Mapping and Ablation of Premature Ventricular Complexes: State of the Art.

Premature ventricular complexes (PVCs) are common arrhythmias in clinical practice. Although benign and asymptomatic in most cases, PVCs may result in disabling symptoms, left ventricular systolic dysfunction, or PVC-induced ventricular fibrillation. Catheter ablation has emerged as a first-line therapy in such cases, with high rates of efficacy and low risk of complications. Significant progress in mapping and ablation technology has been made in the past 2 decades, along with the development of a growing body of knowledge and accumulated experience regarding PVC sites of origin, anatomical relationships, electrocardiographic characterization, and mapping/ablation strategies. This paper provides an overview of the main indications for catheter ablation of PVCs, electrocardiographic features, PVC mapping techniques, and contemporary ablation approaches. The authors also review the most common sites of PVC origin and the main considerations and challenges with ablation in each location.

Predicted Deleterious Variants in Cardiomyopathy Genes Prognosticate Mortality and Composite Outcomes in UK Biobank.

BACKGROUND: Inherited cardiomyopathies present with broad variation of phenotype. Data are limited regarding genetic screening strategies and outcomes associated with predicted deleterious variants in cardiomyopathy-associated genes in the general population. OBJECTIVES: The authors aimed to determine the risk of mortality and composite cardiomyopathy-related outcomes associated with predicted deleterious variants in cardiomyopathy-associated genes in the UK Biobank. METHODS: Using whole exome sequencing data, variants in dilated, hypertrophic, and arrhythmogenic right ventricular cardiomyopathy-associated genes with at least moderate evidence of disease causality according to ClinGen Expert Panel curations were annotated using REVEL (≥0.65) and ANNOVAR (predicted loss-of-function) considering gene-disease mechanisms. Genotype-positive and genotype-negative groups were compared using time-to-event analyses for the primary (all-cause mortality) and secondary outcomes (diagnosis of cardiomyopathy; composite outcome of diagnosis of cardiomyopathy, heart failure, arrhythmia, stroke, and death). RESULTS: Among 200,619 participants (age at recruitment 56.46 ± 8.1 years), 5,292 (2.64%) were found to host ≥1 predicted deleterious variants in cardiomyopathy-associated genes (CMP-G+). After adjusting for age and sex, CMP-G+ individuals had higher risk for all-cause mortality (HR: 1.13 [95% CI: 1.01-1.25]; P = 0.027), increased risk for being diagnosed with cardiomyopathy later in life (HR: 5.75 [95% CI: 4.58-7.23]; P < 0.0001), and elevated risk for composite outcome (HR: 1.29 [95% CI: 1.20-1.39]; P < 0.0001) than CMP-G- individuals. The higher risk for being diagnosed with cardiomyopathy and composite outcomes in the genotype-positive subjects remained consistent across all cardiomyopathy subgroups. CONCLUSIONS: Adults with predicted deleterious variants in cardiomyopathy-associated genes exhibited a slightly higher risk of mortality and a significantly increased risk of developing cardiomyopathy, and cardiomyopathy-related composite outcomes, in comparison with genotype-negative controls.

Catheter ablation of intramural outflow tract premature ventricular complexes: a multicentre study.

AIMS: Ablation of outflow tract ventricular arrhythmias may be limited by a deep intramural location of the arrhythmogenic source. This study evaluates the acute and long-term outcomes of patients undergoing ablation of intramural outflow tract premature ventricular complexes (PVCs). METHODS AND RESULTS: This multicenter series included patients with structurally normal heart or nonischemic cardiomyopathy and intramural outflow tract PVCs defined by: (a) ≥ 2 of the following criteria: (1) earliest endocardial or epicardial activation < 20ms pre-QRS; (2) Similar activation in different chambers; (3) no/transient PVC suppression with ablation at earliest endocardial/epicardial site; or (b) earliest ventricular activation recorded in a septal coronary vein. Ninety-two patients were included, with a mean PVC burden of 21.5±10.9%. Twenty-six patients had had previous ablations. All PVCs had inferior axis, with LBBB pattern in 68%. In 29 patients (32%) direct mapping of the intramural septum was performed using an insulated wire or multielectrode catheter, and in 13 of these cases the earliest activation was recorded within a septal vein. Most patients required special ablation techniques (one or more), including sequential unipolar ablation in 73%, low-ionic irrigation in 26%, bipolar ablation in 15% and ethanol ablation in 1%. Acute PVC suppression was achieved in 75% of patients. Following the procedure, the PVC burden was reduced to 5.8±8.4%. The mean follow-up was 15±14 months and 16 patients underwent a repeat ablation. CONCLUSION: Ablation of intramural PVCs is challenging; acute arrhythmia elimination is achieved in 3/4 patients, and non-conventional approaches are often necessary for success.

