Rapid deployment aortic valve implantation in complex patients with infective endocarditis or aortic valve insufficiency.

BACKGROUND: New prosthetic valves and surgical approaches that shorten operation time and improve the outcome of patients with aortic valve (AV) infective endocarditis (IE) and AV insufficiency (AVI) are crucial. The aim of this study was to evaluate the outcome of patients with AV IE or AVI treated with the EDWARDS INTUITY Rapid-Deployment AV prosthesis for this off-label indication. METHODS: This single-centre retrospective study analyzed data from patients who underwent AV replacement with the EDWARDS INTUITY Rapid-Deployment AV prosthesis for AV IE or regurgitation. (n = 8 for IE and n = 6 for AVI). RESULTS: Heart-lung machine times were significantly shorter in the AVI group (111.3 ± 20.7 min) compared to the IE group (171.9 ± 52.4 min) (p = 0.02). Aortic cross-clamp followed a similar trend (73.7 ± 9.9 min for AVI vs. 113.4 ± 35.6 min for IE) (p = 0.02). The length of ICU stay was also shorter in the AVI group (3.8 ± 2.6 days) compared to the IE group (16.9 ± 8.9 days) (p = 0.005). Postoperative echocardiography revealed no paravalvular leakage or significant valvular dysfunction in any patient. One patient died postoperatively from aspiration pneumonia. CONCLUSION: The INTUITY valve demonstrates as a safe option for complex AV IE and AVI surgery. Further prospective studies with larger patient cohorts are necessary to confirm these findings and explore the long-term benefits of this approach.

Hormonal changes in the first 24 postoperative hours after cardiac surgical procedures.

Background: Hormone level changes after heart surgeries are a widely observed phenomenon due to neurohormonal feedback mechanisms that may affect postoperative morbidity and mortality. The current study aimed to analyze the changes in thyroid and sex hormones in the first 24 postoperative hours after heart surgery. Methods: This prospective, observational study (registered on ClinicalTrials.gov: NCT03736499; 09/11/2018) included 49 patients who underwent elective cardiac surgical procedures at a tertiary heart center between March 2019 and December 2019. Thyroid hormones, including thyroid-stimulating hormone (TSH), triiodothyronine (T3), and thyroxine (T4), and sex hormones, including prolactin (PRL) and total testosterone, were measured preoperatively and at 24 h postoperatively. Results: Significant decreases in serum TSH (P < 0.001), T3 (P < 0.001) and total testosterone (P < 0.001) levels were noted, whereas T4 (P = 0.554) and PRL (P = 0.616) did not significantly change. Intensive care unit (ICU) hours (P < 0.001), mechanical ventilation (P < 0.001) and Vasoactive-Inotropic Score (VIS) (P = 0.006) were associated with postoperative T3 level. ICU hours were associated with postoperative T4 level (P = 0.028). Postoperative and delta testosterone levels were in connection with lengths of stay in ICU (P = 0.032, P = 0.010 respectively). Model for End-Stage Liver Disease (MELD) scores were associated with thyroid hormone levels and serum testosterone. Conclusions: T3 may represent a marker of nonthyroidal illness syndrome and testosterone may reflect hepatic dysfunction. In addition, PRL may act as a stress hormone in female patients.

Comprehensive frailty assessment with multidimensional frailty domains as a predictor of mortality among vascular and cardiac surgical patients.

Purpose: The frailty concept has become a fundamental part of daily clinical practice. In this study our purpose was to create a risk estimation method with a comprehensive aspect of patients' preoperative frailty. Patients and methods: In our prospective, observational study, patients were enrolled between September 2014 and August 2017 in the Department of Cardiac Surgery and Department of Vascular Surgery at Semmelweis University, Budapest, Hungary. A comprehensive frailty score was built from four main domains: biological, functional-nutritional, cognitive-psychological and sociological. Each domain contained numerous indicators. In addition, the EUROSCORE for cardiac patients and the Vascular POSSUM for vascular patients were calculated and adjusted for mortality. Results: Data from 228 participants were included for statistical analysis. A total of 161 patients underwent vascular surgery, and 67 underwent cardiac surgery. The preoperatively estimated mortality was not significantly different (median: 2.700, IQR (interquartile range): 2.000-4.900 vs. 3.000, IQR: 1.140-6.000, P = 0.266). The comprehensive frailty index was significantly different (0.400 (0.358-0.467) vs. 0.348 (0.303-0.460), P = 0.001). In deceased patients had elevated comprehensive frailty index (0.371 (0.316-0.445) vs. 0.423 (0.365-0.500), P < 0.001). In the multivariate Cox model an increased risk for mortality in quartiles 2, 3 and 4 compared with quartile 1 as a reference was found (AHR (95% CI): 1.974 (0.982-3.969), 2.306 (1.155-4.603), and 3.058 (1.556-6.010), respectively). Conclusion: The comprehensive frailty index developed in this study could be an important predictor of long-term mortality after vascular or cardiac surgery. Accurate frailty estimation could make the traditional risk scoring systems more accurate and reliable.

