Aortopulmonary Septal Defect Workup
- Author: Barry A Love, MD; Chief Editor: Stuart Berger, MD more...
Preoperatively in patients with an aortopulmonary septal defect (APSD), obtain a CBC count, cross match, and urinalysis.
If the infant has been on diuretics and digoxin preoperatively, checking electrolyte and digoxin levels is prudent.
Infants with congestive heart failure (CHF) are at risk for potassium depletion from diuretics and may be at risk for digoxin toxicity from routine dosing during decreased renal perfusion. The combination of a volume-loaded circulation with potassium depletion and an elevated serum digoxin level may be a risk factor for ventricular fibrillation at the time of sternotomy.
Radiography typically reveals cardiomegaly with increased pulmonary blood flow. Pulmonary congestion is proportional to the degree of CHF.
ECG typically reveals biventricular hypertrophy or right ventricular hypertrophy. Electrical evidence for left atrial enlargement may present.
In infants and children, this test (if performed by experienced personnel and reviewed by a skilled pediatric echocardiographer) should reveal the defect and associated abnormalities in virtually all cases.[17, 18] See the image below.
Doppler color flow mapping shows retrograde flow in the transverse arch during diastole. By contrast, in an infant with a large left-sided patent ductus arteriosus (PDA) and a left aortic arch, diastolic flow in the arch is antegrade. For patients with poor echocardiographic windows, transesophageal echocardiography or cardiac MRI may be an option.
When an aortopulmonary septal defect is found, the echocardiographer should be especially careful to look for other abnormalities, which are present in 50% of cases of aortopulmonary septal defect. In addition to identifying the aortopulmonary septal defect, the ventricular septum, origins of the pulmonary arteries, aortic arch, presence of a PDA, and origins of the coronary arteries should be carefully scrutinized.
In addition, 3-dimensional echocardiography has been used to help characterize an aortopulmonary septal defect.
Cardiac catheterization (CC) should not be required if anatomy has been well defined by noninvasive means and if the clinical and noninvasive data are both consistent with low pulmonary vascular resistance (PVR). If questions or additional incompletely characterized defects are noted, careful hemodynamic and angiographic assessment may be helpful.
Beyond infancy, CC may be required to exclude irreversible pulmonary vascular obstructive disease (PVOD) before performing surgical repair. In older children with a diagnosis of a nonrestrictive aortopulmonary window, CC is advisable to establish pulmonary vascular reactivity before performing surgical repair. In the rare instance of a small restrictive defect, catheter therapy with device occlusion of the defect may be an option.
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