ABSTRACT
BACKGROUND: Pulmonary embolism (PE) is a major cause of mortality with presentation varying between few or no symptoms to sudden death. This makes timely and appropriate treatment extremely important. Multidisciplinary PE response teams (PERT) have emerged to improve the management of acute PE. This study aims to describe the experience of a large multihospital single-network institution with PERT. METHODS: A retrospective cohort study of patients admitted for submassive and massive PE between 2012 and 2019 was conducted. The cohort was divided based on time of diagnosis and hospital into two groups: non-PERT included patients treated at hospitals that did not initiate PERT and patients diagnosed before the introduction of PERT (June 1, 2014); and the PERT group included those admitted after June 1, 2014, to a hospital with PERT. Patients with low-risk PE and those who had admissions in both time periods were excluded. Primary outcomes included all-cause mortality at 30, 60, and 90 days. Secondary outcomes included causes of death, intensive care unit (ICU) admission, ICU length of stay (LOS), total hospital LOS, type of treatment, and specialty consultations. RESULTS: We analyzed 5190 patients, with 819 (15.8%) being in the PERT group. Patients in the PERT group were more likely to receive extensive workup that included troponin-I (66.3% vs 42.3%; P < .001) and brain natriuretic peptide (50.4% vs 20.3%; P < .001). They also more often received catheter-directed interventions (12% vs 6.2%; P < .001) rather than anticoagulation monotherapy. Mortality outcomes were similar between both groups at all measured timepoints. Rates of ICU admission (65.2% vs 29.7%; P < .001), ICU LOS (median, 64.7 hours; interquartile range [IQR], 41.9-89.1 hours vs median, 38 hours; IQR, 22-66.4 hours; P < .001), and total hospital LOS (median, 5 days; IQR, 3-8 days vs median, 4 days; IQR, 2-6 days; P < .001) were all higher among the PERT group. Patients in the PERT group were more likely to receive vascular surgery consultation (5.3% vs 0.8%; P < .001) and the consultation occurred earlier in the admission when compared with the non-PERT group (median, 0 days; IQR, 0-1 days vs median, 1 day; IQR, 0-1; P = .04). CONCLUSIONS: The data presented here showed that there was no difference in mortality after PERT implementation. These results suggest that the presence of PERT increases the number of patients receiving a full PE workup with cardiac biomarkers. PERT also leads to more specialty consultations and more advanced therapies such as catheter-directed interventions. Further research is needed to assess the effect of PERT on long-term survival of patients with massive and submassive PE.
Subject(s)
Patient Care Team , Pulmonary Embolism , Humans , Retrospective Studies , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/therapy , Hospitalization , Length of Stay , Thrombolytic Therapy/adverse effects , Thrombolytic Therapy/methodsABSTRACT
Background: Interhospital transfer (IHT) of patients with acute life-threatening pulmonary embolism (PE) is necessary to facilitate specialized care and access to advanced therapies. Our goal was to understand what barriers and facilitators may exist during this transfer process from the perspective of both receiving and referring physicians. Methods: This qualitative descriptive study explored physician experience taking care of patients with life threatening PE. Subject matter expert physicians across several different specialties from academic and community United States hospitals participated in qualitative semi-structured interviews. Interview transcripts were subsequently analyzed using inductive qualitative description approach. Results: Four major themes were identified as barriers that impede IHT among patients with life threatening PE. Inefficient communication which mainly pertained to difficulty when multiple points of contact were required to complete a transfer. Subjectivity in the indication for transfer which highlighted the importance of physicians understanding how to use standardized risk stratification tools and to properly triage these patients. Delays in data acquisition were identified in regards to both obtaining clinical information and imaging in a timely fashion. Operation barriers which included difficulty finding available beds for transfer and poor weather conditions inhibiting transportation. In contrast, two main facilitators to transfer were identified: good communication and reliance on colleagues and dedicated team for transferring and treating PE patients. Conclusion: The most prominent themes identified as barriers to IHT for patients with acute life-threatening PE were: (1) inefficient communication, (2) subjectivity in the indication for transfer, (3) delays in data acquisition (imaging or clinical), and (4) operational barriers. Themes identified as facilitators that enable the transfer of patients were: (1) good communication and (2) a dedicated transfer team. The themes presented in our study are useful in identifying opportunities to optimize the IHT of patients with acute PE and improve patient care. These opportunities include instituting educational programs, streamlining the transfer process, and formulating a consensus statement to serve as a guideline regarding IHT of patients with acute PE.
