Marfan and Loeys-Dietz aortic phenotype: A potential tool for diagnosis and management.

Objective: In heritable aortic diseases, different vascular involvement may occur with potential variable implications in aortic dilation/dissection risk. This study aimed to analyze the aortic anatomy of individuals with Marfan syndrome and Loeys-Dietz syndrome to identify possible morphological differences. Methods: Computed tomography and magnetic resonance imaging of the thoracoabdominal aorta from the proximal supra-aortic vessels to the femoral bifurcation level of 114 patients with Marfan and Loeys-Dietz syndromes and 20 matched control subjects were examined. Aortic diameters, areas, length, and tortuosity were measured in different aortic segments using specific vessel analysis software. Results: Patients with Marfan syndrome showed a higher prevalence of ascending aorta and aortic root dilation (P = .011), larger and longer aortic roots (P = .013) with pear-shaped phenotype, larger isthmus/descending aorta diameter ratio (P = .015), and larger suprarenal aorta and iliac arteries. Patients with Loeys-Dietz syndrome showed longer indexed segments and a significantly longer arch (P = .006) with type 2/3 arch prevalence (P = .097). Measurement ratios analysis provided cut-off values (aortic root to ascending aorta length/aortic root diameter, aortic root/sinotubular junction, aortic root/ascending aorta diameter) differentiating patients with Marfan syndrome from patients with Loeys-Dietz syndrome, even in the early stage of the disease. Conclusions: Both syndromes show peculiar anatomic patterns at different aortic levels irrespective of aortic dilation and disease severity. These features may represent the expression of different genetic mutations on aortic development, with a potential impact on prognosis and possibly contributing to better management of the diseases. The systematic adoption of whole body imaging with magnetic resonance or computed tomography should always be considered, because they allow a complete vascular assessment with practical indicators of differential diagnosis.

Devices for thoracic endovascular aortic repair of type B aortic dissection: is there any chance for Marfan syndrome?

INTRODUCTION: Type B aortic dissection (TBAD) is a threatening event that may lead to death for aortic rupture or multivisceral malperfusion in the acute phase and offers a poor prognosis for long term survivors. Thoracic endovascular aortic repair (TEVAR) has become the preferential therapy in acute and chronic complicated cases for its less invasivity compared to open surgery. However TEVAR is still encumbered by a significant number of reinterventions, caused by aortic neck aneurysmal degeneration, endoleaks and stent-graft induced new entry (SINE). This is even more true in patients with particularly fragile aortic wall like Marfan Syndrome (MS) in contrast to the excellent results of elective open surgery. AREAS COVERED: This review analyzes the current available TEVAR devices and techniques in TBAD and their technological advancements, especially those most suitable to TBAD anatomy, according to current literature. EXPERT OPINION: The continuous technological evolution of materials and device solutions ensures solid results of TEVAR in acute TBAD and may also potentially overcome some of the limitations of endovascular devices in this setting, paving the way for safer and more durable results and allowing for expanded indications in the future, even in the delicate population of collagenopathies, especially in MS.

Intramural Hematoma as Unexpected Complication of COVID-19 Infection.

Novel coronavirus disease-2019 (COVID-19) is an ominous infectious disease that seems capable to attack any organ system, leading in the most severe cases to patient death. COVID-19 has been associated with multiple cardiovascular complications of inflammatory and immune origin, leading to a wide spectrum of vascular damage, myocardial injury, stroke, and pulmonary obstruction. We report the case of a patient with COVID-19 infection who developed an acute aortic syndrome with the characteristics of aortic intramural hematoma.

The Clinical Impact of Imaging Surveillance and Clinic Visit Frequency after Acute Aortic Dissection.

