Clinical and epidemiological features of heart-hand syndrome, an updated analysis in China
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RESEARCH ARTICLE
Open Access
Clinical and epidemiological features of heart-hand syndrome, an updated analysis in China Yaobin Yin1†, Jianguang Ji2,3†, Junhui Zhao1, Shanlin Chen1*† and Wen Tian1*†
Abstract Background: The purpose of this study was to prospectively recruit patients treated with limb malformation and to explore the prevalence and the clinical and epidemiological features of Heart-Hand Syndrome (HHS) in China. Methods: The consecutive patients treated for congenital upper limb malformation in Beijing Ji Shui Tan Hospital from October 1st, 2016 to October 1st, 2019 were prospectively recruited. We reviewed the patients’ medical records and identified patients with abnormal electrocardiogram (ECG) and/or abnormal ultrasonic cardiogram as well as their basic demographic and clinical characteristics. Results: A total 1653 (1053 male and 600 female) patients with congenital upper extremity malformations were prospectively recruited. Among them, 200 (12.1%) had abnormal ultrasonic cardiogram (181patients, 10.9%) and/or abnormal ECG (19 patients, 1.1%). The commonest type of abnormal heart structure was atrial septal defect (69/181 38.1%), and the commonest abnormal ECG was wave patterns (7/19, 36.8%). HHS patients had a higher comorbidity rate (11%) than non-HHS patients (6.9%). Patients with HHS were classified into four groups by the types of congenital upper extremity malformations, among which the most common group was thumb type (121/200, 60.5%). Conclusions: HHS occurred frequently among patients with congenital upper extremity malformation in China, particularly for those with multiple congenital malformations. The commonest type of hand malformations of HHS patients was thumb malformation. Keywords: Heart-hand syndrome (HHS), Congenital malformations, Clinical epidemiology, Classification
Background Heart-hand syndrome (HHS) is characterized by the cooccurrence of a limb malformation and a congenital cardiac disease, presenting with substantial clinical and genetic heterogeneity [1]. HHS is a broad category of diseases which might be included in many different syndromes, such as the vertebral abnormalities, anal atresia, * Correspondence: [email protected]; [email protected] † Yaobin Yin, Jianguang Ji shared the first arthorship, Shanlin Chen and Wen Tian contributed equally to this work. 1 Department of Hand Surgery, Beijing Ji Shui Tan Hospital, Xin jie kou dong jie 31, Xi Cheng Qu, Beijing 100035, China Full list of author information is available at the end of the article
cardiac abnormalities, tracheoesophageal fistula and/or esophageal atresia, renal agenesis and dysplasia, and limb defect syndrome (VACTERL syndrome) [2], the “longthumb” brachydactyly syndrome [3], thrombocytopeniaabsent radius syndrome(TRS) [4], the Fanconi anemia syndrome [5], et al. Most congenital upper extremity malformations require surgical treatment, it is thus necessary to have a full understanding of the potential complications, such as simultaneous congenital heart disease, during anesthesia and operation. As
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