Chronic Thromboembolic Pulmonary Hypertension and ChronicThromboembolic Pulmonary Disease / 罕见病研究

Chronic thromboembolic pulmonary hypertension (CTEPH) and chronic thromboembolic pulmonary disease (CTEPD) are diseases that occur after acute pulmonary embolism resulting from residual and organized intra-pulmonary thrombus, with or without pulmonary hypertension(PH). The prognosis of these diseases has been dramatically improved with the continuous progress of treatment and the updated concept of the comprehensive treatment strategy. However, there is a lack of awareness of these diseases among medical practitioners, and a lack of epidemiological data in China, with unknown pathogenesis and non-specific clinical manifestations. Therefore, it is easy to misdiagnosis, underdiagnosis and delay diagnosis in clinical practice. To enhance the understanding of medical personnel, this paper summarizes the definition, epidemiology, pathology, pathogenesis, diagnosis and treatment of CTEPH and CTEPD.

The Use of Ketamine as an Induction Agent for Anesthesia in Pulmonary Thromboendarterectomy Surgery: A Case Series

Pulmonary thromboendarterectomy (PTE) surgery is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH). The induction of anesthesia in patients with severe pulmonary hypertension (PHT) can be challenging, with a risk of cardiovascular collapse. The administration of ketamine in patients with PHT is controversial, with some recommendations contraindicating its use. However, ketamine has been used safely in children with severe PHT. We present a retrospective case series of adult patients with severe PHT presenting for PTE surgery, using intravenous ketamine as a co?induction anesthetic agent.

Clinical study on the changes of left ventricular function and recovery after pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension / 中华超声影像学杂志

Objective:To assess the configuration and systolic function of the left ventricle in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by routine ultrasound, two-dimensional speckle tracking imaging and three-dimensional echocardiography, and to observe the recovery after pulmonary endarterectomy (PEA).Methods:The patients who were diagnosed with CTEPH, underwent PEA and had no left heart disease were enrolled as the CTEPH group ( n=30) in the China-Japan Friendship Hospital from November 2016 and June 2021. The right heart catheterization data before and after surgery were recorded. In the meantime, gender- and age-matched healthy individuals who sought for physical examination during the same period were included as the control group ( n=23). Echocardiography findings before and after PEA were comparatively analyzed and compared between the two groups, including left ventricular end-diastolic diameter (LVEDd), right and left ventricular cross-section ratio (RVd/LVd), left ventricular global longitudinal strain (LVGLS), left ventricular end-diastolic/systolic volume index (LVEDVi/LVESVi), left ventricular ejection fraction (LVEF) and left ventricular stroke volume (LVSV). Associations between the mean pulmonary arterial pressure (mPAP)/pulmonary vascular resistance (PVR) and left ventricular function were discussed. Results:When compared with the control group, the LVEDd, LVEDVi, LVESVi, LVSV, LVGLS and the mitral early to late diastolic flow velocity ratio (E/A) in the CTEPH group were lower (all P<0.05). There were no significant differences between the two groups regarding LVEF, cardiac output (CO), and cardiac index (CI) (all P>0.05). There were no statistical differences of the left ventricular volume and LVSV between PEA group and the control group (both P>0.05), while the LVGLS and E/A remained lower (both P<0.05). Correlation analysis showed negative associations between mPAP and LVSV as well as E/A ( r=-0.490, -0.455; both P<0.05). Conclusions:There are changes in left ventricular configuration with abnormal filling pattern and potential systolic dysfunction in CTEPH patients. The PEA surgery could lead to recovery of the left ventricular configuration and volume, but the filling pattern and LVGLS at follow-up can not recover completely.

El impacto de CoViD-19 en la hipertensión arterial pulmonar

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.

Chronic thromboembolic pulmonary hypertension: a review

Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension resulting from obstruction of the pulmonary artery by fibrotic thromboembolic material, usually initiated by recurrent or incomplete resolution of pulmonary embolism. This distinct form of pulmonary hypertension is classified under Group 4 of the World Health Organization classification. Further investigations are usually initiated, with transthoracic echocardiography followed by right heart catheterisation and pulmonary angiography as the gold standard. Definitive treatment is usually in the form of surgical pulmonary endarterectomy. Inoperable CTEPH is medically treated with pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators and prostacyclin. Recent developments have made balloon pulmonary angioplasty a viable option as well.

