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ABSTRACT: 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.
Korean Circulation Journal 01/2013; 43(1):57-61.
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ABSTRACT: The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean ± SD, 34.9 ± 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 ± 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 ± 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 ± 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 ± 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 ± 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.
Heart Surgery Forum 04/2012; 15(2):E111-5. · 0.63 Impact Factor
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ABSTRACT: Recently, a revised Ghent nosology has been established for the diagnosis of Marfan syndrome (MFS) that puts more weight on the aortic root aneurysm and ectopia lentis. We compared the application of the Ghent and revised Ghent nosologies in adult Korean patients for whom there is suspicion of MFS. From January 1995 to June 2010, we enrolled 106 patients older than 20 years for whom there was suspicion of MFS, and who had undergone genetic analysis of the fibrillin-1 gene (FBN1). Of 106 patients, 86 patients (81%) fulfilled the criteria of the Ghent nosology, and 84 patients (79%) met the criteria of the revised Ghent nosology. The two patients who met the Ghent nosology criteria, but not the criteria of the revised Ghent nosology were diagnosed with Loeys-Dietz syndrome and MASS phenotype. The level of agreement between both nosologies was very high (κ = 0.94, 95% confidence interval: 0.86 to 1.0). Marfan-like syndromes were diagnosed in 30% (6/20 patients) with negative Ghent and revised Ghent criteria and no FBN1 mutations. These results suggest that adult Korean patients who fulfill the old Ghent criteria almost all fulfill the new criteria for the diagnosis of MFS.
American Journal of Medical Genetics Part A 12/2011; 158A(5):989-95. · 2.39 Impact Factor
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ABSTRACT: Loeys-Dietz syndrome (LDS) is an inherited disorder that is characterized by the triad of arterial tortuosity and aneurysms, hypertelorism and a bifid uvula or cleft palate. The disease is caused by heterozygous mutations in the genes encoding transforming growth factor β receptors 1 and 2 (TGFBR1 and TGFBR2, respectively). However, studies of patients with LDS are limited in Korea. From June 2000 to December 2010, 13 patients (10 probands) diagnosed with LDS were enrolled. The multidisciplinary data of the patients were reviewed retrospectively. The frequency of each clinical manifestation in Korean patients with LDS was compared with Western populations as described in the report by Loeys et al. Twelve (92%) of the 13 LDS patients had arterial tortuosity, 9 (69%) patients had hypertelorism and 11 (85%) patients had bifid uvula or cleft palate. Mutations in either TGFBR1 or TGFBR2 were detected in nine probands (90%). Of the mutations, five novel mutations were detected; three in TGFBR2 and two in TGFBR1. Blue sclera and atrial septal defect were not observed in the Korean patients, and the frequency of blue sclera was significantly lower in our Korean population than previously-described Western population (0 vs 40%; P=0.005). Despite the restricted number of patients in our study, we identified five novel mutations in the TGFBR1 and TGFBR2 genes and, except for blue sclera, no differences in phenotype are apparent between Korean patients and Western patients.
