Aortopulmonary Septal Defect

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Aortopulmonary septal defect (APSD), an uncommon congenital cardiac defect, is a deficiency in the septum between the aorta and pulmonary artery, resulting in a communication between the two. This defect is present as an isolated lesion in about one half of patients and in conjunction with another defect or more complex heart disease in the other half of patients.

Developmentally, the defect results from incomplete separation of the common tube of the truncus arteriosus and the aorticopulmonary trunk. [1] During early embryonic development, the aorta and pulmonary arteries separate by growth of a spiral septum dividing the common trunk into the aorta and the pulmonary artery. The spiral septum is created by fusion of a truncal septum growing cephalad from the semilunar valves and the aorticopulmonary spiral septum growing caudally from the pulmonary bifurcation. Incomplete development of these septa results in aortopulmonary septal defect.

van Mierop subdivided aortopulmonary septal defect into 3 subtypes. [2] The first subtype is believed to result from nonfusion between the aorticopulmonary septum above and the truncal septum below, resulting in a small-to-moderate defect midway between the semilunar valves and the pulmonary bifurcation. The second type is also believed to arise from a failure of fusion of the aorticopulmonary septum above and the truncal septum below; however, this failure of fusion results in a large, nonrestrictive defect without a continuous posterior border, in which the defect describes more than one spiral turn. The third type is absence of the aorticopulmonary septum; the defect is large and without a posterior border, and the right pulmonary artery may arise directly from the aorta. Although this classification system may correlate with the various embryologic origins of aortopulmonary septal defect itself, it does not account for other anomalies encountered with aortopulmonary septal defect.

Patent ductus arteriosus (PDA) is encountered in almost three fourths of patients with aortopulmonary septal defect. [3, 4] An interrupted aortic arch type A or severe coarctation is present in 10-15% of patients with aortopulmonary septal defect. [5] Discontinuity of the aorta in interrupted aortic arch type A occurs distal to the left subclavian artery, as in a severe form of aortic coarctation. This is quite different developmentally from interrupted aortic arch type B, in which discontinuity occurs between the left carotid artery and left subclavian arteries. Interrupted aortic arch type B is frequently associated with DiGeorge/velocardiofacial/22q-chromosome arm deletion, unlike interrupted aortic arch type A. When interrupted aortic arch occurs without a ventricular septal defect (VSD), an aortopulmonary septal defect is usually present. [6]

Tetralogy of Fallot and anomalous coronary from pulmonary artery are each present in about 5% of cases. [7, 8] Other reported anomalies associated with aortopulmonary septal defect include VSD, aortic atresia, transposition of the great arteries, [9, 10] double aortic arch, and other more complex heart diseases.

Aortopulmonary septal defect has been described in other mammals including dogs, cats, and horses. [11]

The fetus is unaffected by this defect. Problems arise after birth with the fall in pulmonary vascular resistance (PVR) that typically takes place over the first days and weeks of life. As PVR falls, progressive shunting of blood from the systemic circuit to the pulmonary circuit results in pulmonary edema and signs and symptoms of congestive heart failure (CHF) similar to those seen with a large VSD or PDA. Left untreated, irreversible pulmonary vascular obstructive disease (PVOD) is likely to develop. In some cases, PVR does not fall significantly after birth and the phase of CHF is not apparent. In these instances, PVOD is a consequence nonetheless.

United States

Aortopulmonary septal defect is a rare defect that comprises about 0.1-0.3% of congenital heart diseases in children. No attempt to assess regional or worldwide variation in incidence has been made.

International

A large case series from India reported an overall frequency of surgery for aortopulmonary septal defect of 0.6% of all surgeries performed for congenital heart disease. [12]

Left untreated, an aortopulmonary window results in irreversible pulmonary vascular changes and early mortality. With surgical treatment in the absence of PVOD, the prognosis for isolated aortopulmonary window is good. In the presence of more complex heart disease, prognosis depends more on the nature of other lesions.

No racial predilection is observed.

The male-to-female ratio is approximately 1.8:1.

