Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair.

Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 ± 9.2 [range: 19-58] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 ± 59.9 ms vs. 133.5 ± 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning-Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 ± 20.7 ms vs. 38.4 ± 18.3 ms; p = 0.019; SDNN: 183.5 ± 58.4 ms vs. 136.3 ± 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning-Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 ± 58.4 ms vs. 84.5 ± 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.

Specific Inhibitors of Mitochondrial Deacylase Sirtuin 4 Endowed with Cellular Activity.

Sirtuins are NAD+-dependent protein lysine deacylases implicated in aging-related diseases. Mammalian Sirtuin 4 (Sirt4) is located in mitochondria and a potential therapeutic target for cancer and metabolic diseases, but no potent and selective Sirt4 inhibitors have been reported. Here, we describe the identification of potent Sirt4-specific small-molecule inhibitors. Testing hits from a target-based virtual screen revealed 12 active compounds. A focused screen based on two top compounds, followed by structure-assisted design of derivatives, yielded four first-in-class potent Sirt4 inhibitors. Kinetic analyses indicate compound competition with the acyl peptide substrate, consistent with the docking models and implicating Sirt4's unique acyl binding site. The compounds indeed show preference for Sirt4 over other isoforms, with one of them (69) being highly isoform selective, and they are active in cells. Our results provide first lead compounds and mechanistic insights for optimization toward Sirt4-specific inhibitors useful as experimental tools and potential therapeutics.

Comparative Study of 2D-Cine and 3D-wh Volumetry: Revealing Systemic Error of 2D-Cine Volumetry.

This study investigates the crucial factors influencing the end-systolic and end-diastolic volumes in MRI volumetry and their direct effects on the derived functional parameters. Through the simultaneous acquisition of 2D-cine and 3D whole-heart slices in end-diastole and end-systole, we present a novel direct comparison of the volumetric measurements from both methods. A prospective study was conducted with 18 healthy participants. Both 2D-cine and 3D whole-heart sequences were obtained. Despite the differences in the creation of 3D volumes and trigger points, the impact on the LV volume was minimal (134.9 mL ± 16.9 mL vs. 136.6 mL ± 16.6 mL, p < 0.01 for end-diastole; 50.6 mL ± 11.0 mL vs. 51.6 mL ± 11.2 mL, p = 0.03 for end-systole). In our healthy patient cohort, a systematic underestimation of the end-systolic volume resulted in a significant overestimation of the SV (5.6 mL ± 2.6 mL, p < 0.01). The functional calculations from the 3D whole-heart method proved to be highly accurate and correlated well with function measurements from the phase-contrast sequences. Our study is the first to demonstrate the superiority of 3D whole-heart volumetry over 2D-cine volumetry and sheds light on the systematic error inherent in 2D-cine measurements.

Rare Case Report: Left Atrial Sarcoma Obstructing the Left Ventricular Inflow.

Malignant cardiac tumors of the heart are extremely rare and may present tremendous diagnostic and therapeutic challenges. These tumors are able to infiltrate the heart and metastasize systemically. Early detection is often elusive as the clinical presentation is highly variable, posing significant diagnostic and therapeutic difficulties. Despite a multidisciplinary approach, the prognosis for patients with malignant cardiac tumors remains guarded. Early diagnosis and a multidisciplinary approach involving cardiac surgeons, oncologists and critical care specialists are crucial in the management of this disease. Further research is needed to better understand the pathomechanisms of tumor-related complications and to develop effective treatment strategies to improve patient outcomes. The rare case of a 78-year-old woman with left atrial tumor requiring emergency surgery for acutely developing mitral valve obstruction is presented. Pathology confirmed an undifferentiated pleomorphic sarcoma. This patient tragically did not survive, highlighting the difficulties of managing such a rare and deceptive heart disease.

Left Atrial Appendage Occlusion on a Beating Heart during Minimally Invasive Valve Surgery Using an Aortic Endoclamp: A Case Report.

