221 results on '"Cromme-Dijkhuis A"'
Search Results
2. Afwijkingen aan hart en bloedvaten bij het syndroom van Turner
- Author
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Cromme-Dijkhuis, A. H. and Daniëls, O.
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- 2002
- Full Text
- View/download PDF
3. Katheterinterventioneller Verschluß des Vorhofseptumdefektes vom Sekundumtyp – Erfahrungen mit “CardioSeal” und “Angel Wings”
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Eicken, A., Witsenburg, M., Sebening, W., Cromme-Dijkhuis, A. C., Vogt, M., Henze, R., and Hess, J.
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- 1999
- Full Text
- View/download PDF
4. Secundum atrial septal defect is a dynamic three-dimensional entity
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Dall'Agata, A., McGhie, J., Taams, M.A., Cromme-Dijkhuis, A.H., Spitaels, S.E.C., Breburda, C.S.M., Roelandt, J.R.T.C., and Bogers, A.J.J.C.
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Atrial septal defects ,Three-dimensional display systems -- Evaluation ,Echocardiography -- Innovations ,Health - Published
- 1999
5. Prostacyclin therapy increases right ventricular capillarisation in a model for flow-associated pulmonary hypertension☆
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van Albada, Mirjam E., Berger, Rolf M.F., Niggebrugge, Marnix, van Veghel, Richard, Cromme-Dijkhuis, Adri H., and Schoemaker, Regien G.
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- 2006
- Full Text
- View/download PDF
6. Pulmonary Arterial Wall Distensibility Assessed by Intravascular Ultrasound in Children With Congenital Heart Disease*: An Indicator for Pulmonary Vascular Disease?
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Berger, Rolf M. F., Cromme-Dijkhuis, Adri H., Hop, Wim C. J., Kruit, Marco N., and Hess, John
- Published
- 2002
7. Recombinant human α-glucosidase from rabbit milk in Pompe patients
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Van den Hout, Hannerieke, Reuser, Arnold J J, Vulto, Arnold G, Loonen, M Christa B, Cromme-Dijkhuis, Adri, and Van der Ploeg, Ans T
- Published
- 2000
8. The effects of long-term growth hormone treatment on cardiac left ventricular dimensions and blood pressure in girls with Turner's syndrome
- Author
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Sas, Theo C., Cromme-Dijkhuis, Adri H., de Muinck Keizer-Schrama, Sabine M., Stijnen, Theo, van Teunenbroek, Arne, and Drop, Stenvert L.
- Published
- 1999
9. Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations
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Cromme-Dijkhuis, Adri H., Henkens, Cilia M.A., Bijleveld, Charles M.A., Hillege, Hans L., Bom, Victor J.J., and van der Meer, Jan
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Thromboembolism -- Risk factors ,Surgery -- Complications ,Blood coagulation factors -- Abnormalities ,Thrombosis -- Risk factors ,Heart - Published
- 1990
10. A broad and strong humoral immune response to donor HLA after implantation of cryopreserved human heart valve allografts
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Welters, Marij J.P, Oei, Frans B.S, Witvliet, Marian D, Vaessen, Lenard M.B, Cromme-Dijkhuis, Adri H, Bogers, A.d J.J.C, Weimar, Willem, and Claas, Frans H.J
- Published
- 2002
- Full Text
- View/download PDF
11. DECISION MAKING FOR THE SURGICAL MANAGEMENT OF AORTIC COARCTATION ASSOCIATED WITH VENTRICULAR SEPTAL DEFECT
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Brouwer, René M. H. J., Cromme-Dijkhuis, Adri H., Erasmus, Michiel E., Contant, Caroline, Bogers J. J. C., Elzenga, Nynke J., Ebels, Tjark, and Eijgelaar, Anton
- Published
- 1996
12. Right ventricular outflow tract reconstruction with an allograft conduit
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Gerestein, Cornelis G, Takkenberg, Johanna J.M, Oei, Frans B.S, Cromme-Dijkhuis, Adri H, Spitaels, Silja E.C, van Herwerden, Lex A, Steyerberg, Ewout W, and Bogers, Ad J.J.C
- Published
- 2001
- Full Text
- View/download PDF
13. Prostaglandin E1 and gastric outlet obstruction in infants
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Merkus, Peter J.F.M., Cromme-Dijkhuis, Adri H., Robben, Simon G.F., and Hess, John.
- Published
- 1993
14. Short- and midterm results of balloon valvuloplasty for valvular aortic stenosis in children
- Author
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Witsenburg, Maarten, Cromme-Dijkhuis, Adri H., Frohn-Mulder, Ingrid M.E., and Hess, John
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Balloon dilatation -- Usage ,Arteries -- Stenosis ,Congenital heart disease in children ,Health - Abstract
During a 27-month period, 21 consecutive children (aged 0.1 to 15.7 years) with isolated valvular aortic stenosis underwent percutaneous transfemoral balloon valvuloplasty. Ten children had undergone earlier surgical valvulotomy. The indication for treatment was ST-T-segment changes at rest or during bicycle-ergometry, a continuous-wave Doppler-derived transvalvular gradient>60 mm Hg or syncope, or a combination. Mean peak systolic left ventricular pressure decreased from 165 [+ or -] 19 to 131 [+ or -] 19 mm Hg (p
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- 1992
15. Use of three-dimensional echocardiography for analysis of outflow obstruction in congenital heart disease
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Dall’Agata, Anita, Cromme-Dijkhuis, Adri H, Meijboom, Folkert J, Spitaels, Silja E.C, McGhie, Jackie S, Roelandt, Jos R.T.C, and Bogers, Ad J.J.C
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- 1999
- Full Text
- View/download PDF
16. Long-Term Follow-Up of 133 Atrioventricular Septal Defects
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Ebels, T., Cromme-Dijkhuis, A. H., Eijgelaar, A., Schasfoort-van Leeuwen, M. J. M., Meijboom, E. J., Homan van der Heide, J. N., Ghosh, Probal K., editor, and Unger, Felix, editor
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- 1989
- Full Text
- View/download PDF
17. Treprostinil in advanced experimental pulmonary hypertension: Beneficial outcome without reversed pulmonary vascular remodeling
- Author
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Mirjam E. van Albada, Adri H. Cromme-Dijkhuis, Regien G. Schoemaker, Ro F. M. E. Berger, Richard van Veghel, Internal Medicine, Pediatrics, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), and Schoemaker lab
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Pulmonary Circulation ,GROWTH-FACTOR ,PHARMACOKINETICS ,medicine.medical_specialty ,surgical arteriovenous shunt ,Hypertension, Pulmonary ,medicine.medical_treatment ,Hemodynamics ,Blood Pressure ,Prostacyclin ,THERAPY ,DISEASE ,pulmonary vascular histopathology ,INTRAVENOUS EPOPROSTENOL PROSTACYCLIN ,Right ventricular hypertrophy ,Internal medicine ,pulmonary hypertension ,Animals ,Medicine ,Rats, Wistar ,Saline ,Pharmacology ,ANALOGS ,prostacyclin ,business.industry ,PROLIFERATION ,CONTINUOUS SUBCUTANEOUS INFUSION ,medicine.disease ,Epoprostenol ,monocrotaline ,Pulmonary hypertension ,Rats ,Disease Models, Animal ,Treatment Outcome ,HEMODYNAMICS ,Anesthesia ,Heart failure ,Cardiology ,ARTERIAL-HYPERTENSION ,Histopathology ,Cardiology and Cardiovascular Medicine ,business ,Treprostinil ,medicine.drug - Abstract
Introduction: Beneficial effects of treprostmil, a stable prostacyclin analogue, were demonstrated in patients with pulmonary arterial hypertension (PAH). Although regression of pulmonary vascular remodeling has been suggested as therapeutic mechanism, its mode of action remains unknown.Methods: Flow-associated PAH was created in rats by injection of monocrotaline (60 mg/kg) combined with an abdominal aortocaval shunt. Subsequently, rats were treated with subcutaneous treprostinil (50 ng/kg/min, treated; n = 8) or saline (untreated; n = 9). A control group underwent sham-surgery (n = 8). Animals were sacrificed at symptoms of cardiac failure, together with their matched controls.Results: Dyspnea and weight loss determined the moment of sacrifice in 8/9 untreated animals (89%) versus in one of eight treated animals (13%; log-rank test survival curves; P = 0.02). Mean pulmonary arterial pressure increased in the model (42 +/- 2 mm Hg in untreated vs. 18 +/- 1 in controls; P Conclusions: Treprostinil treatment improved clinical course and ameliorated symptoms of heart failure in a model of advanced PAR However, beneficial effects were not associated with reversed structural remodelling of the pulmonary vasculature.
