Surgical Treatment of Another Sequalae of COVID-19: Post-COVID CTEPH.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is still an ongoing entity and every day we face new sequalae of the disease. We hereby present surgical results of patients who are treated for post-COVID chronic thromboembolic pulmonary hypertension. METHODS: Data were collected among patients who underwent pulmonary endarterectomy and had a diagnosis of post-COVID chronic thromboembolic pulmonary hypertension. All data were retrospectively reviewed from a prospectively conducted database. Operative mortality was described as death in hospital or within 30 days of surgery. RESULTS: Eleven patients (seven males, four females; median age, 52 [22-63] years) were identified. Pulmonary vascular resistance improved significantly from 572 dyn/s/cm-5 (240-1,192) to 240 (195-377) dyn/s/cm-5 (p < 0.005). Significant difference was also detected in median mPAP, as it decreased from 40 mm Hg (24-54) to 24 mm Hg (15-36) following surgery (p < 0.005). Mortality was observed in one patient due to sepsis on the fifth postoperative day. Median time from COVID-19 disease to surgery was 12 months (6-24). Median length of hospital stay of the survivors was 10 days (8-14). CONCLUSION: In the new era of chronic thromboembolic pulmonary hypertension, hybrid approach including surgery, balloon pulmonary angioplasty, and medical treatment has been recommended. pulmonary endarterectomy is still the only curative treatment when the disease is surgically accessible. We hereby report the first publication of post-COVID chronic thromboembolic pulmonary hypertension patients who were surgically treated. As we see a lot of long-term symptoms and clinical manifestations in patients who had COVID-19, we should always remember chronic thromboembolic pulmonary hypertension in the differential diagnosis.

Evaluation of Asymmetric Dimethylarginine Levels in Patients With Chronic Thromboembolic Pulmonary Hypertension Undergoing Pulmonary Endarterectomy.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary embolism, and pulmonary endarterectomy (PEA) is the surgical treatment. Asymmetric dimethylarginine (ADMA) levels are increased in pulmonary hypertension. This study aimed to investigate serum ADMA levels in patients with CTEPH, the effect of PEA on ADMA, and its prognostic value in long-term mortality. METHOD: Eighty (80) patients with CTEPH and 32 healthy controls were included. Preoperative serum ADMA levels, determined using an enzyme-linked immunosorbent assay, were compared between patients with CTEPH and controls. Of 80 patients, 64 had PEA. Pre- and 6-month postoperative serum ADMA levels, 6-minute walk distance (6MWD), and haemodynamic parameters were collected from patients undergoing PEA. Patients were followed-up for survival analysis. RESULTS: Mean ± standard deviation serum ADMA levels were significantly higher in patients with CTEPH compared with controls (0.79±0.32 µmol/L vs 0.52±0.12 µmol/L; p=0.0001). Statistically significant differences were observed between preoperative and postoperative serum ADMA levels (0.78±0.30 µmol/L vs 0.62±0.22 µmol/L; p=0.0001), 6MWD (p=0.0001), and pulmonary vascular resistance (p=0.0001) in 60 patients who underwent and survived PEA. The decrease in serum ADMA levels and increase in 6MWD were significantly correlated (r=-0.286, p=0.027). No other correlation was found. Perioperative mortality was 6.3%, and the survival rate with a mean follow-up of 34.57±8.20 months was 93.3%. Patients with serum ADMA levels >0.8 µmol/L had a significantly lower survival rate (logrank: 5.86; p=0.015). CONCLUSIONS: Levels of circulating ADMA might add diagnostic and prognostic information in CTEPH. Pulmonary endarterectomy is associated with an improvement in serum ADMA levels. Preoperative serum ADMA levels may be useful for estimating the outcome of PEA.

Outcomes of Pulmonary Endarterectomy Operation Concomitant with Cardiac Procedures.

