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Early post-operative sinus node dysfunction (SND) is common in paediatric patients undergoing surgical correction of congenital heart defects (CHD). At present, the pathophysiology of these arrhythmias is incompletely understood. In this case series, we present three paediatric patients in whom we performed intraoperative epicardial mapping and who developed early post-operative SND. All patients had either an inferior or multiple sinoatrial node (SAN) exit sites, in addition to extensive conduction disorders at superior and inferior right atrium. Our findings contribute to the hypothesis that pre-existing alterations in SAN exit sites in combination with atrial conduction disorders may predispose paediatric patients with CHD for early post-operative SND. Such insights in the development of arrhythmias are crucial as it may be the first step in identifying high-risk patients.

Patients with congenital heart disease (CHD) are prone to develop atrial and ventricular arrhythmias. Multiple factors throughout life contribute to arrhythmogenicity substrate such as (i) (longstanding) volume and/or pressure overload, (ii) scar tissue, (iii) ageing-related structural remodelling, (iv) cardiovascular risk factors and (v) tachycardia-induced remodelling. At present, it is unknown whether, and to what extent, paediatric patients with CHD have atrial or ventricular conduction disorders early in life and whether there is a correlation between duration of volume/pressure overload and extensiveness of conduction disorders. To investigate this, we initiated high-resolution intraoperative epicardial mapping in paediatric patients with CHD undergoing primary open-heart surgery.

This study sought to investigate whether pediatric patients with congenital heart disease (CHD) already have atrial conduction disorders early in life. The authors conducted first-in-children epicardial mapping in 10 pediatric patients with CHD undergoing primary open heart surgery. Areas of conduction delay (CD) and block (CB) were present in all patients and were particularly observed at Bachmann's bundle (CD: 4.9%; CB: 2.3%), followed by the right atrium (CD: 3.7%; CB: 1.6%) and, to a lesser degree, the left atrium (CD: 1.8%; CB: 1.0%). Conduction abnormalities may by aggravated over time (e.g., aging, residual lesions, or valvular dysfunction), predisposing these patients to atrial arrhythmias early in life.

BACKGROUND: The aim of this study is to evaluate the effects of minimal access mitral valve surgery (MAMVS) versus conventional surgery with or without concomitant tricuspid valve plasty (TVP) in consecutive patients with mitral regurgitation (MR) on clinical and echocardiographic outcome. METHODS: One-hundred-and-twenty patients operated for MR (91 conventional and 29 MAMVS) were followed by echocardiography and quality of life assessment before and 6 months after surgery. RESULTS: Patients in the MAMVS group were younger, more often in NYHA functional class I-II and had lower NT-proBNP levels. Only four patients (all in the conventional group) underwent mitral valve replacement. There were no significant differences in complications between MAMVS and conventional surgery. At 6 months, comparable MR reduction and left ventricular remodeling data were seen, left atrial remodeling was most prominent in the MAMVS group, 71 [55-90] to 43 [35-58] versus 69 [53-89] to 49 [41-70] mL/m2 in the conventional group (P

Background. The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses.Methods. A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated.Results. Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]).Conclusions. Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk. (C) 2020 by The Society of Thoracic Surgeons. Published by Elsevier Inc.

Background There is an ever-growing number of patients requiring aortic valve replacement (AVR). Limited data is available on the long-term outcomes and structural integrity of bioprosthetic valves in younger patients undergoing surgical AVR. Methods The INSPIRIS RESILIA Durability Registry (INDURE) is a prospective, open-label, multicentre, international registry with a follow-up of 5 years to assess clinical outcomes of patients younger than 60 years who undergo surgical AVR using the INSPIRIS RESILIA aortic valve. INDURE will be conducted across 20–22 sites in Europe and Canada and intends to enrol minimum of 400 patients. Patients will be included if they are scheduled to undergo AVR with or without concomitant root replacement and/or coronary bypass surgery. The primary objectives are to 1) determine VARC-2 defined time-related valve safety at one-year (depicted as freedom from events) and 2) determine freedom from stage 3 structural valve degeneration (SVD) presenting as morphological abnormalities and severe haemodynamic valve degeneration at 5 years. Secondary objectives include the assessment of the haemodynamic performance of the valve, all stages of SVD, potential valve-in-valve procedures, clinical outcomes (in terms of New York Heart Association [NYHA] function class and freedom from valve-related rehospitalisation) and change in patient quality-of-life. Discussion INDURE is a prospective, multicentre registry in Europe and Canada, which will provide much needed data on the long-term performance of bioprosthetic valves in general and the INSPIRIS RESILIA valve in particular. The data may help to gather a deeper understanding of the longevity of bioprosthetic valves and may expand the use of bioprosthetic valves in patients under the age of 60 years. Trial registration ClinicalTrials.gov identifier: NCT03666741 (registration received September, 12th, 2018).

