| Participant: | Cooke, Rhonda |
CLINICOPATHOLOGIC CORRELATION OF A CALCIUM CHANNEL RESPONSIVE PATIENT WITH IDIOPATHIC PULMONARY ARTERY HYPERTENSION (IPAH)
Rhonda K. Cooke, MD1, A.G. Duarte, M.D. 2, M.A. Eltorky, M.D. 1, N. Boroumand1, M.D., and D.F. Cowan, M.D. 1
1Departments of Pathology and 2Pulmonary Medicine, UTMB
Background: We describe the clinical and pathologic findings of a patient with IPAH initially responsive to calcium channel blockers and treated with combination therapy for 12 years. Methods: Retrospective case report correlating 15 years of clinical history with postmortem anatomic and pathologic findings. Results: The patient was diagnosed with IPAH by right heart catheterization. She responded to calcium channel blockers and was sustained on a multi-drug regimen for 12 years, until she contracted a respiratory illness with pericardial effusion and died. Autopsy revealed cardiomegaly and right heart dilatation and hypertrophy. The main pulmonary trunk exhibited aneurysmal dilatation to 15 cm with eight intimal tears of varying degrees of healing, the largest more than 9 cm in length. Medial disruption with associated hemorrhage was found in some of the tears, while others showed mature scar formation. Conclusions: Intimal injury and repair of the large and medium sized arteries was observed in a patient living with IPAH. Supported by the Departments of Pathology and Pulmonary Medicine.