Background : COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. Aims : The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design : This is a retrospective, multicentric, observational study. Methods : We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions : The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Background : Outcome data of children with heart disease who acquired COVID-19 infection are limited. Aims : We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection. Settings and Design : This is a retrospective, multicentric, observational study. Materials and Methods : The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group. Results : From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3–96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients (n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% (n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period (P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9–41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1–814.7, P = 0.046). Conclusions : Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes.

Background : Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. Methods : Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1–4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. Objectives : A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. Results : Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1–85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. Conclusion : Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.

Background : Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI. Methods : Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed. Results : Seven patients aged 17–60 years (median 26 years) had stenosed RV-PA conduits implanted 5–17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50–137 mmHg) to 12.7 ± 6.4 mmHg (5–20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10–22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events. Conclusions : Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Background : Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). Methods : Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. Results : Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure (n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1–39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. Conclusions : Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients.

Background : During repair of tetralogy of fallot (TOF) we modified surgical strategies to preserve the valve and annulus if the pulmonary valve leaflets are pliable and not significantly dysplastic. Methods : Initially, the repair was done from the main pulmonary artery (Group-1, 215 patients) and later through an additional incision in the infundibulum of the right ventricle (Group-2, 73 patients). Recently, we changed the approach to commissurotomy of the fused leaflets by releasing the supra valvar tethering and delamination of the cuspal apparatus till the base to improve the mobility of the cusps and do a controlled commissurotomy (Group-3, 14 patients). With delamination, we could extend the limit of the repair to a z-score of -3.5. Results : There was no hospital mortality; two patients died at home after discharge. A mean follow-up of 42.01 months ± 19.25 is available for 198 patients (92%) for group 1, 16.03 ± 7.45 for group 2, and 4.07 ± 2.09 for group 3. The re-intervention-free survival is 94.4% in group 1. The z value improved from -3 (-3–-2) to -1.2 (-3 – 0), P = 0.001 in Group 1, from -2.8 (-3–-2.4) to -1 (-1.1–-0.7), P = 0.001 in Group 2 and from –3 (-4–-3) to -1, P = 0.001 in Group 3. In all the groups, there was trivial or mild pulmonary regurgitation. Conclusions : During repair of TOF, adequate valve/annulus sparing is possible if the repair is done from both the main pulmonary artery and infundibular incisions using the delamination technique.

Objectives : Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects. Aim of our study was to evaluate the effect of concomitant PVR with TOF repair on right ventricular (RV) parameters, cardiopulmonary exercise capacity, and bioprosthetic valve durability at mid-term. Materials and Methods: Between January 2013 and August 2018, 37 adolescents and adults with TOF who had hypoplastic pulmonary annulus underwent concomitant TOF repair with PVR at our institute. We retrospectively collected the data from the hospital records including follow-up. Results : Mean age of the patients was 18.48 ± 7.53 years. Bioprosthetic valve size ranged from 19 mm to 25 mm. There was no early or late mortality. No patient had developed significant perioperative complications. At a mean follow-up of 53.3 ± 16.4 months, there was no significant change in mean QRS duration, RV function, RV end-systolic and end-diastolic dimensions, RV myocardial performance index, and functional status (including NYHA class and 6-min walk test) compared to at-discharge values. Four patients developed prosthetic valve degeneration with mild PR and without significant increase in gradient. Conclusion : Concomitant PVR with TOF repair in adult provides excellent mid-term outcome, with a minimal rate of pulmonary valve degeneration. It not only eases the early postoperative course but also preserves the RV function as well as functional status at mid-term.

Aims : The utility of beta-blocker therapy in infants with heart failure (HF) due to significant left-to-right shunt lesions is not known. The study aimed to assess the efficacy and safety of propranolol in infants with HF due to moderate-to-large ventricular septal defect (VSD). Methods : The prospective randomized trial included 80 infants with HF and moderate-to-large VSD, randomly allocated to receive either conventional therapy alone (n = 40) or propranolol plus conventional therapy (n = 40). The primary endpoint was a composite of all-cause mortality, hospitalization for HF and/or chest infection, and referral for surgery. The secondary clinical outcomes were the individual components of the composite endpoint. In addition, the patients were followed up to detect safety outcomes, for example, bronchospasm, bradyarrhythmia, and worsening HF symptoms. Results : The addition of propranolol therapy to the conventional medications did not result in significant improvement in the primary composite endpoint (32.50% vs. 52.50%; P = 0.07). There was a trend toward improvement, but the study is underpowered for this important question. However, propranolol therapy significantly decreased the risk of hospitalization (12.50% vs. 32.50%; P = 0.03) and worsening of Ross HF class (5.41% vs. 28.21%; P = 0.01) as compared to conventional therapy (estimated number needed to treat = 5). Propranolol did not result in any significant safety concerns in these infants except bronchospasm in an infant. Conclusions : Propranolol therapy in infants with significant left-to-right shunt may prevent worsening in HF symptoms and hospitalization and is well tolerated. However, it does not reduce mortality or need for surgery.

