Large Aorto-Pulmonary Window (APW) associated with severe pulmonary hypertension: A case report

Abstract

Background: APW is a rare congenital cardiac abnormality, accounting for 0.2% to 0.6% of patients. Hence the reported surgical experience is minimal. It is a communication b/w pulmonary artery (PA) and the ascending aorta (Ao), in the presence of two separate semi lunar valves. Heart failure and irreversible pulmonary hypertension are the fearsome outcomes of untreated APW. Therefore, surgical closure is the first-line curative choice. We present a case of young boy who presented late in age (his second decade of life) with severe signs and symptoms. Case Presentation: A 12-year-old male patient from Baluchistan (balochi) presented with complaints of high grade fever for one week, palpitations since 12 days, along with past history of shortness of breath. On examination patient was anemic, cyanosed with raised JVP. On precordial examination apex beat was localized at the 6th intercostal space shifted to mid-axillary line. S1 was normal but S2 was loud with prominent pulmonary component. Grade IV/VI continuous murmur was heard in the left upper sterna border. Chest X-ray showed cardiomegaly with prominent pulmonary artery (PA) and bilateral lung congestion. Echocardiography showed enlarged and hypertrophied right ventricle associated with dilated PA and a large APW was seen with severe pulmonary artery pressure (PAP) of 102 mmHg. Diagnosis of APW was confirmed on cardiac catheterization. Furthermore, cardiac CT was performed which showed type I APW with severe PH and large patent PDA (patent ductus arteriosus). Patient then underwent repair of APW associated with reactive pulmonary hypertension that had a complete repair utilizing a trans-aortic approach. PDA was also ligated during the same procedure. Patient symptoms remarkably improved with a reduction PAP (40mmHg) after surgery and at 6 weeks follow-up. Follow-up echocardiography showed complete closure of the APW, and pharmacological therapy was no longer required in the patient. Conclusion: APW should be suspected in any child, who comes with repeated chest infections and signs of congestive heart failure. It should be surgically corrected early, in order to prevent the development of obstructive pulmonary vascular disease. There is conflict of interest.