• Journal of Veterinary Clinics
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• v.41 no.2
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• pp.106-111
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• 2024

A 4-year-old neutered female domestic shorthair cat weighing 5.1 kg was referred to Jeju National University Hospital with acute onset respiratory distress, weakness, and anorexia. The patient had a history of stressful antecedent events that involved bullying by a newly introduced cat. Thoracic radiography and echocardiography revealed a stage C hypertrophic cardiomyopathy phenotype based on the American College of Veterinary Internal Medicine classification system with pulmonary edema, pleural effusion, and pericardial effusion at the same time. The patient was treated with furosemide, pimobendan, and rivaroxaban. Pericardiocentesis was performed because pericardial effusion was identified. Reevaluation after 30 days revealed a normal respiratory rate on physical examination, normal cardiac shape on thoracic radiographs, and normal cardiac measurements on echocardiography. The patient was tentatively diagnosed with transient myocardial thickening (TMT) and all medications were discontinued. Six months after the initial hospitalization, the cat continued to do well without any clinical signs or left ventricular wall thickening. This case is the first report describing feline TMT in Korea. Moreover, it involves a rare case in which pulmonary edema, pleural effusion, and pericardial effusion, which induce cardiac tamponade, occurred simultaneously due to TMT-related congestive heart failure.

• Korean Journal of Radiology
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• v.23 no.3
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• pp.298-307
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• 2022

Objective: This study aimed to evaluate the effect of implementing the consensus statement from the Asian Society of Cardiovascular Imaging-Practical Tutorial 2020 (ASCI-PT 2020) on the reliability of cardiac MR with late gadolinium enhancement (CMR-LGE) myocardial viability scoring between observers in the context of ischemic cardiomyopathy. Materials and Methods: A total of 17 cardiovascular imaging experts from five different countries evaluated CMR obtained in 26 patients (male:female, 23:3; median age [interquartile range], 55.5 years [50-61.8]) with ischemic cardiomyopathy. For LGE scoring, based on the 17 segments, the extent of LGE in each segment was graded using a five-point scoring system ranging from 0 to 4 before and after exposure according to the consensus statement. All scoring was performed via web-based review. Scores for slices, vascular territories, and total scores were obtained as the sum of the relevant segmental scores. Interobserver reliability for segment scores was assessed using Fleiss' kappa, while the intraclass correlation coefficient (ICC) was used for slice score, vascular territory score, and total score. Inter-observer agreement was assessed using the limits of agreement from the mean (LoA). Results: Interobserver reliability (Fleiss' kappa) in each segment ranged 0.242-0.662 before the consensus and increased to 0.301-0.774 after the consensus. The interobserver reliability (ICC) for each slice, each vascular territory, and total score increased after the consensus (slice, 0.728-0.805 and 0.849-0.884; vascular territory, 0.756-0.902 and 0.852-0.941; total score, 0.847 and 0.913, before and after implementing the consensus statement, respectively. Interobserver agreement in scoring also improved with the implementation of the consensus for all slices, vascular territories, and total score. The LoA for the total score narrowed from ± 10.36 points to ± 7.12 points. Conclusion: The interobserver reliability and agreement for CMR-LGE scoring for ischemic cardiomyopathy improved when following guidance from the ASCI-PT 2020 consensus statement.

• Journal of Veterinary Clinics
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• v.23 no.3
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• pp.349-354
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• 2006

A 9-month-old, female Miniature Pinscher(MP) dog weighing 1.97kg was presented because of periodic syncopal episode for 5 months. This case was diagnosed as primary dilated cardiomyopathy based on respiratory distress history, weak femoral pulse, generalized cardiomegaly, pulmonary edema, marked dilation of left atrium(LA) and left ventricle(LV), decreased wall thickness of LV and interventricular septum(IVS), increased EPSS in echocardiography, and young age of onset in the absence of other cardiovascular disorders. The patient was stabilized by application of diuretics(Furosemide, 2 mg/kg, SC, q 1 hr) and venodilator(Nitroglycerine patch, 0.5 mg/kg, q 12 hrs). Clinical signs were improved with medical management of positive inotropic vasodilator(Pimobendan, 0.2 mg/kg, PO, q 12 hrs) and angiotensin-converting enzyme(ACE) inhibitor(benazepril, 0.5 mg/kg, PO, q 12 hrs), potassium gluconate gel(2 mEq/dog, PO, q 12 hrs) and, L-carnitine(50 mg/kg, PO, q 12 hrs). The dog still maintains stable clinical status 10 months after the first visit. We report the rare case of DCM in small breed dog, which corresponds to the diagnosis and treatment of typical DCM in large breed dog.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.876-884
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• 1997

