Screening

Screening for pulmonary hypertension

Screening appropriate patient populations may lead to the early identification of pulmonary hypertension in asymptomatic or minimally symptomatic individuals, or in symptomatic patients in whom the diagnosis was not previously suspected. This could allow early initiation of treatments at a time when dynamic or reversible pathogenic mechanisms are present, increasing the likelihood of a successful treatment outcome. Screening tests should be noninvasive and low risk, if possible, and have a relatively high sensitivity and specificity for detecting pulmonary hypertension.

Screening may be appropriate in groups of patients at increased risk of developing pulmonary hypertension. In such instances, general screening should always begin with a thorough clinical interview to elicit symptoms consistent with pulmonary hypertension, and a thorough physical examination to elicit physical findings consistent with the diagnosis. When the history and physical examination are inconclusive, further diagnostic testing may be appropriate.

The following recommendations are made regarding specific subgroups of patients. A transthoracic echocardiogram is currently the preferred screening test for the presence of pulmonary hypertension.

1. Connective Tissue Diseases
The Scleroderma Spectrum of Diseases:
Because of the high prevalence of pulmonary hypertension in these patients, as well as the availability of effective treatments, a transthoracic echocardiogram is recommended to be performed annually in patients with or without symptoms of pulmonary hypertension.

Systemic Lupus, Rheumatoid Arthritis, and Other Connective Tissue Diseases:
Because of the low prevalence of pulmonary hypertension, and the lack of established effective treatment, a transthoracic echocardiogram is recommended only if patients have symptoms suggestive of pulmonary hypertension.

2. Families of Documented PPH
A detailed family history should be taken at the time the diagnosis of PPH is made in the proband. It is reasonable to consider a transthoracic echocardiogram in the first degree relatives at the time of diagnosis, at any time symptoms consistent with pulmonary hypertension arise, or every three to five years in asymptomatic individuals. In addition, relatives should be made aware of symptoms consistent with pulmonary hypertension. The basis for these recommendations is the greater prevalence of familial PPH than previously reported, and the availability of effective treatments. In addition, screening asymptomatic family members will help gather additionalinformation about the prevalence of familial PPH and the effectiveness of early intervention.

3. Liver Disease/Portal Hypertension
Because pulmonary hypertension in these patients renders them at very high risk for liver transplantation, and because there is effectivetreatment available, a transthoracic echocardiogram should be performed in all patients when they are evaluated for liver transplantation.

4. HIV Infection
Because of the low prevalence of pulmonaryhypertension in this subgroup, a transthoracic echocardiogram is recommended only in subjects who are HIV positive if they have symptoms consistent with pulmonaryhypertension.

5. Patients with a History of Intravenous Drug
Use Because the prevalence of pulmonary hypertension is uncertain in this subgroup, a transthoracic echocardiogram is recommended only in those patients who have symptoms consistent with pulmonary hypertension.

6. Patients with a History of Appetite-Suppressant Drug Use
Because of the low prevalence of pulmonary hypertension in this subgroup, a transthoracic echocardiogram is recommended only in patients who have symptoms consistent with pulmonary hypertension.