Changes in Coronary Perfusion after Occlusion of Coronary Arteries in Kawasaki Disease

PURPOSE: Myocardial infarction in children with total occlusion of a coronary artery after Kawasaki disease is rare due to multiple collateral vessels. We aimed to investigate the changes in coronary perfusion associated with coronary artery occlusion after Kawasaki disease. MATERIALS AND METHODS: Eleven patients with coronary artery occlusion after Kawasaki disease were investigated. Serial coronary angiographies after total occlusion of a coronary artery were reviewed and the changes were described in all patients with additive information collected. RESULTS: The median age at the occlusion was 5.9 years old. The interval to occlusion was 6.2+/-6.9 years. Four left anterior descending coronary artery total occlusions and 10 right coronary artery total occlusions were detected. Immediate coronary artery bypass graft for left anterior descending coronary artery total occlusion made right coronary total occlusion occurred in all except one patient and the intervals thereof were 1 year, 1.8 years, and 4 years. Collaterals to the left coronary artery regressed after recanalization, while new collaterals to the right coronary artery developed. In three, collaterals to the right coronary artery decreased without recanalization without clinical signs. CONCLUSION: The right coronary artery should be followed up carefully because of possible occlusion of new onset or changes in collaterals.

Amiodarone and Catheter Ablation as Cardiac Resynchronization Therapy for Children with Dilated Cardiomyopathy and Wolff-Parkinson-White Syndrome

Preexcitation by accessory pathways (APs) is known to cause dyssynchrony of the ventricle, related to ventricular dysfunction. Correction of ventricular dyssynchrony can improve heart failure in cases of dilated cardiomyopathy (DCMP) with preexcitation. Here, we report the first case of a child with DCMP and Wolff-Parkinson-White (WPW) syndrome treated with amiodarone and radiofrequency catheter ablation (RFCA) in Korea. A 7-year-old boy, who suffered from DCMP and WPW syndrome, showed improved left ventricular function and clinical functional class after treatment with amiodarone to eliminate preexcitation. QRS duration and left ventricular ejection fraction (LVEF) were inversely correlated with amiodarone dosage. After confirming the reduction of preexcitation effects in DCMP, successful RFCA of the right anterior AP resulted in LVEF improvement, along with the disappearance of preexcitation. Our findings suggest that ventricular dyssynchrony, caused by preexcitation in DCMP with WPW syndrome, can worsen ventricular function and amiodarone, as well as RFCA, which should be considered as a treatment option, even in young children.

Pulmonary Hypertension Secondary to Bronchopulmonary Dysplasia in Very Low Birth Weight Infants (<1,500 g)

PURPOSE: Although infants with bronchopulmonary dysplasia (BPD) are at risk of developing secondary pulmonary hypertension (PH), which is associated with significant morbidity and mortality, little has been reported about the incidence, clinical course and prognosis of PH secondary to BPD in premature infants. This study was done to investigate the incidence, risk factors, clinical course, and the ultimate prognosis of PH developed secondary to BPD in very low birth weight infants ( or =3 m/s and a flattening of the intraventricular septum by conducting Doppler echocardiography. RESULTS: The incidence of pulmonary hypertension was 6% in VLBWI with BPD and it developed in moderate to severe BPD. The diagnosis of pulmonary hypertension was made on postnatal 133 days (range 40-224 days) and the risk factors related to developing pulmonary hypertension were severe BPD, small for gestational age and outborn infants. The mortality rate was 57% and especially higher in severe BPD (70%). The time to recovery spent 3 months (range 1-10 months) in survived patients. CONCLUSION: Based on the results of this research, pulmonary hypertension secondary to BPD in VLBWI related to severity of BPD and had a poor prognosis. We expect that regular long-term echocardiography may be helpful in treating reversible in VLBWI with moderate to severe BPD.

Dual Pulsed-Wave Doppler Tracing of Right Ventricular Inflow and Outflow: Single Cardiac Cycle Right Ventricular Tei Index and Evaluation of Right Ventricular Function

