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1.
Cardiol Young ; 29(9): 1208-1210, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31379312

RESUMO

We present the case of a 3-year-old boy with bicuspid aortic valve, aortic coarctation, and left ventricular non-compaction. The diagnosis was made post-natally with ultrasonography and was verified by cardiac MRI. Aortic coarctation was initially repaired surgically. At age 3 months, recoarctation and heart failure developed. Balloon angioplasty was performed with immediate improvement. At age 3 years, the patient remains asymptomatic and normotensive.


Assuntos
Anormalidades Múltiplas , Coartação Aórtica/diagnóstico , Valva Aórtica/anormalidades , Ecocardiografia Doppler/métodos , Doenças das Valvas Cardíacas/diagnóstico , Imagem Cinética por Ressonância Magnética/métodos , Coartação Aórtica/cirurgia , Procedimentos Cirúrgicos Cardíacos/métodos , Pré-Escolar , Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/cirurgia , Masculino
2.
J Cardiovasc Pharmacol Ther ; 24(1): 31-36, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29911432

RESUMO

In 2 distinct entities, left ventricular noncompaction (LVNC) and peripartum cardiomyopathy (PPCM), routine anticoagulation therapy is often used in current practices. However, our systematic review showed that LVNC itself was not associated with the increase in thromboembolism event rates and therapeutic anticoagulation therapy should not be considered only for LVNC, unless there is risk factor for thromboembolism. Current literature justifies prophylactic therapeutic anticoagulation in LVNC with low left ventricular ejection fraction (EF < 40%) and/or atrial fibrillation. Although not specifically studied, the presence of intracardiac thrombi by echocardiography or other imaging studies should also prompt anticoagulation therapy. There is limited evidence available for the use of anticoagulation in patients with PPCM, but our systematic review showed that anticoagulation should be recommended only for patients with PPCM especially with an EF < 35% until EF is recovered, as well as for patients with PPCM treated with bromocriptine.


Assuntos
Anticoagulantes/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Cardiomiopatias/tratamento farmacológico , Miocárdio Ventricular não Compactado Isolado/tratamento farmacológico , Transtornos Puerperais/tratamento farmacológico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anticoagulantes/efeitos adversos , Cardiomiopatias/sangue , Cardiomiopatias/diagnóstico , Cardiomiopatias/fisiopatologia , Tomada de Decisão Clínica , Feminino , Humanos , Miocárdio Ventricular não Compactado Isolado/sangue , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Período Periparto , Gravidez , Transtornos Puerperais/sangue , Transtornos Puerperais/diagnóstico , Transtornos Puerperais/fisiopatologia , Recuperação de Função Fisiológica , Volume Sistólico , Resultado do Tratamento , Função Ventricular Esquerda/efeitos dos fármacos , Adulto Jovem
7.
PLoS Genet ; 14(7): e1007502, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29979676

RESUMO

Left ventricular non-compaction (LVNC) is a rare cardiomyopathy associated with a hypertrabeculated phenotype and a large spectrum of symptoms. It is still unclear whether LVNC results from a defect of ventricular trabeculae development and the mechanistic basis that underlies the varying severity of this pathology is unknown. To investigate these issues, we inactivated the cardiac transcription factor Nkx2-5 in trabecular myocardium at different stages of trabecular morphogenesis using an inducible Cx40-creERT2 allele. Conditional deletion of Nkx2-5 at embryonic stages, during trabecular formation, provokes a severe hypertrabeculated phenotype associated with subendocardial fibrosis and Purkinje fiber hypoplasia. A milder phenotype was observed after Nkx2-5 deletion at fetal stages, during trabecular compaction. A longitudinal study of cardiac function in adult Nkx2-5 conditional mutant mice demonstrates that excessive trabeculation is associated with complex ventricular conduction defects, progressively leading to strain defects, and, in 50% of mutant mice, to heart failure. Progressive impaired cardiac function correlates with conduction and strain defects independently of the degree of hypertrabeculation. Transcriptomic analysis of molecular pathways reflects myocardial remodeling with a larger number of differentially expressed genes in the severe versus mild phenotype and identifies Six1 as being upregulated in hypertrabeculated hearts. Our results provide insights into the etiology of LVNC and link its pathogenicity with compromised trabecular development including compaction defects and ventricular conduction system hypoplasia.