ABSTRACT
Pulmonary embolism response teams (PERTs) have emerged as a multidisciplinary, multispecialty team of experts in the care of highly complex symptomatic acute pulmonary embolism (PE), with a centralized unique activation process, providing rapid multimodality assessment and risk stratification, formulating the best individualized diagnostic and therapeutic approach, streamlining the care in challenging clinical case scenarios (e.g., intermediate-high risk and high-risk PE), and facilitating the implementation of the recommended therapeutic strategies on time. PERTs are currently changing how complex acute PE cases are approached. The structure, organization, and function of a given PERT may vary from hospital to hospital, depending on local expertise, specific resources, and infrastructure for a given academic hospital center. Current emerging data demonstrate the value of PERTs in improving time to PE diagnosis; shorter time to initiation of anticoagulation reducing hospital length of stay; increasing use of advanced therapies without an increase in bleeding; and in some reports, decreasing mortality. Importantly, PERTs are positively impacting outcomes by changing the paradigm of care for acute PE through global adoption by the health-care community.
Subject(s)
Pulmonary Embolism , Humans , Pulmonary Embolism/diagnosis , Pulmonary Embolism/drug therapy , Hemorrhage , Acute Disease , Anticoagulants/therapeutic useABSTRACT
OBJECTIVES: The aim of this trial was to determine whether ultrasound-assisted thrombolysis (USAT) is superior to standard catheter-directed thrombolysis (SCDT) in pulmonary arterial thrombus reduction for patients with submassive pulmonary embolism (sPE). BACKGROUND: Catheter-directed therapy has been increasingly used in sPE and massive pulmonary embolism as a decompensation prevention and potentially lifesaving procedure. It is unproved whether USAT is superior to SCDT using traditional multiple-side-hole catheters in the treatment of patients with pulmonary embolism. METHODS: Adults with sPE were enrolled. Participants were randomized 1:1 to USAT or SCDT. The primary outcome was 48-hour clearance of pulmonary thrombus assessed by pre- and postprocedural computed tomographic angiography using a refined Miller score. Secondary outcomes included improvement in right ventricular-to-left ventricular ratio, intensive care unit and hospital stay, bleeding, and adverse events up to 90 days. RESULTS: Eighty-one patients with acute sPE were randomized and were available for analysis. The mean total dose of alteplase for USAT was 19 ± 7 mg and for SCDT was 18 ± 7 mg (P = 0.53), infused over 14 ± 6 and 14 ± 5 hours, respectively (P = 0.99). In the USAT group, the mean raw pulmonary arterial thrombus score was reduced from 31 ± 4 at baseline to 22 ± 7 (P < 0.001). In the SCDT group, the score was reduced from 33 ± 4 to 23 ± 7 (P < 0.001). There was no significant difference in mean thrombus score reduction between the 2 groups (P = 0.76). The mean reduction in right ventricular/left ventricular ratio from baseline (1.54 ± 0.30 for USAT, 1.69 ± 0.44 for SCDT) to 48 hours was 0.37 ± 0.34 in the USAT group and 0.59 ± 0.42 in the SCDT group (P = 0.01). Major bleeding (1 stroke and 1 vaginal bleed requiring transfusion) occurred in 2 patients, both in the USAT group. CONCLUSIONS: In the SUNSET sPE (Standard vs. Ultrasound-Assisted Catheter Thrombolysis for Submassive Pulmonary Embolism) trial, patients undergoing USAT had similar pulmonary arterial thrombus reduction compared with those undergoing SCDT, using comparable mean lytic doses and durations of lysis.
Subject(s)
Pulmonary Embolism , Thrombolytic Therapy , Adult , Female , Humans , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/drug therapy , Retrospective Studies , Thrombolytic Therapy/adverse effects , Tissue Plasminogen Activator/adverse effects , Treatment OutcomeABSTRACT
Venous thromboembolism associated with COVID-19, particularly acute pulmonary embolism, may represent a challenging and complex clinical scenario. The benefits of having a multidisciplinary pulmonary embolism response team (PERT) can be important during such a pandemic. The aim of PERT in the care of such patients is to provide fast, appropriate, multidisciplinary, team-based approach, with the common goal to tailor the best therapeutic decision making, prioritizing always optimal patient care, especially given lack of evidence-based clinical practice guidelines in the setting of COVID-19, which potentially confers a significant prothrombotic state. Herein, we would like to briefly emphasize the importance and potential critical role of PERT in the care of patients in which these two devastating illnesses are present together.
