Successful hybrid management of hypertensive patent ductus arteriosus with unilateral absence of the right pulmonary artery - A case report.

Unilateral absence of pulmonary artery (UAPA) can be either isolated or associated with other congenital cardiac defects. It is a rare congenital lesion with a diverse clinical presentation. We present a case of absent right pulmonary artery and large patent ductus arteriosus (PDA) with severe unilateral pulmonary hypertension. We describe our strategy of staged ductus arteriosus closure in this patient and successful outcome on long term follow up.

Transcatheter management of combined patent ductus arteriosus and left pulmonary artery stenosis in congenital rubella syndrome: A series of three patients and an insight into case selection for intervention.

Transcatheter closure of patent ductus arteriosus (PDA) is now the standard of care with some exceptions. Best treatment for peripheral pulmonary artery (PA) stenosis in small children is still debatable. Surgical augmentation, balloon dilatation, and stenting are the available options with each having its own risks and limitations. Here, we are reporting complete transcatheter management of three cases of congenital rubella syndrome who had PDA and left branch PA stenosis by device closure and stent placement, with successful outcome in two cases and complication, leading to surgery in one. We also share our understanding of case selection for a better outcome.

New-onset headache after percutaneous atrial septal defect closure.

Background: Percutaneous closure of atrial septal defect (ASD) is a well-established procedure both in children and adults with very good long-term outcomes. Migraine headache (MHA) can be precipitated after ASD device closure and the mechanism is not fully understood. Methods: We reviewed medical records of all patients undergoing ASD device closure from January 2015 to January 2021 for new onset headache after the procedure. Diagnosis of migraine was established by guidelines of the International Headache Society. Results: Out of 325 patients undergoing ASD device closure, five patients (1.5%) of various age groups (range 3.5-35 years) complained of severe migraine-like headaches within 2 weeks of the procedure. MHA was reported exclusively by females. All the patients were treated with oral paracetamol for the management of headaches. Three of 5 (60%) patients had a positive family history of migraine. Three patients reported complete disappearance of symptoms within 4-6 months. While two others (40%) had ongoing symptoms at 6 months follow-up. Conclusions: MHA can be precipitated after ASD device closure. The risk is specifically high in females with a family history of migraine. Dual antiplatelet drugs for the first 3 months after device closure may prevent such episodes. Prospective studies are needed to establish the universal role of dual antiplatelet drugs after ASD device closure.

Transcatheter closure of ventricular septal defects in children less than 10 kg: experience from a tertiary care referral hospital in Eastern India.

OBJECTIVE: To assess the feasibility of percutaneous transcatheter device closure of ventricular septal defects in children weighing less than 10 kg. BACKGROUND: Although percutaneous transcatheter device closure of ventricular septal defect is a well-established method of treatment in older children and adolescents, there is limited data on device closure in small children weighing less than 10 kg. We present our institutional experience of transcatheter VSD closure in children weighing less than 10 kg. METHOD: Medical records were reviewed for 16 children, who were selected for device closure of ventricular septal defects based on the inclusion criteria. RESULTS: Out of 65 patients with a diagnosis of ventricular septal defect, 16 children less than 10 kg were attempted for percutaneous device closure. In 13 patients, the device was successfully released, and 3 patients needed surgical closure of the defect. Mean age and weight of the patients were 17.3 ± 12.7 months and 6.8 ± 3.2 kg, respectively. Mean defect size was 6 mm (range 3-10). There was no incidence of device embolisation or heart block or death. Five patients had residual left-to-right shunt immediately after the device release, which got closed by the first month's follow-up. We had one accidental perforation of right ventricular free wall at the time of crossing of the defect, which was successfully repaired surgically. CONCLUSION: Percutaneous device closure of ventricular septal defect in small children with weight below 10 kg is feasible with good short-term outcome. Careful patient selection is essential for procedural success and to avoid complications.

Hyperviscosity syndrome revisited.

Secondary erythrocytosis occurs in cyanotic heart disease as a physiological response to chronic hypoxia, and this leads to hyperviscosity and various complications of the same. Microvascular stasis due to hyperviscosity results in symptoms including headache, fatigue, paraesthesia, and loss of vision. An important and dreadful feature of hyperviscosity is overt thrombosis in organ systems, resulting in cerebrovascular accident and myocardial infarction. Limited body iron store in a state of secondary erythrocytosis brings forth iron-deficient microcytic red cells; these being more rigid and less deformable than normocytic cells, further aggravate vascular occlusion. The management of hyperviscosity syndrome starts with intravenous hydration and correction of latent iron deficiency. However, therapeutic phlebotomy may be employed as a rescue measure if symptoms persist despite correction of dehydration and anemia. We present a series of four patients with uncorrected cyanotic congenital heart disease who presented with a spectrum of features of hyperviscosity and discuss pathophysiology, clinical features, and management of hyperviscosity in detail.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

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.

Outcome of COVID-19-positive children with heart disease and grown-ups with congenital heart disease: A multicentric study from India.

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.

Rare presentation of transposition of great arteries and total anomalous pulmonary venous connection in an 18-year-old.

Transposition of great arteries (TGA) in association with total anomalous Pulmonary venous connection (TAPVC) is a rare anomaly, that is seldom reported in adulthood. While both the conditions are life threatening on their own, in combination they compensate for each other and can often present after weeks or months after birth. Transthoracic Echocardiography (TTE) can reliably diagnose this anomaly. Cardiac MRI can also be used to confirm the diagnosis. We describe a rare combination of TGA and TAPVC in an 18-year-old patient.

Sinus Node Dysfunction After Extracardiac Conduit and Lateral Tunnel Fontan Operation: The Importance of the Type of Prior Superior Cavopulmonary Anastomosis.

This review summarizes the influence of the type of prior superior cavopulmonary anastomosis in the studies that report a difference in the occurrence of sinus node dysfunction among extracardiac conduit Fontan and lateral tunnel Fontan operations along with clinical implications.