• Journal of Dental Anesthesia and Pain Medicine
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• v.23 no.1
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• pp.45-51
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• 2023

Andersen-Tawil syndrome (ATS) is a rare genetic disease characterized by a triad of episodic flaccid muscle weakness, ventricular arrhythmias, and physical anomalies. ATS patients have various cardiac arrhythmias that can cause sudden death. Implantation of an implantable cardioverter-defibrillator (ICD) is required when life-threatening cardiac arrhythmias do not respond to medical treatment. An 11-year-old girl underwent surgery for an ICD implantation. For general anesthesia in ATS patients, anesthesiologists should focus on the potentially difficult airway, serious cardiac arrhythmias, such as ventricular tachycardia (VT), and delayed recovery from neuromuscular blockade. We followed the difficult airway algorithm, avoided drugs that can precipitate QT prolongation and fatal cardiac arrhythmias, and tried to maintain normoxia, normocarbia, normothermia, normoglycemia, and pain control for prevention of sympathetic stimulation. We report the successful application of general anesthesia for ICD implantation in a pediatric patient with ATS and recurrent VT.

• The Korean Journal of Pain
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• v.13 no.1
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• pp.115-118
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• 2000

Stellate ganglion block is most commonly used in pain clinic because it is an easy procedure and it has broad indications reported that Angina pectoris, tachyarrhythmia and long QT syndrome (LQTS) are indicated. LQTS is a disorder of the abnormalities of cardiac sympathetic innervation and of myocardial repolarization. LQTS is characterized by marked prolongation of the QT interval, often manifestating as syncope, seizures, or sudden death due to polymorphic ventricular tachyarrhythmia known as torsades de pointes. Treatment of symptomatic patients usually begin with beta blocker. The elective treatment of LQTS patients unresponsive to beta blocker is the left cardiac sympathetic denervation. We report a case of LQTS patient who had received stellate ganglion block.

• Journal of the Semiconductor & Display Technology
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• v.12 no.2
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• pp.73-78
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• 2013

This paper proposes a portable electrocardiograph and smart device-based heart health monitoring and risk notification system. The proposed system consists of a portable electrocardiograph and a smart device for a system user, and a web-based monitoring system for observers. This system can improve the convenience and efficiency of measurement by using a light-weight portable electrocardiograph and a smart device. In addition, any authorized person such as caregiver or family member who is not related to medical institution can monitor users'heart health in real-time using the web-based monitoring system. Therefore, a user and authorized remote observers can efficiently monitor and manage user's heart health in daily-life even without any medical institution's help, and can preemptively deal with any possible dangerous situations, such as degeneration of a cardiac disorder and sudden cardiac death.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.22 no.1
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• pp.21-27
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• 2022

Very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency (VLCADD) leads to a defective 𝛽-oxidation, specifically during prolonged fasting, infection, or exercise. Patients with VLCADD usually suffer from cardiomyopathy, hypoketotic hypoglycemia, hepatic dysfunction, exercise intolerance, muscle pain, and rhabdomyolysis, and sometimes succumb to sudden death. VLCADD is generally classified into three phenotypes: severe early-onset cardiac and multiorgan failure, hypoketotic hypoglycemia, and later-onset episodic myopathy. Diagnostic evaluation comprises acylcarnitine analysis, genetic analysis, and VLCAD activity assay. In the acylcarnitine analysis, the key metabolites are C14:1, C14:2, C14, and C12:1. A C14:1 level >1 mmol/L strongly suggests VLCADD. Various treatment recommendations are available for this condition. Dietary management includes decreasing fat content, increasing medium-chain triglyceride levels, and decreasing fasting periods. Supplementation with L-carnitine is controversial. Triheptanoin (a seven-carbon fatty acid triglyceride) treatment demonstrates improvement of cardiac functions. Bezafibrate may improve the quality of life of patients with VLCAD.

• Journal of Chest Surgery
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• v.28 no.9
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• pp.817-821
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• 1995

The CarboMedics Medical valve has become our mechanical valvular prosthesis of choice because of favorable hemodynamic results that associated with marked clinical improvement and low incidence of thromboembolism after it,s uses. The data for this study was collected from August 1988 to July 1993,five years period. There were total of 57 patients[female 40,male 17 in this series with 4 isolated aortic valve,26 isolated mitral valve,11 double valve and a triple valve replacement. The mean follow up time was 32 months. Postoperatively,58% of cases were in New York Heart Association[NYHA functional class I,and mild and moderate symptoms[NYHA class II were present in 36% and there were very few patients remaining in higher functional classes. In postoperative echocardiographic study, showed marked improved cardiac function. The overall early mortality was 3.5% and the late mortality was one case after triple valve replacement due to sudden death. The causes of early death were attributed to early prosthetic valve endocarditis and heart failure.

