• Journal of Chest Surgery
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• v.46 no.6
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• pp.457-460
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• 2013

Dynamic left ventricular (LV) outflow tract obstruction is a characteristic feature of hypertrophic cardiomyopathy; however, it can also occur in association with hyperdynamic LV contraction and/or changes in the cardiac loading condition, even in a structurally normal or near-normal heart. Here, we report a case of anemia-induced systolic anterior motion of the mitral valve and the resultant intraventricular obstruction in a patient who underwent coronary artery bypass grafting and suffered from anemia associated with recurrent gastrointestinal bleeding.

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.128-131
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• 2017

Mid-ventricular obstruction (MVO) rarely occurs in patients without hypertrophic cardiomyopathy. Increased cardiac contractility may play an important role in causing MVO. We experienced a case of severe chest pain and MVO in a 50-year-old female patient. She had hypertension, diabetes, stroke and peripheral artery disease. Her blood pressure was very high (222/122 mmHg) with severe fluctuation. The transthoracic echocardiography revealed MVO accompanied by hyper-dynamic left ventricular systolic function. We regarded her chest pain and MVO as secondary findings related to other diseases. Coronary angiography and several tests for uncontrolled hypertension were performed, and those evaluations revealed that she had coronary artery disease and hyperthyroidism. We considered that the increase in the myocardial oxygen demand in response to the increase in cardiac contractility and workload associated with hyperthyroidism aggravated her symptoms and MVO. She was treated with methimazole and beta blockers and her symptoms dramatically improved.

• Bulletin of the Korean Chemical Society
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• v.32 no.7
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• pp.2274-2278
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• 2011

Verapamil is a calcium channel blocker and is classified as a class IV anti-arrhythmic agent. It is used in the control of supra ventricular tachyarrhythmias, and in the management of classical and variant angina pectoris. It is also used in the treatment of hypertension and used as an important therapeutic agent for angina pectoris, ischemic heart disease, hypertension and hypertrophic cardiomyopathy. Verapamil commonly co-administered with NSAIDs (non-steroidal anti-inflammatory drugs) i.e. diclofenac sodium, flurbiprofen, Ibuprofen, mefanamic acid and meloxicam. A simple and rapid RP-HPLC method for simultaneous determination and quantification of verapamil and NSAIDs was developed and validated. The mobile phase constituted of acetonitrile: water (55:45) whose pH was adjusted at 2.7 and pumped at a flow rate of 2.0 mL $min^{-1}$ at 230 nm. The proposed method is simple, precise, accurate, low cost and least time consuming for the simultaneous determination of verapamil and NSAIDs which can be effectively applied for the analysis of human serum.

• Clinical and Experimental Pediatrics
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• v.56 no.2
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• pp.52-59
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• 2013

Cardiomyopathy (CMP) is a heterogeneous disease caused by a functional abnormality of the cardiac muscle. CMP is of 2 major types, dilated and hypertrophic, and is further classified as either primary or secondary. Secondary CMP is caused by extrinsic factors, including infection, ischemia, hypertension, and metabolic disorders. Primary CMP is diagnosed when the extrinsic factors of secondary CMP are absent. Furthermore, the World Health Organization, American Heart Association, and European Cardiology Association have different systems for clinically classifying primary CMP. Primary CMP is rare and associated with a family history of the disease, implying that genetic factors might affect its incidence. In addition, the incidence of CMP varies widely according to patient ethnicity. Genetic testing plays an important role in the care of patients with CMP and their families because it confirms diagnosis, determines the appropriate care for the patient, and possibly affects patient prognosis. The diagnosis and genetic identification of CMP in patients' families allow the possibility to identify novel genes that may lead to new treatments. This review focuses on the epidemiology, pathophysiology, diagnosis, and treatment of CMP, with the aim of providing pediatricians with insights that may be helpful in the early identification and management of idiopathic CMP in children.

• Journal of Chest Surgery
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• v.42 no.6
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• pp.719-724
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• 2009

Background: As the patients who undergo heart transplantation have achieved better survival in recent years, growing number of recipients are at a risk for experiencing surgical complications in addition to rejection and infection. In this paper, we report on our experience with the surgical complications that occurred in heart transplant recipients. Material and Method: From April 1994 to September 2003, 37 heart transplantations were performed at our center by a single surgeon. The indications for transplantation were dilated cardiomyopathy, ischemic cardiomyopathy, valvular cardiomyopathy and familial hypertrophic cardiomyopathy. Result: Twenty postoperative complications required surgeries in 15 patients (41%). The types of operations required were; redo-sternotomy for bleeding (5), pericardiostomy for effusion (4), implantation of a permanent pacemaker (1), right lower lobe lobectomy for aspergilloma (1), removal of urinary stone (1), cholecystectomy for gall bladder stone (1), drainage of a perianal abscess (1), paranasal sinus drainage (1), total hip replacement (1), partial gingivectomy due to gingival hypertrophy (1), urethrostomy (1), herniated intervertebral disc operation (1) and total hysterectomy for myoma uteri (1). The locations of the complications were mediastinal in 10 (27%) cases and extramediastihalin 10 (27%) cases. Conclusion: The relatively high incidence of extrathoracic complications associated with heart transplantation emphasizes the importance of a multidisciplinary approach to the improve long-term survival when managing those complex patients.

