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Revue Medicale de Liege Mar 2019Pulmonary arterial hypertension (PAH) is a rare vascular lung disease with a complex etiopathogeny characterized by an increased pulmonary arterial pressure of 25 mmHg...
Pulmonary arterial hypertension (PAH) is a rare vascular lung disease with a complex etiopathogeny characterized by an increased pulmonary arterial pressure of 25 mmHg or above assessed by right heart catheterization. The diagnosis is difficult due to the atypical presentation with shortness of breath requiring a sequential approach bringing at the end the clinician to perform a right heart catheterization. Nowadays, several therapies have proven to be efficient for treating PAH. Recently, international recommendations have moved to an initial combination therapy reducing the overall morbi-mortality of the patients. Therefore, early therapy appears to be a priority in PAH underlying the need for increasing the global knowledge around PAH.
Topics: Cardiac Catheterization; Humans; Hypertension, Pulmonary
PubMed: 30897313
DOI: No ID Found -
Pneumologie (Stuttgart, Germany) Nov 2023The recently published new European guidelines for diagnosis and treatment of pulmonary hypertension now offer the so far most extensive description of genetic testing...
The recently published new European guidelines for diagnosis and treatment of pulmonary hypertension now offer the so far most extensive description of genetic testing and counselling for pulmonary arterial hypertension patients. In addition, the importance of a clinical screening of healthy mutation carriers is highlighted as well as the genetic testing of patients with a suspicion of pulmonary veno-occlusive disease. We frame the respective parts of the guidelines on genetic testing and counselling in the context of recent data and provide comments. Finally, we give an outlook on novel molecular approaches starting from Sotatercept, addressing ion channels and novel therapeutic developments.
Topics: Humans; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary; Pulmonary Veno-Occlusive Disease
PubMed: 37963476
DOI: 10.1055/a-2145-4663 -
American Journal of Respiratory and... Sep 2022
Topics: Child; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Registries
PubMed: 35737949
DOI: 10.1164/rccm.202206-1180ED -
The European Respiratory Journal Mar 2011The characteristics of pulmonary arterial hypertension (PAH), including pathology, symptoms, diagnosis and treatment are reviewed in children and adults. The... (Comparative Study)
Comparative Study Review
The characteristics of pulmonary arterial hypertension (PAH), including pathology, symptoms, diagnosis and treatment are reviewed in children and adults. The histopathology seen in adults is also observed in children, although children have more medial hypertrophy at presentation. Both populations have vascular and endothelial dysfunction. Several unique disease states are present in children, as lung growth abnormalities contribute to pulmonary hypertension. Although both children and adults present at diagnosis with elevations in pulmonary vascular resistance and pulmonary artery pressure, children have less heart failure. Dyspnoea on exertion is the most frequent symptom in children and adults with PAH, but heart failure with oedema occurs more frequently in adults. However, in idiopathic PAH, syncope is more common in children. Haemodynamic assessment remains the gold standard for diagnosis, but the definition of vasoreactivity in adults may not apply to young children. Targeted PAH therapies approved for adults are associated with clinically meaningful effects in paediatric observational studies; children now survive as long as adults with current treatment guidelines. In conclusion, there are more similarities than differences in the characteristics of PAH in children and adults, resulting in guidelines recommending similar diagnostic and therapeutic algorithms in children (based on expert opinion) and adults (evidence-based).
Topics: Adult; Algorithms; Anticoagulants; Cardiology; Child; Evidence-Based Medicine; Familial Primary Pulmonary Hypertension; Heart Diseases; Humans; Hypertension, Pulmonary; Models, Genetic; Pediatrics; Quality of Life; Treatment Outcome
PubMed: 21357924
DOI: 10.1183/09031936.00056110 -
European Respiratory Review : An... Dec 2016http://ow.ly/1RWN305ibQ3
http://ow.ly/1RWN305ibQ3
Topics: Arterial Pressure; Humans; Hypertension, Pulmonary; Prognosis; Pulmonary Artery; Risk Factors
PubMed: 27903657
DOI: 10.1183/16000617.0102-2016 -
Journal of the American Heart... Jun 2021Pulmonary hypertension (PH) attributable to left heart disease (LHD) is believed to be the most common form of PH and is strongly associated with increased mortality and... (Review)
Review
Pulmonary hypertension (PH) attributable to left heart disease (LHD) is believed to be the most common form of PH and is strongly associated with increased mortality and morbidity in this patient population. Specific therapies for PH-LHD have not yet been identified and the use of pulmonary artery hypertension-targeted therapies in PH-LHD are not recommended. Endothelin receptor antagonists, phosphodiesterase-5 inhibitors, guanylate cyclase stimulators, and prostacyclins have all been studied in PH-LHD with conflicting results. Understanding the mechanisms underlying PH-LHD could potentially provide novel therapeutic targets. Fibrosis, oxidative stress, and metabolic syndrome have been proposed as pathophysiological components of PH-LHD. Genetic associations have also been identified, offering additional mechanisms with biological plausibility. This review summarizes the evidence and challenges for treatment of PH-LHD and focuses on underlying mechanisms on the horizon that could develop into potential therapeutic targets for this disease.
