Effect of beta-blockers and angiotensin receptor blockers in reducing the aortic growth rate in children with bicuspid aortic valve-related aortopathy.

AIMS: The aim of this study is to evaluate the effect of beta-blockers and angiotensin receptor blockers in reducing the aortic growth rate in children with bicuspid aortic valve (BAV)-related aortopathy and ascending phenotype. METHODS: Consecutive paediatric patients (≤16 years) with BAV and ascending aorta (AsAo) dilation (z-score > 3) were enrolled in this observational retrospective cohort study. Patients receiving prophylactic treatment with either atenolol (0.5 to 1.0 mg/kg/daily) or losartan (0.7 to 1.4 mg/kg/daily) were compared with those who did not receive medical prophylaxis (control group). The primary outcome of interest was the annual rate of change in maximal AsAo diameter z-score in the treatment and control groups. RESULTS: From a cohort of 1005 patients, 120 (mean age 11.3 ± 4.5 years, 82% males) fulfilled the inclusion criteria and were included in the study. Patients in the treatment and control group had similar age, sex, family history of BAV, BAV morphology, and baseline AsAo diameter. During a median follow-up of 7.1 years (interquartile range 3.8-10.2), no differences were observed in the annual growth rate of aortic diameter z-score between patients on treatment and controls. The prevalence of aortic diameter progression was similar in the treatment and control groups, and treatment with atenolol or losartan was not associated with a lower rate of aortic disease progression. CONCLUSIONS: The findings revealed no significant difference in the annual aortic growth rate between treated and untreated patients. Larger cohort studies or, ideally, randomized clinical controlled trials are needed to validate these findings.

Open aortic arch repair without circulatory arrest by frozen elephant trunk in Ishimaru zone 0.

BACKGROUND: Open arch surgery is technically demanding for the surgeon and surgically and biologically invasive for the patient, requiring a variably long period of hypothermic circulatory arrest. CASE PRESENTATION: Here we present a case of an elderly patient with chronic renal failure and multiple splanchnic artery disease successfully treated for a rupturing pseudoaneurysm of the aortic arch with a technique that we developed for particularly frail patients. The procedure includes: triple supra-aortic vessel perfusion; distal thoracic aorta antegrade perfusion; balloon endo-clamping of the descending aorta; and anastomosis of an off-the-shelf hybrid arch prosthesis in Ishimaru zone 0. These maneuvers allowed to maintain an extracorporeal circulation in the phase of distal anastomosis, instead of a period of circulatory arrest, employing just mild hypothermia: technical details are depicted and discussed also in comparison with other methods proposed in the literature. CONCLUSIONS: Being able to take advantage of both open surgery advancements and endovascular methods is the key to cardiovascular surgery success today in front of complex pathologies of the aorta: increasing safety and reducing invasiveness of therapeutic options may progressively extend surgical candidacy to the frailest patients.

[Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes]. / Resumen: Consenso internacional para la nomenclatura y clasificación de la válvula aórtica bicúspide congénita y su aortopatía, con fines clínicos, quirúrgicos, intervencionistas y de investigación.

This consensus of nomenclature and classification for congenital bicuspid aortic valve and its aortopathy is evidence-based and intended for universal use by physicians (both pediatricians and adults), echocardiographers, advanced cardiovascular imaging specialists, interventional cardiologists, cardiovascular surgeons, pathologists, geneticists, and researchers spanning these areas of clinical and basic research. In addition, as long as new key and reference research is available, this international consensus may be subject to change based on evidence-based data1.

Este consenso de nomenclatura y clasificación para la válvula aórtica bicúspide congénita y su aortopatía está basado en la evidencia y destinado a ser utilizado universalmente por médicos (tanto pediatras como de adultos), médicos ecocardiografistas, especialistas en imágenes avanzadas cardiovasculares, cardiólogos intervencionistas, cirujanos cardiovasculares, patólogos, genetistas e investigadores que abarcan estas áreas de investigación clínica y básica. Siempre y cuando se disponga de nueva investigación clave y de referencia, este consenso internacional puede estar sujeto a cambios de acuerdo con datos basados en la evidencia1.

Non-A Non-B Acute Aortic Dissection: Is There Some Confusion in the Radiologist's Mind?

