Added Value of CCTA-Derived Features to Predict MACEs in Stable Patients Undergoing Coronary Computed Tomography.

Clinical evidence has emphasized the importance of coronary plaques' characteristics, rather than lumen stenosis, for the outcome of cardiovascular events. Coronary computed tomographic angiography (CCTA) has a well-established role as a non-invasive tool for assessing plaques. The aim of this study was to compare clinical characteristics and CCTA-derived information of stable patients with non-severe plaques in predicting major adverse cardiac events (MACEs) during follow-up. We retrospectively selected 371 patients (64% male) who underwent CCTA in our center from March 2016 to January 2021 with Coronary Artery Disease­Reporting and Data System (CAD-RADS) 0 to 3. Of those, 198 patients (53% male) had CAD-RADS 0 to 1. Among them, 183 (49%) had normal pericoronary fat attenuation index (pFAI), while 15 (60% male) had pFAI ≥ 70.1 Hounsfield unit (HU). The remaining 173 patients (76% male) had CAD-RADS 2 to 3 and were divided into patients with at least one low attenuation plaque (LAP) and patients without LAPs (n-LAP). Compared to n-LAP, patients with LAPs had higher pFAI (p = 0.005) and had more plaques than patients with n-LAP. Presence of LAPs was significantly higher in elderly (p < 0.001), males (p < 0.001) and patients with traditional risk factors (hypertension p = 0.0001, hyperlipemia p = 0.0003, smoking p = 0.0003, diabetes p = <0.0001, familiarity p = 0.0007). Among patients with CAD-RADS 0 to 1, the ones with pFAI ≥ 70.1 HU were more often hyperlipidemic (p = 0.05) and smokers (p = 0.007). Follow-up (25,4 months, range: 17.6−39.2 months) demonstrated that LAP and pFAI ≥ 70.1 significantly and independently (p = 0.04) predisposed to outcomes (overall mortality and interventional procedures). There is an added value of CCTA-derived features in stratifying cardiovascular risk in low- to intermediate-risk patients with non-severe, non-calcified coronary plaques. This is of utmost clinical relevance as it is possible to identify a subset of patients with increased risk who need strengthening in therapeutic management and closer follow-up even in the absence of severe CAD. Further studies are needed to evaluate the effect of medical treatments on pericoronary inflammation and plaque composition.

Surgical management of failing Fontan circulation: results from 30 cases with 285 patient-years follow-up.

OBJECTIVES: Fontan patients are known to suffer from clinical attrition over the years, which has been characterized as Fontan failure. We sought to evaluate the clinical outcomes of such Fontan patients undergoing surgical management in a 25-year, single-centre experience. METHODS: A retrospective single-centre analysis of patients undergoing surgical treatment for failing Fontan between 1995 and 2020, including any reoperations when ventricular function was preserved, or a heart transplant (HTx), when ventricular contractility was impaired. We analysed survival, indications for surgery and early and late complication rates. RESULTS: We collected 30 patients (mean age 24.7 years) who required surgery after a mean time of 19.3 years from the original Fontan procedure: Fontan conversion in 21 (70%, extracardiac conduit in 19, lateral tunnel in 2), a HTx in 4 (13.3%) and other reoperations in 5 (16.7%). The most common indications for surgery were tachyarrhythmias (63.3%) and severe right atrial dilatation (63.3%). Overall survival at the 1-, 5-, 10- and 20-year follow-up examinations were 75.9% [95% confidence interval (CI): 91.4-60.4%], 75.9% (95% CI: 91.4-60.4%), 70% (95% CI: 78-52%) and 70% (95% CI: 78-52%), respectively. The most frequent complications were postoperative tachyarrhythmias (50%) and late Fontan-associated liver disease (56.5%). HTx and Fontan conversion provided comparably good outcomes compared to other reoperations (P = 0.022). CONCLUSIONS: Surgery for failing Fontan can be performed effectively with overall good long-term survival. However, early and late morbidities are still a significant burden. Because other reoperations performed when patients presented with contraindications for a HTx have carried high mortality, close clinical follow-up is mandatory, and an earlier indication for Fontan conversion or a HTx is advisable to optimize outcomes.

