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. 2021 Oct 31;10(21):5130.
doi: 10.3390/jcm10215130.

Non-Invasive Risk Prediction Based on Right Ventricular Function in Patients with Pulmonary Arterial Hypertension

Vazhma Qaderi  1   2 , Jessica Weimann  1 , Lars Harbaum  3   4 , Benedikt N Schrage  1   5 , Dorit Knappe  1 , Jan K Hennigs  3   4 , Christoph Sinning  1 , Renate B Schnabel  1   5 , Stefan Blankenberg  1   5 , Paulus Kirchhof  1   5   6 , Hans Klose  3   4 , Christina Magnussen  1   5
Affiliations

Non-Invasive Risk Prediction Based on Right Ventricular Function in Patients with Pulmonary Arterial Hypertension

Vazhma Qaderi et al. J Clin Med. .

Abstract

Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients.

Methods and results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters. We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. The analyses included a conventional model using guideline-recommended variables and an extended echocardiographic model. Based on the final model a 12-point risk score was derived, indicating the association with the primary outcome within five years. During a median follow-up time of 4.2 years 74 patients died or underwent lung transplantation. The conventional model resulted in a C-Index of 0.539, whereas the extended echocardiographic model improved the discrimination (C-index 0.639, p-value 0.017). Ultimately, the newly developed risk score included WHO functional class, 6-min walking distance, N-terminal brain natriuretic peptide concentrations, pericardial effusion, right atrial area, tricuspid annular plane systolic excursion, and fractional area change.

Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients. Independent validation of this finding is warranted.

Keywords: echocardiography; fractional area change; pulmonary arterial hypertension; risk score; tricuspid annular plane systolic excursion.

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Conflict of interest statement

C.M. reports speaker fees from AstraZeneca, Novartis and Heinen & Loewenstein unrelated to the current work. D.K. reports speaker fees from AstraZeneca unrelated to the current work. B.S. reports speakers fee from AstraZeneca and Abiomed, unrelated to the current work. LH reports speaker fees from Johnson & Johnson and OMT, research funding from MSD, and a fellowship from the European Respiratory Society (ERS) in the past 3 years not related to the current work. C.S. reports personal fees from Johnson & Johnson and personal fees from Astra Zeneca, outside the submitted work. R.B.S. has received funding from the European Research Council (ERC) under the European Union’s Horizon 2020 research and innovation programme under the grant agreement No 648131, from the European Union’s Horizon 2020 research and innovation programme under the grant agreement No 847770 (AFFECT-EU) and German Center for Cardiovascular Research (DZHK e.V.) (81Z1710103); German Ministry of Research and Education (BMBF 01ZX1408A) and ERACoSysMed3 (031L0239). R.B.S. has received lecture fees and advisory board fees from BMS/Pfizer outside this work. P.K. is partially supported by European Union BigData@Heart (grant agreement EU IMI 116074), British Heart Foundation (PG/17/30/32961 and PG/20/22/35093; AA/18/2/34218), German Centre for Cardiovascular Research supported by the German Ministry of Education and Research (DZHK), and Leducq Foundation. P.K. receives research support for basic, translational, and clinical research projects from European Union, British Heart Foundation, Leducq Foundation, Medical Research Council (UK), and German Centre for Cardiovascular Research, from several drug and device companies active in atrial fibrillation, and has received honoraria from several such companies in the past, but not in the last three years. P.K. is listed as inventor on two patents held by University of Birmingham (Atrial Fibrillation Therapy WO 2015140571, Markers for Atrial Fibrillation WO 2016012783). S.B. has received research funding from Abbott Diagnostics, Bayer, SIEMENS, Singulex and Thermo Fisher. He received honoraria for lectures from Abbott, Abbott Diagnostics, Astra Zeneca, Bayer, AMGEN, Medtronic, Pfizer, Roche, SIEMENS Diagnostics, SIEMENS, Thermo Fisher and as member of Advisory Boards and for consulting for Bayer, Novartis and Thermo Fisher. All other authors report no relationships that could be construed as a conflict of interest.

Figures

Figure 1
Figure 1
Predictors of death or lung transplantation in univariable Cox regression analyses. Legend: This figure displays the association of each variable with all-cause mortality and lung transplantation based on univariable Cox regression analyses. Abbreviations: HR = Hazard Ratio, CI = Confidence Interval, No. = Number, BP = blood pressure, BMI = body-mass-index, 6MWD = 6-min walk distance, FC = functional class, NT-proBNP = N-terminal-pro hormone brain peptide, eGFR = estimated glomerular filtration rate, RA = right atrial, RV = right ventricular, VCI = vena cava inferior, FAC = fractional area change, TAPSE = tricuspid annular plane systolic excursion, DLCO = diffusing capacity of the lungs for carbon monoxide, FVC = forced vital capacity.
Figure 2
Figure 2
Point-based score to calculate the individual risk of all-cause mortality and lung transplantation within 5 years in patients with PAH. Legend: We developed a point-based score to assess the risk of death or lung transplantation within 5 years in patients with pulmonary arterial hypertension. The score ranges from 0 to 12 points and indicates the relative risk. Abbreviations: TAPSE = tricuspid annular plane systolic excursion, FAC = fractional area change, NT-proBNP = N-terminal-pro hormone brain peptide, LTx = lung transplantation.
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