• Korean Circulation Journal
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• v.52 no.8
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• pp.563-575
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• 2022

Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM. SCD in HCM can be effectively prevented with implantable cardioverter-defibrillators. However, appropriate patient selection is important for primary prevention, and the 5-year SCD risk score and the presence of major SCD risk factors should be considered. Anticoagulation should be initiated in all HCM patients with atrial fibrillation regardless of the CHA2DS2-VASc score, and non-vitamin K antagonist oral anticoagulants are the first option. Symptomatic dynamic LVOT obstruction is first treated medically with negative inotropes, and if symptoms persist, septal reduction therapy is considered. The recently approved myosin inhibitor mavacamten is promising. HF in HCM is usually related to diastolic dysfunction, while about 5% of HCM patients show reduced left ventricular ejection fraction <50%, also referred to as "end-stage" HCM. Myocardial fibrosis plays an important role in the progression to advanced HF in patients with HCM. Patients who do not respond to guideline-directed medical therapy can be considered for heart transplantation. The development of imaging techniques, such as myocardial deformation on echocardiography and late gadolinium enhancement on cardiac magnetic resonance, can provide better risk evaluation and decision-making for management strategies in HCM.

• Journal of Chest Surgery
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• v.39 no.10 s.267
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• pp.775-778
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• 2006

Hypertrophic cardiomyopathy is characterized by inappropriate hypertrophy of the myocardium and is associated with various clinical presentations ranging from complete absence of symptoms to sudden, unexpected death. These are caused by dynamic obstruction of the left ventricular outflow tract and surgical approaches were initiated. But, the complete resection of hypertrophied midventricular septum is impossible by standard, transaortic approach, because of narrow vision and limited approach. And it leads to inadequate excision, will leave residual left ventricular out-flow tract obstruction or systolic anterior motion of mitral leaflet, and limit symptomatic improvement and patient's survival. We report a case of extended septal myectomy for hypertrophic cardiomyopathy of mid-septum in a child. The extended septal myectomy was performed by aortotomy and left ventricular apical incision, and made possible the complete resection of mid-ventricular septum, abnormal papillary muscles and chordae. The patient's symptom was improved and the postoperative course was uneventful.

• Journal of Cardiovascular Imaging
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• v.31 no.4
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• pp.217-219
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• 2023

• Journal of Veterinary Clinics
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• v.34 no.4
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• pp.275-278
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• 2017

An 8-year old female Korean Short Hair cat with a history of paralysis of both hind limbs less than 1 hour before admission was referred. On physical examination, the left hind limb was cold and there was no pulsation or mobility. On abdominal ultrasound examination, a thrombus 8 mm in length was found at the aortic bifurcation. The patient was diagnosed with hypertrophic cardiomyopathy (HCM) and cardiogenic pulmonary edema through radiologic evaluation and echocardiography. A tissue plasminogen activator (tPA) was applied intravenously using an accelerated dosing protocol (1 mg administered intravenously [IV] bolus, 2.5 mg IV over 30 min, 1.5 mg IV over 1 h) to treat the feline arterial thromboembolism. Within 12 h after administration of tPA, pulsation and mobility of both hind limbs were normal, without any noticeable complications. Clopidogrel was prescribed to prevent additional thrombus formation, and pimobendan, benazepril, and furosemide were prescribed for administration at home. The patient was discharged and survived 377 days.

• Neonatal Medicine
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• v.28 no.2
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• pp.77-82
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• 2021

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

• 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.

• Journal of Veterinary Clinics
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• v.26 no.4
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• pp.362-366
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• 2009

Hypertrophic cardiomyopathy (HCM) is the most often seen type of cardiomyopathy in cats. The cause is unknown but a genetic basis is thought to underlie some cases. Thromboembolism (TE) is a troubling complication in cats with myocardial disease. Two cats referred to Seoul National University Hospital for Animals with the paralysis of bilateral hindlimbs after vomiting. The cats were depressed and the bilateral hindlimbs were cyanotic, cool and painful. Heart murmur sounds were auscultated in both cases. Through radiographic and echocardiographic evaluation, HCM was diagnosed. TE at the distal aortic trifurcation was also visualized on abdominal ultrasonography. Both cats were expired and HCM and saddle thrombus were confirmed by postmortem examination in one cat.

