A NEW CASE OF PARTIAL ANOMALOUS PULMONARY VENOUS RETURN IN THE FORM OF INCOMPLETE CONGENITAL PULMONARY VENOLOBAR SYNDROME WITH THE DEVELOPMENT OF SECONDARY PULMONARY VENO-OCCLUSIVE DISEASE

Abstract

Partial anomalous pulmonary venous return (PAPVR) is a vascular developmental abnormality. As a result of it one or more pulmonary veins do not drain into the left atrium. A particularly rare and difficult form of PAPVR for diagnosis is congenital pulmonary venolobar syndrome (CPVS). However, its manifestations include a number of typical combinations of cardiovascular and pulmonary pathologies such as hypoplasia of the right lung, PAPVR of the right lung into the inferior vena cava («scimitar sign»), hypo or aplasia of the right pulmonary artery. The paper presents an observation, which was not previously described in the literature, of the detection of Partial anomalous pulmonary venous return in the form of incomplete is congenital pulmonary venolobar syndrome with the development of secondary pulmonary veno occlusive disease.