41 results on '"Sojak V"'
Search Results
2. Safety and effects of two red blood cell transfusion strategies in pediatric cardiac surgery patients: a randomized controlled trial
- Author
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de Gast-Bakker, D. H., de Wilde, R. B. P., Hazekamp, M. G., Sojak, V., Zwaginga, J. J., Wolterbeek, R., and de Jonge, E.
- Subjects
Congenital heart disease -- Analysis ,Medical research -- Analysis ,Genetic disorders -- Analysis ,Coronary artery bypass -- Analysis ,Medical centers -- Analysis ,Pediatric cardiology -- Analysis ,Cardiac patients -- Analysis ,Surgery -- Analysis ,Medicine, Experimental -- Analysis ,Medical care, Cost of -- Analysis ,Biological products -- Analysis ,Children -- Health aspects ,Hospital patients -- Analysis ,Health care industry - Abstract
Objective To investigate the safety and effects of a restrictive red blood cell (RBC) transfusion strategy in pediatric cardiac surgery patients. Design Randomized controlled trial. Setting Pediatric ICU in an academic tertiary care center, Leiden University Medical Center, Leiden, The Netherlands. Patients One hundred seven patients with non-cyanotic congenital heart defects between 6 weeks and 6 years of age. One hundred three patients underwent corrective surgery on cardiopulmonary bypass. Interventions Prior to surgery patients were randomly assigned to one of two groups with specific RBC transfusion thresholds: Hb 10.8 g/dl (6.8 mmol/l) and Hb 8.0 g/dl (5.0 mmol/l). Measurements Length of stay in hospital (primary outcome), length of stay in PICU, duration of ventilation (secondary outcome), incidence of adverse events and complications related to randomization (intention to treat analysis). Results In the restrictive transfusion group, mean volume of transfused RBC was 186 (±70) ml per patient and in the liberal transfusion group 258 (±87) ml per patient, (95 % CI 40.6-104.6), p < 0.001. Length of hospital stay was shorter in patients with a restrictive RBC transfusion strategy: median 8 (IQR 7-11) vs. 9 (IQR 7-14) days, p = 0.047. All other outcome measures and incidence of adverse effects were equal in both RBC transfusion groups. Cost of blood products for the liberal transfusion group was 438.35 (±203.39) vs. 316.27 (±189.96) euros (95 % CI 46.61-197.51) per patient in the restrictive transfusion group, p = 0.002. Conclusions For patients with a non-cyanotic congenital heart defect undergoing elective cardiac surgery, a restrictive RBC transfusion policy (threshold of Hb 8.0 g/dl) during the entire perioperative period is safe, leads to a shorter hospital stay and is less expensive., Author(s): D. H. de Gast-Bakker [sup.1], R. B. P. de Wilde [sup.1] [sup.6], M. G. Hazekamp [sup.2], V. Sojak [sup.2], J. J. Zwaginga [sup.3] [sup.4], R. Wolterbeek [sup.5], E. de [...]
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- 2013
- Full Text
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3. The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study
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Vida, Vladimiro L, Guariento, Alvise, Milanesi, Ornella, Gregori, Dario, Stellin, Giovanni, Zucchetta F, Zanotto L, Padalino MA, Castaldi B, Bosiznik S, Crepaz R, Stuefer J, de Maria Garcia Gonzales F, Castaneda AR, Crupi G, Agnoletti G, Bondanza S, Marasini M, Zannini L, Butera G, Frigiola A, Varrica A, Chiappa E, Pilati M, Carotti A, Matteo T, Prandstraller D, Gargiulo G, Giovanna Russo M, Santoro G, Caianiello G, Spadoni I, Murzi B, Arcieri L, Pozzi M, Porcedda G, Berggren H, Carrel T, Kadner A, Çiçek S, Zorman Y, Fragata J, Gordo A, Hazekamp M, Sojak V, Hraska V, Asfour B, Maruszewski B, Kozlowski M, Metras D, Pretre R, Rubay J, Sairanen H, Sarris G, Schreiber C, Ono M, Meyns B, Van den Bossche K, Tlaskal T, Lo Rito M, Joon Yoo S, Van Arsdell GS, Calderone C, Iwamoto Y, Leon-Wyss J, Di Filippo S, Leconte C, Mulder BJ, Ebels T, Arrigoni S, Valsangiacomo E, Hitendu D, Konstantinov IE, Gamillscheg A, Gabriela D, Herberg U, Dulac Y, Edmerger J, Zarate Fuentes A, Miguel Gil Jaurena J, Bo I, Ghez O, Rigby ML, Bacha EA, Kalfa D, Speggiorin S, Bu'Lock F, Al-Ahmadi M, Di Salvo G, Surmacz R, Yemets IM, Mykychak YB, Lugones I, Cameron DE, Vricella LA, Troconis CJ, Thiene G, Angelini A, Zanotto L., Vida, Vladimiro L, Guariento, Alvise, Milanesi, Ornella, Gregori, Dario, Stellin, Giovanni, Zucchetta F, Zanotto L, Padalino MA, Castaldi B, Bosiznik S, Crepaz R, Stuefer J, de Maria Garcia Gonzales F, Castaneda AR, Crupi G, Agnoletti G, Bondanza S, Marasini M, Zannini L, Butera G, Frigiola A, Varrica A, Chiappa E, Pilati M, Carotti A, Matteo T, Prandstraller D, Gargiulo G, Giovanna Russo M, Santoro G, Caianiello G, Spadoni I, Murzi B, Arcieri L, Pozzi M, Porcedda G, Berggren H, Carrel T, Kadner A, Çiçek S, Zorman Y, Fragata J, Gordo A, Hazekamp M, Sojak V, Hraska V, Asfour B, Maruszewski B, Kozlowski M, Metras D, Pretre R, Rubay J, Sairanen H, Sarris G, Schreiber C, Ono M, Meyns B, Van den Bossche K, Tlaskal T, Lo Rito M, Joon Yoo S, Van Arsdell GS, Calderone C, Iwamoto Y, Leon-Wyss J, Di Filippo S, Leconte C, Mulder BJ, Ebels T, Arrigoni S, Valsangiacomo E, Hitendu D, Konstantinov IE, Gamillscheg A, Gabriela D, Herberg U, Dulac Y, Edmerger J, Zarate Fuentes A, Miguel Gil Jaurena J, Bo I, Ghez O, Rigby ML, Bacha EA, Kalfa D, Speggiorin S, Bu'Lock F, Al-Ahmadi M, Di Salvo G, Surmacz R, Yemets IM, Mykychak YB, Lugones I, Cameron DE, Vricella LA, Troconis CJ, Thiene G, Angelini A, Zanotto L., Cardiology, APH - Personalized Medicine, APH - Aging & Later Life, ACS - Heart failure & arrhythmias, Cardiothoracic Surgery, Zucchetta, F, Zanotto, L, Padalino, Ma, Castaldi, B, Bosiznik, S, Crepaz, R, Stuefer, J, De Maria Garcia Gonzales, F, Castaneda, Ar, Crupi, G, Agnoletti, G, Bondanza, S, Marasini, M, Zannini, LUIGI PIERO, Butera, G, Frigiola, A, Varrica, A, Chiappa, E, Pilati, M, Carotti, A, Matteo, T, Prandstraller, D, Gargiulo, G, Giovanna Russo, M, Santoro, G, Caianiello, G, Spadoni, I, Murzi, B, Arcieri, L, Pozzi, M, Porcedda, G, Berggren, H, Carrel, T, Kadner, A, Çiçek, S, Zorman, Y, Fragata, J, Gordo, A, Hazekamp, M, Sojak, V, Hraska, V, Asfour, B, Maruszewski, B, Kozlowski, M, Metras, D, Pretre, R, Rubay, J, Sairanen, H, Sarris, G, Schreiber, C, Ono, M, Meyns, B, Van Den Bossche, K, Tlaskal, T, Lo Rito, M, Joon Yoo, S, Van Arsdell, G, Calderone, C, Iwamoto, Y, Leon-wyss, J, Di Filippo, S, Leconte, C, Mulder, Bj, Ebels, T, Arrigoni, S, Valsangiacomo, E, Hitendu, D, Konstantinov, Ie, Gamillscheg, A, Gabriela, D, Herberg, U, Dulac, Y, Edmerger, J, Zarate Fuentes, A, Miguel Gil Jaurena, J, Bo, I, Ghez, O, Rigby, Ml, Bacha, Ea, Kalfa, D, Speggiorin, S, Bu'Lock, F, Al-ahmadi, M, Di Salvo, G, Surmacz, R, Yemets, Im, Mykychak, Yb, Lugones, I, Cameron, De, Vricella, La, Troconis, Cj, Thiene, G, Angelini, A, Zanotto, L., and University of Zurich
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Male ,Pediatrics ,medicine.medical_specialty ,Time Factors ,Natural history ,610 Medicine & health ,Congenital heart defect ,Multi-centre study ,Surgery ,030204 cardiovascular system & hematology ,Asymptomatic ,2705 Cardiology and Cardiovascular Medicine ,03 medical and health sciences ,0302 clinical medicine ,Interquartile range ,Scimitar syndrome ,medicine ,Humans ,Registries ,Cardiac Surgical Procedures ,Retrospective Studies ,business.industry ,Incidence (epidemiology) ,Incidence ,Scimitar Syndrome ,Infant, Newborn ,Infant ,Retrospective cohort study ,Odds ratio ,medicine.disease ,Pulmonary hypertension ,Echocardiography, Doppler ,Europe ,Survival Rate ,Stenosis ,Treatment Outcome ,030228 respiratory system ,10036 Medical Clinic ,Pulmonary Veins ,Child, Preschool ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P