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- 2006
18. The role of increased pulmonary blood flow in pulmonary arterial hypertension
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Rudolphus Berger, Adri H. Cromme-Dijkhuis, Regien G. Schoemaker, M. E. van Albada, Mariska Kemna, R. Van Veghel, Internal Medicine, Pediatrics, Faculteit Medische Wetenschappen/UMCG, and Schoemaker lab
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EXPRESSION ,Pulmonary and Respiratory Medicine ,Male ,Pathology ,medicine.medical_specialty ,Pulmonary Circulation ,Hypertension, Pulmonary ,Blood Pressure ,Pulmonary Artery ,TOXICITY ,DISEASE ,pulmonary vascular histopathology ,Right ventricular hypertrophy ,pulmonary arterial hypertension ,medicine.artery ,Internal medicine ,INJURY ,medicine ,Animals ,Heart Atria ,NITRIC-OXIDE SYNTHASE ,Rats, Wistar ,Pulmonary wedge pressure ,Lung ,Ultrasonography ,Monocrotaline ,business.industry ,animal model ,right heart failure ,Heart Bypass, Right ,FISTULA ,medicine.disease ,congenital heart disease ,Pulmonary hypertension ,Heart Valves ,plexogenic arteriopathy ,Rats ,Disease Models, Animal ,medicine.anatomical_structure ,HEMODYNAMICS ,Heart failure ,Pulmonary artery ,Cardiology ,business ,DIFFERS ,Blood vessel ,Artery - Abstract
Chronic increased pulmonary blood flow is considered a pre-requisite for the induction of advanced vascular lesions in pulmonary arterial hypertension in congenital heart defects. The aim of the present study was to characterise the effects of increased pulmonary flow induced by an aortocaval shunt in the monocrotaline rat model for pulmonary hypertension in terms of survival, haemodynamics, pathology and histology.Male Wistar rats were injected with monocrotaline followed by the creation of an abdominal aortocaval shunt. Animals were sacrificed when displaying symptoms of weight loss or dyspnoea, 4-5 weeks after the creation of the shunt.Echocardiography identified increased ventricular dimensions in shunted rats and right ventricular hypertrophy in monocrotaline-treated rats. At similar pulmonary artery pressures, shunted monocrotaline rats displayed higher morbidity and mortality, increased pulmonary-to-systemic artery pressure ratios and increased right ventricular hypertrophy compared with nonshunted monocrotaline rats. Histological assessment demonstrated increased number and diameter of pre-acinar pulmonary arteries. Intra-acinar vessel remodelling and occlusion occurred to a similar extent in shunted and nonshunted monocrotaline rats.In conclusion, increased pulmonary blood flow in monocrotaline-induced pulmonary hypertension is associated with increased morbidity, mortality, and unfavourable haemodynamic and cardiac effects. These effects could be attributed to more pronounced right heart failure rather than to altered intra-acinar pulmonary vessel remodelling.
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- 2005
19. Neonatale enterovirusinfecties, een case report en nieuwe mogelijkheden voor diagnostiek en behandeling
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A. C. T. M. Vossen, C. M. P. Buysse, M. W. M. Eling, N. G. Hartwig, and A. H. Cromme-Dijkhuis
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Gynecology ,medicine.medical_specialty ,business.industry ,Pediatrics, Perinatology and Child Health ,Medicine ,business - Abstract
Een neonaat wordt beschreven met een ernstig verlopen enterovirusinfectie die restloos herstelde, aansluitend aan intensieve ondersteunende therapie, immunoglobulinen en pleconaril, een nog niet geregistreerd anti-picornavirusmiddel. De laatste ontwikkelingen met betrekking tot de diagnostiek en behandeling van enterovirusinfecties in het algemeen worden besproken en er wordt een kritische blik geworpen op het gevoerde beleid.
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- 2004
20. First clinical test with recombinant human [Alpha]-glucosidase from rabbit milk shows therapeutic effect in Pompe patients
- Author
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van den Hout, H., Reuser, A., Vulto, A., Arts, W.F., Cromme-Dijkhuis, A., Hop, W., and van der Ploeg, A.
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Glucosidases -- Health aspects ,Glycogen metabolism -- Abnormalities ,Enzymes -- Health aspects ,Biological sciences - Published
- 2000
21. Recombinant human a-glucosidase from rabbit milk in Pompe patients
- Author
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van den Hout, Hannerieke, Reuser, Arnold J J, Vulto, Arnold G, Loonen, M Christa B, Cromme-Dijkhuis, Adri, and Van der Ploeg, Ans T
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Lysosomes -- Physiological aspects ,Glucosidases -- Physiological aspects ,Rabbits -- Health aspects ,Milk -- Origin - Published
- 2000
22. Pulmonary Arterial Wall Distensibility Assessed by Intravascular Ultrasound in Children With Congenital Heart Disease
- Author
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Adri H. Cromme-Dijkhuis, Marco N. Kruit, Wim C. J. Hop, Rolf M. F. Berger, and John Hess
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Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Lung ,business.industry ,Hemodynamics ,Critical Care and Intensive Care Medicine ,medicine.disease ,Pulmonary hypertension ,Pulse pressure ,medicine.anatomical_structure ,Blood pressure ,Internal medicine ,medicine.artery ,Pulmonary artery ,medicine ,Vascular resistance ,Cardiology ,Cardiology and Cardiovascular Medicine ,business ,Pulmonary wedge pressure - Abstract
Background: Both pulmonary hypertension and pulmonary overflow are associated with functional and structural changes of the pulmonary arterial wall. Current techniques to evaluate the pulmonary vasculature neglect the pulsatile nature of pulmonary flow. Study objectives: To determine whether the dynamic properties of the pulmonary arterial wall are altered in patients with abnormal pulmonary hemodynamics due to congenital heart defects, and whether these changes are associated with the progression of pulmonary vascular disease (PVD). Patients and methods: In 43 children with PVD due to congenital heart defects and 12 control subjects, pulmonary arterial pulsatility (the relative increase in vessel area during the cardiac cycle) and distensibility (the inverse of the stress/strain elastic modulus) were determined with intravascular ultrasound. Results were correlated with clinical and hemodynamic parameters. Results: Pulsatility correlated with pulmonary pulse pressure (p < 0.001), pulmonary-to-systemic vascular resistance ratio (PVR/SVR) [p = 0.001], and hemoglobin concentration (p = 0.01). However, when corrected for these variables, pulsatility did not differ between patients and control subjects. In contrast, arterial wall distensibility decreased with the severity of PVD and correlated independently with pulmonary-to-systemic arterial pressure ratio (p < 0.001) and PVR/SVR (p = 0.03), and with hemoglobin concentration (p < 0.01). Adjusted for hemodynamic variables, distensibility was still decreased in patients with PVD compared to control subjects. Conclusions: These results demonstrate that pulmonary arterial wall distensibility is progressively decreased in PVD; moreover, this decreased distensibility is, in part, related to increased distending pressure as a result of pulmonary hypertension but also, in part, to stiffening of the arterial wall during the disease process. Arterial wall distensibility may be of additional value in the evaluation of pulmonary vasculature and ventricular workload.
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- 2002
23. A broad and strong humoral immune response to donor HLA after implantation of cryopreserved human heart valve allografts
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Frans H.J. Claas, Marian D. Witvliet, Marij J. P. Welters, Ad J.J.C. Bogers, Willem Weimar, Lenard M.B. Vaessen, Frans B S Oei, Adri H. Cromme-Dijkhuis, Internal Medicine, Cardiothoracic Surgery, and Pediatrics
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Adult ,Male ,Heart disease ,Adolescent ,medicine.medical_treatment ,Immunology ,Human leukocyte antigen ,Valve replacement ,Antibody Specificity ,HLA Antigens ,Isoantibodies ,medicine ,Immunology and Allergy ,Humans ,Transplantation, Homologous ,Child ,Aged ,Cryopreservation ,biology ,Panel reactive antibody ,Antibody titer ,Infant ,Immunosuppression ,General Medicine ,Middle Aged ,medicine.disease ,Heart Valves ,Tissue Donors ,Transplantation ,Child, Preschool ,biology.protein ,Female ,Antibody - Abstract
Cryopreserved human heart valves are used for valve replacement in patients with congenital or acquired heart disease. Although no blood group or human leukocyte antigens (HLA) matching is performed and no immunosuppression is administered, the clinical results are relatively good. After valve replacement, the majority of the patients develop HLA antibodies, whereas a smaller group of patients shows valve-related events at the long term after right ventricular outflow tract reconstruction. Therefore, we hypothesized that not the mere presence, but rather the titers of antidonor HLA antibodies may be related to valve allograft failure. The presence and specificity of HLA class I antibodies were determined by complement-dependent microlymphocytotoxicity (CDC) test in longitudinally taken peripheral blood samples of 35 valve allograft recipients. In eight patients with an antibody response specific against donor-HLA class I, the titers were measured by this CDC method after stepwise dilution of the plasma. Panel reactive antibodies of more than 10% were found in 31 of 35 (89%) valve allograft recipients. From these 31 patients, 24 (77%) developed donor-specific HLA class I antibodies. All eight selected patients had detectable donor-specific antibody titers, ranging from 1:2 to 1:8,000. Two donor valve recipients before retransplantation had (donor-specific) HLA antibodies and showed high titers of 1:256 and 1:8,000 shortly after the second allograft valve replacement, which was associated with an early graft failure in the latter patient. We conclude that transplantation of cryopreserved human heart valve allografts leads to a broad and strong humoral response, which is probably the result of a lack of immunosuppressive therapy after valve transplantation. Patients receiving a second or following valve allograft appeared to be sensitized and developed early and high allo-antibody titers after second valve allograft implantation. Valve failure was diagnosed in a patient with extremely high titers. These findings suggest that preoperative cross-matching may identify patients with high donor-specific HLA antibody titers and may reduce the risk for early recurrent graft failure.
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- 2002
24. Afwijkingen aan hart en bloedvaten bij het syndroom van Turner
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O. Daniëls, A. H. Cromme-Dijkhuis, and Pediatrics
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Heartfunction and circulation ,Hartfunctie en circulatie ,media_common.quotation_subject ,Pediatrics, Perinatology and Child Health ,Art ,Theology ,media_common - Abstract
De prevalentie van aangeboren hartafwijkingen bij meisjes geboren met het syndroom van Turner (ts) is hoog, volgens de literatuur ongeveer 20 tot 25%. Hierbij komen vooral de obstructieve afwijkingen van de linker harthelft voor. Ook dilatatie van de aortawand met kans op dissectie en ruptuur wordt vaker gerapporteerd, hoewel harde gegevens over de prevalentie ontbreken. Aangeraden wordt om meisjes met ts zo snel mogelijk cardiologisch te onderzoeken als de diagnose is gesteld. Is op jonge leeftijd geen aangeboren afwijking aanwezig, dan wordt geadviseerd het cardiologische onderzoek te herhalen tijdens de puberteit. Vervolgens moet het aortaonderzoek iedere 3 tot 5 jaar worden herhaald, maar zeker voor en tijdens een geplande zwangerschap. Hypertensie komt bij ongeveer 25% van de meisjes met ts voor. Geregelde bloeddrukcontrole is dus gewenst. Een nadelig effect van de groeihormoonbehandeling op de hartspier is tot op heden niet aangetoond, maar langetermijnonderzoek hiernaar is zeker noodzakelijk.
