Hedinger Syndrome-Lessons Learnt: A Single-Center Experience.

BACKGROUND: Hedinger syndrome (HS) or carcinoid heart disease (CD) is a rare and challenging manifestation of malignant neuroendocrine tumours (NETs) involving the heart. We aimed to report our experience with surgical strategies and midterm results in HS patients. METHODS: Eleven patients (58 ± 11 (range 41 to 79 years); 5 females) with HS who underwent cardiac surgery in our department between 07/2005 and 05/2023 were analysed. RESULTS: All patients showed a New York Heart Association (NYHA) class III-IV and in all the tricuspid valve (TV) was involved. Four patients received a TV replacement, and three TV reconstruction. Recently, to preserve the geometry and function of the compromised right ventricle (RV), we have applied the TV "bio-prosthesis in native-valve" implantation technique with the preservation of the valve apparatus (tricuspid valve implantation: TVI) in four cases. Concomitant procedures included pulmonary valve replacement in four, pulmonary implantation in one, and aortic valve replacement in three cases. To treat RV failure, we adapted a combined TandemHeart®-CytoSorb® haemoperfusion strategy in Patient #10 and venoarterial extracorporeal membrane oxygenation (V-A ECMO) support avoidance, after experiencing an ECMO-induced carcinoid-storm-related death in Patient #8. Mortality at 30 days was 18% (2/11). The median follow up was 2 ± 2.1 years (range 1 month to 6 years) with an overall mortality during the follow-up period of 72.7% (8/11). CONCLUSIONS: HS surgery, despite being a high-risk procedure, can efficiently prolong survival, and represents a safe and feasible procedure. However, patient selection seems to be crucial. Further follow up and larger cohorts are needed.

Incidence of malignancies after lung transplantation and their effect on the outcome. 26 years' experience.

Background: Malignancy is a significant, life-limiting complication after lung transplantation (LuTx) and the second common long-term cause of death. We aimed to investigate its incidence and effect on the outcome. Methods: This is a retrospective observational study. Between 1996 and 2022, n = 627 lung transplantations (LuTx) were performed in our department. We used our institutional database to identify recipients with malignancies after LuTx and examined the malignancies' incidence and mortality. Results: N = 59 malignancies occurred in n = 55 (8.8%) LuTx recipients. The post-LTx malignancies incidence was 9.4% (59/627). We report the following rates based on their location: n = 17/55 (28,8% of all recipients diagnosed with malignancies) skin, n = 10/55 (16,95%) gastrointestinal, n = 9/55 (15,3%) respiratory, n = 5/55 (8,48%) lymphatic, n = 13/55 (23,6%) other, n = 5 (8,48%) multiple synchronous.During this study period, a total of n = 328 deaths after LuTx was determined. N = 29 (8,84% of all deaths) were malignancy induced, corresponding to a total malignancy-induced mortality of 4.6% (n = 29/627). The majority of deaths were attributed to GI adenocarcinoma and PTLD. Malignancies' origin, primary COPD diagnosis, type, and specific age group were significantly survival-related (p-values <0.05). The most affected organ was skin and showed the best prognosis. PTLD had the fastest and pancreatic the latest onset. Conclusions: This is the first report of its kind in a large cohort of german LuTx recipients. The prevalence ranking of the three commonest malignancy were skin > colorectal > PTLD. Post-LTx malignancy was the second commonest cause of death. Further studies are needed, while post-LuTx malignomas remain a serious impairment of long-term LuTx survival.

Conventional vs. Sutureless Aortic Valve Bioprosthesis: Is Faster Better?

Purpose: The benefits of sutureless compared to conventional aortic valve prosthesis replacement remain controversial. Supposed advantages of sutureless aortic valve replacement include shortened cross-clamp and implantation time, as well as improved overall safety and good post-operative performance. We aimed to compare the early outcomes and performance of sutureless aortic valve replacement (su-AVR) with the sutureless Perceval (Corcym, Milan, Italy) vs. the conventional AVR with a conventional counterpart, in this case, the Labcor Dokimos Plus (LDP) aortic bioprosthesis. Methods: We compared two types of aortic valve prostheses, the sutureless (Corcym, Milan, Italy) and the conventional valve Labcor Dokimos Plus (LDP), implanted between August 2014 and May 2019 in our Department of Cardiac Surgery at RWTH Aachen University Hospital. Data were collected from 141 patients who received the Perceval (Corcym, Milan, Italy) and 138 who received the Labcor Dokimos Plus (LDP) aortic bioprosthesis. After matching the two groups considering STS mortality risk and pre-operative LDH levels, 201 patients were included in our final study cohort. Seventy-one patients (17 from the Perceval group and 54 from the Dokimos group) were excluded due to the lack of complete data, particularly standardized echocardiographic data (n = 71). Primary endpoints were 30-day mortality, length of hospital stay, and pacemaker implantation. Secondary endpoints were echocardiographic parameters, major adverse cardiovascular events, and prosthesis failure (grade II aortic regurgitation, paravalvular leak with reintervention). Results: Bypass and cross-clamp time proved to be shorter in the Perceval group, while hospital stays were longer. The faster implantation had no effect on the 30-day mortality primary endpoint. Transvalvular gradients were significantly higher in the Perceval group, in addition to a smaller effective orifice area. The LDH values were remarkably higher post-operatively in the Perceval group. Conclusions: Regarding the clinical outcomes, Perceval was equivalent and not superior to the Dokimus bioprosthesis. The suitability of a Perceval prosthesis implantation must be determined on a case-by-case basis and reserved for elderly patients with increased comorbidity.

