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Objective: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. Methods: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. Results: Sixty‐three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3–32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in‐utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty‐eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0–40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty‐eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow‐up of 23.3 (2.0–112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow‐up assessment, at a median (range) gestational age of 24.3 (8.3–48.7) months. Conclusions: FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Except for projections from the Global Burden of Disease (GBD) study, congenital heart disease (CHD) burden remains undetermined in Latin America and Caribbean (LAC), especially for complex types such as functionally univentricular hearts (FUH) responsible for higher disease morbidity and mortality. To describe CHD prevalence, characteristics and related infant mortality in French Guiana, a LAC territory. Population-based registry analysis of all fetal and live birth (LB) CHD cases from January 2012 to December 2016. Overall, 231 CHD were diagnosed, among which 56 fetal diagnoses and 215 resulting LB. Among all CHD (n = 231), there were 18.6% of chromosomal or genetic anomalies, and 6.5% of termination of pregnancy for fetal anomaly. Total and LB CHD prevalence were respectively 68.4 [95% CI: 67.9–68.8] and 65.2 [95% CI: 64.7–65.7] per 10,000, similar to GBD projections for TLA. The total infant mortality in the study sample was 9.4/10,000 LB [95% CI: 9.1–9.7]. Children with FUH presented the highest mortality rates. When compared to mainland France, total FUH prevalence for French Guiana was significantly higher (P = 0.03), as were LB FUH prevalence and related infant mortality respectively two to three-fold higher (P < 0.01). However, no significant difference was observed for FUH prevalence when compared to GBD projections for TLA. FUH infant mortality could not be contrasted due to the lack of GBD data. This first population-based study ever performed in this part of the world suggests a distinct profile for CHD in the LAC region, with elevated prevalence and mortality rates linked to FUH. Another potential determinant of the excess mortality risk might be the frequent presence of chromosomal or genetic anomalies. This constitutes a stepping-stone towards better understanding CHD burden and underlying specificities while providing future keys towards reducing infant mortality in French Guiana and others surrounding countries. [ABSTRACT FROM AUTHOR]

Ebstein's anomaly of the tricuspid valve (TV) is a congenital cardiac malformation of the tricuspid valve (TV) and right ventricle (RV) characterized by downward displacement of the functional annulus usually involving septal and inferior leaflets. Ebstein's anatomical spectrum is highly variable. This anatomical study aimed to unravel the morphogenetic mechanisms underlying this variability. Forty-two hearts exhibiting Ebstein anomaly from the Reference Center for Complex Congenital Heart Defects (CHD) collection were macroscopically analysed: 28 isolated and 14 associated with another CHD. Tricuspid leaflets, right ventricle (RV), and associated lesions were analysed. Measurements were related to fetal heart reference values according to gestational age. Specimens were classified depending on the distal insertion of the anterior leaflet: linear, hyphenated or focal. Among isolated Ebstein hearts, distal insertion of the anterior leaflet was linear in 36%, hyphenated in 29% and focal in 36%. Linear insertion was always associated with anterior TV orifice and absent inferior leaflet, with absent septal leaflet in 80%. Atrialisation (thin, paper-like, inferior RV wall) was found in 90% of the linear forms and 37.5% of the hyphenated forms, which were associated with various degrees of inferior and septal leaflets non-delaminationanomalies. In focal forms, only the septal leaflet was involved, and there was no atrialisation. Ventricular septal defect was found in 3 specimens, Uhl-like RV anterior wall in 2, including 1 with left ventricle non compaction. Associated Ebstein anomalies were predominantly found in pulmonary atresia with intact ventricular septum; there was no atrialisation and septal leaflet only was involved. Ebstein anomaly corresponds to an arrest in TV development, early in linear forms, late in focal forms, intermediate in hyphenated forms. Atrialisation was always associated with absent inferior leaflet, underlining developmental interaction between endocardial cushions and myocardium at the RV inferior wall. [ABSTRACT FROM AUTHOR]

