Distal Aortopulmonary Window: a Morphological Variation
Milind Chaudhari, MD,
Asif Hasan, FRCS1
Department of Pediatric Cardiology
1 Department of Cardiothoracic Surgery, Freeman Hospital, Newcastle upon Tyne, England, UK
Milind Chaudhari, MD, Tel: +44 191 233 7484, Fax: +44 191 233 1417, Email: milind.chaudhari{at}nuth.nhs.uk, Department of Pediatric Cardiology, Freeman Hospital, Newcastle upon Tyne, NE7 7DN, UK.
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ABSTRACT
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Several variations in the morphology of aortopulmonary window have been documented. We describe a unique variation of a large aortopulmonary window, and its successful surgical correction.
Key Words: Aortopulmonary Septal Defect • Heart Defects • Congenital • Heart Failure • Congestive • Hypertension • Pulmonary
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INTRODUCTION
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Aortopulmonary window is a rare congenital cardiac anomaly characterized by a communication between the ascending aorta and the pulmonary trunk, with normal separation of the aortic and pulmonary valves. The size and location of the defect may vary, but it is limited to the area between the 2 semilunar valves and the branch pulmonary arteries.1,2 A morphological variation of aortopulmonary window in association with a right aortic arch is described.
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CASE REPORT
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A 4-month-old boy was admitted in cardiac failure with a heart rate of 170 beats per minute, respiratory rate of 60 breaths per minute, and moderate subcostal recession. Cardiovascular examination revealed a hyperdynamic precordium, good volume pulse with no radio-femoral delay, a grade 3/6 pansystolic murmur, systemic saturation of 95% in air, and blood pressure of 92/54 mm Hg. An electrocardiogram showed sinus rhythm with right axis deviation and right ventricular hypertrophy. Chest radiography confirmed cardiomegaly (cardiothoracic ratio, 0.61) and pulmonary plethora. An echocardiogram revealed normal systemic and pulmonary venous connections, atrioventricular and ventriculoarterial concordance, and normal intracardiac anatomy. The right ventricle was enlarged with moderate tricuspid regurgitation. The aortic arch was right-sided with a mirror-image branching pattern and no coarctation. A large aortopulmonary left-to-right shunt was noted on color Doppler. Cardiac catheterization revealed a large left-to-right shunt at the great arterial level (pulmonary systemic flow ratio, 5 : 1). The pulmonary artery pressure was elevated to 65/27(46) mm Hg, with simultaneous aortic pressure of 70/27(48) mm Hg. A left ventriculogram revealed normal left ventricular size and function, normal aortic valve, a right aortic arch with a mirror-image branching pattern, and a large distal aortopulmonary window (Figure 1A
). A right ventriculogram confirmed enlargement of the ventricle with normal function. The pulmonary valve was normal with confluent branch pulmonary arteries. A typical catheter course was observed from the right ventricular infundibulum through to the pulmonary valve and the main pulmonary trunk into the ascending aorta via a large distal aortopulmonary window (Figure 1B). The patient was operated on through a median sternotomy with cardiopulmonary bypass and moderate hypothermia. The top of the main pulmonary artery was directly continuous with the ascending aorta on its left lateral aspect. A large distal aortopulmonary window measuring approximately 15 mm at its maximum dimension was located between the ascending aorta and the pulmonary trunk, beyond the origin of the right pulmonary artery. The aortic and pulmonary valves were normally separated, and the main pulmonary arterial trunk gave rise to confluent branch pulmonary arteries. A left-sided ductal ligament was seen connecting the left pulmonary artery to the left subclavian artery. An incision was made in the anterior wall of the aortopulmonary window, and the pulmonary artery and aorta were separated. The aortic defect was repaired with a patch of bovine pericardium, and the pulmonary artery was directly oversewn. The patient was separated from cardiopulmonary bypass without difficulty. His postoperative progress was uneventful, and he was discharged from the hospital 8 days later. At the 2-year postoperative follow-up, he was symptom-free with a normal cardiovascular examination.
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Figure 1. (A) left ventricular angiogram showing right aortic arch and aortopulmonary window. (B) Aortogram with the catheter through the pulmonary artery and aortopulmonary window. AO = aorta, APW = aortopulmonary window, LV = left ventricle, PA = pulmonary artery.
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DISCUSSION
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Aortopulmonary window results from a malformation in the division of the aortopulmonary trunk during embryogenesis. Several classifications of aortopulmonary window have been reported based on pathologic, angiographic, and morphological anatomy.1–3 Proximal defects are the most common and are situated between the ascending aorta and the main pulmonary artery, just above the sinus of Valsalva, with a little inferior margin. Distal defects are uncommon and are situated between the ascending aorta and the origin of the right pulmonary artery from the pulmonary trunk. They have a well-formed inferior rim, but little superior rim.
A large window ductus can simulate the clinical and echocardiographic findings of a large distal aortopulmonary window.4 Morphologically, however, this defect shows distinct characteristics. In window ductus, a large window-type communication is located underneath the aortic arch, just distal to the level corresponding to the origin of the left subclavian artery and the main pulmonary artery, above the ostium of the left pulmonary artery. In this case, a large communication was seen between the ascending aorta and the main pulmonary artery, above the origin of the right pulmonary artery. As there was no defect in the aortopulmonary septum, embryologically this could represent persistence of the 5th aortic arch. We believe this is a previously unreported variation of distal aortopulmonary window.
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REFERENCES
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- Richardson JV, Doty DB, Rossi NP, Ehrenhaft JL. The spectrum of anomalies of aortopulmonary septation. J Thorac Cardiovasc Surg 1979;78:21–7.[Abstract]
- Kutsche LM, Van Mierop LH. Anatomy and pathogenesis of aorticopulmonary septal defect. Am J Cardiol 1987;59:443–7.[Medline]
- Mori K, Ando M, Takao A, Ishikawa S, Imai Y. Distal type of aortopulmonary window. Report of 4 cases. Br Heart J 1978;40:681–9.[Abstract/Free Full Text]
- Grünenfelder J, Bartram U, Van Praagh R, Bove KE, Bailey WW, Meyer RA, et al. The large window ductus: a surgical trap. Ann Thorac Surg 1998;65:1790–1.[Abstract/Free Full Text]
Asian Cardiovasc Thorac Ann 2009;
17:413-414
© 2009 by SAGE Publications
DOI: 10.1177/0218492309345214
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ABSTRACT
INTRODUCTION
