Purpose : Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. Methods : Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. Results : Ten(67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. $O_2$ saturation and pH were lower and cardiothoracic(CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia(P<0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but $O_2$ saturation was lower in functional atresia group(P<0.05). 13 patients(87%) were managed with $PGE_1$. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period(mean 37 months). 3 patients(20%) died and none of patients without pulmonary atresia died. Conclusion : We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.
Purpose : Low dose aspirin is used due to its antiplatelet effect for the subacute phase of Kawasaki disease(KD). It is usually used for 6-8 weeks, then various hematologic laboratory tests and follow up echocardiography for evaluating coronary abnormalities are performed. Our review investigated the usefulness of various follow up laboratory tests performed at 6 weeks after the onset of KD. Methods : Two hundred eighty-two children diagnosed and hospitalized with KD were identified by reviewing patient's charts. Cases which were diagnosed between January 1997 and December 2004 were included in this study. We reviewed laboratory data including leukocytes, platelet counts, antistreptolysin O(ASO), erythrocyte sedimentation rate(ESR), C-reactive protein(CRP), aspartate aminotransferase(AST), alanine aminotransferase(ALT), urinalysis, and echocardiograms performed at admission and 6 weeks after the onset of KD. Paired t-test and Fisher's exact test, as well as logistic regression tests, were used for the statistical analysis. Results : At 6th week data, ESR and CRP were still elevated in 35(12.4 percent) and 12(4.3 percent) patients, respectively. Sterile pyuria were all normalized. But, 36 patients(12.8 percent) showed thrombocytosis, 22(7.8 percent) elevated AST, 15(5.3 percent) leukocytosis, and 6(2.1 percent) coronary abnormalities. Coronary abnormalities at the 6th week were only shown in patients with initial abnormalites. Younger age and initial thrombocytosis were risk factors for thrombocytosis at the 6th week. Conclusion : All children with initial coronary abonormalites should have an echocardiogram at 6 weeks after the onset of fever. In view of case-effectiveness, additional echocardiographic studies are justified only if abnormalities are present at admission. ESR, CRP, and urinalysis performed at the 6 weeks after onset of KD is not significant for clinical information of progression. Platelet count should be estimated at 6th week for a judgement of continuous antiplatelet therapy.
Choi, Deok Young;Choi, Jae Young;Kim, Mi Jin;Sul, Jun Hee;Lee, Sung Kyu
Clinical and Experimental Pediatrics
/
v.46
no.9
/
pp.871-875
/
2003
Purpose : This study was performed to follow the natural course and size change of isolated atrial septal defect(ASD) secundum. Methods : Among the newly diagnosed with ASD secundum at the division of pediatric cardiology in Yonsei cardiovascular hospital from January 1996 to December 2000, 89 patients with pure ASD secundum were checked by the serial echocardiographic evaluation to measure the size change. Results : There was a statistical difference(P<0.05) in the rate of spontaneous closure and size change of the defect between the group of bigger defect(>8 mm) and the group of smaller defect (${\leq}8mm$). There was also a statistical difference(P<0.05) in the rate of spontaneous closure and size change of the defect between the group of younger than three years of age and the group of older than three years of age. The initial size of the defect and change of size were the significant influencing factors in the rate of spontaneous closing of ASD. In the group of patients who have persistent ASD, the size of defect showed a tendency of increasing size of defect with the age(P<0.005); however in the correction of these values by the body surface area of each patients, there was no statistical difference. Conclusion : The possibility of spontaneous closure of pure ASD secundum was higher in the group of patients who have smaller defect and who were younger. The ASD secundum that did not have spontaneous closure showed an increase in size with the growth of the patients; however when this size was corrected by the body surface area, there was no statistical difference. The closure of the defect was at 26.2 months of age so close follow-up of the patients is important.
