Severe Ventricular Outflow Tract Obstruction Research Articles

Right atrium giant blood cyst in a young female with acute pericarditis, complicated with fatal cardiac tamponade

Blood cysts (BC) of the heart are exceedingly rare in adults. The BC are benign cardiovascular tumors which are congenital in origin and are incidental autopsy findings in approximately 50% of infants under 2months of age. They usually regress spontaneously and are very rare after 2years of age [1,2]. Their usual size ranges from microscopic to 3mm in diameter. They are typically asymptomatic [1]. When found in adults, they can be large and may result in severe ventricular outflow tract obstruction [3].

310 Aorto-pulmonary anastomosis in tuberous sclerosis and cardiac tumor with severe right ventricular outflow tract obstruction (a case report)

Primary cardiac tumors are rare with an estimated incidence of 0.27% in pediatric autopsies. The most common type of cardiac tumor identified in infancy and childhood is rhabdomyoma. We report a case of prenatal diagnosis of multiples cardiac rhabdomyoma at 29 weeks of gestation. Full-term delivery was straightforward without hydrops and dysrhythmia. In the immediate post-natal period, cyanosis appeared, and echocardiography showed multiple cardiac rhabdomyoma and severe right ventricular outflow tract obstruction (Image 1). Aprostadil perfusion was necessary. Aorto-pulmonary anastomisis was performed with success. The diagnosis of cardiac rhabdomyoma was confirmed histologically and tuberous sclerosis by molecular genetic analysis. Renal echocardiography was normal and cerebral MRI was not perfomed. Fourteen months later, neurodevelopment was normal. Echocardiography confirmed regression of the cardiac tumors with disappearance of the severe right ventricular outflow tract obstruction. ECG monitoring was normal. The natural history of most cardiac rhabdomyoma is favorable with tumors regressing (completely or partially). Surgical resection of the tumors is required for severe ventricular outflow tract obstruction. Aorto-pulmonary anastomosis, as in our case with severe right ventricular outflow tract obstruction, is an alternative treatment because regression and even complete resolution of more than 80% of the tumors occurs during infancy and early childhood.