- Published
- 2002
25. Is there a place for pediatric valvotomy in the autograft era
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Johanna J.M. Takkenberg, Ad J.J.C. Bogers, Adri H. Cromme-Dijkhuis, Maarten Witsenburg, Arie-Pieter Kappetein, P.L. de Jong, Cardiothoracic Surgery, and Pediatrics
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Pulmonary and Respiratory Medicine ,Aortic valve ,Male ,medicine.medical_specialty ,Time Factors ,Adolescent ,medicine.medical_treatment ,Aortic Valve Insufficiency ,Regurgitation (circulation) ,Transplantation, Autologous ,Catheterization ,Internal medicine ,medicine ,Humans ,Child ,Retrospective Studies ,business.industry ,General Medicine ,Aortic Valve Stenosis ,medicine.disease ,Surgery ,Valvulotomy ,Pulmonary Valve Stenosis ,Stenosis ,medicine.anatomical_structure ,El Niño ,Echocardiography ,Pulmonary valve ,Aortic Valve ,Child, Preschool ,Pulmonary valve stenosis ,cardiovascular system ,Vomiting ,Cardiology ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Objective: Valvotomy and the autograft procedure are the most common surgical treatment options for children with valvular aortic stenosis. We evaluated the results of these surgical procedures in our institution. Methods: Retrospective analysis was done of all patients presenting with aortic stenosis and operated upon before the age of 18. In 11 patients a valvotomy was performed and in 36 an autograft procedure. Results: There was no hospital mortality. Mean follow-up in the valvotomy group was 4.8 years (SD 3.3), in the autograft group 4.5 years (SD 3.3). During follow-up one patient died suddenly 2 months after valvotomy. Two patients in the autograft group died (not valve-related). After valvotomy three patients underwent a balloon valvotomy, in one followed by an autograft procedure and one patient had a repeat valvotomy. In the autograft group one patient was reoperated for severe aortic regurgitation and moderate pulmonary stenosis. At last echocardiography after valvotomy (eight remaining patients) in only two patients (25%) no aortic stenosis or regurgitation was present. In the remaining six patients aortic stenosis is mild in two and moderate in three, including one with moderate aortic regurgitation. In one patient without stenosis, moderate aortic regurgitation was seen. No pulmonary stenosis or regurgitation is present. Echocardiography after autografting (33 remaining patients) showed no aortic stenosis. Aortic regurgitation was mild in seven patients, moderate in two, severe in one. Pulmonary stenosis was present in two patients (16%). Pulmonary regurgitation was mild in three patients and moderate in one. Conclusions: In selected patients with valvular aortic stenosis who are beyond infancy, valvotomy may be adequate and may postpone further surgery for a significant length of time. After valvotomy the main problem is residual aortic stenosis while after autografting a shift occurs to aortic regurgitation and problems related to the pulmonary valve. Careful clinical and echocardiographic follow-up is therefore warranted in young patients after the autograft procedure. q 2001 Elsevier Science B.V. All rights reserved.
- Published
- 2001
26. Katheterinterventioneller Verschluß des Vorhofseptumdefektes vom Sekundumtyp - Erfahrungen mit 'CardioSeal' und 'Angel Wings'
- Author
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Adri H. Cromme-Dijkhuis, Maarten Witsenburg, J. Hess, Walter Sebening, M. Vogt, R. Henze, Andreas Eicken, and Pediatrics
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medicine.medical_specialty ,business.industry ,Interrupted aortic arch ,Septum secundum ,Foramen secundum ,Balloon ,medicine.disease ,Persistent fetal circulation ,Surgery ,medicine.anatomical_structure ,Occlusion ,Heart catheterization ,Medicine ,Atrium (heart) ,Cardiology and Cardiovascular Medicine ,business - Abstract
Between July 1996 and July 1998, 34 patients (female n = 16, male n = 18) were scheduled for catheter--interventional occlusion (ASDO) of an atrium septum defect of secundum type (ASD II). Median age was 7.9 years (2.5-54.6), weight 23.1 kg (9-97). 9 defects were closed with "Angel Wings" (AW) and 25 ASD occlusions were attempted with "CardioSeal" (CS). 30 patients had simple ASD II, 3 persisting foramen ovale with suspected paradoxal cerebral embolization and 1 significant residual shunt at atrial level after corrective heart surgery for interrupted aortic arch type B. ASDO was successful in 31/34 cases (91%). One CS was removed surgically due to significant left to right shunt after release and two CS devices were withdrawn back into the sheath. Median ASD diameter was 11.9 mm (6-16), balloon occlusive diameter was 15 mm (6-20) and median left to right shunt was 40% (6-64%). On follow up minimal left to right shunt was seen in 5/31 patients (16%). In selected patients catheter--interventional ASDO may be a good alternative to surgical ASD closure.
- Published
- 1999
27. The effects of long-term growth hormone treatment on cardiac left ventricular dimensions and blood pressure in girls with Turner’s syndrome
- Author
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Stenvert L. S. Drop, Theo Stijnen, Arne van Teunenbroek, Theo C. J. Sas, Sabine M.P.F. de Muinck Keizer-Schrama, and Adri H. Cromme-Dijkhuis
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medicine.medical_specialty ,business.industry ,Diastole ,medicine.disease ,Short stature ,law.invention ,Muscle hypertrophy ,medicine.anatomical_structure ,Blood pressure ,Endocrinology ,El Niño ,Randomized controlled trial ,Ventricle ,law ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Turner syndrome ,medicine ,Cardiology ,medicine.symptom ,business - Abstract
Objective: To assess the effects of long-term growth hormone (GH) treatment for short stature on left ventricular (LV) dimensions and systemic blood pressure (BP) in girls with Turner's syndrome without clinically relevant cardiac abnormalities. Study design: LV dimensions measured by echocardiography and systemic BP were assessed before and during 7 years of GH treatment in 68 girls with Turner's syndrome participating in a randomized dose-response study. These previously untreated girls, age 2 to 11 years, were randomly assigned to 1 of 3 GH dosage groups: group A, 4 IU/m 2 /d; group B, first year 4 IU/m 2 /d, thereafter 6 IU/m 2 /d; group C, first year 4 IU/m 2 /d, second year 6 IU/m 2 /d, thereafter 8 IU/m 2 /d. After the first 4 years, girls ≥12 years of age began receiving 17β-estradiol, 5 μg/kg body weight per day, for induction of puberty. Results: At baseline the LV dimensions of almost every girl were within the normal range, and the mean SD scores were close to zero. During 7 years of GH treatment, the growth of the left ventricle was comparable to that of healthy girls. No signs of LV hypertrophy were found. Before the start of GH treatment, mean BP was within the normal range but significantly higher than in healthy control subjects. Diastolic BP and systolic BP were above the 90th percentile in 23% and 28% of the girls, respectively. After 7 years of treatment, these percentages were 14% and 36%, respectively (not significantly different from baseline). The SD score of the diastolic BP showed a small decrease after 7 years of treatment. The growth of the left ventricle and the development of BP were not different between the GH dosage groups. Conclusions: Long-term GH treatment, even at dosages up to 8 IU/m 2 /d, does not result in LV hypertrophy or hypertension in girls with Turner's syndrome. Continued observation into adulthood is recommended to monitor the further development of the relatively high BP and to ensure that GH treatment has no long-term negative effect on the heart. (J Pediatr 1999;135:470-76)
- Published
- 1999
28. Secundum atrial septal defect is a dynamic three-dimensional entity
- Author
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C.S.M. Breburda, Anita Dall’Agata, Ad J.J.C. Bogers, Meindert A. Taams, J.S. McGhie, Jos R.T.C. Roelandt, Adri H. Cromme-Dijkhuis, and S.E.C. Spitaels
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Adult ,Male ,medicine.medical_specialty ,Adolescent ,Echocardiography, Three-Dimensional ,Septum secundum ,Foramen secundum ,Atrial septum defect ,Heart Septal Defects, Atrial ,Persistent fetal circulation ,Superoinferior ,2d echocardiography ,Heart Septum ,medicine ,Humans ,Child ,Observer Variation ,Cardiac cycle ,business.industry ,Middle Aged ,medicine.disease ,Surgery ,Echocardiography ,Child, Preschool ,Linear Models ,Gross anatomy ,Female ,Cardiology and Cardiovascular Medicine ,business ,Nuclear medicine - Abstract
The aim of this study was to evaluate the diagnostic relevance of 3-dimensional (3D) echocardiography in the assessment of secundum atrial septum defect (ASD2).Twenty-three patients (age 2 to 58 years) with an ASD2 were studied by transthoracic (n = 9) or transesophageal (n = 14) echocardiography for the acquisition of a 3D data set before undergoing surgical repair. Qualitative (location, shape, and structure) and quantitative (largest and smallest anteroposterior and superoinferior diameters) characteristics were analyzed and compared with surgical findings. Intraobserver and interobserver variability were assessed. The gross anatomy of the ASD2, shown by the 3D images, was confirmed by the surgeon in 21 of 23 patients, but the presence of membranous or fenestrated remnants of the valvula foramina ovalis in the defect was not optimally visualized in 7 patients. Three-dimensional echocardiography revealed changes in diameter and shape of the ASD2 during the cardiac cycle. The measured largest and smallest anteroposterior diameters and their intraobserver and interobserver agreement were 274 +/- 12 mm, r = 0. 95 (P.001), r = 0.92 (P.001), and 194 +/- 9 mm, r = 0.96 (P. 001), r = 0.94 (P.001), respectively. The measured largest and smallest superoinferior diameter and their intraobserver and interobserver agreement were 304 +/- 26 mm, r = 0.90 (P.001), r = 0.97 (P.001), and 204 +/- 10 mm, r = 0.83 (P.001), r = 0.84 ( P.001), respectively. The correlation coefficient between 2D and 3D echocardiography for the largest anteroposterior and superoinferior diameter was r = 0.69 (P.001) and r = 0.68 (P =.05), respectively. The correlation coefficient between the measurements from 3D reconstructions and direct surgical measurements was r = 0.20 (P = not significant) and r = 0.57 (P.05), whereas between 2D and surgery was r = 0.50 (P.05) and r = 0.26 (P = not significant).ASD2 has a complex morphology. Three-dimensional echocardiography provides better qualitative and quantitative information on its dynamic geometry, location, and extension as compared with standard 2D echocardiography and might be useful for device selection during catheter-based closure of ASD2.