Journal of Human Genetics 11/2011; 57(1):52-6. · 2.57 Impact Factor
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Young Bae Sohn,
Eun Wha Choi,
Su Jin Kim,
Sung Won Park,
Se-Hwa Kim,
Sung-Yoon Cho,
Soo In Jeong,
June Huh,
I-Seok Kang, Heung Jae Lee,
Kyung-Hoon Paik,
Dong-Kyu Jin
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ABSTRACT: Hunter syndrome (mucopolysaccharidosis II, MPS II) is a rare, X-linked disorder of glycosaminoglycan (GAG) catabolism caused by a deficiency in the activity of the lysosomal enzyme, iduronate-2-sulfatase (I2S). In this study, the medical records of 75 Korean patients with Hunter syndrome (74 males, 1 female) were retrospectively reviewed to investigate the frequency of organ involvement and survival at a single center. The three most common symptoms of organ involvement were hepatosplenomegaly (99%), facial dysmorphism (97%), and frequent otitis media (91%). Cardiovascular involvement was also common including valvular abnormalities (89%), left ventricular hypertrophy (68%), and hypertension (30%). The 19 patients who died had a median age of 16.8 years at the time of death. Four of them died within 1 year of the start of enzyme replacement therapy; autopsy showed myocardial infarction with severe coronary artery disease in one patient. Two other patients died due to pneumonia and sleep apnea. In one case, the cause of death was not investigated. The high incidence of hypertension, and the presence of valvular heart disease indicates that close cardiac monitoring is mandatory in all patients with Hunter syndrome, especially relatively older patients even if they are being treated with enzyme replacement therapy. © 2011 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part A 11/2011; · 2.39 Impact Factor
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ABSTRACT: Fragmented QRS (fQRS) on 12-lead electrocardiography reflects conduction delay caused by myocardial fibrosis and dysfunction. Ventricular fibrosis detected by late gadolinium enhancement (LGE) cardiac magnetic resonance (CMR) is reportedly correlated with worse clinical outcomes in adults with repaired tetralogy of Fallot (TOF). The aim of this study was to assess whether the presence of fQRS is associated with right ventricular (RV) fibrosis or dysfunction in this patient group. In 37 consecutive patients (median age 30 years, median age at repair 6.6 years), the number of leads showing fQRS, defined as the presence of >2 notches on the R/S wave in ≥2 contiguous leads, was counted. RV systolic function, dilatation, and LGE score were measured using LGE CMR. Ventricular LGE was observed mainly at the previous surgical sites: the RV outflow tract (33 of 37), ventricular septal defect patch region (15 of 37), and RV anterior wall (11 of 37). Fragmented QRS was found mostly in the right and mid precordial leads. The fQRS group (n = 20) demonstrated higher RV LGE scores (p <0.001) and lower RV ejection fractions (p = 0.02) and a trend toward larger RV end-diastolic and end-systolic volumes (p = 0.12 and p = 0.06, respectively) compared to the non-fQRS group (n = 17). The number of electrocardiographic leads showing fQRS was positively correlated with RV LGE score (r = 0.75, p <0.001). The presence of fQRS remained independently associated with the presence of supramedian RV LGE score, even after adjusting for relevant parameters. In conclusion, fQRS was closely associated with more extensive RV fibrosis and dysfunction in adults with repaired tetralogy of Fallot.
The American journal of cardiology 09/2011; 109(1):110-5. · 3.58 Impact Factor
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ABSTRACT: Factors related to prolonged pleural drainage after the Fontan operation have not been clearly defined. We investigated perioperative variables to establish factors predicting operative morbidity including prolonged chest tube drainage. Also, we pursued the fate of the fenestration during the follow-up period.
We retrospectively reviewed 52 patients who had undergone a fenestrated extracardiac Fontan procedure between August 1998 and June 2008. The median age at the time of surgery was 34.8 (range: 18.5 ∼ 156) months and the median body weight 13.2 kg (range: 9.5 ∼ 33). A multivariable logistic regression model was used to compare demographic, anatomic, and physiological variables for postoperative morbidity.
Operative mortality occurred in one patient (1.9%). The mean duration of respiratory support, chest tube drainage, and hospital stay was 13 hours (range: 4 to 328 hours), six days (range: 2 to 45 days), and 16 days (range: 7 to 444 days), respectively. Statistically, an operation without previous bidirectional cavopulmonary shunt (OR 30, 95% CI 3.1 to 289) was the only independent risk factor for prolonged pleural drainage. Aortic cross-clamp time was identified as a risk factor for prolonged mechanical ventilatory support. During a median follow-up at 62 months (range: 17 to 137 months), there was one late death (1.9%). Twenty-two patients (43%) underwent intervention for fenestration closure.
Previous bidirectional cavopulmonary shunt and shortened aortic cross-clamp time may reduce postoperative morbidity including prolonged chest tube drainage and mechanical ventilator support after the fenestrated extracardiac conduit Fontan procedure.
