Materiały źródłowe

• #1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7797595/

  This systematic review of literature and online reports critically appraised incidence and prevalence estimates of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension to identify the most accurate estimates. […] Most studies were based on European data and very few included children. In adults, the range of estimates per million was approximately 20-fold for pulmonary arterial hypertension incidence (1.532) and prevalence (12.4268) and of similar magnitude for chronic thromboembolic pulmonary hypertension incidence (0.939) and prevalence (14.5144). […] These estimates were considered the most reliable and consistent for the scientific community to plan for resource allocation and improve detection rates. […] While awareness, diagnosis and clinical management of PAH and CTEPH have greatly improved over the past few decades, there is still a lack of consensus in the scientific community on which estimates could reflect the true incidence and prevalence of PAH and CTEPH.

• #2 Pulmonary Hypertension Surveillance

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4122278/

  Pulmonary hypertension (PH) is an uncommon but progressive condition, and much of what we know about it comes from specialized disease registries. […] With expanding research into the diagnosis and treatment of PH, it is important to provide updated surveillance on the impact of this disease on hospitalizations and mortality. This study, which builds on previous PH surveillance of mortality and hospitalization, analyzed mortality data from the National Vital Statistics System and data from the National Hospital Discharge Survey between 2001 and 2010. […] The decline in death rates associated with PH among men from 1980 to 2005 has reversed and now shows a significant increasing trend. Similarly, the death rates for women with PH have continued to increase significantly during the past decade.

• #3 Prevalence of Pulmonary Hypertension in the General Population: The Rotterdam Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130072

  Overall, we found that pulmonary hypertension as assessed by echocardiography has a prevalence of 2.6% in the general population in the Netherlands. The prevalence of ePH was higher in older persons, and in those with COPD, LV systolic or diastolic dysfunction. Factors independently associated with higher ePASP in a multivariate model were older age, higher BMI, left ventricular diastolic dysfunction, COPD and systemic hypertension. […] Given the population aging and the known association of PH with heart and lung diseases, an increasing PH prevalence could be expected.

• #4 Frontiers | The prevalence and survival of pulmonary hypertension due to left heart failure: A retrospective analysis of a multicenter prospective cohort study

  https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2022.908215/full

  Introduction: Pulmonary hypertension (PH) has become an increasingly common global health issue. It is estimated having the prevalence of about 1% of the global population increases up to 10% in individuals older than 65 years. […] The overall incidence of left heart failure (LHF) is increasing due to the rapid global rise in the number of people older than 65 years, LHF is becoming a leading cause of PH, affecting around 5% of individuals aged 65 years or older. […] Conclusion: Group 2 PH is commonly identified in patients with LHF and its prevalence is lower in patients with CAD than that without CAD. The mortality is still high in patients with PH-LHF, highlighting that the development of risk table to screen high-risk patients for closer monitoring and intensification of management may improve prognosis. FC, hemoglobin, and sPAP are independent risk predictors of mortality for PH-LHF. These findings may be useful for risk stratification in future clinical trial enrollment.

• #5 The epidemiology and pathogenesis of pulmonary arterial hypertension (group 1) – UpToDate

  https://www.uptodate.com/contents/the-epidemiology-and-pathogenesis-of-pulmonary-arterial-hypertension-group-1

  The epidemiology and pathogenesis of pulmonary arterial hypertension (group 1) […] Pulmonary hypertension (PH) is a disease characterized by elevated pulmonary artery pressure (mean pulmonary artery pressure ≥20 mmHg at rest). The World Health Organization (WHO) classifies patients with PH into five groups (table 1) based upon etiology: […] Group 1 – Pulmonary arterial hypertension (PAH) […] Group 2 – PH due to left heart disease […] Group 3 – PH due to chronic lung disease and/or hypoxemia […] Group 4 – PH due to pulmonary artery obstructions.

