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 and hemodynamic outcomes and mortality risk factors in patients undergoing pulmonary thromboendarterectomy / Resultados clínicos y hemodinámicos y factores de riesgo de mortalidad en pacientes sometidos a tromboendarterectomía pulmonar

Abstract Background: Pulmonary thromboendarterectomy is the current treatment of choice in patients with chronic thromboembolic pulmonary hypertension. The objective of the present study was to analyze the clinical and hemodynamic outcomes and the risk factors for mortality in a cardiovascular center in Colombia. Methods: Cohort study, conducted between 2001 and 2019. All operated patients were included in the study. Risk factors associated with mortality were established by means of a multivariate regression using the COX method and survival was established using the Kaplan-Meier method. p < 0.05 was considered statistically significant. Results: Seventy-three patients were operated. Median age was 51 years, 55% of females, 79% had functional Class III and IV. The mean pulmonary arterial pressure was 50 mmHg and 640 dyn.s.cm−5 for pulmonary vascular resistance (PVR). After the intervention, there was a decrease in mean pulmonary artery pressure (p ≤ 0.001) and in PVR (p = 0.357); 21% had evidence of residual pulmonary hypertension. Only 8% and 6% continued with functional Class III and IV at 6 and 12 months, respectively. There were 15 deaths (19.1%; 12% at 30 days). The factors associated with mortality were the diastolic diameter of the right ventricle measured postoperatively (hazard ratio [HR] 10.88 95% confidence interval [CI] 1.97-62, p = 0.007), time of invasive mechanical ventilation (HR 1.06 95% CI 1.02-1.09 p = 0.004), and the presence of complications during the surgical procedure (HR 5.62 95% CI 1.94-16.22 p = 0.001). Conclusions: Pulmonary thromboendarterectomy is associated with excellent clinical and hemodynamic outcomes. The mortality risk factors found are not those usually described in the literature.

Resumen Antecedentes: La tromboendarterectomía pulmonar es el tratamiento de elección actual en pacientes con hipertensión pulmonar tromboembólica crónica. El objetivo del presente estudio fue analizar los resultados clínicos y hemodinámicos y los factores de riesgo de mortalidad en un centro cardiovascular de Colombia. Métodos: Estudio de cohorte entre 2001 y 2019. Se incluyeron todos los pacientes operados. Los factores de riesgo asociados a la mortalidad se establecieron mediante una regresión multivariante mediante el método COX y la supervivencia se estableció mediante el método de Kaplan-Meier. Los valores de p < 0.05 se consideraron estadísticamente significativos. Resultados: se operaron 73 pacientes. La mediana de edad fue de 51 años, 55% mujeres, 79% tenían clase funcional III y IV. La presión arterial pulmonar media fue de 50 mmHg y 640 dyn.s.cm−5 para la resistencia vascular pulmonar. Después de la intervención, hubo una disminución en la presión arterial pulmonar media (p ≤ 0.001) y en la resistencia vascular pulmonar (p = 0.357). El 21% tenía evidencia de hipertensión pulmonar residual. Solo el 8% y el 6% continuaron con clase funcional III y IV a los 6 y 12 meses respectivamente. Hubo 15 muertes (19.1%; 12% a los 30 días). Los factores asociados con la mortalidad fueron el diámetro diastólico del ventrículo derecho medido en el postoperatorio (HR 10.88 IC 95% 1.97-62, p = 0.007), el tiempo de ventilación mecánica invasiva (HR 1.06 IC 95% 1.02-1.09 p = 0.004) y el presencia de complicaciones durante el procedimiento quirúrgico (HR 5.62 IC 95% 1.94-16.22 p = 0.001). Conclusiones: La tromboendartectomía pulmonar se asocia con excelentes resultados clínicos y hemodinámicos. Los factores de riesgo de mortalidad encontrados no son los habitualmente descritos en la literatura.