Background : Hemodynamically significant patent ductus arteriosus (PDA) is frequently encountered in preterm infants sometimes requiring surgical attention. Although PDA ligation is regularly performed in the operating room, conducting it at the bedside in a neonatal intensive care unit (NICU) and its anesthetic management remains challenging. Aim : We aim to discuss the anesthetic considerations in patients undergoing bedside PDA ligation and describe our experience highlighting the feasibility and safety of this procedure. Setting and Design : The study was conducted in the NICU in a tertiary care hospital; This was a retrospective, observational study. Methods : Preterm infants scheduled for bedside PDA ligation using a predefined anesthesia protocol between August 2005 and October 2020 were included. Statistical Analysis Used: Quantitative data were presented as median with interquartile range and categorical data were presented as numbers and percentage thereof. Results : Sixty-six premature infants underwent bedside PDA ligation. Thirty-day mortality was 4.5% (3 infants), but there were no procedural deaths. One (1.5%) patient had intraoperative endotracheal tube dislodgement. Three (4.5%) infants had postoperative pneumothorax requiring an additional chest tube insertion. Twenty-one (32%) patients required initiation of postoperative inotrope/vasodilator therapy within 6 h. Three postligation cardiac syndromes (≥ Grade-III mitral regurgitation with left ventricular dysfunction and hypotension) occurred. Conclusions : Although anesthesia for preterm neonates undergoing bedside PDA ligation poses unique challenges, it can be safely conducted by following a predetermined standardized anesthesia protocol. Its successful conduct requires utmost vigilance and pristine understanding of the principles of neonatal and cardiac care.

Background : Pulmonary vascular resistance, an important determinant of shunting across ventricular septal defects (VSD), rises at both extremes of lung volume. Aims : We sought to determine the effect of changes in tidal volumes (VT) on pulmonary blood flow (Qp), systemic blood flow (Qs), and shunt (Qp/Qs) in children with VSD. Setting : Single-center teaching hospital. Design : Prospective observational study. Methods : Thirty children with a mean age of 11.8 ± 5 months undergoing surgical closure of VSD were studied. Hemodynamics and shunt-related parameters were assessed using transthoracic echocardiography measured at three different VT i.e. 10, 8, and 6-ml/kg keeping the minute ventilation constant. Results : Reduction in VT from 10 to 8 to 6 ml/kg led to a reduction in gradient across VSD measuring 23.5, 20 and 13 mmHg respectively (P < 0.001). Similarly, right ventricluar outflow tract (RVOT) diameter, RVOT velocity time integral, Qp (57.3 ± 18.1, 50.6 ± 16.9, 39.9 ± 14.7 mL; P < 0.001), Qs (24.1 ± 10.4, 20.0 ± 8.7, 15.3 ± 6.9 mL; P < 0.001) and peak airway pressure (17.2 ± 1.5, 15.8 ± 1.3, 14.5 ± 1.2 cmHg; P < 0.001) showed progressive decline with decreasing VT from 10 to 8 to 6 ml/kg, respectively. However, Qp/Qs (2.4 ± 0.4, 2.6 ± 0.4, 2.6 ± 0.4) demonstrated a minor increasing trend. Conclusion : Lower V_T reduces the gradient across VSD, the pulmonary blood flow, and the peak airway pressure. Hence, ventilation with lower V_T and higher respiratory rate maintaining adequate minute ventilation might be preferable in children with VSD. Further studies are required to confirm the findings of this pilot study.

Background : Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. Objectives : The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. Methods : This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications. Results : A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19–35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left subclavian artery (ALSA) was suspected in 31 fetuses, double aortic arch (DAA) in 3, and circumflex aorta in 1. Twenty-six (74%) patients had successful deliveries. One patient had a spontaneous miscarriage, 2 underwent termination, and 6 were lost to follow-up. Postnatal assessment showed RAA with ALSA in 18, DAA in 5, circumflex aorta in 2, and no abnormality in 1. Twenty-two (86%) were operated (RAA with ALSA: 17, DAA: 4, and circumflex aorta: 1) and four were waiting for surgery. Two patients died due to prematurity-related complications. All survivors are symptom free during follow-up (median: 2.24; range: 0.2–5.6 years). Conclusions : Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs.