Atrial fibrillation after orthotopic heart transplantatation: Pathophysiology and clinical impact.

BACKGROUND: Atrial fibrillation (AF) is a well-established post-cardiac surgery complication. Orthotopic heart transplantation (OHT) represents a peculiar condition where surgical thoracic veins isolation and autonomic denervation occur. This study aims at investigating AF incidence in OHT in order to define its risk factors and to evaluate its prognostic impact. METHODS: 278 patients affected by OHT were recruited in our Cardiac Surgery Unit and retrospectively analyzed, using clinical, surgical and instrumental data. RESULTS: The patients cohort showed 45 post-operative (16.5%) and 20 late AF cases (7.2%). Only paroxysmal AF episodes were observed. Elderly donors and acute rejection resulted as risk factors in patients with post-operative AF episodes, who presented higher all-cause mortality at 11 years post-OHT (p < 0.001, Kaplan Meier analysis). The majority of late AF episodes occurred during hospitalization, due to renal failure or infections and more frequently in male patients; no significant correlation was observed with acute or chronic rejection or other characteristics. CONCLUSION: Pulmonary vein isolation and vagal denervation lead to low AF incidence in OHT recipients. Acute rejection and graft status are the main risk factors for post-operative AF episodes, while other systemic conditions act as late AF triggers. The occurrence of AF episodes is associated with poor outcome and AF should be considered as a marker of clinical frailty.

'Hot phase' clinical presentation in arrhythmogenic cardiomyopathy.

AIMS: The aim of this study is to evaluate the clinical features of patients affected by arrhythmogenic cardiomyopathy (AC), presenting with chest pain and myocardial enzyme release in the setting of normal coronary arteries ('hot phase'). METHODS AND RESULTS: We collected detailed anamnestic, clinical, instrumental, genetic, and histopathological findings as well as follow-up data in a series of AC patients who experienced a hot phase. A total of 23 subjects (12 males, mean age at the first episode 27 ± 16 years) were identified among 560 AC probands and family members (5%). At first episode, 10 patients (43%) already fulfilled AC diagnostic criteria. Twelve-lead electrocardiogram recorded during symptoms showed ST-segment elevation in 11 patients (48%). Endomyocardial biopsy was performed in 11 patients, 8 of them during the acute phase showing histologic evidence of virus-negative myocarditis in 88%. Cardiac magnetic resonance was performed in 21 patients, 12 of them during the acute phase; oedema and/or hyperaemia were detected in 7 (58%) and late gadolinium enhancement in 11 (92%). At the end of follow-up (mean 17 years, range 1-32), 12 additional patients achieved an AC diagnosis. Genetic testing was positive in 77% of cases and pathogenic mutations in desmoplakin gene were the most frequent. No patient complained of sustained ventricular arrhythmias or died suddenly during the 'hot phase'. CONCLUSION: 'Hot phase' represents an uncommon clinical presentation of AC, which often occurs in paediatric patients and carriers of desmoplakin gene mutations. Tissue characterization, family history, and genetic test represent fundamental diagnostic tools for differential diagnosis.

First-degree atrioventricular block on basal electrocardiogram predicts future arrhythmic events in patients with Brugada syndrome: a long-term follow-up study from the Veneto region of Northeastern Italy.

