Right ventricular function in Marfan patients remains stable despite multiple cardiac interventions.

OBJECTIVES: Mitral valve regurgitation and left ventricular dysfunction are cardiovascular symptoms of Marfan syndrome. There is a paucity of information on tricuspid valve regurgitation and right ventricular function. In patients with Marfan syndrome, we looked at long-term changes in right ventricular function, tricuspid valve regurgitation and freedom from tricuspid valve repair. METHODS: Retrospective-observational single-centre analysis on right ventricular function and tricuspid regurgitation in Marfan patients who underwent surgery with cardioplegic arrest between 1995 and 2020. Patients were followed-up from 1st operation until death, with echocardiographic changes analysed longitudinally. Composite end point was tricuspid annular plane systolic excursion (TAPSE) ≤16 mm, severe tricuspid regurgitation or tricuspid repair. RESULTS: The study included 135 patients who underwent 193 operations, 58 of those were reoperations in 40 patients. Median age at 1st operation was 35 years [interquartile range (IQR) 26-46], median follow-up was 8.0 years (IQR 3.0-16.0) and median time to 1st reoperation was 7.5 years (IQR 3.4-12.5). The composite end point occurred in 81 observations in 40 patients, mostly as a recurrent event, after median 7.0 years (IQR 1.0-13.0). Ten-year cumulative incidence for composite end point was 22.0% (95% CI 15-31) and 9.0% (95% CI 4.4-16) for new-onset TAPSE ≤16 mm, but no significant change in TAPSE was observed at 10 years. Tricuspid regurgitation was associated with increased risk of annual progression (P < 0.001), but not clinically relevant at 10 years. Actuarial 10-year survival was 91.1%. CONCLUSIONS: In Marfan patients with a history of cardiac surgery and subsequent reoperations, the right ventricular function remains stable. The incidence of severe tricuspid regurgitation and tricuspid repair remain low.

Evaluation of custom-made Relay® stent-grafts for aortic arch landing zones 0 and I: experience from two high-volume aortic centres.

OBJECTIVES: To report experience with a Relay® stent-graft custom-made platform in treating different aortic arch pathology in 2 high-volume aortic centres. METHODS: A retrospective analysis of all patients treated between July 2016 and July 2023 with custom-made Relay® stent-graft (custom-made device). Underlying aortic arch pathology was an aneurysm, penetrating aortic ulcer, and dissection. Three custom-made device designs were used: proximal scallop, fenestrations, and inner branches. The endpoints were technical success, perioperative stroke, death, and reintervention rate. RESULTS: Thirty-five patients (89% males) with a mean age of 70 ± 11 years were treated.Indication for treatment was penetrating aortic ulcer in 14 patients (40%), aneurysm in 11 patients (31%) and aortic dissection in 10 patients (29%). The technical success rate was 100%. Twenty-eight patients (80%) had proximal sealing in zone 0, and 7 (20%) had proximal sealing in zone 1. Nine patients (25.6%) had proximal scallops, 9 (25.6%) had 1 big fenestration and 17 (48.8%) had a branched device; 1 with single branch, 15 with double branches and 1 with triple branches. Thirty patients (86%) had previous or simultaneous left subclavian artery revascularization. No patient died during 30 days. Two patients (5.7%) had stroke postoperatively; both recovered without disabling deficits. The mean follow-up was 35 ± 26 months. Six patients (17.1%) died during follow-up. One patient required reinforcement of the bridging stent in the left common carotid artery and one additional vascular plugging of the left subclavian artery. Three patients received distal extension. CONCLUSIONS: The Relay® stent-graft custom-made platform showed a good performance in our study with a high technical success rate, low perioperative stroke and mortality, and low reintervention rates during the follow-up.

Endovascular aortic repair in patients with Marfan and Loeys-Dietz syndrome is safe and durable when employed by a multi-disciplinary aortic team.

OBJECTIVES: The aim of this study was to report on mid-term outcomes after endovascular aortic repair (EVAR) in patients with Marfan (MFS) or Loeys-Dietz (LDS) syndrome. METHODS: We analysed data from 2 European centres of patients with MFS and LDS undergoing EVAR. Patients were analysed based on (i) timing of the procedure (planned versus emergency procedure) and (ii) the nature of the landing zone (safe versus non-safe). The primary end-point was freedom from reintervention. Secondary end-points were freedom from stroke, bleeding and death. RESULTS: A population of 419 patients with MFS (n = 352) or LDS (n = 67) was analysed for the purpose of this study. Thirty-nine patients (9%) underwent EVAR. Indications for thoracic endovascular aortic repair or EVAR were aortic dissection in 13 (33%) patients, aortic aneurysm in 22 (57%) patients and others (intercostal patch aneurysm, penetrating atherosclerotic ulcer, pseudoaneurysm, kinking of frozen elephant trunk (FET)) in 4 (10%) patients. Thoracic endovascular repair was performed in 34 patients, and abdominal endovascular aortic repair was performed in 5 patients. Mean age at 1st thoracic endovascular aortic repair/EVAR was 48.5 ± 15.4 years. Mean follow-up after 1st thoracic endovascular aortic repair/EVAR was 5.9 ± 4.4 years. There was no statistically significant difference in the rate of reinterventions between patients with non-safe landing zone and the patients with safe proximal landing zone (P = 0.609). Furthermore, there was no increased probability for reintervention after planned endovascular intervention compared to emergency procedures (P = 0.916). Mean time to reintervention, either open surgical or endovascular, after planned endovascular intervention was in median 3.9 years (95% confidence interval 2.0-5.9 years) and 2.0 years (95% confidence interval -1.1 to 5.1 years) (P = 0.23) after emergency procedures. CONCLUSIONS: EVAR in patients with MFS and LDS and a safe landing zone is feasible and safe. Endovascular treatment is a viable option when employed by a multi-disciplinary aortic team even if the landing zone is in native tissue.

The non to moderately dilated root in acute type A aortic dissection: outcomes of the PENN-BERN registry in young, non-syndromic patients.

OBJECTIVES: There is an ongoing debate regarding whether patients benefit more from root replacement compared to a reconstruction of the sinuses of Valsalva in acute type A aortic dissection (aTAAD). In those with known or suspected connective tissue disorders, root replacement is considered appropriate. However, there are currently no diameter-based guidelines regarding the best approach in patients with minimally to moderately dilated root and no connective tissue disorders. METHODS: From January 2005 to December 2022, a two-centre registry of aTAAD was created. Patients were included based on their age (≤60 years), the absence of root entry and dilatation >50 mm and the absence of syndromic hereditable aortic disease. Patients were divided into 2 groups based on the proximal procedure, root reconstruction and root replacement. Propensity score pair matching was performed based on preoperative characteristics. RESULTS: Cumulative incidence of reintervention at 10 years was slightly higher after root reconstruction 13% vs 3.9% in the matched group (P = 0.040). Survival at 10 years was not affected by the procedure independently of the matching 72.1% vs 71.4% (P = 0.2). Uni- and multivariate Cox regressions showed that a root diameter of >40 mm was associated with a hazard ratio of 7.7 (95% confidence interval 2.6-23) and 5.4 (7-17), respectively, for reoperation for aneurysm and pseudoaneurysm. CONCLUSIONS: Rate of reoperation due to proximal pseudoaneurysm and aneurysm could be significantly reduced with a lower threshold of 40 mm to replace the aortic root in aTAAD than in elective cases.

Hyperoxia-induced deterioration of diastolic function in anaesthetised patients with coronary artery disease - Randomised crossover trial.

Background: There are no current recommendations for oxygen titration in patients with stable coronary artery disease. This study investigates the effect of iatrogenic hyperoxia on cardiac function in patients with coronary artery disease undergoing general anaesthesia. Methods: Patients scheduled for elective coronary artery bypass graft surgery were prospectively recruited into this randomised crossover clinical trial. All patients were exposed to inspired oxygen fractions of 0.3 (normoxaemia) and 0.8 (hyperoxia) in randomised order. A transoesophageal echocardiographic imaging protocol was performed during each exposure. Primary analysis investigated changes in 3D peak strain, whereas secondary analyses investigated other systolic and diastolic responses. Results: There was no statistical difference in systolic function between normoxaemia and hyperoxia. However, the response in systolic function to hyperoxia was dependent on ventricular function at normoxaemia. Patients with a normoxaemic left ventricular (LV) global longitudinal strain (GLS) poorer than the derived cut-off (>-15.4%) improved with hyperoxia (P<0.01), whereas in patients with normoxaemic LV-GLS <-15.4%, LV-GLS worsened with transition to hyperoxia (P<0.01). The same was seen for right ventricular GLS with a cut-off at -24.1%. Diastolic function worsened during hyperoxia indicated by a significant increase of averaged E/e' (8.6 [2.6]. vs 8.2 [2.4], P=0.01) and E/A ratio (1.4 (0.4) vs 1.3 (0.4), P=0.01). Conclusions: Although the response of biventricular systolic variables is dependent on systolic function at normoxaemia, diastolic function consistently worsens under hyperoxia. In coronary artery disease, intraoperative strain analysis may offer guidance for oxygen titration. Clinical trial registration: NCT04424433.

The impact of sex and gender on aortic events in patients with Marfan syndrome.

