• Title/Summary/Keyword: Cardiac hypertrophy

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A Case of Complicated Congenital Cardiac Anormaly Occurred in Shepherd Mongrel Dog (세퍼드 잡종견에 발생한 복합적 선천성 심장기형의 일예)

  • Kim Duck-Hwan;Kim Kyo-Joon;Kwon Oh-Deog;Jun Moo-Hyung;Park Chung-Hee;Lee Byung-Chul;Choi Hi-In;Park Kyung-Soo
    • Journal of Veterinary Clinics
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    • v.5 no.2
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    • pp.111-118
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    • 1988
  • One four-month-old female shepherd mongrel dog with chief complaints of abdominal distension and dysponea was referred. The authors performed physical examination, hematological examination, X-ray examination, exploratory laparotomy, electrocardiography and angiography as antemortem investigation in addition to postmortem examination. This patient revealed complicated congenital cardiac anormaly including patent ductus arteriosus, both atrial and ventricular hypertrophy, distension in the base of the pulmonary artery, pulmonary stenosis, aortic insufficiency, patent foramen ovate and coronary arteriovenous fistula.

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The Role of Cardiac MRI in the Diagnosis of Fabry Disease (파브리병에서의 심장 자기공명영상의 역할)

  • Yoo Jin Hong;Young Jin Kim
    • 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.

A Review of Total Correction in 48 Cases of Tetralogy of Fallot (Fallot 사징증(四徵症) 외과적(外科的) 완전교정(完全矯正)에 대한 임상적(臨床的) 고찰(考察))

  • Suh, Kyung Phill
    • Journal of Chest Surgery
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    • v.9 no.2
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    • pp.198-206
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    • 1976
  • A Total of Forty eight patients underwent open-heart surgery for correction of tetralogy of Fallot at the Seoul National University Hospital from January 1974 to October 1976, with an overall survival rate of 77 per cent. Operative mortality varied according to severity of the lesion, age of the patient, nature of previous surgical treatment and presence or absence of an outflow tract patch across the pulmonary valve ring. Eleven patients died in the early postoperative period and thirty seven patients were discharged from the hospital alive. A patch of the right ventricular outflow tract and pulmonary annulus was required to relieve pulmonic stenosis in 24 patients. There were 10 deaths in this group (42%) as compared to 1 death in the group of 24 patients who were corrected without a patch. Operative mortality was especially higher when an inlay patch was placed across the pulmonary valve ring. This may be related to the possibly greater anatomic severity of these cases and to the longer operating time when a patch was used. The electrocardiogram showed right ventricular hypertrophy in 35 cyanotic patients. Intraventricular conduction was normal in 34 patients before operation. It was normal postoperatively in only 5 of 34 patients in this group who survived surgery. Complete right bundle branch block appeared at operation in 21 patients, and 8 patients developed incomplete right bundle branch block. Major causes of death were progressive cardiac failure (4), Complete atrioventricular dissociation (3), bleeding (2), cardiac tamponade (1), and sudden cardiac arrest (1)

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A Study on MCG Imaging (MCG 영상진단 검사에 관한 연구)

  • Kim, Jong-Gyu
    • Korean Journal of Clinical Laboratory Science
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    • v.38 no.2
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    • pp.135-140
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    • 2006
  • Magnetocardiography (MCG) is the measurement and analysis of the magnetic component of the electro-magnetic field of the human heart, usually conducted externally, using extremely sensitive devices such as a Superconducting Quantum Interference Device (SQUID). MCG is a totally noninvasive method, it uses neither radiation nor ultrasonics. The magnetic activity of the heart is registered from outside the thorax. MCG has a very high sensitivity and a high spatial resolution for very a small, local myocardial current. In comparison to the electrical signals measured by an ECG, the magnetic signal does not disturb the boundaries of tissues with different electrical properties. MCG measures the myocardial function rather than describing the morphology. MCG is a relatively new technique that promises good spatial resolution and extremely high temporal resolution, thus complementing other heart activity measurement techniques such as Electrocardiography (ECG). The clinical uses of MCG are in detecting various cardiac disorders including myocardial infarction, ventricular hypertrophy, ventricular conduction defects, Wolff-Parkinson-White (WPW) syndrome, sudden cardiac death and fetal magnetocardiography. Magnetocardiography may be used alone or together with electrcardiography for the measurement of spontaneous or overloaded activity and for research or clinical purposes.

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Gene Expression Changes in Peripheral Blood Mononuclear Cells from Cynomolgus Monkeys Following Astemizole Exposure