Cuidado de enfermería postoperación de Tromboendarterectomia Pulmonar Bilateral. Estudio de caso fundamentado en los principios de Henderson / Nursing care after Bilateral Pulmonary Thromboendarterectomy. Case study based on Henderson's principles / Cuidados de enfermagem após tromboendarterectomia pulmonar bilateral. Estudo de caso baseado nos princípios de Henderson

Resumen Introducción La tromboembolia pulmonar (TEP) es un padecimiento que se presenta con frecuencia de manera silenciosa, el 50 % o más de los casos no se diagnostica. Se define como la oclusión total o parcial de la circulación pulmonar, ocasionada por un coágulo sanguíneo proveniente de la circulación venosa sistémica, incluidas las cavidades derechas y que, de acuerdo a su magnitud, puede o no originar síntomas. Está considerada como una urgencia cardiovascular y constituye una de las principales causas de morbimortalidad en pacientes hospitalizados. Objetivo Desarrollar un Estudio de caso basado en el Proceso de Atención de Enfermería en la persona postoperada de Tromboendarterectomía Pulmonar Bilateral en Unidad de cuidados intensivos postquirúrgica de un Instituto Nacional de Tercer Nivel de la Ciudad de México. Método Estudio de caso basado en las etapas del proceso enfermero, se utilizó una Guía de valoración de las 14 necesidades de Virginia Henderson, jerarquización de Diagnósticos enfermeros, se ofreció una atención integral enfocada en cuidados especializados para cubrir las necesidades más afectadas en la persona. Conclusiones La aplicación del proceso de atención enfermero con base en la valoración de las 14 necesidades de Virginia Henderson, identificó que las necesidades más afectadas fueron; Oxigenación/Circulación y Evitar peligros, con la jerarquización se determinó que es imprescindible el conocimiento de enfermería en intervenciones especificas en el proceso postoperatorio de Tromboendarterectomía como: la detección del riesgo de alteraciones del ritmo cardiaco, valoración e intervenciones en insuficiencia respiratoria aguda, la propensión a eventos trombóticos secundarios a afección genética, para el logro de la recuperación de la salud de manera exitosa.

Abstract Introduction Since a pulmonary embolism (PE) can develop silently, more than 50 % of cases are not diagnosed until clear signs and symptoms are present. It is defined as the partial or total blockage of pulmonary circulation due to a blood clot which originated from the venous circulation elsewhere. Considered a cardiovascular urgency, it is a main cause of morbidity and mortality among hospitalized patients. Objective Based on the Nursing Process, to carry out a study case on a person who underwent surgery for Bilateral Pulmonary Thromboendarterectomy in the ICU of a Third Level of Care National Institute of Mexico City. Method This is a study case which followed the stages of the Nursing Process. An assessment guide of the 14 needs proposed by Virginia Henderson was utilized. Nursing diagnoses were ranked. An integral and specialized care addressing the patient's most important needs was offered. Conclusions The most important needs identified were: promoting oxygenation and circulation, and preventing risks. From the diagnoses analysis, it was determined that nursing knowledge is crucial in post thromboendarterectomy interventions, in terms of heart rate alteration risk detection, acute respiratory insufficiency assessment, and secondary thrombotic events prevention, all these in order to achieve a successful recovery.

Resumo Introdução A trombo embolia pulmonar (TEP) é uma doença que se apresenta com frequência de maneira silenciosa, o 50 % ou mais dos casos não se diagnostica. Define-se como a oclusão total ou parcial da circulação pulmonar, ocasionada por um coágulo sanguíneo proveniente da circulação venosa sistémica, incluídas as cavidades direitas e que, conforme sua magnitude, pode ou não originar sintomas. É considerada como uma urgência cardiovascular e constitui uma das principais causas de morbimortalidade em pacientes hospitalizados. Objetivo Desenvolver um Estudo de caso baseado no Processo de Atenção de Enfermagem na pessoa pós-perada de Tromboendarterectomia Pulmonar Bilateral na Unidade de cuidados intensivos post-cirúrgica de um Instituto Nacional de Terceiro Nível da Cidade do México. Método Estudo de Caso baseado nas etapas do processo enfermeiro, utilizou-se uma Guia de avaliação das 14 necessidades de Virginia Henderson, hierarquização de Diagnósticos enfermeiros, ofereceu-se uma atenção integral focalizada em cuidados especializados para cobrir as necessidades mais afetadas na pessoa. Conclusões A aplicação do processo de atenção enfermeiro com base na avaliação das 14 necessidades de Virginia Henderson, identificou que as necessidades mais afetadas foram; Oxigenação/Circulação e Evitar perigos, com a hierarquização determinou-se que é imprescindível o conhecimento de enfermagem em intervenções especificas no processo pós-operatório de Tromboendarterectomia como: a detecção do risco de alterações do ritmo cardíaco, avaliação e intervenções em insuficiência respiratória aguda, a propensão a eventos trombóticos secundários a afecção genética, para o sucesso da recuperação da saúde de maneira bem-sucedida.

