Pulmonary Hypertension Subtypes Associated with Hereditary Haemorrhagic Telangiectasia: Haemodynamic Profiles and Survival Probability

Overview

Journal PLoS One

Specialties General Medicine
Science

Date 2017 Oct 6

PMID 28981519

Citations 15

Authors

Sabine Revuz

Evelyne Decullier

Isabelle Ginon

Nicolas Lamblin

Pierre-Yves Hatron

Pierre Kaminsky

Marie-France Carette

Pascal Lacombe

Anne-Claire Simon

Sophie Riviere

Jean-Robert Harle

Alain Fraisse

Christian Lavigne

Vanessa Leguy-Seguin

Ari Chaouat

Chahera Khouatra

Sophie Dupuis-Girod

Eric Hachulla

Affiliations

Soon will be listed here.

Abstract

Background: Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT).

Methods And Results: We conducted a retrospective study of all suspected cases of PH (echocardiographically estimated systolic pulmonary artery pressure [sPAP] ≥ 40 mmHg) in patients with definite HHT recorded in the French National Reference Centre for HHT database. When right heart catheterization (RHC) was performed, PH cases were confirmed and classified among the PH groups according to the European guidelines. Among 2,598 patients in the database, 110 (4.2%) had suspected PH. Forty-seven of these 110 patients had RHC: 38/47 (81%) had a confirmed diagnosis of PH. The majority of these had isolated post-capillary PH (n = 20). We identified for the first time other haemodynamic profiles: pre-capillary pulmonary arterial hypertension (PAH) cases (n = 3) with slightly raised pulmonary vascular resistances (PVR), and combined post- and pre-capillary PH cases (n = 4). Compared to controls, survival probability was lower in patients with PAH.

Conclusion: This study revealed the diversity of PH mechanisms in HHT. The description of combined post- and pre-capillary PH with/or without high cardiac output (CO) suggests either a continuum between the pre- and post-capillary haemodynamic profiles or a different course in response to high CO.

Citing Articles

Pulmonary vascular manifestations of hereditary haemorrhagic telangiectasia.

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Hereditary hemorrhagic telangiectasia with hepatic arteriovenous shunt diagnosed due to liver damage.

Hagiwara S, Takase T, Oda I, Komeda Y, Nishida N, Yoshida A Clin J Gastroenterol. 2024; 17(3):477-483.

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Long Term Survival of Heritable Pulmonary Arterial Hypertension Associated with Hereditary Hemorrhagic Telangiectasia: A Case Series.

Jamindar P, Pope M, Gossage J J Clin Med. 2024; 13(1).

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Pulmonary hypertension in hereditary hemorrhagic telangiectasia: A clinical review.

Mathavan A, Mathavan A, Reddy R, Jones K, Eagan C, Alnuaimat H Pulm Circ. 2023; 13(4):e12301.

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Cardiac and Hemodynamic Manifestations of Hereditary Hemorrhagic Telangiectasia.

Farhan A, Latif M, Minhas A, Weiss C Int J Angiol. 2022; 31(2):75-82.

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