A systematic review of microRNAs in patients with hypertrophic cardiomyopathy.

BACKGROUND: Several microRNAs (miRNA) have been associated with hypertrophic cardiomyopathy (HCM), but studies differ regarding methods employed. In an attempt to understand their role in the disease, we performed a systematic review of studies assessing miRNAs and their association with HCM. METHODS: The literature search was based on The Medical Subject Headings (MeSH) terms "Hypertrophic Cardiomyopathy" and "MicroRNA" combined with other synonyms on Embase, Medline and LILACS databases in April 2020. The selected studies and data extraction were independently evaluated. Only human reports with a clear definition of HCM diagnosis were included. RESULTS: The search found 68 studies, 13 fulfilled the selection criteria, with a total of 329 patients. Eighty-seven miRNA were differentially expressed in HCM patients, being mir-21, mir-29a and mir-133 the most reported. The miRNA were mainly up-regulated, where mir-29a was up-regulated in 6 studies, followed by mir-133 in 4 and mir-21 in 3. The other miRNAs were mainly up-regulated. Blood samples were evaluated in the majority of patients (86%), but a greater number of miRNAs (79%) were assessed in myocardium. Six studies evaluating the phenotype correlation demonstrated that several miRNAs, mainly mir-1-3p, mir-19b, mir-21, mir-29a, mir-155, and mir-221, were related to either hypertrophy or fibrosis. Mir-29a showed a more consistent phenotypic correlation. CONCLUSION: Eighty-seven miRNAs were differentially expressed in HCM patients, the majority in up-regulation. Mir-21, mir-29a and mir-133 were the most reported. Correlation with left ventricular hypertrophy and fibrosis was evaluated in six studies for several miRNAs, nevertheless, mir-29a showed more consistent findings and seems to be a promising biomarker.

Ventricular arrhythmias and left ventricular hypertrophy in hypertrophic cardiomyopathy.

BACKGROUND: In hypertrophic cardiomyopathy (HCM), the degree of left ventricular hypertrophy (LVH) could influence the development of ventricular arrhythmias. OBJECTIVE: In HCM, analyze the association between the occurrence of ventricular arrhythmias determined by Holter electrocardiogram (ECG-Holter) and the degree of LVH determined by maximum wall thickness (MWT) in echocardiography and body mass index (BMI). METHODS: Fifty-four consecutive patients with HCM underwent 24-hour ECG-Holter and echocardiography for assessment of level of LVH through MWT and BMI. Two levels were established for the occurrence of Ventricular Arrhythmias: I - alone or paired extrasystoles and II - Non- Sustained Ventricular Tachycardia (NSVT). RESULTS: In 13 patients (24%) with NSVT (level II), there was a higher frequency of MWT of the left ventricle (LV) > 21 mm (n = 10, 77%, 25 ± 4 mm) and LLLV = 144 g/m² (n = 10, 77%, 200 ± 30 g/m²), in comparison with those presenting with extrasystole arrhythmias (level I) (n = 41, 76%), in which these measures were identified in, respectively, 37 % (n= 15, 23 ± 1 mm), p = 0.023, and 39% (n = 16, 192 ± 53 g / m²) of the cases (p = 0.026). The cut-off values mentioned were determined by the ROC curve with a confidence interval of 95%. NSVT was more common in patients with MWTLV > 21 mm and LLLV > 144 g/m² (8 of 13, 62%) than in those with (4 of 13, 31%) or without (1 of 13; 8%) echocardiographic variables above cut-off values (p = 0.04). CONCLUSION: In HCM, occurrence of ventricular arrhythmias by Holter was associated with the degree of LVH assessed by echocardiography through MWT and BMI.

The diagnosis of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy is a prevalent genetic disease characterized by left ventricular hypertrophy, presenting dynamic obstruction of outflow tract with subaortic gradient happening at rest in 30% of the cases. It is attributed to the intricate interaction between the anterior mitral leaflet, the interventricular septum and altered flow vectors generated in left ventricle along with changes in outflow tract geometry. Mitral regurgitation in varying degrees is found with or without association with structural deformities of the valve apparatus. The exercise echocardiogram evidences latent obstruction easily induced by exercise in 60 to 75% of non-obstructive forms. The determination of the gradient under this condition must be considered in routine investigation of patients with mild or no obstruction at rest. The evaluation of hypertrophic cardiomyopathy incorporates methods based on the ultrasound image, which, along with MRI, allow recognizing ventricular obstruction generating mechanisms, thus facilitating the diagnosis and management of obstructive and latent obstructive forms.