• The Korean Journal of Nuclear Medicine
• /
• v.38 no.3
• /
• pp.225-232
• /
• 2004

Purpose : Transient wall motion abnormality and contractile dysfunction of the left ventricle (LV) can be observed in patients with coronary artery disease due to post-stress myocardial stunning. To understand clinical characteristics of stress induced LV dysfunction, we have compared the findings of exercise stress test, myocardial perfusion SPECT and coronary angiography between subjects with and without post-stress LV dysfunction. Materials and Methods : Among subjects who underwent exercise stress test, myocardial perfusion SPECT and coronary angiography within a month of interval, we enrolled 36 patients with post-stress LV election fraction (LVEF) was $\geq5%$ lower than rest (stunning group) and 16 patients with difference of post-stress and rest LVEF was lesser than 1 %(non-stunning group) for this study. Treadmill exercise stress gated myocardial perfusion SPECT was performed with dual head SPECT camera using 740 MBq Tc-99m MIBI and coronary angiography was also performed by conventional Judkins method. Results : Stunning group had a significantly higher incidence of hypercholesterolemia than non-stunning group(45.5 vs. 7.1%, p=0.01). Stunning group also had higher incidence of diabetes mellitus and lower incidence of hypertension, but these were not statistically significant. Stunning group had larger and more severe perfusion defect in stress perfusion myocardial SPECT than non-stunning group(extent 18.2 vs. 9.2%, p=0.029; severity 13.5 vs. 6.9, p=0.040). Stunning group also had higher degree of reversibility of perfusion defect, higher incidence of positive exercise stress test and higher incidence of having severe stenosis ($80{\sim}99%$) in coronary angiography than non-stunning group, but these were not statistically significant. In stunning group, all of 4 patients without perfusion defect had significant coronary artery stenosis and had received revascularization treatment. Conclusion : Patients with post-stress LV dysfunction had larger and more severe perfusion defect and severe coronary artery stenosis than patients without post-stress LV dysfunction. All of the patients without perfusion defect in stunning group had significant coronary artery stenosis and needed revascularization. Therefore, we suggest that invasive diagnostic procedures and therapeutic interventions might be needed in patients with post-stress LV dysfunction.

• 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.

• Nuclear Medicine and Molecular Imaging
• /
• v.43 no.2
• /
• pp.112-119
• /
• 2009

Purpose: This study was performed to investigate the clinical significance of reverse redistribution(RR) phenomenon detected on delayed Tc-99m tetrofosmin myocardial single photon emission computed tomography(SPEG) in patients with acute myocardial infarction after revascularization. Materials and Methods: A Tc-99m tetrofrosmin myocardial SPECT was performed in 67 consecutive patients after revascularization for acute myocardial infarction. Myocardial SPECT imaging was performed for early imaging at 40 min and for delayed imaging at 180 min after reinjection at myocardial stress. Regional myocardial uptakes were scored by 4-point scoring in the left ventricular wall divided into 17 segments. Reverse redistribution was defined as an increase of more than 2 point in the activity score on the delayed image. Follow-up myocardial SPECT and coronary angiography(CAG) were performed 9 months later. Results: On myocardial SPECT performed following revascularization, RR was observed in 100 of all 319 segments(31%) and in 43 patients(64%). The abnormalities of perfusion and regional wall motion were more severe in the patients with RR compared to those without RR(p<0.05). On follow-up myocardial SPECT, the myocardial perfusion, regional wall motion, and myocardial thickness were significantly improved in the patients with RR(p<0.05) however, these changes were not significant in those without RR. There was no significant difference between the patients with RR and those without RR in the occurrence of restenosis on CAG. Conclusions: In patients with acute myocardial infarction, the regions showing the RR phenomenon on delayed Tc-99m tetrofosmin SPECT may reflect viable myocardium and indicate recovery of salvaged myocardium.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.103-108
• /
• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Radiation Oncology Journal
• /
• v.20 no.2
• /
• pp.93-99
• /
• 2002