Journal of Cardiac Surgery 08/2011; 26(5):509-14. · 0.87 Impact Factor
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ABSTRACT: The optimal surgical strategy for coarctation of the aorta (CoA) with ventricular septal defect (VSD) is controversial. The aim of this study is to evaluate the clinical outcome of a single-stage repair of CoA with VSD.
We reviewed 72 patients who underwent single-stage repair for CoA with VSD between January 1995 and December 2007. There were 43 males and 29 females. The median age of the patients was 28 days (range = 3 to 188) and median weight was 3.7 kg (range = 2.16 to 5.6). Deep hypothermic circulatory arrest was used in 22 patients and selective antegrade cerebral perfusion was performed in 43 patients.
There were no operative deaths and one late death at a median follow-up of 60 months (range = 16 to 158). Postoperative complications were left main bronchus compression requiring aortopexy in one patient and recoarctation requiring balloon dilatation in one patient. Subaortic stenosis occurred in two patients and surgical repair was performed.
One-stage simultaneous repair can be performed with a low risk at an early age.
Journal of Cardiac Surgery 04/2011; 26(4):420-4. · 0.87 Impact Factor
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ABSTRACT: The purpose of this study was to analyze the MDCT and MRI findings in patients with unroofed coronary sinus syndrome.
This retrospective study included 11 patients with unroofed coronary sinus syndrome (10 adults, one child) without persistent left superior vena cava and one adult with communication of the left atrium and coronary sinus via an anomalous vein (unroofed coronary sinus variant). Four patients underwent contrast-enhanced ECG-gated MDCT; six, MRI; and two patients, both CT and MRI. We also measured the coronary sinus on the CT scans of 28 adults with normal cardiac anatomy and 10 adults with persistent left superior vena cava and compared the measurements with those in the patients with unroofed coronary sinus syndrome. Seven patients underwent surgical treatment of unroofed coronary sinus syndrome.
At echocardiography, unroofed coronary sinus syndrome was not clearly discriminated from atrial septal defect in two patients and was not suspected in three patients. CT and MRI showed that 11 patients had a defect in which the coronary sinus communicated with the left atrium and that the other patient had atresia of the coronary sinus orifice with an anomalous vein connecting the coronary sinus and left atrium. In patients with unroofed coronary sinus syndrome, the mean standardized diameter of the coronary sinus according to the patient's body surface area was 15 ± 4 mm/m², similar to that of the control group with persistent left superior vena cava (15 ± 6 mm/m²; p = 0.97) and significantly greater than that of the group with normal cardiac anatomy (7 ± 2 mm/m²; p < 0.0001).
CT and MRI facilitate definite diagnosis of unroofed coronary sinus syndrome.
American Journal of Roentgenology 11/2010; 195(5):W331-6. · 2.78 Impact Factor
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ABSTRACT: 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)).
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.
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) (β=-0.60, p<0.001), mid-RV dimension (β=-0.26, p=0.012), left ventricular ejection fraction (β=0.22, p=0.023), and early diastolic tricuspid annular velocity (β=0.21, p=0.048).
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.
Korean Circulation Journal 08/2010; 40(8):391-8.
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ABSTRACT: The aim of this study was to investigate clinical manifestation and causes of chest pain in adults with congenital heart disease. Of 966 patients in our Adult Congenital Heart Clinic, 50 patients had cyanosis (cyanosis [+]), and 916 patients did not have cyanosis (cyanosis [-]). Fifty-four patients (16 male and 38 female) with a history of chest pain requiring medical care were selected: 33 were in the cyanosis (+) group, and 21 were in the cyanosis (-) group. Medical records were reviewed. The causes of chest pain were (no. cyanosis [+], no. cyanosis [-], respectively) as follows: idiopathic (n = 19, n = 10), pulmonary hemorrhage (n = 6, n = 0), pulmonary tuberculosis (n = 2, n = 0), pulmonary thromboembolism (n = 4, n = 0), localized musculoskeletal (n = 1, n = 1), myocardial ischemia (n = 1, n = 1), and arrhythmia (n = 2, n = 9). Patients with cyanosis had much higher frequency of chest pain compared with patients without cyanosis (33 of 50 vs. 21 of 916, p < 0.0001). Chest pain in adults with congenital heart disease has diverse causes. Meticulous evaluation of chest pain in adults with congenital heart disease is required to differentiate correctable causes from idiopathic or incurable ones.
