Papers by ferdinando pasquale
Journal of Cardiovascular Medicine, Nov 1, 2007
Heart, Feb 9, 2021
ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry di... more ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).MethodsIn this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.ResultsShort PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ2 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78.ConclusionsStandard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL ≥1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis.
Frontiers in Cardiovascular Medicine, Jun 21, 2023
Background: Electrocardiogram (ECG) has proven to be useful for early detection of cardiac involv... more Background: Electrocardiogram (ECG) has proven to be useful for early detection of cardiac involvement in Anderson-Fabry disease (AFD); however, little evidence is available on the association between ECG alterations and the progression of the disease. Aim and Methods: To perform a cross sectional comparison of ECG abnormalities throughout different left ventricular hypertrophy (LVH) severity subgroups, providing ECG patterns specific of the progressive AFD stages. 189 AFD patients from a multicenter cohort underwent comprehensive ECG analysis, echocardiography, and clinical evaluation. Results: The study cohort (39% males, median age 47 years, 68% classical AFD) was divided into 4 groups according to different degree of left ventricular (LV) thickness: group A ≤ 9 mm (n = 52, 28%); group B 10-14 mm (n = 76, 40%); group C 15-19 mm (n = 46, 24%); group D ≥ 20 mm (n = 15, 8%). The most frequent conduction delay was right bundle branch block (RBBB), incomplete in groups B and C (20%,22%) and complete RBBB in group D (54%, p < 0.001); none of the patients had left bundle branch block (LBBB). Left anterior fascicular block, LVH criteria, negative T waves, ST depression were more common in the advanced stages of the disease (p < 0.001). Summarizing our results, we suggested ECG patterns representative of the different AFD stages as assessed by the increases in LV thickness over time (Central Figure). Patients from group A showed mostly a normal ECG (77%) or minor anomalies like LVH criteria (8%) and delta wave/slurred QR onset + borderline PR (8%). Differently, patients from groups B and C exhibited more heterogeneous ECG patterns: LVH (17%; 7% respectively); LVH + LV strain (9%; 17%); incomplete RBBB + repolarization abnormalities (8%; 9%), more frequently associated with LVH criteria in group C than B (8%; 15%). Finally, patients from group D showed very peculiar ECG patterns,
BMJ Open, 2012
Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruct... more Effects of myocardial fibrosis assessed by MRI on dynamic left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy: a retrospective database analysis. BMJ Open 2012;2: e001267.
Orphanet Journal of Rare Diseases, Nov 19, 2019
Objective: To investigate differences in cardiac manifestations of patients affected by laminopat... more Objective: To investigate differences in cardiac manifestations of patients affected by laminopathy, according to the presence or absence of neuromuscular involvement at presentation. Methods: We prospectively analyzed 40 consecutive patients with a diagnosis of laminopathy followed at a single centre between 1998 and 2017. Additionally, reports of clinical evaluations and tests prior to referral at our centre were retrospectively evaluated. Results: Clinical onset was cardiac in 26 cases and neuromuscular in 14. Patients with neuromuscular presentation experienced first symptoms earlier in life (11 vs 39 years; p < 0.0001) and developed atrial fibrillation/flutter (AF) and required pacemaker implantation at a younger age (28 vs 41 years [p = 0.013] and 30 vs 44 years [p = 0.086] respectively), despite a similar overall prevalence of AF (57% vs 65%; p = 0.735) and atrio-ventricular (A-V) block (50% vs 65%; p = 0.500). Those with a neuromuscular presentation developed a cardiomyopathy less frequently (43% vs 73%; p = 0.089) and had a lower rate of sustained ventricular tachyarrhythmias (7% vs 23%; p = 0.387). In patients with neuromuscular onset rhythm disturbances occurred usually before evidence of cardiomyopathy. Despite these differences, the need for heart transplantation and median age at intervention were similar in the two groups (29% vs 23% [p = 0.717] and 43 vs 46 years [p = 0.593] respectively). Conclusions: In patients with laminopathy, the type of disease onset was a marker for a different natural history. Specifically, patients with neuromuscular presentation had an earlier cardiac involvement, characterized by a linear and progressive evolution from rhythm disorders (AF and/or A-V block) to cardiomyopathy.