• #6 Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults – UpToDate

  https://www.uptodate.com/contents/pulmonary-hypertension-due-to-lung-disease-and-or-hypoxemia-group-3-pulmonary-hypertension-epidemiology-pathogenesis-and-diagnostic-evaluation-in-adults

  Pulmonary hypertension due to lung disease and/or hypoxemia (group 3 pulmonary hypertension): Epidemiology, pathogenesis, and diagnostic evaluation in adults […] Patients with pulmonary hypertension (PH) due to diffuse lung disease (eg, chronic obstructive pulmonary disease, interstitial lung disease, or overlap syndromes) or conditions that cause hypoxemia (eg, obstructive sleep apnea, alveolar hypoventilation disorders) are classified as having group 3 PH (table 1). […] The prevalence, pathogenesis, and diagnosis of PH due to lung disease and/or hypoxemia are presented here. The treatment and prognosis of patients with group 3 PH are reviewed separately. […] Classification – Patients with PH are classified into five groups based upon etiology. Patients in group 1 are considered to have pulmonary arterial hypertension (PAH; also sometimes referred to as pre-capillary PH), whereas patients in group 2 (due to left-sided heart disease), group 3 (due to chronic lung disorders and hypoxemia), group 4 (due to pulmonary artery obstructions), and group 5 (due to unidentified or mixed mechanisms) are considered to have PH (table 1). When all five groups are discussed collectively, the term PH is used.

• #7 Epidemiology and Disease Classification of Pulmonary Hypertension | SpringerLink

  https://link.springer.com/chapter/10.1007/978-1-4939-2636-7_2

  Numerous conditions lead to a sustained increase in pulmonary arterial pressure. Pulmonary hypertension is defined as an elevation of mean pulmonary arterial pressure to 25 mmHg or greater. Currently, the pulmonary hypertensive diseases are organized into five groups that include (1) pulmonary arterial hypertension, (2) pulmonary hypertension owing to left-sided heart disease, (3) pulmonary hypertension owing to chronic lung disease, (4) chronic thromboembolic pulmonary hypertension, and (5) miscellaneous causes. Group 2 and 3 represent the most common causes of pulmonary hypertension. Group 1 (PAH) is exceedingly rare but normally progresses to severe pulmonary hypertension and right ventricular failure. PAH is seen more frequently in patients with connective tissue disease, HIV infection, portal hypertension, congenital cardiac shunts, and those who have used amphetamine-like drugs.

• #8 Pulmonary Arterial Hypertension: Updates in Epidemiology and Evaluation of Patients

  https://www.ajmc.com/view/pulmonary-arterial-hypertension-updates-in-epidemiology-and-evaluation-of-patients

  PAH is a rare disorder found in 15 to 50 persons per million within the United States and Europe. Idiopathic, heritable, and anorexigen-induced PAH make up 52.6% of all PAH cases. Generally, PAH affects women aged between 30 and 60 years. However, it can occur in males and is often associated with worse clinical outcomes. The National Institutes of Health (NIH) was a landmark registry that collected PAH data between 1981 and 1985. This registry included 187 individuals having PAH of various etiologies. The registry largely consisted of females, found a mean age of PAH presentation of 36 years, and was primarily Caucasian. PAH-specific therapies were not available at this time, and registry participants had a median survival of 2.8 years (1 year, 68%; 3 year, 48%; and 5 year, 34%). […] […]

• #9

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7797595/

  The published estimates of PAH epidemiology in adults are summarised in Table 1. […] The ranges of estimates for PAH incidence and prevalence were 1.532 and 12.4268ppm, respectively. National systematic registries reported PAH adult incidence to be between 5.8 and 13.7ppm (four studies), while estimates from all non-systematic registries except one (BNP-PL; 5.2ppm) were below 4ppm (1.53.9ppm; seven studies). […] The ranges of CTEPH incidence and prevalence in adults were 0.939ppm and 14.5144ppm, respectively. […] According to national systematic registries (three studies), the incidence of CTEPH was between 3.1 and 6.0ppm and prevalence ranged from 15.7 to 38.4ppm.

• #10 Pulmonary Arterial Hypertension: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/303098-overview

  The overall prevalence of pulmonary arterial hypertension (PAH) is difficult to determine given the diseases heterogeneity and likely underdiagnosis. […] Worldwide, schistosomiasis is likely the most prevalent cause of PAH, with studies suggesting that over 7% of patients with hepatosplenic schistosomiasis have pulmonary hypertension. […] Data registries in areas without endemic schistosomiasis such as the United States and Europe report a PAH prevalence ranging from 6.6-26 cases per million adults. […] The majority of these cases are idiopathic. While approximately 10% are classified as heritable, it is likely that this number will increase with time as genetic testing becomes more widespread. […] Studies have also estimated the prevalence of specific subgroups of PAH. An observational study of 277 patients with HIV infection found that 0.46% of patients had pulmonary hypertension.

