Rare presentation of eosinophilic granulomatosis polyangiitis with left ventricular endomyocardial fibrosis in a child.

Eosinophilic granulomatosis polyangiitis represents less than 2% of vasculitis cases in childhood. Children have worse long-term outcomes and higher mortality. Cardiac involvement portends a worse prognosis. We describe here an adolescent girl who presented with heart failure and stroke. Her blood investigations showed eosinophilia and high IgE levels. Cardiac evaluation revealed myocarditis, intracardiac thrombus, and endomyocardial fibrosis, a rare presentation of this disease in childhood.

Clinical characteristics and outcomes of patients admitted with acute heart failure: insights from a single-center heart failure registry in South India.

BACKGROUND: The epidemiology of HF in India is largely unexplored. Current resources are based on a few hospital-based and a community-based registry from North India. Thus, we present the data from a single hospital-based registry in South India. Patients admitted with acute heart failure over a period of 1 year were enrolled in the registry and were characterized based on their ejection fraction (EF) measured by echocardiogram. The clinical profile of the patients was assessed, including their in-hospital outcomes. One-way ANOVA and univariate analysis were performed for comparison between three EF-based groups and for the assessment of in-hospital outcomes. RESULTS: A total of 449 patients were enrolled in the registry, of which 296, 90, and 63 patients were categorized as, HFrEF, HFmrEF, and HFpEF, respectively. The prevalence of HFrEF was higher (65.99%). The mean age (SD) of the study cohort was 59.9±13.3. The majority of the patients presented with acute denovo HF (67%) and were more likely to be males (65.9%). The majority of patients presented with warm and wet clinical phenotype (86.4%). In hospital mortality was higher in HFmrEF (3.3%). CONCLUSION: Patients with HFrEF had high adherence to guideline-directed medical therapy (GDMT). HFrEF patients were also likely to have longer hospital stay along with a worsening of renal function. The in-hospital mortality was comparable between the EF-based groups. Additionally, the association of clinical phenotypes with outcome highlighted that patients in warm and wet phenotype had a longer length of hospital stay, whereas the mortality and worsening renal function rates were found to be significantly higher in the cold and wet group.

Supraventricular tachycardia in one of the twins: The ethical dilemmas involved in treatment.

The pediatric cardiologist now has an important role to play in fetal medicine. They are often called upon to manage fetal cardiac problems such as arrhythmias or perform fetal cardiac interventions such as balloon valvuloplasty or atrial septostomy. In these scenarios, it becomes very important for the pediatric cardiologist to understand the concepts of "fetus as a patient," "viability," etc., and their implications in management. We try to shed light on these principles through our case scenario of managing supraventricular tachycardia in one of the fetuses of a twin pregnancy.

Common arterial trunk with intact septum and hypoplastic right ventricle: An uncommon embryological entity.

Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major outflow cushions. This invariably leads to the presence of an associated large juxta-arterial ventricular septal defect. Overriding of the ventricular septum leads to its biventricular origin in more than 2/3rd of cases. Uncommonly, CAT may be associated with an intact ventricular septum (IVS) and univentricular origin. We describe an infant with CAT, intact IVS, hypoplastic right ventricle, and Ebstein's anomaly of the tricuspid valve who presented to us and palliated successfully. The embryological mechanisms are discussed in this report.

Prospective concurrent head-to head comparison of three different types of nitinol occluder device for transcatheter closure of secundum atrial septal defects.

AIMS: Previous comparisons between AMPLATZER septal occluders and other designs were retrospective, non-randomised, non-concurrent and involved fewer patients. A prospective concurrent head-to-head comparison of AMPLATZER (ASO), Cera (CSO) and Figulla (FSO) septal occluders was planned to study the patient outcomes. METHODS AND RESULTS: The three occluders were serially allocated in a cycle of three to consecutively included patients. Demographic, procedural details and complications were analysed. After calculating a sample size of 122 patients in each group, additional patients were recruited to ensure at least 80% follow-up. Four hundred and fifty (450) consecutive patients equally divided among the three designs were comparable in all parameters. There were no major complications and procedural success was 99.6%. The defects and device sizes were similar in all groups; the delivery system was significantly smaller with the ASO. The FSO needed special deployment techniques less often and formed a cobra deformity more often, though this was not statistically significant. Patient outcome was similar among the groups at a follow-up of 12-47 months. CONCLUSIONS: The new occluders are comparable to the ASO with good outcomes and low complication rates in the current era. The new modified structural designs do not show any advantages in terms of procedural complications on early and midterm follow-up, but long-term studies are warranted.

Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma.

BACKGROUND: Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein. OBJECTIVE: The aim of the study is to observe the efficacy of everolimus in infants with significant CR. MATERIALS AND METHODS: This is a single-center prospective observational study including infants with significant CR causing either clinical symptoms or obstruction to the blood flow. Everolimus was administered at a dose of 4.5 mg/M2/wk till the symptomatic improvement. Liver and renal function tests were monitored during treatment. RESULTS: There were six cases of suspected CR included in the study. Median age and weight of patients were 5 days (range: 1-90 days) and 3.2 kg (range: 2.2-4.5 kg), respectively. One patient was excluded after surgical excision biopsy during concomitant closure of associated large perimembranous ventricular septal defect confirmed it as a fibroma. The remaining all five cases showed regression of tumor during mean follow-up of 6.1 ± 5.1 months. One child developed varicella infection necessitating temporary discontinuation of medicine during follow-up. One case had sudden death at 4 months of age. CONCLUSION: Everolimus appears to be useful in selected cases of symptomatic CR. Multicentric studies are needed to determine its safety and efficacy in larger population.