- Published
- 1999
29. Use of three-dimensional echocardiography for analysis of outflow obstruction in congenital heart disease
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Jos R.T.C. Roelandt, Adri H. Cromme-Dijkhuis, Jackie S. McGhie, Ad J.J.C. Bogers, S.E.C. Spitaels, Anita Dall’Agata, Folkert J. Meijboom, Cardiothoracic Surgery, Pediatrics, and Cardiology
- Subjects
Adult ,Male ,Aortic valve ,medicine.medical_specialty ,Adolescent ,Heart disease ,Echocardiography, Three-Dimensional ,Ventricular Outflow Obstruction ,Internal medicine ,Image Processing, Computer-Assisted ,medicine ,Humans ,Ventricular outflow tract ,Child ,Pulmonary Valve ,business.industry ,Infant ,Three dimensional echocardiography ,Aortic Valve Stenosis ,Aortic Stenosis, Subvalvular ,medicine.disease ,medicine.anatomical_structure ,Aortic Valve ,Child, Preschool ,Pulmonary valve ,Aortic valve stenosis ,Cardiology ,Feasibility Studies ,Female ,Cardiology and Cardiovascular Medicine ,business ,3d echocardiography - Abstract
To evaluate the feasibility and accuracy of 3-dimensional (3D) echocardiography in analysis of left and right ventricular outflow tract (LVOT and RVOT) obstruction, 3D echocardiography was performed in 28 patients (age 4 months to 36 years) with outflow tract pathology. Type of lesion and relation to valves were assessed. Length and degree of obstruction were measured. Three-D data sets were adequate for reconstruction in 25 of 28 patients; 47 reconstructions were made. In 13 patients with LVOT obstruction, 3D echocardiography was used to study subvalvular details in 8, valvular in 13, and supravalvular in 1. Four of these 13 patients had complex subaortic obstruction. In 12 patients with RVOT lesions, 3D echocardiography was used to study subvalvular details in 11, valvular in 12, and supravalvular in 2. Three-dimensional reconstructions were suitable for analysis in 100% of subvalvular LVOT, 77% valvular LVOT, 100% supravalvular LVOT, 100% subvalvular RVOT, 50% valvular RVOT, and 50% supravalvular RVOT. Twenty patients underwent operation, and surgical findings served as morphologic control for thirty-four 3D reconstructions (LVOT 17, RVOT 17). Operative findings revealed an accuracy at subvalvular LVOT of 100%, valvular LVOT 90%, supravalvular LVOT 100%, subvalvular RVOT 100%, valvular RVOT 100%, and supravalvular RVOT 100%. Quantitative measurements could adequately be performed. Three-D echocardiography is feasible and accurate for analyzing both outflow tracts of the heart. Particularly, generation of nonconventional horizontal cross sections allows a good definition of extension and severity of lesions.
- Published
- 1999
30. Three-dimensional echocardiography enhances the assessment of ventricular septal defect
- Author
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Ad J.J.C. Bogers, Adrie H. Cromme-Dijkhuis, Goris Bol-Raap, Youssef F.M. Nosir, Jackie S. McGhie, Folkert J. Meijboom, Anita Dall’Agata, Jos R.T.C. Roelandt, Cardiothoracic Surgery, Pediatrics, and Cardiology
- Subjects
Adult ,Heart Septal Defects, Ventricular ,Male ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Echocardiography, Three-Dimensional ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Child ,Tricuspid valve ,business.industry ,Infant, Newborn ,Infant ,Three dimensional echocardiography ,Middle Aged ,Infant newborn ,medicine.anatomical_structure ,cardiovascular system ,Cardiology ,Female ,Tricuspid Valve ,Cardiology and Cardiovascular Medicine ,business - Abstract
By 3-dimensional echocardiography, the location, relation to the aortic and tricuspid valve, and the size of the ventricular septal defect was assessed and compared with 2-dimensional echocardiography and intraoperative findings. We concluded that 3-dimensional echocardiography accurately assesses the anatomy of the ventricular septal defect, provides additional information, and can be considered a valuable preoperative diagnostic tool.
- Published
- 1999
31. Clinical Outcome and Left Ventricular Function After Pulmonary Autograft Implantation in Children
- Author
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John Hess, Maarten Witsenburg, Ad J.J.C. Bogers, S.E.C. Spitaels, Adri H. Cromme-Dijkhuis, Egbert Bos, Raymond B. Hokken, Cardiothoracic Surgery, Cardiology, and Pediatrics
- Subjects
Male ,Reoperation ,Pulmonary and Respiratory Medicine ,Aortic valve ,medicine.medical_specialty ,Adolescent ,Heart Ventricles ,medicine.medical_treatment ,Left ventricular hypertrophy ,Transplantation, Autologous ,Disease-Free Survival ,Ventricular Function, Left ,Aortic valve replacement ,Actuarial Analysis ,Right ventricular hypertrophy ,Internal medicine ,medicine ,Humans ,Child ,Pulmonary Valve ,Cardiopulmonary Bypass ,business.industry ,Infant ,Aortic Valve Stenosis ,medicine.disease ,Autotransplantation ,Surgery ,Transplantation ,Stenosis ,Treatment Outcome ,medicine.anatomical_structure ,Echocardiography ,Aortic Valve ,Child, Preschool ,Pulmonary valve ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background . Aortic root replacement with a pulmonary autograft is an alternative treatment for children with aortic valve or root disease, or both. Methods . Twenty-six patients (18 boys and 8 girls) with a mean age of 10.9 years (range, 0.3 to 16.9 years) underwent this procedures in a 7-year period. The mean follow-up period was 3.2 years (range, 0.2 to 7.5 years). Results . During follow-up 3 patients died and one autograft was replaced with a mechanical valve. The actuarial survival and actuarial event-free survival rates were 87% and 79%, respectively, at both 5 and 7 years. None of the surviving patients had complaints, and all have done well and are living normal lives. Electrocardiographic signs of myocardial ischemia and left ventricular hypertrophy were not present. Echocardiography showed autograft valve regurgitation to be absent or trivial (n = 17) or mild (n = 5). Stenosis was not present. Increasing autograft annulus diameters were noted during follow-up, but this was not related to the severity of autograft regurgitation. Left ventricular dimensions and function were within normal limits later than 1 year after the operation. Only 2 patients had a moderate pulmonary stenosis without right ventricular hypertrophy. Conclusions . The surgical results, clinical outcome, valve function, and left ventricular function in our patients have been good. This procedure is recommended as a method of aortic valve replacement in children. (Ann Thorac Surg 1997;63:1713–7)
- Published
- 1997
32. Repair of aortic arch interruption by direct anastomosis1
- Author
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Raymond B. Hokken, Carolien M.E Contant, Adri H. Cromme-Dijkhuis, and Ad J.J.C. Bogers
- Subjects
Pulmonary and Respiratory Medicine ,Aortic arch ,medicine.medical_specialty ,Aorta ,business.industry ,medicine.medical_treatment ,Interrupted aortic arch ,General Medicine ,medicine.disease ,Surgery ,Stenosis ,Surgical anastomosis ,Median sternotomy ,medicine.artery ,Ascending aorta ,medicine ,Thoracic aorta ,Cardiology and Cardiovascular Medicine ,business - Abstract
Objective: Evaluation of surgical treatment of interrupted aortic arch (IAA) by direct anastomosis. Methods: A consecutive series of 17 infants with IAA (type A in eight patients, type B in nine) were operated upon. The mean age at arch repair was 1.0 month (range 0.2-7.7), mean weight was 3.7 kg (range 2.2-6.2). All arch repairs were done by direct anastomosis. This included a persistent arterial duct in one and a subdavian turnup in another case. The aortic reconstruction included reimplantation of a lusoric artery in three patients, patch enlargement of the ascending aorta in three and of the complete arch in one patient. The arch repair was done through a lateral thoracotomy in three patients. In 14 patients the aortic repair was part of a single-stage approach through a median sternotomy using cardiopulmonary bypass and circulatory arrest. Results: There was no operative mortality. One patient (single-stage approach) died 2 days after operation due to respiratory problems caused by tracheobronchomalacy. One patient (lateral approach) died suddenly 3 months after aortic repair and banding. Median follow up was 4.8 years (range 0.1-12.9). In five patients restenosis of the aortic arch developed, all within 1.5 years after repair. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from restenosis was 61% at 5 years with a 70% confidence limit (CL 70% ) of 46-75. All restenoses were balloon dilated, but two needed redo surgery, which was done by the median approach. In three patients discrete subaortic stenosis developed. This was not correlated with the type of interruption, weight at operation, age at operation or the surgical approach. The actuarial freedom from subaortic stenosis was 68% at 5 years (CL 70% = 54-83). These stenoses were treated by enucleation, followed in one patient by a pulmonary autograft procedure for recurrent root stenosis after another year. At the end of follow up all patients were thriving well, lacked symptoms, were normotensive and had normal femoral artery pulsations. Conclusions: IAA can be treated well with primary anastomosis. Possible restenosis of the aortic arch can adequately be treated by percutaneous balloon dilatation or redo surgery if necessary. Arch repair by median single-stage approach has our preference.
