A Hipertensão Pulmonar Tromboembólica Crônica (HPTEC) acontece quando coágulos de sangue antigos e organizados (que parecem cicatrizes) permanecem nas artérias dos pulmões, entupindo-as. Isso faz com que os vasos sanguíneos dos pulmões fiquem mais estreitos e rígidos, aumentando a dificuldade para o sangue passar (um problema chamado de resistência vascular pulmonar, RVP). Essa situação leva a um aumento da pressão nos pulmões (hipertensão pulmonar, HP) e, com o tempo, à perda progressiva da força do lado direito do coração (insuficiência cardíaca direita).
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A hipertensão pulmonar tromboembólica crônica (HPTEC) ocorre quando coágulos de sangue endurecidos permanecem nos vasos pulmonares, dificultando a circulação e sobrecarregando o coração. Quem convive com a doença costuma perceber cansaço intenso, falta de ar aos esforços físicos, palpitações e episódios de desmaio. No Brasil, a CONITEC avaliou e recusou a incorporação do medicamento riociguate no SUS, e não há protocolo com medicamentos específicos dispensados na rede pública. Apesar disso, o acompanhamento clínico e estudos sobre procedimentos vasculares continuam em andamento em centros especializados.
A Hipertensão Pulmonar Tromboembólica Crônica (HPTEC) acontece quando coágulos de sangue antigos e organizados (que parecem cicatrizes) permanecem nas artérias dos pulmões, entupindo-as. Isso faz com que os vasos sanguíneos dos pulmões fiquem mais estreitos e rígidos, aumentando a dificuldade para o sangue passar (um problema chamado de resistência vascular pulmonar, RVP). Essa situação leva a um aumento da pressão nos pulmões (hipertensão pulmonar, HP) e, com o tempo, à perda progressiva da força do lado direito do coração (insuficiência cardíaca direita).
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Sinais e sintomas
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Características mais comuns
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A Phase 2, Randomized, Clinical Trial of Inhaled Nitric Oxide for Acute Severe Right Heart Failure With Pulmonary Hypertension (PHiNO Study).
Acute right heart failure (RHF) is life threatening in patients with pulmonary hypertension (PH). This study investigated the efficacy and safety of inhaled nitric oxide (iNO; INOflo®for inhalation 800 ppm), a rapid pulmonary vasodilator, in patients with acute severe RHF due to PH. In this Phase 2 randomized controlled trial, 30 patients with acute severe RHF due to PH (pulmonary arterial hypertension or chronic thromboembolic PH) were randomized 1 : 1 to either an iNO or control group (which did not receive iNO). The primary endpoint was the change in pulmonary vascular resistance (PVR) from baseline to 30 min after either iNO initiation or patient registration and assignment (control group), which differed significantly between the iNO and control groups (mean [±SD] -2.41±2.47 vs. 0.8±1.03 Wood units, respectively; between group difference -3.21 [95% confidence interval -4.633, -1.785] Wood units). Serum B-type natriuretic peptide (BNP) levels and inferior vena cava diameter (secondary endpoints) decreased significantly in the iNO group over the 7-day study period. No serious adverse events, including methemoglobinemia, were observed. iNO significantly reduced PVR in patients with acute severe RHF due to PH, without serious adverse events. Serum BNP levels and inferior vena cava diameter improved in the iNO group. These findings suggest that iNO is a promising acute treatment option for RHF due to PH.
Prevalence, Clinical Features, and Treatment Response of Chronic Thromboembolic Pulmonary Hypertension in Patients With Myeloproliferative Neoplasm.