Topics: Disease Management; Endothelin Receptor Antagonists; Guanylate Cyclase; Humans; Hypertension, Pulmonary; Oxidative Stress; Phosphodiesterase 5 Inhibitors; Prognosis; Pulmonary Wedge Pressure; Ventricular Dysfunction, Left; Ventricular Function, Left
PubMed: 34032129
DOI: 10.1161/JAHA.120.020633 -
Arquivos Brasileiros de Cardiologia 2019The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of... (Review)
Review
The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of PH. When direct involvement of pulmonary circulation is suspected, invasive hemodynamic monitoring is recommended to establish the diagnosis. This assessent provides, in addition to the diagnostic confirmation, the correct identification of the vascular territory predominantly involved (arterial pulmonary or postcapillary). Treatment with specific medication for PH (phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues) has been proven effective in patients with pulmonary arterial hypertension, but its use in patients with PH due to left heart disease can even be damaging. In this review, we discuss the diagnosis criteria, how etiological investigation should be carried out, the clinical classification and, finally, the therapeutic recommendations for PH.
Topics: Echocardiography; Heart Diseases; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Pulmonary Disease, Chronic Obstructive; Risk Assessment
PubMed: 31621783
DOI: 10.5935/abc.20190188 -
Allergology International : Official... Dec 2011Pulmonary hypertension was defined as mean pulmonary artery pressure ≥ 25 mmHg at the 4th World Symposium on Pulmonary Hypertension. In 2009, the European Society of... (Review)
Review
Pulmonary hypertension was defined as mean pulmonary artery pressure ≥ 25 mmHg at the 4th World Symposium on Pulmonary Hypertension. In 2009, the European Society of Cardiology and European Respiratory Society jointly created guidelines for practical pulmonary hypertension classifications and treatments based on the discussions at the 4th World Symposium. This classification is characterized by division into five groups: Pulmonary arterial hypertension (PAH); Pulmonary hypertension due to left heart disease; Pulmonary hypertension due to lung disease and/or hypoxia; Chronic thromboembolic pulmonary hypertension; and Pulmonary hypertension with unclear and/or multifactorial mechanisms. PAH is a common and fatal complication of connective tissue disease (CTD), but pulmonary hypertension in CTD consists of PAH, pulmonary hypertension caused by myocardial involvement, pulmonary veno-occlusive disorder, pulmonary hypertension due to interstitial lung disease. PAH has been studied widely in SSc and the estimated prevalence of 7-12%. Treatment of CTD associated PAH (CTD-PAH) consists of general therapeutic options and specific treatment. Specific treatment of CTD-PAH patients is targeted to produce vasodilatation. Calcium channel blockers (CCBs) are indicated in cases where a sufficient decrease in pulmonary arterial pressure is seen in vasoreactivity testing. If vasoreactivity is absent in CTD-PAH patients, the treatment consists of the endothelin receptor antagonists, the prostacyclin analogues and phosphodiesterase-type 5 inhibitors. Few data are available to support the use of immunosuppression in CTD-PAH. However, some case reports suggested that a minority of CTD-PAH patients could benefit from immunosuppressive therapy. The treatment of CTD-PAH patients may differ from the treatment of idiopathic PAH.
Topics: Connective Tissue Diseases; Evidence-Based Medicine; Humans; Hypertension, Pulmonary
PubMed: 22015568
DOI: 10.2332/allergolint.11-RAI-0362 -
European Respiratory Review : An... Dec 2010
Topics: Epoprostenol; Humans; Hypertension, Pulmonary; Prognosis; Severity of Illness Index; World Health Organization
PubMed: 21119183
DOI: 10.1183/09059180.00009510 -
Congestive Heart Failure (Greenwich,... 2011Pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequently encountered in patients with advanced heart failure (HF). Both conditions aggravate... (Review)
Review
Pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequently encountered in patients with advanced heart failure (HF). Both conditions aggravate prognosis and influence clinical decisions. Echocardiography is the screening tool of choice for pulmonary pressures and RV function, although invasive assessment of PH is necessary when advanced therapies are considered. Reversibility of PH in response to short-term pharmacologic treatment or even to long-term unloading after left ventricular assist device (LVAD) implantation is a favorable prognostic sign for both medically treated patients and heart transplant candidates. Although patients with severe PH secondary to HF have not derived benefit from pulmonary arterial hypertension therapies thus far, agents that modulate the cyclic guanosine monophosphate pathway, including phosphodiesterase 5A inhibitors, hold promise and are being actively investigated in advanced HF. Therapies that lead to reduction in left-sided pressures, including cardiac resynchronization and LVAD placement, also have a favorable effect on pulmonary pressures and RV function. However, no specific medical treatment for RV dysfunction exists to date, highlighting an important gap in the management of patients with advanced HF.
Topics: Comorbidity; Heart Failure; Humans; Hypertension, Pulmonary; Phosphodiesterase 5 Inhibitors; Prevalence; Prognosis; Vascular Resistance; Ventricular Dysfunction, Left; Ventricular Dysfunction, Right; Ventricular Function, Right
PubMed: 21790969
DOI: 10.1111/j.1751-7133.2011.00234.x