• Korean Circulation Journal
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• v.52 no.8
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• pp.563-575
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• 2022

Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM. SCD in HCM can be effectively prevented with implantable cardioverter-defibrillators. However, appropriate patient selection is important for primary prevention, and the 5-year SCD risk score and the presence of major SCD risk factors should be considered. Anticoagulation should be initiated in all HCM patients with atrial fibrillation regardless of the CHA2DS2-VASc score, and non-vitamin K antagonist oral anticoagulants are the first option. Symptomatic dynamic LVOT obstruction is first treated medically with negative inotropes, and if symptoms persist, septal reduction therapy is considered. The recently approved myosin inhibitor mavacamten is promising. HF in HCM is usually related to diastolic dysfunction, while about 5% of HCM patients show reduced left ventricular ejection fraction <50%, also referred to as "end-stage" HCM. Myocardial fibrosis plays an important role in the progression to advanced HF in patients with HCM. Patients who do not respond to guideline-directed medical therapy can be considered for heart transplantation. The development of imaging techniques, such as myocardial deformation on echocardiography and late gadolinium enhancement on cardiac magnetic resonance, can provide better risk evaluation and decision-making for management strategies in HCM.

• Journal of Chest Surgery
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• v.49 no.5
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• pp.350-355
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• 2016

Background: Mitral stenosis (MS) remains one of the important heart diseases. There are many factors that influence the clinical outcomes, and little is known about how left ventricular (LV) dysfunction clinically affects the prognosis of the patient with MS after mitral valve replacement (MVR). We reviewed our clinical experiences of MVR in patients with MS who had LV dysfunction. Methods: Between January 1991 and January 2013, 110 patients with MS who underwent MVR were analyzed and divided into two groups according to ejection fraction (EF). Group 1 ($EF{\leq}45%$) included 13 patients and group 2 (EF>45%) included 97 patients. Results: Thromboembolism occurred in 8 patients after MVR (group 1: n=3, 23.1%; group 2: n=5, 5.2%) and its incidence was significantly higher in group 1 than in group 2 (p=0.014). There were 3 deaths each in groups 1 and 2 during follow-up. The overall rate of cardiac-related death in group 1 was significantly higher than in group 2 (group 1: n=3, 23.1%; group 2: n=3, 3.1%; p=0.007). The cumulative survival rate at 1 and 15 years was 83.9% and 69.9% in group 1 and 97.9% and 96.3% in group 2 (p=0.004). The Cox regression analysis revealed that survival was significantly associated with postoperative stroke (p=0.011, odds ratio=10.304). Conclusion: This study identified postoperative stroke as an adverse prognostic factor in patients with MS after MVR, and a s more prevalent in patients with LV dysfunction. Postoperative stroke should be reduced to improve clinical outcomes for patients. Preventive care should be made in multiple ways, such as management of LV dysfunction, atrial fibrillation, and anticoagulation.

• Clinical and Experimental Pediatrics
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• v.55 no.9
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• pp.350-353
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• 2012

An 18-year-old boy was admitted with chest discomfort, nausea, and dyspnea at rest. At the age of 3 years, he underwent muscle biopsy and dystrophin gene analysis owing to an enlarged calf muscle and elevated serum kinase level (6,378 U/L) without overt weakness; based on the results, Becker muscular dystrophy (BMD) was diagnosed. The dystrophin gene showed deletion of exons 45 to 49. He remained ambulant and could step upstairs without significant difficulties. A chest roentgenogram showed cardiomegaly (cardiothoracic ratio, 54%), and his electrocardiogram (ECG) showed abnormal ST-T wave, biatrial enlargement, and left ventricular hypertrophy. The 2-dimensional and M-mode ECGs showed a severely dilated left ventricular cavity with diffuse hypokinesis. The systolic indices were reduced, including fractional shortening (9%) and ejection fraction (19%). Despite receiving intensive medical treatment, he died from congestive heart failure 5 months after the initial cardiac symptoms. We report a case of BMD with early-onset dilated cardiomyopathy associated with deletion of exons 45 to 49. Early cardiomyopathy can occur in BMD patients with certain genotypes; therefore, careful follow-up is required even in patients with mild phenotypes of BMD.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.299-302
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• 1999

