Extract
We appreciate the thoughtful comments of J.C. Grignola and colleagues in their letter addressing our article [1]. First, we agree that pulmonary hypertension associated with left heart disease (PH-LHD) is associated with global pulmonary vascular remodelling involving pulmonary arteries, veins and capillaries, as recently reported by Fayyaz et al. [2]. In fact, we have previously observed increased wall thickness and intimal fibrosis of small pulmonary arteries, even in patients with isolated post-capillary pulmonary hypertension (Ipc-PH) or “passive” pulmonary hypertension (PH) [3]. However, significant pre-capillary pulmonary vascular disease appeared to only be present in patients with combined post and pre-capillary PH (Cpc-PH), particularly those with elevated diastolic pulmonary vascular pressure gradient (DPG) [3]. Of note, in the study by Fayyaz et al. [2], diagnosis of PH was solely based on echocardiography. Furthermore, in their study, DPG was 3 mmHg and pulmonary vascular resistance (PVR) was 3.9 WU in the subgroup of 30 patients with available invasive haemodynamic data, thus resembling the haemodynamic phenotype of Ipc-PH. Application of these data are therefore limited to Ipc-PH and cannot be applied to Cpc-PH.
Abstract
Haemodynamic estimates of pulmonary vascular disease are a major requirement for the understanding and management of patients with pulmonary hypertension due to left heart disease http://ow.ly/ABsl30nuXX2
Footnotes
Conflict of interest: C. Gerges reports receiving the following during the conduct of the study: grants from United Therapeutics Corporation, Bayer Healthcare and Actelion Pharmaceuticals, and personal fees from GlaxoSmithKline, AOPOrphan and Actelion.
Conflict of interest: M. Gerges reports receiving the following during the conduct of the study: grants from United Therapeutics Corporation, Bayer Healthcare and Actelion Pharmaceuticals. M. Gerges reports receiving the following outside the submitted work: personal fees from GlaxoSmithKline, AOPOrphan and Actelion.
Conflict of interest: I.M. Lang reports receiving the following during the conduct of the study: grants from United Therapeutics Corporation, Bayer AG and Actelion Pharmaceuticals. I.M. Lang reports receiving the following outside the submitted work: grants, personal fees and non-financial support from Actelion, AOPOrphan Pharmaceuticals, and United Therapeutics Corporation; and other support from AstraZeneca, Servier, Medtronic and Novartis.
Support statement: This research was funded by educational grants from Actelion Pharmaceuticals Ltd (00283GMS&C), Bayer HealthCare Pharmaceuticals (grant number 15662 and IIR 18495), SFB-54 and United Therapeutics Corporation (grant number REG-NC-002).
- Received December 17, 2018.
- Accepted December 18, 2018.
- Copyright ©ERS 2019
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