THROMBOENDARTERECTOMY FOR CHRONIC PULMONARY THROMBOEMBOLISM

Objective To evaluate the improving reliability and safety of thromboendarterectomy and perioperative management for chronic pulmonary thromboembolism. Methods The clinical data of 12 cases with chronic pulmonary thromboembolism, who underwent thromboendarterec- tomy assisted by low flow or circulation arrest with deep hypothermia, were reviewed retrospectively. Results Pulmonary artery pressure decreased 20 to 40 mmHg immediately after surgical procedures in 9 cases. The postoperative pulmonary edema at various degrees happened in 12 cases, among them, 1 died of severe lung infection and pulmonary re-embolism at 19 days postoperation. Computed tomography pulmonary angiography and angiography of 11 cases indicated that the original obstruction of pulmonary artery disappeared. During the follow-up period of 2 months to 5 years, the clinical symptoms and activity was improved. Conclusion Thromboendarterectomy is an effective treatment for chronic pulmonary thromboembolism. The outcome of the surgical procedure needs to be further investigated and followed up regularly according to an evaluative system, because it might be influenced by multiple factors.

Treatment and Clinical Management of Chronic Thromboembolic Pulmonary Hypertension:An Update of Literature Review

Chronic thromboembolic pulmonary hypertension(CTEPH)is a chronic,progressive,debilitating,and life-threa-tening complication of pulmonary embolism(PE).Recent technological advances have permitted various treat-ment options for the treatment of CTEPH,including surgery,angioplasty,and medical treatment,depending on the location and characteristics of lesions.Pulmonary endarterectomy(PEA)is the treatment of choice for CTEPH,as it offers excellent long-term outcomes and a high probability of recovery.Moreover,various medical and interventional therapies are currently being developed for patients with inoperable CTEPH.This review mainly summarizes the current treatment approaches of CTEPH,offering more options for specialist physicians to,thus,better manage chronic thromboembolic syndromes.

Pulmonary artery stent thrombosis and symptomatic pulmonary hypertension following COVID-19 infection in Alagille patient:A case report

BACKGROUND Alagille syndrome is a multisystem disease that results in various vascular anomalies,commonly involving the cardiac and pulmonary systems.To the best of our knowledge,there is no literature regarding the cardiovascular outcomes of these patients in association with coronavirus disease 2019(COVID-19).CASE SUMMARY A 34-year-old woman with a history of Alagille syndrome who underwent successful atrial septal defect with partial anomalous pulmonary veins and patent ductus arteriosus repair,as well as left pulmonary artery catheterization and stenting in childhood due to pulmonary stenosis.The patient was without any respiratory symptoms and was a dancer prior to contracting COVID-19.Several weeks after her COVID-19 infection,she developed left pulmonary artery stent thrombosis and subsequent symptomatic pulmonary hypertension.A treatment strategy of anticoagulation alongside pharmacological agents for pulmonary hypertension for 3 months followed by balloon pulmonary artery angioplasty to reopen the stenosis was unsuccessful.CONCLUSION In the era of COVID-19,patients with pulmonary vascular malformations and endovascular stents are at an increased risk for chronic thromboembolic disease.Patients may benefit from prophylactic antiplatelet or anticoagulation therapy.Stent thrombosis is a devastating phenomenon and should be treated urgently and aggressively with balloon pulmonary angioplasty,and/or a thrombolytic agent.

Balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension:State of the art

Chronic thromboembolic pulmonary hypertension(CTEPH)is a complex chronic disease in which pulmonary artery stenosis or obstruction caused by organized thrombus can lead to increased pulmonary artery pressure and pulmonary vascular resistance,ultimately triggering progressive right heart failure and death.Currently,its exact mechanism is not fully understood.Pulmonary endarterectomy(PEA)has immediate effects with low perioperative mortality and satisfactory prognosis in experienced expert centers for CTEPH patients with proximal lesions.Nevertheless,37%of patients are deemed unsuitable for PEA surgery due to comorbidities and other factors,and nearly half of the operated patients have residual or recurrent pulmonary hypertension.Riociguat is the only approved drug for CTEPH,although its effect is limited.Balloon pulmonary angioplasty(BPA)is a promising alternative treatment for patients with CTEPH.After more than 30 years of development and refinements,emerging evidence has confirmed its role in patients with inoperable CTEPH or residual/recurrent pulmonary hypertension,with acceptable complications and comparable longterm prognosis to PEA.This review summarizes the pathophysiology of CTEPH,BPA history and development,therapeutic principles,indications and contraindications,interventional procedures,imaging modalities,efficacy and prognosis,complications and management,bridging and hybrid therapies,ongoing clinical trials and future prospects.

