Employment status and contributing factors among adults with congenital heart disease in Japan.

BACKGROUND: Working is an important concern in transitional care for adults with congenital heart disease (ACHD) because work connects people with society. Employment status is correlated with gender, but studies on patient employment by gender have rarely been conducted. This study therefore aimed to examine the employment status of ACHD by gender and to explore the factors influencing this status. METHODS: In this study, 193 Japanese ACHD (mean age: men-33.62 years, women-32.69 years; 89 men, no students included) completed a questionnaire including questions about employment status, an evaluation of hindrances to employment, the Linear Analog Scale to assess quality of life (QOL), and the Satisfaction with Life Scale (SWLS). RESULTS: In the study sample, 13 of 89 (14.6%) men and 13 of 104 (12.5%) women did not have a job. These rates were higher than the national standard rates in Japan (men: 5.0%, women: 2.9%). Of these patients, only one man and one woman listed their illness as a reason for their unemployment. The factors thought to explain unemployment were age for men and disease severity for women (P < 0.05 for both). Unemployed patients had significantly lower QOL and SWLS scores. CONCLUSIONS: Most ACHD can join the workforce but a higher percentage of ACHD do not work and find it challenging to have a career compared with the general population. Moreover, because unemployed patients have low QOL and SWLS scores, obtaining work is crucial to enable these people to have mentally and emotionally stable and fulfilling lives.

Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial.

BACKGROUND: Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery abnormalities. METHODS: We did a randomised, open-label, blinded endpoints trial involving 22 hospitals in Japan between May 29, 2014, and Dec 27, 2016. Eligible patients predicted to be at higher risk for intravenous immunoglobulin (IVIG) resistance were randomly assigned to IVIG plus ciclosporin (5 mg/kg per day for 5 days; study treatment) or IVIG (conventional treatment) groups, stratified by risk score, age, and sex. The primary endpoint was incidence of coronary artery abnormalities using Japanese criteria during the 12-week trial, assessed in participants who received at least one dose of study drug and who visited the study institution at least once during treatment. This trial is registered to Center for Clinical Trials, Japan Medical Association, number JMA-IIA00174. FINDINGS: We enrolled 175 participants. One patient withdrew consent after enrolment and was excluded and one patient (in the study treatment group) was excluded from analysis because of lost echocardiography data. Incidence of coronary artery abnormalities was lower in the study treatment group than in the conventional treatment group (12 [14%] of 86 patients vs 27 [31%] of 87 patients; risk ratio 0·46; 95% CI 0·25-0·86; p=0·010). No difference was found in the incidence of adverse events between the groups (9% vs 7%; p=0·78). INTERPRETATION: Combined primary therapy with IVIG and ciclosporin was safe and effective for favourable coronary artery outcomes in Kawasaki disease patients who were predicted to be unresponsive to IVIG. FUNDING: Japan Agency for Medical Research and Development (grant CCT-B-2503).

Surgical treatment for adult congenital heart disease: consideration for indications and procedures.

The number of the adult patients with congenital heart diseases (ACHD) continues to grow owing to improvement of surgical results and medical management. Corrective surgery for complex CHD does not always mean complete cure. It is not rare that the patients will visit the cardiology institutes because of secondary lesions due to residua or sequela in adults. Some patients with CHD remain unrepairable with different degree of heart failure and pulmonary arterial hypertension. Association of arrhythmias is common in ACHD patients and sometimes critical. We experienced 265 surgical procedures for ACHD patients at our center between 1999 and 2015. Of these procedures, palliative surgery was performed in 3%, palliation to corrective surgery in 6%, primary repair in 57%, and redo surgery in 34%. Hospital mortality within 30 days in this period was 1.1%. Surgery for ACHD patients is safe, beneficial and low-risk treatment; however, tailored procedures for the individual patient are essential to obtain the optimal quality. A comprehensive multidisciplinary approach is required to fulfill this goal.

Vasospastic angina in a 13-year-old female patient whose only symptom was toothache.

Vasospastic angina was confirmed in a 13-year-old female patient at autopsy. The patient's only symptom was recurrent toothache, which began when she was 10 years old. In November 2000, she was evaluated at our medical center; however, all examinations were judged normal. Six months later, she suffered a severe toothache. She went to school the next morning after the symptoms improved. She lost consciousness at school and was given cardiopulmonary resuscitation but could not be revived. At autopsy, her three coronary vessels showed marked intimal hyperplasia, and multiple focal myocardial infarctions were observed in the lateral wall of the left ventricle. The patient's only clinical symptom was toothache and none of the physicians realized that this was caused by angina. Vasospastic angina begins at a young age and is one of the causes of sudden death in adolescents.