Jérôme Le Bidois

Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction

BACKGROUND Valved conduits from the right ventricle to the pulmonary artery are frequently used in paediatric cardiac surgery. However, stenosis and insufficiency of the conduit usually occur in the follow-up and lead to reoperations. Conduit stenting can delay surgical replacement, but it aggravates pulmonary insufficiency. We developed an innovative system for percutaneous stent implantation combined with valve replacement. METHODS A 12-year-old boy with stenosis and insufficiency of a prosthetic conduit from the right ventricle to the pulmonary artery underwent percutaneous implantation of a bovine jugular valve in the conduit. FINDINGS Angiography, haemodynamic assessment, and echocardiography after the procedure showed no insufficiency of the implanted valve, and partial relief of the conduit stenosis. There were no complications after 1 month of follow-up, and the patient is presently in good physical condition. INTERPRETATION We have shown that percutaneous valve replacement in the pulmonary position is possible. With further technical improvements, this new technique might also be used for valve replacement in other cardiac and non-cardiac positions.

Pediatric cardiac transplantation for congenital heart defects: surgical considerations and results.

Among 54 children who underwent 55 heart transplantations, 24 (44%) (mean age, 4.9 +/- 4.8 years; range, 9 days to 18 years) had congenital defects with the following diagnoses: single-ventricle variants (6), hypoplastic left heart syndrome variants (5), transposition complex (6), and miscellaneous defects (7). Twenty patients (83%) had undergone 43 prior operations. Additional surgical procedures included repositioning of transposed great arteries (11), reconstruction of the aortic pathway (4), reconstruction of the pulmonary pathway (8), correction of situs inversus (1), and correction of anomalous pulmonary (1) or systemic (1) venous drainage. Reconstructive procedures were performed using donor or recipient tissue or both. There were six early deaths (hyperacute rejection, 1 patient; pulmonary hypertension, 1; graft failure, 2 patients; infection, 2) and six late deaths (sudden death, 2; chronic rejection, 2; nonspecific graft dysfunction, 1; lymphoproliferative disease, 1). The survival rate was 43% +/- 12% at 3 years. No deaths were related to surgical technique. Survival was not significantly different in pediatric recipients with cardiomyopathy (67% +/- 9%; p = 0.22). Accelerated coronary artery disease was noted in 4 operative survivors (22%; 70% confidence limits, 12% to 36%). All late survivors were free from cardiac symptoms after a mean follow-up of 34 +/- 24 months (range, 6 to 71 months). Based on this study, we reached three conclusions. (1) Careful planning of both harvesting and transplantation procedures allows heart transplantation in recipients with congenital heart diseases. (2) The surgical technique may be demanding, but the early risk is not increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Foetal echocardiographic assessment of tetralogy of Fallot and post-natal outcome

AIMS Outcome of foetuses diagnosed with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA-VSD) and the reliability of foetal echocardiography to predict post-natal surgical outcome. METHODS AND RESULTS Outcome of 218 foetuses having been diagnosed with TOF (n = 153) or PA-VSD (n = 65) was reviewed. Abnormal karyotyping, 22q11 deletion, and extracardiac anomalies were found, respectively, in 11, 18, and 46%. Pregnancy was terminated in 75 cases (34%), and in three cases foetuses died in utero. Presence or absence and confluence of PA branches were confirmed after birth or pregnancy termination in all but five (5%) cases. Main pulmonary trunk (MPA) was incorrectly described in 11 (10%) cases and major aorto-pulmonary collateral arteries in 16 (13%) cases. Among live born infants, 110 (88%) were operated and 92 (74%) underwent complete repair in the first year of life. Size of confluent PAs and presence of MPA were related to the probability of having a complete repair in the first year of life. CONCLUSION Foetal diagnosis of TOF and PA-VSD has a major impact on pregnancy outcome, as associated anomalies are frequently found. Pre-natally determined size of PA branches and presence of MPA are good predictors of complete repair in the first year of life.

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies

BACKGROUND Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes. OBJECTIVES This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies. METHODS We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period. Pre-natal echocardiographic and final diagnoses were compared. RESULTS For live births, we identified 368 (29.3%) discordances between pre- and post-natal diagnoses. The pre-natal diagnosis was different from the post-natal diagnosis in 36 cases (2.9%) and partially different with a major impact on neonatal treatment of the CHD in 97 cases (7.7%). In 235 cases (18.7%), the diagnosis was partially different with no impact on neonatal planned treatment. The discordances had a negative impact on late care strategy in 62 cases (4.9%): more complex CHD that was unsuitable for biventricular repair, leading to unplanned compassionate care, additional surgery or increase of the complexity level of the Aristotle score. A positive impact was found in 31 cases (2.5%): less complex CHD that allowed biventricular repair, fewer surgical procedures, or decrease of the complexity of the Aristotle score. For 275 patients (21.9%), there was no impact on late care strategy. Of the 872 terminations of pregnancy and intrauterine fetal deaths, 189 fetopsies were available: 16 (8.5%) different diagnoses, 27 (14.3%) major differences, and 60 (31.7%) minor differences. CONCLUSIONS Correcting fetal cardiac diagnosis after birth can lead to significant changes in neonatal (10.6%) and late (7.4%) care strategies. Tools should be developed to try to improve the accuracy of pre-natal diagnosis of CHD. Clinicians should be cautious when predicting required treatment and outcomes during pre-natal counseling.