Subject(s)
COVID-19/therapy , Pulmonary Embolism/therapy , Thromboembolism/therapy , Venous Thromboembolism/therapy , Acute Disease , Anticoagulants/therapeutic use , COVID-19/complications , Cardiology/organization & administration , Decision Making , Evidence-Based Medicine , Humans , Interdisciplinary Communication , Practice Guidelines as Topic , Pulmonary Embolism/complications , Pulmonary Medicine/organization & administration , Quality of Life , SARS-CoV-2 , Thromboembolism/complications , Thrombolytic Therapy , Treatment Outcome , Venous Thromboembolism/complicationsABSTRACT
Chronic thromboembolic pulmonary hypertension (CTEPH) represents the later stage consequence of at least one or more unresolved episodes of acute pulmonary embolism; thus, indefinite anticoagulation is strongly recommended by current practice guidelines. Historically, vitamin K antagonists have been widely used in these patients. However, recent data indicate a shift toward direct oral anticoagulants (DOACs), despite lack of data on the safety and efficacy in this patient population. Herein, we briefly discuss the current rationale for oral anticoagulation use in CTEPH, addressing important issues and controversies involved with the use of DOACs, opening a strategy for further clinical research in the field of oral anticoagulation.
Subject(s)
Hypertension, Pulmonary , Administration, Oral , Anticoagulants/therapeutic use , Humans , Hypertension, Pulmonary/drug therapy , Pulmonary Embolism/complications , Pulmonary Embolism/drug therapy , Venous Thromboembolism/drug therapy , Vitamin KABSTRACT
Pulmonary embolism (PE) is a major source of morbidity and mortality. The presentation of acute PE varies, ranging from few or no symptoms to sudden death. Patient outcome depends on how well the right ventricle can sustain the increased afterload caused by the embolic burden. Careful risk stratification is critical, and the PE response team (PERT) concept offers a rapid and multidisciplinary approach. Anticoagulation is essential unless contraindicated; thrombolysis, surgical embolectomy, and catheter-directed approaches are also available. Clinical consensus statements have been published that offer a guide to PE management, but areas remain for which the evidence is inadequate. Although the management of low-risk and high-risk patients is more straightforward, optimal management of intermediate-risk patients remains controversial. In this document, we offer a case-based approach to PE management, beginning with diagnosis and risk stratification, followed by therapeutic alternatives, and finishing with follow-up care.
Subject(s)
Pulmonary Embolism/therapy , Aged , Anticoagulants/therapeutic use , Clinical Protocols , Embolectomy , Humans , Male , Patient Selection , Thrombolytic TherapyABSTRACT
Perioperative acute pulmonary embolism represents a relatively rare complication; however, it could be very serious and devastating in some cases. Its diagnosis could be particularly challenging, especially in the intraoperative period. Herein, we emphasize some key concepts with the aim to perform an early and appropriate risk stratification, diagnostic and therapeutic approach in a multidisciplinary fashion, a brief overview on thromboprophylaxis, with the main objective to improve outcomes and survival in these challenging patients.
ABSTRACT
BACKGROUND: Catheter-directed thrombolysis (CDL) is increasingly being used for the treatment of submassive and massive pulmonary embolism. Although this therapy has been shown to be effective at reducing right ventricle strain, the impact on clinical outcomes remains unclear. We therefore aimed to evaluate the outcomes of CDL compared to standard anticoagulation for submassive pulmonary embolism patients in a large cohort. METHODS: We conducted a retrospective observational study of consecutive patients with a primary diagnosis of submassive pulmonary embolism admitted to an intensive care unit within our health system between June 2014 and April 2016. We compared the outcome of patients treated with systemic anticoagulation (medical therapy) vs. catheter-based delivery of tissue plasminogen activator (tPA) (CDL). CDL patients were matched with medical therapy controls using a propensity-score matching algorithm based on the components of the simplified pulmonary embolism severity index (sPESI) score. RESULTS: Unadjusted mortality rates were 3.0% for CDL vs. 10.4% for medical therapy at 30 days and 8.1% for CDL vs. 22.9% for medical therapy at 1 year. In the propensity-score matched cohort, mortality rates were 3.1% for CDL vs. 6.1% for medical therapy at 30 days and 8.2% for CDL vs. 18.2% for medical therapy at 1 year. Length of stay was significantly shorter in the CDL group. The index admission bleeding and transfusion rates were not increased in the CDL group. CONCLUSIONS: In patients presenting with acute submassive pulmonary embolism who are admitted to an intensive care unit, the group treated with CDL experienced reduced mortality at 30 days and 1 year when compared to medical therapy without increase in bleeding. Further randomized studies are required to confirm these findings.