- Published
- 2018
4. P0519 / #2100: EFFECTIVE TREATMENT OF HYPERTENSION WITH LABETALOL FOLLOWING AORTIC COARCTATION REPAIR.
- Author
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Siersma, C., primary, Brouwer, C., additional, Sojak, V., additional, and Roeleveld, P.P., additional
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- 2021
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5. Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study
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Protopapas, E.M., Anderson, R.H., Backer, C.L., Fragata, J., Hakim, N., Vida, V.L., Sarris, G.E., Barron, D.J., Berggren, H., Hazekamp, M.G., Ilyin, V., Kornoukhov, O.J., Kostolny, M., Lazarov, S., Rito, M. lo, Monge, M.C., Mykychak, Y., Nosal, M., Pretre, R., Polimenakos, A.C., Sojak, V., Stellin, G., Veshti, A., Yemets, I., and ECHSA WSPCHS Study Grp
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Male ,Time Factors ,medicine.medical_treatment ,030204 cardiovascular system & hematology ,Ventricular Function, Left ,0302 clinical medicine ,Aortic valve repair ,Postoperative Complications ,Child ,Aorta ,Surgical treatment ,General Medicine ,Aorto-ventricular tunnel ,Aortic orifice ,Aortic valvuloplasty ,Europe ,medicine.anatomical_structure ,Treatment Outcome ,Aortic valve stenosis ,Right coronary artery ,Aortic Valve ,Child, Preschool ,cardiovascular system ,Female ,Cardiology and Cardiovascular Medicine ,Pulmonary and Respiratory Medicine ,Adult ,Heart Defects, Congenital ,medicine.medical_specialty ,Adolescent ,Heart Ventricles ,03 medical and health sciences ,Young Adult ,medicine.artery ,Ascending aorta ,medicine ,Humans ,Cardiac Surgical Procedures ,Coronary sinus ,Retrospective Studies ,business.industry ,Hemodynamics ,Infant, Newborn ,Infant ,Recovery of Function ,medicine.disease ,United States ,Surgery ,030228 respiratory system ,business - Abstract
Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day–25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month–12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results.
- Published
- 2020
6. Dealing with kinked and swirled pulmonary vessels: surgical treatment of arterial tortuosity syndrome: a case report
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Ricciardi, G., primary, Sojak, V., additional, and Hazekamp, M. G., additional
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- 2020
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7. 031 * A SINGLE-CENTRE 37-YEAR EXPERIENCE WITH REOPERATION FOR ATRIOVENTRICULAR SEPTAL DEFECT
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Sojak, V., primary, Koolbergen, D. R., additional, Bruggemans, E., additional, Yazdanbakhsh, A., additional, Kooij, M., additional, and Hazekamp, M., additional
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- 2014
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8. Surgery for truncus arteriosus
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Sojak, V., primary, Lugo, J., additional, Koolbergen, D., additional, and Hazekamp, M., additional
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- 2012
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9. Mid-term results of bidirectional cavopulmonary anastomosis and hemi-Mustard procedure in anatomical correction of congenitally corrected transposition of the great arteries
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Sojak, V., primary, Kuipers, I., additional, Koolbergen, D., additional, Rijlaarsdam, M., additional, Hruda, J., additional, Blom, N., additional, and Hazekamp, M., additional
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- 2012
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10. Safety and effects of two red blood cell transfusion strategies in pediatric cardiac surgery patients: a randomized controlled trial.
- Author
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Gast-Bakker, D., Wilde, R., Hazekamp, M., Sojak, V., Zwaginga, J., Wolterbeek, R., Jonge, E., and Gesink-van der Veer, B.
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RED blood cell transfusion ,BLOOD transfusion ,PEDIATRIC cardiology ,PEDIATRIC surgery ,CARDIAC surgery patients ,RANDOMIZED controlled trials - Abstract
Objective: To investigate the safety and effects of a restrictive red blood cell (RBC) transfusion strategy in pediatric cardiac surgery patients. Design: Randomized controlled trial. Setting: Pediatric ICU in an academic tertiary care center, Leiden University Medical Center, Leiden, The Netherlands. Patients: One hundred seven patients with non-cyanotic congenital heart defects between 6 weeks and 6 years of age. One hundred three patients underwent corrective surgery on cardiopulmonary bypass. Interventions: Prior to surgery patients were randomly assigned to one of two groups with specific RBC transfusion thresholds: Hb 10.8 g/dl (6.8 mmol/l) and Hb 8.0 g/dl (5.0 mmol/l). Measurements: Length of stay in hospital (primary outcome), length of stay in PICU, duration of ventilation (secondary outcome), incidence of adverse events and complications related to randomization (intention to treat analysis). Results: In the restrictive transfusion group, mean volume of transfused RBC was 186 (±70) ml per patient and in the liberal transfusion group 258 (±87) ml per patient, (95 % CI 40.6-104.6), p < 0.001. Length of hospital stay was shorter in patients with a restrictive RBC transfusion strategy: median 8 (IQR 7-11) vs. 9 (IQR 7-14) days, p = 0.047. All other outcome measures and incidence of adverse effects were equal in both RBC transfusion groups. Cost of blood products for the liberal transfusion group was 438.35 (±203.39) vs. 316.27 (±189.96) euros (95 % CI 46.61-197.51) per patient in the restrictive transfusion group, p = 0.002. Conclusions: For patients with a non-cyanotic congenital heart defect undergoing elective cardiac surgery, a restrictive RBC transfusion policy (threshold of Hb 8.0 g/dl) during the entire perioperative period is safe, leads to a shorter hospital stay and is less expensive. [ABSTRACT FROM AUTHOR]
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- 2013
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11. Histidine production by a regulatory mutant of Streptomyces coelicolor
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Derkos-Sojak, V, primary, Korajlija, J, additional, and Delíc, V, additional
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- 1977
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12. 20-year follow-up and comparison of homografts with bovine jugular vein conduits in children less than 2 years of age.
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Sabateen F, Nosal M, and Sojak V
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- Humans, Child, Animals, Cattle, Infant, Infant, Newborn, Retrospective Studies, Follow-Up Studies, Jugular Veins transplantation, Treatment Outcome, Allografts, Reoperation, Bioprosthesis, Ventricular Outflow Obstruction surgery
- Abstract
Objectives: Repair of congenital heart defects involving right ventricular outflow tract (RVOT) may require pulmonary valve replacement at time of primary repair or reoperation. This study compares the outcomes of cryopreserved homografts with bovine jugular vein conduits (BJVC) in children < 2 years of age with RVOT reconstruction., Methods: Retrospective, single-center review of 70 conduits implanted in 63 patients undergoing right ventricle-to-pulmonary artery reconstruction with valved conduit from 2002 to 2022., Results: A total of 70 conduits were implanted in 63 patients, with mean age of 4.5 ± 6.9 months (range 1 day ‒ 23.5 months). The following conduits were used; homografts 38 (54.2 %), BJVC 32 (45.8 %). During mean follow-up of 6.2 ± 5.6 years, there were 12 deaths, 24 conduit reoperations, and 25 catheter reinterventions. Overall survival, reoperation-free, and catheter reintervention-free survival at 15 years was 82.7 %, 31.2 %, and 25.7 %, respectively. Multivariate analysis revealed that low patient weight, age < 30 days at repair, ventilation time, and ICU length of stay were associated with increased risk of death., Conclusion: The performance of homografts and BJVC is comparable in patients ent between the two groups (Tab. 3, Fig. 3, Ref. 16).
