Relationship Between Electrocardiographic Findings and Cardiac Magnetic Resonance Phenotypes in Arrhythmogenic Cardiomyopathy

Abstract

Background The new designation of arrhythmogenic cardiomyopathy defines a broader spectrum of disease phenotypes, which include right dominant, biventricular, and left dominant variants. We evaluated the relationship between electrocardiographic findings and contrast‐enhanced cardiac magnetic resonance phenotypes in arrhythmogenic cardiomyopathy. Methods and Results We studied a consecutive cohort of patients with a definite diagnosis of arrhythmogenic cardiomyopathy, according to 2010 International Task Force criteria, who underwent electrocardiography and contrast‐enhanced cardiac magnetic resonance. Both depolarization and repolarization electrocardiographic abnormalities were correlated with the severity of dilatation/dysfunction, either global or regional, of both ventricles and the presence and regional distribution of late gadolinium enhancement. The study population included 79 patients (60% men). There was a statistically significant relationship between the presence and extent of T‐wave inversion across a 12‐lead ECG and increasing values of median right ventricular ( RV ) end‐diastolic volume ( P <0.001) and decreasing values of RV ejection fraction ( P <0.001). The extent of T‐wave inversion to lateral leads predicted a more severe RV dilatation rather than a left ventricular involvement because of the leftward displacement of the dilated RV , as evidenced by contrast‐enhanced cardiac magnetic resonance. A terminal activation delay of >55 ms in the right precordial leads (V1‐V3) was associated with higher RV volume ( P =0.014) and lower RV ejection fraction ( P =0.053). Low QRS voltages in limb leads predicted the presence ( P =0.004) and amount ( P <0.001) of left ventricular late gadolinium enhancement. Conclusions The study results indicated that electrocardiographic abnormalities predict the arrhythmogenic cardiomyopathy phenotype in terms of severity of RV disease and left ventricular involvement, which are among the most important determinants of the disease outcome.

Arrhythmogenic right ventricular cardiomyopathy: evaluation of the current diagnostic criteria and differential diagnosis

“…Rather than an ECG marker of advanced RV disease, low QRS voltages indicate LV involvement (regardless of the RV disease severity) and reflect loss of myocardium/electrical voltages of the LV wall and replacement by electrically inert fibrofatty scar tissue. [54][55][56][57][58] The ECG abnormalities resulting from delayed RV activation/conduction include RBBB (usually incomplete and rarely complete), QRS fragmentation, prolongation of right precordial QRS duration with a delayed S-wave upstroke, terminal activation duration (TAD) > _55 ms, and epsilon waves. The accuracy of the presence of epsilon waves as a diagnostic tool has been questioned since these discrete signals are related to ECG filtering and sampling rate, giving rise to large interobserver variability.…”

Screening young athletes for diseases at risk of sudden cardiac death: role of stress testing for ventricular arrhythmias

“…Although ECG abnormalities are reported in up to 85% of patients with overt arrhythmogenic cardiomyopathy, there is a correlation between the severity of the phenotype and the probability of exhibiting ECG changes. 26 Therefore, it is not surprising that young athletes at the beginning of the disease's natural history may still show an unremarkable ECG.…”

A paradigm shift in the concept of arrhythmogenic cardiomyopathy: expanding the clinical and genetic spectrum, new diagnostic criteria for left ventricular phenotypes

“…Однако результаты анализа клинических и трансляционных исследований в последние годы позволили значительно расши-рить фенотипический спектр АПЖК [4]. Так, в большинстве случаев АПЖК, согласно данным метаанализа многоцентровых исследований с использованием магнитно-резонансной томографии (МРТ) сердца и гистологической оценки миокарда, были выявлены признаки вовлечения левого желудочка (ЛЖ) различной степени выраженности [5][6][7]. Такие подтипы заболевания, как аритмогенная левожелудочковая кардиомиопатия (АЛЖК) и бивентрикулярная аритмогенная кардиомиопатия (АКМП), первоначально рассматривались как атипичные формы заболевания.…”

“…Так, в 2019г эксперты двенадцати международных кардиологических Ассоциаций во главе с рабочей группой Общества сердечного ритма (HRS) предложили обновленные рекомендации по диагностике, стратификации риска и лечению АКМП [11]. В документе HRS консенсуса принят общий термин "аритмогенная кардиомиопатия" для обозначения спектра аритмогенных генетических заболеваний с вовлечением одного или обоих желудочков [5,6,9]. Эксперты HRS предложили три основных фенотипа АКМП: 1) с преобладающим поражени-группа HRS предложила несколько иной подход к генетическому тестированию при АКМП.…”