Neonatal management and outcomes of prenatally diagnosed CHDs

OBJECTIVES The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD. METHODS A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention. RESULTS The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital. CONCLUSION Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Aortic coarctation, multiple ventricular septal defects, and anomalous coronary artery arising from the right pulmonary artery ☆ ☆☆ ★ ★★

The literature reveals no reports of a left coronary artery arising from the right pulmonary artery in association with aortic coarctation and multiple ventricular septal defects. The patient we describe here died of left myocardial infarction after palliative operation because this defect was unanticipated. Case report. The patient was admitted at the age of 12 days with a diagnosis of aortic coarctation and multiple ventricular septal defects. She had classic signs of aortic coarctation and predominantly left-sided heart failure, which responded partially to prostaglandin E1 and diuretics. Chest radiography revealed severe cardiomegaly and symmetrically plethoric lungs. The electrocardiogram showed sinus rhythm at 140 beats/min, right ventricular hypertrophy, and no signs of myocardial ischemia. Echocardiography demonstrated an aortic coarctation with long, severe hypoplasia of the arch, a large ductus arteriosus, and multiple ventricular septal defects. The left ventricle was neither hypokinetic nor hypoplastic. No coexisting mitral regurgitation was documented. There was also a left superior caval vein draining into the coronary sinus and a large ostium secundum–type atrial septal defect. The patient underwent pulmonary artery banding, division of the ductus arteriosus, and aortic coartectomy. Within 2 hours of the patient’s return to the intensive care unit, her condition deteriorated rapidly and dramatically, with a global but predominantly left-sided intractable heart failure, complete atrioventricular block, and cardiogenic shock. The postoperative electrocardiogram revealed signs of severe myocardial anterolateral ischemia and necrosis and a third-degree atrioventricular block (Fig. 1). Creatine kinase had increased to 530 U/L (normal range 15 to 100 U/L) with an MB fraction of 365 U/L (68%), representing a significant acute myocardial necrotic phenomenon. Postoperative cross-sectional echocardiography visualized a mildly dilated and severely hypokinetic left ventricle (less than 10% of shortening fraction) with severe impairment of diastolic function. The pulmonary artery banding was adequate, and there was no residual aortic coarctation. The right coronary artery was predominant, and the left coronary artery’s origin was not seen from the corresponding aortic Valsalva sinus. This raised suspicion of an anomalous left coronary artery arising from the pulmonary artery, which would provide an explanation for the myocardial infarction. Unfortunately, the child’s deteriorating condition did not allow performance of any further investigations. Despite maximum hemodynamic support and intravascular cardiac pacing with a 5F bipolar balloon pacing electrode (USCI International, BARD, C.R. Bard Ireland Ltd., Galway, Ireland), she died on the third postoperative day. Postmortem examination. The heart weighed 30 gm. There was, as expected, an abnormal left superior caval vein draining into the coronary sinus, a large ostium secundum–type atrial septal defect, and one large perimembranous and multiple muscular ventricular septal defects. The right ventricle was predominant, and the left one was small but not hypoplastic. Necrotic patches, confirmed by histologic analysis, were evident in the left anterolateral wall. The endocardium did not exhibit fibroelastosis. The aortic coarctation was well repaired, and the pulmonary banding was found to be in place. A tortuous, dilated right predominant coronary artery originated from the right aortic Valsalva sinus. The left coronary artery arose from the posterior right pulmonary artery wall immediately after the pulmonary bifurcation and followed a tortuous course over the anterior interventricular groove (Fig. 2). Discussion. Within the wide variety of left coronary anomalous origin, left coronary artery arising from the right pulmonary artery is extremely uncommon. The chief reason for this report, however, is the association of this origin with lesions producing high pulmonary flow, with consequent lack of clinical data suggesting such a diagnosis. Many factors led to misdiagnosis. First, this finding is not classically anticipated in cases of ventricular septal defects and aortic coarctation. Second, the left-to-right shunt provided normal perfusion to the aberrant coronary artery before the operation. Until palliation, this child’s left coronary blood flow was normal, and so were the left ventricular myocardial perfusion and oxygenation, because the pulmonary and aortic pressures and saturations were similar. The fall in pulmonary pressure to a low level after the pulmonary artery banding was therefore accompanied by a decline in the left coronary flow, with drops in coronary perfusion pressure and oxygen saturation leading to left ventricular ischemia and myocardial infarction. A similar physiopathologic condition related to closure of From the Department of Pediatric Cardiac Surgery, Centre Medico-Chirurgical de la Porte de Choisy, and the Department of Pediatric Cardiology, Institut de Puericulture de Paris, Paris, France.

