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Circulation. Heart Failure Jul 2023Patients with pulmonary hypertension associated with congenital heart disease make up an increasing proportion of the total pulmonary hypertension population who bring... (Review)
Review
Patients with pulmonary hypertension associated with congenital heart disease make up an increasing proportion of the total pulmonary hypertension population who bring with them added complexity because of underlying anatomical and hemodynamic abnormalities. Currently, no consensus recommendations are available on how to best manage this group of patients for either the primary cardiologist or pulmonary hypertension subspecialist, including timing of referral. The purposes of this document are (1) to describe the various pulmonary hypertension groups and subgroups associated with congenital heart disease, (2) to describe imaging modalities used in patient evaluation, (3) to elucidate medical and surgical management considerations, (4) to highlight disparities within this population, and (5) to identify gaps and future research needs of patients with pulmonary hypertension associated with congenital heart disease.
Topics: United States; Humans; Hypertension, Pulmonary; American Heart Association; Heart Failure; Heart Defects, Congenital; Hemodynamics
PubMed: 37357777
DOI: 10.1161/HHF.0000000000000080 -
Clinics in Chest Medicine Mar 2021Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary vascular disease (CTED) are rare manifestations of venous thromboembolism.... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary vascular disease (CTED) are rare manifestations of venous thromboembolism. Presumably, CTEPH and CTED are variants of the same pathophysiological mechanism. CTEPH and CTED can be near-cured by pulmonary endarterectomy, balloon pulmonary angioplasty, and medical treatment with Riociguat or subcutaneous treprostinil, which are the approved drugs.
Topics: Chronic Disease; Humans; Hypertension, Pulmonary; Pulmonary Embolism; Venous Thromboembolism
PubMed: 33541619
DOI: 10.1016/j.ccm.2020.11.014 -
European Heart Journal Mar 2016In patients with left ventricular heart failure (HF), the development of pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequent and have... (Review)
Review
In patients with left ventricular heart failure (HF), the development of pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequent and have important impact on disease progression, morbidity, and mortality, and therefore warrant clinical attention. Pulmonary hypertension related to left heart disease (LHD) by far represents the most common form of PH, accounting for 65-80% of cases. The proper distinction between pulmonary arterial hypertension and PH-LHD may be challenging, yet it has direct therapeutic consequences. Despite recent advances in the pathophysiological understanding and clinical assessment, and adjustments in the haemodynamic definitions and classification of PH-LHD, the haemodynamic interrelations in combined post- and pre-capillary PH are complex, definitions and prognostic significance of haemodynamic variables characterizing the degree of pre-capillary PH in LHD remain suboptimal, and there are currently no evidence-based recommendations for the management of PH-LHD. Here, we highlight the prevalence and significance of PH and RV dysfunction in patients with both HF with reduced ejection fraction (HFrEF) and HF with preserved ejection fraction (HFpEF), and provide insights into the complex pathophysiology of cardiopulmonary interaction in LHD, which may lead to the evolution from a 'left ventricular phenotype' to a 'right ventricular phenotype' across the natural history of HF. Furthermore, we propose to better define the individual phenotype of PH by integrating the clinical context, non-invasive assessment, and invasive haemodynamic variables in a structured diagnostic work-up. Finally, we challenge current definitions and diagnostic short falls, and discuss gaps in evidence, therapeutic options and the necessity for future developments in this context.