ABSTRACT
BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary arterial pressures and is managed by vasodilator therapies. Current guidelines encourage PAH management in specialty care centers (SCCs), but evidence is sparse regarding improvement in clinical outcomes and correlation to vasodilator use with referral. RESEARCH QUESTION: Is PAH management at SCCs associated with improved clinical outcomes? STUDY DESIGNAND METHODS: A single-center, retrospective study was performed at the University of Pittsburgh Medical Center (UPMC; overseeing 40 hospitals). Patients with PAH were identified between 2008 and 2018 and classified into an SCC or non-SCC cohort. Cox proportional hazard modeling was done to compare for all-cause mortality, as was negative binomial regression modeling for hospitalizations. Vasodilator therapy was included to adjust outcomes. RESULTS: Of 580 patients with PAH at UPMC, 455 (78%) were treated at the SCC, comprising a younger (58.8 vs 64.8 years; P < .001) and more often female (68.4% vs 51.2%; P < .001) population with more comorbidities without differences in race or income. SCC patients demonstrated improved survival (hazard ratio, 0.68; P = .012) and fewer hospitalizations (incidence ratio, 0.54; P < .001), and provided more frequent disease monitoring. Early patient referral to SCC (< 6 months from time of diagnosis) was associated with improved outcomes compared with non-SCC patients. SCC patients were more frequently prescribed vasodilators (P < .001) and carried more diagnostic PAH coding (P < .001). Vasodilators were associated with improved outcomes irrespective of location but without statistical significance when comparing between locations (P > .05). INTERPRETATION: The UPMC SCC demonstrated improved outcomes in mortality and hospitalizations. The SCC benefit was multifactorial, with more frequent vasodilator therapy and disease monitoring. These findings provide robust evidence for early and regular referral of patients with PAH to SCCs.
Subject(s)
Pulmonary Arterial Hypertension/mortality , Pulmonary Arterial Hypertension/therapy , Tertiary Care Centers , Aged , Female , Hospitalization , Humans , Male , Middle Aged , Proportional Hazards Models , Pulmonary Arterial Hypertension/diagnosis , Retrospective Studies , Survival Rate , Treatment Outcome , Vasodilator Agents/therapeutic useABSTRACT
Pulmonary embolism represents the third most common cause of cardiovascular death in the United States. Reperfusion therapeutic strategies such as systemic thrombolysis, catheter directed therapies, surgical pulmonary embolectomy, and cardiopulmonary support devices are currently available for patients with high- and intermediate-high-risk pulmonary embolism. However, deciding on optimal therapy may be challenging. Pulmonary embolism response teams have been designed to facilitate multidisciplinary decision-making with the goal to improve quality of care for complex cases with pulmonary embolism. Herein, we discuss the current role and strategies on how to leverage the strengths from pulmonary embolism response teams, its possible worldwide adoption, and implementation to improve survival and change the paradigm in the care of a potentially deadly disease.
Subject(s)
Embolectomy , Emergency Medical Services , Pulmonary Embolism/therapy , Reperfusion , Disease-Free Survival , Humans , Pulmonary Embolism/mortality , Survival RateABSTRACT
Venous thromboembolism (VTE) is a common cause of morbidity and mortality. Presence of preexisting conditions, such as cardiopulmonary diseases, cirrhosis, renal dysfunction, and asplenia, commonly occurs in VTE patients. Moreover, these conditions often are risk factors for developing VTE. These preexisting conditions make VTE diagnosis and treatment challenging and worsen outcomes. Current guidelines do not include detailed features in the diagnosis and management of patients with preexisting conditions. This review discusses presence of VTE in patients with preexisting cardiopulmonary diseases, cirrhosis, renal dysfunction, and asplenia.