Stereotactic Arrhythmia Radioablation Treatment of Ventricular Tachycardia: Current Technology and Evolving Indications.

Ventricular tachycardia in patients with structural heart disease is a significant cause of morbidity and mortality. According to current guidelines, cardioverter defibrillator implantation, antiarrhythmic drugs, and catheter ablation are established therapies in the management of ventricular arrhythmias but their efficacy is limited in some cases. Sustained ventricular tachycardia can be terminated by cardioverter-defibrillator therapies although shocks in particular have been demonstrated to increase mortality and worsen patients' quality of life. Antiarrhythmic drugs have important side effects and relatively low efficacy, while catheter ablation, even if it is actually an established treatment, is an invasive procedure with intrinsic procedural risks and is frequently affected by patients' hemodynamic instability. Stereotactic arrhythmia radioablation for ventricular arrhythmias was developed as bail-out therapy in patients unresponsive to traditional treatments. Radiotherapy has been mainly applied in the oncological field, but new current perspectives have developed in the field of ventricular arrhythmias. Stereotactic arrhythmia radioablation provides an alternative non-invasive and painless therapeutic strategy for the treatment of previously detected cardiac arrhythmic substrate by three-dimensional intracardiac mapping or different tools. Since preliminary experiences have been reported, several retrospective studies, registries, and case reports have been published in the literature. Although, for now, stereotactic arrhythmia radioablation is considered an alternative palliative treatment for patients with refractory ventricular tachycardia and no other therapeutic options, this research field is currently extremely promising.

Pacing maneuvers from the aortic cusps: A useful technique during ablation of focal arrhythmias arising from the aortic root.

BACKGROUND: The ablation of arrhythmias arising near the His-bundle region in the non-coronary aortic cusp (NCAC) is challenging. Among the aortic sinuses of Valsalva, the NCAC is located between the right atrium and the left atrium. For this reason, pacing from the NCAC should result in atrial capture while pacing from the right and left coronary cusps (LCC) may result in ventricular capture. The objective of this study is to prove that atrial capture during pacing from the sinuses of Valsalva may be used to confirm a correct position in the non-coronary cusp. METHODS: A total of 30 patients with structurally normal heart undergoing left sided ablation procedures, underwent electrophysiological study of the aortic cusps. Each of the aortic valve cusps was paced with increasing outputs and analyzed offline to determine their unique electrocardiographic characteristics. RESULTS: In the NCAC, we obtained an atrial capture in 29 out of 30 patients (96.6%). The atrial capture was highly specific for a position in the NCAC (p < .001). The median output required to capture from the cusp was 10 mA (range 5-25 mA) at a pulse width of 2 ms. In the right coronary cusp (RCC) and LCC, we obtained ventricular capture in all patients (100%). The median output required to capture from the cusp was 10 mA (range 10-25 mA) in RCC and 25 mA (range 10-25 mA) in LCC at a pulse width of 2 ms. CONCLUSIONS: Atrial capture during a pacemapping from the aortic cusps can be considered proof of a correct position in NCAC.

Do oral anticoagulants impact outcomes and false lumen patency after repair of acute type A aortic dissection?

