Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study

Maron, Bradley A.; Brittain, Evan L.; Hess, Edward; Waldo, Stephen W.; Barón, Anna E.; Huang, Shi; Goldstein, Ronald H.; Assad, Tufik R.; Wertheim, Bradley M.; Alba, George A.; Leopold, Jane A.; Olschewski, Horst; Galiè, Nazzareno; Simonneau, Gérald; Kovàcs, Gabór; Tedford, Ryan J.; Humbert, Marc; Choudhary, Gaurav

doi:10.1016/s2213-2600(20)30317-9

Cited by 145 publications

(112 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…1 This case illustrates that PVR ranging between 2 and 3 WU in the highrisk population of BMPR2 mutation carriers might represent an early abnormal haemodynamic status, associated with disease progression and clinical outcomes. 1,34 Like in systemic sclerosis 24 , our study suggests that exercise PH may be a possible early marker of pulmonary vascular remodelling in BMPR2 mutation carriers. Indeed, 16•7% of the 12 subjects with exercise PH at inclusion subsequently developed PAH during long-term follow-up versus 2•5% among the 40 subjects with normal exercise haemodynamics.…”

Section: Discussionmentioning

confidence: 69%

Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation

et al. 2020

Self Cite

View full text Add to dashboard Cite

BackgroundHeritable pulmonary arterial hypertension (PAH) is most commonly due to heterozygous mutations of the BMPR2 gene. Based on expert consensus, guidelines recommend annual screening echocardiography in asymptomatic BMPR2 mutation carriers. The main objectives of this study were to evaluate characteristics of asymptomatic BMPR2 mutation carriers, assess their risk of occurrence of PAH, and detect PAH at an early stage in this high-risk population.MethodsAsymptomatic BMPR2 mutation carriers underwent screening at baseline and annually for a minimum of 2 years (DELPHI-2 study, NCT01600898). Annual screening included clinical assessment, electrocardiogram, pulmonary function tests, 6-minute walk distance, cardiopulmonary exercise test, chest X-ray, echocardiography, and NT pro-BNP level. Right heart catheterisation (RHC) was performed based on predefined criteria. An optional RHC at rest and exercise was proposed at baseline.ResultsFifty-five subjects (26 males, median age 37 years) were included. At baseline, no PAH was suspected based on echocardiography and NT pro-BNP levels. All subjects accepted RHC at inclusion, which identified two mild PAH cases (3·6%), and 12 subjects with exercise pulmonary hypertension (21·8%). At long term follow-up (118·8 patients.year follow-up), three additional cases were diagnosed, yielding a PAH incidence of 2·3%/year (0·99%/year in men and 3·5%/year in women). All PAH cases have remained at low-risk status on oral therapy at last follow-up.ConclusionsAsymptomatic BMPR2 mutation carriers have a significant risk of developing incident PAH. International multicenter studies are needed to confirm that refined multimodal screening programs with regular follow-up allow early detection of PAH.

show abstract

Section: Discussionmentioning

confidence: 69%

Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…Total pulmonary arterial compliance (PAC=1/pulmonary arterial stiffness) is defined as the relation between changes in arterial volume per unit change in arterial pressure, and it is currently estimated by the stroke volume over pulmonary arterial pulse pressure ratio (SV/PP) [ 9 – 11 ]. In patients with pulmonary hypertension, increased PVR and reduced PAC may contribute to right ventricular dysfunction and poor prognosis [ 12 – 19 ].…”

Section: Introductionmentioning

confidence: 99%

The isobaric pulmonary arterial compliance in pulmonary hypertension

et al. 2021

Self Cite

View full text Add to dashboard Cite

Pulmonary hypertension (PH) is associated with stiffening of pulmonary arteries which increases right ventricular pulsatile loading. High pulmonary artery wedge pressure (PAWP) in postcapillary PH (Pc-PH) further decreases PA compliance (PAC) at a given pulmonary vascular resistance (PVR) compared to precapillary PH, thus responsible for a higher total arterial load. In all other vascular beds, arterial compliance is considered as mainly determined by the distending pressure, due to non-linear stress-strain behaviour of arteries. We tested the applicability, advantages and drawbacks of two comparison methods of PAC depending on the level of mean PA pressure mPAP (isobaric PAC) or PVR.Right heart catheterisation data including PAC (stroke volume/pulse pressure) were obtained in 112Pc-PH (of whom 61 had combined postcapillary and precapillary PH) and 719 idiopathic pulmonary arterial hypertension (iPAH).PAC could be compared over the same mPAP range (25–66 mmHg) in 792/831 patients (95.3%) and over the same PVR range (3–10.7 WU) in only 520/831 patients (62.6%). The main assumption underlying comparisons at a given PVR was not verified as the PVR×PAC product (RC-time) was not constant but on the contrary more variable than mPAP. In the 788/831 (94.8%) patients studied over the same PAC range (0.62–6.5 mL·mmHg−1), PVR and thus total arterial load tended to be higher in iPAH.Our study favours comparing PAC at fixed mPAP level (isobaric PAC) rather than at fixed PVR. A reappraisal of the effects of PAWP on the pulsatile and total arterial load put on the right heart is needed, and this point deserves further studies.

show abstract

“…However, mean PAP used in isolation cannot characterize the severity of disease or de ne the pathological process, since it changes depending on various hemodynamic factors such as cardiac output 2 , and as the disease progresses, an increase in PAP usually lag behind pathological changes in the pulmonary arterial tree, leading to a diagnostic delay 3 . Mean PAP and PVR have been shown to correlate, but not closely, with the degree of right ventricular dysfunction and adverse clinical outcomes 4,5 . These inherent limitations may be because they do not represent all components of load faced by the right ventricle.…”

Section: Introductionmentioning

confidence: 99%

A New Echo-Doppler Method of Assessing Pulmonary Arterial Wave Reflection: In Vivo Validation

Yoshida¹,

Uejima²,

Komeda³

et al. 2021

Preprint

View full text Add to dashboard Cite

Background: Pulmonary arterial (PA) wave reflection provides additional information for assessing right ventricular afterload, but its applications is hampered by the need for invasive pressure and flow measurements. We tested the hypothesis that PA pressure and flow waveforms estimated by Doppler echocardiography could be used to quantify PA wave reflection. Methods: Doppler echocardiographic images of tricuspid regurgitation and right ventricular outflow tract flow used to estimate PA pressure and flow waveforms were acquired simultaneously with direct measurements with a dual sensor-tipped catheter under various hemodynamic conditions in a canine model of pulmonary hypertension (n=8). Wave separation analysis was performed on echo-Doppler derived as well as catheter derived waveforms to separate PA pressure into forward (Pf) and backward (Pb) pressures and derive wave reflection coefficient (RC) defined as Pb divided by Pf.Results: RC by echo-Doppler agreed well with RC indices by catheter (RC: bias = 0.13, 95% limits of agreement = -0.25 to 0.26). RC correlated negatively with pulmonary arterial compliance and right ventricular systolic function. Conclusions: This echo-Doppler method yields accurate measurement of reflected wave in the pulmonary circulation, paving the way to a more integrative assessment of pulmonary hemodynamics in the clinical setting.

show abstract

Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study

Cited by 145 publications

References 31 publications

Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation

Screening for pulmonary arterial hypertension in adults carrying a BMPR2 mutation

The isobaric pulmonary arterial compliance in pulmonary hypertension

A New Echo-Doppler Method of Assessing Pulmonary Arterial Wave Reflection: In Vivo Validation

Contact Info

Product

Resources

About