BACKGROUND: The aim of this study was to analyze the results of pulmonary endarterectomy (PEA) performed simultaneously with additional cardiac procedures in a single tertiary-level center. METHODS: Data of patients who underwent PEA with additional cardiac procedures for chronic thromboembolic pulmonary hypertension (CTEPH) in our clinic were retrospectively reviewed using patient records. RESULTS: Between March 2011 and April 2019, 56 patients underwent PEA with additional cardiac surgery. The most common additional procedure was coronary artery bypass grafting (21 patients; 38%). The median intensive care unit and hospital stays were 4 (3-6) days and 10 (8-14) days. Mortality was recorded in six patients (11%). In multivariate analysis, only preoperative pulmonary vascular resistance (PVR) (p = 0.02; odds ratio [OR]: 1.003) and cardiopulmonary bypass duration (p = 0.02; OR: 1.028) were associated with mortality. When the cutoff value of 1000 dyn.s.cm-5 was taken in the receiver operating characteristic curve analysis, preoperative PVR predicted mortality with 83% sensitivity and 94% specificity (area under curve = 0.89; p < 0.01). CONCLUSION: PEA for CTEPH may be performed safely with other cardiac operations. This type of surgery is a complex procedure that should be performed only in expert centers. Patients with high preoperative PVR are at increased risk of perioperative complications.

Outcomes of Patients with Behçet's Syndrome after Pulmonary Endarterectomy.

BACKGROUND: Behçet's syndrome (BS) is a multisystem disorder and is not known as a risk factor for chronic thromboembolic pulmonary hypertension (CTEPH), for which the treatment of choice is pulmonary endarterectomy (PEA). The aim of this study was to review our experience in the surgical treatment of CTEPH in patients with BS. METHODS: Data were collected prospectively for consecutive patients with BS who underwent PEA over a 6-year period. RESULTS: We identified nine patients (seven males, two females, mean age: 34.7 ± 9.9 years) with BS. The mean disease duration before PEA was 88.0 ± 70.2 months. All patients but one received immunosuppressive therapy before the surgery. Exercise-induced dyspnea presented symptoms in six patients. One patient had associated intracardiac thrombosis. PEA was bilateral in five patients, unilateral in three, and lobar in one. No perioperative mortality was observed; however, one patient died four weeks after PEA due to massive hemoptysis. Morbidity was observed in two patients. The systolic pulmonary artery pressure fell significantly from 59.0 ± 22.7 mm Hg to 30.0 ± 6.5 mm Hg after surgery (p = 0.031). Pulmonary vascular resistance also improved significantly from 611.8 ± 300.2 to 234.7 ± 94.9 dyn/s/cm5 (p = 0.031). After a median follow-up of 29.4 months, all patients improved to the New York Heart Association (NYHA) functional class I and II. CONCLUSION: Patients with BS may suffer recurrent pulmonary embolism and develop CTEPH. In patients who do not respond to anticoagulation or immunosuppressive therapy, PEA may be a therapeutic option when thrombotic lesions are surgically accessible. Due to the high risk of perioperative mortality, the procedure should be undertaken in centers with experience.

Evaluation of apoptotic molecular pathways for smooth muscle cells isolated from thoracic aortic aneurysms in response to oxidized sterols.

Oxysterols, oxygenated derivatives of cholesterol, are found abundantly in the plasma and atherosclerotic plaques, a common risk factor for thoracic aortic aneurysms (TAAs). Among the oxysterols, namely 7-ketocholesterol (7-KC) and 25-hydroxycholesterol (25-OHC), lead both to induction of reactive oxygen species (ROS) in cells and to apoptosis in smooth muscle cells (SMCs) probably due to increased oxidative stress. Since loss of SMCs through apoptosis is a major event in TAA formation, it is important to understand the molecular pathways of apoptosis in response to ROS in TAAs. Very little is known about the effect of oxysterols on TAA SMCs. Therefore, we investigated molecular pathways participating in the oxysterol induced cell death of TAAs. Our results showed that TAA SMCs died mainly as a result of apoptosis as suggested by cellular shrinkage, blebbing, DNA condensation/fragmentation in response to oxysterol treatment. There was no significant difference in oxysterol induced cell death between TAA and control SMCs. Addition of antioxidant molecules prevented cell death, hence ROS appears to be involved in the apoptosis of these cells. While oxysterol treatment increased caspase 3 activity, cell death was not rescued in its absence. Efficient silencing of other targets including apoptotic proteins (p53, Bax), and survival proteins (Akt1, Akt2) showed that apoptosis can occur through p53, and Bax independent pathways. Silencing Akt1 or Akt2 did not lead to further cell death. These results indicate that oxysterols can induce several cell death pathways in TAA SMCs.

