REVIEWPulmonary Hypertension in Patients With Interstitial Lung Diseases
Section snippets
PREVALENCE
The overall prevalence of PH in ILD is not fully known and varies among the ILDs. In a prospective observational study, Leuchte et al19 reported that 31.8% of 88 consecutive patients with various ILDs undergoing right heart catheterization had significant PH (defined as mean PAP >35 mm Hg). Data are available regarding the prevalence of PH in several specific ILDs (Table 1). Perhaps the best known association is with PLCH. Pulmonary Langerhans cell histiocytosis (also known as histiocytosis X,
PATHOGENESIS
The pathogenesis of PH in ILD is incompletely understood and may involve multiple diverse mechanisms, some of which are peculiar to specific forms of ILD, as discussed subsequently. In advanced lung disease, PH generally results from chronic hypoxic pulmonary vasoconstriction and vascular remodeling.4, 48, 49 This vascular remodeling involves all layers of the pulmonary arterial wall and includes intimal thickening and medial hypertrophy. However, PH can be seen in the absence of hypoxemia in
DIAGNOSIS
Because exertional dyspnea is the most common symptom associated with both PH and ILD, the presence of coexisting PH could easily be overlooked in patients with ILD. Furthermore, the development of both PH and ILD is typically insidious and associated with minimal symptoms and signs in earlier stages. Advanced stages of PH are easier to recognize and are associated with symptoms of leg swelling, abdominal bloating and distention, anorexia, plethora, and profound fatigue as right ventricular
TREATMENT
Management of PH in patients with ILD is guided by the clinical context, mechanism underlying PH, severity of PH, and results of vasoreactivity testing performed during right heart catheterization. To a certain extent, PH in ILD is treatable whether one is addressing the PH itself, the underlying ILD, or both (Table 2). Some of the vasomodulating agents used in the treatment of pulmonary arterial hypertension, eg, bosentan, may have beneficial effects on the underlying parenchymal lung disease,
PROGNOSIS
Pulmonary hypertension causes disability and premature death in patients with or without underlying lung disease. Prognosis in patients with ILD and PH is affected by both PH and underlying ILD. The presence of PH in patients with ILD correlates with worse prognosis.11, 12, 13, 14, 16, 17, 114 For example, an increasing PH correlated with worse survival in patients with IPF; those with systolic PAP greater than 50 mm Hg had a median survival of less than 1 year.14 Whether vasomodulator therapy
CONCLUSION
Pulmonary hypertension is an underrecognized complication in patients with ILDs and may arise through multiple mechanisms. The prevalence and clinical implications of PH in patients with ILDs require further clarification. Although some themes may be similar in the pathogenesis of PH in patients with ILDs, there are likely mechanisms peculiar to specific ILDs, and these need to be studied. The presence of PH adds to the symptoms, functional impairment, morbidity, and mortality in these
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Cited by (86)
Pulmonary hypertension due to silicosis and right upper pulmonary artery occlusion with bronchial anthracofibrosis
2021, Respiratory Medicine Case ReportsCitation Excerpt :It is usually not associated with artery occlusion. On the other hand, pulmonary hypertension (PH) can occur with pneumoconiosis, which is associated with interstitial lung disease and hypoxic vasoconstriction [4]. Here, we report a rare case of PH due to silicosis and right upper pulmonary artery occlusion with bronchial anthracofibrosis.
Acute Respiratory Failure in Interstitial Lung Disease Complicated by Pulmonary Hypertension
2020, Respiratory MedicineCitation Excerpt :Shorr et al. (2005) reported ~75% of pre-transplant patients with advanced sarcoidosis had PH on RHC [17]. The reported incidence of PH in systemic sclerosis (SSc) patients is up to 45% [22,26] and moderate to severe PH is reported in 17.9% of SSc patients [19]. The prevalence of PH in SSc-ILD has been estimated at 12% and is associated with increased risk of overall mortality [27].
Increasing Opportunity for Lung Transplant in Interstitial Lung Disease With Pulmonary Hypertension
2018, Annals of Thoracic SurgeryCitation Excerpt :The use of ECMO as BTT is increasing as outcomes for its use improve for a wide variety of conditions [6–8]. A significant proportion of patients with ILD have associated PH, and the PH in these patients is an independent risk factor for pretransplant mortality [10, 11]. There is a notably different response to mechanical circulatory support in patients with end-stage ILD-PH, which may be in part due to the presence of right ventricular strain and reduced cardiac output.
Pulmonary hypertension
2018, Cardiac Intensive Care
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Dr Krowka has received grant support from CoTherix, Inc. Dr McGoon is a consultant for Medtronic, Inc, a steering committee member for CoTherix, Inc, and Myogen, a data safety and monitoring committee member for United Therapeutics, and a central event committee member for Actelion; he has received grant/research support from Medtronic, Inc, CoTherix, Inc, and Myogen.