Surgical experience with cor triatriatum repair beyond infancy.

ABSTRACT

BACKGROUND: Cor triatriatum is an exceedingly rare congenital heart defect which can present at any age depending upon the presence of associated cardiac anomalies and the size of communication between the common chamber and the left atrium. Our aim is to present 10 years surgical outcome of cor triatriatum repair in patients who present beyond infancy. METHODS: On going through our institute's surgical database from January 2009 to June 2019 for cor triatriatum, 14 patients were found to have undergone the surgical correction. Their demographic profile and midterm outcome in terms of mortality, functional status, and cardiac function was assessed. RESULTS: There were total 14 patients with slight female predominance (57.14%) who underwent cor triatriatum surgical repair. The mean age at the time of surgery was 12.14 ± 9.97 years ranging from 1 to 29 years. Associated congenital cardiac defects were seen in 12 (85.71%) patients with atrial septal defect being the most common. Partial anomalous pulmonary venous connection was seen in 4 (28.57%) patients. Eight (57.14%) patients had severe pulmonary hypertension preoperatively. All but two patients showed marked reduction in pulmonary artery pressures postoperatively. All the patients underwent excision of the membrane and repair of associated cardiac defects simultaneously. There was no early or midterm mortality or any reintervention over a mean follow-up period of 58.06 ± 30.73 months (range 20-120 months). CONCLUSION: The early and midterm surgical outcome of cor triatriatum is excellent even in patients who present beyond infancy with reversible pulmonary artery hypertension.