Yves d'Udekem

The Fontan Procedure Contemporary Techniques Have Improved Long-Term Outcomes

Background— To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection. Methods and Results— Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12±6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed. Conclusions— The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.

Long-term results of surgery for active infective endocarditis

OBJECTIVE This paper was undertaken to determine the long-term outcome of active infective endocarditis treated with antibiotic and radical excision of infected tissues by surgery. METHODS From October 1978 to August 1994, 122 consecutive patients were operated on during the acute phase of infective endocarditis. There were 85 men and 37 women whose mean age was 50 years, ranging from 20 to 79. Surgery was needed because of one or more of the following complications: cardiogenic/septic shock in 19 patients, congestive heart failure in 68, persistent sepsis in 64, peripheral embolization in 20, and cerebral embolization in 10. The offending microorganism was identified in 110 patients, staphylococci were the most common ones. Seventy-six patients had native valve endocarditis and 46 had prosthetic valve endocarditis. Simple valve replacement or repair was performed in 60 patients and radical resection of the valve and surrounding tissues with reconstruction of the heart with either fresh autologous pericardium or glutaraldehyde-fixed bovine pericardium was performed in 62 with paravalvular abscess. Pulmonary autograft and aortic homograft were used in only three patients, the remaining patients had either bioprostheses or mechanical heart valves if valve repair was not feasible. RESULTS There were nine deaths, for an operative mortality of 7.4%. Logistic regression analysis identified preoperative shock and renal failure as predictors of operative mortality. Operative survivors were followed up from 4 to 173 months, mean of 56.4. The actuarial survival at 10 years was 61 +/- 6%. Logistic regression analysis identified preoperative New York Heart Association functional class IV and perioperative renal failure as predictors of late mortality. Eight patients developed recurrent endocarditis 10-102 months postoperatively. The freedom from recurrent endocarditis at 10 years was 79 +/- 9%. All patients who developed this late complication had paravalvular abscess at the time of original operation. CONCLUSIONS These data suggest that surgery for active infective endocarditis yield a high probability of eradicating the infection with relatively low operative mortality and good long-term results.

Outcomes of the Arterial Switch Operation for Transposition of the Great Arteries: 25 Years of Experience

BACKGROUND Studies on long-term outcomes of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are uncommon. Thus, we sought to determine the long-term outcomes for patients after ASO performed at a single institution over a 25-year period. METHODS From 1983 to 2009, 618 patients underwent the ASO for TGA and were reviewed retrospectively. RESULTS Overall early mortality was 2.8%. Risk factors for early death on multivariate analysis were resection of left ventricular outflow tract obstruction at time of ASO (p = 0.001), weight less than 2.5 kg at time of ASO (p < 0.001), associated aortic arch obstruction (p = 0.043), and the need for postoperative extracorporeal membrane oxygenation (p < 0.001). Mean follow-up time was 10.6 years (range 2 months to 26.1 years). Late mortality was 0.9%. Reintervention was significantly higher (p < 0.001) in patients with ventricular septal defect or arch obstruction versus those without them (25.2% and 23.4% vs 5.9% at 15- year follow-up). Risk factors for late reintervention were left ventricular outflow tract obstruction at time of ASO (p < 0.001) and a greater circulatory arrest time (p < 0.001). Freedom from at least moderate neoaortic valve regurgitation for the entire cohort was 98.7% (95% confidence interval 96.8 to 99.5%) at 20 years. Mild neoaortic regurgitation was seen in 25.6% of patients at mean follow-up. All patients were free of arrhythmia and heart failure symptoms at last follow-up. CONCLUSIONS The ASO can be performed with good long-term results. Patients with associated ventricular septal defect and aortic arch obstruction warrant close follow-up.

Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance.

