Sudden collapse in a 16-year-old Girl: Case report

Introduction: Left Ventricular Non-Compaction(LVNC) is a genetic cardiomyopathy caused by disruption in the embryogenesis of the endocardium and the myocardium between the 5th and 8thweek of gestation.It was known as“spongy myocardium”and is characterized by trabeculations and deep intertrabecular recesses in the ventricular myocardium,which may lead to myocardial scaring,ventricular dysfunction and arrhythmia.The isolated form of the disease is rare.The most frequent presentation is heart failure but arrhytmias and thromboembolic events can present as first clinical features.

Objective: We present a case of a rare arrhythiogenic congenital cardiomyopathy.

Material: We present a 16-year-old girl who was brought to our emergency department(ER) after sudden collapse while walking.During the transportation the rescuers began CPR and the automatic external defibrillator(AED) gave an electric shock.When the patient arrived in the ER she held a Glascow Coma Scale(GCS):3/15 with rhythmic pulse,blood pressure(BP):120/60mmHg,SO2:97% and no responsive pupils.Due to her age,substance abuse was thought and Naloxone and Anexate were administered offering no result.After 15minutes she reacted with decerebration while stimulated with pain and the pupils became responsive to light.

Results: She was immediatelly intubated and after a brain CT-scan that was negative for trauma, hemorrhage or tumor she was transferred to an Intensive Care Unit(ICU).She became alert after 48hours and showed no neurological dysfunction.A heart MRI revealed asymmetrical septal inferior and lateral segments with large trabeculations and normal systolic function of the left ventricle.The Ejection Fraction(EF) was normal(69%) and a DDDR pacemaker was placed.Since then she remains symptom-free, in all her follow-ups in our cardiology department.

Conclusion: A congenital heart disease can present lethally at any age and should be suspected at all cases of sudden collapse.The only mandatory is proper CPR and if done flawlessly,the necessary time for acquiring the diagnosis will be allowed.