Patients with myeloproliferative neoplasms (MPN) are known to complicate pulmonary hypertension (PH). Half of patients with MPN-associated PH have chronic thromboembolic PH (CTEPH); however, the prevalence, clinical characteristics, and treatment response remain unclear. Nagoya PH registry database was retrospectively analyzed. Of 129 CTEPH patients, 82 were included. Clinical characteristics and treatment response were compared between CTEPH patients with MPN (MPN group) and those without MPN (non-MPN group). As the treatment course at Nagoya University Hospital, medical treatment was initiated before performing balloon pulmonary angioplasty (BPA)/pulmonary endarterectomy (PEA). Right heart catheterization was performed at pre-medication, post-medication, and post-BPA/PEA. Seven patients had concomitant MPN as the underlying cause for CTEPH, accounting for 5.4% (7/129) of CTEPH cases. Baseline clinical characteristics were comparable between the MPN and non-MPN groups. No significant difference was observed in ∆pulmonary vascular resistance (PVR) from baseline to post-medical treatment (-3.59 WU in the MPN group vs. -3.80 WU in the non-MPN group; P=0.67) and baseline to post-BPA/PEA (-6.92 WU in the MPN group vs. -7.60 WU in the non-MPN group; P=0.35). As hemodynamic responses to treatment in CTEPH patients with MPN were comparable with those without MPN, their prognosis may improve with multimodal CTEPH treatment similar to non-MPN cases.
Residual physiological abnormalities after pulmonary endarterectomy and balloon pulmonary angioplasty in CTEPH.
Pulmonary endarterectomy (PEA) is the first-line treatment for chronic thromboembolic pulmonary hypertension (CTEPH), while balloon pulmonary angioplasty (BPA) is an established alternative for inoperable patients. Although both interventions improve resting pulmonary hemodynamics, the extent of long-term physiological recovery during exercise and the persistence of functional limitations remain incompletely characterized. Prospective single-center registry (2017-2023) including 14 patients completing BPA (71 sessions) and 15 undergoing PEA, with median follow-up of 50 months (IQR 36-61). Clinical assessment included resting hemodynamics, invasive exercise right heart catheterization to derive the exercise slope of the mean pulmonary arterial pressure to cardiac output relashionship (mPAP/CO slope), and health-related quality of life (HRQOL) evaluated using the SF-36 questionnaire. Analyses were descriptive and focused on within-pathway changes over time. Both BPA and PEA significantly reduced mPAP (44.8 ± 12.4 → 26.1 ± 9.3 mmHg; 42.1 ± 12.9 → 22.6 ± 5.4 mmHg, both p < 0.001) and pulmonary vascular resistance (9.8 ± 4.6 → 3.0 ± 1.3 WU; 9.0 ± 5.4 → 2.9 ± 1.9 WU, both p < 0.001) at long term follow-up. Despite sustained improvements in resting hemodynamics, abnormal exercise pulmonary vascular responses persisted, with mean mPAP/CO slopes of 7.0 ± 5.6 mmHg/L/min after BPA and 4.0 ± 2.3 mmHg/L/min after PEA. Physical HRQOL remained impaired at long-term follow-up, with Physical Component Summary (PCS) scores below population norms in both pathways (44.4 ± 12.7 after BPA and 44.5 ± 7.3 after PEA). BPA and PEA provide durable improvements in resting pulmonary hemodynamics; however, incomplete physiological recovery is common, with persistent exercise abnormalities and reduced physical quality of life at long-term follow-up.
Clinical Profile and Admission Outcomes of Patients With Acute Pulmonary Embolism at a Resource-Constrained Hospital in Ghana: A Retrospective Pilot Study.
Pulmonary embolism is a cardiovascular disease associated with significant morbidity and mortality. There is a paucity of literature regarding this condition from the Ghanaian perspective. This study aimed at evaluating the clinical profile and admission outcomes of patients with acute pulmonary embolism at the Methodist Hospital, Wenchi, Ghana. A retrospective pilot study was conducted by analyzing medical records of patients diagnosed with acute pulmonary embolism from September 2023 to March 2025. Data on demographics, clinical manifestations, risk factors, investigations, treatment, and outcomes of admission were collected. Out of 17 patients enrolled in the study, 11 (64.7%) were females. The mean age was 58.8 ± 13.0 years. The commonest risk factors were age > 65 years (n = 6), immobilization (n = 5), obesity (n = 4), and recent surgery (n = 3). Hypertension and diabetes mellitus were the predominant comorbidities. Dyspnea (76.5%, n = 13) and chest pain (n = 5) were the most frequent presenting symptoms while tachypnea (70.6%, n = 12), tachycardia (70.6%, n = 12), and hypoxia (52.9%, n = 9) were the commonest clinical signs. Three patients had hemodynamic instability. Pretest probability assessment was infrequently performed (n = 7). Sinus tachycardia (76.5%, n = 13) was the predominant electrocardiographic abnormality. The majority (58.8%, n = 10) had intermediate-risk pulmonary embolism whereas 3 patients were classified as having high-risk pulmonary embolism. Two in-hospital mortalities were recorded. Clinical features and predisposing factors were largely similar to those described in the literature. Also, pretest probability assessment was underutilized. In-hospital mortality rate (11.8%) was relatively high. Large-scale, multicenter prospective studies are recommended to assess long-term complications of acute pulmonary embolism such as recurrence and chronic thromboembolic pulmonary hypertension.
Notch signaling enhances PASMC proliferation and vascular remodeling in CTEPH.
The Notch signaling pathway is implicated in pulmonary vascular remodeling (PVR) in Chronic Thromboembolic Pulmonary Hypertension (CTEPH). This study investigated the role of Notch signaling in PVR using a rat model of CTEPH and performed measurements including Right Ventricular Systolic Pressure (RVSP), Mean Pulmonary Arterial Pressure (MPAP), and histological analysis of pulmonary small arteries. Western blot analysis revealed elevated levels of Notch1, Notch3, Jagged1, and Hes1 proteins in CTEPH rats. Furthermore, the Notch pathway proteins (Notch1, Notch3, Jagged1, and Hes1) were even more elevated in the CTEPH + VPA group. In contrast, protein levels were reduced in the CTEPH + DAPT (a γ-secretase inhibitor) group. The medial wall thickness percentage (MWT%) and markers of smooth muscle cell proliferation, including PCNA and α-SMA, were significantly increased in the CTEPH and CTEPH + VPA groups and decreased after DAPT treatment. These findings suggest that activation of the Notch signaling pathway promotes the proliferation of Pulmonary Artery Smooth Muscle Cells (PASMCs), contributing to PVR in CTEPH. Inhibition of Notch signaling with DAPT mitigated vascular remodeling, suggesting a potential therapeutic target for CTEPH.
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The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationA Phase 2, Randomized, Clinical Trial of Inhaled Nitric Oxide for Acute Severe Right Heart Failure With Pulmonary Hypertension (PHiNO Study).
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European radiology experimentalAnimal models of chronic thromboembolic pulmonary hypertension.
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European journal of internal medicineRespond to "comments and opinions regarding "High antiphospholipid antibody titers and outcomes of pulmonary endarterectomy: a single-center retrospective observational cohort study" by Miard et al.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation[Use of modified balloon pulmonary angioplasty in the treatment of chronic thromboembolic pulmonary hypertension: a retrospective study from a single center].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesApplication of murine hindlimb deep vein thrombosis model to assess temporal evolution and hemodynamic sequelae of pulmonary embolism.
Biochemical and biophysical research communicationsChronic thromboembolic pulmonary hypertension: insights from the 100 most cited papers: a bibliometric perspective.
Journal of thoracic diseaseRole of PKM2 Tetramer in Modulating Endothelial Mitochondrial Dysfunction in a Rat Model of Chronic Thromboembolic Pulmonary Hypertension.
Physiological researchPerioperative Use of Pulmonary Artery Catheter in a Patient With Severe Chronic Thromboembolic Pulmonary Hypertension Undergoing Major Gynecological Surgery: A Case Report.
CureusPrevalence, Clinical Features, and Treatment Response of Chronic Thromboembolic Pulmonary Hypertension in Patients With Myeloproliferative Neoplasm.
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Open life sciencesPost-pulmonary embolism syndrome: a practical narrative review.