Incidental findings on routine preoperative noncontrast chest computed tomography and chest radiography prior to cardiac surgery in the multicenter randomized controlled CRICKET study.

OBJECTIVE: To describe the prevalence and consequences of incidental findings when implementing routine noncontrast CT prior to cardiac surgery. METHODS: In the multicenter randomized controlled CRICKET study, 862 adult patients scheduled for cardiac surgery were randomized 1:1 to undergo standard of care (SoC), which included a chest-radiograph, or an additional preoperative noncontrast chest CT-scan (SoC+CT). In this subanalysis, all incidental findings detected on the chest radiograph and CT-scan were analyzed. The influence of smoking status on incidental findings was also evaluated, adjusting for sex, age, and group allocation. RESULTS: Incidental findings were observed in 11.4% (n = 49) of patients in the SoC+CT group and in 3.7% (n = 16) of patients in the SoC-group (p < 0.001). The largest difference was observed in findings requiring follow-up (SoC+CT 7.7% (n = 33) vs SoC 2.3% (n = 10), p < 0.001). Clinically relevant findings changing the surgical approach or requiring specific treatment were observed in 10 patients (1.2%, SoC+CT: 1.6% SoC: 0.7%), including lung cancer in 0.5% of patients (n = 4) and aortic dilatation requiring replacement in 0.2% of patients (n = 2). Incidental findings were more frequent in patients who stopped smoking (OR 1.91, 1.03-3.63) or who actively smoked (OR 3.91, 1.85-8.23). CONCLUSIONS: Routine CT-screening increases the rate of incidental findings, mainly by identifying more pulmonary findings requiring follow-up. Incidental findings are more prevalent in patients with a history of smoking, and preoperative CT might increase the yield of identifying lung cancer in these patients. Incidental findings, but not specifically the use of routine CT, are associated with delay of surgery. KEY POINTS: • Clinically relevant incidental findings are identified more often after a routine preoperative CT-scan, when compared to a standard of care workup, with some findings changing patient management. • Patients with a history of smoking have a higher rate of incidental findings and a lung cancer rate comparable to that of lung cancer screening trials. • We observed no clear delay in the time to surgery when adding routine CT screening.

HTAD patient pathway: Strategy for diagnostic work-up of patients and families with (suspected) heritable thoracic aortic diseases (HTAD). A statement from the HTAD working group of VASCERN.

Heritable thoracic aortic diseases (HTAD) are rare pathologies associated with thoracic aortic aneurysms and dissection, which can be syndromic or non-syndromic. They may result from genetic defects. Associated genes identified to date are classified into those encoding components of the (a) extracellular matrix (b) TGFß pathway and (c) smooth muscle contractile mechanism. Timely diagnosis allows for prompt aortic surveillance and prophylactic surgery, hence improving life expectancy and reducing maternal complications as well as providing reassurance to family members when a diagnosis is ruled out. This document is an expert opinion reflecting strategies put forward by medical experts and patient representatives involved in the HTAD Rare Disease Working Group of VASCERN. It aims to provide a patient pathway that improves patient care by diminishing time to diagnosis, facilitating the establishment of a correct diagnosis using molecular genetics when possible, excluding the diagnosis in unaffected persons through appropriate family screening and avoiding overuse of resources. It is being recommended that patients are referred to an expert centre for further evaluation if they meet at least one of the following criteria: (1) thoracic aortic dissection (<70 years if hypertensive; all ages if non-hypertensive), (2) thoracic aortic aneurysm (all adults with Z score >3.5 or 2.5-3.5 if non-hypertensive or hypertensive and <60 years; all children with Z score >3), (3) family history of HTAD with/without a pathogenic variant in a gene linked to HTAD, (4) ectopia lentis without other obvious explanation and (5) a systemic score of >5 in adults and >3 in children. Aortic imaging primarily relies on transthoracic echocardiography with magnetic resonance imaging or computed tomography as needed. Genetic testing should be considered in those with a high suspicion of underlying genetic aortopathy. Though panels vary among centers, for patients with thoracic aortic aneurysm or dissection or systemic features these should include genes with a definitive or strong association to HTAD. Genetic cascade screening and serial aortic imaging should be considered for family screening and follow-up. In conclusion, the implementation of these strategies should help standardise the diagnostic work-up and follow-up of patients with suspected HTAD and the screening of their relatives.

Association between Hepatic Venous Congestion and Adverse Outcomes after Cardiac Surgery.

