• Journal of Yeungnam Medical Science
• /
• v.33 no.2
• /
• pp.125-129
• /
• 2016

Stress induced cardiomyopathy (SC) is characterized by transient left ventricular (LV) dysfunction in the absence of coronary artery disease. We report on a patient with panhypopituitarism who developed SC resulting from withdrawal of hormonal replacement therapy (HRT). A 52-year-old male visited our hospital for progressively worsening dyspnea. The patient had discontinued HRT 7 days ago, which had been administered for 18 months after transsphenoidal adenomectomy for pituitary macroadenoma. Initial electrocardiogram showed marked sinus bradycardia. Transthoracic echocardiography showed apical ballooning with an LV ejection fraction of 25%. No significant obstructive lesions were observed on coronary angiography. With a clinical diagnosis of SC associated with panhypopituitarism, HRT was restarted, including glucocorticoid and thyroxine, along with standard heart failure management. His LV function had normalized at 2-month follow-up. He remains asymptomatic and administration of beta-blocker and angiotensin converting enzyme inhibitor were discontinued He currently only requires HRT.

• Journal of Dental Anesthesia and Pain Medicine
• /
• v.15 no.3
• /
• pp.161-165
• /
• 2015

Long QT syndrome (LQTs) is a rare congenital disorder of the heart's electrical activity. Patients with LQTs are at increased risk of developing fatal ventricular arrhythmias. Elevated levels of sympathetic stimulation can exacerbate this risk. Successful behavior management is indispensable in the treatment of patients with LQTs. However, many drugs involved in pharmacologic behavior management are known to adversely affect the QT interval. Therefore, careful selection of a sedative drug is essential in avoiding such incidences. A 10-year-old boy with a known diagnosis of LQTs required restorative treatment due to dental caries at the permanent molar. He required sedation since treatment was painful and dental phobia can trigger sympathetic stimulation, creating a dangerous situation for patients with LQTs. Therefore, the treatment was performed over two sessions under moderate sedation involving propofol combined with nitrous oxide. Restorative treatment was successful without any complications under sedation with a target-controlled infusion (TCI) of propofol. There was no significant QT prolongation during pulpal treatment. Propofol TCI may be a good candidate for sedation in patients with LQTs.

• Journal of Chest Surgery
• /
• v.9 no.2
• /
• pp.207-214
• /
• 1976

Operations for cardiac valvular disease has been progressed in various ways. Since 1949 when Lord Russel operated mitral stenosis by closed technique at Johns Hopkins Hospital then much progress has been achieved and that nowadays severely diseased cardiac valve has been replaced by prosthetic valve, which is almost ideal in hemodynamic aspect, but still it has many problems such as thromboembolism, destruction of red blood cell, pressure gradient, and disturbance of left ventricular function, so in case of delicate situations, valve replacement should be decided carefully. Besides prosthetic valve, there are some kinds of reconstructive procedures and these have been resulted in better prognosis than prosthetic valve replacement in selected cases. So, authors have reviewed 61 Cases of cardiac patients who have been operated reconstructive valvular surgery by cardiopulmonary bypass, at Yonsei University, from Jan. 1963 to Mar. 1976. Out of 61 cases, 9 patients were replaced by prosthetic valve and rest of the patients were operated upon in various reconstructive procedures such as commissurotomy, valvotomy, valvuloplasty, and annuloplasty. Twenty cases of congenital heart diseases with valvular lesion, which had been operated for valvular lesion were also included in this statistics. Out of 9 cases of prosthetic valvular replacement five cases of prosthetic valvular replacement was done combined with other reconstructive procedures after attempted valvuloplasty. Comparative prognosis of both procedures are somewhat variable by reporters, average 19% of mortality after reconstructive surgery and 38% of mortality after prosthetic valve replacement in long term results. Most common cause of death in postoperative period was low output syndrome in both cases. It seems that good preoperative evaluation and proper reconstructive surgery will afford good prognosis in selected cardiac valvular diseased patient.

