Chest
Volume 114, Issue 3, Supplement, September 1998, Pages 184S-194S
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Brenot Memorial Symposium on the Pathogenesis of Primary Pulmonary Hypertension
Primary Pulmonary Hypertension: Insights Into Pathogenesis From Epidemiology

https://doi.org/10.1378/chest.114.3_Supplement.184SGet rights and content

Primary pulmonary hypertension (PPH) is a rare disease that affects young people predominantly of female gender. Early epidemiologic studies have shown that the diagnosis is usually made 1 to 2 years after symptoms onset, and the mean survival is reduced to 2 to 3 years thereafter. New insights into the pathogenesis of PPH by epidemiologic studies may be obtained through the utilization of informatic technologies coupled to a clear definition of the disease. Early stages of precapillary pulmonary hypertension could be identified through screening tests like echocardiography in populations with higher incidence, such as familial PPH and the conditions associated with pulmonary hypertension. These latter conditions are hemodynamically and pathologically similar to the primary form, and they can give insight into several possible aspects of the pathogenesis of PPH. Prospective registries are very useful in coordinating the collection of epidemiologic data, and new technologies, such as informatics, may improve the management and the continuous updating of the databases.

Section snippets

HEMODYNAMIC, CLINICAL, AND PATHOLOGIC DEFINITIONS

To be effective, epidemiologic studies need precise definition(s) of the disease(s) to be analyzed in order to obtain a uniform group(s) of patients with regard to the established variables. In fact, depending on definitions used, parameters such as annual incidence, prevalence, clinical picture, treatment, and prognosis may vary widely.

Primary pulmonary hypertension was defined by an expert Committee of the World Health Organization in 1973 as “pulmonary hypertension of unexplained (unknown)

EARLY DIAGNOSIS

One of the requisites of good epidemiologic studies is the possibility of identifying and analyzing any stage of a disease. The clinical identification of the early stages may be one of the opportunities we have to study the early pathophysiologic events and to better understand the initiating processes. Unfortunately, patients with PPH usually become symptomatic when the pathologic and pathophysiologic processes are fully expressed and few, if any, traces of the original triggering mechanisms

Anorectic Agents

Among the patients with associated precapillary pulmonary hypertension, those who have taken appetite suppressant drugs are generally considered to have PPH; for example, they were included in the National Institutes of Health registry on PPH.8 The clinical presentation, hemodynamics, prognosis, and the histopathology of the lung vessels of these patients are indistinguishable from those with PPH,17, 24 and only in rare cases has regression of the pulmonary hypertension been observed with

REGISTRIES OF PPH CASES

The classical procedure to collect data on rare diseases with short spontaneous survival is to implement prospective registries with standardized protocols in order to obtain a significant and uniform patient population linking the experiences of multiple centers. A typical example is the National Institutes of Health registry on PPH8 that enrolled 194 patients between July 1, 1981, and December 31, 1985, who were followed through August 8, 1988.51 This registry has allowed the clarification of

CONCLUSIONS

New insights into the pathogenesis of PPH by epidemiologic studies may be obtained through the utilization of informatic technologies coupled with a clear definition of the disease. Early stages of precapillary pulmonary hypertension could be identified through screening tests like echocardiography in populations with higher incidence, such as familial PPH and the conditions associated with pulmonary hypertension. These latter conditions are hemodynamically and pathologically similar to the

REFERENCES (56)

  • KimKK et al.

    Membrano-proliferative glomerulonephritis and plexogenic pulmonary arteriopathy in a homosexual man with acquired immunodeficiency syndrome

    Hum Pathol

    (1987)
  • ChenW et al.

    Detection of a patent foramen ovale by contrast transesophageal echocardiography

    Chest

    (1992)
  • WoodP

    Pulmonary hypertension

    Br Med Bull

    (1952)
  • WoodP

    Primary pulmonary hypertension, with special reference to the vasoconstrictive factor

    Br Heart J

    (1958)
  • RubinLJ

    Pathology and pathophysiology of primary pulmonary hypertension

    Am J Cardiol

    (1995)
  • The international primary pulmonary hypertension study (IPPHS)

    Chest

    (1994)
  • RichS et al.

    Primary pulmonary hypertension: a national prospective study

    Ann Intern Med

    (1987)
  • SimonsonJ et al.

    Pulmonary hypertension in systemic lupus erythematosus

    J Rheumatol

    (1989)
  • AlpertMA et al.

    Cardiovascular manifestations of mixed connective tissue disease in adults

    Circulation

    (1983)
  • McDonnelPJ et al.

    Primary pulmonary hypertension and cirrhosis: are they related?

    Am Rev Respir Dis

    (1983)
  • RubinLJ

    Primary pulmonary hypertension

    N Engl J Med

    (1997)
  • AbenhaimL et al.

    Appetite suppressant drugs and the risk of primary pulmonary hypertension

    N Engl J Med

    (1996)
  • PietraGG et al.

    Histopathology of primary pulmonary hypertension: a qualitative and quantitative study of pulmonary blood vessels from 58 patients in the National Heart, Lung, and Blood Institute primary pulmonary hypertension registry

    Circulation

    (1989)
  • LoydJE et al.

    Heterogeneity of pathological lesions in familial primary pulmonary hypertension

    Am Rev Respir Dis

    (1988)
  • PalevskyHI et al.

    Primary pulmonary hypertension: vascular structure, morphometry, and responsiveness to vasodilator agents

    Circulation

    (1989)
  • Barst RJ, Loyd JE. Genetics and immunogenetic aspects of primary pulmonary hypertension. Chest 1998;...
  • NicholsCW et al.

    Localization of the gene for familial primary pulmonary hypertension to chromosome 2q31-32 [letter]

    Nat Genet

    (1997)
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