Revista clinica espanola[Rehabilitation after pulmonary Embolism].
Die Rehabilitation2026 AHA/ACC/ACCP/ACEP/CHEST/SCAI/SHM/SIR/SVM/SVN Guideline for the Evaluation and Management of Acute Pulmonary Embolism in Adults: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.
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European heart journal. Case reportsDiagnostic Performance of Quantitative Lung Perfusion SPECT/CT for Chronic Thromboembolic Pulmonary Hypertension: A Pilot Study.
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Echocardiography (Mount Kisco, N.Y.)Expanding the boundaries of PTE: successful integration with multiple major cardiac procedures.
Journal of surgical case reportsObstructive sleep apnea and echocardiographic indicators of elevated pulmonary hypertension probability after pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension.
Sleep medicineTemporal Trends in Age Profile and Safety of Balloon Pulmonary Angioplasty for CTEPH.
JACC. AsiaImpact of Preoperative Hemodynamic Values on Development of Reperfusion Edema After Pulmonary Endarterectomy.
Pulmonary circulationVascular Obstruction Scoring on Dual-energy CT, Cone-beam CT and Digital Subtraction Angiography: Correlation with Invasive Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension.
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ERJ open researchHow long does chronic thromboembolic pulmonary hypertension take to develop? Two cases of post-thrombotic complete occlusion of the left pulmonary artery.
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Frontiers in medicineRole and interpretation of lung scintigraphy for long-term management of pulmonary embolism.
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The European respiratory journalDevelopment and Characterization of a Novel Chronic Thromboembolic Pulmonary Hypertension Rat Model: Identifying Sell-Podxl as Potential Regulators.
Hypertension (Dallas, Tex. : 1979)Stress testing in the diagnosis and early screening of chronic thromboembolic pulmonary disease with and without pulmonary hypertension.
European journal of clinical investigationRole of hsa-miR-122-5p in chronic thromboembolic pulmonary hypertension and chronic thromboembolic disease: Targeting PIK3CG in the PI3K/Akt signaling pathway.
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The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of...Prognostic Value of Acute Vasoreactivity in Chronic Thromboembolic Pulmonary Hypertension.
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Kardiologia polskaComparative outcomes of ultrasound-assisted catheter-directed thrombolysis between patients with acute and acute-on-chronic pulmonary embolism.
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Pulmonary circulationInflammation-Based Hematologic Indices as Prognostic Markers in Pulmonary Arterial and Chronic Thromboembolic Pulmonary Hypertension: A Hypothesis-Generating Registry Study.
International journal of molecular sciencesGenetic Analysis of Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Single-Center Observational Study.
GenesResults of the MACiTEPH study of macitentan for the treatment of inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationDual-energy computed tomography (CT) versus cone-beam computed tomography (CT) in chronic thromboembolic pulmonary hypertension: diagnostic accuracy compared with digital subtraction angiography.
Clinical radiologyEnhancing methodological rigour in meta-analyses of renal dysfunction and outcomes in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
The European respiratory journalQuantifying right ventricular fibrosis burden in chronic thromboembolic pulmonary hypertension via fibroblast activation protein inhibitor single photon emission computed tomography/computed tomography: Correlation with echocardiographic parameters and balloon pulmonary angiography response.
Journal of nuclear cardiology : official publication of the American Society of Nuclear CardiologyThe relationship between preoperative clinical, respiratory, and hemodynamic parameters and the surgical anatomical classification of thrombi in patients with chronic thromboembolic pulmonary hypertension.
BMC pulmonary medicineCardiovascular MRI-based Biventricular Perfusion Assessment in Two Patients with Chronic Thromboembolic Pulmonary Hypertension Undergoing Pulmonary Thromboendarterectomy.
Radiology. Cardiothoracic imagingA scoping review of photon-counting detector computed tomography in cardiovascular imaging.
Diagnostic and interventional radiology (Ankara, Turkey)Circulating Pin1 Levels in Patients With Chronic Thromboembolic Pulmonary Hypertension: A Case-Control Study.
