• Journal of the Korean Society of Radiology
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• v.81 no.2
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• pp.302-309
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• 2020

Fabry disease is a rare X-linked metabolic disorder that is characterized by the accumulation of glycosphingolipids in various organs, resulting from the deficiency of alpha-galactosidase A. Cardiac involvement is relatively common; myocardial inflammation, left ventricular hypertrophy, and myocardial fibrosis secondary to abnormal lipid deposition in myocytes are often observed. Hence, the diagnosis of cardiac involvement is crucial for evaluating patient prognosis. Cardiac MRI is the standard technique for measuring the function, volume, and mass of the ventricles. It is also useful for myocardial tissue characterizations. The evaluation of native myocardial T1 values can facilitate early diagnosis of cardiac involvement, while measurements of left ventricular myocardial mass can be used to monitor treatment outcomes, in patients with Fabry disease. Consequently, cardiac MRI can provide useful information for diagnosing, monitoring, and treating patients with Fabry disease.

• Nuclear Medicine and Molecular Imaging
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• v.40 no.3
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• pp.155-162
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• 2006

Purpose: Diabetes mellitus (DM) is a critical disease with higher rates of cardiovascular morbidity and mortality due to myocardial ischemia and infarction. There is glowing interest in how to determine high-risk patients who are candidates for screening testing. This study was performed to evaluate the incidence of coronary artery disease (CAD) in diabetic patients detected by Tc-99m MIBI myocardial perfusion SPECT (MPS) and to assess risk factors of CAD and cardiac hard events. Subjects and Methods: 203 diabetic patients (64 male, mean age $64.1{\pm}9.0$ years) who underwent MPS were included between Jan 2000 and July 2004. Cardiac death and nonfatal myocardial infarction (MI) were considered as hard events, and coronary angioplasty and bypass surgery >60 days after testing were considered as soft events. The mean follow-up period was $36{\pm}18$ months. Patients underwent exercise (n=6) or adenosine stress (n=197) myocardial perfusion SPECT. Results: Perfusion defects on MPS were detected in 28.6% (58/203) of the patients. There was no cardiac death but 11 hard events were observed. The annual cardiac hard event rate was 1.1%. In univariate analysis of clinical factors, typical anginal pain, peripheral vascular disease, peripheral polyneuropathy, and resting ECG abnormality were significantly associated with the ocurrence of hard events. Anginal pain, peripheral vascular disease, and resting ECG abnormality remained independent predictors of nonfatal MIs with multivariate analysis. Abnormal SPECT results were significantly associated with high prevalence of hard events but not independent predictors on uni- and multivariate analyses. Conclusion: Patients who were male, had longer diabetes duration (especially over 20 years), peripheral vascular disease, peripheral polyneuropathy, or resting ECG abnormality had higher incidence of CAD. Among clinical factors in diabetic patients, typical angina, peripheral vascular disease, peripheral polyneuropathy, and resting ECG abnormality were strong predictors of hard events.

• Journal of Chest Surgery
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• v.30 no.12
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• pp.1205-1213
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• 1997

This study reviewed useful aspects of the intraoperative transesophageal echocardiography among the patients in whom heart surgery were undertaken between January 1996 and July 1996 at St.Pauls hospital, Medical College of Catholic University, Seoul, Korea. During that period, 61 patients were operated on because of valvular heart disease(25 patients), coronary artery disease(22 patients), congenital heart disease(13 patients), and combined coronary artery disease and valvular heart disease(1 patient). Two patients(1 redo-VSD and 1 valvular heart diease) needed repeated aortic cross clamping and complementary procedures because of incomplete initial procedures. There was no incidence of air embolism. We could observe significant relationship of cardiac output monitoring methods either by thermodilution technique and transesophageal echocardiography by linear regression analysis(p<0.001). We tested myocardial response(percentage of systolic wall thickness, PSWT) with low dose dobutamine challenge to predict post-CABG myocardial perfusion. And the test showed statistically significant resp.onse(sensitivity 76%, specificity 94.7%, positive predictive value 95%, negative predictive value 75%). These results suggest that cardiac surgeon could draw more benefits by intraoperative transesophageal echocardiography.

• Pediatric Infection and Vaccine
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• v.27 no.3
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• pp.184-189
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• 2020

Lyme disease is a common vector-borne disease caused by Borrelia burgdorferi. Erythema migrans represents the most common manifestation during the early phase of this disease; however, systemic manifestations involving the nervous system, joints, or heart are known to occur. We report a case of Lyme disease accompanied by cardiac complications in a 13-year-old Korean male adolescent. The patient developed annular erythematous lesions on his lower extremities after a field trip during his visit to Connecticut, USA, for a boarding school camp, and his skin lesions were consistent with erythema migrans. Indirect immunofluorescence assay and Western blot analysis for Lyme immunoglobulin M showed positive results. Electrocardiography revealed a first-degree atrioventricular block, and he was diagnosed with Lyme carditis and received a 4-week course of oral doxycycline. Follow-up electrocardiography performed a week later revealed normal findings, and the patient showed an uneventful recovery. Lyme carditis often presents as an asymptomatic heart block that can progress to an advanced heart block; however, this condition is reversible with appropriate antibiotic treatment.

