The prompt and appropriate management of acute type A aortic dissections is imperative for patient survival. Advances in medical technology have broadened the adjuncts available to treat the spectrum of pathology within this population. The role of medical management prior to surgical intervention and the components of operative management, including cannulation strategies, neuroprotection, and the extent of aortic intervention, have been explored in-depth within the literature. More recent work has identified novel open and endovascular techniques available to treat acute type A dissections. This review aims to summarize the literature, with a particular focus on innovation in cardiac surgery and its role in the care of this high-risk population.

Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype–phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with PTPN11-related Noonan syndrome and hypoplastic left heart syndrome variants.

Nearly one in five children with CHD is born with white matter injury that can be recognised on postnatal MRI by the presence of T1 hyperintense lesions. This pattern of white matter injury is known to portend poor neurodevelopmental outcomes, but the exact aetiology and histologic characterisation of these lesions have never been described. A fetal sheep was cannulated at gestational age 110 days onto a pumpless extracorporeal oxygenator via the umbilical vessels and supported in a fluid environment for 14.5 days. The fetus was supported under hypoxic conditions (mean oxygen delivery 16 ml/kg/day) to simulate the in utero conditions of CHD. At necropsy, the brain was fixed, imaged with MRI, and then stained to histologically identify areas of injury. Under hypoxemic in utero conditions, the fetus developed a T1 hyperintense lesion in its right frontal lobe. Histologically, this lesion was characterised by microvascular proliferation and astrocytosis without gliosis. These findings may provide valuable insight into the aetiology of white matter injury in neonates with CHD.

Concurrent aortic and mitral valve repair presents specific technical challenges to surgeons. Here we evaluate the outcomes of patients undergoing combined valve-sparing aortic root reimplantation and mitral valve repair. We performed a retrospective review of patients at our center between 2006 and 2021 who underwent concomitant valve-sparing aortic root replacement and mitral valve repair. Patient characteristics, including preoperative and postoperative valve function, and operative outcomes were analyzed. Between 2006 and 2021, 14 patients underwent valve-sparing aortic root replacement with concurrent mitral valve repair. Mean age of the patients at operation was 39 ± 20 years. Almost half of the patients (6/14) had Marfan disease. The primary indication for operation in all patients was aortic root dilation. Preoperatively, all patients (14/14) had moderate to severe mitral regurgitation, and half (7/14) had moderate to severe aortic regurgitation. Patients underwent mitral annuloplasty alone (9/14) or had concurrent leaflet repair (5/14). The median length of intubation was 0 day (interquartile range, 0-1 day), and median length of stay was 7 days (interquartile range, 6-10 days). At average follow-up of 6.8 ± 4.2 years, 13 of 14 patients had none to trace aortic and mitral regurgitation. One patient required reoperation for late recurrence of aortic and mitral regurgitation. There was no early or late cardiovascular mortality. Mitral valve repair can be performed safely with excellent short-term and long-term results in selected patients undergoing valve-sparing aortic root reimplantation. For durable bivalvular repair to be achieved, specific technical considerations should be adhered to.

Objectives: Sex-related disparities are well established in cardiovascular disease, but their impact on outcomes after valve-sparing root replacement (VSRR) is not well defined. In a multicentre international cohort, we evaluated sex-based differences in clinical characteristics, radiographic features and outcomes after elective VSRR. Methods: We retrospectively analysed all patients who underwent VSRR between 2004 and 2024 at 3 high-volume centres in the United States and Europe. Baseline, operative, and imaging data were compared between sexes. The primary end-point was a composite of all-cause mortality, 30-day cerebrovascular accident, and Zone 0-3 aortic reintervention or aortic valve reintervention. Kaplan-Meier and competing risk analyses were performed, with 4:1 propensity score matching (calliper 0.2) to adjust for baseline imbalances. Results: A total of 1019 patients (191 women, 19%) undergoing VSRR were included in the study. Women were younger (46.7 vs 50.4 years, P = .002), more likely to present with heritable thoracic aortic disease (HTAD) (51% vs 17%, P < .001), and had smaller aortic diameters but larger indexed dimensions compared with men (all P ≤ .001). Aortic valve leaflet repair was performed less frequently in women (17% vs 34%, P < .001). After matching, no significant sex differences were observed in early outcomes. At 10 years, freedom from the composite end-point was similar between women and men (82.8% vs 83.9%, P = .740), as were survival (95.0% vs 96.4%, P = .920) and cumulative incidences of reintervention or dissection. Age thresholds for adverse events were lower in women (>50 years) than in men (>59 years). Conclusions: Only one-fifth of VSRR patients were women, who presented younger with more HTAD and smaller absolute aortic diameters. Despite these differences, long-term outcomes were equivalent, suggesting that sex alone does not determine surgical results. Earlier surgical referral guided by indexed aortic measurements may improve recognition and outcomes in women.