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Case Reports
. 2021 Oct 29;14(10):e245566.
doi: 10.1136/bcr-2021-245566.

Loeys-Dietz syndrome and isolated severe ostial left main coronary stenosis presenting as ventricular fibrillation arrest and biventricular takotsubo syndrome in a 25-year-old patient

Ahmed Gargoum  1 , Idris Bare  1 , Christopher Pekrul  1 , Shravan Nosib  2
Affiliations
Case Reports

Loeys-Dietz syndrome and isolated severe ostial left main coronary stenosis presenting as ventricular fibrillation arrest and biventricular takotsubo syndrome in a 25-year-old patient

Ahmed Gargoum et al. BMJ Case Rep. .

Abstract

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.

Keywords: arrhythmias; heart failure; interventional cardiology; ischaemic heart disease.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
ECG showing ventricular fibrillation prior to defibrillation.
Figure 2
Figure 2
Postresuscitation ECG shows T-wave inversions anterolaterally with prolonged QT interval.
Figure 3
Figure 3
Hypermobility of the wrist joint with positive thumb sign.
Figure 4
Figure 4
Severe damping of coronary pressure with ventricularisation after cannulation of the left main coronary ostium.
Figure 5
Figure 5
Intravascular ultrasound (IVUS) showing severe ostial left main stenosis (blue arrow) and fibrous plaque (red arrow). Measurements confirmed severe stenosis (right image).
Figure 6
Figure 6
Right and left internal carotid arteries with two 180° turns (red arrows).
See this image and copyright information in PMC

References

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