Management of massive pulmonary hemorrhage following pulmonary endarterectomy.

Massive pulmonary hemorrhage is a potentially fatal complication of pulmonary endarterectomy. We describe a successful management in which the patient's right lower lobe bronchus was occluded with a Fogarty balloon catheter while on peripheral venoarterial extracorporeal membrane oxygenation.

Surgical treatment of double valve endocarditis.

BACKGROUND: We have retrospectively analyzed the results of the operations made for aortic infective endocarditis with mitral involvement in a single center in 19 years. METHODS: From May 1992 to January 2011, we have operated on 72 patients with infective endocarditis of the aortic valve with mitral valve involvement. Fifty-two patients (72.2%) were male and the mean age was 40.5 ± 15.5 (9-73) years. The blood cultures were positive in 33 patients (45.8%) and the most commonly identified microorganism was Streptococcus. Nine patients (12.5%) had prosthetic valve endocarditis. The mean duration of follow-up was 6.8 ± 4.7 (0.1-16.9) years, adding up to a total of 156.1 patient/years. RESULTS: A total of 155 procedures were performed on these 72 patients. The most commonly performed procedure was aortic valve replacement, in 63 patients (87.5%). Aortic annular involvement was present in 9 cases (12.5%). In-hospital mortality was seen in 13 patients (18.1%). Postoperatively, 13 (18.1%) patients had low cardiac output, 9 (12.5%) had heart block, and only 1 of them required permanent pacemaker implantation. The actuarial survival rates for 1, 5, and 10 years were 96.4% ± 2.5%, 84.4% ± 5.1%, and 77.4 ± 6.7%, respectively. CONCLUSIONS: Double-valve endocarditis is a serious condition and the surgeon must be aware of the high rates of mortality and morbidity in these patients. Although no association was found, heart blocks and septic embolization must be handled with caution. The patients generally do well after surgery, and recurrences and reoperations decrease by the second year after operation.

Prognostic significance of the absence of normal septal Q waves before aortic valve replacement.

PURPOSE: Aim of this study was to investigate the prognostic significance of absence of septal Q waves in patients scheduled for aortic valve replacement. MATERIAL AND METHODS: Sixty-one patients who underwent isolated aortic valve replacement for aortic stenosis were retrospectively evaluated. Septal Q waves were defined as Q waves of<2mm in amplitude and<40ms in width and absence of septal Q waves was defined as simultaneous loss of Q waves from at least three of the leads I, aVL, V5 and V6. Septal Q waves were absent in 17 patients (Group AQ, 27.8%) and were present in 44 patients (Group PQ, 72.1 %) preoperatively. Newly developed AV block>1st degree and newly developed left bundle branch block were primary endpoints. RESULTS: Preoperatively, absence of normal septal Q waves was significantly associated with increased risk of postoperative AV block (HR: 11.18, range 1.37-91.21, 95% CI, p=0.02) whereas it was not associated with increased risk for newly developed LBBB (HR: 3.15 0.62-15.83, 95% CI, p=0.16). CONCLUSION: Absence of normal septal Q waves in the preoperative ECG may predict further delay in conduction which might develop in the early postoperative course of aortic valve replacement.

The Effect of Perioperative Fluid Management and Operative Modifications on Mortality and Morbidity in Patients Undergoing Pulmonary Endarterectomy.

INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe disease treated with pulmonary endarterectomy. Our study aims to reveal the differences in liquid modalities and operation modifications, which can affect the patients' mortality and morbidity. METHODS: One hundred twenty-five patients who were diagnosed with CTEPH and underwent pulmonary thromboendarterectomy (PTE) at our center between February 2011 and September 2013 were included in this retrospective study with prospective observation. They were in New York Heart Association functional class II, III, or IV, and mean pulmonary artery pressure was > 40 mmHg. There were two groups, the crystalloid (Group 1) and colloid (Group 2) liquid groups, depending on the treatment fluids. P-value < 0.05 was considered statistically significant. RESULTS: Although the two different fluid types did not show a significant difference in mortality between groups, fluid balance sheets significantly affected the intragroup mortality rate. Negative fluid balance significantly decreased mortality in Group 1 (P<0.01). There was no difference in mortality in positive or negative fluid balance in Group 2 (P>0.05). Mean duration of stay in the intensive care unit (ICU) for Group 1 was 6.2 days and for Group 2 was 5.4 days (P>0.05). Readmission rate to the ICU for respiratory or non-respiratory reasons was 8.3% (n=4) in Group 1 and 11.7% (n=9) in Group 2 (P>0.05). CONCLUSION: Changes in fluid management have an etiological significance on possible complications in patient follow-up. We believe that as new approaches are reported, the number of comorbid events will decrease.

The angiogenic gene profile of pulmonary endarterectomy specimens: Initial study.

Objectives: The underlying mechanisms for the development of chronic thromboembolic pulmonary hypertension and prognostic biomarkers are not clear yet. Thus, our aim is to assess and identify new biomarkers for the expression of 84 key genes linked to angiogenesis. Methods: Patients who had levels more than 1000 dynes·sec·cm-5 were included in the test group, and the other patients were included in the control group. Twelve specimens were taken from the patients. RT2 Profiler PCR Array (Qiagen) was used to quantify the expression of the 84 key genes. Results: Eight patients (6 male, 2 female, median age 54.4 ± 13.1 years) who underwent pulmonary endarterectomy were included. Pulmonary vascular resistance improved significantly from 811 ± 390 dyn/s/cm-5 to 413.3 ± 144.9 dyn/s/cm-5 (P < .005). A difference was also detected in median mean pulmonary arterial pressure, which decreased from 49.8 ± 9 mm Hg to 32.62 ± 2.50 mm Hg (P > .005) after surgery. Median length of hospital stay was 11.62 ± 2.97 days. The test group had a distinct pattern of impaired angiogenic and antiangiogenic genes. The expression levels of TGFA, TGFB1, THBS2, THBS1, TGFBR1, SERPINE1, SERPINF1, TGFB2, TIMP2, VEGFC, IFNA1, TNF, CXCL10, NOS3, IGF1, and MMP14 were downregulated in the specimens from the patients who had higher pulmonary vascular resistance values, whereas some genes, including PDGFA, showed upregulation that was statistically nonsignificant in the same group. Conclusions: These results can lead to the development of new markers that could predict adverse outcomes of patients with CTEPH. Identification of new markers that are related to worse outcomes would enable screening patients for early diagnosis and treatment.

Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?

Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

Extracellular matrix collagen biomarker levels in patients who underwent pulmonary endarterectomy.