Anesthetic management of pulmonary endarterectomy in a patient with suprasystemic pulmonary pressure: a case report

Pulmonary endarterectomy (PEA) is a potentially curative procedure for chronic thromboembolic pulmonary hypertension. A 34-year Indian male presented with supra-systemic pulmonary pressure was taken for PEA after achievable optimization. A successful PEA was performed with the help of total cardiac arrest. Patient developed reperfusion pulmonary edema post operatively, which was managed medically. The problems encountered by anesthesiologists in this case were preoperative pulmonary hypertension with chronic hypoxia and postoperative management of reperfusion pulmonary edema.

Lung cavities in chronic thromboembolic pulmonary hypertension

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.

Perioperative anesthesia management for pulmonary endarterectomy: Adopting an established European Protocol for the Asian Population

Background: Anesthesia for pulmonary endarterectomy (PEA) has always been one of the challenges of anesthesia. As one of the leading cardiothoracic institutions in Southeast Asia, our hospital has vast interest in this subject. A local multidisciplinary team was deployed to an expert center in the United Kingdom (UK), and the experience was then integrated to the care of our patients. We present a case series of ten patients undergoing anesthesia for PEA, a first for our institution, and discuss techniques as well as potential complications. Methods: Patients with chronic thromboembolic pulmonary hypertension were reviewed by a multidisciplinary team, and those who were suitable for surgical intervention subsequently underwent PEA. A total of ten patients were identified and operated on. The perioperative management and conduction of anesthesia for all patients followed a protocol adapted from the expert center in the UK, with revisions to cater to our Asian population. Results: In the ten patients operated on, eight of them were successfully extubated on the first postoperative day. Apart from one incident of prolonged ventilator usage due to reperfusion lung injury and pneumonia, there were no major respiratory or hemodynamic complications. Certainly, six of the ten patients developed subdural hemorrhage after the commencement of enoxaparin, although none of them sustained any permanent neurological deficits. Conclusion: We have demonstrated that with careful planning and a well-outlined protocol, anesthesia for PEA in an Asian population can be achieved with favorable outcomes. Further fine-tuning of the protocol is still required based on local expertise.

Hipertensión pulmonar tromboembólica crónica: Del diagnóstico al pronóstico

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.

The clinical characteristics of antiphospholipid syndrome associated with chronic thromboembolic pulmonary hypertension / 中华内科杂志

Objective To analyze the clinical characteristics of antiphospholipid syndrome (APS) patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods A total of 22 APS patients with CTEPH were enrolled in our study,who were admitted in Peking Union Medical College Hospital from January 2012 to August 2018.Diagnoses were confirmed by computed tomographic pulmonary angiography (CTPA),or pulmonary angiography.Demographic characteristics,clinical manifestations,laboratory tests,therapy,World Health Organization (WHO) functional class were retrospectively collected.Results There were 15 females and 7 males with a median age of 29-year-old.Chest pain (6 cases),dyspnea on exertion (22 cases),cough (6 cases) and hemoptysis (9 cases) were the most common clinical manifestations.Lupus anticoagulant (LA),anticardiolipin (ACL) antibodies and anti-beta 2 glycoprotein Ⅰ (anti-β2 GP Ⅰ) antibodies were all positive in 12 patients,two of three antibodies positive in 5 patients,only one positive in 5 patients.The WHO functional classes were Ⅱ-Ⅳ before treatment.Anticoagulants were administrated in all patients.After multidisciplinary evaluation,9 patients underwent pulmonary thromboendarterectomy (PTE),who all had a good outcome.Symptoms in eleven over thirteen patients with only anticoagulants improved.Three patients developed cardiac deterioration while other 3 patients died of right heart failure during follow-up.Conclusion Pulmonary embolism is one of the most common thrombotic events in APS patients.It is important to recognize symptoms and signs related to pulmonary embolism and start anticoagulation as soon as possible.Standard anticoagulation improves symptoms but can't reverse the process of pulmonary hypertension.Some patients may benefit from PTE after anticoagulation and multidisciplinary evaluation.

Pulmonary balloon angioplasty for patients with chronic thromboembolic pulmonary hypertension accompanying with progressed pulmonary hypertension after pulmonary endarterectomy / 中国胸心血管外科临床杂志