• Safety and Health at Work
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• v.3 no.1
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• pp.58-66
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• 2012

Objectives: The purpose of this study was to review clinical characteristics and working environments of sudden cardiac death (SCD) cases associated with a tire manufacturer in Korea, and review possible occupational risk factors for cardiovascular disease including nanoparticles (ultrafine particles, UFPs). Methods: We reviewed (i) the clinical course of SCD cases and (ii) occupational and non-occupational risk factors including chemicals, the physical work environment, and job characteristics. Results: Possible occupational factors were chemicals, UFPs of rubber fume, a hot environment, shift work, overworking, and noise exposure. The mean diameter of rubber fume (63-73 nm) was (larger than diesel exhaust [12 nm] and outdoor dust [50 nm]). The concentration of carbon disulfide, carbon monoxide and styrene were lower than the limit of detection. Five SCD cases were exposed to shift work and overworking. Most of the cases had several non-occupational factors such as hypertension, overweight and smoking. Conclusion: The diameter of rubber fume was larger than outdoor and the diesel exhaust, the most well known particulate having a causal relationship with cardiovascular disease. The possibility of a causal relation between UFPs of rubber fume and SCD was not supported in this study. However, it is necessary to continue studying the relationship between large sized UFPs and SCD.

• The Korean Journal of Physiology and Pharmacology
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• v.6 no.1
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• pp.1-7
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• 2002

Congenital Long QT syndrome (LQTs) is a relatively rare pathologic disorder but results frequently in sudden cardiac death. Of the six LQTs that have been clinically described, five have been worked out for their genetic and biophysical profile. Most are generated by mutations which cause a loss of function in two delayed $K^+$ currents, $i_{Ks}\;and\;i_{Kr}.$ One syndrome is generated by mutations in the $Na^+$ channel which causes essentially a gain of function in the channel. Clinically the syndromes are characterized by slowed repolarization of the cardiac ventricular action potential and the occurrence of typical arrhythmias with undulating peaks in the electrocardiogram, called Torsade de Pointes. Arrhythmias are initiated by early or delayed afterdepolarizations and continue as reentry. Triggers for cardiac events are exercise (swimming; LQT1), emotion (arousal; LQT2) and rest/sleep (LQT3). ${\beta}-blockers$ have a high efficacy in the treatment of LQT1 and LQT2. In LQT3 their use is questionable. The study of congenital LQTsyndromes is a remarkable example of how basic and clinical science converge and take profit of each other's contribution.

• Journal of Chest Surgery
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• v.31 no.7
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• pp.735-738
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• 1998

Intracardiac hemangiomas are very rare primary cardiac tumor and there have been at least 37 reports of surgically resected cardiac hemangiomas. Most cardiac hemangiomas are asymptomatic. In symptomatic patients, symptoms are related to the location of tumor and outflow tract obstruction or obstruction of inferior and/or superior vena cava. Sudden death may occur due to conduction disturbances. The principle of treatment is surgical resection, and the prognosis is dependent upon the size, location and multiplicity of the tumor. A 40 year old man was admitted due to chest contusion and was found to have an intracardiac mass during echocardiographic examination. The mass was successfully removed and pathologic examination showed benign hemangioma. The patient was recovered uneventfully in postoperative period and was followed up for 1 year without evidence of recurrence.

• Investigative Magnetic Resonance Imaging
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• v.23 no.2
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• pp.100-113
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• 2019

Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.

• Korean Journal of Radiology
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• v.22 no.10
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• pp.1609-1618
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• 2021

Objective: Arrhythmogenic mitral valve prolapse (MVP) is an important cause of sudden cardiac death characterized by fibrosis of the papillary muscles or left ventricle (LV) wall, and an association between late gadolinium enhancement (LGE) of the LV papillary muscles and ventricular arrhythmia in MVP has been reported. However, LGE of the papillary muscles may be observed in other causes of mitral regurgitation, and it is not limited to patients with MVP. This study was to evaluate the association of LGE of the LV papillary muscles or ventricular wall on cardiac magnetic resonance imaging (CMR) and ventricular arrhythmia in patients with mitral regurgitation. Materials and Methods: This study included 88 patients (mean age ± standard deviation, 58.3 ± 12.0 years; male, 42%) with mitral regurgitation who underwent CMR. They were allocated to the MVP (n = 43) and non-MVP (n = 45) groups, and their LGE images on CMR, clinical characteristics, echocardiographic findings, and presence of arrhythmia were compared. Results: LV myocardial wall enhancement was more frequent in the MVP group than in the non-MVP group (28% vs. 11%, p = 0.046). Papillary muscle enhancement was observed in 7 (7.9%) patients. Of the 43 patients with MVP, 15 (34.8%) showed LGE in the papillary muscles or LV myocardium, including 12 (27.9%) with LV myocardial wall enhancement and 4 (9.3%) with papillary muscle enhancement. One patient with bilateral diffuse papillary muscle enhancement experienced sudden cardiac arrest due to ventricular fibrillation. Univariable logistic regression analysis showed that high systolic blood pressure (BP; odds ratio [OR], 1.05; 95% confidence interval [CI], 1.01-1.09; p = 0.027) and ventricular arrhythmia (OR, 6.84; 95% CI, 1.29-36.19; p = 0.024) were significantly associated with LGE of the papillary muscles. Conclusion: LGE of the papillary muscles was present not only in patients with MVP, but also in patients with other etiologies of mitral regurgitation, and it was associated with high systolic BP and ventricular arrhythmia. Papillary muscle enhancement on CMR should not be overlooked.