Congenital thrombotic occlusion of the ascending aorta and the aortic arch.
In a one-day-old male newborn with severe heart failure, the skin of the upper right thorax was pink, whereas the remaining areas were cyanotic. No peripheral pulses were palpable and the blood pressure could not be measured. On cardiac catheterization, systolic and diastolic pressures were elevated in the left ventricle (137/4/12 mmHg), but in the descending aorta, reached via a patent ductus arteriosus, the pressure was only 55/45 mmHg. O2 saturation was 97% in the left ventricle and 67% in the descending aorta. Angiocardiography showed an extreme obstruction of the ascending aorta and the aortic arch. The infant died on the second day. Postmortem examination revealed a wall-adherent calcified thrombus that totally occluded the lumen. No etiologic explanation could be obtained from the histologic examination, anamnestic data, or clinical findings.
D000328:Adult; D001011:Aorta; D001013:Aorta, Thoracic; D001018:Aortic Diseases / Q000151:congenital* / Q000209:etiology; D001157:Arterial Occlusive Diseases / Q000151:congenital* / Q000209:etiology; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013927:Thrombosis / Q000151:congenital* / Q000209:etiology