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Cardiac Calcifications in Adults with Congenital Heart Defects
Dan G Halpern1,2,3,4, Michael L Steigner5,6, Sanjay P Prabhu6,7
1Adult Congenital Heart Disease and Pulmonary Hypertension Service, Department of Cardiology, Boston Children's Hospital, Boston, Mass, USA.
Insights
Cardiac calcification is common in adults with congenital heart disease. This study found calcification linked to surgery, regional dysfunction, and sudden death in these patients.
Area of Science:
- Cardiovascular Research
- Medical Imaging
- Congenital Heart Disease
Background:
- Recent autopsy findings suggested a high prevalence of calcification in adult congenital heart disease (ACHD) patients.
- Understanding calcification patterns is crucial for managing ACHD.
Purpose of the Study:
- To investigate the type and extent of cardiac calcification in adult congenital heart disease patients.
- To correlate calcification patterns with clinical data and outcomes.
Main Methods:
- Computed tomography (CT) was used to examine seven adult heart specimens (>18 years) with congenital heart defects.
- Three-dimensional (3D) data sets were reformatted for detailed analysis of calcium deposition patterns.
- Clinical data, including diagnosis and surgical history, were recorded.
Main Results:
- Five of seven hearts exhibited extensive calcifications.
- Calcification patterns included atherosclerosis (oldest patients), surgery-associated (four of five post-surgery hearts), and remote myocardial calcification.
- Myocardial calcification correlated with regional dysfunction and was observed in patients with sudden, unexpected death.
Conclusions:
- Cardiac calcification is frequent in adults with congenital heart defects.
- Calcification is often, but not always, associated with prior cardiac surgery.
- In this series, calcification was linked to regional dysfunction and sudden death.
Objective:
We investigated the type and extent of calcification in a series of heart specimens from adult congenital heart disease patients because recent autopsy observations suggested a high prevalence of calcification.
Design:
We used computed tomography to examine seven heart specimens from adults (>18 years old) with a congenital heart defect collected with permission from the family during a recent 3-year period. Clinical data regarding diagnosis, history, and imaging studies were recorded. The 3D data sets were reviewed after reformatting as maximum intensity projection and volumetric renderings to determine the pattern and extent of calcium deposition.
Results:
Five of the seven hearts had extensive calcifications in one or more of three patterns: atherosclerosis associated in the three oldest cases; surgery associated in four of five hearts that had undergone heart surgery; and myocardial calcification remote from surgical sites in two cases. Myocardial calcification was associated with regional dysfunction and was present in the three patients that died suddenly and unexpectedly.
Conclusion:
Cardiac calcification was frequent in our series of heart specimens from adults with congenital heart defects, was often but not uniformly associated with prior surgery, and, in our small series, was associated with regional dysfunction and sudden death.
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