A hipertensão arterial pulmonar (HAP) é um grupo de doenças caracterizadas por pressão média da artéria pulmonar >20 mmHg e resistência arterial pulmonar elevada, levando à insuficiência cardíaca direita. A HAP é progressiva e potencialmente fatal. A HAP pode ser idiopática e/ou familiar, ter características evidentes de envolvimento venoso/capilar (doença veno-oclusiva pulmonar, PVOD/hemangiomatose capilar pulmonar, HCP), induzida por medicamento ou toxina (HAP induzida por medicamento ou toxina), ou associada a outras doenças como cardiopatia congênita, doença do tecido conjuntivo, HIV, esquistossomose, hipertensão portal (HAP associada a outras doenças).
Introdução
O que você precisa saber de cara
A hipertensão arterial pulmonar é uma doença rara caracterizada pelo aumento da pressão nos vasos que levam sangue do coração aos pulmões, sobrecarregando o lado direito do coração. A pessoa pode notar sintomas frequentes como falta de ar, inchaço nas pernas, cansaço extremo aos esforços físicos e palpitações. O diagnóstico pode ocorrer em qualquer idade e conta com testes genéticos específicos para identificar possíveis causas hereditárias. No Brasil, o SUS entrega pelo Componente Especializado medicamentos como Bosentana, Ambrisentana, Sildenafila, Iloprosta, Macitentana, Riociguate e Selexipague.
A hipertensão arterial pulmonar (HAP) é um grupo de doenças caracterizadas por pressão média da artéria pulmonar >20 mmHg e resistência arterial pulmonar elevada, levando à insuficiência cardíaca direita. A HAP é progressiva e potencialmente fatal. A HAP pode ser idiopática e/ou familiar, ter características evidentes de envolvimento venoso/capilar (doença veno-oclusiva pulmonar, PVOD/hemangiomatose capilar pulmonar, HCP), induzida por medicamento ou toxina (HAP induzida por medicamento ou toxina), ou associada a outras doenças como cardiopatia congênita, doença do tecido conjuntivo, HIV, esquistossomose, hipertensão portal (HAP associada a outras doenças).
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 41 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 95 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
12 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.
Curadoria gene-doença
fontes oficiaisLipodystrophy, congenital generalized, 3
A form of congenital generalized lipodystrophy, a metabolic disorder characterized by a near complete absence of adipose tissue, extreme insulin resistance, hypertriglyceridemia, hepatic steatosis and diabetes mellitus. CGL3 inheritance is autosomal recessive.
Telangiectasia, hereditary hemorrhagic, 1
A multisystemic vascular dysplasia leading to dilation of permanent blood vessels and arteriovenous malformations of skin, mucosa, and viscera. The disease is characterized by recurrent epistaxis and gastro-intestinal hemorrhage. Visceral involvement includes arteriovenous malformations of the lung, liver, and brain.
Ischiocoxopodopatellar syndrome with or without pulmonary arterial hypertension
An autosomal dominant bone disease characterized by patellar aplasia or hypoplasia and by anomalies of the pelvis and feet, including disrupted ossification of the ischia and inferior pubic rami.
Vesicoureteral reflux 3
A disease belonging to the group of congenital anomalies of the kidney and urinary tract. It is characterized by the reflux of urine from the bladder into the ureters and sometimes into the kidneys, and is a risk factor for urinary tract infections. Primary disease results from a developmental defect of the ureterovesical junction. In combination with intrarenal reflux, the resulting inflammatory reaction may result in renal injury or scarring, also called reflux nephropathy. Extensive renal scarring impairs renal function and may predispose patients to hypertension, proteinuria, renal insufficiency and end-stage renal disease.
Pulmonary venoocclusive disease 2, autosomal recessive
A disease characterized by widespread fibrous obstruction and intimal thickening of septal veins and preseptal venules, a low diffusing capacity for carbon monoxide, occult alveolar hemorrhage, and nodular ground-glass opacities, septal lines and lymph node enlargement showed by high-resolution computed tomography of the chest. It is frequently associated with pulmonary capillary dilatation and proliferation, and is a rare and devastating cause of pulmonary hypertension.
Telangiectasia, hereditary hemorrhagic, 2
A multisystemic vascular dysplasia leading to dilation of permanent blood vessels and arteriovenous malformations of skin, mucosa, and viscera. The disease is characterized by recurrent epistaxis and gastro-intestinal hemorrhage. Visceral involvement includes arteriovenous malformations of the lung, liver, and brain.
Telangiectasia, hereditary hemorrhagic, 5
A multisystemic vascular dysplasia leading to dilation of permanent blood vessels and arteriovenous malformations of skin, mucosa, and viscera. The disease is characterized by recurrent epistaxis and gastro-intestinal hemorrhage. Visceral involvement includes arteriovenous malformations of the lung, liver, and brain.
Pulmonary hypertension, primary, 5
A form of primary pulmonary hypertension, a disease defined by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. Primary pulmonary hypertension exhibits incomplete penetrance, sex bias and variable age of onset, both within and between families. PPH5 is an autosomal recessive form characterized by the onset in infancy. Death in early childhood is common.
