scholarly journals Pulmonary hypertension phenotypes in patients with systemic sclerosis

2021 ◽  
Vol 30 (161) ◽  
pp. 210053
Author(s):  
Ashraful Haque ◽  
David G. Kiely ◽  
Gabor Kovacs ◽  
A.A. Roger Thompson ◽  
Robin Condliffe
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Left Heart ◽  
Significant Morbidity ◽  
Early Disease ◽  
Pulmonary Haemodynamics ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a heterogenous condition and several different forms can be associated with SSc, including pulmonary arterial hypertension (PAH) resulting from a pulmonary arterial vasculopathy, PH due to left heart disease and PH due to interstitial lung disease. The incidence of pulmonary veno-occlusive disease is also increased. Accurate and early diagnosis to allow optimal treatment is, therefore, essential. Recent changes to diagnostic haemodynamic criteria at the 6th World Symposium on Pulmonary Hypertension have resulted in therapeutic uncertainty regarding patients with borderline pulmonary haemodynamics. Furthermore, the optimal pulmonary vascular resistance threshold for diagnosing PAH and the role of exercise in identifying early disease require further elucidation. In this article we review the epidemiology, diagnosis, outcomes and treatment of the spectrum of pulmonary vascular phenotypes associated with SSc.

scholarly journals Pulmonary Hypertension Due to Left Heart Disease—Combine or Not Combined? DPG In or Out? A Practical Approach to the Patient With Suspected Left Heart Disease

2019 ◽  
Vol 18 (3) ◽  
pp. 87-91
Author(s):  
Thenappan Thenappan
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Left Heart ◽  
Common Cause ◽  
Pulmonary Vasodilators ◽  
Left Heart Disease ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) due to left heart disease (LHD) is the most common cause of PH in clinical practice. The definition and classification of PH-LHD has evolved in the last 5 years from the 5th World Symposium on PH (WSPH) in 2013 to the most recent 6th WSPH in 2018. Differentiation of PH-LHD, especially PH due to heart failure with preserved ejection from pulmonary arterial hypertension and chronic thromboembolic PH, can be very challenging. Finally, there is unclarity on the role of pulmonary vasodilators in the treatment of PH-LHD. The 6th WSPH consensus proceedings addresses all these topics in a detailed manner. In this article, we review the changes proposed by the 6th WSPH consensus proceedings in the definition, classification, diagnostic evaluation, and treatment of PH-LHD.

scholarly journals Under pressure: pulmonary hypertension associated with left heart disease

2015 ◽  
Vol 24 (138) ◽  
pp. 665-673 ◽  
Author(s):  
Harrison W. Farber ◽  
Simon Gibbs
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Survival Rates ◽  
Left Ventricular ◽  
Left Heart ◽  
Mean Pulmonary Arterial Pressure ◽  
Left Heart Disease ◽  
Right Heart Catheterisation ◽  
Pulmonary Capillary ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) associated with left heart disease (PH-LHD) is the most common type of PH, but its natural history is not well understood. PH-LHD is diagnosed by right heart catheterisation with a mean pulmonary arterial pressure ≥25 mmHg and a pulmonary capillary wedge pressure >15 mmHg. The primary causes of PH-LHD are left ventricular dysfunction of systolic and diastolic origin, and valvular disease. Prognosis is poor and survival rates are low. Limited progress has been made towards specific therapies for PH-LHD, and management focuses on addressing the underlying cause of the disease with supportive therapies, surgery and pharmacological treatments. Clinical trials of therapies for pulmonary arterial hypertension in patients with PH-LHD have thus far been limited and have provided disappointing or conflicting results. Robust, long-term clinical studies in appropriate target populations have the potential to improve the outlook for patients with PH-LHD. Herein, we discuss the knowledge gaps in our understanding of PH-LHD, and describe the current unmet needs and challenges that are faced by clinicians when identifying and managing patients with this disease.

scholarly journals Clinical phenotypes and outcomes of pulmonary hypertension due to left heart disease: role of the pre-capillary component