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- 2023
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13. Treatment of Post-Coarctectomy Hypertension With Labetalol-A 9-Year Single-Center Experience.
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Siersma C, Brouwer CNM, Sojak V, Ten Harkel ADJ, and Roeleveld PP
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- Child, Humans, Nitroprusside pharmacology, Nitroprusside therapeutic use, Retrospective Studies, Postoperative Complications drug therapy, Antihypertensive Agents therapeutic use, Blood Pressure, Labetalol pharmacology, Labetalol therapeutic use, Hypertension drug therapy, Hypertension etiology
- Abstract
Background: Although considering the pathophysiology of post-coarctectomy hypertension, β-blockers should be effective, experience with labetalol for treatment is limited in the literature., Methods: Retrospective collection and analysis of data in children aged ≤6 years following coarctectomy in our tertiary care university medical center between January 2009 and June 2018., Results: 96 patients were included, 45 were treated with intravenous labetalol and 51 received no treatment. Median time to maximum dose received (median 1.1 mg/kg/h) was 2.7 h, and median time to the reduction of labetalol dose was 8.3 h. No antihypertensives had to be added. In one child, labetalol was switched to nitroprusside due to bronchoconstriction. Of patients receiving intravenous labetalol, 48% had been switched to oral labetalol at discharge., Conclusions: Intravenous labetalol is a fast, effective, and safe drug to treat hypertension following aortic coarctation repair. Labetalol is easily converted to oral therapy when the continuation of treatment is considered necessary.
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- 2022
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14. Left heart growth and biventricular repair after hybrid palliation.
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Sojak V, Bokenkamp R, Kuipers I, Schneider A, and Hazekamp M
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- Aortic Valve diagnostic imaging, Aortic Valve surgery, Heart Septal Defects, Ventricular diagnostic imaging, Heart Septal Defects, Ventricular surgery, Heart Ventricles diagnostic imaging, Heart Ventricles surgery, Humans, Hypoplastic Left Heart Syndrome, Infant, Treatment Outcome, Palliative Care
- Abstract
Objectives: We evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia., Methods: Between September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross-Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair., Results: Twenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair., Conclusions: Hybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2021
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15. We can, but should we?
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Hazekamp MG and Sojak V
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- Humans, Pulmonary Valve, Tetralogy of Fallot
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- 2021
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16. Surgical Management of Aorto-Ventricular Tunnel. A Multicenter Study.
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Protopapas EM, Anderson RH, Backer CL, Fragata J, Hakim N, Vida VL, Sarris GE, Barron DJ, Berggren H, Hazekamp MG, Ilyin V, Kornoukhov OJ, Kostolny M, Lazarov S, Lo Rito M, Monge MC, Mykychak Y, Nosal M, Prêtre R, Polimenakos AC, Sojak V, Stellin G, Veshti A, and Yemets I
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- Adolescent, Adult, Aorta abnormalities, Aorta diagnostic imaging, Aorta physiopathology, Aortic Valve diagnostic imaging, Aortic Valve physiopathology, Child, Child, Preschool, Europe, Female, Heart Defects, Congenital diagnostic imaging, Heart Defects, Congenital mortality, Heart Defects, Congenital physiopathology, Heart Ventricles abnormalities, Heart Ventricles diagnostic imaging, Heart Ventricles physiopathology, Hemodynamics, Humans, Infant, Infant, Newborn, Male, Postoperative Complications mortality, Postoperative Complications surgery, Recovery of Function, Retrospective Studies, Time Factors, Treatment Outcome, United States, Ventricular Function, Left, Young Adult, Aorta surgery, Aortic Valve surgery, Cardiac Surgical Procedures adverse effects, Cardiac Surgical Procedures mortality, Heart Defects, Congenital surgery, Heart Ventricles surgery
- Abstract
Aorto-ventricular tunnel (AoVT), a rare congenital anomaly, is a channel originating in the ascending aorta just above the sinotubular junction and leading to the cavity of the left ventricle (AoLVT), or, rarely, the right (AoRVT). This study reviews our collective 30-year experience with the surgical treatment of AoVT. Data were submitted by 15 participating centers on 42 patients who underwent correction of AoVT between 1987 and 2018. Of these, 36 had AoLVT, and 6 AoRVT. The tunnel originated above the right coronary sinus in 28 (77.8%) patients. For AoLVT, most operations were performed early (median age 25 days, range: 1 day-25 years). In contrast, AoRVT was diagnosed and repaired later (median age 6 years, range: 1 month-12 years). Surgically important coronary ostial displacement was common. Patch closure of the aortic orifice only was the commonest surgical repair for AoLVT (23 patients), while in AoRVT, both orifices or only the ventricular one was closed. Aortic valvar insufficiency, severe or moderate, coexisted in 11 (30.5%) patients with AoLVT, and aortic valvuloplasty was performed in 8, mainly due to aortic valve stenosis. Aortic valvar insufficiency at discharge ranged from trivial to mild in almost all patients. Early mortality was 7.14%, with 3 patients with AoLVT succumbing to cardiac failure. There were 2 early reoperations and 1 late death. AoVT is a rare malformation. AoLVT usually necessitates surgery in early life. AoRVT is rarer, diagnosed and repaired later in life. Surgical repair by patch closure, with concomitant aortic valve repair as needed, is associated with good results., (Copyright © 2020 Elsevier Inc. All rights reserved.)
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- 2020
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17. Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.
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Padalino MA, Franchetti N, Hazekamp M, Sojak V, Carrel T, Frigiola A, Lo Rito M, Horer J, Roussin R, Cleuziou J, Meyns B, Fragata J, Telles H, Polimenakos AC, Francois K, Veshti A, Salminen J, Rocafort AG, Nosal M, Vedovelli L, Guariento A, Vida VL, Sarris GE, Boccuzzo G, and Stellin G
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- Adolescent, Adult, Cardiology, Europe, Female, Humans, Male, Middle Aged, Retrospective Studies, Societies, Medical, Time Factors, Treatment Outcome, Vascular Surgical Procedures methods, Young Adult, Abnormalities, Multiple surgery, Aorta, Thoracic abnormalities, Aorta, Thoracic surgery, Coronary Vessel Anomalies surgery
- Abstract
Objectives: We sought to describe early and late outcomes in a large surgical series of patients with anomalous aortic origin of coronary arteries., Methods: We performed a retrospective multicentre study including surgical patients with anomalous aortic origin of coronary arteries since 1991. Patients with isolated high coronary takeoff and associated major congenital heart disease were excluded., Results: We collected 156 surgical patients (median age 39.5 years, interquartile range 15-53) affected by anomalous right (67.9%), anomalous left (22.4%) and other anatomical abnormalities (9.6%). An interarterial course occurred in 86.5%, an intramural course in 62.8% and symptoms in 85.9%. The operations included coronary unroofing (56.4%), reimplantation (19.2%), coronary bypass graft (15.4%) and other (9.0%). Two patients with preoperative cardiac failure died postoperatively (1.3%). All survivors were discharged home in good clinical condition. At a median follow-up of 2 years (interquartile range 1-5, 88.5% complete), there were 3 deaths (2.2%), 9 reinterventions in 8 patients (5 interventional, 3 surgical); 91.2% are in New York Heart Association functional class ≤ II, but symptoms persisted in 14.2%; 48.1% of them returned to sport activity. On Kaplan-Meier analysis, event-free survival at follow-up was 74.6%. Morbidity was not significantly different among age classes, anatomical variants and types of surgical procedures. Furthermore, return to sport activity was significantly higher in younger patients who participated in sports preoperatively., Conclusions: Surgical repair of anomalous aortic origin of coronary arteries is effective and has few complications. Unroofing and coronary reimplantation are safe and are the most common procedures. The occurrence of late adverse events is not negligible, and long-term surveillance is mandatory. Most young athletes can return to an unrestrained lifestyle., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2019
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18. Anomalous aortic origin of coronary arteries: Early results on clinical management from an international multicenter study.
