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Acute pulmonary embolism (APE) is one of the leading causes of cardiovascular emergencies and the third leading cause of death. Although efforts focus on treating the acute event, patients who survive APE may develop long-term sequelae. Research reveals that approximately half of patients who have suffered an APE do not regain their previous level of function and experience a reduction in their quality of life for several years after the episode. Acute pulmonary embolism can be classified according to the risk of short-term mortality, with most mortality and morbidity concentrated in high-risk and intermediate-risk cases. The first-line treatment for APE is systemic anticoagulation. However, identifying and more aggressively treating people with intermediate to high risk, who have a more favorable risk profile for reperfusion treatments, could reduce short-term mortality and mitigate post-pulmonary embolism syndrome (PPES). Post-pulmonary embolism syndrome refers to a variety of persistent symptoms and functional limitations that occur after an APE. The presence of persistent dyspnea, functional limitations, and/or decreased quality of life after an APE has been recently termed "PPES," although this entity encompasses different manifestations. The most severe cause of persistent dyspnea is chronic thromboembolic pulmonary hypertension, where increased pulmonary artery pressure is due to the fibrotic organization of unresolved APE. Post-PE Syndrome is not always systematically addressed in management guidelines, and its prevalence may be underestimated. More research is needed to fully understand its causes and risk factors. Interventions such as cardiopulmonary rehabilitation have been suggested to improve the quality of life of patients with PPES. A comprehensive, evidence-based approach is essential to effectively prevent and manage PPES and improve the long-term outcomes and well-being of affected patients.
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Embolia Pulmonar , Calidad de Vida , Humanos , Embolia Pulmonar/terapia , Embolia Pulmonar/fisiopatología , Factores de Riesgo , Síndrome , Resultado del Tratamiento , Anticoagulantes/uso terapéutico , Anticoagulantes/efectos adversos , Disnea/etiología , Disnea/fisiopatología , Medición de Riesgo , Factores de Tiempo , Hipertensión Pulmonar/fisiopatología , Hipertensión Pulmonar/etiología , Estado FuncionalRESUMEN
Background: Cardiopulmonary exercise testing (CPET) assesses exercise capacity and causes of exercise limitation in patients with pulmonary hypertension (PH). At altitude, changes occur in the ventilatory pattern and a decrease in arterial oxygen pressure in healthy; these changes are increased in patients with cardiopulmonary disease. Our objective was to compare the response to exercise and gas exchange between patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) residing at the altitude of Bogotá (2640 m). Methods: All patients performed an incremental CPET with measurement of oxygen consumption ( VO 2 ), dead space (VD/VT), ventilatory equivalents (VE/ VCO 2 ), and alveolar-arterial oxygen gradient ( PA-aO 2 ). X 2 test and one-way analysis of variance were used for comparisons between PAH and CTEPH. Results: We included 53 patients, 29 with PAH, 24 with CTEPH, and 102 controls as a reference of the normal response to exercise at altitude. CTEPH patients had a higher New York Health Association (NYHA) functional class than PAH (p = 0.037). There were no differences between patients with PAH and CTEPH in hemodynamics and VO 2 % of predicted (67.8 ± 18.7 vs. 66.0 ± 19.8, p < 0.05), but those with CTEPH had higher dyspnea, VD/VT (0.36 ± 0.09 vs. 0.23 ± 0.9, p < 0.001), VE/ VCO 2 (45.8 ± 7.1 vs. 39.3 ± 5.6, p < 0.001), and PA-aO 2 (19.9 ± 7.6 vs. 13.5 ± 7.6, p < 0.001) than PAH patients. Conclusions: At altitude, patients with PH present severe alterations in gas exchange during exercise. There were no differences in exercise capacity between PAH and CTEPH, but patients with CTEPH had more dyspnea and greater alterations in gas exchange during exercise. CPET made it possible to identify alterations related to the pathophysiology of CTEPH that could explain the functional class and dyspnea in these patients.
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PURPOSE: Pulmonary thromboendarterectomy (PTE) is the treatment for patients with chronic thromboembolic disease. In the immediate postoperative period, some patients may still experience life-threatening complications such as reperfusion lung injury, airway bleeding, and persistent pulmonary hypertension with consequent right ventricular dysfunction. These issues may require support with extracorporeal membrane oxygenation (ECMO) as a bridge to recovery or lung transplantation. This study aims to analyze our series of PTEs that require ECMO. METHODS: A descriptive and retrospective analysis of all PTE performed at the Favaloro Foundation University Hospital was conducted between March 2013 and December 2023. RESULTS: A total of 42 patients underwent PTE with a median age of 47 years (interquartile range: 26-76). The incidence of patients with ECMO was 26.6%, of which 53.6% were veno-venous (VV) ECMO. Preoperatively, a low cardiac index (CI), high right and left filling pressures, and high total pulmonary vascular resistances (PVRs) were associated with ECMO with a statistically significant relationship. The hospital mortality was 11.9%, and the mortality in the ECMO group was 45.5%, with a statistically significant relationship. Veno-arterial ECMO has a worse prognosis than VV ECMO. CONCLUSIONS: Preoperatively, a low CI, high right and left filling pressures, and high total PVRs were associated with ECMO after PTE.
Asunto(s)
Endarterectomía , Oxigenación por Membrana Extracorpórea , Mortalidad Hospitalaria , Embolia Pulmonar , Humanos , Oxigenación por Membrana Extracorpórea/mortalidad , Oxigenación por Membrana Extracorpórea/efectos adversos , Persona de Mediana Edad , Endarterectomía/efectos adversos , Endarterectomía/mortalidad , Masculino , Estudios Retrospectivos , Femenino , Resultado del Tratamiento , Adulto , Anciano , Embolia Pulmonar/mortalidad , Embolia Pulmonar/cirugía , Embolia Pulmonar/fisiopatología , Factores de Tiempo , Factores de Riesgo , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/cirugía , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/fisiopatología , Hipertensión Pulmonar/cirugíaRESUMEN
Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.