Pulmonary hypertension, primary, 4
A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
Pulmonary hypertension, primary, 1
A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
Pulmonary hypertension, primary, 6
A form of primary pulmonary hypertension, a disease defined by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. Primary pulmonary hypertension exhibits incomplete penetrance, sex bias and variable age of onset, both within and between families. PPH6 is an autosomal recessive form.
Pulmonary hypertension, primary, 2
A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial pulmonary hypertension is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.
Medicamentos aprovados (FDA)
3 medicamentos encontrados nos registros da FDA americana.
Variantes genéticas (ClinVar)
982 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 445 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
47 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Hipertensão arterial pulmonar
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712 ensaios clínicos encontrados, 150 ativos.
Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 12.293
A Phase 2, Randomized, Clinical Trial of Inhaled Nitric Oxide for Acute Severe Right Heart Failure With Pulmonary Hypertension (PHiNO Study).
O estudo PHiNO, de Fase 2, demonstrou que o óxido nítrico inalado (iNO) é um tratamento agudo promissor para pacientes com insuficiência cardíaca direita (ICD) grave devido à hipertensão pulmonar. Ele reduziu significativamente a resistência vascular pulmonar (um fator chave na ICD) em apenas 30 minutos, melhorando também marcadores cardíacos importantes ao longo de 7 dias, sem causar eventos adversos graves. Estes resultados sugerem que o iNO pode ser uma opção terapêutica segura e eficaz para essa condição de risco de vida.
🇧🇷 traduzidoWhen atrioventricular block paves the way to a more severe diagnosis: a case report.
Este caso atípico de esclerose sistêmica (ESc) difusa demonstra que um bloqueio atrioventricular (AV) completo pode ser a manifestação inicial, levando à descoberta de hipertensão pulmonar grave e fibrose. É crucial que médicos considerem doenças autoimunes sistêmicas em pacientes com bloqueio AV inexplicado, especialmente com sinais cutâneos, para um diagnóstico precoce. Essa abordagem integrada e multidisciplinar é vital para pacientes, permitindo tratar as múltiplas complicações (cardíacas, pulmonares e gastrointestinais) e estabilizar a condição, melhorando a qualidade de vida.
🇧🇷 traduzidoThe emerging role of integrin signaling in pulmonary vascular disease.
A hipertensão arterial pulmonar (HAP) é uma doença grave e incurável, e estudos recentes indicam que as integrinas desempenham um papel promissor em sua patogênese, promovendo a proliferação celular, inflamação e fibrose nos vasos pulmonares. Este artigo de revisão explora a base biológica das integrinas na doença vascular pulmonar e as posiciona como potenciais alvos de tratamento, discutindo caminhos intracelulares e estudos terapêuticos recentes em animais com o objetivo de desenvolver novas terapias.
🇧🇷 traduzidoEndothelial C-type natriuretic peptide/guanylyl cyclase-B signaling prevents pulmonary arterial hypertension.
Este estudo revela que a via de sinalização do peptídeo natriurético tipo C (CNP) e GC-B, presente nas células que revestem os vasos sanguíneos (endoteliais), é crucial para prevenir a Hipertensão Arterial Pulmonar (HAP). Em pacientes e modelos da doença, essa via protetora está reduzida. A administração de CNP demonstrou a capacidade de prevenir e tratar a HAP, inclusive com benefícios adicionais quando combinada a terapias existentes. Isso sugere que o CNP possui um potencial terapêutico significativo e promissor para pacientes com HAP.
🇧🇷 traduzidoIron Deficiency in Pulmonary Hypertension-Prevalence, Impact on Prognosis and Disease Burden in Pulmonary Arterial Hypertension and Pulmonary Hypertension Related to Hypoxia: A Review.
A hipertensão arterial pulmonar (HAP) é uma condição grave, e embora existam tratamentos avançados, fatores modificáveis também são cruciais para o manejo. A deficiência de ferro (DF) é um déficit nutricional comum que está sendo cada vez mais associado ao prognóstico e à carga da doença na HAP e na HP relacionada à hipóxia. Isso sugere que a DF pode ser um fator importante e potencialmente tratável, influenciando a qualidade de vida e o desfecho dos pacientes, embora mais estudos sejam necessários para definições precisas e manejo ideal.
🇧🇷 traduzidoPublicações recentes
The Impact of Fluid Balance on Clinical Outcomes in ICU Patients with Pulmonary Arterial Hypertension: A Retrospective Analysis using MIMIC-IV.
📖 RevisãoIntermittent fasting and cardiometabolic responses under cardiac stress: a systematic review of preclinical studies.
🥉 Relato de casoIdentification of RUNX1 as a Biomarker for Right Ventricular Remodeling in Pulmonary Arterial Hypertension Using WGCNA.
Biological Sex Differences in the US-CTEPH-R.
Echocardiographic Assessment of Pulmonary Arterial Hypertension After Complete Atrioventricular Septal Defect Repair.
📚 EuropePMC10.723 artigos no totalmostrando 197
THBS4 Regulates Pulmonary Hypertension via TGF-β/SMAD2 Signaling.
Hypertension (Dallas, Tex. : 1979)Reduction in microbiota-derived short-chain fatty acids contributes to the pathogenesis of pulmonary arterial hypertension.
Respiratory researchMnTBAP attenuates pulmonary hypertension-induced right ventricular dysfunction and remodeling by modulating the CaMKIIδ-SERCA2a signaling pathway.
