Clinical, hemodynamic, echocardiographic, angiographic profiles and post-operative outcomes among DCRV patients from a tertiary care referral center in India

Background: This retrospective study of data from 2006 to 2018 at a tertiary care referral center in India aims to document the contemporary clinical and hemodynamic profile of patients who were diagnosed with double chamber right ventricle (DCRV) based on echocardiography and cardiac catheterization. Patients were followed up and their outcomes were studied to document their short and long term outcomes.Methods: It is a retrospective observational study of patients diagnosed with DCRV in a tertiary care hospital in India. The diagnosis of DCRV was based on the following criteria: an echocardiographic diagnosis of DCRV with doppler evidence of a mid ventricular gradient; cardiac catheterization revealing a systolic pressure gradient between right ventricular inflow and outflow tracts; a right ventricular angiogram demonstrating an anomalous muscle bundle causing obstruction well below the infundibulum. All the patients were followed up for their long term outcomes.Results: All the patients underwent echocardiography and cardiac catheterization for confirmation of the diagnosis. Sixty percent of our patients presented during adulthood, which is very unusual presentation of this disease entity. Median age of our patient cohort was 23.5 years. Patients presenting during adulthood have atypical symptoms. Dyspnea was the most common presenting symptom in this study. Right ventricular hypertrophy (73.3%)and right bundle branch pattern (26.6%) were the common electrocardiographic findings in our patients. DCRV is commonly associated with other anomalies. Ventricular septal defect (VSD) was the commonest associated anomaly, which was seen in 80% of our patients. Mean gradient cross the anomalous muscle bundle was 67.5 mmHg. Three of our patients (20%) had no associated anomaly, which is very rare in DCRV. Eight patients underwent surgical correction with significant reduction in gradients in all and no perioperative mortality. Median follow up of 8 years showed no adverse outcomes and no progression of gradients.Conclusions: This study describes in detail the clinical profile, echocardiographic and angiographic identification of anomalous muscle bundles in DCRV patients, which will help the young readers in identifying this often missed diagnosis. It highlights the unusual presentation during adulthood with atypical symptoms in DCRV patients with excellent long-term outcomes on follow up.

A Rare Case of Double-Chambered Right Ventricle Associated with Ventricular Septal Defect and congenital Absence of the Pulmonary Valve

Double-chambered right ventricle (DCRV) is a rare congenital heart disorder involving 2 different right ventricle (RV) pressure compartments that is often associated with ventricular septal defect (VSD). Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.

Anomalous muscle bundle in the left ventricle causing subaortic obstruction

We report a 14-year-old boy in whom an anomalous muscle bundle in the left ventricle caused subaortic obstruction. It was diagnosed by echocardiography and resected at operation. The anomalous muscle most likely represents the hypertrophied anterolateral muscle bundle.