Risk of infective endocarditis after hybrid melody mitral valve replacement in infants: the French experience.

OBJECTIVES: Surgical management of mitral valve disease is challenging in infants <1 year old. We aimed at reviewing the French experience with Melody mitral valve replacement in critically ill infants. METHODS: A retrospective cohort study reporting the French experience with Melody mitral valve replacement. RESULTS: Seven symptomatic infants [complete atrioventricular septal defect (n = 4, Down syndrome: n = 3), hammock valve (n = 3)] underwent Melody mitral valve replacement [age: 3 months (28 days to 8 months), weight: 4.3 kg (3.2-6.4 kg)] because of severe mitral valve regurgitation (6) or mixed valve disease (1) and 14 mm (11-16 mm) mitral valve annulus. In 2 patients whose valve was felt irreparable, Melody mitral valve replacement was performed straightaway. The others underwent 2 (1-3) previous attempts of valve repair; 3 were on extracorporeal membrane oxygenation. Melody mitral valve replacement led to competent valve and low gradient [3 mmHg, (1-4 mmHg)]. One patient died 3 days post-implant from extracorporeal membrane oxygenation-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, 1 underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical mitral valve replacement at 2 years; another is currently planned for transcatheter Melody valve dilation. CONCLUSIONS: Melody mitral valve replacement may be considered in selected infants with small mitral valve annulus as an alternative to mechanical mitral valve replacement. Our experience highlights a high-risk of late infective endocarditis that deserves further consideration.

Post-ligation cardiac syndrome after surgical versus transcatheter closure of patent ductus arteriosus in low body weight premature infants: a multicenter retrospective cohort study.

Transcatheter patent ductus arteriosus (PDA) closure is a safe and effective alternative to surgical ligation in low-body-weight infants. Post-ligation cardiac syndrome (PLCS) is defined as severe hemodynamic and respiratory collapse within 24 h of PDA closure, requiring initiation or an increase of an inotropic agent by > 20% of preligation dosing and an absolute increase of at least 20% in ventilation parameters compared with the preoperative value. Whilst PLCS is routinely observed after surgery, its incidence remains poorly described following transcatheter closure. This study aimed to compare the incidence of PLCS after surgical versus transcatheter closure of PDA in low-body-weight premature infants. Propensity scores were used to compare surgical (N = 78) and transcatheter (N = 76) groups of preterm infants who underwent PDA closure at a procedural weight less than 2000 g in two tertiary institutions between 2009 and 2021. The primary outcome was the incidence of PLCS. Secondary outcomes included overall mortality before discharge, risk factors for PLCS, and post-procedural complications. Procedural success was 100% in both groups. After matching, transcatheter group experienced no PLCS vs 15% in the surgical group (p = 0.012). Furthermore, overall mortality (2% vs 17%; p = 0.03) and major complications (2% vs 23%; p = 0.002) were higher in the surgical group. Surgery (100% vs 47%; p < 0.01), gestation age (25 ± 1 vs 26 ± 2 weeks, p < 0.05) and inotropic support before closure (90% vs 29%; p < 0.001) were associated with PLCS occurrence.          Conclusion: Transcatheter PDA closure may be equally effective but safer than surgical PDA closure in low-body-weight premature infants. What is Known: • Post-ligation cardiac syndrome is a serious and common complication of surgical closure of the ductus arteriosus in preterm infants. • Transcatheter closure of preterm ductus arteriosus is a safe and effective technique that is becoming more and more common worldwide. What is New: • Device closure is safer than surgical ligation for patent ductus arteriosus closure in preterm infants and may be the first-line non-pharmacological therapeutic option in this indication in experienced teams. • Our findings should encourage neonatologists and pediatric cardiologists to start and/or strengthen a durable interventional program for transcatheter PDA closure in premature infants.

Transcatheter Closure of Superior Sinus Venosus Defects.

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes.

Effectiveness of intraoperative use of dexmedetomidine in reducing the incidence of tachyarrhythmia after congenital cardiac surgery in neonates and infants: a doubly robust method estimation analysis.