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Padalino MA, Franchetti N, Sarris GE, Hazekamp M, Carrel T, Frigiola A, Horer J, Roussin R, Cleuziou J, Meyns B, Fragata J, Telles H, Polimenakos AC, Francois K, Veshti A, Salminen J, Rocafort AG, Nosal M, Vedovelli L, Protopapas E, Tumbarello R, Merola A, Pegoraro C, Motta R, Boccuzzo G, Sojak V, Rito ML, Caldaroni F, Corrado D, Basso C, and Stellin G
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- Adolescent, Adult, Child, Female, Follow-Up Studies, Humans, Longitudinal Studies, Male, Middle Aged, Retrospective Studies, Young Adult, Coronary Vessel Anomalies diagnosis, Coronary Vessel Anomalies therapy, Disease Management, Internationality
- Abstract
Background: Anomalous aortic origin of coronary arteries (AAOCA) is a rare abnormality, whose optimal management is still undefined. We describe early outcomes in patients treated with different management strategies., Methods: This is a retrospective clinical multicenter study including patients with AAOCA, undergoing or not surgical treatment. Patients with isolated high coronary take off and associated major congenital heart disease were excluded. Preoperative, intraoperative, anatomical and postoperative data were retrieved from a common database., Results: Among 217 patients, 156 underwent Surgical repair (median age 39 years, IQR: 15-53), while 61 were Medical (median age 15 years, IQR: 8-52), in whom AAOCA was incidentally diagnosed during screening or clinical evaluations. Surgical patients were more often symptomatic when compared to medical ones (87.2% vs 44.3%, p < 0.001). Coronary unroofing was the most frequent procedure (56.4%). Operative mortality was 1.3% (2 patients with preoperative severe heart failure). At a median follow up of 18 months (range 0.1-23 years), 89.9% of survivors are in NYHA ≤ II, while only 3 elderly surgical patients died late. Return to sport activity was significantly higher in Surgical patients (48.1% vs 18.2%, p < 0.001)., Conclusions: Surgery for AAOCA is safe and with low morbidity. When compared to Medical patients, who remain on exercise restriction and medical therapy, surgical patients have a benefit in terms of symptoms and return to normal life. Since the long term-risk of sudden cardiac death is still unknown, we currently recommend accurate long term surveillance in all patients with AAOCA., (Copyright © 2019 The Authors. Published by Elsevier B.V. All rights reserved.)
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- 2019
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19. A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome.
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Rijnberg FM, Blom NA, Sojak V, Bruggemans EF, Kuipers IM, Rammeloo LAJ, Jongbloed MRM, Bouma BJ, and Hazekamp MG
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- Adolescent, Adult, Child, Child, Preschool, Female, Follow-Up Studies, Heart Defects, Congenital mortality, Humans, Male, Morbidity trends, Postoperative Complications epidemiology, Retrospective Studies, Risk Factors, Survival Rate trends, Tachycardia epidemiology, Treatment Outcome, Fontan Procedure adverse effects, Forecasting, Heart Defects, Congenital surgery, Postoperative Complications etiology, Tachycardia etiology
- Abstract
Objectives: This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity., Methods: Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016., Results: The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1-Q3 7.6-17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18-44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63-96%) at 20 years for lateral tunnel and 99% (95% CI 96-100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III-IV) at 15 years was 65% (95% CI 52-76%) for atriopulmonary connection/Björk, 90% (95% CI 73-97%) for lateral tunnel and 90% (95% CI 82-94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2-5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4-9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4-9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0-11.1; P < 0.001)., Conclusions: With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2019
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20. Biventricular repair after the hybrid Norwood procedure.
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Sojak V, Bokenkamp R, Kuipers I, Schneider A, and Hazekamp M
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- Humans, Infant, Newborn, Reoperation statistics & numerical data, Retrospective Studies, Ventricular Outflow Obstruction mortality, Ventricular Outflow Obstruction surgery, Heart Defects, Congenital mortality, Heart Defects, Congenital surgery, Heart Ventricles surgery, Norwood Procedures methods, Norwood Procedures mortality
- Abstract
Objectives: We analysed the outcomes of patients undergoing biventricular repair (BVR) after an initial hybrid Norwood approach as a salvage procedure in extremely sick infants; or as the initial palliation in patients with uncertain feasibility of single-stage BVR due to severe left ventricular outflow tract obstruction; or as part of a left ventricle (LV) recruitment strategy in patients with borderline LVs., Methods: Between September 2010 and July 2018, 26 patients underwent BVR after initial hybrid palliation at a median age of 13 days. The rationale for the hybrid approach was to promote the growth of the LV in 10 patients and that of the left ventricular outflow tract and/or aortic valve in 12 patients and to be a salvage procedure in 4 patients. Significant growth of the LV was noted during the interstage period, which had a median length of 65 days (P = 0.008). Fourteen patients underwent aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients underwent the Yasui procedure; 4 patients had the Ross-Konno procedure; 2 patients had an arterial switch operation; and 1 patient had truncus arteriosus repair., Results: Twenty-two patients (84.6%) are alive at a median follow-up period of 1.8 (range 0.04-6.2) years. There were 2 early and 2 late deaths. Nineteen catheter-based reinterventions and 15 reoperations were performed after BVR., Conclusions: The hybrid Norwood procedure permits stabilization of critical infants. It allows for growth of left ventricular structures in some patients with borderline left hearts and in those with severe left ventricular outflow tract obstruction. More patients may eventually have BVR than was thought during the newborn period., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2019
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21. Aortic coarctation repair through left thoracotomy: results in the modern era.
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Farag ES, Kluin J, de Heer F, Ahmed Y, Sojak V, Koolbergen DR, Blom NA, de Mol BAJM, Ten Harkel ADJ, and Hazekamp MG
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- Child, Preschool, Female, Humans, Hypertension, Infant, Infant, Newborn, Kaplan-Meier Estimate, Male, Postoperative Complications, Reoperation adverse effects, Reoperation mortality, Retrospective Studies, Treatment Outcome, Aortic Coarctation mortality, Aortic Coarctation surgery, Thoracotomy adverse effects, Thoracotomy mortality
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Objectives: Surgical repair of coarctation of the aorta (CoA) is often possible through left thoracotomy and without the use of cardiopulmonary bypass. Recent studies reporting the outcome after CoA repair through left thoracotomy are limited. Therefore, the aim of this study is to evaluate the results of CoA repair through left thoracotomy in children who were operated on in our centre over the past 21 years., Methods: From January 1995 to December 2016, 292 patients younger than 18 years underwent primary CoA repair through left thoracotomy at our 2 institutions. Peri- and postoperative data and follow-up data collected from our hospital and the referring hospitals were retrospectively reviewed., Results: Median age at operation was 64 days (range 2 days-17 years). Most patients underwent the resection of the CoA followed by an (extended) end-to-end anastomosis (93%). Six patients died perioperatively and 2 more patients died during the follow-up, of which 7 patients had other major comorbidities. Actuarial survival was 97% at 5 years, 96% at 10 years and 96% at 15 years. Second arch interventions due to recoarctation were performed in 9.9% (n = 29) of patients, consisting of balloon dilatation in all but 2 patients. Recoarctation occurred significantly more often after initial repair in the neonatal period (21%) and could occur as late as 14 years after initial surgery. There were 7 re-recoarctations, and 14% of patients were on hypertensive medication during the follow-up., Conclusions: Repair of CoA through left thoracotomy is a safe procedure with low rates of mortality. The long-term follow-up is necessary due to the significant risk of recoarctation requiring reintervention.
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- 2019
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22. Abnormal blood flow and wall shear stress are present in corrected aortic coarctation despite successful surgical repair.
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Farag ES, van Ooij P, Boekholdt SM, Planken RN, Dukker KC, Bouma BJ, Groenink M, Koolbergen DR, Sojak V, Nederveen AJ, Hazekamp MG, de Mol BA, and Kluin J
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- Aorta diagnostic imaging, Aortic Coarctation diagnostic imaging, Aortic Coarctation surgery, Humans, Magnetic Resonance Imaging, Stress, Mechanical, Aorta physiopathology, Aortic Coarctation physiopathology, Hemorheology
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- 2019
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23. Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients.
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Rijnberg FM, Sojak V, Blom NA, and Hazekamp MG
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- Child, Preschool, Female, Follow-Up Studies, Heart Ventricles surgery, Humans, Infant, Infant, Newborn, Male, Treatment Outcome, Aortic Stenosis, Subvalvular surgery, Cardiac Surgical Procedures methods, Forecasting, Heart Defects, Congenital surgery, Heart Ventricles abnormalities
- Abstract
Background: Single ventricle patients with unrestrictive pulmonary blood flow and (potential) subaortic stenosis are challenging to manage and optimal surgical strategy is unknown. Direct relief of subaortic stenosis by enlargement of the ventricular septal defect and/or subaortic chamber has generally been replaced by a Damus-Kaye-Stansel or Norwood procedure due to concerns of iatrogenic heart block, reobstruction, or ventricular dysfunction. Studies reporting long-term outcome after the direct approach are limited. The aim of our study was to describe and analyze our experience with direct relief of subaortic stenosis in single ventricle patients., Methods: Demographic data, characteristics, and pre-operative, operative and outcome details were collected for children undergoing direct relief of subaortic stenosis between 1989 and 2016., Results: Twenty-three patients (median age: 7.4 months, range: 10 days to 5.5 years) underwent direct relief of subaortic stenosis. Complete follow-up was available for all patients (median: 15.6 years, range: 34 days to 26.3 years). Seven (30%) patients had recurrence of subaortic stenosis. One (4%) patient developed complete heart block and one patient developed moderate ventricular dysfunction. Five (50%) patients developed a (pseudo)aneurysm at site of the patch and ventriculotomy. There were two perioperative deaths. Eighty-six percent of patients underwent a successful Fontan procedure., Conclusions: Direct relief of subaortic stenosis is associated with a substantial risk of reobstruction and patch (pseudo)aneurysm formation. However, risk of heart block is low and long-term outcome is good with the majority of patients reaching Fontan completion. In our opinion, the direct approach appears to be a good and relatively simple procedure in selected cases for the treatment of subaortic stenosis.
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- 2018
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24. Nikaidoh vs Réparation à l'Etage Ventriculaire vs Rastelli.
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Hazekamp MG, Nevvazhay T, and Sojak V
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- Humans, Arterial Switch Operation, Heart Septal Defects, Ventricular surgery, Transposition of Great Vessels surgery, Ventricular Outflow Obstruction surgery
- Abstract
This review describes the different surgical options for transposition of the great arteries, ventricular septal defect (VSD), and left ventricular outflow tract obstruction. When the pulmonary valve can be used, an arterial switch operation with VSD closure and resection of pulmonary stenosis may be possible. This is not the scope of our review: we focus on the Rastelli, REV (Réparation à l'Etage Ventriculaire), and Nikaidoh techniques, and we also describe the "en bloc rotation" technique. Each of these procedures has a different history, and these techniques have not been used uniformly around the world. We describe the advantages and disadvantages of each technique together with their outcomes as reported in the literature. Some forms of transposition of the great arteries, VSD, and left ventricular outflow tract obstruction can only be corrected by the Nikaidoh operation, although this operation can be definitely contraindicated in other instances. Surgical eras and length of follow-up are not the same for all procedures, and there has been surgical bias in choosing 1 technique over another. This makes comparison between techniques difficult, although certain trends are observed., (Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.)
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- 2018
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25. Fifteen years' experience with the use of artificial chords for valve reconstruction in children.
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Kluin J, Sojak V, Koolbergen DR, Boon R, Bökenkamp R, and Hazekamp MG
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- Adolescent, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Mitral Valve Insufficiency diagnosis, Polytetrafluoroethylene, Retrospective Studies, Treatment Outcome, Tricuspid Valve diagnostic imaging, Tricuspid Valve Insufficiency diagnosis, Balloon Valvuloplasty methods, Chordae Tendineae, Forecasting, Mitral Valve surgery, Mitral Valve Insufficiency surgery, Tricuspid Valve surgery, Tricuspid Valve Insufficiency surgery
- Abstract
Objectives: To retrospectively review our experience with artificial chords in mitral and tricuspid reconstructive surgery in children., Methods: All consecutive paediatric (<18 years) patients who underwent mitral or tricuspid valve reconstruction with the use of artificial chords in our centre in the past 15 years were retrospectively analysed., Results: Thirty-nine patients (age 3 days to 17 years) underwent reconstruction of the mitral (n = 27) or tricuspid (n = 12) valve using artificial chords. Mean number of chords was 3.5 ± 1.7. In 26 of 27 mitral valve patients, chords were placed on the anterior leaflet, in one on the posterior leaflet. In 10 of the 12 tricupid valve, patients chords were placed on the anterior leaflet and in 2 on the septal leaflet. All mitral patients underwent annuloplasty (10 bilateral Wooler-Kay and 17 rigid ring annuloplasty). Ten of the 12 tricuspid patients underwent annuloplasty (1 rigid ring and 9 commissural plication). Follow-up was after 8.7 ± 5.5 years. There was no early or late mortality. The actuarial freedom from reoperation rates at 1, 5 and 10 years were 95%, 91% and 81%, respectively. No reoperations occurred in the tricuspid group. In the mitral group, there were 2 early failures and 3 late reoperations due to mitral stenosis. Restricted leaflet motion probably caused by the artificial chords was seen in only 1 patient., Conclusions: Our data show that long-term durability of mitral and tricuspid valve reconstruction using artificial chords in children is good. Despite patient growth, restricted leaflet motion by the artificial chords does not seem to form a major problem., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2017
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26. Early and late outcomes after surgical repair of congenital supravalvular aortic stenosis: a European Congenital Heart Surgeons Association multicentric study.
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Padalino MA, Frigo AC, Comisso M, Kostolny M, Omeje I, Schreiber C, Pabst von Ohain J, Cleuziou J, Barron DJ, Meyns B, Hraska V, Maruszewski B, Kozlowski M, Vricella LA, Hibino N, Collica S, Berggren H, Synnergren M, Lazarov S, Kalfa D, Bacha E, Pizarro C, Hazekamp M, Sojak V, Jacobs JP, Nosal M, Fragata J, Cicek S, Sarris GE, Zografos P, Vida VL, and Stellin G
- Subjects
- Adolescent, Aortic Stenosis, Supravalvular mortality, Child, Child, Preschool, Europe epidemiology, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Prevalence, Retrospective Studies, Survival Rate trends, Aortic Stenosis, Supravalvular surgery, Cardiac Surgical Procedures methods, Cardiology, Forecasting, Postoperative Complications epidemiology, Societies, Medical
- Abstract
Objectives: Our goal was to evaluate the early and late results of the surgical management of congenital supravalvular aortic stenosis (SVAS)., Methods: We performed a retrospective, multicentre study using data from the European Congenital Heart Surgeons Association. Exclusion criteria were age >18 years, operation before 1990 and redo supravalvular aortic stenosis operations. Multivariate Cox regression analysis was performed to detect independent predictors of adverse events., Results: Of a total of 301 patients (male/female = 194/107; median age 3.9 years, range 13 days-17.9 years), 17.6% had a prior surgical or interventional procedure. Pulmonary artery stenosis was present in 41.5% and coronary anomalies in 13.6%. The operation consisted of a single patch repair in 36.7%, a pantaloon-shaped patch in 36.7%, a 3-patch technique in 14.3% and other techniques in 11.7%. Postoperative complications occurred in 14.9%, and the early mortality rate was 5%. At a median follow-up of 13 years (interquartile range 3.5-7.8; follow-up completed 79.1%), there were 10 late deaths (4.2%). A surgical reoperation or an interventional cardiology procedure occurred in 12.6% and 7.2%, respectively. No significant differences in outcomes between the techniques were found. Age at repair <12 months and pulmonary artery stenosis were associated with an increased risk of early (P = 0.0001) and overall mortality (P = 0.025), respectively. Having an operation after 2005 and co-existing pulmonary artery stenosis were significant predictors of late reintervention (P = 0.0110 and P = 0.001, respectively)., Conclusions: Surgical repair of congenital stenosis is an effective procedure with acceptable surgical risk and good late survival, but late morbidity is not negligible, especially in infants and when associated pulmonary artery stenosis is present., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
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- 2017
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27. Left-Sided Reoperations After Arterial Switch Operation: A European Multicenter Study.
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Vida VL, Zanotto L, Zanotto L, Stellin G, Padalino M, Sarris G, Protopapas E, Prospero C, Pizarro C, Woodford E, Tlaskal T, Berggren H, Kostolny M, Omeje I, Asfour B, Kadner A, Carrel T, Schoof PH, Nosal M, Fragata J, Kozłowski M, Maruszewski B, Vricella LA, Cameron DE, Sojak V, Hazekamp M, Salminen J, Mattila IP, Cleuziou J, Myers PO, and Hraska V
- Subjects
- Adolescent, Aortic Valve Insufficiency epidemiology, Aortic Valve Insufficiency etiology, Child, Child, Preschool, Europe epidemiology, Female, Follow-Up Studies, Humans, Incidence, Infant, Male, Postoperative Complications epidemiology, Postoperative Complications etiology, Prognosis, Retrospective Studies, Risk Factors, Survival Rate trends, Aortic Valve Insufficiency surgery, Arterial Switch Operation adverse effects, Double Outlet Right Ventricle surgery, Postoperative Complications surgery, Reoperation methods, Transposition of Great Vessels surgery
- Abstract
Background: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type., Methods: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%])., Results: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%])., Conclusions: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)
- Published
- 2017
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28. Valve-sparing root replacement in children.