- Published
- 1997
33. In utero diagnosis of infra-diaphragmatic total anomalous pulmonary venous return
- Author
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Marja W. Wessels, A. H. Cromme-Dijkhuis, J. W. Wladimiroff, and Ingrid M.E. Frohn-Mulder
- Subjects
Fetus ,medicine.medical_specialty ,Radiological and Ultrasound Technology ,medicine.diagnostic_test ,business.industry ,Ultrasound ,Obstetrics and Gynecology ,Diaphragmatic breathing ,General Medicine ,medicine.anatomical_structure ,Reproductive Medicine ,Ventricle ,In utero ,Internal medicine ,medicine ,Cardiology ,Anomalous pulmonary venous return ,Gestation ,Radiology, Nuclear Medicine and imaging ,Radiology ,business ,Fetal echocardiography - Abstract
This report describes the diagnosis of infra-diaphragmatic total anomalous pulmonary venous return in a fetus at 25 weeks of gestation. Abnormal venous pathways were visualized with real-time, pulsed and color Doppler ultrasound. The growth of cardiac structures, especially the left ventricle, was evaluated during subsequent ultrasound examinations.
- Published
- 1996
34. Decision making for the surgical management of aortic coarctation associated with ventricular septal defect
- Author
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Nynke J. Elzenga, Tjark Ebels, Michiel E. Erasmus, Ad J.J.C. Bogers, A Eijgelaar, Adri H. Cromme-Dijkhuis, Caroline Contant, René M.H.J. Brouwer, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), and Groningen Institute for Organ Transplantation (GIOT)
- Subjects
Heart Septal Defects, Ventricular ,Male ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,BALLOON ANGIOPLASTY ,Time Factors ,Heart disease ,Coarctation of the aorta ,INFANTS ,Aortic Coarctation ,AGE ,Recurrence ,Risk Factors ,medicine.artery ,Internal medicine ,medicine ,Humans ,Hospital Mortality ,cardiovascular diseases ,Risk factor ,Probability ,Retrospective Studies ,REPAIR ,Heart septal defect ,business.industry ,Infant, Newborn ,Infant ,Retrospective cohort study ,medicine.disease ,Survival Analysis ,Surgery ,LIFE ,1ST 3 MONTHS ,Pulmonary artery ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Complication ,Shunt (electrical) ,Follow-Up Studies - Abstract
Coarctation of the aorta and associated ventricular septal defect may be repaired simultaneously or by initial coarctation repair with or without banding of the pulmonary artery. The question is whether specific preoperative criteria can enable the surgeon to choose the optimal surgical management. Between 1980 and 1993, 80 infants younger than 3 months with coarctation and ventricular septal defect were treated surgically. In 64 infants (multistage group), simple coarctation repair was performed through a posterolateral approach, with concomitant banding of the pulmonary artery in 10 infants. Twenty ventricular septal defects were closed as a secondary procedure and four were closed as a tertiary procedure. Sixteen infants (single-stage group) underwent one-stage repair through an anterior midline approach. The total in-hospital mortality rate was 7.5%. Freedom from recoarctation after 5 years was 91.3% in the multistage group versus 60.0% in the single-stage group (p = 0.018). Freedom from secondary ventricular septal defect treatment in the multistage group after 5 years was 40.7%, versus 100% in the single-stage group (p = 0.016). Thirty-seven ventricular septal defects (47.8%) closed spontaneously. In particular, the preoperative left-to-right shunt and extension of the perimembranous VSD into the inlet or outlet were risk factors for the need for eventual surgical ventricular septal defect closure after initial coarctation repair. On the basis of these two risk factors, the probability of the need for eventual surgical treatment of ventricular septal defect after initial coarctation repair can be calculated. This policy offers a well-considered choice between single-stage and multistage repair, weighing the risk of secondary ventricular septal defect treatment versus the risk of recoarctation. Finally, the number of surgical procedures per infant will be as low as possible. (J THORAC CARDIOVASC SURG 1996;111:168-75)
- Published
- 1996
35. Allograft reconstruction of the right ventricular outflow tract
- Author
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J. Hess, Ad J.J.C. Bogers, Adri H. Cromme-Dijkhuis, Raymond B. Hokken, Egbert Bos, Ewout W. Steyerberg, L. A. Van Herwerden, and Tineke P. Willems
- Subjects
Adult ,Male ,Pulmonary and Respiratory Medicine ,Aortic valve ,medicine.medical_specialty ,Adolescent ,Ventricular Dysfunction, Right ,Persistent truncus arteriosus ,Risk Factors ,Internal medicine ,medicine ,Humans ,Transplantation, Homologous ,Ventricular outflow tract ,Tricuspid atresia ,Tetralogy of Fallot ,Pulmonary Valve ,business.industry ,Graft Survival ,Age Factors ,General Medicine ,Middle Aged ,Prognosis ,medicine.disease ,Surgery ,Survival Rate ,Transplantation ,surgical procedures, operative ,medicine.anatomical_structure ,Evaluation Studies as Topic ,Great arteries ,Aortic Valve ,Child, Preschool ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,Pulmonary atresia ,business ,Follow-Up Studies - Abstract
Objective. Evaluation of allograft reconstruction of the right ventricular outflow tract (RVOT). Methods. From 1986 to April 1995, 201 allografts (146 pulmonary, 55 aortic) were implanted in 189 patients for conduit reconstruction of the RVOT in congenital heart disease or in the pulmonary autograft procedure. The mean age at allograft implantation was 16 years (range 2 weeks - 54 years). The primary diagnoses of these patients were truncus arteriosus (n=19, 10%), transposition of the great arteries (TGA) with ventricular septal defect (VSD) and pulmonary atresia (PA) or stenosis (PS) (n= 14, 7%), PA with VSD (n = 26, 14%), PA or PS with intact septum (n = 7, 4%), tetralogy of Fallot (n = 44, 23%), corrected TGA with PA or PS (n = 11, 6%), tricuspid atresia (n = 9, 5%), aortic valve pathology for pulmonary autograft procedure (n = 55, 29%), and miscellaneous (n =4, 2%). The allograft implantation was a reoperation in 54 patients (29%). Results. The mean follow-up was 2.5 years (range 4 weeks-9 years). Six patients died in hospital (3.2%). Patient survival at 5 years was 91% (95% CL 86-95%). Freedom from all valve-related events (2 deaths, 17 reoperations, one endocarditis), as determined during reoperation or autopsy at 5 years was 78% (95% CL 65-86%). Freedom from structural allograft failure was 83% (2 deaths, Allografts implanted for congenital right heart defects failed earlier than allografts used for pulmonary auto- graft procedures (P= 0.05). Aortic allografts showed structural failure more often than pulmonary allografts (P=0.05). There were more valve- more often than pulmonary allografts related events in patients of a younrelated at implantation (P= 0.02) and in those allograft valves from younger donors (P=0.004). Conclusions. Allograft RVOT reconstruction is an adequate surgical therapy. The allograft should preferably be pulmonary. A younger age at implantation is a risk factor for allograft failure. Donor age may be a thus-far underestimated risk factor for allograft degeneration.
