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Isolated non-compaction of the ventricular myocardium (INVM), sometimes referred to as 'spongy myocardium', is a congenital and exceedingly rare cardiomyopathy. Isolated ventricular non-compaction occurs in the absence of other structural heart diseases and, hypothetically, it is due to the arrest of myocardial morphogenesis. Isolated non-compaction of the ventricular myocardium may manifest itself from infancy to young adulthood with a high mortality rate. Both sexes are affected. In our study, we present a case of INVM (left and right ventricles) in a 3-year-old girl, diagnosed by two-dimensional echocardiography. The anomaly presented as a restrictive cardiomyopathy. The girl was admitted to our hospital with heart failure, when she was 10 months old. She was treated with dopamine, digoxin, furosemide, spironolactone, and acenocoumarol and her condition improved. Presently, the girl remains asymptomatic and for 3 years of follow-up, her development has been almost normal. We here describe the genetic background of this disorder (based on a literature review).

We describe the clinical and genetic characteristics of the family, in which the diagnosis of LQT1 had been made. The electrocardiogram (ECG) characteristics of this patient indicated the likelihood of LQTS1. Polymorphic ventricular extrasystolies and episodes of polymorphic non-sustained ventricular tachycardia were confirmed by Holter ECG monitoring. On the exertional electrocardiogram polymorphic ventricular tachycardia (torsade de pointes) was recorded. Direct sequencing of both DNA strands revealed the absence of mutations or polymorphisms in the KCNQ1, HERG, and SCN5A genes.

Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue. In this study, we present a case of ARVD in 17 year old girl, who was admitted to the hospital after syncope with ventricular arrhythmia. The echocardiography did not demonstrate structural cardiac abnormalities but the magnetic resonance recently showed thinning of the right ventricular wall. The girl was treated with the lidocaine, amiodarone and next, after radiofrequency catheter ablation she was receiving metoprolol. The girl has remained asymptomatic for four years of follow-up.

We would like to express our gratitude and appreciation to the authors of the article ‘Genetic background of the left venricular hypertrabeculation/noncompation with stroke’ for fruitful comments of our manuscript.1 We agree with Finsterer et al. that in a single patient ventricular non-compaction is an acquired phenomenon. In our case of a 3-year-old girl with both chambers affected and heart failure, the disorder has not disappeared after treatment. Two-dimensional echocardiograms disclosed numerous prominent trabeculations in both ventricles with deep intertrabecular recesses and thickened endocardium. The echocardiographic images were diagnostic2 of a restrictive filling pattern. Mitral inflow velocities demonstrated a decrease in E/A ratio, consistent with restrictive left ventricular physiology. The left and right atria were enlarged. (LA-20 mm, Ao-14 mm). Left ventricular systolic function was depressed, with an ejection fraction (EF) of 48–50%. During follow-up, the EF of the left ventricle increased to 60–65% and now to 75%. But the ventricular morphology and restrictive filling pattern did …