Changes in the Etiology and Clinical Characteristics of Pediatric Infective Endocarditis in South Korea.

OBJECTIVE: Changes in the epidemiology, clinical manifestations, and outcomes of infective endocarditis (IE) have been reported in recent years. This study was performed to analyze changing trends in the etiology of IE in children in South Korea. STUDY DESIGN: A retrospective study of children with IE was conducted from 2 hospitals in Korea from 2004 to 2019. IE was defined according to the Modified Duke criteria. We analyzed the etiology and clinical outcome of 96 cases and compared the etiology with that during 1987-1997, as described in a previous study. RESULTS: A total of 96 cases in 89 children satisfied the modified Duke criteria. The median age was 9.5 years. Preexisting structural heart diseases were identified in 81 cases (84.4%). Ten of the 15 cases with normal heart anatomy had a history of indwelling central venous catheters and underlying noncardiac conditions. Of the 72 identified organisms, common pathogens included Staphylococcus aureus, 24 (32.9%); viridans streptococci, 22 (30.2%); coagulase-negative staphylococci, 14 (19.2%); and Candida species, 7 (9.6). Sixty-three cases (66.7%) required surgical intervention, and the overall fatality rate was 7.9%. Compared with the period of 1987-1997, an increasing trend in the proportion of staphylococci as the etiology of IE in 2004-2019 was observed (26.7% vs. 51.8%, P = 0.029). In addition, S. aureus was associated with the high rate of developing complications (P < 0.05). CONCLUSION: In South Korea, Gram-positive cocci represent 85% of all etiologic agents of IE in children with an increasing trend in the proportion of staphylococci.

Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era.

Patients with Eisenmenger syndrome (ES) have a higher mortality rate than patients with simple congenital heart disease (CHD). To determine factors associated with death in the era of advanced pulmonary vasodilator treatment, we analyzed the characteristics of adult ES patients depending on underlying CHD. Simple septal defects and patent ductus arteriosus were classified as simple CHD, and other conditions were classified as complex CHD. Sixty-seven adult ES patients (50.7% women) were reviewed retrospectively. CHD was diagnosed at a median of 10.0 years of age and ES was diagnosed at 18.6 years. Thirteen patients (19.4%) died; the median age was 38.6 years (IQR 32.2-47.8). In a multivariate analysis, patients with SpO2 < 85% had a higher mortality rate than others [hazard ratio (HR) 9.7; 95% confidence interval (CI) 1.002-95.2, p = 0.05]. In simple CHD patients, those with a low platelet count (< 100 × 109/L) or low SpO2 (< 85%) were at a higher risk of death than those without (HR 16.32, 95% CI 1.25-2266.31, p = 0.032; and HR 38.91, 95% CI 3.44-5219.41, p = 0.001, respectively). Advanced pulmonary vasodilators were used more in survivors than in non-survivors (48.1% vs. 15.4%, p = 0.032). Low SpO2 and platelet count were related to mortality in adult ES, especially in those with simple CHD. Therefore, careful attention should be paid to the care of adult ES patients with this tendency; active pulmonary vasodilator treatment should be considered.

The outcome of percutaneous stent implantation in congenital heart disease: experience of a single institute.

PURPOSE: The efficacy of percutaneous stent implantation for congenital heart disease (CHD) in Korea, where stent availability is limited, has not been determined. This study evaluated the acute and midterm results of stent implantation in different CHD subgroups. METHODS: Stents were implanted in 75 patients with 81 lesions: (1) pulmonary artery stenosis (PAS) group, 56 lesions in 51 patients; (2) coarctation of the aorta (CoA) group, 5 lesions in 5 patients; (3) Fontan group, 13 lesions in 12 patients; (4) ductal stent group, 3 lesions in 3 patients; and (5) other CHD group, 4 lesions in 4 patients. Mean follow-up duration was 2.1 years (0.1-4 years). Medical records were reviewed retrospectively. RESULTS: The minimum lumen diameter (MLD) in PAS and CoA increased from 5.0±1.9 mm and 8.4±1.6 mm to 10.1±3.6 mm and 12.3±2.5 mm, respectively (P<0.01). In the PAS group, pressure gradient decreased from 25.7±15.6 mmHg to 10.4±10.1 mmHg, and right ventricular to aortic pressure ratio from 0.56±0.21 to 0.46±0.19. In the CoA group, the pressure gradient decreased from 50±33 mmHg to 17±8 mmHg. In the ductal stent group, the MLD of the ductus increased from 2.3 mm to 4.3 mm and arterial oxygen saturation from 40%-70% to 90%. No deaths were associated with stent implantation. Stent migration occurred in 3 patients, but repositioning was successful in all. Stent redilation was performed successfully in 26 cases after 29±12 months. CONCLUSION: Percutaneous stent implantation was safe and effective, with acceptable short and mid-term outcomes in Korean CHD patients.

Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm).

BACKGROUND AND OBJECTIVES: Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. SUBJECTS AND METHODS: The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. RESULTS: The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. CONCLUSIONS: Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms.

The mental health and quality of life of adult patients with congenital heart disease.

BACKGROUND: The quality of life (QoL) of adults with congenital heart disease (CHD) has gained significant interest. In addition to medical problems, many patients with CHD face psychosocial, educational, and behavioral challenges. However, few studies have examined the relationship between disease severity and QoL in adults with CHD. METHODS: Eighty-five patients (50 men, 35 women) aged 20-52 years (median, 26.5 years) were enrolled. Patients underwent a QoL, Beck Depression Inventory (BDI), and Beck Anxiety Inventory (BAI) survey. The scores were compared with those of age- and gender-matched population data according to the degree of underlying CHD. Disease severity was classified in relation to initial diagnosis, illness course, and current functional status (New York Heart Association [NYHA] class, ability index, CHD functional index, ventricular ejection fraction, and peak VO2). RESULTS: There was no significant correlation between disease severity and current functional status assessed by BDI and BAI. Patients who stated that they were religious had better scores for resilience (p=0.031), physical QoL (p=0.008), and environmental QoL (p=0.025). Environmental QoL scores were higher in patients who fully understood their disease (p=0.004). Current NYHA functional class was associated with scores for psychological resilience. CONCLUSIONS: CHD severity had a detrimental impact on resilience only when measured in terms of poor functional status. The initial diagnosis and course of the illness influence QoL and perceived health. Good psychosocial adaptation could be the result of close family relationships and involvement, making mental adjustment easier.

Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea.

PURPOSE: The use of implantable cardioverter defibrillators (ICDs) to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. METHODS: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. RESULTS: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5±5.4 years (range, 2 to 22 years). The follow-up duration was 28.9±20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1), cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years). Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia. During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. CONCLUSION: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

Aortic valve replacement surgery for a case of infantile Takayasu arteritis.

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.