OBJECTIVES: The aim of this study was to explore sex and gender differences regarding aortic events in Marfan patients. METHODS: We analysed all data from our connective tissue disorder database. Only patients with Marfan syndrome were included. For analysis, patients were divided by sex. Female patients were further divided into 2 subgroups: with versus without children. Aortic events were defined as Stanford type A aortic dissection (TAAD) or type B aortic dissection (TBAD) or any aortic intervention. RESULTS: A population of 183 Marfan patients was analysed for the purpose of this study. One hundred four (57%) were male and 79 (43%) were female patients. Thirty-seven (47%) of the 79 female patients had at least 1 child. Male patients had a significantly higher probability of experiencing an aortic event (P = 0.015) compared to female patients. However, there was no increased probability for recurrent events in male patients compared to female patients (P = 0.063). Follow-up revealed no sex and gender differences in the occurrence of Stanford TAAD or TBAD between male and female patients (P = 0.324/P = 0.534). While 11% of women with children suffered from peripartum aortic events, 24% experienced Stanford TAAD unrelated to pregnancy. CONCLUSIONS: Male patients have a higher risk of aortic events than female patients. The majority of women were not aware of their Marfan syndrome diagnosis before conceiving. One out of 10 women suffered from peripartum Stanford TAAD or TBAD. Twice as many female patients with children suffered from aortic dissection unrelated to childbirth. There were no sex and gender differences affecting mortality in Marfan patients.

One-Year Outcome of Postoperative Stroke and Nerve Injury After Supraclavicular Revascularization of The Left Subclavian Artery for Proximal Landing Zone Extension in Thoracic Endovascular Aortic Repair.

OBJECTIVE: To assess the outcome of stroke and nerve injury after supraclavicular revascularization of the left subclavian artery for proximal landing zone extension in thoracic endovascular aortic repair (TEVAR). METHODS: Retrospective analysis of all patients undergoing left-sided carotid-subclavian bypass (CSB) and subclavian-carotid transposition (SCT) with simultaneous or staged TEVAR between January 2010 and June 2019. Endpoints were perioperative cerebrovascular events and nerve injuries, patency and re-intervention due to the debranching, and mortality at 30 days and during follow-up. RESULTS: Forty-eight patients (median age 66 years, 81 % male) had 25 (52%) CSB and 23 (48%) SCT. TEVAR was performed simultaneously in 39 (81%) patients, 11 (23%) of them in an emergent setting. There were 7 (15%) re-interventions within 30 days: 3 due to local hematoma, one for bypass occlusion, 2 for stenosis (of which one was not confirmed intraoperatively), and one after initially abandoned SCT with subsequent CSB on the next day. 30-day mortality was 2%; 1 patient died on the first postoperative day after emergency coronary artery bypass surgery and multiorgan failure. 4 (8%) patients suffered postoperative strokes; 3 occurred after simultaneous emergency procedures and none was fatal. There were 9 (19%) left neck nerve injuries in 8 patients, 5 patients had SCT and 3 CSB. During a median follow-up of 37.5 months (IQR 23-83) with a Follow-up Index of 0.77, there were no reinterventions or occlusions, and no graft infections. Primary patency was 90% and primary assisted patency 98% during follow-up. 8 patients died during follow-up, all of them with patent cervical debranching. CONCLUSION: Supraclavicular LSA revascularization for proximal landing zone extension in TEVAR is safe with an acceptable rate of early re-interventions. There is higher risk for perioperative stroke during concomitant emergency LSA revascularization and TEVAR. Left neck nerve injuries are common complications but resolve completely in vast majority of the cases during first postoperative year. During follow-up, excellent patency could be expected.

Perfusion management during cardiopulmonary bypass in a patient with high cardiac output due to arteriovenous malformations.

We present a case of a 37-year-old woman diagnosed with Parkes-Weber syndrome characterized by high-flow arterio-venous malformations of the pelvic region with an estimated cardiac output of 7.2-8.5 l/min. Due to concomitant Marfan syndrome, the patient also presented an aortic root aneurysm needing surgical treatment under cardiopulmonary bypass. Concerns arose regarding the ability to maintain sufficient perfusion pressure during cardiopulmonary bypass due to the arterio-venous malformations. To address this, an intravascular balloon occlusion at the level of the aortic bifurcation was placed in order to limit arterial inflow into the arterio-venous malformations and achieve optimal perfusion pressure during cardiopulmonary bypass. The patient did not experience any complications and recovered completely after surgery.

Study design for a randomized crossover study investigating myocardial strain analysis in patients with coronary artery disease at hyperoxia and normoxemia prior to coronary artery bypass graft surgery (StrECHO-O2).