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.

Association of deep vein thrombosis with the efficacy of pulmonary thromboendarterectomy / 中华胸心血管外科杂志

Objective To retrospectively analysis whether the existence of deep vein thrombosis(DVT) in chronic thromboembolic pulmonary hypertension (CTEPH)) has an influence on the efficacy of pulmonary thromboendarterectomy.Methods The data of 75 patients with CTEPH having undergone PTE in Beijing Chaoyang Hospital from Sep 2004 to Mar 2016 were retrospectively reviewed.We classified them into two different groups which were DVT (+)group (32) and DVT (-) group (43) according to the present of DVT.Clinical data,hemodynamics,blood gas analysis and so on of both groups were compared.Results There were 1 patient died after PEA surgery in DVT(+)group and 7 patients died after PEA surgery in DVT (-) group(3.1％ vs 16.3％,P =0.033).The incidence of severe pulmonary reperfusion injury and the neurological disorders postoperatively in DVT(-) group was significantly higher than that in DVT(+) group (46.5 ％ vs 21.9％,P =0.028;32.6％ vs 12.5％,P =0.044).Compared with DVT(+) group,the content of C-react protein (CRP) was higher in DVT (-) group [(0.9 ± 0.7) mg/dl vs (0.5 ± 0.4) mg/dl,P =0.005].Furthermore,DVT(-) group had a significantly greater proportion of Jamieson Ⅲ or V than DVT(+) group,while the cardiopulnonary bypass time [(289.8 ± 54.3) min vs (259.8 ±45.5) min,P=0.014],aorta cross clamp time[(137.3 ±31.6) min vs(119.5±29.3) min,P=0.015] and deep hypothermia circulatory arrest were significantly longer in DVT(-) group than in DVT(+) group.Postoperative hemodynamics and blood gas index were obvious improved in both groups,but the postoperative pulmonary artery systolic pressure (PAPS) [(59.6 ± 17.9) mmHg vs (48.5 ± 9.7) mmHg,P =0.001],the pulmonary vascular resistance (PVR) [(30.44 ± 22.97) kPa · S · L-1 vs (18.34 ±8.09) kPa · S · L 1,P =0.002] in DVT(-) group were significantly higher than those in DVT(+) group.In addition,the incidence of residual pulmonary hypertension in DVT(-) group was significantly higher than in DVT(+) group.Conclusion Pulmonary thromboendarterectomy for patients with CTEPH results in significant pulmonary hemodynamic improvement with favorable outcomes of heart and lung function.The recovery of the PAPS,PVR in patients with DVT are significantly better than those in patients without DVT,and the incidence of postoperative complication is significantly lower in patients with DVT.

Pulmonary Thromboembolectomy in Chronic Thromboembolic Pulmonary Hypertension: A Case Report and Review of Literature.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of severe pulmonary hypertention (PH) resulting in significant morbidity and mortality. Chronic thromboembolic PH occurs when a pulmonary embolism fails to undergo complete thrombolysis leading to vascular occlusion and pulmonary hypertension. Despite the fact that CTEPH is a potential consequence of pulmonary embolus, diagnosis requires a high degree of vigilance as many patients will not have a history of thromboembolic disease. The ventilation perfusion scan is used to evaluate for the possibility of CTEPH although right heart catheterization and pulmonary artery (PA) angiogram are needed to confirm the diagnosis. Pulmonary thromboendarterectomy is the first-line treatment for patients who are surgical candidates. This case report and review describes the pathophysiology, risk factors, diagnosis, and management of CTEPH. As it is a potentially curable cause of PH, its accurate diagnosis is vital. The gold standard and effective treatment for CTEPH is pulmonary endarterectomy (PEA). Pulmonary endarterectomy is an uncommon procedure with less than 50 years of experience worldwide. Research on the development of new surgical approaches is essential. In the present case, a new successful surgical technique for PEA was introduced. Conclusion: The surgical procedure used on the present patient was a unique technique. We do not claim that our technique is better than the original San Diego technique, but it is suggested as a modification that may improve patient survival. However, this procedure has its own limitations and cannot be used for clots that are located distally. Therefore, further experience should be obtained in order to overcome the limitations and improve the applicability of the technique.

