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BackgroundWe used structural equation modeling to evaluate the quality of life (QOL) for patients with Marfan syndrome (MFS). The goal was to provide guidelines to facilitate the development of interventions and strategies to improve the QOL for patients with MFS.MethodsThe participants fulfilled the Ghent 2 criteria for MFS and they comprised patients who visited the cardiology outpatient department of a tertiary hospital in Seoul, Korea, between August 17, 2013 and April 17, 2014. Demographic, social support, disease-related factors, biobehavioral factors, and QOL data were collected in one-on-one interviews.ResultsThe final analyses included 218 patients. Anxious and depressed patients comprised 63.8 and 71.5 % of the sample, respectively. For the hypothetical model, the goodness-of-fit index = 0.91, normal fit index = 0.93, and comparative fit index = 0.90. The outcome was suitable for the recommended level, so the hypothetical model appeared to fit the data. In patients with MFS, the QOL was affected significantly by social support, disease-related factors, and biobehavioral factors. These variables explained 72.4 % of the QOL in patients with MFS. Biobehavioral factors had the strongest and most direct effects on QOL.ConclusionTo improve QOL in patients with MFS, comprehensive interventions are necessary to assess and manage biobehavioral factors, social support, and disease-related factors.

Chylothorax is a rare but serious postoperative condition with a high rate of morbidity that may lead to death of children with congenital heart disease. Here we reviewed nine consecutive cases with chylothorax in infants and children following cardiac surgery from March 2002 to February 2003. Somatostatin was added to conservative treatment proctocol to increase effectiveness of therapy in all cases. The duration of somatostatin therapy varied from 7 to 32 days. All cases of chylothorax were successfully treated with intravenous infusion of somatostatin as an adjunctive treatment. Even though two cases showed rebound phenomena, we avoided any surgical procedure in the nine patients who treated with conservative management combined with somatostatin. No significant side effects of somatostatin were observed. It seems that somatostatin is effective, noninvasive and safe therapeutic modality. It can be used as an adjunctive treatment to conservative management to control postoperative chylothorax in children with congenital heart disease.

Pregnancy outcomes in patients with congenital heart disease have not been fully assessed in Korea. Forty-nine pregnancies that occurred in 34 women with congenital heart disease who registered at our hospital between September 1995 and April 2006 were reviewed. Spontaneous abortions occurred in two pregnancies at 6+1 and 7 weeks, and another two underwent elective pregnancy termination. One maternal death in puerperium occurred in a woman with Eisenmenger syndrome. Maternal cardiac complications were noted in 18.4%, pulmonary edema in 16.3%, symptomatic arrhythmia in 6.1%, deterioration of New York Heart Association (NYHA) functional class by ≥2 in 2.0%, and cardiac death in 2.0%. Independent predictors of adverse maternal cardiac events were an NYHA functional class of ≥3 (odds ratio [OR], 20.3), right ventricular dilation (OR, 21.2), and pulmonary hypertension (OR, 21.8). Neonatal complications occurred in 22.4% of pregnancies and included preterm delivery (16.3%), small for gestational age (12.2%), and neonatal death (2.0%). Independent predictors of adverse neonatal events were pulmonary hypertension (OR, 6.8) and NYHA functional class ≥3 (OR, 23.0). Pregnancy in women with congenital heart disease was found to be significantly associated with maternal cardiac and neonatal complications. Pre-pregnancy counseling and multidisciplinary care involving cardiologists and obstetricians are recommended for women with congenital heart disease contemplating pregnancy.

Hunter syndrome (mucopolysaccharidosis II, MPS II) is a rare, X-linked disorder of glycosaminoglycan (GAG) catabolism caused by a deficiency in the activity of the lysosomal enzyme, iduronate-2-sulfatase (I2S). In this study, the medical records of 75 Korean patients with Hunter syndrome (74 males, 1 female) were retrospectively reviewed to investigate the frequency of organ involvement and survival at a single center. The three most common symptoms of organ involvement were hepatosplenomegaly (99%), facial dysmorphism (97%), and frequent otitis media (91%). Cardiovascular involvement was also common including valvular abnormalities (89%), left ventricular hypertrophy (68%), and hypertension (30%). The 19 patients who died had a median age of 16.8 years at the time of death. Four of them died within 1 year of the start of enzyme replacement therapy; autopsy showed myocardial infarction with severe coronary artery disease in one patient. Two other patients died due to pneumonia and sleep apnea. In one case, the cause of death was not investigated. The high incidence of hypertension, and the presence of valvular heart disease indicates that close cardiac monitoring is mandatory in all patients with Hunter syndrome, especially relatively older patients even if they are being treated with enzyme replacement therapy.

Based on this study, slide tracheoplasty seems to be an effective technique for CTS. However, shortening of the trachea after reconstruction may give rise to recurrent obstruction.

Given the high rate of aspiration in the infants who underwent open heart procedures, monitoring and prompt recognition of the signs and the risk factors of dysphagia may substantially improve infant care with oral feeding and reduce the duration of hospital stays.

Home orthostatic self-training was ineffective in reducing the positive response rate of head-up tilt test in patients with recurrent neurally mediated syncope.