Decreased pulmonary arterial proportional pulse pressure is associated with increased mortality in group 1 pulmonary hypertension

Mwansa, Hunter; Bilchick, Kenneth C.; Parker, Alex M.; Harding, William C.; Ruth, Benjamin; Kennedy, Jamie L.W.; Mysore, Manu; Kwon, Younghoon; Mihalek, Andrew; Mazimba, Sula

doi:10.1002/clc.22752

Cited by 6 publications

(3 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Consistent with this, we found that ARVC HT patients had a median age of 48 years, and compared with non-ARVC HT transplants, they had fewer co-morbidities (significantly less diabetes and tobacco use) and were more likely to have implantable cardioverter defibrillators. Reflective of their intrinsic RV-dominant pathology, ARVC patients in this study had significantly lower PA pressures compared with non-ARVC patients while having significantly narrower PA pulse pressures (an indirect measure of the combined effects of RV contractility and pulmonary vascular distensibility 29 ) and significantly impaired cardiac output, likely driving need for HT. Likewise, we have demonstrated that patients with ARVC can have impaired ventilatory efficiency, which is in turn associated with HF and need for HT.…”

Section: Discussionmentioning

confidence: 82%

Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy: a contemporary national analysis

et al. 2022

View full text Add to dashboard Cite

Aims Heart failure is an increasingly recognized later stage manifestation of arrhythmogenic right ventricular cardiomyopathy (ARVC) that can require heart transplantation (HT) to appropriately treat. We aimed to study contemporary ARVC HT outcomes in a national registry. Methods and resultsThe United Network for Organ Sharing registry was queried for HT recipients from 1/1994 through 2/2020. ARVC patients were compared with non-ARVC dilated, restrictive, and hypertrophic cardiomyopathy HT patients (HT for ischaemic and valvular disease was excluded from analysis). Post-HT survival was assessed using Kaplan-Meier estimates. A total of 189 of 252 (75%) waitlisted ARVC patients (median age 48 years, 65% male) underwent HT, representing 0.3% of the total 65 559 HT during the study time period. Annual frequency of HT for ARVC increased significantly over time. ARVC patients had less diabetes (5% vs. 17%, P < 0.001), less cigarette use (15% vs. 23%, P < 0.001), lower pulmonary artery and pulmonary capillary wedge pressures, and lower cardiac output than the 33 659 non-ARVC patients (P < 0.001). Ventricular assist device use was significantly lower in ARVC patients (8% vs. 32%, P < 0.001); 1 and 5 year post-HT survival was 97% and 93% for ARVC vs. 95% and 82% for non-ARVC HT recipients (P < 0.001). On adjusted multivariable Cox regression, ARVC had decreased risk of post-HT death compared with non-ARVC aetiologies (hazard ratio 0.48, 95% confidence interval 0.28-0.82, P = 0.008). Patients with ARVC also had lower risk of death or graft failure than non-ARVC patients (hazard ratio 0.51, 95% confidence interval 0.32-0.81, P = 0.004). Conclusions In the largest series of HT in ARVC, we found that HT is increasingly performed in ARVC, with higher survival compared with other cardiomyopathy aetiologies. The right ventricular predominant pathophysiology may require unique considerations for heart failure management, including HT.

show abstract

Section: Discussionmentioning

confidence: 82%

Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy: a contemporary national analysis

et al. 2022

View full text Add to dashboard Cite

show abstract

“…(19)(20)(21)(22) A recent iteration utilized the PAPP indexed to systolic pulmonary artery pressure (SPAP) with higher values predicting more favorable outcomes in pulmonary arterial hypertension cohorts. (23) The role of PAPP or PAPP indices as indicators of RV function remain undefined in ECMO.…”

Section: Abbreviationsmentioning

confidence: 99%

Pulmonary Artery Pressures and Mortality during VA ECMO: An ELSO Registry Analysis

