Abstract
BackgroundThe 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.MethodsThe 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.ResultsA 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%).ConclusionsHHS 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.
Highlights
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
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 necessary to have a full understanding of the potential complications, such as simultaneous congenital heart disease, during anesthesia and operation
In our previous retrospective study, we found that the prevalence of Heart-hand syndrome (HHS) was around 11.8% among 1462 patients treated with congenital upper extremity malformations [7]
Summary
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. In light of the increased number of patients with congenital upper extremity malformation in China, we expected that more HHS cases might be diagnosed in Chinese patients We planned this cohort study and aimed (1) to prospectively recruit patients with congenital upper extremity malformation and explore the prevalence of HHS, (2) to update the characteristics and the clinical features of HHS in China, (3) to make a new classification for HHS based on the clinical characteristics of hand malformations as previous classifications of HHS do not meet the need of HHS patients in China
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