• #11 Global, regional, and national burden of pulmonary arterial hypertension, 1990–2021 | Institute for Health Metrics and Evaluation

  https://www.healthdata.org/research-analysis/library/global-regional-and-national-burden-pulmonary-arterial-hypertension-1990

  In 2021, there were 22,000 deaths from pulmonary arterial hypertension globally. […] This study reports the global burden of PAH, leveraging all available data and using methodology of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) to understand the epidemiology of this under-researched and morbid disease. […] In 2021, there were an estimated 192000 (95% uncertainty interval [UI] 155000236000) prevalent cases of PAH globally. […] In 2021, there were 22000 deaths (1820025400) attributed to PAH globally, with an age-standardised mortality rate of 027 deaths from PAH per 100000 population (023032). […] PAH is a rare but fatal disease that accounts for a considerable health-associated burden worldwide. PAH is disproportionally diagnosed among females and older adults.

• #12 Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry | BMC Pulmonary Medicine | Full Text

  https://bmcpulmmed.biomedcentral.com/articles/10.1186/1471-2466-14-45

  Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Epidemiological data from national registries are growing worldwide, but are still unavailable in Eastern Europe. […] This is the first study in Eastern Europe to describe the prevalence, incidence and survival of patients with PAH from a national representative registry. This registry from the Czech Republic highlights that diagnosis of PAH is frequently made late in the disease continuum when patients have significant functional impairment. […] The estimated incidence of PAH in adults in the Czech Republic was 10.7 cases per million persons overall in 2007 (7.5 per million men and 13.7 per million women). The estimated prevalence of PAH in the Czech adult population was 22.4 cases per million persons overall (16 per million men and 28.5 per million women).

• #13 Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

  https://www.mdpi.com/2077-0383/9/1/173

  Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. […] The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females ≥65 years old. […] A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. […] The mean prevalence of PAH was 30.8 per million adults and the estimated incidence rate was 5.2 per million adults per year. […] The present analysis of the BNP-PL registry shows demographics, treatment and the burden of coexisting diseases in a large group of Caucasian adults of Central-Eastern European origin with all types of PAH.

• #14 Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

  https://www.mdpi.com/2077-0383/9/1/173

  Both, the prevalence and incidence of PAH were the highest among older population which confirms the changing demographics of PAH found previously in the Western countries. […] Our study shows the epidemiology of PAH in a Central-Eastern post-communist European country with a relatively short history of availability of PAH therapies. […] It estimates the prevalence of adult PAH at a similar level to that observed in the Western countries but highlights heterogeneity in the geographic distribution of PAH, which suggests that an important proportion of patients may still have not been diagnosed.

• #15 :: KCJ :: Korean Circulation Journal

  https://e-kcj.org/DOIx.php?id=10.4070/kcj.2022.0231

  The incidence and survival of pulmonary arterial hypertension (PAH) in Korea is not well understood. […] We analyzed how the epidemiology and clinical features of PAH has changed through the rapidly advancing healthcare infrastructure in South Korea. […] The prevalence and incidence of PAH increased 75- and 12-fold, respectively. […] The proportion of patients on combination PAH-specific drug therapy has increased by 2.6-fold in 2018 compared to when 2007, when the prescription was first approved by the government. […] This nationwide study delineated that the prevalence and incidence of PAH have grown rapidly in Korea since the early 2000s. […] The 5-year survival rate and median survival duration were 71.8% and 13.1 years, respectively. […] The overall prevalence of PAH per million people has risen from 0.4 in 2002 to 29.9 in 2018 (75-fold increase).