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Kluin J, Koolbergen DR, Sojak V, and Hazekamp MG
- Subjects
- Adolescent, Aorta diagnostic imaging, Aortic Valve diagnostic imaging, Aortic Valve Insufficiency diagnostic imaging, Blood Vessel Prosthesis, Child, Child, Preschool, Echocardiography, Female, Follow-Up Studies, Heart Defects, Congenital diagnostic imaging, Heart Defects, Congenital surgery, Humans, Infant, Male, Marfan Syndrome diagnostic imaging, Marfan Syndrome surgery, Prosthesis Design, Recurrence, Retrospective Studies, Treatment Outcome, Aorta surgery, Aortic Valve surgery, Aortic Valve Insufficiency surgery, Blood Vessel Prosthesis Implantation methods
- Abstract
Objectives: In children, words of caution have been raised about valve-sparing procedures especially regarding the valve-remodelling technique. This study reviewed our experience with the valve-sparing reimplantation technique in children., Methods: All consecutive paediatric (<18 years) patients who underwent valve-sparing root replacement in our centre in the past 12.5 years were retrospectively analysed., Results: Nineteen patients (median age 13.2 years, 10 months to 17.9 years) underwent valve-sparing root replacement using the reimplantation technique. Seventeen had connective tissue disease. An adult-sized vascular prosthesis could be implanted in all cases. Additional cusp repair was required in 3 patients. Follow-up was 4.4 ± 3.8 years. There was no perioperative mortality and one late death. Of the 3 patients that needed cusp repair, 1 developed Grade 3 aortic valve regurgitation (AR) and required aortic valve replacement and 2 had Grade 1 AR. Ninety-four percent of the other patients had Grade 0 AR at latest follow-up, 1 patient (6%) had Grade 1 AR., Conclusions: Our data show that valve-sparing root surgery using the reimplantation technique can be performed safely in children. Mid-term follow-up yields stable and favourable results. When leaflet reconstruction is necessary on top of the reimplantation procedure, rate of recurrent AR seems to be higher., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
- Published
- 2016
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29. Reoperation for right ventricular outflow tract obstruction after arterial switch operation for transposition of the great arteries and aortic arch obstruction.
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Bokenkamp R, Aguilar E, van der Palen RL, Sojak V, Bruggemans EF, Hruda J, Kuipers IM, and Hazekamp MG
- Subjects
- Child, Child, Preschool, Female, Follow-Up Studies, Humans, Infant, Infant, Newborn, Male, Retrospective Studies, Aortic Diseases surgery, Arterial Switch Operation adverse effects, Reoperation mortality, Transposition of Great Vessels surgery, Ventricular Outflow Obstruction mortality, Ventricular Outflow Obstruction surgery
- Abstract
Objectives: Right ventricular outflow tract obstruction (RVOTO) is one of the reasons for late reinterventions after repair of transposition of the great arteries (TGA) with aortic arch obstruction (AAO). The aim of the present study was to identify predictors of reoperation for RVOTO in patients who underwent arterial switch operation (ASO) and arch repair for TGA or Taussig-Bing anomaly with AAO., Methods: Between 1977 and 2015, 45 patients [TGA/intact ventricular septum (IVS) 5, TGA/ventricular septal defect (VSD) 13, Taussig-Bing 27] with coarctation (21), arch hypoplasia (5), coarctation and hypoplasia (12) and aortic arch interruption (7) underwent ASO and arch repair. The median age at the ASO was 19 days (range, 1 day to 12.7 years). AAO was repaired concomitantly with ASO in 36 patients. Operation reports and 2D-echocardiographic data were retrospectively reviewed to determine the following parameters: position of the great arteries, coronary artery anatomy, and diameters of RVOT, aortic annulus, aortic sinotubular-junction, pulmonary annulus and transverse aortic arch previous to ASO. The median follow-up time was 6 years (range, 0-30 years). Four patients were lost to follow-up; reliable echo data were available in 24 subjects. Cox proportional hazard models were performed to examine predictors of reoperation for RVOTO., Results: Thirty-day mortality rate after ASO was 13% (n = 6), and late mortality rate 9% (n = 4). Ten patients (TGA/VSD 2, Taussig-Bing 8) had 14 reoperations for RVOTO. One patient died after reoperation. Taussig-Bing anomaly was a significant predictor of reoperation for RVOTO [hazard ratio (HR) = 5.5, 95% confidence interval (CI) = 1.15-26.38, P = 0.033]. Higher preoperative aortic annulus Z-score significantly decreased the reoperation risk (HR = 0.6, 95% CI = 0.42-0.93, P = 0.020). In reoperated patients, the mean gradient across the RVOT reduced from 84 ± 12.2 mmHg prior to reoperation to 15.29 ± 13.70 mmHg at latest follow-up., Conclusions: Taussig-Bing anomaly and smaller preoperative aortic annulus diameter (Z-score) were significant predictors of reoperation for RVOTO in patients after ASO for TGA or Taussig-Bing anomaly with AAO. In Taussig-Bing hearts, the more complex anatomy often necessitates modifications of the operation technique, sometimes precluding RVOT relief at primary ASO. During follow-up, the possibility of recurrent RVOTO should always be considered in this specific patient population. Yet, in case of a reoperation for RVOTO, the surgical relief is in general effective., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
- Published
- 2016
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30. A single-centre 37-year experience with reoperation after primary repair of atrioventricular septal defect.
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Sojak V, Kooij M, Yazdanbakhsh A, Koolbergen DR, Bruggemans EF, and Hazekamp MG
- Subjects
- Adolescent, Adult, Aged, Child, Child, Preschool, Female, Follow-Up Studies, Heart Septal Defects mortality, Heart Septal Defects physiopathology, Humans, Infant, Male, Middle Aged, Reoperation, Retrospective Studies, Survival Analysis, Treatment Outcome, Young Adult, Heart Septal Defects surgery, Heart Valve Prosthesis Implantation mortality, Pacemaker, Artificial
- Abstract
Objectives: To evaluate our experience with patients reoperated after primary repair of atrioventricular septal defect (AVSD) and identify predictors of poor outcome., Methods: Between 1976 and 2014, 69 patients were reoperated after primary repair of partial (n = 28), intermediate (n = 15) or complete (n = 26) AVSD., Results: Median age at first reoperation was 62.4 (range, 1.6-845) months, median interval to first reoperation was 22.3 (range, 0.2-598) months. Main indications for first reoperation included left atrioventricular valve (LAVV) pathology (66%), residual septal defect (19%) and left ventricle outflow tract obstruction (LVOTO; 4%). Procedures to address LAVV pathology included various valvuloplasties in 47 (77%) patients and valve replacement in 14 (23%) patients. A second, third, fourth and fifth reoperation was required in 27, 12, 4 and 1 patient, respectively. Most common procedures were LAVV replacement (LAVVR), LVOTO relief, pacemaker implantation and right atrioventricular valve procedure. Freedom from reoperation after LAVV valvuloplasty (LAVVP) was 84 and 62% at 1 and 10 years, respectively. There were 10 early and 4 late deaths. Estimated overall survival at 1, 5 and 10 years was 87, 83 and 83%, respectively. Double orifice LAVV (DOLAVV) was a risk factor for in-hospital and overall mortality [odds ratio (OR) = 14.5; 95% confidence interval (CI) = 1.2-178.7; P = 0.037 and hazard ratio (HR) = 6.8; 95% CI = 1.5-31.7; P = 0.015, respectively]. Patients with LAVVP and LAVVR differed significantly in overall survival (P = 0.014). At the last follow-up (median, 9.8; range, 0-34 years), 84% survivors were in New York Heart Association Class I or II., Conclusions: Many patients reoperated after primary AVSD repair needed surgical reintervention. LAVV pathology was the most common indication for reoperation. DOLAVV was a risk factor for mortality. Particular AVSD type did not appear to be a risk factor for mortality or LAVVP failure. There is some evidence for the close relationship between LAVV pathology and LVOTO in subjects undergoing reoperation after primary AVSD repair as some patients with initial LAVV problems needed LVOTO repair later on and vice versa., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
- Published
- 2016
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31. Partial and intermediate atrioventricular septal defects without major associated cardiac anomalies.
