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Persisting spongy myocardium. A case indicating the difficulty of antenatal diagnosis
N Winer1, M Lefèvre, M F Nomballais
1Service de Gynécologie-obstétrique B, Centre Hospitalier Régional et Universitaire, Nantes, France.
Insights
Persisting spongy myocardium, a rare cardiomyopathy, is difficult to detect before birth. This case highlights its antenatal diagnosis via fetopathology, leading to therapeutic abortion.
Area of Science:
- Cardiology
- Developmental Biology
- Medical Genetics
Background:
- Persisting spongy myocardium (PSM), or noncompaction of the ventricular myocardium, is a rare cardiomyopathy.
- It is characterized by excessive trabeculae and intertrabecular recesses, stemming from abnormal embryonic endomyocardial morphogenesis.
- Most reported cases are diagnosed postnatally and associated with severe cardiovascular complications.
Observation:
- Antenatal detection of PSM is challenging, particularly when isolated without other fetal malformations.
- This report details a case where PSM was suspected antenatally.
- The diagnosis was confirmed through detailed fetopathologic examination after therapeutic abortion.
Findings:
- The study confirms the difficulty of antenatal diagnosis for isolated persisting spongy myocardium.
- Fetopathology proved crucial for definitive diagnosis in this complex case.
- The case underscores the potential for severe cardiac anomalies to manifest during embryonic development.
Implications:
- This case highlights the importance of advanced fetopathology in diagnosing rare congenital heart conditions.
- Early diagnosis, though challenging, could inform crucial reproductive decisions.
- Further research into antenatal diagnostic markers for PSM is warranted to improve outcomes.
Abstract:
Persisting spongy myocardium (also known as noncompaction of the ventricular myocardium) is a rare and special form of cardiomyopathy. The few cases reported in the literature were detected postnatally and involved a high rate of cardiovascular complications. This anomaly of endomyocardial morphogenesis, which occurs during the embryonic phase at the stage of cardiac partitioning, is characterised by an excessive number of prominent trabeculae and by intertrabecular recesses within the myocardial wall. Antenatal detection is difficult in the absence of an associated malformation, which is the general situation. We report a case of antenatal cardiomyopathy which led to therapeutic abortion. The diagnosis of persisting spongy myocardium was made during fetopathologic examination.