Background. Hyperthophic cardiomyopathy (HCM) is the most common genetic cardiac disease, defined by the presence of increased left ventricular wall thickness that is not solely explained by abnormal loading conditions. Purpose of the study The aim of this study was to analyze a large cohort of patients with HCM, evaluated at cardiomyopathies Outpatient Center of the University-Hospital of Padua, to compare the differences in terms of clinical characteristics, echocardiographic- electrocardiographic parameters and outcome in terms of survival based on arrhythmic and heart failure events and overall survival, between the patients with obstructive forms and those with non-obstructive forms. Methods. From 1 January 2018 to 31 December 2022 we recorded at least one cardiological examination, 12-lead ECG, 24-hour HOLTER dynamic ECG (or telemetric control of any PM-ICD) and TTE of 291 patients with HCM evaluated at cardiomyopathies Outpatient Center of the University-Hospital of Padua. Based on the presence of a peak LVOT gradient ≥ 30 mmHg we categorized our cohort of patients in two sub-population: patients with obstructive HCM (O-HCM) and those without obstruction (Non O-HCM). Results. From the total cohort of 291 patients, 205 patients (70%) belonged to " Non O-HCM" group and 86 patients (30%) to "O-HCM" group. Median follow-up duration was 6.4. Based on this study, compared to "Non O-HCM", "O- HCM" patients were significantly older (p=0.013 at diagnosis; p=0.02 at last visit) and they are more symptomatic in terms of chest pain (p= 0.007), dyspnea (p<0.001 at diagnosis; p= 0.06 at the last visit) and syncope (p<0.001). Regarding echocardiographic characteristics, the patients affected by obstructive HCM showed not only more severe hypertrophy in terms of higher LVWT (p<0.001 at diagnosis; p=0.002 at last visit), but also larger LA volume (p= 0.037 at last visit) and diameter (p= 0.01 at last visit), affected by a more severe mitral valve regurgitation (p<0.001 at diagnosis and at follow-up) and were more frequently affected by apical aneurisms (p=0.004). Concerning electrocardiographic abnormalities, the "O-HCM" showed more often LBBB (p< 0.001) and got PM (p=0.006). Among O-HCM, patients who remain "Still Obstructive" during follow-up, were more symptomatic in terms of dyspnea (p= 0.01 at last visit); had higher degree of mitral regurgitation (p=0.01) and just one patient in 15 who underwent surgical septal myectomy continued to show a significant gradient. From our statistical analyses we concluded that the patients with “O-HCM” had statistically significant lower rate of survival based on heart failure events, while for the survival based on arrhythmic events and the cardiac overall survival the difference was not statistically significant. Conclusion. Obstructive HCM patients, although treated with "optimal medical therapy" currently available, remain more symptomatic and tend to have a worse prognosis in terms of progressive heart failure when compared to non-obstructive ones.
Introduzione. La Cardiomiopatia Ipertrofica (CMI) rappresenta la più frequente malattia cardiaca geneticamente determinata ed è definita dalla presenza di aumentati spessori parietali a carico del ventricolo sinistro (VS) o di entrambi i ventricoli, non causata da condizioni di sovraccarico emodinamico. Obiettivi. Lo scopo di questo studio è quello di descrivere un’ampia popolazione di pazienti consecutivi affetti da CMI afferenti all’ambulatorio specialistico dell’Azienda Ospedaliera-Università di Padova, al fine di identificare le differenze nelle caratteristiche clinico-strumentali e prognostiche, in termini di sopravvivenza per eventi di scompenso cardiaco, eventi aritmici e la sopravvivenza per tutte le morti cardiache, tra la forma ostruttiva e la forma non ostruttiva di CMI. Metodi. Dal 1 Gennaio 2018 fino al 31 Dicembre 2022 una popolazione di 291 pazienti consecutivi con diagnosi di CMI è stata valutata presso l’ambulatorio specialistico per la CMI dell’Azienda Ospedaliera-Università di Padova. Abbiamo diviso questi pazienti in due gruppi: pazienti con “CMI ostruttiva” e pazienti con “CMI non ostruttiva”. Risultati. Dai 291 pazienti partecipanti a questo studio, 205 (70%) avevano una forma non ostruttiva per cui classificati nella coorte “CMI non ostruttiva” e 86 (30%) una forma ostruttiva per cui classificati nella coorte “CMI ostruttiva”. Il periodo di osservazione è compreso in un range tra i 6 mesi ed i 17 anni (comprendendo anche i pazienti già in follow-up), con una media di 6.4. Da questa analisi statistica è emersa che i pazienti della coorte “CMI ostruttiva” avevano un’età significativamente maggiore (p=0.013 alla diagnosi e p=0.02 all’ultimo controllo) ed erano più sintomatici in termini di dolore toracico (p=0.007), dispnea (p<0.001 alla diagnosi e p= 0.06 all’ultimo controllo) e sincope non vaso-vagale (p<0.001) rispetto alla coorte “CMI non ostruttiva”. Per quanto riguarda le caratteristiche ecocardiografiche i pazienti della coorte “CMI ostruttiva” avevano uno spessore del ventricolo sinistro maggiore (p<0.001 