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Chronic thromboembolic pulmonary hypertension (CTEPH) is a subtype of pulmonary hypertension characterized by the obstruction of pulmonary arteries secondary to chronic thromboembolism. Pulmonary thromboendarterectomy surgery (PTE) is the main treatment for patients with CTEPH, as it removes the chronic thrombi from the pulmonary arteries. Pulmonary reperfusion syndrome is a common complication of the surgery, which involves the development of pulmonary edema in the area where blood perfusion improves after the surgery. The incidence of this syndrome varies from 8 to 91% depending on the criteria used for diagnosis, and it is one of the most serious complications of pulmonary thromboendarterectomy. In such cases, circulatory support with extracorporeal membrane oxygenation (ECMO) has become a valuable therapeutic modality. We present the case of a 60-year-old woman with a history of acute pulmonary embolism due to deep vein thrombosis of the right pelvic limb who was diagnosed later with CTEPH who was admitted for scheduled surgical treatment involving bilateral PTE. However, during the immediate postoperative period, she developed cardiogenic shock and refractory hypoxemia secondary to pulmonary reperfusion syndrome following the surgical procedure. As a result, she required veno-venous ECMO circulatory support for 6 days, leading to resolution of the pulmonary condition and clinical improvement.
La hipertensión pulmonar tromboembólica crónica (HPTEC) es un subtipo de hipertensión pulmonar caracterizada por la obstrucción de las arterias pulmonares secundaria a tromboembolias crónicas. La cirugía de tromboendarterectomía pulmonar (TEAP) es el tratamiento principal para los pacientes con HPTEC, elimina los trombos crónicos de las arterias pulmonares. El síndrome de reperfusión pulmonar es una complicación común de la cirugía, se trata del desarrollo de edema pulmonar en el área en la que la perfusión sanguínea mejora después de la cirugía. La incidencia del síndrome varía del 8 al 91% según los criterios utilizados para diagnosticarlo y es una de las complicaciones más graves de la tromboendarterectomía pulmonar. En tales casos, el soporte circulatorio con oxigenación por membrana extracorpórea (ECMO) se ha convertido en una valiosa modalidad terapéutica. Presentamos el caso de una paciente de 60 años de edad con antecedente de tromboembolia pulmonar aguda secundaria a trombosis venosa profunda de miembro pélvico derecho a quien durante el seguimiento se realizó el diagnóstico de HPTEC e ingresó de manera programada para tratamiento quirúrgico con realización de TEAP bilateral, sin embargo durante el posquirúrgico inmediato presentó choque cardiogénico e hipoxemia refractaria secundarios a síndrome de reperfusión pulmonar, por lo cual requirió soporte circulatorio con ECMO venovenosa durante seis días, con resolución del cuadro pulmonar y mejoría clínica.
Asunto(s)
Endarterectomía , Oxigenación por Membrana Extracorpórea , Complicaciones Posoperatorias , Edema Pulmonar , Embolia Pulmonar , Humanos , Femenino , Oxigenación por Membrana Extracorpórea/métodos , Endarterectomía/métodos , Persona de Mediana Edad , Edema Pulmonar/etiología , Embolia Pulmonar/etiología , Complicaciones Posoperatorias/etiología , Hipertensión Pulmonar/etiología , Arteria Pulmonar/cirugíaRESUMEN
Introducción: La tromboendarterectomía pulmonar (TEA) es la principal herramienta terapéutica en hipertensión pulmonar tromboembólica crónica (HPTEC), potencialmente curativa. Se analiza la experiencia de 13 años de TEAs de pacientes uruguayos en el marco del convenio con el Hospital Universitario Fundación Favaloro (HUFF-Argentina). Metodología: Estudio analítico, observacional y retrospectivo de todas las TEAs realizadas entre 2011 y 2023. Resultados: Se realizaron 15 TEAs. 46 ± 17 años, 67% hombres. Diez pacientes tenían antecedentes de enfermedad tromboembólica previa y 8 hipercoagulabilidad. El tiempo desde el inicio de los síntomas al diagnóstico fue de 36 (22-78) meses. Ochenta por ciento se encontraba en CF III, con una distancia total recorrida en la prueba de la marcha de 6 minutos de 375 (272-458) metros, severidad ecocardiográfica (TAPSE/PAPs 0,22 ± 0,08 mm/mmHg) y hemodinámica (RVP 11 ± 5 UW) que mejoraron significativamente en el posoperatorio temprano. La mortalidad intrahospitalaria fue del 20% que se mantuvo en el seguimiento de 34 (7-97) meses excepto un paciente que murió por una nueva embolia de pulmón a los 3 años. Todos los fallecidos tenían una hemodinamia preoperatoria significativamente más grave (RVP 19 ± 6 UW, índice cardíaco 1,5 ± 0,4 L/min/m2). Seis sobrevivientes (50%) presentaron HP residual asociada a una mayor PAPm y RVP preoperatorias y menor recuperación funcional y ecocardiográfica a corto plazo (P <0,05), dos de los cuales recibieron terapia específica posterior al procedimiento. Conclusiones: La TEA determinó mejoría clínica, ecocardiográfica y hemodinámica. Seis supervivientes (50%) normalizaron la hemodinamia de reposo. La severidad hemodinámica preoperatoria dado por las lesiones obstructivas y microangiopatía distales se asoció con una alta tasa de complicaciones y mortalidad perioperatorias. La reducción del retardo en el diagnóstico y tratamiento junto con una mayor tasa de TEAs/año podrían reducir las complicaciones y mejorar el pronóstico.