Tromboendarterectomía pulmonar: tratamiento de elección para la hipertensión pulmonar tromboembólica crónica / Pulmonary thromboendarterectomy is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension

La tromboendarterectomía pulmonar es la terapéutica de elección para pacientes con hipertensión pulmonar tromboembólica crónica. Objetivos: Reportar la experiencia en tromboendarterectomía pulmonar a 18 años de seguimiento. Material y Métodos: Se analizaron retrospectivamente 41 pacientes entre Noviembre 1992 y Agosto 2010.Criterios de inclusión: presión arterial pulmonar media >30 mmHg y resistenciavascular pulmonar (RVP) >300 dinas/sg. cm-5. La angiografía pulmonar determinó la factibilidad quirúrgica. La supervivencia se calculó utilizando el método de Kaplan-Meier y para comparar 2 grupos log-rank test.Resultados: El 44% de los pacientes estaban en clase funcional IV. Los cambios hemodinámicos posteriores a la tromboendarterectomía fueron significativos p <0.001. La PAPm 53 ± 2 a 29 ± 2 mmHg, la RVP 857 ± 65 a 245 ± 25 dinas/sg. cm-5 y el IC 2,3 ± 0,1a 3 ± 0,1. La mortalidad hospitalaria fue 15%, en CF II-III 4% y en CF IV 33% (p=0.01). La mortalidad alejada fue 11%. El 93% de los pacientes mejoró su clase funcional. La supervivencia global a 1, 2, 5, 10 años fue del 85%, 82%, 75% y 71% respectivamente.La supervivencia por clase funcional mostró diferencias significativas (p=0.02). Conclusión: En nuestra experiencia la tromboendarterectomía pulmonar mostró beneficios en esta población de alto riesgo.

Pulmonary thromboendarterectomy is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension. Objective: To report our 18-years experience in pulmonary thromboendarterectomy. Material and Methods: Forty one patients were analyzed between November/1992 andAugust/2010. Inclusion criteria: mean pulmonary artery pressure (PAPm) >30 mmHg and pulmonary vascular resistance (PVR) >300 dinas/sec.cm-5. Pulmonary angiography determined the surgical feasibility. Survival was calculated using the Kaplan-Meiermethod and to compare the 2 groups log rank test. results: Pulmonary thromboendarterectomy is the treatment of choice for patients with chronicthromboembolic pulmonary hypertension. Objective: To report our 18-years experience in pulmonary thromboendarterectomy. Material and Methods: Forty one patients were analyzed between November/1992 andAugust/2010. Inclusion criteria: mean pulmonary artery pressure (PAPm) >30 mmHg and pulmonary vascular resistance (PVR) >300 dinas/sec.cm-5. Pulmonary angiography determined the surgical feasibility. Survival was calculated using the Kaplan-Meiermethod and to compare the 2 groups log rank test. Results: Forty four percent of the patients were classified in class IV. The hemodynamic changesafter pulmonary thromboendarterectomy were significant (p < 0.001). PAPm 53 ± 2 vs. 29 ± 2 mm Hg, PVR 857 ± 65 vs. 245 ± 25 dinas/sec. cm-5 and CI 2.3 ± 0.1 vs. 3 ± 0.1. In-hospital mortality was 15%; in classes II - III was 4%, and in class IV 33% (p = 0.01). Late mortality was 12%. Ninety three percent of the patients improved their functional class. Overall survival at 1,2, 5 and 10 years was 85%, 82%, 75% and 71% respectively. Survival according to functional class showed significant differences (p = 0.02).Conclusion: In our experience pulmonary thromboendarterectomy showed benefits even in thishigh risk population.