Role of pulmonary perfusion magnetic resonance imaging for the diagnosis of pulmonary hypertension:A review

Among five types of pulmonary hypertension,chronic thromboembolic pulmonary hypertension(CTEPH)is the only curable form,but prompt and accurate diagnosis can be challenging.Computed tomography and nuclear medicine-based techniques are standard imaging modalities to non-invasively diagnose CTEPH,however these are limited by radiation exposure,subjective qualitative bias,and lack of cardiac functional assessment.This review aims to assess the methodology,diagnostic accuracy of pulmonary perfusion imaging in the current literature and discuss its advantages,limitations and future research scope.

Pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension： preliminary exploration in China

Background Pulmonary endarterectomy is safe and effective surgical treatment for chronic thromboembolic pulmonary hypertension. This study aimed to evaluate the efficacy of pulmonary endarterectomy in treatment of thromboembolic pulmonary hypertension. Methods A retrospective study of 15 patients who underwent pulmonary endarterectomy in Beijing Chaoyang Hospital was performed. Obvious pulmonary hypertension and hypoxemia were observed in all patients. Bilateral pulmonary endarterectomy was performed under cardiopulmonary bypass with profound hypothermic circulatory arrest. Results Two patients （2/15） died of residual postoperative pulmonary hypertension and bleeding complication. The other 13 cases had significant decrease in systolic pulmonary artery pressure （（92.8＋_27.4） mmHg vs. （49.3＋18.6） mmHg） and pulmonary vascular resistance （（938.7±464.1） dynes.s.cm5 vs. （316.8±153.3） dynes＇s.cm5）, great improvement in cardiac index （（2.31：LK）.69） L.min-l.m2 vs. （3.85±1.21） L.min-l.m2）, arterial oxygen saturation （0.67±O.11 vs. 0.96±0.22） and mixed venous 02 saturation （0.52±0.12 vs. 0.74±0.16） postoperatively compared to preoperative data. Mid-term follow-up showed that the cardiac function of all cases returned to NYHA class I or II, with great improvement in 6-minute walking distance （（138±36） m） and quality of life. Conclusions Bilateral pulmonary endarterectomy using cardiopulmonary bypass with the aid of deep hypothermia and circulatory arrest can effectively reduce pulmonary hypertension and provide good mid-term hemodynamic and symptomatic results with low surgical mortality rate and few complications.

Serum Bilirubin and 6-min Walk Distance as Prognostic Predictors for Inoperable Chronic Thromboembolic Pulmonary Hypertension： A Prospective Cohort Study