AIMS: This study was designed to assess the prognostic value of clinical and electrocardiographic parameters in Brugada syndrome (BrS). METHODS AND RESULTS: The study population included 272 consecutive patients (82% males; mean age 43 ± 12 years), with either a spontaneous (n = 137, 50%) or drug-induced (n = 135, 50%) Type 1 Brugada electrocardiogram (ECG) pattern. The study combined endpoint included sudden cardiac death (SCD), cardiac arrest, and appropriate intervention of implantable cardioverter-defibrillator (ICD). A first-degree atrioventricular (AV) block (PR = 219 ± 17 ms) was documented at basal ECG in 45 patients (16.5%); 27 of these underwent an electrophysiological study with recording in 21 (78%) of an HV interval ≥55 ms (mean 61 ± 3 ms). Patients with first-degree AV block had a wider QRS complex (median 110 ms vs. 95 ms; P = 0.04) and more often showed a left anterior hemiblock pattern (n = 13, 29% vs. n = 35, 16%; P = 0.056). During a mean follow-up of 85 ± 55 months, 17 patients (6.3%) experienced ≥1 major arrhythmic events (appropriate ICD intervention, n = 13 and SCD, n = 4). At univariate analysis, the occurrence of major arrhythmic events was significantly associated with a history of syncope or cardiac arrest (P < 0.001), Type 1 ECG pattern (P = 0.04), and first-degree AV block (P < 0.001). Univariate and multivariable predictors of events included a history of syncope or cardiac arrest [hazard ratio (HR) 5.8, 95% confidence interval (95% CI) 2.04-16.5; P < 0.001; and HR 6.68, 95% CI 2.34-19.1; P < 0.001; respectively], a spontaneous Type 1 ECG pattern (HR 1.56, 95% CI 1.03-4.24; P = 0.033; and HR 1.84, 95% CI 1.01-4.29; P = 0.044; respectively) and a first-degree AV block at baseline ECG (HR 3.84, 95% CI 1.47-9.99; P = 0.006; and HR 4.65, 95% CI 2.34-19.1; P = 0.002; respectively). CONCLUSION: Besides a history of cardiac arrest or syncope, first-degree AV block on basal ECG is an independent predictor of malignant arrhythmic events and a stronger marker of arrhythmic risk than a spontaneous 'coved-type' ECG pattern in patients with BrS.

Is the time between first diagnosis of paroxysmal atrial fibrillation and cryoballoon ablation a predictor of efficacy?

AIMS: Cryoablation is an indicated therapy for the treatment of recurrent atrial fibrillation through pulmonary vein isolation; however, the optimal time between first diagnosis of atrial fibrillation and cryoablation is still unknown. We aimed to assess the clinical efficacy and safety of early versus later treatment of patients with paroxysmal atrial fibrillation by cryoablation. METHODS: Five hundred and ten patients underwent atrial fibrillation cryoablation and were prospectively followed for at least 6 months in 43 Italian cardiology centers. The population was divided into two groups according to the time since the first diagnosis of atrial fibrillation until the index cryoablation procedure. An early-treatment group had an elapsed time of 15 months or less from atrial fibrillation diagnosis until cryoablation, and the late-treatment group had an elapsed time of greater than 15 months. During the evaluation, clinical efficacy was defined as atrial fibrillation recurrence outside a landmark 90-day blanking period, and safety was defined as the reporting of all procedure-related complications. RESULTS: In the total cohort, cryoablation was performed after a median of 36 months from the point of the patient diagnosis with drug refractory symptomatic recurrent atrial fibrillation. The early-treatment group was composed of 130 (25%) patients, whereas the late-treatment group had 380 (75%) patients. Both cohorts had similar baseline clinical characteristics. Of 510 patients, 22 had a complication related to the procedure with no difference between the two groups. Multivariable analysis showed that the risk of atrial fibrillation recurrence was significantly higher in the late-treatment group (hazard ratio: 1.77; 95% confidence interval 1.00-3.13) CONCLUSION: In our multicenter observational examination, cryoablation was well tolerated and effective in the treatment of patients with drug refractory symptomatic paroxysmal atrial fibrillation. Reducing the time between diagnosis and ablation brought about a treatment that had a lower risk of atrial fibrillation recurrence with no change in safety.(Italian ClinicalService Project: NCT01007474).