Coarctation of aorta(CoA), complicated by endarteritis in a children is very rare. Here we present a case of endarteritis in an unoperated CoA in a four year old boy. CoA had been diagnosed in the referring hospital, yet the diagnosis of endocarditis distal to CoA, was made in the tertiary center using modified transthoracic echo windows or focused views. After six weeks of intravenous antibiotic treatment, a coarctectomy and end‐to‐end anastomosis was performed and he recovered clinically well. This case report concludes that echocardiography remains as the standard diagnostic method for identifying intracardiac manifestations of infective endocarditis/endarteritis. Last but foremost, it delineates the importance of modified transthoracic echo windows or focused views in identifying the unusual position of endocarditis.

There is no published evidence on pregnancy after right ventricular outflow tract (RVOT) reconstruction with an allograft.The aim of this study was to describe pregnancy outcomes in women with allografts in the RVOT position.A retrospective cohort study of consecutive female patients who received allografts in the RVOT position was conducted. All patients between 18 and 50 years of age were screened for cardiac, obstetric, and fetal outcomes of completed (≥20 weeks' gestation) pregnancies.In total, 196 women met the inclusion criteria, of whom 56 had 89 completed pregnancies. Information could be retrieved in 84 cases (94.4%). Mean maternal age was 29.6 ± 4.3 years, with 80 patients (95.2%) in New York Heart Association functional class I or II. The most common diagnosis was tetralogy of Fallot. All women survived pregnancy. There were 2 cases (2.4%) of heart failure (arrhythmic and diastolic dysfunction), 1 case (1.2%) of infection (chorioamnionitis), and 3 cases (3.6%) of pre-eclampsia. No other cardiac or obstetric events were reported. All children were born alive at a median gestational age of 38.4 weeks (interquartile range: 36.9 to 39.6 weeks), with a median birthweight of 2,930 g (interquartile range: 2,535 to 3,385 g). Seventeen (20.2%) were small for gestational age, and 20 (23.8%) were premature. Neonatal death was reported in 2 children (2.5%). Preconception pulmonary regurgitation was associated with an increased probability of pre-term labor (odds ratio: 2.610; 95% confidence interval: 1.318 to 5.172). Compared with the general Dutch population, pre-term delivery (25.0% vs. 7.4%, p 0.001) and children small for gestational age (20.2% vs. 10.0%, p = 0.002) were more common.Women in good cardiac health after RVOT reconstruction with allografts can safely experience pregnancy and labor. The higher incidence of pre-term delivery and children small for gestational age warrants special attention.

There is very limited published evidence about the influence of pregnancy on allograft durability in right ventricular outflow tract position. We present the first study using mixed and joint modeling.This retrospective study compared clinical and valve related outcomes of all consecutive female hospital survivors in their fertile life phase (18-50 years) based on pregnancy. Serial echocardiographic measurements of pulmonary gradient and regurgitation were analyzed for their association with valve replacement using joint models for longitudinal and time-to-event data. Occurrence of first pregnancy was included as a time-dependent intermediate event in both the longitudinal and survival analyses of the joint model to assess its impact on the hemodynamic and clinical outcome.In total, 196 consecutive women in their fertile life-phase with an allograft were included. Complete information of 176 (90%) allografts in 165 women was available, including 1395 echocardiograms. Of these women, 51 (30.9%) women had 84 completed pregnancies at an average age of 29.1 ± 3.9 (SD) years; 8.1 ± 6.1 years since allograft implantation. Tetralogy of Fallot was the most common diagnosis in both groups. After a mean follow-up of 15.2 years (range 0.1-30), 7 (13.7%) parous women underwent valve replacement versus 20 (17.5%) nulliparous women. During this follow-up, the mean allograft gradient in parous (24.2 mm Hg) and nulliparous (21.0 mm Hg) women was comparable (P = .225). A 1-mm Hg increase in pulmonary gradient increased the instantaneous risk of pulmonary valve replacement (PVR) by a ratio of 1.051 (P .001), regardless of pregnancy. Similarly, development of moderate or severe regurgitation increased the risk of PVR (P = .038), regardless of pregnancy. Pregnancy was not associated with a change in the allograft gradient (P = .258), regurgitation grade (P = .774), or hazard of PVR (P = .796) during follow-up.Pregnancy is not associated with impaired allograft durability in women with good cardiac health.

Mechanical complications are rare, however dreaded, and potentially lethal consequences of acute myocardial infarction (AMI). They manifest as a tear (rupture) of the necrotic myocardium with ventricular septal rupture (VSR), free wall rupture, and/or ischemic mitral regurgitation (IMR) as a consequence. The introduction and routine use of primary reperfusion therapies such as thrombolysis and especially primary PCI in the management of AMI have reduced the incidence of excessive myocardial necrosis and the subsequent rupture of the acutely ischemic myocardium. However clinicians can still expect to encounter this life-threatening entity in a minority (around 1%) of patients following AMI. As survival of medically treated patients is extremely poor, diagnosis of an acute/subacute event requires urgent surgical referral, and in most cases necessitates life-saving emergency surgery. Mortality after surgical treatment, however, is also high, rendering decision-making in this condition often complex and not always straightforward. Features of different mechanical complications are discussed in this chapter, but all combinations can be encountered in clinical practice.

