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Background: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country., Methods: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality., Results: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030)., Conclusion: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns., (© 2024. The Author(s).)

Background: Kommerell's aneurysm is a saccular or fusiform dilatation found in 3-8% of Kommerell's diverticulum cases. A non-dissecting rupture rate of 6% has been reported. If ruptured, emergent surgical correction is usually granted. However, evidence regarding the optimal surgical approach in this acute setting is scarce. In this case report series, we aim to describe our experience managing type-1 non-dissecting ruptured Kommerell's aneurysm with hybrid emergent surgical approaches., Cases Presentation: From January 2005 to December 2020, three cases of type-1 non-dissecting ruptured Kommerell's aneurysm requiring emergent surgical repair were identified. The mean age was 66.67 ± 7.76 years, and 3/3 were male. The most common symptoms were atypical chest pain, dyspnoea, and headache (2/3). The mean aneurysm's diameter was 63.67 ± 5.69 mm. Frozen Elephant Trunk was the preferred surgical approach (2/3). The Non-Frozen Elephant Trunk patient underwent a hybrid procedure consisting of a supra-aortic debranching and a zone-2 stent-graft deployment. We found a mean clamp time of 140 ± 60.75 min, cardiac arrest time of 51.33 ± 3.06 min, and a hospital stay of 13.67 ± 5.51 days. The most common complications were surgical-site infection and shock (2/3). Only one patient died (1/3)., Conclusion: Evidence of management for non-dissecting ruptured Kommerell's aneurysms is scarce. Additional, robust, and more extensive studies are required. The selection of the appropriate surgical approach is challenging, and each patient should be individualized. Frozen Elephant Trunk was feasible for patients requiring emergent surgical repair in our centre. However, other hybrid or open procedures can be performed., (© 2023. The Author(s).)

Objective: Cardiac paraganglioma is a rare tumor that most surgeons have limited experience treating. The objective of this study is to examine the management and outcomes for cardiac paraganglioma treatment when cared for by a multidisciplinary cardiac tumor team., Methods: We reviewed our institutionally approved cardiac tumor database from March 2004 to June 2020 for cardiac paraganglioma. These prospectively collected data were retrospectively reviewed. Patient characteristics were presented for individual patients and as summary statistics. Demographic and clinical data were also reported as median and interquartile range for continuous variables and frequencies and proportions for categoric variables. Kaplan-Meier curves were used to depict the patient survival from surgery., Results: There were 21 cases of primary cardiac paraganglioma, 19 of whom had surgical resection with 3 refusing offered surgery. Of 19 resected tumors, 13 originated from the left atrium and 6 originated from the roots of the pulmonary artery and the aorta. Complex procedures were required, including aortic and pulmonary root replacement and 8 autotransplants. All tumors had complete gross resection with no identifiable disease left behind, but 4 of these had microscopically positive margins. None of the patients had local recurrence of disease. There was 1 case of metastatic paraganglioma with death at 4 years postsurgery. Operative mortality was 10.6%. Survival from surgery was 88.2%, 71.8%, and 71.8% and 1, 5, and 10 years, respectively., Conclusions: Cardiac paraganglioma presents a surgical challenge. Mortality and long-term survival after surgical resection are acceptable but may require complex resection and reconstruction., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Aims: To assess treatment decision and outcome in patients referred for transcatheter aortic valve implantation (TAVI) in addition to predictive factors of mortality after TAVI., Methods: Three-centre prospective observational study including 358 patients. Endpoints were defined according to the Valve Academic Research Consortium., Results: Of the 358 patients referred for TAVI, TAVI was performed in 235 patients (65%), surgical aortic valve replacement (AVR) in 24 (7%) and medical therapy (MT) in 99 (28%). Reasons to decline TAVI in favour of AVR/MT were patient preference (29%), peripheral vascular disease (15%) and non-severe aortic stenosis (11%). The logistic EuroSCORE was significantly higher in patients who underwent TAVI and MT in comparison with those undergoing AVR (19 vs. 10%, p = 0.007). At 30 days, all-cause mortality and the combined safety endpoint were 9 and 24% after TAVI and 8 and 25% after AVR, respectively. All-cause mortality was significantly lower in the TAVI group compared with the MT group at 6 months, 1 year and 2 years (12% vs. 22%, 21% vs. 33% and 31% vs. 55%, respectively, p < 0.001). Multivariable analysis revealed that blood transfusion (HR: 1.19; 95% CI: 1.05-1.33), pre-existing renal failure (HR: 1.18; 95% CI: 1.06-1.33) and STS score (HR: 1.06; 95% CI: 1.02-1.10) were independent predictors of mortality at a median of 10 (IQR: 3-23) months after TAVI., Conclusions: Approximately two-thirds of the patients referred for TAVI receive this treatment with gratifying short- and long-term survival. Another 7% underwent AVR. Prognosis is poor in patients who do not receive valve replacement therapy.

