Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study.

BACKGROUND: Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. OBJECTIVE: This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. METHODS: A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years' observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. RESULTS: The most common symptoms were typical of those expected for CTEPH. Almost 90% of patients underwent right heart catheterization at diagnosis or initial study visit. In total, 66 patients (31%) underwent PEA before the initial visit; 95 patients (45%) were considered operable, 115 (54%) were inoperable, and two (1%) had no operability data. Only 26 patients (12%) had been assessed for BPA at their initial visit. PH-targeted therapy was documented at diagnosis for 77 patients (36%), most commonly a phosphodiesterase type 5 inhibitor (23%). Use of PH-targeted therapy increased to 142 patients (67%) at the initial visit, remaining similar after 3 years. Use of riociguat increased from 6% of patients at diagnosis to 38% at 3 years. Between baseline and end of observation, results for patients with paired data showed an increase in 6MWD. Survival at the end of observation was 88%. CONCLUSIONS: These data highlight the current diagnosis and management of CTEPH in the participating countries. They show that early CTEPH diagnosis remains challenging, and use of off-label PH-targeted therapy is common. CLINICALTRIALS: gov: NCT02637050; registered December 2015.

Which Diastolic Pressure Should Be Used to Assess Diastolic Function?

BACKGROUND: Although high left ventricular filling pressures [left ventricular (LV) end-diastolic pressure or pulmonary capillary wedge pressure (PCWP)] are widely taken as surrogates for LV diastolic dysfunction, the actual distending pressure that governs LV diastolic stretch is transmural pressure difference (∆PTM). Clinically, preferring ∆PTM over PCWP may improve diagnostic and therapeutic decision-making. We aimed to compare the clinical implications of diastolic function characterization based on PCWP or ∆PTM. METHODS: We retrospectively screened our hospital database for adult patients with a clinical diagnosis of heart failure who underwent right heart catheterization. Echocardiographic diastolic dysfunction was graded according to the current guidelines. LV end-diastolic properties were assessed with construction of complete end-diastolic pressure-volume relationship (EDPVR) curves using the single-beat method. Survival status was checked via the electronic national health-care system. RESULTS: A total of 693 cases were identified in our database; the final study population comprised 621 cases. ∆PTM-based, but not PCWP-based, EDPVR diastolic stiffness constants were significantly predictive of advanced diastolic dysfunction. PCWP-based diastolic stiffness constants were not able to predict 5-year mortality, whereas ∆PTM-based EDPVR stiffness constants and volumes all turned out to have significant predictive power for 5-year mortality. CONCLUSION: Left ventricular diastolic function assessment can be improved using ∆PTM instead of PCWP. As ∆PTM ultimately linked to right-sided functions, this approach emphasizes the limitations of taking LV diastolic function as an isolated phenomenon and underlines the need for a complete hemodynamic assessment involving the right heart in therapeutic and prognostic decision-making processes.

Is Pulmonary Capillary Wedge Pressure a Reliable Indicator of Postcapillary Pulmonary Hypertension?

Although current pulmonary hypertension (PH) guidelines recommend a pulmonary capillary wedge pressure (PCWP) >15 mm Hg for the detection of a postcapillary component, the rationale of this recommendation may not be quite compatible with the peculiar hemodynamics of PH. We hypothesize that a high PCWP alone does not necessarily indicate left-sided disease, and this diagnosis can be improved using left ventricle transmural pressure difference (∆ PTM). In this 2-center, retrospective, observational study, we enrolled 1,070 patients with PH who underwent heart catheterization, with the final study population comprising 961 cases. ∆ PTM was calculated as PCWP minus right atrial pressure. The patients with group II PH had significantly higher ∆ PTM values (12.6 ± 6.6 mm Hg) compared with the other groups (1.1 ± 4.8 in group I, 12.4 ± 6.6 in group II, 2.5 ± 6.4 in group III, and 0.8 ± 8.0 in group IV, p <0.001) despite overlapping PCWP values. A ∆ PTM cutoff of 7 mm Hg identifies left heart disease when PCWP is >15 (area under curve 0.825, 95% confidence interval 0.784 to 0.866, p <0.001). Five-year mortality was significantly higher in patients with high ∆ PTM and PCWP subgroups compared with low ∆ PTM plus high PCWP (26.1% vs 18.5%, p = 0.027) and low ∆ PTM and PCWP subgroups (26.1% vs 15.6%, p <0.001). ∆ PTM has supplementary discriminatory power in distinguishing patients with and without postcapillary PH. In conclusion, a new approach utilizing ∆ PTM may improve our understanding of PH pathophysiology and may identify a subpopulation that may potentially benefit from PH-specific treatments.