• #16 :: KCJ :: Korean Circulation Journal

  https://e-kcj.org/DOIx.php?id=10.4070/kcj.2022.0231

  The incidence of PAH has increased over time regardless of sex or age. […] The overall incidence (per million person-years) increased by 12-fold between 2004 (0.5) and 2018 (6.3). […] The 5-year survival of incident PAH patients ranged from 67.0% to 78.6%, except in 2004 and 2005. […] The observed rapid increase in the prevalence and incidence of PAH in Korea is unprecedented when compared to other countries. […] The Korean government currently reimburses 90% of the medical costs for patients with PAH, which allows physicians to prescribe any type of PAH-specific drug that best suits the patient regardless of its cost. […] The findings were generally consistent in East Asian reports: 71.8% in Korea (current study), 20-73% in China, 74% in Japan, and 78% in Taiwan, suggesting that the prognosis of PAH in East Asian countries is now comparable to that in western countries. […] In conclusion, the prevalence and incidence of PAH and use of combination therapy have steadily increased in Korea since the early 2000s.

• #17 Pulmonary Arterial Hypertension: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/303098-overview

  In comparison with prior studies, no change in prevalence rate was seen with modern highly active antiretroviral treatment (HAART). […] In scleroderma patients, the incidence has been estimated to be 6-60% of all patients, with the variance based on the extent of disease. […] Women are more likely to have PAH, with registries reporting a 65-80% female predominance of the disease. […] Interestingly, while prior studies suggested a mean age of diagnosis in the thirties, current registries suggest a mean age of diagnosis in the fifties. […] Although PAH can affect all races, data from the US REVEAL registry suggest a white predominance (73% white vs 12% African American, 9% Latino, and 3% Asian).

• #18 Epidemiology and Disease Classification of Pulmonary Hypertension | Thoracic Key

  https://thoracickey.com/epidemiology-and-disease-classification-of-pulmonary-hypertension/

  Epidemiology and Disease Classification of Pulmonary Hypertension […] The first classification of PH was described in 1973 and categorized patients as having primary or secondary pulmonary hypertension according to the presence or absence of an identifiable cause for the disease. […] The updated classification for pulmonary hypertension, derived from the last world symposium on PH, is presented in Table 2.2. […] Our knowledge of the epidemiology of PAH has changed dramatically over the last 30 years. […] The French Registry confirmed the female predominance, but described a mean age at diagnosis of 50 years, with older patients presenting worse prognosis. […] Both registries also demonstrated disturbing data concerning PAH diagnosis. Although an increase in disease awareness is believed to have occurred during the last decade, the appropriate diagnosis still takes about 2 years with most of the patients being diagnosed in functional class III and IV. This is particularly alarming if one takes into consideration that the worse the functional class at the diagnosis, the worse the survival despite all available medical treatments. Therefore, late diagnosis is a true problem, more specifically for IPAH, and might be one of the scenarios to be changed in the near future, in order to better improve survival.

• #19 Pulmonary Arterial Hypertension: Updates in Epidemiology and Evaluation of Patients

  https://www.ajmc.com/view/pulmonary-arterial-hypertension-updates-in-epidemiology-and-evaluation-of-patients

  Significant progress in the field of PAH pathophysiology and treatment have occurred in the 2 decades since the NIH registry. Contemporary PAH registries vary in their study populations, study design, and cohorts. The 2002 French Network on Pulmonary Arterial Hypertension (French PAH) registry included 674 people with PAH. The French PAH registry found an estimated survival rate among those with idiopathic/familial/anorexigen-associated PAH of 82.9% at 1 year and 58.2% at 3 years. This is a markedly higher survival rate compared with the NIH registry but consistent with other more recent PAH registry findings. […] […] […] REVEAL (Registry to Evaluate Early and Long-Term PAH Disease Management) is a contemporary, multicenter, observational, US-based registry that began in 2006. In contrast to the NIH registry, REVEAL was specifically designed to ascertain demographics, longitudinal clinical course, and management of PAH from a current US perspective. Baseline characteristics for the 2967 individuals who met traditional hemodynamic criteria included a mean age of 53 (14 years) and female sex in 79.5% with a female-to-male ratio of 4.8:1. In addition, 46% of individuals had idiopathic PAH, 25% were associated with connective tissue diseases, and 10% were associated with congenital heart diseases. There was also a mean duration between symptom onset and diagnosis of 2.8 years. Results of the REVEAL Registry showed a 1-year survival rate of 91% among 2716 individuals who were enrolled consecutively. An additional analysis assessing long-term survival (N = 2635, between March 2006 and December 2009) found survival rates of 85% at year 3, 68% at year 5, and 49% at year 7 from time of diagnosis. These increases in survival were attributed to various factors, including changes in treatment, increased patient support, and potentially, a difference in the PAH population cohort.