Aortic valvuloplasty has recently gained attention as an attractive alternative procedure for aortic valvular disease. Between March 1995 to August 1996, 14 patients with pure aortic regurgitation(AR) underwent aortic alvuloplasty using leaflet extension with glutaraldehydepreserved autologous pericardium. There were 11 males and 3 females, and the mean age was 34.8 $\pm$ 15.3 years. Preoperative echocardiography and cardiac catheterization revealed that the degree of AR was mean 3.4$\pm$0.65, and more than moderate degree of mitral regurgitation(MR) were detected in 4 patients. In 12 patients, 3 leaflets were extended and in another 2 patients only one deformed leaflet was extended. Concomitant mitral valvuloplasty (MVP) was performed in 4 patients. The competency of the aortic valve after completion of repair was evaluated by the transesophageal echocardiography in operating theater, and there was no aortic and mitral stenosis or regurgitation. In an early postoperative echocardiography, trivial AR was detected in 3 patients and mild MR in 1 patient. The end-systolic and end-diastolic dimensions of the left ventricle were decreased significantly(p<0.05) as compared with those of preoperative values. T ere was no mortality and no significant postoperative complication encountered. Late complication developed in 2 patients during the follow-up period(mean 7.9$\pm$ 5.9 months). One patient underwent AVR on postoperative 7th month due to endocarditis, and the another patient with Behcet's disease underwent Ross operation at postoperative 4th month. In conclusion, AVP of leaflet extension technique offers an excellent early clinical result and represents a good alterna!ivy surgical treatment for the pure AR especially in young age group, although long-term follow-up is necessary to determine the durability of glutaraldehyde-preserved autologous pericardium as a valve leaflet.
Recently, autologous bone marrow cell transplantation (CTx) for angiogenesis and myogenesis in ischemic myocardium has been extensively investigated to improve heart functions. This study was designed to evaluate the effects of CTx with off-pump coronary artery bypass grafting (OPCAB) in patients who were not feasible for complete revascularization. Material and Method: Four male patients underwent CTx and OPCAB simultaneously. Bone marrow was aspirated from iliac bone. Mean 1.5 ${\times}$ 10$^{9}$ mononuclear cells including mean 6.7 ${\times}$ 10$^{6}$ CD34 + cells and 3.7 ${\times}$ 10$^{6}$ AC133 + cells were obtained and concentrated with 10 cc. These cells were transplanted into non-graftable ischemic myocardium after OPCAB. The heart function of all patients were evaluated using the MIBI scan, echocardiogram and MRI preoperatively. The effects of CTx was evaluated using MIBI scan and echocardiogram at 1 month postoperatively. Result: An average of 2 grafts were bypassed to left anterior descending artery territory. Other territories were transplanted with isolated mononuclear cell. All patients had uncomplicated postoperative course. After 1 month follow up, there were improvement in symptom, ejection fraction (from 49% to 55%) on echocardiogram and myocardial perfusion on MIBI scan in all patients. Conclusion: These preliminary data showed improvement of heart function and myocardial perfusion and also showed the feasibility and safety of combined therapy with OPCAB and CTx in ischemic myocardium. However, the effectiveness of CTx alone cannot be readily assessed. Further randomized, controlled studies are required to evaluate the effectiveness of CTx alone.
Background: This study was undertaken in infant patients with isolated ventricular septal defect(VSD) to determine the effect of surgical closure on ventricular systolic time interval, as a parameter for ventricular performance, by echocardiography. Material and Method: Thirty patients were enrolled. Mean age of patients at operation was 6.5$\pm$3.2 months and all patients had non-restrictive VSD. We checked the left atrium/aorta(LA/Ao) ratio, left ventricle ejection fraction(EF), left ventricular systolic time interval(LVSTI), and right ventricular systolic time interval(RVSTI). Echocardiographic studies were done before surgical correction and postoperative periods(postopl: within 2 weeks, postop2: between 4 and 6 months, postop3: between 1 and 2 years). Result: LA/Ao ratio decreased significantly at immediate postoperative period compared to preoperative period and sustained during further follow-up period(from 1.74$\pm$0.37 to 1.36$\pm$0.24*, 1.32$\pm$0.22*, and 1.27$\pm$0.19*, p<0.01). LV EF had not changed during follow-up periods(from 65.1$\pm$7.0 to 62.3$\pm$9.5, 62.8$\pm$5.7, and 64.1$\pm$6.9). LVSTI decreased significantly at postop2 and sustained during further follow-up period (from 0.46$\pm$0.13 to 0.46$\pm$0.11, 0.37$\pm$0.08*, and 0.34$\pm$0.07*, p<0.01). RVSTI decreased significantly at postop3(0.33$\pm$0.08 to 0.32$\pm$0.08, 0.31$\pm$0.07, and 0.27$\pm$0.05*, p<0.01). Conclusion: We found that right and left ventricular systolic time intervals had decreased over the period of 1 year after surgical correction of VSD. Therefore, it is necessary to observe the change of ventricular function during that period.
Purpose : Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. Methods : The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. Results : Age at diagnosis was $45{\pm}53months$(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. Conclusion : INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.