OBJECTIVE: The study objective was to analyze the effects of chronic oral anticoagulation on long-term outcomes after repair of type A acute aortic dissection and its influence on false lumen fate. METHODS: We studied 188 patients (median age, 62 years; 74% were male) who underwent repair of type A aortic dissection; patients receiving postoperative chronic oral anticoagulation (n = 59) were compared with those receiving antiplatelet therapy alone (n = 129). RESULTS: Median age was similar: 60 years (18-79 years; OAC group) versus 64 years (22-86; no-OAC group) (P = .11); patients taking anticoagulants were more frequently male (88% vs 67%, P = .003). After a median follow-up of 8.4 years (2 months to 30 years), 58 patients died, 18 of aortic-related causes, and 37 patients underwent aortic reintervention. After multivariable adjustment, anticoagulation showed no significant effect on long-term survival (hazard ratio, 0.85; 95% confidence interval, 0.41-1.76; P = .66) or risk of reintervention (hazard ratio, 0.55; 95% confidence interval, 0.27-1.15; P = .11). Analysis of 127 postoperative computed tomography scans showed a patent false lumen in 53% of anticoagulated patients versus 38% of nonanticoagulated patients (P = .09): partially thrombosed in 8% versus 28% (P = .01) and thrombosed in 39% versus 34% (P = .63), respectively. In patients with a control computed tomography, there were 6 late aortic-related deaths, 1 among anticoagulated patients and 5 in those who were not. CONCLUSIONS: Chronic anticoagulation after repair of type A acute aortic dissection favors persistent late false lumen patency, which is not a risk factor for late mortality or reoperation. Chronic anticoagulation can be administered safely to patients with repaired type A acute aortic dissection regardless of its specific indication.

Mechanisms of Ventricular Arrhythmias and Implications for Catheter Ablation.

Ventricular arrhythmias present with a wide spectrum of clinical manifestations, from mildly symptomatic frequent premature ventricular contractions to life-threatening events. Pathophysiologically, idiopathic ventricular arrhythmias occur in the absence of structural heart disease or ion channelopathies. Ventricular arrhythmias in the context of structural heart disease are usually determined by scar-related reentry and are associated with increased mortality. Catheter ablation is safe and highly effective in treating ventricular arrhythmias. The proper characterization of the arrhythmogenic substrate is essential for accurate procedural planning. We provide an overview on the main mechanisms of ventricular arrhythmias and their implications for catheter ablation.

Frequency, Penetrance, and Variable Expressivity of Dilated Cardiomyopathy-Associated Putative Pathogenic Gene Variants in UK Biobank Participants.

BACKGROUND: There is a paucity of data regarding the phenotype of dilated cardiomyopathy (DCM) gene variants in the general population. We aimed to determine the frequency and penetrance of DCM-associated putative pathogenic gene variants in a general adult population, with a focus on the expression of clinical and subclinical phenotype, including structural, functional, and arrhythmic disease features. METHODS: UK Biobank participants who had undergone whole exome sequencing, ECG, and cardiovascular magnetic resonance imaging were selected for study. Three variant-calling strategies (1 primary and 2 secondary) were used to identify participants with putative pathogenic variants in 44 DCM genes. The observed phenotype was graded DCM (clinical or cardiovascular magnetic resonance diagnosis); early DCM features, including arrhythmia or conduction disease, isolated ventricular dilation, and hypokinetic nondilated cardiomyopathy; or phenotype-negative. RESULTS: Among 18 665 individuals included in the study, 1463 (7.8%) possessed ≥1 putative pathogenic variant in 44 DCM genes by the main variant calling strategy. A clinical diagnosis of DCM was present in 0.34% and early DCM features in 5.7% of individuals with putative pathogenic variants. ECG and cardiovascular magnetic resonance analysis revealed evidence of subclinical DCM in an additional 1.6% and early DCM features in an additional 15.9% of individuals with putative pathogenic variants. Arrhythmias or conduction disease (15.2%) were the most common early DCM features, followed by hypokinetic nondilated cardiomyopathy (4%). The combined clinical/subclinical penetrance was ≤30% with all 3 variant filtering strategies. Clinical DCM was slightly more prevalent among participants with putative pathogenic variants in definitive/strong evidence genes as compared with those with variants in moderate/limited evidence genes. CONCLUSIONS: In the UK Biobank, ≈1 of 6 of adults with putative pathogenic variants in DCM genes exhibited early DCM features potentially associated with DCM genotype, most commonly manifesting with arrhythmias in the absence of substantial ventricular dilation or dysfunction.

