Patient Registry for Primary Pulmonary Hypertension
(PPH Registry)
Objectives:
Establish a patient registry to evaluate the natural
history, etiology, pathogenesis and treatment of primary pulmonary
hypertension. Specific aims included the characterization of the demographic,
medical history, family history, physical and laboratory findings of patients
at time of diagnosis, and to characterize the survival duration of patients by
traits evaluated at diagnosis and by medical interventions.
Background:
There are several known causes of pulmonary
hypertension: chronic obstructive pulmonary disease, congenital heart disease,
mitral stenosis, left ventricular dysfunction and recurrent pulmonary emboli.
Primary Pulmonary Hypertension is a disorder of unknown etiology which is
diagnosed only after the known causes of pulmonary hypertension have been
eliminated. Prior to the PPH registry, little was known regarding the
epidemiology, etiology, natural history or ultimate survival among patients
with PPH. In 1973, the WHO met to review the current state of knowledge on PPH
and proposed the establishment of a multicenter collaborative study. The NHLBI
PPH registry enrolled patients in the registry from 1981 to 1985.
Subjects:
Patients were enrolled into the registry from 32
medical centers throughout the US. Pulmonary hypertension was defined as a mean
pulmonary arterial pressure of >25 mmHg at rest or 30 mmHg with exercise at
catheterization. The diagnosis of primary pulmonary hypertension was only
accepted after the following secondary causes of pulmonary hypertension had
been exlcuded: pulmonary hypertension within the first year of life, congenital
abnormalities of the heart, lungs, or diaphragm, pulmonary thromboembolic
disease, diagnosis of sickle cell anemia, history of intravenous drug abuse,
obstructive lung disease, interstitial lung disease, arterial hypoxemia,
collagen vascular disease, parasitic disease affecting the lungs, pulmonary
artery or valve stenosis, or pulmonary venous hypertension. Baseline and
follow-up data collected on patients include demographic characteristics, chest
radiograph, pulmonary function tests, lung perfusion scan or pulmonary
angiogram, intracardiac left-to-right shunt, pulmonary hemodynamics, as well as
a history, physical findings and other laboratory measurements. Patients were
followed for approximately 5 years.
Conclusions:
There were 1.7 females for each male in the registry,
and females tended to present with more severe symptoms. The mean time from
onset of symptoms to diagnosis was 2 years. Right ventricular hypertrophy was
found in 87% of patients and right atrial pressure was elevated in 72% of
patients. The estimated median survival was 2.8 years with single year survival
rates of: 1 year, 68%; 3 years, 48%; and 5 years, 34%. (Ann Intern Med, 1987;
107:216-23, Ann Intern Med, 1991; 115:343-49).
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Study Documentation |
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Data Distribution Agreement |
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