• Journal of Chest Surgery
• /
• v.32 no.7
• /
• pp.665-667
• /
• 1999

We report a case of ruptured sinus of Valsalva aneurysm in 48-year-old male, presenting the symptom of congestive heart failure. Echocardiography showing a hairpin-like sac ex tended from the left coronary sinus to the left ventricle adjacent to the anterior mitral valve leaflet, suggested ruptured sinus of Valsalva aneurysm or aorto-left ventricular tunnel. Operative findings revealed that left sinus of Valsalva aneurysm had multiple openings faced left ventricle, 7 mm in diameter, 20 mm in length. The proximal opening was closed with bovine pericardium and distal openings were closed with spaghetti pledgeted reinforced sutures. The patient was discharged on the 14th postoperative day, and follow up for 8 months uneventfully. This case was reported for its rarity and to describe the techniques of surgical repair.

• Journal of Chest Surgery
• /
• v.15 no.1
• /
• pp.61-66
• /
• 1982

The present study was performed to evaluate hemodynamic effects on the pericardial patch graft for stenosis of right ventricle outflow tract in 19 patients of tetralogy of Fallot. The stenosis of right ventricle outflow tract was associated with or without pulmonary annular nar-rowing, pulmonary valvular stenosis, and hypoplastic narrowing of pulmonary artery. Total correction of tetralogy of Fallot was performed under cardiopulmonary bypass with moderate hypothermia and cardioplegic cardiac arrest. Ventricular septal defects were closed with Teflon patch graft. The chamber pressures in the heart were measured before and after a total correction of tetralogy of Fallot. The data of pressure measurement and the results of postoperative observation of pericardial patch were as followings: 1. Systolic and diastolic pressure of right ventricle was decreased after operation from $96.0{\pm}14.7/10.0{\pm}14.4mmHg$ to $61.0{\pm}13.1/8.0{\pm}9.3mmHg$. 2. Systolic and diastolic pressure of pulmonary artery was increased after operation from $18.0{\pm}5.6/10.0{\pm}5.5mmHg$ to $31.0{\pm}10.7/14.0{\pm}4.9mmHg$. 3. Preoperative pressure gradient between right ventricle and pulmonary artery was decreased immediately after operation from 78.0mmHg to 30.0mmHg. 4. It was observed that excellent widening effects of right ventricle outflow tract was resulted from pericardial patch graft. 5. No postoperative bleeding from pericardial patch graft was observed. 6. Aneurysm formation of pericardial patch was not be observed during 1 to 6 years postoperative periods.

• Journal of Chest Surgery
• /
• v.46 no.2
• /
• pp.117-123
• /
• 2013

Background: We examined perioperative predictors of sustained sinus rhythm (SR) in patients undergoing the Cox maze operation and concomitant cardiac surgery for structural heart disease. Materials and Methods: From October 1999 to December 2008, 90 patients with atrial fibrillation (AF) underwent the Cox maze operation and other concomitant cardiac surgery. Eighty-nine patients, all except for one postoperative death, were followed-up with serial electrocardiographic studies, 24-hour Holter monitoring tests, and regular echocardiographic studies. Results: Eighty-nine patients undergoing the maze operation were divided into two groups according to the presence of SR. At the time of last follow-up (mean follow-up period, $51.0{\pm}30.8$ months), 79 patients (88.8%) showed SR (SR group) and 10 patients (11.2%) had recurrent AF (AF group). Factors predictive of sustained SR were the immediate postoperative conversion to SR (odds ratio, 97.2; p=0.001) and the presence of SR at the 6th month postoperatively (odds ratio, 155.7; p=0.002). Duration of AF, mitral valve surgery, number of valves undergoing surgery, left atrial dimension, and perioperative left ventricular dimensions and ejection fractions were not predictors of postoperative maintenance of SR. Conclusion: Immediate postoperative SR conversion and the presence of SR at the 6th postoperative month were independent predictors of sustained SR after the maze operation.

