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[Isolated abnormality ("noncompaction") of the myocardium in 3 children]
A Hussein1, A A Schmaltz, E Trowitzsch
1Abt. Kinderkardiologie, Pulmologie und Schlaflabor, Vestische Kinderklinik, Universität Witten-Herdecke.
Insights
Isolated myocardial noncompaction, a rare heart condition, was found in three asymptomatic children. Early detection and management are key for these rare congenital heart defects.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Myocardial noncompaction is a rare congenital heart malformation characterized by a spongy appearance of the myocardium.
- It can occur in isolation or be associated with other congenital heart defects.
- Understanding its presentation in children is crucial for early diagnosis and management.
Observation:
- Three asymptomatic children were diagnosed with isolated myocardial noncompaction via echocardiography at varying ages.
- One infant presented with severe biventricular and septal involvement and depressed myocardial function, remaining asymptomatic on therapy.
- Two other children showed isolated left ventricular involvement with normal myocardial function, one with epilepsy-encephalopathy and the other with Wolff-Parkinson-White syndrome.
Findings:
- Isolated myocardial noncompaction can present asymptomatically in children.
- The extent of myocardial involvement and function may vary.
- Novel associations with epilepsy-encephalopathy and Wolff-Parkinson-White syndrome were observed.
Implications:
- Early echocardiographic detection of myocardial noncompaction is vital in pediatric patients.
- Management strategies may involve anticongestive therapy, similar to dilated cardiomyopathy.
- Further research is needed to understand the long-term outcomes and specific associations of this rare condition.
Unlabelled:
In three asymptomatic children an isolated myocardial noncompaction was detected by echocardiography at age 11 months, 5 weeks and 5.5 years. In the first male infant both ventricles and septum were severely affected and myocardial function was depressed. Nevertheless, during a follow up of 16 months he remained asymptomatic on anticongestive therapy. In the other two children apex and lateral wall of the left ventricle were affected and myocardial function was still normal. The second boy had also an infantile epilepsy-encephalopathy syndrome and the third child (a girl) had a Wolff-Parkinson-White syndrome; an association of either syndromes with myocardial noncompaction has not been reported earlier.
Discussion:
Myocardial noncompaction (spongy myocardium) is a rare maldevelopment, which occurs either associated with certain congenital heart defects or, even more rarely, isolated, as the two cases reported here. Myocardial failure, severe arrhythmias or thromboembolism may occur at any age and determine the outcome. Clinical course, therapy and prognosis are similar to dilatative cardiomyopathy, which represents an important differential diagnosis.