Gi Beom Kim

Pulmonary hypertension in preterm infants with bronchopulmonary dysplasia.

Background and Objectives With the increasing survival of preterm infants, pulmonary hypertension (PH) related to bronchopulmonary dysplasia (BPD) has become an important complication. The aim of this study was to investigate the characteristics and outcome of PH in preterm infants with BPD and to identify the risk factors for PH. Subjects and Methods We reviewed the records of 116 preterm infants with BPD cared for at a single tertiary center between 2004 and 2008. Results Twenty-nine (25%) infants had PH >2 months after birth. PH occurred initially at a median age of 65 days (range, 7-232 days). Severe BPD, a birth weight <800 g, long-term ventilator care and oxygen supplementation, a high ventilator setting, infection, and a patent ductus arteriosus (PDA) were related to PH based on univariate analysis (p<0.05). The infants who had longer oxygen supplementation were significantly more likely to have PH (odds ratio, 18.5; 95% confidence interval, 4.1-84.6; p<0.001). PH was improved in 76% of infants after a median of 85 days (range, 20-765 days). Four infants (14%) died. The death of 3 infants was attributed to PH. Conclusion BPD was frequently complicated by PH. Although PH resolved in the majority of infants, PH in preterm infants with BPD can be fatal. Regular screening for PH and adequate management are required.

Epidemiologic features of Kawasaki disease in South Korea: data from nationwide survey, 2009-2011.

Background: To assess the recent epidemiologic features of Kawasaki disease (KD) in South Korea from the nationwide survey conducted between 2009 and 2011. Methods: We collected data regarding the incidence, symptoms, treatment and coronary complications associated with acute KD by sending questionnaires to the 100 hospitals that have pediatric residency programs from 2009 to 2011. Results: We received complete responses from 73 hospitals and partial responses from 14 hospitals. A total of 13,031 patients of KD were reported from the 87 hospitals (3941 in 2009, 4635 in 2010 and 4455 in 2011). The male to female ratio was 1.44:1, and the median age at diagnosis was 28 months. From the questionnaires with complete responses, we noted that the incidence of KD per 100,000 children <5 years of age was 115.4 in 2009, 132.9 in 2010 and 134.4 in 2011 (average rate, 127.7). KD occurred more frequently during summer (June, July and August) and during winter (December and January). The recurrence rate was 3.83%. The standard dose of intravenous immunoglobulin was administered to 93.6% of the patients, and nonresponder rate was 11.6%. Coronary aneurysm occurred in 1.9% of the patients and giant aneurysm developed in 26 patients (0.26%) over 3 years, and 2 patients had myocardial infarction. No mortality was reported. Conclusions: The average annual incidence of KD in South Korea has continuously increased to 134.4 per 100,000 children <5 years of age in 2011, which is the second highest incidence of KD worldwide, following its incidence in Japan.

Clinical features of congenital portosystemic shunt in children

Clinical features, images, complications, treatments, and prognosis of 10 children with congenital portosystemic shunt (CPSS) were reviewed. Nine children were diagnosed with intrahepatic shunts while one presented with extrahepatic shunt. CPSS was detected by prenatal ultrasonography in four infants. Three infants presented with galactosemia without an enzyme deficiency. Two children presented with mental retardation and attention deficit hyperactivity disorder. Pulmonary hypertension developed in two patients. Spontaneous closure occurred in four infants with intrahepatic shunts including patent ductus venosus. The shunts were closed using transcatheter embolizations in four patients with intrahepatic shunts. Conclusion: Intrahepatic shunts may close spontaneously. Transcatheter embolization is effective for the treatment of symptomatic intrahepatic shunts.

Pulmonary hypertension in infants with bronchopulmonary dysplasia.

An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type natriuretic peptide (BNP) or N-terminal-proBNP must be conducted in the BPD patients with greater than moderate degree to prevent PH and to ensure early treatment if PH is present. In the BPD patients with significant PH, multi-modality treatment, including treatment for correcting an underlying disease, oxygen supply, use of diverse selective pulmonary vasodilators (inhaled nitric oxide, inhaled prostacyclins, sildenafil, and endothelin-receptor antagonist) and other methods, is mandatory.