The clinical features of neurological disorders after pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension / 中华胸心血管外科杂志

Objective Investigate the clinical features of the neurological disorders in patients after pulmonary thromboendarterectomy (PTE) for chronic pulmonary thromboembolism , analyze the factorial. Methods A retrospective study was made of 26 patients who underwent PTE between 2002 and 2010 in Beijing Chao-Yang hospital. The symptom of neurological system disorders occurred in the survivors were been investigate. The preoperative conditions and the perioperative conditions of all survivors were investigated. Compared the differences between the groups with neurological system disorders and the groups without. Results There were 22 patients of 26 survived after the surgery. Cardiac function of the survivors improved, and the quality of life improved significantly. 5 cases of the 22 survivors had symptoms of neurological system disorders. 3 patients showed lethargy, delirium, memory disorders, brain CT found no abnormal, symptoms recovered within 2 weeks. The fourth patient showed ataxia, unsteady gait, dance-like movements symmetry, in addition to the above symptoms, brain CT no abnormal showed,brain MRI showed bilateral abnormal signal in the midbrain, basal ganglia, symptoms improved and discharged after 8 weeks, the symptoms completely disappeared 6 months, and the abnormal signals in brain in MRI disappeared. The fifth patient with similar symptoms to the fourth, brain CT no abnormal found, be discharged 10 weeks after the operation, recovery is poor, living part of themselves. The postoperative neurological disorders occurred in the patients of Jamieson surgery type Ⅲ type, Ⅳ type of larger proportion, (P = 0.024), longer circulatory arrest surgery (P = 0.034). Conclusion The neurological disorders postoperative PTE often showed diffuse symmetric cerebral cortex and basal ganglia nerve dysfunction, brain MRI showed abnormal signal corresponding region, the majority of symptoms disappeared in 2 - 8 weeks, abnormal signal in brain MRI could disappeared after 6 months. Neurological dysfunction occurs in patients with more difficult surgical procedure, longer circulatory arrest, suggesting that with the surgery cerebral ischemia and hypoxia related.

Postoperative management of pulmonary endarterectomy and outcome.

Pulmonary artery thromboendarterectomy (PTE) has been regarded as a promising, potentially curative surgical procedure. However, PTE is associated with specific postoperative complications, such as reperfusion pulmonary edema and right heart failure leading to a considerable mortality of 7-24%. Despite its limitations PTE is a better surgical alternative to lung transplantation which carries high morbidity and mortality. The aim of the study is to analyze the efficacy, safety, morbidity and survival associated in the postoperative period and quality of life after six months of PTE in Indian patients. Forty-one patients with surgically correctable chronic thromboembolic pulmonary hypertension underwent pulmonary endarterectomy. All patients were in New York Heart Association (NYHA) Class II, III or IV. Preoperative mean pulmonary artery pressure was 40.98 ± 9.29 mmHg and mean pulmonary vascular resistance was 418.39 ± 95.88 dynes/sec/cm -5 . All patients were followed up to six months and a telephonic survey was conducted using a standard questionnaire. They were assessed and classified as per NYHA grading. There was a significant reduction in the mean pulmonary artery pressure (from 40.98 ± 9.29 mmHg to 24.13 ± 7.36 mmHg, P < 0.001) and pulmonary vascular resistance (from 418.39 ± 95.88 dynes/sec/cm -5 to 142.45 ± 36.27 dynes/sec/cm -5 , P < 0.001) with a concomitant increase in the cardiac index (from 1.99 ± 0.20 L/min/m 2 to 3.28 ± 0.56 L/min/m 2 , P < 0.001) during the postoperative period. The mortality rate in our study was 12.19% (five patients). Ninety per cent of the patients reported a significant improvement in the quality of life and exercise tolerance after surgery compared to the preoperative state. Pulmonary endarterectomy is an effective and potentially curative surgical treatment for patients with severe chronic thromboembolic pulmonary hypertension. The current techniques of operation make the procedure relatively safe and long-term survival, NYHA functional status and exercise capacity improve significantly.