OBJECTIVES This study examined survival after surgical palliation in children with single-ventricle physiology. BACKGROUND Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear. METHODS In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states. RESULTS After 2000, the population comprised more patients with dominant right ventricle (66% vs. 36%) and hypoplastic left heart syndrome (HLHS) (47% vs. 13%). Median age at bidirectional cavopulmonary shunt (BCPS) decreased from 15 months (10 to 22 months) before 2000 to 4 months (3.3 to 9 months) thereafter. Survival rates at 1, 5, and 10 years were, respectively, 82% (95% confidence interval [CI]: 79% to 85%), 74% (95% CI: 70% to 78%), and 71% (95% CI: 67% to 75%). Throughout the study, atrioventricular valve regurgitation (hazard ratio [HR]: 1.8; p = 0.008), not having transposition (HR: 2.0; p = 0.013), and heterotaxia (HR: 2.0; p = 0.026) were predictors of mortality. The most potent risk factor was right ventricular (RV) dominance (HR: 2.2; p = 0.001) because of its impact before BCPS. HR for death in patients with RV dominance went from 2.8 (95% CI: 1.4 to 5.7; p = 0.005) before BCPS to 1.0 (95% CI: 0.5 to 2.1; p = 0.98) thereafter. Survival of patients with RV dominance, adjusted for the risk factors noted here, improved over the study period (p = 0.001). CONCLUSIONS Considerable mortality is still observed during the first years of life among patients with single ventricle. RV dominance is the most important risk factor for death but only before BCPS.

A three-stage intrathymic development pathway for the mucosal-associated invariant T cell lineage

Mucosal-associated invariant T cells (MAIT cells) detect microbial vitamin B2 derivatives presented by the antigen-presenting molecule MR1. Here we defined three developmental stages and checkpoints for the MAIT cell lineage in humans and mice. Stage 1 and stage 2 MAIT cells predominated in thymus, while stage 3 cells progressively increased in abundance extrathymically. Transition through each checkpoint was regulated by MR1, whereas the final checkpoint that generated mature functional MAIT cells was controlled by multiple factors, including the transcription factor PLZF and microbial colonization. Furthermore, stage 3 MAIT cell populations were expanded in mice deficient in the antigen-presenting molecule CD1d, suggestive of a niche shared by MAIT cells and natural killer T cells (NKT cells). Accordingly, this study maps the developmental pathway and checkpoints that control the generation of functional MAIT cells.

Surgical treatment of congenital mitral valve disease: Midterm results of a repair-oriented policy

OBJECTIVE Management of congenital mitral valve disease is challenging because of a wide morphologic spectrum, frequent associated lesions, and small patient size. We evaluated the results of a repair-oriented policy. METHODS All consecutive patients with congenital mitral valve disease who underwent surgery between 1996 and 2006 were studied retrospectively. Patients with atrioventricular canal, atrioventricular discordance, or ischemic regurgitation were excluded. RESULTS During this period, 71 children (median age 2.9 years, range 3 days-20.8 years) underwent surgery. All but 1 underwent primary mitral valve repair. Twenty-two (30%) were younger than 12 months. Associated cardiac lesions were present in 45 children (63%) and were addressed concurrently in 35; previous cardiac procedures had been performed in 17 patients (24%). Mitral incompetence was predominant in 60 (85%) and stenosis in 11 (15%). During a median follow-up of 47.8 months (range 2-120 months), 14 patients underwent 17 mitral reinterventions: 14 repairs and 3 replacements. After 60 months, overall survival was 94% +/- 2.8%; freedoms from reoperation and prosthesis implantation were 76% +/- 5.6% and 94% +/- 3.6%, respectively. There were 4 deaths, and all survivors remain in New York Heart Association class I or II with moderate (6 patients) or less mitral dysfunction. CONCLUSION Surgical repair of the congenital mitral valve can be successfully performed with low mortality, satisfactory valvular function at midterm follow-up, and acceptable reoperation rate while obviating risks associated with valvular prostheses. Suboptimal primary repair was significant predictor for reoperation but re-repair was often successful.

Initial results of primary device closure of large muscular ventricular septal defects in early infancy using perventricular access

Objectives: To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants. Background: Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants. Methods: Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent perventricular muscular VSD closure under transesophageal echo (TEE) guidance. Results: Eight infants underwent the procedure. The median age was 14 weeks (2–41) with median weight of 4 kg (3–6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4–10 mm). A single device (6–12 mm) was deployed in each patient, the size chosen being 1–2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5–66) post device implantation revealed no patients with a hemodynamically significant shunt. Conclusion: Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass.

