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Background : Selecting the device size using a sizing balloon could oversize the ostium secundum atrial septal defect (OSASD) with floppy margins and at times may lead to complications. Identifying the firm margins using trans-esophageal echocardiography (TEE) and selecting appropriate-sized device optimizes ASD device closure. This retrospective study was undertaken to document the safety and feasibility of device closure without balloon sizing the defect. Methods : Sixty-one consecutive patients who underwent trans-catheter closure of OSASD guided by balloon sizing of the defect and intra procedural fluoroscopy (group I) and 67 consecutive patients in whom TEE was used for defect sizing and as intraprocedural imaging during device deployment (group II) were compared. The procedural success rate, device characteristics, and complications were compared between the two groups. Results : The procedure was successful in 79.7 % patients. The success rate in group II (60 of 67, 89.6%) was significantly higher than in group I (41 of 61, 67.2 %) (P = 0.002). Mean upsizing of ASD device was significantly lower in group II (P < 0.001). TEE also provided better success rate with smaller device in subjects with large ASD (>25 mm) and in those who were younger than 14 years of age. There were four cases of device embolization (two in each group); of which one died in group II despite successful surgical retrieval. Conclusion : Balloon sizing may not be essential for successful ASD device closure. TEE-guided sizing of ASD and device deployment provides better success rate with relatively smaller sized device.

Introduction and Aims: Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome., Methods and Results: We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5-9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up ( n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias ( P value- 0.05). The 5-year survival rate of DCM patients was 59%., Conclusion: A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Annals of Pediatric Cardiology.)

The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Pediatric Cardiology.)

Competing Interests: There are no conflicts of interest.

Background: Three-dimensional echocardiography. (3DE) is comparable to cardiac magnetic resonance imaging for estimating ventricular volume in congenital heart diseases. However, there are limited data on estimation of ventricular volumes by 3DE in univentricular heart and change in ventricular volumes after surgical creation of cavopulmonary connection. We sought to quantify the unloading of the single ventricle of left ventricular. (LV) morphology by 3DE after superior cavopulmonary anastomosis. (SCPA) or Fontan operation over a period of 3 months and thereby derive a preliminary 3DE data set on this patient subset., Patients and Methods: Eighteen patients with functional single ventricle of LV morphology, who underwent SCPA or completion of Fontan circulation, were included in the study. Volume of the ventricle was estimated by 3DE before surgery and after surgery. (in the early postoperative phase and 3 months after surgery), and indexed end-diastolic volume. (EDV), end-systolic volume. (ESV), and ejection fraction. (EF) were derived., Results: Twelve patients underwent SCPA and six patients underwent staged completion of Fontan circulation. Before surgery, EDV was similar in both groups. There was a significant fall in EDV immediately after SCPA (from 48.3 ± 14.9 ml/m 2 to 39.5 ± 12.3 ml/m 2 ). However, EDV increased at 3 months' follow-up to 41.3 ± 10.5 ml/m 2 . There was no significant fall in EDV immediately after Fontan operation (47.2 ± 10.1 ml/m 2 -46.6 ± 14.2 ml/m 2 ), but EDV continued to fall at 3 months of follow-up (44.7 ± 10. ml/m 2 ). There was no significant change in ESV in either group, but EF fell significantly after SCPA., Conclusions: We provide preliminary information on 3DE volume data of single ventricle of LV morphology and the pattern of unloading after SCPA and Fontan operation. Immediate significant volume unloading occurred after SCPA which tended to catch-up after 3 months, whereas continued fall in ventricular volume with time was noted after Fontan., Competing Interests: There are no conflicts of interest.

A 44-year-old woman presented with features of congestive heart failure. Echocardiography revealed severe right ventricular dysfunction along with passive minimally pulsatile pulmonary blood flow suggesting very high systemic venous pressures. This was confirmed with cardiac catheterization in which the pressures of superior vena cava and inferior vena cava (19 mmHg) were higher than the pulmonary artery pressures (17 mmHg). Elevation of systemic venous pressures above the pulmonary venous pressures, Fontan paradox, to drive the forward flow, is a specific feature of artificially created cavopulmonary shunts. Late stage of isolated right ventricular cardiomyopathy resulted in the spontaneous evolution of Fontan circulation with a nonfunctional right ventricle in this patient.

Ebstein's anomaly was diagnosed in a fetus at 24 weeks of gestation. There was significant cardiomegaly and severe tricuspid regurgitation (TR). There was functional pulmonary atresia with severe pulmonary regurgitation (PR) and this was causing a circular shunt. There was no fetal hydrops.

A nineteen-month-old girl was taken up for patent ductus arteriosus (PDA) device closure. A diagnostic catheter from the right femoral venous access entered the superior vena cava (SVC), through the azygos vein suggesting interruption of inferior vena caval with azygos continuity. Therefore, the PDA device was closed from the right jugular venous access. However, a postprocedure echocardiogram (echo) showed a patent inferior vena caval connection into the right atrium. An angiogram from femoral veins showed communication between the iliac veins and the azygos system, in addition to normal drainage into the inferior vena cava (IVC). Congenital communication between the iliac veins and the azygos system can mimic IVC interruption. An attempt to theoretically explain the embryological origin of the communication has been made.

An eight-year-old boy was evaluated for unexplained hemoptysis and cyanosis. A contrast echocardiogram was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed persistent ductus venosus (PDV) and aortopulmonary collaterals. Both the PDV and aortopulmonary collaterals were closed percutaneously. PDV is amenable for device closure after detailed anatomical evaluation. Prior to closure, it is important to ensure adequate portal vein arborization into the liver and normal portal pressure after test balloon occlusion.

Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus.

Background: Right ventricular (RV) dysfunction after surgical repair of Tetralogy of Fallot (TOF) is often asymptomatic and may be detected by tissue Doppler imaging (TDI). The severity of RV dysfunction is more after intracardiac repair with transannular patch (TAP)., Methods: One hundred seventy-three adult patients who have undergone surgical repair for TOF were prospectively analyzed for RV function using 2D echocardiography and TDI. RV function was compared between patients who have undergone intracardiac repair with and without TAP., Results: In both the patient sub-groups, TDI derived myocardial performance index (MPI) and myocardial velocities were abnormal even when 2D echocardiography derived RV functional area change was normal. TDI derived MPI was significantly higher (0.5 ± 0.1 vs. 0.4 ± 0 P < 0.001) and Systolic tricuspid annular velocity (Sa) (9.2 ± 1.3 vs. 10.8 ± 1.6 P < 0.001) was significantly lower in the TAP group. Older age at surgery and severity of pulmonary regurgitation on follow-up were among the significant predictors of TDI derived MPI., Conclusions: Asymptomatic RV dysfunction in surgically repaired adult TOF atients can be detected by TDI. Extent of RV dysfunction was significantly greater with patients requiring TAP, in those operated at older age, and in patients with severe pulmonary regurgitation.