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Left Ventricular Noncompaction Is More Prevalent in Ventricular Septal Defect Than Other Congenital Heart Defects: A
Laís Costa Marques1, Gabriel Romero Liguori1,2, Ana Carolina Amarante Souza1
1Laboratory of Pathology, Heart Institute (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo 05403-000, Brazil.
Insights
Left ventricular noncompaction (LVNC) morphology is more common in hearts with congenital heart defects (CHD), particularly ventricular septal defects (VSDs). This study found LVNC prevalence varied by diagnostic criteria across different CHDs.
Area of Science:
- Cardiology
- Developmental Biology
- Pathology
Background:
- Left ventricular noncompaction (LVNC) is a rare cardiomyopathy with prominent trabeculae and deep recesses.
- LVNC is associated with arrhythmias, thromboembolism, and heart failure.
- The prevalence of LVNC morphology in congenital heart defects (CHDs) requires further investigation.
Purpose of the Study:
- To determine the prevalence of LVNC morphology in various types of congenital heart defects (CHDs).
- To compare the prevalence of LVNC in CHD hearts versus control hearts.
- To assess the association between specific CHDs and LVNC morphology.
Main Methods:
- Examined 259 postnatal hearts with CHDs (VSD, ASD, AVSD, TGA, ISOM, EB, TF) and 11 control hearts.
- Assessed LVNC morphology using criteria proposed by Chin, Jenni, and Petersen.
- Measured left ventricular myocardial wall thickness (compacted and non-compacted).
Main Results:
- LVNC morphology was detected in hearts with CHDs, with prevalence varying by defect type and diagnostic criteria.
- Ventricular septal defect (VSD) hearts showed a significantly higher risk of LVNC compared to controls (Chin and Jenni criteria).
- No other CHD group demonstrated a significantly increased risk of LVNC compared to controls.
Conclusions:
- LVNC morphology is present in hearts with congenital heart defects, notably more frequent in VSDs.
- The prevalence of LVNC in CHDs is dependent on the diagnostic criteria used.
- This morphological study highlights an association but does not correlate with clinical symptoms or CHD severity.
Abstract:
Left ventricular noncompaction (LVNC) is a condition characterized by prominent ventricular trabeculae and deep intertrabecular recesses and has been described as a possible substrate for arrhythmias, thromboembolism, and heart failure. Herein, we explored the prevalence of LVNC morphology among hearts with congenital heart defects (CHD). We examined 259 postnatal hearts with one of the following CHD: isolated ventricular septal defect (VSD); isolated atrial septal defect (ASD); atrioventricular septal defect (AVSD); transposition of the great arteries (TGA); isomerism of the atrial appendages (ISOM); Ebstein's malformation (EB); Tetralogy of Fallot (TF). Eleven hearts from children who died of non-cardiovascular causes were used as controls. The thickness of the compacted and non-compacted left ventricular myocardial wall was determined and the specimens classified as presenting or not LVNC morphology according to three criteria, as proposed by Chin, Jenni, and Petersen. Normal hearts did not present LVNC, but the CHD group presented different percentages of LVNC in at least one diagnostic criterium. The prevalence of LVNC was respectively, according to Chin's, Jenni´s and Petersen´s methods: for VSD-54.2%, 35.4%, and 12.5%; ASD-8.3%, 8.3%, and 8.3%; AVSD-2.9%, 2.9%, and 0.0%; TGA-22.6%, 17%, and 5.7%; ISOM-7.1%, 7.1%, and 7.1%; EB-28.6%, 9.5%, and 0.0%; TF-5.9%. 2.9%, and 2.9%. VSD hearts showed a significantly greater risk of presenting LVNC when compared to controls (Chin and Jenni criteria). No other CHD presented similar risk. Current results show some agreement with previous studies, such as LVNC morphology being more prevalent in VSDs. Nonetheless, this is a morphological study and cannot be correlated with symptoms or severity of the CHD.
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