INTRODUCTION: Hepatic venous flow patterns reflect pressure changes in the right ventricle and are also markers of systemic venous congestion. Fluid management is crucial in patients undergoing cardiac surgery. METHODS: Our goal was to determine which factors are associated with the increased congestion of the liver as measured by Doppler ultrasound in patients undergoing cardiac surgery. This prospective, observational study included 41 patients without preexisting liver disease who underwent cardiac surgery between 1 January 2021 and 30 September 2021 at a tertiary heart center. In addition to routine echocardiographic examination, we recorded the maximal velocity and velocity time integral (VTI) of the standard four waves seen in the common hepatic vein (flow profile) using Doppler ultrasound preoperatively and at the 20-24th hour of the postoperative period. The ratios of the retrograde and anterograde hepatic venous waves were calculated, and the waveforms were compared to the baseline value and expressed as a delta ratio. Demographic data, pre- and postoperative echocardiographic parameters, intraoperative variables (procedure, cardiopulmonary bypass time), postoperative factors (fluid balance, vasoactive medication requirement, ventilation time and parameters) and perioperative laboratory parameters (liver and kidney function tests, albumin) were used in the analysis. RESULTS: Of the 41 patients, 20 (48.7%) were males, and the median age of the patients was 65.9 years (IQR: 59.8-69.9 years). Retrograde VTI growth showed a correlation with positive fluid balance (0.89 (95% CI 0.785-0.995) c-index. After comparing the postoperative echocardiographic parameters of the two subgroups, right ventricular and atrial diameters were significantly greater in the "retrograde VTI growth" group. The ejection fraction and decrement in ejection fraction to preoperative parameters were significantly different between the two groups. (p = 0.001 and 0.003). Ventilation times were longer in the retrograde VTI group. The postoperative vs. baseline delta VTI ratio of the hepatic vein correlated with positive fluid balance, maximum central venous pressure, and ejection fraction. (B = -0.099, 95% CI = -0.022-0.002, p = 0.022, B = 0.011, 95% CI = 0.001-0.021, p = 0.022, B = 0.091, 95% CI = 0.052-0.213, p = 0.002, respectively.) Conclusion: The increase of the retrograde hepatic flow during the first 24 h following cardiac surgery was associated with positive fluid balance and the decrease of the right ventricular function. Measurement of venous congestion or venous abdominal insufficiency seems to be a useful tool in guiding fluid therapy and hemodynamic management.

Case report: angiosarcoma of the pulmonary artery diagnosed as pulmonary artery emboli.

BACKGROUND: Angiosarcomas are the most common malignant tumors of the heart and great vessels. Late onset and unspecific symptoms are reasons why a diagnosis is made rather late at a time when most tumors have already metastasized. We report a rare case of an angiosarcoma presenting as pulmonary artery emboli. CASE PRESENTATION: A 66-year-old patient was initially admitted to the hospital with a STEMI. Days later a successful reanimation due to ventricular fibrillation followed. An emboli of the pulmonary artery was diagnosed as the cause and after a lysis therapy the patient was discharged. A few weeks later the patient suffered an episode of absolute arrhythmia. TTE as well as CT-Scan showed an emboli of the pulmonary artery and a pericardial effusion with compression to the right ventricle. Intraoperative findings, showed an infiltrating tumor of the pulmonary artery, the pulmonary valve, the RVOT, the LA and LV. A resection of the tumor from the pulmonary artery, valve and RVOT was carried out. A new pulmonary valve was implanted with the reconstruction of the RVOT and pulmonary artery. Due to LV infiltration, only a palliative surgical approach was possible. Despite an uncomplicated postoperative course, the patient died at home two months later. CONCLUSION: Although a rarity, a tumor of the pulmonary artery should be taken into consideration as a differential diagnosis to pulmonary artery emboli. Development of better diagnostic tools (specific tumor markers) and more effective chemotherapeutic agents is necessary to improve the prognosis of these patients.

Long-Term Outcomes Stratified by Age in Patients with a Mechanical versus Biological Mitral Valve Replacement.

