Asian Cardiovasc Thorac Ann 2006;14:e27-e29
© 2006 Asia Publishing EXchange Ltd
Total Anomalous Pulmonary Venous Return in an Adult
Motoyuki Hisagi, MD,
Kazuhiko Higuchi, MD,
Kenzi Koseni, MD,
Hirotaka Inaba, MD
Department of Cardiac Surgery Asahi General Hospital Chiba, Japan
For reprint information contact: Motoyuki Hisagi, MD Tel: 81 35 800 8654 Fax: 81 35 684 3989 Email: mhisagi-tky{at}umin.ac.jp, Department of Cardiothoracic Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
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ABSTRACT
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A 47-year-old man with total anomalous pulmonary venous return met all the conditions necessary for long survival. In particular, his pulmonary vascular resistance was almost normal because of right ventricular outflow tract obstruction. Surgical treatment was performed with a good result.
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INTRODUCTION
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Total anomalous pulmonary venous return (TAPVR) is a relatively uncommon congenital heart defect. The natural history indicates that only 50% of patients survive beyond 3 months, and only 20% survive the first year of life.1 Several factors are necessary for long survival, including a large atrial septal defect (ASD), absence of pulmonary venous obstruction, and low pulmonary arterial resistance.1–4 An unusual surgical case of TAPVR with right ventricular outflow tract obstruction (RVOTO) in an adult is described.
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CASE REPORT
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A heart murmur had been detected during childhood in our patient, and surgery had been recommended. He had no therapy or follow-up, and although he had been a sick child, his condition normalized in junior high school. He and his parents thought that the disease was cured. However, at the age of 47, he felt a little general fatigue, so he was referred to our hospital. Standard and three-dimensional computed tomography showed a common pulmonary venous chamber that had no connection with the left atrium. There was a large ASD (15 x 25 mm) and a vertical vein with no stenosis (Figure 1
). Right ventriculography indicated RVOTO (Figure 2). Pulmonary arteriography revealed stenosis of the pulmonary valve. Preoperative catheter data are listed in Table 1. The main pulmonary artery pressure was 33/11(20) mm Hg, which was almost normal, and the pressure gradient between the pulmonary artery and the right ventricular inflow was over 50 mm Hg. The pulmonary/systemic blood flow ratio was 1.39 and pulmonary vascular resistance was 2.0 Wood units·m–2 which was not excessive. Transthoracic and transesophageal echocardiography showed dilatation of the right heart, ASD, and RVOTO. The patient was diagnosed with Darling type Ia TAPVR, ASD, valvular pulmonary stenosis, and RVOTO. He underwent total correction with anastomosis of the common pulmonary venous chamber to the left atrium, ASD patch closure, right ventricular outflow tract reconstruction, and ligation of the vertical vein. Reconstruction of the right ventricular outflow tract involved pulmonary valve commissurotomy and resection of an abnormal muscle bundle. The postoperative course was uneventful; postoperative catheter data are given in Table 1. On the 10th postoperative day, the pressure gradient between the pulmonary artery and the right ventricle was approximately 40 mm Hg, and right ventriculography showed slight residual RVOTO. The patient was discharged on the 14th postoperative day. Six months later, there was no evidence of stenosis (Figure 1, Table 1).
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Figure 1. Three-dimensional computed tomography (A) anteroposterior and (B) posteroanterior, showing a common pulmonary venous chamber and vertical vein, with no stenosis.
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Figure 2. Right ventriculography showing right ventricular outflow tract obstruction preoperatively (A), but no ventricular outflow tract obstruction on the postoperative ventriculography (B).
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DISCUSSION
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Total anomalous pulmonary venous return is a relatively uncommon anomaly, accounting for only 1.5% to 3% of cases of congenital heart disease. As a result of the low survival rate in untreated cases, it is unusual to encounter a patient with TAPVR who has remained without symptoms until adulthood.1 This patient fulfilled all the conditions for long survival: a large ASD, no obstruction of pulmonary venous return, and low pulmonary vascular resistance.1–4 However, in many cases of TAPVR in an adult, even without pulmonary venous obstruction, the pulmonary vascular bed is damaged due to the large shunt flow over a long period of time.1 In this case, there was no pulmonary hypertension and the pulmonary vascular resistance was almost normal, which was due to stenosis of the pulmonary valve and RVOTO. As the pulmonary valve had sclerotic changes pathologically, the stenosis may have been acquired. The abnormal muscle bundle resected at the operation was found to be myocardial tissue with lipofuscin. It is not clear whether the RVOTO was congenital or acquired. However, these features protected the pulmonary vascular bed, so the patient survived for 47 years.
There are many case reports of TAPVR in adults, but few in which it was associated with pulmonary stenosis (Table 2). The pulmonary stenosis in these reports was valvular or relative.5,6 To the best of our knowledge, there has been no other report of TAPVR associated with RVOTO in an adult. We suspect there is a tendency for patients with pulmonary stenosis or RVOTO to recover early and uneventfully (similar to a patient with ASD), in contrast to those with pulmonary hypertension.
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REFERENCES
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ABSTRACT
INTRODUCTION
