• Journal of Genetic Medicine
• /
• v.6 no.1
• /
• pp.95-99
• /
• 2009

Prenatal diagnosis of rare autosome mosaicism involvingchromosomes other than chromosome 13, 18, 21 or the sex chromosome is encountered prognostic dilemma during genetic counseling. We report four cases of level III uncommon mosaicism of trisomy 5, 16 and 20,diagnosed prenatally. In case 1 with mosaic trisomy 20, there was a higher mosaic ratio of trisomy 20 in the repeat amniocentesis (62.1%) than in the first (36.6%) with normal fetal ultrasound finding except for a relatively small aorta on a 3-vessel view of the fetal heart. Case 2 showed a low rate of mosaic trisomy 20 (5.25%) in cultured amniocytes but normal karyotype in the repeat amniocentesis, who delivered a normal healthy baby. Case 3 showed a 13.6% of trisomy 16 mosaicism in the 30 cells of cultured amniocytes. Sixty cells from a fetal blood sample at termination showed non-mosaic 46,XX normal karyotype, while skin fibroblasts had 22.5% trisomy 16 in 40 metaphases. The autopsy showed ventricular septal defect (VSD). Case 4 with low grade mosaicism (10.5%) of trisomy 5 resulted in elective termination, though the ultrasoumd showed growsly normal fetus. Although level III mosaicism is regarded as true mosaicism, it is difficult to predict the outcome of the fetus with rare mosaic autosome trisomy. Therefore mosaic autosome trisomy of fetus should be carefully interpreted with more various approaches including repeat sampling and targeted fetal ultrasound.

• The Korean Journal of Nuclear Medicine
• /
• v.37 no.2
• /
• pp.73-82
• /
• 2003

Objectives: A new software (Cardiac SPECT Analyzer: CSA) was developed for quantification of volumes and election fraction on gated myocardial SPECT. Volumes and ejection fraction by CSA were validated by comparing with those quantified by Quantitative Gated SPECT (QGS) software. Materials and Methods: Gated myocardial SPECT was peformed in 40 patients with ejection fraction from 15% to 85%. In 26 patients, gated myocardial SPECT was acquired again with the patients in situ. A cylinder model was used to eliminate noise semi-automatically and profile data was extracted using Gaussian fitting after smoothing. The boundary points of endo- and epicardium were found using an iterative learning algorithm. Enddiastolic (EDV) and endsystolic volumes (ESV) and election fraction (EF) were calculated. These values were compared with those calculated by QGS and the same gated SPECT data was repeatedly quantified by CSA and variation of the values on sequential measurements of the same patients on the repeated acquisition. Results: From the 40 patient data, EF, EDV and ESV by CSA were correlated with those by QGS with the correlation coefficients of 0.97, 0.92, 0.96. Two standard deviation (SD) of EF on Bland Altman plot was 10.1%. Repeated measurements of EF, EDV, and ESV by CSA were correlated with each other with the coefficients of 0.96, 0.99, and 0.99 for EF, EDV and ESV respectively. On repeated acquisition, reproducibility was also excellent with correlation coefficients of 0.89, 0.97, 0.98, and coefficient of variation of 8.2%, 5.4mL, 8.5mL and 2SD of 10.6%, 21.2mL, and 16.4mL on Bland Altman plot for EF, EDV and ESV. Conclusion: We developed the software of CSA for quantification of volumes and ejection fraction on gated myocardial SPECT. Volumes and ejection fraction quantified using this software was found valid for its correctness and precision.

• The Korean Journal of Nuclear Medicine
• /
• v.38 no.3
• /
• pp.218-224
• /
• 2004