The Pathogenesis and Long-Term Consequences of COVID-19 Cardiac Injury.

The mechanisms of coronavirus disease-2019 (COVID-19)-related myocardial injury comprise both direct viral invasion and indirect (hypercoagulability and immune-mediated) cellular injuries. Some patients with COVID-19 cardiac involvement have poor clinical outcomes, with preliminary data suggesting long-term structural and functional changes. These include persistent myocardial fibrosis, edema, and intraventricular thrombi with embolic events, while functionally, the left ventricle is enlarged, with a reduced ejection fraction and new-onset arrhythmias reported in a number of patients. Myocarditis post-COVID-19 vaccination is rare but more common among young male patients. Larger studies, including prospective data from biobanks, will be useful in expanding these early findings and determining their validity.

Prevalence and prognostic significance of ischemic late gadolinium enhancement pattern in non-ischemic dilated cardiomyopathy.

BACKGROUND: Typical late gadolinium enhancement (LGE) patterns in dilated cardiomyopathy (DCM) include intramyocardial and subepicardial distribution. However, the ischemic pattern of LGE (subendocardial and transmural) has also been reported in DCM without coronary artery disease (CAD), but its correlates and prognostic significance are still not known. On these bases, this study sought to describe the prevalence and prognostic significance of the ischemic LGE pattern in DCM. METHODS: A total of 611 DCM patients with available cardiac magnetic resonance were retrospectively analyzed. A composite of all-cause-death, major ventricular arrhythmias (MVAs), heart transplantation (HTx) or ventricular assist device (VAD) implantation was the primary outcome of the study. Secondary outcomes were a composite of sudden cardiac death or MVAs and a composite of death for refractory heart failure, HTx or VAD implantation. RESULTS: Ischemic LGE was found in 7% of DCM patients without significant CAD or history of myocardial infarction, most commonly inferior/inferolateral/anterolateral. Compared to patients with non-ischemic LGE, those with ischemic LGE had higher prevalence of hypertension and atrial fibrillation or flutter. Ischemic LGE was associated with worse long-term outcomes compared to non-ischemic LGE (36% vs 23% risk of primary outcome events at 5 years respectively, P = .006), and remained an independent predictor of primary outcome after adjustment for clinically and statistically significant variables (adjusted hazard ratio 2.059 [1.055-4.015], P = .034 with respect to non-ischemic LGE). CONCLUSIONS: The ischemic pattern of LGE is not uncommon among DCM patients without CAD and is independently associated with worse long-term outcomes.

Outcomes Associated With Catheter Ablation of Ventricular Tachycardia in Patients With Cardiac Sarcoidosis.

Importance: Ventricular tachycardia (VT) is associated with high mortality in patients with cardiac sarcoidosis (CS), and medical management of CS-associated VT is limited by high failure rates. The role of catheter ablation has been investigated in small, single-center studies. Objective: To investigate outcomes associated with VT ablation in patients with CS. Design, Setting, and Participants: This cohort study from the Cardiac Sarcoidosis Consortium registry (2003-2019) included 16 tertiary referral centers in the US, Europe, and Asia. A total of 158 consecutive patients with CS and VT were included (33% female; mean [SD] age, 52 [11] years; 53% with ejection fraction [EF] <50%). Exposures: Catheter ablation of CS-associated VT and, as appropriate, medical treatment. Main Outcomes and Measures: Immediate and short-term outcomes included procedural success, elimination of VT storm, and reduction in defibrillator shocks. The primary long-term outcome was the composite of VT recurrence, heart transplant (HT), or death. Results: Complete procedural success (no inducible VT postablation) was achieved in 85 patients (54%). Sixty-five patients (41%) had preablation VT storm that did not recur postablation in 53 (82%). Defibrillator shocks were significantly reduced from a median (IQR) of 2 (1-5) to 0 (0-0) in the 30 days before and after ablation (P < .001). During median (IQR) follow-up of 2.5 (1.1-4.9) years, 73 patients (46%) experienced VT recurrence and 81 (51%) experienced the composite primary outcome. One- and 2-year rates of survival free of VT recurrence, HT, or death were 60% and 52%, respectively. EF less than 50% and myocardial inflammation on preprocedural 18F-fluorodeoxyglucose positron emission tomography were significantly associated with adverse prognosis in multivariable analysis for the primary outcome (HR, 2.24; 95% CI, 1.37-3.64; P = .001 and HR, 2.93; 95% CI, 1.31-6.55; P = .009, respectively). History of hypertension was associated with a favorable long-term outcome (adjusted HR, 0.51; 95% CI, 0.28-0.92; P = .02). Conclusions and Relevance: In this observational study of selected patients with CS and VT, catheter ablation was associated with reductions in defibrillator shocks and recurrent VT storm. Preablation LV dysfunction and myocardial inflammation were associated with adverse long-term prognosis. These data support the role of catheter ablation in conjunction with medical therapy in the management of CS-associated VT.