Pulmonary circulationCatheter-directed versus standard therapy in intermediate-high-risk pulmonary embolism: A retrospective cohort study.
Archives of cardiovascular diseasesAmerican Lung Association Pulmonary Hypertension Roundtable: Executive Summary.
Pulmonary circulationProximal pulmonary artery wall shear stress derived from computational fluid dynamics: A noninvasive biomarker for CTEPH and perfusion mismatch.
Physiological reportsIn situ pulmonary arterial thrombosis in pulmonary arterial hypertension: Diagnostic differentiation, imaging criteria, and management-A narrative review with an institutional case-series snapshot.
Current problems in cardiologyPulmonary artery fresh floating thrombus in patients with chronic thromboembolic pulmonary hypertension on low-dose direct oral anticoagulants: A single-center angiographic observational study.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationCharacteristics of ferritinophagy-related genes in chronic thromboembolic pulmonary hypertension, a study based on bioinformatics with machine learning model.
Respiratory medicineMagnetic resonance direct thrombus imaging to distinguish acute pulmonary embolism from chronic thromboembolic pulmonary hypertension: a case report.
European heart journal. Case reportsThe activin pathway in chronic thromboembolic pulmonary hypertension: a potential biomarker for residual pulmonary hypertension after endarterectomy.
ERJ open researchFrom Pathways to Patients: Contemporary Approaches to Pulmonary Hypertension.
Cardiology in reviewBalloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension: A Nationwide Prospective Multicenter Registry in Japan (J-BPA).
Circulation. Cardiovascular interventionsChronic thromboembolic pulmonary hypertension associated with venous thoracic outlet syndrome: a retrospective case series.
The European respiratory journalIncreased lung [18F]-FDG uptake in chronic thromboembolic pulmonary hypertension with distal involvement.
The European respiratory journalThe Predictive Value of Quantitative Analysis of SPECT V/Q Imaging for Postoperative Persistent/Recurrent Pulmonary Hypertension in Patients With Chronic Thromboembolic Pulmonary Hypertension After Pulmonary Endarterectomy.
Pulmonary circulationEmergency Pulmonary Thromboendarterectomy in Malignancy.
JACC. Case reportsCausal Effect of Pulmonary Hypertension on Brain Structure: A Mendelian Randomization Study Combined With Brain Magnetic Resonance Imaging.
CureusThe expanding role of intraoperative transesophageal echocardiography in pulmonary endarterectomy for CTEPH.
Indian journal of thoracic and cardiovascular surgeryBridging to a delayed pulmonary endarterectomy in massive acute-on-chronic pulmonary thromboembolism: a case report.
Journal of cardiothoracic surgeryA Tale of Two Pathologies: Unraveling CTEPH and PVOD in Pulmonary Hypertension.
American journal of respiratory and critical care medicineDiagnosis model for assessing chronic thromboembolic pulmonary hypertension in high-altitude pulmonary embolism patients: a machine learning approach.
Frontiers in medicineFactors affecting patient outcomes in pulmonary artery thromboendarterectomy under deep hypothermic circulatory arrest and cardiopulmonary bypass support----a single center's experience.
BMC cardiovascular disordersPulmonary Blood Volumes on CT Predict Residual Pulmonary Hypertension Post-Pulmonary Endarterectomy.
American journal of respiratory cell and molecular biologyImpact of Elevated Pulmonary Arterial Wedge Pressure on Safety and Efficacy of Balloon Pulmonary Angioplasty in the Treatment of Chronic Thromboembolic Pulmonary Hypertension.
ChestIntravenous Treprostinil in Precapillary and Postcapillary Pulmonary Hypertension Due to Cardiac Amyloidosis and Aortic Stenosis.
JACC. Case reportsLung transplantation for chronic thromboembolic pulmonary hypertension (CTEPH): A treatment of last resort.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationPatient-Specific Haemodynamic Modeling to Estimate the Extent of Microvascular Disease and Response to Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension.