Owyang

Rippon

Teran

et al. 2023

Preprint

View full text Add to dashboard Cite

Background: Systemic hemodynamics and specific ventilator settings have been shown to predict survival during venoarterial extracorporeal membrane oxygenation (VA ECMO). While these factors are intertwined with right ventricular (RV) function, the independent relationship between RV function and survival during VA ECMO is unknown. Objectives: To identify the relationship between RV function with mortality and duration of ECMO support. Methods: Cardiac ECMO runs in adults from the Extracorporeal Life Support Organization (ELSO) Registry between 2010 and 2022 were queried. RV function was quantified via pulmonary artery pulse pressure (PAPP) for pre-ECMO and on-ECMO periods. A multivariable model was adjusted for Society for Cardiovascular Angiography and Interventions (SCAI) stage, age, gender, and concurrent clinical data (i.e., pulmonary vasodilators and systemic pulse pressure). The primary outcome was in-hospital mortality. Results: A total of 4,442 ECMO runs met inclusion criteria and had documentation of hemodynamic and illness severity variables. The mortality rate was 55%; non-survivors were more likely to be older, have a worse SCAI stage, and have longer pre-ECMO endotracheal intubation times (P < 0.05 for all) than survivors. Improving PAPP from pre-ECMO to on-ECMO time (? PAPP) was associated with reduced mortality per 10 mm Hg increase (OR: 0.91 [95% CI: 0.86-0.96]; P=0.002). Increasing on-ECMO PAPP was associated with longer time on ECMO per 10 mm Hg (Beta: 15 [95% CI: 7.7-21]; P<0.001). Conclusions: Early improvements in RV function from pre-ECMO values were associated with mortality reduction during cardiac ECMO. Incorporation of ? PAPP into risk prediction models should be considered.

show abstract

“…[20][21][22][23] A recent iteration utilized the PAPP indexed to systolic pulmonary artery pressure (SPAP) with higher values predicting more favorable outcomes in pulmonary arterial hypertension cohorts. 24 The role of PAPP or PAPP indices as indicators of right heart function remains undefined in ECMO.…”

mentioning

confidence: 99%

Pulmonary Artery Pressures and Mortality During Venoarterial ECMO: An ELSO Registry Analysis

Owyang,

Rippon,

Teran

et al. 2024

Circ: Heart Failure

View full text Add to dashboard Cite

BACKGROUND: Systemic hemodynamics and specific ventilator settings have been shown to predict survival during venoarterial extracorporeal membrane oxygenation (ECMO). How the right heart (the right ventricle and pulmonary artery) affect survival during venoarterial ECMO is unknown. We aimed to identify the relationship between right heart function with mortality and the duration of ECMO support. METHODS: Cardiac ECMO runs in adults from the Extracorporeal Life Support Organization Registry between 2010 and 2022 were queried. Right heart function was quantified via pulmonary artery pulse pressure (PAPP) for pre-ECMO and on-ECMO periods. A multivariable model was adjusted for modified Society for Cardiovascular Angiography and Interventions stage, age, sex, and concurrent clinical data (ie, pulmonary vasodilators and systemic pulse pressure). The primary outcome was in-hospital mortality. RESULTS: A total of 4442 ECMO runs met inclusion criteria and had documentation of hemodynamic and illness severity variables. The mortality rate was 55%; nonsurvivors were more likely to be older, have a worse Society for Cardiovascular Angiography and Interventions stage, and have longer pre-ECMO endotracheal intubation times ( P <0.05 for all) than survivors. Increasing PAPP from pre-ECMO to on-ECMO time (ΔPAPP) was associated with reduced mortality per 2 mm Hg increase (odds ratio, 0.98 [95% CI, 0.97–0.99]; P =0.002). Higher on-ECMO PAPP was associated with mortality reduction across quartiles with the greatest reduction in the third PAPP quartile (odds ratio, 0.75 [95% CI, 0.63–0.90]; P =0.002) and longer time on ECMO per 10 mm Hg (beta, 15 [95% CI, 7.7–21]; P <0.001). CONCLUSIONS: Early on-ECMO right heart function and interval improvement from pre-ECMO values were associated with mortality reduction during cardiac ECMO. Incorporation of right heart metrics into risk prediction models should be considered.

show abstract

Decreased pulmonary arterial proportional pulse pressure is associated with increased mortality in group 1 pulmonary hypertension

Cited by 6 publications

References 23 publications

Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy: a contemporary national analysis

Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy: a contemporary national analysis

Pulmonary Artery Pressures and Mortality during VA ECMO: An ELSO Registry Analysis

Pulmonary Artery Pressures and Mortality During Venoarterial ECMO: An ELSO Registry Analysis

Contact Info

Product

Resources

About