Substrate Characterization and Outcome of Catheter Ablation of Ventricular Tachycardia in Patients With Nonischemic Cardiomyopathy and Isolated Epicardial Scar.

BACKGROUND: The substrate for ventricular tachycardia (VT) in left ventricular (LV) nonischemic cardiomyopathy may be epicardial. We assessed the prevalence, location, endocardial electrograms, and VT ablation outcomes in LV nonischemic cardiomyopathy with isolated epicardial substrate. METHODS: Forty-seven of 531 (9%) patients with LV nonischemic cardiomyopathy and VT demonstrated normal endocardial (>1.5 mV)/abnormal epicardial bipolar low-voltage area (LVA, <1.0 mV and signal abnormality). Abnormal endocardial unipolar LVA (≤8.3 mV) and endocardial bipolar split electrograms and predictors of ablation success were assessed. RESULTS: Epicardial bipolar LVA (27.3 cm2 [interquartile range, 15.8-50.0]) localized to basal (40), mid (8), and apical (3) LV with basal inferolateral LV most common (28/47, 60%). Of 44 endocardial maps available, 40 (91%) had endocardial unipolar LVA (24.5 cm2 [interquartile range, 9.4-68.5]) and 29 (67%) had characteristic normal amplitude endocardial split electrograms opposite the epicardial LVA. At mean of 34 months, the VT-free survival was 55% after one and 72% after multiple procedures. Greater endocardial unipolar LVA than epicardial bipolar LVA (hazard ratio, 10.66 [CI, 2.63-43.12], P=0.001) and number of inducible VTs (hazard ratio, 1.96 [CI, 1.27-3.00], P=0.002) were associated with VT recurrence. CONCLUSIONS: In patients with LV nonischemic cardiomyopathy and VT, the substrate may be confined to epicardial and commonly basal inferolateral. LV endocardial unipolar LVA and normal amplitude bipolar split electrograms identify epicardial LVA. Ablation targeting epicardial VT and substrate achieves good long-term VT-free survival. Greater endocardial unipolar than epicardial bipolar LVA and more inducible VTs predict VT recurrence.

An Integrated Approach for Treatment of Acute Type A Aortic Dissection.

Background and objective: We reviewed a single-institution experience to verify the impact of surgery during different time intervals on early and late results in the treatment of patients with type A acute aortic dissection (A-AAD). Materials and Methods: From 2004 to 2021, a total of 258 patients underwent repair of A-AAD; patients were equally distributed among three periods: 2004-2010 (Era 1, n = 90), 2011-2016 (Era 2, n = 87), and 2017-2021 (Era 3, n = 81). The primary end-point was to assess whether through the years changes in indications, surgical strategies and techniques and increasing experience have influenced early and late outcomes of A-AAD repair. Results: Axillary artery cannulation was almost routinely used in Eras 2 (86%) and 3 (91%) while one femoral artery was mainly cannulated in Era 1 (91%) (p < 0.01). Retrograde cerebral perfusion was predominantly used in Era 1 (60%) while antegrade cerebral perfusion was preferred in Eras 2 (94%,) and 3 (100%); (p < 0.01). There was a significant increase of arch replacement procedures from Era 1 (11%) to Eras 2 (33%) and 3 (48%) (p < 0.01). A frozen elephant trunk was mainly performed in Era 3. Hospital mortality was 13% in Era 1, 11% in Era 2, and 4% in Era 3 (p = 0.07). Actuarial survival at 3 years is 74%, in Era 1, 78% in Era 2, and 89% in Era 3 (p = 0.05). Conclusions: With increasing experience and a more aggressive approach, including total arch replacement, repair of A-AAD can be performed with low operative mortality in many patients. Patient care and treatment by a specific team organization allows a faster diagnosis and referral for surgery allowing to further improve early and late outcomes.

