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1.
Clin Cardiol ; 42(5): 530-535, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30851055

RESUMO

BACKGROUND: Titin-truncating variants (TTNtv) have been recognized as the most prevalent genetic cause of dilated cardiomyopathy. However, their effects on phenotypes of left ventricular non-compaction cardiomyopathy (LVNC) remain largely unknown. HYPOTHESIS: The presence of TTNtv may have an effect on the phenotype of LVNC. METHODS: TTN was comprehensively screened by targeted sequencing in a cohort of 83 adult patients with LVNC. Baseline and follow-up data of all participants were collected. The primary endpoint was a composite of death and heart transplantation. The secondary endpoint was heart failure (HF) events, a composite of HF-related death, heart transplantation, and HF hospitalization. RESULTS: Overall, 13 TTNtv were identified in 13 patients, with 9 TTNtv located in the A-band of titin. There was no significant difference in baseline characteristics between patients with and without TTNtv. During a median follow-up of 4.4 years, no significant difference in death and heart transplantation between the two groups was observed. However, more HF events occurred in TTNtv carriers than in non-carriers (P = 0.006). Multivariable analyses showed that TTNtv were associated with an increased risk of HF events independent of sex, age, and baseline cardiac function (hazard ratio: 3.25, 95% confidence interval: 1.50-7.01, P = 0.003). Sensitivity analysis excluding non-A-band TTNtv yielded similar results, but with less strength. CONCLUSIONS: The presence of TTNtv may be a genetic modifier of LVNC and confer a higher risk of HF events among adult patients. Studies of larger cohorts are needed to confirm our findings.


Assuntos
Conectina/genética , Variação Genética , Insuficiência Cardíaca/genética , Miocárdio Ventricular não Compactado Isolado/genética , Adulto , Progressão da Doença , Feminino , Predisposição Genética para Doença , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/cirurgia , Transplante de Coração , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/cirurgia , Masculino , Pessoa de Meia-Idade , Admissão do Paciente , Fenótipo , Medição de Risco , Fatores de Risco
3.
Circulation ; 138(4): 367-376, 2018 07 24.
Artigo em Inglês | MEDLINE | ID: mdl-29514799

RESUMO

BACKGROUND: Long-term outcomes for childhood left ventricular noncompaction (LVNC) are uncertain. We examined late outcomes for children with LVNC enrolled in a national population-based study. METHODS: The National Australian Childhood Cardiomyopathy Study includes all children in Australia with primary cardiomyopathy diagnosed before 10 years of age between 1987 and 1996. Outcomes for subjects with LVNC with a dilated phenotype (LVNC-D) were compared with outcomes for those with dilated cardiomyopathy. Propensity-score analysis was used for risk factor adjustment. RESULTS: There were 29 subjects with LVNC (9.2% of all cardiomyopathy subjects), with a mean annual incidence of newly diagnosed cases of 0.11 per 100 000 at-risk individuals. Congestive heart failure was the initial symptom in 24 of 29 subjects (83%), and 27 (93%) had LVNC-D. The median age at diagnosis was 0.3 (interquartile interval, 0.08-1.3) years. The median duration of follow-up was 6.8 (interquartile interval, 0.7-24.0) years for all subjects and 24.7 (interquartile interval, 23.3 - 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% confidence interval [CI], 30-65) at 10 years after diagnosis and 45% (95% CI, 27-63) at 15 years. In competing-risk analysis, 21% of subjects with LVNC were alive with normal left ventricular systolic function, and 31% were alive with abnormal function at 15 years. Propensity-score matching between subjects with LVNC-D and those with dilated cardiomyopathy suggested a lower freedom from death/transplantation at 15 years after diagnosis in the subjects with LVNC-D (LVNC-D, 46% [95% CI, 26-66] versus dilated cardiomyopathy, 70% [95% CI, 42-97]; P=0.08). Using propensity-score inverse probability of treatment-weighted Cox regression, we found evidence that LVNC-D was associated with a greater risk of death or transplantation (hazard ratio, 2.3; 95% CI, 1.4-3.8; P=0.0012). CONCLUSIONS: Symptomatic children with LVNC usually present in early infancy with a predominant dilated phenotype. Long-term outcomes are worse than for matched children with dilated cardiomyopathy.