Pulmonary Vein Isolation with the Cryoballoon Technique: Feasibility, Procedural Outcomes, and Adoption in the Real World: Data from One Shot Technologies TO Pulmonary Vein Isolation (1STOP) Project.

BACKGROUND: Catheter ablation (CA) is recommended for patients with drug refractory symptomatic atrial fibrillation (AF). "One Shot" catheters have been introduced to simplify CA and cryoballoon ablation (CBA) is spreading rapidly. Few real-world data are available on standard clinical practice, mainly from single-center experience. We aimed to evaluate clinical settings, demographics, and acute procedural outcomes in a large cohort of patients treated with CBA. METHODS: A total of 903 patients (73% male, mean age 59 ± 11) underwent pulmonary vein CBA. Correlations between the patient's inclusion time and clinical characteristics, procedure duration, acute success rate, and intraprocedural complications were evaluated. RESULTS: Seventy-seven percent of patients were affected by paroxysmal AF and 23% by persistent AF. Overall, acute success rate was 97.9% and periprocedural complications were observed in 35 (3.9%) patients, 13 (1.4%) of which were classified as major complications. With respect to the patient's inclusion time analysis, an increase in treatment of persistent AF was observed, a significant decrease in CBA times (procedure, ablation, and fluoroscopy: 136.0 ± 46.5 minutes, 28.8 ± 19.6 minutes, and 34.3 ± 15.4 minutes, respectively) was observed, with comparable acute success rate and intraprocedural complications over time. The rate of major complications was extremely low (1.4%); no death, atrioesophageal fistula, stroke, or other major periinterventional or late complications occurred. CONCLUSION: This series represents the largest experience of CBA in the treatment of AF that also describes the adoption curve of this relatively recent technology. CBA showed an excellent safety profile when performed in a large real-world clinical setting, with satisfactory acute success rate and, on average, short procedural times. CLINICAL TRIAL REGISTRATION: clinicaltrials.gov (NCT01007474).

Successful transvenous mechanical lead extraction and stent implantation in a patient after Mustard palliation for D-transposition of great arteries and superior vena cava syndrome.

: We report a successful combined approach of transvenous mechanical pacing lead extraction and stent angioplasty for superior baffle occlusion in a young woman with D-transposition of great artery after Mustard procedure. After having extracted the pacing leads, the baffle was easily stented, thanks to the channel left by the extracted leads. Eventually, a new pacing lead was implanted into the pulmonic ventricle through the stented baffle. Our report demonstrates the safeness and feasibility of a combined interventional approach in avoiding the need for surgery.

Prognostic value of endocardial voltage mapping in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.

BACKGROUND: Endocardial voltage mapping (EVM) identifies low-voltage right ventricular (RV) areas, which may represent the electroanatomic scar substrate of life-threatening tachyarrhythmias. We prospectively assessed the prognostic value of EVM in a consecutive series of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). METHODS AND RESULTS: We studied 69 consecutive ARVC/D patients (47 males; median age 35 years [28-45]) who underwent electrophysiological study and both bipolar and unipolar EVM. The extent of confluent bipolar (<1.5 mV) and unipolar (<6.0 mV) low-voltage electrograms was estimated using the CARTO-incorporated area calculation software. Fifty-three patients (77%) showed ≥1 RV electroanatomic scars with an estimated burden of bipolar versus unipolar low-voltage areas of 24.8% (7.2-31.5) and 64.8% (39.8-95.3), respectively (P=0.009). In the remaining patients with normal bipolar EVM (n=16; 23%), the use of unipolar EVM unmasked ≥1 region of low-voltage electrogram affecting 26.2% (11.6-38.2) of RV wall. During a median follow-up of 41 (28-56) months, 19 (27.5%) patients experienced arrhythmic events, such as sudden death (n=1), appropriate implantable cardioverter defibrillator interventions (n=7), or sustained ventricular tachycardia (n=11). Univariate predictors of arrhythmic outcome included previous cardiac arrest or syncope (hazard ratio=3.4; 95% confidence interval, 1.4-8.8; P=0.03) and extent of bipolar low-voltage areas (hazard ratio=1.7 per 5%; 95% confidence interval, 1.5-2; P<0.001), whereas the only independent predictor was the bipolar low-voltage electrogram burden (hazard ratio=1.6 per 5%; 95% confidence interval, 1.2-1.9; P<0.001). Patients with normal bipolar EVM had an uneventful clinical course. CONCLUSIONS: The extent of bipolar RV endocardial low-voltage area was a powerful predictor of arrhythmic outcome in ARVC/D, independently of history and RV dilatation/dysfunction. A normal bipolar EVM characterized a low-risk subgroup of ARVC/D patients.