Subject(s)
Heterotaxy Syndrome/complications , Kidney Failure, Chronic/complications , Liver Cirrhosis/complications , Venous Thromboembolism/diagnosis , Heterotaxy Syndrome/pathology , Humans , Kidney Failure, Chronic/pathology , Liver Cirrhosis/pathology , Risk Factors , Venous Thromboembolism/pathologyABSTRACT
OBJECTIVE: During the past few years, there has been a surge in the use of catheter-directed thrombolysis (CDT) for acute pulmonary embolism (PE), in the form of either standard CDT or ultrasound-assisted CDT (usCDT). This is a systematic review and meta-analysis of all published series on contemporary CDT for acute PE seeking to determine their clinical efficacy, stratifying by PE severity and CDT modality. METHODS: A comprehensive MEDLINE and Embase search was performed to identify studies that reported outcomes of CDT for acute PE published from 2009 to July 2017. Outcomes included clinical success (in-hospital survival with stabilization of hemodynamics, without decompensation or any major complication), in-hospital mortality, major bleeding, right ventricular/left ventricular ratio, and Miller score changes after CDT. Meta-analyses assumed random effects. RESULTS: Twenty studies with 1168 patients were included in the meta-analysis. Available for subgroup analysis were 210 patients with high-risk PE and 945 patients with intermediate-risk PE; 181 patients received CDT using a standard multiside hole catheter, and 850 received usCDT. The pooled average right ventricular/left ventricular improvement and Miller score drop after CDT were 0.30 (95% confidence interval [CI], 0.22-0.39) and 8.8 (95% CI, 7.1-10.5). For high-risk PE, the pooled estimate for clinical success was 81.3% (95% CI, 72.5%-89.1%), the 30-day mortality estimate was 8.0% (95% CI, 3.2%-14.0%), and major bleeding was 6.7% (95% CI, 1.0%-15.3%). For intermediate-risk PE, the pooled estimate for clinical success was 97.5% (95% CI, 95.3%-99.1%), the 30-day mortality was 0% (95% CI, 0%-0.5%), and major bleeding was 1.4% (95% CI, 0.3%-2.8%). In high-risk PE, clinical success for CDT and usCDT was 70.8% (95% CI, 53.4%-85.8%) and 83.1% (95% CI, 68.5%-94.5%), respectively. In intermediate-risk PE, clinical success for CDT and usCDT was 95.0% (95% CI, 88.5%-99.2%) and 97.5% (95% CI, 95.0%-99.4%), respectively. CONCLUSIONS: Catheter thrombolysis has high clinical success rates in both high- and intermediate-risk PE, but higher mortality and bleeding rates should be anticipated in high-risk PE. Ultrasound-assisted thrombolysis may be more effective than standard CDT in the higher risk population.
Subject(s)
Catheterization, Peripheral , Fibrinolytic Agents/administration & dosage , Pulmonary Embolism/drug therapy , Thrombolytic Therapy/methods , Aged , Catheterization, Peripheral/adverse effects , Catheterization, Peripheral/mortality , Female , Fibrinolytic Agents/adverse effects , Hemorrhage/chemically induced , Humans , Male , Middle Aged , Pulmonary Embolism/diagnostic imaging , Pulmonary Embolism/mortality , Pulmonary Embolism/physiopathology , Risk Factors , Thrombolytic Therapy/adverse effects , Thrombolytic Therapy/mortality , Time Factors , Treatment Outcome , Ultrasonography, InterventionalABSTRACT
BACKGROUND: Little data exist on the use of direct oral anticoagulant (DOAC) factor Xa inhibitors for submassive pulmonary embolism (PE) after catheter-directed thrombolysis (CDT). The objective of this evaluation was to determine whether the transition from parenteral anticoagulation to DOACs for submassive PE after CDT would decrease hospital length of stay (LOS) compared to warfarin. METHODS: A retrospective review of patients diagnosed with submassive PE who underwent CDT was conducted from January 1, 2012, to February 28, 2017. Hospital LOS and major and minor bleeding events were recorded during hospitalization and at 90 days. RESULTS: Sixty-two patients met the inclusion criteria, 36 in warfarin group and 26 in the DOAC group. Overall, patients receiving rivaroxaban or apixaban had a shorter median hospital LOS compared to warfarin (4.0 vs 6.1 days, P = .002). In the multivariate regression analysis, administration of DOAC was an independent predictor of decreased hospital LOS, ß: -2.1, 95% confidence interval (-3.5 to -0.7). CONCLUSION: Among patients with submassive PE, initiation of a DOAC shortly after CDT may result in a decreased hospital LOS compared to parenterally bridged warfarin.
