Annals of Pediatric Cardiology最新文献

End-stage heart failure due to left ventricular noncompaction (LVNC) poses unique challenges for ventricular assist device implantation, like inflow cannula obstruction due to trabeculations. We report a case of an 11-year-old boy with LVNC who underwent successful HeartWare implantation as a bridge to transplant for high pulmonary vascular resistance and had successful heart transplantation after 4 years of HeartWare support.

PostGlenn thrombosis is an infrequent, serious complication requiring immediate attention, which is not uncommon in a resource-limited setting where patients are often lost to follow-up and have poor compliance with medications. This rare complication has infrequently been reported in the past. We describe a case of a massive thromboembolic event in a patient presenting acutely in a decompensated state 14 years after initial palliation by Glenn shunt. We report an innovative strategy of using transcatheter mechanical thrombectomy using a rotational thrombectomy device and chemical thrombolysis for the successful salvation of the Glenn circuit.

Background: Children with one functional ventricle might receive the Fontan procedure as palliative management to prolong their lives. However, the heart remains functionally univentricular, which may result in disrupted absorption of nutrients, including Vitamin D. Individuals with Fontan circulation have limited physical activity and sun exposure, which might further contribute to Vitamin D deficiency.

Objective: This study aimed to estimate the prevalence of Vitamin D deficiency among individuals with Fontan circulation and analyze probable contributing factors to it through meta-regression analysis.

Methods: A predetermined search strategy was applied on PubMed, Scopus, Scilit, Web of Science, Europe PMC, and Google Scholar to identify relevant literature as of July 2024. In general, studies reporting the prevalence or incidence of Vitamin D deficiency (<20 ng/mL) among Fontan patients were eligible for inclusion. Only cohort and cross-sectional studies were included. The pooled estimates were carried out using a restricted maximum-likelihood model and Freeman-Tukey double-arcsine transformation (FTT).

Results: Six studies were included, comprising 255 individuals with the Fontan circulation. The prevalence of Vitamin D deficiency was 51% (95% confidence interval: 35%- 67%) with noticeable heterogeneity (I ² = 84.38%; p-Het <0.001). Extracardiac conduit/lateral tunnel (P = 0.003) was the main contributor to the heterogeneity.

Conclusion: Vitamin D deficiency is highly prevalent among individuals with Fontan palliation, highlighting the need for active monitoring and investigation into the benefits of Vitamin D supplementation.PROSPERO registration: CRD42024574724.

Transcatheter pulmonary valve implantation (TPVI) is the standard of care in patients with repaired tetralogy of Fallot (rTOF) presenting with right ventricular outflow tract (RVOT) dysfunction. However, the feasibility of TPVI is limited by the high cost and nonavailability of larger-sized valves for dilated native RVOT of rTOF patients. We report the first successful TPVI with a custom-made 35 mm balloon-expandable valve (Myval™) in a 30-year-old rTOF patient with severe pulmonary regurgitation and RV dysfunction.

India, owing to its population structure, faces an enormous burden of children born with congenital heart disease (CHD). Systematic challenges such as limited public health infrastructure, a shortage of trained specialists, and high out-of-pocket expenditures hinder uniform access to comprehensive CHD care. Despite these limitations, Indian pediatric cardiologists have delivered innovative and often cost-effective solutions to challenging clinical problems. Indigenous devices such as the MyVal transcatheter heart valve, Konar-MF (multifunction) occluder, and Zephyr large-diameter stents are significant achievements in their respective fields. Static balloon dilatation of interatrial septum and balloon-assisted atrial septal defect (ASD) device implantation are prime examples of many innovations that were established by Indians and followed across the world. India also helped consolidate significant interventions in children, such as percutaneous transvenous mitral commissurotomy and interventions for aortoarteritis. Notably, Indian centers have published some of the largest series on transcatheter closure of sinus venosus ASD and ruptured sinus of Valsalva aneurysm. Close collaboration with adult coronary interventionalists has facilitated innovations borrowed from coronary chronic total occlusion hardware and techniques to recanalize ductus arteriosus or membranous-type pulmonary atresia. This manuscript discusses some of India's innovative contributions to the field of pediatric cardiac interventions.

A unicuspid aortic valve (UAV) in adults is a very rare form of aortic valve (AV) malformation. UAV has two distinct subtypes, acommissural UAV and unicommissural, and can be differentiated by anatomical features, imaging modalities, and clinical presentation. With the development of significant AV lesion (s), surgical or transcatheter intervention will be required. The first part is a summarized review of UAV (anatomical features, clinical presentation, diagnostic modalities, and management). In the second part, we present a series of four patients diagnosed with UAV (3 unicommissural and 1 acommissural). The first case underwent balloon aortic valvuloplasty during childhood and surgical AV replacement later, with the progression to severe aortic stenosis (AS). The second case underwent a Ross procedure. The third and fourth cases were asymptomatic with moderate AS and mild-to-moderate AR and were kept on follow-up. In all the cases, transesophageal echocardiography confirmed the diagnosis of UAV with detailed morphological and functional assessment of AV.

Background: The ductus venosus (DV) is a pivotal component of fetal circulation. Absent DV (ADV) is associated with structural defects, portal vein (PV) anomalies, and chromosomal anomalies. This observational study aims to investigate the impact of ADV on fetal circulation and postnatal outcomes.

Materials and methods: This observational study was conducted from August 2016 to January 2020 at a fetal and pediatric cardiac center. The DV was evaluated as part of routine fetal echocardiography. Cases of ADV were identified. Blood flow and exit points of the umbilical vein were studied. Cardiothoracic ratio, hydrops, and PV were assessed during the initial and follow-up scans. The postnatal evaluation included an ultrasound abdomen and computed tomography with triple-phase imaging to assess portosystemic shunts (PSSs). Serum ammonia levels were monitored.

Results: Twelve patients with ADV were identified. The median maternal age and median gestational age were 27.5 years and 22 weeks, respectively. Four patients had intrahepatic drainage, while eight had extrahepatic drainage. All patients (100%) exhibited cardiomegaly, but none developed hydrops. Four patients had persistent PSS postnatally. All four patients with PSS had asymptomatic hyperammonemia. Two patients underwent transcatheter closure of PSS. The intrahepatic variant showed good PV anatomy with no evidence of PSS.

Conclusions: DV evaluation should be performed during fetal echocardiography. ADV can lead to cardiomegaly and dilation of the right atrium and ventricle. PSS can be a potential sequela of the extrahepatic variant of ADV.