Summary Introduction: Pulmonary thromboendarterectomy (PTE) is the main therapeutic tool for chronic thromboembolic pulmonary hypertension (CTEPH), and is potentially curative. The experience of 13 years of PTEs on Uruguayan patients under the agreement with the University Hospital Fundación Favaloro (HUFF-Argentina) is analyzed. Methodology: Analytical, observational, and retrospective study of all PTEs performed between 2011 and 2023. Results: Fifteen PTEs were performed. Age was 46 ± 17 years, 67% male. Ten patients had a history of prior thromboembolic disease, and 8 had hypercoagulability. The time from symptom onset to diagnosis was 36 (22-78) months. Eighty percent were in NYHA Functional Class III, with a total distance walked in the 6-minute walk test of 375 (272-458) meters, echocardiographic severity (TAPSE/sPAP 0,22 ± 0,08 mm/mmHg), and hemodynamic severity (PVR 11 ± 5 WU) which significantly improved in the early postoperative period. In-hospital mortality was 20% and remained at a follow-up of 34 (7-97) months, except for one patient who died from a new pulmonary embolism at 3 years. All deceased patients had significantly more severe preoperative hemodynamics (PVR 19 ± 6 WU, cardiac index 1,5 ± 0,4 L/min/m²). Six survivors (50%) presented with residual PH associated with higher preoperative mPAP and PVR and lower short-term functional and echocardiographic recovery (P <0,05), two of whom received specific therapy post-procedure. Conclusions: PTE resulted in clinical, echocardiographic, and hemodynamic improvement. Six survivors (50%) normalized resting hemodynamics. Preoperative hemodynamic severity due to distal obstructive lesions and microangiopathy was associated with a high rate of perioperative complications and mortality. Reducing the delay in diagnosis and treatment and a higher rate of PTEs/year could reduce complications and improve prognosis.
Introdução: A tromboendarterectomia pulmonar (TEP) é a principal ferramenta terapêutica na hipertensão pulmonar tromboembólica crônica (HPTEC), potencialmente curativa. Analisa-se a experiência de 13 anos de TEPs em pacientes uruguaios no âmbito do convênio com o Hospital Universitário Fundação Favaloro (HUFF-Argentina). Metodologia: Estudo analítico, observacional e retrospectivo de todas as TEPs realizadas entre 2011 e 2023. Resultados: Foram realizadas 15 TEPs. Idade de 46 ± 17 anos, 67% homens. Dez pacientes tinham antecedentes de doença tromboembólica prévia e 8 hipercoagulabilidade. O tempo desde o início dos sintomas até o diagnóstico foi de 36 (22-78) meses. Oitenta por cento encontravam-se em Classe Funcional III, com uma distância total percorrida no teste de caminhada de 6 minutos de 375 (272-458) metros, gravidade ecocardiográfica (TAPSE/sPAP 0,22 ± 0,08 mm/mmHg) e hemodinâmica (RVP 11 ± 5 UW) que melhoraram significativamente no pós-operatório precoce. A mortalidade intra-hospitalar foi de 20% e manteve-se no seguimento de 34 (7-97) meses, exceto um paciente que faleceu por uma nova embolia pulmonar aos 3 anos. Todos os falecidos apresentavam hemodinâmica pré-operatória significativamente mais grave (RVP 19 ± 6 UW, índice cardíaco 1,5 ± 0,4 L/min/m²). Seis sobreviventes (50%) apresentaram HP residual associada a maior PAPm e RVP pré-operatórias e menor recuperação funcional e ecocardiográfica a curto prazo (P <0,05), dois dos quais receberam terapia específica pós-procedimento. Conclusões: A TEP resultou em melhora clínica, ecocardiográfica e hemodinâmica. Seis sobreviventes (50%) normalizaram a hemodinâmica de repouso. A gravidade hemodinâmica pré-operatória devido a lesões obstrutivas e microangiopatia distal foi associada a uma alta taxa de complicações e mortalidade perioperatória. A redução do atraso no diagnóstico e tratamento juntamente com uma maior taxa de TEPs/ano poderia reduzir as complicações e melhorar o prognóstico.
Asunto(s)
Endarterectomía , Hipertensión Pulmonar/terapia , Argentina , Uruguay , Enfermedad Crónica , Estudios Retrospectivos , Estudio ObservacionalRESUMEN
Objectives: We sought to evaluate the efficacy and safety of refined balloon pulmonary angioplasty (BPA) in the treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH). Background: BPA is rapidly evolving therapeutic option for patients with nonsurgical CTEPH. There are few US studies that have reported on the outcomes of this novel therapeutic option. Methods: This is a retrospective study of CTEPH patients that underwent BPA at Temple University Hospital. The primary efficacy endpoint was the change in pulmonary vascular resistance (PVR) after BPA as compared to baseline and the primary safety endpoint was the rate of hemoptysis within 24 hours. Secondary endpoints included death, WHO functional class, and 6-minute walk distance (6MWD). We used logistic regression to evaluate factors associated with a hemodynamic and functional response. Results: A total of 211 BPA sessions were performed on 77 patients (average 2.7 ± 1.7 sessions/patient). After BPA the mean PVR improved by 26% (P<0.001) while the mean 6MWD improved by 71.7 meters (P <0.001) and WHO functional class improved by one functional class (P <0.001). Ten sessions (4.7%) were complicated by hemoptysis. The independent factors associated with a improved functional and hemodynamic response included the pre-procedural use of riociguat, reduce baseline PA compliance and > 3 BPA sessions per patient. Conclusion: This single center study from the US showed that BPA with refined techniques in patients with CTEPH was safe and was associated with significant improvements in pulmonary hemodynamics and functional capacity.