@#Objective    To describe the effect of sequential pulmonary balloon angioplasty for patients with chronic thromboembolic pulmonary hypertension, who was accompanied with progressed pulmonary hypertension after pulmonary endarterectomy surgeries. Methods    From 2014 to December 2017, 7 patients were treated with a combination therapy of pulmonary endarterectomy and sequential pulmonary balloon angioplasty. There were 1 male and 6 females at age of 58 (43–59) years. A follow-up period of more than 1 year was accomplished. The result of right sided heart catheterization and ultrasonic cardiogram between and after the pulmonary endarterectomy or balloon angioplasty was collected. Results    Seven patients were treated with a combination of pulmonary endarterectomy and sequential pulmonary balloon angioplasty, which included 1 patient of single pulmonary balloon angioplasty and 6 patients of multiple pulmonary balloon angioplasties. The balloon dilation times was 2 (2–6), and the number of segments during each single balloon dilatation was 3–5, compared with the first clinical results before  the first balloon dilation, systolic pulmonary artery pressure [53 (47–75) mm Hg vs. 45 (40–54) mm Hg, P=0.042), mean pulmonaryartery pressure [38 (29–47) mm Hg vs. 29 (25–39) mm Hg, P=0.043], N terminal-B type natriuretic peptide [1 872 (1 598–2 898) pg/ml vs. 164 (72–334) pg/ml, P=0.018] improved significantly after the last balloon angioplasty. Heart function classification (NYHA) of all the 7 patients were recovered to Ⅰ-Ⅱclasses (P<0.05). Conclusion    Sequential pulmonary balloon angioplasty after pulmonary endarterectomy can further reduce the patient's right heart after load, improve the heart function for patients with progressed pulmonary hypertension after pulmonary endarterectomy surgeries.

Interpretation of expert ideas in the Sixth World Symposium of Pulmonary Hypertension（Ⅳ）——update on the diagnosis and treatment strategies of chronic thromboembolic pulmonary hypertension / 中国实用内科杂志

Chronic thromboembolic pulmonary hypertension(CTEPH)is a complication of pulmonary embolism. It is emphasized that the need for a multidisciplinary team involving diagnosis and treatment. The understanding and management of CTEPH have been updated on the 6 th World Symposium on Pulmonary Hypertension(WSPH)in 2018. Key diagnostic steps in early identification and accurate diagnosis are emphasised. An updated treatment algorithm is proposed. Combined with clinical practice, the article will give an interpretation of diagnosis and treatment of CTEPH based on this consensus opinions.

Impact of Balloon Pulmonary Angioplasty on Hemodynamics and Clinical Outcomes in Patients with Chronic Thromboembolic Pulmonary Hypertension: the Initial Korean Experience

BACKGROUND: The treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) is pulmonary endarterectomy (PEA). However, not all patients are eligible for PEA, and some patients experience recurrence of pulmonary hypertension even after PEA. METHODS: Patients who underwent balloon pulmonary angioplasty (BPA) between December 2015 and April 2017 were enrolled from the Samsung Medical Center CTEPH registry. Enrolled patients underwent right heart catheterization, echocardiography, and 6-minute walk distance (6MWD) at baseline, 4 and 24 weeks after their first BPA session. We compared clinical and hemodynamic parameters at the baseline and last BPA session. RESULTS: Fifty-two BPA sessions were performed in 15 patients, six of whom had a history of PEA. BPA resulted in improvements in World Health Organization (WHO) functional class (2.9 ± 0.8 to 1.7 ± 0.6, P = 0.002), 6MWD (387.0 ± 86.4 to 453.4 ± 64.8 m, P = 0.01), tricuspid annular plane systolic excursion (14.1 ± 3.6 to 15.6 ± 4.3 mm, P = 0.03) and hemodynamics, including a decline in mean pulmonary artery pressure (41.1 ± 13.1 to 32.1 ± 9.5 mmHg, P < 0.001) and in pulmonary vascular resistance (607.4 ± 452.3 to 406.7 ± 265.4 dyne.sec.cm−5, P = 0.01) but not in cardiac index (2.94 ± 0.79 to 2.96 ± 0.93 L/min/m2, P = 0.92). Six cases of complications were recorded, including two cases of reperfusion injury. CONCLUSION: BPA might be a safe and effective treatment strategy for both inoperable CTEPH patients and patients with residual pulmonary hypertension after PEA.

Acute Pulmonary Thromboembolism Associated with Chronic Thromboembolic Pulmonary Hypertension Treated by Emergency Embolectomy and Delayed Pulmonary Endarterectomy / 日本心臓血管外科学会雑誌

A 46-year-old man who had been hospitalized for treatment of pneumonia was referred to our hospital with a diagnosis of acute pulmonary thromboembolism. Multi-detector row computed tomography revealed massive thrombi in the bilateral main pulmonary arteries. Transthoracic echocardiography showed dilated right heart chambers and a large floating thrombus in the right atrium. Emergency thromboembolectomy was performed. Although fresh thrombi were removed from the right atrium and left main pulmonary artery, organized old thrombi were present in the bilateral pulmonary arteries, leading to a diagnosis of chronic thromboembolic pulmonary hypertension. Because no instruments were available for pulmonary endarterectomy, the surgery was terminated with percutaneous cardiopulmonary support. Five days postoperatively, pulmonary endarterectomy was performed under deep hypothermic circulatory arrest. The postoperative course was uneventful, and the patient returned to his preoperative lifestyle without the need for oxygen support.