Assuntos
Cardiomiopatia Dilatada , Insuficiência Cardíaca , Miocárdio Ventricular não Compactado Isolado , Austrália/epidemiologia , Cardiomiopatia Dilatada/diagnóstico por imagem , Cardiomiopatia Dilatada/mortalidade , Cardiomiopatia Dilatada/fisiopatologia , Cardiomiopatia Dilatada/terapia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/mortalidade , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/terapia , Transplante de Coração , Humanos , Incidência , Lactente , Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/terapia , Estudos Longitudinais , Masculino , Fenótipo , Prognóstico , Medição de Risco , Fatores de Risco , Fatores de Tempo , Função Ventricular Esquerda
5.
Circ Cardiovasc Imaging ; 10(9)2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28899950

RESUMO

BACKGROUND: Presence of prominent left ventricular trabeculation satisfying criteria for left ventricular noncompaction (LVNC) on routine cardiac magnetic resonance examination is frequently encountered; however, the clinical and prognostic significance of these findings remain elusive. This registry aimed to assess LVNC prevalence by 4 current criteria and to prospectively evaluate an association between diagnosis of LVNC by these criteria and adverse events. METHODS AND RESULTS: There were 700 patients referred for cardiac magnetic resonance: 42% were women, median age was 70 years (range, 45-71 years), mean left ventricular ejection fraction was 51% (±17%), and 32% had late gadolinium enhancement on cardiac magnetic resonance. The cohort underwent diagnostic assessment for LVNC by 4 separate imaging criteria-referenced by their authors as Petersen, Stacey, Jacquier, and Captur, with LVNC prevalence of 39%, 23%, 25% and 3%, respectively. Primary clinical outcome was combined end point of time to death, ischemic stroke, ventricular tachycardia/ventricular fibrillation, and heart failure hospitalization. Secondary clinical outcomes were (1) all-cause mortality and (2) time to the first occurrence of any of the following events: cardiac death, ischemic stroke, ventricular tachycardia/ventricular fibrillation, or heart failure hospitalization. During a median follow-up of 7 years, there were no statistically significant differences in assessed outcomes noted between patients with and without LVNC irrespective of the applied criteria. CONCLUSIONS: Current criteria for the diagnosis of LVNC leads to highly variable disease prevalence in patients referred for cardiac magnetic resonance. The diagnosis of LVNC, by any current criteria, was not associated with adverse clinical events on nearly 7 years of follow-up. Limited conclusions can be made for Captur criteria due to low observed prevalence.


Assuntos
Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/epidemiologia , Imagem Cinética por Ressonância Magnética , Encaminhamento e Consulta , Idoso , Isquemia Encefálica/epidemiologia , Meios de Contraste/administração & dosagem , Intervalo Livre de Doença , Feminino , Insuficiência Cardíaca/epidemiologia , Hospitalização , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Pessoa de Meia-Idade , Cidade de Nova Iorque/epidemiologia , Variações Dependentes do Observador , Valor Preditivo dos Testes , Prevalência , Modelos de Riscos Proporcionais , Estudos Prospectivos , Sistema de Registros , Reprodutibilidade dos Testes , Fatores de Risco , Acidente Vascular Cerebral/epidemiologia , Volume Sistólico , Taquicardia Ventricular/epidemiologia , Fatores de Tempo , Fibrilação Ventricular/epidemiologia , Função Ventricular Esquerda
6.
J Am Heart Assoc ; 6(9)2017 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-28855170

