Double outlet right ventricle (DORV) is a highly complex congenital heart disease (CHD) entity, gaining increasing interest due to the rapid progress of cardiac surgery. The number of patients operated for this congenital defect has been growing since 1980s and over following decades with active transitioning of this cohort into the adult medicine. However, the diversity of initial anomaly and performed interventions makes challenging the management of these patients. This is particularly important in the regions where specialized adult CHD cardiology still remains underdeveloped. In this review, we observe the basic principles of DORV nomenclature, main types of the operations and possible late complications. The article focuses on adult patients and offers illustrations from clinical practice.
A 25‐year‐old pregnant woman with low oxygen saturation due to unclear congenital heart disease was admitted. During work‐up the rare anomaly of superior vena cava drainage to the left atrium was diagnosed by transthoracic bubble test with agitated saline. The pathology was later confirmed by contrast multislice computed tomography angiography after delivery.
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