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Sfyridis P, Sojak V, and Hazekamp M
- Subjects
- Adolescent, Adult, Aged, Child, Child, Preschool, Heart Valves abnormalities, Heart Ventricles abnormalities, Humans, Infant, Middle Aged, Reoperation, Treatment Outcome, Young Adult, Heart Septal Defects surgery, Heart Valves surgery, Heart Ventricles surgery
- Abstract
Partial and intermediate atrioventricular septal defects (p-i AVSDs) constitute approximately 20-40% of all AVSDs. Children with p-i AVSDs are usually asymptomatic and typically undergo surgery at the preschool age or earlier if the signs of heart failure have developed. Surgical treatment for repair of p-i AVSDs has been successful for more than 60 years and is mainly directed towards closing septal defects, and maintaining or creating competent, non-stenotic left and/or right atrioventricular valves. By most measures, the outcomes of surgical management of p-i AVSDs have improved over the last 5 decades. In spite of significantly reduced mortality, the need for reoperation and long-term morbidity remains an issue in some patients from this population. The purpose of this article is to review current options and outcomes concerning the surgical management of the p-AVSD and i-AVSD variants without major associated cardiac malformations., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)
- Published
- 2015
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32. Peri-operative care in adults with congenital heart disease: room for improvement in after care.
- Author
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Schoormans D, Smets EM, Zwart R, Sprangers MA, Veelenturg TH, de Mol BA, Hazekamp MG, Koolbergen DR, Sojak V, Bouma BJ, Groenink M, Boekholdt MS, Backx AP, and Mulder BJ
- Subjects
- Adult, Aftercare psychology, Cardiac Surgical Procedures nursing, Cardiac Surgical Procedures psychology, Female, Heart Defects, Congenital nursing, Heart Defects, Congenital psychology, Humans, Linear Models, Male, Middle Aged, Perioperative Care nursing, Perioperative Care psychology, Surveys and Questionnaires, Aftercare methods, Cardiac Surgical Procedures rehabilitation, Heart Defects, Congenital surgery, Patient Satisfaction, Perioperative Care methods
- Abstract
Background: Patient satisfaction with care has received little attention within the field of congenital heart disease. Our objective was to examine patient satisfaction with the care received when undergoing open-heart surgery in order to identify the best and worst aspects of peri-operative care. Moreover, we examined whether having contact with a specialised nurse in addition to usual care is associated with higher patient satisfaction levels., Methods: Patient satisfaction was measured by the Satisfaction with Hospital Care Questionnaire, evaluating nine aspects of care by answering individual items and giving overall grades. A top 10 of the best and worst items was selected. Linear regression analyses were used to examine the relationship between having contact with a specialised nurse and patient satisfaction (9 grades), independent of patient characteristics--sex, age, educational level, and health status., Results: Data were available for 75 patients. Grades ranged from 6.74 for "discharge and after care" to 8.18 for "medical care". In all, 21% of patients were dissatisfied with the clarity of the information about lifestyle adjustments given by the surgeon. However, patients who had contact with a specialised nurse were more satisfied with the provided information (B-coefficient is 0.497, p-value is 0.038), independent of patient characteristics., Conclusions: Patients were satisfied with the received care, although there is room for improvement, especially in discharge and after care and the clarity of the information provided by the surgeon. This gap in care can be compensated for by specialised nurses, as patients who were counselled by a specialised nurse were more satisfied with the provided information.
- Published
- 2013
- Full Text
- View/download PDF
33. Small atrial septal defect associated with heart failure in an infant with a marginal left ventricle.
- Author
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Kingma SD, Rammeloo LA, Sojak V, and Hruda J
- Abstract
Atrial septal defect (ASD) is usually asymptomatic in infancy, unless pulmonary hypertension or severe co-morbidity is present. We report a case of a 4-week-old infant with moderate-sized ASD, small patent ductus arteriosus (PDA), and a borderline sized left ventricle that developed heart failure. Despite the relatively small diameter of the ASD, this defect influenced the mechanism of heart failure significantly. After surgical closure of both PDA and ASD, the signs of pulmonary hypertension resolved and the patient developed a normal sized left ventricle. This report illustrates that the presence of a small ASD in combination with a marginal left ventricle may result in inadequate left ventricular filling, pulmonary hypertension and heart failure.
- Published
- 2012
- Full Text
- View/download PDF
34. Simultaneous repair of supravalvar aortic and supravalvar pulmonary stenosis using the 3-patch technique.
- Author
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Sojak V, Koolbergen D, Filippini L, and Hazekamp M
- Abstract
Patients with Williams-Beuren syndrome may have bilateral outflow tract obstruction. We report a simultaneous repair of supravalvar aortic and supravalvar pulmonary stenosis using the 3-patch technique.
- Published
- 2010
- Full Text
- View/download PDF
35. Cardiac resynchronization therapy for the failing Fontan patient.
- Author
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Sojak V, Mazic U, Cesen M, Schrader J, and Danojevic N
- Subjects
- Child, Child, Preschool, Electrocardiography, Electrodes, Implanted, Follow-Up Studies, Humans, Male, Reoperation, Cardiac Output, Low therapy, Fontan Procedure, Heart Defects, Congenital surgery, Heart Failure therapy, Heart Ventricles abnormalities, Pacemaker, Artificial, Postoperative Complications therapy
- Abstract
Myocardial dysfunction is the leading cause of death in single-ventricle patients. Heart transplantation has traditionally been reserved for Fontan patients with end-stage myocardial dysfunction. Cardiac resynchronization therapy with multisite pacing was found to improve the myocardial performance in Fontan patients in acute postoperative settings; however, its role is unclear in chronic Fontan patients with progressive myocardial dysfunction. We present a case in which cardiac resynchronization therapy improved both hemodynamics and clinical condition in a Fontan patient with advanced myocardial dysfunction.
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- 2008
- Full Text
- View/download PDF
36. Outcomes after surgical repair of sinus venosus atrial septal defect in children.
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Sojak V, Sagat M, Balazova E, and Siman J
- Subjects
- Adolescent, Adult, Cardiac Surgical Procedures, Child, Child, Preschool, Female, Heart Septal Defects, Atrial pathology, Humans, Infant, Male, Treatment Outcome, Heart Septal Defects, Atrial surgery
- Abstract
Objectives: Review of outcomes for children undergoing surgical repair of sinus venosus atrial septal defect (SVASD) and proposal of optimal treatment strategy., Background: Unlike secundum-type atrial septal defect (ASD II), surgical correction of SVASD is more challenging and carries higher risk of postoperative complications. Several surgical techniques have been proposed to avoid them; however, the data on long-term follow-up are limited., Methods: Between 1992-2005, 85 patients underwent surgery for SVASD at our institution. 78 and 7 operations were performed through full and partial sternotomy, respectively. The mean age was 7.7 years (2 months -19 years). All patients with SVASD and no partial anomalous pulmonary venous drainage (PAPVD) or PAPVD in right atrium (n = 24) underwent the single-patch repair. Among patients with SVASD and PAPVD in SVC, 22 and 39 patients underwent intracaval baffle and Warden procedure, respectively. Clinical, electrocardiographic and echocardiographic follow-ups were available for all patients., Results: There were no deaths, reoperations, residual atrial septal defects and pulmonary vein stenosis. Two patients had SVC stenosis. Permanent arrhythmias were noted in 24 patients (28.2%). The rate of new arrhythmias in the intracaval baffle and Warden subgroup was 54.6% vs 23.1% (p = 0.01). Moreover, two and one patient from the intracaval baffle subgroup receive antiarrhythmics and had a pacemaker implanted, respectively., Conclusion: Surgical treatment of SVASD is associated with excellent results and minimal morbidity. The Warden procedure is preferred in patients with SVASD and PAPVD in SVC because of lower incidence of postoperative arrhythmias (Tab. 1, Fig. 4, Ref. 23). Full Text (Free, PDF) www.bmj.sk.
- Published
- 2008
37. Arrhythmias in congenital heart defects.
- Author
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Kaldararova M, Balazova E, Bordacova L, Lakomy M, Hraska V, Nosal M, Sojak V, Valentik P, Masura J, Milovsky V, and Vrsanska V
- Subjects
- Arrhythmias, Cardiac physiopathology, Arrhythmias, Cardiac surgery, Heart Defects, Congenital physiopathology, Heart Defects, Congenital surgery, Humans, Arrhythmias, Cardiac etiology, Heart Defects, Congenital complications
- Abstract
Aim of the Study: Evaluation of the incidence and severity of late arrhythmias in patients with predisposing congenital heart defects--either due to the anatomy of the defect itself or as a result of a particular type of surgical intervention., Patients and Methods: In a retrospective long-term study authors analyzed 158 patients (divided into 5 groups) with congenital heart defects after surgical correction. Evaluated were: the incidence of rhythm disturbances, the type of arrhythmia and the need for medication or intervention., Results: The most rhythm disturbances occurred in patients after physiological correction of D-transposition of the great arteries (68.5%) and these patients also mostly needed medication or pacemaker implantation; followed were by patients with hypoplastic left heart syndrome after Fontan procedure (40%), then were patients after long-term correction of tetralogy of Fallot (31.1%), atrial septal defect sinus venosus type with partial anomalous pulmonary venous return after Warden correction (25.7%) and congenitally corrected L-transposition of the great arteries (25 %). Most of these arrhythmias were asymptomatic and there was no need to treat them. There was an increased incidence of arrhythmias with time (p < 0.05)., Discussion: During childhood in patients after surgical correction late arrhythmias mostly do not represent a severe problem, but with time, when reaching adulthood, this may be an issue. It is therefore very important to understand the anatomy, physiology and the arrhythmogenic substrate of every high risk congenital heart defect (Tab. 2, Fig. 6, Ref. 10).