BACKGROUND AND OBJECTIVES: The reliability and usefulness of the right ventricular (RV) Tei index (RTX) remains controversial because it has not been possible to simultaneously measure RV inflow and outflow. However, dual pulsed-wave Doppler (DPD) enables flow velocities to be obtained at different sampling sites simultaneously. In this study we evaluated the feasibility and reliability of RTX values obtained by DPD (RTX(DPD)). SUBJECTS AND METHODS: Forty-one patients who underwent cardiac catheterization and echocardiography for RV volume or pressure overloading conditions were evaluated. Symptom-limited exercise treadmill testing with expired gas analysis was performed and maximal exercise capacity was measured. RESULTS: RTX by conventional flow Doppler (RTX(CFD), 0.262+/-0.164) was similar to RTX(DPD) (0.253+/-0.117, p=NS), whereas RTX by tissue Doppler echocardiography (RTX(TDE), 0.447+/-0.125) was significantly larger than RTX(DPD) (p<0.001). Based on multiple regression analysis, maximal exercise capacity was independently related to RTX(DPD) (beta=-0.60, p<0.001), mid-RV dimension (beta=-0.26, p=0.012), left ventricular ejection fraction (beta=0.22, p=0.023), and early diastolic tricuspid annular velocity (beta=0.21, p=0.048). CONCLUSION: It is feasible and reliable to evaluate RV function using RTX(DPD) values. However, to evaluate the clinical usefulness of RTX(DPD), additional studies are required with a large number of patients and long-term follow-up.

Surgical outcome of severe pulmonary arterial hypertension secondary to left-to-right shunt lesions / 소아과

PURPOSE: Despite recent advances in pulmonary hypertension management and surgery, appropriate guidelines remain to be developed for operability in congenital heart disease with pulmonary artery hypertension (PAH). Our aim was to evaluate clinical outcomes of patients with severe PAH who underwent surgical closure of left-to-right shunt lesions (LRSL) on the basis of pulmonary reactivity. METHODS: We retrospectively reviewed 21 patients who underwent surgical closure of LRSL with severe PAH (> or =8 Wood unit) from January 1995 to April 2009. The median age at operation was 26 years. Atrial septal defect, ventricular septal defect (VSD), VSD and patent ductus arteriosus (PDA), and PDA was present in 11, 4, 4, and 2 patients, respectively. RESULTS: Operability was based on vasoreactivity of PAH. Of the 21 patients, 5 showed response to pulmonary vasodilator therapy and 8 showed vasoreactivity after balloon occlusion of defects. The remaining 8 patients were considered operable because of significant left-to-right shunt (Qp/Qs > or =1.5). Five patients underwent total closure of defects and 16 were left with small residual shunts. The median follow-up duration was 32 months. There was no significant postoperative mortality or morbidity. Systolic pulmonary artery pressure (PAP) decreased in all but 2 patients. All patients except 1 showed improvement of New York Heart Association functional class. CONCLUSION: Closure of LRSL in patients with severe PAH on the basis of pulmonary vasoreactivity seems reasonable. PAP and clinical symptoms improved in most patients. Further research is needed for the evaluation of long-term results.

A Case of Pulmonary Sequestration and Congenital Lobar Emphysema Presenting with Congestive Heart Failure and Pulmonary Hypertension / 소아알레르기및호흡기학회지

Congenital cystic disease of the lung represents pulmonary sequestration, congenital lobar emphysema, bronchogenic cyst and congenital cystic adenomatoid malformation. It has a variety of clinical manifestations from immediate postnatal respiratory distress to heart failure. Pulmonary sequestration sometimes causes heart failure in neonates through a shunt between an anomalous systemic feeding artery and the pulmonary venous system. We hereby report a case with both pulmonary sequestration and congenital lobar emphysema presenting with congestive heart failure and pulmonary hypertension, which improved after lobectomy.

Clinical course and prognosis of hemodynamically significant congenital heart defects in very low birth weight infants / 소아과

PURPOSE: This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). METHODS: Medical records of 1,098 VLBWI with birth weight or =Gr III), and periventricular leukomalacia. Cardiac surgery was performed on 13 patients (39%). Nine patients received staged operations, and 10 patients received early intervention. The overall mortality in patients who had CHD was higher than in the patients who did not have CHD (27% vs. 16%). In patients with CHD, congenital abnormalities or chromosomal disorders were more important factors for increased mortality (86% vs. 11%) than the degree of complexity of CHD (19% vs. 42%). CONCLUSION: The most important prognostic factors of VLBWI with CHD are the associated congenital abnormalities or chromosomal disorders.