Objectives: Balancing anticoagulation and reoperation risks determines prostheses choice (mechanical/biological) for mitral valve replacement. We aimed to re-evaluate the outcomes after biological versus mechanical mitral valve replacement. Methods: We compared long-term benefits and risks of mechanical and biological prostheses in 2056 patients (52% men, 48% women; 65.4 ± 12.1 years) who underwent mitral valve replacements between 1993−2017, in a retrospective single-centre study. Data sources included prospective institutional database, social registry, general practitioner data and follow-up questionnaire. Patients were stratified by age: < = 39 y (n = 82), 40−49 y (n = 164), 50−59 y (n = 335), 60−69 y (n = 593), 70−79 y (n = 743) and > = 80 y (n = 139). Long-term outcomes (mortality, reoperations, bleeding) were analysed. Results: Altogether, 1308 mechanical (53% men, 47% women; 61.5 ± 11.7 years) and 748 biological (50% men, 50% women; 72.3 ± 9.6 years) valves were implanted. The reason for valve replacement was stenosis in 162, insufficiency in 823 and combined in 323 cases for mechanical, while it was 46, 567 and 135 for biological valves, respectively. Overall cumulative survival was higher with mechanical prosthesis (mean: 139 ± 4 vs. 102 ± 5 months, 10 y: 55% vs. 33%, p < 0.0001). Subgroup analysis revealed higher survival among patients receiving mechanical prosthesis up to 60 years (< = 39 y p = 0.047, 40−49 y p < 0.0001, 50−59 y p = 0.001). In patients 60−69 years, overall survival did not differ; however, in survivors beyond 8 years, mechanical prosthesis showed improved survival (p = 0.014). While between 70−79 years survival was nearly identical, for above 80 years, patients had a higher survival with biological prosthesis (p = 0.014). Conclusion: The present data demonstrated a higher survival of mechanical prosthesis in a wide range of patients after mitral valve replacement.

Preventive Impella® Support in High-Risk Patients Undergoing Cardiac Surgery.

BACKGROUND: Patients with severely reduced LV-EF ≤ 30% undergoing CABG have a high risk for postoperative cardiogenic shock. The optimal timing of an adequate hemodynamic support has an impact on short- and midterm mortality after CABG. This study aimed to assess the prophylactic use of the Impella pump in high-risk patients undergoing elective cardiac surgery. METHODS: In this single-center retrospective study, 14 patients with LV-EF (≤30%) undergoing cardiac surgery received a prophylactic, perioperative Impella (5.0, 5.5) support between 2020 and 2022. RESULTS: The mean age at surgery was 64.2 ± 2.6 years, the mean preoperative LV-EF was 20.7% ± 1.56%. The duration of Impella support was 4 (3-7.8) days and the 30-day survival rate was 92.85%. Acute renal failure occurred in four patients who were dialyzed on average for 1.2 ± 0.7 days. Mechanical ventilation was needed for 1.75 (0.9-2.7) days. Time to inotrope/vasopressor independence was 2 (0.97-7.25) days with a highest lactate level (24 h postoperatively) of 3.8 ± 0.6 mmol/l. Postoperative LV-EF showed a significant improvement when compared to preoperative LV-EF (29.1% ± 2.6% vs. 20.7% ± 1.56% (p = 0.022)). CONCLUSION: The prophylactic Impella application seems to be a safe approach to improve the outcomes of this patient population.

Poor Preoperative Nutritional Status, but Not Hormone Levels, Are Associated With Mortality After Cardiac Surgery.

OBJECTIVES: The authors' aim was to examine the preoperative hormone and nutritional status in patients undergoing elective cardiac surgery. DESIGN AND SETTINGS: The authors' research was a single-center, prospective, observational study (ClinicalTrials.gov: NCT03736499). PARTICIPANTS & INTERVENTIONS: The authors examined 252 patients who underwent elective cardiac surgery. Preoperative thyroid-stimulating hormone (TSH), free triiodothyronine (fT3), free thyroxine (fT4), prolactin, and testosterone levels were collected and analyzed after the surgery. The Geriatric Nutritional Risk Index (GNRI), Controlling Nutritional Status (CONUT), and Prognostic Nutritional Index (PNI) were all calculated as a sum and groups. Frailty was calculated based on the modified Frailty Index-11. The primary outcome was overall mortality. MEASUREMENTS AND MAIN RESULTS: The mean age of the patients was 64.23 years (standard deviation: 11.07 years). Thirty-three patients (13.01%) died during the median follow-up time of 20.48 months (interquartile range: 18.90-22.98 months). Thyroid hormones were examined as continuous variables and also in 3 groups based on low, normal, and high hormone levels. Continuous TSH (p = 0.230), continuous fT3 (p = 0.492), and continuous fT4 (p = 0.657) were not significantly associated with total mortality. After adjustment for the European System for Cardiac Operative Risk Evaluation II and postoperative complications, the following nutritional scores were associated with total mortality: GNRI < 91 (adjusted hazard ratio [AHR]: 4.384; 95% confidence interval [CI]: 1.866-10.303, p = 0.001), the higher CONUT group (AHR: 1.736; 95% CI: 1.736-2.866, p = 0.031), and a PNI < 48 points (AHR: 3.465; 95% CI: 1.735-6.918, p < 0.001). The modified Frailty Index-11 was not associated with mortality. CONCLUSIONS: Before cardiac surgery, nutritional status should be assessed because the findings may help to decrease mortality. The hormone levels were not associated with mortality.

Perioperative Factor Concentrate Use is Associated With More Beneficial Outcomes and Reduced Complication Rates Compared With a Pure Blood Product-Based Strategy in Patients Undergoing Elective Cardiac Surgery: A Propensity Score-Matched Cohort Study.

