Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

Tanabe, Nobuhiro; Taniguchi, Hiroyuki; Tsujino, Ichizo; Sakamaki, Fumio; Emoto, Noriaki; Kimura, Hiroshi; Takamura, Kei; Hanaoka, Masayuki; Nishimura, Masaharu

doi:10.1111/resp.12530

Cited by 47 publications

(36 citation statements)

References 19 publications

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“…It is unclear whether PH-targeted therapies affect the outcomes of patients with PH-IIP as suggested by preliminary data from Japan [ 19 ]. Due to the lack of a control group, our data do not allow us to draw such conclusions.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

et al. 2015

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BackgroundPulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.MethodsWe retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).ResultsCompared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.ConclusionsPatients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.Trial Registrationclinicaltrials.gov NCT01347216

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Section: Discussionmentioning

confidence: 99%

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

et al. 2015

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“…Generally speaking, severe PH was defined by a mPAP ≥35 mm Hg. [ 14 , 15 ] However, an mPAP >45 mm Hg or even higher can lead to poor prognosis in patients with PH, and clinical combination therapy and/or surgical lung transplantation surgery is more likely to be used for the treatment. [ 16 – 18 ]…”

Section: Discussionmentioning

confidence: 99%

Clinical study of right ventricular longitudinal strain for assessing right ventricular dysfunction and hemodynamics in pulmonary hypertension

Wang²,

Ye³

et al. 2016

Medicine

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This study aimed to appraise the application of right ventricular longitudinal strain for assessing right ventricular dysfunction and severe hemodynamic changes in pulmonary hypertension. The study included 53 patients clinically diagnosed with PH. Tissue Doppler–derived tricuspid lateral annular systolic velocity (s’), early diastolic peak velocity (e’), late diastolic peak velocity (a’), tricuspid annular plane systolic excursion (TAPSE), RV index of myocardial performance (RIMP), and right ventricular fractional area change (FAC) were determined. The STI parameter was RV free wall longitudinal peak systolic strain (RV LPSS). The patients were assigned into two groups based on a RV LPSS value of − 19%.RV LPSS, s’, TAPSE, RIMP, FAC, a’ and e’/a’ showed significant differences. PH patients with an RV LPSS≥ −19% exhibited a lower RV function (P < 0.05). RV LPSS was negatively correlated with TAPSE (r = −0.326, P < 0.05) and FAC (r = −0.495, P < 0.001) and positively correlated with RIMP (r = 0.508, P < 0.001). The optimal cut-off value of RV LPSS to reveal an mPAP ≥ 45 mmHg defined based on the receiver operating characteristic curve analysis was − 19.26% with a sensitivity of 83.9% and a specificity of 73.4%.Distinguishing the degree of RV dysfunction by 2D-STI may help physicians to determine the state of cardiac function and degree of PH in patients and offer a basis for subsequent clinical diagnosis and therapy. Our study demonstrates the superiority of RV LPSS for uncovering severe PH over the traditional echocardiographic parameters.

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“…21 Conversely, some basic and observational data suggested that beneficial effects existed for PAH-approved drugs, particularly sildenafil, in this group. 22,23 Consequently, the use of PAH-approved drugs in patients with lung-PH has been allowed in Japan since 2009, but only when clinical data suggest that severe PH cannot be explained by the lung disease(s) alone. This unique situation in Japan has enabled further examination of the effects of these PAH drugs on the CMR-derived RV morphology and function in patients with severe lung-PH under the current Japanese medical insurance system.…”

Section: Discussionmentioning

confidence: 99%

The Effects of Pulmonary Vasodilating Agents on Right Ventricular Parameters in Severe Group 3 Pulmonary Hypertension: A Pilot Study

et al. 2016

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Pulmonary arterial hypertension (PAH)-approved vasodilators improve right ventricular (RV) function in patients with PAH. However, whether PAH-approved drugs ameliorate RV morphology and function in lung disease-associated pulmonary hypertension (lung-PH) remains unclear. We aimed to prospectively evaluate the changes in RV volume and ejection fraction (RVEF) in 14 consecutive severe lung-PH patients treated with PAH-approved vasodilators. Severe lung-PH was defined as a mean pulmonary arterial pressure (MPAP) of ≥35 mmHg or an MPAP of ≥25 mmHg with a cardiac index (L/min/m 2 ) of <2. Right heart catheterization and cardiac magnetic resonance (CMR) imaging were performed at baseline and at 3 months after starting sildenafil with or without other PAH-approved drugs. Follow-up was conducted at 3 months in 11 participants; compared with baseline values, MPAP and pulmonary vascular resistance (PVR) decreased by 18% and 37%, respectively. Baseline CMR imaging revealed an elevated RV end-diastolic volume index (RVEDVI; mL/m 2 ) of 117.5 ± 35.9 and a below-average RVEF of 25.2% ± 7.2%; after 3 months, RVEDVI decreased by 23.7% (P = 0.0061) and RVEF increased by 32.9% (P = 0.0165). Among the 11 patients, 3 were thought to be a stable and homogenous subset in terms of background lung disease and medical management administered. These 3 patients exhibited similar ameliorations in PVR and RVEF, compared with the other 8 patients. PAH-approved drug treatment may improve RV dilatation and systolic function among patients with severe lung-PH. This study was approved by University Hospital Medical Information Network Clinical Trials Registry (UMIN-CTR) on September 1, 2013 (UMIN000011541).

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Multi‐institutional retrospective cohort study of patients with severe pulmonary hypertension associated with respiratory diseases

Cited by 47 publications

References 19 publications

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias

Clinical study of right ventricular longitudinal strain for assessing right ventricular dysfunction and hemodynamics in pulmonary hypertension

The Effects of Pulmonary Vasodilating Agents on Right Ventricular Parameters in Severe Group 3 Pulmonary Hypertension: A Pilot Study

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