Cor triatriatum sinistrum in childhood. A single institution's experience.

Introduction: Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients.

Objective: To analyze the experience with CTS at a single pediatric centre, highlighting symptoms on presentation, mode of diagnosis and outcome.

Methods: Hospital databases were searched to identify patients with CTS who were diagnosed between 1954 and 2005. Medical records with demographic data, clinical evaluation, diagnostic approach, interventions and autopsy results were reviewed.

Results: Between 1954 and 2005, 82 patients (43 female children, 52%) with CTS were diagnosed at the institution. Patients were born between 1951 and 2004, and the median age at presentation was eight months (range one day to 16.1 years). The majority of patients (77%) presented with associated cardiac lesions. Of 82 patients, 57 (70%) underwent resection of the fibromuscular diaphragm, 14 (17%) did not require surgery and 11 (13%) did not survive the time to intervention. Nineteen patients (23%) died a median of two months (range one day to 5.5 years) after presentation. Nine of these patients (11%) died before surgery was attempted. Most patients were completely asymptomatic at the time of the last follow-up at a median of three years (range one day to 18 years). Echocardiography significantly facilitated the diagnosis and subsequent management of patients with CTS.

Conclusions: Despite the diversity in presentation and complexity of associated lesions, the outcome for patients with CTS is favourable.