BACKGROUND: Supplemental oxygen (O2) is used routinely during anesthesia. In the treatment of acute myocardial infarction, it has been established that hyperoxia is to be avoided, whereas information on benefit and risk of hyperoxia in patients with stable coronary artery disease (CAD) remain scarce, especially in the setting of general anesthesia. This study will compare the immediate effects of normoxemia and hyperoxia on cardiac function, with a primary focus on changes in peak longitudinal left-ventricular strain, in anesthetized stable chronic CAD patients using peri-operative transesophageal echocardiography (TEE). METHODS: A single-center randomized cross-over clinical trial will be conducted, enrolling 106 patients undergoing elective coronary artery bypass graft surgery. After the induction of anesthesia and prior to the start of surgery, cardiac function will be assessed by 2D and 3D TEE. Images will be acquired at two different oxygen states for each patient in randomized order. The fraction of inspired oxygen (FIO2) will be titrated to a normoxemic state (oxygen saturation of 95-98%) and adjusted to a hyperoxic state (FIO2 = 0.8). TEE images will be analyzed in a blinded manner for standard cardiac function and strain parameters. CONCLUSION: By using myocardial strain assessed by TEE, early and subtle signs of biventricular systolic and diastolic dysfunction can be promptly measured intraoperatively prior to the onset of severe signs of ischemia. The results may help anesthesiologists to better understand the effects of FIO2 on cardiac function and potentially tailor oxygen therapy to patients with CAD undergoing general anesthesia.

Long-term outcome of patients with Marfan syndrome with previous aortic surgery but native aortic roots.

OBJECTIVES: The goal of this study was to report the long-term outcomes of patients with Marfan syndrome who had aortic surgery on any aortic segment except for the replacement of the aortic root itself. METHODS: An observational retrospective single-centre study was conducted with 115 Marfan syndrome patients who underwent 189 major aortic interventions from 1995 until 2018. Patients without aortic root replacement were identified and aortic root growth was analysed over time. RESULTS: Eleven of 115 patients (9.5%) did not have aortic root replacement during a follow-up of 10.5 [standard deviation (SD) 5.7] years and a mean age at last follow-up of 53.9 (SD 13.4) years. Patients without root replacement did not suffer less frequently from any type of acute aortic dissection (type A 27% vs 25%, P = 0.999; type B 36% vs 25%, P = 0.474). Patients with native aortic roots did not undergo fewer aortic interventions than those with aortic root replacement [12/11, mean 1.09 (SD 0.54) operations/patient vs 177/104, mean 1.7 (SD 1.3); P = 0.128]. Progression of the aortic root dimension was 0.5 (SD 0.3) mm/year in the group of patients with native aortic roots. CONCLUSIONS: Current data suggest that 10% of patients with Marfan syndrome with previous aortic surgery will be free from aortic root replacement until the sixth decade of life.

Management of the aortic arch in patients with Loeys-Dietz syndrome.

OBJECTIVES: We sought to develop strategies for management of the aortic arch in patients with Loeys-Dietz syndrome (LDS) through a review of our clinical experience with these patients and a comparison with our experience in patients with Marfan syndrome (MFS). METHODS: We reviewed hospital and follow-up records of 79 patients with LDS and compared them with 256 patients with MFS who served as reference controls. RESULTS: In the LDS group, 16% of patients presented initially with acute aortic dissection (AAD) (67% type A, 33% type B) or developed AAD during follow-up, compared with 10% of patients with MFS (95% type A, 5% type B). There was no difference between patients with LDS or MFS in need for subsequent arch interventions after aortic root surgery (46% vs 50%, P = 1.0). Among the patients who never had AAD, the need for arch repair at initial root surgery was greater in patients with LDS (5% vs 0.4%, P = .04), as was the need for any subsequent aortic surgery (12% vs 1.3%, P = .0004). Late mortality in patients with LDS after arch repair was greater than in those patients who had no arch intervention (33% vs 6%, P = .007). CONCLUSIONS: In the absence of dissection, patients with LDS have a greater rate of arch intervention after root surgery than patients with MFS. After a dissection, arch reintervention rates are similar in the 2 groups. Arch intervention portends greater late mortality in LDS.

Standards of reporting in open and endovascular aortic surgery (STORAGE guidelines).

The number of patients undergoing surgery on the thoracic and thoraco-abdominal aorta has been steadily increasing over the past decade. This document aims to give guidance to authors reporting on results in aortic surgery by clarifying definitions of aortic pathologies, open and endovascular techniques and by listing clinical parameters that should be provided for full presentation of patients' clinical profile and in particular, their outcome. The aim is to help find a common language in the treatment of aortic disease and to contribute to a better understanding of this patient population.