Bendopnea Predicts Right Ventricular Energy Failure in Patients with Pulmonary Hypertension.

OBJECTIVE: The development of right ventricular failure has a significant adverse prognostic impact on the course of pulmonary hypertension. Right ventricular energy failure has been shown to double the mortality of pulmonary hypertension even after correction for many established risk predictors. We hypothesize that bendopnea may indicate right ventricular energy failure in patients with pulmonary hypertension. METHODS: We prospectively enrolled patients with pulmonary hypertension who were admitted to our pulmonary hypertension outpatient clinic between January 2021 and June 2021. Bendopnea was assessed by asking patients to bend forward and report any shortness of breath within 30 seconds. Routine physical examination, laboratory tests, echocardiography, and right heart catheterization parameters were collected. RESULTS: A total of 167 patients were enrolled into the study. Bendopnea and right ventricular energy failure was present in 79 (47.3%) and 43 (25.7%) patients, respectively. Bendopnea accurately predicted the presence of right ventricular energy failure (area under the curve, 0.667; 95% CI, 0.574-0.760; P < 0.001) and had a significantly superior diagnostic power compared with many other symptoms and signs. CONCLUSIONS: Our study shows that bendopnea predicts right ventricular energy failure in patients with pulmonary hypertension and can be added to our physical examination armamentarium as an easy, rapid, and noninvasive prognostic tool.

Evaluation of Pulmonary Hypertension with Exercise Right Heart Catheterization in an Adult Patient with Cor Triatriatum Sinister.

Cor triatriatum sinister (CTS) is a rare adult congenital heart disease. The usual presentation may vary according to the size of the hole in the membrane in the left atrium and the pressure gradient. In addition to acute clinical presentations including acute pulmonary edema and sudden cardiac death, patients may present with chronic findings such as right heart failure due to pulmonary hypertension. The development of pulmonary hypertension is an important indicator of mortality. In cases where non-invasive methods are not sufficient for the diagnosis of pulmonary hypertension, exercise right heart catheterization may also be used. We present a patient with CTS, in whom the final decision was made with the help of an exercise right heart catheterization.

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.

Determinants of Severe Nocturnal Hypoxemia in Adults with Chronic Thromboembolic Pulmonary Hypertension and Sleep-Related Breathing Disorders.

