Background: Despite the increased use of guideline directed medical therapy (GDMT) (defined as renin angiotensin aldosterone system inhibitor, beta blocker, mineralocorticoid receptor antagonist, and sodium-glucose cotransporter-2 inhibitors) in adult heart failure, there is no consensus regarding their use in adults with single ventricular failure, and pediatric data is scarce. Objectives: This was a single center observational study to evaluate the feasibility of GDMT in children with single ventricular dysfunction. Methods: Consecutive patients aged <= 19 years with single ventricular physiology at various stages of surgical palliation, with ejection fraction <= 50% and received at least three of the four pillars of GDMT for more than thirty consecutive days, were considered for inclusion in this study. Results: Ten consecutive children constituted the study cohort. The primary composite outcome of worsening heart failure, heart transplantation, and mortality was identified in 40% of patients at 1-year follow-up. The median ejection fraction improved from 32% to 50%, and the cardiothoracic ratio reduced from 59 to 55%, predominantly in those with left ventricular morphology. The NT-proBNP levels reduced from 5530 to 1400 pg/ ml. The patients demonstrated a mild decrease in systolic blood pressure on follow-up but remained above the 15th centile. The serum creatinine levels and estimated glomerular filtration rate showed a biphasic pattern with an initial worsening followed by normalization to baseline levels at 1-year follow-up with continued GDMT. Conclusions: The improvements in ejection fraction observed in children with single ventricle physiology and ventricular dysfunction with quadruple GDMT suggest the need for larger prospective trials in children.

We attempted to study the influence of pre-operative mitral regurgitation on the recovery of ventricular function in paediatric patients with repaired anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Eighty-eight patients under 18 years who underwent ALCAPA repair from 2003 to 2022 were included. Baseline clinical and echocardiographic data, including MR and left ventricular ejection fraction (LVEF), were recorded pre-operatively and at multiple post-operative follow-ups (discharge, 1 month, 6 months, 1 year, and annually thereafter). Friedman’s Test and correlation/regression analyses were used to assess the impact of pre-operative MR on ventricular function recovery. The median age at surgery was 6 months(range 1-197), and the median hospital stay was 17 days. 56.81

The Occlunix® septal occluder (OSO) is a new generation double disk occluder device for catheter closure of the secundum atrial septal defect (ASD). Fifty-six consecutive patients who underwent ASD device closure with the OSO between February 2022 and April 2024 were evaluated in this single-center observational study. All procedures were done under transesophageal echocardiography guidance. The OSO was successfully deployed in all patients (technical success 100%). Fifty-five patients had complete closure of the intervened ASD after device closure (procedural success 98.2%). The sole patient with trivial intradevice shunt had complete seal of the defect at 1-month follow-up. There were no device related complications - cardiac, vascular or systemic. During the follow-up period of 32.2 ± 9.1 months, the device position was satisfactory in all, with no complications. The OSO was associated with lower procedural cost than one of the most used conventional ASD occluders used in the country.

BACKGROUND:Fungal infective endocarditis is rare but highly lethal, with limited evidence to guide management. Diagnosis is often delayed, source control may be difficult, and effective antifungal options may be limited. CASE SUMMARY:We describe 8 patients with fungal infective endocarditis, ranging from a 40-day-old infant to a 56-year-old adult. Organisms included Candida parapsilosis in 3 patients, multidrug-resistant Candida auris, Candida glabrata, Candida albicans, Fusarium species, and Apiosporium mycotoxinivorans. Infection involved native valves, prosthetic valves, aortic root grafts, and intracardiac device leads. Five patients underwent definitive source control. In 1 patient, fungal prosthetic valve endocarditis initially mimicked prosthetic valve thrombosis, leading to thrombolysis before infection was recognized. In-hospital mortality was 50%. DISCUSSION:Outcomes appeared to depend on early recognition, antifungal susceptibility, and feasibility of source control. These cases emphasize the need for microbiological suspicion, multimodality imaging, and multidisciplinary decision-making in fungal infective endocarditis.

BACKGROUND:KLHL24 (Kelch-like family member 24)-associated hypertrophic cardiomyopathy (HCM) is a recently recognized genetic disorder characterized by early presentation and a disproportionate risk of malignant ventricular arrhythmias due to impaired cytoskeletal protein turnover. CASE SUMMARY:A 16-year-old asymptomatic young man without a family history of HCM was evaluated after detection of a cardiac murmur. Imaging revealed asymmetric septal hypertrophy with minimal fibrosis and preserved systolic function. Genetic testing identified compound heterozygous truncating KLHL24 variants. Despite low conventional risk markers, genotype-directed assessment indicated high arrhythmic risk, prompting prophylactic implantable cardioverter-defibrillator (ICD) placement. The patient remained clinically stable at follow-up. DISCUSSION:This case illustrates the limitations of phenotype-based risk stratification and highlights the importance of genotype-informed decision-making in arrhythmogenic cardiomyopathies. TAKE-HOME MESSAGES:KLHL24-associated HCM is uniquely arrhythmogenic, with malignant ventricular arrhythmias potentially preceding structural severity. Early genetic testing enables genotype-guided preventive strategies, including timely prophylactic ICD implantation, even in apparently low-risk phenotypes.