Clinical and haemodynamic evaluation of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy: Is schistosomiasis hypertension an important confounding factor?

INTRODUCTION: Chronic thromboembolic pulmonary hypertension is a disease affecting approximately 4,000 people per year in the United States. The incidence rate in Brazil, however, is unknown. The estimated survival for patients with chronic thromboembolic pulmonary hypertension without treatment is approximately three years. Pulmonary thromboendarterectomy for select patients is a potentially curative procedure when correctly applied. In Brazil, the clinical and hemodynamic profiles of chronic thromboembolic pulmonary hypertension patients have yet to be described. OBJECTIVES: To evaluate the clinical and hemodynamic characteristics of chronic thromboembolic pulmonary hypertension patients scheduled for pulmonary thromboendarterectomy in a referral center for chronic thromboembolic pulmonary hypertension treatment in Brazil. METHODS: From December 2006 to November 2009, patients were evaluated and scheduled for pulmonary thromboendarterectomy. The subjects were classified according to gender, age and functional class and were tested for thrombofilia and brain natriuretic peptide levels. RESULTS: Thirty-five consecutive chronic thromboembolic pulmonary hypertension patients were evaluated. Two patients tested positive for schistosomiasis, and 31 were enrolled in the study (19 female, 12 male). The majority of patients were categorized in functional classes III and IV. Hemodynamic data showed a mean pulmonary vascular resistance (PVR) of 970.8 ± 494.36 dynas·s·cm-5 and a low cardiac output of 3.378 ± 1.13 L/min. Linear regression revealed a direct relation between cardiac output and pulmonary vascular resistance. Paradoxical septal movement was strongly correlated with pulmonary vascular resistance and cardiac output (p=0.001). Brain natriuretic peptide serum levels were elevated in 19 of 27 patients. CONCLUSIONS: In a referral center for pulmonary hypertension in Brazil, chronic thromboembolic pulmonary hypertension patients evaluated for pulmonary thromboendarterectomy had a hemodynamically severe status and had elevated brain natriuretic peptide serum levels. There was a predominance of females in our cohort, and the prevalence of hematological disorders and schistosomiasis was low (less than 10 percent).

A case of chronic thromboembolic pulmonary hypertension (CTEPH) with antithrombin III deficiency / 대한내과학회지

Presented is a case study of a 39-year-old man with chronic thromboembolic pulmonary hypertension (CTEPH) and an underlying antithrombin III (AT III) deficiency. The subject presented with severe dyspnea (NYHA functional class III). A diagnostic workup led to a diagnosis of pulmonary thromboembolism and severe pulmonary hypertension with right ventricular failure. Genetic analysis revealed a novel nonsense mutation (c.243G>A) in SERPINC1. Pulmonary thromboendarterectomy was performed following the insertion of an inferior vena cava filter. After one year, the subject remained in NYHA functional class I and exhibited normal right ventricular function. This is the first report of a genetically confirmed AT III deficiency complicated by CTEPH in Korea.

Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a Patient with Antiphospholipid Syndrome and Systemic Lupus Erythematosus / 대한흉부외과학회지

Antiphospholipid syndrome (APS) is defined as venous and/or arterial thromboses, recurrent fetal losses, thrombocytopenia in combination with repeatedly positive tests for the lupus anticoagulant (LAC), and anticardiolipin antibodies (aCL). The pulmonary manifestations in APS are relatively rare. We report a rare case of antiphopholipid syndrome with systemic lupus erythematosus in a patient who presented with pulmonary hypertension secondary to a chronic pulmonary thromboembolism. A bilateral thromboendarterectomy was performed satisfactorily and the incision was extended to the left intrapleural pulmonary artery.

