• Niaz Ali

• Muhammad Zahid Ullah

• Muhammad Aasim

• Syed Mumtaz Anwar Shah

• Amir Mohammad Khan

• Zainab Sultana

• A. Muzahir

• M. Rehman

• Abdul Waheed Sahibzada

Abstract

Pulmonary artery(PA) dissection is a rare but fatal pathology. Diagnosis is rarely made during life as false lumen tends to rupture resulting in cardiac tamponade and sudden death. Congenital heart diseases like Patent Ductus Arteriosus (PDA) can lead to pulmonary hypertension which is the main cause of PA dissection. A 35 years old male presented to us with acute right sided chest pain and dyspnoea.Patient was diagnosed with some congenital heart disease in childhood but was not treated. CT Angiography confirmed main pulmonary artery dissection which was extending to right pulmonary artery. While in the ward, the patient deteriorated, went into shock, resuscitated, shifted to operation room. Median sternotomy was done, cardiopulmonary bypass established, patent ductus arteriosus closed and PA repair using T-graft done. But the patient did not come off cardiopulmonary bypass and died. Pulmonary artery dissection is an extremely rare condition but mostly fatal. The main cause is chronic pulmonary hypertension either primary or secondary to congenital heart diseases, though there are other causes as well like right heart endocarditis, amyloidosis, trauma, severe atherosclerosis etc. The progression and natural course is not known as there are very few cases reported in literature. Optimum management for Pulmonary artery dissection has to be defined because of its association with congenital heart diseases, so that prompt measures can be taken to save precious lives.

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