RESUMO

BACKGROUND: Left ventricular noncompaction (LVNC) has since been classified as a primary genetic cardiomyopathy, but the genetic basis is not fully evaluated. The aim of the present study was to identify the genetic spectrum using next-generation sequencing and to evaluate genotype-phenotype correlations in LVNC patients. METHODS AND RESULTS: Using next-generation sequencing, we targeted and sequenced 73 genes related to cardiomyopathy in 102 unrelated LVNC patients. We identified 43 pathogenic variants in 16 genes in 39 patients (38%); 28 were novel variants. Sarcomere gene variants accounted for 63%, and variants in genes associated with channelopathies accounted for 12%. MYH7 and TAZ pathogenic variants were the most common, and rare variant collapsing analysis showed variants in these genes contributed to the risk of LVNC, although patients carrying MYH7 and TAZ pathogenic variants displayed different phenotypes. Patients with pathogenic variants had early age of onset and more severely decreased left ventricular ejection fractions. Survival analysis showed poorer prognosis in patients with pathogenic variants, especially those with multiple variants: All died before their first birthdays. Adverse events were noted in 17 patients, including 13 deaths, 3 heart transplants, and 1 implantable cardioverter-defibrillator insertion. Congestive heart failure at diagnosis and pathogenic variants were independent risk factors for these adverse events. CONCLUSIONS: Next-generation sequencing revealed a wide spectrum of genetic variations and a high incidence of pathogenic variants in LVNC patients. These pathogenic variants were independent risk factors for adverse events. Patients harboring pathogenic variants showed poor prognosis and should be followed closely.


Assuntos
Análise Mutacional de DNA/métodos , Sequenciamento de Nucleotídeos em Larga Escala , Miocárdio Ventricular não Compactado Isolado/genética , Mutação , Polimorfismo de Nucleotídeo Único , Função Ventricular Esquerda/genética , Pré-Escolar , Desfibriladores Implantáveis , Intervalo Livre de Doença , Cardioversão Elétrica/instrumentação , Feminino , Frequência do Gene , Estudos de Associação Genética , Marcadores Genéticos , Predisposição Genética para Doença , Transplante de Coração , Humanos , Lactente , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/terapia , Japão , Estimativa de Kaplan-Meier , Masculino , Fenótipo , Valor Preditivo dos Testes , Fatores de Tempo
7.
Acta Cardiol ; 70(5): 588-93, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26567819

RESUMO

OBJECTIVE: Although the clinical importance of left ventricular noncompaction cardiomyopathy (LVNC) is known, few data exist that describe the prognosis associated with intermediate levels of LV trabeculations that do not meet criteria for LVNC. METHODS: Trabeculation/possible LVNC by CMR was retrospectively observed among 122 consecutive cases. We assessed the end-systolic noncompacted-to-compacted ratios (ESNCCR) along with deaths, embolic events, congestive heart failure (CHF) readmissions, ventricular arrhythmias, myocardial thickening (MT), and ejection fraction (EF). ESNCCRs were categorized as follows: <1, 1<1.5, 1.5<2, ≥2. General linear models were used to compare combined events (death, CHF readmission, embolism, ventricular arrhythmia) between categories of ESNCCR. There were 3 models used: model 1: unadjusted; model 2: adjusted for age, race, gender, body surface area, LV ejection fraction, and trabeculated segments; model 3: model 2+adjustment for myocardial thickening. RESULTS: In model 1, those with an ESNCCR<1 had a lower association with composite clinical events than those with a ratio between 1.5<2 and those≥2 (P<0.002 and P<0.001, respectively). In model 2, the lower association continued, (P=0.009 and P<0.001, respectively), but in model 3, those with a ratio from 1.5-2 only had a trend towards a higher association with composite clinical events than those with a ratio<1 (P=-0.09). Those with a ratio≥2 continued to have a higher association (P=-0.001). CONCLUSION: Patients with intermediate trabeculations not meeting criteria for LVNC had a higher association with composite clinical events, but it was mediated by decreased myocardial thickening in the associated compacted layer.