As a congenital disease, all cases are present from birth. The diagnosis is typically made in infancy but may be delayed if persistently elevated PVR occurs. Because of improved fetal ultrasonography, prenatal diagnosis of aortopulmonary septal defect has also been reported. [13, 14]

Bohuta L, Hussein A, Fricke TA, d’Udekem Y, Bennett M, Brizard C, et al. Surgical repair of truncus arteriosus associated with interrupted aortic arch: long-term outcomes. Ann Thorac Surg. 2011 May. 91(5):1473-7. [Medline].

Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol. 1987 Feb 15. 59(5):443-7. [Medline].

Mert M, Paker T, Akcevin A, et al. Diagnosis, management, and results of treatment for aortopulmonary window. Cardiol Young. 2004 Oct. 14(5):506-11. [Medline].

Dipchand AI, Giuffre M, Freedom RM. Tetralogy of Fallot with non-confluent pulmonary arteries and aortopulmonary septal defect. Cardiol Young. 1999 Jan. 9(1):75-7. [Medline].

Erez E, Dagan O, Georghiou GP, et al. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg. 2004 Feb. 77(2):484-7. [Medline].

Murin P, Sinzobahamvya N, Blaschczok HC, Photiadis J, Haun C, Asfour B, et al. Aortopulmonary Window Associated with Interrupted Aortic Arch: Report of Surgical Repair of Eight Cases and Review of Literature. Thorac Cardiovasc Surg. 2012 Jan 17. [Medline].

Carminati M, Borghi A, Valsecchi O, et al. Aortopulmonary window coexisting with tetralogy of Fallot: echocardiographic diagnosis. Pediatr Cardiol. 1990 Jan. 11(1):41-3. [Medline].

Antonetti I, Lorch D, Coe B, Maxey TS, Nallamshetty L, Dadlani GH, et al. Unrepaired Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries in an Adult Patient. Congenit Heart Dis. 2011 Dec 18. [Medline].

Marangi D, Peterson RJ, Ceithaml EL, Marvin WJ Jr. Surgical repair of d-transposition with aortopulmonary window: a case report. J Thorac Cardiovasc Surg. 1996 Mar. 111(3):671-2. [Medline].

Bajpai P, Shah S, Misri A, Rao S, Suresh P, Maheshwari S. Assessment of operability in d-transposition of great arteries with ventricular septal defect: A practical method. Ann Pediatr Cardiol. 2011 Jan. 4(1):41-4. [Medline]. [Full Text].

Nelson AW. Aorticopulmonary window in a dog. J Am Vet Med Assoc. 1986 May 1. 188(9):1055-8. [Medline].

Kiran VS, Singh MK, Shah S, John C, Maheshwari S. Lessons learned from a series of patients with missed aortopulmonary windows. Cardiol Young. 2008 Oct. 18(5):480-4. [Medline].

Valsangiacomo ER, Smallhorn JF. Images in cardiovascular medicine. Prenatal diagnosis of aortopulmonary window by fetal echocardiography. Circulation. 2002 Jun 18. 105(24):E192. [Medline].

Alvarez R, Garcia-Diaz L, Coserria F, Hosseinpour R, Antinolo G. Aortopulmonary window with atrial septal defect: prenatal diagnosis, management and outcome. Fetal Diagn Ther. 2011. 30(4):306-8. [Medline].

Waters BL, Allen EF, Gibson PC, Johnston T. Autopsy findings in a severely affected infant with a 2q terminal deletion. Am J Med Genet. 1993 Nov 15. 47(7):1099-103. [Medline].

Bergstrom CS, Saunders RA, Hutchinson AK, Lambert SR. Iris hypoplasia and aorticopulmonary septal defect: a neurocristopathy. J AAPOS. 2005 Jun. 9(3):264-7. [Medline].

Balaji S, Burch M, Sullivan ID. Accuracy of cross-sectional echocardiography in diagnosis of aortopulmonary window. Am J Cardiol. 1991 Mar 15. 67(7):650-3. [Medline].

Horimi H, Hasegawa T, Shiraishi H, et al. Detection of aortopulmonary window with ventricular septal defect by Doppler color flow imaging. Chest. 1992 Jan. 101(1):280-1. [Medline].