Background: Over the past decade, the use of mechanical circulatory support in patients with postcardiotomy cardiogenic shock has resulted in hospital discharge rates of 25% to 40%. In an attempt to improve patient survival, we initiated a program of early insertion of an implantable Thermocardiosystems Incorporated Heartmate left ventricular assist device in patients who have circulatory failure after having undergone high-risk cardiac operations., Methods: Between April 1993 and February 1997, 12 patients underwent insertion of an implantable left ventricular assist device for postcardiotomy cardiogenic shock after coronary artery bypass grafting. Indications for insertion included postoperative cardiogenic shock (7 patients), postoperative cardiac arrest (3 patients), and failure to wean from cardiopulmonary bypass (2 patients)., Results: The median time to device insertion was 3.5 days. The mean duration of left ventricular assist device support was 103 +/- 19 days (range, 2 to 225 days). Nine of 11 patients (82%) survived to undergo either transplantation (8 patients) or explantation (1 patient), with successful hospital discharge of all 9 patients. The major complication was device-related infection (42%). A single thromboembolism occurred in a patient with an infection., Conclusions: Long-term outcome after postcardiotomy cardiogenic shock is improved substantially with the use of an implantable left ventricular assist device early in the postoperative course. Access to such a device is an important consideration before undertaking a high-risk cardiac operation, and early implantation of the device is a critical factor in ensuring long-term survival.

Objectives: Our recent experience with outpatient left ventricular assist device (LVAD) support is presented to demonstrate the possibilities and limitations of long-term outpatient mechanical circulatory assistance., Background: The experience with inpatient LVAD support as a bridge to transplantation has proved the efficacy of such therapy in improving circulatory hemodynamic status, restoring normal end-organ function and facilitating patient rehabilitation. With miniaturization of the power supplies and controllers, such mechanical circulatory support can now be accomplished in an outpatient setting., Methods: Between March 1993 and February 1997, 32 patients (26 male, 6 female, mean [+/-SEM] age 49 +/- 15 years) underwent implantation of the ThermoCardiosystems (TCI) Heartmate vented electric (VE) LVAD. The VE LVAD is powered by batteries worn on shoulder holsters and is operated by a belt-mounted system controller, allowing unrestricted patient ambulation and hospital discharge., Results: Mean duration of support was 122 +/- 26 days (range 3 to 605), with a survival rate to transplantation or explantation of 78%. Nineteen patients were discharged from the hospital on mean postoperative day 41 +/- 4 (range 17 to 68), for an outpatient support time of 108 +/- 30 days (range 2 to 466). Four patients underwent early transplantation and could not participate in the discharge program, and three patients currently await discharge. The complication rate was not statistically different from that encountered in our previous 52 patients with a pneumatic LVAD., Conclusions: Outpatient LVAD support is safe and provides improved quality of life for patients awaiting transplantation. Wearable and totally implantable LVADs should be studied as permanent treatment options for patients who are not candidates for heart transplantation.