• Journal of Chest Surgery
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• v.30 no.1
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• pp.92-96
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• 1997

The prognosis of Behfet's disease characterized by recurrent orogenltal ulcers and ocular and skin lesions depends upon the complications in the central nervous system, the gastrointestinal tract And the vascular system. Cardiac involvement, especially aortic regurgitation, is quite uncommon and hemodynamic instability is usually treated with ope heart surgery. But serious postoperative complications had been reported in many cases, which are prosthetic valve detachment, paravalvular leakage, conduction disturbance, and false aneurysm. Many efforts to prevent the complications have been made such as application of cryopreseved homograft. We have described an experience of root replacement with homograft in d 39 year-old male patient for prosthetic valve detachment because of Behfet's aorlitis with a review of the literatures regarding treatment, complication, and prognosis.

• Journal of Chest Surgery
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• v.29 no.2
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• pp.219-222
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• 1996

A 34-year-old man was admitted to the hospital because of ascites, abdominal fullness. computed tomography and cavography revealed inferior vena cavil occlusion just above the hepatic vein and diagnosed as Budd-Chiari syndrome. conservative medical therapy failed to control the symptoms produced from both portal hypertension and versa caval stasis. Therefore, under extracorporeal circulation with moderate hypothermia and normal cardiac contraction, membranoto y and inferior vena casa venoplasty with Gore-tex (10mm) was performed. Postoperatively, physical examination revealed oral ulceration, subcutaneous thrombophlebitis, folliculitic lesions. uveitis And increased reactivity of the skin to needle punctures. 10 month later, superior vena ciiva obstruction symptom was found. Hehcet's disease was diagnosed.

• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.93-97
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• 2008

Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition, or unusual symptoms such as diabetes mellitus develops in a cyanotic patient with congenital heart disease, pheochromocytoma must be ruled out. We report two patients presenting with cyanotic single-ventricle heart disease with pheochromocytoma.

• Journal of Genetic Medicine
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• v.6 no.2
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• pp.161-165
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• 2009

Pompe disease (glycogen storage disease type II) is an autosomal recessive disorder caused by deficiency of acid-${\alpha}$-glucosidase (GAA) resulting in lysosomal glycogen accumulation in multiple tissue, particularly cardiac and skeletal muscle. The classic infantile form of Pompe disease is characterized by marked cardiomegaly, respiratory failure and severe generalized hypotonia. Most patients die from cardiorespiratory failure or respiratory infection within the first year or two of life without treatment. A "non-classic" phenotype presents with less severe clinical feature and slow progression of disease. We report two patients with non-classic infantile Pompe disease from one family manifested hypertrophic cardiomyopathy and progressive proximal weakness.

• Clinical and Experimental Pediatrics
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• v.53 no.3
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• pp.380-391
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• 2010

Purpose : Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. Methods : On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. Results : During the winter season, RSV caused 20-0% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. Conclusion : To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1411-1416
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• 2002

Purpose : Although the use of intravenous gamma-globulin(IVGG) in Kawasaki disease(KD) is effective in reducing clinical symptoms and coronary artery complications, 20-30% of patients have persistent or recrudescent fever and ongoing clinical symptoms. In these patients, the additional infusion of IVGG is considered. The authors studied the characteristics of patients who received IVGG retreatment, and compared them with the patients who did not need IVGG retreatment, for determination of IVGG retreatment. Methods : We reviewed the medical records of 117 KD patients who could be followed up at least six months. We studied the conventional laboratory findings, electrocardiogram(EKG), signal averaged ECG(SAECG) and echocardiogram. Results : Twenty three patients had early cardiac complications during the six months of follow-up. Four patients had late cardiac complications after six months. The early cardiac complication rate was higher in the IVGG retreatment group than the single infusion group(P<0.0001). The late complication rate was also higher in the retreatment group(P<0.0001). The patients who received methyl-prednisolone(m-PD) pulse therapy had much higher rates of early and late cardiac complications than those who received a single IVGG infusion. Among the clinical data and laboratory findings, only CRP increased significantly in patients who have had the cardiac complications. The IVGG retreatment group had increased CRP than the single infusion group. Conclusion : The patients with increased initial CRP may have an increased incidence of complications and an increased possibility of IVGG retreatment. We thought that retreated KD patients might have inflammations severe enough to need high dose IVGG as shown by high CRP levels, and IVGG retreatment could not prevent coronary artery lesions sufficiently.