Two cases are described of a most unusual variant of two-chambered right ventricle. In both the ventricular septal defect was between the distal chamber of the right ventricle and the left ventricle. However the extensive dividing 'septum' between proximal and distal parts of the right ventricle converted the latter, haemodynamically, into part of the left ventricle. In the first case the dista...

Sinus of Valsalva (SV) rupture is a rare, cardiac complication after surgical repair of complex congenital heart disease. This paper reports a 4-year-old male child with double outlet right ventricle (RV) and pulmonary stenosis with superior-inferior arrangement of the ventricles, who was submitted to surgical repair using the "reparation a l'etage ventriculaire" procedure. A few months after a...

A parachute deformity of the tricuspid valve occurred in a heart with atrioventricular concordance, double outlet right ventricle, and straddling mitral valve. Although to the best of our knowledge parachute deformity of the tricuspid valve has not previously been reported, in this case its presence was insignificant in relation to the other lesions.

Anatomical studies were made on 70 necropsied hearts with atrioventricular defects from patients with situs solitus and atrioventricular concordance, all having a common atrioventricular orifice. The arterial connections were concordant in 68 and were double outlet right ventricle in two; cases with arterial discordance (transposition) or single outlet of the heart were excluded. It proved pos...

Closure of residual ventricular septal defect with an occluder is traditionally performed by a percutaneous transcatheter approach under radiographic guidance. However, this procedure may be of limited use in cases with unusually shaped defects and in patients with low body weight. Here, we report minimally invasive surgical device closure of a 6 mm residual ventricular septal defect under tran...

Ectopia cordis is a rare disease that occurs in 5.5 to 7.9 per million live births. Only 267 cases had been reported as of 2001, most (95%) associated with other cardiac anomalies. We studied the cardiac malformations associated in 6 patients with ectopia cordis. Depending on where the defect was located, the cases of ectopia were classified into four groups: cervical, thoracic, thoraco-abdomin...

In a previous study the possibility that tetralogy of Fallot and transposition of the great arteries may arise as a result of embryonic arrests in the normal rotation of the junction of the outflow tract and the great arteries was investigated. The results suggested that the development of other transposition complexes such as double-outlet right ventricle might also be related to arrests in th...

Ivemark syndrome or right atrial isomerism is a rare syndrome of asplenia / hyposplenia with malformation of heart and abnormal arrangement of internal organs of chest and abdomen and is classified under heterotaxy disorder. We describe here the case of a 14 year old boy diagnosed with asplenia, dextrocardia with double outlet right ventricle and midline liver.