Propionibacterium acnes, a common human skin organism [Perry A.L., Lambert P.A., Under the microscope Propionibacterium acnes, Lett App Microbiol 2006; 42:185-186], mostly considered a contaminant, has rarely been associated with cases of infectious endocarditis [Vanagt W.Y., Daenen W.J., Delhaas T., Propionibacterium acnes endocarditis on an annuloplasty ring in an adolescent boy, Heart 2004; 90:56]. We report on a 48-year old man with a history of mitral valve replacement who acutely developed dyspnoea, tachypnoea and forward failure. Transesophageal echocardiography showed a dehiscence of the St. Jude mechanical mitral prosthesis necessitating urgent surgery. The prosthesis was replaced by another St. Jude mechanical valve. Blood cultures were initially negative but after a prolonged incubation period the tissue cultures became positive for Propionibacterium acnes. In cases of valvular dehiscence without macroscopic signs of endocarditis, communication between clinicians and the laboratory is important in order to incubate blood and tissue samples for a longer period of time to be able to detect exceptional causes of endocarditis.

OBJECTIVES: Guidelines recommend surgical mitral valve repair in selected patients with asymptomatic severe mitral valve regurgitation (MR), but the role of repair remains a matter of debate. Survival analyses of operated asymptomatic patients have been reported, but long-term haemodynamics and quality of life are not well defined. The aim of this study was to report the long-term follow-up focusing on these aspects. METHODS: Our database identified patients who underwent primary isolated mitral valve repair for severe MR and were asymptomatic by New York Heart Association Class I and in sinus rhythm. To obtain sufficient length of follow-up, only patients operated on before 2006 returned for an echocardiogram and quality-of-life assessment (SF-36). RESULTS: Between May 1991 and December 2005, 46 asymptomatic patients with severe MR and a normal left ventricular function (ejection fraction >60%) were operated on. Mean age was 50.2 ± 13.2 years and 89% of patients were male. There were no operative deaths. Mean follow-up was 8.4 ± 3.9 years with 386 patient-years, survival was 93.3% at 12 years and comparable with the general age-matched Dutch population. Follow-up echocardiography showed that 92% had no to mild MR, and 3 patients had moderate MR. Left ventricular function was good/impaired/moderate in 66/29/5% of patients. Quality-of-life SF-36 assessment showed that mean physical and mental health components were 83 ± 17 and 79 ± 17, which was comparable with that of the general age- and gender-matched Dutch population. CONCLUSIONS: Our experience shows that mitral valve repair for severe MR in asymptomatic patients is safe, and has satisfactory long-term survival with a low recurrence rate of MR, good left ventricular function, and excellent quality of life that is comparable with the general Dutch population.

The optimal surgical strategy for the management of ischemic mitral regurgitation (IMR) is still debated. The purpose of this study was to perform a meta-analysis summarizing the evidence favoring one technique over another (repair vs replacement). A search of the English literature in PubMed was performed using 'ischemic mitral regurgitation' and 'repair or replacement or annuloplasty' in the title/abstract field. Articles were excluded if they lacked a direct comparison of repair versus replacement, or used Teflon/pericardial strip or suture annuloplasty in10% of the repairs. Nine articles were selected for the final analysis. All studies except one were relatively recent (2004-2009). The patient characteristics between treatment groups were similar across studies. All studies excluded patients with degenerative etiology and used a rigorous definition of IMR. Most patients had concomitant coronary artery bypass graft. In the patients with mitral valve replacement, at least the posterior and, in many cases, the entire subvalvular apparatus were preserved. Mean ejection fraction and proportion of patients with severe ventricular dysfunction were similar between the repair and replacement groups. The odds ratios for the studies, comparing replacement to repair, ranged from 0.884 to 17.241 for short-term mortality and the hazard ratios ranged from 0.677 to 3.205 for long-term mortality. There was a significantly increased likelihood of both short-term mortality (summary odds ratio 2.667 (95% confidence interval (CI) 1.859-3.817)) and long-term mortality (summary hazard ratio 1.352 (95% CI 1.131-1.618)) for the replacement group compared to the repair group. Based on the meta-analysis of the current relevant literature, mitral valve repair for IMR is associated with better short-term and long-term survival compared to mitral valve replacement. Our conclusion should be interpreted in the context of the inherent limitations of a meta-analysis of retrospective studies including heterogeneity of patient characteristics, which may have influenced the physician's decision to perform mitral valve repair or replacement. In the absence of any published randomized studies, mitral procedure selection should be individualized.