Acyanotic Heart Disease With Normal Vascularity
Alexander J. Towbin, MD
DIFFERENTIAL DIAGNOSIS
Common
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Aortic Coarctation
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Aortic Stenosis
Less Common
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Interrupted Aortic Arch
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Pulmonary Stenosis
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
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Obstructive lesions cause acyanotic heart disease with normal pulmonary vascularity
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Patients with small, left-to-right shunts have normal vascularity
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In neonates, increased pulmonary vascular resistance causes left-to-right shunt to have normal vascularity
Helpful Clues for Common Diagnoses
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Aortic Coarctation
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Stenosis in proximal descending aorta
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Usually just beyond origin of left subclavian artery
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5-8% of congenital heart defects (CHD)
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2x more common in males
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Associations: Turner syndrome, bicuspid aortic valve, ventricular septal defect
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Severe coarct presents when ductus closes
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Mild coarct presents with upper extremity hypertension and ↓ lower extremity pulses
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Rib notching not usually seen on chest x-ray (CXR) until after age 6
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Treatment options: Surgical repair, angioplasty, stent placement
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Aortic Stenosis
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Types: Supravalvular, valvular, or subaortic
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Valvular aortic stenosis is most common
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Accounts for 3-6% of CHD
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4x more common in males
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˜ 20% have associated cardiac anomaly
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Severity related to degree of obstruction
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CXR: Normal or with cardiomegaly, vascular congestion, and poststenotic dilation of ascending aorta
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Subaortic stenosis can be discrete or diffuse
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Supravalvular is least common
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Helpful Clues for Less Common Diagnoses
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Interrupted Aortic Arch
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Discontinuity of aorta (1% of all CHD)
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Associations: DiGeorge syndrome and 22q11 deletion
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3 types: Isolated, simple, and complex
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Isolated: No other cardiac anomalies
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Simple: Associated with ventricular septal defect and patent ductus arteriosus
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Complex: Associated with complex CHD
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Pulmonary Stenosis
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Types: Valvular, subvalvular, supravalvular, or in branch pulmonary arteries
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Valvular stenosis is most common
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7-9% of all CHD
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Presents with asymptomatic murmur
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CXR: Dilated main pulmonary artery
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Treatment: Balloon valvuloplasty
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Image Gallery
Sagittal MIP of T1 C+ subtraction MR shows a focal area of stenosis in the proximal descending aorta
 just distal to the origin of the left subclavian artery  . Stay updated, free articles. Join our Telegram channel
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