Assuntos
Ventrículos do Coração/patologia , Miocárdio Ventricular não Compactado Isolado/patologia , Imagem Cinética por Ressonância Magnética , Miocárdio/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/terapia , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Função Ventricular Esquerda
8.
J Card Fail ; 21(11): 877-84, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26164213

RESUMO

BACKGROUND: Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children. METHODS AND RESULTS: According to diagnoses from the National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC. Each LVNC patient was also classified as having an associated echocardiographically diagnosed cardiomyopathy phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, or indeterminate. The time to death or transplantation differed among the phenotypic groups (P = .035). Time to listing for cardiac transplantation significantly differed by phenotype (P < .001), as did time to transplantation (P = .015). The hazard ratio for death/transplantation (with isolated LVNC as the reference group) was 4.26 (95% confidence interval [CI] 0.78-23.3) for HCM, 6.35 (95% CI 1.52-26.6) for DCM, and 5.66 (95% CI 1.04-30.9) for the indeterminate phenotype. Most events occurred in the 1st year after diagnosis. CONCLUSIONS: LVNC is present in at least 5% of children with cardiomyopathy. The specific LVNC-associated cardiomyopathy phenotype predicts the risk of death or transplantation and should inform clinical management.


Assuntos
Cardiomiopatias/genética , Cardiomiopatias/mortalidade , Miocárdio Ventricular não Compactado Isolado/genética , Miocárdio Ventricular não Compactado Isolado/mortalidade , Fenótipo , Sistema de Registros , Canadá , Cardiomiopatias/fisiopatologia , Causas de Morte , Criança , Pré-Escolar , Feminino , Predisposição Genética para Doença/epidemiologia , Humanos , Incidência , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Estimativa de Kaplan-Meier , Masculino , Análise Multivariada , Pediatria , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Análise de Sobrevida , Estados Unidos
9.
J Cardiovasc Magn Reson ; 17: 44, 2015 May 30.
Artigo em Inglês | MEDLINE | ID: mdl-26024839

RESUMO

BACKGROUND: Although cardiovascular magnetic resonance (CMR) is showing increasingly diagnostic potential in left ventricular non-compaction (LVNC), relatively little research relevant to CMR is conducted in children with LVNC. This study was performed to characterize and compare CMR features and clinical outcomes in children with LVNC with and without late gadolinium enhancement (LGE). METHODS: A cohort of 40 consecutive children (age, 13.7 ± 3.3 years; 29 boys and 11 girls) with isolated LVNC underwent a baseline CMR scan with subsequent clinical follow-up. Short-axis cine images were used to calculate left ventricular (LV) ejection fraction (EF), end-diastolic volume (EDV), end-systolic volume (ESV), myocardial mass, ratio of non-compacted-to-compacted myocardial thickness (NC/C ratio), and number of non-compacted segments. The LGE images were analyzed to assess visually presence and patterns of LGE. The primary end point was a composite of cardiac death and heart transplantation. RESULTS: The LGE was present in 10 (25%) children, and 46 (27%) segments were involved, including 23 non-compacted segments and 23 normal segments. Compared with LGE- cohort, LGE+ cohort had significantly lower LVEF (23.8 ± 10.7% vs. 42.9 ± 16.7%, p < 0.001) and greater LVEDV (169.2 ± 65.1 vs. 118.2 ± 48.9 mL/m2, p = 0.010), LVESV (131.3 ± 55.5 vs. 73.3 ± 46.7 mL/m2, p = 0.002), and sphericity indices (0.75 ± 0.19 vs. 0.60 ± 0.20, p = 0.045). There were no differences in terms of number and distribution of non-compacted segments, NC/C ratio, and myocardial mass index between LGE+ and LGE- cohort. In the LGE+ cohort, adverse events occurred in 6 patients compared to 2 events in the LGE- cohort. Kaplan-Meier analysis showed a significant difference in outcome between LGE+ and LGE- cohort for cardiac death and heart transplantation (p = 0.011). CONCLUSIONS: The LGE was present in up to one-fourth of children with LVNC, and the LGE+ children exhibited a more maladaptive LV remodeling and a higher incidence of cardiovascular death and heart transplantation.