Assuntos
Regulação da Expressão Gênica no Desenvolvimento , Insuficiência Cardíaca/genética , Ventrículos do Coração/embriologia , Proteína Homeobox Nkx-2.5/metabolismo , Miocárdio Ventricular não Compactado Isolado/genética , Morfogênese/genética , Animais , Modelos Animais de Doenças , Feminino , Fibrose , Perfilação da Expressão Gênica , Ventrículos do Coração/patologia , Proteína Homeobox Nkx-2.5/genética , Proteínas de Homeodomínio/metabolismo , Humanos , Miocárdio Ventricular não Compactado Isolado/complicações , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/patologia , Camundongos , Camundongos Knockout , Miocárdio/metabolismo , Miocárdio/patologia , Ramos Subendocárdicos/patologia , Deleção de Sequência , Índice de Gravidade de Doença , Regulação para Cima
8.
G Ital Cardiol (Rome) ; 19(6): 371-378, 2018 Jun.
Artigo em Italiano | MEDLINE | ID: mdl-29912226

RESUMO

Hypertrabeculation is a feature of the left ventricle that, by itself, does not coincide with left ventricular non compaction (LVNC), which represents a specific cardiomyopathy. Nowadays, in the absence of gold standard diagnostic criteria, the clinician must integrate imaging aspects together with medical history. The family inheritance for LVNC, presence of neuromuscular disorders, symptoms or signs of heart failure, thromboembolic events, unexplained syncope, pathological findings at rest ECG, Holter ECG, stress test, systolic/diastolic dysfunction at rest echocardiogram, late gadolinium enhancement at cardiac magnetic resonance, and identification of specific mutations are all considered features useful for the diagnosis. Many aspects are not fully understood: multicenter studies, registers and observational studies are needed for a better comprehension of the pathology, adequate risk stratification and targeted follow-up.


Assuntos
Ecocardiografia/métodos , Eletrocardiografia/métodos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Meios de Contraste/administração & dosagem , Eletrocardiografia Ambulatorial/métodos , Teste de Esforço/métodos , Gadolínio/administração & dosagem , Humanos , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Imagem por Ressonância Magnética/métodos
10.
Int Heart J ; 59(4): 862-867, 2018 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-29794382

RESUMO

Little is known about pregnancies of left ventricular noncompaction cardiomyopathy (LVNC), much less cases in which LVNC was definitively diagnosed prepregnancy. We report the cases of three pregnant Japanese women definitively diagnosed with LVNC prepregnancy. Case 1 presented LVNC with restrictive phenotype. Her pregnancy was terminated due to exacerbated pulmonary hypertension and low output status at 30 weeks' gestation. Case 2 presented isolated LVNC with nonsustained ventricle tachycardia. A cesarean section was performed at 36 weeks' gestation because of placenta previa. Case 3 presented dilated LVNC. Labor induction was performed because of decreased left ventricular ejection fraction, leading to a vaginal delivery at 37 weeks' gestation. In all cases, no thromboembolic event was identified during pregnancy; two patients received anticoagulants. We reviewed all English-literature cases of pregnant women definitively diagnosed with LVNC prepregnancy to analyze causes of adverse pregnancy outcomes and the necessity of anticoagulation. Four of the six pregnancies identified were terminated due to exacerbated cardiomyopathy phenotypes and not complications due to noncompaction itself, resulting in three cases' preterm deliveries. No thromboembolic event was identified by maintenance of the anticoagulation strategy determined prepregnancy. In pregnancies with LVNC, the possibility of a severe cardiac event and the indications for termination of the pregnancy can depend on the cardiomyopathy phenotypes, not noncompaction itself. Anticoagulation only because of the pregnancy itself may be redundant. In the management of LVNC during pregnancy, close monitoring of the condition of different phenotypes and reassessment of the necessity of anticoagulation can contribute to the pregnancy outcome.