Analysis of Clinical Course and the Prognosis of Pulmonary Hypertension in Infants with Bronchopulmonary Dysplasia: A Single Center Study / 소아알레르기및호흡기학회지

PURPOSE: The aim of our study was to determine the clinical course and the prognosis of pulmonary hypertension (PHT) with bronchopulmonary dysplasia (BPD). METHODS: Two hundred forty infants who were admitted to the neonatal intensive care unit (NICU) of Samsung Medical Center from January 2002 to December 2006 and were diagnosed with BPD were enrolled in this study. We investigated their medical records retrospectively to investigate any difference between BPD with PHT group and BPD without PHT group in clinical characteristics, mortality and morbidity. RESULTS: Nineteen (7.9%) of the 240 patients with BPD developed PHT. The ratio of females to males was 2.8:1. The severity of BPD, the small birth weight for gestational age, the duration of mechanical ventilation and the maximal peak inspiratory pressure were significantly associated with the development of PHT. (P=0.000, P=0.007 and P=0.000, respectively) The mortality was higher in the BPD with PHT group than in the BPD without PHT group. (P= 0.000) BPD with PHT group required longer duration of oxygen therapy and had more rehospitalization for respiratory illness than the others. (P=0.014) More patients were admitted to the pediatric intensive care unit and received mechanical ventilation therapy in the BPD with PHT group. (P=0.001 and P=0.020, respectively) CONCLUSIONS: PHT is one of the causes associated with high mortality and morbidity in BPD patients. Therefore, the physician should be alert to the development of PHT in premature infants with severe BPD, especially if the baby is female, less than 500 g of birth weight, small for gestational age, or supported by mechanical ventilation for a prolonged duration

A Case of Congenital Extra Hepatic Portocaval Shunt(Abernethy Malformation Type 2) with a very LargeLiver Mass and an Atrial Septal Defect / 대한소아소화기영양학회지

Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.

Epidemiologic study of Kawasaki disease in 6 months old and younger infants / 소아과

PURPOSE: The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants 6 months of age. RESULTS: A total of 1,739 patients 6 months old with data from 1,739 KD patients < or =6 months old showed significantly higher incidences of CA abnormalities and CA aneurysms in the younger patients.

Outcomes of Pregnancy in Women with Congenital Heart Disease: A Single Center Experience in Korea

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 > or =2 in 2.0%, and cardiac death in 2.0%. Independent predictors of adverse maternal cardiac events were an NYHA functional class of > or =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 > or =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.

Outcome Following Surgical Closure of Patent Ductus Arteriosus in Very Low Birth Weight Infants in Neonatal Intensive Care Unit

PURPOSE: The aims of this study were to determine the factors affecting the outcome of patent ductus arteriosus ligation in very low birth weight infants (VLBWI) and demonstrate the safety of PDA ligation in VLBWI performed in the neonatal intensive care unit (NICU). MATERIALS AND METHODS: From October 1994 to July 2006, medical records of 94 VLBWI weighing < 1,500g who underwent PDA ligation in the NICU of Samsung Medical Center were reviewed retrospectively. Factors affecting the final outcome of PDA ligation were evaluated by dividing the infants into 3 groups according to mortality and major morbidities as follows: mortality group (Mo), major morbidity group (Mb), and no major morbidity group (NM). RESULTS: In the Mo group, birth weight was significantly lower and the preoperative mean FiO(2) and mean dopamine dose were significantly higher than those in the other 2 groups. There was no significant difference in gestational age, incidence of RDS, number of courses of indomethacin, surgery-related factors, including weight and age at surgery, perioperative vital signs, and complications after surgery between the 3 groups. During surgery in the NICU, there were no significant hemodynamic instability or serious acute complications. CONCLUSION: The factors affecting the outcome of surgery in VLBWI are not the factors related to surgery but the preoperative conditions related to the underlying prematurity. PDA ligation of VLBWI performed in the NICU is safe without serious complications.

The Short Term Growth of Neo-aorta and its Implication to Aortic Insufficiency after Arterial Switch Operation in Complete Transposition of Great Arteries

PURPOSE: This study was aimed to assess the growth of neo-aorta and its implication to neo-aortic insufficiency in children with complete transposition of great arteries (TGA) after arterial switch operation (ASO). METHOD: We retrospectively reviewed medical records, echocardiograms and cardiac catheterization of 40 patients who had underwent ASO at Samsung Medical Center from 1995 through 2001. Pulmonary artery and aorta were evaluated regarding its stenosis and regurgitation, respectively. The growth of the neo-aortic valve, sinus of neo-aortic Valsalva, and the site of aortic anastomosis were evaluated by measuring the change of the diameter. RESULT: The mean duration of follow-up after ASO was 17.2+/-12.4 months (range 1.2-67 months). Aortic insufficiency (AI) developed in 45%, in which all were mild. The neo-aortic annulus (originally pulmonary annulus) had grown as normal pulmonic valve does do (diameter of pulmonary valve annulus preoperatively, 8.9+/-1.22 mm; postoperatively at more than 6 months, 12.8+/-2.2mm). The anastomotic site of neo-aorta showed a growth curve equivalent to that of sinotubular junction of normal aorta (preoperative diameter, 7.7+/-1.4 mm; postoperatively at more than 6months, 12.7+/-3.1mm). However, the growth rate of sinus of Valsalva showed a extremely higher compared to that of normal aorta (preoperative diameter, 10.5+/-1.2 mm; postoperatively at more than 6 months, 18.8+/-2.6 mm). No significant relations could be revealed between the change of dimension of aortic root and aortic insufficiency. CONCLUSION: Aortic insufficiency was not uncommon but mild aortic dilatation was not significantly associated with AI. Although the short term result is encouraging, long-term surveillance for aortic root dilatation and aortic insufficiency remains necessary.