OBJECTIVES: The antiarrhythmic effects of dexmedetomidine (DEX) have been suggested, but there are controversial reports on the effectiveness of intraoperative use of DEX to reduce the incidence of postoperative tachyarrhythmia (POT). METHODS: From a local European Congenital Heart Surgery Association database, we included patients operated for congenital heart diseases under cardiopulmonary bypass within a 5-year period (2017-2021), during which intraoperative use of high dose of DEX (1-1.4 µg/kg/h) was implemented. A doubly robust matching estimation of the causal effect of DEX on the incidence of POT was conducted. We combined a multimodal estimation model in patients not exposed to DEX (disease risk score) as well as a regression analysis in a matched cohort for patients exposured to DEX. RESULTS: From a cohort of 593 surgeries (514 patients) occurring during the study period, doubly matched analysis consisted of the analysis of 426 surgeries conducted under DEX or not (213 per group). The probability of developing POT in patients exposed to DEX was 6.6% (95% confidence interval 0.032-0.099) vs 14.5% (95% confidence interval 0.098-0.193) in the group of patients not exposed to DEX. The doubly robust matched estimation method showed a mean reduction of 8.8% (95% confidence interval -0.137 to -0.023) of POT when DEX is used for intraoperative anaesthesia. CONCLUSIONS: The use of high doses of DEX during anaesthesia for congenital heart surgery in neonates and infants is associated with a moderate but significant reduction of POT.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

Health-related quality of life and physical activity in children with inherited cardiac arrhythmia or inherited cardiomyopathy: the prospective multicentre controlled QUALIMYORYTHM study rationale, design and methods.

BACKGROUND: Advances in paediatric cardiology have improved the prognosis of children with inherited cardiac disorders. However, health-related quality of life (QoL) and physical activity have been scarcely analysed in children with inherited cardiac arrhythmia or inherited cardiomyopathy. Moreover, current guidelines on the eligibility of young athletes with inherited cardiac disorders for sports participation mainly rely on expert opinions and remain controversial. METHODS: The QUALIMYORYTHM trial is a multicentre observational controlled study. The main objective is to compare the QoL of children aged 6 to 17 years old with inherited cardiac arrhythmia (long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, or arrhythmogenic right ventricular dysplasia), or inherited cardiomyopathy (hypertrophic, dilated, or restrictive cardiomyopathy), to that of age and gender-matched healthy subjects. The secondary objective is to assess their QoL according to the disease's clinical and genetic characteristics, the level of physical activity and motivation for sports, the exercise capacity, and the socio-demographic data. Participants will wear a fitness tracker (ActiGraph GT3X accelerometer) for 2 weeks. A total of 214 children are required to observe a significant difference of 7 ± 15 points in the PedsQL, with a power of 90% and an alpha risk of 5%. DISCUSSION: After focusing on the survival in children with inherited cardiac disorders, current research is expanding to patient-reported outcomes and secondary prevention. The QUALIMYORYTHM trial intends to improve the level of evidence for future guidelines on sports eligibility in this population. Trial registration ClinicalTrials.gov Identifier: NCT04712136, registered on January 15th, 2021 ( https://clinicaltrials.gov/ct2/show/NCT04712136 ).

Improved ventilation in premature babies after transcatheter versus surgical closure of patent ductus arteriosus.

AIMS: Patent ductus arteriosus (PDA) is common in preterm infants and can contribute to morbidity and mortality. We aimed to compare results and outcome of transcatheter closure using the Amplatzer Piccolo Occluder versus surgical closure in 2 matched groups of preterm infants weighing <3000 g. METHODS AND RESULTS: A total of 147 babies from three tertiary centres were retrospectively analysed. Sixty-four babies undergoing catheter closure were compared with 83 matched surgical cases. Patent ductus arteriosus closure was successful in all cases. During neonatal unit course, mortality was 6.3% (n = 4) after catheterization and 12% (n = 10) after surgery (p = 0.24). Median duration of mechanical ventilation was shorter after catheterisation than after surgery (3 vs 5 days, p = 0.035). Before 4 weeks of age the difference between transcatheter and surgical closure for mechanical ventilation was even more pronounced (3 vs 9 days, p = 0.022). Additionally, when catheterisation was performed before 4 weeks, babies were discharged home earlier as compared to those who underwent closure later in life (39+1 vs. 42+1 weeks, p = 0.021). Such difference was not found in the surgical group. CONCLUSIONS: Transcatheter closure of patent ductus arteriosus is safe, effective and is associated with shorter mechanical ventilation than after surgery. Hospital stay might be shorter when performed earlier in life.

Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Presenting in Adulthood: a French Nationwide Retrospective Study.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease usually diagnosed during the first months of life. Without surgical treatment, ALCAPA carries a high mortality risk, and disease presentation in adulthood is rare. We describe the diagnosis and management of patients presenting with ALCAPA in adulthood. This multicenter French nationwide retrospective study included adult patients diagnosed from 1980 to 2014. Eleven adult patients (mean age: 38 ± 17 years) were analyzed. All patients were symptomatic, presenting with chest pain, palpitations, heart failure, or syncope. Electrocardiogram was abnormal in 8 (73%) patients. Echocardiogram showed a mildly depressed left ventricular ejection fraction of 50 ± 13%, kinetic abnormalities in 5 (45%) patients, and significant mitral regurgitation in 8 (73%) patients. Coronary angiography was performed in 10 (91%) patients and confirmed the diagnosis. Computerized tomography scan, magnetic resonance imaging, and myocardial scintigraphy were performed when deemed necessary. Ten patients underwent reconstructive surgery, but 1 patient was not operated because of age. Four patients experienced postoperative complications including cardiogenic shock, heart failure, renal failure, or additional surgery. After a median follow-up of 2.5 years, all 10 operated patients were alive and asymptomatic, and the nonoperated patient had died at the age of 70 from syncope related to ventricular tachycardia. ALCAPA may be diagnosed in adults. Although complications may occur postoperatively, long-term outcome is favorable in adult patients undergoing surgical correction. Surgery should be discussed as first-line therapy in adults with ALCAPA.

Loss-of-Function KCNE2 Variants: True Monogenic Culprits of Long-QT Syndrome or Proarrhythmic Variants Requiring Secondary Provocation?

BACKGROUND: Insight into type 6 long-QT syndrome (LQT6), stemming from mutations in the KCNE2-encoded voltage-gated channel ß-subunit, is limited. We sought to further characterize its clinical phenotype. METHODS AND RESULTS: Individuals with reported pathogenic KCNE2 mutations identified during arrhythmia evaluation were collected from inherited arrhythmia clinics and the Rochester long-QT syndrome (LQTS) registry. Previously reported LQT6 cases were identified through a search of the MEDLINE database. Clinical features were assessed, while reported KCNE2 mutations were evaluated for genotype-phenotype segregation and classified according to the contemporary American College of Medical Genetics guidelines. Twenty-seven probands possessed reported pathogenic KCNE2 mutations, while a MEDLINE search identified 17 additional LQT6 cases providing clinical and genetic data. Sixteen probands had normal resting QTc values and only developed QT prolongation and malignant arrhythmias after exposure to QT-prolonging stressors, 10 had other LQTS pathogenic mutations, and 10 did not have an LQTS phenotype. Although the remaining 8 subjects had an LQTS phenotype, evidence suggested that the KCNE2 variant was not the underlying culprit. The collective frequency of KCNE2 variants implicated in LQT6 in the Exome Aggregation Consortium database was 1.4%, in comparison with a 0.0005% estimated clinical prevalence for LQT6. CONCLUSIONS: On the basis of clinical phenotype, the high allelic frequencies of LQT6 mutations in the Exome Aggregation Consortium database, and absence of previous documentation of genotype-phenotype segregation, our findings suggest that many KCNE2 variants, and perhaps all, have been erroneously designated as LQTS-causative mutations. Instead, KCNE2 variants may confer proarrhythmic susceptibility when provoked by additional environmental/acquired or genetic factors, or both.