• #20 Pulmonary Hypertension in Women | Methodist DeBakey Cardiovascular J

  https://journal.houstonmethodist.org/articles/10.14797/mdcvj.1308

  Pulmonary hypertension (PH) affects all age groups, with an estimated prevalence of 1% of the worlds population. The prevalence is higher in patients aged 65 years due to increased cardiac and pulmonary etiologies, with lung disease and chronic obstructive pulmonary disease (COPD) being a common cause of PH. […] PH is divided into five groups. Group 1, or PAH, has an incidence of 6 cases/million adults, a prevalence of 48 to 55 cases/million adults, and predominantly affects women. […] Females are known to have a higher incidence of PAH, which is reflected globally across registries in the US, Europe, and Asia. Female-to-male prevalence varies by ethnicity, with one paper citing 3.2:1 female-to-male predominance in Whites, 4.7:1 in Hispanics, and 5.5:1 in Blacks. Data from various registries showed that most patients enrolled with PAH were female, such as the French and Scottish registries (65%), US REVEAL registry (80%), German Registry COMPERA, Spanish and Latvian registries (73%), and Japanese registry (76%). However, despite female predominance, women had better outcomes compared with male patients, a finding consistently reflected across all registries. This observation has led to a phenomenon called the estrogen paradox.

• #21 Epidemiology of Pulmonary Hypertension: From Quaternary Referral Centre to the Community | Thoracic Key

  https://thoracickey.com/epidemiology-of-pulmonary-hypertension-from-quaternary-referral-centre-to-the-community/

  Multiple registries have captured survival in both the pre- and post-PAH treatment eras. […] Comparing these estimates, it appears that short-term survival has improved over time and is approximately 90 % at 1 year and 75 % at 3 years. […] Single center US-based registries including various WHO Group 1 etiologies have reported slightly older average age (4th5th decade of life) than that reported in the NIH registry, but confirmed 70 % female predominance, as have other national reports. […] In patients with IPAH, the average age at enrollment was 5315 years of age and 80 % were female, signaling a possible shift in demographics as compared to earlier PAH registries. […] AA have higher rates of hospitalization and death as compared to whites, and appear to have a less robust response to treatment with endothelin receptor antagonists in PAH clinical trials. […] While neither sex nor race/ethnicity were associated with delay in diagnosis (2 years between symptom onset and evaluation for PAH) in the REVEAL registry, it is unknown whether these possible differences in outcomes are due to true differences in disease biology, variable therapeutic responses, and/or confounding by socioeconomic status such as limited access to care as has been noted in other cardiopulmonary diseases. […] After IPAH, CTD-PAH patients tend to be the second most represented subgroup in registries, allowing for comparisons between these groups. […] Survival is poorer in CTD-PAH as compared to idiopathic disease and, among CTD-PAH, lowest in patients with systemic sclerosis.

• #22 Epidemiology of Pediatric Pulmonary Hypertension | SpringerLink

  https://link.springer.com/10.1007/978-1-4471-4999-6_204-2

  Pulmonary hypertension (PH) is a rare condition. Data on adult PH patients is abundant, but data on pediatric PH are scarce. Nevertheless, in the past decades, increasing data concerning pediatric PH have become available, enabling this review of the epidemiology, characterization, and outcome of pediatric pulmonary hypertension. […] Pediatric PH frequently co-occurs with genetic and syndromal abnormalities, including Down syndrome. […] Outcome of pediatric PAH differs between patients with different types of congenital heart disease. […] However, currently reported survival rates vary, and critical appraisal of patient populations, survival data, and proposed treatment approaches is necessary. […] Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005.

• #23 Pulmonary hypertension in the global population of adolescents and adults living with HIV: a systematic review and meta-analysis | Scientific Reports

  https://www.nature.com/articles/s41598-019-44300-5

  A systematic review and meta-analysis was conducted to estimate the prevalence of PH in adolescents and adults living with HIV at the global level. […] One study reported the prevalence of PH among HIV-infected adults based on RHC: 0.5% (95% confidence interval (CI): 0.30.6). The global prevalence of PH based on echography was 8.3% (95% CI: 4.612.8; 22 studies) among HIV-infected adults. […] Among HIV-infected adolescents, the prevalence of PH based on echography was 14.0% (95% CI: 2.233.1; 2 studies). This study suggests a high prevalence of PH in the global adolescent and adult population infected with HIV. […] The exact pathogenesis of HIV-associated PH is still unknown, but HIV proteins such as Tat and Nef are probably implicated. […] To date, it is unclear whether HIV infection is a cause or contributor of PH.