Spontaneous Closure of Iatrogenic Coronary Artery Fistula to Left Ventricle After Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy

Cases of iatrogenic coronary artery fistulas draining into the left ventricle after surgical myectomy for hypertrophic obstructive cardiomyopathy have been published as sporadic reports. However, its management scheme and prognosis are not clear because of the low incidence. A 46-yr-old woman was hospitalized for evaluation of chest pain and shortness of breath for 3 months. Transthoracic echocardiographic examination showed typical hypertrophic obstructive cardiomyopathy with a peak pressure gradient of 71 mmHg across the left ventricular outflow tract. The patient underwent surgical septal myectomy. Postoperative color Doppler imaging revealed a diastolic blood flow from the interventricular septal myocardium to the left ventricular cavity, i.e. iatrogenic coronary artery fistula to the left ventricle. Ten days later, the fistula closed spontaneously which was diagnosed by transthoracic echocardiography and confirmed by coronary angiography.

A Case of Axenfeld-Rieger Syndrome with Severe Mitral Regurgitation

Axenfeld-Rieger syndrome is a rare autosomal dominant inherited developmental disorder characterized by ocular and systemic abnormalities. In ocular anomaly, it includes a prominent and anteriorly displaced Schwalbe line and an iridocorneal synechiae, iris hypoplasia, corectopia and secondary glaucoma. Extraocular developmental abnormalities of the syndrome are hearing loss, congenital heart disease, dental anomalies, developmental delay, and a characteristic facial appearance. We herein reported a familial case of Axenfeld-Rieger syndrome that had a mitral valve prolapse with severe mitral regurgitation finally requiring mitral valve replacement.

Proximal Interruption of Right Pulmonary Artery with a Remnant of Patent Ductus Arteriosus

Proximal interruption of unilateral pulmonary artery is an uncommon developmental anomaly. Patients without associated cardiac defects may remain asymptomatic but many develop recurrent pulmonary infections, hemoptysis, pneumonitis, bronchiectasis or pulmonary hypertension. We herein reported two cases of interruption of right pulmonary artery which were diagnosed beyond infancy. A remnant of patent ductus arteriosus was revealed on an angiogram in both cases. Surgical intervention could not be considered due to severe hypoplastic right pulmonary artery. Early surgical intervention should be considered to rescue a pulmonary circulation and prevent secondary lung hypoplasia.

The Relationship Between the Serum Level of Cytokines (Interleukin-2, Interleukin-6, Interleukin-10, Interferon-gamma) and the Erythematous Induration of BCG Site in the Children with Kawasaki Disease

PURPOSE: This study was aimed to investigate the clinical findings and the changes of serum levels of inflammatory cytokines according to the presence of erythematous induration at BCG (Bacillus Calmette-Guerin) site in the children with Kawasaki disease. METHODS: Thirty-one patients with Kawasaki disease were classified into a group with erythematous induration at BCG site (BCG (+), n=14, M: F=11: 3) and a group without skin changes at BCG site (BCG (-), n=17, M: F=12: 5). Levels of various laboratory parameters and cytokines including interleukin-2 (IL-2), interleukin-6 (IL-6), interleukin-10 (IL-10) and interferon-gamma (IFN-gamma) were determined four times on admission day, 3-4 days, 2 weeks and 8 weeks after intravenous immunoglobuin injection. RESULTS: The age of onset in BCG (+) group was significantly younger than BCG (-) group (15+/-8 months vs. 31+/-16 months, P<0.05). There were no significant differences in other clinical findings and laboratory parameters except elevated ESR in BCG (-) group. There was no difference in complications of coronary arteries and cardiac function between two groups. The serum levels of IL-2, IL-6 and IL-10 were increased in all patients at the diagnosis, but there were no significant differences between BCG (-) and BCG (-) groups. The serial changes of cytokines did not show any significant differences. CONCLUSION: In Kawasaki disease, BCG site injection seems to develop in younger age and not to be related to the differences of immunologic inflammatory response.