Purpose : We intended to decrease late CNS reaction after radical radiotherapy for an intracranial germinoma by using combined neoadjuvant chemotherapy and involved-field radiotherapy. The efficacy in terms of its acute toxicity and short-term relapse patterns was analyzed. Materials and Methods : Eighteen patients were treated with combined neoadjuvant chemotherapy and radiotherapy between 1995 and 2001. The chemotherapy regimen used was the Children's Cancer Group (CCG) 9921A (cisplatin, cyclophosphamide, VP-16, vincristine) for 5 patients younger than 16 years, BEP (bleomycin, VP-16, cisplatin) for 12 patients, and EP (VP-16, cisplatin) for 1 patient. The radiotherapy covered the whole craniospinal axis for 5 patients, the whole brain for 1, and the partial brain (involved field) for 12. the primary lesion received tumour doses between 3,960 and 5,400 cGy. Results : The male to female ratio was 16:2 and the median age was 16 years old. The tumors were located in the pineal gland in 12 patients, in the suprasellar region in 1, in the basal ganglia In 1, in the thalamus in 1. Three patients had multiple lesions and ventricular seedings were shown at MRI. In 3 patients, tumor cells were detected in the cerebrospinal fluid and MRI detected a spinal seeding in 2 patients. The response to neoadjuvant chemotherapy was complete remission in 5 patients, partial remission in 12, and no response in 1. However, after radiotherapy, all except 1 patient experienced complete remission. The toxicity during or after chemotherapy greater than or equal to grade III was remarkable; hematologic toxicity was observed in 11 patients, liver toxicity in none, kidney toxicity in none, and gastrointestinal toxicity in one. One patient suffered from bleomycin-induced pneumonitis. Radiotherapy was therefore stopped and the patient eventually died of respiratory failure. The other 17 are alive without any evidence of disease or relapse during an average of 20 months follow-up. Conclusion : A high response rate and disease control was experienced, which was the same as observed other studies and the morbidity from chemotherapy-induced toxicity was similar. With these results, the results from adjuvant chemotherapy and involved-field radiotherapy cannot be concluded to be equal to those from extended-field radiotherapy. The long term follow-up study on later complications are required in order to draw definite conclusions on the optimal management with minimum side effects.

• 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.37 no.10
• /
• pp.833-838
• /
• 2004

Background: Despite the excellent early results after the repair of congenital bicuspid aortic valve (BAV) disease, the mid-term durability of the repaired valve has still controversies. Material and Method: To evaluate the mid-term results of BAV repair, retrospective review of medical records and echocardiographic data were done. Between 1994 and 2003, twenty-two patients underwent reparative procedure for either regurgitant or stenotic congenital bicuspid aortic valve (BAV). Result: Mean age was $41\pm14$ years with male predominance (Male=17, Female=5). The pathophysiologies of the BAV were regurgitation-dominant in 20 (91%) and stenosis-dominant in 2 (9%) cases. Various repair techniques were used for raphe, prolapsed leaflet, thickened leaflet, and commissures; 1) release of raphe in 19 (86%), 2) wedge resection and primary repair in 11 (50%), pericardial patch reinforcement after plication of the leaflet in 6 (27%), and plication of the leaflet in 3 (14%), 3) slicing of thickened leaflet was used in 12 (55%) cases, 4) commissuroplasty in 8 (36%), and commissurotomy in 6 (27%) cases. There was no in-hospital mortality. During the mean follow-up of $38\pm17$ months, one patient underwent aortic valve replacement after developing acute severe regurgitation from dehiscence of the suture on postoperative 2 months. New York Heart Association functional class was improved from $1.9\pm0.6$ to $1.2\pm0.5$ (p<0.01). Left ventricular end-systolic and diastolic dimension (LVESD/LVEDD) were also improved from $45\pm9$ and $67\pm10$ to $37\pm10$ and $56\pm10,$ respectively (p<0.01). The grade of aortic regurgitation (AR) was improved from preoperative $(3.1\pm1.2)$ to post-bypass $(0.9\pm0.7).$ However, the grade at last follow-up $(1.7\pm1.1)$ was deteriorated during the follow-up period (p<0.01). Freedom from grade III and more AR at one, three, and four year were 89.7%, 89.7%, and 39.9% respectively. Conclusion: Midterm clinical result of the BAV repair was favorable. But, the durability of the repaired valve was not satisfactory.