Pre-operative stabilisation and effective transport to a tertiary referral centre are key to survival in newborns with critical congenital heart disease (CHD) in resource limited settings. There is an urgent need for organized transport services (ambulances and personnel, structured communication and documentation, state and insurance participation). Transport of these patients for long distances by road, without specialised ambulances with appropriate monitoring equipment or trained medical personnel may result in destabilisation of these patients when they arrive at the tertiary centre. Time critical transfers are needed for transposition of great arteries (TGA) with restrictive foramen ovale, duct dependent circulation with no response to prostaglandin and obstructed total anomalous pulmonary venous connection (TAPVC). In this article, the authors look at ways to optimise safe transport of these newborns by quick initial stabilisation with specific attention to prostaglandins, oxygen and inotropes to improve outcomes of transferred neonates with CHD.

Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease, is classically associated with decreased pulmonary blood flow. Classical TOF is characterized by antegrade pulmonary blood flow and right ventricular outflow tract obstruction at varying levels. In the modern era, most children with TOF undergo definitive intracardiac repair during infancy, and pulmonary artery hypertension (PAH) is virtually unheard of in postoperative TOF in the absence of major aortopulmonary collaterals. We report the circumstances in which PAH can occur after definitive repair of classical TOF in the modern era.

Quadricuspid aortic valve (QAV) is a rare congenital anomaly with <200 cases reported to date. Most patients are asymptomatic. Symptoms depend on the functionality of the valve and associated heart defects, most often presenting as aortic regurgitation followed by aortic stenosis. Management guidelines are ill-defined. We present two cases of QAV with rare associations (ventricular septal defect) and variable clinical presentation.

ABSTRACT Introduction: Permanent pacemaker implantation (PPI) in neonates is challenging with respect to indications, device selection, implantation technique, and long-term outcomes. Complex anatomy, the need for long-term pacing with high rates, and a problematic postoperative period are the major problems. Methods: We prospectively followed up 22 newborns who underwent PPI below 28 days of life at our institute. Results: The median age at implantation was 2 days (interquartile range 1–9 days), and 9% were born preterm. The average heart rate before implantation was 46.4 ± 7.2 bpm. Maternal lupus antibodies were positive in 8 (36.4%) neonates, whereas 11 (50.0%) had associated congenital heart disease. Nineteen neonates underwent single chamber (VVI) and three underwent dual chamber (DDD) pacemaker implantation. Over a median follow-up of 46 months (range 2–123 months), the average ventricular pacing percentage was 87.5 ± 24.9%, with a stable pacing threshold. Seven children underwent pulse generator replacement due to battery depletion at a median age of 47 months. Pacing-induced ventricular dysfunction was seen in five children at a median age of 23.6 months, and two underwent upgradation to cardiac resynchronization therapy. Overall mortality was 13.6%, all due to tissue hypoperfusion and lactic acidosis in the postimplantation period. Conclusions: PPI in neonates has a favorable outcome with excellent lead survival. Overall mortality is 13.6%, which is predominantly in the postimplantation period and related to myocardial dysfunction.

An 18-month-old boy presented with recurrent episodes of irritability and documented fast heart rate suggestive of supraventricular tachycardia. Cardiovascular examination revealed significant cardiomegaly, normal heart sounds and no murmurs. The differential diagnosis of marked right atrial dilatation and management principles of idiopathic dilatation of the right atrium are described.

Truncus arteriosus is an exceedingly rare congenital heart disease involving the conotruncal septum of the developing heart. Clinical presentations vary depending on associated anomalies. Surgical management is extremely challenging and differs with anatomic subtypes. We present a case of truncus arteriosus––Vaan Praagh type A4, quadricuspid truncal valve with moderate truncal stenosis and regurgitation, type B interrupted aortic arch who underwent bilateral pulmonary artery banding.

Aneurysms of the coronary arteries and abdominal aorta are extremely rare in infancy. Due to the rarity of the anomaly, there are no existing guidelines on management of these aneurysms. We describe the challenges in diagnosis, evaluation and management of an infantile Marfan with this rare presentation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0–8.5) and 8 days (IQR 4.0–39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0–3.0) and 3.0 kg (IQR 2.5–3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group ( p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups ( p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable ( p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures.

Total anomalous pulmonary venous connection (TAPVC) and anomalous pulmonary venous drainage are not synonymous. This has been described in the setting of right isomerism (bilateral right sidedness) where the pulmonary veins are connected anomalously but drain normally to the left-sided morphological right atrium. We describe another situation in right isomerism where normal pulmonary venous drainage is present in the setting of TAPVC.