Key result
Surgical repair of cor triatriatum with pulmonary hypertension drops sPAP ~70 mmHg, improving exercise tolerance.
Case Report (n=1)
No
Surgical correction is a viable and successful treatment for adults with cor triatriatum complicated by pulmonary thrombotic hypertension and atrial septal defect.
May support surgery in rare adult cor triatriatum with pulmonary thrombotic hypertension; leaves open confirmation in larger series.
We herein present a case of a successful correction of cor triatriatum associated with thrombotic pulmonary hypertension diagnosed in an adult female patient. We confirmed diagnosis using transthoracic and transesophageal echocardiography in addition to cardiac computed tomography and magnetic resonance imaging. Surgical repair comprised excision of the fibromuscular membranous septum in the left atrium, patch closure of an atrial septal defect, and reconstruction of the pulmonary arteries with a vascular graft. Cor triatriatum complicated pulmonary thrombotic hypertension with atrial septal defect is amenable to surgical correction with satisfactory results.
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Park et al. (2011) conducted a case report in Cor triatriatum with pulmonary artery thrombosis and severe pulmonary hypertension (n=1). Surgical correction (septum excision, ASD closure, and pulmonary artery reconstruction) was evaluated on Reduction in pulmonary arterial pressure and clinical improvement. Surgical correction of cor triatriatum complicated by pulmonary thrombotic hypertension successfully reduced systolic pulmonary arterial pressure from 122 mmHg to 50-55 mmHg and significantly improved exercise tolerance.
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