OBJECTIVES: We aimed to investigate the occurrence of sleep-related breathing disorders (SRBDs) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and addressed the effect of pulmonary hemodynamics and SRBD indices on the severity of nocturnal hypoxemia (NH). METHODS: An overnight polysomnography (PSG) was conducted in patients with CTEPH, who were eligible for pulmonary endarterectomy. Pulmonary hemodynamics (mean pulmonary arterial pressure (mPAP), pulmonary arterial wedge pressure (PAWP), pulmonary vascular resistance (PVR) measured with right heart catheterization (RHC)), PSG variables (apnea-hypopnea index (AHI)), lung function and carbon monoxide diffusion capacity (DLCO) values, as well as demographics and comorbidities were entered into a logistic regression model to address the determinants of severe NH (nocturnal oxyhemoglobin saturation (SpO2) < 90% under >20% of total sleep time (TST)). SRBDs were defined as obstructive sleep apnea (OSA; as an AHI ≥ 15 events/h), central sleep apnea with Cheyne-Stokes respiration (CSA-CSR; CSR pattern ≥ 50% of TST), obesity hypoventilation syndrome (OHS), and isolated sleep-related hypoxemia (ISRH; SpO2 < 88% under >5 min without OSA, CSA, or OHS). RESULTS: In all, 50 consecutive patients (34 men and 16 women; mean age 54.0 (SD 15.1) years) were included. The average mPAP was 43.8 (SD 16.8) mmHg. SRBD was observed in 40 (80%) patients, of whom 27 had OSA, 2 CSA-CSR, and 11 ISRH. None had OHS. Severe NH was observed in 31 (62%) patients. Among the variables tested, age (odds ratio (OR) 1.08, 95% confidence interval [CI] 1.01-1.15; p = 0.031), mPAP (OR 1.11 [95% CI 1.02-1.12; p = 0.012]), and AHI (OR 1.17 [95% CI 1.02-1.35; p = 0.031]) were independent determinants of severe NH. CONCLUSIONS: Severe NH is highly prevalent in patients with CTEPH. Early screening for SRBDs and intervention with nocturnal supplemental oxygen and/or positive airway pressure as well as pulmonary endarterectomy may reduce adverse outcomes in patients with CTEPH.

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.

Right Ventricular Energy Failure Predicts Mortality in Patients With Pulmonary Hypertension.

Right ventricular (RV) failure has a significant adverse impact on pulmonary hypertension (PH) prognosis. None of the currently used parameters directly assess whether RV fails to provide enough energy output to propel the blood through diseased pulmonary vascular system. Furthermore, most of the current parameters are affected by the volume status of the patient. We aimed to explore whether RV energy failure has a predictive power for mortality on top of the established prognostic risk parameters in patients with PH. We screened 723 cases from our database. A total of 3 sets of binary regression analyses were executed to determine the hazard ratios (HRs) of RV energy failure for 5-year mortality in clinical, echocardiographic, and hemodynamic context, using adjustment variables chosen according to previous studies. The final study population encompassed 549 cases. A total of 77 patients died during the 5-year follow-up (14%). RV energy failure was observed in 146 of 549 patients (26.6%). In the univariate model, RV energy failure strongly associated with increased long-term mortality (HR 4.25, 95% confidence interval [CI] 2.58 to 7.00, p <0.001). It also emerged as a significant predictor of long-term mortality in clinical and hemodynamic multivariate models (HR 2.59, 95% CI 1.43 to 4.67, p = 0.002 and HR 2.05, 95% CI 1.15 to 3.63, p = 0.015, respectively). In conclusion, our study indicates that the presence of RV energy failure independently predicts long-term mortality in PH.

Are Guideline-recommended Risk Classification Schemes in Pulmonary Hypertension Adequately Robust to Guide the Real-world Setting?