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.

Intra-atrial reentrant tachycardia in adult patients after Fontan operation

BACKGROUND Atrial tachyarrhythmia is a major late complication in adult Fontan patients. This study examined the clinical features and risk factors of late intra-atrial reentrant tachyarrhythmia (IART) in adult patients after Fontan surgery and the mid-term outcome of Fontan conversion with or without antiarrhythmic surgery in these patients. METHODS We conducted a retrospective study on adult patients who were born before 1994 and survived at least 3 months after a Fontan operation at Seoul National University Childrens Hospital. RESULTS We followed 160 patients over 20.9 ± 4.1 years. Sustained atrial tachycardia was identified in 51 patients, and IART was found in 41, appearing a mean 13.6 years after surgery. By the 25 year follow-up, 40% had developed IART. The incidence of IART significantly increased over time. Patients with an atriopulmonary connection (APC) (n=65) had significantly longer follow-up duration and higher incidence of IART than patients with a lateral tunnel (n=86) or extracardiac conduit Fontan (n=9). On multivariate analysis, APC, sinus node dysfunction, and nonsustained atrial tachycardia were found to be significantly associated with IART. Twenty-four patients with IART underwent Fontan conversion. Over the follow-up period, IART severity scores in the 22 patients who survived after Fontan conversion decreased significantly, and New York Heart Association functional class significantly improved. On multivariate analysis, protein losing enteropathy and ventricular dysfunction were found to be significant risk factors for mortality. CONCLUSIONS IART was common in adult Fontan patients, and Fontan conversion with or without antiarrhythmic surgery and pacemaker placement helped to control it.

Rehabilitation of pulmonary artery in congenital unilateral absence of intrapericardial pulmonary artery.

OBJECTIVE We evaluated the efficacy of the early rehabilitation of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery and the factors affecting pulmonary artery growth. METHODS We retrospectively reviewed the medical records and imaging modalities of 15 patients with unilateral absent intrapericardial pulmonary artery (7 left and 8 right; median age at diagnosis, 5 months) from 1991 to 2008. RESULTS The remnant pulmonary artery was found in 12 patients (mean diameter, 2.6 ± 0.7 mm) at the hilum. Eleven patients underwent operation (main pulmonary artery flap angioplasty in 5 patients; tube graft interposition in 6 patients), and 4 patients were inoperable. Transcatheter balloon angioplasty or stent implantation was required for the remaining pulmonary artery stenosis in 6 patients (55%). The last ipsilateral lung perfusion proportion at lung perfusion scan was 39% (range, 15%-51%), and the Z value of the last ipsilateral pulmonary artery diameter was -0.5 (range, -4.2 to 2). The patients with a smaller initial remnant pulmonary artery required more interventions (P = .003). The final perfusion proportion of affected lung was higher in the patients treated early (≤6 months, n = 7) than in those treated late (>6 months, n = 4) (41.9% ± 8.5% vs 24.9% ± 10.7%, respectively, P = .024). The patients with graft interposition showed a lower perfusion proportion of affected lung than those with main pulmonary artery flap angioplasty (P = .017). CONCLUSIONS In patients with unilateral absent intrapericardial pulmonary artery, early and aggressive management of combined surgical reconstruction and transcatheter intervention improved pulmonary artery growth and lung perfusion.