Journal of the American College of Cardiology, 2019
BACKGROUND Scientific guidelines consider atrial fibrillation (AF) complicating degenerative mitr... more BACKGROUND Scientific guidelines consider atrial fibrillation (AF) complicating degenerative mitral regurgitation (DMR) a debated indication for surgery. OBJECTIVES This study analyzed the prognostic/therapeutic implications of AF at DMR diagnosis and long-term. METHODS Patients were enrolled in the MIDA (Mitral Regurgitation International Database) registry, which reported the consecutive, multicenter, international experience with DMR due to flail leaflets echocardiographically diagnosed. RESULTS Among 2,425 patients (age 67 AE 13 years; 71% male, 67% asymptomatic, ejection fraction 64 AE 10%), 1,646 presented at diagnosis with sinus rhythm (SR), 317 with paroxysmal AD, and 462 with persistent AF. Underlying clinical/instrumental characteristics progressively worsened from SR to paroxysmal to persistent AF. During follow-up, paroxysmal and persistent AF were associated with excess mortality (10-year survival in SR and in paroxysmal and persistent AF was 74 AE 1%, 59 AE 3%, and 46 AE 2%, respectively; p < 0.0001), that persisted 20 years post-diagnosis and independently of all baseline characteristics (p values <0.0001). Surgery (n ¼ 1,889, repair 88%) was associated with better survival versus medical management, regardless of all baseline characteristics and rhythm (adjusted hazard ratio: 0.26; 95% confidence interval: 0.23 to 0.30; p < 0.0001) but post-surgical outcome remained affected by AF (10-year post-surgical survival in SR and in paroxysmal and persistent AF was 82 AE 1%, 70 AE 4%, and 57 AE 3%, respectively; p < 0.0001). CONCLUSIONS AF is a frequent occurrence at DMR diagnosis. Although AF is associated with older age and more severe presentation of DMR, it is independently associated with excess mortality long-term after diagnosis. Surgery is followed by improved survival in each cardiac rhythm subset, but persistence of excess risk is observed for each type of AF. Our study indicates that detection of AF, even paroxysmal, should trigger prompt consideration for surgery.
Circulation, 2016
Background: Mitral valve (MV) repair is preferred over replacement in clinical guidelines and is ... more Background: Mitral valve (MV) repair is preferred over replacement in clinical guidelines and is an important determinant of the indication for surgery in degenerative mitral regurgitation. However, the level of evidence supporting current recommendations is low, and recent data cast doubts on its validity in the current era. Accordingly, the aim of the present study was to analyze very long-term outcome after MV repair and replacement for degenerative mitral regurgitation with a flail leaflet. Methods: MIDA (Mitral Regurgitation International Database) is a multicenter registry enrolling patients with degenerative mitral regurgitation with a flail leaflet in 6 tertiary European and US centers. We analyzed the outcome after MV repair (n=1709) and replacement (n=213) overall, by propensity score matching, and by inverse probability-of-treatment weighting. Results: At baseline, patients undergoing MV repair were younger, had more comorbidities, and were more likely to present with a p...
European Heart Journal, Oct 1, 2022
JACC: Cardiovascular Imaging
EuroIntervention : journal of EuroPCR in collaboration with the Working Group on Interventional Cardiology of the European Society of Cardiology, Jan 3, 2017
Treatment of advanced ischemic cardiomyopathy is often challenging, with recurrent episodes of ca... more Treatment of advanced ischemic cardiomyopathy is often challenging, with recurrent episodes of cardiac decompensation and consequent need for multiple hospitalizations. Mixed outcomes have been previously reported after conventional surgical left ventricle(LV) reconstruction. Aim of this report is to describe a novel hybrid transcatheter technique to reconstruct the remodeled LV. In this manuscript, we provide a detailed step-by-step description of a novel procedure, called Less Invasive Ventricular Enhancement(LIVE), which reconstructs the remodeled LV by plication of anteroseptal LV scar, thus reducing the enlarged volume, decreasing wall stress and increasing ejection fraction. The procedure relies on the micro-anchoring technology of the Revivent-TC™ System (BioVentrix Inc.), and is performed by both a interventional cardiologist and cardiothoracic surgeon cooperating. After surgical access by means of a left mini-thoracotomy and percutaneous access via the right internal jugula...
Giornale italiano di cardiologia (2006), Mar 1, 2022
Mitral valve prolapse is a relatively common disease with a good overall prognosis. However, in s... more Mitral valve prolapse is a relatively common disease with a good overall prognosis. However, in specific clinical and instrumental contexts, patients at high risk of ventricular arrhythmias and sudden cardiac death can be identified. Female sex, history of palpitations or syncope, bi-leaflet myxomatous valve, ECG repolarization abnormalities in the inferior leads, complex ventricular arrhythmias, left ventricular fibrosis detected by cardiac magnetic resonance correlate with a higher risk clinical profile. Additionally, morpho-functional abnormalities of the mitral valve annulus, particularly mitral annulus disjunction, may cause a mechanical stretch at the inferior basal ventricular wall and posterior papillary muscles, predisposing to myocardial fibrosis and arrhythmias. A risk stratification strategy is needed to identify patients with mitral valve prolapse and/or mitral annulus disjunction at high risk of arrhythmias; however, few data are available. Further prospective multicenter studies are warranted, focusing on medical therapy, the role of implantable cardioverter-defibrillators for primary prevention, efficacy of targeted catheter ablation or mitral valve surgery.
