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Virtual Pediatric Hospital: Paediapaedia: Coarctation of the Aorta Paediapaedia: Cardiovascular Diseases

Coarctation of the Aorta, Infantile

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Infantile type presents in the neonate or young infant with congestive heart failure and bounding upper extremity pulses and diminished lower extremity pulses. This infantile type is also known as preductal coarcation. The infantile type is the most frequent cause of pulmonary venous hypertension and congestive heart failure that presents in the second and third week of life.

Etiology/Pathophysiology:
Usually caused by a congenital indentation in the posterior wall of the aorta that usually is distal to the origin of the left subclavian artery at the level of the ductus arteriosus. Infantile type coarctation causes a diffuse narrowing while adult type coarctation causes a focal narrowing.

Seventy-five percent of patients have a bicuspid aortic valve, and ASD / VSD / PDA / mitral valve deformities can also be seen.

Since the ductus arteriosus is still often open, a pressure gradient is not present across the coarctation, and collateral circulation does not develop. Right ventricular volume overload and dilation develops because almost all blood reaching the descending aorta must come through the ductus arteriosus from the right ventricle. As the ductus begins to close, a pressure gradient develps across the coarctation and the relatively underdeveloped left ventricle begins to fail. Increased left atrial pressure is then transmitted to the pulmonary veins with progressive venous engorgement and pulmonary edema. Post stenotic dilation of the aorta is not seen due to prenatal lack of flow across the coarctation.

Pathology:
Not applicable

Imaging Findings:
The infantile type usually presents with congestive heart failure and cardiomegaly.

 

DDX:

References:
See References Chapter.

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