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OBJECTIVES: Due to Pulmonary Artery Endothelial Cell (PAEC) dysfunction, Pulmonary Hypertension (PH) persists even after the Pulmonary Embolism (PE) has been relieved. However, the mechanism behind this remains unclear. METHOD: Here, the authors incubated Human PAECs (HPAECs) with thrombin to simulate the process of arterial thrombosis. RESULTS: CCK8 results showed a decrease in the viability of HPAECs after thrombin incubation. In addition, the expression of Tissue Factor (TF), Monocyte Chemoattractant Protein 1 (MCP-1), VCAM-1, ICAM-1, cleaved caspase 3, cleaved caspase 9, and Bax protein were all increased after thrombin incubation, while Bcl-2 was decreased. The effects of 3-MA treatment further suggested that autophagy might mediate the partial protective effects of Resveratrol on HPAECs. To observe the effects of Resveratrol in vivo, the authors established a Chronic Thromboembolic Pulmonary Hypertension (CTEPH) model by repeatedly injecting autologous blood clots into a rat's left jugular vein. The results exhibited that Mean Pulmonary Arterial Pressure (mPAP) and vessel Wall Area/Total Area (WA/TA) ratio were both decreased after Resveratrol treatment. Moreover, Resveratrol could reduce the concentration and activity of TF, vWF, P-selectin, and promote these Superoxide Dismutase (SOD) in plasma. Western blot analysis of inflammation, platelet activation, autophagy, and apoptosis-associated proteins in pulmonary artery tissue validated the results in PHAECs. CONCLUSIONS: These findings suggested that reduced autophagy, increased oxidative stress, increased platelet activation, and increased inflammation were involved in CTEPH-induced HPAEC dysfunction and the development of PH, while Resveratrol could improve PAEC dysfunction and PH.
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Hipertensión Pulmonar , Embolia Pulmonar , Animales , Autofagia , Células Endoteliales , Humanos , Inflamación , Estrés Oxidativo , Arteria Pulmonar , Ratas , Resveratrol , TrombinaRESUMEN
BACKGROUND: Patients with acute decompensation of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) admitted to intensive care unit (ICU) have high in-hospital mortality. We hypothesized that pulmonary hypertension (PH) severity, measured by a simplified version of European Society of Cardiology/European Respiratory Society (ESC/ERS) risk assessment, and the severity of organ dysfunction upon ICU admission, measured by sequential organ failure assessment score (SOFA) were associated with in-hospital mortality in decompensated patients with PAH and CTEPH. We also described clinical and laboratory variables during ICU stay. METHODS: Observational study including adults with decompensated PAH or CTEPH with unplanned ICU admission between 2014 and 2019. Multivariate logistic regression models were used to evaluate the association of ESC/ERS risk assessment and SOFA score with in-hospital mortality. ESC/ERS risk assessment and SOFA score were included in a decision tree to predict in-hospital mortality. RESULTS: 73 patients were included. In-hospital mortality was 41.1%. ESC/ERS high-risk group (adjusted odds ratio = 95.52) and SOFA score (adjusted odds ratio = 1.80) were associated with in-hospital mortality. The decision tree identified four groups with in-hospital mortality between 8.1% and 100%. Nonsurvivors had a lower central venous oxygen saturation, higher arterial lactate and higher brain natriuretic peptide in the end of first week in the ICU. CONCLUSIONS: High-risk on a simplified version of ERS/ESC risk assessment and SOFA score upon ICU admission are associate with in-hospital mortality. A decision tree based on ESC/ERS risk assessment and SOFA score identifies four groups with in-hospital mortality between 8.1% and 100%.
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Hipertensión Pulmonar/mortalidad , Unidades de Cuidados Intensivos , Adulto , Brasil/epidemiología , Estudios de Cohortes , Femenino , Mortalidad Hospitalaria , Humanos , Ácido Láctico/sangre , Masculino , Persona de Mediana Edad , Péptido Natriurético Encefálico/sangre , Puntuaciones en la Disfunción de Órganos , Saturación de Oxígeno , Pronóstico , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
La enfermedad por coronavirus 2019 (CoViD-19) está causada por el virus del síndrome respiratorio agudo severo por coronavirus 2 (SARS-CoV-2), siendo particularmente perjudicial para los pacientes con enfermedad cardiovascular subyacente, y provocando una causa de morbilidad y mortalidad significativas en todo el mundo. Este virus lleva a una neumopatía, al tiempo que causa lesiones agudas de miocardio y daño crónico al sistema cardiovascular. Como consecuencia del daño del parénquima pulmonar y de la circulación pulmonar alterada, puede desarrollarse hipertensión pulmonar (HP), con su respectiva consecuencia. La fisiopatología de este tipo de HP es compleja y multifactorial, considerándose factores potenciales para las alteraciones de la circulación pulmonar. En estudios recientes, la prevalencia evidenciada de HP en pacientes con CoViD-19 es de alrededor del 12%, pero su evolución aún no está clara. La pandemia de CoViD-19 ha tenido un impacto significativo en todos los aspectos de la HP, desde el diagnóstico y manejo hasta la observación de un mayor riesgo de muerte en pacientes con hipertensión arterial pulmonar (HAP). En una encuesta de 77 centros de atención médica integral de HAP, la incidencia de infección por CoViD-19 fue de 2,1 casos por cada 1000 pacientes con HAP, similar a la incidencia de infección por CoViD-19 en la población general. Si bien, esta pandemia ha alterado el estándar de atención médica de rutina y de manejo agudo, particularmente, en aquellos pacientes con HAP, los riesgos asociados con CoViD-19 son significativos, presentándose nuevos desafíos en el cuidado de pacientes con HP. Dado que los pacientes con HAP han demostrado tener peores resultados en el ámbito de esta pandemia, es esencial trabajar de manera proactiva para disminuir el riesgo de infección por CoViD-19, mientras se continúa brindando un alto nivel de atención médica. El impacto de CoViD-19 en la prestación de atención médica y en la sociedad en general requirió que se establecieran nuevos protocolos para el tratamiento de HAP para disminuir el riesgo de exposición o transmisión de CoViD-19. De manera similar, ha habido una disminución en las pruebas de pacientes estables. Actualmente, la forma en que brindamos la atención médica se evidencia en un aumento de las visitas de telemedicina, una menor exposición a los entornos de atención médica para los pacientes y los profesionales de la salud, ayudando a nuestra necesidad continua de brindar servicios a los pacientes dentro del entorno de CoViD-19 y adaptándonos a una forma diferente de interactuar, ampliando nuestra comprensión de la mejor manera de cuidar a nuestros pacientes.
