-
American Family Physician Sep 2016Pulmonary hypertension is a common, complex group of disorders that result from different pathophysiologic mechanisms but are all defined by a mean pulmonary arterial...
Pulmonary hypertension is a common, complex group of disorders that result from different pathophysiologic mechanisms but are all defined by a mean pulmonary arterial pressure of 25 mm Hg or greater. Patients often initially present to family physicians; however, because the symptoms are typically nonspecific or easily attributable to comorbid conditions, diagnosis can be challenging and requires a stepwise evaluation. There is limited evidence to support screening of asymptomatic individuals. Echocardiography is recommended as the initial step in the evaluation of patients with suspected pulmonary hypertension. A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization. For certain categories of pulmonary hypertension, particularly pulmonary arterial hypertension, treatment options are rapidly evolving, and early diagnosis and prompt referral to an expert center are critical to ensure the best prognosis. There are no directed therapies for many other categories of pulmonary hypertension; therefore, family physicians have a central role in managing contributing comorbidities. Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health.
Topics: Electrocardiography; Humans; Hypertension, Pulmonary; Practice Guidelines as Topic
PubMed: 27637122
DOI: No ID Found -
Methodist DeBakey Cardiovascular Journal 2021
Topics: Humans; Hypertension, Pulmonary
PubMed: 34326926
DOI: 10.14797/IAME5992 -
Journal of the American College of... Dec 2013Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and... (Review)
Review
Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have permitted comparisons between populations in different eras and environments. Inclusion of patients with all subtypes of PAH has also allowed comparisons of these subpopulations. We describe herein the basic methodology by which PAH registries have been conducted, review key insights provided by registries, summarize issues related to interpretation and comparison of the results, and discuss the utility of data to predict survival outcomes. Potential sources of bias, particularly related to the inclusion of incident and/or prevalent patients and missing data, are addressed. A fundamental observation of current registries is that survival in the modern treatment era has improved compared with that observed previously and that outcomes among PAH subpopulations vary substantially. Continuing systematic clinical surveillance of PAH will be important as treatment evolves and as understanding of mechanisms advance. Considerations for future directions of registry studies include enrollment of a broader population of patients with pulmonary hypertension of all clinical types and severity and continued globalization and collaboration of registry databases.
Topics: Animals; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Predictive Value of Tests; Registries
PubMed: 24355642
DOI: 10.1016/j.jacc.2013.10.023 -
Reumatismo Jan 2021The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance...
The term pulmonary arterial hypertension (PAH) identifies a heterogeneous group of diseases characterized by a progressive increase in pulmonary arterial resistance (PVR), which causes a significant burden in terms of quality of life, right heart failure and premature death. The pathogenesis of PAH is not completely clear: the remodeling of the small pulmonary vessels is crucial, causing an increase in the resistance of the pulmonary circle. Its diagnosis is based on cardiac catheterization of the right heart. According to the present hemodynamic definition of pulmonary hypertension (PH) proposed by the Guidelines of the European Society of Cardiology/European Respiratory Society (ESC-ERS), the mean pulmonary arterial pressure (mPAP) values are ≥25 mmHg. In case of PAH, apart from an mPAP value ≥25 mmHg, patients must have a >3 Wood units increase in PVR and normal pressure values of the left heart. PH is a pathophysiological condition observed in more than 40 different diseases, while PAH is a primary disease of the pulmonary bloodstream potentially treatable with specific drugs. PAH is a severe complication of systemic sclerosis (SSc) affecting about 10% of the patients. Due to the devastating nature of SSc-PAH, there is a clear need to systematically adopt appropriate screening programs. In fact, despite awareness of the negative impact of SSc-PAH on quality of life and survival, as well as on the severity of lung function, at the moment standardized and shared guidelines and/or screening programs for the diagnosis and the subsequent early treatment of PAH in SSc are not available. The aim of the present paper is to highlight the lights and shadows of SSc-PAH, unraveling the unmet clinical needs on this topic with a proposal of clinical-diagnostic and therapeutic guidelines.
