Genetic testing in specific cardiomyopathies

An increasing number of genetic tests for cardiomyopathies are becoming available for clinical use. This commentary will give a short overview of indications and challenges concerning genetic testing for these conditions

Left ventricular mechanical dispersion by tissue Doppler imaging: a novel approach for identifying high-risk individuals with long QT syndrome

Forutsigelse av livstruende hjerterytmeforstyrrelser Hjertespesialist og forsker Kristina Hermann Haugaa har i sin doktorgrad funnet en ny metode som kan brukes til å forutsi hvilke pasienter som kommer til å få alvorlige hjerterytmeforstyrrelser: Ultralyd av hjertet med ny metode kan avsløre hvem som har risiko for hjerterytmeforstyrrelser og hvem som ikke har det. Plutselig hjertedød på grunn av hjerterytmeforstyrrelser er en av de vanligste dødsårsakene i Norge og i den øvrige vestlige verden. Den største risikogruppen er personer som har hatt hjerteinfarkt. Plutselig hjertedød hos yngre skyldes ofte arvelige hjertesykdommer. I avhandlingen “Prediction of cardiac ventricular arrhythmias by echocardiography in patients at risk” undersøker Kristina Haugaa både yngre pasienter med arvelige hjerterytmeforstyrrelser og pasienter som har gjennomgått hjerteinfarkt med den nye metoden for hjerteultralyd. Pasientene ble fulgt i over to år etter hjerteinfarkt. Studiene viser at ujevn hjertekontraksjon er en risikomarkør for å få hjerterytmeforstyrrelser og at den nye metoden vurderer risikoen bedre enn dagens metoder. Med bedre risikovurdering kan man bedre fordele resursene for behandling. Behandlingen innebærer oftest at pasientene i tillegg til medisin får operert inn en automatisk hjertestarter. Den nye metoden som er brukt i avhandlingen vil kunne forbedre utvelgelsen av pasienter med høy risiko for død slik at disse kan utstyres med hjertestarter

Mechanical Dispersion Assessed by Myocardial Strain in Patients After Myocardial Infarction for Risk Prediction of Ventricular Arrhythmia

ObjectivesThe aim of this study was to investigate whether myocardial strain echocardiography can predict ventricular arrhythmias in patients after myocardial infarction (MI).BackgroundLeft ventricular (LV) ejection fraction (EF) is insufficient for selecting patients for implantable cardioverter-defibrillator (ICD) therapy after MI. Electrical dispersion in infarcted myocardium facilitates malignant arrhythmia. Myocardial strain by echocardiography can quantify detailed regional and global myocardial function and timing. We hypothesized that electrical abnormalities in patients after MI will lead to LV mechanical dispersion, which can be measured as regional heterogeneity of contraction by myocardial strain.MethodsWe prospectively included 85 post-MI patients, 44 meeting primary and 41 meeting secondary ICD prevention criteria. After 2.3 years (range 0.6 to 5.5 years) of follow-up, 47 patients had no and 38 patients had 1 or more recorded arrhythmias requiring appropriate ICD therapy. Longitudinal strain was measured by speckle tracking echocardiography. The SD of time to maximum myocardial shortening in a 16-segment LV model was calculated as a parameter of mechanical dispersion. Global strain was calculated as average strain in a 16-segment LV model.ResultsThe EF did not differ between ICD patients with and without arrhythmias occurring during follow-up (34 ± 11% vs. 35 ± 9%, p = 0.70). Mechanical dispersion was greater in ICD patients with recorded ventricular arrhythmias compared with those without (85 ± 29 ms vs. 56 ± 13 ms, p < 0.001). By Cox regression, mechanical dispersion was a strong and independent predictor of arrhythmias requiring ICD therapy (hazard ratio: 1.25 per 10-ms increase, 95% confidence interval: 1.1 to 1.4, p < 0.001). In patients with an EF >35%, global strain showed better LV function in those without recorded arrhythmias (−14.0% ± 4.0% vs. −12.0 ± 3.0%, p = 0.05), whereas the EF did not differ (44 ± 8% vs. 41 ± 5%, p = 0.23).ConclusionsMechanical dispersion was more pronounced in post-MI patients with recurrent arrhythmias. Global strain was a marker of arrhythmias in post-MI patients with relatively preserved ventricular function. These novel parameters assessed by myocardial strain may add important information about susceptibility for ventricular arrhythmias after MI