Congenital cardiovascular anomalies
Updates to Article Attributes
Congenital cardiovascular anomalies are relatively common, with an incidence of up to 1% if small muscular VSDs are included. As a group, there is a much greater frequency in syndromic infants and in those that are stillborn.
Clinical presentation
Broadly, congenital cardiovascular anomalies can be clinically divided into:
Depending on the lesion and its severity, patients may be asymptomatic or conversely, may present with rapidly deteriorating congestive cardiac failure.
Pathology
These defects as a group have a heterogeneous aetiology with many resulting from an interplay between both genetic and environmental factors.
Subtypes
A list of anomalies and their approximate occurrences (as a percentage of all congenital cardiac disease) are given below:
- ventricular septal defect (VSD): 40%
- atrial septal defect (ASD): 10%
- congenital pulmonary stenosis: 8 %
- patent ductus arteriosus (PDA): 7%
- transposition of the great arteries (TGA): 7%
- tetralogy of Fallot (TOF): 5%
- coarctation of the aorta: 5%
- atrioventricular septal defect (AVSD): 4%
- congenital aortic stenosis: 4%
- hypoplastic left heart syndrome (HLHS): 4%
- double outlet right ventricle (DORV): 2%
- interrupted aortic arch (IAA): 1.5%
- truncus arteriosus: 1%
- total anomalous pulmonary venous return (TAPVR): 1%
- tricuspid atresia: 1%
- pulmonary atresia: ?
- Ebstein anomaly: 0.7%
- Bland-White-Garland syndrome: ~0.5%
- cor triatriatum: ~0.1%
- partial anomalous pulmonary venous return (PAPVR): 0.5%
- double outlet left ventricle (DOLV): ?
- aortopulmonary septal defect: <1%
- Gerbode defect: <1%
- Shone complex: <1%
- hypoplastic right heart syndrome (HRHS): ?
- aortoventricular tunnel <0.1%
Other valvular anomalies
Percentages may not necessarynecessarily add up to a 100 due to overlap and simultaneous occurrence of abnormalities.
Associations
Congenital cardiac anomalies can be found with many aneuploidicaneuploid conditions which include:
- trisomy 18: up to 90% can have cardiac anomalies
- trisomy 13: up to 90%
- trisomy 21: up to 50%
- Turner syndrome: up to 40%
-<a title="hypoplastic right heart syndrome (HRHS)" href="/articles/hypoplastic-right-heart-syndrome-hrhs">hypoplastic right heart syndrome (HRHS)</a>: ?</li>- +<a href="/articles/hypoplastic-right-heart-syndrome-hrhs">hypoplastic right heart syndrome (HRHS)</a>: ?</li>
-</ul><p>Percentages may not necessary add up to a 100 due to overlap and simultaneous occurrence of abnormalities.</p><h5>Associations</h5><p>Congenital cardiac anomalies can be found with many aneuploidic conditions which include:</p><ul>- +</ul><p>Percentages may not necessarily add up to 100 due to overlap and simultaneous occurrence of abnormalities.</p><h5>Associations</h5><p>Congenital cardiac anomalies can be found with many aneuploid conditions which include:</p><ul>