Category: Case Report
We present a case of an unusual form of the ductus arteriosus termed “window ductus,” and highlight the importance of early recognition echocardiogram. The patient presented as a 22 week fetus due to advanced maternal age, maternal cardiomyopathy, and an abnormal 4-chamber view on the fetal anatomy ultrasound. Fetal images were consistent with polyvalvular disease with redundancy of the mitral, tricuspid and aortic valves. There was an extremely abnormal pulmonic valve that was thickened, stenotic, and regurgitant due to relative immobility of the cusps, creating a narrow, tunnel-like orifice and post-stenotic dilated main pulmonary artery. The right ventricle was hypertrophic and stiff with decreased systolic function; the left ventricle appeared normal. There was concern for a ductal-dependent lesion given the narrow diameter of the effective pulmonary valve orifice. The ductal arch was not well-identified antenatally, but assumed to be patent due to a normal Doppler flow.
The infant was born at 35 weeks due to maternal pre-eclampsia, and significantly cyanotic. Postnatal echo confirmed the aforementioned findings, with the additional finding of a direct communication between the descending aorta and left pulmonary artery. The communication had the appearance an aortopulmonary (AP) window, although in the wrong location, making this connection embryologically different than the usual AP window (neural crest abnormality) or PDA (abnormality of the sixth aortic arch). Prostaglandin therapy was initiated, although its efficacy was uncertain. The patient was urgently transferred to a tertiary center offering surgical repair. At surgery, the lesion was identified as a window ductus, was separated and patched.
This case highlights the importance of appropriate antenatal diagnosis of the fetal ductal arch, particularly in light of a ductal-dependent lesion.
Dorrough, Storm; Gavin McCullough; and Michelle Grenier. "The Window Ductus, a Rare Finding, and the Importance of Perinatal Echocardiography." (2020). https://digitalrepository.unm.edu/hsc_2020_pediatric_research/22