Diagnóstico prenatal de miocardiopatía hipertrófica septal en hijos de madres diabéticas a través de estudio ecocardiógráfico fetal y su relación con los valores de hemoglobina glicosilada materna / Prenatal diagnosis of septal hypertrophic cardiomyopathy in children of diabetic mothers through a fetal echocardiographic study and its relation to the values of maternal glycosylated hemoglobin

Introducción: Los niveles elevados de glicemia materna generan un incremento en el metabolismo ycrecimiento de las estructuras cardiacas fetales. Por lo que un adecuado control metabólico de la madre sepuede relacionar con el crecimineto del ventrículo izquierdo. Objetivos: Evaluar la frecuencia demiocardiopatia hipertrófica en fetos de madres con diabetes durante el embarazo, a través deecocardiograma fetal. Correlacionar la presencia de miocardiopatia hipertrófica con el grado de controlmetabólico materno. Determinar si existe relación entre los niveles de hemoglobina glucosilada materna y/olos niveles de glucosa preprandial con la presencia de hipetrofia septal. Resultados: 44% de pacientes condiabetes mostraron hipertrofia septal, siendo estos casos con niveles de hemoglobina glicosilada elevados.Miocardiopatía hipertrófica septal se encontró en 89% de pacientes con diabetes gestacional, 5% enpacientes con diabetes mellitus tipo II, 5% en pacientes con intolerancia a los carbohidratos y no seobservaron casos en los pacientes con diabetes tipo I; No se encontró cambios en la frecuencia cardiacafetal, ni arritmias en los fetos hijos de madre diabética similar a lo observado en los controles. Conclusión:Hemoglobina glicosilada de 6% - 8% se correlacionaron con mayor riesgo de miocardiopatía hipertróficaseptal. Ecocardiograma fetal es adecuado para diagnóstico prenatal.

Background: Elevated levels of maternal glucose generate an increase, in the metabolism and growth of fetalcardiac structures. An adequate metabolic control of the mother may be related to growth of the left ventricle.Objectives: Evaluate the frequency of hypertrophic cardiomyopathy in fetuses of mothers with diabetesduring pregnancy, through fetal echocardiography. Correlate the presence of hypertrophic cardiomyopathywith the degree of maternal metabolic control. To determine the correlation between maternal glycatedhemoglobin levels and / or preprandial glucose levels with the presence of septal hypertrophy. Results: 44%of patients with diabetes show septal hypertrophy, and these cases at high levels of glycated hemoglobin.Septal hypertrophic cardiomyopathy was found in 89% of patients with gestational diabetes, 5% in patientswith diabetes mellitus type II, 5% in patients with carbohydrate intolerance and no cases were observed inpatients with type I diabetes; No changes were found in the fetal heart rate or fetal arrhythmias in children ofdiabetic mother similar to that observed in controls. Conclusion: Glycated hemoglobin between 6% - 8%,was correlated with increased risk of septal hypertrophic cardiomyopathy. Fetal echocardiogram is suitable forprenatal diagnosis.

Gradientes intraventriculares e qualidade de vida no acompanhamento a longo prazo da redução não-cirúrgica do septo intraventricular na cardiomiopatia hipertrófica obstrutiva / Intraventricular gradients and quality of life in the long-term follow-up of nonsurgical intraventricular septum reduction in patients with hypertrophic cardiomyopathy

Introdução: Cerca de 30% dos pacientes com cardiomiopatia hipertrófica obstrutiva têm uma obstrução dinâmica do trato de saída do ventrículo esquerdo (TSVE). Pacientes sintomáticos são tratados com agentes inotrópicos negativos, entretanto alguns pacientes não respondem à terapia medicamentosa. A miectomia cirúrgica foi, até a década de 1990, o tratamento padrão para esses pacientes. A ablação septal percutânea com álcool (ASA) foi introduzida como terapia alternativa menos invasiva. Este estudo teve como objetivo avaliar a evolução clínica de pacientes submetidos a ASA em nosso centro.Método: Foram incluídos sete pacientes com cardiomiopatia hipertrófica obstrutiva, analisando-se os sintomas, os gradientes intraventriculares e a qualidade de vida antes e depois da ASA. O acompanhamento médio foi de 3,3 + 1,8 anos. Resultados: Houve melhora significativa da classe da New York Heart Association (NYHA) durante o acompanhamento clínico. O gradiente médio do TSVE antes da ASA, em repouso, foi de 67 + 22 mmHg, e com a manobra de Valsalva foi de 131 + 27 mmHg. Na evolução tardia, o gradiente médio do TSVE, em repouso, foi de 33 + 37 mmHg e com a manobra de Valsalva, 37 + 47 mmHg. O consumo máximo de oxigênio foi de 14,7 ml/min antes da ASA e durante o acompanhamento aumentou para 25,4 ml/min. Conclusão: Em nosso estudo, a ASA foi um tratamento seguro e eficaz para a cardiomiopatia hipertrófica obstrutiva, propiciando alívio substancial dos sintomas e da obstrução do trato de saída, e melhora da capacidade de exercício e da qualidade de vida, com baixas taxas de complicações.

Background: About 30% of patients with hypertrophic obstructive cardiomyopathy (HOCM) have dynamic obstruction of the left ventricular outflow tract (LVOT). Symptomaticpatients are treated with negative inotropic agents, however, some patients do not respond to medical therapy. Surgical myectomy was the standard therapy for these patients until the nineties. Percutaneous alcohol septal ablation (ASA) was introduced as a less invasive alternative therapy. This study was aimed at assessing the clinical course of patients undergoing ASA at our center. Method: Seven patients with HOCM were included and intraventricular gradients and quality of life before and after ASA were evaluated. Mean follow-up was 3.3 + 1.8 years. Results: There was a significant improvement in the NewYork Heart Association (NYHA) class during the followup. The mean LVOT gradient before ASA at rest was 67 +22 mmHg and 131 + 27 mmHg with Valsalva maneuver. In the late follow-up, the mean LVOT gradient at rest was33 + 37 mmHg and 37 + 47 mmHg with Valsalva maneuver. Peak oxygen uptake was 14.7 ml/min prior ASA, increasing to 25.4 ml/min during the follow-up. Conclusion: In our study, ASA was a safe and effective treatment for HOCM providing substantial relief of symptoms, outflow tract obstructionand improvement of exercise capacity and quality of life with low complication rates.

Troponin I release at rest and after exercise in patients with hypertrophic cardiomyopathy and the effect of betablockade

PURPOSE OF THE WORK: In patients with hypertrophic cardiomyopathy ischemia may occur due to massive heart weight, myocyte disarray or small vessel disease. We detected elevated troponin levels in some of these patients and hypothesized that troponin release would rise after exercise and diminish after betablockade. METHODS AND RESULTS: In 5 of 7 young patients (6 males) with hypertrophic cardiomyopathy and no overt coronary artery disease we found elevated troponin levels after physical exercise; the peak was between 6 and 9 hours and levels returned to pre-exercise values within 24 hours. Troponin release was consistently diminished after use of a betablocker. CONCLUSIONS: Increased troponin release may be present in patients with hypertrophic cardiomyopathy and is temporarily enhanced by exercise and diminishes with betablockade.