Unilateral Chronic Thromboembolic Pulmonary Disease: Do Patients Benefit From Thromboendarterectomy? Case Series From Three CTEPH Centres.

BACKGROUND: Unilateral chronic thromboembolism pulmonary disease (CTEPD) is very rare. There is limited information on the safety and efficacy of pulmonary endarterectomy (PEA) in this population. This study investigated the effectiveness of PEA in this unique disease. METHODS: This multicentre study included patients with unilateral CTEPD from three referral centres in the United States, Turkey, and Iran. The patients' demographic information, 6-minute walk test distance (6MWD), New York Heart Association (NYHA) functional class, and haemodynamics were evaluated. RESULT: Of the 1,031 patients who had undergone PEA, 39 patients (3.7%) had pure unilateral involvement, of whom 28 were female (71.8%). There was a significant improvement in the mean pulmonary artery pressure (mPAP, 26 mmHg vs 21 mmHg; p=0.011) and pulmonary vascular resistance (PVR, 202 vs 136 dynes∗sec1∗cm-5; p=0.014). There was also a significant improvement in NYHA functional class (p<0.001) and 6MWD (360 vs 409 m; p<0.001). In the nine patients with normal haemodynamic parameters at rest, there was no significant change in median 6MWD (448.5 vs 449 m; p=0.208), mPAP (19 mmHg vs 16.5 mmHg; p=0.397), and PVR (129 vs 84.5 dynes∗sec1∗cm-5; p=0.128). The most common postoperative complication was ipsilateral pleural effusion. One patient needed extracorporeal membrane oxygenation support. No patient died within the 1-year follow up. CONCLUSION: Pulmonary endarterectomy is a safe and effective procedure for improving the symptoms and haemodynamic parameters of patients with unilateral CTEPH. Symptomatic patients with unilateral chronic thromboembolic disease are suitable for PEA.

Pulmonary Endarterectomy: Risk Factors for Early and Late Mortality.

Background Pulmonary endarterectomy is a potentially curative treatment option for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to identify predictors of short- and long-term outcomes after pulmonary endarterectomy for CTEPH patients, including age. Methods Patients who underwent surgery between March 2014 and January 2024 were included in the study. Perioperative and follow-up data were retrospectively studied, including age, in-hospital mortality, one- and five-year survival, and the length of intensive care unit and hospital stays after pulmonary endarterectomy. Results In total, 834 consecutive patients (mean age 51±15.3 years) underwent pulmonary endarterectomy and were included in the analysis. The in-hospital mortality rate was 7.8% (n = 65), while overall mortality rates at one and five years were 10.6% and 11.3%, respectively. The in-hospital mortality rate was 6.7% for patients < 70 years compared to 12.4% for patients ≥70 years (p=0.029). In the multivariate analysis of mortality, age (p=0.007), and length of intensive care unit stay (p= 0.028) emerged as independent predictors of in-hospital mortality, while the Charlson Comorbidity Index (p<0.001) and six-minute walk distance (p=0.005) were also significant predictors of one-year survival. Conclusion Despite higher short-term mortality rates, pulmonary endarterectomy was feasible and well tolerated among elderly patients. Despite surgical advancements, careful patient selection remains crucial, particularly in the presence of comorbidities. Significant clinical and hemodynamic improvements were observed, along with favorable long-term survival outcomes.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Correlation Between Perfusion Abnormalities Extent in Ventilation/Perfusion SPECT/CT with Hemodynamic Parameters in Patients with Chronic Thromboembolic Pulmonary Hypertension

Objectives: Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension with persistent pulmonary vascular obstruction and exercise intolerance, which may benefit from pulmonary endarterectomy (PEA). Ventilation/perfusion (V/Q) scan is the preferred screening test of CTEPH, which can be used to assess the anatomical extent of the disease. This study aimed to analyze the correlation between the extent of mismatched Q defects in V/Q single photon emission computed tomography/computed tomography (SPECT/CT) with preoperative clinical and hemodynamic parameters in patients with CTEPH. Methods: A total of 102 patients with CTEPH prior to PEA having V/Q SPECT/CT scans were retrospectively reviewed. Age, gender, New York Heart Association classification, intraoperative right-sided heart catheterization (mPAP and PVR), and 6-minute walk test (6MWT) findings were obtained from clinical records of patients. Results: Linear regression analysis showed a significant but weak correlation between the preoperative mPAP and PVR with the extent of mismatched Q defects in V/Q SPECT/CT (rs=0.09474 with p=0.0016 and rs=0.045 with p=0.045, respectively). No significant correlation was found between 6MWT and extent of mismatched Q defects in V/Q SPECT/CT (p>0.05). Conclusion: A quantitative assessment of Q defects on V/Q SPECT/CT might provide information about hemodynamic parameters in patients with CTEPH.

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.

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.