BACKGROUND: Guidelines recommend frequent follow-up after acute aortic dissection (AAD), but optimal rates of follow-up are not clear. METHODS: We examined rates of imaging and clinic visits in 267 individuals surviving AAD during recommended intervals (≤1, > 1-3, > 3-6, > 6-12 months, then annually), frequency of adverse imaging findings, and the relationship between follow-up and mortality. RESULTS: Type A and B AAD were noted in 46 and 54% of patients, respectively. Mean follow-up was 54.7 ± 13.3 months, with 52 deaths. Adverse imaging findings peaked at 6 to 12 months (5.6%), but rarely resulted in an intervention (3.4% peak at 6-12 months). Compared with those with less frequent imaging, patients with imaging for 33 to 66% of intervals (p = 0.22) or ≥66% of intervals (p = 0.77) had similar adjusted survival. In comparison to patients with fewer clinic visits, those with visits in 33 to 66% of intervals experienced lower adjusted mortality (hazards ratio: 0.47, 95% confidence interval: 0.23-0.97, p = 0.04), with no difference seen in those with ≥66% (vs. < 33%) interval visits (p = 0.47). Imaging at 6 to 12 months (vs. none) was associated with decreased adjusted mortality (hazards ratio: 0.50, 95% confidence interval: 0.27-0.91, p = 0.02), while imaging during other intervals, or clinic visits during any specific intervals, was not associated with a difference in mortality (p > 0.05 for each). CONCLUSIONS: Adverse imaging findings following AAD are common, but rarely require prompt intervention. Patients with the lowest and highest rates of clinic visits experienced increased mortality. While the overall rate of surveillance imaging did not correlate with mortality, adverse imaging findings and related interventions peaked at 6 to 12 months after AAD, and imaging during this time was associated with improved survival.

One-Year Clinical Outcomes of Forty-Eight Millimeter Everolimus-Eluting Stent Implanted in Very Long Lesions: A Propensity-Matched Comparison (The FREIUS Study).

BACKGROUND: Long coronary lesions still remain a challenge, with poor immediate results and suboptimal outcomes when compared to class A/B1 lesions. The presence of overlapped segments of metal struts and polymer might trigger an abnormal inflammatory reaction, resulting in a higher restenosis rate. The aim of our study was to evaluate the safety, feasibility, and cost effectiveness of a 48 mm everolimus-eluting stent (EES) during treatment of very long coronary lesions. METHODS AND RESULTS: The FREIUS study is a prospective data collection of consecutive patients undergoing 48 mm EES implantation in six high-volume European centers. Each patient was matched through a propensity score to a comparable patient treated with two or more second-generation overlapped drug-eluting stents. The primary endpoint was the combined incidence of cardiac death, target-vessel myocardial infarction, and target-lesion revascularization (device-oriented composite endpoint [DOCE]). The secondary endpoints were all-cause death, each individual component of the primary endpoint, and definite/probable stent thrombosis. From January 2014 to April 2015, a total of 218 patients were treated with at least one 48 mm EES and were compared with 218 matched controls. Overall, 9% of patients reached the primary endpoint. Cumulative survival free from DOCE incidence did not differ between the two groups (7% in the cases vs 10.5% in the controls; P=.10). After multivariable analysis, only clinical presentation with myocardial infarction (hazard ratio [HR], 1.8; 95% confidence interval [CI], 1.5-2.1; P=.01) and stent number (HR, 1.4; 95% CI, 1.1-1.8; P=.02) emerged as independent predictors of DOCE. CONCLUSION: The use of 48 mm EES offers a safe and effective strategy for the treatment of very long coronary lesions.

Thoracic endovascular repair versus medical management for acute uncomplicated type B aortic dissection.

BACKGROUND: Current treatment options and outcomes for acute uncomplicated thoracic Type-B aortic dissection (TBAD) remain unclear between medical management (MED) and thoracic endovascular aortic repair (TEVAR). In this study we aim to compare both strategies in terms of all-cause mortality, aortic dilation, and aortic rupture. METHODS: MEDLINE, EMBASE, and the Cochrane Central Register of Controlled Trials were queried from January 1990 through March 2017. Only studies comparing TEVAR to MED for acute uncomplicated TBAD were included. Random-effects meta-analysis was used to pool outcomes across studies. Study outcomes included short (1 month), intermediate (1 year), and mid-term (2-5 year) all-cause mortality. Additional outcomes included aortic dilation and rupture at 1 year. RESULTS: A total of 1,960 patients (64.3 years; 75.8% male) were included from six studies (one prospective and five retrospective). No difference was observed in short-term (odd ratio [OR] 0.73 with 95% confidence interval [CI] 0.47 to 1.12, P = 0.15), intermediate (OR 0.99 with 95% CI 0.56 to 1.73, P = 0.96), or mid-term all-cause mortality (OR 1.12 with 95% CI 0.54 to 2.32, P = 0.75). No difference in aortic dilation with either modality was noted at 1-year (OR 1.11 with 95% CI 0.76 to 1.64, P = 0.59). TEVAR was associated with a significantly lower 1-year risk of aortic rupture (OR 2.49 with 95% CI 1.23 to 5.06, P = 0.01). CONCLUSION: There were no short, intermediate, or mid-term differences in mortality between TEVAR or MED in patients with acute uncomplicated TBAD. Although the dilation rate was similar between both groups, TEVAR was associated with lower likelihood of aortic rupture at 1 year.