Progressive Atrial Conduction Defects Associated With Bone Malformation Caused by a Connexin-45 Mutation.

BACKGROUND: Inherited cardiac conduction disease is a rare bradyarrhythmia associated with mutations in various genes that affect action potential propagation. It is often characterized by isolated conduction disturbance of the His-Purkinje system, but it is rarely described as a syndromic form. OBJECTIVES: The authors sought to identify the genetic defect in families with a novel bradyarrhythmia syndrome associated with bone malformation. METHODS: The authors genetically screened 15 European cases with genotype-negative de novo atrioventricular (AV) block and their parents by trio whole-exome sequencing, plus 31 Japanese cases with genotype-negative familial AV block or sick sinus syndrome by targeted exon sequencing of 457 susceptibility genes. Functional consequences of the mutation were evaluated using an in vitro cell expression system and in vivo knockout mice. RESULTS: The authors identified a connexin-45 (Cx45) mutation (p.R75H) in 2 unrelated families (a de novo French case and a 3-generation Japanese family) who presented with progressive AV block, which resulted in atrial standstill without ventricular conduction abnormalities. Affected individuals shared a common extracardiac phenotype: a brachyfacial pattern, finger deformity, and dental dysplasia. Mutant Cx45 expressed in Neuro-2a cells showed normal hemichannel assembly and plaque formation. However, Lucifer yellow dye transfer and gap junction conductance between cell pairs were severely impaired, which suggested that mutant Cx45 impedes gap junction communication in a dominant-negative manner. Tamoxifen-induced, cardiac-specific Cx45 knockout mice showed sinus node dysfunction and atrial arrhythmia, recapitulating the intra-atrial disturbance. CONCLUSIONS: Altogether, the authors showed that Cx45 mutant p.R75H is responsible for a novel disease entity of progressive atrial conduction system defects associated with craniofacial and dentodigital malformation.

Long-term outcomes of the arterial switch operation for transposition of the great arteries and ventricular septal defect and/or aortic arch obstruction.

OBJECTIVES: Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be clarified. METHODS: A retrospective study was conducted in patients operated on between 1982 and 1998. Overall 220 postoperative survivors, 79.1% with a ventricular septal defect, 13.2% with multiple ventricular septal defects, and 29.1% with aortic arch obstruction, were followed for 17 years (0-28 years). RESULTS: The conditional survival rate was 96.7% [95% confidence interval (CI): 94.4-99.1] at 25 years. Late sudden death occurred in 2 asymptomatic patients. The cumulative incidence rate of death or reinterventions was 3.8% (95% CI: 2.9-4.8) at 25 years, with age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. The cumulative incidence rate of neoaortic regurgitation was 41.6% (95% CI: 20.5-62.8) at 25 years with an aorto-pulmonary diameter mismatch at the time of the ASO, age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. At the last follow-up, 53 patients (24.1%) had neoaortic root dilatation with an aortic sinus z-score ≥3 and 6 of them had a Bentall operation at a median delay of 14.1 years since the ASO. The only independent factors for neoaortic root dilatation were male sex and an aorto-pulmonary diameter mismatch at the time of the ASO. CONCLUSIONS: Despite a continual rate of reinterventions, long-term survival and cardiovascular outcome are excellent after ASO for complex TGA. Dilatation of the neoaortic root and neoaortic regurgitation may be observed with time and 2 late sudden deaths occurred, justifying a close follow-up in all patients.

Cardiac imaging of congenital heart diseases during interventional procedures continues to evolve: Pros and cons of the main techniques.