• 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.39 no.6 s.263
• /
• pp.449-455
• /
• 2006

Background: Female sex was known to be a risk factor for mortality after coronary bypass grafting (CABG), and women showed higher in-hospital mortality than men. Material and Method: Between 1992 and 1996, 147 consecutive patients (98 men and 49 women) undergoing CABG were included in the study. Most patients had undergone CABG with left internal thoracic artery and saphenous vein under cardiopulmonary bypass. We examined the influence of gender on survival after CABG and looked for risk factors for survival. Result: There was no in-hospital mortality in women, but 3 death (3.0%) in men. During the mean follow-up period of $138.5{\pm}23.0$ months, mortality was lower in women than in men (20.4% vs 44.9%, p=0.004), and the most common cause of death in women was chronic renal failure (40%). Survival in women at 1, 5, 10, and f4 years was 100%, $98.0{\pm}2.0%,\;81.2{\pm}5.6%,\;and\;78.4{\pm}6.1%$, respectively, which was better than in men (p=0.004). Although preoperative left ventricular ejection fraction was higher in women than in men, this did not affect early and long-term survival difference between two sexes (p=0.15). Risk factor for long-term survival in women was diabetes (p=0.033) and in men number of diseased coronary artery (p=0.006). Conclusion: Long-term survival after CABG was better in women than men. Risk factor for long-term survival in women was morbid disease rather than cardiac disease.

• Journal of Chest Surgery
• /
• v.35 no.5
• /
• pp.356-364
• /
• 2002

Background: This study was undertaken to investigate the outcome of composite graft aortic root replacement using coronary button reimplantation technique for the treatment of aneurysms of the ascending aorta involving the aortic root. Material and Method: Between April 1995 and September 2001, 54 patients having aortic root replacement with a composite valve graft using direct coronary button reimplantation were reviewed retrospectively. Left ventricular dysfunction was present in 14 patients(25.9%), aortic regurgitation in 48(89%), and Marfan's syndrome in 17(31.5%). The indications for operation were annuloaortic ectasia in 29 patients(53.7%), aortic dissection in 11(20.4%), aneurysms of the ascending aorta involving aortic root in 12(22.2%), and aortitis in 2(3.7%). Six patients(11.1%) had previous cardiac or ascending aortic operations. Concomitant procedures were arch replacement in 21 patients(38.9%), coronary artery bypass graft in 7(13%), mitral valve repair or replacement in 4(7.4%), and others in 6. The mean time of circulatory arrest, total bypass, and aortic crossclamp were 18 $\pm$ 9 minutes, 177 $\pm$ 42 minutes, and 127 $\pm$ 31 minutes, respectively. Result: There was 1 early death(1.9%). Mean follow-up was 24.6$\pm$ 19.5 months. There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage. The Kaplan-Meier survival rate was 98.0 $\pm$ 2.0% and 93.1 $\pm$ 5.1% at 1 and 6 years, respectively. Two patients required reoperation owing to a false aneurysm at the root anastomosis site and a malfunction of prosthetic aortic valve(3.8%). Staged operation for dissection of the remaining thoracoabdominal aorta was performed in 1 patient. The freedom rate from reoperation was 97.8 $\pm$ 2.0% and 65.3 $\pm$ 26.7% at 1 and 6 years, respectively.