Fourteen patients underwent orthotopic heart transplantation between March 1994 and May 1996 in Seoul National University Hospital. There were 9 male and 5 female patients, and the mean age was 40.8 $\pm$ 12.4 years ranged from 12 to 56 years. All patient were in NYHA Fc III or IV preoperatively. The underlying heart diseases were dilated cardiomyopathy in 11 and restrictive cardiomyopathy in 3. The mean age of donors was 24.9$\pm$ 10.2 years and the causes of the brain death were head trauma by traffic accidents in 8, subarachnoid hemorrhage in 2, 1 asphyxia, 1 fall down injury, 1 brain tumo , and 1 drowning, respectively The blood type was identical in 11, compatible in 2, and incompatible in 1 patient. The direct bicaval anastomosis technique was used in 11 cases, and standard right atrial anastomosis was done in the remaining 3 cases. The graft ischemic time was 158$\pm$44 minutes ranged 94 to 220 minutes. There were two hospital deaths(14.3%). The causes of deaths were 1 right ventricular failure followed by suspected cyclosporine induced hemolytic uremic syndrome and rejection, and 1 delayed massive bleeding, probably from rupture of the anastomotic pseudoaneurysm, respectively. The follow-up duration was 16$\pm$9 months(3 to 28 months). There was one late death(8.3%). All the other patients were in NYHA Fc I except one patient who was in hospital because of the acute rejection. The actuarial survival rates including hospital deaths were 93.7% at 1 month, 86.9% at 6 months, and 77$\pm$12% at 2 years. Conclusively, heart transplantation is the good strategy for the management of end stage heart disease with acceptable operative mortality and early follow-up results.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.329-334
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• 2008

Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is expected. Kwashiorkor has been known as a common disease of poor countries. To date, in fact, there has been no report of kwashiorkor leading to death in technically advanced countries. We here report a fatal case of a baby girl admitted with kwashiorkor. She had been fed only with cereal grain mixed with juice, without any protein supplement, for 2 months. This diet was deficient not because of poverty, but due to the illiteracy of her parents. The patient suffered from diarrhea, whole body edema, hypothermia, and dermatitis. Laboratory findings revealed an immune-deficient state featuring leukopenia and decreased immunoglobulin. Blood and urine cultures revealed Alcaligenes Xylosoxidans growth. The patient was fed frequent small amounts of protein-containing formula and intravenous albumin and micronutrients were administered for nutritional support. She was also treated with intravenous immunoglobulin and antibiotics in order to control infection. Nevertheless, she developed dilated cardiomyopathy and multi-organ failure and died. We review this case in light of the literature.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.336-342
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• 2002

Background: Recently, cell transplantation has been extensively investigated to improve heart function in dysfunctional heart. This study was designed to compare the effects of smooth muscle cells (SMC) and heart cells (HC) transplantation in dilated cardiomyopathic hamsters. Material and Method: HC and SMC were isolated from heart and ductus deferens of BIO 53.58 hamsters, and cultured for transplantation. HC and SMC or culture medium were transplanted into the left ventricle of 17 weeks old adult hamsters in HC transplanted (HCTx), SMC transplantation (SMCTX), and control groups (Con) (N = 10 each). Cyclosporine (5 mg/Kg) was administered subcutaneously for HCTx. Sham operated hamsters (N=10) underwent the surgery but did not receive an injection. At 4 weeks after transplantation, heart function was evaluated in all groups using a Langendorff perfusion apparatus. Result: Histology showed severe focal myocardial necrosis in all groups. HCTx and SMCTx formed huge muscle tissue in dilated myocardium. SMCTx and HCTx had better heart function than Con and sham (p<0.01). And SMCTx had better peak systolic pressure (p<0.05) antral developed pressure (p<0.05) than HCTx. But sham and Con did not any statistical make difference. Conclusion: SMCTx and HCTx formed muscle tissue and improved ventricular function in hamsters with dilated cardiomyopathy And SMCTx showed better heart function in peak systolic pressure and developed pressure than HCTx.

• Investigative Magnetic Resonance Imaging
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• v.16 no.2
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• pp.189-194
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• 2012

We report a case of cardiac lymphoma in a 40-year-old man, who had a mediastinal mass which was diagnosed as sclerosing mediastinitis pathologically. The mediastinal mass caused right pulmonary arterial stenosis. The patient developed myocardial hypertrophy and echocardiography showed restrictive physiology and severely decreased left ventricle ejection fraction, 6 months later. MRI showed global left ventricular myocardial hypertrophy and diffuse late gadolinium hyperenhancement after administration of contrast material. Thus, non-ischemic cardiomyopathy was suspected on MRI. However, pathology confirmed the myocardial abnormality as lymphoma after myocardial biopsy. Because a basal part of the left ventricle and global subendocardial myocardium were not involved on contrast-enhanced delayed MRI, the MRI abnormalities could be differentiated from amyloidosis and other myocardial diseases. The peculiar non-mass forming diffuse hypertrophy pattern of cardiac lymphoma has not been known in the MRI literature.