Pediatric Cardiology 02/2010; 31(5):620-5. · 1.30 Impact Factor
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ABSTRACT: The CoaguChek XS international normalized ratio (INR) assay was compared to INR assay by a standard laboratory method in children with heart disease on anticoagulant therapy. The data comprised 120 pairs of INR values for 42 patients (age <16 yr) who attended a cardiology clinic between 1 May 2007 and 30 January 2008. Parallel INR assays by the CoaguChek XS and the standard method were performed within 1 hr by a single qualified technician and the paired results were evaluated by linear regression and Bland-Altman analysis. The mean difference in the INR values was -0.08 +/- 0.04 units (p = 0.63); the difference between the two results was consistently <0.5 INR units. The slope of the regression line was 0.98 (95% CI: 0.96 to 1.01) and the y-intercept was 0.014 (95% CI: -0.01 to 0.04). In the Bland-Altman analysis, the mean difference in INR between the two methods was 0.08 units and values for 99.4% of the patients fell within the limit of agreement (-0.17 to 0.28 units). In summary, INR assays in children by the CoaguChek XS device are as accurate as the standard method, but faster and more convenient.
Annals of clinical and laboratory science 01/2010; 40(3):247-51. · 0.96 Impact Factor
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ABSTRACT: Intracardiac thrombosis in patients with a dilated cardiomyopathy can be life threatening. This study investigated the incidence, risk factors, and outcome of intracardiac thrombosis in children with dilated cardiomyopathy. A retrospective review of the clinical records was performed in 83 children with dilated cardiomyopathy diagnosed from January 1995 to December 2008. Intracardiac thrombi were detected in 5 patients (6.0%). The intracardiac thrombi were found mainly in the left ventricle (n = 3). One patient had a thrombus in the left atrium at the time of diagnosis, and a right ventricular thrombus was found in 1 patient with unrepaired ventricular septal defect complicated by pulmonary hypertension. Intracardiac thrombosis developed during rapid deterioration of ventricular function, and all patients had a poor ejection fraction of the left ventricle. All patients were treated with heparinization, and thrombectomy was performed in 1 patient. Three patients achieved complete resolution of the thrombus without further embolic complications. Careful evaluation and aggressive anticoagulation are necessary for the prevention of intracardiac thrombosis in children with poor ventricular function, especially during rapid deterioration of ventricular function.
Pediatric Cardiology 11/2009; 31(2):264-9. · 1.30 Impact Factor
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Hye Soo,
Ji Eun Kim,
Soo Kyoung,
Hyun Ju Seo,
M D Yoo,
Jin Jeong,
Seo Heui Chio,
Soo In Jeong,
Hoon Kim,
M D Ji,
Hyuk Yang,
June Huh,
Yun Sil Chang,
Tae Gook,
Seok Kang,
Soon Park,
Pyo Won Park, Heung Jae Lee
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ABSTRACT: P u r p o s e: This study investigated the clinical course and prognostic factor of very low birth weight infants (VLBWI) with hemodynamically significant congenital heart defects (CHDs). Me t h o d s: Medical records of 1,098 VLBWI with birth weight <1,500 g who had been admitted to the neonatal intensive care unit of Samsung Medical Center from October 1994 to December 2007 were reviewed retrospectively. The data for these patients with hemodynamically significant CHD (n=33) were compared with those without CHD (n=1,065). Re s u l t s: The incidence of CHD was 3.0% (33 patients) 7 patients (21%) had CHD combined with the congenital abnorma-lities or chromosomal disorders. The most common CHD was a ventricular septal defect. The incidence of intrauterine growth retardation was higher in patients with CHD than in patients without CHD (34% vs. 20%), but there were no significant differ-ences in gestational age, birth weight, respiratory distress syndrome, bronchopulmonary dysplasia, necrotizing enterocolitis, severe intraventricular hemorrhage (≥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%).
