Dilated-Hypokinetic Evolution of Hypertrophic Cardiomyopathy Prevalence, Incidence, Risk Factors, and Prognostic Implications in Pediatric and Adult Patients

ObjectivesThis study sought to investigate the incidence, risk factors, and prognosis of dilated-hypokinetic evolution in a large cohort of patients with hypertrophic cardiomyopathy (HCM) followed up at a cardiology center serving both the pediatric and the adult population.BackgroundThe available data on this evolution of HCM mainly regards prevalence (rather than incidence) in adults, with very little being known about the pediatric population.MethodsA total of 222 consecutive HCM patients (65% men, 19% ≤18 years old) were prospectively evaluated for a mean follow-up of 11 ± 9 years.ResultsA diagnosis of dilated-hypokinetic HCM was made in 12 patients at first evaluation (11 without previous septal myectomy surgery; prevalence, 4.9%). Twelve of the 210 patients with classic HCM at first evaluation underwent dilated-hypokinetic evolution (incidence, 5.3/1,000 patient-years). Patients with prevalent/incident dilated-hypokinetic evolution were younger at first evaluation (32 ± 14 years vs. 41 ± 21 years, p = 0.04) and more often had a family history of HCM (61% vs. 26%, p = 0.002) or sudden death (43% vs. 19%, p = 0.01) with respect to patients who maintained classic HCM. Moreover, they showed greater interventricular septum (23 ± 3 mm vs. 19 ± 6 mm, p = 0.004) and posterior wall (15 ± 3 mm vs. 13 ± 4 mm, p = 0.006) thickness. Cardiovascular death-free survival was lower among patients with dilated-hypokinetic HCM (p < 0.04). Cox proportional hazards regression analysis identified left ventricular wall thickness (hazard ratio [HR] = 1.07; 95% confidence interval [CI], 1.01 to 1.14; p = 0.03) and end-diastolic diameter (HR = 1.08; 95% CI 1.04 to 1.11; p = 0.0001) as independent predictors of cardiovascular death.ConclusionsDilated-hypokinetic evolution is rare but not exceptional in HCM. Young age at diagnosis, family history of HCM, and greater wall thickness are incremental risk factors for dilated-hypokinetic HCM, which carries an ominous prognosis

Cardiologia

I progressi costantemente realizzati nei diversi ambiti della fisiopatologia e della cardiologia clinica hanno reso indispensabile la pubblicazione di un manuale per gli studenti del Corso di Laurea in Medicina e Chirurgia aggiornato e innovativo, che si ponga come testo di riferimento per lo studio della patologia cardiovascolare. Ideato e realizzato in sintonia con il programma dei corsi e le esigenze di apprendimento, chiaro nell'esposizione, accurato e documentato nella presentazione delle applicazioni strumentali, arricchito dagli apparati didattici che caratterizzano la collana, "Core Curriculum. Cardiologia" affronta e risolve ad alto livello gli argomenti cardine della disciplina, come gli sviluppi nel trattamento medicamentoso e interventistico delle aritmie, delle cardiopatie congenite e della cardiopatia ischemica, le possibilità di trattamento chirurgico e interventistico delle valvulopatie, il riconoscimento e la classificazione delle cardiomiopatie, le possibilità preventive e terapeutiche dello scompenso cardiaco grave e dell'ipertensione polmonare

Rasmussen's Aneurysm in Childhood: A Case Report

In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosi

Determinants of Cardiopulmonary Functional Improvement After Transcatheter Atrial Septal Defect Closure in Asymptomatic Adults.

none8mixedA.GIARDINI; A.DONTI; S.SPECCHIA; D.PRANDSTRALLER; R.FORMIGARI; G.BRONZETTI; M.BONVICINI; PICCHIO F.A.GIARDINI; A.DONTI; S.SPECCHIA; D.PRANDSTRALLER; R.FORMIGARI; G.BRONZETTI; M.BONVICINI; PICCHIO F