Assuntos
Meios de Contraste , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Imagem Cinética por Ressonância Magnética , Contração Miocárdica , Miocárdio/patologia , Volume Sistólico , Função Ventricular Esquerda , Adolescente , Fatores Etários , Criança , China , Progressão da Doença , Feminino , Gadolínio DTPA , Transplante de Coração , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/patologia , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/cirurgia , Estimativa de Kaplan-Meier , Masculino , Valor Preditivo dos Testes , Estudos Prospectivos , Fatores de Risco , Fatores de Tempo
12.
Heart Vessels ; 29(5): 645-52, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24085471

RESUMO

This study was undertaken to evaluate the clinical course of isolated left ventricular noncompaction (ILVNC) and to identify the predictors for adverse outcomes in an adult cohort with ILVNC. Between March 2003 and April 2012, 106 adult patients diagnosed with ILVNC at Fuwai Hospital were included in this study. The medical history, electrocardiograms, and echocardiograms of these patients were retrospectively analyzed by chart review. Of these patients, 64 (60 %) were in New York Heart Association (NYHA) functional class III/IV and 84 (79 %) had systolic dysfunction (left ventricular ejection fraction (LVEF) <50 %). During a follow-up of 2.9 ± 2.1 years, 28 (26 %) patients died or underwent heart transplantation. The annual incidence of death or transplantation was 9.1 %. The determinants of death or heart transplantation included NYHA functional class III/IV (hazard ratio (HR) 4.52; 95 % confidence interval (CI) 1.57-13.04; P = 0.005), decreased left ventricular ejection fraction (HR 0.94; 95 % CI 0.90-0.97; P = 0.001), dilated left ventricular end-diastolic diameter (HR, 1.06; 95 % CI, 1.02-1.09; P = 0.001), increased left atrial diameter (HR 1.08; 95 % CI 1.03-1.14; P = 0.001), reduced systolic blood pressure (HR 0.96; 95 % CI 0.94-0.99; P = 0.003), the presence of pulmonary hypertension (HR 3.50; 95 % CI 1.63-7.51; P = 0.001), and right bundle branch block (HR 7.79; 95 % CI 2.56-23.76; P < 0.001). In conclusion, this study demonstrates that ILVNC is related to a high incidence of death or heart transplantation. Advanced heart failure, a dilated left heart with systolic dysfunction, reduced systolic blood pressure, pulmonary hypertension, and right bundle branch block predict adverse outcomes of ILVNC.


Assuntos
Miocárdio Ventricular não Compactado Isolado/diagnóstico , Adolescente , Adulto , Idoso , Pressão Arterial , Bloqueio de Ramo/diagnóstico , Bloqueio de Ramo/fisiopatologia , China , Progressão da Doença , Ecocardiografia , Eletrocardiografia , Feminino , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Transplante de Coração , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Hipertrofia Ventricular Esquerda/diagnóstico , Hipertrofia Ventricular Esquerda/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/terapia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Fatores de Risco , Volume Sistólico , Fatores de Tempo , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/fisiopatologia , Função Ventricular Esquerda , Adulto Jovem
13.
Circulation ; 127(22): 2202-8, 2013 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-23633270

RESUMO

BACKGROUND: Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death. METHODS AND RESULTS: We retrospectively reviewed all children diagnosed with left ventricular noncompaction at Texas Children's Hospital from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty-two children were diagnosed with isolated left ventricular noncompaction over the study period. Thirty-one (12.8%) died, and 13 (5.4%) were received a transplant. One hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (hazard ratio, 11; P<0.001). ECG abnormalities were present in 87%, with ventricular hypertrophy and repolarization abnormalities occurring most commonly. Repolarization abnormalities were associated with increased mortality (hazard ratio, 2.1; P=0.02). Eighty children (33.1%) had an arrhythmia, and those with arrhythmias had increased mortality (hazard ratio, 2.8; P=0.002). Forty-two (17.4%) had ventricular tachycardia, with 5 presenting with resuscitated sudden cardiac death. In total, there were 15 cases of sudden cardiac death in the cohort (6.2%). Nearly all patients with sudden death (14 of 15) had abnormal cardiac dimensions or cardiac dysfunction. No patient with normal cardiac dimensions and function without preceding arrhythmias died. CONCLUSIONS: Left ventricular noncompaction has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death.