Topics: Heart Failure; Hemodynamics; Humans; Hypertension, Pulmonary; Mitral Valve Insufficiency; Phenotype; Pulmonary Circulation; Ventricular Dysfunction, Left
PubMed: 26508169
DOI: 10.1093/eurheartj/ehv512 -
International Journal of Environmental... Feb 2021Alveolar hypoxia is the most prominent feature of high altitude environment with well-known consequences for the cardio-pulmonary system, including development of... (Review)
Review
Alveolar hypoxia is the most prominent feature of high altitude environment with well-known consequences for the cardio-pulmonary system, including development of pulmonary hypertension. Pulmonary hypertension due to an exaggerated hypoxic pulmonary vasoconstriction contributes to high altitude pulmonary edema (HAPE), a life-threatening disorder, occurring at high altitudes in non-acclimatized healthy individuals. Despite a strong physiologic rationale for using vasodilators for prevention and treatment of HAPE, no systematic studies of their efficacy have been conducted to date. Calcium-channel blockers are currently recommended for drug prophylaxis in high-risk individuals with a clear history of recurrent HAPE based on the extensive clinical experience with nifedipine in HAPE prevention in susceptible individuals. Chronic exposure to hypoxia induces pulmonary vascular remodeling and development of pulmonary hypertension, which places an increased pressure load on the right ventricle leading to right heart failure. Further, pulmonary hypertension along with excessive erythrocytosis may complicate chronic mountain sickness, another high altitude maladaptation disorder. Importantly, other causes than hypoxia may potentially underlie and/or contribute to pulmonary hypertension at high altitude, such as chronic heart and lung diseases, thrombotic or embolic diseases. Extensive clinical experience with drugs in patients with pulmonary arterial hypertension suggests their potential for treatment of high altitude pulmonary hypertension. Small studies have demonstrated their efficacy in reducing pulmonary artery pressure in high altitude residents. However, no drugs have been approved to date for the therapy of chronic high altitude pulmonary hypertension. This work provides a literature review on the role of pulmonary hypertension in the pathogenesis of acute and chronic high altitude maladaptation disorders and summarizes current knowledge regarding potential treatment options.
Topics: Altitude; Altitude Sickness; Humans; Hypertension, Pulmonary; Hypoxia; Pulmonary Edema
PubMed: 33578749
DOI: 10.3390/ijerph18041692 -
European Journal of Clinical... Apr 2021Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic... (Review)
Review
Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be.
Topics: Connective Tissue Diseases; Dermatomyositis; Disease Management; Humans; Hypertension, Pulmonary; Lupus Erythematosus, Systemic; Mass Screening; Mixed Connective Tissue Disease; Prognosis; Pulmonary Arterial Hypertension; Scleroderma, Systemic; Sjogren's Syndrome
PubMed: 33216992
DOI: 10.1111/eci.13453 -
Medicina (Kaunas, Lithuania) Aug 2019Pulmonary hypertension (PH) is a frequently encountered complication of chronic obstructive pulmonary disease (COPD) and is associated with worsened clinical symptoms... (Review)
Review
Pulmonary hypertension (PH) is a frequently encountered complication of chronic obstructive pulmonary disease (COPD) and is associated with worsened clinical symptoms and prognosis. The prevalence of PH-COPD is not concretely established as classification criteria vary historically, but the presence of severe disease out of proportion to underlying COPD is relatively rare. Right heart catheterization, the gold standard in diagnosis of PH, is infrequently performed in COPD, and the overlap in the clinical symptoms of PH and COPD presents diagnostic challenges. Proven treatments are limited. Trials exploring the use of vasodilator therapy in this patient group generally demonstrate improvements in hemodynamics accompanied by worsening gas exchange without clearly demonstrated improvements in clinically meaningful outcomes. In-depth workup of underlying pulmonary hypertension and use of pulmonary vasodilator medications may be appropriate on an individual basis. We present a case study and a review and discussion of the pertinent literature on this topic.
Topics: Case-Control Studies; Humans; Hypertension, Pulmonary; Prognosis; Pulmonary Disease, Chronic Obstructive
PubMed: 31382489
DOI: 10.3390/medicina55080432 -
European Respiratory Review : An... Sep 2021Pulmonary hypertension (PH) commonly affects patients with systemic sclerosis (SSc) and is associated with significant morbidity and increased mortality. PH is a... (Review)
Review
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.