OBJECTIVES: The role of extracellular matrix collagen biomarkers in chronic thromboembolic pulmonary hypertension (CTEPH) is not well known. Our goal was to investigate the matrix metalloproteinase (MMP)-2 and -9 protein levels in patients with CTETH. METHODS: This is a prospective, cross-sectional study. Patients with CTETH who underwent pulmonary endarterectomy comprise group 1, and the control group included patients who underwent lung surgery without pulmonary hypertension (group 2) between March 2020 and March 2021. In addition to serum levels of MMP-9, the pulmonary endarterectomy and control pulmonary artery tissue samples were measured by the enzyme-linked immunosorbent assay  4pl, cubic, quadratic and Western blot techniques. Levels of MMP-2, which consist of pro MMP-2/ß-actin and active MMP-2/ß-actin and MMP-9/ß-actin, were measured only in the tissue samples. RESULTS: Forty-eight patients were enrolled consecutively in group 1 (n: 24) and group 2 (n: 24). The serum concentrations of MMP-9 were similar in both groups. Similarly, a comparison of tissue sample levels of pro MMP-2/ß-actin (P = 0.496) and active MMP-2/ß-actin (P = 0.216) showed no significant difference between the groups. The tissue samples from patients with CTETH had significantly lower amounts of MMP-9/ß-actin compared to the control group (P = 0.001). CONCLUSIONS: This study indicates that serum levels of extracellular matrix collagen biomarkers were similar in patients with CTETH who were candidates for surgery and in patients who had non-pulmonary hypertension who underwent lung surgery. Differences in levels of MMP-9/ß-actin in tissue samples may play a role in pulmonary vascular remodelling in operable patients.

Midterm results of axilloaxillary cardiopulmonary bypass.

BACKGROUND: Total axilloaxillary cardiopulmonary bypass (CPB) is an alternative peripheral cannulation technique that has the advantages of antegrade flow during CPB, monohemispherical brain perfusion in case of circulatory arrest, and achieving excellent decompression of the heart during sternotomy. The results of this strategy, particularly beyond the immediately postoperative period, are not well known. METHODS: Eleven patients with huge aortic aneurysms (>80 mm) and/or acute-subacute ascending aorta dissections underwent surgery with totally axilloaxillary CPB. Short- and midterm outcomes, including survival and complications relating to axilloaxillary cannulation, were reported. RESULTS: All attempts at axillary artery cannulation were successful. Ten of the 11 axillary vein cannulation attempts were successful, and the target pump flow was achieved via the axillary vein alone. Postoperatively, clinical examinations revealed no cases of arm ischemia or compartment syndrome. Three patients (27.3%) experienced ipsilateral brachial plexus neuropathy that produced right hand weakness. The neuropathy was transient in 2 patients, and the symptoms resolved completely. Hospital death occurred in 1 (9.1%) of the 11 patients. The mean (±SD) follow-up time was 956 ± 292 days. One of the survivors died on postoperative day 105 from subacute graft infection and sepsis. The right arms of all 9 of the living patients were examined physically and by Doppler ultrasonography. We found a chronic recanalized thrombotic change in the subclavian vein in 1 patient (11.1%), who had no complaints. CONCLUSIONS: Axilloaxillary CPB is an alternative technique that can be used under certain conditions. Adding axillary venous cannulation to axillary artery cannulation at least does not increase the risk of a procedure that uses the axillary artery alone, either in the early or mid term.

Diagnosis of Pulmonary Artery Sarcoma with Multi-modality Imaging.

Pulmonary artery sarcoma is an extremely uncommon malignancy with a poor prognosis. It is often difficult to distinguish it from pulmonary thromboembolic disease because of nonspe cific signs and symptoms as well as similar imaging findings. We present a 46-year-old man who had initially been diagnosed with presumed asthma that later proved to be pulmonary artery sarcoma. The patient was evaluated with multi-modality imaging studies which showed a mass in the pulmonary artery, its extension, mobility and invasion, and attachment to the artery wall. Pulmonary artery mass was excised and pulmonary artery endarterectomy was performed. The histopathological diagnosis was undifferentiated sarcoma with pleomorphic morphology.

Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience.

BACKGROUND: Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. RESULTS: We identified 9 patients (6 women; median age, 48 years [range, 23-55]) with IPV. The diagnosis was confirmed after histopathologic examination of all surgical materials. The mean duration of disease before surgery was 88.0 ± 70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in 6 patients and unilateral in 3. No deaths occurred; however 1 patient had pulmonary artery stenosis, and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30 mm Hg (range, 19-67) to 21 mm Hg (range, 15-49) after surgery (P < .05). Pulmonary vascular resistance also improved significantly from 270 dyn/s/cm-5 (range, 160-1600) to 153 dyn/s/cm-5 (range, 94-548; P < .05). After a median follow-up of 41 months, all but 1 patient had improved to the New York Heart Association functional class I. CONCLUSIONS: IPV can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

Is There Any Role of Pulmonary Endarterectomy in Pulmonary Arterial Hydatidosis?