Biochemical pharmacologyUpdates in the Treatment of Pediatric Pulmonary Arterial Hypertension.
Cardiology in reviewDilemmas of Self-Management in Patients with Pulmonary Arterial Hypertension: A Descriptive Phenomenological Study.
Patient preference and adherenceMarked clinical and haemodynamic improvement with sotatercept in severe refractory pulmonary arterial hypertension associated with corrected complex congenital heart disease.
Respiratory medicine case reportsMultifaceted mechanisms of plant metabolites in pulmonary arterial hypertension: a critical review beyond vasodilation.
Frontiers in pharmacologyA Phase 2, Randomized, Clinical Trial of Inhaled Nitric Oxide for Acute Severe Right Heart Failure With Pulmonary Hypertension (PHiNO Study).
Circulation journal : official journal of the Japanese Circulation SocietyClinical and hemodynamic evaluation of schistosomiasis-associated pulmonary arterial hypertension from Egyptian pulmonary hypertension centers: epidemiology, risk factors, and survival determinants.
Scientific reportsSotatercept improves right ventricular function but is associated with new or worsening pericardial effusions: a CMR study in intermediate-high risk PAH.
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonancePreoperative pulmonary hemodynamics and clinical decision making to determine operability and risk of long-term pulmonary hypertension in infants with open shunt under 1 year.
International journal of cardiologyCardiac MRI differentiates classical idiopathic pulmonary arterial hypertension from lung phenotype and group 3 pulmonary hypertension.
Respiratory medicine and researchLoss of productivity among commercially insured patients with pulmonary arterial hypertension in the United States.
Journal of medical economicsNuclear export by COPS5/CSN5/JAB1 mediates vascular smooth muscle cell dedifferentiation in neointimal hyperplasia.
Cardiovascular researchSphingosine-1-phosphate induces pulmonary artery smooth muscle cell proliferation, migration and pulmonary arterial remodeling by modulating sonic hedgehog signaling effector FoxM1.
Chinese medical journal[Sleep disorders dynamics in patients with chronic thromboembolic pulmonary hypertension after surgical treatment].
Terapevticheskii arkhivNeonatal pulmonary vascular remodeling induced by increased blood flow is associated with an antiviral-like immune signature.
Frontiers in immunologyWhen atrioventricular block paves the way to a more severe diagnosis: a case report.
European heart journal. Case reportsInsight of traditional Chinese medicine in treating pulmonary hypertension: Achievements from 2021 to 2025.
Journal of ethnopharmacologyS- nitrosylation of Annexin A2 at Cys133 ameliorates pulmonary arterial hypertension by inhibiting the WNT/β-catenin pathway.
Respiratory researchHarnessing metabolomics and proteomics in a clinical trial for pulmonary arterial hypertension: insights from post-hoc analysis of the REHAB-PH trial.
EBioMedicineThe emerging role of integrin signaling in pulmonary vascular disease.
American journal of physiology. Lung cellular and molecular physiologyHydroxychloroquine withdrawal triggers pregnancy-associated pulmonary arterial hypertension in systemic lupus erythematosus: a case report and exploration of the Complement-EndMT axis.
Frontiers in pharmacologyMyocardial fibrosis and glycolysis: a bibliometric study and visualization analysis (2000-2024).
Journal of translational medicineEndothelial C-type natriuretic peptide/guanylyl cyclase-B signaling prevents pulmonary arterial hypertension.
Nature communicationsProvider networks for pulmonary hypertension in Massachusetts: implications for improving referrals to expert care.
Annals of the American Thoracic SocietyCritical Care Management of the Patient with Pulmonary Hypertension.
Journal of intensive care medicineTargeting autoimmunity: Phosphodiesterase inhibitors as immunomodulators-a review of current insights and future direction.
Autoimmunity reviewsMolecular dynamics simulations refine the pathogenicity of ACVRL1 kinase domain variants by quantifying impacts on ATP binding in pulmonary arterial hypertension.
Journal of structural biologyEnhanced Mitochondrial Mrs2-Mg2+ Signaling Drives Mitochondrial Dysfunction in Pulmonary Arterial Hypertension Rats.
Hypertension (Dallas, Tex. : 1979)Exercise testing in chronic thromboembolic pulmonary hypertension and chronic thromboembolic pulmonary disease without pulmonary hypertension: a comprehensive systematic review and meta-analysis.
European journal of internal medicineRight atrial phasic strain in risk stratification of patients with Pulmonary Arterial Hypertension.
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonanceIdentification of Key Genes and Pathways Associated With Gender Differences in Pulmonary Arterial Hypertension Based on Bioinformatic Approaches.
BioMed research internationalIron Deficiency in Pulmonary Hypertension-Prevalence, Impact on Prognosis and Disease Burden in Pulmonary Arterial Hypertension and Pulmonary Hypertension Related to Hypoxia: A Review.
International journal of molecular sciencesStress Echocardiography in the Diagnosis and Evaluation of Pulmonary Hypertension: Practical Recommendations, Haemodynamic Phenotyping, and Application in Adults and Children.
Diagnostics (Basel, Switzerland)The Role of the Apelin Receptor in the Pathophysiology of Pulmonary Arterial Hypertension.
CellsValidation Analysis of the Polish-Translated Version of EmPHasis-10 Health-Related Quality of Life Questionnaire in Patients with Pulmonary Arterial Hypertension.
