Pulmonary Hypertension in Patients With Idiopathic Pulmonary Fibrosis

Nadrous, Hassan F.; Pellikka, Patricia A.; Krowka, Michael J.; Swanson, Karen L.; Chaowalit, Nithima; Decker, Paul A.; Ryu, Jay H.

doi:10.1378/chest.128.4.2393

Cited by 374 publications

(264 citation statements)

References 39 publications

Supporting

Mentioning

246

Contrasting

Unclassified

Order By: Relevance

“…Despite its critical role, the epidemiology of PH in IPF has not been extensively studied, and the reported incidence is wide, ranging from 32 to 84% [10,11]. Several factors account for this wide range in the prevalence of PH in IPF.…”

Section: Definition and Epidemiologymentioning

confidence: 99%

“…Pulmonary hypertension (PH) is recognized as a severe complication of IPF [6,9]. The development of PH during the course of the disease has a negative impact on the functional status and quality of life of IPF patients and is associated with poor survival [10,11,12,13]. The approval of new drugs for the treatment of pulmonary arterial hypertension (PAH) has renewed interest in IPF and associated PH and raised the possibility of therapeutic intervention for these patients.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

2011

View full text Add to dashboard Cite

Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal disease with a poor prognosis. Pulmonary hypertension (PH) often complicates the course of IPF and may even be found in patients with preserved lung function. Possible pathogenetic mechanisms of PH in IPF include vascular destruction, pulmonary hypoxic vasoconstriction and vascular remodeling due to overexpression of cytokines and growth factors. PH in IPF patients is associated with decreased exercise capacity and a worse prognosis. Due to its prognostic significance, it seems important to investigate for PH in these patients. As the symptoms of PH in IPF are nonspecific, the development of PH in a patient with known IPF can be easily overlooked. Noninvasive methods provide clues for the diagnosis, but their sensitivity is limited. Doppler echocardiography is a useful tool for the detection of PH which also provides additional information regarding associated cardiac abnormalities. However, right heart catheterization remains the gold standard diagnostic test. Therapeutic options for PH in IPF are limited. Long-term oxygen administration for the correction of hypoxemia should be recommended. The availability of new pharmacological agents in the treatment of PH has raised the possibility of therapy in patients with IPF and associated PH. Whether these PH-targeted therapies may be of benefit in this patient group, in terms of improving functional outcomes and survival, remains uncertain.

show abstract

Section: Definition and Epidemiologymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

2011

View full text Add to dashboard Cite

show abstract

“…[2][3][4] Pulmonary Hypertension (PH) is an important comorbidity of advanced IPF that has a significant negative impact on survival. [5][6][7] Variable prevalence (range, 32%-84%) of PH has been reported. 5,6 Combined Pulmonary Fibrosis and Emphysema (CPFE) has been proposed as a new phenotype of pulmonary fibrosis, defined by the presence of emphysema of upper lobe and fibrosis of the lower lobe.…”

Section: Introductionmentioning

confidence: 99%

“…[5][6][7] Variable prevalence (range, 32%-84%) of PH has been reported. 5,6 Combined Pulmonary Fibrosis and Emphysema (CPFE) has been proposed as a new phenotype of pulmonary fibrosis, defined by the presence of emphysema of upper lobe and fibrosis of the lower lobe. 8 PH involvement is more frequent in IPF with emphysema than in IPF without emphysema, and PH is believed to be a poor prognostic factor of CPFE.…”

Section: Introductionmentioning

confidence: 99%

“…9,10 Although echocardiography is inferior to RHC in accuracy, 11,12 several reports have suggested that it can provide a useful prognostic value of IPF. 6,13 However, most previous studies on the relationship between PH and IPF were cross-sectional analyses. Therefore, it is unknown whether clinical parameters, including Pulmonary Arterial Pressure (PAP), change serially during the natural course of IPF.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Serial Measurements of Tricuspid Regurgitation Pressure Gradient by Echocardiography Predict Prognosis In Idiopathic Pulmonary Fibrosis

Kitamura

Otsuka²,

Yamada³

et al. 2016

Pulm Res Respir Med Open J

View full text Add to dashboard Cite

Background and Objectives: Idiopathic Pulmonary Fibrosis (IPF), especially with emphysema, reportedly involved with Pulmonary Hypertension (PH). However, it is not elucidated whether pulmonary arterial pressure changes serially during the course and influence on prognosis in IPF. We examined whether serial measurements of Tricuspid Regurgitation Pressure Gradient (TRPG) by echocardiography were meaningful predictors of IPF patient survival. Methods: We retrospectively investigated 83 IPF patients. The echocardiographic TRPG cutoff was set at 30 mmHg, and the subjects were divided into two groups: high TRPG and normal TRPG. We also evaluated the relationship between serial TRPG changes during follow-up. Results: A total of 28 patients were included in the high TRPG group. The high TRPG group showed significantly lower %FVC and %DLco, higher A-aDO 2 , shorter 6-minute walk test distance, and more frequent emphysema than the normal TRPG group. The high TRPG group also had poorer survival than the normal TRPG group. A multivariate Cox proportional hazard model demonstrated that TRPG, %FVC, and A-aDO 2 significantly affected patient survival. Thirty-six patients underwent echocardiography twice. At the time of the second echocardiography, 7 patients with normal TRPG at baseline (n=22) increased to a TRPG of more than 30 mmHg. These patients had significantly showed poorer survival. Conclusions: TRPG is an independent prognostic factor in IPF. Emphysema involvement, decreased DLco, and decreased FVC were associated with an increase in TRPG. Serial measurements of TRPG are recommended for the early detection of PH and predict prognosis in IPF patients.

show abstract