OBJECTIVE: The goal of this study was to compare factor concentrate (FC)-based and blood product-based hemostasis management of coagulopathy in cardiac surgical patients in terms of postoperative bleeding, required blood products, and outcome. DESIGN: Retrospective, propensity score-matched analysis. SETTING: Single, tertiary, academic medical center. PARTICIPANTS: One hundred eighteen matched pairs of 433 consecutive patients scheduled for cardiac surgery in two isolated periods with distinct strategies of hemostasis management. INTERVENTIONS: Patients received either blood product-based (period I) or FC-based (period II) hemostasis management to treat perioperative coagulopathy. MEASUREMENTS AND MAIN RESULTS: Patients treated with FC management experienced less postoperative blood loss (907 v 1,153 mL, p = 0.014) and required less red blood cell and fresh frozen plasma transfusion (2.3 v 3.7 units p < 0.0001, and 2.0 v 3.4 units p < 0.0001, respectively) compared with subjects in the blood product-based management group. The frequency of Stage 3 acute kidney injury and 30-day mortality rate were significantly higher in the blood product-based group than in the FC management group (6.8% v 0.8%, p = 0.016, and 7.2% v 0.8%, p = 0.022, respectively). FC management-related thromboembolic events were not registered. The FC strategy was associated with a 2.19-fold decrease in the odds of massive postoperative bleeding (p < 0.0001), a 2.56-fold decrease in the odds of polytransfusion (p < 0.0001), and a 13.16-fold decrease in the odds of early postoperative death (p = 0.003). CONCLUSIONS: FC-based versus blood product-based management is associated with reduced blood product needs and fewer complications, and was not linked to a higher frequency of thromboembolic events or a decrease in long-term survival in cardiac surgical patients developing perioperative coagulopathy and bleeding.

Blood-filled cyst of the tricuspid valve: Multiple cardiac disorders, one surgical case.

BACKGROUND: Intracardiac blood cysts are an exceedingly rare occurrence in adulthood. Detailed imaging modalities aid in the diagnosis of such incidental lesions and procedure planning. METHODS: We report the case of a 51-year-old male accusing dyspnea on exertion as a sole symptom which led to the discovery of multiple cardiac anomalies, namely, severe aortic valve insufficiency on a bicuspid aortic valve, ascending aortic aneurysm, a cystic mass on the tricuspid valve, patent foramen ovale, and an occluded right coronary artery. RESULTS: The disorders were managed in a single surgical intervention, the resected mass being confirmed as a blood cyst. CONCLUSIONS: Our case presents a unique association of cardiac disorders, including a highly uncommon intracardiac blood-filled cyst, and underlines the importance of multimodality imaging and interdisciplinary approach in the successful management of such complex cases.

Case Report: Morphological Characterization and Long-Term Observation of Bilateral Sequential Internal Mammary Artery Aneurysms in a Patient With Confirmed FBN1 Mutation.

Marfan syndrome (MFS) is a genetically determined connective tissue disorder that leads to ocular, skeletal, and severe cardiovascular involvement. High mortality of MFS is associated with aortic dissection and aneurysm characteristic to the syndrome. In MFS, only a few cases of peripheral arterial involvement have been reported so far, mostly without a genetically confirmed diagnosis. We report a 41-year-old MFS patient with a saccular pearl-string-like aneurysm on the right internal mammary artery (RIMA) and a single aneurysm on the left internal mammary artery (LIMA). To our knowledge this is the first reported case on internal mammary artery aneurysms with this special morphology and with follow-up and blood pressure control as primary therapeutic approach in a patient with genetically confirmed MFS. The aneurysms with the above described morphology first appeared as small aneurysms on a CT scan 6 years after a cardiac operation. Due to the lack of guidelines, based on the asymptomatic state of the patient, the increased tortuosity of the affected vessels and the history of prior cardiac surgery, we decided to closely monitor these aneurysms with blood pressure control and without carrying out any interventions. On the CT scans done 3, 11, 12, 17, and 32 months after identifying the aneurysms, no progression of these structures was detected. Our findings confirm the possibility of the occurrence of internal mammary artery aneurysms in patients with FBN1 mutation and we believe that monitoring these aneurysms with blood pressure management can be a suitable option in selected cases.

Potential predictors of severe cardiovascular involvement in Marfan syndrome: the emphasized role of genotype-phenotype correlations in improving risk stratification-a literature review.