- Published
- 2007
38. Recoarctation and patients' freedom from re-intervention--a study of patients undergoing surgery for coarctation of the aorta at the Department of Cardiac Surgery of the Children's University Hospital, Bratislava.
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Omeje IC, Kaldararova M, Sagat M, Sojak V, Nosal M, Siman J, and Hraska V
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- Child, Child, Preschool, Follow-Up Studies, Humans, Infant, Newborn, Recurrence, Reoperation, Aortic Coarctation surgery
- Abstract
Background: Many children and young adults have undergone surgery for coarctation of the aorta. Individual surgical techniques employed in the treatment of patients and their effects on incidence of recoarctation were reviewed., Methods and Data: Over the last ten years, a total of 201 cases of aortic coarctation were surgically treated at our department. The three forms of coarctation of the aorta recognized by the Society for Cardiothoracic Surgeons namely: isolated coarctation, coarctation with ventricular septal defects and coarctation with complex cardiac anomalies, were represented., Results: 19 cases of recoarctation were recorded over the period of follow-up, representing 10% of all operated patients. On univariate analysis, the risk of recoarctation was closely linked with the following variables: use of resection and end-to-end anastomosis (p=0.01), age at operation less than one month (p=0.0002) and weight at operation less than 3 kg (p=0.01). The risk of recoarctation was found to be highest when resection and end-to-end anastomosis was employed in neonates (p<0.0001). Most cases of recoarctation as shown by the Kaplan-Meier plot occurred within the first year after surgery., Conclusion: The use of simple resection and end-to-end anastomosis in neonates is associated with a high risk of recoarctation. Hence, our preferred surgical technique in neonates is the extended resection and end-to-end anastomosis, which is associated with a considerably lower risk of recoarctation in this age group. (Tab. 4, Fig. 5, Re. 7)
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- 2003
39. [Surgical treatment of total anomalous pulmonary venous return].
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Hraska V, Sagat M, Sojak V, Kostolny M, Nosal M, Kantorova A, Nagi A, Krajci M, Siman J, and Kunovsky P
- Subjects
- Heart Defects, Congenital mortality, Humans, Infant, Infant, Newborn, Postoperative Complications, Survival Rate, Heart Defects, Congenital surgery, Pulmonary Veins abnormalities, Pulmonary Veins surgery
- Abstract
Background: Total abnormal pulmonary venous return (TAPVR), mainly the obstructive type represents the most riskful critical congenital heart defect requiring urgent surgery immediately after birth., The Aim of the Study: Analysis of surgical correction of TAPVR results performed from December 1992 to December 1998., Methods: Twenty-seven patients underwent surgery for TAPVR. 13 of them (48%) presented with hemodynamically severe obstruction. Mean age in the group with obstruction was 3.6 +/- 3.2 days with mean weight of 3282 +/- 537 grams., Results: From the 27 studied patients 5 (18.5%) died. Mean duration of the study in the whole group is 1.91 +/- 2.01 years. Actuarial survival in the first month is 85%, in the second month 81% and remains identical in the 1., 2., 3., 4., 5., 6. year of the study. Univariate analysis identified operation before the year 1996 (p = 0.0056) as a risk factor of immediate mortality. Introduction of ultrafiltration significantly eliminated mortality (p = 0.0101). Remaining variables (age, weight, sex, obstructive TAPVR, TAPVR, extracorporeal circulation duration, pulmonary hypertension) did not significantly influence the survival (p more than 0.05). Multivariate analysis defined operation before the year 1996 as the sole risk factor of mortality (p = 0.0033). In patients operated on in the year 1996 (n = 15) was the survival in the studied period 100%., Conclusion: Since the year 1996 the results of surgical treatment of TAPVR significantly improved. The key role in the improvement have better urgent diagnostic and surgery, improvement of surgical technique and myocardial protection, introduction of modified ultrafiltration and the quality of postoperative care. Psychomotor development of children after correction is comparable with healthy population, all patients are in NYHA I class. (Tab. 2, Fig. 4, Ref. 9.)
- Published
- 1999
40. Surgical treatment of transposition of the great arteries.
- Author
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Hraska V, Sojak V, Kostolny M, Nosal M, Sagat M, Petko M, Kantorova A, Nagi A, Kaldararova M, and Siman J
- Subjects
- Follow-Up Studies, Humans, Infant, Infant, Newborn, Survival Rate, Transposition of Great Vessels mortality, Transposition of Great Vessels surgery
- Abstract
Background: With regard to risk of the failure of systemic right ventricle after physiological correction of transposition of great arteries, anatomic repair is a current method of choice., Objective of Study: Analysis of results of surgical correction of transposition of great arteries performed between 1992 and October 1998., Method: A total of 111 patients were operated on for transposition of the great arteries. In the 1st group of patients (n = 21, mean age was 135 +/- 55 days), physiological correction according to Senning was performed. Patients of the 2nd group (n = 90, mean age was 15.4 +/- 21.6 days) underwent anatomic repair., Results: Early mortality was 6% (7 patients). Mean follow-up is 2.95 years (1.9 SD) ranging from 0.2 years to 6.1 years. Actuarial 1-month survival in the whole cohort (n = 111) is 94%, and it remains unchanged at 1, 2, 3, 4, 5, and 6 years of follow-up. Patients, who underwent surgery after 1997, show significantly better survival compared to those operated before 1997 (p = 0.0997). Thus, a date of operation (before 1997) is the only significant risk factor for death. Survival in patients operated after 1997 (n = 40) is 98%. All patients belonging to the 2nd group are in functional group NYHA 1., Conclusion: Anatomic repair of transposition of the great arteries is a method of choice for treatment of this congenital heart defect. Left ventricle becomes systemic ventricle, which is essential in view of long-term performance. Psychomotor development of children, who underwent ASO, is comparable with that of healthy population. (Tab. 3, Fig. 3, Ref. 18.)
- Published
- 1999
41. Biochemical and genetic studies of a histidine regulatory mutant of Streptomyces coelicolor A3 (2).
- Author
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Derkos-Sojak V, Pigac J, and Delić V
- Subjects
- Crosses, Genetic, DNA Restriction Enzymes, Operon, Plasmids, Species Specificity, Streptomyces metabolism, ATP Phosphoribosyltransferase genetics, Alcohol Oxidoreductases genetics, Genes, Genes, Bacterial, Genes, Regulator, Histidine metabolism, Hydro-Lyases genetics, Mutation, Pentosyltransferases genetics, Streptomyces genetics
- Abstract
The specific activities of three enzymes in a histidine regulatory mutant RF59 of Streptomyces coelicolor A3(2) resistant to the histidine analogue 1,2,4-triazolealanine (TRA) were measured and compared to the activity of the wild type strain. The first enzyme of the histidine pathway, phosphorybosyl-ATP-pyrophosphorylase (PR-ATP-pyrophosphorylase), of mutant RF59 and the wild type was sensitive to allosteric inhibition by L-histidine and hence feed-back inhibition was not affected by mutation, although the specific activity in the mutant was 2.9 fold higher than in the wild type. The other two enzymes coded by genes from the histidine operon were significantly derepressed. The enzyme D-erythroimidazoleglycerol phosphate dehydrase (IGP-dehydrase) in mutant RF59 had a 4.9 fold higher specific activity than in the wild type strain. The specific activity of the last enzyme of the pathway, histidinol-dehydrogenase (Hol-dehydrogenase), in the mutant was 4.7 fold derepressed compared to the wild type strain. The results of genetic crosses revealed the mapping of RF59 regulatory mutation between argA1 and cysD18 on S. coelicolor chromosome, suggesting that the mutant RF59 is a regulatory mutant unable to fully repress genes of the histidine pathway.
- Published
- 1985
- Full Text
- View/download PDF
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