BACKGROUND: The aim of this study is to define and determine the rate of acute non-A-non-B aortic dissections, and to evaluate CT angiography findings and possible complications, as well as to discuss management strategies and currently available therapy. Non-A non-B type of aortic dissection is still a grey area in the radiologist's mind, such that it is not entirely clear what should be reported and completed in terms of this disease. METHODS: A retrospective single-center study including 36 pre-treatment CT angiograms of consecutive patients (mean age: 61 years) between January 2012 and December 2022 with aortic dissection involving the aortic arch with/without the thoracic descending/abdominal aorta (type non-A non-B). RESULTS: According to the dissection anatomy, we identified three modalities of spontaneous acute non-A-non-B anatomical configurations. Configuration 1 (n = 25) with descending-entry tear and retrograde arch extension (DTA entry). Configuration 2 (n = 4) with Arch entry tear and isolated arch involvement (Arch alone). Configuration 3 (n = 7) with Arch entry and anterograde descending (±abdominal) aorta involvement (Arch entry). CT angiogram findings, management, and treatment options are described. CONCLUSIONS: Acute non-A non-B dissection represents an infrequent occurrence of aortic arch dissection (with or without involvement of the descending aorta) that does not extend to the ascending aorta. The complete understanding of its natural progression, distinct CT angiography subtypes, optimal management, and treatment strategies remains incomplete. Within our series, patients frequently exhibit a complex clinical course, often necessitating a more assertive approach to treatment compared to type B dissections.

The Novel Role of Noncoding RNAs in Modulating Platelet Function: Implications in Activation and Aggregation.

It is currently believed that plaque complication, with the consequent superimposed thrombosis, is a key factor in the clinical occurrence of acute coronary syndromes (ACSs). Platelets are major players in this process. Despite the considerable progress made by the new antithrombotic strategies (P2Y12 receptor inhibitors, new oral anticoagulants, thrombin direct inhibitors, etc.) in terms of a reduction in major cardiovascular events, a significant number of patients with previous ACSs treated with these drugs continue to experience events, indicating that the mechanisms of platelet remain largely unknown. In the last decade, our knowledge of platelet pathophysiology has improved. It has been reported that, in response to physiological and pathological stimuli, platelet activation is accompanied by de novo protein synthesis, through a rapid and particularly well-regulated translation of resident mRNAs of megakaryocytic derivation. Although the platelets are anucleate, they indeed contain an important fraction of mRNAs that can be quickly used for protein synthesis following their activation. A better understanding of the pathophysiology of platelet activation and the interaction with the main cellular components of the vascular wall will open up new perspectives in the treatment of the majority of thrombotic disorders, such as ACSs, stroke, and peripheral artery diseases before and after the acute event. In the present review, we will discuss the novel role of noncoding RNAs in modulating platelet function, highlighting the possible implications in activation and aggregation.

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.

Prolonged Periods of Antegrade Cerebral Perfusion Are Safe During Elective Arch Surgery.

BACKGROUND: Cerebral circulatory arrest times >40 minutes during aortic surgery have previously been shown to be associated with increased morbidity and mortality. The purpose of this study was to redefine what would constitute a safe period of circulatory arrest for patients who underwent elective proximal aortic operations requiring antegrade cerebral perfusion (ACP). METHODS: The ARCH International aortic database was queried, and 2008 patients undergoing elective arch operations with circulatory arrest using ACP were identified. Circulatory arrest time was categorized a priori in 10-minute intervals. To further determine the impact of this variable on outcomes, hierarchical multivariable regression analysis was performed. RESULTS: Unadjusted mortality increased with increasing circulatory arrest time from 4.8% (<40 minutes) to 13.5% (>90 minutes; P < .001), but risk of stroke was not impacted (P = .4). When treated as a continuous variable, mortality increased significantly with increasing circulatory arrest time, whereas the risk of permanent stroke did not. Using <40 minutes as the reference, multivariable analysis showed no statistical increase in mortality for ranges up to 80 minutes of circulatory arrest. The risk of permanent stroke was not significantly higher for any time interval >40 minutes up to 90 minutes. CONCLUSIONS: In this series of patients who underwent elective proximal aortic surgery using ACP, periods of circulatory arrest up to at least 80 minutes were not associated with significant increases in mortality or permanent stroke. Modern perfusion strategies have allowed for increased safety during elective arch cases requiring prolonged periods of circulatory arrest.

Acute type A aortic dissection in bicuspid versus tricuspid aortic valve patients: focus on geometrical features of the aorta.