Transcatheter Closure of Fenestration with Detachable Coils After the Fontan Operation

We report our experience in 13 patients who underwent transcatheter closure of Fontan fenestration with the Cook(R) detachable coils. These patients underwent the extracardiac type Fontan operation with a short conduit fenestration (n=7) or lateral tunnel type with a punch-hole fenestration (n=6). Fenestration closure was done at the mean age of 5.1+/-2.4 yr, average of 32 months after the Fontan operation. We used one to three coils depending on the fenestration type, size, and residual shunt. Aortic oxygen saturations increased by an average of 5.4 (2-9)% and mean pressures in the Fontan circuit increased by an average of 2.1 (0-6) mmHg. During fol-low-up (median of 23 months), five patients (4 in extracardiac, 1 in lateral tunnel) had complete occlusion of the fenestration on echocardiography. There was no immediate or late complication. Transcatheter closure of fenestration in Fontan operation using the Cook(R) detachable coil is a safe and feasible technique. However, the coil was ineffective for closure of a punch-hole fenestration in the lateral tunnel type operation. In the conduit type fenestration, some modification of fenestration method instead of a short conduit for coil closure or use of new device is necessary to increase complete closure rate.

Development and Assessment of Clinical Pathway for Simple Congenital Heart Surgery

PURPOSE: To standardize hospital management of patients with simple congenital heart disease (CHD), we developed a protocol called the 'clinical pathway (CP)'and evaluated quality improvement in patient outcomes. METHODS: The study included a group of 60 patients with simple CHD who had surgery between June 1 and October 31, 1998. The control group included 48 patients who had surgery for the same disease during the corresponding time in 1997. Two CPs were developed according to state and place of residence of patients. Information on patients was reviewed including: education, physical examination, hospital stay, type of care and date of discharge from the hospital. The effect of each protocol on standard procedures was reviewed. After patient discharge additional performance information was assessed including: procedures used, duration of hospital stay, medical cost, treatment outcome, complications and parent satisfaction. RESULTS: The duration of hospital stay prior to surgery (1.8 days vs. 3.0 days), the ICU stay (1.3 days vs. 3.6 days), number of ward days after surgery (5.3 days vs. 7.9 days) and total number of hospital days (8.5 days vs. 14.7 days) were significantly shortened in the study group compared to the control group. The intubation time (P=0.000) and the frequency of laboratory studies (P=0.000) during the hospitalization decreased in the study group compared to the control group. The average medical costs of cases was also significantly lower in the study group (P=0.001). There were no deaths, readmissions or serious complications in the study group. A questionnaire survey of patient family members showed that they were satisfied with care; with more than 85 percent response rate to questionnaire items. CONCLUSION: Application of our new CP protocol shortens hospital stay and reduces medical costs while improving the quality of care for patients and their families. This CP protocol should now be considered for more complex CHD.

Assessment of Systemic Right Ventricular Function using Strain/Strain Rate in Congenitally Corrected Transposition of the Great Arteries

PURPOSE: In patients with the congenitally corrected transposition of great arteries (CCTGA), systemic ventricle tends to be hypertrophic due to longstanding systemic high pressure that may lead to dysfunction of ventricle. The aim of this study was to assess the global and segmental wall function of systemic right ventricle in CCTGA. METHODS: Twelve patients with the CCTGA patients were enrolled. Ejection fraction of systemic right ventricle, myocardial performance index (MPI), strain and strain rate were assessed. RESULTS: The median age was 32.5 years with a range of 4 to 66 years. Ejection fraction (EF) using M-mode and Simpsons method was significantly lower in the CCTGA patients (48.5+/-4.6% and 51.2+/-4.1%, respectively, vs 63.7+/-3.6% and 66.6+/-3.8% respectively). 5 patients had diastolic dysfunction (3/12; pseudo-normalization pattern, 2/12; relaxation abnormality). Strain and strain rate revealed segmental systemic right ventricle dysfunction, and were lower in the CCTGA patients at mid and apical segment of septum and apical segment of anterior wall. The extent of dysfunctional segment was related to the age and MPI. CONCLUSION: There was global dysfunction in the CCTGA patients and it was related with age. Segmental dysfunction was especially shown at mid and apical segment of septum and apical segment of anterior wall and more frequently at apical segment of each wall. The extent of dysfunctional segment was related with age.