Extended versus limited arch replacement in acute Type A aortic dissection.

OBJECTIVES: The recommended extent of surgical resection and reconstruction of the arch in acute DeBakey Type I aortic dissection is an ongoing controversy. However, several recent reports indicate a trend towards a more extensive arch operation in several institutions. We have analysed the recent data from the International Registry of Acute Aortic Dissection to assess the choice of procedure over time and to evaluate the surgical outcome in a 'real-world' database. Our aim was to compare short- and mid-term outcomes of limited repairs versus complete arch surgery. METHODS: Of the 1241 patients included in the 'Interventional Cohort' of the International Registry of Acute Aortic Dissection from March 1996 to March 2015, 907 underwent ascending aortic or hemiarch replacement (Group A) and 334 had extended arch replacement (Group B). An extended resection was a surgeon's 'judgement call'. Logistic regression analysis, propensity-adjusted multivariable comparisons and Kaplan-Meier curves were used for analyses. RESULTS: Overall in-hospital mortality was 14.2% with no difference between groups (Group A 13.1%, Group B 17.1%). Coma/altered consciousness (odds ratio 3.16, 95% confidence interval 1.60-6.25, P = 0.001), hypotension, tamponade or shock (2.03, 1.11-3.73, P = 0.022) and any pulse deficit (1.92, 1.04-3.54, P = 0.038) were predictors of in-hospital mortality in a propensity score-adjusted multivariable analysis. Overall 5-year survival was 69.4% in the ascending group and 73.1% in the total arch group (P = 0.83 by Kaplan-Meier analysis). For survivors of the index hospitalization, the 5-year freedom from death, aortic rupture and reintervention were 71.1% in Group A and 76.4% in Group B (P = 0.54 by Kaplan-Meier analysis). CONCLUSIONS: Selective, or 'surgeon's choice', extended arch replacement had no discernible acute downside compared with less extensive surgery. Whether extended arch replacement improves the prognosis beyond 5 years remains to be settled.

Polymer-free amphilimus-eluting stent versus biodegradable polymer biolimus-eluting stent in patients with and without diabetes mellitus.

OBJECTIVES: This study sought to compare clinical outcome of polymer-free amphilimus-eluting stent (PF-AES) versus biodegradable-polymer biolimus-eluting stent (BD-BES) in "all-comer" diabetes mellitus (DM) and non-DM patients who underwent percutaneous coronary intervention. BACKGROUND: The PF-AES has shown promising preliminary results in patients with DM. METHODS: Data from 2 multicentre-national registries (the ASTUTE and the INSPIRE-1) were used to analyse 1776 patients stratified in non-DM and DM. A double 1:1 propensity-score matched analysis (PF-AES vs. BD-BES) was performed in each group to adjust for clinical and procedural characteristics. Primary stent-efficacy and stent-safety endpoints were 1-year target-lesion revascularization (TLR) and target-lesion failure (TLF, composed of cardiac-death, target-vessel myocardial infarction and any TLR). RESULTS: After propensity-score matching, 850 patients were stratified as non-DM (425 PF-AES/425 BD-BES) and 480 as DM patients (240 PF-AES/240 BD-BES). Both TLF (20 of 425 [5%] vs. 24 of 425 [6%]; Plog-rank=0.527) and TLR (9 of 425 [2%] vs. 18 of 425 [4%]; Plog-rank=0.079) were similar between PF-AES and BD-BES in non-DM patients. In DM, TLF (12 of 240 [5%] vs. 31 of 240 [13%]; Plog-rank=0.002) and TLR (9 of 240 [4%] vs. 21 of 240 [9%]; Plog-rank=0.019) were significantly lower in PF-AES compared to BD-BES. Upon multivariate analysis, the most powerful predictors of TLF were chronic kidney disease in non-DM (OR 4.24, 95% CI: 2.07-8.70, p<0.001) and stent type in DM patients (OR 2.76, 1.36-5.56, p=0.005). CONCLUSIONS: This matched-cohort study suggests that PF-AES has better safety and efficacy profile than BD-BES in patients with DM.

