Primary pulmonary hypertension. Vascular structure, morphometry, and responsiveness to vasodilator agents.

• DOI: 10.1161/01.cir.80.5.1207
• Publication Date: 2011-06-17T20:29:13Z
• Authors: H I Palevsky; B L Schloo; G G Pietra; K T Weber; J S Janicki; E Rubin; A P Fishman
• Keywords: Right ventricular hypertrophy; Lung biopsy

The use of pharmacologic agents in the treatment pulmonary hypertension has not proved to be uniformly successful or predictable. One possible reason for vagaries response is that vascular lesions are consistent. We examined relation between structure resistance vessels unexplained (primary) and vasodilators. Our study involved 19 patients with clinically (mean pressure, 59 +/- 14 mm Hg). After characterizing them performing control hemodynamic measurements, we determined acute effects a series vasodilator have different mechanisms action. In 16 patients, lung biopsy material was related studies; nine including six who had undergone open biopsy, studies were pathologic changes found at autopsy. Histologic specimens from all evaluated qualitatively sorted into three subsets hypertensive arteriopathy: medial hypertrophy (with minimal intimal proliferation), arteriopathy plexiform (associated predominantly concentric laminar proliferation fibrosis), microthrombotic eccentric fibrosis). biopsies also quantitated by morphometric techniques. Using decrease calculated more than 30% accompanied mean arterial pressure least 10% define vasodilation, only four responders. varied considerably their responses agents. Patients similar clinical profiles differed respect nature obstructive Qualitative histologic examination tissue did provide basis predicting how individual would respond However, quantitative morphologic analysis initial prove helpful responsiveness subsequent course these hypertension. An area 18% cross-sectional an 85% predictive value identifying poorly during first 36 months follow-up.