Garver KA, Hernandez RJ, Vermilion RP, Goble M. Images in cardiovascular medicine. Correlative imaging of aortopulmonary window: demonstration with echocardiography, angiography, and MRI. Circulation. 1997 Aug 5. 96(3):1036-7. [Medline].

Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/Real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008 Jan. 25(1):96-9. [Medline].

Stamato T, Benson LN, Smallhorn JF, Freedom RM. Transcatheter closure of an aortopulmonary window with a modified double umbrella occluder system. Cathet Cardiovasc Diagn. 1995 Jun. 35(2):165-7. [Medline].

Naik GD, Chandra VS, Shenoy A, et al. Transcatheter closure of aortopulmonary window using Amplatzer device. Catheter Cardiovasc Interv. 2003 Jul. 59(3):402-5. [Medline].

Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv. 2008 Feb 15. 71(3):405-11. [Medline].

Backer CL, Mavroudis C. Surgical management of aortopulmonary window: a 40-year experience. Eur J Cardiothorac Surg. 2002 May. 21(5):773-9. [Medline].

Di Bella I, Gladstone DJ. Surgical management of aortopulmonary window. Ann Thorac Surg. 1998 Mar. 65(3):768-70. [Medline].

Hew CC, Bacha EA, Zurakowski D, et al. Optimal surgical approach for repair of aortopulmonary window. Cardiol Young. 2001 Jul. 11(4):385-90. [Medline].

Prasad TR, Valiathan MS, Shyamakrishnan KG, Venkitachalam CG. Surgical management of aortopulmonary septal defect. Ann Thorac Surg. 1989 Jun. 47(6):877-9. [Medline].

Tkebuchava T, von Segesser LK, Vogt PR, et al. Congenital aortopulmonary window: diagnosis, surgical technique and long-term results. Eur J Cardiothorac Surg. 1997 Feb. 11(2):293-7. [Medline].

Roubertie F, Kalfa D, Vergnat M, Ly M, Lambert V, Belli E. Aortopulmonary window and the interrupted aortic arch: midterm results with use of the single-patch technique. Ann Thorac Surg. 2015 Jan. 99(1):186-91. [Medline].

Li X, Zhu D, Feng Y. Transcatheter closure of late-onset residual aortopulmonary septal defect using a muscular ventricular septal occluder. Int Heart J. 2014. 55(1):89-91. [Medline].

Nishimura RA, Otto CM, Bonow RO, et al, for the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014 Jun 10. 63(22):e57-185. [Medline].

Nishimura RA, Carabello BA, Faxon DP, et al. ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. 2008 Aug 19. 118(8):887-96. [Medline]. [Full Text].

Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007 Oct 9. 116(15):1736-54. [Medline]. [Full Text].

Chopra PS, Reed WH, Wilson AD, Rao PS. Delayed presentation of anomalous circumflex coronary artery arising from pulmonary artery following repair of aortopulmonary window in infancy. Chest. 1994 Dec. 106(6):1920-2. [Medline].

Grunenfelder J, Zund G, Vogt PR, Turina MI. Aortopulmonary window with anomalous origin of the right coronary artery. Ann Thorac Surg. 1999 Jan. 67(1):233-5. [Medline].

Izumoto H, Ishihara K, Fujii Y, et al. AP Window and Anomalous Origin of the Right Coronary Artery From the Window. Annals of Thoracic Surgery. 1999. 68:557-9. [Medline].

McElhinney DB, Reddy VM, Tworetzky W, et al. Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants Am J Cardiol</i>. 1998 Jan 15. 81(2):195-201. [Medline].

Tulloh RM, Rigby ML. Transcatheter umbrella closure of aorto-pulmonary window. Heart. 1997 May. 77(5):479-80. [Medline].

van Son JA, Puga FJ, Danielson GK, et al. Aortopulmonary window: factors associated with early and late success after surgical treatment. Mayo Clin Proc. 1993 Feb. 68(2):128-33. [Medline].

Barry A Love, MD Assistant Professor , Department of Medicine, Division of Cardiology, Assistant Professor, Division Pediatric Cardiology, Director, Pediatric Electrophysiology Service, Department of Pediatrics, Division of Pediatric Cardiology, Mount Sinai School of Medicine

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Disclosure: Nothing to disclose.

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children’s Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Aortopulmonary Septal Defect

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