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Cor triatriatum sinistrum is a very rare condition constituting 0.1-0.4% of congenital heart disease. Patients most commonly present with breathlessness, and may be falsely diagnosed with asthma. Pulmonary hypertension may coexist due to left ventricular inflow obstruction at the level of the membrane.
A 2-year-old boy presented to his local hospital with a 1-week history of coryzal symptoms. A chest x ray demonstrated cardiomegaly, prompting cardiological referral. Admission examination confirmed 4 cm hepatomegaly, loud second heart sound and weight on the 0.4th centile. The resting respiratory rate was elevated at 40 breaths per minute.
Live three-dimensional echocardiography using a high frequency matrix probe (X7 probe, IE33 ultrasound system, Philips Inc., Andover, Massachusetts, USA) demonstrated the cortriatriatum permitting cropping and adjustment of imaging projection to facilitate full diagnosis (see figure). The pulmonary veins drained to the posterior aspect of the divided left atrium. There was no interatrial communication, and a restrictive opening in the cor triatriatum membrane leading to elevated pulmonary artery pressures (tricuspid regurgitant pressure drop 70 mm Hg) with right ventricular dilation and dysfunction.
Surgical repair was undertaken without other cardiac imaging modalities with an excellent postoperative result.
This article has been adapted from M Vogel, J M Simpson and D Anderson. Live three-dimensional echocardiography of cor triatriatum in a child Heart 2008;94:794
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