Utility of stress perfusion-cardiac magnetic resonance in follow-up of patients undergoing percutaneous coronary interventions of the left main coronary artery.

To assess the accuracy of cardiac magnetic resonance (CMR) for the diagnosis of angiographic stenosis after percutaneous coronary intervention (PCI) of left main coronary artery (LMCA). Patients undergone in the last year PCI of unprotected LMCA and scheduled for conventional X-ray coronary angiography (CXA) were evaluated with stress perfusion CMR within 2 weeks before CXA. Main contraindications to CMR were exclusion criteria. Stress perfusion CMR was performed to follow a bolus of contrast Gadobutrol after 3 min of adenosine infusion. Between the 50 patients enrolled, only 1 did not finish the CMR protocol and 49 patients with median age 71 (65-75) years (38 male, 11 female) were analyzed. Between 784 coronary angiographic segments evaluated we found 75 stenosis or occlusions (prevalence 9.5%), but only 13 stenosis or occlusions in proximal segments (prevalence 6.6%). Patients with coronary stenosis (n = 12, 24%) showed a significantly (p = 0.002) higher prevalence of diabetes (7 of 12, 58%). At CMR examination, late gadolinium enhancement was present in 25 (51%), reversible perfusion defects in 12 (24%), and fixed perfusion defects in 6 subjects (12%). The only patient with LMCA restenosis resulted positive at perfusion CMR. The accuracy of stress perfusion CMR in diagnosis of coronary stenosis was higher when the analysis was performed only in proximal coronary arteries (95%, CI 86-99) compared to overall vessels (84%, CI 70-92). Stress perfusion CMR could strongly reduce the need for elective CXA in follow up of LMCA PCI and should be validated in further multicenter prospective studies.

Safety and efficacy of drug eluting stents in patients with spontaneous coronary artery dissection.

AIMS: Given the different pathogenesis, use of drug eluting stent (DES) in patients with Spontaneous Coronary Artery Dissection SCAD may delay the healing of the dissected vessel. Aim of our study was to compare the safety and the efficacy of DES vs. bare metal stent (BMS) in a cohort of patients who underwent stenting for SCAD. METHODS AND RESULTS: Consecutive patients with SCAD between January 1995 and August 2014 were retrospectively identified in 12 centers and included. Major Adverse Cardiac Events (MACE) was the primary end point. A total of 238 SCAD patients were identified: of them 108 patients underwent PCI with DES or BMS. Overall 24 patients (22.2%) suffered an intra-procedural complication without any differences between the 2 groups. At median follow-up of 1201days (Inter Quartile Range 541-2760), incidence of the primary endpoint showed a trend towards less events in the DES-treated patients (38.7% vs. 25.9% p=0.14) mainly driven by the benefit of DES in terms of TVR (17.6% vs. 4%, p=0.08), mortality (16.8% vs. 9.3%, p=0.4), and MI rate (16% vs. 8.4%, p=0.33). STEMI at presentation (HR 6.4, CI 95% 1.29-31.9, p=0.02) but not kind of stent (HR 0.97, CI 95% 0.2-4.7, p=0.9) emerged as independently related to prognosis at multivariable analysis. CONCLUSIONS: In SCAD patients use of DES seems to be as safe as BMS with trend of better efficacy in the long term.

Midterm and one-year outcome of amphilimus polymer free drug eluting stent in patients needing short dual antiplatelet therapy. Insight from the ASTUTE registry (AmphilimuS iTalian mUlticenTer rEgistry).