• The Korean Journal of Physiology and Pharmacology
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• v.5 no.5
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• pp.397-405
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• 2001

The ryanodine receptor, a $Ca^{2+}$ release channel of the sarcoplasmic reticulum (SR), is responsible for the rapid release of $Ca^{2+}$ that activates cardiac muscle contraction. In the excitation-contraction coupling cascade, activation of SR $Ca^{2+}$ release channel is initiated by the activity of sarcolemmal $Ca^{2+}$ channels, the dihydropyridine receptors. Previous study showed that the relaxation defect of diabetic heart was due to the changes of the expressional levels of SR $Ca^{2+}$ATPase and phospholamban. In the diabetic heart contractile abnormalities were also observed, and one of the mechanisms for these changes could include alterations in the expression and/or activity levels of various $Ca^{2+}$ regulatory proteins involving cardiac contraction. In the present study, underlying mechanisms for the functional derangement of the diabetic cardiomyopathy were investigated with respect to ryanodine receptor, and dihydropyridine receptor at the transcriptional and translational levels. Quantitative changes of ryanodine receptors and the dihydropyridine receptors, and the functional consequences of those changes in diabetic heart were investigated. The levels of protein and mRNA of the ryanodine receptor in diabetic rats were comparable to these of the control. However, the binding capacity of ryanodine was significantly decreased in diabetic rat hearts. Furthermore, the reduction in the binding capacity of ryanodine receptor was completely restored by insulin. This result suggests that there were no transcriptional and translational changes but functional changes, such as conformational changes of the $Ca^{2+}$ release channel, which might be regulated by insulin. The protein level of the dihydropyridine receptor and the binding capacity of nitrendipine in the sarcolemmal membranes of diabetic rats were not different as compared to these of the control. In conclusion, in diabetic hearts, $Ca^{2+}$ release processes are impaired, which are likely to lead to functional derangement of contraction of heart. This dysregulation of intracellular $Ca^{2+}$ concentration could explain for clinical findings of diabetic cardiomyopathy and provide the scientific basis for more effective treatments of diabetic patients. In view of these results, insulin may be involved in the control of intracellular $Ca^{2+}$ in the cardiomyocyte via unknown mechanism, which needs further study.

• Kosin Medical Journal
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• v.33 no.3
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• pp.328-336
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• 2018

Objectives: Electrocardiograhy (ECG) is the first step in hypertrophic cardiomyopathy (HCMP) diagnosis. For various reasons, the T wave inversion (TWI) and ECG change with time and HCMP is not easy to diagnosis. The aim of this retrospective study was to investigate the association between TWI on ECG and apical HCMP. Methods: A total of 4,730 ECGs presenting TWI from January 2011 to March 2013 in Pusan National University Hospital were enrolled. 133 patients who were examined by both echocardiography and coronary angiogram were analyzed. Patients were divided into two groups: Group A (TWI ${\geq}$ 10 mm) and Group B (5 mm ${\leq}$ TWI < 10 mm). HCMP is defined by a wall thickness ${\geq}15mm$ in one or more LV myocardial segments. Apical HCMP is defined to be hypertrophy that is confined to LV apex. The patients who had ECGs with at least one month interval were divided 3 groups: Normal T wave, Abnormal T wave, and Persistent TWI. The prevalence of Apical HCMP and coronary artery disease (CAD) was reviewed among the three groups. Results: In this study there were a total 133 patients, with patients divided into Group A which had 15 patients and Group B which had 118 patients. Among the 23 patients with apical HCMP, three patients were Group A and twenty patients were Group B (P = 0.769). Regarding constancy of TWI, persistent TWI group was higher in apical HCMP than in other groups (P = 0.038). CAD had no difference between groups (P = 0.889). Conclusions: T wave negativity was not associated with incidence of apical HCMP. However, apical HCMP was diagnosed more frequently in patients with persistent TWI. Further follow up echocardiographic study is needed to evaluate the progression of apical HCMP in patients with TWI.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.21 no.1
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• pp.22-27
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• 2021

Propionic acidemia (PA) is an inherited autosomal recessive disorder, due to the deficiency of propionyl-CoA carboxylase (PCC). PCC is the enzyme which catalyzes the conversion of propionyl-CoA to D-methylmalonyl-CoA, and it is critical for the metabolism of amino acids, odd-chain fatty acids, and side chains of cholesterol. The clinical manifestations present mostly at the neonatal period with life-threatening metabolic acidosis and hyperammonemia. Here, we described a case of a 16-year-old Korean boy with late-onset PA who presented with embolic cerebral infarction due to dilated cardiomyopathy (DCMP) with left ventricular noncompaction. And he has family history of sudden cardiac death, so we performed metabolic screening and genetic tests. Elevated levels of 3-hydroxypropionic acid, methylcitric acid and propionylglycerine were detected in urine. Plasma acylcarnitine profile showed elevated propionylcarnitine (C3). Diagnosis of PA was confirmed by genetic analysis, which revealed compound heterozygous mutations, c.[1151T>G] (p.[Phe384Cys]) and c.[1228C>T] (p.[Arg410Trp]) in PCCB gene. His heart function is in improving state and the results of biochemical analysis are stable with heart failure medication and metabolic managements. We present a case of patient without episodes of metabolic decompensation who manifests DCMP as the first symptom of PA.