Transition of the ST segment from a J wave to a coved-type elevation before ventricular fibrillation induced by coronary vasospasm in the precordial leads

We present the case of a previously healthy 25-year-old woman who presented with an out-of-hospital ventricular fibrillation arrest. Postresuscitation ECG did not show any evidence of ST segment elevation. Echocardiogram showed regional wall abnormalities in keeping with takotsubo syndrome (TTS). Urgent coronary angiogram to rule out malignant congenital coronary artery anomaly revealed an isolated severe ostial left main coronary artery (LMCA) stenosis, a rare disease, approximately 0.2% in previous case series. The LMCA was aneurysmal. Genetic studies revealed a novel frameshift pathogenic variant in the transforming growth factor B two ligand gene (TGFB2) gene, suggestive of Loeys-Dietz syndrome (LDS) type 4, an aggressive vascular disease. Ostial LMCA stenosis has not been previously reported in LDS, and we outline the management of this unique disease combination. We also reflect on its presentation as TTS and infer that TTS and acute coronary syndromes are not mutually exclusive.

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Abstract 2769: Bradycardia-Dependent Augmentation of J-wave Elevation in Patients with Idiopathic Ventricular Fibrillation

Circulation ◽

10.1161/circ.118.suppl_18.s_774-d ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Koichi Mizumaki ◽

Akira Fujiki ◽

Masao Sakabe ◽

Tamotsu Sakamoto ◽

Yosuke Nakatani ◽

...

Keyword(s):

Ventricular Fibrillation ◽

Linear Regression ◽

Wave Amplitude ◽

Idiopathic Ventricular Fibrillation ◽

Cardioverter Defibrillator ◽

Rate Dependent ◽

Wave Elevation ◽

Male Patients ◽

And Control ◽

J Wave

Although J-wave elevation (JWE) in the inferior or lateral leads is characteristic of subtype of idiopathic ventricular fibrillation (IVF), difference between malignant and benign JWE remains to be elucidated. So, we assessed the hypothesis that the rate dependent changes in JWE could be different between patients with IVF and control subjects. Six male patients with IVF and 18 age matched male controls with JWE (>2 mm) in the lateral leads were studied. CM-5 lead digital Holter ECG was recorded and J wave amplitude was automatically measured. J/RR relationships were evaluated by means of J/RR linear regression lines for 24 hours, and according to four 6-hour periods. From J-RR linear regression lines, J-RR slope (mm/sec), J wave amplitude (mm) at RR intervals of both 0.6 sec and 1.2 sec (J (0.6) and J (1.2) ) were determined. In all 6 patients with IVF, the time of spontaneous episodes of VF were investigated from the implantable cardioverter defibrillator (ICD) stored data. For 24 hours, J-RR slope was greater in IVF patients than in controls (3.5±0.7 vs 2.4±0.8, p<0.01). (J (0.6) ) wasn’t different between 2 groups; however, J (1.2) was greater in IVF patients than in controls (2.8±0.9 vs 2.0±0.6, p<0.05). In IVF patients, ST/RR slope was highest during 18:00–24:00; however, these circadian changes were blunted in controls. J(1.2) was greater during 18:00 – 6:00 in IVF patients than in controls. Seven (59%) of total 9 episodes of spontaneous VF of IVF patients occurred during 18:00 – 6:00. In patients with subtype of IVF, JWE augmented during bradycardia especially at night. This could relate to nighttime occurrence of IVF.

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