Concomitant LAA occlusion has been shown to be an effective and safe treatment for patients with atrial fibrillation during cardiac surgery to prevent embolic stroke. Minimally invasive procedures are challenging due to restricted access to and visibility of the surgical site. Also, aortic endoclamping has been developed as an alternative surgical approach to exoclamping. The aim of this article is to demonstrate the method of beating heart LAA occlusion with the Atriclip® (AtriCure, Mason, OH, USA) device during minimally invasive mitral valve surgery while using the endoclamping alternative for aortic cross-clamping.

A Dual-Pathogen Mitral Valve Endocarditis Caused by Coxiella burnetii and Streptococcus gordonii-Which Came First?

Infective endocarditis (IE) is still a life-threatening disease with high morbidity and mortality. While usually caused by a single bacterium, poly-microbial infective endocarditis (IE) is rare. Here, we report a (blood-culture-negative) dual pathogen mitral valve IE caused by Coxiella burnetii and Streptococcus gordonii: A 53-year-old woman was presented to an internal medicine department with abdominal pain for further evaluation. Within the diagnostic work up, transthoracic echocardiography (TTE) revealed an irregularly shaped echogenic mass (5 × 13 mm) adherent to the edge of the posterior mitral valve leaflet and protruding into the left atrium. As infected endocarditis was suspected, blood cultures were initially obtained, but they remained negative. Chronic Q fever infection was diagnosed using serologic testing. After the occurrence of cerebral thromboembolic events, the patient was admitted for mitral valve surgery. Intraoperatively, a massively destructed mitral valve with adhering vegetations was noted. Examination of the mitral valve by broad-range bacterial polymerase chain reaction (PCR) and amplicon sequencing confirmed Coxiella burnetii infection and yielded Streptococcus gordonii as the second pathogen. Based on the detailed diagnosis, appropriate antibiotic therapy of both pathogens was initiated, and the patient could be discharged uneventfully on the 11th postoperative day after a successful minimal-invasive mitral valve replacement.

Novel 1,4-Dihydropyridines as Specific Binders and Activators of SIRT3 Impair Cell Viability and Clonogenicity and Downregulate Hypoxia-Induced Targets in Cancer Cells.

The mitochondrial SIRT3 modulates several biological pathways such as cancer, metabolism, and hypoxia-related diseases. Recently, we discovered new 1,4-dihydropyridines, compounds 2 and 3, the latter being a SIRT3-specific activator. In the present work, a novel 2- and 3-related small series of compounds have been developed, with 3c displaying the strongest SIRT3 binding and activation, with a KD of 29 µM and 387% of enzyme activation. Differently, 3d was the best in enhancing glutamate dehydrogenase activity and deacetylating K68- and K122-acMnSOD in triple-negative MDA-MB-231 breast cancer cells. Tested in CAL-62 thyroid cancer and MDA-MB-231 cells, 3d displayed the strongest time- and dose-dependent reduction of cell viability and clonogenicity at a single-digit micromolar level, along with cell death, in both normoxia and hypoxia conditions. Moreover, 3d downregulated not only hypoxia-induced factors, such as HIF-1α, EPAS-1, and CA-IX, but also epithelial-mesenchymal transition master regulators and extracellular matrix components such as SNAIL1, ZEB1, SLUG, COL1A2, MMP2, and MMP9, markedly hampering MDA-MB-231 cell migration.

Transmural fibre orientations based on Laplace-Dirichlet-Rule-Based-Methods and their influence on human heart simulations.

It is well known that the orthotropic tissue structure decisively influences the mechanical and electrical properties of the heart. Numerous approaches to compute the orthotropic tissue structure in computational heart models have been developed in the past decades. In this study, we investigate to what extent different Laplace-Dirichlet-Rule-Based-Methods (LDRBMs) influence the local orthotropic tissue structure and thus the electromechanical behaviour of the subsequent cardiac simulation. In detail, we are utilising three Laplace-Dirichlet-Rule-Based-Methods and compare: (i) the local myofibre orientation; (ii) important global characteristics (ejection fraction, peak pressure, apex shortening, myocardial volume reduction, fractional wall thickening); (iii) local characteristics (active fibre stress, fibre strain). We observe that the orthotropic tissue structures for the three LDRBMs show significant differences in the local myofibre orientation. The global characteristics myocardial volume reduction and peak pressure are rather insensitive to a change in local myofibre orientation, while the ejection fraction is moderately influenced by the different LDRBMs. Moreover, the apical shortening and fractional wall thickening exhibit a sensitive behaviour to a change in the local myofibre orientation. The highest sensitivity can be observed for the local characteristics.