Introduction : Myocarditis remains an under-diagnosed entity among children. We evaluated the spectrum of electrocardiogram (ECG) changes and arrhythmias in children with myocarditis. Methods : A single-center prospective observational study was conducted over a period of 18 months at a public university hospital, which included all cases with myocarditis from the ages of 1 month to 12 years. Myocarditis was diagnosed according to standard criteria. Arrhythmias were detected by 12-lead ECG or by multiparameter monitors. Results : There were 63 children with myocarditis. Sinus tachycardia remained the most important ECG finding (61, 96.8%) followed by ST-T changes (30, 47.6%), low voltage QRS complexes (23, 36.5%), and premature complexes (11, 17.4%). Sustained arrhythmias were seen in 14/63 (22.2%) of the children (Group A), while the remaining 49 patients were designated as Group B. There were 11 (17.5%) cases with sustained tachyarrhythmias, comprising 5 with supraventricular tachycardia, 4 with ventricular tachycardia, and 2 with atrial flutter/fibrillation. Bradyarrhythmias were seen in 3 patients, including 2 children with atrioventricular block and 1 with severe sinus bradycardia. A longer hospital stay of 18.5 (4.75) days vs. 13 (4) days, P = 0.001), and more ST-T changes [12 (85.7%) vs. 18 (36.73%), P = 0.003] were seen in Group A. Multivariate regression analysis found only the presence of ST-T changes as predictors for arrhythmia. Conclusions : A variety of arrhythmias and other ECG changes were commonly seen in children with myocarditis. Sustained arrhythmias were seen in one-fifth of the patients, being associated with ST-T changes and a longer hospital stay.

Junctional ectopic tachycardia (JET) is more common in its postoperative form. A thorough understanding of its etiology, pathophysiology, and management strategies is essential. Classically, postoperative JET is considered to arise from surgical trauma. Genetic susceptibility and an intrinsic morphologic/functional defect in the conduction system inherent in congenital heart diseases likely play a significant role. The devastating effects on postoperative hemodynamics warrant prompt attention. A multipronged management approach with general measures, pharmacotherapy, and pacing has decreased morbidity and mortality. Amiodarone and procainamide remain the preferred drugs, while ivabradine appears promising. Carefully planned randomized trials can go a long way in developing a systematic management protocol for postoperative JET.

The advent of the arterial switch operation for the treatment of transposition of great arteries (TGA) made the atrial switch operation largely redundant and its use in the developed world is now confined to the atrial component of the double-switch operation for congenitally corrected TGA. In resource-limited countries, however, it remains relevant as a treatment option for patients presenting late with transposition and a regressed left ventricle. The operation is intricate with many potential pitfalls, and this “how I do it” article describes the operative steps in detail to enable surgeons to learn this procedure and make it a part of their surgical armamentarium.

Virtual reality (VR) is increasingly used for presurgical planning and teaching during surgery. However, VR aided presurgical planning toolbox for left ventricular assist device (LVAD) implantation is not widely available. We investigated the use of a VR environment with wearable headsets and touch controllers in simulating an implant in an 11-year-old boy. The technology played a significant role in the optimal positioning of the LVAD.

The multi-inflammatory syndrome in children is a poorly understood febrile illness potentially linked to an immune response to COVID-19 infection. The disease is characterized by fever and elevated acute-phase reactants. A number of children with clinical and laboratory evidence of cardiovascular involvement have normal echocardiograms by conventional assessment. The peak left atrial longitudinal strain obtained by atrial deformation analysis could potentially be diagnostic of this condition in children who do not have abnormalities identified on conventional assessment.

Perimembranous ventricular septal defect (pmVSD) is a common congenital heart disease that is sometimes associated with indirect left ventricle (LV) to right atrium (RA) shunt (indirect Gerbode defect). This defect has a rare chance of spontaneous closure and therefore was usually closed surgically in the past, but more recently transcatheter closure has been reported by a few authors. In our study, we have described a series of 14 children (age ranging from 1.2 to 12 years and weight ranging from 7.2 to 25.5 kg) with the above-mentioned defect which were closed by various interventional devices. The procedures were successful in complete elimination of pmVSD and immediate reduction of indirect LV-RA shunts with negligible residual tricuspid regurgitation on follow-up. In our midterm experience, the judicious use of double-disc devices is efficacious for occluding pmVSD associated with indirect Gerbode defect.

