Obstructive congenital heart lesions
- Lesion
Some babies are born with a narrowing in the aorta, a valve or a chamber. There is no hole, but the heart must push against an obstacle.
What it is
Congenital obstructions narrow a valve, an outflow tract or a great vessel. Coarctation of the aorta is a narrowing near the ductus arteriosus; the infantile (preductal) form presents with heart failure and lower-body cyanosis in newborns, the adult (postductal) form with hypertension in the arms and weak leg pulses. Pulmonary stenosis ranges from mild valve narrowing to atresia. Aortic stenosis can be valvular, subvalvular or supravalvular (Williams syndrome). Hypoplastic left heart syndrome is the severe end, with an underdeveloped left ventricle and aorta.
Structure
- Coarctation: a sharp ridge-like infolding of the aortic media, with dilation of the aorta beyond it and of the arch vessels before it.
- Congenital aortic valve stenosis: a thickened unicuspid or bicuspid valve; left ventricle hypertrophied.
Congenital obstructions compared
| Lesion | Site | Presentation | Associations |
|---|---|---|---|
| Coarctation, infantile (preductal) | Aortic isthmus before the ductus | Newborn failure, cyanosis of the lower body | PDA dependence, Turner syndrome |
| Coarctation, adult (postductal) | Just beyond the ductus (ligamentum) | Upper-limb hypertension, weak femoral pulses, rib notching | Bicuspid aortic valve, berry aneurysms |
| Pulmonary stenosis or atresia | Pulmonary valve or infundibulum | From a murmur to cyanosis (atresia) | Noonan syndrome, tetralogy |
| Aortic stenosis (valvular, sub-, supravalvular) | Valve, below the valve, or above it | Murmur, left ventricular hypertrophy, sudden death | Bicuspid valve; Williams syndrome (supravalvular) |
| Hypoplastic left heart syndrome | Small LV, mitral and aortic atresia or stenosis | Shock as the ductus closes | Ductus-dependent systemic circulation |
Clinical notes
- Untreated adult coarctation leads to hypertension, heart failure, aortic dissection, intracranial hemorrhage and endocarditis.
Test yourself
Think of the answer, then tap the question to see it.
What causes rib notching in adult coarctation?
Enlarged, tortuous intercostal arteries carrying collateral flow erode the undersurface of the ribs.
Which chromosomal syndrome is strongly associated with coarctation?
Turner syndrome (45,X).
What type of aortic stenosis occurs in Williams syndrome?
Supravalvular aortic stenosis, from the elastin gene deletion.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Coarctation of the Aorta (StatPearls (NCBI Bookshelf), 2025); Congenital heart disease: MedlinePlus Medical Encyclopedia (MedlinePlus, US National Library of Medicine, 2025).
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Representative model, for educational purposes only.