Journal of clinical medicineClinically Actionable Explainable AI in Pulmonary Arterial Hypertension: Endpoints, Calibration, and External Validation. Reply to Pagnoni et al. Toward Clinically Actionable Explainable AI in Pulmonary Arterial Hypertension: Endpoints, Calibration, and External Validation. Comment on "Ledziński et al. Personalized Medicine in Pulmonary Arterial Hypertension: Utilizing Artificial Intelligence for Death Prevention. J. Clin. Med. 2025, 14, 8325".
Journal of clinical medicineToward Clinically Actionable Explainable AI in Pulmonary Arterial Hypertension: Endpoints, Calibration, and External Validation. Comment on Ledziński et al. Personalized Medicine in Pulmonary Arterial Hypertension: Utilizing Artificial Intelligence for Death Prevention. J. Clin. Med. 2025, 14, 8325.
Journal of clinical medicineNailfold capillaroscopy and organ involvement in systemic sclerosis: a systematic review.
Best practice & research. Clinical rheumatologyLong-Term Treatment with Single-Tablet Combination of Macitentan and Tadalafil in Pulmonary Arterial Hypertension: Results from A DUE and Its Open-Label Period.
Advances in therapyProtocol for an open-label, randomised, controlled trial to evaluate the efficacy and safety of sotatercept add-on therapy compared with pulmonary vasodilator-based standard of care for pulmonary vasodilator-resistant pulmonary arterial hypertension associated with unrepaired congenital shunts (atrial septal defect, ventricular septal defect or patent ductus arteriosus), including Eisenmenger syndrome: the SuMILE trial.
BMJ openHow I do it: How to care for the patient with methamphetamine-associated PAH.
ChestLong-term efficacy of the treat-to-close strategy for patients with atrial septal defect-pulmonary artery hypertension and characteristics of indicated populations.
Clinics (Sao Paulo, Brazil)Targeting the Hippo pathway in pulmonary arterial hypertension: emerging pharmacological strategies.
Journal of hypertensionSotatercept in Patients With Eisenmenger Syndrome.
Pulmonary circulationResting Oxygen Consumption Estimates in Scleroderma Can Lead to Underestimation of Cardiac Output.
Pulmonary circulationTime to Therapy Activation and Initial Combination Therapy (TITANIC) for Patients With Pulmonary Arterial Hypertension.
Pulmonary circulation[Mechanisms of Qibai Pingfei Capsules in inhibiting pyroptosis of pulmonaryartery adventitial fibroblasts via regulating NLRP3/Caspase-1/ GSDMD pathway to intervene in COPD complicated by pulmonary arterial hypertension].
Zhongguo Zhong yao za zhi = Zhongguo zhongyao zazhi = China journal of Chinese materia medicaDiamond in the Rough or Just the Flavor of the Week?: Transforming Growth Factor 15 as a Biomarker in Pulmonary Arterial Hypertension.
ChestPulmonary hypertension associated with nonparenchymal restrictive lung diseases.
European respiratory review : an official journal of the European Respiratory SocietyPulmonary Hypertension Associated With Left Heart Disease: Challenges, Emerging Strategies, and Future Directions.
Journal of the American College of CardiologyCase Report: Rethinking pulmonary arterial hypertension: immune and metabolic adaptations in a 34-year case of insidious progression.
Frontiers in cardiovascular medicineSmoking exposure on diagnosis and survival of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Data from REHAP registry.
PulmonologySotatercept for Decompensated Pulmonary Arterial Hypertension Requiring VA ECMO: First Canadian ICU Experience.
Pulmonary circulationIndustry Payments and Prescribing Patterns of Pulmonary Hypertension Therapies in the United States.
Pulmonary circulationFerroptosis of smooth muscle cells in vascular diseases: from basic principles to clinical translation.
Cell death discoveryThe Minimal Important Difference in N-Terminal Pro-Brain Natriuretic Peptide in Pulmonary Hypertension.
ChestRisk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease. A scientific statement of the ESC Working Group on Pulmonary Circulation & Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease, and the Association of Cardiovascular Nursing & Allied Professions of the ESC.
European journal of heart failurePulmonary Artery Denervation for Refractory Sjögren's Syndrome-Associated Pulmonary Arterial Hypertension: A Case Report of Combined Hemodynamic and Renal Recovery.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsAlternative Polyadenylation Signatures Distinguish Maladaptive Right Ventricular Remodeling in Pulmonary Hypertension: Implications for RNA-Based Diagnostics and Therapeutics.
British journal of biomedical scienceCase Report: A pediatric case of chronic active Epstein-Barr virus infection complicated by pulmonary arterial hypertension.
Frontiers in cardiovascular medicineA Critical Contribution of Cardiac Myofibroblasts in Right Ventricular Failure and the Role of UCP2 SNPs in the Predisposition to RV Decompensation in Pulmonary Arterial Hypertension.
CirculationRectus sheath block a retrospective review of a novel adjunct for treatment of the injection site pain associated with treprostinil subcutaneous infusion in pulmonary hypertension case series.
Pain managementMacitentan vs Standard of Care in Pediatric Pulmonary Arterial Hypertension (TOMORROW): A Randomized Clinical Trial.
