Korean Circ J.  2017 Jan;47(1):136-140. 10.4070/kcj.2016.0123.

Prophylactic Pulmonary Artery Reduction in a Young Female with Severe Pulmonary Hypertension from Complete Atrioventricular Septal Defect

• Lee SH
• Kwon BS
• Kim GB
• Bae EJ
• Noh CI
• Kim WH

• KMID: 2365293
• DOI: http://doi.org/10.4070/kcj.2016.0123

Abstract

Management of severely dilated pulmonary artery (PA) associated with severe pulmonary hypertension from congenital heart disease remains controversial, primarily due to its rare nature and concern for perioperative unpredictable complications. Herein, we report a 25 year-old female with a severely dilated PA (up to 73 mm), who was successfully treated by a PA graft replacement by creating a Y-shaped conduit using a 28 mm hemashield tube in the main PA and a 20 mm hemashield tube in both proximal parts of the branch PA.

Keyword

Pulmonary arterial hypertension; Congenital heart defects; Pulmonary surgical procedure

MeSH Terms

Female*
Heart Defects, Congenital
Humans
Hypertension, Pulmonary*
Pulmonary Artery*
Pulmonary Surgical Procedures
Transplants

Figure

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  Fig. 1 Chest radiography. (A) Preoperative radiograph shows cardiomegaly and dilated main pulmonary trunk and its branches in central and peripheral localizations. (B) Postoperative radiograph (18 months after the operation) shows a reduction in the size of the main pulmonary trunk and arteries.

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  Fig. 2 Cardiac CT with 3D reconstruction. (A, B) Preoperative image shows severely dilated main pulmonary artery (measuring 73 mm) and its branches at the level of aorticopulmonary window. (C, D) Postoperative image (19 months after the operation) revealed a decreased size of neo-main pulmonary artery (measuring 28 mm) and proximal branch pulmonary arteries with remained aneurysm of lobar pulmonary arteries. CT: computed tomography.

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  Fig. 3 Operative findings and procedures. (A) Postoperative diagnosis was a left isomerism with inferior vena cava interruption, dextrocardia, single atrium, complete atrioventricular septal defect with another muscular ventricular septal defect, bilateral superior vena cava, and pulmonary artery aneurysm with severe pulmonary hypertension. (B) Y-shaped graft replacement by a creation of 28 mm main pulmonary artery trunk using 20 mm hemashield. (C) Mitral valve cleft closure and atrial patch partitioning with 8 mm fenestration. (D) Right-sided superior vena cava division and anastomosis to right atrium auricle with 16 mm polytetrafluoroethylene graft interposition. MV: mitral valve, CS: coronary sinus.

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  Fig. 4 Pulmonary artery specimen. Resected pulmonary artery revealed huge pulmonary aneurysm with myxoid degeneration of the vascular wall.

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