Pulmonary circulationThe Surgical Management of Chronic Thromboembolic Pulmonary Hypertension.
Journal of clinical medicinePulmonary endarterectomy for chronic thromboembolic pulmonary hypertension after bilateral lung transplantation.
JHLT openProteomic insights into platelet dysregulation and pathogenic mechanisms of chronic thromboembolic pulmonary hypertension.
Journal of translational medicineEvaluation of gene and microRNA expression patterns in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension as rare diseases.
Respiratory medicineLung transplantation for chronic thromboembolic pulmonary hypertension-a case series.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationAn Unusual Initial Manifestation of Pulmonary Embolism: Aseptic Cavitary Infarction With Pulmonary Hypertension-Case Report and Literature Review.
Respirology case reportsQuantitative Evaluation of Lung Perfusion by Dynamic Chest Radiography in Chronic Thromboembolic Pulmonary Hypertension: Comparison With Lung Perfusion Scintigraphy.
Pulmonary circulation[Chronic Thromboembolic Pulmonary Hypertension].
Pneumologie (Stuttgart, Germany)[Innovations in the Field of Pulmonary Hypertension - Critical Assessment of the Results of the 7th World Symposium on Pulmonary Hypertension].
Pneumologie (Stuttgart, Germany)Use of direct anticoagulants in chronic thromboembolic pulmonary hypertension: An anatomopathological study of endarterectomy material.
International journal of cardiologyThe influence factors on the resolution of pulmonary embolism by CT pulmonary angiography.
Thrombosis journalBalloon Pulmonary Angioplasty Versus Riociguat in Inoperable Chronic Thromboembolic Pulmonary Hypertension: Systematic Review and Meta-Analysis of Randomized Controlled Trials.
Cardiology in reviewLong-Term Outcomes of Mechanical Thrombectomy Versus Catheter-Directed Thrombolysis.
Journal of the Society for Cardiovascular Angiography & InterventionsEndothelin-1 overexpression in pulmonary endarterectomy specimens of CTEPH patients is associated with pulmonary hypertension development.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationTricuspid Regurgitation and Pulmonary Haemodynamics After Pulmonary Endarterectomy.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryCongenital Thrombophilia in Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Systematic Review of Prevalence, Clinical Phenotype, and Surgical Outcomes.
BiomedicinesA machine learning approach to identify patients at risk for long-term consequences after pulmonary embolism.
Scientific reportsPractical guide to management after an acute pulmonary embolism.
BMJ open respiratory researchImaging in chronic thromboembolic pulmonary hypertension: review of the current literature.
Diagnostic and interventional radiology (Ankara, Turkey)Gas exchange dynamics and responses to exercise in chronic thromboembolic pulmonary hypertension.
Respiratory physiology & neurobiologyEffectiveness and Safety of Reduced-Dose and Slow-Infusion Intravenous Tissue-Type Plasminogen Activator Regimen in Patients with Acute Pulmonary Embolism at Intermediate-High Risk.
Anatolian journal of cardiologyA Rare Case of Chronic Thromboembolic Pulmonary Hypertension in an Elderly With Atrial Septal Defect and Eisenmenger Syndrome.
CureusLong-term in-hospital mortality and chronic thromboembolic pulmonary hypertension after COVID-19-associated pulmonary embolism in France: a nationwide study.
ERJ open researchEffects of Web-Based Symptom Monitoring Program on Symptom Interference, Physical Activity, and Emergency Department Readmissions in Patients With Pre-Capillary Pulmonary Hypertension: Randomized Controlled Trial.
Journal of medical Internet researchPulmonary thrombectomy and extracorporeal membrane oxygenation: a case study.
The journal of extra-corporeal technologyEvaluating NT-proBNP-to-Albumin (NTAR) and RDW-to-eGFR (RGR) Ratios as Biomarkers for Predicting Hospitalization Duration and Mortality in Pulmonary Arterial Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH).
Diagnostics (Basel, Switzerland)Riociguat for CTEPH: A retrospective cohort study from a single reference center in Brazil.