The Journal of pediatricsTherapeutic effects of fingolimod through sphingosine-1-phosphate signaling in pulmonary arterial hypertension.
Journal of pharmacological sciencesBMPR2 Splice-Site Variant in a Patient With Pulmonary Arteriovenous Malformation and Delayed-Onset Pulmonary Arterial Hypertension: A Case Report and Mechanistic Phenocopy Hypothesis.
American journal of medical genetics. Part AInterpretive considerations on real-world use of selexipag and parenteral prostacyclin in pulmonary arterial hypertension.
Journal of cardiologyMethamphetamine-associated PAH on the rise in the US: geographic trends & disparities in patient demographics and treatment strategies.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationThe critical role of HIBCH in pulmonary arterial hypertension.
Toxicology and applied pharmacologyParadigm shift: from pulmonary vasodilation to cardiopulmonary dual-track therapy-a comprehensive review of pathophysiology and advances in pulmonary hypertension-associated right heart failure.
Respiratory researchLGALS3BP promotes M1 polarization of macrophages and interacts with LGALS3, damaging endothelial function and exacerbating pulmonary arterial hypertension in systemic lupus erythematosus.
Pathology, research and practicePAH-former: Transfer learning for efficient discovery of pulmonary arterial hypertension-associated genes.
PloS oneEmergency cesarean section in pregnant women with severe pulmonary hypertension: the potential role of extracorporeal life support.
Ulusal travma ve acil cerrahi dergisi = Turkish journal of trauma & emergency surgery : TJTESLoss of endothelin-2 in pulmonary neuroendocrine cells promotes the development of hypoxia-induced pulmonary hypertension.
Clinical science (London, England : 1979)A circulating MicroRNA signature for the diagnosis of pulmonary arterial hypertension and functional characterization of candidate miR-3168.
Scientific reportsThe clinical phenotype of anti-Th/To+ patients in systemic sclerosis: a case-control study within the European Scleroderma Trials and Research cohort.
Therapeutic advances in musculoskeletal diseaseFerroptosis inhibitors: latest advances and therapeutic potential for pulmonary arterial hypertension.
Drug discovery todayPulmonary arterial flow alterations in systemic lupus erythematosus on 4D flow CMR: a case-control study.
European radiology experimentalTreatment Paradigm Shifts in Pulmonary Arterial Hypertension: Evidence From Asia on Vasodilators to Antiremodeling Therapies.
JACC. AsiaReal-world use of theophylline in critically ill patients with sinus pauses: a case series.
Frontiers in cardiovascular medicineUnilateral Pulmonary Artery Agenesis in an Adult: A Case Report and Review of Literature.
Case reports in radiologyIdentification of Novel Drug Targets for Pulmonary Arterial Hypertension through Integrated Plasma Proteomics.
Journal of proteome researchImpact of Pulmonary Veno-Occlusive Disease on Posttransplant Survival in Pulmonary Hypertension.
Clinical transplantationReduced gastrointestinal iron uptake in pulmonary arterial hypertension: a prospective cross-sectional study.
Respiratory researchThe impact of age on six-minute walk distance, functional class, and right ventricular function in pulmonary arterial hypertension.
Respiratory medicineThe Pathophysiology, Mechanism, Diagnosis, and Management of Pulmonary Arterial Hypertension: A Comprehensive Literature Review.
US cardiologyReal-world Persistence on Selexipag for Pulmonary Arterial Hypertension in Canada.
CJC openHepatocyte growth factor may contribute to male protection against pulmonary arterial hypertension.
Biology of sex differences2025 expert consensus on the use of parenteral prostanoids in incident and prevalent PAH patients: The Italian perspective.
Vascular pharmacologyEvaluation of non-invasive techniques for the diagnosis of pulmonary complications in systemic sclerosis and scleroderma-like overlap syndromes: role of lung ultrasound.
Journal of autoimmunityImpact of ionic imbalances on rat cardiomyocyte function in pulmonary arterial hypertension: insights from energy dispersive X-ray spectroscopy and scanning electron microscopy analysis.
European journal of applied physiologyFeasibility and safety of right heart catheterization via median cubital vein in patients with pulmonary hypertension.
BMC cardiovascular disordersCirculating fatty acid profiles and risk of pulmonary arterial hypertension: Evidence from a large prospective cohort study.
MedicineRecurrent Paracentral Acute Middle Maculopathy Secondary to Pulmonary Arterial Hypertension: A Case Report.
Retinal cases & brief reportsNew phenotype and outcomes in connective tissue disease-associated pulmonary arterial hypertension: relevance of age-related comorbidities.
Rheumatology (Oxford, England)Trends and cross-country disparity in the burden of pulmonary arterial hypertension among women of childbearing age from 1990 to 2021.
Frontiers in global women's healthA Case of Antibody-Mediated Recurrent Hypoglycemia in a Patient With Mixed Connective Tissue Disease.
CureusCo-Spray-Dried Macitentan-Tadalafil with Leucine Microparticles for Inhalable Delivery in Pulmonary Arterial Hypertension.
PharmaceuticsMitochondrial Transplantation from Bone Marrow Mesenchymal Stromal Cells Combined with Sildenafil Attenuated Vascular Remodeling and Improved Right Ventricular Dysfunction in Experimental Pulmonary Arterial Hypertension.