• Journal of Chest Surgery
• /
• v.50 no.3
• /
• pp.144-152
• /
• 2017

Background: The ubiquitin-proteasome system (UPS) is an important pathway of proteolysis in pathologic hypertrophic cardiomyocytes. We hypothesize that MG132, a proteasome inhibitor, might prevent hypertrophic cardiomyopathy (CMP) by blocking the UPS. Nuclear factor kappa-light-chain-enhancer of activated B cells ($NF-{\kappa}B$) and androgen receptor (AR) have been reported to be mediators of CMP and heart failure. This study drew upon pathophysiologic studies and the analysis of $NF-{\kappa}B$ and AR to assess the cardioprotective effects of MG132 in a left ventricular hypertrophy (LVH) rat model. Methods: We constructed a transverse aortic constriction (TAC)-induced LVH rat model with 3 groups: sham (TAC-sham, n=10), control (TAC-cont, n=10), and MG132 administration (TAC-MG132, n=10). MG-132 (0.1 mg/kg) was injected for 4 weeks in the TAC-MG132 group. Pathophysiologic evaluations were performed and the expression of AR and $NF-{\kappa}B$ was measured in the left ventricle. Results: Fibrosis was prevalent in the pathologic examination of the TAC-cont model, and it was reduced in the TAC-MG132 group, although not significantly. Less expression of AR, but not $NF-{\kappa}B$, was found in the TAC-MG132 group than in the TAC-cont group (p<0.05). Conclusion: MG-132 was found to suppress AR in the TAC-CMP model by blocking the UPS, which reduced fibrosis. However, $NF-{\kappa}B$ expression levels were not related to UPS function.

• Clinical and Experimental Pediatrics
• /
• v.45 no.6
• /
• pp.790-795
• /
• 2002

Sporadic reports of hypernatremic dehydration in breastfed newborn infants have appeared in medical literature for at least 3 decades. We report the first case of hypernatremic dehydration resulting from inadequate breast-feeding in Korea. A 14-day old baby, born to a mentally retarded mother, was transferred to our hospital with a body weight loss of 460 g since birth(17%) and a serum sodium(Na) level of 179 mEq/L, after initial hydration at another hospital. On admission, a cardiac murmur was heard and an enlarged liver was palpated. Cardiac ultrasonogram revealed ventricular septal defect and ostium secundum atrial septal defect. During hydration, a seizure-like attack developed. Serum Na decreased to 135 mEq/L on the 5th day of admission. Brain ultrasonography and brain magnetic resonance image revealed no remarkable abnormalities. Electroencephalography was normal. She suffered from prerenal azotemia, hyperglycemia and disseminated intravascular coagulation at admission but was treated successfully. Heart failure was also controlled with dobutamine, diuretics and digoxin.

• Clinical and Experimental Pediatrics
• /
• v.59 no.1
• /
• pp.16-23
• /
• 2016

Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

• The Korean Journal of Physiology and Pharmacology
• /
• v.12 no.2
• /
• pp.51-58
• /
• 2008

Cardiac fibroblasts constitute one of the largest cell populations in the heart, and contribute to structural, biochemical, mechanical and electrical properties of the myocardium. Nonetheless, their cardiac functions, especially electrophysiological properties, have often been disregarded in studies. $Ca^{2+}$-activated $K^+\;(K_{Ca})$ channels can control $Ca^{2+}$ influx as well as a number of $Ca^{2+}$-dependent physiological processes. We, therefore, attempted to identify and characterize $K_{Ca}$ channels in rat Cardiac fibroblasts. First, we showed that the cells cultured from the rat ventricle were cardiac fibroblasts by immunostaining for discoidin domain receptor 2 (DDR-2), a specific fibroblast marker. Secondly, we detected the expression of various $K_{Ca}$ channels by reverse transcription polymerase chain reaction (RT-PCR), and found all three family members of $K_{Ca}$ channels, including large conductance $K_{Ca}$ (BK-${\alpha}1-\;and\;-{\beta}1{\sim}4$subunits), intermediate conductance $K_{Ca}$ (IK), and small conductance $K_{Ca}$ (SK$1{\sim}4$ subunits) channels. Thirdly, we recorded BK, IK, and SK channels by whole cell mode patch clamp technique using their specific blockers. Finally, we performed cell proliferation assay to evaluate the effects of the channels on cell proliferation, and found that the inhibition of IK channel increased the cell proliferation. These results showed the existence of BK, IK, and SK channels in rat ventricular fibroblasts and involvement of IK channel in cell proliferation.