Sudden cardiac death (SCD) accounts for 15%–60% of mortality in patients with heart disease. Generally, this has been attributed to ventricular tachyarrhythmia. However, ventricular tachyarrhythmia has been documented or strongly suspected on clinical grounds in a relatively small proportion of SCD patients (8%–50%). Attempted prophylaxis of SCD by implantation of cardioverter-defibrillator is associated with variable success in different subsets of high-risk cardiac patients (30%–70%). A significant number of SCD, therefore, appear to be due to catastrophic circulatory failure. Multiple interdependent compensatory mechanisms help to maintain circulation in advanced cardiac disease. Rapid, unexpected, and massive breakdown of the compensated state can be precipitated by small and often imperceptible triggers. The initial critical but stable state followed by rapid circulatory failure and death has been considered to be analogous to snow avalanches. It is typically described in patients with left ventricular (LV) dysfunction (ischemic or nonischemic). It is now recognized that SCD can also happen in conditions where the right ventricle (RV) takes the brunt of the hemodynamic load. Advanced pulmonary arterial hypertension and operated patients of tetralogy of Fallot with pulmonary regurgitation are of particular interest to pediatric cardiologists. A large amount of data is available on LV changes and mechanics, while relatively little information is available on the mechanisms of RV adaptation to increased load and RV failure. Whether the triggers and the decompensatory processes are similar for the two ventricles is a moot point. This article highlights the currently available knowledge on the pathophysiology of SCD in RV overload states, with special reference to RV adaptive and decompensatory mechanisms, and therapeutic measures that can potentially interrupt the vicious downward course (cardiac avalanches).

In the midst of the COVID-19 pandemic, we report two cases of children presenting with typical diagnosis of pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) who suffered from complete heart block requiring pacemaker placement which is an unusual presentation of PIMS-TS. We also compared it with the already existing data with similar manifestations. PIMS-TS is reported to occur in children with predominantly gastrointestinal symptoms, hemodynamic instability, and myocardial dysfunction. The implications of development of atrioventricular block during critical illness in PIMS-TS are yet unknown. Both patients had an otherwise normal cardiac structure and had no gastrointestinal symptoms but suffered complete heart block without any other identifiable etiology, both requiring temporary pacemaker placement. While one child recovered completely with medical management, the other child required permanent pacemaker placement. While we cannot be certain that COVID-19 was the cause, complete heart block appeared to be temporally related to COVID-19 infection in both patients, and hence, it is important for pediatricians to be aware of the potential manifestation of this disease.

The pulmonary dominant variant of the common arterial trunk has always been reported to be associated with aortic coarctation, or interruption of the aortic arch, along with a duct-dependent systemic circulation. This mandates a complex surgical repair with attendant high surgical mortality. We report a 23-day-old baby with a pulmonary dominant trunk with mild aortic hypoplasia but with an arch free of coarctation or interruption, who underwent successful surgical repair. In the preoperative evaluation of a common arterial trunk, pulmonary dominance may not necessarily denote an adverse risk factor when the aorta is only mildly hypoplastic.

Portosystemic shunts are rare congenital malformations that come to attention due to various hepatic and extrahepatic manifestations. Management of this malformation is dictated by the presence and adequacy of intrahepatic portal radicals. Staged occlusion of the shunt is recommended in patients with severely hypoplastic portal radicals. We describe a novel transcatheter technique that we improvised for staged occlusion of Abernethy malformation in a 2-year-old girl.

This report describes two cases of tachycardia-induced cardiomyopathy secondary to incessant ectopic atrial tachycardia (EAT) in an infant presenting with severe left ventricular dysfunction and hemodynamic instability. The two cases were managed differently. The first required mechanical ventilation and was resistant to conventional antiarrhythmic drugs. After the initiation of enteral ivabradine (0.15mg/kg) the heart rate slowed with significant improvement in hemodynamics, peripheral perfusion and sinus rhythm was restored after 12 hours. Ivabradine was continued and the patient was discharged home after 10 days of hospitalization. The second case was managed by early initiation of ivabradine and resulted in restoration of sinus rhythm within 4 hours, thus avoiding trials of conventional anti-arrhythmic drugs with unstable hemodynamic profile. The infant was discharged after 5 days of hospitalization on ivabradine..

Kawasaki disease (KD) is the most common pediatric vasculitis with coronary involvement feared as the most serious complication. The reported case describes a child presenting initially with atypical KD and coronary artery aneurysms. He was treated with intravenous immunoglobulin and aspirin. In spite of adequate compliance and no clinical recurrence, serial echocardiography revealed nonregression of aneurysm and new-onset moderate aortic regurgitation (AR) in the subacute phase produced by prolapse of noncoronary cusp of the aortic valve. AR without aortic root dilatation from persistent inflammation of the valve leaflets in KD is a rare phenomenon. This case demonstrates unusual cardiac manifestations of KD and reoriented our protocol for long-term surveillance in infantile KD.

Diagnosis of isolated total anomalous pulmonary venous connections (TAPVCs) is relatively rare in fetal life, especially in early gestation. We report a case of a fetus diagnosed with the supracardiac type of TAPVC at 23 weeks gestation, with evidence of obstruction to connection of the common vertical vein to the superior vena cava. The neonate had a critical presentation at birth and underwent an emergency surgical repair immediately after birth with excellent outcome on short term follow-up with the resolution of pulmonary artery hypertension.