International journal of molecular sciencesDysregulated Klotho and FGF23 signaling aggravates vascular remodeling in age-related pulmonary hypertension.
Cardiovascular researchA blood biomarker to specifically identify idiopathic pulmonary arterial hypertension.
Nature cardiovascular researchCorrigendum to "Real-world observational study on pulmonary arterial hypertension: Italian cohort treated with macitentan and/or selexipag as a part of a combination treatment (INSPECTIO)" [Vascular Pharmacology 162 (2026) 107585].
Vascular pharmacologyAdventitial Fibrosis and Fibroblast Mechanosensitivity Are Shaped by Sex and Hormonal Status in Pulmonary Arterial Hypertension.
CellsPulmonary Arterial Hypertension: Reconfiguring the Vascular Landscape to Reverse Remodeling.
CellsThe prevalence and long-term response to calcium channel blockers in patients with pulmonary arterial hypertension and positive vasoreactivity test - results of multicenter national registry (BNP-PL).
Orphanet journal of rare diseasesPharmacokinetics, Safety Profile, and Tolerability of MN-08 Tablets after Single and Multiple Ascending Doses in Healthy Chinese Volunteers.
Clinical drug investigationRisk Averse: Assessing Risk as a Surrogate Measure in Pulmonary Arterial Hypertension.
American journal of respiratory and critical care medicineStudy of Risk Assessment Scores as Surrogate Endpoints in Pulmonary Arterial Hypertension Trials.
American journal of respiratory and critical care medicinePotts shunt in Pulmonary Arterial Hypertension: Timing and risk assessment.
American journal of respiratory and critical care medicinePhase 2b trial of inhaled imatinib for treatment of pulmonary arterial hypertension.
American journal of respiratory and critical care medicineRespiratory complications of systemic autoimmune diseases: the emerging and important role of palliative care.
Current opinion in supportive and palliative careNeutrophil-mediated delivery of curcumin-loaded manganese-doped zeolitic imidazolate framework-8 nanozymes for pulmonary arterial hypertension.
Journal of nanobiotechnologyPrevious resistance exercise training mitigates progression of right ventricle dysfunction and remodeling in male rats with pulmonary arterial hypertension.
Physiological reportsSotatercept for the treatment of portopulmonary hypertension: a case report.
Communications medicineSotatercept Versus Selexipag in Severe Pulmonary Arterial Hypertension: An Indirect Comparison of Efficacy Based on an Artificial-Intelligence Method That Reconstructed Patient-Level Data From Three Randomized Trials.
CureusPulmonary Venous Vasculopathy in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension With Reduced Diffusion Capacity for Carbon Monoxide.
Pulmonary circulationPlasma GDF-15 and PSP-D Predict the Development of Pulmonary Arterial Hypertension in Systemic Sclerosis.
Pulmonary circulationAlterations in gut microbiota and plasma metabolites in pulmonary arterial hypertension secondary to congenital left-to-right shunt heart disease: potential mechanisms and biomarkers.
Frontiers in geneticsTransoral videolaryngoscopic surgery for hypopharyngeal anaplastic metastasis from papillary thyroid carcinoma.
JCEM case reportsPulmonary Arterial Hypertension and Cancer: Unveiling Parallels in Epidemiology, Clinical Pathways, and Therapeutic Strategies.
Journal of market access & health policyIndividualised interventional strategies and clinical outcomes in paediatric patients with pulmonary arterial hypertension.
Cardiology in the youngBioinformatics-based identification and experimental validation of biomarkers in pulmonary arterial hypertension.
European journal of medical researchThe role of pericytes in homeostasis and cardiovascular disease: A comprehensive review.
Journal of advanced researchA Rare Case of Reversible Pulmonary Hypertension Phenotype in a Child with Scurvy: Aetiologies Insights.
Reports (MDPI)Validity and reliability testing of the Thai version of the emPHasis-10 questionnaire for patients with pulmonary arterial hypertension.
Journal of patient-reported outcomesPulmonary hypertension associated with hereditary hemorrhagic telangiectasia: from genetics to clinical management.
The European respiratory journalValue of right heart haemodynamics for risk stratification of patients with pulmonary arterial hypertension at follow-up.
ESC heart failureAgreement of thermodilution and direct Fick methods for cardiac output across varying haemodynamic conditions.
ESC heart failureEvaluation of LCN2 and miR-8078 as diagnostic biomarkers for congenital heart disease-associated pulmonary arterial hypertension.
ESC heart failureCurrent State of RV Multimodality Imaging in Pediatric Pulmonary Hypertension: Current Evidence, Knowledge Gaps, and Future Research Directions.
Circulation. Cardiovascular imagingPerformance of Guideline-Recommended Approaches to Echocardiographic Investigation for Pulmonary Hypertension: Analysis of the CIPHER Study.
Pulmonary circulationDivergent Paths: A Survey of Cardiologist and Obstetrician Decision-Making in High-Risk Pregnancies with Cardiovascular Disease.
International journal of women's healthSotatercept for pulmonary arterial hypertension.
European journal of internal medicineManagement of pulmonary arterial hypertension in systemic sclerosis: from classical treatments to new horizons.
European respiratory review : an official journal of the European Respiratory SocietyThe Sigma-1 receptor agonist PRE084 improves cardiopulmonary function and remodelling in an experimental model of pulmonary arterial hypertension.