BACKGROUND: Hydatid cyst is a zoonosis caused by Echinococcus granulosis. Pulmonary artery involvement is a rare condition. The aim of this study was to review the investigators' experience with the surgical treatment of pulmonary arterial hydatidosis. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy (PEA) and who had a diagnosis of hydatidosis at or after PEA. RESULTS: A total of 8 patients (2 male and 6 female; mean age, 31.25 ± 13.68 years) with hydatidosis were defined. Only 1 patient presented with hemoptysis, whereas the rest of the patients reported exertional dyspnea as their main symptom. Cardiac hydatidosis associated with pulmonary arterial involvement was noted in 1 patient. The mean time interval for duration of disease was 12 ± 24.29 months before PEA. Mortality was observed in 2 patients as a result of massive hemoptysis in 1 patient and right-sided heart failure in the other. No anaphylactic reaction was observed. Significant difference was detected in mean pulmonary vascular resistance as a decline from 442.38 ± 474.20 dyn/s/cm-5 to 357.25 ± 285.34 dyn/s/cm-5 after PEA (P = .011). Two patients had recurrence of the disease after a median follow-up of 9.1 months All survivors improved to New York Heart Association functional classes I and II. CONCLUSION: Pulmonary arterial hydatidosis may mimic chronic thromboembolic pulmonary hypertension, and in these patients the diagnosis can be made with PEA. Pulmonary endarterectomy may be a therapeutic option for patients who do not respond to medical therapy if the cystic lesions are surgically accessible. PEA should be performed only in expert centers because of the high risk of perioperative morbidity, mortality, and postoperative recurrence.

Pulmonary Endarterectomy in Patients with Antiphospholipid Syndrome-Associated Chronic Thromboembolic Pulmonary Hypertension.

BACKGROUND: Antiphospholipid syndrome is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thrombo-embolic pulmonary hypertension. Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. The aim of this study is to evalu-ate the efficacy and risk of pulmonary endarterectomy in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. METHODS: Data were prospectively collected and retrospectively analyzed, for patientswho underwent pulmonary endarterectomy between March 2011 and March 2020. RESULTS: Seventeen patients (4 male and 13 female) were identified. Thirteen patients had primary antiphospholipid syndrome and 4 had secondary antiphospholipid syndrome. The mean age was 34.82 ± 10.07 years and the mean time interval between the diagno-sis and surgery was 26.94 ± 17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, 5 patients had a history of recurrent abortions, and 2 patients had hemoptysis. Following surgery, mean pulmo-nary artery pressure decreased from 47.82 ± 13.11 mm Hg to 22.24 ± 4.56 mm Hg (P < .001), and pulmonary vascular resistance improved from 756.50 ± 393.91 dyn/s/cm-5 to 298.31 ±132.84 dyn/s/cm-5 (P < .001). There was no in-hospital mortality with a mean follow-up of 75.29 ± 40.21 months. The functional capacity of all patients improved from 269.46 ±111.7 m to 490 ± 105.34 m on a 6-minute walking test. CONCLUSIONS: Pulmonary endarterectomy is a safe and curative treatment in patientswith antiphospholipid syndrome-associated chronic thromboembolic pulmonary hyper-tension. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced chronic thromboembolic pulmonary hypertension team is critical in the management of these unique patients.

Repair of pulmonary artery aneurysms.

