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Review
. 2012 Jul;8(3):413-25.
doi: 10.1016/j.hfc.2012.04.001.

Pulmonary arterial hypertension in connective tissue diseases

Stephen C Mathai  1 , Paul M Hassoun
Affiliations
Review

Pulmonary arterial hypertension in connective tissue diseases

Stephen C Mathai et al. Heart Fail Clin. 2012 Jul.

Abstract

Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs). Approximately 10% of patients with systemic sclerosis develop PAH, the prevalence being much lower in other CTDs. However, PAH is an important contributor to morbidity and mortality in all forms of CTD. Despite similarities in presentation, hemodynamic perturbations, and pathogenesis, patients with CTD-associated PAH (CTD-PAH) usually have a poorer response to PAH-specific medications and poorer prognosis than patients with idiopathic PAH (IPAH). Select patients with CTD-PAH may be candidates for lung transplantation, but results are less favorable than for IPAH because of comorbidities and complications specifically associated with CTD.

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Figures

Figure 1
Figure 1. Algorithm for Detection of PAH in Patients with Systemic Sclerosis
Algorithm showing routine clinical tests in patients with systemic sclerosis aimed at early detection of pulmonary arterial hypertension or other causes of cardiac dysfunction (e.g., left ventricular dysfunction) Abbreviations: PFTs-Pulmonary function tests; DLCO-single breath diffusing capacity to carbon monoxide; FVC-forced vital capacity; RV-right ventricle; TRV-tricuspid regurgitation jet; RHC-right heart catheterization.
Figure 2
Figure 2. Survival in SSc-PAH compared to SLE-PAH
Survival from diagnosis for patients with PAH associated with SSc compared to PAH associated with SLE. From Condliffe R et al. Am J Resp Crit Care Med 2009 (permission pending)
See this image and copyright information in PMC

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