Heart transplantation was planned for a 10-year old boy who had dilated cardiomyopathy with severe congestive heart failure and had been on dopamine for 1month. However, partial left ventriculectomy and mitral annuloplasty were performed instead, because there was no donor heart of the adequate size and the symptoms were aggravated. The clinical symptoms were markedly improved after the surgery. Comparing the postoperative echocardiographic results with the preoperative results, there were remarkable changes in the left ventricular ejection fraction(preoperative LV EF 17% to postoperative 3 months 29%, 6 months 35%, 1 year 36%) and the left ventricular end-diastolic dimension(preoperative 72 mm to postoperative 3 months 59 mm, 6 months 61 mm, 1 year 61 mm). Partial left ventriculectomy and mitral annuloplasty reduced the cardiac loading in the dilated cardiomyopathy. Partial left ventriculectomy and mitral annuloplasty may be considered as one of the alternative surgical metho s to carry over until a heart transplantation can be performed, especially for children.

• Journal of Chest Surgery
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• v.40 no.4 s.273
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• pp.280-287
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• 2007

Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

• Journal of Chest Surgery
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• v.35 no.7
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• pp.523-529
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• 2002

Background: Previous reports present that the early results of coronary artery bypass grafting (CABG) has been improving with the accumulation of surgical experience. We conducted a retrospective analysis of the patients who received CABG to evaluate the recent results of CABG. Material and Method: Between January 1996 and August 2001, 154 patients underwent CABG at Hanyang University Hospital. There were 47 patients(group I) who were operated between 1996 and 1998, and 107 patients(group II) who were operated thereafter. The preoperative diagnosis, operative procedure, mortality, and complications were analyzed retrospectively. Result: There were 35 males and 12 females in group I, and 78 males and 29 females in group II, which shows similar ratio of sexes between the two groups. The average age of patients for group I and group II was $55.9{\pm}6.2$ years and $61.0{\pm}8.0$ years, respectively, showing a significant increase in group II(p<0.05). The average left ventricular ejection fraction(LVEF) for group I and group II was $54.6{\pm}11.8$% and $56.9{\pm}13.0$%, respectively. The number of patients who had previous MI in group I and group II were 13 patients(27.7%) and 14 patients(13.1%), respectively, which shows a significant difference (p<0.05). All procedures were performed using the cardiopulmonary bypass(CPB) and moderate systemic hypothermia. Myocardial protection was achieved using intermittent hypothermic ischemia under ventricular fibrillation state or cold crystalloid cardioplegic solution for most of group I patients, whereas cold blood cardioplegic solution was used for group II patients. The mean CPB times for group I and group II were $149.2{\pm}48.7$ minutes and $113.1{\pm}30.6$ minutes, respectively. The mean aortic cross clamp times for group I and group II were $81.3{\pm}26.5$ minutes $72.2{\pm}23.9$ minutes, respectively. These figures show that CPB and aortic cross clamp times were significantly reduced in group II(p<0.05). The use of the left internal thoracic artery(LITA) was increased from 42%(20/47) for group I to 81% (87/107) for group II. The mean number of grafts also significantly increased from $2.5{\pm}0.6$ for group I to $3.0{\pm}1.1$ for group II(p<0.05). Intra-aortic balloon pump(IABP) was applied in 7 cases in group I and 17 cases in group II. Of these, 28.6%(2/7) and 52.9%(9/17) were broadly applied preoperatively in patients with LVEF<40% or congestive heart failure. The operative mortalities for group I and II were 10.6%(5/47) and 0.9%(1/107), respectively, which shows significant decrease for group II(p.0.05). Conclusion: This report suggest that CABG using CPB can recently be performed more safely in virtue of the accumulation of surgical experience with reduction in CPB and aortic cross clamp times and improved surgical techniques and myocardial protection. And we think that the optimal treatment of patients with left ventricular dysfunction associated with congestive heart failure and the extended application of IABP, especially have contributed to the reduction of operative mortality and morbidity.