Endovascular stenting of an HVAD™ outflow graft pseudoaneurysm that exerts compression and kinking stenosis on the soft portion of the prosthesis.

Key Clinical Message: Complex presentations of MCS patients may necessitate a multidisciplinary approach involving HF cardiologists, CT surgeons, advanced cardiac imagers, and interventional cardiologists in order to define the optimal management strategy. Abstract: Left ventricle assist devices (LVADs) provide life-sustaining treatment for patients with terminal heart failure, but their intricacy allows for complications. One complication is LVAD outflow graft obstruction due to the graft's intraluminal thrombus or extraluminal compression. It may be treated endovascularly with stenting. We report an endovascular stenting of an outflow tract in HVAD™ (HeartWare Inc.) due to a pseudoaneurysm causing compression and kinking stenosis.

Corrigendum: long-term cultivation of human atrial myocardium.

[This corrects the article DOI: 10.3389/fphys.2022.839139.].

MELD-score for risk stratification in cardiac surgery.

The outcome of the patients undergoing cardiac surgery with cardiopulmonary bypass (CPB) is also influenced by the renal and hepatic organ functions. Risk stratification, using scores such as EURO Score II or STS Short-Term Risk Calculator for patients undergoing cardiac surgery with cardiopulmonary bypass, ignores the quantitative renal and hepatic function; therefore, MELD-Score was applied in these cases. We retrospectively examined patient data using the MELD score as a predictor of mortality. To perform a univariate analysis of the data, patients were classified into three groups based on the MELD Score: MELD < 10 (Group 1), MELD 10 to 19 (Group 2), and MELD ≥ 20 (Group 3). A total of 11,477 participants were included in the study, though several patients with either missing MELD scores or lack of creatinine, bilirubin, or INR levels were dropped from the original cohort. Eventually, 10,882 patients were included in the analysis. The primary outcome was defined as postoperative, in-hospital mortality. Secondary outcomes such as postoperative bleeding, including the requirement for repeat thoracotomy, postoperative neurological complications, and assessment of catecholamines on weaning from cardiopulmonary bypass/ requirement of mechanical circulatory support were examined. A higher MELD score was associated with increased postoperative mortality. Patients with MELD > 20 experienced a 31.2% postoperative mortality, compared to Group 1 (4.6%) and Group 2 (17.5%). The highest rates of postoperative bleeding (13.8%) and, repeat thoracotomy (13.2%) & postoperative pneumonia (17.4%) were seen in Group 3 (threefold increase when compared to Group 1, renal failure requiring dialysis (N = 235, 2.7% in Group 1 v/s N = 78, 22.9% in Group 3) or requiring high dose catecholamines post-operatively or mechanical circulatory support (IABP/ECLS). Incidentally, an increased MELD Score was not associated with a significant increase in the postoperative incidence of stroke/TIA or the presence of sternal wound infections/complications. A higher mortality was observed in patients with reduced liver and renal function, with a significant increase in patients with a MELD score > 20. As the current risk stratification scores do not consider this, we recommend applying the MELD score before considering patients for cardiac surgery.

The lost balloon at midnight: a case report reveals the inevitability of heart team existence.

BACKGROUND: Dislodgement of a coronary stent-balloon catheter during percutaneous coronary intervention (PCI) is rare but is a life-threatening complication. A 57- year-old male presented with a non-ST elevation myocardial infarction (NSTEMI). Coronary angiography revealed total thrombotic occlusion of the Right coronary artery (RCA). Following the balloon dilatation of the RCA and while trying to retrieve the balloon catheter, the balloon was dislodged from the catheter shaft and entrapped in the coronary vessel. Under cardiopulmonary bypass, with antegrade cardioplegic arrest, the balloon was extracted through a coronary arteriotomy. Right coronary revascularization was done with reversed saphenous vein graft (SVG). DISCUSSION: Given the variety of equipment that can be retained in the coronary artery and the multitude of mechanisms by which it may be entrapped, there are no straightforward techniques applicable to all situations. Specific guidelines or recommendations on properly managing these potentially life-threatening complications do not exist. However, the most crucial issue in the management of these cases is the hemodynamic status of the patient as well as the coronary flow in the vessel with entrapped device or stent. In our case, the RCA was retrogradely perfused from the left coronary artery, which provided time to transfer the patient to cardiovascular surgical backup.