• Journal of Veterinary Clinics
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• v.31 no.2
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• pp.85-89
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• 2014

Arterial thromboembolism (ATE) is a common and fatal complication of hypertrophic cardiomyopathy (HCM) in cats. Therefore in this study, we evaluated the hypercoagulability (using plasma concentration of D-dimer) in HCM cats with different stage of heart failure and left atrial enlargement and also investigated the any correlation with echocardiographic indices (including left free wall thickness at diastole, interventricular septal thickness at diastole, LA to Ao ratio, heart failure stage, existence of systolic anterior motion of mitral valve). The median plasma D-dimer concentration in this study population was $0.51{\pm}0.70$ (range 0 to 2.50) ug/mL in the control group, $1.47{\pm}1.29$ (range 0.3 to 5.79) ug/mL in the HCM group, $1.48{\pm}1.65$ (range 0.3 to 5.79) ug/mL in the ISACHC I group, $1.62{\pm}0.4$ (range 1.31 to 2.07) ug/mL in the ISACHC II group, $1.36{\pm}0.91$ (range 0.3 to 2.31) ug/mL in the ISACHC III group, $1.90{\pm}1.60$ (range 0.3 to 5.79) ug/mL in the cat with LA dilation, $1.72{\pm}0.72$ (range 0.6 to 2.31) ug/mL in cats with SEC-T, $1.19{\pm}0.70$ (range 0.3 to 2.31) ug/mL in the cats with SAM, and $1.63{\pm}0.80$ (range 0.6 to 2.31) ug/mL in the cats with ATE. Our study found the median and mean concentration of plasma D-dimer was higher in cat with HCM, ATE, SECT and SAM and clearly provides evidence of hypercoagulability in cats with HCM, although the severity was not correlated to the dilation of LA and the presence of heart failure. This is the first study evaluating the hypercoagulability in cats with HCM in Korea.

• Journal of Chest Surgery
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• v.56 no.5
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• pp.322-327
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• 2023

Background: Superior vena cava (SVC) stenosis during follow-up is a major concern after heart transplantation, and many technical modifications have been introduced. We analyzed the surgical results of the SVC intima layer-only suture technique in heart transplantation. Methods: We performed SVC anastomosis with sutures placed only in the intima during heart transplantation. We measured the area of the SVC at 3 different points (above the anastomosis, at the anastomosis, and below the anastomosis) in an axial view by freely drawing regions of interest, and then evaluated the degree of stenosis. Patients who underwent cardiac computed tomography (CT) at 2 years postoperatively between June 2017 and May 2020 were included in this study. Results: We performed heart transplantation in 41 patients. Among them, 24 patients (16 males and 8 females) underwent follow-up cardiac CT at 2 years postoperatively. The mean age at operation was 49.4±4.9 years. The diagnoses at time of operation were dilated cardiomyopathy (n=12), ischemic heart disease (n=8), valvular heart disease (n=2), hypertrophic cardiomyopathy (n=1), and congenital heart disease (n=1). No cases of postoperative bleeding requiring intervention occurred. The mean CT follow-up duration was 1.9±0.7 years. At follow-up, the mean areas at the 3 key points were 2.7±0.8 cm², 2.7±0.8 cm², and 2.7±1.0 cm² (p=0.996). There were no SVC stenosis-related symptoms during follow-up. Conclusion: The suture technique using only the SVC intimal layer is a safe and effective method for use in heart transplantation.

• Neonatal Medicine
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• v.15 no.2
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• pp.200-206
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• 2008

Human chromosome 9 is characterized by a high degree of morphologic heteromorphisms, including variation in the size of the heterochromatin. We present a case of a de novo short arm addition of chromosome 9, [46, XY, add(9)(p13)], associated with multiple anomalies, including trigonocephaly, upward slant of the palpebral fissures, patent ductus arteriosus, pulmonary hypertension, hypertrophic cardiomyopathy, umbilical hernia, ambiguous genitalia, and sensorineural hearing and visual loss. This mutation affects the pericentric region of the heterochromatin. This patient exhibited a clinically important breakpoint of the heterochromatic region of chromosome 9 short arm and the associated anomalies.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.23 no.1
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• pp.31-35
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• 2012

Noonan syndrome is characterized by short stature, typical facial dysmorphology, and congenital heart defects. The main facial features of Noonan syndrome are hypertelorism with down-slanting palpebral fissures, ptosis, and low-set posteriorly-rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency, and lymphatic dysplasias. The patient is a 10-year-old boy. He had experienced repeated febrile convulsions. He had typical facial features, a short stature, chest deformity, cryptorchidism, vesicoureteral reflux, and mental retardation. His language and motor development were delayed. When he went to school, it was difficult for him to pay attention, follow directions, and organize tasks. He also displayed behavior such as squirming, leaving his seat in class, and running around inappropriately. Clinical observation is important for the diagnosis, so we report a patient who was diagnosed with Noonan syndrome, mental retardation, and attention-deficit hyperactivity disorder.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.18-22
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• 2005

Pompe disease is a genetic disorder caused by a deficiency of acid ${\alpha}$-glucosidase (GAA). This enzyme defect results in lysosomal glycogen accumulation in multiple tissues and cell types, with cardiac, skeletal, and smooth muscle cells the most seriously affected. Infantile-onset Pompe disease is uniformly lethal. Affected infants present in the first few months of life with hypotonia, generalized muscle weakness, and a hypertrophic cardiomyopathy, followed by death from cardiorespiratory failure or respiratory infection, usually by 1 year of age. Late-onset forms is characterized by a lack of severe cardiac involvement and a less severe short-term prognosis. Enzyme replacement therapy for Pompe disease is intended to address directly the underlying metabolic defect via intravenous infusions of recombinant human GAA to provide the missing enzyme. We experienced one case of Pompe disease in 3-years old boy that has improved his exercise ability and cardiac function after GAA enzyme replacement therapy.