BACKGROUND AND AIM OF THE STUDY: We have presented the results of pulmonary arterial aneurysm repairs performed over eight years. METHODS: From 2002 to December 2010, we performed nine operations for pulmonary artery aneurysms in our department. The mean age of the patients was 37.8 ± 17.1 years and four were females. Five patients had pulmonic valve stenosis, three patients had concomitant pathologies on the aortic or mitral valves, and two had isolated pulmonary arterial aneurysm. Aneurysm repairs were performed by plication in eight patients and with Dacron patch repair in one patient. RESULTS: There were no postoperative deaths. Three patients required inotropic support postoperatively. One patient had pulmonary morbidity. The results of the pathology revealed intimal hyalinization, edema, and elastic fiber degeneration in the media, fibrosis in the adventitia, and increased vascularity. All patients were in New York Heart Association Class I or II except for one patient who had a prolonged intensive care stay. The mean duration of follow-up was 48.6 ± 33.2 months (range, 2 to 107) adding up to a total of 36.4 patient/years. The mean diameter of the pulmonary artery in the recent imaging was 3.6 ± 0.4 cm. CONCLUSION: Pulmonary artery aneurysms are rare entities which are usually associated with other congenital defects. While surgery is the preferred treatment for symptomatic aneurysms, controversy exists as to the indications for repair in asymptomatic patients.

Chronic thromboembolic pulmonary hypertension: evaluation of V/Q SPECT/CT and V/Q Quotient SPECT findings with postoperative results of pulmonary endarterectomy.

OBJECTIVES: We aimed to perform a comparison between V/Q single-photon emission computed tomography/computed tomography (SPECT/CT) and V/Q Quotient single-photon emission computerized tomography (SPECT) in the detection of chronic thromboembolic pulmonary hypertension (CTEPH) and in depicting the extent of the disease on per-segment basis in patients with CTEPH. METHODS: Between January 2015 and November 2019, a total of 412 patients with pulmonary hypertension secondary to CTEPH at the preoperative assessment underwent pulmonary endarterectomy (PEA), of whom 92 consecutive patients with their V/Q SPECT/CT scans have been performed in our institution prior to PEA were included in this study. Histopathological findings and post-PEA fully resected surgical specimens were used as the reference standard. RESULTS: On a per-patient basis analysis, V/Q SPECT/CT and V/Q Quotient SPECT both revealed CTEPH in the same 85 of the 92 patients (κ = 1) with a detection rate of 92.4%. In six of these patients, chronic thromboembolic disease could not be reported on both of these two methods due to extensive 'matched' V/Q defects. On a per-segment basis analysis, V/Q SPECT/CT and V/Q Quotient SPECT showed a sensitivity of 75.8 and 73.1%, respectively. Correlation analysis results showed a significant correlation (κ = 0.933) between these two methods on a per-segment basis analysis. CONCLUSION: In the light of histopathological findings and post-PEA surgical specimen examinations, the results of the present study indicated that both V/Q SPECT/CT and V/Q Quotient SPECT showed relatively high efficacy for the detection of CTEPH on per-patient and per-segment bases with an excellent agreement.

Raising the bar to ultradisciplinary collaborations in management of chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension is an underdiagnosed and potentially fatal subgroup of pulmonary hypertension, if left untreated. Clinical signs include exertional dyspnea and non-specific symptoms. Diagnosis requires multimodality imaging and heart catheterization. Pulmonary endarterectomy, an open heart surgery, is the gold standard treatment of choice in selected patients in specialized centers. Targeted medical therapy and balloon pulmonary angioplasty can be effective in high-risk patients with significant comorbidities, distal pulmonary vascular obstructions, or recurrent/persistent pulmonary hypertension after pulmonary endarterectomy. Currently, there is a limited number of data regarding novel coronavirus-2019 infection in patients with chronic thromboembolic pulmonary hypertension and the changing spectrum of the disease during the pandemic. Challenging times during this outbreak due to healthcare crisis and relatively higher case-fatality rates require convergence; that is an ultradisciplinary collaboration, which crosses disciplinary and sectorial boundaries to develop integrated knowledge and new paradigms. Management strategies for the "new normal" such as virtual care, preparedness for further threats, redesigned standards and working conditions, reevaluation of specific recommendations, and online collaborations for optimal decisions for chronic thromboembolic pulmonary hypertension patients may change the poor outcomes.