Korean Journal of Pediatrics 01/2009; 52.
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Young Bin Song,
Seung Woo Park,
Jun Hyung Kim,
Dae-Hee Shin,
Sung Won Cho,
Jin-Oh Choi,
Sang-Chol Lee,
Ju Ryoung Moon,
June Huh,
I-Seok Kang, Heung Jae Lee
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ABSTRACT: 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.
Journal of Korean Medical Science 10/2008; 23(5):808-13. · 0.99 Impact Factor
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Ga Yeun Lee,
Young Bae Sohn,
Myo Jing Kim,
Ga Won Jeon,
Jae Won Shim,
Yun Sil Chang,
June Huh,
I-Seok Kang,
Ji-Hyuk Yang,
Tae-Gook Jun,
Pyo Won Park,
Won Soon Park, Heung Jae Lee
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ABSTRACT: 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).
From October 1994 to July 2006, medical records of 94 VLBWI weighing <1,500 g 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).
In the Mo group, birth weight was significantly lower and the preoperative mean FiO2 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.
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.
Yonsei Medical Journal 05/2008; 49(2):265-71. · 1.14 Impact Factor
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In Sook Park,
Young Hwue Kim,
Jae Kon Ko,
Jung Yun Choi,
Soo Jin Kim,
Hong Ryang Kil,
June Huh, Heung Jae Lee,
Jae Sook Ma,
Sang Bum Lee,
Eul Ju Seo,
Han Wook Yoo
11/2007: pages 242 - 243; , ISBN: 9780470988664
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Yong-Won Park,
Ji-Whan Han,
In-Sook Park,
Chang-Hwi Kim,
Sung-Ho Cha,
Jae-Sook Ma,
Joon-Sung Lee,
Tae-Chan Kwon,
Sang-Bum Lee,
Chul-Ho Kim, Heung-Jae Lee,
Yong-Soo Yun
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ABSTRACT: The aim of this study was to investigate the incidence rate and epidemiologic patterns of Kawasaki disease in Korea for the 3-year-period, 2003-2005.
The questionnaire for an epidemiologic survey on Kawasaki disease was distributed to all 102 Korean hospitals that conduct pediatric residency programs, and obtained data were analyzed upon receipt.
The 9662 patients of Kawasaki disease from 85 hospitals that responded (response rate, 83.3%) consisted of 5877 males and 3785 females (male:female ratio, 1.55:1). The incidence rate per 100,000 children <5-year-old was 104.2 in 2003, 106.4 in 2004, and 104.6 in 2005 (average rate, 105.0). Their mean age of onset was 33.3 months, and the proportions of sibling cases and recurrent cases were 0.29% and 2.0%, respectively. Coronary arterial abnormalities were detected at follow-up by echocardiogram in 18.8% of all such cases including dilatations of 18.0% and aneurysms of 2.5%.
The average annual rate of incidence, 105.0/100,000 in children <5-year-old is the second highest reported rate in the world.
The Pediatric Infectious Disease Journal 09/2007; 26(9):821-3. · 3.58 Impact Factor
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ABSTRACT: 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.
Journal of Korean Medical Science 01/2007; 21(6):1111-4. · 0.99 Impact Factor
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ABSTRACT: We report our experience in 13 patients who underwent transcatheter closure of Fontan fenestration with the Cook 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 follow-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 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.
Journal of Korean Medical Science 11/2006; 21(5):859-64. · 0.99 Impact Factor