• Journal of Veterinary Clinics
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• v.26 no.2
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• pp.170-175
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• 2009

A 7-year-old, intact male Pomeranian(weighing 2.2 kg), was presented with clinical signs of prolonged anorexia, polydypsia/polyuria, severe azotemia, proteinuria and heart murmur. Diagnostic studies found chronic kidney disease, severe hypertension and hypertrophic cardiomyopathy. The dog was treated with 2-day peritoneal dialysis, blood transfusion, anti-hypertensive therapy with amlodipine and conservative therapies direct to chronic renal failure. This is a rare case of hypertensive cardiomyopathy complicated with chronic kidney disease in dogs.

• Journal of Veterinary Clinics
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• v.31 no.4
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• pp.298-302
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• 2014

An 8-year-old castrated male domestic shorthair cat (Case 1) and 3-year-old castrated male Siamese cat (Case 2) was presented with acute paresis of the hindlimbs, constant open-mouth breathing, and hemoptysis. Heart murmur (Case 1) and gallop sound (Case 2) was ausculated on the left heart base. Radiographs revealed alveolar infiltration of the caudodorsal lung lobes with aerophagea in Case 1 and prominent cardiomegaly in Case 2. Marked concentric hypertrophy of the ventricular septum and free wall, and left atrial enlargement was detected through echocardiography in both cats. Based on the examinations including echocardiography, those cats were diagnosed as hypertropic cardiomyopathy. Abdominal ultrasound revealed echogenic material in the aortic trifurcation region, aortic thromboembolism (ATE). Although prognosis of those animals was guarded, interventional therapeutic approach through direct endovascular thrombolytic therapy was attempted. ATE was visualized through angiography; however dissolving the embolus using interventional thrombolytic approach was not successful due to the extensive thrombus.

• Annals of Occupational and Environmental Medicine
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• v.35
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• pp.34.1-34.8
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• 2023

Background: Hydrogen sulfide is a toxic substance that humans can be exposed to occupationally, and cases of hydrogen sulfide poisoning of workers in industrial sites are commonly reported. However, there have been no cases of poisoning of the public due to an unauthorized discharge of wastewater, so it is important to describe this incident. Case presentation: In a small village in Jeollanam-do, Republic of Korea, accounts of a terrible stench had been reported. A 26-year-old man who lived and worked in a foul-smelling area was taken to the emergency room with a headache, dizziness, nausea, and repeated syncope. A subsequent police and Ministry of Environment investigation determined that the cause of the stench was the unauthorized discharge of 9 tons of wastewater containing hydrogen sulfide through a stormwater pipe while the villagers were sleeping. The patient had no previous medical history or experience of symptoms. Leukocytes and cardiac markers were elevated, an electrocardiogram indicated biatrial enlargement, left ventricular hypertrophy, and corrected QT interval prolongation. Myocardial hypertrophy was detected on a chest computed tomography scan, and hypertrophic cardiomyopathy was confirmed on echocardiography. After hospitalization, cardiac marker concentrations declined, symptoms improved, and the patient was discharged after 7 days of hospitalization. There was no recurrence of symptoms after discharge. Conclusions: We suspect that previously unrecognized heart disease manifested or was aggravated in this patient due to exposure to hydrogen sulfide. Attention should be paid to the possibility of unauthorized discharge of hydrogen sulfide, etc., in occasional local incidents and damage to public health. In the event of such an accident, it is necessary to have government guidelines in place to investigate health impact and follow-up clinical management of exposed residents.