Molecular Mechanism of Sirtuin 1 Inhibition by Human Immunodeficiency Virus 1 Tat Protein.

Sirtuins are NAD+-dependent protein lysine deacylases implicated in metabolic regulation and aging-related dysfunctions. The nuclear isoform Sirt1 deacetylates histones and transcription factors and contributes, e.g., to brain and immune cell functions. Upon infection by human immunodeficiency virus 1 (HIV1), Sirt1 deacetylates the viral transactivator of transcription (Tat) protein to promote the expression of the viral genome. Tat, in turn, inhibits Sirt1, leading to the T cell hyperactivation associated with HIV infection. Here, we describe the molecular mechanism of Tat-dependent sirtuin inhibition. Using Tat-derived peptides and recombinant Tat protein, we mapped the inhibitory activity to Tat residues 34-59, comprising Tat core and basic regions and including the Sirt1 deacetylation site Lys50. Tat binds to the sirtuin catalytic core and inhibits Sirt1, Sirt2, and Sirt3 with comparable potencies. Biochemical data and crystal structures of sirtuin complexes with Tat peptides reveal that Tat exploits its intrinsically extended basic region for binding to the sirtuin substrate binding cleft through substrate-like ß-strand interactions, supported by charge complementarity. Tat Lys50 is positioned in the sirtuin substrate lysine pocket, although binding and inhibition do not require prior acetylation and rely on subtle differences to the binding of regular substrates. Our results provide mechanistic insights into sirtuin regulation by Tat, improving our understanding of physiological sirtuin regulation and the role of this interaction during HIV1 infection.

Interleukin-3 protects against viral pneumonia in sepsis by enhancing plasmacytoid dendritic cell recruitment into the lungs and T cell priming.

Rationale: Sepsis, a global health burden, is often complicated by viral infections leading to increased long-term morbidity and mortality. Interleukin-3 (IL-3) has been identified as an important mediator amplifying acute inflammation in sepsis; however, its function in the host response to viral infections during sepsis remains elusive. Objectives: To investigate the role of IL-3 during viral pneumonia in sepsis. Methods: We included septic patients from two different cohorts and used in vitro and in vivo assays. The obtained data were substantiated using a second model (SARS-CoV-2 infections). Measurements and main results: Low plasma IL-3 levels were associated with increased herpes simplex virus (HSV) airway infections in septic patients, resulting in reduced overall survival. Likewise, Il-3-deficient septic mice were more susceptible to pulmonary HSV-1 infection and exhibited higher pulmonary inflammation than control mice. Mechanistically, IL-3 increases innate antiviral immunity by promoting the recruitment of circulating plasmacytoid dendritic cells (pDCs) into the airways and by enhancing pDC-mediated T cell activation upon viral stimulation. Interestingly, the ability of IL-3 to improve adaptive immunity was confirmed in patients with SARS-CoV-2 infections. Conclusion: Our study identifies IL-3 as a predictive disease marker for viral reactivation in sepsis and reveals that IL-3 improves antiviral immunity by enhancing the recruitment and the function of pDCs.

Single-Center Retrospective Analysis of Acute Type A Aortic Dissection Outcome and Reoperation Focusing on Extended Versus Limited Initial Repair.

