Open AccessPulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability
Author(s) -
Sabine Revuz,
Évelyne Decullier,
Isabelle Gi,
Nicolas Lamblin,
PierreYves Hatron,
P. Kaminsky,
Marie-France Carette,
Pascal Lacombe,
Anne-Claire Simon,
Sophie Rivière,
JeanRobert Harlé,
Alain Fraisse,
Christian Lavigne,
V. Leguy-Seguin,
Ari Chaouat,
C. Khouatra,
Sophie DupuisGirod,
Éric Hachulla
Publication year - 2017
Publication title -
plos one
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.99
H-Index - 332
ISSN - 1932-6203
DOI - 10.1371/journal.pone.0184227
Subject(s) - pulmonary hypertension , telangiectasia , medicine , hemodynamics , pulmonary artery , cardiology , right heart catheterization , vascular resistance , pulmonary wedge pressure , cardiac catheterization , heart catheterization , blood pressure , surgery
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.