Abstract
Introduction
Double outlet right ventricle is a rare cause of acute cerebrovascular disease among young adults.
Case. We report a case of a 21 year old male who presented with aphasia and left sided weakness. This was associated with cyanosis, easy fatigablity, recurrent epistaxis and loss of consciousness since childhood. On admission, patient’s vital sign was stable at 100/60, 90 bpm regular, 20 cycles per minute afebrile 80% oxygen saturation on room air. Patient is conscious, aphasic but follows simple commands. He had central cyanosis, clubbing of the upper and lower extremities, adynamic precordium, single s2, right ventricular heave, grade 3/6 systolic ejection murmur at the 2nd left intercoastal space parasternal line, 4/6 holosystolic murmur at the 4th intercoastal space parasternal line, and clear breath sounds. Neurologic examination revealed a 0/5 motor response on both left upper and lower extremity. Cranial MRI showed acute ischemic infarcts at the right frontal centrum semiovale and left cerebral hemisphere. During his admission, cardiac work up was done to investigate cardioembolic cause of the acute cerebrovascular disease. Transesophageal echocardiogram revealed congenital heart disease situs solitus with atrioventricular discordance; double outlet right ventricle, pulmonic valve stenosis, interatrial septal aneurysm with atrial septal defect and persistent left superior vena cava syndrome. During his hospital course, patient was started on aspirin, citicholine and atorvastatin for his cerebrovascular disease which was noted to improve after 2 days. Patient was also referred to the thoracovascular surgery team for comanagement who did modified Blalock Tausig shunting. Patient was referred to cardiac rehabilitation post procedure. His vital signs remained to be stable, his oxygen saturation increased to 90% at room air, and his functional capacity improved. Patient was discharged stable.
Discussion
Double outlet right ventricle is a rare complex congenital heart disease characterized by a conotruncal malseptation from which more than or equal to 50% of each great artery arises from the morphologic right ventricle. Associated defects like atrial septal defect and persistent left superior vena cava were reported in some literature. Cerebrovascular infarcts in complex cyanotic congenital heart disease might be secondary to polycythemia and/or paradoxical emboli bypassing the interatrial defect. Prompt prophylaxis such as periodic phlebotomy and/or anticoagulation in patients high risk for deep vein thrombosis must be evaluated for each patient.
High prevalence rate of left superior vena cava determined by echocardiography in patients with congenital heart disease in Saudi Arabia