Assuntos
Cardiomiopatias/mortalidade , Morte Súbita Cardíaca/epidemiologia , Miocárdio Ventricular não Compactado Isolado/mortalidade , Disfunção Ventricular Esquerda/mortalidade , Adolescente , Flutter Atrial/mortalidade , Cardiomiopatias/diagnóstico por imagem , Criança , Pré-Escolar , Ecocardiografia Doppler , Feminino , Hospitais Pediátricos/estatística & dados numéricos , Humanos , Incidência , Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Encaminhamento e Consulta/estatística & dados numéricos , Estudos Retrospectivos , Fatores de Risco , Taquicardia/mortalidade , Centros de Atenção Terciária/estatística & dados numéricos , Disfunção Ventricular Esquerda/diagnóstico por imagem
14.
Circ J ; 76(7): 1556-62, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22664784

RESUMO

Diagnosing left ventricular noncompaction (LVNC) cardiomyopathy is a challenge for the medical community because the condition shares morphologic features of hypertrophic and dilated cardiomyopathies. The uncertainty surrounding the diagnosis of LVNC is related to the lack of a "perfect diagnostic tool," such as a reproducible genetic marker. The diagnosis requires expertise in the broad spectrum of overlapping cardiomyopathies. The demarcation between LVNC and normal phenotypic variations is often indistinct. Echocardiography, used in routine clinical practice to identify the typical morphologic features of LVNC, can be overly sensitive and lack specificity with the presently defined measurements and ratios used to diagnose LVNC. The available diagnostic criteria show a propensity toward overdiagnosing LVNC. The complex clinical sequelae of atrial and ventricular arrhythmias, heart failure, thromboembolic events and sudden death associated with LVNC make a valid and reproducible diagnosis critical. The trend to using a morphologic/pathophysiologic, instead of a solely morphologic, approach holds promise in the quest for an accurate, reliable diagnosis of LVNC. We must understand the distinction between morphological findings and morphological findings with pathophysiology. Our future understanding of LVNC depends on an integration of cardiac morphology, physiology, pathophysiology and evolving genetics.


Assuntos
Diagnóstico por Imagem , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Adolescente , Adulto , Arritmias Cardíacas/etiologia , Arritmias Cardíacas/mortalidade , Fenômenos Biomecânicos , Morte Súbita Cardíaca/etiologia , Diagnóstico Diferencial , Diagnóstico por Imagem/métodos , Ecocardiografia , Fibrose , Predisposição Genética para Doença , Humanos , Miocárdio Ventricular não Compactado Isolado/complicações , Miocárdio Ventricular não Compactado Isolado/genética , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Imagem por Ressonância Magnética , Masculino , Miocárdio/patologia , Fenótipo , Valor Preditivo dos Testes , Prognóstico , Função Ventricular Esquerda , Adulto Jovem
15.
Can J Cardiol ; 28(4): 508-14, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22445100

