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.

Impact of surgically placed radiopaque markers during aortic root surgery on facilitating secondary diagnostic and therapeutic interventions.

BACKGROUND: Implantation of radiopaque markers during aortic root surgery might possibly facilitate upcoming coronary angiography or transcatheter aortic valve implantation. Aim of this study was to report the impact of surgically placed radiopaque markers on procedural characteristics and on angiographic outcomes. METHODS: We retrospectively analyzed baseline characteristics, preoperative and postoperative data as well as procedural findings. In addition, a subgroup analysis of all patients who underwent coronary angiography after aortic root surgery was performed to report radiation time and contrast media used. RESULTS: A total of 469 patients underwent aortic root surgery between January 2008 and April 2020. Patients were divided into two groups: group w/ markers (n = 182) and group w/o markers (n = 287). A propensity score matching was performed resulting in a total of 28 patients w/ markers and 28 patients w/o markers. Aortic cross-clamp time did not differ statistically significantly between the group w/o markers and the group w/ markers (124.0 [96.0-150.0] versus 123.0 [110.0-149.0] min, p = 0.09). There was no increased probability for requirement of postoperative angiography in the group w/o markers compared to the group w/ markers (11.8% versus 15.4%, p = 0.27). There was no statistically significant difference in the radiation time 5.5 [3-6.5] versus 5 [2.5-7.5] min, p = 0.62) nor in the amount of contrast media used (85 [77.5-100] versus 80 [60-90] ml, p = 0.07). CONCLUSIONS: Surgically placed radiopaque markers during aortic root surgery do not increase operative risk and have the potential for facilitating secondary diagnostic and therapeutic interventions.

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.

Outcome of Stanford type B dissection in patients with Marfan syndrome.

OBJECTIVES: The aim of this study was to determine the outcome of Stanford type B aortic dissection in patients with Marfan syndrome (MFS) and to evaluate aortic diameters at time of dissection as well as the impact of previous aortic root replacement. METHODS: Analysis of all patients with MFS fulfilling Ghent criteria seen at this institution since 1995 until 2022. RESULTS: Thirty-six (19%) out of 188 patients with MFS suffered from Stanford type B aortic dissection during the study period. The Mean aortic diameter at the time of dissection was 39.0 mm [95% confidence interval (CI): 35.6-42.3]. The mean pre-dissection diameter (available in 25% of patients) was 32.1 mm (95% CI: 28.0-36.3) and the mean expansion was 19% (95% CI: 11.9-26.2). There was no correlation between age and diameter at the time of dissection (<20, 21-30, 31-40, 41-50, 51-60, <61 years; P = 0.78). Freedom from intervention after dissection was 53%, 44% and 33% at 1, 5 and 10 years. Aortic growth rate in those patients that had to undergo intervention within the 1st year after dissection was 10.2 mm/year (95% CI: 4.4-15.9) compared to 5.8 mm/year (95% CI: 3.3-8.3, P = 0.109) in those thereafter. The mean time between dissection and intervention was 1.8 years (95% CI: 0.6-3.0). While type B dissection seems more frequent after previous elective aortic repair (58% vs 42%), there was no difference between valve-sparing root replacement (VSRR) compared to Bentall procedures [Hazard ratio (HR) for VSRR 0.78, 95% CI: 0.31-2.0, P-value = 0.61]. The mean age of the entire population at the end of follow-up was 42 years (95% CI: 39.2-44.7). The mean follow-up time was 9 years (95% CI: 7.8-10.4). CONCLUSIONS: Stanford type B dissection in patients with MFS occurs far below accepted thresholds for intervention. Risk for type B dissection is present throughout lifetime and two-thirds of patients need an intervention after dissection. There is no difference in freedom from type B dissection between a Bentall procedure and a VSRR.

Acute type A aortic dissection in adolescents and young adults under 30 years of age: demographics, aetiology and postoperative outcomes of 139 cases.

OBJECTIVES: The prevalence and aetiology of acute aortic dissection type A (AADA) in patients ≤30 years is unknown. The aims of this clinical study were to determine the prevalence and potential aetiology of AADA in surgically treated patients ≤30 years and to evaluate the respective postoperative outcomes in this selective group of patients in a large multicentre study. METHODS: Retrospective data collection was performed at 16 participating international aortic institutions. All patients ≤30 years at the time of dissection onset were included. The postoperative results were analysed with regard to connective tissue disease (CTD). RESULTS: The overall prevalence of AADA ≤30 years was 1.8% (139 out of 7914 patients), including 51 (36.7%) patients who were retrospectively diagnosed with CTD. Cumulative postoperative mortality was 8.6%, 2.2% and 1.4%. Actuarial survival was 80% at 10 years postoperatively. Non-CTD patients (n = 88) had a significantly higher incidence of arterial hypertension (46.6% vs 9.8%; P < 0.001) while AADA affected the aortic root (P < 0.001) and arch (P = 0.029) significantly more often in the CTD group. A positive family history of aortic disease was present in 9.4% of the study cohort (n = 13). CONCLUSIONS: The prevalence of AADA in surgically treated patients ≤30 years is <2% with CTD and arterial hypertension as the 2 most prevalent triggers of AADA. Open surgery may be performed with good early results and excellent mid- to long-term outcomes.

Treatment of infected hybrid arch prosthesis with self-assembled bovine elephant trunk grafts.

Graft infections are associated with severe morbidity and mortality. The widespread use of the frozen elephant technique increases the incidence of complex aortic patients to suffer from graft infections. Surgery of these patients is challenging. Removal of the stent graft portion of the frozen elephant technique prosthesis via sternotomy carries the risk of irreparable damage to the descending aorta. There is currently no single-stage surgical strategy that allows for the removal of all infected material apart from a hemi-clamshell approach. This approach is technically demanding and associated with significant morbidity and mortality. This results in conservative treatment in a substantial number of patients. Pericardial tube grafts have shown to be an excellent option in treating graft infections in various aortic segments with promising results concerning freedom of re-infection and survival. We report a single-stage, trans-sternal approach to remove all infected materials and simultaneously treat the descending aorta to prevent aortic catastrophe in 2 consecutive cases.

Thoracoabdominal aneurysm causing functional mitral valve stenosis after total arch replacement.

Large thoracic and thoraco-abdominal aneurysms may compress adjacent mediastinal structures. We present a case of a large thoraco-abdominal aneurysm compressing the left atrium and leading to functional mitral valve stenosis after total aortic arch repair, requiring urgent open thoraco-abdominal aneurysm repair.

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.

Providing safe perioperative care in cardiac surgery during the COVID-19 pandemic.

The coronavirus disease 2019 (COVID-19) pandemic has potentiated the need for implementation of strict safety measures in the medical care of surgical patients - and especially in cardiac surgery patients, who are at a higher risk of COVID-19-associated morbidity and mortality. Such measures not only require minimization of patients' exposure to COVID-19 but also careful balancing of the risks of postponing nonemergent surgical procedures and providing appropriate and timely surgical care. We provide an overview of current evidence for preoperative strategies used in cardiac surgery patients, including risk stratification, telemedicine, logistical challenges during inpatient care, appropriate screening capacity, and decision-making on when to safely operate on COVID-19 patients. Further, we focus on perioperative measures such as safe operating room management and address the dilemma over when to perform cardiovascular surgical procedures in patients at risk.

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.