Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

HAN Yuehu , WEI Dongming , PENG Langang , DUAN Le , YIN Qiang , SUN Guocheng , ZHU Hailong ,  GU Chunhu

Department of Cardiac Surgery, The First Affiliated Hospital of Air Force Medical University, Xi’an, 710032, P.R.China;

Corresponding author：

GU Chunhu, Email: guchunhu@163.com

Keywords：

Aortopulmonary window; congenital heart disease; pulmonary hypertension; surgical treatment

DOI：

10.7507/1007-4848.202002103

Video：

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Objective To study the diagnosis and treatment of aortopulmonary window (APW) associated with severe pulmonary hypertension.Methods The clinical data of 23 patients with APW undergoing surgical treatment in The First Affiliated Hospital of Air Force Medical University from 2010 to 2018 were retrospectively reviewed. There were 9 male and 14 female patients. The age was 3-132 (4.63±2.14) months. The weight was 3.3-35.0 (17.3±3.6) kg.Results Windows were situated in the proximal of semilunar valve (type Ⅰ) in 8 patients, and distal of the aorta (type Ⅱ) in 14 patients, from proximal to distal (type Ⅲ) in only 1 patient. Eleven patients were isolated APW, the others were combined with cardiac defects. The mean pulmonary artery pressure was 68.4±7.5 mm Hg. All patients underwent surgical correction under general anesthesia and hypothermia cardiopulmonary bypass. All patients were discharged uneventfully, with an average follow-up time of 4 years. The patients showed good outcomes and no residual shunt after surgery, and the pulmonary artery pressure decreased to normal.Conclusion APW is an uncommon congenital cardiac anomaly. The clinical presentation is an excessive left-to-right shunt, and most patients present early in life. Development of pulmonary hypertension and pulmonary vascular resistance is usually rapid. Operative treatment is indicated as soon as the diagnosis is established, regardless of the patient’s age, and most patients after surgery have a good long-term outcome.

Citation： HAN Yuehu, WEI Dongming, PENG Langang, DUAN Le, YIN Qiang, SUN Guocheng, ZHU Hailong, GU Chunhu. Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2021, 28(1): 70-74. doi: 10.7507/1007-4848.202002103 Copy

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1. Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorti-copulmonary septal defect. Am J Cardiol, 1987, 59(5): 443-447.
2. Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40‐year experience. Eur J Cardiothorac Surg, 2002, 21(5): 773-779.
3. Castaneda AR, Jonas RA, Mayer JE Jr, et al, chief editor. Cardiac Surgery of the Neonate and Infant. Philadelphia, PA: W.B. Saunders Company, 1994.
4. Gowda D, Gajjar T, Rao JN, et al. Surgical management of aortopulmonary window: 24 years of experience and lessons learned. Interact Cardiovasc Thorac Surg, 2017, 25(2): 302-309.
5. Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulumonary window: diagnosis, surgical technique and long‐term results. Eur J Cardiothorac Surg, 1997, 11(2): 293-297.
6. Jacobs JP, Quintessenza JA, Gaynor JW, et al. Congenital heart surgery nomenclature and database project: ventricular septal defect. Ann Thorac Surg, 2000, 69(3): 25-35.
7. Brooks MM, Heymann MA, Chief editor. Aortopulmonary window. In: Emmanouilides GC, Riemenschneider TA, Allen HD, et al. Moss and Adams’ Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult (5th ed). Baltimore: Willians & Wilkins, 1994.764-768.
8. Ding WX, Su ZK, Cao DF, et al. One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch. Ann Thorac Surg, 1990, 49(4): 664-666.
9. Soares AM, Atik E, Cort^ez TM, et al. Aortopulmonary window. Clinical and surgical assessment of 18 cases. Arq Bras Cardiol, 1999, 73: 59-74.
10. Patra S, Agrawal N, Mahimarangaiah J, et al. Type Ⅰ aortopulmonary window presenting with very early onset Eisenmenger's syndrome. BMJ Case Rep, 2014, 2014: bcr2013201492.
11. Aizaz K, Al Qethamy H, Al Jubair K, et al. Aortopulmonary window repair: 15-year surgical experience. Asian Cardiovasc Thorac Ann, 2001, 9: 111-114.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Diagnosis and treatment of aortopulmonary window associated with severe pulmonary hypertension

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