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American Journal of Respiratory and... Aug 2023
Topics: Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial
PubMed: 37343302
DOI: 10.1164/rccm.202306-1005ED -
Disease-a-month : DM Jul 2023Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/hemodynamic criteria that are a consequence of several etiologies. Systemic... (Review)
Review
Pulmonary Arterial Hypertension (PAH) is a clinical syndrome consisting of physiologic/hemodynamic criteria that are a consequence of several etiologies. Systemic Sclerosis (SSc), one of the most common causes of PAH, is an autoimmune disorder of the connective tissue leading to fibrosis that involves the skin, gastrointestinal tract, lungs, heart, kidney etc. SSc has an annual prevalence of one to five cases for every 1000 individuals and nearly 15 percent of all cases develop PAH. At its core, Pulmonary hypertension (PH) in SSc is an obliterative vasculopathy in small to medium-sized pulmonary arterioles. A host of other local and systemic mechanisms operate in concert to gradually alter the hemodynamics resulting in elevated pulmonary vascular resistance and thus right ventricular afterload. A diagnosis of PAH in SSc is virtually a death sentence, with studies reporting a mortality rate of 50 per cent in the 3 years of diagnosis. Therefore, developing and implementing a robust screening and diagnosis protocol is crucial in the fight against this pervasive disease. This review aims to summarize the current literature of PAH in SSc, with a special focus on the screening and diagnosis protocols, newer treatment options and prognostic indicators for the same.
Topics: Humans; Hypertension, Pulmonary; Scleroderma, Systemic; Prognosis
PubMed: 36163292
DOI: 10.1016/j.disamonth.2022.101468 -
Cardiology Clinics Feb 2022Pulmonary hypertension (PH) due to left heart disease (LHD; group 2 PH) is a common complication of heart failure with reduced ejection fraction and heart failure with... (Review)
Review
Pulmonary hypertension (PH) due to left heart disease (LHD; group 2 PH) is a common complication of heart failure with reduced ejection fraction and heart failure with preserved ejection fraction and is often related to disease severity and duration of these diseases. PH due to LHD is associated with negative impact on outcomes in addition to worse symptoms and exercise capacity. Risk factors for group 2 PH are older age, hypertension, atrial fibrillation, and features of metabolic syndrome. The main mechanisms for group 2 PH are believed to be vascular remodeling secondary to sustained elevated intravascular pressure.
Topics: Aged; Heart Diseases; Heart Failure; Humans; Hypertension, Pulmonary; Risk Factors; Stroke Volume; Ventricular Dysfunction, Left
PubMed: 34809918
DOI: 10.1016/j.ccl.2021.08.007 -
Current Opinion in Cardiology Mar 2021Pulmonary hypertension (PH) occurs frequently in heart failure (HF) and confers worse prognosis. It becomes important to adequately identify these patients to optimize... (Review)
Review
PURPOSE OF REVIEW
Pulmonary hypertension (PH) occurs frequently in heart failure (HF) and confers worse prognosis. It becomes important to adequately identify these patients to optimize treatment. The purpose of this review is to inform about the updated classification of PH in left heart disease, in addition to current and upcoming trials regarding treatment.
RECENT FINDINGS
The updated classification of PH due to left heart disease now utilizes pulmonary vascular resistance instead of diastolic pulmonary gradient to differentiate between isolated postcapillary and combined pre and postcapillary PH. In regards to treatment, recent clinical trials continue to provide data that pulmonary vasodilators do not improve outcomes in this population.
SUMMARY
Management of underlying heart disease and optimal control of comorbidities continues to be the mainstay of treatment in PH due to HF. At this time, current data does not support the use of PH-directed therapies.
Topics: Diastole; Heart Diseases; Heart Failure; Humans; Hypertension, Pulmonary; Vascular Resistance
PubMed: 33394713
DOI: 10.1097/HCO.0000000000000834 -
Heart Failure Clinics Jan 2023Biological sex and sociocultural gender are emerging as pivotal modifiers of health and diseases. Sex-based differences exist in the development, pathogenesis, and... (Review)
Review
Biological sex and sociocultural gender are emerging as pivotal modifiers of health and diseases. Sex-based differences exist in the development, pathogenesis, and management of individuals with pulmonary arterial hypertension (PAH). The interplay between gender domains (ie, identity, roles, relations, and institutionalized gender) and PAH has been barely investigated. The aim of this narrative review is to describe up-to-date evidence on the integration of sex and gender in PAH research, highlighting areas for future investigation.
