Surgical Correction of Total Anomalous Pulmonary Venous Return in an Adult Patient

doi:10.21203/rs.3.rs-1732884/v2

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Case Report

Surgical Correction of Total Anomalous Pulmonary Venous Return in an Adult Patient

https://doi.org/10.21203/rs.3.rs-1732884/v2

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Background: Total anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. Most untreated patients with large atrial septal defects and no pulmonary venous obstruction have pulmonary vascular damage from pulmonary over circulation.

Case presentation: 44-year-old TAPVR patient admitted to our medical center. A snowman-shaped heart, including cardiomegaly and an increase in pulmonary blood flow, was seen in the chest X-ray. A large-sized (around 3 cm) atrial septal defect (ASD) with dilated right atrium, right ventricle, and pulmonary artery was detected on echocardiography. Heart computed tomography was performed for further evaluation, and supra-cardiac type TAPVR without any obstructive lesion was identified.

Conclusions: TAPVR in an adult patient is extremely rare, and this patient was treated successfully with surgical correction and is doing well. A sinus rhythm and mild mitral valve regurgitation have remained during 2.5 years of outpatient follow-up.

TAPVR

Adult congenital heart surgery

vascular anomalies

complex congenital heart disease

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).

TAPVR is a rare anomaly accounting for only 1.5–2.2% of congenital heart disease [1]. Almost all patients with TAPVR present with symptoms that include cyanosis and heart failure in the neonatal period, which require early surgical correction. A large ASD and no obstruction of the pulmonary venous drainage pathway are the two major factors affecting survival. However, most untreated patients with large ASDs and no pulmonary venous obstruction have pulmonary over-circulation, leading to pulmonary vascular damage [2]. Thus, it is unusual to encounter adult patients without symptoms.

Fortunately, this patient had a relatively good LV size with a large ASD, no pulmonary venous pathway obstruction, and low pulmonary arterial pressure despite having no pulmonary stenosis. Thus, this patient had a good recovery. Severe mitral valve regurgitation was seen in this patient upon admission. However, the degree of mitral valve regurgitation improved after rhythm conversion. The mitral valve morphology in the operative field showed diffuse thickening with a good coaptation margin. There was no evidence of rheumatic history. Further follow-up is required.

There are few adult TAPVR patients worldwide. The cases reported previously showed a favorable prognosis in the postoperative phase. However, approximately 10–15% of the patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive [3]. In the three years of follow-up after surgery, this patient did not show any complications, including pulmonary venous obstruction or mitral valve regurgitation. However, long-term surveillance and monitoring are required.

NT-proBNP: NT- amino-terminal pro-brain natriuretic peptide; ASD: Atrial septal defect; LV: Left ventricle; TAPVR: Total anomalous pulmonary vein return; CPB: Cardiopulmonary bypass; POD: Postoperative day.

Acknowledgements: Not applicable.

Funding: No.

Availability of data and materials: Not applicable.

Ethics approval and consent to participate: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Competing interests: The authors declare that they have no competing interest

Author contributions: Woosung Jang was the operator of this case and the corresponding author of this article. Yohan Bae was a major contributor in writing the manuscript. Kyungsub Song made critical revisions to the manuscript and collect data. All authors read and approved the final manuscript.

Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002; doi:10.1016/s0735-1097(02)01886-7
Hisagi M, Higuchi K, Koseni K, Inaba H. Total anomalous pulmonary venous return in an adult. Asian Cardiovasc Thorac Ann. 2006; doi:10.1177/021849230601400228
Ashfaq A, Shah N, Khan MZ, Atiq M, Amanullah MM. Successful surgical intervention of total anomalous pulmonary venous drainage in the third decade of life. Ann Med Surg. 2013; doi:10.1016/s2049-0801(13)70039-6

No competing interests reported.

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Editorial decision: Major revision
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Surgical Correction of Total Anomalous Pulmonary Venous Return in an Adult Patient

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