• #24 Pulmonary hypertension in the global population of adolescents and adults living with HIV: a systematic review and meta-analysis | Scientific Reports

  https://www.nature.com/articles/s41598-019-44300-5

  This first global systematic review and meta-analysis of data from 42,642 adolescents and adults living with HIV from 17 countries showed an overall PH prevalence of 8.3% in adults and 14.0% in adolescents (based on transthoracic echocardiography), with substantial heterogeneity between studies. […] In the general global population, the prevalence of PH is around 1%. […] Evidence suggests HIV infection as the leading infectious cause of PH. […] Our findings have important implications for healthcare providers, health policy makers and researchers. […] This systematic review and meta-analysis compiled data from 17 countries and pointed a high prevalence of PH in people living with HIV.

• #25 Group 2 Pulmonary Hypertension: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/2500055-overview

  Pulmonary hypertension (PH) is divided into five distinct groups, which are categorized according to similar pathophysiologic changes, clinical presentation, and available therapies. Pulmonary hypertension due to left-sided heart disease (PH-LHD), classified by the World Health Organization (WHO) into group 2 pulmonary hypertension, is the most common cause of pulmonary hypertension and is associated with impaired exercise capacity and reduced survival. […] There are approximately 6.5 million people in Europe and 5 million people in the United States with heart failure. About 60% of patients with heart failure with reduced ejection fraction (HFrEF) have been noted to have pulmonary hypertension at presentation. PH has an estimated prevalence of 65% in patients with aortic valve disease and up to 100% prevalence in patients with symptomatic mitral valve disease.

• #26 Group 2 Pulmonary Hypertension: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/2500055-overview

  The development of pulmonary hypertension due to left-sided heart disease (PH-LHD) is associated with a poor prognosis, particularly in relation to patients with LHD without associated development of pulmonary hypertension. Patients with heart failure with reduced ejection fraction (HFrEF) and pulmonary hypertension have a higher mortality rate (28%) than those without pulmonary hypertension (17%) at 28 months. […] Patients with confirmed left heart disease, atrial fribrillation, previous cardiac intervention and structural LHD are at increased risk for PH. Noncardiac risk factors for the development of PH-LHD include older age 60 years old, female, obesity, hypertension, dyslipidemia, and diabetes.

• #27 Pulmonary hypertension in low- and middle-income countries with focus on sub-Saharan Africa – Dzudie – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/29088/html

  Pulmonary hypertension (PH) is a devastating, progressive disease with increasingly debilitating symptoms and usually shortened overall life expectancy. This article reviews the global epidemiology of PH with focus on low- and middle-income countries (LMICs) and sub-Sahara African in particular. […] Despite existing epidemiological data of PH risk factors suggesting a high prevalence in sub-Saharan Africa (SSA), the available literature is limited. International registries in LMICs like the pan African pulmonary hypertension cohort (PAPUCO) study are essential to provide information about the causes, treatment, outcome, and the natural course of PH in Africa and other parts of the world. […] It is estimated that about 80% of the global burden of the disease is in LMICs where PH is known to be highly associated with CHD, rheumatic heart disease, schistosomiasis and HIV.

• #28 Pulmonary hypertension in low- and middle-income countries with focus on sub-Saharan Africa – Dzudie – Cardiovascular Diagnosis and Therapy

  https://cdt.amegroups.org/article/view/29088/html

  The exact global burden of PH remains unknown, and most likely largely underestimated due to the above-mentioned circumstances in LMICs. […] Overall, LHD still remains the dominant risk factor for PH (PH-LHD), followed by chronic lung disease and hypoxia (PH-LD), while PAH remains rare even in high HIV prevalent settings in SSA. […] The PAPUCO study described the causes, treatment and outcome of PH in Africa in both adult and pediatric populations. […] The high prevalence of PH-LHD in LMICs is expected due to a higher prevalence of uncontrolled hypertension which is also a principal cause of heart failure according to the sub-Saharan acute heart failure (THESUS-HF) study, a study conducted in nine African countries. […] A South African study identified PH as one of the commonest causes of death accounting for 31% of total cardiovascular deaths.

