Spontaneous closure of ventricular septal defect in a case of double outlet right ventricle.

Abstract

SUMMARY A 5 year old child, previously diagnosed as having tetralogy of Fallot, was admitted to hospital in severe congestive heart failure. The electrocardiogram showed left anterior hemiblock and incomplete right bundle-branch block, neither of which was previously present. The child died in intractable congestive heart failure and the necropsy showed a double outlet right ventricle with complete spontaneous closure of the subaortic ventricular septal defect by fibrous tissue. The possible mechanism involved in the production of this unusual complication of double outlet right ventricle is discussed, together with an explanation for the electrocardiographic changes.It is well known that an isolated ventricular septal defect can reduce in size spontaneously, and even close completely.'"3 Occasionally, this development has been described in patients with complex congenital heart disease, in whom the ventricular septal defect was physiologically advantageous such as the "classic" double outlet right ventricle, that is with bilateral conus,46 or tricuspid atresia.7 8 Conversely, complete spontaneous closure of a ventricular septal defect has never been described, to our knowledge, in patients with tetralogy of Fallot or with double outlet right ventricle and infundibular features of Fallot's tetralogy.We report the clinical and anatomical findings of a case of double outlet right ventricle with some infundibular features of Fallot's tetralogy, in which there was spontaneous closure of the ventricular septal defect.Case report A 5 year old girl was born with a cyanotic cardiac defect. She was admitted to our hospital in congestive heart failure.A heart murmur had been heard since birth.Cyanosis appeared when she was 5 months old. At 3 years of age she underwent a right heart catheterisation in another institution which disclosed tetralogy of Fallot. The electrocardiogram showed right ventricular hypertrophy including QRS right axis deviation (Fig. IA) There was oedema at the ankles, hepatomegaly, ascites, and bilateral pleural effusions. The precordium was hyperactive. The first heart sound was normal and the second sound was single. A 3/6 ejection systolic murmur and a third heart sound were heard in the third left intercostal space on the sternal border.The chest x-ray film showed cardiac enlargement, pulmonary venous congestion, bilateral pleural effusions, and a left aortic arch.The electrocardiogram recorded on admission (Fig. IB) showed normal sinus rhythm, PR interval 0-18 s, left atrial enlargement, QRS width 0-08 s, and QRS mean frontal axis -60°. The QRS pattern indicated left anterior hemiblock; QRS morphology was Qrs, QS, and rS in VI, V2, and V6, respectively, indicating a posterior, rightward, and counterclockwise inscription of the QRS forces in the transverse plane.Another electrocardiogram was recorded four days later (Fig. IC) and disclosed an incomplete right ventricular conduction defect (qR morphology in VI, with tall R wave and mild increase in QRS width). A more pronounced superior deviation...

Double Outlet Right Ventricle with Intact Atrial Septum and Restrictive Ventricular Septal Defect: An Analysis of Two Cases

“…The different mechanisms for spontaneous closure include apposition to and eventual fusion with the rims of the defects by leaflets of the tricuspid or mitral valves, progressive muscular ''encroachment'' of the margins of the defects with subsequent fibrosis, and covering by endocardial proliferation [12]. In a case reported by Marino et al [8], the patient initially was diagnosed as having tetralogy of Fallot at three years of age and there was late development of severe congestive heart failure and fatal evolution at five years of age. Necropsy showed DORV with complete spontaneous closure of the subaortic VSD by accessory endocardial fibrous tissue.…”

“…1), who did not have the initial vectors, a finding consistent with septal fibrosis, similar to cases with severe aortic stenosis. Chest roentgenograms usually reveal biventricular enlargement, left atrial enlargement, and increased pulmonary vascularity [8,9]. Echocardiography plays an important role in the diagnosis of anatomical details such as the specific relationship of VSD to the cardiac outlets and possible atrioventricular valve malformations, thus aiding in establishing the precise method of surgical repair.…”

Double-Outlet Right Ventricle and Double-Outlet Left Ventricle

“…Occasionally the VSD can become restrictive, causing left ventricular outlet obstruction similar to subaortic stenosis. 30,55 …”

Disadvantageous closure of the interventricular communication in double outlet right ventricle.

“…In 12 of these there was atresia or stenosis of the mitral valve and either an absent left ventricle as judged by gross examination or no details of ventricular morphology.4-9 Four cases had two well formed ventricles and an intact ventricular septum. '0- 13 We report a further case of double outlet right ventricle, in which the only communication between the two ventricles was iatrogenic.…”