Min-Tai Lin

Prevalence and the Long-Term Coronary Risks of Patients With Kawasaki Disease in a General Population <40 Years A National Database Study

Background— Patients with Kawasaki disease (kDa) may develop coronary arterial lesions and subsequent coronary events. The first reported case in Taiwan was in 1976, and the annual incidence from 2003 to 2006 was 69/100 000 children < 5 years. A population study from Taiwan, a country with a high incidence of kDa, national health insurance, and easily accessible medical care, would adequately reflect the long-term risk. Methods and Results— We retrieved the data of kDa patients from a national health insurance 2000 to 2010 database of Taiwan, a country with a child health index similar to those in the United States. The occurrence of coronary complications and interventions was identified by the respective International Classification of Diseases, Ninth Revision, codes. The prevalence of kDa in the population < 40 years was 34.9/100 000 (male/female ratio, 1.47). Coronary complications occurred in 1254 patients (5.37%; male/female ratio, 2.19), with an average annual risk of 2.4% (2.7% for males and 2.0% for females). An acute myocardial infarction occurred in 19 patients (0.08%; 18 males and 1 female), of whom one third were aged between 10 and 15 years (median, 15.7 years; range, 0.7–36.7 years). A coronary intervention was performed by catheterization in 18 patients (all males) at a median age of 24.5 years and by surgery in 10 patients (male/female ratio, 4.0) at a median age of 21.7 years, with mortality at discharge being 0% and 25%, respectively. Conclusions— This study estimated the overall prevalence of kDa (≈1/2940) in a population < 40 years. They, particularly the males, carry long-term coronary risks from a young age. Risk stratification for a timely coronary intervention and risk modification are mandatory.

Outcome in Children Bridged and Nonbridged to Cardiac Transplantation

BACKGROUND Heart transplantation (HTx) in children with end-stage heart disease has become an accepted treatment option. OBJECTIVE To evaluate our results of pediatric cardiac transplantation with vs without bridge methods. PATIENTS AND METHODS The study included 31 patients (34 transplantations) younger than 18 years who underwent orthotopic HTx between March 1995 and December 2008. Ten patients were girls, and 21 were boys. Preoperative diagnoses included cardiomyopathy (n=20), congenital heart disease (n=7), hypertrophic cardiomyopathy (n=2), restrictive cardiomyopathy (n=1), and ischemic cardiomyopathy (n=1). Mean (SD) ischemia time was 185 (72) minutes. Thirty-day mortality was 6%, and was due to primary graft failure (n=2). Overall follow-up was 4.36 (3.93) years. Eleven patients underwent bridge techniques before HTx, and 11 patients required perioperative extracorporeal membrane oxygenation or ventricular assist device support. RESULTS In the group that received extracorporeal membrane oxygenation, 8 patients (73%) were successfully weaned and discharged with excellent functional class. There were no differences in operative mortality, functional class, survival, rejection, and infection rates between the bridged and nonbridged groups. Overall actuarial 1- and 5-year survival rates were 93% and 83%, respectively. All survivors had good functional class. CONCLUSION Our findings demonstrate satisfactory medium-term outcome of HTx in selected pediatric patients with end-stage heart disease. Using bridge methods in children at high risk can increase the opportunity to receive a donor heart. These bridge methods achieve similar postoperative outcomes.

The Outcome of Heart Transplantation in Hepatitis C-Positive Recipients

BACKGROUND Clinical outcomes of heart transplantation (HTx) among recipients with chronic hepatitis C virus (HCV) infection are poorly understood especially in Asia. Therefore, this study evaluated these clinical outcomes. METHODS Using retrospective chart review we collected data on 385 patients including 20 HCV-positive recipients at the time of transplantation. We obtained information on demographics features, serial transaminases, graft function, patient survival as well as the incidences of acute hepatitis and transplant coronary artery disease. RESULTS Between 1987 and 2010, the 20 HCV-positive patients had a median age at transplantation of 52 years (range, 30-63). Seventeen were men and three women. All the patients were classified as Child-Pugh class A; two had cirrhosis prior to HTx. Over a mean follow-up of 63 months (range, 2 days to 187 months), there were 11 deaths, including two hospital mortalities and nine subsequent deaths. Only one mortality (5%) was related to Child-Pugh class C cirrhosis, despite liver transplantation. Among the other 19 deceased or surviving recipients, there was no evidence of hepatic dysfunction or hepatocellular carcinoma. Transplant coronary artery disease was detected in six patients (30%). There was no significant difference in Kaplan-Meier actuarial survival between the HCV-positive and HCV-negative recipients (P = .59). CONCLUSIONS There was no significant difference in patient survival or graft function between HCV-positive and HCV-negative HTx recipients. Additionally, HCV-positive recipients were not at an increased risk of hepatic failure or accelerated transplant coronary artery disease.

Steroid Pulse Therapy Combined with Plasmapheresis for Clinically Compromised Patients after Heart Transplantation

BACKGROUND The most serious complication after heart transplantation is allograft dysfunction. Patients presenting with compromised hemodynamics show a high incidence of mortality. The most common reason for allograft dysfunction is rejection. We have employed steroid pulse therapy combined with plasmapheresis for hemodynamically compromised patients after heart transplantation. METHODS AND RESULTS Steroid pulse therapy and plasmapheresis were performed on 35 patients who underwent orthotopic heart transplantation for graft dysfunction. Thus treatment rescued ventricular function and improved the ejection fraction in 77% of patients, among who ever 71.4% showed improved New York Heart Association (NYHA) functional class. CONCLUSIONS Steroid pulse therapy combined with plasmapheresis improved the cardiac contractility and NYHA functional class of most heart transplant recipients with graft dysfunction.

Arterial switch operation for transposition of the great arteries: experience from 2000-2002 in Taiwan.

Arterial switch operation (ASO) is considered the procedure of choice for transposition of great arteries (TGA). The results and long-term prognosis improved with recent advances in perioperative management. We herein analyze the clinical outcome of patients undergoing ASO at our institution during the past 3 years. From 2000 to 2002, 44 patients (30 male and 14 female) of TGA received ASO. Age at operation varied from 4 days to 6.6 years (median 14 days) with body weight ranged from 2.25kg to 18.1kg (median 3.3kg). Palliative procedure prior to ASO was performed in 8 patients 18.8%. Normal coronary artery pattern was found in 28 patients (63.6%). The early mortality was 11%(5/44). Only associated ventricular septal defect (VSD) was a significant predictor for operative mortality (p=0.012). With a follow-up ranged from 11 to 44 months, the gradient of neo-pulmonary artery stenosis was 16.5±18.2 mmHg. Four patients (10%) received balloon dilatation and the other three (7.7%) underwent reoperation. The gradient of neo-aortic stenosis was 16.5 ±18.2 mmHg that needed to be dilated in three patients (7.7%). The probability free from reintervention was 73% at the 3d postoperative year. One patient had moderate degree of pulmonary valve regurgitation and six had moderate neo-aortic valve regurgitation. In conclusion, the ASO can be performed in infants with satisfactory results, even in those with a body weight less than 2.5kg. Only associated VSD was shown to be a risk factor.

Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study

Objective To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third‐highest incidence of Kawasaki disease worldwide. Study design We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000‐2014 in the Taiwan national database. Results Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P < .001) and exhibited an increasing trend with birth year (from 2.28‰ for 2000 to 3.67‰ for 2009). The incidence ratio was 1.535 in boys and 1.055 in each increasing year. Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1‐4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%. Conclusions The postnatal risk of Kawasaki disease was 3‰‐4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood.