Case report
A 22 year-old-man was admitted to our institute because of exertional dyspnea, which occurred after recruited the military service. On his examination the clubbing and cyanosis in the tongue and lips were observed. Mesocardiac 2-3/6 grade systolic murmur was heard throughout the systole. ECG revealed left axis deviation, LVH by voltage, and tall T waves. Transthoracic echocardiography demonstrated a membranous type VSD, and LVEF of 50%. A cardiac computed tomography (CT), which performed to assess whether VSD is appropriate for percutaneous closure, revealed a perimembranous inlet type VSD (7 mm), a large sinus venosus ASD (27 mm), small right ventricle cavity (23mm), and pulmonary trunks diameter of 20 mm. In addition to the findings detected on cardiac CT, cardiac MRG also revealed the appearance of the dysplastic tricuspid valve, and 13 mm apical displacement of tricuspid septal leaflet (Ebstein’s anomaly). Although right ventricle and pulmonary vascular bed preserved, clubbing and central cyanosis was present. Therefore, we performed a right cardiac catheterization. CO (fick): 1.91 L/min/m2, Qp/Qs: 1.24, PVR: 1.27 Wood Units, SVR:45 Wood Units, Transpulmonary gradient: 3 mmHg. LV pressure was substantially higher than RV. Oximetry run was performed and interestingly demonstrated that while PO2 in the pulmonary vein was 96.6%, PO2 in the left atrium was 59%. Afterwards, we injected opaque into the superior vena cava, and observed that blood flow passed directly to the left atrium. Interestingly, we observed mild methemoglobinemia (3.5%) during oximetry run. However, we did not consider that this level of MetHb would be responsible for cyanosis. Although cardiac CT and MRI did not any membrane or similar structure, a transesophageal echocardiography demonstrated a membrane in the right atrium, which directed the blood flow coming from the SVC into the left atrium, suggesting cor triatriatum dextrum. The patient was referred to cardiac surgery.
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