We compared rest perfusion PET with redistribution perfusion SPECT to investigate the concordant rate between PET and SPECT images and analyze the discordant pattern. Materials and Methods: Rest N-13 ammonia and F-18 FDG PET were performed on 18 patients with old myocardial infarction and left ventricular dysfunction whose dipyridamole - 4hr redistribution TI-201 SPECT showed one or more severe fixed defects. Regional perfusion and metabolism were evaluated visually and quantitatively with 5-segment myocardial model. Results: There were high concordant rate in uptake pattern (80/90 segments, 88.9%) and high correlation coefficient on quantitative analysis (R=0.81, p<0.001) between redistribution TI-201 SPECT and N-13 ammonia PET images. Nine of 18 patients had SPECT-PET concordant pattern (Group I). Ten segments (9 in inferior wall, 1 in apex) from the remaining 9 patients showed SPECT-PET discordant pattern with abnormal TI-201 defect and near normal N-13 ammonia uptake (Group II). The diastolic and systolic left ventricular dimensions were significantly increased in Group II compared to those of Group I. When attenuation uncorrected N-13 ammonia PET images were reconstructed in Group II, it resulted in PET images with severe inferior wall defects nearly identical to those seen in redistribution TI-201 SPECT images. Conclusion: Redistribution TI-201 SPECT images showed high concordant rate and correlation with rest N-13 ammonia PET images. Most of discordant segments had fixed thallium defects in inferior wall with nearly normal N-13 ammonia uptake, which may result from severe left ventricular dilatation and attenuation by the left hemidiaphragm and cardiac blood pool.

• Journal of Yeungnam Medical Science
• /
• v.16 no.2
• /
• pp.309-317
• /
• 1999

Background: Left ventricular diastolic filling is an important determinant for maintenance of cardiac output during hemodialysis. Few investigators have studied the influence of hemodialysis on diastolic function. To evaluate the change of left ventricular systolic and diastolic function. we performed M-mode and Doppler echocardiographic studies before and after hemodialysis. Methods: The study population consisted of 30 patients(15 patients were male, mean age $45{\pm}10$ years) with CRF on maintenance hemodialysis. They have normal left ventricular systolic function(Fractional shortening>30%) and no evidence of valvular heart disease or regional wall motion abnormalities. The ejection fraction (EF) was measured using M-mode echocardiography and Doppler indices such as peak E velocity, peak A velocity, isovolumetric relaxation time(IVRT), deceleration time(DT). and left ventricular ejection time(LVET) obtained from Doppler echocardiography. The index of myocardial performance (IMP) was calculated from each of the Doppler velocity indices. Results: The weight reduction after hemodialysis was $2.1{\pm}1.0kg$(p<0.0001), After hemodialysis, there was some decrease in blood pressure(p<0.05), but no significant change in heart rate, EF and fractional shortening, mean VCF, peak A velocity, and DT. And significant reduction in peak E velocity, E/A ratio(p<0.0001. p<0.001), and significant increase in IVRT and IMP(p<0.05, p<0.0001) were noted. Conclusion: In conclusion, preload reduction is the main mechanism that accounts for changes in Doppler diastolic indices after hemodialysis. And an increased IMP suggests that diastolic function may be aggravated after hemodialysis, and that implies impaired left ventricular filling and disturbed left ventricular compliance.

• The Korean Journal of Nuclear Medicine Technology
• /
• v.16 no.2
• /
• pp.49-56
• /
• 2012

Purpose : Gated myocardial perfusion SPECT provides not only myocardial perfusion status, but various functional parameters of left ventricle (LV). The purpose of this study was to analyze ejection fraction (EF) for correlation and difference between $^{201}Tl$ gated myocardial perfusion SPECT and echocardiography depending on extent of perfusion defect, gender and LV volumes. Materials and Methods : From April 2011 to May 2012, we analyzed 291 patients (male:female =165:126; mean: $64.6{\pm}10.8$ years) who were examined both $^{201}Tl$ gated myocardial perfusion SPECT and echocardiography at less than 7 days apart in our hospital. 101 patients showed perfusion defect and the rest of the people without any defect. We applied automatic analysis (Quantitative gated SPECT, QGS), and calculated EF, End-diastolic volume (EDV) and End-systolic volume (ESV) from Stress (G-Stress) and Rest (G-Rest) studies. And we analyzed the correlation and difference for EF between $^{201}Tl$ gated SPECT and echocardiography. Results : The correlation of LVEF among G-Stress, G-Rest and echocardiography was quite a good (G-Stress vs. G-Rest: r=0.909, G-Stress vs. echocardiography: r=0.833, G-Rest vs. echocardiography: r=0.825). And there were significant differences in EDV, ESV and EF in total patients (p<0.01). The normal group showed significant difference in EF (p<0.01) and the group with perfusion defect also demonstrated significant difference (a group with reversible defect: p<0.01, fixed defect: p<0.01) depending on extent of perfusion defect. We analyzed difference in normal group by gender. In normal group, there was no significant difference (p>0.05) in EF from men. However, there was a significant difference (p<0.01) from women. When we classified two groups by average size of EDV in Korean women, there was no significant difference in a group of above average size of EDV (p>0.05). Conclusion : When compared among Stress and Rest of $^{201}Tl$ gated SPECT and echocardiography, we confirmed that there was a good correlation for LVEF. But there were significant differences among three studies. And extent of perfusion defect, gender and LV volumes are independent determinants of the accuracy of LVEF. So, it is hard to compare and interchange quantitative indices among modalities. We should take additional researches to prove results of our study.