Topics: Male; Female; Humans; Hypertension, Pulmonary; Sex Characteristics
PubMed: 36435566
DOI: 10.1016/j.hfc.2022.09.002 -
Methodist DeBakey Cardiovascular Journal 2024Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance. Females have a higher incidence of PAH, which is reflected globally across registries in the United States, Europe, and Asia. However, despite female predominance, women had better outcomes compared with male patients, a finding that has been labeled the "estrogen paradox." Special considerations should be given to women with PAH regarding sexual health, contraception, family planning, and treatment before, during, and after pregnancy. Pregnant women with PAH should be referred to a pulmonary hypertension care center; a multidisciplinary team approach is recommended, and Cesarean section is the preferred mode of delivery. While pregnancy outcomes have improved over the years with PAH-specific therapy, pregnancy portends a high-risk for those with PAH. Continued research is needed to tailor PAH treatment for women.
Topics: Female; Pregnancy; Humans; Male; Hypertension, Pulmonary; Cesarean Section; Pregnancy Outcome; Familial Primary Pulmonary Hypertension; Hypertension
PubMed: 38495664
DOI: 10.14797/mdcvj.1308 -
Methodist DeBakey Cardiovascular Journal 2021Pulmonary hypertension (PH) is a rare heterogenous disease characterized by elevated blood pressure in the lungs. Patients with PH require careful evaluation and... (Review)
Review
Pulmonary hypertension (PH) is a rare heterogenous disease characterized by elevated blood pressure in the lungs. Patients with PH require careful evaluation and management at an expert center. Understanding of the mechanisms underlying the development of PH has increased over the past two decades, and several treatment options for pulmonary arterial hypertension have emerged. Despite this progress, PH continues to carry high morbidity and mortality. The 6th World Symposium on Pulmonary Hypertension that occurred in late 2018 modified the clinical classification of PH into five groups. In this review, we focus on the evaluation and diagnosis of PH and discuss the updated clinical classification.
Topics: Humans; Hypertension, Pulmonary
PubMed: 34326927
DOI: 10.14797/OCDF4453 -
Vascular Pharmacology Dec 2022Pulmonary arterial hypertension is a rare dyspnea-fatigue syndrome defined by an increase in mean pulmonary artery pressure above 20 mmHg combined with an increase in...
Pulmonary arterial hypertension is a rare dyspnea-fatigue syndrome defined by an increase in mean pulmonary artery pressure above 20 mmHg combined with an increase in pulmonary vascular resistance higher than 2 Wood units. The condition is of poor prognosis and still incurable in spite of progress achieved in recent decades. The approach is currently optimized by multi-drug combinations titrated on serial risk assessments using recently validated scores. In this issue of Vascular Pharmacology argument is made based on retrospective registry data from three reference centers in favor of initial multi-drug therapies including a parenteral prostanoid dosed to decrease mPAP to normal. This objective was achieved in only a minority of patients, but improved outcome was demonstrated when mPAP can be brought to below 35 mmHg. This data suggest that pulmonary artery pressure-directed multi-drug therapies in PAH may reverse right heart remodeling and limit progression, or even reverse pulmonary vascular disease. However, further studies are needed to validate mPAP as a primary endpoint in PAH drug trials. In the meantime, aggressive initial prescription of parenteral prostanoids combined with one or two oral drugs targeting the pulmonary circulation under careful clinical, imaging and hemodynamic follow-up may be the best therapeutic strategy.
Topics: Humans; Pulmonary Arterial Hypertension; Hypertension, Pulmonary; Pulmonary Artery; Retrospective Studies; Vascular Resistance
PubMed: 36270620
DOI: 10.1016/j.vph.2022.107124 -
Current Opinion in Pulmonary Medicine Sep 2023The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis.
RECENT FINDINGS
The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme.
SUMMARY
Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Prognosis; Familial Primary Pulmonary Hypertension; Echocardiography; Ventricular Dysfunction, Right
PubMed: 37410491
DOI: 10.1097/MCP.0000000000000980 -
Clinics in Chest Medicine Mar 2021Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in... (Review)
Review
Presently, with increasing survival of patients with congenital heart disease (CHD), pulmonary arterial hypertension (PAH) associated with CHD is commonly encountered in children and adults. This increased prevalence is seen despite significant advances in early diagnosis and surgical correction of patients with structural CHD. PAH is the cause of significant morbidity and mortality in these patients and comes in many forms. With the increased availability of targeted therapies for PAH, there is hope for improved hemodynamics, exercise capacity, quality of life, and possibly survival for these patients. There may also be opportunities for combined medical and interventional/surgical approaches for some.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; Exercise; Heart Defects, Congenital; Hemodynamics; Humans; Hypertension, Pulmonary; Longevity; Middle Aged; Patients; Quality of Life; Young Adult
PubMed: 33541620
DOI: 10.1016/j.ccm.2020.11.005