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Erschienen in:
01.02.2006 | Images in Pediatric Cardiology
Anatomically Corrected Malposition of the Great Arteries in the Setting of Aortopulmonary Window Associated with Holoprosencephaly
Erschienen in: Pediatric Cardiology | Ausgabe 1/2006
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AA was diagnosed with a heart murmur at birth and echocardiography showed an aortopulmonary (AP) window with malposition of the great arteries (Fig. 1). Cardiac catheterization was performed to delineate the anatomy (Fig. 2) and hemodynamics. He had a normal neurological examination and there was no history of any seizure or focal neurological deficit. However, he was found to have holoprosencephaly on head ultrasound, which was confirmed by head computed tomography. After consultation with his family, surgical repair was not performed and the patient was offered comfort care and subsequently died. No postmortem was performed at the request of the family.
Fig. 1
(Top) Suprastrenal long-axis view demonstrating the aortopulmonary window (arrow). AO, aorta; LPA, left pulmonary artery. (Bottom) Subcostal short-axis view representing the anatomically corrected malposition with solitus atria and atrioventricular concordance. The great arteries arise from their appropriate ventricles but are in approximately the same frontal plane. Neither semilunar valve is in fibrous continuity with an atrioventricular valve (bilateral conus). The aorta is anterior and to the right of the pulmonary artery. MV, mitral valve; TV, tricuspid valve; AV, Aortic valve; PV, pulmonic valve; R, right; L, left; I, inferior; S, superior; A, anterior; P, posterior.
Fig. 2
Aortogram (lateral view) showing the aortopulmonary window (between the two arrows) and the aorta (AO) is anterior to the pulmonary artery (PA).
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