• Journal of Chest Surgery
• /
• v.38 no.5 s.250
• /
• pp.357-365
• /
• 2005

Background: Patients with mitral regurgitation (MR) in the setting of coronary artery disease have a dismal long-term prognosis whether treated medically or surgically. Moreover, the optimal management of moderate ischemic MR at the time of coronary artery bypass grafting (CABG) remains the subjects of controversy. Thus, the present retrospective study was undertaken to determine whether mitral valve surgery for moderate ischemic MR at the time of CABG would be preferable to CABG alone in terms of clinical outcome. Material and Method: Between January 1997 and December 2003, 34 patients with moderate (Gr 3/4) ischemic MR underwent CABG alone (Group I, n=23) or CABG plus mitral valve surgery (Group II, n=11). Operative mortality, long-term survival and echocardiographic parameters were used to evaluate the efficacy of mitral valve surgery in patients with moderate ischemic MR. The mean follow-up durations of each group were $69.3\pm4.3$ months and $53.1\pm4.9$ months respectively. Result: There was no hospital mortality in both groups. There was one case of late mortality in Group I. The mean number of bypass graft was similar ($3.8\pm1.2\;vs\;3.7\pm1.3$ respectively). Cardiopulmonary bypass time was longer in group II (p=0.014). In group II, all of the patients received mitral annuloplasty using ring. On immediate postoperative echo-cardiogram, mitral regurgitation was reduced more in group II (p=0.002). Echocardiogram performed at last follow-up state showed no difference except the grade of MR between the two groups. Actuarial survival of both groups at 5 years was similar ($95.5\%\;vs\;100\%$, p=0.48). Conclusion: This study shows that in selected patients with moderate ischemic MR, CABG without mitral valve surgery might be sufficient. However, patients with low EF and NYHA functional class pre-operatively had tendency of significant residual MR, so mitral valve surgery should be necessary in these patients, and moreover, MR severity and left ventricle volume decreased more in mitral valve surgery group. Therefore, more large-scale studies are necessary to determine these effects on the ventricular function and long-term survival.

• Journal of Chest Surgery
• /
• v.39 no.6 s.263
• /
• pp.434-439
• /
• 2006

Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.

• Journal of Chest Surgery
• /
• v.35 no.5
• /
• pp.329-335
• /
• 2002

Background: The surgical technique for biventricular assist device(BVAD) implantation has mainly consisted of cannulation procedures. A median sternotomy has been the technique of choice as it gives a surgeon an excellent exposure of the heart. However, considering that most patients require a future sternotomy or already have a previous sternotomy, sternotomy-related complication remains a major concern in BVAD implantation. Based on this consideration as well as the clinical experiences of conventional heart surgery, the authors have hypothesized that the cardiac chambers for BVAD cannulation can be approached from the right side of the heart. The purpose of this studs to develop a novel surgical technique of right thoracotomy for BVAD implantation in an animals study. Material and Method: For last two years, 16 (11 calves, 3 canines, and 2 sheep) out of 30 experimental animals with AnyHeart implantation underwent a right thoracotomy. The device was used as an implantable BVAD in 14 animals, a wearable BVAD in 1, and an implantable LVAD in 1. The chest cavity was entered through the 4th intercostal space or the 5th periosteal bed. As for the BVAD use, a right inflow cannula was inserted into the right atrial free wall and a right outflow cannula was grafted onto the main pulmonary artery. A left inflow cannula was inserted into the interatrial groove and a left outflow cannula was grafted on the innominate artery of the ascending aorta. The connecting tubes were brought out through the thoracotomy wound and connected to the pump located in the subcutaneous pocket at the right flank. Result: Except for the 5 animals for a lilting test or during the early learning curve, all recovered smoothly from the procedures. The inflow drainage allowed the pump output 6.5 L/min at the maximum with 3-3.5 L/min in an average. Of the survivors, there noted no procedure-related mortality or morbidity. Necropsy findings demonstrated the well-positioned cannula tips in the each cardiac chamber

