Summary: A 16-year-old female presented with progressive dyspnea and syncope
episodes. The patient had had a personal history of a murmur since
childhood, which was clinically diagnosed without additional studies,
and had remained healthy until the onset of symptoms. Echocardiography
revealed severe pulmonary hypertension, and chest radiography identified
signs of pulmonary hypertension. Therefore, the patient underwent
surgery to close the defect with a pericardial patch. After the
procedure, the patient had a healthy recovery and was eventually discharged.
Aortopulmonary window with severe nonreactive pulmonary hypertension
As a ligament injury was suspected computed tomography angiography
(CTA) demonstrated a large communication between the ascending aorta and
the pulmonary artery trunk (Figures 1 and 2), with a 32-mm diameter
defect in the antero-posterior axis and signs of pulmonary hypertension.
Right heart catheterization showed a significant short circuit
between the ascending aorta and the pulmonary artery trunk (Figure
3) with severe pulmonary hypertension nonreactive to intravenous nitrates
and 100% oxygen.
Aortopulmonary window is caused by the failure to fuse of the two
opposing conotruncal ridges that are responsible for separating the
truncus arteriosus into the aorta and pulmonary artery. This defect may
be found anywhere from just above the semilunar valves to themore distal
ascending aorta and main pulmonary artery.1
It is a rare lesion and its prevalence among patients with congenital
heart disease is only 0.1% to 0.2%. Currently, approximately 300 cases
are cited in the literature1 in which aortopulmonary window
occurs as an isolated lesion. It can also be associated with other
cardiac abnormalities in one-third to one-half of cases. The most common
associated lesions are arch abnormalities, specifically interrupted
aortic arch, and coarctation of the aorta.1-2 Abnormal origin
of the coronary arteries is commonly associated with aortopulmonary
window. The coronary arteries may arise from the edge of the defect or on
the pulmonary artery side of the defect. In addition, a variety of
other lesions can occur, though rarely, with aortopulmonary window. These
include ventricular septal defect (VSD), tetralogy of Fallot,
and transposition of the great vessels.3
Despite the rarity of aortopulmonary window, the condition has been
classified into 3 types: proximal, or type I; distal, or type II;
and total, or type III. It has been suggested that, when associated with
interrupted aortic arch, aortopulmonary windows are larger with
greater distal extension.4
In most patients, these alterations cause a severe short circuit from
left to right—similar to that of patients with left-to-right
shunts, such as patent ductus arteriosus or VSD—leading to the
development of congestive heart failure with dyspnea and hepatomegaly5 mainly
during the first year of life or later in childhood. Pulmonary
hypertension can be fatal in almost all cases if not treated early in
childhood or adolescence;6 the few surviving adult patients have symptoms associated with severe pulmonary hypertension, making these cases inoperable.7 There are some reports in the literature of successful surgery in adult patients8
and some are candidates for heart-lung transplant. Physical examination
demonstrates a tachypneic infant with accessory respiratory muscle use.
Cardiac examination reveals an enlarged heart and, like patients with
patent ductus arteriosus, the pulses are bounding. A systolic murmur can
be heard along the left sternal border; however, unlike patients with a
patent ductus arteriosus, a diastolic component to the murmur is rare.6
Chest x-rays reveal cardiomegaly and increased pulmonary vascular
markings consistent with increased pulmonary blood flow. Diagnosisis
made with echocardiography. The location and size of the communication,
as well as associated anomalies, are carefully identified. Cardiac
catheterization is rarely indicated and reserved for patients who
present after early infancy and, therefore, are at risk for elevated
pulmonary vascular resistance, or any patient in whom the anatomy cannot
be adequately defined by echocardiography.9
Multidetector CT (MDCT) and magnetic resonance imaging (MRI) can
noninvasively demonstrate the defect and signs of pulmonary
hypertension. Furthermore, MRI permits evaluation of the direction and
quantification of the flow and the presence of other
Aortopulmonary window is a rare defect caused by failure of the 2
opposing conotruncal ridges to fuse, which may occur as an isolated
lesion or in association with other cardiac abnormalities. Currently,
early mortality following repair of simple aortopulmonary window
approaches zero and depends on the presence of associated lesions.
Long-term outcomes should be excellent. MDCT and MRI are useful
noninvasive techniques that can could demonstrate the defect and signs
of pulmonary hypertension.
- Moruno Tirado A, Santos De Soto J, Grueso Montero J, et al. Aortopulmonary window: Clinical assessment and surgical results. Rev Esp Cardiol. 2002;55:266-270.
- Soares AM, Atik E, Cortez TM, et al. Aortopulmonary window. Clinical and surgical assessment of 18 cases. Arq Bras Cardiol. 1999;73:59-74.
- Erez E, Dagan O, Georghiou GP, et al. Surgical management of aortopulmonary window and associated lesions. Ann Thorac Surg. 2004;77:484-487.
- Mori K, Ando M, Takao A, et al. Distal type of aortopulmonary window. Report of 4 cases. Br Heart J. 1978;40:681-689.
- Santos W, Rossi R, Abecasis M, et al. Aortopulmonary window—a review of nine cases. Rev Port Cardiol. 2008;27:1453-1462.
Morrow AG, Greenfield LJ, Braunwald E. Congenital aortopulmonary septal
defect. Clinical and hemodynamic findings, surgical technic, and
results of operative correction. Circulation. 1962;25:463-476.
- Perloff JK. The clinical recognition of congenital heart disease. 4th ed. W.B. Saunders; Philadelphia, PA: 1994.
- Aggarwal SK, Mishra J, Sai V, et al. Aortopulmonary window in adults: Diagnosis and treatment of late-presenting patients. Congenit Heart Dis. 2008;3:341-346.
- Trehan V, Nigam A, Tyagi S. Percutaneous closure of nonrestrictive aortopulmonary window in three infants. Catheter Cardiovasc Interv. 2008;71:405-411.
- Wang ZJ, Reddy GP, Gotway MB, et al. Cardiovascular shunts: MR imaging evaluation. Radiographics. 2003;23 Spec No:S181-194.