Coronavirus disease 2019 (CoViD-19) causes severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), being particularly harmful for patients with underlying cardiovascular disease, and causing a cause of significant morbidity and mortality throughout the world. This virus leads to lung disease, while causing acute myocardial injury and chronic damage to the cardiovascular system. As a consequence of the damage to the lung parenchyma and altered pulmonary circulation, pulmonary hypertension (PH) can develop, with its respective consequence. The pathophysiology of this type of PH is complex and multifactorial, considering potential factors for alterations in pulmonary circulation. In recent studies, the evidenced prevalence of PH in patients with CoViD-19 is around 12%, but its evolution is not yet clear. The CoViD-19 pandemic has had a significant impact on all aspects of PH, from diagnosis and management to observing an increased risk of death in patients with pulmonary arterial hypertension (PAH). In a survey of 77 comprehensive PAH healthcare centers, the incidence of CoViD-19 infection was 2.1 cases per 1,000 PAH patients, similar to the incidence of CoViD-19 infection in the general population. Although this pandemic has altered the standard of routine medical care and acute management, particularly in those patients with PAH, the risks associated with CoViD-19 are significant, presenting new challenges in the care of patients with PH. Since PAH patients have been shown to have worse outcomes in the setting of this pandemic, it is essential to work proactively to decrease the risk of CoViD-19 infection, while continuing to provide a high level of medical care. The impact of CoViD-19 on the provision of health care and on society in general required that new protocols be established for the treatment of PAH to reduce the risk of exposure or transmission of CoViD-19. Similarly, there has been a decline in stable patient testing. Currently, the way we provide healthcare is evidenced by an increase in telemedicine visits, less exposure to healthcare settings for patients and healthcare professionals, aiding our continued need to provide services to patients. patients within the CoViD-19 environment and adapting to a different way of interacting, broadening our understanding of the best way to care for our patients
A doença coronavírus 2019 (CoViD-19) causa síndrome respiratória aguda grave coronavírus 2 (SARS-CoV-2), sendo particularmente prejudicial para pacientes com doença cardiovascular subjacente e causando uma importante morbidade e mortalidade em todo o mundo. Este vírus leva à doença pulmonar, enquanto causa lesão aguda do miocárdio e dano crônico ao sistema cardiovascular. Como consequência do dano ao parênquima pulmonar e da circulação pulmonar alterada, pode ocorrer hipertensão pulmonar (HP), com suas respectivas consequências. A fisiopatologia desse tipo de HP é complexa e multifatorial, considerando fatores potenciais para alterações da circulação pulmonar. Em estudos recentes, a prevalência de HP evidenciada em pacientes com CoViD-19 gira em torno de 12%, mas sua evolução ainda não está clara. A pandemia CoViD-19 teve um impacto significativo em todos os aspectos da HP, desde o diagnóstico e tratamento até a observação de um risco aumentado de morte em pacientes com hipertensão arterial pulmonar (HAP). Em uma pesquisa com 77 centros de saúde com HAP abrangentes, a incidência de infecção por CoViD-19 foi de 2,1 casos por 1.000 pacientes com HAP, semelhante à incidência de infecção por CoViD-19 na população em geral. Embora essa pandemia tenha alterado o padrão de cuidados médicos de rotina e tratamento agudo, particularmente em pacientes com HAP, os riscos associados ao CoViD-19 são significativos, apresentando novos desafios no cuidado de pacientes com HP. Como os pacientes com HAP demonstraram ter resultados piores no cenário dessa pandemia, é essencial trabalhar proativamente para diminuir o risco de infecção por CoViD-19, enquanto continua a fornecer um alto nível de cuidados médicos. O impacto do CoViD-19 na prestação de cuidados de saúde e na sociedade em geral exigiu o estabelecimento de novos protocolos para o tratamento da HAP para reduzir o risco de exposição ou transmissão do CoViD-19. Da mesma forma, houve um declínio nos testes de pacientes estáveis. Atualmente, a forma como prestamos serviços de saúde é evidenciada por um aumento nas visitas de telemedicina, menos exposição aos ambientes de saúde para pacientes e profissionais de saúde, auxiliando nossa necessidade contínua de fornecer serviços aos pacientes. Pacientes dentro do ambiente CoViD-19 e adaptando-se de uma maneira diferente de interagir, ampliando nosso entendimento sobre a melhor forma de cuidar de nossos pacientes.
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OBJECTIVES: To describe the characteristics of patients who undergo balloon pulmonary angioplasty (BPA) for inoperable chronic thromboembolic pulmonary hypertension (CTEPH) and report the mid-term outcomes. BACKGROUND: BPA has been recently introduced in Latin America. Mid-term results have not been published. METHODS: Prospective Chilean Registry of inoperable CTEPH patients who underwent BPA. Clinical variables were analyzed at baseline, after each procedure and at follow-up. Hemodynamic variables were recorded before and after the last BPA. RESULTS: Between August 2016 and September 2019, 22 patients (17 women), 59 ± 12.7 years, underwent 81 BPA and were followed for as long as 33.1 months (mean 17.3 ± 7.5). Mean pulmonary artery pressure decreased by 17.4% (51.1 ± 12 vs. 42.2 ± 13 mmHg, p = .001), pulmonary vascular resistance by 23.9% (766.7 ± 351 vs. 583 ± 346 dynes/s/cm-5 , p = .001), cardiac index increased by 8% (2.3 ± 0.54 vs. 2.5 ± 0.54 L/min/m2 , p = .012), N-terminal pro-B-type natriuretic peptide decreased by 73.8% (1,685 ± 1,045 vs. 441.8 ± 276 pg/dl, p = .006), and 6-min walk distance improved by 135 m (316.7 ± 94 vs. 451.1 ± 113 m, p = .001). One patient (4.5%) developed lung reperfusion injury and four patients (18.2%) had minor bleeding (hemoptysis), after the procedure. There was no mortality associated with BPA. CONCLUSIONS: Our results confirm that BPA for inoperable CTEPH is a relatively safe procedure that improves clinical and hemodynamic parameters in the mid-term. This therapy should be considered as an alternative, mainly in places where access to PAH therapy or surgery is restricted.
