International Journal of Cardiology, 31 (1991) 91-99 0 1991 Elsevier Science Publishers B.V. 0167-5273/91/$03.50 ADONIS 016752739100106N 97 CARD10 01229 zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA Brief Reports Coexistence of divided left atrium (car triatriatum) and tetralogy of Fallot Maria AngClica Binotto, Vera Demarchi Aiello and Munir Ebaid Division of Pediatric Cardiology, Heart Institute, Sao Paula, Brazil (Received 2 April 1990; revision accepted 23 October 1990) zyxwvutsrqponmlkjihgfedcbaZYXWV The association of a partitioned left atrium (COT tiatriatum) and tetralogy of Fallot has been reported a few times in the literature. In all of these cases, there was no clinical evidence of an obstructive lesion in the left heart, raising difficulties in establishing the proper diagnosis. We call attention to this rare combination of anomalies and to the particular morphological presentation of the dividing atrial shelf, which was partially formed by the wall of the left superior caval vein. Key words: Partitioned left atrium; Cor triatriatum; Tetralogy of Fallot Introduction Partitioning of the atrial chambers (car triatriatum) is an uncommon malformation. Even rarer is its associa- tion with tetralogy of Fallot. Most of the cases previ- ously described have been diagnosed at autopsy [l-4]. Since preoperative demonstration of a division within the left atrium is crucial for a successful repair of tetralogy of Fallot, this report is aimed at calling atten- tion to this rare combination of anomalies. Case Report A 17-year-old young woman was admitted to the Heart ,Institute for repair of a cyanotic heart defect. In spite of early detection of a cardiac anomaly and re- peated hypercyanotic attacks, a proposal of surgical treatment was refused when she was three years old. Physical examination revealed moderate underdevel- opment, marked generalized cyanosis, clubbing of fingers and toes, and dyspnoea. The cardiac impulse was in the right precordium. The first and the second Correspondence to: M.A. Binotto, M.D, Instituto do Cora@o do Hospital das Clinicas da FMUSP, Avenida Dr. Endas de Carvalho Aguiar 44, CEP 05403~S%o Paulo, SP-Brazil. sounds were increased. A grade 3/6 ejection systolic murmur was heard maximally at the third intercostal space just to the right of the sternum. On chest X-ray, the pulmonary vascular markings were reduced. A right-sided heart was present, with mild cardiomegaly. Electra and vectorcardiography showed a + 120 degrees frontal QRS axis with a pattern of marked right ventric- ular hypertrophy. Echocardiographic and angiographic findings were compatible with tetralogy of Fallot with small bilar pulmonary arteries. An additional apical ventricular septal defect was also detected. The patient underwent emergency surgical treatment subsequent to cardiac catheterisation because of a severe hypercyanotic attack unresponsive to routine medical management. Because of the small caliber of the hilar pulmonary arteries, the procedure included only in- fundibulectomy with patch-widening of the sub- pulmonary outflow tract. A severe pulmonary conges- tion just within the first 24 hours after surgery was partially controlled with vasoactive agents. Banding of the pulmonary trunk was performed on the third day with relief of symptoms. The patient died on the sixth day after a generalized seizure, in the absence of any haemodynamic abnormalities. At autopsy, the heart showed usual atria1 arrange-