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.

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.

BackgroundThe Center for Epidemiological Studies Depression Scale (CES-D) is an instrument that is commonly used to screen for depression in patients with chronic disease, but the characteristics of the CES-D in adults with congenital heart disease (CHD) have not yet been studied. The aim of this study was to investigate the criterion validities and the predictive powers of the CES-D for depression and anxiety disorders in adults with CHD.MethodsTwo hundred patients were screened with the CES-D and secondarily interviewed with a diagnostic instrument, i.e., the Mini International Neuropsychiatric Instrument. The sensitivity and specificity values of the CES-D were calculated by cross-tabulation at different cutoff scores. Receiver operating characteristic (ROC) curves were used to assess the optimal cutoff point for each disorder and to assess the predictive power of the instrument.ResultsThe CES-D exhibited satisfactory criterion validities for depression and for all combinations of depression and/or anxiety. With a desired sensitivity of at least 80%, the optimal cutoff scores were 18. The predictive power of the CES-D in the patients was best for major depression and dysthymia (area under the ROC curve: 0.92) followed by the score for any combination of depression and/or anxiety (0.88).ConclusionThe use of CES-D to simultaneously screen for both depression and anxiety disorders may be useful in adults with CHD. Trial registration: CESDEP 212. Registered 2 March 2014 (retrospectively registered).

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