Asunto(s)
Angioplastia de Balón , Hipertensión Pulmonar , Embolia Pulmonar , Angioplastia de Balón/efectos adversos , Enfermedad Crónica , Femenino , Estudios de Seguimiento , Humanos , Hipertensión Pulmonar/diagnóstico por imagen , Hipertensión Pulmonar/etiología , América Latina , Pulmón , Estudios Prospectivos , Arteria Pulmonar/diagnóstico por imagen , Arteria Pulmonar/cirugía , Embolia Pulmonar/diagnóstico , Embolia Pulmonar/diagnóstico por imagen , Resultado del TratamientoRESUMEN
OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.
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Humanos , Masculino , Femenino , Embolia Pulmonar/diagnóstico , Tromboembolia/etiología , Enfermedad Granulomatosa Crónica/patología , Hipertensión Pulmonar/diagnóstico , Embolia Pulmonar/complicaciones , Embolia Pulmonar/terapia , Angiografía/métodos , Tomografía Computarizada por Rayos X/métodos , Enfermedad Crónica , Estudios Retrospectivos , Resultado del Tratamiento , Imagen de Perfusión , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/terapia , Pulmón/irrigación sanguínea , Anticoagulantes/uso terapéuticoRESUMEN
La hipertensión pulmonar tromboembólica crónica (HPTC) es una enfermedad que remodela de manera obstructiva a las arterias pulmonares de gran y pequeño calibre como consecuencia de un tromboembolismo pulmonar (TEP) importante. Se cree que presenta una incidencia acumulada del 0,1 al 9,1% en los primeros 2 años después de un evento sintomático de TEP. El gran margen de error entre los autores que ofrecen estos números se debe probablemente al sesgo de referencia, a la escasez de síntomas tempranos y a la dificultad de diferenciar al TEP agudo de los síntomas de HPTC preexistente. Aunque su prevalencia exacta y la incidencia anual son desconocidas, algunos datos sugieren que esta condición puede ocurrir en aproximadamente 5 individuos por millón de habitantes por año. En el diagnóstico diferencial de la misma, se debe tener en cuenta distintas condiciones muy diferentes a los fenómenos trombóticos. Estas afecciones incluyen el sarcoma de arteria pulmonar, embolia de células tumorales, parásitos (como el quiste hidatídico), embolia de cuerpo extraño y estenosis congénita o adquirida de la arteria pulmonar. Todo esto hace que el diagnóstico se torne dificultoso y afecte consecutivamente al pronóstico de esta enfermedad. Esta revisión pretende interpretar la metodología diagnóstica usada actualmente en la HPTC y definir las diferentes situaciones pronósticas de la misma.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease that obstructively remodels the pulmonary arteries of large and small caliber as a consequence of an important pulmonary embolism (PE). It is believed to have an accumulated incidence of 0.1 to 9.1% in the first 2 years after a symptomatic event of PE. The large margin of error among the authors offering these numbers is probably due to the reference bias, the shortage of early symptoms, and the difficulty of differentiating acute PE from the symptoms of pre-existing CTEPH. Although its exact prevalence and annual incidence are unknown, some data suggest that this condition can occur in approximately 5 individuals per million inhabitants per year. In the differential diagnosis of the same, must take into account different conditions very different from the thrombotic phenomena. These conditions include pulmonary artery sarcoma, tumor cell embolism, parasites (such as hydatid cyst), foreign body embolism, and congenital or acquired pulmonary artery stenosis. All this makes the diagnosis difficult and affects consecutively the prognosis of this disease. This review aims to interpret the diagnostic methodology currently used in the CTEPH and define the different prognostic situations of it.
A hipertensão pulmonar tromboembólica crônica (HPTC) é uma doença que remodela de forma obstrutiva as artérias pulmonares de grande e pequeno calibre, como resultado de uma embolia pulmonar (EP) significativa. Acredita-se que tenha uma incidência acumulada de 0,1 a 9,1% nos primeiros 2 anos após um evento sintomático de EP. A grande margem de erro entre os autores que oferecem esses números provavelmente se deve ao viés de referência, à falta de sintomas precoces e à dificuldade de diferenciar a EP aguda dos sintomas de HPTC pré-existente. Embora sua prevalência exata e incidência anual sejam desconhecidas, alguns dados sugerem que essa condição pode ocorrer em aproximadamente 5 indivíduos por milhão de habitantes por ano. No diagnóstico diferencial do mesma, deve levar em conta condições distintas muito diferentes dos fenômenos trombóticos. Essas condições incluem sarcoma de artéria pulmonar, embolia de células tumorais, parasitas (como cisto hidático), embolia de corpo estranho e estenose da artéria pulmonar congênita ou adquirida. Tudo isso dificulta o diagnóstico e afeta consecutivamente o prognóstico dessa doença. Esta revisão tem como objetivo interpretar a metodologia diagnóstica atualmente utilizada na HPTC e definir as diferentes situações prognósticas da mesma.