BACKGROUND: To assess clinical outcomes of patients needing short dual antiplatelet therapy (S-DAPT) after PCI with Cre8 polymer-free amphilimus eluting-stent (AES). The Cre8-AES with pure i-Carbofilm coating was supposed to induce faster stent endothelialization and reduce device thrombogenicity. METHODS: We performed a sub-analysis of unrestricted consecutive patients treated with Cre8-AES between August 2011 and January 2015. Two groups were formed: 1) patients discharged with S-DAPT (≤3-month), because of high bleeding risk or attending urgent non-cardiac surgery; and 2) patients discharged with Recommended DAPT duration (R-DAPT; ≥6-month). The primary ischemic- and bleeding-safety endpoints were Target Vessel Failure (TVF, composite endpoint of cardiac-death, target vessel-myocardial infarction and target vessel-revascularization), and major-bleeding (BARC ≥type-3a) at 6-month and 1-year. RESULTS: 106 patients (8.7%) were discharged with ≤3-month DAPT (83±19days; S-DAPT group) and 1102 patients (90.6%) with ≥6-month DAPT (342±62days; R-DAPT group). Between S-DAPT and R-DAPT groups no significant differences were observed in TVF at 1-year (5.7% vs 5.1%); 1-year BARC major bleeding rate was higher in S-DAPT group (3.4% vs 0.2%, p=0.007) with all bleeding events occurred within 3months. The landmark analysis (started at 90days, ended at 1year) showed no differences in BARC major bleedings between groups (0% vs. 0.3%). CONCLUSIONS: The results of this multicenter registry show that the use of Cre8 AES in patients needing short DAPT (≤3-month) was safe regarding ischemic events and could favor a reduction of bleeding events related to the recommended DAPT. A large randomized trial is necessary to support these preliminary findings.

The Role of Cardiac Magnetic Resonance Imaging in Hypertrophic Cardiomyopathy.

Until recently, the only imaging technique for the diagnosis and management of hypertrophic cardiomyopathy (HCM) was two-dimensional echocardiography, and the use of cardiac magnetic resonance imaging (cMRI) was limited to patients with poor acoustic windows. Now, cMRI has gained an essential role in the diagnosis of HCM, providing superior visualization of myocardial hypertrophy-even in remote zones of the left ventricle-and visualization of subtle changes in thickness and contractility over time. The morphologic accuracy of cMRI allows for the differentiation of HCM from other pathologic conditions with hypertrophic phenotype. Moreover, cMRI sheds light on the in vivo fibrotic changes in cardiac ultrastructure, offering an important advantage in the understanding of pathologic mechanisms of the disease, allowing early identification, risk stratification, and timely therapeutic management.

IRAD experience on surgical type A acute dissection patients: results and predictors of mortality.

Type A acute aortic dissection (TAAD) is a disease that has a catastrophic impact on a patient's life and emergent surgery represents a key goal of early treatment. Despite continuous improvements in imaging techniques, medical therapy and surgical management, early mortality in patients undergoing TAAD repair still remains high, ranging from 17% to 26%. In this setting, the International Registry of Acute Aortic Dissection (IRAD), the largest worldwide registry for acute aortic dissection, was established to assess clinical characteristics, management and outcomes of TAAD patients. The present review aimed to evaluate and comment on outcomes of TAAD surgery as reported from IRAD series.

One-year clinical outcome of amphilimus polymer-free drug-eluting stent in diabetes mellitus patients: Insight from the ASTUTE registry (AmphilimuS iTalian mUlticenTre rEgistry).

BACKGROUND: Despite several improvements in drug-eluting stent (DES) technology, patients with diabetes mellitus (DM) are affected by higher rate of adverse events after PCI with DES, mainly in terms of target lesion revascularization (TLR). The Cre8 stent, a polymer-free amphilimus-eluting stent (AES), has shown promising preliminary results in DM patients. The ASTUTE registry was conceived to assess clinical performance of this polymer-free AES in DM in a "real-world" multicenter-independent cohort of patients. METHODS: 1216 consecutive patients (1637 lesions) treated with Cre8 between August 2011 and January 2015 were retrospectively enrolled and divided in two groups: DM and non-DM patients. The primary and secondary endpoints were target lesion failure (TLF) and TLR at 1-year, respectively. RESULTS: 1-Year clinical outcome was available for 407 (80%) DM and 566 (84%) non-DM patients. Diabetic patients were more frequently affected by hypertension (p<0.001) and dyslipidemia (p<0.001) and more frequently dialyzed (p=0.010). At 1-year, TLF occurred in 50 (5.1%) overall patients, and was similar in DM and non-DM patients (4.9 vs 5.3%, respectively, p=0.788). The secondary endpoint (TLR) occurred in 3% of overall patients and similarly in DM and non-DM patients (3.7 vs 2.5%, respectively, p=0.273). Primary and secondary endpoints were similar also between insulin and non-insulin dependent DM patients. CONCLUSIONS: The results of this large, real-world, multicenter-independent registry show that Cre8 AES has similar safety and efficacy profile in DM as well as non-DM patients, reducing the adverse outcomes related to DM in patients undergoing PCI. A large randomized trial will be necessary to definitely prove these preliminary findings.