• #29 Pulmonary Hypertension Surveillance

  https://stacks.cdc.gov/view/cdc/26606

  Pulmonary hypertension (PH) is an uncommon but progressive condition, and much of what we know about it comes from specialized disease registries. […] With expanding research into the diagnosis and treatment of PH, it is important to provide updated surveillance on the impact of this disease on hospitalizations and mortality. […] This study, which builds on previous PH surveillance of mortality and hospitalization, analyzed mortality data from the National Vital Statistics System and data from the National Hospital Discharge Survey between 2001 and 2010. […] The decline in death rates associated with PH among men from 1980 to 2005 has reversed and now shows a significant increasing trend. […] Similarly, the death rates for women with PH have continued to increase significantly during the past decade.

• #30 Pulmonary Hypertension Surveillance

  https://stacks.cdc.gov/view/cdc/26606

  PH-associated mortality rates for those aged 85 years and older have accelerated compared with rates for younger age groups. […] There have been significant declines in PH-associated mortality rates for those with pulmonary embolism and emphysema. […] Rates of hospitalization for PH have increased significantly for both men and women during the past decade; for those aged 85 years and older, hospitalization rates have nearly doubled. […] Continued surveillance helps us understand and address the evolving trends in hospitalization and mortality associated with PH and PH-associated conditions, especially regarding sex, age, and race/ethnicity disparities.

• #31 Survival, morbidity, and quality of life in pulmonary arterial hypertension patients: a systematic review of outcomes reported by population-based observational studies | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-024-02994-w

  Comprehensive summaries on real-world outcomes in pulmonary arterial hypertension (PAH)a rare, incurable condition, are lacking. […] We conducted a systematic literature review to describe current survival, morbidity, and quality of life (QoL) outcomes in adult and pediatric PAH patients. […] The search yielded 7473 records. Following screening and full text review, 22 unique studies with 31 individual reports of outcomes were included. […] Survival was the most frequently reported outcome (n=22). Global adult 1-, 3-, and 5-year survival ranged from 85 to 99% (n=15), 65 to 95% (n=14), and 50 to 86% (n=9), respectively. […] Subgroup meta-analysis showed that 1-, 3-, and 5-year survival in Europe was 90% (95% CI 8694%; n=8), 78% (95% CI 6886%; n=8), and 61% (95% CI 4972%; n=6), respectively; 1-year survival in North America was 88% (95% CI 8393%; n=3) and 3-year survival in Asia was 85% (95% CI 8288%; n=3).

• #32 Pulmonary Hypertension: Diagnosis and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2016/0915/p463.html

  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. […] There is limited evidence to support screening of asymptomatic individuals. […] Family physicians most often encounter pulmonary hypertension in patients with chronic disease such as heart failure, obstructive lung disease, and thromboembolism. Pulmonary hypertension is most prevalent in those with left heart disease (group 2). […] The prevalence of pulmonary hypertension increases with age. A population-based study in Minnesota estimated that the total prevalence could reach 10% to 20% in the general population. […] There is limited evidence to guide screening for pulmonary hypertension in asymptomatic individuals, even in high-risk groups, which leads to significant delays in diagnosis.

• #33 Pulmonary hypertension associated mortality in the United States from 2003 to 2020: an observational analysis of time trends and disparities – Singh – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/75990/html

  Our study indicates a rise in PH-associated mortality from 2003 to 2020 in the US population. […] Overall, PH was more often reported as the underlying cause of death in females, with the sex gap widening over the study period. […] WHO groups 25 PH mortality rates increased, and there was a steeper rise in females as compared to males. […] Our study documents nineteen times higher mortality rates in groups 25 PH in comparison to the PAH group, a 55% increase in mortality burden related to groups 25 PH, and an increase in the median age of death. […] These findings signal that the aging population and comorbid clinical conditions such as left-sided heart disease, systemic hypertension, obstructive sleep apnea, chronic obstructive lung diseases, and interstitial lung diseases are responsible for the increasing mortality burden from secondary PH.