BACKGROUND: Marfan syndrome (MFS) is a genetically determined systemic connective tissue disorder, caused by a mutation in the FBN1 gene. In MFS mainly the cardiovascular, musculoskeletal and ocular systems are affected. The most dangerous manifestation of MFS is aortic dissection, which needs to be prevented by a prophylactic aortic root replacement. MAIN BODY: The indication criteria for the prophylactic procedure is currently based on aortic diameter, however aortic dissections below the threshold defined in the guidelines have been reported, highlighting the need for a more accurate risk stratification system to predict the occurrence of aortic complications. The aim of this review is to present the current knowledge on the possible predictors of severe cardiovascular manifestations in MFS patients, demonstrating the wide range of molecular and radiological differences between people with MFS and healthy individuals, and more importantly between MFS patients with and without advanced aortic manifestations. These differences originating from the underlying common molecular pathological processes can be assessed by laboratory (e.g. genetic testing) and imaging techniques to serve as biomarkers of severe aortic involvement. In this review we paid special attention to the rapidly expanding field of genotype-phenotype correlations for aortic features as by collecting and presenting the ever growing number of correlations, future perspectives for risk stratification can be outlined. CONCLUSIONS: Data on promising biomarkers of severe aortic complications of MFS have been accumulating steadily. However, more unifying studies are required to further evaluate the applicability of the discussed predictors with the aim of improving the risk stratification and therefore the life expectancy and quality of life of MFS patients.

Changing strategies in aortic root reconstruction in Marfan syndrome / Stratégiai szemléletváltás a Marfan-szindrómás betegeken végzett aortagyök-rekonstrukciókban.

Összefoglaló. Bevezetés: A Marfan-szindróma autoszomális domináns módon öröklodo, szisztémás kötoszöveti betegség. A hosszú távú túlélés szempontjából fontos a nagyérkatasztrófák megelozése. Szívsebészeti szempontból a legfontosabb elváltozás az aortagyök tágulata. Aortagyök-rekonstrukciós beavatkozásaink Bentall-DeBono-, David I. és módosított Yacoub-mutétek, melyek mind preventív jelleggel, mind dissectio esetén jó eredménnyel végezhetok. Célkituzés: A marfanos betegeknél eltéro technikával végzett aortagyök-rekonstrukciós mutéteink összehasonlítása. Módszer: A Semmelweis Egyetem Városmajori Szív- és Érgyógyászati Klinikáján 1993 és 2020 között Marfan-szindrómásoknál elvégzett Bentall-DeBono-, David I. és módosított Yacoub-féle aortagyök-rekonstrukciókat elemeztük. A mutét szerinti csoportok életkora a beavatkozás idején 29,69 (21,98-41,25) év, 29,15 ± 11,99 év és 35,29 ± 14,14 év volt, a fenti sorrendben. Az adatok forrásául a Magyar Marfan Regiszter és az Aortagyök-rekonstrukciós Regiszter szolgált. Eredmények: Az utánkövetési ido 132 ± 81,04 hónap volt a Bentall-, 76 ± 27,77 hónap a David-, valamint 4,5 (0,75-11,75) hónap a Yacoub-mutét esetén. A David- és a Yacoub-beavatkozások gyakrabban voltak profilaktikusak, mint a Bentall-operációk (p = 0,0153; p = 0,0085). A Bentall-mutéteknél ritkább volt a primer mutét esetleges késobbi elégtelenségébol fakadó reoperáció, mint a David-operációknál (p<0,001). David-beavatkozásnál a Bentall-mutéthez képest hosszabb volt a cardiopulmonaris bypass (p = 0,0013) és az aortalefogás ideje (p = 0,0048), valamint David- és Yacoub-mutét esetén gyakrabban lépett fel korai posztoperatív szövodmény, mint Bentall-operációnál (p = 0,0005; p = 0,0037). A késoi szövodmények és a túlélés tekintetében a csoportok nem különböztek. Következtetés: Marfan-szindrómában a leggyakrabban halált okozó szövodmény az akut aortaruptura, illetve akut aortadissectio. Eredményeink alapján mindhárom profilaktikus aortagyök-rekonstrukciós mutéti típus jól reprodukálható és jó eredménnyel végezheto Marfan-szindrómában. Orv Hetil. 2021; 162(18): 696-704. INTRODUCTION: Marfan syndrome is an autosomal dominant, systemic connective tissue disorder. Preventing vascular complications is essential for long-term survival. Aortic dilation is the main cardiac surgical manifestation. Bentall-DeBono, David I and modified Yacoub aortic root reconstructions treat and prevent aortic dissections with great outcomes. OBJECTIVE: Comparing results of aortic root reconstructions in Marfan syndrome. METHOD: We analysed the data of Bentall-DeBono, David I and modified Yacoub operations performed in Marfan syndrome at the Heart and Vascular Center, Semmelweis University between 1993 and 2020. Ages of surgical groups at the time of operation were 29.69 (21.98-41.25) years, 29.15 ± 11.99 years and 35.29 ± 14.14 years, respectively. Data were obtained from the Hungarian Marfan Register and the Aortic Root Reconstruction Register. RESULTS: Follow-up time was 132 ± 81.04 months for Bentall, 76 ± 27.77 months for David and 4.5 (0.75-11.75) months for Yacoub groups. David and Yacoub operations were prophylactic more frequently than Bentall ones (p = 0.0153; p = 0.0085). Freedom from reoperation after primary surgery insufficiency was more common for Bentall than for David procedure (p<0.001). Compared to Bentall, David surgeries required longer cardiopulmonary bypass (p = 0.0013) and aortic cross clamp time (p = 0.0048), more early postoperative complications occurred after David and Yacoub, than after Bentall operations (p = 0.0005; p = 0.0037). Late complications and survival did not differ among the groups. CONCLUSION: In Marfan syndrome, acute aortic rupture and dissection are the main contributors to mortality. Based on our results, the prophylactic aortic root reconstructions are reproducible and can be performed with great outcomes. Orv Hetil. 2021; 162(18): 696-704.