Clinics (Sao Paulo, Brazil)Spatial heterogeneity and distribution of CT-Based pulmonary vascular volumes in chronic thromboembolic pulmonary hypertension.
European journal of radiologyClinical impact of mean pulmonary arterial pressure after balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension.
Open heartCT scoring system to defined thrombus distribution in chronic thromboembolic pulmonary hypertension.
The British journal of radiologyChronic Thromboembolic Pulmonary Hypertension in Latvia: Epidemiological Insights and Diagnostic Challenges From 2024.
Pulmonary circulationHigh Antiphospholipid Antibody Titers and Outcomes of Pulmonary Endarterectomy: A Single-Center Retrospective Observational Cohort Study.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationRenal Outcomes After Lung or Combined Heart-Lung Transplantation in Pulmonary Hypertension.
Pulmonary circulationSequential Combination of Balloon Pulmonary Angioplasty for Distal Lesions in the Left Pulmonary Artery Followed by Pulmonary Endarterectomy for Central Lesions in the Right Pulmonary Artery: A Case Report.
Pulmonary circulationPulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: Technical challenges and controversies.
JHLT openKlippel-Trenaunay Syndrome with Chronic Thromboembolic Pulmonary Hypertension Treated with Balloon Pulmonary Angioplasty.
Pneumologie (Stuttgart, Germany)Assessment of Right Ventricular Pressure in Chronic Thromboembolic Pulmonary Hypertension: Comparison of Diagnostic Modalities and Balloon Pulmonary Angioplasty Outcomes.
Diagnostics (Basel, Switzerland)The Effect of Eccentric Cycling on Cerebral and Muscle Tissue Oxygenation in Patients with Pulmonary Hypertension and Healthy Individuals: A Randomized Controlled Crossover Trial.
Journal of clinical medicineEcho-Doppler Predictors of Residual Pulmonary Hypertension After Pulmonary Thromboendarterectomy.
Journal of clinical medicineImpact of the Balloon Pulmonary Angioplasty on the Diagnosis of Chronic Thromboembolic Pulmonary Hypertension: From the COMMAND VTE Registry-2.
Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & InterventionsUtility of machine learning for predicting severe chronic thromboembolic pulmonary hypertension based on CT metrics in a surgical cohort.
European radiologyCombined multimodal therapy in high-risk patients with chronic thromboembolic pulmonary hypertension and both distal and proximal lesions: A prospective observational cohort study.
The Journal of heart and lung transplantation : the official publication of the International Society for Heart TransplantationHemodynamics assessment using 4D flow CMR before and after balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: a retrospective observational study.
BMC cardiovascular disordersDetectability of subsegmental lesions in patients with inoperable CTEPH: Comparison between ultra-high-resolution vs. conventional CT.
JHLT openPathogenesis, Diagnosis, and Management of Chronic Thromboembolic Pulmonary Hypertension.
Cardiac electrophysiology clinicsManagement of Complications of Balloon Pulmonary Angioplasty.
Cardiac electrophysiology clinicsOutpatient Follow-up of Pulmonary Embolism: Putting It all Together.
Cardiac electrophysiology clinicsEvolving Role and Clinical Evidence in the Global Practice of Balloon Pulmonary Angioplasty.
Cardiac electrophysiology clinicsAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Hipertensão pulmonar tromboembólica crônica 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
Existe um documento oficial para "Hipertensão Arterial Pulmonar". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
A condição é causada pelo acúmulo e permanência de coágulos organizados que cicatrizam e obstruem as artérias dos pulmões. Isso eleva a resistência para a passagem do sangue, resultando em hipertensão pulmonar e sobrecarga no 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 pulmonar tromboembólica crônica. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hipertensao-pulmonar-tromboembolica-cronica
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hipertensão pulmonar tromboembólica crônica
📋 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
- Genética mendeliana
- fonte: OMIM
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata
- Medicamentos aprovados FDA
- fonte: FDA OpenFDA
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
