(0.8
Mb)Pillekamp F, 1 Hannes T,1
Koch D,2 Brockmeier K,1 Sreeram N.1 Transcatheter
closure of symptomatic aortopulmonary window in an infant. Images Paediatr
Cardiol 2008;35:11-17
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(0.8
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Pillekamp F, 1 Hannes T,1
Koch D,2 Brockmeier K,1 Sreeram N.1 Transcatheter
closure of symptomatic aortopulmonary window in an infant. Images Paediatr
Cardiol 2008;35:11-17
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| 1Pediatric Cardiology, University of Cologne, 50931 Cologne, Germany | |
| 2Pediatric Cardiologist, 51607 Cologne, Germany |
MeSH
| Heart Catheterization | Prosthesis Implantation/instrumentation/*methods | Blood Vessel Prosthesis Implantation |
| Heart defects, congenital |
Abstract
An aortopulmonary window is a rare congenital cardiac defect. In the
majority of symptomatic neonates and infants, primary surgical repair is
the treatment of choice. In selected infants, catheter closure of the defect
with a device may be feasible. We report on the successful closure of an
AP window in a 12 month old infant, using a 6mm Amplatzer septal occluder.
The procedure and follow-up were uneventful..
Introduction
Aortopulmonary window is a rare congenital cardiac malformation that
typically requires surgical treatment. We report on an infant with an intermediate-type
of aortopulmonary window who presented with mild clinical signs of heart
failure, in whom catheter occlusion was successfully achieved with a 6mm
Amplatzer septal occluder at one year of age.1
Case report
The patient presented to the cardiologist with a murmur at 6 weeks
of age. Minimal feeding difficulties were also present. The 12 lead electrocardiogram
showed mild biventricular hypertrophy. Two-dimensional echocardiography
identified an aortopulmonary window (diameter varying between 4 and 6mm)
with a turbulent left-to-right shunt and a peak Doppler gradient of > 50
mmHg (Fig. 1).
The left atrium and ventricle were enlarged. Anticongestive medication with furosemide (1 mg/kg d) and spironolactone (3 mg/kg d) was started. Serial echocardiographic studies continued to show a restrictive defect, without evidence for pulmonary hypertension. At 12 months of age (body weight: 9.2 kg) cardiac catheterization was performed to electively close the defect.
Procedure
Under general anesthesia, the femoral artery and vein were percutaneously
cannulated. heparin (100 units/kg) was administered after vascular access
was obtained. Ascending aortography demonstrated a 5mm aortopulmonary window
(Fig. 2, 3).
Over this wire, a 6F Amplatzer delivery system (Aga Medical Corporation,
USA) was advanced from the femoral vein, and through the AP window into
the descending aorta. A 6mm Amplatzer septal occluder was introduced into
the delivery catheter, and the distal (left atrial) disk was opened in
the aorta. The device was then retracted towards the defect, and the proximal
(right atrial) disk was opened on the pulmonary arterial side of the defect.
Appropriate device position was confirmed both by transthoracic echocardiography
(Fig. 1) and aortography, prior to release of the device (Fig. 4, 5).
The procedure was uncomplicated. The patient was discharged from hospital
on the following day, taking oral acetylsalicylic acid (aspirin 5 mg/kg
day) for 6 months.
Discussion
Aortopulmonary window is a rare congenital heart defect occurring in
0.1-0.2% of patients with congenital heart disease.2-4 In about
half the patients other cardiac abnormalities, especially an interruption
of the aortic arch type A, may be associated with an aortopulmonary window.2,4
Large defects resulting in severe left-to-right shunting typically present
within the first weeks or months of life because of congestive heart failure.
In contrast, a heart murmur without symptoms might be the only sign in
small lesions. Early surgical treatment is recommended for the larger defects,
to avoid the risk of progressive pulmonary vascular disease, but requires
the use of cardiopulmonary bypass.5,6 In the absence of evidence
for pulmonary hypertension, it may be justifiable to delay treatment.
There have been isolated reports of transcatheter closure of aortopulmonary windows.7-9 The majority however, have either been small defects, or have been closed beyond infancy.7-9 With current advances in interventional catheterisation techniques and devices used to occlude intracardiac shunts, such defects may also be closed relatively early in life by percutaneous catheterisation. In considering defects amenable for transcatheter closure, it is important to bear in mind the relationship of the coronary artery origins to the margins of the window. More distally located defects are ideal for catheter closure, and do not carry the risk of the device impinging on a coronary artery. An Amplatzer PDA device might also be a good alternative to the use of the ASD device as reported here. No obstruction to aortic or pulmonary flow has been reported in any of the descriptions of device closure of aortopulmonary windows to date.
References
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