Assessment of Pulmonary Artery Morphology by Optical Coherence Tomography in a Patient with Idiopathic Pulmonary Arterial Hypertension

Abstract

We assessed the pulmonary vascular morphology by optical coherence tomography （OCT） in a 10-year-old boy with idiopathic pulmonary arterial hypertension. The thickness of the intima and media was 0.18 mm and 0.19 mm, respectively, in the distal pulmonary artery. The findings suggested that pulmonary artery lesions involved medial hypertrophy and intimal thickening （Heath-Edwards grade II or III）. Pulmonary arterial pressure was greater than aortic pressure. The patient had poor response to the acute vasodilator test using intravenous epoprostenol administration. The hemodynamic result was compatible with the OCT findings. We propose OCT as a useful tool in diagnosing pulmonary artery hypertension and may provide helpful information for the pulmonary arterial remodeling process, severity, therapeutic strategy, and prognosis.