One of many causes of right heart failure (RHF) is partial anomalous pulmonary venous connection (PAPVC). We present a rare entity of isolated supra-cardiac PAPVC, as right pulmonary vein drains into the superior vena cava (SVC) with intact atrial septum, precipitating RHF.
A 55-year-old man with hypertension, diabetes mellitus, coronary artery disease, presenting with syncope. On examination blood pressure was 90/44 mm Hg and heart rate of 44 bpm, lungs were clear on auscultation, jugular venous distension was present, prominent S2 heart sound, and bilateral pitting edema of the lower extremities. Laboratory studies were significant for brain natriuretic peptide (BNP) of 504 pg/mL, troponin I of 0.06 ng/mL, and glycated hemoglobin (HgA1c) of 11.9%. Electrocardiography was significant for left atrial dilation and right ventricular hypertrophy. Transthoracic echocardiography (TTE) showed severe right ventricular dilation, left ventricular hypertrophy, and severe tricuspic regurgitation with pulmonary artery systolic pressure of 85 mm Hg. Additionally, on the TTE ejection fraction was noted to be 55% with no evidence of atrial septal defect (ASD). Cardiac catheterization and computed tomography angiogram (CTA) revealed severe pulmonary hypertension and drainage of the right pulmonary vein into the superior vena cava.
We have described a case of an isolated supra-cardiac variant of right pulmonary vein draining into the SVC. ASD is absent in isolated form of PAPVC, our case demonstrated an intact atrial septum in a supra-cardiac variant. Studies have indicated that 82% of patients with PAPVC have an ASD, distinguishing our case as an uncommon entity.
Patient was discharged with optimized doses of bumetanide, metoprolol, and was offered surgery for the definitive treatment. Surgical prognosis is excellent and the perioperative mortality rate is less than 0.1%⇓.
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