- Published
- 1996
36. Evaluation of the Pulmonary Vasculature and Dynamics with Intravascular Ultrasound Imaging in Children and Infants
- Author
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A M Van Vliet, Rudolphus Berger, A H Cromme-Dijkhuis, and John Hess
- Subjects
medicine.medical_specialty ,Adolescent ,Heart disease ,medicine.medical_treatment ,Hemodynamics ,Computer Systems ,medicine.artery ,Intravascular ultrasound ,medicine ,Humans ,Vascular Diseases ,Child ,Lung ,Ultrasonography, Interventional ,Cardiac catheterization ,medicine.diagnostic_test ,Vascular disease ,business.industry ,Infant ,medicine.disease ,medicine.anatomical_structure ,Evaluation Studies as Topic ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Pulmonary artery ,Vascular resistance ,Feasibility Studies ,Radiology ,business - Abstract
The ability to assess the pulmonary vasculature in pulmonary vascular disease by hemodynamic or histologic evaluation is limited. We sought to determine the feasibility of intravascular ultrasound techniques in infants and children and to assess simultaneously the morphology and dynamics of pulmonary arteries. Patients were seen in the Department of Pediatrics, Division of Pediatric Cardiology, Sophia Children's Hospital, Rotterdam, The Netherlands. We performed intravascular ultrasound imaging in 11 pediatric patients with congenital heart disease undergoing cardiac catheterization. Luminal diameter, area, and pulsatility were determined at two to five sites in the pulmonary branches. Pulmonary vascular reaction to 100% oxygen inhalation was studied. Patients weighed 4.1-51.0 kg (21.8 + 16.3 kg, mean + SD). Luminal diameters, areas, and pulsatilities could be determined reproducibly in arteries with diameters from 1.6 to 9.3 mm. In total 39 sites were studied in 11 patients. Pulsatility was related to vessel size (r = 0.81), although a substantial interindividual variation was present. After 100% oxygen inhalation, pulsatility increased in all arteries (from 20.0 2 3.3% to 25.9 + 2.9%,p < 0.05), and vasodilatation could be directly visualized, most prominently in the smallest arteries (percentage change in diameter, mean 7.4 + 2.8% versus -2.8 2 3.1% in the largest arteries, p < 0.001). Measurement of wall thickness was not feasible, but specific changes in the appearance of the wall structure could be recognized in a patient with severely elevated pulmonary vascular resistance. The specific advantages of intravascular ultrasound in assessing pulmonary vascular disease are discussed. We conclude that intravascular ultrasound imaging of the pulmonary vasculature is feasible in infants and children and provides a unique opportunity to assess directly pulmonary dynamics in vivo. Therefore, it may be a valuable tool in evaluating the pulmonary vasculature and its responses to normal and pathologic conditions. (Pediatr Res 38
- Published
- 1995
37. Aortic root replacement with pulmonary autograft in children
- Author
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Maarten Witsenburg, Adri H. Cromme-Dijkhuis, Ad J.J.C. Bogers, John Hess, Paul H. Schoof, Egbert Bos, and Eric J.M. Thijssen
- Subjects
musculoskeletal diseases ,Pulmonary and Respiratory Medicine ,Aortic valve ,medicine.medical_specialty ,business.industry ,Restrictive cardiomyopathy ,Endocardial fibroelastosis ,medicine.disease ,Surgery ,Stenosis ,surgical procedures, operative ,medicine.anatomical_structure ,Aortic valve replacement ,Heart failure ,Internal medicine ,medicine ,Cardiology ,Rheumatic fever ,Cardiology and Cardiovascular Medicine ,business ,Juvenile rheumatoid arthritis - Abstract
Between September 1988 and February 1993, 14 patients whose ages ranged from.3 months to 16 years (mean 11.1 ± 4.3 years) underwent replacement of the aortic root with the autologous pulmonary root for aortic valve disease. The follow-up was 4 years (cumulative total of 25.2 patient-years). There was no early mortality. Late mortality (one patient) was 7.1 % (95% confidence limits 0 % to 21 %). This patient had juvenile rheumatoid arthritis and died of consequent congestive heart failure with autograft failure 6 months after operation: Event-free survival after 4 years was 78.6% (95% confidence limits 50% to 95%). One patient was reoperated on because of autograft failure caused by a relapse of rheumatic fever. One patient operated on for critical neonatal aortic stenosis has subnormal exercise tolerance because of restrictive cardiomyopathy and pulmonary homograft regurgitation. The other 12 patients were in New York Heart Association functional class I at the end of follow-up. There was no prevalence of bacterial endocarditis. There were no signs of primary structural degeneration of the pulmonary autograft. During follow-up, in eight patients, increased anulus diameter of the pulmonary autograft could be demonstrated by precordial two-dimensional echocardiography, suggesting growth of the autograft. Our experience shows that aortic root replacement with the pulmonary autograft can be done with low mortality and morbidity in children with aortic valve disease. The operation seems to be contraindicated in children with juvenile rheumatoid arthritis because of the risk of recurrence of rheumatic disease in the autograft: The pulmonary autograft has also been shown to be susceptible to recurrence of rheumatic inflammation in children with a history of acute rheumatic fever. Despite pulmonary autograft replacement of the aortic valve in infants with critical valvular aortic stenosis and endocardial fibroelastosis, clinical results may be poor. Growth of the autograft is suggested by echocardiography follow-up. We consider aortic root replacement with the pulmonary autograft the procedure of choice in children who require aortic valve replacement.
- Published
- 1994
38. Specific sequelae after Fontan operation at mid- and long-term follow-up
- Author
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Adri H. Cromme-Dijkhuis, K. Hählen, A. A. Eygelaar, C. M. A. Henkens, Margreet Th.E. Bink-Boelkens, John Hess, and Egbert Bos
- Subjects
Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Hemodynamics ,Sequela ,medicine.disease ,Thrombosis ,Protein C deficiency ,Internal medicine ,Ambulatory ,medicine ,Cardiology ,Surgery ,Cardiology and Cardiovascular Medicine ,business ,Liver function tests ,Electrocardiography ,Coagulation Disorder - Abstract
Patients who have undergone a Fontan-type operation usually have an elevated systemic venous pressure. To determine the sequelae of this nonphysiologic condition, we evaluated 66 patients 1 to 14 years after a Fontan-type operation. Fifty-one patients were apparently in good clinical condition, and 15 patients had symptoms and were restricted in their daily life. Bicycle exercise capacity, tested in 41 patients, ranged from 50% to 110% (mean 85%) of the predicted value for length. In 16 patients, a decreased capacity (< 85%) was, among others, related to arrhythmias and the presence of protein-losing enteropathy. A 24-hour ambulatory electrocardiogram was available in 56 patients and found to be normal in 32 (57%) patients. Arrhythmias were present in 21 patients, six of whom had symptoms. Three patients had previous pacemaker implantation. One or more abnormalities in liver enzyme and function tests were present in 40 patients (61%) and in coagulation factors in 46 patients (69%). The most pronounced was a protein C deficiency, a known thrombotic risk factor, present in 41 patients. The occurrence of arrhythmias increased with time of follow-up (p < 0.004), the occurrence of protein C deficiency decreased with time (p < 0.0001), and the occurrence of abnormal liver enzyme and function tests was not related to time of follow-up. With regard to age at operation, arrhythmias did not occur in patients who underwent operation at a mean age of 4 +/- 1.9 years (standard deviation), in contrast to patients who underwent operation at a mean age of 7.6 +/- 4 years (standard deviation) (p < 0.001). The occurrence of the two other types of sequelae was not related to the age at operation. With regard to the type of operation, only patients with a valved right atrium-to-pulmonary artery connection had a higher prevalence of arrhythmias than patients with a nonvalved or direct right atrium-to-pulmonary artery connection (p < or = 0.001). The latter patients also had a higher prevalence of protein C deficiency (p < or = 0.001). No relationship was found among the other types of operation, the underlying structure, or the hemodynamic condition measured at rest and the presence of arrhythmias, abnormal liver enzyme and function tests, or protein C deficiency. This point survey shows that even patients with an apparently good clinical condition are at risk for arrhythmias, abnormal liver enzyme and function tests, and coagulation factor abnormalities. Serial statement of affairs is recommended to ensure that adequate preventive measures can be taken.
- Published
- 1993
39. Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations
- Author
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Hans L. Hillege, J. vd Meer, C.M.A. Bijleveld, C. M. A. Henkens, A.H. Cromme-Dijkhuis, and V. J. J. Bom
- Subjects
Adult ,Male ,Reoperation ,medicine.medical_specialty ,Time Factors ,Adolescent ,Heart Diseases ,medicine.drug_class ,Postoperative Complications ,Liver Function Tests ,Risk Factors ,Thromboembolism ,Internal medicine ,medicine ,Humans ,Risk factor ,Child ,Factor X Deficiency ,Hypoprothrombinemias ,Thrombotic risk ,Antithrombin III Deficiency ,business.industry ,Anastomosis, Surgical ,Anticoagulant ,Antithrombin ,Protein C Deficiency ,General Medicine ,Blood Coagulation Disorders ,Surgery ,Fontan operations ,Coagulation ,Child, Preschool ,Cardiology ,Female ,Tricuspid Valve ,Abnormality ,business ,Complication ,Follow-Up Studies ,medicine.drug - Abstract
Severe thromboembolism occurred in 3 of 37 patients who had undergone Fontan-type operations for correction of congenital heart defects several months to years after the operation. The patients were screened for coagulation factor abnormalities to find out whether known prothrombotic risk factors could explain the high frequency of thromboembolism. 63 abnormalities were found in 24 of the 37 patients. The commonest and most pronounced abnormality was deficiency of protein C, a known risk factor. Concentrations of antithrombin III and factors II and X were significantly lower in protein-C-deficient patients than in those with normal protein C concentrations. These findings suggest that the high thrombotic risk in these patients is caused or at least associated with an imbalance between procoagulant and anticoagulant factors.
- Published
- 1990
40. Long-term intravenous treatment of pompe disease with recombinant human [alpha]-glucosidase from milk
- Author
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Van den Hout, Johanna M.P., Kamphoven, Joep H.J., Winkel, Leon P.F., Arts, Willem F.M., De Klerk, Johannes B.C., Loonen, Christa B., Vulto, Arnold G., Cromme-Dijkhuis, Adri, WeisglasKuperns, Nynke, Hop, Wim, Van Hirtum, Hans, Van Diggelen, Otto P., Boer, Marijke, Kroos, Marian A., Van Doom, Pieter A., Van der Voort, Edwin, Sibbles, Barbara, Van Corven, Emiel J.J.M., Brakenhoff, Just P.J., Van Hove, Johan, Smeitink, Jan A.M., de Jong, Gerard, Reuser, Arnold J.J., and Van der Ploeg, Arts T.
