A case of incomplete cor triatriatum sinister associated with large secundum atrial septal defect in an adult patient/Eriskin bir hastada büyük atriyal septal defekt ile iliskili inkomplet kor triatriyatum sinister olgusu

A 38-year-old-woman presented with dyspnea and palpitation. On physical examination the blood pressure was 120/70 mmHg and the heart rate was regular at 95 beats/min. Cardiac auscultation revealed normal S1, widely split S2 and grade 3/6 systolic murmur along the left sternal edge. Transthoracic echocardiography showed the fibromuscular membrane located above the mitral valve and secundum atrial septal defect (ASD) (Fig. 1, Video 1. See corresponding video/movie images at www.anakarder.com). A minimal pulmonary regurgitation and a moderate tricuspid regurgitation were found. The systolic pressure gradient over the tricuspid valve was 47 mm Hg. The ratio of pulmonary blood flow to systemic blood flow (Qp/Qs) was 2,6. Transesophageal echocardiography clearly showed cor triatriatum sinistra and a large ASD (25mm) (Fig. 2, 3; Video 2, 3. See corresponding video/movie images at www.anakarder.com). The Doppler echocardiography did not show significant gradient through fibromuscular membrane in left atrium. The patient underwent elective surgical treatment with excision of the membrane through a left atriotomy and closure of an ASD.

Cor triatriatum sinistrum is rare congenital cardiac malformation. It occurs in about 0.1-0.4% of all patients with congenital heart disease (CHD). Incomplete cor triatriatum sinister is extremely rare. The clinical symptoms depend on the size of the orifice, the morphology of the defect including the presence and location of an ASD, and on associated CHD. Incomplete cases have been described; in these patients the orifice was wide without a pressure drop between the proximal and distal chamber.

We reported a case of incomplete cor triatriatum sinister associated with large secundum ASD in an adult.