Pulmonary arterial hypertension and pulmonary hypertension due to left heart disease: so near and yet so far. Authors' reply

Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common type of PH and is defined as mean pulmonary artery systolic pressure of >20 mm Hg and pulmonary capillary wedge pressure >15 mm Hg during right heart catheterization. LHD may lead to elevated left atrial pressure alone, which in the absence of intrinsic pulmonary vascular disease will result in PH without changes in pulmonary vascular resistance. Persistent elevation in left atrial pressure may, however, also be associated with subsequent pulmonary vascular remodeling, vasoconstriction, and an increase in pulmonary vascular resistance. Hence, there are 2 subgroups of PH due to LHD, isolated postcapillary PH and combined post- and precapillary PH, with these groups have differing clinical implications. Differentiation of pulmonary arterial hypertension and PH due to LHD is critical to guide management planning; however, this may be challenging. Older patients, patients with metabolic syndrome, and patients with imaging and clinical features consistent with left ventricular dysfunction are suggestive of LHD etiology rather than pulmonary arterial hypertension. Hemodynamic measures such as diastolic pressure gradient, transpulmonary gradient, and pulmonary vascular resistance may assist to differentiate pre- from postcapillary PH and offer prognostic insights. However, these are influenced by fluid status and heart failure treatment. Pulmonary arterial hypertension therapies have been trialed in the treatment with concerning results reflecting disease heterogeneity, variation in inclusion criteria, and mixed end point criteria. The aim of this review is to provide an updated definition, discuss possible pathophysiology, clinical aspects, and the available treatment options for PH due to LHD.

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Clinical Features of the Systemic Sclerosis Associated Pulmonary Arterial Hypertension with Left Heart Disease

Journal of Cardiac Failure ◽

10.1016/j.cardfail.2017.08.322 ◽

2017 ◽

Vol 23 (10) ◽

pp. S65

Author(s):

Hiromu Yanagisawa ◽

Hideyuki Kinoshita ◽

Kouichirou Kuwahara ◽

Hajime Yoshifuji ◽

Kenji Moriuti ◽

...

Keyword(s):

Heart Disease ◽

Systemic Sclerosis ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Clinical Features ◽

Left Heart ◽

Left Heart Disease ◽

Pulmonary Arterial

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Noninvasive identification of left-sided heart failure in a population suspected of pulmonary arterial hypertension

European Respiratory Journal ◽

10.1183/09031936.00202814 ◽

2015 ◽

Vol 46 (2) ◽

pp. 422-430 ◽

Cited By ~ 24

Author(s):

Wouter Jacobs ◽

Thelma C. Konings ◽

Martijn W. Heymans ◽

Anco Boonstra ◽

Harm Jan Bogaard ◽

...

Keyword(s):

Heart Failure ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Risk Score ◽

Left Heart ◽

Score System ◽

Pulmonary Arterial ◽

Risk Score System

Exclusion of pulmonary hypertension secondary to left-sided heart disease (left heart failure (LHF)) is pivotal in the diagnosis of pulmonary arterial hypertension (PAH). In case of doubt, invasive measurements are recommended. The aim of the present study was to investigate whether it is possible to diagnose LHF using noninvasive parameters in a population suspected of PAH.300 PAH and 80 LHF patients attended our pulmonary hypertension clinic before August 2010, and were used to build the predictive model. 79 PAH and 55 LHF patients attended our clinic from August 2010, and were used for prospective validation.A medical history of left heart disease, S deflection in V1 plus R deflection in V6 in millimetres on ECG, and left atrial dilation or left valvular heart disease that is worse than mild on echocardiography were independent predictors of LHF. The derived risk score system showed good predictive characteristics: R2=0.66 and area under the curve 0.93. In patients with a risk score ≥72, there is 100% certainty that the cause of pulmonary hypertension is LHF. Using this risk score system, the number of right heart catheterisations in LHF may be reduced by 20%.In a population referred under suspicion of PAH, a predictive model incorporating medical history, ECG and echocardiography data can diagnose LHF noninvasively in a substantial percentage of cases.

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Pulmonary arterial hypertension-associated genetic variants in combined post-capillary and pre-capillary pulmonary hypertension: a case report

Pulmonary Circulation ◽

10.1177/2045894021996571 ◽

2021 ◽

Vol 11 (1) ◽

pp. 204589402199657

Author(s):

Chomette L ◽

Caravita S ◽

Dewachter C ◽

Abramowicz M ◽

Vachiery JL ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Heart Disease ◽

Case Report ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Genetic Variants ◽

Cardiac Transplantation ◽

Predisposing Factors ◽

Left Heart ◽

Pulmonary Arterial

Predisposing factors for the development of a pre-capillary component in pulmonary hypertension associated with left heart disease remain elusive. We report the case of a patient with persistent combined post-capillary and pre-capillary pulmonary hypertension after cardiac transplantation, in whom a rare BMPR2 variant was found.

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