• Journal of Chest Surgery
• /
• v.31 no.2
• /
• pp.95-101
• /
• 1998

Ischemic preconditioning is known to have protective effect on myocardial function at prolonged ischemic insult but the mechanism of the effect is not clearly known. The effect of the preconditioning on the global ischemia using cardioplegic solution is not well known. To evaluate the effect of global myocardial preconditioning on the functional recovery after cardioplegic arrest and two hours of hypothermic storage, we used the isolated rat heart and two hours cardioplegic arrest time at $0^{\circ}C$. In the experimental group(n=10), after baseline functional data was obtained, ischemic preconditioning was induced with 1 min of global normothermic ischemia for three times before the arrest period. In the control group(n=10), hearts underwent no ischemic precondi- tioning. After 2 hrs of cardioplegic arrest and storage in the $0^{\circ}C$ cardioplegic solution reperfusion was done and hemodynamic data were collected at post-reperfusion 20 min. Heart with ischemic preconditioning showed improved functional recovery at post reperfusion 20 min in peak developed pressure and dP/dT. In percent change of the peak pressure, preconditioning group showed 93.20$\pm$15.7% recovery rate compared to baseline data, and control group showed 67.3$\pm$15.6% recovery rate. In percent change of the dP/dT, control group showed 54.7$\pm$18.2% recovery rate and preconditioning group showed 78.1$\pm$15.1% recovery rate. Percent changes in heart rate and coronary flow showed no significant difference between two groups and there was no significant differences in amount of cardioplegic delivery between groups. Our data suggest ischemic preconditioning may have protective effect on recovery state after cardioplegic arrest and 2 hr ischemic storage of isolated rat heart and its mechanism is not related to the amount of the cardioplegic delivery amount.

• Journal of Chest Surgery
• /
• v.40 no.4 s.273
• /
• pp.288-291
• /
• 2007

Background: Myxoma makes up close to 50% of adult primary cardiac tumors, and this mainly occurs in the left atrium, and rarely in the right atrium or ventricle. The patients clinically present with symptoms of hemodynamic obstruction, embolization or constitutional changes. Diagnosis is currently established most appropriately with 2-D echocardiography. Surgical resection of myxoma is a safe and effective treatment, Material and Method: We reviewed our clinical experience in the diagnosis and management of 57 cases of cardiac myxoma that were seen over a 20-year period from July 1984 to July 2004. Result: The mean age of the patients was $53.5{\pm}14.0$ years (range: 12 to 76 years). There were 38 (67%) females and 19 (33%) males. The preoperative symptoms included dyspnea on exertion in 27 patients, palpitation in 4, chest pain in 9 and syncopal episode in 4. The diagnosis was made by echocardiography alone in 51, and by combination of echocardiography, CT and angiography in 6. The tumor attachment sites were the interatrial septum in 50, the mital valve annulus in 3 and the left atrial wall in cases, The tumor was excised successfully via biatriotomy in 33 (58%), left atriotomy in 15 (26%), the septal approach via right atriotomy in 3, Inverted T incision in 3 and the extended septal approach in 3. The follow-up time ranged from 1 to 229 months (mean follow-up: $84.0{\pm}71.3$ months). There were no early and late deaths and no recurrence during the follow-up period except for follow-up loss in 5 patients. Conclusion: It's concluded that excision of cardiac myxoma is curative and the long-term survival is excellent. Immediate surgical treatment was indicated because of the high risk of embolization or of sudden cardiac death. Radical tumor excision may prevent recurrences.