Assuntos
Anticoagulantes/administração & dosagem , Miocárdio Ventricular não Compactado Isolado , Complicações Cardiovasculares na Gravidez , Tromboembolia/prevenção & controle , Adulto , Cesárea/métodos , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Miocárdio Ventricular não Compactado Isolado/complicações , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Trabalho de Parto Induzido/métodos , Administração dos Cuidados ao Paciente/métodos , Gravidez , Complicações Cardiovasculares na Gravidez/diagnóstico , Complicações Cardiovasculares na Gravidez/fisiopatologia , Complicações Cardiovasculares na Gravidez/terapia , Resultado da Gravidez , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/etiologia , Tromboembolia/etiologia , Disfunção Ventricular Esquerda/diagnóstico , Disfunção Ventricular Esquerda/etiologia
11.
Med. clín (Ed. impr.) ; 150(9): 354-360, mayo 2018. ilus
Artigo em Espanhol | IBECS | ID: ibc-173389

RESUMO

La miocardiopatía no compactada es una entidad heterogénea y compleja de la que existen todavía muchas dudas por resolver. Mientras que la American Heart Association la incluye entre las miocardiopatías de origen genético, la Sociedad Europea de Cardiología la considera como una miocardiopatía no clasificada. Puede presentarse tanto de forma esporádica como familiar, aislada o asociada con otras cardiopatías, afectar solo al ventrículo izquierdo o a ambos y puede, en ocasiones, aparecer como un fenotipo mixto en pacientes con otras miocardiopatías. A sus diferentes manifestaciones morfológicas se asocian también diferentes formas de presentación clínica e, incluso, la no compactación del ventrículo izquierdo puede estar desencadenada por otros procesos fisiológicos o patológicos. El objeto de esta revisión es una puesta al día de esta entidad y de sus controversias


Non-compaction cardiomyopathy is a heterogeneous and complex entity concerning which there are still many doubts to be resolved. While the American Heart Association includes it among genetic cardiomyopathies, the European Society of Cardiology treats it as an unclassified cardiomyopathy. It may present in a sporadic or familial form, isolated or associated with other heart diseases, affecting only the left ventricle or both and can sometimes appear as a mixed phenotype in patients with other cardiomyopathies. Different forms of clinical presentation are also associated with its different morphological manifestations, and even non-compaction of the left ventricle may be triggered by other physiological or pathological processes. The purpose of this review is an update of this entity and its controversies


Assuntos
Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/epidemiologia , Ventrículos do Coração/fisiopatologia , Ecocardiografia/métodos , Espectroscopia de Ressonância Magnética/métodos , Anticoagulantes
12.
Echocardiography ; 35(7): 941-948, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29577407

RESUMO

BACKGROUND: Left ventricular noncompaction (LVNC) is associated with progressive LV systolic dysfunction and dilated cardiomyopathy. We aimed to investigate the echocardiographic and clinical characteristics associated with LV ejection fraction (LVEF) and moderate or greater systolic dysfunction in patients with LVNC. METHODS: Our institutional echocardiography database was retrospectively reviewed between 2008 and 2014, and 62 patients with LVNC were identified. Forty-three (69%) had moderate or greater LV systolic dysfunction (LVEF ≤ 40%) and were compared with 19 (31%) patients with preserved or mildly reduced LVEF (>40%). Linear regression analyses were utilized to identify markers associated with LVEF. RESULTS: The mean age was 63 ± 17 years and noncompacted-to-compacted ratio was 2.3 ± 0.5, and was larger in patients with LVEF ≤ 40% (2.4 vs 2.1; P = .02). Patients with LVEF ≤ 40% were older, had more congestive heart failure, significant QRS interval prolongation, and greater LV remodeling and worse mean global longitudinal strain (GLS). Multivariate regression analysis revealed increased age (standardized regression coefficient (ß) = -0.17; P = .04) and QRS duration (ß = -0.13; P = .08), congestive heart failure (ß = -0.18; P = .04), and worsened GLS (ß = -0.40; P = .001) were independently associated with decreased LVEF in the cohort (overall model fit R2  = 0.71; P < .0001). Increased age (ß = -0.49; P = .01) and QRS duration (ß = -0.50; P = .002), and worsened GLS (ß = -0.33; P = .04), were also associated with a lower LVEF in patients with LVEF > 40%. CONCLUSIONS: The independent markers associated with LVEF and moderate or greater LV systolic dysfunction in patients with LVNC, in particular GLS and QRS duration, may detect high-risk candidates for more aggressive clinical surveillance and medical therapy.