Background: To evaluate the efficacy of arterial switch operation for transposition of great arteries, serial echocardiographic studies were performed in 8 patients who underwent the surgery between 1989 and 1998 at Dept. of Thoracic & Cardiovascular Surgery, Yeungnam University Hospital in Daegu City, Korea. Material and Method: Follow-up period ranged from 6 months to 11 years(average of 4.1 years). Body weight ranged from 2.6kg to 4.8kg, with average of 3.6kg. 5 of 8 patients were preoperatively diagnosed as TGA+VSD, and 3 as TGA+IVS. LV function was evaluated by the measurement of LV shortening fraction, LVSTI, and LVEF. RVSTI was also measured. Postoperative function of valve and growth of great vessels were analyzed by the measurement of PSPGV, valvular regurgitation, LA/AO ratio, root dimension of aorta and pulmonary artery, comparing with the age matched controls, respectively. Result: LVEF had an average of 65.0+9.03% which is tended to increase serially. LVAOPG had an average of 15.9mmHg. RVPAPG, 27.5mmHg. From the measurement of aortic root dimension of 6 patients at end-systole, aortic root growth was assumed to increase more than the mean value of normal growth. PA root dimension at end-systole showed a similar growth progress when compared with age matched normal controls. Postoperative pulmonic valve regurgitation was noted in 5 of total 8 patients, in which 1 patient who showed grade 2 and 4 showed below grade 1. AR, in 6 patients and all grade 1 Except 1 patient, all the valvular regurgitations were below grade 1, which was presumed to be clinically insignificant.
Background: In the operation for coronary artery stenosis, the procedures for mitral regurgitation are restricted to cases of more than moderate mitral regurgitation or for the lesions in leaflets. This is based on the belief that the less than mild regurgitation are a form of reversible change results from ischemia with coronary artery stenosis. We studied the changes and prognostic factors of mitral regurgitation in patients with coronary artery stenosis and mitral regurgitation who underwent coronary artery bypass surgery alone. Material and Method: We reviewed the medical records of 90 patients with coronary artery stenosis and mitral regurgitation who underwent coronary artery bypass surgery alone by a single surgeon from Jan. 1995 to Dec. 2002, We grouped the patients according to the postoperative changes of mitral regurgitation, and then we statistically compared the findings of echocardiogram between preoperative and last follow up. Result: There were 24 cases with progression of mitral regurgitation, 12 cases without changes, 54 cases with improvements of mitral regurgitation in total 90 patients. The bypass to LAD was proven as the significant prognostic factor of mitral regurgitation. The preoperative end diastolic left ventricular volume index were higher in aggravated group with 105.38$\pm$38.89 $m\ell$ compared to 71.75$\pm$28,45 $m\ell$ in improvement group, and 84.00$\pm$11.66 $m\ell$ in no change group. The grade of preoperative mitral regurgitation did not show significant differences among the groups. Conclusion: The mitral regurgitation in patient with coronary artery stenosis can be improved after the coronary artery bypass surgery alone. However, the expectation of improvements based on the degree of preparative mitral regurgitation can not be justified, therefore, the procedures for mitral regurgitation should be aggressively considered even in the cases of mild mitral regurgitation. Also, further study should be performed to identify the exact prognostic factors of mitral regurgitation including the left ventricular volume index, and whether the left anterior descending artery has been bypassed.
Purpose We considered the correlation of Ejection Fraction (EF) which was analyzed by Multi Gated Blood Pool Scan (MUGA) and Echocardiography (ECHO) for the patients who were classified according to the condition of cardiac function. Materials and Methods We analyzed the patients (female 60) who were diagnosed with breast cancer and were examined by both MUGA and ECHO. The 30 patients (age: $58.27{\pm}13.48$) who were analyzed into less than 50% to 70% of EF were categorized as normal group and the other 30 patients (age: $53.70{\pm}8.45$) who were analyzed into less than 50% of EF were categorized as abnormal group. Statistical analysis with SPSS ver. 18 was applied. Results Each of the value of mean and standard deviation of normal group was $66.43{\pm}5.80$ (MUGA), $60.50{\pm}4.93$ (ECHO). There was a significant difference (p<0.001). Each of the value of mean and standard deviation of abnormal group was $41.93{\pm}7.58$ (MUGA), $41.70{\pm}11.49$ (ECHO). There was no significant difference (p>0.001). In the result, all 30 cases of normal group showed the same reading. 8 out of 30 cases in abnormal group showed inconsistency of the reading. Conclusion We could confirm the correlation of the EF in MUGA and ECHO statistically. There was difference between abnormal groups from the result of reading. If we are aware of the result according to the different cardiac function categorization, MUGA and ECHO can be used as even more accurate interchangeable test.
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