Background： Inoperable chronic thromboembolic pulmonary hypertension （CTEPH） is a severe clinical syndrome characterized by right cardiac failure and possibly subsequent liver dysfunction. However, whether serum markers of liver dysfunction can predict prognosis in inoperable CTEPH patients has not been determined. Our study aimed to evaluate the potential role of liver function markers （such as serum levels of transaminase, bilirubin, and gamma-glutamyl transpeptidase [GGT]） combined with 6-min walk test in the prediction of prognosis in patients with inoperable CTEPH. Methods： From June 2005 to May 2013, 77 consecutive patients with inoperable CTEPH without confounding co-morbidities were recruited for this prospective cohort study. Baseline clinical characteristics and 6-min walk distance （6MWD） results were collected. Serum biomarkers of liver function, including levels of aspartate aminotransferase, alanine aminotransferase, GGT, uric acid, and serum bilirubin, were also determined at enrollment. All-cause mortality was recorded during the follow-up period. Results： During the follow-up, 22 patients （29%） died. Cox regression analyses demonstrated that increased serum concentration of total bilirubin （hazard ratio [HR] = 7.755, P 〈 0.001）, elevated N-terminal of the prohormone brain natriuretic peptide （HR = 1.001, P = 0.001）, decreased 6MWD （HR = 0.990, P 〈 0.001）, increased central venous pressure （HR = 1.074, P = 0.040）, and higher pulmonary vascular resistance （HR = 1.001, P = 0.018） were associated with an increased risk of mortality. Serum concentrations of total bilirubin （HR = 4.755, P = 0.007） and 6MWD （HR = 0.994, P = 0.017） were independent prognostic predictors for CTEPH patients. Patients with hyperbilirubinemia （≥23.7 μmol/L） had markedly worse survival than those with normobilirubinemia. Conclusion： Elevated serum bilirubin and decreased 6MWD are potential predictors for poor prognosis in inoperable CTEPH.

A male with chronic thromboembolic pulmonary hypertension caused by isolated noncompaction of ventricular myocardium

Noncompaction of the ventricular myocardium （NVM） is a rare disease of endomyocardialmorphogenesis characterized by numerous, prominent trabeculations and deep intertrabecular recesses. It is commonly associated with congenital heart disease. Isolated noncompaction of the ventricular myocardium （INVM） is thought to be caused by arrest of normal embryogenesis of the endocardium and myocardimn. A significant amount of data on INVM are available for the left ventricle but right ventricular involvement is rare.

Pulmonary oligemia maneuver can alleviate pulmonary artery injury during pulmonary thromboendarterectomy procedure

Background Pulmonary thromboendarterectomy （PTE） has evolved as a treatment of choice for chronic thromboembolic pulmonary hypertension （CTEPH）. This study aimed to characterize if pulmonary oligemia maneuver （POM） can alleviate pulmonary artery injury during PTE procedure. Methods A total of 112 cases of CTEPH admitted to Beijing Anzhen Hospital from March 2002 to August 2011 received PTE procedure. They were retrospectively classified as non-POM group （group A, n=55） or POM group （group B, n=57）. Members from group B received POM during rewarming period, whereas members from group A did not. Results There were three （5.45%） early deaths in group A, no death in group B （0） （Fisher＇s exact test, P=0.118）. Six patients in group A needed extracorporeal membrane oxygenation （ECMO） as life support after the PTE procedure, no patients in group B needed ECMO （Fisher＇s exact test, P=0.013）. The patients in group B had a shorter intubation and ICU stay, lower mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR）, higher partial pressure of oxygen in artery （PaO2） and arterial oxygen saturation （SaO2） and less medical expenditure than patients in group A. With a mean follow-up time of （58.3 ± 30.6） months, two patients in group A and one patient in group B died. The difference of the actuarial survival after the procedure between the two groups did not reach statistical significance. Three months post the PTE procedure, the difference of residual occluded pulmonary segment between the two groups did not reach statistical significance （P=0.393）. Conclusion POM can alleviate pulmonary artery injury, shorten ICU stay and intubation time, and lower down the rate of ECMO after PTE procedure.

Pulmonary artery injury management during pulmonary thromboendarterectomy

Pulmonary thromboendarterectomy （PEA） is the recommended treatment for chronic thromboembolic pulmonary hypertension （CTEPH）.1 Pulmonary artery injury is a major risk factor during PEA performed at less experienced surgical centers and is associated with adverse short-term consequences.In-hospital mortality may be as high as 15% in some PEA case series,with pulmonary artery injury and persistent pulmonary hypertension as the principal etiologies.2 Many techniques have been used to prevent pulmonary artery injury during PEA,but once the injury occurs,the prognosis is poor and may even result in death.Out of 202 PEA procedures performed at our surgical center,we successfully managed two cases of severe pulmonary artery injury and report our experience in the present case series.Keywords：chronic thromboembolic pulmonary hypertension; pulmonary thromboendarterectomy; pulmonary artery injury