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Hipertensión Pulmonar , Pirazoles , Hemodinámica , Humanos , Pirimidinas , Citrato de SildenafilRESUMEN
La hipertensión pulmonar tromboembólica crónica presenta un tratamiento curativo, siendo la endarterectomía pulmonar (EP) la opción terapéutica establecida. Sin embargo, la angioplastia con balón a arterias pulmonares puede ser considerada una alternativa viable en pacientes inoperables o en aquellos que persisten con hipertensión pulmonar tras una EP. Se reporta a continuación el primer caso de angioplastia con balón a arterias pulmonares realizada en un centro de Argentina.
Chronic thromboembolic pulmonary hypertension presents a curative treatment, with pulmonary endarterectomy being the established therapeutic option. However, balloon angioplasty to pulmonary arteries may be considered an alternative therapeutic option for patients with inoperable or residual disease after surgery. Herewith, we report the first case of chronic thromboembolic pulmonary hypertension treated with balloon pulmonary angioplasty in Argentina.
A hipertensão pulmonar tromboembólica crônica tem um tratamento curativo, a endarterectomia pulmonar (EP), a opção terapêutica estabelecida. No entanto, angioplastia com balão de artérias pulmonares pode ser considerado uma alternativa viável em pacientes inoperáveis ou aqueles que persistem com a hipertensão pulmonar após EP. Relatamos o primeiro caso de angioplastia pulmonar com balão no centro da Argentina.
Asunto(s)
Humanos , Angioplastia de Balón , Endarterectomía , Hipertensión PulmonarRESUMEN
Resumen: La hipertensión pulmonar tromboembólica crónica (HPTEC) es un subtipo de hipertensión pulmonar (HP) caracterizada por la obstrucción mecánica de las arterias pulmonares causada por una tromboembolia pulmonar. Sin tratamiento es una enfermedad progresiva y devastadora, y es el único subgrupo de HP potencialmente curable mediante la endarterectomía pulmonar. La magnitud, así como la recurrencia de la embolia pulmonar, son determinantes y contribuyen al desarrollo de la HPTEC aun cuando está asociada solo a algunos factores trombofílicos. Una hipótesis es que la enfermedad es consecuencia de la resolución incompleta y la organización del trombo, favorecido por fenómenos de inflamación, inmunitarios y/o genéticos que promueven el desarrollo de estenosis de tipo fibroso que culminan con el remodelado vascular oclusivo de vasos proximales y distales. Los mecanismos involucrados en la fallida resolución del trombo aún no están esclarecidos. Los pacientes con HPTEC con frecuencia exhiben HP severa que no puede ser explicada por el grado de obstrucción vascular demostrada en estudios por imagen. En tales casos la arteriopatía pulmonar y las lesiones trombóticas obstructivas, distales al nivel subsegmentario, pueden contribuir al incremento fuera de proporción de las resistencias vasculares pulmonares. Los procesos que llevan al desarrollo de la arteriopatía pulmonar y los cambios microvasculares que ocurren en la HPTEC explican el comportamiento progresivo de la HP y el deterioro clínico gradual con pobre pronóstico para los pacientes, así como también la falta de correlación entre la magnitud de la obstrucción vascular y la medición de parámetros hemodinámicos, aun en ausencia de tromboembolismo venoso recurrente. Esta revisión resume los aspectos más relevantes y actuales de la patobiología y fisiopatología de la HPTEC.
Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subtype of pulmonary hypertension characterized by the presence of mechanical obstruction of the major pulmonary vessels caused by venous thromboembolism. CTEPH is a progressive and devastating disease if not treated, and is the only subset of PH potentially curable by a surgical procedure known as pulmonary endarterectomy. The clot burden and pulmonary embolism recurrence may contribute to the development of CTEPH however only few thrombophilic factors have been found to be associated. A current hypothesis is that CTEPH results from the incomplete resolution and organization of thrombus modified by inflammatory, immunologic and genetic mechanisms, leading to the development of fibrotic stenosis and adaptive vascular remodeling of resistance vessels. The causes of thrombus non-resolution have yet to be fully clarified. CTEPH patients often display severe PH that cannot be fully explained by the degree of pulmonary vascular obstruction apparent on imaging studies. In such cases, the small vessel disease and distal obstructive thrombotic lesions beyond the sub-segmental level may contribute for out of proportion elevated PVR. The processes implicated in the development of arteriopathy and microvascular changes might explain the progressive nature of PH and gradual clinical deterioration with poor prognosis, as well as lack of correlation between measurable hemodynamic parameters and vascular obstruction even in the absence of recurrent venous thromboembolism. This review summarizes the most relevant up-to-date aspects on pathobiology and pathophysiology of CTEPH.
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Humanos , Embolia Pulmonar/complicaciones , Embolia Pulmonar/fisiopatología , Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/fisiopatología , Enfermedad Aguda , Enfermedad CrónicaRESUMEN
Abstract: Objective: REMEHIP is a prospective, multicentre registry on pulmonary hypertension. The main objective will be to identify the clinical profile, medical care, therapeutic trends and outcomes in adult and pediatric Mexican patients with well-characterized pulmonary hypertension. Methods: REMEHIP a multicenter registry began in 2015 with a planned recruitment time of 12 months and a 4-year follow-up. The study population will comprise a longitudinal cohort study, collecting data on patients with prevalent and incident pulmonary hypertension. Will be included patients of age >2 years and diagnosis of pulmonary hypertension by right heart catheterization within Group 1 and Group 4 of the World Health Organization classification. The structure, data collection and data analysis will be based on quality current recommendations for registries. The protocol has been approved by institutional ethics committees in all participant centers. All patients will sign an informed consent form. Currently in Mexico, there is a need of observational registries that include patients with treatment in the everyday clinical practice so the data could be validated and additional information could be obtained versus the one from the clinical trials. In this way, REMEHIP emerges as a link among randomized clinical trials developed by experts and previous Mexican experience.