McGraw-Hill eBooks, 2006
Approccio pratico ai problemi cardiologici connessi a oltre 175 condizioni patologiche e relativi... more Approccio pratico ai problemi cardiologici connessi a oltre 175 condizioni patologiche e relativi sintomi - Ciascuna patologia, ordinata alfabeticamente, viene rappresentata su due pagine affiancate in cui vengono riportate le informazioni essenziali su diagnosi e terapia - Tra le caratteristiche pi\uf9 significative del volume: percorsi diagnostici, diagnosi differenziali, raccomandazioni terapeutiche (incluse le indicazioni per procedure operative), trattamento delle complicanze e prognosi - Guida alla scelta delle indagini di laboratorio, delle metodiche di imaging e delle altre procedure diagnostiche - Utili suggerimenti sull\u2019opportunit\ue0 di una consulenza specialistica e sui criteri di ospedalizzazione e dimissione dei pazient
European Heart Journal, 2017
2. Sleep apnea screening should be considered as integral part of diagnostic and treatment manage... more 2. Sleep apnea screening should be considered as integral part of diagnostic and treatment management in HCM patients. 3. Our criteria may help identify those patients that may most benefit from sleep apnea treatment. 4. Further research is needed to explore whether proper diagnosis and subsequent treatment may improve long-term outcomes in patients with HCM Acknowledgement/Funding: Supported by European Regional Development Fund-Project FNUSA-ICRC (No. CZ.
European Heart Journal, 2020
Background Anderson Fabry (AF) disease is a X-linked lysosomal storage disorder with multiorgan i... more Background Anderson Fabry (AF) disease is a X-linked lysosomal storage disorder with multiorgan involvement. Cardiac disease, mainly represented by left ventricular hypertrophy (LVH) and arrhythmias, is the most frequent cause of premature death. It is well know that specific therapy is less effective after the development of LVH and myocardial fibrosis, therefore early cardiac detection (before LVH) is important. New cardiac magnetic resonance (CMR) parametric imaging techniques (T1 and T2 maps) enable myocardial tissue changes associated with AF disease. Purpose To evaluate the relationship between CMR tissue characterization and clinical and instrumental manifestations of AF disease to find early markers of cardiac involvement. Methods 31 AF patients (9 males, mean age 49±16 years) underwent ECG, echocardiogram and contrast CMR. TnI, BNP, pro-BNP and serum lyso-Gb3 were dosed. T1 mapping was performed in a pre-contrast acquisition with the modified Look-Locker inversion recovery ...
Circulation: Cardiovascular Imaging
Open Heart
ObjectivesTo describe a cohort of patients with arrhythmogenic left ventricular cardiomyopathy (A... more ObjectivesTo describe a cohort of patients with arrhythmogenic left ventricular cardiomyopathy (ALVC), focusing on the spectrum of the clinical presentations.MethodsPatients were retrospectively evaluated between January 2012 and June 2020. Diagnosis was based on (1) ≥3 contiguous segments with subepicardial/midwall late gadolinium enhancement in the left ventricle (LV) at cardiac magnetic resonance plus a likely pathogenic/pathogenic arrhythmogenic cardiomyopathy (AC) associated genetic mutation and/or familial history of AC and/or red flags for ALVC (ie, negative T waves in V4-6/aVL, low voltages in limb leads, right bundle branch block like ventricular tachycardia) or (2) pathology examination of explanted hearts or autoptic cases suffering sudden cardiac death (SCD). Significant right ventricular involvement was an exclusion criterion.ResultsFifty-two patients (63% males, age 45 years (31–53)) composed the study cohort. Twenty-one (41%) had normal echocardiogram, 13 (25%) a hypo...
A 40 years old Afro-Caribbean woman from Nigeria living in Italy for a few months presented becau... more A 40 years old Afro-Caribbean woman from Nigeria living in Italy for a few months presented because of pelvic pain due to uterine myoma. In view of surgical treatment she had ECG, chest X-ray and standard blood test done. The ECG showed first degree atrio-ventricular block and pathologic Q waves in inferior leads. The radiograph documented some lung congestion. She was asymptomatic and the family history for cardiovascular disease was negative. Echocardiography was performed and revealed asymmetrical septal hypertrophy (maximal wall thickness 24mm) with some left ventricular (LV) apical hypertrophy and right ventricular (RV) involvement with apical obliteration. Differential diagnoses were considered, specifically hypertrophic cardiomyopathy (HCM) with biventricular hypertrophy, cardiac mass or tumor, endomyocardial fibrosis (EMF) with biventricular involvement or the coexistence of HCM with asymmetrical septal hypertrophy and EMF limited to the RV. Contrast echocardiography with So...
Heart
ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry di... more ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).MethodsIn this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.ResultsShort PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ2 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predi...
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Papers by ferdinando pasquale