Assuntos
Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Volume Sistólico/fisiologia , Função Ventricular Esquerda/fisiologia , Idoso , Feminino , Ventrículos do Coração/fisiopatologia , Humanos , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Estudos Retrospectivos , Índice de Gravidade de Doença , Sístole
13.
Adv Clin Exp Med ; 27(3): 415-422, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29558024

RESUMO

Left ventricular noncompaction (LVNC) is a unique inherited cardiomyopathy, characterized by an increased risk of adverse cardiovascular events such as heart failure, arrhythmia or sudden cardiac death. Although in comparison to dilated cardiomyopathy, the number of clinical studies concerning LVNC is still small, it is quickly increasing, which reflects a huge effort of the cardiovascular society to develop data to improve understanding of this cardiomyopathy. However, the predictors of adverse outcomes in LVNC are not well established. The aim of this review is to systematize the available data obtained from the medical literature in order to establish a proper prognosis, so that affected patients can receive the most appropriate treatment. The review considers issues connected with various areas of risk in LVNC, referring to its incidence and prevalence, comorbidity, genetics, morphology, symptoms, thromboembolic events, incidence of arrhythmia, sudden cardiac death, and mortality. Beginning with a genetic approach to the disease, passing through diagnostic tools, and finishing with issues relating to invasive methods of treatment, the article points out the most important and valuable clues for predicting a poor prognosis in LVNC. The review confirms that LVNC is not a disease, but a type of cardiac abnormality laden with a variety of prognostic factors of poor outcomes in terms of life-threatening ventricular arrhythmia and progression of heart failure. Thus, establishing a proper prognosis for individual patients is crucial for implementing the most appropriate treatment, and it should be based on the outcomes of a variety of clinical tests.


Assuntos
Cardiomiopatias , Morte Súbita Cardíaca/etiologia , Ventrículos do Coração/fisiopatologia , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Insuficiência Cardíaca , Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/terapia
14.
Congenit Heart Dis ; 13(3): 440-443, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29468808

RESUMO

OBJECTIVES: Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD. METHODS: We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015. Demographics, hemoglobin phenotype and treatment information was recorded. LV systolic function, evidence of LVHT, and possible pulmonary arterial hypertension was assessed. RESULTS: Two hundred thirty-six patients had echocardiograms available for interpretation. One hundred twenty-one (51.3%) were female; mean age was 11.3 years (± 4.1 years). Twenty-six patients (11%) had features of LVHT on echocardiography. Eleven patients (4.7%) had borderline features of LVHT. Mean LVEDD across the whole cohort was 4.2 ± 0.69 cm, LVEDD z-score of 1.44 ± 1.9, and mean LVSF was 37.3% ±15.7%. There were no significant differences in terms of age, LVEDD, LVEDD z-score, or LVSF between patients with and those without LVHT. CONCLUSIONS: The prevalence of LVHT/noncompaction in children with SCD is lower than the adult population and LV systolic function is well preserved throughout our patient group. The mechanism behind the development of LVHT in this population remains speculative. Further work is required in this field. Sickle cell patients require longitudinal evaluation to ascertain changes in left ventricular function and the presence of LVHT/noncompaction.


Assuntos
Anemia Falciforme/epidemiologia , Ecocardiografia/métodos , Ventrículos do Coração/diagnóstico por imagem , Miocárdio Ventricular não Compactado Isolado/epidemiologia , Função Ventricular Esquerda/fisiologia , Anemia Falciforme/diagnóstico , Criança , Comorbidade/tendências , Feminino , Humanos , Irlanda/epidemiologia , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/fisiopatologia , Masculino , Taxa de Sobrevida/tendências
15.
J Am Coll Cardiol ; 71(7): 711-722, 2018 02 20.
Artigo em Inglês | MEDLINE | ID: mdl-29447731