Effects of Pulmonary Thromboendarterectomy in Patients with Chronic Thromboembolic Pulmonary Hypertension / 결핵및호흡기질환

BACKGROUND: Bilateral pulmonary thromboendarterectomy(PTE) is recognized as the definitive treatment for chronic thromboembolic pulmonary hypertension (CTEPH). We investigated the symptomatic, hemodynamic and prognostic effects of PTE in comparison with medical treatment. METHODs: Twenty-four patients diagnosed with CTEPH from 1995 to 2003 at the Asan Medical Center were divided into two groups: patients treated with PTE(PTE group, n=12) and those not treated with PTE(Med group, n=12). The serial changes in dyspnea, the tricuspid regurgitation maximal velocity (TRVmax) and survival of the PTE and Med groups were compared retrospectively. RESULTS: In PTE group, during a follow-up period of 1 year, the New York Heart Association(NYHA) functional class significantly improved, while there was no significant improvement in the Med group. PTE significantly lowered the TRVmax from 4.23+/-0.54 m/sec to 3.22+/-0.70 m/sec over a follow up period of 2 years. (p=0.028) However, the TRVmax in the medically treated group did not show any significant improvement, changing from 3.98+/-0.68 m/sec to 4.27+/-0.95 m/sec during 1 year. The 5-year survival of the PTE group was 77.9% compared with 64.3% in the medically treated group. CONCLUSION: PTE provides substantial long-term improvement in dyspnea and the echocardiographic changes compared with medically treated patients.

Tromboendarterettomía pulmonar bilateral en un aso de hipertensión arterial puulmonar tromboembólica crónica

El embolismo pulmonar, problema de salud pública mundial, presenta una incidencia anual estimada en 100,000 casos en Francia, 65,000 en Inglaterra y Gales, 630,000 en Estados Unidos y 60,000 nuevos casos por año en Italia. La mortalidad en casos no tratados asciende a 30% y con tratamiento adecuado es del 2-8%. La hipertensión arterial pulmonar tromboembólica crónica acontece en el 0.1 al 0.5% de los pacientes que sobreviven al evento embólico y es el resultado de la obstrucción de las arterias pulmonares principales por la presencia de émbolos pulmonares. La obstrucción del lecho vascular pulmonar contribuye al desarrollo de la hipertensión arterial pulmonar, la cual determina diversos grados de disfunción ventricular derecha y modifica la sobrevida en estos pacientes. La hipertensión arterial pulmonar tromboembólica crónica es potencialmente corregible mediante cirugía: tromboendarterectomía pulmonar con circulación extracorpórea, hipotermia profunda a 16°C, y perfusión cerebral retrógrada. En el presente artículo se reporta un caso de tromboembolia pulmonar crónica con obstrucción de 70% del lecho vascular pulmonar y disfunción ventricular derecha, sometido a cirugía de tromboendarterectomía pulmonar bilateral, observándose cambios ostensibles hacia la mejoría en los parámetros hemodinámicos, respiratorios y estado funcional. Estas variables mejoran la calidad de vida y sobrevida.

Pulmonary embolism is a global public health problem with estimated annual incidence of 100,000 cases ion France, 65,000 in England and Wales, 630,000 in the United States and 60,000 new cases per year in Italy. Mortality of untreated cases can be up to 30% and with adequate treatment of 2-8%. Chronic thromboembolic pulmonary arterial hypertension occurs in 0.1 to 0.5% of patients who survive to the embolic event, and is the result of main pulmonary artery obstruction due to the presence of pulmonary embolia. Obstruction of the pulmonary vascular bed contributes to the development of pulmonary arterial hypertension, which determines diverse degrees of right ventricular dysfunction and modifies patient survival expectancy. Chronic thromboembolic pulmonary arterial hypertension is potentially corrigible by pulmonary thromboendaterectomy with extracorporeal circulation, deep hypothermia at 16°C and retrograde cerebral perfusion. The present article reports a case of chronic pulmonary thromboembolia with 70% obstruction of the pulmonary vascular bed and right ventricle dysfunction, subjected to bilateral pulmonary thromboendarterectomy. Visible improvements in hemodynamic, respiratory and functional state parameters were observed. These variables increase life quality and survival expectancy.