Background: Pulmonary hypertension is a complex syndrome that encompasses a diverse group of pathophysiologies predisposed by different environmental and genetic factors. It is not clear to which extent the universal risk classification schemes can be applied to cohorts in individual pulmonary hypertension centers with differing environmental backgrounds, genetic pools, referral networks. Aims: To explore whether the recommended risk classification schemes could reliably be used for mortality prediction in an unselected pulmonary hypertension population of a tertiary pulmonary hypertension center. Study Design: A retrospective cross-sectional study. Methods: We retrospectively screened our hospital database for the patients with pulmonary hypertension between 2015 and 2022. The grouping of pulmonary hypertension was made as follows in accordance with current guidelines: Group 1: patients with pulmonary arterial hypertension, Group 2: patients with pulmonary hypertension associated with left heart disease, Group 3: patients with pulmonary hypertension associated with lung disease and/or hypoxia, and Group 4: patients with pulmonary hypertension associated with pulmonary artery obstructions. Then, we compared the predicted and observed mortality rates of four different risk classification schemes (REVEAL, REVEAL-Lite, ESC/ERS and COMPERA). Results: We identified 723 cases in our pulmonary hypertension database, the final study population consisted of 549 patients. The REVEAL, REVEAL-Lite and European Society of Cardiology/European Respiratory Society risk scores significantly underestimated the mortality risk in the low-risk stratum (5.3% vs. 1.9%, P < 0.001; 5.3% vs. 2.9%, P = 0.015 and 6.3% vs. 1%, P < 0.001, respectively) and overestimated the mortality risk in the high-risk stratum (11.8% vs. 25.8%, P < 0.001; 10.4% vs. 25.1%, P < 0.001 and 13.2% vs. 30%, P < 0.001, respectively). Although the COMPERA 4-strata model significantly underestimated the risk in low- and intermediate-low risk strata (4.9% vs. 1.5%, P < 0.001 and 6.8% vs. 2.8%, P = 0.001, respectively), it was accurate in intermediate-high and high-risk groups (10.1% vs. 8.7%, P = 0.592 and 15.6% vs. 22%, P = 0.384, respectively). The analyses limited only to group 1 pulmonary hypertension patients gave similar results. Conclusion: The established risk classification schemes may not perform as good as expected in unselected pulmonary hypertension populations and this may have important implications on management decisions. Tertiary centers should not uncritically accept the published risk prediction models and consider modifying current risk scores according to their own patient characteristics.

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.

Effects of balloon pulmonary angioplasty procedure on electrocardiographic parameters in patients with chronic thromboembolic pulmonary hypertension.

AIM: The aim of the present study was to evaluate the value of electrocardiography (ECG) in predicting postoperative hemodynamic improvement in patients with chronic thromboembolic pulmonary hypertension (CTEPH) undergoing balloon pulmonary angioplasty (BPA). MATERIAL AND METHODS: A total of 32 patients were included in the study. During ECG analysis, parameters that have been suggested to be related to right ventricular hypertrophy and/or dilatation were evaluated. The significance of the change in each parameter obtained at the pre-BPA visit and at the scheduled control visit 6 months after BPA was tested. In addition to ECG analysis, data related to right heart catheterization (RHC) and echocardiography, B-type natriuretic peptide (BNP) levels and World Health Organization (WHO) functional classifications of all patients were also recorded. The relationship between the amount of possible change in ECG parameters and the amount of possible change in hemodynamic parameters was investigated. RESULTS: The Daniel score, which has been suggested to have prognostic value in acute pulmonary embolism, decreased from 8.22 ± 5.68 to 6.56 ± 5.55 after the BPA procedure (p: 0.035). Among all parameters studied, only T wave height (V2 t) in V2 derivation changed significantly from -0.77 ± 2.39 to 1.27 ± 2.58 mm (p: 0.036). The amount of change in V2 T was found to significantly correlate with the amount of change in systolic right ventricular pressure, mean pulmonary artery pressure, pulmonary vascular resistance, and systemic vascular resistance. CONCLUSION: Postprocedural T wave changes in lead V2 might serve as a marker of hemodynamic improvement in patients with CTEPH who undergo BPA.

Cardiologists' Awareness of the Relationship Between Periodontal Diseases and Cardiovascular Diseases: A Region-Wide Survey in Türkiye.

