Causes of pulmonary hypertension in the elderly

Abstract

Background: Pulmonary hypertension (PH) is common in elderly patients, but a detailed analysis of the causes of PH in the elderly has not been performed. We hypothesized that pulmonary arterial hypertension (PAH) is rare in elderly patients and sought to describe the characteristics of these patients at a large referral center.

Methods: Clinical and hemodynamic data were collected on consecutive patients ≥ 65 years of age referred for evaluation of PH. The subtype of PH was determined after standard evaluation using the World Health Organization (WHO) classification. Patients with PH not meeting criteria for PAH with "out-of-proportion" PH related to group 2 or group 3 disease were classified as "other/mixed PH." A model using age, presence of connective tissue disease, and left atrial size was developed to predict the probability of PAH diagnosis.

Results: Two hundred forty-six elderly patients were evaluated (mean age, 72.9 ± 5.5 years, 78% women); 36 had PAH (15%). Idiopathic PAH was rare (four patients, 1.6%). WHO group 2 PH was the most frequent diagnosis (n = 70, 28% of cohort); mixed/other PH (n = 43, 17%) and WHO group 3 PH (n = 34, 14%) were also common diagnoses. Connective tissue disease strongly predicted PAH diagnosis (OR, 27.2; 95% CI, 9.5-77.6).

Conclusions: PAH is an uncommon cause of PH in elderly patients, most frequently associated with connective tissue disease. WHO group 2 PH and mixed disease are common, highlighting a need for careful phenotyping of elderly patients with PH prior to initiating PAH therapy.

Figure 1

– Causes of PH. This chart shows the final diagnoses of PH in this elderly cohort after complete clinical evaluation. PAH = pulmonary arterial hypertension; PH = pulmonary hypertension.

Figure 2

– Association of variables with PAH diagnosis. Plot of adjusted and unadjusted ORs for our logistic regression model predicting a diagnosis of PAH. Probability of WHO group 1 PH = 1/[1 + exp(−Xβ), where Xβ = 6.42 − 0.0995(age) + 3.3(CTD) − 0.495(left atrial size). CTD = 1 if CTD is present, 0 if CTD is not present. CTD = connective tissue disease; WHO = World Health Organization. See Figure 1 legend for expansion of other abbreviation.

Figure 3

– Receiver operating characteristics curve for full model predicting WHO group 1 PH (PAH). AUC = area under the curve. See Figure 1 and 2 legends for expansion of other abbreviations.

Figure 4

– Receiver operating characteristics curve for the full model and individual variables predicting WHO group 1 PH (PAH). LA = left atrium. See Figure 1 and 2 legends for expansion of other abbreviations.