Unusual Form of Truncus Arteriosus Associated With 22q11 Deletion

A 25-year-old woman was referred at 31 weeks’ gestation for prenatal echocardiography because routine obstetrical sonography had detected a cardiac malformation. Pregnancy to that point had been normal. Four-chamber and great-vessel views allowed the diagnosis of truncus arteriosus with an abnormal dilatation of the pulmonary tree (Figure 1 and Movie I). An in situ hybridization study …

Incidence, risk factors, and mortality of neonatal and late-onset dilated cardiomyopathy associated with cardiac neonatal lupus

BACKGROUND Dilated cardiomyopathy (DCM), a well-known complication of cardiac neonatal lupus, is associated with high mortality rate. Its risk factors remain unclear. METHODS We analyzed occurrence of postnatal DCM among children with high-degree congenital heart block (CHB) and mothers with anti-SSA and/or anti-SSB antibodies. RESULTS Among 187 neonates with CHB, 35 (18.8%, one missing data) had DCM and 22 (11.8%) died during a median follow-up of 7years [range: birth-36years]. On multivariate analysis, factors associated with postnatal DCM were in utero DCM (P=0.0199; HR=3.13 [95% CI: 1.20-8.16]), non-European origin (P=0.0052; HR=4.10 [95% CI: 1.81-9.28]) and pacemaker implantation (P=0.0013; HR=5.48 [95% CI: 1.94-15.47]). Postnatal DCM could be categorized in two subgroups: neonatal DCM (n=13, diagnosed at a median age of 0day [birth-4days]) and late-onset DCM (n=22, diagnosed at a median age of 15.2months [3.6months-22.8years]). Factors associated with neonatal DCM were in utero DCM, hydrops, endocardial fibroelastosis and pericardial effusion, whereas those associated with late-onset DCM were non-European origin, in utero mitral valve insufficiency, and pacemaker implantation. Fluorinated steroids showed no protective effect against late-onset DCM (P=0.27; HR=1.65 [95% CI: 0.63-4.25]). Probability of survival at 10years was 23.1% for newborns diagnosed neonatally with DCM, 53.9% for those who developed late-onset DCM, and 98.6% for those without DCM. CONCLUSION Neonatal and late-onset DCM appear to be two different entities. None of the known risk factors associated with neonatal DCM predicted late-onset DCM. Long-term follow-up of cardiac function is warranted in all children with CHB.

P7 Impact of precision prenatal diagnostic of congenital heart diseases on perinatal and long-term management

Objective To evaluate the impact of precising prenatal diagnosis of congenital heart diseases (CHD) on perinatal and long-term management. Methods Over a 10-year period, 1258 neonates with a prenatally diagnosed CHD and 189 fetal autopsies after termination of pregnancy were included. Changes in CHD diagnosis were classified as totally different, similar but leading to changes in neonatal management, and similar without changes on initial management. The impact on long-term outcome was considered negative if the final diagnosis was a more complex CHD precluding the planned biventricular repair, or if additional surgical interventions were needed, or if the complexity level of the Aristotle score was increased. The impact on outcome was considered positive if biventricular repair was possible while not planned prenatally, or if the number of surgical interventions was reduced, or if the complexity level of the Aristotle score was lower. Results The post-natal diagnosis was imprecise in 30.2% of the cases: completely different in 2.9%, led to changes in initial management in 8%, and did not affect initial management in 19.3%. Imprecision in the prenatal diagnosis had a negative impact on long-term outcome in 4.9% of the cases, and a positive impact in 4.1%. In the fetal autopsy group (mean term 26 weeks), the diagnosis was imprecise in 54.5% of the cases: completely different in 8.5%, could have led to changes in postnatal management in 14.3%, and with minor differences that would not have led to changes in management in 31.7%. In both groups, the most frequent differences were anomalies of the outflow tract anatomy (43%), and the systemic or pulmonary veins (25%). Conclusion Imprecision of prenatal diagnosis of CHD changes early management in 11% of the cases, and impacts long-term outcome in 9% of the cases. Improvement of CHD diagnosis for anatomy of the outflow tract and main veins should help to reduce impact on postnatal management and outcome.