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Review
. 2025 Feb 17:19:e18743064359315.
doi: 10.2174/0118743064359315250210080743. eCollection 2025.

Revisiting Acute Decompensated Right Ventricle Failure in Pulmonary Arterial Hypertension

Rohit Masih  1 , Vivek Paudyal  2 , Yogendra Mani Basnet  3 , Shaleen Sunesara  4 , Munish Sharma  5 , Salim Surani  6
Affiliations
Review

Revisiting Acute Decompensated Right Ventricle Failure in Pulmonary Arterial Hypertension

Rohit Masih et al. Open Respir Med J. .

Abstract

Pulmonary Arterial Hypertension (PAH) is a progressive vascular disease characterized by elevated Pulmonary Vascular Resistance (PVR) leading to Right Ventricular (RV) dysfunction and, ultimately, Right Heart Failure (RHF). Acute decompensation of PAH presents a life-threatening consequence marked by sudden worsening of clinical signs of right heart failure, systemic circulatory insufficiency, and multi-system organ failure. Clinicians are encountering more and more patients with PAH and RHF in the critical care units. These patients require admission and management in a critical care unit until they can be stabilized. The pathogenesis involves an imbalance between RV afterload and its adaptation capacity, ultimately resulting in RV dilation and failure. While the causes of acute decompensation remain subtle in many cases, infections, drug noncompliance, and pulmonary embolism are common culprits. Early identification of signs and symptoms of acute decompensation of RV failure, determination of possible etiology, and timely initiation of optimal treatment approaches are pivotal in avoiding detrimental outcomes. Optimization of pre-load and use of pulmonary vasodilators and inotropic agents are cornerstones of management. In refractory cases, mechanical circulatory support such as Extracorporeal Membrane Oxygenation (ECMO) or Right Ventricular Assist Devices (RVADs) may be necessary. Balloon Atrial Septostomy (BAS) serves as a bridge to definitive therapy, offering decompression of the right atrium and right ventricle. The prognosis of acute decompensated RV failure in PAH patients remains poor, highlighting the critical need for early diagnosis and intervention to improve outcomes. Currently, there are no strict standard guidelines to manage acute decompensated RV failure in PAH patients. We aim to revisit current evidence and practice trends in PAH and its acute decompensation.

Keywords: Balloon Atrial Septostomy (BAS); Extracorporeal Membrane Oxygenation (ECMO); Pulmonary Vascular Resistance (PVR); Pulmonary arterial hypertension; Right heart failure; Right ventricle.

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Conflict of interest statement

Dr. Salim Surani is the Co-Editor and Dr. Munish Sharma is the Editorial Board Manager of the journal The Open Respiratory Medicine Journal.

Figures

Fig. (1)
Fig. (1)
mPAP=(CO×PVR) + PAWP Differences between Normal circulation(A) and Pre-capillary PH(B) [4, 5]. Ao: Aorta, CO: Cardiac Output, LA: Left Atrium, LV: Left Ventricle, mPAP: mean Pulmonary Arterial Pressure, PA: Pulmonary Artery, PAWP: Pulmonary Arterial Wedge Pressure, PC: Pulmonary Capillary, PV: Pulmonary Vein, PVR: Pulmonary Vascular Resistance, RA: Right Atrium, RV: Right Ventricle, VC: Venacava, WU: Woods Unit.
Fig. (2)
Fig. (2)
Clinical features of PAH with progression to RV failure.
Fig. (3)
Fig. (3)
Schematic flowchart depiction of the pathogenesis of Right Ventricular failure in Pulmonary Hypertension. CO: Cardiac Output, LV: Left Ventricle, PVR: Pulmonary Vascular Resistance, RAP: Right Atrial Pressure, RV: Right Ventricle.
Fig. (4)
Fig. (4)
Schematic flowchart of management of Acute Right Heart Failure. ECMO: Extracorporeal membrane oxygenation, IV: intravenous, MCS: Mechanical circulatory support, RV: Right Ventricle, VA-ECMO: Venoarterial extracorporeal membrane oxygenation.
See this image and copyright information in PMC

References

    1. Simonneau G., Montani D., Celermajer D.S., Denton C.P., Gatzoulis M.A., Krowka M., Williams P.G., Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. doi: 10.1183/13993003.01913-2018. - DOI - PMC - PubMed
    1. Hoeper M.M., Humbert M., Souza R., Idrees M., Kawut S.M., Sliwa-Hahnle K., Jing Z.C., Gibbs J.S.R. A global view of pulmonary hypertension. Lancet Respir. Med. 2016;4(4):306–322. doi: 10.1016/S2213-2600(15)00543-3. - DOI - PubMed
    1. Hu Y., Chi L., Kuebler W.M., Goldenberg N.M. Perivascular inflammation in pulmonary arterial hypertension. Cells. 2020;9(11):2338. doi: 10.3390/cells9112338. - DOI - PMC - PubMed
    1. Humbert M., Kovacs G., Hoeper M.M., Badagliacca R., Berger R.M.F., Brida M., Carlsen J., Coats A.J.S., Escribano-Subias P., Ferrari P., Ferreira D.S., Ghofrani H.A., Giannakoulas G., Kiely D.G., Mayer E., Meszaros G., Nagavci B., Olsson K.M., Pepke-Zaba J., Quint J.K., Rådegran G., Simonneau G., Sitbon O., Tonia T., Toshner M., Vachiery J.L., Vonk Noordegraaf A., Delcroix M., Rosenkranz S., Schwerzmann M., Dinh-Xuan A.T., Bush A., Abdelhamid M., Aboyans V., Arbustini E., Asteggiano R., Barberà J.A., Beghetti M., Čelutkienė J., Cikes M., Condliffe R., de Man F., Falk V., Fauchier L., Gaine S., Galié N., Gin-Sing W., Granton J., Grünig E., Hassoun P.M., Hellemons M., Jaarsma T., Kjellström B., Klok F.A., Konradi A., Koskinas K.C., Kotecha D., Lang I., Lewis B.S., Linhart A., Lip G.Y.H., Løchen M.L., Mathioudakis A.G., Mindham R., Moledina S., Naeije R., Nielsen J.C., Olschewski H., Opitz I., Petersen S.E., Prescott E., Rakisheva A., Reis A., Ristić A.D., Roche N., Rodrigues R., Selton-Suty C., Souza R., Swift A.J., Touyz R.M., Ulrich S., Wilkins M.R., Wort S.J. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur. Heart J. 2022;43(38):3618–3731. doi: 10.1093/eurheartj/ehac237. - DOI - PubMed
    1. Kovacs G., Berghold A., Scheidl S., Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: A systematic review. Eur. Respir. J. 2009;34(4):888–894. doi: 10.1183/09031936.00145608. - DOI - PubMed

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