  • Park, Han-Jin;Seo, Jeong-Wook;Oh, Jung-Hwa;Lee, Sun-Hee;Lee, Eun-Hee;Kim, Choong-Yong;Yoon, Seok-Joo
    • Molecular & Cellular Toxicology
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    • v.4 no.4
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    • pp.323-330
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    • 2008
  • Surrogate tissue analysis incorporating -omics technologies has emerged as a potential alternative method for evaluating toxic effect of the tissues which are not accessible for sampling. Among the recent applications, blood including whole blood, peripheral blood lymphocytes and peripheral blood mononuclear cells (PBMCs) was suggested as a suitable surrogate tissue in determining toxicant exposure and effect at the pre- or early clinical stage. In this application, we investigated transcriptomic profiles in astemizole treated Cynomolgus monkey's PBMCs. PBMCs were isolated from 4-6 years old male monkeys at 24 hr after administration45 Helvetica Light (10 mg/kg, 30 mg/kg). Gene expression profiles of astemizole treated monkey's PBMCs were determined using Affymetrix $GeneChip^{(R)}$ Human Genome U133 plus 2.0 arrays. The expression levels of 724 probe sets were significantly altered in PBMCs at 10 or 30 mg/kg after astemizole administration following determination of paired t-test using statistical criteria of ${\geq}$$1.5-fold changes at P<0.05. Gene expression patterns in PBMCs showed a considerable difference between astemizole 10 and 30 mg/kg administration groups in spite of an administration of the same chemical. However, close examination using Ingenuity Pathway Analysis (IPA) software revealed that several gene sets related to cardiotoxicity were deregulated at astemizole 10 and 30 mg/kg administration groups. The deregulation of cardiac hypertrophy related genes such as TXN, GNAQ, and MAP3K5 was observed at 10 mg/kg group. In astemizole 30 mg/kg group, genes involved in cardiotoxicity including cardiac necrosis/cell death, dilation, fibrosis, and hypertrophy were also identified. These results suggest that toxicogenomic approach using PBMCs as surrogate tissues will contribute to assess toxicant exposures and identify biomarkers at the pre-clinical stage.

Effect of adenotonsillar hypertrophy on right ventricle function in children

  • Lee, Jin Hwan;Yoon, Jung Min;Lim, Jae Woo;Ko, Kyung Og;Choi, Seong Jun;Kim, Jong-Yeup;Cheon, Eun Jung
    • Clinical and Experimental Pediatrics
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    • v.57 no.11
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    • pp.484-488
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    • 2014
  • Purpose: Chronic upper airway obstruction causes hypoxemic pulmonary vasoconstriction, which may lead to right ventricle (RV) dysfunction. Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Therefore, we aimed to evaluate RV function in children with ATH. Methods: Twenty-one children (male/female, 15/6; mean age, $92.3{\pm}39.0$ months; age range, 4-15 years) with ATH and 21 healthy age- and gender-matched controls were included in this study. Tricuspid annular plane systolic excursion and RV myocardial performance index were measured by transthoracic echocardiography. Further, the plasma level of N-terminal of probrain natriuretic peptide (NT-proBNP), an indicator of RV function, was determined. Results: The snoring-tiredness during daytime-observed apnea-high blood pressure (STOP) questionnaire was completed by the patients' parents, and loud snoring was noted in the ATH group. The plasma NT-proBNP level was significantly higher in the ATH group than that in the controls ($66.44{\pm}37.63pg/mL$ vs. $27.85{\pm}8.89pg/mL$, P=0.001). The echocardiographic parameters were not significantly different between the groups. Conclusion: We were unable to confirm the significance of echocardiographic evidence of RV dysfunction in the management of children with ATH. However, the plasma NT-proBNP level was significantly higher in the ATH group than that in the control, suggesting that chronic airway obstruction in children may carry a risk for cardiac dysfunction. Therefore, more patients should be examined using transthoracic echocardiography. In addition, pediatricians and otolaryngologists should consider cardiologic aspects during the management of children with severe ATH.

A novel GLA mutation in a Korean boy with an early cardiac manifestation of Fabry disease

  • Kwon, Soonhak;Park, Jin-Sung;Jung, Jae Hun;Hwang, Su Kyeong;Kim, Yeo Hyang;Lee, Yun Jeong
    • Journal of Genetic Medicine
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    • v.15 no.1
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    • pp.28-33
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    • 2018
  • Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of ${\alpha}$-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma ${\alpha}$-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.

clinical analyusis of ventricular septal defect (심실중격결손증의 임상적 고찰)

  • 이승구
    • Journal of Chest Surgery
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    • v.19 no.2
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    • pp.265-272
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    • 1986
  • We operated on 199 patients of VSD from 1976 to April l986. Among them, patients of VSD whose medical records were available were analyzed clinically. Operation on patients of VSD occupied 23.9% of total open heart surgery [832 cases] during those days. Of the 164 patients, 93 patients were male [56.7%]. 71 patients were female [43.3%]. Their age ranged from 6 months to 28 years and the mean age was 9.5 year and 82.2% of the patients were between 2 and 15 year of age. Of the patients, body weight below 10Kg were 19 cases. The most common complaints were frequent URI and DOE. On Kirklin`s anatomical classification, type II defect was most common [60.1%], type I [38.4%], combined type I+II, type III, combined type II+Ill and combined type II+IV in orders. Associated anomaly was found in 66 patients [42.5%>]. Pulmonary stenosis was most commonly associated cardiac anomaly [8.4%] and aortic insufficiency [7.1%], ASD, Lt. SVC and PDA in orders. There were extracardiac anomalies such as polydactyly, cleft palate, hypospadia and congenital aniridia, etc. Relationship between ventricular hypertrophy and defect size and cardiac cath. data was analyzed. The overall mortality was 7.0% [14 cases] and complication rate was 22.5% [35 cases].

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