Spontaneous closure of ventricular septal defects

Indian journal of child health, 2022

D ouble-chambered right ventricle (DCRV) is an extremely rare condition and accounts for 0.5-2% of congenital heart disease, occurring in as many as 10% of patients with ventricular septal defect (VSD) [1] (Figs. 1a-b and 2). DCRV is thought to be caused by an anomalous muscle bundle (AMB) dividing the right ventricle (RV) into high and low pressure chambers, which result in progressive right ventricular outflow obstruction [2]. Male-to-female ratio is 2:1. No inheritance pattern or risk factors are described. Sporadic cases reported in patients with Down and Noonan Syndromes. Associated congenital cardiac abnormalities are found in 80-90% cases. Isolated DCRV is exceptionally rare [3]. VSD is the most common defect, next being pulmonary stenosis. Other associations are double-outlet RV, tetralogy of Fallot, anomalous pulmonary venous drainage, transposition of the great arteries, pulmonary atresia with intact ventricular septum, and Ebstein anomaly [4]. VSD is usually large, Perimembranous and opens into high pressure proximal chamber, but it may open into distal chamber also.As the obstruction worsens across DCRV, associated VSD may progressively become smaller. Few studies reported that asymptomatic adults with AMB and intact ventricular septum may have had VSD that underwent spontaneous closure [5]. The clinical significance of DCRV would depend on degree of obstruction and associated lesions [6]. In general, patients with DCRV are diagnosed in infancy or childhood and isolated DCRV presenting in adulthood is exceedingly scarce. [3,4,7-9]. Perhaps, in our adult female patient, DCRV was present since her birth, but has been unmasked now, on a routine medical checkup. Hence, we are reporting this case of isolated DCRV in an asymptomatic young female in a pediatric journal. CASE REPORT A 22-year-old women was referred to us, for evaluation of murmur, heard over precordium, while she was undergoing treatment of a minor upper respiratory tract illness. Even on deep interrogation, she claimed to be asymptomatic and denied any history of dyspnea, cyanosis, palpitations, chest pain, and loss of consciousness or swelling over feet or face. On physical examination of the lady, she was of average built without any physical deformity, having a weight of 45 kg and height of 144 cm. Her BP was 118 / 76 mmHg in the right upper extremity, in sitting position. Pulse rate was 86/min, RR was 14/min, and SPO 2

The American Journal of Cardiology, 1989

The American Journal of Cardiology, 1968

Anadolu kardiyoloji dergisi : AKD = the Anatolian journal of cardiology, 2005

European Journal of Echocardiography, 2006

The American Journal of Cardiology, 1966

Indian Journal of Child Health

Double-chambered right ventricle (DCRV) is an extremely rare condition caused by anomalous muscular bundle (AMB), dividing the RV into high- and low-pressure chambers. We are highlighting a case series of three rare patients of DCRV, presenting with diverse clinical and echocardiographic features. Case 1 is manifesting as isolated DCRV in an asymptomatic young female adult, with the AMB located in the subvalvular region of the right ventricular outflow tract. Case 2 is of a significantly symptomatic male infant, afflicted with DCRV, and associated with a large apical muscular ventricular septal defect (VSD). The AMB was situated near the RV apex, just proximal to the VSD. Case 3 represented an asymptomatic DCRV in an adolescent female along with a coexisting small hemodynamically insignificant VSD. Individually, all the three patients are exceedingly rare and their clinical and echocardiographic features were peculiarly distinctive, hence making this case series an interesting and e...

European Heart Journal – Cardiovascular Imaging, 2016

Introduction. Double-chambered right ventricle (DCRV) is a very rare congenital defect causing right ventricular outflow tract (RVOT) obstruction where the right ventricle (RV) is divided by aberrant muscle bundles into a high-pressure and low-pressure chamber. Case report. A 44-year-old man was referred to our center for progressive exertional dyspnea. He was previously diagnosed with Eisenmenger syndrome secondary to an uncorrected ventricular septal defect (VSD). Clinical evaluation showed cyanosis, a harsh systolic murmur along the left sternal border and hepatomegaly. The electrocardiogram revealed atrial fibrillation, right bundle branch block and right ventricular hypertrophy. Transthoracic echocardiography (TTE) showed the presence of a discrete obstruction in the RVOT (Fig.A), adjacent to the pulmonary valve, caused by an anomalous muscle bundle, consistent with DCRV. Both the right atrium (RA) and the RV were severely dilated, with marked RV trabeculations and moderate to severe RV systolic dysfunction. Moderate to severe secondary tricuspid regurgitation was also identified. The RV-RA systolic gradient (108 mmHg) was used as a surrogate for the intraventricular gradient. The left ventricular ejection fraction was normal. The patient had a large circumferential pericardial effusion with no signs of cardiac tamponade. The patient underwent right heart catheterization that showed severely increased right ventricular systolic pressure (108 mmHg) but normal pressure and arterial resistance in the pulmonary artery. The right ventriculogram identified an obstruction between the RV and the pulmonary valve (Fig. B). There was no VSD. Since the patient was cyanotic, the presence of an intracardiac shunt was suspected. Transoesophageal echocardiography (TOE) confirmed the diagnosis of DCRV and identified a right-to-left atrial shunt at the level of a patent foramen ovale. For further anatomical assessment of the RV obstruction and RV systolic function, a cardiac magnetic resonance (CMR) study was performed. It showed subinfundibular muscular obstruction and severe RV systolic dysfunction (Fig. C). Surgery was indicated (with a high perioperative risk), and the patient underwent resection of the muscle bundle and tricuspid annuloplasty. Unfortunately, the postoperative evolution was unfavorable, with nonresponsive RV failure and death. Discussion. DCRV is frequently associated (80-90%) with VSD/pulmonary valve stenosis. Isolated DCRV is very uncommon, therefore ruling out associated lesions is paramount. This disease is usually diagnosed in childhood. Hence, there is little data available in adults. Moreover, the presentation is frequently nonspecific. Differential diagnosis must be made with infundibular/ pulmonary valve stenosis. TTE is diagnostic in a few patients (15%), while TOE is superior, defining the full morphological details, including associated defects. Since surgical correction before RV failure carries a small risk and is curative, misdiagnosis must be prevented by accurate imaging of the whole RV, RVOT and pulmonic valve. While TTE is the first-line imaging tool, use of additional imaging techniques: TOE, cardiac catheterization, ventriculography and CMR adds useful information in many cases. Conclusion. This is a very rare presentation of an isolated DCRV in an adult patient. It underlines the importance of using multiple and complementary imaging techniques to establish the correct diagnosis.

Heart, 1971

Echocardiography, 2006