Clinical and pathologic findings of an unusual case of cardiac malformation are presented. The main features were those of atresia of the right atrioventricular valve associated with two distinct atrioventricular orifices connecting the morphologically left atrium to the underlying morphologically left ventricle. Other distinguishing characteristics were ostium secundum atrial septal defect, no...

introduction the eustachian valve (ev) remnant, when present in adults, is usually rudimentary. however, in echocardiographic examinations, it may appear as a mobile long structure in the right atrium, and it rarely protrudes into the right ventricle. when it is quite large, the ev remnant could be misdiagnosed as a right atrial tumor, thrombus, or vegetation. case presentation an 83-year-old p...

A 27-year-old man with complex congenital heart disease (double discordance and complete congenital atrioventricular block) previously implanted with a single-chamber pacemaker was successfully resuscitated from sudden death due to ventricular fibrillation (VF). Echocardiography showed a moderately dilated right systemic ventricle with systolic dysfunction (right ventricular ejection fraction 3...

conclusions full echocardiographic examination of the right ventricle, including rvot is essential for the diagnosis of this disease. surgical correction appears to be a successful strategy for management of this rare condition in adults. case presentation we report a 40-year-old man who was referred to cardiology clinic for work-up of dyspnea and a heart murmur found on his physical examinatio...

A 37 years old Thai pregnant woman with MCDA twins, para 1, was suspected of severe twin-twin transfusion syndrome (TTTS) at 21 weeks’ of gestation. Quintero staging was applied in this cases. Neither of the fetuses exhibited signs of hydrops. Abnormal four-chamber view was demonstrated in a recipient fetus. Two-dimensional and color Doppler showed complex cardiac malformations which include (1...

INTRODUCTION Double chambered right ventricle is a rare congenital cardiac anomaly in which the right ventricle is divided into two chambers by an anomalous muscle bundle. The diagnosis of this disorder is difficult in adults. Calcification of the tricuspid valve is extremely rare, and very few cases have been reported. Most cases of tricuspid valve calcification had a congenital disorder with ...

In the patient with congenital heart disease, the phenomenon termed 'circular shunt' implies that some of the right-to-left shunted blood returns to its chamber of orgin through intracardiac channels or communications, hence bypassing the systemic capillary bed. We are reporting the angiographic appearance of a right-sided 'circular shunt' in 2 patients with pulmonary atresia, diminutive, hyper...

Carpenter syndrome (Acrocephalopolysyndactyly type 2, OMIM 201000) is a rarely seen autosomal recessive disorder. In addition to abnormalities such as acrocephaly, craniosynostosis, facial asymmetry, polydactyly and syndactyly, obesity, hypogonadism, mental retardation, and corneal opacity, it may frequently be accompanied by congenital heart diseases such as ventricular septal defect, patent d...

Pulmonary arterial hypertension (PAH) is a right heart failure syndrome. In early-stage PAH, the right ventricle tends to remain adapted to afterload with increased contractility and little or no increase in right heart chamber dimensions. However, less than optimal right ventricular (RV)-arterial coupling may already cause a decreased aerobic exercise capacity by limiting maximum cardiac outpu...