Time-related changes in neointimal tissue coverage of a novel Sirolimus eluting stent: Serial observations with optical coherence tomography.

BACKGROUND: DES has reduced rates of restenosis compared with BMS but it has been associated with delayed healing and increase of stent thrombosis. The aim of our study was to evaluate the vascular time-related changes following implantation of a new SES coated with an amorphous silicon carbide that allows faster re-endothelisation (Orsiro-Biotronik). METHODS: This prospective registry enrolled STEMI-patients with multi-vessel disease, thus candidates for a two-step procedure. PCI of the culprit lesion was performed with at least one Orsiro stent that was OCT-analysed during the second-step procedure (deferred to 30,90 and 180-days). RESULTS: 16 of the 95 patients with MVD underwent an OCT evaluation of the study device implanted in the culprit lesion during the second-step staged procedure and were enrolled in the present Registry. A total of 3060-struts were analysed. The percentage of uncovered struts was 19.6% at30-days, 1.3% at90-days and 1.8% at180-days (p<0.001). The percentage of cross section with ≥1 uncovered struts were 51.3% at30-days, 6.5% at90-days and 5.7% at180-days (p<0.001). The percentage of cross sections containing thrombus was 6.2% at30-days while no thrombus was detected both at90 and 180-days. CONCLUSIONS: Our data shows that the new Orsiro stent promotes early and persistent strut coverage with low peri-strut thrombus. This pilot OCT evaluation might suggest a low incidence of late adverse events and anticipate safe outcome after early withdrawal of dual antiplatelet therapy.

Current challenges in endovascular therapy for thoracic aneurysms.

Surgical treatment of thoracic aortic aneurysms can be associated with high mortality and complication rates, resulting in prolonged hospital stay. Endovascular techniques for treating thoracic aortic disease have been rapidly embraced, showing limited mortality and major adverse events. Its wide availability and relative ease of application has changed and extended management options for thoracic aortic disease, including inpatients deemed unsuitable for open surgery. Rapid technical evolution has allowed it to be applied to even hostile anatomy and complex cases. However, as it is still a young technology, many unknowns remain, including long-term outcomes with respect to conservative treatment and open surgical repair.

Very high-pressure dilatation for undilatable coronary lesions: indications and results with a new dedicated balloon.

AIMS: Calcific coronary lesions impose a rigid obstacle to optimal balloon and stent expansion and the 20 to 30 atm limit that non-compliant (NC) balloons reach can be insufficient. The aim of our study was to evaluate the safety and efficacy of a new dedicated super high-pressure NC balloon (OPN NC®; SIS Medical AG, Winterthur, Switzerland). METHODS AND RESULTS: We retrospectively evaluated a consecutive series of 91 lesions in which conventional NC balloons at maximal pressure failed to achieve an adequate post-dilatation luminal gain and were therefore treated with an OPN NC balloon up to 40 atm. Angiographic success was defined as residual angiographic diameter stenosis <30%. MLD and %DS were measured at baseline, after NC balloon, OPN NC balloon and stent implantation. Angiographic success was achieved in 84 lesions (92.3%). All of the remaining lesions received rotational atherectomy with the exception of two cases in which rotational atherectomy was not attempted because of small vessel size and excessive tortuosity. MLD and acute gain were significantly greater and %DS was significantly lower post OPN NC balloon compared with conventional NC balloon inflation (p<0.001). No coronary perforations occurred. No acute or 30-day follow-up MACE was reported. CONCLUSIONS: When conventional NC balloons fail, the new OPN NC dedicated high-pressure balloon provides an effective and safe alternative strategy for the dilatation of resistant coronary lesions.