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapieBulk and single-cell transcriptomics for identification of potential diagnostic biomarkers associated with pulmonary arterial hypertension and integrated stress response and experimental validation.
Clinical and experimental hypertension (New York, N.Y. : 1993)Improving risk assessment with right ventricular phenotyping in patients with pulmonary arterial hypertension: minimizing future surprises.
European heart journalSystemic sclerosis-associated pulmonary arterial hypertension and pulmonary fibrosis: exploring biomarker discriminators with advanced omics in a Caucasian cohort.
Frontiers in immunologyAn Under-Recognized Manifestation of Systemic Sclerosis: Macrovascular Peripheral Arterial Disease.
CureusModulation of AKT/GSK-3β signaling by ambrisentan alleviates chronic kidney disease.
Pathology, research and practiceGM-CSF exacerbates pulmonary arterial hypertension via CCL2/CCR2-axis-mediated macrophage NLRP3 inflammasome activation.
International immunopharmacologyGLI1 Promotes Hypoxia Induced Pyroptosis of PASMCs and Aggravates Pulmonary Arterial Hypertension in Rats by Upregulating HDAC1 Expression.
Cardiovascular toxicologyHealthcare Resource Utilization and Medical Costs in Pulmonary Arterial Hypertension Management in Japan: A Retrospective Study Using a Claims Database.
Pulmonary therapyAssociation of Immunoglobulin E With Right Ventricular Dysfunction in Pulmonary Arterial Hypertension.
Pulmonary circulationShunt configuration's role in shaping hemodynamics of reverse Potts shunt in pediatric pulmonary arterial hypertension.
Frontiers in bioengineering and biotechnologyPhase 1 Study Evaluating the Effects of Single-Dose Frespaciguat, an Inhaled Soluble Guanylate Cyclase Stimulator, Co-administered with Multiple-Dose Sildenafil on Systemic Hemodynamics.
Clinical pharmacology in drug developmentGenetic Syndromes and Multimorbidity in Adults with Congenital Heart Disease and Heart Failure: Insights from the PATHFINDER-CHD Registry.
Journal of clinical medicineCardiac Magnetic Resonance Findings and Their Association with Clinical Outcomes in Pediatric Pulmonary Arterial Hypertension: An Exploratory Study.
Journal of clinical medicineComparative analysis of melatonin and sildenafil in a rat model of pulmonary arterial hypertension: Insights into oxidative stress, inflammation, and mitochondrial biogenesis.
Molecular and cellular endocrinologyFirst longitudinal spatial transcriptomics analysis in pulmonary arterial hypertension.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationLow positive affect as a predictor of mortality in pulmonary arterial hypertension: A Taiwanese cohort study integrating psychological and clinical risk factors.
Heart & lung : the journal of critical careTransitioning Prostacyclin Pathway Agents to Oral Selexipag in Patients with Pulmonary Arterial Hypertension: A Systematic Literature Review.
Advances in therapySotatercept Reverses SIN3a Deficiency-Driven PAH by Reprogramming BMPR2/TGF-β-HIF-1α Signaling Pathways.
bioRxiv : the preprint server for biologyA Large Animal Model of Heritable Pulmonary Arterial Hypertension Using Gene-edited BMPR2 Sheep.
bioRxiv : the preprint server for biologyAssociations between leisure sedentary behaviors, physical activity, and chronic respiratory diseases: a Mendelian randomization study.
Archives of medical science : AMSHeart-Lung transplantation: Meeting allocation challenges in France.
JHLT openDevelopment of a Robust, Rapid and Reliable Tandem Mass Spectrometry Method for the Measurement of Sildenafil, Bosentan and their Major Metabolites.
Indian journal of clinical biochemistry : IJCBEchocardiographic Assessment After Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension: Which Parameters Reflect Improved Right Ventricular Function?
Pulmonary circulationSecond- and Third-Generation BCR-ABL Tyrosine Kinase Inhibitors and the Risk of Pulmonary Arterial Hypertension: A Prevalent New-User Design.
CirculationMulti-omics integration study of vascular smooth muscle cell phenotypic conversion identified novel biomarkers in idiopathic pulmonary arterial hypertension.
Respiratory researchEfficacy of fasudil in COPD-associated pulmonary arterial hypertension: meta-analysis of randomized controlled trials.
Frontiers in medicineANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report.
European heart journal. Case reportsBeta arrestin 1 is a key regulator of pulmonary vascular tone.
Proceedings of the National Academy of Sciences of the United States of AmericaA study on the accessibility and utilisation of targeted drugs for pulmonary arterial hypertension in China.
Frontiers in pharmacologyRare TRAF5 coding variants in systemic lupus erythematosus patients aggravate pulmonary hypertension via endothelial dysfunction.
Innovation (Cambridge (Mass.))A Case of IgG4-Related Disease Developing During Long-Term High-Dose Intravenous Epoprostenol Therapy in a Patient With Idiopathic Pulmonary Arterial Hypertension.
Pulmonary circulationAge and onset timing of Raynaud's phenomenon and first non-Raynaud symptom as prognostic factors in systemic sclerosis: a retrospective analysis from the Italian national multicenter Systemic Sclerosis Progression INvestiGation registry of the Italian Society for Rheumatology (SPRING-SIR).
