Isolated ventricular inversion with hypoplastic right ventricle and pulmonary stenosis in an adolescent with situs solitus: a rare combination

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in the literature.

Melody valve insertion for relief of “Tricuspid” stenosis in an unbalanced atrioventricular septal defect

A Melody valve was successfully placed across a very stenotic right-sided component of a common atrioventricular valve because of ongoing troublesome arrhythmias in a young woman with an unbalanced atrioventricular septal defect, a very dilated right atrium and a hypoplastic right ventricle. Four years later, she remains well.

New Horizon of Intervention in Congenital Heart Disease: AFR in a Complex Cyanotic Patient

Double-inlet left ventricle (DILV) is the commonest form of the anatomic univentricular heart which has different ventriculoarterial connection; generally, the most prevalent type is DILV with the hypoplastic right ventricle on the left side. The disease is associated with several heart defects, and the treatment method is different based on the anatomy of the heart, but treatment methods are almost always palliative. In this paper, we described one adult patient with DILV and severe left AV (atrioventricular valve) stenosis who is managed with a novel palliative intervention; it means AFR (atrial flow regulator) device implantation for the first time.

Point ablation for macro-reentrant ventricular tachycardia associated with Ebstein’s anomaly and pulmonary atresia: a case report

We report a case with Ebstein’s anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.

A rhabdomyoma in the right ventricle presenting as hemodynamics of hypoplastic right heart

Rhabdomyomas are the most common paediatric cardiac tumours. The natural history of these tumours is mostly benign, and the tumour usually regresses spontaneously. Although surgical resection of these tumours is one of the considerations in patients with ventricular outflow obstruction, a palliation with Blalock–Taussig shunt is an alternative approach with the hope of regression of the tumour over time. We report a case of prenatally diagnosed rhabdomyomas in the right ventricle and its outflow presenting as hemodynamic simulating hypoplastic right ventricle in a newborn. She required prostaglandin and Blalock–Taussig shunts palliation for pulmonary flow and subsequent regression of tumours.

A Misdiagnosed Case of Double Outlet Right Atrium Associated With Hypoplastic Right Ventricle

Double outlet right atrium (DORA) is a rare congenital heart disease in which the right atrium opens into both ventricles. The reduced leftward motion of the interventricular septum causes a malalignment between the atrial and the ventricular septum at the cardiac crux, which is the pathognomonic feature of this heart defect. We describe a case of significant exertional desaturation in an adult patient who was diagnosed with DORA, restrictive right ventricle, and anomalous tricuspid valve. Subsequently, the patient underwent one-and-a-half ventricular palliation.