OBJECTIVE: The relationship between oral health and general health has gained increased attention in recent years. This study sought to assess the knowledge, attitudes, and practices of cardiologists in Türkiye concerning the link between periodontal disease and cardiovascular disease (CVD). METHODS: After a pilot test, a modified survey was dispatched to 1,894 practicing cardiologists in Türkiye. Two mailings were carried out, and descriptive statistics were used to analyze the data. RESULTS: Of the 1,894 cardiologists surveyed, 166 responded, yielding a response rate of 11.5%. The majority of respondents (77%) were male and held professional positions in academia (45%), as assistant doctors (17.5%), or in private practice (12.7%). Ninety percent of respondents accurately recognized periodontal disease as a chronic, multifactorial inflammatory disease. Meanwhile, 78% concurred that inflammation is a pivotal connection between periodontal disease and CVD. On the topic of whether treating periodontal disease could reduce a patient's CVD risk, 37% of the polled cardiologists expressed uncertainty, while 9% disagreed. Seventy six percent believed that periodontists and cardiologists should collaborate to reduce shared risk factors for both cardiovascular and periodontal diseases. Additionally, 80% expressed interest in deepening their understanding of the link between periodontitis and CVD. CONCLUSION: While the vast majority of participants acknowledged that microbially-associated, host-mediated inflammation is a hallmark of periodontitis, consensus was lacking on inflammation being the primary factor linking periodontal diseases and CVDs. The majority of respondents expressed eagerness understand better the relationship between these two diseases, with the intention of enhancing oral health content in medical school and attending relevant seminars.

Intra-aortic Balloon Occlusion for Refractory Cardiac Arrest in a Patient with Anterior Myocardial Infarction.

Despite recent advances in its management, the outcome of cardiac arrest is often poor despite appropriate cardiopulmonary resuscitation. The arteriovenous perfusion gradient achieved dur ing cardiopulmonary resuscitation is associated with the successful return of spontaneous cir culation. Continuous balloon occlusion of the descending aorta is an experimental method that can occlude the "unnecessary" part of the circulation, thus diverting generated pressure and blood flow to the heart and brain. In this study, we present a case report of a patient unre sponsive to standard cardiopulmonary resuscitation, in whom constant intra-aortic balloon occlusion achieved a return of spontaneous circulation and successful survival.

The Effects of Iron Replacement on Functional Capacity in Patients with Group 1 and Group 4 Pulmonary Hypertension.

OBJECTIVE: Abnormal iron handling complicates pulmonary hypertension (PH), causes functional limitation and poor outcomes. Although preliminary results in group 1 PH patients support the use of iron replacement, whether this applies to other PH subgroups is not known. METHODS: A total of 58 patients with an established diagnosis of group 1 or 4 PH, who had a serum ferritin of <100 ng/mL or 100 to 300 ng/mL in combination with a transferrin saturation (TSAT) <20% and received 500 to 1000 mg of ferric carboxymaltose (FCM) were included in the study. The change in ferritin levels and TSAT were calculated at 12- and 24-weeks follow-up. A six-minute walk test (6MWT) is undertaken at the first, 12-week and 24-week follow-up visits. RESULTS: In group 1 PH patients, ferritin levels increased from 14 ng/mL-1 to 133 and 90 ng/mL-1 at 12- and 24-weeks, respectively ( P < .001 for both). In group 4 PH patients, ferritin levels increased from 22.1 ng/mL-1 to 145 and 88.9 ng/mL-1 at 12- and 24-weeks, respectively ( P < .001 for both). 6MWT distances were 356, 412, and 350 m in group 1 PH patients and 260, 315 and 290 m in group 4 PH patients. Although the difference between baseline and 12-week 6MWT was significant in both groups ( P < .001 for both), this difference was lost at 24-week. CONCLUSION: Our study indicates that there is no difference in response to iron replacement in patients with group 1 and group 4 PH patients, in terms of treatment success and functional status.

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.

Characteristics, management, and outcomes of patients with left-sided infective endocarditis complicated by heart failure: a substudy of the ESC-EORP EURO-ENDO (European infective endocarditis) registry.