RESUMO

BACKGROUND: Fragmented QRS complexes (fQRS) were proven to be associated with the prognosis of several heart diseases. However, no data is available regarding fQRS in left ventricular noncompaction cardiomyopathy (LVNC), in which the outcome varies greatly and a simple yet practicable prognostic predictor is needed. The purpose of this study was to determine the prognostic value of fQRS in LVNC patients. METHODS: Sixty-four LVNC patients were evaluated. Fragmented narrow QRS (f-nQRS) included single or multiple notches in the R or S wave in at least 2 contiguous electrocardiogram (ECG) leads and QRS duration < 120 ms, fragmented wide QRS (f-wQRS) included more than 2 notches and QRS duration > 120 ms. RESULTS: f-nQRS and f-wQRS was present in 24 (38%) and 7 (11%) patients respectively. During follow-up, 13 patients died and 7 patients underwent heart transplantation. Kaplan-Meier analysis revealed that compared with the non-f-nQRS group, the f-nQRS group associated with a significantly lower survival (P = 0.005). The f-wQRS group also demonstrated a substantially lower survival as compared with the non-f-wQRS group (P = 0.02). Multivariate analysis indicated f-nQRS was an independent predictor of all-cause mortality (HR: 5.33; P = 0.045). CONCLUSIONS: In LVNC patients, the presence of f-nQRS has significant prognostic value and may provide a valid method of risk stratification.


Assuntos
Eletrocardiografia , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Processamento de Sinais Assistido por Computador , Adulto , Terapia de Ressincronização Cardíaca , Causas de Morte , Desfibriladores Implantáveis , Ecocardiografia , Eletrocardiografia Ambulatorial , Endocárdio/diagnóstico por imagem , Feminino , Seguimentos , Transplante de Coração , Ventrículos do Coração/diagnóstico por imagem , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/terapia , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Prognóstico
16.
J Am Soc Echocardiogr ; 25(2): 194-202, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22036126

RESUMO

BACKGROUND: Left ventricular noncompaction (LVNC) cardiomyopathy is variably defined by numerous trabeculations, deep intertrabecular recesses, and noncompacted-to-compacted (NC/C) ratio >2. Limited studies exist on the reproducibility of diagnosing LVNC. METHODS: Clinical records of patients diagnosed with LVNC by echocardiography were reviewed. Blinded review of the index echocardiogram for all patients and a 1:1 match without LVNC was performed independently by two observers, measuring the number of trabeculations and the NC/C ratio. RESULTS: A total of 104 patients with LVNC were included in the study, 52 with no congenital heart disease (NCongHD) and 52 with congenital heart disease (CongHD). The duration of follow-up was 7.2 years (range, 0.5-23.1 years) for NCongHD and 8.2 years (range, 0-33.3 years) for CongHD. Agreement between observers in determining zero to three versus more than three trabeculations was 59% (NCongHD) and 73% (CongHD). Agreement in measuring an NC/C ratio ≤ 2 versus > 2 was 79% (NCongHD) and 74% (CongHD). Agreement with the original reader in diagnosing LVNC was 67%. There was no association between the number of trabeculations or the NC/C ratio and the likelihood of a major event. Patients with moderate or severe left ventricular dysfunction at the time of diagnosis were more likely to undergo cardiac transplantation or die compared with those with normal or mild dysfunction (NCongHD, 22% vs 0%, P = .01; CongHD, 39% vs 3%, P = .001). CONCLUSIONS: The reproducibility of making measurements to diagnose LVNC by accepted criteria is poor. Heart transplantation and death are associated with significant ventricular dysfunction and not with increased trabeculations or NC/C ratios.


Assuntos
Ecocardiografia/estatística & dados numéricos , Insuficiência Cardíaca/diagnóstico por imagem , Insuficiência Cardíaca/mortalidade , Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/mortalidade , Adolescente , Adulto , Boston/epidemiologia , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Reprodutibilidade dos Testes , Medição de Risco , Fatores de Risco , Sensibilidade e Especificidade , Método Simples-Cego , Análise de Sobrevida , Taxa de Sobrevida , Adulto Jovem
17.
J Card Fail ; 17(9): 771-8, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21872148