Role of an aggressive rhythm control strategy on sinus rhythm maintenance following intra-operative radiofrequency ablation of atrial fibrillation in patients undergoing surgical correction of valvular disease.

OBJECTIVE: The purpose of this study was to evaluate the impact of adhering to a strict follow up regimen on mid-term efficacy of intraoperative radiofrequency ablation in maintaining sinus rhythm for patients with atrial fibrillation undergoing cardiac surgery. METHODS: We enrolled 52 patients with atrial fibrillation and valve disease divided in 2 groups: group 1 (26 patients) underwent intraoperative radiofrequency ablation and was followed by a team of skilled cardiologists who treated recurrences with amiodarone and electrical cardioversion; group 2 (26 patients) was treated with intraoperative radiofrequency ablation but patients were thereafter treated by their local hospital or personal cardiologist. RESULTS: There were no intraoperative deaths or complications related to radiofrequency ablation. Twenty four months after the operation, freedom from atrial fibrillation was 60% in group 1 and 32% in group 2 (p=0.02). Group 1 also showed a reduction in atrial volume (p≤0.05). CONCLUSION: Postoperative follow up with electrical and pharmacologic cardioversion combined with radiofrequency ablation achieves higher rate of sinus rhythm maintenance. After ablation, electrical cardioversion is effective even in patients with enlarged left atrium, long history of atrial fibrillation, or previous failed electrical cardioversion.

Imaging study of ventricular scar in arrhythmogenic right ventricular cardiomyopathy: comparison of 3D standard electroanatomical voltage mapping and contrast-enhanced cardiac magnetic resonance.

BACKGROUND: The hallmark lesion of arrhythmogenic right ventricular cardiomyopathy (ARVC) is fibrofatty scar replacement. We compared endocardial voltage mapping (EVM) and contrast-enhanced cardiac magnetic resonance (CE-CMR) for imaging scar lesions in ARVC patients. METHODS AND RESULTS: We studied 23 consecutive ARVC patients (16 males; mean age, 38±12 years) who underwent RV EVM and CE-CMR and 37 control subjects. In 21 (91%) of 23 ARVC patients, RV EVM was abnormal, with a total of 45 electroanatomical scars (EAS): 17 (38%) in the inferobasal region, 12 (26.6%) in the anterolateral region, 8 (17.7%) in the RV outflow tract (RVOT), and 8 (17.7%) in the apex. RV delayed contrast enhancement (DCE) was found in 9 (39%) of 23 patients, with a total of 23 RV DCE scars: 4 (17.4%) in the inferobasal region, 9 (39.1%) in the anterolateral region, 4 (17.4%) in the RVOT, and 6 (26.1%) in the apex. There was a mismatch in 24 RV scars, with 22 EAS not confirmed by DCE and 2 DCE scars (both in the RVOT) undetected by EVM. In 9 (75%) of 12 patients with abnormal RV EVM/normal RV DCE, ≥1 DCEs were identified in the left ventricle (LV). Overall, ventricular DCE was detected in 78% of patients. No control subjects showed either EAS or DCE. CONCLUSIONS: EVM and CE-CMR allow identification of RV scar lesions in most ARVC patients. CE-CMR is less sensitive than EVM in identifying RV scar lesions. The high prevalence of LV DCE confirms the frequent biventricular involvement and indicates the diagnostic relevance of LV scar detection by CE-CMR.