Comparison between arch zones in modified frozen elephant trunk procedure for complex thoracic aortic diseases

Abstract Introduction: The aim of this study is to compare postoperative outcomes and follow-up of two different modifications facilitating surgical technique of frozen elephant trunk (FET) procedure for complex thoracic aortic diseases - zone 0 (fixation with total arch debranching) and zone 3 (fixation with islet-shape arch repair). Methods: From May 2012 to December 2018, data were collected from 139 patients who had been treated with FET procedure for complex thoracic aortic diseases. According to Ishimaru arch map, patients with proximal anastomotic site of hybrid graft at zone 0 and zone 3 were grouped as Group A (n=58, 41.7%) and Group B (n=81, 58.3%), respectively. Mean age of study population was 54.7±11.4 years, and 111 patients were male (79.9%). Results: In-hospital mortality was observed in 20 (14.4%) patients (n=12, acute type A aortic dissection, and n=4, previous aortic dissection surgery). There was no significant difference between both groups in terms of in-hospital mortality. Four patients from Group A and three patients from Group B had permanent neurological deficit (P=0.32). Three patients from both groups had transient spinal cord ischemia (P=0.334). Although mean total perfusion time was longer in Group A, duration of visceral ischemia, when compared with Group B, was shorter (P<0.001). Five-year survival rate was 82.8% in Group A and 81.5% in Group B (P=0.876). Conclusion: FET procedure is a feasible repair technique in the treatment of complex aortic diseases, providing satisfactory early results. Because of its advantageous aspects, zone 0 fixation with debranching is the preferred technique in our clinic.

Comparison between Arch Zones in Modified Frozen Elephant Trunk Procedure for Complex Thoracic Aortic Diseases.

INTRODUCTION: The aim of this study is to compare postoperative outcomes and follow-up of two different modifications facilitating surgical technique of frozen elephant trunk (FET) procedure for complex thoracic aortic diseases - zone 0 (fixation with total arch debranching) and zone 3 (fixation with islet-shape arch repair). METHODS: From May 2012 to December 2018, data were collected from 139 patients who had been treated with FET procedure for complex thoracic aortic diseases. According to Ishimaru arch map, patients with proximal anastomotic site of hybrid graft at zone 0 and zone 3 were grouped as Group A (n=58, 41.7%) and Group B (n=81, 58.3%), respectively. Mean age of study population was 54.7±11.4 years, and 111 patients were male (79.9%). RESULTS: In-hospital mortality was observed in 20 (14.4%) patients (n=12, acute type A aortic dissection, and n=4, previous aortic dissection surgery). There was no significant difference between both groups in terms of in-hospital mortality. Four patients from Group A and three patients from Group B had permanent neurological deficit (P=0.32). Three patients from both groups had transient spinal cord ischemia (P=0.334). Although mean total perfusion time was longer in Group A, duration of visceral ischemia, when compared with Group B, was shorter (P<0.001). Five-year survival rate was 82.8% in Group A and 81.5% in Group B (P=0.876). CONCLUSION: FET procedure is a feasible repair technique in the treatment of complex aortic diseases, providing satisfactory early results. Because of its advantageous aspects, zone 0 fixation with debranching is the preferred technique in our clinic.

Repair of residual aortic dissections with frozen elephant trunk technique.

BACKGROUND: In this study, we present our mid-term results of reoperation with the frozen elephant trunk procedure due to patent false lumen-related complications in patients previously undergoing supracoronary aortic repair for acute type A aortic dissection. METHODS: Between January 2013 and September 2018, a total of 23 patients (17 males, 6 females; mean age 51.5±9.7 years; range, 30 to 67 years) who underwent ascending aortic replacement due to type A aortic dissection and, later, frozen elephant trunk procedure for residual distal dissection were included. For diagnostic purposes and follow-up, computed tomography angiography was performed in all patients, and both re-entry and aortic diameters were evaluated. Echocardiography was used to evaluate cardiac function and valve pathologies. RESULTS: The Ishimaru zone 0 (n=11, 47.8%), Ishimaru zone 1 (n=1, 4.3%), Ishimaru zone 2 (n=4, 17.4%), and Ishimaru zone 3 (n=7, 30.4%) were used for frozen elephant trunk stent graft fixation. The mean duration of cardiopulmonary bypass and antegrade selective cerebral perfusion was 223.9±71.2 min and 88.9±60.3 min, respectively. In-hospital mortality was 13%, while there was one (4.3%) aortic-related death and four (17.4%) re-interventions during follow-up. CONCLUSION: Early repair should be considered in the presence of persistent dissections due to alarmingly high mortality rates of reoperations. Reoperation with the frozen elephant trunk procedure has acceptable results and the decision of the procedure to be performed should be based on preoperative risk factors of the patient.

Chronic Thromboembolic Pulmonary Hypertension Secondary to Thrombophilia and Incidentally Diagnosed Atrial Septal Defect.

A 46-year-old man developed chronic thromboembolic pulmonary hypertension and atrial fibrillation after acute pulmonary embolism. He was found incidentally to have an isolated secundum atrial septal defect, as well as a homozygous mutation for the plasminogen activator inhibitor-1 gene. He was successfully treated with pulmonary endarterectomy and atrial septal defect repair. He has continued to do well on a regimen of dabigatran. (Level of Difficulty: Beginner.).