[Isolated non-compaction of the ventricular myocardium]. / Miocardio ventricular no compacto aislado.

Isolated non-compaction of the ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations, probably due to arrest of normal endomyocardial embryogenesis. We report two cases of isolated non-compaction of the ventricular myocardium.The first patient had a brother who died in the first year of life after heart transplantation due to refractory heart failure caused by restrictive biventricular cardiomyopathy associated with a right ventricular diverticulum. This could have been a case of isolated non-compaction of the ventricular myocardium that was not diagnosed. The patient, who is 8 years old, is asymptomatic and echocardiogram (ECG) showed multiple prominent ventricular trabeculations in the apical portion of the left ventricle, slightly depressed systolic and diastolic function and restrictive ventricular filling pattern.The second patient is a 7-year-old boy who presents recurrent syncopes. ECG showed multiple prominent ventricular trabeculations, restrictive ventricular filling pattern and normal systolic function. The Holter ECG recording showed multiple premature ventricular complexes and nonsustained ventricular tachycardia. An implantable subcutaneous Holter recorder was inserted, which was associated with an episode of aborted sudden death and polymorphic ventricular tachycardia followed by asystole. The patient was then treated with an implantable defibrillator.

[Clinical presentation, diagnosis and treatment of four cases of cor triatriatum]. / Presentación clínica, diagnóstico y tratamiento de cuatro casos de cor triatriatum.

We report four cases of cor triatriatum. In two of them the fibromuscular membrane was obstructive and associated with an atrial septal defect between the left atrial accessory chamber and the right atrium; in those cases the clinical findings were due to the pulmonary hypervascularity. The third case was associated to a small patent foramen ovale, and the clinical findings were due to the obstruction to flow through the membrane in the left atrium, producing venocapilar pulmonary hypertension. In these three cases surgical treatment was carried out through a right atriotomy. In all of them a good corelationship was found between the echocardiographic and the surgical findings. The follow-up has been good, all previous symptoms disappearing, remaining symptom free. The fourth case is an asymptomatic 5-year-old girl, with a non-obstructive membrane and without any associated anomalies.