- Subjects
Metabolism, Inborn errors of -- Care and treatment ,Glucosidases -- Testing ,Glycogenosis -- Care and treatment - Abstract
Objective. Recent reports warn that the worldwide cell culture capacity is insufficient to fulfill the increasing demand for human protein drugs. Production in milk of transgenic animals is an attractive alternative. Kilogram quantities of product per year can be obtained at relatively low costs, even in small animals such as rabbits. We tested the long-term safety and efficacy of recombinant human [alpha]-glucosidase (rhAGLU) from rabbit milk for the treatment of the lysosomal storage disorder Pompe disease. The disease occurs with an estimated frequency of 1 in 40 000 and is designated as orphan disease. The classic infantile form leads to death at a median age of 6 to 8 months and is diagnosed by absence of [alpha]-glucosidase activity and presence of fully deleterious mutations in the [alpha]-glucosidase gene. Cardiac hypertrophy is characteristically present. Loss of muscle strength prevents infants from achieving developmental milestones such as sitting, standing, and walking. Milder forms of the disease are associated with less severe mutations and partial deficiency of [alpha]-glucosidase. Methods. In the beginning of 1999, 4 critically ill patients with infantile Pompe disease (2.5-8 months of age) were enrolled in a single-center open-label study and treated intravenously with rhAGLU in a dose of 15 to 40 mg/kg/week. Results. Genotypes of patients were consistent with the most severe form of Pompe disease. Additional molecular analysis failed to detect processed forms of [alpha]-glucosidase (95, 76, and 70 kDa) in 3 of the 4 patients and revealed only a trace amount of the 95-kDa biosynthetic intermediate form in the fourth (patient 1). With the more sensitive detection method, (35) S-methionine incorporation, we could detect low-level synthesis of [alpha]-glucosidase in 3 of the 4 patients (patients 1, 2, and 4) with some posttranslation modification from 110 kDa to 95 kDa in 1 of them (patient 1). One patient (patient 3) remained totally deficient with both detection methods (negative for cross-reactive immunologic material [CRIM negative]). The [alpha]-glucosidase activity in skeletal muscle and fibroblasts of all 4 patients was below the lower limit of detection (3 years of treatment. Anti-rhAGLU immunoglobulin G titers initially increased during the first 20 to 48 weeks of therapy but declined thereafter. There was no consistent difference in antibody formation comparing CRIM-negative with CRIM-positive patients. Muscle [alpha]-glucosidase activity increased from 42[degrees]C, unstable blood pressure, and coma. The respiratory course of patient 1 remained uneventful. The 2 older patients, who both were hypercapnic (partial pressure of carbon dioxide: 10.6 and 9.8 kPa; normal range: 4.5-6.8 kPa) at start of treatment, became ventilator dependent before the first infusion (patient 2) and after 10 weeks of therapy (patient 4). Patient 4 was gradually weaned from the ventilator after 1 year of high-dose treatment and was eventually completely ventilator-free for 5 days, but this situation could not be maintained. Currently, both patients are completely ventilator dependent. The most remarkable progress in motor function was seen in the younger patients (patients I and 3). They achieved motor milestones that are unmet in infantile Pompe disease. Patient 1 learned to crawl (12 months), walk (16 months), squat (18 months), and climb stairs (22 months), and patient 3 learned to sit unsupported. The Alberta Infant Motor Scale score for patients 2, 3, and 4 remained far below p5. Patient 1 followed the p5 of normal. Conclusion. Our study shows that a safe and effective medicine can be produced in the milk of mammals and encourages additional development of enzyme replacement therapy for the several forms of Pompe disease. Restoration of skeletal muscle function and prevention of pulmonary insufficiency require dosing in the range of 20 to 40 mg/kg/week. The effect depends on residual muscle function at the start of treatment. Early start of treatment is required. Pediatrics 2004;113:e448-e457. URL: http:/Iwww.pediatrics.org/cgi/content/full/113/5/e448; transgenesis, enzyme therapy, lysosomal, muscular dystrophy, glycogen storage disease.
- Published
- 2004
41. Prostacyclin therapy increases right ventricular capillarisation in a model for flow-associated pulmonary hypertension
- Author
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Richard van Veghel, Regien G. Schoemaker, Rolf M. F. Berger, Mirjam E. van Albada, Adri H. Cromme-Dijkhuis, Marnix J. N. Niggebrugge, Pediatrics, Internal Medicine, Faculteit Medische Wetenschappen/UMCG, Cardiovascular Centre (CVC), Vascular Ageing Programme (VAP), and Schoemaker lab
- Subjects
Male ,Vascular Endothelial Growth Factor A ,Hemodynamics ,Gene Expression ,heart failure ,Blood Pressure ,angiogenesis ,cyclo-oxygenase ,Lung ,IN-VIVO ,Arachidonic Acid ,Ventricular Remodeling ,Respiratory disease ,Organ Size ,Survival Rate ,medicine.anatomical_structure ,Echocardiography ,Cardiology ,cardiovascular system ,histopathology ,medicine.drug ,medicine.medical_specialty ,GROWTH-FACTOR ,RAT-HEART ,Heart Ventricles ,Hypertension, Pulmonary ,THROMBOXANE PRODUCTION ,INHIBITION ,Pulmonary Artery ,INTRAVENOUS EPOPROSTENOL PROSTACYCLIN ,Right ventricular hypertrophy ,Internal medicine ,medicine.artery ,medicine ,Animals ,Iloprost ,Rats, Wistar ,Pharmacology ,business.industry ,Myocardium ,SYNTHASE EXPRESSION ,medicine.disease ,Pulmonary hypertension ,Capillaries ,Rats ,ASPIRIN ,Disease Models, Animal ,Receptors, Vascular Endothelial Growth Factor ,MYOCARDIAL-INFARCTION ,pulmonary circulation ,Heart failure ,Pulmonary artery ,ARTERIAL-HYPERTENSION ,business ,Platelet Aggregation Inhibitors - Abstract
Pulmonary hypertension, and consequently right ventricular failure, complicates several congenital heart defects. Although intervention in the prostacyclin-thromboxane ratio is known to improve outcome, the underlying mechanism is not clear. Therefore, effects of acetyl salicylic acid and iloprost are studied in an animal model for flow-associated pulmonary hypertension. Male Wistar rats with flow-associated pulmonary hypertension, an aortocaval shunt in addition to monocrotaline induced pulmonary hypertension, were treated with low-dose aspirin (25 mg/kg/day) or iloprost (72 microg/kg/day). Effects on pulmonary hemodynamics and pulmonary vascular remodeling as well as right ventricular hemodynamics and remodeling were evaluated. Ninety percent (n=7/8) of the untreated pulmonary hypertensive rats developed dyspnea and pleural fluid, whereas this was seen in 50% (n=4/8, ns) and 10% (n=1/8, P
- Published
- 2006
42. A randomized, placebo-controlled GH trial in very preterm infants who were at risk for bronchopulmonary dysplasia and were treated with dexamethasone
- Author
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Adri H Cromme-Dijkhuis, Wim C.J. Hop, Anita C. S. Hokken-Koelega, Marianne W A Huysman, Pieter J J Sauer, Epidemiology, and Pediatrics
- Subjects
Male ,Time Factors ,Anti-Inflammatory Agents ,CHILDREN ,Gastroenterology ,Cardiovascular System ,Dexamethasone ,law.invention ,Placebos ,Randomized controlled trial ,law ,Birth Weight ,Hormone metabolism ,Insulin-Like Growth Factor I ,Bronchopulmonary Dysplasia ,musculoskeletal system ,RENAL-TRANSPLANT ,Growth hormone treatment ,SURVIVAL ,Female ,medicine.symptom ,Ventilator Weaning ,Infant, Premature ,medicine.drug ,musculoskeletal diseases ,BIRTH-WEIGHT INFANTS ,medicine.medical_specialty ,GROWTH-HORMONE TREATMENT ,Birth weight ,CORTICOSTEROIDS ,Placebo ,Double-Blind Method ,Internal medicine ,medicine ,Humans ,I IGF-I ,SUPPRESSION ,business.industry ,Infant, Newborn ,Infant ,medicine.disease ,Hormones ,Low birth weight ,Endocrinology ,Insulin-Like Growth Factor Binding Protein 3 ,Bronchopulmonary dysplasia ,CHRONIC LUNG-DISEASE ,Growth Hormone ,Pediatrics, Perinatology and Child Health ,business ,RETARDATION - Abstract
Very preterm infants who develop bronchopulmonary dysplasia are often treated with dexamethasone (DEXA) to wean them from the ventilator. As DEXA has growth-suppressive and catabolic effects, which might have long-term consequences on growth and organ development, we investigated whether high-dose GH treatment could overcome these effects. In a randomized, double-blind, placebo-controlled trial, 30 ventilated very low birth weight infants were assigned to receive either GH or placebo treatment after start of DEXA. DEXA was given for 24 d (starting dose 0.5 mg (.) kg(-1) d tapering off every third day). Simultaneously, high-dose GH (0.3 mg (.) kg(-1) (.) d(-1)) or placebo was administered during 6 wk. During high-dose DEXA treatment (dose 0.5-0.3 mg (.) kg(-1) (.) d(-1)), no gain in head circumference, weight, crown-heel length, and knee-heel length occurred in the GH and placebo groups. Growth during the 6-wk study period was not different between the GH and the placebo groups. Two patients in the placebo group died, but the number and the severity of adverse effects was not statistically different between the GH and placebo groups. In conclusion, high-dose GH treatment did not improve growth in DEXA-treated very preterm infants and thus cannot be recommended to prevent growth failure in these infants. During high-dose DEXA, a complete growth arrest occurred, including stunting of head growth. Growth in head circumference and weight with lower dose DEXA was comparable to growth after discontinuation of DEXA.