Study on expression profile of long non-coding RNA in chronic thromboembolic pulmonary hypertension / 中华胸心血管外科杂志

Objective To investigate the aberrant expression of long non-coding RNA(lncRNA) in chronic thromboembolic pulmonary hypertension,and explore the lncRNA role in pathogenesis of CTEPH.Methods A total of 5 pulmonary artery endarterium tissue of CTEPH patients and 5 pulmonary artery endarterium tissue of healthy controls were collected.Using high-throughput gene microarray technology to detect two groups of lncRNA and mRNA expression spectrum,build lncRNA-mRNA express network,Pathway and GO analysis to explore the gene function.Results Differential expression of 185 lncRNA was observed in the CTEPH tissues compared with healthy control tissues.Further analysis identified 464 regulated enhancerlike lncRNA and overlapping,antisense or nearby mRNA pairs.Coexpression networks were subsequently constructed and investigated.The expression levels of the lncRNA,NR_036693,NR_027783,NR_033766 and NR_001284,were significantly altered.Gene ontology and pathway analysis demonstrated the potential role of lncRNA in the regulation of central process,including inflammatory response,response to endogenous stimulus and antigen processing and presentation.Conclusion Differentially expressed lncRNA may exert a partial role in CTEPH,the results of this study will help in the future about diagnosis and treatment of CTEPH.

Angioplastia con balón a arterias pulmonares en una paciente con hipertensión pulmonar tromboembólica crónica / Angioplastia com balão de artérias pulmonares em uma paciente com hipertensão pulmonar tromboembólica crônica / A case of balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension

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.

De la tromboembolia pulmonar aguda a la hipertensión pulmonar tromboembólica crónica: implicaciones en la patobiología y fisiopatología / From acute pulmonary embolism to chronic thromboembolic pulmonary hypertension: Pathobiology and pathophysiology

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.

Mexican registry of pulmonary hypertension REMEHIP / Registro Mexicano de Hipertensión Pulmonar REMEHIP

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.

Lung transplantation for chronic thromboembolic pulmonary hypertension: a report of 6 cases / 中华器官移植杂志

Objective To investigate the selection of recipients,operative technique,and perioperative management of lung transplantation for chronic thromboembolic pulmonary hypertension.Methods This article reviews the clinical data of 6 patients with chronic thromboembolic pulmonary hypertension receiving lung transplantation from January 2012 to June 2016.From January 2012 to June 2016,6 patients with chronic thromboembolic pulmonary hypertension underwent lung transplantation at Department of Thoracic Surgery,Affiliated Wuxi People's Hospital,Nanjing Medical University.There were 4 male and 2 female patients aged from 34 to 59 years.Chest enhancement of CT or CTPA in 6 patients prior to transplantation suggests a different degree of pulmonary embolism.The mean pulmonary artery pressure (mPAP) was＞30 mmHg,NYHA 11Ⅰ or Ⅳ.Four patients received bilateral sequence lung transplantation (BSLT) under extracorporeal membrane oxygenation (ECMO) support.Two patients received single lung transplantation (SLT).We analyzed the differences of oxygenation index,pulmonary systolic pressure,pulmonary artery diastolic pressure,mean pulmonary arterial pressure,and central venous pressure before and after treatment.Using paired t test to compare these indicators,P＜0.05 was considered statistically significant.Results The oxygenation index increased from (195 ± 85) to (440 ± 140) mmHg after treatment,the difference was statistically significant (P＜0.05).the The systolic pressure of pulmonary artery was decreased from (108 ± 28) mmHg to (56 ± 16) mmHg,and the difference was statistically significant (P＜0.05).the The diastolic pressure of pulmonary artery was decreased from (72 ± 18) mmHg to (25 ± 10) mmHg,and the difference was statistically significant (P＜0.05).the The mean pulmonary artery pressure was decreased from (84 ± 27) mmHg to (36 ± 10) mmHg,and the difference was statistically significant (P＜0.05).the The central venous pressure was decreased from (17.5 ± 4.5) mmHg to (8.5 ± 1.5) mmHg,and the difference was statistically significant (P＜0.05).Finally,5 patients were discharged from the hospital,and the pulmonary valve? functioned? well.1 patients died of shock and systemic failure 3 days after operation.Conclusion Lung transplantation is effective in the treatment of chronic thromboembolic pulmonary hypertension,and long-term survival requires further investigations.

Hipertensión pulmonar tromboembólica crónica / Chronic thromboembolic pulmonary hypertension

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.