Surgical valvotomy and repair for neonatal and infant congenital aortic stenosis achieves better results than interventional catheterization.

OBJECTIVES This study sought to compare outcomes after surgical valvuloplasty and balloon dilation of the aortic valve in neonates and infants. BACKGROUND Surgical techniques of aortic valve repair have improved and there is today controversy on the best approach to treat neonatal congenital aortic valve stenosis. METHODS Retrospective review of data and follow-up of 123 consecutive neonates and infants (35 females, 88 males) undergoing intervention for congenital aortic stenosis. RESULTS From 1977 to 2009, 123 consecutive neonates (<30 days) and infants (31 days to 1 year) underwent relief of congenital aortic stenosis. Median age at procedure was 27 days (6 to 76 days). Twenty-year survival was 80 ± 7%. Fifty-four patients required a re-intervention and freedom from re-intervention was 55 ± 6% at 10 years and 40 ± 6% at 20 years. By multivariate analysis, having the relief of stenosis by balloon valvuloplasty and undergoing initial treatment as a neonate were predictive of re-intervention. Freedom from re-intervention at 5 years was 27% after balloon valvuloplasty versus 65% after surgery. At latest follow-up, an additional 16 patients had moderate or severe stenosis and 8 had regurgitation. Freedom from re-intervention or stenosis was 39 ± 5% at 15 years. By multivariate analysis, balloon valvuloplasty (p < 0.001) and treatment as a neonate (p = 0.003) were again predictive of stenosis or re-intervention. Thirty-five patients ultimately needed a valve replacement. Significant predictor of the requirement of valve replacement was unicuspid aortic valve (p < 0.001). Freedom from valve replacement was 55 ± 7% at 20 years. CONCLUSIONS Surgical valvuloplasty remains the best approach to treat neonates and infants with congenital aortic stenosis. After surgery, a higher proportion of patients remain free of re-intervention than after interventional catheterization and the relief of their stenosis lasts longer.

The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes

OBJECTIVES To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients. METHODS All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed. RESULTS A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5). CONCLUSIONS The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent.

Outcomes of Surgery for Simple Total Anomalous Pulmonary Venous Drainage in Neonates

BACKGROUND Repair of total anomalous pulmonary venous drainage (TAPVD) in neonates remains a challenge as it is often associated with severe obstruction. We describe a large cohort of neonates who underwent TAPVD repair at a single institution. METHODS From 1973 to 2008, 112 patients underwent simple TAPVD surgery during the first month of life. Data collection occurred retrospectively. RESULTS Preoperative pulmonary venous obstruction (PVO) occurred in 89 (79.5%) patients. There were 12 (10.7%) early deaths. Significant risk factors were bypass time greater than 65 minutes (p=0.014) and emergent surgery (p=0.005). Hospital mortality was unchanged throughout the 3 eras (1973 to 1988, 1989 to 1998, 1999 to 2008), despite an increase in patients with preoperative acidosis (p=0.004) and severe TAPVD obstruction (p=0.038) during the recent 10 years. There were 6 (6.25%) late deaths within 2 years of repair. Survival at 20 years was 83.4% (95% confidence interval 75 to 89). Risk factors for late death were operative weight 2.5 kg or less (p=0.004) and postoperative pulmonary hypertensive crisis (p=0.02). Reoperation for recurrent PVO was required in 13 patients (11.9%). Risk factors were operative weight 2.5 kg or less (p=0.035) and postoperative pulmonary hypertensive crisis (p=0.002). Follow-up was 96% complete and survivors (n=90) were asymptomatic at a median age of 11.7 years. CONCLUSIONS Hospital mortality remained unchanged over the 36-year period. Survival beyond 2 years offers excellent outcome. Risk factors for mortality were the preoperative clinical status, prolonged bypass time, persisting micro-obstruction, and low operative weight. A reduction in mortality will likely require development of effective medical management for patients who have peripheral PVO not amenable to surgical repair.