Journal of Pediatric Cardiology and Cardiac Surgery

Online ISSN: 2433-1783 Print ISSN: 2433-2720
Japanese Society of Pediatric Cardiology and Cardiac Surgery
Japanese Society of Pediatric Cardiology and Cardiac Surgery Academy Center, 358-5 Yamabuki-cho, Shinju-ku, Tokyo 162-0801, Japan
Journal of Pediatric Cardiology and Cardiac Surgery 6(1): 6-10 (2022)

Original ArticleOriginal Article

Psychiatric Disorders in Patients with 22q11.2 Deletion Syndrome Concomitant with Congenital Heart Disease in Japan

1Department of Cardiology, Tokyo Metropolitan Children's Medical Center ◇ Tokyo, Japan

2Department of Pediatric Cardiology and Adult Congenital Cardiology, Tokyo Women’s Medical University ◇ Tokyo, Japan

3Department of Pediatrics, Nippon Medical School Tama Nagayama Hospital ◇ Tokyo, Japan

4Department of Pediatrics, Toho University Omori Medical Center ◇ Tokyo, Japan

5Department of Development Strategy, Center for Clinical Research and Development, National Center for Child Health and Development ◇ Tokyo, Japan

6Department of Pediatric Cardiology, Sakakibara Heart Institute ◇ Tokyo, Japan

7Department of Pediatrics, Keio University School of Medicine ◇ Tokyo, Japan

8Department of Pediatrics, The University of Tokyo Hospital ◇ Tokyo, Japan

受付日:2021年6月19日Received: June 19, 2021
受理日:2021年8月27日Accepted: August 27, 2021
発行日:2022年1月31日Published: January 31, 2022

Background: Since most patients with 22q11.2 deletion syndrome (22q11DS) have congenital heart defects (CHD), they are usually followed by pediatric cardiologists. Throughout their life, about two-thirds of patients with 22q11DS suffer from developmental and psychiatric disorders. In this setting, pediatric cardiologists may be the first health care providers who need to identify psychiatric problems, but the actual state of psychiatric care in routine cardiology check-up remains unknown.

Patients and Methods: We retrospectively reviewed the numbers of 22q11DS patients with both psychiatric disorders and CHD in 12 collaborating facilities specialized in CHD in Japan (primary survey). In two elective hospitals where a comprehensive healthcare program for patients with 22q11DS has been provided, further surveys were carried out to collect detailed information regarding the clinical profile of psychiatric disorders and CHD (secondary survey).

Results: Two hundred and twenty-two patients with 22q11DS and CHD were reported to have psychiatric disorders. While thirty-nine out of 222 patients (18%) received specialized care by psychiatrists, the rest (82%) were followed by pediatric cardiologists in the primary survey. Twenty-seven patients were included in the secondary survey, and 26 patients (96%) were treated for psychiatric problems by psychiatrists. The median age at onset of psychiatric disorders was 24 years, and 59% of them were diagnosed with schizophrenia.

Conclusions: In this nationwide survey, most of pediatric cardiologists did not consult experts about psychiatric problems of 22q11DS. Aiming to improve prognosis, early collaboration with psychiatrists is necessary for pediatric cardiologists facing psychiatric disorders, especially schizophrenia.

Key words: psychiatric disorders; schizophrenia; congenital heart disease; 22q11.2 deletion syndrome

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