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Influence of Chromosome 22q11.2 Microdeletion on Postoperative Calcium Level After Cardiac-Correction Surgery

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Abstract

One of the most common constitutional chromosomal abnormalities, 22q11.2 microdeletion (del22q11.2) syndrome has diverse medical complications, such as congenital heart defect, hypocalcaemia, and immune deficiency, which require coordinated multidisciplinary care. Until now, the natural history of hypocalcaemia in chromosome del22q11.2 syndrome had been only partly documented, but there has been limited recognition of the importance of calcium status during the postoperative period when altered calcium status may be associated with serious complications. The goals of our study were (1) to delineate the clinical characteristics of serum calcium in patients with del22q11.2 during the postoperative period and (2) to make recommendations for the investigation and management of del22q11.2 patients after cardiac correction. This study included 22 children diagnosed with del22q11.2 syndrome and 110 children without del22q11.2 syndrome from Nanjing Children’s Hospital. Clinical examinations and blood ionized calcium testing were reviewed retrospectively. A comparative study of postoperative calcium levels and complications of del22q11.2 patients with nondeletion patients was performed. Association between postoperative hypocalcaemia and adverse incidents after cardiac correction was also examined. Postoperative hypocalcaemia was observed among 86.4% of del22q11.2 patients and among only 47.3% of nondeletion subjects. The difference was statistically significant (P = 0.0017). Patients with del22q11.2 syndrome also had a much sharper decrease in serum calcium levels during the first 6 h after surgery than nondeletion patients. Postoperative clinical analysis showed that del22q11.2 patients with hypocalcaemia experience more postoperative complications (18 of 19) and greater mortality (5 of 19) after cardiac correction than del22q11.2 patients without normal calcium levels and nondeletion patients. Del22q11.2 children have high susceptibility of hypocalcaemia during the postoperative period, and this low calcium status after cardiac correction may be associated with significant risk of postoperative complications and mortality in patients with del22q11.2.

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Abbreviations

CHD:

Congenital heart defect

PTH:

Parathyroid hormone

CFMSA:

Competitive fluorescent multiplex STRP assay

TOF:

Tetralogy of Fallot

VSD:

Ventricular septal defect

FISH:

Fluorescent in situ hybridization

SPSS:

Statistical Package for the Social Sciences

CPB:

Cardiopulmonary bypass

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Acknowledgments

We thank all of the CHD patients and family members who participated in the studies. This work was supported by grants from the Foundation of Nanjing Medical, Pharmaceutical and Health Research Program (Grant No. YKK07052), National Natural Science Foundation of China (Grants No. 30772156 and 81070241), and Nature Science Foundation of Jiangsu Province (Grants No. BK2007011 and SBK200921216).

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Authors and Affiliations

  1. Department of Pathology, Nanjing University Medical School, Nanjing, 210093, People’s Republic of China

    Li Shen, Chi Yang, Yibing Ding, Huacheng Hou, Zhijuan Ge & Long Yi

  2. Department of Cardiothoracic Surgery, Nanjing Children’s Hospital, Nanjing Medical University, Nanjing, 210008, People’s Republic of China

    Li Shen, Haitao Gu, Yongzhong Jiang & Xuming Mo

  3. Department of Cardiothoracic Surgery, First Affiliated Hospital of Nanjing Medical University, Nanjing, People’s Republic of China

    Haitao Gu

  4. Department of Cardiothoracic Surgery, Drum Tower Hospital Affiliated to Nanjing University Medical School, Nanjing, People’s Republic of China

    Dongjing Wang, Chi Yang & Shilin Chen

  5. Center of Prenatal Diagnosis, Nanjing Maternity and Child Health Hospital, Nanjing Medical University, Nanjing, People’s Republic of China

    Li Shen & Zhengfeng Xu

  6. Department of Cardiothoracic Surgery, Jinling Hospital, Clinical Medicine School, Nanjing University, Nanjing, People’s Republic of China

    Hua Jing & Shilin Chen

Authors
  1. Li Shen
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  2. Haitao Gu
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  3. Dongjing Wang
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  4. Chi Yang
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  5. Zhengfeng Xu
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  6. Hua Jing
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  7. Yongzhong Jiang
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  8. Yibing Ding
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  9. Huacheng Hou
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  10. Zhijuan Ge
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  11. Shilin Chen
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  12. Xuming Mo
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  13. Long Yi
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Corresponding authors

Correspondence to Xuming Mo or Long Yi.

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Shen, L., Gu, H., Wang, D. et al. Influence of Chromosome 22q11.2 Microdeletion on Postoperative Calcium Level After Cardiac-Correction Surgery. Pediatr Cardiol 32, 904–909 (2011). https://doi.org/10.1007/s00246-011-0012-y

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  • DOI: https://doi.org/10.1007/s00246-011-0012-y

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