Behçet's disease: successful aortic root reconstruction in severely dilated aortoventricular junction after aortic valve replacement with novel surgical method - case report.

BACKGROUND: Behçet's disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet's disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. CASE PRESENTATION: Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special "skirted" conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. CONCLUSIONS: The surgical treatment of cardiovascular manifestations of Behçet's disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.

Needle in the heart: a rare case of cardiac tamponade caused by a migrated foreign body and mimicking ST segment elevation myocardial infarction.

BACKGROUND: Pericardial tamponade is a serious condition which may eventually lead to severe haemodynamic disturbances and cardiac arrest. It is most often caused by the accumulation of fluid inside the pericardium, as a result of different aetiological factors such as pericarditis, neoplastic diseases, lymphatic dysfunctions, or idiopathic pericardial disease. Pericardial tamponade can develop after cardiac surgical procedures or as a complication of myocardial infarction. Collection of blood inside the pericardial sack can be the result of pericardial or cardiac trauma. It is exceedingly rare for the injury to be caused by a migrating foreign body. Although a typical picture of pericardial tamponade has been previously described, the disorder may clinically resemble an acute myocardial infarction. CASE PRESENTATION: We report the case of a 58-year-old female patient complaining of new onset thoracic pain and shortness of breath. Electrocardiographic examination results were suggestive of an acute inferior myocardial infarction. However, echocardiography revealed significant pericardial tamponade. The cause was found to be a needle which remained inside the pelvis following a previous cesarean delivery, which the patient had undergone 18 years prior. In emergency setting, the needle was removed and the pericardial tamponade was resolved. Due to the prompt and efficient management, the patient had an uneventful postoperative recovery and presented no recurrence at the follow-up examinations. CONCLUSIONS: The migration of foreign bodies through tissues is exceedingly rare. If present, it may cause life-threatening complications. Since the aetiology of pericardial tamponade is vast, a thorough assessment is highly important. Therefore, echocardiography is the imaging modality of choice. We wish to highlight the possibility of migrating foreign bodies as probable cause for pericardial tamponade, as well as the importance of echocardiographic methods in the fast-track evaluation of such critical conditions.

Methane supplementation improves graft function in experimental heart transplantation.

BACKGROUND: Maintenance of cell viability during cold storage is a key issue in organ transplantation. Methane (CH4) bioactivity has recently been recognized in ischemia/reperfusion conditions; we therefore hypothesized that cold storage in CH4-enriched preservation solution can provide an increased defense against organ dysfunction during experimental heart transplantation (HTX). METHODS: The hearts of donor Lewis rats were stored for 60 minutes in cold histidine-tryptophan-ketoglutarate (Custodiol [CS]) or CH4-saturated CS solution (CS-CH4) (n = 12 each). Standard heterotopic HTX was performed, and 60 minutes later, the left ventricular (LV) pressure-volume relationships LV systolic pressure (LVSP), systolic pressure increment (dP/dtmax), diastolic pressure decrement, and coronary blood flow (CBF) were measured. Tissue samples were taken to detect proinflammatory parameters, structural damage (by light microscopy), endoplasmic reticulum (ER) stress, and apoptosis markers (CCAAT/enhancer binding protein [C/EBP] homologous protein, GRP78, glycogen synthase kinase-3ß, very low-density lipoprotein receptor, caspase 3 and 9, B-cell lymphoma 2, and bcl-2-like protein 4), whereas mitochondrial functional changes were analyzed by high-resolution respirometry. RESULTS: LVSP and dP/dtmax increased significantly at the largest pre-load volumes in CS-CH4 grafts as compared with the CS group (114.5 ± 16.6 mm Hg vs 82.8 ± 4.6 mm Hg and 3,133 ± 430 mm Hg/s vs 1,739 ± 169 mm Hg/s, respectively); the diastolic function and CBF (2.4 ± 0.4 ml/min/g vs 1.3 ± 0.3 ml/min/g) also improved. Mitochondrial oxidative phosphorylation capacity was more preserved (58.5 ± 9.4 pmol/s/ml vs 27.7 ± 6.6 pmol/s/ml), and cytochrome c release was reduced in CS-CH4 storage. Signs of HTX-caused myocardial damage, level of ER stress, and the transcription of proapoptotic proteins were significantly lower in CS-CH4 grafts. CONCLUSION: The addition of CH4 during 1 hour of cold storage improved early in vitro graft function and reduced mitochondrial dysfunction and activation of inflammation. Evidence shows that CH4 reduced ER stress-linked proapoptotic signaling.

