Surgical Management of Primary Adult Presentation of Severe Tricuspid Valve and Right Ventricular Hypoplasia in Patient Presenting With Massive Right Atrial Thrombus

Abstract

Purpose: We report the surgical management of a massive right atrial thrombus secondary to undiagnosed right ventricle and tricuspid valve hypoplasia in a 41-year-old female presenting with a right atrial mass and congestive hepatic failure. Methods: After presenting with increasing abdominal distension and massive abdominal ascites secondary to congestive hepatic failure, transthoracic echocardiogram demonstrated a large echogenic mass in the right atrium and severe tricuspid valve stenosis. Patient underwent surgical management after cardiac MRI and endovascular biopsy were inconclusive. Primary median sternotomy was performed with CPB introduced via ascending aortic cannulation, SVC cannulation, IVC cannulation via right femoral cut down and cooling to 30 degrees celsius. A right atriotomy was performed and right atrial thrombus was removed with evidence of severe right atrial hypertrophy and severe tricuspid stenosis appearing congenital in nature. The right ventricle was mild to moderately hypoplastic however the pulmonary valve was normal. Subsequent bidirectional Glenn procedure was achieved by anastomosing the SVC to the right pulmonary artery. Upon rewarming and weaning off CPB, SVC pressures were measured at 18 mmHg with excellent haemodynamics. Results: Patient was extubated the following day and had an uneventful recovery. She was discharged home on therapeutic enoxaparin after repeat abdominal ultrasound demonstrated improved hepatic ductal flow. Conclusions: The successful surgical management of this instance of adult congenital heart disease demonstrates that congenital heart disease should be considered in unusual presentations of heart failure even in adult populations with no prior history of congenital disease.