OBJECTIVES: This retrospective observational study aimed to assess the geometrical features, including diameters, length and angulation, of the ascending aorta with bicuspid and tricuspid aortic valve and to identify imaging markers potentially predicting whether aortopathy is likely to evolve chronically or to complicate with acute type A dissection. METHODS: Angio-computed-tomography scans of 354 patients (from 3 centres) with non-dilated (n = 97), aneurysmal (n = 100) or dissected aorta (n = 157) were reviewed. Diameters were measured at root, sinotubular junction, ascending, brachio-cephalic trunk origin; centreline lengths of the root and tubular tract and ascending-arch angle (between the ascending tubular tract axis and the proximal arch axis) were also measured. For 12 dissection patients, pre-dissection scans were available to investigate predisposing aortic geometry. Statistical analysis included: tricuspid versus bicuspid comparisons in each subgroup; univariate and multivariate analyses of the predictors of ascending-arch angle narrowing; and estimation of diagnostic accuracy of the angle parameter. RESULTS: Diameters and lengths were similar between aneurysms and dissections, whereas dissections showed a significant ascending-arch angle narrowing (117 ± 13° in tricuspid, 115 ± 14° in bicuspid) compared to non-dilated and aneurysmal aortas (all P < 0.001). The best angle cut-off to discriminate dissection patients was 131° (96% sensitivity). In patients with a pre-dissection scan, Asc-Arch narrowing was already present before dissection. In non-dissected aortas over-angulation was predicted by root phenotype dilatation both in bicuspid and tricuspid patients. Bicuspid patients with non-dilated aorta showed elongated root (P = 0.027), a feature significantly correlated with Asc-Arch angle narrowing (P = 0.008). CONCLUSIONS: The ascending-arch angle is promising as a risk marker for dissection to be used along with diameter. Its narrowing seems to be associated with elongation of the root, a feature that bicuspid patients can show even without significant dilatation. Root phenotype aortopathy may be at higher risk also with tricuspid aortic valve.

Prognostic role of endocarditis in isolated tricuspid valve surgery. A propensity-weighted study.

OBJECTIVES: The role of the underlying etiology in isolated tricuspid valve surgery has not been investigated extensively in current literature. Aim of this study was to analyse outcomes of patients undergoing surgery due to endocarditis compared to other pathologies. METHODS: The SURTRI study is a multicenter study enrolling adult patients who underwent isolated tricuspid valve surgery (n = 406, 55 ± 16 y.o.; 56% female) at 13 international sites. Propensity weighted analysis was performed to compare groups (IE group n = 107 vs Not-IE group n = 299). RESULTS: No difference was found regarding the 30-day mortality (Group IE: 2.8% vs Group Not-IE = 6.8%; OR = 0.45) and major adverse events. Weighted cumulative incidence of cardiac death was significantly higher for patients with endocarditis (p = 0.01). The composite endpoint of cardiac death and reoperation at 6 years was reduced in the Group IE (63.2 ± 6.8% vs 78.9 ± 3.1%; p = 0.022). Repair strategy resulted in an increased late survival even in IE cases. CONCLUSIONS: Data from SURTRI study report acceptable 30-day results but significantly reduced late survival in the setting of endocarditis of the tricuspid valve. Multi-disciplinary approach, repair strategy and earlier treatment may improve outcomes.

Survival and Recurrence of Endocarditis following Mechanical vs. Biological Aortic Valve Replacement for Endocarditis in Patients Aged 40 to 65 Years: Data from the INFECT-Registry.

BACKGROUND: Infective endocarditis (IE) is a serious disease, and in many cases, surgery is necessary. Whether the type of prosthesis implanted for aortic valve replacement (AVR) for IE impacts patient survival is a matter of debate. The aim of the present study is to quantify differences in long-term survival and recurrence of endocarditis AVR for IE according to prosthesis type among patients aged 40 to 65 years. METHODS: This was an analysis of the INFECT-REGISTRY. Trends in proportion to the use of mechanical prostheses versus biological ones over time were tested by applying the sieve bootstrapped t-test. Confounders were adjusted using the optimal full-matching propensity score. The difference in overall survival was compared using the Cox model, whereas the differences in recurrence of endocarditis were evaluated using the Gray test. RESULTS: Overall, 4365 patients were diagnosed and operated on for IE from 2000 to 2021. Of these, 549, aged between 40 and 65 years, underwent AVR. A total of 268 (48.8%) received mechanical prostheses, and 281 (51.2%) received biological ones. A significant trend in the reduction of implantation of mechanical vs. biological prostheses was observed during the study period (p < 0.0001). Long-term survival was significantly higher among patients receiving a mechanical prosthesis than those receiving a biological prosthesis (hazard ratio [HR] 0.546, 95% CI: 0.322-0.926, p = 0.025). Mechanical prostheses were associated with significantly less recurrent endocarditis after AVR than biological prostheses (HR 0.268, 95%CI: 0.077-0.933, p = 0.039). CONCLUSIONS: The present analysis of the INFECT-REGISTRY shows increased survival and reduced recurrence of endocarditis after a mechanical aortic valve prosthesis implant for IE in middle-aged patients.