• #34 Prevalence of Pulmonary Hypertension in the General Population: The Rotterdam Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130072

  Pulmonary hypertension is characterized by increased pulmonary artery pressure and carries an increased mortality. Population-based studies into pulmonary hypertension are scarce and little is known about its prevalence in the general population. We aimed to describe the distribution of echocardiographically-assessed pulmonary artery systolic pressure (ePASP) in the general population, to estimate the prevalence of pulmonary hypertension, and to identify associated factors. […] In this large population-based study, we show that pulmonary hypertension as measured by echocardiography has a low prevalence in the overall general population in the Netherlands, but estimates may be higher in specific subgroups, especially in those with underlying diseases. Increased pulmonary arterial pressure is likely to gain importance in the near future due to population aging and the accompanying prevalences of underlying disorders.

• #35 Long COVID May Cause Long-Term Changes in the Heart and Lungs and May Lead to Cardiac & Pulmonary Diseases

  https://yubanet.com/scitech/long-covid-may-cause-long-term-changes-in-the-heart-and-lungs-and-may-lead-to-cardiac-pulmonary-diseases/

  Patients suffering from long COVID may exhibit persistent inflammation in the heart and lungs for up to a year following SARS-CoV-2 infection even when standard medical tests return normal results potentially placing them at elevated risk for future cardiac and pulmonary conditions. […] These abnormalities could serve as early warning signs of diseases such as heart failure, valvular heart disease, and pulmonary hypertension. […] We believe long COVID results in an inflammatory response that may predispose patients to premature coronary artery disease, pulmonary hypertension, and valvular damage such as stenosis or regurgitation. […] This study highlights the unique power of hybrid PET/MRI imaging to uncover hidden disease processes in long COVID patients. […] These findings should change how we approach care and surveillance not only recognizing SARS-CoV-2 as a potential long-term cardiovascular risk factor, but also integrating molecular imaging into post-COVID evaluation protocols. […] The Mount Sinai team continues to follow this patient cohort to assess long-term outcomes and is exploring whether these imaging and biomarker patterns can help predict who is most at risk for developing chronic cardiovascular or pulmonary disease after COVID-19.

• #36 Long COVID May Cause Long-Term Changes in the Heart and Lungs and May Lead to Cardiac and Pulmonary Diseases | Mount Sinai – New York

  https://www.mountsinai.org/about/newsroom/2025/long-covid-may-cause-long-term-changes-in-the-heart-and-lungs-and-may-lead-to-cardiac-and-pulmonary-diseases

  Patients suffering from long COVID may exhibit persistent inflammation in the heart and lungs for up to a year following SARS-CoV-2 infection—even when standard medical tests return normal results—potentially placing them at elevated risk for future cardiac and pulmonary conditions. […] These abnormalities could serve as early warning signs of diseases such as heart failure, valvular heart disease, and pulmonary hypertension. […] This study brings us closer to understanding how SARS-CoV-2 affects the heart and lungs over time. […] We believe long COVID results in an inflammatory response that may predispose patients to premature coronary artery disease, pulmonary hypertension, and valvular damage such as stenosis or regurgitation. […] This study highlights the unique power of hybrid PET/MRI imaging to uncover hidden disease processes in long COVID patients.

• #37 Top End Pulmonary Hypertension Study: Understanding Epidemiology, Therapeutic Gaps and Prognosis in Remote Australian Setting | medRxiv

  https://www.medrxiv.org/content/10.1101/2020.09.19.20197988v1.full-text

  Pulmonary hypertension (PH) is prevalent in Northern Australia, with a high frequency of modifiable risk factors and other treatable conditions. The epidemiology of PH has not been previously studied in this region. The prevalence of PH was 955 per 100,000 population (with corresponding prevalence of 1587 for Indigenous people). The study provides rich and comprehensive information regarding the prevalence and the root causes of PH in our region and complements the findings of the previously published Central Australian Study to complete the picture for the whole NT. The Indigenous population not only had a higher prevalence (1.6%), but also a higher mortality risk associated with PH. The burden of risk factors and comorbidities were high in this region where approximately 30% of the population was Indigenous and had a high prevalence of rheumatic heart disease. The prevalence of all types of PH in Top End was approximately three times higher than observed in the Armadale echo study and higher than reported from Central Australia. Better understanding of PH epidemiology will allow better planning and management of scarce health resources and thence hopefully improve the outcomes.

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