BACKGROUND: The optimal management strategy for acute aortic type A dissection remains controversial. Whether a limited primary (index) repair would increase the need for late aortic reintervention is still an open debate. METHODS: A total of 393 consecutive adult patients with acute type A aortic dissection who underwent cardiac surgery were analyzed. Our research hypothesis was whether limited aortic index repair (i.e., isolated aorta ascending replacement without an open distal anastomosis with and without a concomitant aortic valve replacement, including hemiarch replacement procedure) is associated with a higher incidence of late aortic reoperation compared with extended repair (i.e., any other surgical procedure that goes beyond that limited approach). RESULTS: Type of the initial repair had no statically significant relationship with in-hospital mortality with a P-value of 0.12, however in multivariable analysis, cross-clamp time had a statistically significant relation with mortality (P = 0.4). From the patients who survived until discharge (N = 311), 40 patients needed a reoperation on the aorta; the mean interval until reoperation was 4.5 years. The relationship between the type of the initial repair and the need for reoperation didn't reach a statically significant value (P = 0.9). In-hospitable mortality after the second operation was 10% (N = 4). CONCLUSION: We reached two conclusions. 1) An extended prophylactic repair in the initial operation of an acute type A aortic dissection might not lead to a lower incidence of reoperations on the aorta and could increase in-hospital mortality by increasing cross-clamp time, and 2) Reoperation on the aorta could be done safely with acceptable mortality outcomes.

Pericardial tamponade, a diagnostic chameleon: from the historical perspectives to contemporary management.

BACKGROUND: Pericardial tamponade (PT) early after cardiac surgery is a challenging clinical entity, not infrequently misrecognized and often only detected late in its course. Because the clinical signs of pericardial tamponade can be very unspecific, a high degree of initial suspicion is required to establish the diagnosis. In addition to clinical examination the deployment of imaging techniques is almost always mandatory in order to avoid delays in diagnosis and to initiate any necessary interventions, such as pericardiocentesis or direct cardiac surgical interventions. After a brief overview of how knowledge of PT has developed throughout history, we report on an atypical life-threatening cardiac tamponade after cardiac surgery. A 74-year-old woman was admitted for elective biological aortic valve replacement and aorto-coronary-bypass grafting (left internal mammary artery to left anterior descending artery, single vein graft to right coronary artery). On the 10th postoperative day, the patient unexpectedly deteriorated. She rapidly developed epigastric pain radiating to the left upper abdomen, and features of low peripheral perfusion and shock. There were no clear signs of pericardial tamponade either clinically or echocardiographically. Therefore, for further differential diagnosis, a contrast-enhanced computed tomography scan was performed under clinical suspicion of acute abdomen. Unexpectedly, active bleeding distally from the right coronary anastomosis was revealed. While the patient was prepared for operative revision, she needed cardiopulmonary resuscitation, which was successful. Intraoperatively, the source of bleeding was located and surgically relieved. The subsequent postoperative course was uneventful. CONCLUSIONS: In the first days after cardiac surgery, the occurrence of life-threatening situations, such as cardiac tamponade, must be expected. Especially if the symptoms are atypical, the entire diagnostic armamentarium must be applied to identify the origin of the complaints, which may be cardiac, but also non-cardiac. CENTRAL MESSAGE: A high level of suspicion, immediate diagnostic confirmation, and rapid treatment are required to recognize and successfully treat such an emergency (Fig. 5). PERSPECTIVE: Pericardial tamponade should always be considered as a complication of cardiac surgery, even when symptoms are atypical. The full range of diagnostic tools must be used to identify the origin of the complaints, which may be cardiac, but also non-cardiac (Fig. 5).

Intraoperative visualization of a deformed left main stent during surgical aortic valve replacement.

BACKGROUND: While coronary artery bypass grafting is typically considered first choice for the treatment of left main stenosis, there is a trend towards left main stenting due to a steadily aging population in western countries with a high operative risk and patients with single vessel coronary artery disease affecting the left main artery. Nevertheless left main stenting remains controversial, especially in patients with concomitant indications for open-heart surgery. CASE PRESENTATION: We want to present a case of a 78-year-old male patient with high-grade aortic stenosis who underwent surgical aortic valve replacement at our heart center due to anatomical contraindications for transcatheter aortic valve replacement. Stenting of the left main coronary artery was performed three years earlier due to single vessel coronary artery disease while moderate aortic valve stenosis was under surveillance at the time of the intervention. Intraoperatively we found the stent to be deformed inside the left main coronary artery, covering nearly 25% of the coronary ostium. So injection of cardioplegia directly into this ostium, as we perform normally, was not possible without further damaging the stent and/or the opening of the ostium. We had to insert cardioplegia via the retrograde way, so via the coronary sinus. CONCLUSION: While left main stenting can be reasonable for a specific population of patients, it should be used cautiously in patients with concomitant indications for open-heart surgery in the near future and a low perioperative risk profile.