Thromboendarterectomy in a Patient with Unilateral Chronic Thromboembolic Pulmonary Hypertension / 대한마취과학회지

Chronic thromboembolic pulmonary hypertension (CTEPH) is considered to be an aberrant outcome of acute pulmonary thromboembolism, due to inadequate thrombus dissolution. However, the mechanism of thrombi dissolution failure remains unclear. With respect to inherited thrombophilia, the co-occurrence of natural anticoagulant deficiencies with CTEPH was found to be rare. Pulmonary thromboendarterectomy (PTE) is a potentially curative surgical procedure for CTEPH, but it is associated with considerable mortality due to postoperative complications, such as reperfusion pulmonary edema and right heart failure. The postoperative course after PTE poses a unique series of ventilatory care and hemodynamic management challenges. We present the case of a 42-year-old woman with unilateral CTEPH combined with thrombophilia (Protein S deficiency). Successful PTE was followed by independent lung ventilation with unilateral nitric oxide (NO) inhalation, which resulted in functional improvement without postoperative complications.

Two or Three Times of Short Deep Hypothermic Circulatory Arrest (DHCA)within 20 Minutes, Total Not Exceeding 60 Minutes, Does Not Impair Brain Function in Adults / 대한마취과학회지

BACKGROUND: Deep Hypothermic circulatory Arrest (DHCA) is widely used during heart surgery in neonates and infants and complicated surgeries in adults such as a pulmonary thromboendarterectomy (PTE). However safe limits of time and temperature during DHCA still remain controversial. Futhermore it's effects on neurologic outcome has been controversial. METHODS: On the postoperative 3 days, we examined the neurologic function of 12 patients who received a PTE. During the PTE, DHCA was done 2 or 3 times within 20 minutes. Between DHCAs the patients were perfused at a low flow rate with 18degreesC blood until their venous saturation returned to 90%. Neurologic evaluations included level of consciousness, Glasgow coma scale (GCS), and motor and sensory functions. RESULTS: Neurologic functions of all of the patients assessed on the 3 days postoperatively was grossly normal. All 12 patients were oriented to time, place, and person with normal gross motor and sensory functions. CONCLUSIONS: No significant neurologic complications were related to the technique of two or three times of short DHCA within 20 minutes, for a total not exceeding 60 minutes.

Pulmonary Thromboendarterectomy Under Total Circulatory Arrest / 대한흉부외과학회지

Besides lung transplantation, pulmonary thromboembolectomy is the only effective therapeutic option for chronic thromboembolic pulmonary hypertension. It is however associated with a considerably high hospital mortality between 6.6 to 23%. Proper patient selection is critical when considering a patient for pulmonary thromboembolectomy. And It cannot be overemphasised that the key to the success of the operation is complete endarterectomy of the entire pulmonary arterial tree. We report that pulmonary thromboendarterectomy under total circulatory arrest was an effective and safe method in the surgical correction of the chronic thromboembolic pulmonary hypertension and enabled complete removal of superimposed peripheral organized thrombi in a good operative field.

Massive Endobronchial Hemorrhage After Pulmonary Thromboendarterectomy in Chronic Pulmonary Embolism / 대한흉부외과학회지

Massive endobronchial hemorrhage after a successful pulmonary thromboendarterectomy is a rare but catastrophic complication. We experienced this complication in a patient with chronic pulmonary embolism, but it was managed successfully using extracorporeal membrane oxygenation and differential ventilation.

Thromboendarterectomy of Chronic Pulmonary Thromboembolism: A Case Report / 대한흉부외과학회지

Chronic pulmonary artery thromboembolism is a relatively rare phenomenon causing hypoxia and pulmonary hypertension that eventually leads to respiratory failure and right heart failure. Patients with acute pulmonary artery thromboembolism are generally treated with antithrombotics and thrombolytics. However, in cases with chronic pulmonary artery thromboembolism the fibrinized thrombus is so strongly adhered to the pulmonary artery wall that medical treatment becomes ineffective and surgical treatment must then be considered. We report a 47year old patient, with a history of repeated admission due to unresolved chronic obstructive pulmonary disease and chronic pulmonary artery thromboembolism at a local hospital, who underwent a successful endarterectomy of pulmonary artery thromboemboli using intermittent total circulatory arrest.