Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.
Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT).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 record...
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doaj-9fbe1814329f4108896a85e2d1ca701b2020-11-24T21:34:06ZengPublic Library of Science (PLoS)PLoS ONE1932-62032017-01-011210e018422710.1371/journal.pone.0184227Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.Sabine RevuzEvelyne DecullierIsabelle GinonNicolas LamblinPierre-Yves HatronPierre KaminskyMarie-France CarettePascal LacombeAnne-Claire SimonSophie RivièreJean-Robert HarléAlain FraisseChristian LavigneVanessa Leguy-SeguinAri ChaouatChahera KhouatraSophie Dupuis-GirodEric HachullaDifferent pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT).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.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.http://europepmc.org/articles/PMC5628806?pdf=render |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sabine Revuz Evelyne Decullier Isabelle Ginon Nicolas Lamblin Pierre-Yves Hatron Pierre Kaminsky Marie-France Carette Pascal Lacombe Anne-Claire Simon Sophie Rivière Jean-Robert Harlé Alain Fraisse Christian Lavigne Vanessa Leguy-Seguin Ari Chaouat Chahera Khouatra Sophie Dupuis-Girod Eric Hachulla |
spellingShingle |
Sabine Revuz Evelyne Decullier Isabelle Ginon Nicolas Lamblin Pierre-Yves Hatron Pierre Kaminsky Marie-France Carette Pascal Lacombe Anne-Claire Simon Sophie Rivière Jean-Robert Harlé Alain Fraisse Christian Lavigne Vanessa Leguy-Seguin Ari Chaouat Chahera Khouatra Sophie Dupuis-Girod Eric Hachulla Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. PLoS ONE |
author_facet |
Sabine Revuz Evelyne Decullier Isabelle Ginon Nicolas Lamblin Pierre-Yves Hatron Pierre Kaminsky Marie-France Carette Pascal Lacombe Anne-Claire Simon Sophie Rivière Jean-Robert Harlé Alain Fraisse Christian Lavigne Vanessa Leguy-Seguin Ari Chaouat Chahera Khouatra Sophie Dupuis-Girod Eric Hachulla |
author_sort |
Sabine Revuz |
title |
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. |
title_short |
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. |
title_full |
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. |
title_fullStr |
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. |
title_full_unstemmed |
Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability. |
title_sort |
pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: haemodynamic profiles and survival probability. |
publisher |
Public Library of Science (PLoS) |
series |
PLoS ONE |
issn |
1932-6203 |
publishDate |
2017-01-01 |
description |
Different pulmonary hypertension (PH) mechanisms are associated with hereditary haemorrhagic telangiectasia (HHT).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.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. |
url |
http://europepmc.org/articles/PMC5628806?pdf=render |
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