- Published
- 2005
43. Development of left atrioventricular valve regurgitation after correction of atrioventricular septal defect
- Author
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Ad J.J.C. Bogers, A. Derk Jan Ten Harkel, Bianca C. C. Heinerman, Adri H. Cromme-Dijkhuis, Wim C. J. Hop, Pediatrics, Epidemiology, and Cardiothoracic Surgery
- Subjects
Heart Septal Defects, Ventricular ,Male ,Reoperation ,Pulmonary and Respiratory Medicine ,Thorax ,congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,Adolescent ,Heart disease ,Gauche effect ,Regurgitation (circulation) ,Heart Septal Defects, Atrial ,Risk Factors ,Internal medicine ,medicine ,Humans ,cardiovascular diseases ,Atrioventricular Septal Defect ,Cardiac Surgical Procedures ,Child ,Survival rate ,Retrospective Studies ,Atrioventricular valve ,Heart septal defect ,business.industry ,Infant ,Mitral Valve Insufficiency ,medicine.disease ,Surgery ,Survival Rate ,Treatment Outcome ,Child, Preschool ,Multivariate Analysis ,cardiovascular system ,Cardiology ,Female ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
Background Left-sided atrioventricular valve regurgitation is the main indication for reoperation in patients after repair of both partial and complete atrioventricular septal defect. Until now, the timing for reoperation is difficult. We sought to determine the outcome of severe residual left-sided atrioventricular valve regurgitation, either medically treated or reoperation. In this regard risk factors were determined for severe residual left-sided atrioventricular valve regurgitation and reoperation, and the most appropriate strategy for patients with postoperative severe left-sided atrioventricular valve regurgitation was identified. Methods Retrospective review of clinical, operative, and echocardiographic data was performed. From 1990 until 2001 164 patients underwent correction of their atrioventricular septal defect. Results Five patients died in the immediate postoperative period, and 2 patients were lost to follow-up. During follow-up (median, 66 months; range, 9 months to 12 years), 30 patients (19%) had severe left-sided atrioventricular valve regurgitation. Sixteen patients had severe left-sided atrioventricular valve regurgitation in the immediate postoperative period; 4 of them showed spontaneous regression to near-normal valve function during follow-up. Fourteen patients exhibited left-sided atrioventricular valve regurgitation during follow-up; 8 of them remained stable with medication only. Fifteen of the 30 patients with severe left-sided atrioventricular valve regurgitation underwent reoperation. A significant risk factor for the development of severe left-sided atrioventricular valve regurgitation and reoperation was the presence of preoperative severe left-sided atrioventricular valve regurgitation. Conclusions Severe left-sided atrioventricular valve regurgitation develops in a significant number of patients after correction of atrioventricular septal defect, and preoperative severe left-sided atrioventricular valve regurgitation is an important risk factor. Although reoperation usually results in good valve function, spontaneous regression after the immediate postoperative period is possible and should be given a fair chance.
- Published
- 2005
44. Neonatale anterovirusinfecties, een case report en nieuwe mogeljkheden voor diagnostiek en behandeling
- Author
-
Elink-Schuurman, BEE, Cromme-Dijkhuis, AH, Buysse, Corinne, Hartwig, Nico, Vossen, ACTM, Pediatrics, and Virology
- Published
- 2004
45. Pulmonary arterial wall distensibility assessed by intravascular ultrasound in children with congenital heart disease: an indicator for pulmonary vascular disease?
- Author
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Rolf M F, Berger, Adri H, Cromme-Dijkhuis, Wim C J, Hop, Marco N, Kruit, and John, Hess
- Subjects
Heart Defects, Congenital ,Pulmonary Circulation ,Adolescent ,Case-Control Studies ,Child, Preschool ,Pulsatile Flow ,Humans ,Vascular Resistance ,Vascular Diseases ,Pulmonary Artery ,Child ,Ultrasonography, Interventional - Abstract
Both pulmonary hypertension and pulmonary overflow are associated with functional and structural changes of the pulmonary arterial wall. Current techniques to evaluate the pulmonary vasculature neglect the pulsatile nature of pulmonary flow.To determine whether the dynamic properties of the pulmonary arterial wall are altered in patients with abnormal pulmonary hemodynamics due to congenital heart defects, and whether these changes are associated with the progression of pulmonary vascular disease (PVD).In 43 children with PVD due to congenital heart defects and 12 control subjects, pulmonary arterial pulsatility (the relative increase in vessel area during the cardiac cycle) and distensibility (the inverse of the stress/strain elastic modulus) were determined with intravascular ultrasound. Results were correlated with clinical and hemodynamic parameters.Pulsatility correlated with pulmonary pulse pressure (p0.001), pulmonary-to-systemic vascular resistance ratio (PVR/SVR) [p = 0.001], and hemoglobin concentration (p = 0.01). However, when corrected for these variables, pulsatility did not differ between patients and control subjects. In contrast, arterial wall distensibility decreased with the severity of PVD and correlated independently with pulmonary-to-systemic arterial pressure ratio (p0.001) and PVR/SVR (p = 0.03), and with hemoglobin concentration (p0.01). Adjusted for hemodynamic variables, distensibility was still decreased in patients with PVD compared to control subjects.These results demonstrate that pulmonary arterial wall distensibility is progressively decreased in PVD; moreover, this decreased distensibility is, in part, related to increased distending pressure as a result of pulmonary hypertension but also, in part, to stiffening of the arterial wall during the disease process. Arterial wall distensibility may be of additional value in the evaluation of pulmonary vasculature and ventricular workload.
- Published
- 2002
46. Growth hormone, good or not so good for the heart?
- Author
-
Cromme-Dijkhuis, AH and Pediatrics
- Published
- 2001
47. Beeldvorming in de kindercardiologie: nieuwe ontwikkelingen
- Author
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Cromme-Dijkhuis, AH, Helbing, W.A., Suijlekom - Smit, L.W.A., Pediatrics, and Cardiology
- Published
- 2000
48. Follow-up of AVSD
- Author
-
Cromme-Dijkhuis, AH, van Heukelum, A, Akkersdijk, GP (George), Bogers, Ad, den Hamer, I, Ebels, Tj, Bartelings, M.M., Wenink, A.C.G., Pediatrics, and Cardiothoracic Surgery
- Published
- 2000
49. The value of 2-D Doppler echocardiography in the evaluation of asymptomatic patients with Mustard operation for transposition of the great arteries
- Author
-
A. H. Cromme-Dijkhuis, M. Th. E. Bink-Boekens, M. Talsma, and M. Schasfoort-Van Leeuwen
- Subjects
Cardiac Catheterization ,medicine.medical_specialty ,Transposition of Great Vessels ,medicine.medical_treatment ,Ventricular outflow tract obstruction ,Doppler echocardiography ,Asymptomatic ,Postoperative Complications ,Tricuspid Valve Insufficiency ,Risk Factors ,Internal medicine ,Humans ,Medicine ,Heart Atria ,Child ,Mustard procedure ,Cardiac catheterization ,medicine.diagnostic_test ,business.industry ,Hemodynamics ,Transposition of the great vessels ,medicine.disease ,Echocardiography, Doppler ,Great arteries ,cardiovascular system ,Cardiology ,Radiology ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - Abstract
2-D Doppler echocardiography was used to assess the occurrence of haemodynamic abnormalities in 45 asymptomatic patients, aged 4 to 16 years (median 7.4) after a Mustard operation for transposition of the great arteries. The findings were compared with those derived from cardiac catheterization. Thirty-five cardiac lesions were correctly diagnosed by 2-D Doppler echocardiography in 23 patients, but on six occasions, minor abnormalities were missed. 2-D Doppler echocardiography demonstrated systemic venous pathway obstruction of more than 3 mmHg at cardiac catheterization in nine patients, and in five of the six patients with pulmonary venous channel obstruction. A left ventricular outflow tract obstruction (pressure difference greater than 15 mmHg) was diagnosed correctly by Doppler echocardiography in seven patients. Baffle leakage was found in two patients with a left to right shunt of 25% or more of pulmonary blood flow, but was missed in five out of nine patients with small shunts. Tricuspid regurgitation was well defined in eight patients. The absence of symptoms and a routine examination after a Mustard operation do not rule out haemodynamic abnormalities. However, these, with the possible exception of minor baffle leakage, can be detected by 2-D Doppler echocardiography.
- Published
- 1991
50. The effects of long-term growth hormone treatment on cardiac left ventricular dimensions and blood pressure in girls with Turner's syndrome. Dutch Working Group on Growth Hormone
- Author
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T C, Sas, A H, Cromme-Dijkhuis, S M, de Muinck Keizer-Schrama, T, Stijnen, A, van Teunenbroek, and S L, Drop
- Subjects
Dose-Response Relationship, Drug ,Echocardiography ,Human Growth Hormone ,Heart Ventricles ,Humans ,Turner Syndrome ,Blood Pressure ,Female ,Hypertrophy, Left Ventricular ,Child - Abstract
To assess the effects of long-term growth hormone (GH) treatment for short stature on left ventricular (LV) dimensions and systemic blood pressure (BP) in girls with Turner's syndrome without clinically relevant cardiac abnormalities.LV dimensions measured by echocardiography and systemic BP were assessed before and during 7 years of GH treatment in 68 girls with Turner's syndrome participating in a randomized dose-response study. These previously untreated girls, age 2 to 11 years, were randomly assigned to 1 of 3 GH dosage groups: group A, 4 IU/m(2)/d; group B, first year 4 IU/m(2)/d, thereafter 6 IU/m(2)/d; group C, first year 4 IU/m(2)/d, second year 6 IU/m(2)/d, thereafter 8 IU/m(2)/d. After the first 4 years, girls/=12 years of age began receiving 17beta-estradiol, 5 microg/kg body weight per day, for induction of puberty.At baseline the LV dimensions of almost every girl were within the normal range, and the mean SD scores were close to zero. During 7 years of GH treatment, the growth of the left ventricle was comparable to that of healthy girls. No signs of LV hypertrophy were found. Before the start of GH treatment, mean BP was within the normal range but significantly higher than in healthy control subjects. Diastolic BP and systolic BP were above the 90th percentile in 23% and 28% of the girls, respectively. After 7 years of treatment, these percentages were 14% and 36%, respectively (not significantly different from baseline). The SD score of the diastolic BP showed a small decrease after 7 years of treatment. The growth of the left ventricle and the development of BP were not different between the GH dosage groups.Long-term GH treatment, even at dosages up to 8 IU/m(2)/d, does not result in LV hypertrophy or hypertension in girls with Turner's syndrome. Continued observation into adulthood is recommended to monitor the further development of the relatively high BP and to ensure that GH treatment has no long-term negative effect on the heart.
- Published
- 1999
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