alla diagnosi e p=0.002 all’ultimo controllo), aumentate dimensioni dell’atrio sinistro sia in termini di diametro (p=0.01 all’ultimo controllo) che di volume (p=0.037 all’ultimo controllo) ed erano caratterizzati da un grado di rigurgito mitralico più severo (p<0.001 alla diagnosi e all’ultimo controllo) e da più frequente presenza di aneurisma apicale (p=0.004) rispetto alla coorte “CMI non ostruttiva”. Per quanto riguarda le caratteristiche elettrocardiografiche i pazienti della coorte “CMI ostruttiva” avevano maggiore prevalenza di blocco di branca sinistra (p<0.001) e andavano più frequentemente incontro ad impianto di pacemaker-ICD (p=0.006) rispetto alla coorte “CMI non ostruttiva”. Tra i pazienti con “CMI ostruttiva”, quelli che rimanevano “ancora ostruttivi” dopo terapia rappresentano circa la metà della coorte (48%), risultavano essere significativamente più sintomatici in termine di dispnea (p=0.01 all’ultimo controllo) e presentavano un rigurgito mitralico più severo (p=0.011) rispetto a quelli “non più ostruttivi”. Da notare che solo un paziente sottoposto a miectomia ha continuato ad avere un gradiente significativo dopo l’intervento. I pazienti con ostruzione presentano un rischio significativamente maggiore di sviluppare eventi di scompenso cardiaco (HR 2.5 CI 1.1-5.8, p 0.03). Per quanto riguarda la sopravvivenza per eventi aritmici e la sopravvivenza per tutte le morti cardiache, pur essendoci una più bassa sopravvivenza nei pazienti con forma ostruttiva questa non raggiunge la significatività statistica. Conclusioni. I pazienti con CMI ostruttiva, nonostante la terapia medica ottimale disponibile, rimangono più sintomatici e tendono ad avere una peggiore prognosi in termini di progressione verso lo scompenso cardiaco quando confrontati con i pazienti non ostruttivi.
Descrizione e follow-up di una popolazione di pazienti con cardiomiopatia ipertrofica ostruttiva
LLESHI, MARIZA
2022/2023
Abstract
Background. Hyperthophic cardiomyopathy (HCM) is the most common genetic cardiac disease, defined by the presence of increased left ventricular wall thickness that is not solely explained by abnormal loading conditions. Purpose of the study The aim of this study was to analyze a large cohort of patients with HCM, evaluated at cardiomyopathies Outpatient Center of the University-Hospital of Padua, to compare the differences in terms of clinical characteristics, echocardiographic- electrocardiographic parameters and outcome in terms of survival based on arrhythmic and heart failure events and overall survival, between the patients with obstructive forms and those with non-obstructive forms. Methods. From 1 January 2018 to 31 December 2022 we recorded at least one cardiological examination, 12-lead ECG, 24-hour HOLTER dynamic ECG (or telemetric control of any PM-ICD) and TTE of 291 patients with HCM evaluated at cardiomyopathies Outpatient Center of the University-Hospital of Padua. Based on the presence of a peak LVOT gradient ≥ 30 mmHg we categorized our cohort of patients in two sub-population: patients with obstructive HCM (O-HCM) and those without obstruction (Non O-HCM). Results. From the total cohort of 291 patients, 205 patients (70%) belonged to " Non O-HCM" group and 86 patients (30%) to "O-HCM" group. Median follow-up duration was 6.4. Based on this study, compared to "Non O-HCM", "O- HCM" patients were significantly older (p=0.013 at diagnosis; p=0.02 at last visit) and they are more symptomatic in terms of chest pain (p= 0.007), dyspnea (p<0.001 at diagnosis; p= 0.06 at the last visit) and syncope (p<0.001). Regarding echocardiographic characteristics, the patients affected by obstructive HCM showed not only more severe hypertrophy in terms of higher LVWT (p<0.001 at diagnosis; p=0.002 at last visit), but also larger LA volume (p= 0.037 at last visit) and diameter (p= 0.01 at last visit), affected by a more severe mitral valve regurgitation (p<0.001 at diagnosis and at follow-up) and were more frequently affected by apical aneurisms (p=0.004). Concerning electrocardiographic abnormalities, the "O-HCM" showed more often LBBB (p< 0.001) and got PM (p=0.006). Among O-HCM, patients who remain "Still Obstructive" during follow-up, were more symptomatic in terms of dyspnea (p= 0.01 at last visit); had higher degree of mitral regurgitation (p=0.01) and just one patient in 15 who underwent surgical septal myectomy continued to show a significant gradient. From our statistical analyses we concluded that the patients with “O-HCM” had statistically significant lower rate of survival based on heart failure events, while for the survival based on arrhythmic events and the cardiac overall survival the difference was not statistically significant. Conclusion. Obstructive HCM patients, although treated with "optimal medical therapy" currently available, remain more symptomatic and tend to have a worse prognosis in terms of progressive heart failure when compared to non-obstructive ones.File | Dimensione | Formato | |
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https://hdl.handle.net/20.500.12608/54921