Advertisement

 

 

Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.

Pulmonary hypertension subtypes associated with hereditary haemorrhagic telangiectasia: Haemodynamic profiles and survival probability.
Author Information (click to view)

Revuz S, Decullier E, Ginon I, Lamblin N, Hatron PY, Kaminsky P, Carette MF, Lacombe P, Simon AC, Rivière S, Harlé JR, Fraisse A, Lavigne C, Leguy-Seguin V, Chaouat A, Khouatra C, Dupuis-Girod S, Hachulla E,


Revuz S, Decullier E, Ginon I, Lamblin N, Hatron PY, Kaminsky P, Carette MF, Lacombe P, Simon AC, Rivière S, Harlé JR, Fraisse A, Lavigne C, Leguy-Seguin V, Chaouat A, Khouatra C, Dupuis-Girod S, Hachulla E, (click to view)

Revuz S, Decullier E, Ginon I, Lamblin N, Hatron PY, Kaminsky P, Carette MF, Lacombe P, Simon AC, Rivière S, Harlé JR, Fraisse A, Lavigne C, Leguy-Seguin V, Chaouat A, Khouatra C, Dupuis-Girod S, Hachulla E,

Advertisement

PloS one 2017 10 0512(10) e0184227 doi 10.1371/journal.pone.0184227
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.

Submit a Comment

Your email address will not be published. Required fields are marked *

two × four =

[ HIDE/SHOW ]