Presented by: Alexandros Protonotarios, MD; Adalena Tsatsopoulou, MD; Aris Anastasakis, MD
A young woman of 28 years old suffered cardiac arrest as soon as she started dancing.Her history started with asymptomatic frequent monomorphic VES from RVOT (4.500/24hrs) at the age of 14 years (Figure 1).
Figure 1. Resting 12-lead ECG recording, at initial assessment VES (14-year-old). Monomorphic VES (20.000/24h) of LBBB morphology and inferior axis.
Two-dimensional echocardiography revealed no functional/structural abnormality of the right or left ventricle. At that time she suffered a self-terminated episode of VT run ≤2 min, with LBBB pattern, at the beginning of stress test (Figure 2).
Figure 2. RVOT Tachycardia at the beginning of Stress Test (14-year-old), the episode of sustained VT lasted ≤2 min and it was spontaneously terminated.
On EPS that followed, there was not inducible VT. The site of origin of extra-systoles was located on the anterior aspect of the infundibular area where application of radiofrequency energy was performed. However, persistent monomorphic VES from RVOT remained for the years following (Figure 3).
Figure 3. Resting 12-lead ECG showing monomorphic VES from RVOT, 7 years after ablation
From the family history, juvenile SCD was recorded in two 5th degree relatives; in one of them complete AV block had been documented and a pacemaker had been implanted. Cardiac evaluation of the first and second-degree relatives revealed no abnormality suggestive of a cardiomyopathy or ion-channel disorder.
Genetic screening for desmosomal and lamin A/C mutations was negative.
For the following 14 years, she remained asymptomatic on bisoprolol, avoiding athletics. She was followed-up yearly with 12-lead resting ECG, SAECG, 24-hour Holter monitoring, and two-dimensional echocardiography. Repolarization abnormalities on 12-lead resting ECG precordial leads developed at the beginning of follow-up and remained thereafter (Figure 4).
Figure 4. Resting 12-lead ECG recordings at 18 (A) and 28 (B) years of age. Inverted T waves in V1-V4. Flattened T in left precordial and limp leads.
No depolarization abnormalities were ever recorded. Two-dimensional echocardiography never revealed any RV/LV functional/structural alteration. At last follow-up, 2 months before the event, her two-dimensional echo revealed no wall motion abnormalities or any hypertrophy, RVOT-PLAX=24mm, RVOT-PSAX=27mm, LVEDD/BSA=31mm/m 2, EF=55%-60%. CMR with contrast enhancement, one year before the event, revealed normal right and left ventricular structure and function without any late enhancement sign; RVEDV=65ml/m 2, RVEF=71%, LVEDV=85ml/m 2, LVEF=55% (Figure 5).
Figure 5. Contrast enhanced CMR with no signs of RV or LV delayed enhancement
At the age of 28, she suffered sudden cardiac death as soon as she started dancing.
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