Correlative Imaging of Aortopulmonary Window
Demonstration With Echocardiography, Angiography, and MRI
A 9-month-old previously healthy girl presented to the emergency room in congestive heart failure. The parents had previously noticed sweating during feedings with occasional increased respiratory rate. Physical examination showed her to be at less than the 5th percentile in weight and height for age, acyanotic, and tachypneic. Her precordium was hyperdynamic, with a normal S1, a narrowly split S2, a grade III/VI continuous murmur at the upper left sternal border radiating to the axilla and back, and a grade III/IV diastolic rumble at the apex. The liver edge was 3 cm below the costal margin. Pulses were normal and symmetrical.
The ECG showed sinus rhythm, a right ventricular conduction defect, and left ventricular hypertrophy with ST changes diffusely throughout the inferolateral leads. Two-dimensional transthoracic echocardiography demonstrated a large aortopulmonary window with a large left-to-right shunt, a dilated left atrium and ventricle, a dilated right pulmonary artery, and no atrial septal defect, ventricular septal defect, or patent ductus arteriosus. Cardiac catheterization was done because of the late presentation and confirmed the echocardiographic findings, with additional demonstration of normal coronary arteries and evidence of low pulmonary vascular resistance. Axial and coronal T1-weighted and fast gradient-recalled sequence with cardiac-triggered segmented acquisition (FastCard) with a 1.5-T GE MRI scanner also demonstrated the exact location and size of the aortopulmonary window. The child then underwent uncomplicated Gortex patch closure of the defect via a transpulmonary approach.
Aortopulmonary window should be considered in the differential diagnosis of a large patent ductus arteriosus or ventricular septal defect. In contrast to truncus arteriosus, both aortic and pulmonary valves are present and normally related. The classic form of aortopulmonary window involves a windowlike communication between the left wall of the ascending aorta and the right wall of the main pulmonary artery ≈1.5 cm above the semilunar valves. Less common types have a more distal and posterior communication between the posterior wall of the ascending aorta and the proximal right pulmonary artery or anomalous origin of the pulmonary artery from the aorta. Coexistent conditions include patent ductus arteriosus, ventricular septal defects, tetralogy of Fallot, coarctation, or interruption of the aortic arch. Surgical correction is determined by the location of the aortopulmonary window as well as associated anomalies.
The purpose of these images was to demonstrate the ability of MRI to fully depict the important anatomic features of an aortopulmonary window. In most cases, noninvasive imaging with echocardiography and/or MRI will adequately define the anatomy before surgery.
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Reprint requests to Kimberly A. Garver, MD, Section of Pediatric Radiology, C.S. Mott Children’s Hospital, University of Michigan Hospitals, Ann Arbor, MI 48109-0252.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC 4-265, Houston, TX 77030.
- Copyright © 1997 by American Heart Association