Prognostic Role of Myocardial Edema as Evidenced by Early Cardiac Magnetic Resonance in Survivors of Out-of-Hospital Cardiac Arrest: A Multicenter Study.

Background Sudden cardiac arrest (SCA) may be caused by an acute and reversible myocardial injury, a chronic and irreversible myocardial damage, or a primary ventricular arrhythmia. Cardiac magnetic resonance imaging may identify myocardial edema (ME), which denotes acute and reversible myocardial damage. We evaluated the arrhythmic outcome of SCA survivors during follow-up and tested the prognostic role of ME. Methods and Results We included a consecutive series of 101 (71% men, median age 47 years) SCA survivors from 9 collaborative centers who underwent early (<1 month) cardiac magnetic resonance imaging and received an implantable cardioverter-defibrillator (ICD). On T2-weighted sequences, ME was found in 18 of 101 (18%) patients. According to cardiac magnetic resonance imaging findings, the arrhythmic SCA was ascribed to acute myocardial injury (either ischemic [n=10] or inflammatory [n=8]), to chronic structural heart diseases (ischemic heart disease [n=11], cardiomyopathy [n=20], or other [n=23]), or to primarily arrhythmic syndrome (n=29). During a follow-up of 47 months (28 to 67 months), 24 of 101 (24%) patients received an appropriate ICD intervention. ME was associated with a significantly higher survival free from both any ICD interventions (log-rank=0.04) and ICD shocks (log-rank=0.03) and remained an independent predictor of better arrhythmic outcome after adjustment for left ventricular ejection fraction and late gadolinium enhancement. The risk of appropriate ICD intervention was unrelated to the type of underlying heart disease. Conclusions ME on early cardiac magnetic resonance imaging, which denotes an acute and transient arrhythmogenic substrate, predicted a favorable long-term arrhythmic outcome of SCA survivors. These findings may have a substantial impact on future guidelines on the management of SCA survivors.

Prognostic role of global longitudinal strain by feature tracking in patients with hypertrophic cardiomyopathy: The STRAIN-HCM study.

BACKGROUND: The assessment of myocardial fiber deformation with cardiac magnetic resonance feature tracking (CMR-FT) has shown to be promising in terms of prognostic information in several structural heart diseases. However, little is known about its role in hypertrophic cardiomyopathy (HCM). Aims of the present study were: 1) to assess the prognostic role of CMR-FT derived strain parameters in patients with HCM. METHODS: CMR was performed in 130 consecutive HCM patients (93 males, mean age (54 ± 17 years) with an estimated 5-year risk of sudden cardiac death (SCD) <6% according to the HCM Risk-SCD calculator. 2D- and 3D-Global Radial (GRS), Longitudinal (GLS) and Circumferential (GCS) Strain was evaluated by FT analysis. The primary outcome of the study was a composite of major adverse cardiac events (MACE) including SCD, resuscitated cardiac arrest due to ventricular fibrillation (VF) or hemodynamically unstable ventricular tachycardia (VT), and hospitalization for heart failure. RESULTS: After a median follow-up of 51.7 (37.1-68.8) months, 4 (3%) patients died (all of them suffered from SCD) and 36 (28%) were hospitalized for heart failure. After multivariable adjustment for clinical and imaging covariates, among all strain parameters, only GLS remained a significant independent predictor of outcome events in both the model including 2D strain (HR 1.12, 95% CI 1.03-1.23, p = 0.01) and the model including 3D strain (HR 1.14, 95% CI 1.01-1.30, p = 0.04). The addition of 2D-GLS into the model with clinical and imaging predictors resulted in a significant increase in the C-statistic (from 0.48 to 0.65, p = 0.03). CONCLUSION: CMR-FT derived GLS is a powerful independent predictor of MACE in patients with HCM, incremental to common clinical and CMR risk factors including left ventricular ejection fraction and late gadolinium enhancement.

