We present the interesting case of a 49 years old male, an ex-professional football player, referred to our clinic with the suspicion of acute coronary syndrome, after a syncope followed by constrictive thoracic pain and palpitations.
He was known to our clinic since 2011 (8 years ago), when he suffered a cardiac arrest after being hit in the chest by a football during a football match. He was resuscitated on the field, requiring 10 external electrical shocks for an incessant ventricular tachycardia (VT). After reestablishment of the sinus rhythm, he was addressed to our clinic for further investigation. Coronary angiography showed normal coronary arteries. Taking into consideration the young age, the morphology of the VT (left bundle branch block morphology - right ventricle (RV) origin and superior axis) and the mild dilation of the RV, a suspicion of ARVC was raised. Electrophysiological study was performed, but no ventricular arrhythmias were further induced. The patient was started on amiodarone and reevaluation with cardiac MRI was recommended for the confirmation of ARVC. Furthermore, complete cessation of professional football was advised. Despite this, between 2011 and 2019 the patient did not return for reevaluation, stopped any medical treatment and resumed the endurance physical activity.
In February 2019 he presents a syncopal episode while undergoing regular house activities, followed by constrictive thoracic pain and palpitations. A suspicion of acute coronary syndrome was raised, so the patient was referred to our clinic. During transportation, two episodes of sustained VT were recorded, requiring two electrical shocks and intravenous amiodarone, with reestablishment of sinus rhythm.
On admission in our department, he was hemodynamically stable, with normal blood pressure and heart rate, without angina. No cardiac murmurs or pulmonary rales were found on auscultation.
The electrocardiogram showed sinus rhythm 60 bpm, sequelae of anterior myocardial infarction, negative T waves in V1-V2 (Figure 1.)
By analyzing the ECG trace from the ambulance, a wide complex tachycardia with a left bundle branch block morphology and inferior axis was observed, suggesting a VT originating in the RVOT.
The blood tests revealed hypopotassemia (3.4 mmol/l), mild dyslipidemia, normal myocardial and hepatic enzymes, normal TSH.
At echocardiography we found an extremely dilated RV, with dyskinesia of the apex and apical half of the RV free wall, with aneurisms at this level. A focused examination of the RV showed a dilated RVOT measured both in PLAX (32 mm) and PSAX (41 mm), with a severely reduced systolic function - fractional area change 20%, TAPSE 16 mm, S' velocity 8,6 cm/s. The LV was non dilated, with a normal ejection fraction and without kinetic abnormalities but with a small degree of trabeculation at the apical level. No signs of aortic dissection or significant valvopathies were found (Figure 2).
Chest X ray was normal. In order to exclude an ischemic etiology of the VT, we decided to perform a coronary angiography, which was normal.
At this moment, the differential diagnosis of ARVC included myocarditis, sarcoidosis, idiopathic RV VT, athlete's heart, excluded by the normal LV function and walls without hypertrophy, normal myocardial enzymes and chest X ray.
In order to confirm our suspicion and further risk stratification, we decided to perform a cardiac MRI, which showed an extremely dilated RVOT (43 mm) and RV (EDV/BSA 223 ml/m2), with aneurisms and hypokinesia, dyskinesia and zones of akinesia located at the level of the RV free wall (Figure 3). The systolic function of the RV was found to be severely depressed - RV ejection fraction 26%, global longitudinal strain -7%, global circumferential strain -4%. Furthermore, inhomogeneous adipose infiltrations of the RV free wall and at the apical segment of the lateral wall of the LV, with extensive fibrosis at this level were observed (Figure 4). According to the 2010 Task Force Criteria for diagnosis of ARVD, the patient had 3 major criteria and 2 minor criteria, establishing a certain diagnosis.
Repeating the electrophysiological study and further ablation of the arrhythmogenic substrate was considered. Given the possible epicardial and endocardial substrate, the extensive, inhomogeneous fibrosis lesions at biventricular level, the intense trabeculation and parietal aneurisms, and the fact that in our center there is no epicardial approach available, we decided that the risks outweigh the possible benefits of the procedure.
Taking all of the above into consideration and according to the risk stratification system recommended by the ESC/AHA, our patient was found to be at the highest arrhythmic risk (8-10%/year), with a class I indication for implantable cardioverter defibrillator. The implantation was performed without immediate periprocedural complications.
At 48 hours after the implantation, the patient developed atrocious epigastric pain, with no response to routine pain killers. An emergency ECG was performed, showing pacing and sensing malfunction and the echocardiography showed no pericardial fluid. The abdominal ultrasound showed no free fluid in the abdominal cavity and no signs of acute abdominal emergency. The thoracic radioscopy showed displacement of the ventricular probe, suggesting a myocardial perforation (Figure 5).
Given the severe alteration of the patients' status and high risk, emergency thoracotomy with successful withdrawal of the ventricular probe was performed. The myocardial perforation was then sutured.
The postoperative evolution of the patients was slowly favorable, without repeating the ventricular arrhythmias. He was discharged after 14 days and treatment with a beta blocker, amiodarone, spironolactone and atorvastatin was recommended. As future perspectives, the patient will be referred for evaluation in a center where epicardial approach for ablation is feasible and implantation of a subcutaneous defibrillator will be performed as soon as the device will be available.