Cardiac catheterization has contributed to the progress made in the management of patients with congenital heart disease (CHD). First, it allowed clarification of the diagnostic assessment of CHD, by offering a better understanding of normal cardiac physiology and the pathophysiology and anatomy of complex malformations. Then, it became an alternative to surgery and a major component of the therapeutic approach for some CHD lesions. Nowadays, techniques have evolved and cardiac catheterization is widely used to percutaneously close intracardiac shunts, to relieve obstructive valvar or vessel lesions, and for transcatheter valve replacement. Accurate imaging is mandatory to guide these procedures. Cardiac imaging during catheterization of CHD must provide accurate images of lesions, surrounding cardiac structures, medical devices and tools used to deliver them. Cardiac imaging has to be 'real-time' with an excellent temporal resolution to ensure 'eyes-hands' synchronization and 'device-target area' accurate positioning. In this comprehensive review, we provide an overview of conventional cardiac imaging tools used in the catheterization laboratory in daily practice, as well as the effect of recent evolution and future imaging modalities.

Late outcomes after arterial switch operation for Taussig-Bing anomaly.

OBJECTIVE: To assess the long-term results of the arterial switch operation (ASO) for Taussig-Bing Anomaly (TBA) and identify risk factors affecting outcomes. METHODS: Retrospective review and late follow-up was performed for all TBA patients from 1997 to 2010 (follow-up >3 years). Selection criteria included the absence of mitro-pulmonary continuity. RESULTS: Sixty-nine children underwent ASO at a median age of 24 days (interquartile range [IR] 11-125), with concomitant repair of aortic arch obstruction in 26 (37.7%). Complex coronary anatomy (n = 38; 55.0%) was common. Nine (13.0%) patients had staged repair. Hospital mortality was 5.8% (95% confidence interval [CI], 1.6%-14.2%; n = 4). Median follow-up was 11.2 years (IR 7.2-13.8). Subsequent mortality was confined to the first postoperative year (n = 5, 86% [95% CI, 78%-95%]), 1-, and 10-year survival). Overall mortality was related to coronary pattern (Yacoub types C and E vs A and D, multivariate, hazard ratio [HR] 12.2 [95% CI, 1.2-122.1], P = .03). At latest follow-up, 96% of the survivors are asymptomatic, with normal ventricular function. Cumulative incidence of reintervention at 10 years was 53% (95% CI, 28%-77%). Concomitant aortic arch obstruction was a predictor of reintervention (multivariate, HR 2.9 [95% CI, 1.1-7.4], P = .03). No mortality occurred upon reinterventions. CONCLUSIONS: In the largest series to date of ASO for TBA, mortality is confined to the first postoperative year, and related to coronary artery pattern. Beyond the first year, needed reinterventions are frequent, but with sustained functional status and no mortality over >10 years follow-up. Aortic arch obstruction is the main predictor for reintervention. Despite a significant rate of early events, favorable long-term outcomes argue for use of the ASO in TBA patients.

Closure of tubular patent ductus arteriosus with the Amplatzer Vascular Plug IV: feasibility and safety.

BACKGROUND: Closure of tubular patent ductus arteriosus remains a challenging procedure. Anecdotal use of Amplatzer Vascular Plug IV for tubular ductus closure has been reported but feasibility and safety in a consecutive patients' series remain unknown. METHODS: We performed a monocenter prospective study at the Marie Lannelongue Hospital in Paris, France. From 2009 to 2014, a total of 47 patients (39 infants, 3 children, and 5 adults) underwent ductus closure with the Plug IV. RESULTS: Ductus morphology was a type E in 34 (72.3%) patients and a type C in 13 (27.7%) patients. Ductus closure occurred in 39 (83.0%) infants at a median age of seven months (range: 3-23 months) and a median weight of 6.9 kg (range: 4.1-17.0 kg). A past history of prematurity and very low birth weight was found in 33 (70.2%) of them. Twelve (25.5%) patients had pulmonary hypertension. Mean Plug IV diameter was 1.9 ± 0.1 mm larger than the mean maximal ductus diameter. Early complete closure of the ductus was obtained in all patients. Early migration of an undersized Plug IV occurred in one (2.1%) patient and was suitable for percutaneous device retrieval. After a mean follow-up of 3.4 ± 1.4 years, all patients are alive and asymptomatic, no late complication occurred. CONCLUSION: Transcatheter closure of tubular ductus with the Amplatzer Vascular Plug IV can be safe and effective, with a 100% early occlusion rate. This device, suitable for a 4F sheath, is a new alternative for tubular ductus closure in low-body-weight infants.

Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients.

OBJECTIVES: Palliative Potts shunt has been proposed in children with suprasystemic pulmonary arterial hypertension (PAH). METHODS: A retrospective multicentre study was performed to assess short- and long-term outcomes after Potts shunt. RESULTS: From 2003 to 2014, 24 children underwent a Potts shunt [19 surgical, median age: 7.7 years (1.5-17 years), median weight: 19.5 kg (10.2-47 kg) and 5 transcatheter, median age: 8.1 years (2.3-9.7 years), median weight: 22 kg (12.5-31 kg)] for drug-refractory PAH. For the first time in humans, we performed an unidirectional valved Potts anastomosis in a child with infrasystemic PAH on intravenous epoprostenol who experienced repeated central line infections. Severe postoperative complications occurred in 6 patients (25.0%, all from the surgical group) including 3 early deaths (12.5%) related to low cardiac output. After a median follow-up (FU) of 2.1 years (range, 3 months to 14.3 years, ≥8 years in 7 patients), World Health Organization (WHO) functional class was dramatically improved in the 21 survivors, all being in WHO-functional class 1 or 2 (P < 0.05); none experienced syncope during the FU; none had overt right ventricular failure; mean 6-min walk distance improved from 42.3 ± 10.0% to 81.2 ± 9.7% of adjusted values for age and sex (P < 0.001), BNP/NT-proBNP levels normalized in all; and weaning of intravenous epoprostenol was obtained in all patients who received triple combination as pre-Potts anastomosis therapy. Finally, all survivors caught up to normal growth curves. Arterial oxygen saturation gradient between upper and lower limbs persisted at the last FU (94.7 ± 3.6% vs 81.6 ± 5.1%, P < 0.001). One patient required double lung transplantation 6 years after a surgical Potts shunt. CONCLUSIONS: Palliative Potts shunt allows prolonged survival and dramatic, long-lasting improvement in functional capacities in children with severe, drug-refractory PAH. The Potts shunt might be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH, leaving the door open for further lung transplantation, if needed.

Mitral disease: the real burden for Ross-Konno procedure in children.

BACKGROUND: Aortic valve replacement and management of complex left ventricular outflow tract obstruction in early life remains a surgical challenge. We assessed our experience with the Ross-Konno procedure. METHODS: Since 2000, 49 consecutive patients (24 neonates and infants) underwent the Ross-Konno procedure. Anatomic and clinical risk factors were analyzed. RESULTS: Median age was 12.2 months (0.0 to 194.4); 82% had previous valvotomy (surgical, n = 26; balloon, n = 7; balloon then surgical, n = 7) and 35% preceding arch repair (n = 17). Fifteen patients (31%) required concomitant procedures: mitral valve repair (n = 11), replacement (n = 1), endocardial fibroelastosis resection (n = 4), and aortic arch repair (n = 2). There were 5 hospital deaths (10.2%). Median follow-up was 63 ± 47 months. There were 4 late deaths (8.1%), all because of persistent pulmonary hypertension despite subsequent mitral procedures. Five-year actuarial survival and freedom from reoperation were 79.7% ± 6.1% and 68.6% ± 9.3%, respectively. Preoperative lower shortening fraction (p = 0.005) was associated with early mortality, while concomitant mitral surgery and pulmonary hypertension (p = 0.002) were associated with late mortality. Sixteen patients underwent 26 reoperations. Autograft function was normal in 30 of the 37 late survivors (81.1%), 4 (10.8%) had grade 2 regurgitation, and 3 (8.1%) required valve replacement. CONCLUSIONS: Ross-Konno in children remains a high-risk procedure. Preoperative ventricular dysfunction is associated with significant early mortality and should favor conservative options. Mitral involvement substantially affects late outcome, survival for these patients relies on the efficacy in relieving mitral disease. Efforts to improve mitral repair in these infants are critically required as an alternative to univentricular pathways.