Resumen: Objetivo: REMEHIP es un registro prospectivo, multicéntrico en hipertensión pulmonar. El objetivo principal será identificar el perfil clínico, atención médica, tendencias terapéuticas y evolución en pacientes mexicanos adultos y pediátricos con hipertensión pulmonar bien caracterizada. Métodos: El REMEHIP comenzó en el año de 2015 y se planea un reclutamiento de 12 meses con un seguimiento de 4 años. La población en estudio será una cohorte longitudinal y se obtendrán datos de pacientes prevalentes e incidentes con hipertensión pulmonar. Se incluirán pacientes con edad > 2 años con diagnóstico de hipertensión pulmonar demostrado por cateterismo cardiaco derecho del Grupo 1 y Grupo 4 de la clasificación de la Organización Mundial de la Salud. La estructura, colección de datos y el análisis se establecerá a través de las recomendaciones actuales de calidad para los registros. El protocolo ha sido aprobado por los comités de ética de todos los centros participantes. Todos los pacientes firmarán un consentimiento informado. Actualmente en México existe una necesidad de registros observacionales que incluyan a pacientes con tratamiento en la práctica clínica cotidiana, de tal forma que los datos obtenidos podrían validarse y el resto de la información podría compararse con la derivada de los estudios clínicos. De esta forma REMEHIP surge como un vínculo entre los estudios clínicos aleatorizados conducidos por expertos y la experiencia mexicana previa.
Asunto(s)
Humanos , Sistema de Registros , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/terapia , Proyectos de Investigación , Estudios Prospectivos , MéxicoRESUMEN
OBJECTIVE: REMEHIP is a prospective, multicentre registry on pulmonary hypertension. The main objective will be to identify the clinical profile, medical care, therapeutic trends and outcomes in adult and pediatric Mexican patients with well-characterized pulmonary hypertension. METHODS: REMEHIP a multicenter registry began in 2015 with a planned recruitment time of 12 months and a 4-year follow-up. The study population will comprise a longitudinal cohort study, collecting data on patients with prevalent and incident pulmonary hypertension. Will be included patients of age >2 years and diagnosis of pulmonary hypertension by right heart catheterization within Group 1 and Group 4 of the World Health Organization classification. The structure, data collection and data analysis will be based on quality current recommendations for registries. The protocol has been approved by institutional ethics committees in all participant centers. All patients will sign an informed consent form. Currently in Mexico, there is a need of observational registries that include patients with treatment in the everyday clinical practice so the data could be validated and additional information could be obtained versus the one from the clinical trials. In this way, REMEHIP emerges as a link among randomized clinical trials developed by experts and previous Mexican experience.
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Hipertensión Pulmonar , Sistema de Registros , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/terapia , México , Estudios Prospectivos , Proyectos de InvestigaciónRESUMEN
Chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subtype of pulmonary hypertension characterized by the presence of mechanical obstruction of the major pulmonary vessels caused by venous thromboembolism. CTEPH is a progressive and devastating disease if not treated, and is the only subset of PH potentially curable by a surgical procedure known as pulmonary endarterectomy. The clot burden and pulmonary embolism recurrence may contribute to the development of CTEPH however only few thrombophilic factors have been found to be associated. A current hypothesis is that CTEPH results from the incomplete resolution and organization of thrombus modified by inflammatory, immunologic and genetic mechanisms, leading to the development of fibrotic stenosis and adaptive vascular remodeling of resistance vessels. The causes of thrombus non-resolution have yet to be fully clarified. CTEPH patients often display severe PH that cannot be fully explained by the degree of pulmonary vascular obstruction apparent on imaging studies. In such cases, the small vessel disease and distal obstructive thrombotic lesions beyond the sub-segmental level may contribute for out of proportion elevated PVR. The processes implicated in the development of arteriopathy and micro-vascular changes might explain the progressive nature of PH and gradual clinical deterioration with poor prognosis, as well as lack of correlation between measurable hemodynamic parameters and vascular obstruction even in the absence of recurrent venous thromboembolism. This review summarizes the most relevant up-to-date aspects on pathobiology and pathophysiology of CTEPH.
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Hipertensión Pulmonar/complicaciones , Hipertensión Pulmonar/fisiopatología , Embolia Pulmonar/complicaciones , Embolia Pulmonar/fisiopatología , Enfermedad Aguda , Enfermedad Crónica , HumanosRESUMEN
A partir del 4to simposio mundial de hipertensión pulmonar (HP) se asignó un grupo propio a la hipertensión pulmonar tromboembólica crónica (HPTEC) en la clasificación de HP, el grupo 4. La HP es un estado hemodinámico definido por una presión media de arteria pulmonar (PAPm) igual o mayor a 25 mm Hg en reposo medida por cateterismo cardiaco derecho (CCD). La HPTEC se desarrolla como consecuencia de una obstrucción en las arterias pulmonares debida a la resolución incompleta de una tromboembolia pulmonar que conduce al remodelado de los vasos pulmonares luego de tres meses de anticoagulación efectiva. En el 5to simposio mundial de HP a la HPTEC se asignó un grupo independiente de trabajo. El presente trabajo busca actualizar los datos epidemiológicos, fisiopatología, características clínicas y tratamiento de este grupo de HP.
Since the 4th world symposium on pulmonary hypertension (PH), chronic thromboembolic pulmonary hypertension (CTEPH) was assigned an own group in the classification of HP, the group 4. PH is a hemodynamic state defined by a mean pulmonary artery pressure (mPAP) equal to or greater than 25 mmHg at rest, measured by right heart catheterization (CCD). CTEPH is developped by the presence of an obstruction in the pulmonary arteries due to incomplete resolution of pulmonary thromboembolic event leading to pulmonary vascular remodeling after three months of effective anticoagulation. Since the 5th World Symposium HP, CTEPH was assigned an independent working group. The objective of this paper is to update the epidemiological data, pathophysiology, clinical features and treatment of this group of pulmonary hypertension.