OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients.
METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients\' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model.
RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070).
CONCLUSIONS: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.

OBJECTIVE: To describe the incidence of acute aortic dissection in a clearly defined population, to assess onset symptoms and admission biochemical marker levels and to analyse variables potentially associated to mortality.
METHODS: Medical records and CT angiograms of all patients hospitalised for acute aortic dissection in the Stockholm County during the 5-year period 2012-2016 were reviewed. The patients were followed until date of death or until 31 December 2020. The annual incidence was determined. Associations between clinical and biochemical variables and 30-day mortality, respectively, were analysed using multivariable logistic regression models.
RESULTS: A total of 344 patients were included. The mean annual incidence of acute aortic dissection was 4.1 per 100 000. Median age was 67 years (range 24-91) and 34% (n=118) were women. Type A dissection was predominant; 220 patients (64%) had type A and 124 (36%) had type B. Painless dissection was more common in type A than in type B (18% vs 15%, p=0.003). Type A dissection patients also more commonly had elevated plasma troponin T (44% vs 21%, p<0.001) and thrombocytopenia (26% vs 15%, p=0.010) than type B dissection patients on admission. Overall, 30-day mortality was 28% in type A and 11% in type B (p<0.001). Both painless dissection (OR 4.30, 95% CI 1.80 to 10.28, p=0.001) and elevated troponin T (OR 3.78, 95% CI 2.01 to 7.12, p<0.001), respectively, were associated with increased 30-day mortality in all acute aortic dissection patients. Thrombocytopenia was associated with elevated 30-day mortality only in patients with type A (OR 3.09, 95% CI 1.53 to 6.21, p=0.002).
CONCLUSIONS: Nearly two-thirds of acute aortic dissection patients had type A. Levels of troponin T and platelets, respectively, paired with presence or absence of typical symptoms may become useful adjuncts in risk stratification of patients with acute aortic dissection.

OBJECTIVE: Cabrol shunt has been introduced for surgical repair of type A aortic dissection (TAAD) without robust evidence supporting its routine preventive use.
METHODS: Adult patients with TAAD from China 5A study were included if surgically repaired between 2016 and 2022. Primary outcome was operative mortality according to Society of Thoracic Surgeons criterion. Overall, we compared clinical outcomes in patients with and without Cabrol shunt, and subgroup analysis were further examined between Cabrol shunt and outcome among patients with or without root replacement.
RESULTS: 3283 patients were finally identified for analysis, with median age of 51 (IQR 41-59) years, 2389 men, and 2201 treated with Cabrol shunt technique. Cabrol shunt-treated patients were more severely ill before surgery than those without Cabrol shunt. Overall, the rate of operative mortality was 6.6% (146/2201 in Cabrol shunt group and 71/1082 in non-Cabrol shunt group), with no association between Cabrol shunt and operative mortality (OR 1.012 (95% CI 0.754 to 1.357); p=0.938). Stratified by root replacement, Cabrol shunt was associated with similar risk of operative mortality either in patients without root replacement (OR 1.054 (0.747 to 1.487); p=0.764) or in patients with root replacement (OR 1.194 (0.563 to 2.536); p=0.644) (P interaction=0.765). Results were similar in multiple sensitivity analysis.
CONCLUSIONS: Cabrol shunt was not associated with either a greatly lowered or an increased risk of operative mortality, regardless of aortic root replacement. Our study did not support the use of Cabrol shunt as a routine preventive strategy in the treatment of TAAD.
BACKGROUND: NCT04398992.

OBJECTIVE: Aortic dissection and aortic aneurysm rupture are aortic emergencies and their clinical outcomes have improved over the past two decades; however, whether this has translated into lower mortality across countries remains an open question. The purpose of this study was to compare mortality trends from aortic dissection and rupture between the UK, Japan, the USA and Canada.
METHODS: We analysed the WHO mortality database to determine trends in mortality from aortic dissection and rupture in four countries from 2000 to 2019. Age-standardised mortality rates per 100 000 persons were calculated, and annual percentage change was estimated using joinpoint regression.
RESULTS: Age-standardised mortality rates per 100 000 persons from aortic dissection and rupture in 2019 were 1.04 and 1.80 in the UK, 2.66 and 1.16 in Japan, 0.76 and 0.52 in the USA, and 0.67 and 0.81 in Canada, respectively. There was significantly decreasing trends in age-standardised mortality from aortic rupture in all four countries and decreasing trends in age-standardised mortality from aortic dissection in the UK over the study period. There was significantly increasing trends in mortality from aortic dissection in Japan over the study period. Joinpoint regression identified significant changes in the aortic dissection trends from decreasing to increasing in the USA from 2010 and Canada from 2012. In sensitivity analyses stratified by sex, similar trends were observed.
CONCLUSIONS: Trends in mortality from aortic rupture are decreasing; however, mortality from aortic dissection is increasing in Japan, the USA and Canada. Further study to explain these trends is warranted.