2017 ◽  
Author(s):  
Sergio Caravita ◽  
Andrea Faini ◽  
Antoine Bondue ◽  
Robert Naeije ◽  
Gianfranco Parati ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Left Heart ◽  
Clinical Phenotypes ◽  
Left Heart Disease

scholarly journals Pulmonary Hypertension Due to Left Heart Disease

Hypertension ◽  
2020 ◽  
Vol 75 (6) ◽  
pp. 1397-1408 ◽  
Author(s):  
Mohammed S. Al-Omary ◽  
Stuart Sugito ◽  
Andrew J. Boyle ◽  
Aaron L. Sverdlov ◽  
Nicholas J. Collins
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Left Atrial ◽  
Left Atrial Pressure ◽  
Left Heart ◽  
Pulmonary Arterial

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.

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

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

scholarly journals The pathophysiology of pulmonary hypertension in left heart disease

2015 ◽  
Vol 309 (9) ◽  
pp. L924-L941 ◽  
Author(s):  
Siegfried Breitling ◽  
Krishnan Ravindran ◽  
Neil M. Goldenberg ◽  
Wolfgang M. Kuebler
Keyword(s):  
Heart Failure ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Fluid Transport ◽  
Poor Response ◽  
Left Heart ◽  
Left Heart Disease ◽  
Wide Range ◽  
Pulmonary Arterial ◽  
Preclinical Animal Models

Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial pressure leading to right-sided heart failure and can arise from a wide range of etiologies. The most common cause of PH, termed Group 2 PH, is left-sided heart failure and is commonly known as pulmonary hypertension with left heart disease (PH-LHD). Importantly, while sharing many clinical features with pulmonary arterial hypertension (PAH), PH-LHD differs significantly at the cellular and physiological levels. These fundamental pathophysiological differences largely account for the poor response to PAH therapies experienced by PH-LHD patients. The relatively high prevalence of this disease, coupled with its unique features compared with PAH, signal the importance of an in-depth understanding of the mechanistic details of PH-LHD. The present review will focus on the current state of knowledge regarding the pathomechanisms of PH-LHD, highlighting work carried out both in human trials and in preclinical animal models. Adaptive processes at the alveolocapillary barrier and in the pulmonary circulation, including alterations in alveolar fluid transport, endothelial junctional integrity, and vasoactive mediator secretion will be discussed in detail, highlighting the aspects that impact the response to, and development of, novel therapeutics.

Pulmonary Hypertension Complicating Connective Tissue Disease

2017 ◽  
Vol 38 (05) ◽  
pp. 619-635 ◽  
Author(s):  
Rajan Saggar ◽  
John Belperio ◽  
Elizabeth Volkmann ◽  
Augustine Chung ◽  
Joseph Lynch
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Pulmonary Complications ◽  
Clinical Markers ◽  
Major Focus ◽  
Specific Autoantibody ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) may complicate connective tissue disease (CTD; particularly systemic sclerosis [scleroderma]), and is associated with increased mortality. More than 70% of cases of PH complicating CTD occur in patients with systemic sclerosis (SSc), which is the major focus of this article. Pulmonary complications (i.e., interstitial lung disease [ILD] and PH) are the leading causes of SSc-related deaths. “Isolated” PH (i.e., without ILD) complicates SSc in 7.5 to 20% of cases; secondary PH may also occur in patients with SSc-associated ILD. Several clinical markers and specific autoantibody profiles have been associated with PH in SSc. The role of PH-specific therapy in improving CTD-PH outcomes is under investigation, as prognosis and responsiveness to therapy appear to be worse in SSc-associated PH compared with idiopathic pulmonary arterial hypertension. We discuss medical therapies for CTD-associated PH and the role of lung transplantation for patients who fail medical therapy.

Pulmonary hypertension due to left heart disease with pulmonary arterial wedge pressure ≤15 mm Hg

Herz ◽  
2020 ◽  
Author(s):  
Dongxu Xu ◽  
Hao Zhang ◽  
Huiling Cheng ◽  
Tianbao Xu ◽  
Wei Sun ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Left Heart ◽  
Left Heart Disease ◽  
Pulmonary Arterial ◽  
Wedge Pressure
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