RESUMO

BACKGROUND: The clinical outcomes of noncompaction cardiomyopathy (NCCM) range from asymptomatic to heart failure, arrhythmias, and sudden cardiac death. Genetics play an important role in NCCM. OBJECTIVES: This study investigated the correlations among genetics, clinical features, and outcomes in adults and children diagnosed with NCCM. METHODS: A retrospective multicenter study from 4 cardiogenetic centers in the Netherlands classified 327 unrelated NCCM patients into 3 categories: 1) genetic, with a mutation in 32% (81 adults; 23 children) of patients; 2) probably genetic, familial cardiomyopathy without a mutation in 16% (45 adults; 8 children) of patients; or 3) sporadic, no family history, without mutation in 52% (149 adults; 21 children) of patients. Clinical features and major adverse cardiac events (MACE) during follow-up were compared across the children and adults. RESULTS: MYH7, MYBPC3, and TTN mutations were the most common mutations (71%) found in genetic NCCM. The risk of having reduced left ventricular (LV) systolic dysfunction was higher for genetic patients compared with the probably genetic and sporadic cases (p = 0.024), with the highest risk in patients with multiple mutations and TTN mutations. Mutations were more frequent in children (p = 0.04) and were associated with MACE (p = 0.025). Adults were more likely to have sporadic NCCM. High risk for cardiac events in children and adults was related to LV systolic dysfunction in mutation carriers, but not in sporadic cases. Patients with MYH7 mutations had low risk for MACE (p = 0.03). CONCLUSIONS: NCCM is a heterogeneous condition, and genetic stratification has a role in clinical care. Distinguishing genetic from nongenetic NCCM complements prediction of outcome and may lead to management and follow-up tailored to genetic status.


Assuntos
Miocárdio Ventricular não Compactado Isolado/epidemiologia , Miocárdio Ventricular não Compactado Isolado/genética , Mutação/genética , Adolescente , Adulto , Criança , Pré-Escolar , Morte Súbita Cardíaca/epidemiologia , Morte Súbita Cardíaca/prevenção & controle , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
16.
RELAMPA, Rev. Lat.-Am. Marcapasso Arritm ; 31(1): 23-26, jan.-mar. 2018. ilus
Artigo em Português | LILACS | ID: biblio-905746

RESUMO

Relatamos o caso de paciente do sexo masculino, com 23 anos de idade, portador de miocárdio não compactado e taquicardia ventricular monomórfica sustentada. O paciente foi submetido a implante de cardiodesfibrilador implantável após diagnóstico confirmado por meio de ressonância nuclear magnética cardíaca e mantido em tratamento clínico com medicação antiarrítmica, sem recorrência de arritmia ventricular no acompanhamento ambulatorial


We report the case of a 23-year-old male patient with noncompacted myocardium and sustained monomorphic ventricular tachycardia. The patient was submitted to mplantable cardioverter defibrillator after diagnosis confirmed by cardiac magnetic resonance imaging and was kept on clinical treatment with antiarrhythmic medication without the recurrence of ventricular arrhythmia in the outpatient follow-up


Assuntos
Humanos , Masculino , Adulto , Cardiomiopatia Dilatada/complicações , Cardiomiopatia Dilatada/diagnóstico , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Taquicardia Ventricular/diagnóstico , Amiodarona/administração & dosagem , Morte Súbita , Ecocardiografia/métodos , Eletrocardiografia/métodos , Cardiopatias Congênitas , Frequência Cardíaca , Metoprolol/administração & dosagem
19.
Med Clin (Barc) ; 150(9): 354-360, 2018 05 11.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-29173988

RESUMO

Non-compaction cardiomyopathy is a heterogeneous and complex entity concerning which there are still many doubts to be resolved. While the American Heart Association includes it among genetic cardiomyopathies, the European Society of Cardiology treats it as an unclassified cardiomyopathy. It may present in a sporadic or familial form, isolated or associated with other heart diseases, affecting only the left ventricle or both and can sometimes appear as a mixed phenotype in patients with other cardiomyopathies. Different forms of clinical presentation are also associated with its different morphological manifestations, and even non-compaction of the left ventricle may be triggered by other physiological or pathological processes. The purpose of this review is an update of this entity and its controversies.


Assuntos
Miocárdio Ventricular não Compactado Isolado , Anticoagulantes/uso terapêutico , Cardiomiopatias/classificação , Gerenciamento Clínico , Coração Fetal/patologia , Cardiopatias Congênitas/complicações , Humanos , Miocárdio Ventricular não Compactado Isolado/diagnóstico , Miocárdio Ventricular não Compactado Isolado/epidemiologia , Miocárdio Ventricular não Compactado Isolado/genética , Miocárdio Ventricular não Compactado Isolado/terapia , Modelos Cardiovasculares , Fenótipo
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