ABSTRACT
@#Objective To evaluate the early- and mid-term outcomes of surgical repair of persistent truncus arteriosus in children in a single institution of China. Methods The clinical data of 27 consecutive patients with persistent truncus arteriosus undergoing surgical repair in Guangzhou Women and Children’s Medical Center from November 2009 to May 2018 were retrospectively reviewed. There were 14 males and 13 females. Median age was 3.0 months (range: 13 days -11 years), of whom 10 (37.0%) were older than 6 months. Results There were three early deaths with a mortality of 11.1%. The main complications included VSD partial repair in 2 patients, complete atrioventricular block in one patient. The mean follow-up time was 24.5±19.3 months (range: 1–76 months). There were three late deaths, and two patients lost follow. Echocardiology showed seven patients of right heart outflow tract obstruction, including three in pulmonary artery trunk, and four of pulmonary artery branches. One patient showed moderate aortic valve regurgitation. None required re-intervention during the follow-up. Survival estimates for the entire cohort following surgery were both 76.1% (95%CI 59.2% to 92.9%) at 1 year and 5 years. Conclusion The surgical repair of persistent truncus arteriosus (PTA) remains challenges. The early- and mid-term outcomes of surgical repair of persistent truncus arteriosus are acceptable. For older children with severe pulmonary artery hypertension and/or trunk valve regurgitation, the risk of death is still higher. Some children have the higher risk of late right heart obstructive lesions.
ABSTRACT
Hereditary angioedema (HAE) is a rare, life-threatening, autosomal dominant disease characterized by recurrent episodes of angioedema, and caused by a deficiency of the plasma protein C1-esterase inhibitor (C1-INH). Clinical manifestation of HAE may first develop during childhood but typically presents around puberty with nonpruritic and non-pitting edema of the subcutaneous and mucosal tissues. Up to now, there has been no published report of HAE case in Taiwan. We reported a 33 year-old female patient who had recurrent painful swelling of face and hands since 27 years of age. She first suffered from sudden onset of painful swelling of the eyelids and lips in August 1998 when she was pregnant for the first time. Subsequently, similar episodes recurred for a few times. Her blood test disclosed that her C3 and C4 were 125 mg/dl and 6 mg/dl, respectively. Her uncle died of laryngeal edema at the age of 30 years. Her father and elder brother also had the similar history of recurrent facial and hand swelling. The C4 levels of her elder brother were 6 mg/dl and 13.3 mg/dl on two separate occasions. The C1-INH antigen serum level and functional assay of the index patient and ten other family members were studied. A total of seven members of the family were confirmed to have type 1 HAE as evidenced by the low C4 and low C1-INH antigenic level and functional activity. Two of the seven cases were asymptomatic up to the date of our report.