Therapeutic advances in musculoskeletal diseaseNatural terpenoids with therapeutic potential against pulmonary arterial hypertension.
Frontiers in pharmacologyPreselected and preferred immersive virtual reality versus narrative alone to induce post-stress relaxation in patients with pulmonary arterial hypertension: A pilot study on perceived stress and heart rate.
British journal of health psychologyAmbrisentan for Early-Stage Low-Risk Pulmonary Arterial Hypertension: Design of the Randomized, Double-Blind, Placebo-Controlled ALEPH Trial.
JACC. AsiaExosomal miR-21 mitigates pulmonary hypertension-induced right-heart remodeling by preserves mitochondrial homeostasis.
Journal of translational medicinePulmonary arterial hypertension: right ventricular phenotyping to improve risk assessment at follow-up.
European heart journalA treprostinil inhaler (Yutrepia) for pulmonary hypertension.
The Medical letter on drugs and therapeuticsNicotinamide phosphoribosyltransferase activates the mitochondrial unfolded protein response to promote pulmonary arterial endothelial cell proliferation.
bioRxiv : the preprint server for biologyEstriol attenuates visceral adiposity and pulmonary artery smooth muscle cell proliferation via ERα-mediated signalling.
European heart journal openCleavage and Polyadenylation Specificity Factor Subunit 5 Regulates Pulmonary Artery Smooth Muscle Expansion and Hypoxic Response.
MedCommPulmonary Veno-Occlusive Disease: A Clue to Underlying Lymphoma.
CureusPerillyl alcohol attenuates hypoxia induced right ventricular dysfunction and remodeling by balancing the renin angiotensin aldosterone system in rats.
Scientific reportsDaily Physical Activity in Pulmonary Arterial Hypertension: Insights from a Multicenter Longitudinal Trial Using Accelerometry.
ChestTransplantation of Ex Vivo Amplified Mononuclear Cells From Peripheral Blood Improves Rat Pulmonary Hypertension via CD14- and CD163-Positive M2 Macrophages.
Respirology (Carlton, Vic.)Angioinvasive pulmonary mucormycosis presenting with massive haemoptysis secondary to pulmonary artery pseudoaneurysm.
BMJ case reportsRegional Disparities in Pulmonary Arterial Hypertension Care in Japan - Beyond the Availability of Targeted Therapies.
Circulation journal : official journal of the Japanese Circulation SocietyReal-world observational study on pulmonary arterial hypertension: Italian cohort treated with macitentan and/or selexipag as a part of a combination treatment (INSPECTIO).
Vascular pharmacologyHemoglobin adduction and impaired oxygen transport define the etiology of pyrrolizidine alkaloid-induced pulmonary arterial hypertension.
Phytomedicine : international journal of phytotherapy and phytopharmacologyPaeonol alleviates pulmonary arterial hypertension by activation of BRCC3.
Phytomedicine : international journal of phytotherapy and phytopharmacologySine oculis homeobox 1 drives endothelial dysfunction in preclinical pulmonary arterial hypertension.
Science translational medicineIL-6 and TGF-β1 as biomarkers of schistosomiasis-associated pulmonary hypertension in a murine model.
Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicasDiscovery Beyond the "Undiscovered Country": Re-Exploring the Right Ventricle Through Metabolomics and Cardiac Magnetic Resonance.
JACC. AsiaLoss of ROR2 Tyrosine Kinase Receptor Is Associated With Endothelial Dysfunction in PAH via Inappropriate Integrin β1 Activation.
Hypertension (Dallas, Tex. : 1979)Clinical relevance of the TAPSE/SPAP ratio in pulmonary arterial hypertension: a single-center retrospective study.
BMC cardiovascular disorders[Research progress on the correlation between Akkermansia muciniphila and cardiovascular diseases].
Nan fang yi ke da xue xue bao = Journal of Southern Medical UniversityNewly diagnosed pulmonary arterial hypertension following liver transplantation: Insights from a multicenter case series.
Liver transplantation : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation SocietySympatho-Excitation in Pulmonary Hypertension: The Potential Role of Pulmonary Arterial Baroreceptors: An Acute Physiologic Intervention Study.
Respirology (Carlton, Vic.)[Role of macrophages in pulmonary arterial hypertension and advances in targeted therapeutic interventions].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesEfficacy and Safety of Oral Treprostinil in Patients with Pulmonary Arterial Hypertension on Background Monotherapy or Dual Therapy.
Advances in therapyCase Report: Immune checkpoint inhibitor-associated pulmonary hypertension.
Frontiers in cardiovascular medicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Associação brasileira dedicada a Hipertensão arterial pulmonar.
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Hipertensão arterial pulmonar e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Este é o documento que organiza diagnóstico, tratamento e dispensação de Hipertensão arterial pulmonar no SUS. O que a farmácia entrega é o que está no PCDT vigente — não o que foi aprovado fora do Brasil.
A hipertensão arterial pulmonar é uma doença caracterizada por pressão média na artéria pulmonar acima de 20 mmHg e alta resistência vascular. Essa alteração provoca sobrecarga progressiva e pode levar à insuficiência cardíaca do ventrículo direito.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Hipertensão arterial pulmonar. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hipertensao-arterial-pulmonar
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hipertensão arterial pulmonar
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