Percutaneous coronary intervention (PCI) of the ostium of the left circumflex artery (LCx) is technically challenging. The aim of this study was to compare long-term clinical outcomes of ostial PCI located in the LCx versus the left anterior descending artery (LAD) in a propensity-matched population.
Consecutive patients with a symptomatic isolated \'de novo\' ostial lesion of the LCx or LAD treated with PCI were included. Patients with a stenosis of >40% in the left main (LM) were excluded. A propensity score matching was performed to compare both groups. The primary endpoint was target lesion revascularisation (TLR); other endpoints included target lesion failure and an analysis of the bifurcation angles.
From 2004 to 2018, 287 consecutive patients with LAD (n=240) or LCx (n=47) ostial lesions treated with PCI were analysed. After the adjustment, 47 matched pairs were obtained. The mean age was 72±12 years and 82% were male. The LM-LAD angle was significantly wider than the LM-LCx angle (128°±23° vs 108°±24°, p=0.002). At a median follow-up of 5.5 (IQR 1.5-9.3) years, the rate of TLR was significantly higher in the LCx group (15% vs 2%); with an HR of 7.5, 95% CI 2.1 to 26.4, p<0.001. Interestingly, in the LCx group, TLR-LM occurred in 43% of the TLR cases; meanwhile, no TLR-LM involvement was found in the LAD group.
Isolated ostial LCx PCI was associated with an increase in the rate of TLR compared with ostial LAD PCI at long-term follow-up. Larger studies evaluating the optimal percutaneous approach at this location are needed.

BACKGROUND: Contemporary management of uncomplicated type B aortic dissections (uTBAD) is based on the acuity and various morphological features. Medical therapy is mandatory, while the risks of early thoracic endovascular aortic repair (TEVAR) are balanced against the potential for rupture, complex surgery, and death. Improved aortic morphology following TEVAR is documented, but evidence for improved overall survival is lacking. The costs and impact on quality of life are also needed.
METHODS: The trial is a randomized, open-label, superiority clinical trial with parallel assignment of subjects at 23 clinical sites in Denmark, Norway, Sweden, Finland, and Iceland. Eligibility includes patients aged ≥ 18 with uTBAD of < 4 weeks duration. Recruited subjects will be randomized to either standard medical therapy (SMT) or SMT + TEVAR, where TEVAR must be performed between 2-12 weeks from the onset of symptoms.
CONCLUSIONS: This trial will evaluate the primary question of whether early TEVAR improves survival at 5 years among uTBAD patients. Moreover, the costs and the impact on quality of life should provide sorely needed data on other factors that play a role in treatment strategy decisions. The common Nordic healthcare model, with inclusion of all aortic centers, provides a favorable setting for carrying out this trial, while the robust healthcare registries ensure data validity.
BACKGROUND: ClinicalTrials.gov NCT05215587. Registered on January 31, 2022.

The primary aim was to gain insight into the growth of the aortic root in children and young adults with Marfan syndrome (MFS). Furthermore, we aimed to identify a clinical profile of patients with MFS who require an aortic root replacement at a young age with specific interest in age, sex, height and fibrillin-1 (FBN1) genotype.
Aortic root dimensions of 97 patients with MFS between 0 year and 20 years and 30 controls were serially assessed with echocardiography. Trends were analysed using a linear mixed-effect model. Additionally, including only patients with MFS, we allowed trends to differ by sex, aortic root replacement and type of FBN1 mutation.
Average aortic root dilatation in patients with MFS became more pronounced after the age of 8 years. In the MFS cohort, male patients had a significantly greater aortic root diameter than female patients, which was in close relationship with patient height. There was no difference in aortic root growth between children with dominant negative (DN) or haploinsufficient FBN1 mutations. However, DN-FBN1 variants resulting in loss of cysteine content were associated with a more severe phenotype. Eleven children needed an aortic root replacement. Compared with patients with MFS without aortic root surgery, these children had a significantly larger aortic root diameter from an early age.
This study provides clinically useful longitudinal growth charts on aortic root growth in children and young adults with MFS. Children requiring prophylactic aortic root replacement during childhood can be identified at a young age. Our growth charts can help clinicians in decision making with regard to follow-up and prophylactic therapy. Loss of cysteine content in the FBN1 protein was associated with larger aortic root dimensions.