Prophylactic implantable defibrillator in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation or sustained ventricular tachycardia.

BACKGROUND: The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue. METHODS AND RESULTS: We studied 106 consecutive patients (62 men and 44 women; age, 35.6±18 years) with arrhythmogenic right ventricular cardiomyopathy/dysplasia who received an ICD based on 1 or more arrhythmic risk factors such as syncope, nonsustained ventricular tachycardia, familial sudden death, and inducibility at programmed ventricular stimulation. During follow-up of 58±35 months, 25 patients (24%) had appropriate ICD interventions and 17 (16%) had shocks for life-threatening VF or ventricular flutter. At 48 months, the actual survival rate was 100% compared with the VF/ventricular flutter-free survival rate of 77% (log-rank P=0.01). Syncope significantly predicted any appropriate ICD interventions (hazard ratio, 2.94; 95% confidence interval, 1.83 to 4.67; P=0.013) and shocks for VF/ventricular flutter (hazard ratio, 3.16; 95% confidence interval, 1.39 to 5.63; P=0.005). The positive predictive value of programmed ventricular stimulation was 35% for any appropriate ICD intervention and 20% for shocks for VF/ventricular flutter, with a negative predictive value of 70% and 74%. None of the 27 asymptomatic patients with isolated familial sudden death had appropriate ICD therapy. Twenty patients (19%) had inappropriate ICD interventions, and 18 (17%) had device-related complications. CONCLUSIONS: One fourth of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior sustained ventricular tachycardia or VF had appropriate ICD interventions. Syncope was an important predictor of life-saving ICD intervention and is an indication for ICD. Prophylactic ICD may not be indicated in asymptomatic patients because of their low arrhythmic risk regardless of familial sudden death and programmed ventricular stimulation findings. Programmed ventricular stimulation had a low predictive accuracy for ICD therapy.

Acute biopsy-proven lymphocytic myocarditis mimicking Takotsubo cardiomyopathy.

Endomyocardial biopsy (EMB), the diagnostic gold standard for myocarditis, has not been systematically performed in the reported case series of Takotsubo cardiomyopathy, although proposed Mayo Criteria specify exclusion of myocarditis. Moreover, there is no specific recommendation for infarct-like acute myocarditis in the recently published guidelines on the role of EMB. Here we present a thoroughly documented case fulfilling both the proposed Mayo criteria for Takotsubo cardiomyopathy and the World Health Organization criteria for active, virus-negative, immune-mediated myocarditis. Since myocarditis can mimic acute myocardial infarction with normal coronary arteries, EMB should be performed to rule out myocarditis in patients presenting with LV apical ballooning syndrome (or Takotsubo cardiomyopathy).

Three-dimensional electroanatomical voltage mapping and histologic evaluation of myocardial substrate in right ventricular outflow tract tachycardia.

