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Brenot Memorial Symposium on the Pathogenesis of Primary Pulmonary HypertensionPrimary Pulmonary Hypertension: Insights Into Pathogenesis From Epidemiology
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
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2013, American Journal of CardiologyCitation Excerpt :The incidence of PAH associated with scleroderma is estimated at 30 to 286 cases/1 million population.6 PAH occurs in both genders and in all age groups; although after puberty, PAH is seen approximately twice as often in females.7 Untreated patients with PAH face an estimated mean survival of 2.8 years, with a 1-, 3-, and 5-year survival rate of 68%, 48%, and 34%, respectively.8
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2010, Clinical Pediatric Emergency MedicineCitation Excerpt :Combined heart and lung transplantation may be performed if there is concern about unrecoverable right ventricular systolic or left ventricular diastolic dysfunction, and this is the reason that the patient in this case study is being evaluated for heart and lung transplantation. The limits for unrecoverable cardiac function in pulmonary hypertension have not been well established, so guidelines for either lung transplantation or cardiac transplantation are institution dependent.8,9 Pulmonary hypertension is an unusual but life-threatening cause of chest pain in children that may present to the ED.