Wearable cardioverter defibrillator after cardiac surgery: Analysis of real-life data from patients at transient risk of sudden cardiac death.

BACKGROUND: Especially in the first 3 months after cardiac surgery, patients are at transient risk of sudden cardiac death (SCD). To close the gap between hospital discharge and the final implantable cardioverter-defibrillator (ICD) decision, guidelines recommend temporarily using a wearable cardioverter-defibrillator (WCD) to protect these patients from SCD. We investigated real-life data on the safety, effectiveness, and compliance of the WCD in this population. METHODS: Data for analysis were collected via the Zoll Patient Management Network (ZPM) from patients who underwent cardiac surgery and who were discharged with a WCD between 2018 and 2021 at the Cardiac Surgery Center of the University of Erlangen in Germany. RESULTS: The majority of the 55 patients were male (90.9%) and underwent a coronary artery bypass graft (80.0%). The number of patients with left ventricular ejection fraction (LVEF) >35% increased from 9.1% at the beginning of WCD use to 58.2% at the end of WCD use. Six ventricular tachycardia (VT) episodes occurred in four patients. The WCD appropriately defibrillated two patients with VT episodes. There were no inadequate shocks and no fatalities during the observation time. WCD wearing compliance was high, with a median wear time of 23.3 h/day. CONCLUSION: This retrospective analysis in a single cardiac surgery center confirms prior data on the safety and effectiveness of the WCD in patients in post-surgery care in a real-life setting. The WCD successfully protected patients from SCD during life-threatening VT episodes. WCD wearing compliance was high.

A Practical Approach to Systemic Mastocytosis Complications in Cardiac Surgery: A Case Report and Systematic Review of the Literature.

(1) Background: Systemic mastocytosis is a rare, non-curable disease with potential life-threatening complications in patients receiving cardiac surgery. (2) Methods: This systematic review of the literature was prompted by the case of a life-threatening anaphylactic reaction during cardiac surgery related to systemic mastocytosis. The search of all types of studies, using several databases (Pubmed, Scopus and Web of Science), was conducted through September 2022 to identify the relevant studies. (3) Results: Twelve studies were included describing cases of patients undergoing cardiac surgery who were diagnosed with systemic mastocytosis. An adverse effect, namely anaphylaxis, has happened in three cases. Different strategies of premedication, intraoperative and postoperative management were used. In our case, the patient was admitted for elective biological aortic valve replacement due to severe aortic stenosis. Intraoperatively, the patient developed an anaphylactic shock during the administration of protamine after separation from the cardiopulmonary bypass. This anaphylaxis reaction was a complication of the pre-existing systemic mastocytosis and could be successfully managed by the administration of epinephrine, antihistamines and corticosteroids. (4) Conclusions: This systematic literature search and case report highlight the importance of careful preoperative planning, as well as coordination between cardiac surgeons, anesthesiologists and hemato-oncological specialists, in patients with rare but complication-prone diseases such as systemic mastocytosis.

Double valve replacement in a case of Hedinger syndrome.

Hedinger Syndrome or carcinoid heart disease is a rare cardiac complication of neuroendocrine tumors (NET) affecting the tricuspid and pulmonary valves. Following is a case description of a patient undergoing treatment for a neuroendocrine tumor with liver metastasis, referred with symptomatic tricuspid valve regurgitation and pulmonary valve stenosis for surgical valve replacement. Planned surgical valve replacement was successfully performed before the onset of severe right ventricular failure or pulmonary hypertension in this case of carcinoid heart disease. An interdisciplinary approach and regular follow up is recommended in such cases.