Pulmonary Vascular Disease

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Abstract

The pulmonary vasculature is an anatomic compartment that is frequently overlooked in the histologic review of lung biopsy samples, other than those obtained specifically to assess pulmonary vascular disease. 1 Though often of a nonspecific nature, the histologic pattern of vascular remodeling may at times suggest its underlying pathogenesis and provide clues to the cause of pulmonary hypertension. 2 Disproportionately severe vascular pathology may further indicate alternate disease processes, such as congestive heart failure or thromboemboli, contributing to the patient’s overall respiratory condition.

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Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.

G D'Alonzo, R Barst, S. Ayres … (1991)

To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival. Registry with prospective follow-up. Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute. Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988. At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals. The estimated median survival of these patients was 2.8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration. Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.

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A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.

R Barst, L Rubin, W. A. Long … (1996)

Primary pulmonary hypertension is a progressive disease for which no treatment has been shown in a prospective, randomized trial to improve survival. We conducted a 12-week prospective, randomized, multicenter open trial comparing the effects of the continuous intravenous infusion of epoprostenol (formerly called prostacyclin) plus conventional therapy with those of conventional therapy alone in 81 patients with severe primary pulmonary hypertension (New York Heart Association functional class III or IV). Exercise capacity was improved in the 41 patients treated with epoprostenol (median distance walked in six minutes, 362 m at 12 weeks vs. 315 m at base line), but it decreased in the 40 patients treated with conventional therapy alone (204 m at 12 weeks vs. 270 m at base line; P < 0.002 for the comparison of the treatment groups). Indexes of the quality of life were improved only in the epoprostenol group (P < 0.01). Hemodynamics improved at 12 weeks in the epoprostenol-treated patients. The changes in mean pulmonary-artery pressure for the epoprostenol and control groups were -8 percent and +3 percent, respectively (difference in mean change, -6.7 mm Hg; 95 percent confidence interval, -10.7 to -2.6 mm Hg; P < 0.002), and the mean changes in pulmonary vascular resistance for the epoprostenol and control groups were -21 percent and +9 percent, respectively (difference in mean change, -4.9 mm Hg/liter/min; 95 percent confidence interval, -7.6 to -2.3 mm Hg/liter/min; P < 0.001). Eight patients died during the study, all of whom had been randomly assigned to conventional therapy (P = 0.003). Serious complications included four episodes of catheter-related sepsis and one thrombotic event. As compared with conventional therapy, the continuous intravenous infusion of epoprostenol produced symptomatic and hemodynamic improvement, as well as improved survival in patients with severe primary pulmonary hypertension.

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Primary pulmonary hypertension. A national prospective study.

S. Rich, D R Dantzker, S. Ayres … (1987)

A national registry was begun in 1981 to collect data from 32 centers on patients diagnosed by uniform criteria as having primary pulmonary hypertension. Entered into the registry were 187 patients with a mean age (+/- SD) of 36 +/- 15 years (range, 1 to 81), and a female-to-male ratio of 1.7:1 overall. The mean interval from onset of symptoms to diagnosis was 2 years. The most frequent presenting symptoms included dyspnea (60%), fatigue (19%), and syncope (or near syncope) (13%). Raynaud phenomenon was present in 10% (95% of whom were female) and a positive antinuclear antibody test, in 29% (69% female). Pulmonary function studies showed mild restriction (forced vital capacity [FVC], 82% of predicted) with a reduced diffusing capacity for carbon monoxide (DLCO), and hypoxemia with hypocapnia. The mean (+/- SD) right atrial pressure was 9.7 +/- 6 mm Hg; mean pulmonary artery pressure, 60 +/- 18 mm Hg; cardiac index, 2.3 +/- 0.9 L/min X m2; and pulmonary vascular resistance index, 26 +/- 14 mm Hg/L/min X m2 for the group. Although no deaths or sustained morbid events occurred during the diagnostic evaluation of the patients, the typically long interval from initial symptoms to diagnosis emphasizes the need to develop strategies to make the diagnosis earlier.

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Journal

Journal ID (publisher-id): 978-0-387-68792-6

Journal ID (doi): 10.1007/978-0-387-68792-6

Journal ID (nlm-ta): Dail and Hammar’s Pulmonary Pathology

Title: Dail and Hammar’s Pulmonary Pathology

Subtitle: Volume I: Nonneoplastic Lung Disease

ISBN (Print): 978-0-387-98395-0

ISBN (Electronic): 978-0-387-68792-6

Publication date (Print): 2008

Pages: 1032-1087

Affiliations

[1 ]GRID grid.67105.35, ISNI 0000000121643847, Department of Pathology, , Case Western Reserve University School of Medicine, ; Cleveland, OH USA

[2 ]GRID grid.411931.f, ISNI 0000000100354528, Department of Pathology, , MetroHealth Medical Center, ; Cleveland, OH USA

[3 ]GRID grid.5386.8, ISNI 000000041936877X, Department of Pathology, , Weill Medical College of Cornell University, ; New York, NY

[4 ]GRID grid.63368.38, ISNI 0000000404450041, Pulmonary Pathology, Department of Pathology, , The Methodist Hospital, ; Houston, TX USA

[5 ]GRID grid.239578.2, ISNI 0000000106754725, Pulmonary Pathology, Department of Anatomic Pathology, , The Cleveland Clinic Foundation, ; Cleveland, OH USA

[6 ]GRID grid.168645.8, ISNI 0000000107420364, Department of Pathology, , University of Massachusetts Medical School, ; Worcester, MA USA

[7 ]GRID grid.241116.1, ISNI 0000000107903411, Department of Pathology, , University of Colorado Health Sciences Center, ; Denver, CO 80262 USA

[8 ]GRID grid.266471.0, ISNI 0000000404133513, Department of Pathology Case Western Reserve, , University School of Medicine, ; Cleveland, OH 44109 USA

[9 ]GRID grid.411931.f, ISNI 0000000100354528, Department of Pathology, , MetroHealth Medical Center, ; Cleveland, OH 44109 USA

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Publisher ID: 28

DOI: 10.1007/978-0-387-68792-6_28

PMC ID: 7120700

SO-VID: dc203d76-eb82-4c44-aac4-21a5452e95b4

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This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

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Keywords: pulmonary artery,pulmonary hypertension,pulmonary arterial hypertension,bronchial artery,primary pulmonary hypertension

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Keywords: pulmonary artery, pulmonary hypertension, pulmonary arterial hypertension, bronchial artery, primary pulmonary hypertension

Pulmonary Vascular Disease

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Most cited references 391

Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.

A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension.

Primary pulmonary hypertension. A national prospective study.

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