Diagnosis and Treatment of Idiopathic Premature Ventricular Contractions: A Stepwise Approach Based on the Site of Origin.

Premature ventricular contractions in the absence of structural heart disease are among the most common arrhythmias in clinical practice, with well-defined sites of origin in the right and left ventricle. In this review, starting from the electrocardiographic localization of premature ventricular contractions, we investigated the mechanisms, prevalence in the general population, diagnostic work-up, prognosis and treatment of premature ventricular contractions, according to current scientific evidence.

Phenotypic Expression, Natural History, and Risk Stratification of Cardiomyopathy Caused by Filamin C Truncating Variants.

BACKGROUND: Filamin C truncating variants (FLNCtv) cause a form of arrhythmogenic cardiomyopathy: the mode of presentation, natural history, and risk stratification of FLNCtv remain incompletely explored. We aimed to develop a risk profile for refractory heart failure and life-threatening arrhythmias in a multicenter cohort of FLNCtv carriers. METHODS: FLNCtv carriers were identified from 10 tertiary care centers for genetic cardiomyopathies. Clinical and outcome data were compiled. Composite outcomes were all-cause mortality/heart transplantation/left ventricle assist device (D/HT/LVAD), nonarrhythmic death/HT/LVAD, and sudden cardiac death/major ventricular arrhythmias. Previously established cohorts of 46 patients with LMNA and 60 with DSP-related arrhythmogenic cardiomyopathies were used for prognostic comparison. RESULTS: Eighty-five patients carrying FLNCtv were included (42±15 years, 53% men, 45% probands). Phenotypes were heterogeneous at presentation: 49% dilated cardiomyopathy, 25% arrhythmogenic left dominant cardiomyopathy, 3% arrhythmogenic right ventricular cardiomyopathy. Left ventricular ejection fraction was <50% in 64% of carriers and 34% had right ventricular fractional area changes (RVFAC=(right ventricular end-diastolic area - right ventricular end-systolic area)/right ventricular end-diastolic area) <35%. During follow-up (median time 61 months), 19 (22%) carriers experienced D/HT/LVAD, 13 (15%) experienced nonarrhythmic death/HT/LVAD, and 23 (27%) experienced sudden cardiac death/major ventricular arrhythmias. The sudden cardiac death/major ventricular arrhythmias incidence of FLNCtv carriers did not significantly differ from LMNA carriers and DSP carriers. In FLNCtv carriers, left ventricular ejection fraction was associated with the risk of D/HT/LVAD and nonarrhythmic death/HT/LVAD. CONCLUSIONS: Among patients referred to tertiary referral centers, FLNCtv arrhythmogenic cardiomyopathy is phenotypically heterogeneous and characterized by a high risk of life-threatening arrhythmias, which does not seem to be associated with the severity of left ventricular dysfunction.

Use of Sutureless and Rapid Deployment Prostheses in Challenging Reoperations.

Sutureless and rapid-deployment bioprostheses have been introduced as alternatives to traditional prosthetic valves to reduce cardiopulmonary and aortic cross-clamp times during aortic valve replacement. These devices have also been employed in extremely demanding surgical settings, as underlined in the present review. Searches on the PubMed and Medline databases aimed to identify, from the English-language literature, the reported cases where both sutureless and rapid-deployment prostheses were employed in challenging surgical situations, usually complex reoperations sometimes even performed as bailout procedures. We have identified 25 patients for whom a sutureless or rapid-deployment prosthesis was used in complex redo procedures: 17 patients with a failing stentless bioprosthesis, 6 patients with a failing homograft, and 2 patients with the failure of a valve-sparing procedure. All patients survived reoperation and were reported to be alive 3 months to 4 years postoperatively. Sutureless and rapid-deployment bioprostheses have proved effective in replacing degenerated stentless bioprostheses and homografts in challenging redo procedures. In these settings, they should be considered as a valid alternative not only to traditional prostheses but also in selected cases to transcatheter valve-in-valve solutions.