Annals of Pediatric Cardiology最新文献

Shone's complex is a rare constellation of multilevel left cardiac obstructive lesions involving both the inflow and outflow. Four primary lesions were included in the initial lesion description. However, other obstruction-causing structural lesions could be infrequently associated. The current report describes a fetal diagnosis of an unusual association of incomplete Shone's complex with cor triatriatum sinister and interrupted aortic arch.

Background: The current recommended secondary prevention of rheumatic fever (RF)/rheumatic heart disease (RHD) includes every 21-28 days injection of benzathine penicillin G (BPG), which causes pain. We evaluated the effect of the coadministration of lidocaine on reducing the pain of BPG injections in children with RF/RHD.

Patients and methods: Children aged 7-18 years who received BPG were eligible for a randomized crossover study. Participants received a secondary prophylaxis dose of intramuscular (IM) BPG with and without additional lidocaine as a diluent (0.2-0.4 mg/kg) and were randomized to the intervention (as a sequence). Participants and staff completing the evaluation were blinded to the sequence. Pain scales were assessed by participants using a Visual Analog Scale (VAS) and staff using the Wong-Baker Faces Pain Scale (WBFPS) at 5 and 60 min after injection.

Results: Forty-two children were included in the study. Both lidocaine and aquadest sterile water groups were well matched in baseline characteristics. The pain scores at 5 min after BPG injection were lower when lidocaine was coadministrated: 2 (min-max 1-9) in lidocaine versus 5 (min-max 1-9) in sterile water (P = 0.001) using VAS score and 2 (min-max 0-10) in lidocaine versus 4 (1-8) in sterile water (P = 0.002) using WBFPS. Scores at 60 min after BPG injection showed no differences between treatments with P = 0.551 and P = 0.167, respectively, using VAS and WBFPS. No adverse event was observed.

Conclusion: The coadministration of lidocaine as diluent resulted in reduced pain 5 min after IM injection of BPG.

Innovation in congenital cardiac surgery took its roots in the 1960s with the development of indigenous heart-lung machines. Over the years, many pioneering advancements showcasing solutions that combine cutting-edge technology with cost-effective approaches have happened in India. Indian surgeons have made significant contributions, including developing tissue-engineered patches, handmade conduits, and indigenous devices for surgical closure of multiple ventricular septal defects. Several new surgical techniques have been described, and Indian surgeons have described some new anatomical entities. Development of frugal technologies has been the game-changer for resource-limited environments. Innovations in imaging, including three-dimensional printing and virtual reality, are transforming preoperative planning and surgical education. Artificial intelligence and machine learning are beginning to influence decision-making and predictive modeling in pediatric cardiac care. Funding initiatives, including government support, public-private partnerships, and philanthropic funding, are making congenital heart disease surgery accessible to a broader population. India's contributions to innovations in congenital cardiac surgery exemplify the spirit of resilience and ingenuity, offering solutions that are impactful locally and inspiring globally.

Background: Atrial flow regulators are used in patients with pulmonary arterial hypertension (PAH) who present with syncope or advanced heart failure. The ThoroughFare atrial pressure controller (Meril Life Sciences, Vapi, India) is a new device for similar use. The differences in structural design include a cable-screw release mechanism and a low profile.

Methods: A multi-institutional study assessed the feasibility and safety of this device and reported short-term follow-up. This device, with an 8-mm fenestration diameter, was deployed after transseptal puncture using a 12F sheath.

Results: Between April 2023 and June 2024, 15 symptomatic patients aged 5-39 years, including four children, received this implant after dual pulmonary vasodilator pharmacotherapy for at least 2 years. Etiology included idiopathic PAH in eight patients, hereditary PAH in two, operated shunt lesions in four, and human immunodeficiency virus-associated PAH in one patient. The key indication was recurrent syncope in 11 and right heart failure in the rest. The mean N-terminal pro-brain natriuretic peptide was 2414 ± 2046 pg/mL. The hemodynamic assessment revealed high right atrial pressures in 80% of patients, low cardiac index in half, and suprasystemic pulmonary pressures in four patients. The procedure was completed in all patients without any complications. Pulse oximeter saturations dropped from 98% ±2% to 92% ±4%. Symptoms improved over a median follow-up of 8 months, and the device patency was confirmed in all patients except one patient who died 5 months postprocedure after a heart-lung transplantation.

Conclusion: ThoroughFare atrial pressure controller implantation was feasible and safe for all patients with severe PAH without any complications. Longer follow-up with more patients is needed to ascertain the functional improvements.

An 11-year-old boy presented with recurrent exertional syncope for 1 month. The baseline electrocardiogram (ECG) suggested a diagnosis of long QT syndrome with macroscopic T-wave alternans. Volatility of blood pressure and left ventricular hypertrophy triggered further investigations, revealing pheochromocytoma as the primary cause. The child underwent laparoscopic resection of the tumor with subsequent resolution of ECG changes and symptoms. The genetic testing was negative for known mutations implicated with prolonged QT interval.

The growing body of evidence suggests that junctophilin-2 (JPH2) variants hold significant potential for diagnostic and therapeutic interventions, particularly within the framework of personalized medicine and genetic screening across diverse populations. Mutations in JPH2 have been associated with a range of clinical phenotypes including early-onset heart failure and cardiomyopathies, a diverse group of diseases affecting heart muscle structure and function that contribute to heart failure and sudden cardiac death. While traditional understanding has centered on sarcomeric gene mutations, recent studies have shifted attention toward calcium-handling genes such as JPH2. This study consolidates insights from original research, preclinical studies, case reports, and editorials to highlight JPH2's impact on cardiomyopathies and associated disease phenotypes.

This hemodynamic round section deals with severe pulmonary arterial hypertension with suprasystemic pulmonary artery pressures in a patient who underwent delayed surgical correction of the double-outlet right ventricle with a large subaortic ventricular septal defect (VSD). Recreation of a moderate-sized VSD by electrocautery-aided fenestration of the surgical patch resulted in effective right ventricular decompression. The changes in the hemodynamics are illustrated in the pressure traces and Doppler echocardiographic images. The changes in cardiac events on the right and left heart due to the right bundle branch block are also illustrated in the manuscript.