Dextrocardia with Situs Inversus Associated with Non-Compaction\nCardiomyopathy
A 53-year-old male patient who, in the past 2 years, has presented brief episodes of\ncollapse and presyncope when performing daily activities, with recent worsening in the\nfrequency of symptoms. Regarding the habits of life, the patient was sedentary, smoker\nand social alcoholic. He sought a general practitioner who requested examinations for\ninitial evaluation. The resting electrocardiogram (ECG) revealed sinus bradycardia\n(heart rate - HR: 39 bpm), deviation of the electrical axis to the right and right\nbundle branch block (). The exercise test\nwas considered abnormal due to arrhythmia (ventricular extrassystoles with periods of\nbigeminy during exercise and recovery). 24-hour Holter monitoring showed average heart\nrate of 37 bpm (minimum of 25 and maximum of 81 bpm) with sinus rhythm interspersed with\njunctional rhythm. Atrioventricular conduction was within the normal range and\nintraventricular conduction revealed right bundle branch disorder. The patient presented\n11,596 pauses lasting longer than 2 seconds, rare and isolated monomorphic ventricular\nextrasystoles and an episode of non-sustained supraventricular tachycardia.\nGiven the clinical suspicion of sinus node disease, the patient was referred to a\ncardiologist, who continued the investigation. Transthoracic echocardiography was\nrequested, which revealed dextrocardia (presence of situs inversus, heart positioned in\nthe right hemithorax with apex facing right); images suggestive of trabeculations in the\nleft ventricle (LV); moderate LV diastolic dysfunction with preserved systolic function;\nleft atrial enlargement (57 mm); mild mitral regurgitation.\nFor diagnostic elucidation and evaluation of cardiomyopathy, CMRI was performed and cine\nsequences were obtained (balanced steady-state free precession - b-SSFP), anatomy with\nblack-blood turbo spin-echo sequences, with T1 and T2 weighting with and without fat\nsuppression (TSE BB-PD and STIR) and delayed enhancement (turbo-field echo with\ninversion pulse and recovery, 10 minutes after injection of 0.2 mmol/kg of gadolinium\ncontrast; TE: 6.1; TR 3.0). Besides confirming dextrocardia associated with situs\ninversus, a slight increase in the left ventricular cavity associated with excessive\ntrabeculations in the middle and apical segments of the inferior, lateral and anterior\nLV walls was observed. The non-compacted/compacted myocardium relationship was higher\n2.3 and the trabecular mass corresponded to 38% of the total left ventricular mass\ncompatible with diagnosis of non-compacted CMP. There were no direct or indirect signs\nof restriction, thrombus or pericardial abnormalities. There were no areas of fibrosis\nor infarction detectable in the sequences after gadolinium injection ().\nElectrophysiological study was performed; programmed ventricular stimulation caused the\ninduction of ventricular fibrillation (two extrasystoles).\nCardioverter defibrillator (CD) was implanted as a normal procedure. Four months after\nthe CD implantation, the patient developed deep vein thrombosis in the left upper limb.\nThe patient was anticoagulated and is currently asymptomatic, with atrial pacing in 100%\nof the time and no sustained ventricular arrhythmias, treated with amiodarone 200 mg and\naspirin 100 mg.