To describe medical management in aortic dissection (AD) and to analyse the possible associations between antihypertensive, antithrombotic, anticoagulant and statin agents, respectively, and long-term survival.
From Swedish medical registers, all patients diagnosed with AD in 2006-2015 were identified. Filled prescriptions prior to admission and within 1 year from discharge in patients discharged and alive at 30 days were registered. Associations between pharmacological treatment and long-term survival were analysed using Cox proportional hazards models.
Of 3951 patients hospitalised with acute AD, 3046 (77%) were discharged and alive at 30 days. In hospitalised patients, mean age was 66 years (SD 13), and 36% (n=1098) were women. Within 1 year from discharge, 96% (n=2939) had at least one antihypertensive drug. Beta blocker was the most commonly used drug type (90%, n=2741). Statin treatment (47%, n=1418) was associated with higher long-term survival; HR 0.74 (95% CI 0.63 to 0.87, p<0.001). The positive association between statins and long-term survival remained, in subgroup analysis, in medically managed patients (HR 0.72 (95% CI 0.60 to 0.86, p<0.001)), but not in patients undergoing surgical repair (HR 0.82 (95% CI 0.58 to 1.14, p=0.230)). Beta blockers were associated with favourable long-term survival in surgically managed patients (HR 0.58 (95% CI 0.35 to 0.97, p=0.038)) but not in medically managed patients (HR 0.93 (95% CI 0.72 to 1.12, p=0.057)). Neither antiplatelet therapy nor anticoagulants were associated with long-term survival.
Statin treatment was associated with favourable long-term outcome in medically managed AD patients, whereas treatment with beta blocker was associated with higher survival only in surgically managed AD patients. Statin use as well as optimal antihypertensive therapy in the chronic stage of the disease need to be further analysed, preferably in randomised controlled trials.

暂无摘要。

Population-based epidemiologic studies of aortic dissections (ADs) are needed. This study aimed to report clinical characteristics, incidences, and mortality rates for adult patients admitted to Danish hospitals with type A AD (TAAD) or type B AD (TBAD) from 1996 through 2016.
We conducted a nationwide, population-based register study. All cases of AD registered with International Classification of Diseases, Tenth Revision codes in the Danish National Patient Registry at time of admission to a hospital with available medical records underwent validation. Data were merged between nationwide health registries including the cause of death registry. Patients with validated AD were matched 1:10 on sex and age with patients with hypertension from the general Danish population.
Of 5018 registered cases of AD, 4183 cases underwent review and 3023 (60.2%) were validated as AD. After exclusions, the distribution of validated TAAD and TBAD was 1620 (60.5%) and 1059 (39.5%; P<0.001), 67.5% and 67.0% of patients were men, and mean ages at dissection were 63.5±12.9 and 67.5±12.2 years (P<0.001), respectively. The most prevalent comorbidities for TAAD were hypertension (55.2%), thoracic aortic aneurysms (14.6%), and chronic obstructive pulmonary disease (13.1%); for TBAD, the most prevalent comorbidities were hypertension (64.1%), aortic aneurysms at any location (7.5% to 12.0%), and chronic obstructive pulmonary disease (15.7%). The overall mean annual incidence rate was 4.2/100 000 patient-years. Incidence was significantly higher for TAAD (2.2/100 000) compared with TBAD (1.5/100 000; P<0.001). The 30-day mortality rates for validated TAAD and TBAD were 22.0% and 13.9% (P<0.001), respectively, with no significant changes over time or between sexes. Adjusted 5-year overall mortality rates for TAAD and TBAD were hazard ratio 3.2 (2.9 to 3.5; P<0.001; aortic-related cause of death, 57.0%) and hazard ratio 2.1 (1.9 to 2.4; P<0.001; aortic-related cause of death, 42.8%), respectively, compared with the general hypertensive population. Among patients who survived 30 days from dissection, the adjusted 5-year overall mortality rates were hazard ratio 1.1 (1.0 to 1.3; P=0.12; aortic-related cause of death, 23.2%) and hazard ratio 1.4 (1.2 to 1.6; P<0.001; aortic-related cause of death, 25.6%) for TAAD and TBAD, respectively.
Hypertension, aortic aneurysms, and chronic obstructive pulmonary disease were the most prevalent comorbidities. The 30-day mortality frequencies were consistent over time with no significant differences between sexes. The 5-year mortality rate was higher for TAAD than TBAD. If the patient survived 30 days from dissection, the mortality rate for patients with TAAD was comparable with that of the general hypertensive population, but the mortality rate was significantly higher in patients with TBAD.