Cor triatriatum sinistrum and persistent left superior vena cava: an original association

Cor triatriatum sinistrum is a rare congenital heart disease usually diagnosed in symptomatic children. Symptoms depend on the degree of obstruction to pulmonary venous return with pulmonary hypertension and other associated abnormalities. Persistent left superior vena cava is quite a common congenital heart disease (about 0.5% in healthy populations). It should be suspected every time a dilated coronary sinus is detected at the echo examination. Transthoracic and transoesophageal examinations visualize the site and the size of the fibrous membrane as well as the degree of obstruction, and allow the evaluation of pulmonary pressures that are very important clues for prognosis and therapy. This case report describes the clinical signs and the diagnostic ultrasound findings evaluated in comparison with magnetic resonance imaging, a well-defined gold standard in heart disease of this uncommon congenital association.