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Cor Triatriatum: Clinical Presentation of 18 Cases

Department of Pediatric Cardiothoracic Surgery 1 Department of Pediatric Cardiology Service 2 Department of Pediatric Medicine Kandang Kerbau Women's and Children's Hospital Singapore, Republic of Singapore 3 Department of Cardiothoracic Surgery Red Cross Memorial Children's Hospital Cape Town, South Africa 4 Department of Pediatric Cardiovascular Surgery WOK Hospital Zabrze, Poland
For reprint information contact: Ryszard W Lupinski, MD, PhD Tel: 65 394 1132 Fax: 65 291 0161 email: richardl{at}kkh.com.sg Department of Pediatric Cardiothoracic Surgery, Kandang Kerbau Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Republic of Singapore.

From 1985 to 1999, 18 patients with cor triatriatum underwent surgical correction. Their ages ranged from 3 months to 9 years (mean, 32.5 months). Eight patients were cyanosed; the other 10 presented with congestive heart failure. Recurrent chest infection was observed in 10 cases, and failure to thrive in 7. One patient had isolated cor triatriatum, and 17 had an associated atrial septal defect. Other associated anomalies included patent ductus arteriosus, ventricular septal defect, and partial and total anomalous pulmonary venous connection. Two deaths occurred perioperatively in patients with associated severe heart defects. Follow-up ranged from 1 month to 10 years. No late events occurred among the survivors, and all were in New York Heart Association functional class I. Their most recent echocardiograms showed no residual obstruction or shunt and good development of the left cardiac chambers. Echocardiography is recommended as the diagnostic modality of choice. Cor triatriatum can be corrected surgically with low mortality and good long-term results.