A 50-year-old unrepaired patient with pulmonary atresia and ventricular septal defect
Journal Title: Journal of Rare Cardiovascular Diseases - Year 2016, Vol 2, Issue 8
Abstract
Pulmonary atresia with ventricular septal defect (PA + VSD) is a cyanotic congenital heart disease, also classified as Tetralogy of Fallot with pulmonary atresia. PA + VSD accounts for about 1–2% of congenital heart defects. The intracardiac anatomy is similar to tetralogy of Fallot but there is no direct communication between the right ventricle and pulmonary arteries. Major problems with surgical treatment are related to complexity of the pulmonary vascular bed. We report a case of a 50-year-old woman with congenital heart disease who was admitted to our Centre in July 2012. Congenital heart disease was first diagnosed at the age of 28 and at that time it was classified as a pulmonary valve atresia with ventricular septal defect with right-to-left shunt and common arterial trunk. Diagnostics performed in our Centre confirmed complicated anatomy of vessels in the chest, especially narrow and hypoplastic major aortopulmonary collateral arteries arising from descending aorta and left subclavian artery. The congenital heart disease was reclassified as a pulmonary atresia with ventricular septal defect. JRCD 2016; 2 (8): 270–274
Authors and Affiliations
Agnieszka Sarnecka, Anna Tyrka, Grzegorz Kopeć, Piotr Podolec
Keywords
Related Articles
- EP ID EP248030
- DOI 10.20418/jrcd.vol2no8.248
- Views 111
- Downloads 0
Hypertrophic cardiomyopathy – symptomatic atrial fibrillation in a patient at high risk of sudden cardiac death
We present the case of a young patient with significant left ventricular hypertrophy as a common representation of the hypertrophic cardiomyopathy (HCM) phenotype. The clinical presentation and diagnostic route of the di...
Pulmonary arterial hypertension in a patient with unilateral pulmonary artery absence (RCD code: II-2A.1)
Pulmonary artery absence is a rare congenital defect, occurring in 1 out of 100 000 individuals. In its isolated unilateral form only one in three cases concerns the left artery. The first symptoms usually appear in youn...
A 34‐year‐old man with non‐obstructive apical hypertrophic cardiomyopathy (RCD code: III‐2A.1)
Hypertrophic cardiomyopathy (HCM) is a primary disease of the myocardium that is defined by the presence of regional (more frequent) or global myocardial hypertrophy, which usually results in functional cardiac impairmen...
Accidentally discovered non‑compaction cardiomyopathy in patient with initial diagnosis of acute coronary syndrome (RCD code: III-5A)
Isolated non‑compaction cardiomyopathy is a rare congenital developmental disorder, which belongs to the group of unclassified cardiomyopathies by the ESC Position Statement. Typical echocardiographic findings include mu...
Comparison of platelet count reduction in patients with essential thrombocythaemia treated with hydroxyurea and thromboreductin. Single centre experience
Essential thrombocythemia is one of the Ph-negative myeloproliferative neoplasms treated with hydroxyurea. An alternative strategy may be a therapy with thromboreductin. The aim of the study was to compare the effectiven...