Double Outlet Right Ventricle with Pulmonary Valve Stenosis in a Young Girl: A Letter to the editor

Double outlet right ventricle (DORV) includesabnormal junctions between the ventricle andarteries, which both the aorta and pulmonary arteryoriginates from the right ventricle structurally. 1About 1 to 1.5 percent of patients with congenitalheart diseases may have this rare condition. It may beassociated with some other congenital anomalies.Some types which are more frequent variants includeDORV are: Pallot type, Taussig-Bing malformationaand Eisenmenger anomaly, and less frequents areassociated with: Non-committed Ventricular septaldefect (VSD) and ect. 2 It requires to be stated thatthe written consent form was taken from the patientbefore decision to write this article. Also, no conflictof interest exists.A 19-year-old girl referred to the cardiology clinic ofFatemeh-Zahra hospital Sari-Iran with chiefcomplaint of palpitation. At first a complete historywas taken. She had not any past history of dyspnea,weakness followed by activity, syncope and chestpain. On cardiovascular examination, distal pulseswere fully palpable. The first and second heartsounds, S1 and S2 were normal, but a systolic sufl inleft intercostal space and left boarder of the sternumwas sound. There were no evidence of clubbing andacrosianosis in physical examinations. Lungauscultation was normal too. Echocardiography wasperformed for evaluating of the cardiac chambers,which revealed extreme hypertrophy of rightventricle (RV), double chamber RV due to muscularband, severe stenosis of RVOT, sub pulmonic PS(PG pick-120 mmHg), no Atrial septal defect (ASD),Patent Foramen Ovale (PFO) and PartiallyAnomalous Pulmonary Venous Connection(PAPVC). Also a dumb shape pulmonary valve,severe interventricular septum (IVS) hypertrophywith paradoxical and abnormal movements due toextra load of right ventricle pressure was seen(Fig.1). A double chamber right ventricle that isdivided by a fibromuscular layer and also pulmonaryvalve stenosis were seen in ventriculography (Fig.2).Therefore, based on these results and for theconfirming of ventricular problem and pulmonaryvalve stenosis in this patent, we decided to perform aright anterior oblique (ROA) angiography, whichrevealed two proximal and distal chambers in theright ventricle (Fig.3).