The best strategy to achieve optimal growth of pulmonary artery branches (PAs) in functionally univentricular heart (FUH) between partial cavopulmonary connection (PCPC) and total cavopulmonary connection (TCPC) is still controversial, especially the preservation of anterograde pulmonary blood flow (APBF). To model the growth of PA in FUH between PCPC and TCPC and to assess whether APBF promotes this growth. Then to determine whether the maintenance of an APBF is associated with higher morbidity and mortality. We retrospectively included all patients with FUH who underwent cardiac catheterization before PCPC and TCPC between 2004 and 2021. A linear regression model was used to model PA growth. We compared mortality and morbidity outcomes between the APBF group and no APBF group using the Kaplan–Meier method. In total, 118 children with FUH with a median follow-up of 8.8 years were included, 49 (41.5%) had maintained APBF. PA branch growth can be considered continuous and linear over time [fractional polynomials (P = 0.2)], estimated at β = 8.5 [0.7–16.2] mm2/year. In multivariate analysis, maintaining an APBF was the only factor associated with increased PA branch growth [β = 55.9 (21.8; 90) mm2 (P = 0.01)], regardless of TCPC timing (Fig. 1). Before PCPC, there was no difference in mean pulmonary artery pressure (mPAP) between groups with and without APBF. Between PCPC and TCPC, there was no significant increase in mPAP [13.2 (12–16) vs. 14.1 (12.4–15.8), P = 0.3] or transpulmonary pressure gradient [7.2 (5.2–9.3) vs. 6.9 (4.6–9.1), P = 0.6] in the APBF group. There was no difference in survival at 6 years after TCPC between the group with APBF [87.6, 95% CI (65.6%–95.9%)] and the group without APBF [82.3 95% CI (67.8–90.6)]. No difference was also found when comparing morbidity characteristics between the two groups, such as length of hospital stay after TCPC (P = 0.7), chylothorax (P = 0.81), hemodynamic contraindications to fenestration closure (P = 0.9), failing Fontan rate (P = 0.38). PAs growth between PCPC and TCPC is significant and can be considered linear over time. By maintaining APBF, growth potential can be achieved much more quickly and without additional risk of morbidity or mortality. [ABSTRACT FROM AUTHOR]

Most patients with Fontan circulation struggle to increase cardiac output during exercise affecting aerobic capacity, response to training and quality of life. This poor cardiac performance affects their aerobic capacity, their response to training and their quality of life. We sought to identify the pre-Fontan and managerial factors that influence their exercise cardiac performance. We retrospectively collected pre and post-Fontan anatomical, anamnestic and hemodynamic data in 57 consecutive young Fontan patients (median age 14.7 years) who underwent cardiopulmonary exercise test. We studied their exercise cardiac performance using a thoracic bioelectrical impedance device that allows measurement of the peak cardiac index (pCI). The median age at Fontan was 6.4 ± 3 years. In multivariable models, pCI was negatively influenced by cyanosis duration (B = −0.240; P = 0.0002). Peak indexed stroke volume (pSVi) was negatively influenced by Norwood procedure (B = -8.415; P = 0.005) and cyanosis duration (B = −1.591; P = 0.0002) and peak heart rate (pHR) by pre-Fontan peripheral oxygen saturation (B = 0.602 P = 0.009). Cyanosis duration ≤ 6.9 years predicted a pSVi ≥ 45 mL/m2 (AUC = 0.747; P = 0.001). Furthermore, pSVi and pHR were correlated with post-Fontan ventricular filling pressure (post-VFP) (respectively rp = −0.539, p = 0.012 and rp = −0.552; P = 0.010) and post-VFP was correlated with bidirectional cavopulmonary shunt duration (rs = 0.498, P = 0.023). In young Fontan patients, the duration of cyanosis and low peripheral oxygen saturation prior to the Fontan procedure affect both components of cardiac exercise performance probably through increased ventricular filling pressure. Shortening the duration of cyanosis could help preserve cardiac performance in the long term. [ABSTRACT FROM AUTHOR]