[Bicuspid aortic valve and its complications: from molecules to surgery]. / La bicuspidia aortica e le sue complicanze: dalle molecole al bisturi.

Bicuspid aortic valve is a relevant condition to the epidemiology of degenerative (stenosis, regurgitation) and infective (endocarditis) aortic valve disease. This review will address the topics of treatment of those valvular complications, focusing on the peculiar aspects distinguishing them from tricuspid aortic valve diseases, in particular transcatheter treatment of stenosis, valve repair/sparing surgery for regurgitation, prevention of endocarditis. Bicuspid aortopathy represents a clinical entity with remarkable prognostic, and likely also pathogenetic, heterogeneity. A deeper understanding of the tissue and molecular factors as well as flow-related and biomechanical aspects of its development and progression could help guiding better risk stratification (identification of more severe forms, prevention of acute aortic dissection), allowing for a personalized therapeutic approach. The present review will also summarize the state of the art about bicuspid aortopathy pathogenesis, moreover forwarding new hypotheses for the interpretation of the many sparse pieces of evidence currently available.

Outcome of patients undergoing isolated tricuspid repair or replacement surgery.

OBJECTIVES: The interest in isolated tricuspid valve disease has rapidly increased recently. However, clinical trials and registry data are rare in the surgical literature. This study aimed to describe the early and long-term outcomes of a real-world experience in isolated tricuspid procedures comparing repair and replacement strategies. METHODS: The Surgical-Tricuspid study is a multicentre retrospective study that enrolled adult patients who had undergone isolated tricuspid valve surgery at 13 international sites. Propensity score-matched analysis was used to compare repair versus replacement. RESULTS: A cohort of 426 patients was enrolled [mean age: 55 (16) years; 56% female]. After matching, 175 comparable pairs were analysed. Preoperative left ventricular ejection fraction was 55(9) vs 56(9) (P = 0.8) while moderate-severe tricuspid regurgitation was present in 95% of cases. The 30-day mortality rate was 4.0% vs 8.0% in the repair and replacement groups, respectively (P = 0.115). The rates of re-exploration for bleeding (6.9% vs 13.1% P = 0.050), permanent pacemaker implantation (5.1% vs 12.0%; P = 0.022) and blood transfusion (46% vs 62%; P = 0.002) were higher in the replacement group. Cumulative survival rates at 3, 5 and 7 years in the repair group were 84 (3)%, 75 (4)% and 56 (9)% vs 71 (4)%, 66 (5)% and 58 (5)% in the replacement group (P = 0.001) while cumulative incidence for reoperation at 10 years did not differ between groups [repair 10 (1)% vs replacement 9 (1)%; P = 0.469]. CONCLUSIONS: The data from the Surgical-Tricuspid study reported a high risk for patients undergoing tricuspid surgery. Isolated valve repair offered reduced early and late mortality with no difference regarding reoperation rate when compared with replacement.

Observed versus predicted mortality after isolated tricuspid valve surgery.

BACKGROUND: Aim of this study is to analyse the performances of Clinical Risk Score (CRS) and European System for Cardiac Operative Risk Evaluation (EuroSCORE)-II in isolated tricuspid surgery. METHODS: Three hundred and eighty-three patients (54 ± 16 year; 54% female) were enrolled. Receiver operating characteristic analysis was performed to evaluate the relationship between the true positive fraction of test results and the false-positive fraction for a procedure. RESULTS: Considering the 30-day mortality the area under the curve was 0.6 (95% confidence interval [CI] 0.50-0.72) for EuroSCORE II and 0.7 (95% CI 0.56-0.84) for CRS-score. The ratio of expected/observed mortality showed underestimation when considering EuroSCORE-II (min. 0.46-max. 0.6). At multivariate analysis, the CRS score (p = .005) was predictor of late cardiac death. CONCLUSION: We suggest using both scores to obtain a range of expected mortality. CRS to speculate on late survival.