Sotos Syndrome, Isolated Left Ventricular Non Compaction Cardiomyopathy and Ventricular Pre Excitation: A Case Report. - Sotos Syndrome, Isolated Left Ventricular Non Compaction Cardiomyopathy and Ventricular Pre Excitation: A Case Report. - Open Access Pub

We report the case of a 22 year-old-male patient affected by Sotos syndrome was preoperatively and urgently evaluated for tracheal stent implantation due to respiratory insufficiency caused by idiopathic tracheal stenosis. Rest electrocardiogram detected a ventricular pre excitation; a transthoracic echocardiography showed a classic pattern fulfilling criteria for isolated left ventricular non compaction. At the best of our knowledge this is the first report of a patient suffering from Sotos syndrome and simultaneously affected by isolated left ventricular non compaction and ventricular pre excitation. DOI 10.14302/issn.2329-9487.jhc-12-155 A 22 years old male affected by Sotos Syndrome underwent urgent preoperative cardiologic evaluation for an endoscopic tracheal stent implantation because of a respiratory insufficiency due to idiopathic tracheal stenosis. Two month before he was discharged from another Hospital with diagnosis of pericarditis and apical hypertrophic cardiomyopathy. At physical examination he presented the common features of Sotos syndrome: characteristic facial appearance, a moderate to severe delayed cognitive and motor development, a severe kyphoscoliosis, epilepsy anamnesis and respiratory tirage due to “idiopathic tracheal stenosis”. Rest electrocardiogram showed sinus tachycardia 110/m and no other anomalies. Nevertheless an ECG performed in the operatory room next day showed sinus tachycardia and a ventricular pre excitation compatible with a right anterolateral accessory pathway (Figure 1). The transthoracic echocardiogram performed in the same day demonstrated only left ventricle posterior apex, inferior and lateral wall mild thickening; furthermore, it showed two layered structure with numerous prominent trabeculae and deep intertrabecular recesses directly communicating filled with left ventricular cavity as assessed by Color Doppler. The ratio between tick non compacted layer and thin compacted part measured at end systole in short axis was > 2, according Jenni criteria for isolated left ventricular non compaction (ILVNC) (Figure 2) 1,2. Figure 1. Sinus tachycardia and ventricular pre excitation Figure 2. Echo image focused on infero-lateral apical segment of left ventricle Left ventricular ejection fraction was normal and any other pathology was excluded.A successful tracheal stent implantation was performed and he was discharged in good condition after two days. Cardiac MRI was not promptly performed because of his psycho-physical condition and recent stent implantation as well as electrophysiological study (because he