Topics: Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial; Phenotype; Pulmonary Arterial Hypertension; Scleroderma, Systemic
PubMed: 34407977
DOI: 10.1183/16000617.0053-2021 -
Clinical Medicine (London, England) Sep 2023Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely... (Review)
Review
Pulmonary hypertension (PH) is common, with an estimated prevalence of approximately 1% that increases with age. Prompt and accurate diagnosis is key to institute timely and appropriate therapy to improve symptoms and prognosis. The international guidelines for the diagnosis and management of PH have recently been updated, with a lowering of the haemodynamic threshold for diagnosis to a mean pulmonary artery pressure >20 mmHg. New diagnostic algorithms and revised indications for screening in at-risk groups have been developed to facilitate early referral to specialist PH centres. This includes fast-track referral pathways for patients who are either clinically high-risk or are at-risk for pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). This review summarises key changes in the PH guidelines for general physicians who are, most often, the first healthcare professionals to encounter these patients and consequently have a key role as referrers into specialist PH services.
Topics: Humans; Hypertension, Pulmonary; Prognosis; Physicians; Chronic Disease
PubMed: 37775164
DOI: 10.7861/clinmed.2023-23.5.Cardio4 -
Journal of the American College of... Sep 2020Pulmonary hypertension (PH) associated with left heart disease, or Group 2 PH, includes heart failure, valvular heart diseases, and congenital heart diseases. Although... (Review)
Review
Pulmonary hypertension (PH) associated with left heart disease, or Group 2 PH, includes heart failure, valvular heart diseases, and congenital heart diseases. Although it is axiomatic that in PH due to heart failure the increase in pulmonary pressure is directly related to an enhanced left atrial pressure, which is common to both heart failure with preserved ejection fraction (HFpEF) and heart failure with reduced ejection fraction (HFrEF), there has been limited attention over the years on the potential differences in terms of driving mechanisms, pathophysiology, and clinical phenotypes. Major differences between HFpEF and HFrEF are the underlying causes, associated comorbidities, and cardiac remodeling. This suggests that despite similar hemodynamic profiles, there may be some disparities in PH development. A focused knowledge on the differences between the 2 syndromes has relevant implications to seek new, personalized, and timely treatments for Group 2 PH. The purpose of the present review is to highlight the mechanisms and clinical phenotypes of PH in HFpEF and HFrEF.
Topics: Heart Failure; Hemodynamics; Humans; Hypertension, Pulmonary; Stroke Volume
PubMed: 32854845
DOI: 10.1016/j.jacc.2020.06.069 -
Journal of the American Heart... Apr 2023The definition of pulmonary hypertension (PH) has changed recently based, in part, on contemporary outcome data and to focus on early disease detection. Now, PH includes... (Review)
Review
The definition of pulmonary hypertension (PH) has changed recently based, in part, on contemporary outcome data and to focus on early disease detection. Now, PH includes patients with mean pulmonary artery pressure >20 mm Hg measured by right heart catheterization. In contrast to the classical era, pulmonary vascular resistance >2.0 Wood units is also used for diagnosis and prognostication. These lowered thresholds aim to identify patients early in the disease course, which is important because delay to diagnosis of PH is common and linked to elevated morbidity and shortened lifespan. This clinical primer highlights key changes in diagnosis and approach to PH management, focusing on concepts that are likely to be encountered frequently in general practice. Specifically, this includes hemodynamic assessment of at-risk patients, pharmacotherapeutic management of pulmonary arterial hypertension, approach to PH in patients with heart failure with preserved ejection fraction, and newly established indications for early referral to PH centers to prompt comanagement of patients with pulmonary vascular disease experts.
Topics: Humans; Hypertension, Pulmonary; Cardiac Catheterization; Hemodynamics; Vascular Resistance; Familial Primary Pulmonary Hypertension; Heart Failure
PubMed: 37026538
DOI: 10.1161/JAHA.122.029024