RESUMO

BACKGROUND: Owing to inconsistent diagnostic criteria and small heterogeneous cohorts, little is known about the long-term outcomes of adult left ventricular noncompaction (LVNC), a rare cardiomyopathy with potentially serious outcomes. This systematic overview aimed to better delineate the natural history of adult LVNC. METHOD AND RESULTS: A comprehensive computerized search using "noncompaction" and its synonyms initially identified 206 articles, with reference lists subsequently hand scanned. These searches yielded 5 studies that were eligible for this systematic overview, identifying adult cohorts with isolated LVNC diagnosed by similar echocardiographic criteria. This combined cohort (n = 241) was followed for a mean duration of 39 months. The annualized event rate was 4% for cardiovascular deaths, 6.2% for cardiovascular death and its surrogates (heart transplantation and appropriate implantable cardioverter-defibrillator shocks), and 8.6% for all cardiovascular events (death, stroke, implantable cardioverter-defibrillator shocks, and heart transplantation.) Familial occurrence of LVNC in first-degree relatives was identified by echocardiography in 30% of index cases who were screened. CONCLUSION: LVNC is an increasingly recognized cardiomyopathy diagnosed by echocardiography and is associated with familial tendencies, arrhythmias, thromboembolism, advanced heart failure, and death.


Assuntos
Miocárdio Ventricular não Compactado Isolado/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/terapia , Adulto , Fatores Etários , Ensaios Clínicos como Assunto/métodos , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Ultrassonografia
18.
Circ J ; 75(7): 1728-34, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-21617326

RESUMO

BACKGROUND: Abnormal baseline electrocardiograms (ECGs) are common in patients with isolated left ventricular noncompaction (IVNC). Whether certain electrocardiographic parameters are associated with a poor clinical outcome, however, remains elusive. The present study was therefore designed to comprehensively assess the predictive value of baseline ECG findings in patients newly diagnosed with IVNC. METHODS AND RESULTS: 74 patients diagnosed with IVNC were included in the analysis. During follow-up, 8 patients (11%) died of a cardiovascular cause or underwent heart transplantation (primary outcome measure). On univariate analysis, several variables, including repolarization abnormalities (ST segment elevation/depression, T-wave inversion) in the inferior leads (5-year estimator: 67.1 ± 10.7% vs. 98 ± 2.2%; P = 0.001), an increase in PQ- (hazard ratio (HR) 1.032, P=0.004) and QTc-duration (HR 1.037, P=0.001), were predictive of cardiovascular death or heart transplantation. On multivariate analysis, only PQ- and QTc-duration and the presence of repolarization abnormalities in the inferior leads remained significantly predictive of a poor outcome. CONCLUSIONS: PQ duration, QTc duration, and repolarization abnormalities in the inferior leads are independently predictive of a poor prognosis in IVNC. Further prospective studies are required to conclusively investigate the usefulness of baseline ECG parameters for risk stratification in patients with IVNC.


Assuntos
Eletrocardiografia/métodos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Adulto , Feminino , Seguimentos , Humanos , Miocárdio Ventricular não Compactado Isolado/mortalidade , Estimativa de Kaplan-Meier , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Sensibilidade e Especificidade
20.
Interact Cardiovasc Thorac Surg ; 12(3): 370-3, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21172941

RESUMO

Isolated left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep intratrabecular recesses. In this study, we aimed to identify the clinical characteristics of children with ventricular non-compaction and determine the factors affecting prognosis. We retrospectively evaluated 29 children with LVNC followed at Dr. Sami Ulus Children Hospital Pediatric Cardiology Department from December 2004 to November 2009. There were 13 females (45%) and 16 males (55%) and the mean age at presentation was 4.8±4.6 years (one month-15 years). Although there was no statistical significance; early presentation age and high left ventricular end-diastolic diameter at the diagnosis were associated with poorer prognosis.


Assuntos
Anormalidades Múltiplas , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Adolescente , Idade de Início , Criança , Pré-Escolar , Progressão da Doença , Ecocardiografia Doppler em Cores , Eletrocardiografia , Eletrocardiografia Ambulatorial , Feminino , Humanos , Lactente , Miocárdio Ventricular não Compactado Isolado/mortalidade , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/terapia , Masculino , Miocárdio/patologia , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Turquia , Função Ventricular Esquerda
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