Cardiac Dysfunction After Renal Transplantation; Incomplete Resolution in Pediatric Population

Background. Long-term data of cardiac function after renal transplantation (RT) are limited, especially in children. Thus, we evaluated the status of left ventricular hypertrophy and various indices of left ventricular (LV) function in pediatric RT patients. Methods. Blood pressure, serum biochemical profiles, electrocardiogram, and echocardiogram of 32 pediatric patients (mean age, 15.5±4.4 years) who underwent RT 5.1±2.5 years before and 29 body surface area-matched control subjects were studied. Results. Repolarization abnormalities shown on electrocardiogram of pre-RT patients improved significantly after RT (QTc dispersion 50.8±37.3 to 37.4±11.9 msec, P=0.009). Left ventricular hypertrophy with increased LV mass index of pre-RT patients regressed remarkably after RT (LV mass index 120.9±40.5 to 69.2±14.5 g/m2, P<0.001); still, LV mass was significantly higher in RT patients than the controls (54.0±9.6 g/m2, P<0.001). Compared with the controls, the RT patients showed diastolic dysfunction (lower E/A ratio and higher isovolumic relaxation time) and lower myocardial performance (higher LV Tei index and weaker strain pattern). Patients who had shorter duration of non-RT renal replacement therapy showed better LV function (lower LV Tei index and stronger strain pattern) in the long-term follow-up. Conclusions. Because cardiac dysfunction did not resolve after RT in pediatric population, regular evaluation for cardiovascular function after RT is required. Early RT may also be beneficial to global LV performance after RT.

Epidemiology and Clinical Features of Kawasaki Disease in South Korea, 2012–2014

Background: To reveal the recent epidemiologic features of Kawasaki disease (KD) in South Korea based on data from a nationwide survey. Methods: We collected data between 2012 and 2014 regarding the incidence, symptoms and signs, treatment trends and coronary complications associated with acute KD by sending questionnaires to 97 hospitals with pediatric residency programs as well as 19 community hospitals without residency training. Results: We received full and partial data from 97 and 13 hospitals, respectively (response rate: 94.8%). A total of 14,916 cases of KD were reported by these 110 hospitals (4588 in 2012, 5183 in 2013 and 5145 in 2014). The male-to-female ratio was 1.4:1, and the median age at diagnosis was 29 months. The incidence of KD per 100,000 children younger than 5 years of age were 170.9, 194.9 and 194.7 in 2012, 2013 and 2014, respectively. The recurrence rate was 4.7%. KD occurred more frequently during summer (especially June and July) and winter (December and January) seasons. Intravenous immunoglobulin was administered to 95.4% of the patients, and the nonresponder rate for the first intravenous immunoglobulin was 11.8%. Coronary aneurysm occurred in 1.7% of the patients, and giant aneurysm developed in 19 patients (0.16%) during the 3 years. One patient had myocardial infarction and 1 patient died of suspected coronary aneurysm rupture. Conclusions: The incidence of KD in South Korea increased to 194.7 per 100,000 children younger than 5 years in 2014; meanwhile, the coronary aneurysm rate decreased to 1.7%.

Clinical Course of Vascular Rings and Risk Factors Associated With Mortality

Background and Objectives Vascular rings refer to anomalies of the great arteries that cause respiratory or feeding problems. The purpose of this study was to analyze a series of patients with vascular rings and evaluate associated risk factors for mortality. Subjects and Methods A retrospective review of all patients identified with vascular rings between 1997 and 2010 in the Seoul National University Childrens Hospital. Results Thirty-five patients were diagnosed with vascular rings (median age at diagnosis, 7 months). The vascular rings of 32 patients were confirmed by cardiac computed tomography or magnetic resonance imaging. The types of vascular rings were: a double aortic arch in ten patients, a right aortic arch with persistent left ligamentum arteriosum in seven, an aberrant subclavian artery in seven, a pulmonary sling in eight, and others types in three patients. Eleven patients were asymptomatic. Gastrointestinal and respiratory symptoms were seen in ten and sixteen patients, respectively. Associated cardiovascular anomalies were present in fifteen patients. Twenty patients with definite symptoms underwent surgical treatment. The median interval between diagnosis and operation was 6 days. Four patients eventually died; three deaths were associated with complex heart diseases, and one had pulmonary artery sling with severe tracheal stenosis. Only the presence of a complex heart disease significantly influenced mortality (p=0.002). Conclusion Vascular rings include several types of anomalies, each with a different prognosis and symptoms. The presence of a complex heart disease was significantly associated with mortality. Early diagnosis and timely surgery in symptomatic patients are essential.