Optimising the mutation screening strategy in Marfan syndrome and identifying genotypes with more severe aortic involvement.

BACKGROUND: Marfan syndrome (MFS) is a systemic connective tissue disorder with life-threatening manifestations affecting the ascending aorta. MFS is caused by dominant negative (DN) and haploinsufficient (HI) mutations of the FBN1 gene. Our aim was to identify mutations of MFS patients with high detection rate and to investigate the use of a gene panel for patients with Marfanoid habitus. We also aimed to examine correlations between genotype and cardiovascular manifestations to predict "malignant" mutations. METHODS: 136 individuals were enrolled. In the first phase, next-generation sequencing (NGS) and Sanger sequencing were performed for 57 patients to screen the FBN1 gene, followed by multiplex ligation-dependent probe amplification (MLPA) in negative cases. For repeated negative results, NGS gene panel involving 9 genes was used. In the second phase, 79 patients were tested primarily with the same gene panel, negative samples were tested by MLPA. RESULTS: 84 pathogenic mutations were detected, out of which 78 affected FBN1, 6 non-FBN1 mutations (2 TGFB2, 1 TGFBR2, 2 TGFBR1, 1 SMAD3) are associated with Loeys-Dietz syndrome (LDS). LDS patients had lower systemic score and they were younger, but their aortic involvement did not differ. MLPA detected 4 multi-exon deletions of FBN1 gene, which could not be identified by our first-step screening method. Aortic involvement (aortic dissection and/or dilation) did not differ significantly among HI and DN mutations (p = 0.061). Combined group of HI and DN mutations eliminating a disulphide-bonding cysteine (DN Cys) had significantly higher aortic involvement rate than DN mutations not eliminating a disulphide-bonding cysteine (DN non-Cys) (p < 0.001). Patients with DN Cys required significantly more aortic surgeries than HI and DN non-Cys mutations (p = 0.042 and p = 0.015, respectively). CONCLUSIONS: Due to the relevant number of mutations affecting genes other than FBN1, preferred approach for testing individuals with Marfanoid habitus is using a gene panel rather than single-gene analysis, followed by MLPA for negative samples. DN Cys and HI mutations should be considered as risk factors for aortic involvement. Genetic testing for patients with Marfanoid features and a systemic score under 7 is recommended, as LDS patients may have lower scores, but they may have severe cardiovascular manifestations.

Psychological factors affecting Marfan syndrome patients with or without cardiac surgery.

BACKGROUND: Marfan syndrome (MFS) is a systemic connective tissue disorder belonging to a group of rare diseases. Several psychologically distressing factors can challenge life for MFS patients. The aim of the present study was, therefore, to assess the psychological and psychosocial aspects of MFS with the goal of identifying a means of improving disease management for patients. METHODS: A total of 66 adult patients with MFS were enrolled into the study prospectively and were divided into operated (OP) and non-operated (NOP) subgroups. Multiple questionnaire tests were used to determine the mental and physical state of our patients. Demographic and surgical data were collected. The results of the tests were also compared to the Hungarostudy (HS) population (representing the average Hungarian population) by using a propensity-matched control. RESULTS: OP group scores yielded more alcohol consumption (P<0.001), while NOP group showed more sleep disturbances. Scores on the MMSE, BECK, STAI and STAI-T tests showed no significant difference comparing the OP and NOP groups. MFS patients appear to have moderate pain-related disability and mild depressive symptoms and sleep disturbances (P<0.05) compared to the HS group. On 10-point scale, MFS patients were more satisfied with their lives (P<0.001) and considered themselves happier (P<0.001) than the HS population; however, they also spent more days on sick leave and in hospital over the past year. The HS group yielded a higher overall percentage of current smokers and pack-per-year consumption than the MFS patients overall (P=0.003 and P<0.001 respectively). CONCLUSIONS: Marfan patients' psychosocial life differs in many ways (including sleep disturbances, healthier lifestyle, pain-related suffering) from the average Hungarian population. Therefore, as part of a multidisciplinary approach during treatment, modern management of MFS should include psychosocial exploration and psychological support in addition to traditional medical options.