Treprostinil to Reverse Pulmonary Hypertension Associated With Idiopathic Pulmonary Fibrosis as a Bridge to Single-Lung Transplantation
Rajeev SaggarShelley ShapiroDavid J. RossMichael C. FishbeinDavid A. ZismanJoseph P. LynchSam WeigtA. ArdehaliJohn A. BelperioRajan Saggar
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(1) Background: This paper aims to assess temporal trends (2016-2020) in incidence, patient's characteristics, complications, length of hospital stay (LOHS) and in-hospital mortality (IHM) among patients with and without idiopathic pulmonary fibrosis (IPF) undergoing lung transplantation (LTx). We also analyse the effect of the COVID-19 pandemic on LTx in these populations. (2) Methods: A retrospective, population-based observational study was conducted using the Spanish National Hospital Discharge Database. Multivariable adjustment was conducted with logistic regression to analyse the IHM. (3) Results: We identified 1777 admissions for LTx during the study period, of which 573 (32.2%) were performed in patients with IPF. The number of hospital admissions for LTx rose from 2016 to 2020, both in patients with and without IPF, but a marked reduction was observed from year 2019 to year 2020. Over time, the proportion of single LTx decreased and bilateral LTx increased significantly in both groups. The incidence of LTx complications increased significantly over time along with the increase in the incidence of IPF. No significant differences in the incidence of complications or in the IHM between patients with and without IPF were found. Suffering any complication of the LTx and pulmonary hypertension were conditions positively associated with IHM in patients with and without IPF. The IHM remained stable from 2016 to 2020 in both study populations and was not affected by the COVID pandemic. (4) Conclusions: Patients with IPF account for almost a third of all lung transplants. The number of LTx increased over time in patients with and without IPF, but a marked reduction was observed from 2019 to 2020. Although the proportion of LTx complications increased significantly over time in both groups, the IHM did not change. IPF was not associated with increased complications or IHM after LTx.
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Pulmonary hypertension is a serious but often overlooked complication in collagen vascular disease. The understanding of the development of pulmonary hypertension has increased substantially during the last years. Abnormal proliferation of pulmonary vascular cells is now being regarded as a predominant process leading to pulmonary vascular obliteration. Medical therapy focuses on prostacyclin treatment, which has been shown to improve exercise capacity and haemodynamic variables in patients with several collagen vascular diseases and pulmonary arterial hypertension. Continuous intravenous prostacyclin remains the standard treatment of associated pulmonary hypertension but less invasive alternatives such as subcutaneous treprostinil, oral beraprost or aerosolized iloprost, as well as, novel substances such as endothelin receptor antagonists may be appropriate for selected patients.
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Pathophysiology of hypertension
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Idiopathic pulmonary fibrosis (IPF) is a diffuse progressive parenchymal disease. Patients with IPF have a poor prognosis and frequently suffer from pulmonary hypertension (PH) complications, which are associated to significantly worse outcomes. In IPF, the pathogenesis of PH varies and has not been completely understood yet, since it may arise from multiple factors. In these patients, PH is associated with reduced exercise capacity and lower survival rate. Due to its prognostic significance, it would be useful to conduct further research into PH in IPF patients. Though right heart catheterization continues to be the gold standard diagnostic test, the first steps to properly diagnose PH are clinical suspicion (based on increased shortness of breath and reduced diffusing capacity) and echocardiography. The options to treat PH in this population are limited. What is more, to date, there are no approved therapies specifically aimed at PH in IPF patients. Keywords: Doppler echocardiogram, idiopathic pulmonary fibrosis, pulmonary arterial pressure, pulmonary hypertension, right heart catheterization.
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Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that results in progressive respiratory failure and death. Antifibrotic therapy has been shown to slow disease progression [1, 2]. For many patients [3], lung transplantation is the only intervention recognised to provide significant survival benefit [4]. IPF commonly affects older patients, with a median age at diagnosis of 66 years old [5]. Without transplantation, the median survival from diagnosis is 2–4 years [5]. However, registry data indicates impaired outcomes following lung transplantation for older patients 6]. These data have prompted many centres in Europe to avoid offering transplantation to older IPF patients. This study demonstrates a clinically meaningful survival benefit of lung transplantation for IPF patients aged ≥65 years
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Pulmonary hypertension (PH) causes significant morbidity and mortality in children due to right ventricular (RV) failure. We sought to determine the effect of prostacyclin analogues on RV function assessed by echocardiography in children with PH. We conducted a retrospective cohort study of children with PH treated with a prostacyclin analogue (epoprostenol or treprostinil) between January 2001 and August 2015 at our center. Data were collected before initiation of treatment (baseline) and at 1-3 and 6-12 months after. Protocolized echocardiogram measurements including tricuspid annular plane systolic excursion (TAPSE) and RV global longitudinal strain were made with blinding to clinical information. Forty-nine individuals (65% female), aged 0-29 years at the time of prostacyclin initiation were included. Disease types included pulmonary arterial hypertension (idiopathic [35%], heritable [2%], and congenital heart disease-associated [18%]), developmental lung disease (43%), and chronic thromboembolic PH (2%). Participants received intravenous (IV) epoprostenol (14%) and IV/subcutaneous (SQ) (67%) or inhaled (18%) treprostinil. Over the study period, prostacyclin analogues were associated with improvement in TAPSE ( P = 0.007), RV strain ( P < 0.001), and qualitative RV function ( P = 0.037) by echocardiogram, and BNP ( P < 0.001), functional class ( P = 0.047) and 6-min walk distance ( P = 0.001). TAPSE and strain improved at early follow up ( P = 0.05 and P = 0.002, respectively) despite minimal RV pressure change. In children with PH, prostacyclin analogues are associated with an early and sustained improvement in RV function measured as TAPSE and strain as well as clinical markers of PH severity. RV strain may be a sensitive marker of RV function in this population.
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