OBJECTIVES: We tested whether 3-dimensional electroanatomical voltage mapping (EVM) may help in the differential diagnosis between idiopathic right ventricular outflow tract (RVOT) tachycardia and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). BACKGROUND: Right ventricular EVM has been demonstrated to reliably identify low-voltage regions ("electroanatomical scar"), which in patients with ARVC/D correspond to areas of fibrofatty myocardial replacement. METHODS: The study population comprised 27 patients (15 men and 12 women, age 33.9 +/- 8 years) with RVOT tachycardia and no echocardiographic/angiographic evidence of right ventricular (RV) dilation/dysfunction, who underwent EVM and endomyocardial biopsy (EMB) for characterization of ventricular tachycardia (VT) substrate before catheter ablation. RESULTS: Electroanatomical voltage mapping was normal in 20 of 27 patients (74%, group A), with electrogram voltage >1.5 mV throughout the RV. The other 7 patients (26%, group B) showed >/=1 (1.4 +/- 07) RV electroanatomical scar area(s) (bipolar voltage <0.5 mV) that correlated with fibrofatty myocardial replacement at EMB (p < 0.001). Clinical predictors of RV scar were right precordial QRS prolongation (p < 0.001) and VT inducibility (p = 0.001). Catheter ablation successfully eliminated VT in 18 of 20 patients (90%). During a follow-up of 41 +/- 8 months, 3 of 7 patients (43%) from group B received an implantable defibrillator because of life-threatening ventricular arrhythmias, compared with no patients from group A (p = 0.016). CONCLUSIONS: An early/minor form of ARVC/D may mimic idiopathic RVOT tachycardia. Electroanatomical voltage mapping is able to identify RVOT tachycardia due to concealed ARVC/D by detecting RVOT electroanatomical scars that correlate with fibrofatty myocardial replacement at EMB and predispose to sudden arrhythmic death.

How to deal with recipients of valves prone to structural failure in the 2000s: Padua experience with the TRI Technologies valve.

BACKGROUND: TRI Technologies (TT) valves evidenced a propensity to structural failure, consisting in fracture of the pivoting system, leading to leaflet escape. At our institution, between 2000 and 2002, 36 TT valves were implanted in 34 patients. Here we report the final results of the Tritech survey program. METHODS: In February 2002, the first valve-related death occurred. After the event, patients were enrolled in the TT valve survey program and strictly followed up. The option of a reintervention was advised to each patient and weighed against the redo operative risk. The prophylactic TT valve replacement program took place in two time frames, dependent on each patient's personal choice: between September 2002 and October 2003 (first phase), and between September 2004 and October 2004 (second phase). Overall, 22 (10 women, 12 men) patients underwent reoperation. Mean time interval between TT valve implantation and replacement was 23 +/- 11 months. Patients' mean age was 59 +/- 11 years (median age, 64 years). RESULTS: All recipients adhering to the program successfully underwent reoperation. Operative mortality was 0%. CONCLUSIONS: We believe that the timing of the second surgery was fundamental for the favorable outcome of each patient and the absence of operative mortality. We are convinced that the tailored programming of the reintervention, together with the strong motivation of each patient, contributed in minimizing the risks related to surgery. The circumscribed cohort of patients involved was compatible with a tailored therapeutic plan. We strongly advise prophylactic reoperation of recipients of TT valves.

Three-dimensional electroanatomic voltage mapping increases accuracy of diagnosing arrhythmogenic right ventricular cardiomyopathy/dysplasia.

BACKGROUND: Three-dimensional electroanatomic voltage mapping offers the potential to identify low-voltage areas that correspond to regions of right ventricular (RV) myocardial loss and fibrofatty replacement in patients with arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). METHODS AND RESULTS: Thirty-one consecutive patients (22 men and 9 women; mean age, 30.8+/-7 years) who fulfilled the criteria of the Task Force of the European Society of Cardiology and International Society and Federation of Cardiology (ESC/ISFC) for ARVC/D diagnosis after noninvasive clinical evaluation underwent further invasive study including RV electroanatomic voltage mapping and endomyocardial biopsy (EMB) to validate the diagnosis. Multiple RV endocardial, bipolar electrograms (175+/-23) were sampled during sinus rhythm. Twenty patients (group A; 65%) had an abnormal RV electroanatomic voltage mapping showing > or =1 area (mean 2.25+/-0.7) with low-voltage values (bipolar electrogram amplitude <0.5 mV), surrounded by a border zone (0.5 to 1.5 mV) that transitioned into normal myocardium (>1.5 mV). Low-voltage electrograms appeared fractionated with significantly prolonged duration and delayed activation. In 11 patients (group B; 35%), electroanatomic voltage mapping was normal, with preserved electrogram voltage (4.4+/-0.7 mV) and duration (37.2+/-0.9 ms) throughout the RV. Low-voltage areas in patients from group A corresponded to echocardiographic/angiographic RV wall motion abnormalities and were significantly associated with myocyte loss and fibrofatty replacement at EMB (P<0.0001) and familial ARVC/D (P<0.0001). Patients from group B had sporadic disease and histopathological evidence of inflammatory cardiomyopathy (P<0.0001). During the time interval from onset of symptoms to the invasive study, 11 patients (55%) with electroanatomic low-voltage regions received an implantable cardioverter/defibrillator because of life-threatening ventricular arrhythmias, whereas all but 1 patient with a normal voltage map remained stable on antiarrhythmic drug therapy (P=0.02). CONCLUSIONS: Three-dimensional electroanatomic voltage mapping enhanced accuracy for diagnosing ARVC/D (1) by demonstrating low-voltage areas that were associated with fibrofatty myocardial replacement and (2) by identifying a subset of patients who fulfilled ESC/ISFC Task Force diagnostic criteria but showed a preserved electrogram voltage, an inflammatory cardiomyopathy mimicking ARVC/D, and a better arrhythmic outcome.

Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia.

BACKGROUND: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a condition associated with the risk of sudden death (SD). METHODS AND RESULTS: We conducted a multicenter study of the impact of the implantable cardioverter-defibrillator (ICD) for prevention of SD in 132 patients (93 males and 39 females, age 40+/-15 years) with ARVC/D. Implant indications were a history of cardiac arrest in 13 patients (10%), sustained ventricular tachycardia in 82 (62%), syncope in 21 (16%), and other in 16 (12%). During a mean follow-up of 39+/-25 months, 64 patients (48%) had appropriate ICD interventions, 21 (16%) had inappropriate interventions, and 19 (14%) had ICD-related complications. Fifty-three (83%) of the 64 patients with appropriate interventions received antiarrhythmic drug therapy at the time of first ICD discharge. Programmed ventricular stimulation was of limited value in identifying patients at risk of tachyarrhythmias during the follow-up (positive predictive value 49%, negative predictive value 54%). Four patients (3%) died, and 32 (24%) experienced ventricular fibrillation/flutter that in all likelihood would have been fatal in the absence of the device. At 36 months, the actual patient survival rate was 96% compared with the ventricular fibrillation/flutter-free survival rate of 72% (P<0.001). Patients who received implants because of ventricular tachycardia without hemodynamic compromise had a significantly lower incidence of ventricular fibrillation/flutter (log rank=0.01). History of cardiac arrest or ventricular tachycardia with hemodynamic compromise, younger age, and left ventricular involvement were independent predictors of ventricular fibrillation/flutter. CONCLUSIONS: In patients with ARVC/D, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. Patients who were prone to ventricular fibrillation/flutter could be identified on the basis of clinical presentation, irrespective of programmed ventricular stimulation outcome.

Radiofrequency ablation through a right atrium incision in congenital atrial septal defect.

BACKGROUND: The incidence of atrial fibrillation is similar in the clinical history of patients with atrial septal defect, either surgically corrected and uncorrected. We present an unpublished technique for treating atrial fibrillation by left endocardial radiofrequency ablation through the lone right atrium incision, coupled to atrial septal defect septum primum and secundum closure, thus reducing the surgical trauma related to paraseptal left atrium incision. PATIENTS AND METHODS: We treated 2 patients through a lone right atrium incision by radiofrequency ablation because of congenital atrial septal defect and chronic atrial fibrillation. RESULTS: The patients after defect closure were weaned off cardiopulmonary bypass in sinus rhythm. The postoperative hospital stay was uneventful and at more than 1 year of follow-up they are still in sinus rhythm. CONCLUSIONS: The radiofrequency ablation procedure is possible through the lone right atrium incision, avoiding any further incision and new source of possible bleeding when in presence of interatrial septal defects, and the technique is simple and useful.