Clinical course and prognosis of dilated cardiomyopathy in children

Background: Epidemiology and clinical course of dilated cardiomyopathy (DCM) in children and infants are not well established. Thus, this study aims to investigate the clinical course and prognosis of DCM in childrenMethods: This was a single-center, prospective, observational study conducted at a tertiary-care center in India between February 2011 and September 2012. A total of 31 patients admitted to the paediatric department diagnosed with DCM were included in the study. Patients were divided into three groups based on the age at the time of diagnosis: 0-3 years, >3-12 years and >12-16 years. Among the study population, 28 patients were followed up for a mean period of 1.44 years and three patients were lost to follow-up.Results: Of the 31 patients, 11 patients were male with a mean age of 8.9±6 years and 20 patients were female with a mean age of 8.3±6 years. All patients were presented with same characteristics of New York heart association (NYHA) class III-IV dyspnoea and fatigue. Among 28 patients who were followed-up for a mean period of 1.44 years, 20(71.4%) patients died and eight patients were on follow up. Of the eight patients, five patients were with NYHA class III symptoms and three patients were with NYHA class I-II symptoms.Conclusions: Dilated cardiomyopathy in children is a very serious disease with a grave prognosis. Patients with NYHA III-IV symptoms have a very high mortality rate and potential use of other therapies remains to be fully evaluated in paediatric population

Unguarded left atrioventricular orifice: An unusual cause of hypoplastic left ventricle and double-outlet right ventricle with intact ventricular septum

An unguarded atrioventricular orifice is an extremely rare congenital anomaly characterized by the absence of the atrioventricular valve in varying proportions. While atresia of the mitral or aortic valves are usually described as causes for hypoplastic left heart, our case highlights the role of free atrioventricular valve regurgitation and consequent volume loss of the left heart, giving rise to a small left ventricle. There was an associated double-outlet right ventricle and Type B aortic interruption. While we have attempted to discuss the complex management options in this scenario, the parents decided to withdraw further care

Hutchinson–Gilford progeria syndrome with severe calcific aortic valve stenosis

Hutchinson–Gilford progeria syndrome (HGPS) is a rare premature aging syndrome that results from mutation in the Laminin A gene. This case report of a 12-year-old girl with HGPS is presented for the rarity of the syndrome and the classical clinical features that were observed in the patient. All patients with this condition should undergo early and periodic evaluation for cardiovascular diseases. However, the prognosis is poor and management is mainly conservative. There is no proven therapy available. Mortality in this uniformly fatal condition is primarily due to myocardial infarction, strokes or congestive cardiac failure between ages 7 and 21 years due to the rapidly progressive arteriosclerosis involving the large vessels

Double outlet right ventricle, total anomalous venous return, total anomalous hepatic venous drainage and supra mitral ring in a child with Ivemark`s syndrome

Double outlet right ventricle, total anomalous venous return, total anomalous hepatic venous drainage and supra mitral ring with Ivemark syndrome is an unusual combination of cardiac malformations.These complexities of congenital anomalies can pose problem in preoperative diagnosis and surgicalmanagement. We present investigation and surgical management of a three month old child with thesementioned rear combination of anomalies and a brief revive of literature.</p

Three-dimensional transesophageal echocardiography-guided transcathetar closure of ruptured noncoronary sinus of valsalva aneurysm

Sinus of Valsalva aneurysm accounts for only 1% of congenital cardiac anomalies. Sinus of Valsalva aneurysm can cause aortic insufficiency, coronary artery flow compromise, cardiac arrhythmia, or aneurysm rupture. Three-dimensional transesophageal echocardiography (3DTEE) represents an adjunctive tool to demonstrate the ruptured sinus of Valsalva with better delineation. We present an adult patient with rupture of noncoronary sinus of Valsalva aneurysm into the right atrium (RA). 3DTEE accurately delineated the site of rupture into the RA and showed the exact size and shape of the defect, which helped in the successful transcatheter closure of the defect with a duct occluder device

Giant coronary-pulmonary fistula with pulmonary atresia, ventricular septal defect, and coronary anomaly: A case report and review of literature

Congenital coronary-pulmonary artery fistula is a rare condition and is usually associated with pulmonary atresia. We present a 10-year-old girl with circumflex coronary artery to pulmonary artery (PA) fistula with a giant dilated circumflex coronary artery, ventricular septal defect (VSD), pulmonary atresia, and anomalous originofleft anterior descending (LAD) artery from right coronary sinus. The patient underwent surgical correction. We report the investigation, treatment, and review of literatureof this rare congenital anomaly associated with dilated circumflex coronary artery