A 44-year-old man was admitted for acute onset palpitations and dyspnea. On physical examination, clubbed fingers were observed. The patient’s oxygen desaturation was 89% in room air, and atrial tachycardia was seen on electrocardiography. Blood chemistry evaluation showed elevated liver enzymes (aminotransferase/alanine aminotransferase: 2060/1649 U/L, total bilirubin/ direct bilirubin 4.41/0.84 mg/dL) and amino-terminal pro-brain natriuretic peptide (NT-proBNP) levels up to 6789 pg/mL. A snowman-shaped heart, including cardiomegaly and an increase in pulmonary blood flow, was seen in the chest X-ray (Fig. 1). Cardiomegaly on chest X-ray was detected 23 years ago and noted in his past medical history. However, there was no follow-up.
A large-sized (around 3 cm) atrial septal defect (ASD) with dilated right atrium, right ventricle, and pulmonary artery was detected on echocardiography. In addition, a D-shaped left ventricle (LV) and severe mitral valve regurgitation were also detected. Sinus rhythm recovered after digitalization and diuretic administration. The mitral valve regurgitation was improved to a mild degree on echocardiography, and the liver enzymes were also normalized. Heart computed tomography was performed for further evaluation, and supra-cardiac type total anomalous pulmonary vein return (TAPVR) without any obstructive lesion was identified. The pulmonary venous drainage course, including the superior vena cava was severely dilated. However, the left atrium and left ventricle sizes were relatively good (Fig. 1). The pulmonary arterial pressure was 43/22 mmHg (Mean: 29 mmHg) on right heart catheterization (Table 1).
Table 1
Preoperative cardiac catheterization data
Site | O2 saturation (%) | Pressure systolic/diastolic pressure (mean), mmHg |
Superior vena cava | 90.7 | |
Right atrium | 85.4 | 12/4 (7) |
Right ventricle | 88.7 | 43/8 (20) |
Pulmonary artery | 88.7 | 43/22 (29) |
Left atrium | 89.6 | 12/5 (7) |
Aorta | 90 | 100/60 (73) |
We performed a confluent vein left atrium direct anastomosis and ASD patch closure by the transverse sinus approach without total circulatory arrest. The vertical vein was ligated. The cardiopulmonary bypass (CPB) time and aorta cross-clamp time were 147 and 61 minutes, respectively. We also checked the mitral valve morphology. The mitral valve showed diffuse leaflet thickening at the anterior and posterior valves. However, the coaptation margin of the valve leaflet was good. Thus, we did not perform mitral valve surgery. CPB weaning was smooth. After CPB weaning, there was no problem with the pulmonary venous drainage course, and the mitral valve regurgitation was mild. The patient was extubated on postoperative day (POD) 0 and transferred to the general ward on POD 1. He was discharged on POD 8. The patient is doing well, and sinus rhythm and mild mitral valve regurgitation have remained during 2.5 years of outpatient follow-up (Fig. 2).