AIMS: To evaluate the current management and survival of patients with left-sided infective endocarditis (IE) complicated by congestive heart failure (CHF) in the ESC-EORP European Endocarditis (EURO-ENDO) registry. METHODS AND RESULTS: Among the 3116 patients enrolled in this prospective registry, 2449 (mean age: 60 years, 69% male) with left-sided (native or prosthetic) IE were included in this study. Patients with CHF (n = 698, 28.5%) were older, with more comorbidity and more severe valvular damage (mitro-aortic involvement, vegetations >10 mm and severe regurgitation/new prosthesis dehiscence) than those without CHF (all p ≤ 0.019). Patients with CHF experienced higher 30-day and 1-year mortality than those without (20.5% vs. 9.0% and 36.1% vs. 19.3%, respectively) and CHF remained strongly associated with 30-day (odds ratio[OR] 2.37, 95% confidence interval [CI] [1.73-3.24; p < 0.001) and 1-year mortality (hazard ratio [HR] 1.69, 95% CI 1.39-2.05; p < 0.001) after adjustment for established outcome predictors, including early surgery, or after propensity matching for age, sex, and comorbidity (n = 618 [88.5%] for each group, both p < 0.001). Early surgery, performed on 49% of these patients with IE complicated by CHF, remained associated with a substantial reduction in 30-day mortality following multivariable analysis, after adjustment for age, sex, Charlson comorbidity index, cerebrovascular accident, Staphylococcus aureus IE, streptococcal IE, uncontrolled infection, vegetation size >10 mm, severe valvular regurgitation and/or new prosthetic dehiscence, perivalvular complication, and prosthetic IE (OR 0.22, 95% CI 0.12-0.38; p < 0.001) and in 1-year mortality (HR 0.29, 95% CI 0.20-0.41; p < 0.001). CONCLUSION: Congestive heart failure is common in left-sided IE and is associated with older age, greater comorbidity, more advanced lesions, and markedly higher 30-day and 1-year mortality. Early surgery is strongly associated with lower mortality but is performed on only approximately half of patients with CHF, mainly because of a surgical risk considered prohibitive.

Surgery and outcome of infective endocarditis in octogenarians: prospective data from the ESC EORP EURO-ENDO registry.

PURPOSE: High mortality and a limited performance of valvular surgery are typical features of infective endocarditis (IE) in octogenarians, even though surgical treatment is a major determinant of a successful outcome in IE. METHODS: Data from the prospective multicentre ESC EORP EURO-ENDO registry were used to assess the prognostic role of valvular surgery depending on age. RESULTS: As compared to < 80 yo patients, ≥ 80 yo had lower rates of theoretical indication for valvular surgery (49.1% vs. 60.3%, p < 0.001), of surgery performed (37.0% vs. 75.5%, p < 0.001), and a higher in-hospital (25.9% vs. 15.8%, p < 0.001) and 1-year mortality (41.3% vs. 22.2%, p < 0.001). By multivariable analysis, age per se was not predictive of 1-year mortality, but lack of surgical procedures when indicated was strongly predictive (HR 2.98 [2.43-3.66]). By propensity analysis, 304 ≥ 80 yo were matched to 608 < 80 yo patients. Propensity analysis confirmed the lower rate of indication for valvular surgery (51.3% vs. 57.2%, p = 0.031) and of surgery performed (35.3% vs. 68.4%, p < 0.0001) in ≥ 80 yo. Overall mortality remained higher in ≥ 80 yo (in-hospital: HR 1.50[1.06-2.13], p = 0.0210; 1-yr: HR 1.58[1.21-2.05], p = 0.0006), but was not different from that of < 80 yo among those who had surgery (in-hospital: 19.7% vs. 20.0%, p = 0.4236; 1-year: 27.3% vs. 25.5%, p = 0.7176). CONCLUSION: Although mortality rates are consistently higher in ≥ 80 yo patients than in < 80 yo patients in the general population, mortality of surgery in ≥ 80 yo is similar to < 80 yo after matching patients. These results confirm the importance of a better recognition of surgical indication and of an increased performance of surgery in ≥ 80 yo patients.