Subject(s)
Length of Stay , Pulmonary Embolism/drug therapy , Pyrazoles/administration & dosage , Pyridones/administration & dosage , Rivaroxaban/administration & dosage , Thrombolytic Therapy/adverse effects , Warfarin/administration & dosage , Aged , Female , Humans , Male , Middle Aged , Pulmonary Embolism/etiology , Retrospective StudiesABSTRACT
Pulmonary arterial hypertension (PAH) is a progressive disease defined by an elevation in pulmonary arterial pressure that can lead to right heart failure and death. Ambrisentan is a selective endothelin receptor antagonist approved for the treatment of idiopathic, heritable PAH and connective tissue disease-associated PAH. Ambrisentan has been shown to improve exercise capacity and hemodynamics with an acceptable side-effect profile. It has also proven to be safely used in combination with other PAH-specific medications, especially with phosphodiesterase-5 inhibitors. In the recent randomized trial, AMBITION, it was shown that upfront combination therapy of ambrisentan and tadalafil significantly decreased the risk of clinical failure compared with monotherapy. This review describes the drug profile of ambrisentan and its safety and efficacy in the treatment of PAH.
Subject(s)
Antihypertensive Agents/therapeutic use , Hypertension, Pulmonary/drug therapy , Phenylpropionates/therapeutic use , Pyridazines/therapeutic use , Animals , Antihypertensive Agents/administration & dosage , Antihypertensive Agents/adverse effects , Drug Therapy, Combination , Humans , Hypertension, Pulmonary/complications , Hypertension, Pulmonary/physiopathology , Phenylpropionates/administration & dosage , Phenylpropionates/adverse effects , Pyridazines/administration & dosage , Pyridazines/adverse effects , Randomized Controlled Trials as Topic , Tadalafil/administration & dosage , Tadalafil/therapeutic useABSTRACT
Little is known about the physiologic determinants of 6-minute walk distance in idiopathic pulmonary fibrosis. We investigated the demographic, pulmonary function, echocardiographic, and hemodynamic determinants of 6-minute walk distance in patients with idiopathic pulmonary fibrosis evaluated for lung transplantation. We performed a cross-sectional analysis of 130 patients with idiopathic pulmonary fibrosis who completed a lung transplantation evaluation at the Hospital of the University of Pennsylvania between 2005 and 2010. Multivariable linear regression analysis was used to generate an explanatory model for 6-minute walk distance. After adjustment for age, sex, race, height, and weight, the presence of right ventricular dilation was associated with a decrease of 50.9 m (95% confidence interval [CI], 8.4-93.3) in 6-minute walk distance ([Formula: see text]). For each 200-mL reduction in forced vital capacity, the walk distance decreased by 15.0 m (95% CI, 9.0-21.1; [Formula: see text]). For every increase of 1 Wood unit in pulmonary vascular resistance, the walk distance decreased by 17.3 m (95% CI, 5.1-29.5; [Formula: see text]). Six-minute walk distance in idiopathic pulmonary fibrosis depends in part on circulatory impairment and the degree of restrictive lung disease. Future trials that target right ventricular morphology, pulmonary vascular resistance, and forced vital capacity may potentially improve exercise capacity in patients with idiopathic pulmonary fibrosis.
ABSTRACT
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) can lead to the development of pulmonary hypertension, which is associated with an increased risk of death. In pulmonary arterial hypertension, survival is directly related to the capacity of the right ventricle to adapt to elevated pulmonary vascular load. The relative importance of right ventricular function in IPF is not well understood. Our objective was to evaluate right ventricular echocardiographic and hemodynamic predictors of mortality in a cohort of patients with IPF referred for lung transplant evaluation. METHODS: We performed a retrospective cohort study of 135 patients who met 2011 American Thoracic Society/European Respiratory Society criteria for IPF and who were evaluated for lung transplantation at the Hospital of the University of Pennsylvania. RESULTS: Right ventricle:left ventricle diameter ratio (hazard ratio [HR], 4.5; 95% CI, 1.7-11.9), moderate to severe right atrial and right ventricular dilation (HR, 2.9; 95% CI, 1.4-5.9; and HR, 2.7; 95% CI, 1.4-5.4, respectively) and right ventricular dysfunction (HR, 5.5; 95% CI, 2.6-11.5) were associated with an increased risk of death. Higher pulmonary vascular resistance was also associated with increased mortality (HR per 1 Wood unit, 1.3; 95% CI, 1.1-1.5). These risk factors were independent of age, sex, race, height, weight, FVC, and lung transplantation status. Other hemodynamic indices, such as mean pulmonary artery pressure and cardiac index, were not associated with outcome. CONCLUSIONS: Right-sided heart size and right ventricular dysfunction measured by echocardiography and higher pulmonary vascular resistance by invasive hemodynamic assessment predict mortality in patients with IPF evaluated for lung transplantation.