Stent deformation in a sutureless aortic valve bioprosthesis: a pilot observational analysis using imaging and three-dimensional modelling.

OBJECTIVES: The goal of this analysis of the Perceval aortic bioprosthesis was to investigate the ovalization/deformation of the Perceval prosthesis stent after implantation and its propensity for cusp dysfunction, fluttering, fibrosis and blockage. METHODS: Between August 2014 and May 2019, a total of 134 patients (52% female) underwent aortic valve replacement with the Perceval bioprosthesis. We reconstructed three-dimensional models of the Perceval stent using thorax computed tomography scans for 16 patients employing the software Mimics (Materialise NV, Leuven, Belgium) and analysed the ovality of the stents. Radial force (RF) measurements were performed to compare the stiffness of the Perceval bioprosthesis to that of other valves. RESULTS: The three-dimensional reconstructions showed that all Perceval stents exhibited some degree of deformation and ovalization. Ovality in the annulus and commissure section of the Perceval stents ranged from 6.8% to 45% with mean values of 13.6% and 21.9%, respectively. The RF of the Perceval prosthesis was noticeably lower than that of the Edwards Intuity and several transcatheter aortic valve implantation devices. The stent adopted the preoperative shape of the aorta in the 2 patients for whom pre- and postoperative computed tomography data existed. CONCLUSIONS: The Perceval bioprostheses were deformed to different degrees in all analysed cases. The comparably low RFs might be an explanation for the propensity towards this deformation, which can lead to fluttering, a reduction of the cusps' mobility. This condition could potentially result in fibrosis as well as increased transvalvular pressure gradients and might be the cause for the increase in lactate dehydrogenase and the decrease in platelet count.

Evaluation of Left Ventricular Myocardial Work Performance in Patients Undergoing On-Pump and Off-Pump Coronary Artery Bypass Surgery.

PURPOSE: Benefits of off-pump coronary bypass (OPCAB) over on-pump (ONCAB) remain controversial. We aimed to evaluate the early impacts of OPCAB vs ONCAB for varying left ventricular (LV) function baselines by applying the non-invasive myocardial work (MW) analysis, which enables further insights in cardiac mechanics, contractility, and efficacy. METHODS: We retrospectively analyzed 98 patients (55 ONCAB vs 43 OPCAB). Transthoracic echocardiography (TTE) and concurrent arterial blood pressure measurements taken at rest, prior to, and early after surgery were performed. Global myocardial work index (GMWI), global constructive work (GCW), and global work efficiency (GWE), inter alia, were quantified. RESULTS: Preoperatively, OPCAB patients had significantly lower values than ONCAB patients in terms of GMWI (1404.33 ± 585.41 mmHg% vs 1619.07 ± 535.42 mmHg%, p = 0.039), GWE (90% (60%, 96%) vs 93% (74%, 98%), p = 0.028). After surgery, GMWI was reduced in both groups. However, a more significant GMWI impairment occurred early after ONCAB than after OPCAB (-343.14 ± 35.20 mmHg%, p <0.001 vs -224.04 ± 120.91 mmHg%, p = 0.042). CONCLUSION: Despite lower preoperative LV function in OPCAB patients, GMWIs after OPCAB were superior to ONCAB, indicating better preservation of systolic LV function early after OPCAB by means of contractility compared to ONCAB. Further studies should investigate the long-term course of MW response and their clinical impact.

DNA mismatch repair deficiency in lung and oral cavity carcinomas: the role of histogenetic origin.

DNA mismatch repair system (DNA MMR) is a crucial genetic mechanism for DNA homeostasis in prokaryotic and eukaryotic cells. During DNA replication and also recombination, point intra-nucleotide errors including base deletion, insertion, and mis-incorporation happen. These raised abnormalities in the newly synthesized DNA strand could affect negatively the stability of the molecule and the function of the corresponding genes. DNA MMR proteins prevent these errors by recognizing and repairing them, securing directly the normal anatomy of the DNA double strand and indirectly the expression of the genes. Specific genomic alterations - mutations, loss of heterozygosity (LOH), or promoter hypermethylation - regarding the MMR genes (human homologues) hMLH1, hMSH2, hMSH3, hMSH6, hPMS1 and hPMS2 modify negatively their expression leading to loss of their function in repairing the corresponding base to base errors. The result known as microsatellite instability (MSI) was initially recognized in colonic carcinoma, especially in its inherited aspect - the Lynch syndrome -, the most common form of hereditary colon carcinoma. Since then, acquired deficiencies in specific DNA MMR genes have been detected in a broad spectrum of malignancies including different anatomic regions and histologies such as stomach, prostate, esophageal, endometrial, lung and head & neck. In the current special review we explored the role of DNA MMR deficiency in lung and oral cavity carcinomas in order to identify similarities and differences regarding the corresponding genes alterations.