Topics: Hemodynamics; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Quality of Life; Scleroderma, Systemic
PubMed: 33677950
DOI: 10.4081/reumatismo.2020.1310 -
Blood Feb 2016Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery... (Review)
Review
Pulmonary hypertension affects ∼10% of adult patients with sickle cell disease (SCD), particularly those with the homozygous genotype. An increase in pulmonary artery systolic pressure, estimated noninvasively by echocardiography, helps identify SCD patients at risk for pulmonary hypertension, but definitive diagnosis requires right-heart catheterization. About half of SCD-related pulmonary hypertension patients have precapillary pulmonary hypertension with potential etiologies of (1) a nitric oxide deficiency state and vasculopathy consequent to intravascular hemolysis, (2) chronic pulmonary thromboembolism, or (3) upregulated hypoxic responses secondary to anemia, low O2 saturation, and microvascular obstruction. The remainder have postcapillary pulmonary hypertension secondary to left ventricular dysfunction. Although the pulmonary artery pressure in SCD patients with pulmonary hypertension is only moderately elevated, they have a markedly higher risk of death than patients without pulmonary hypertension. Guidelines for diagnosis and management of SCD-related pulmonary hypertension were published recently by the American Thoracic Society. Management of adults with sickle-related pulmonary hypertension is based on anticoagulation for those with thromboembolism; oxygen therapy for those with low oxygen saturation; treatment of left ventricular failure in those with postcapillary pulmonary hypertension; and hydroxyurea or transfusions to raise the hemoglobin concentration, reduce hemolysis, and prevent vaso-occlusive events that cause additional increases in pulmonary pressure. Randomized trials have not identified drugs to lower pulmonary pressure in SCD patients with precapillary pulmonary hypertension. Patients with hemodynamics of pulmonary arterial hypertension should be referred to specialized centers and considered for treatments known to be effective in other forms of pulmonary arterial hypertension. There have been reports that some of these treatments improve SCD-related pulmonary hypertension.
Topics: Adult; Anemia, Sickle Cell; Homozygote; Humans; Hypertension, Pulmonary
PubMed: 26758918
DOI: 10.1182/blood-2015-08-618561 -
Journal of the American College of... Dec 2013Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of... (Review)
Review
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.
Topics: Age Factors; Animals; Child; Humans; Hypertension, Pulmonary; Incidence; Infant; Infant, Newborn; Netherlands
PubMed: 24355636
DOI: 10.1016/j.jacc.2013.10.028 -
Internal Medicine Journal Jan 2023Pulmonary hypertension (PH) is a common and debilitating medical condition with high mortality. PH research has traditionally focused on pulmonary arterial hypertension... (Review)
Review
Pulmonary hypertension (PH) is a common and debilitating medical condition with high mortality. PH research has traditionally focused on pulmonary arterial hypertension and its management in expert PH centres. Other forms of PH such as PH associated with cardiac or respiratory disease are more common, less well-understood and associated with higher mortality. Epidemiology of PH in disadvantaged, remote and rural regions, remains largely undocumented. In this review, we discuss the unique challenges in identifying PH in rural and disadvantaged populations using the Top End region of the Northern Territory of Australia as an example. We propose a simple diagnostic approach, ideally suited to regions where resource allocation is scarce, using clinical skills, echocardiography, and an escalation algorithm. The brief history, epidemiology and current literature on PH are summarised to inform the busy clinicians. We highlight two case examples from the Top End to illustrate the challenges and potential solutions.
Topics: Humans; Hypertension, Pulmonary; Vulnerable Populations; Northern Territory; Echocardiography; Pulmonary Arterial Hypertension
PubMed: 35762199
DOI: 10.1111/imj.15860 -
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 -
European Respiratory Review : An... Dec 2019http://bit.ly/2OBpapG
http://bit.ly/2OBpapG
Topics: Familial Primary Pulmonary Hypertension; Female; Forecasting; Humans; Male; Prevalence; Pulmonary Arterial Hypertension; Registries; Severity of Illness Index
PubMed: 31852747
DOI: 10.1183/16000617.0128-2019 -
Medical Archives (Sarajevo, Bosnia and... Jul 2016Pulmonary hypertension is a pathophysiological state hemodynamically defined as the increase of the mean pulmonary arterial pressure above 25, or 30 mmHg at rest,... (Review)
Review
Pulmonary hypertension is a pathophysiological state hemodynamically defined as the increase of the mean pulmonary arterial pressure above 25, or 30 mmHg at rest, measured by catheterization of the right heart. Laboratory findings usually reveals polycythemia, the ECG right ventricle hypertrophy, and x-ray characteristic of diseased branches (echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-BNP hormones are potentially helpful tools in identifying PH). Echocardiography can be found the increase of the right atrium and ventricle, right ventricular hypertrophy, abnormal contraction of the interventricular septum, left ventricular diastolic dysfunction and decreased left ventricular size, with reduced volumes of systole and end diastole. Doppler confirming tricuspid regurgitation. Pharmacological therapy would represent a use: Calcium Channel Blockers, Prostacyclin Analogues, Endothelin Receptor Antagonists and Phosphodiesterase-5 Inhibitors. Alpha adrenergic antagonists, endothelial receptor subtype A (Bosentan, Tracller) with treatment of the underlying disease or anticongestive therapy, are recommended. In case of inadequate response to treatment with a specific drug, guidelines recommend the combined use of drugs from the basic three groups, using their synergism.
Topics: Humans; Hypertension, Pulmonary
PubMed: 27703295
DOI: 10.5455/medarh.2016.70.303-307