• 고신대학교 의과대학 학술지
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• 제33권3호
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• pp.328-336
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• 2018

Objectives: Electrocardiograhy (ECG) is the first step in hypertrophic cardiomyopathy (HCMP) diagnosis. For various reasons, the T wave inversion (TWI) and ECG change with time and HCMP is not easy to diagnosis. The aim of this retrospective study was to investigate the association between TWI on ECG and apical HCMP. Methods: A total of 4,730 ECGs presenting TWI from January 2011 to March 2013 in Pusan National University Hospital were enrolled. 133 patients who were examined by both echocardiography and coronary angiogram were analyzed. Patients were divided into two groups: Group A (TWI ${\geq}$ 10 mm) and Group B (5 mm ${\leq}$ TWI < 10 mm). HCMP is defined by a wall thickness ${\geq}15mm$ in one or more LV myocardial segments. Apical HCMP is defined to be hypertrophy that is confined to LV apex. The patients who had ECGs with at least one month interval were divided 3 groups: Normal T wave, Abnormal T wave, and Persistent TWI. The prevalence of Apical HCMP and coronary artery disease (CAD) was reviewed among the three groups. Results: In this study there were a total 133 patients, with patients divided into Group A which had 15 patients and Group B which had 118 patients. Among the 23 patients with apical HCMP, three patients were Group A and twenty patients were Group B (P = 0.769). Regarding constancy of TWI, persistent TWI group was higher in apical HCMP than in other groups (P = 0.038). CAD had no difference between groups (P = 0.889). Conclusions: T wave negativity was not associated with incidence of apical HCMP. However, apical HCMP was diagnosed more frequently in patients with persistent TWI. Further follow up echocardiographic study is needed to evaluate the progression of apical HCMP in patients with TWI.

• Korean Journal of Radiology
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• 제22권7호
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• pp.1044-1053
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• 2021

Objective: Motion-corrected averaging with a single-shot technique was introduced for faster acquisition of late-gadolinium-enhancement (LGE) cardiovascular magnetic resonance (CMR) imaging while free-breathing. We aimed to evaluate the image quality (IQ) of free-breathing motion-corrected single-shot LGE (moco-ss-LGE) in patients with hypertrophic cardiomyopathy (HCM). Materials and Methods: Between April and December 2019, 30 patients (23 men; median age, 48.5; interquartile range [IQR], 36.5-61.3) with HCM were prospectively enrolled. Breath-held single-shot LGE (bh-ss-LGE) and free-breathing moco-ss-LGE images were acquired in random order on a 3T MR system. Semi-quantitative IQ scores, contrast-to-noise ratios (CNRs), and quantitative size of myocardial scar were assessed on pairs of bh-ss-LGE and moco-ss-LGE. The mean ± standard deviation of the parameters was obtained. The results were compared using the Wilcoxon signed-rank test. Results: The moco-ss-LGE images had better IQ scores than the bh-ss-LGE images (4.55 ± 0.55 vs. 3.68 ± 0.45, p < 0.001). The CNR of the scar to the remote myocardium (34.46 ± 11.85 vs. 26.13 ± 10.04, p < 0.001), scar to left ventricle (LV) cavity (13.09 ± 7.95 vs. 9.84 ± 6.65, p = 0.030), and LV cavity to remote myocardium (33.12 ± 15.53 vs. 22.69 ± 11.27, p < 0.001) were consistently greater for moco-ss-LGE images than for bh-ss-LGE images. Measurements of scar size did not differ significantly between LGE pairs using the following three different quantification methods: 1) full width at half-maximum method; 23.84 ± 12.88% vs. 24.05 ± 12.81% (p = 0.820), 2) 6-standard deviation method, 15.14 ± 10.78% vs. 15.99 ± 10.99% (p = 0.186), and 3) 3-standard deviation method; 36.51 ± 17.60% vs. 37.50 ± 17.90% (p = 0.785). Conclusion: Motion-corrected averaging may allow for superior IQ and CNRs with free-breathing in single-shot LGE imaging, with a herald of free-breathing moco-ss-LGE as the scar imaging technique of choice for clinical practice.

• Korean Journal of Radiology
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• 제22권7호
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• pp.1054-1065
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• 2021

Objective: We implemented a novel resectable myocardial model for mock myectomy using a hybrid method of three-dimensional (3D) printing and silicone molding for patients with apical hypertrophic cardiomyopathy (ApHCM). Materials and Methods: From January 2019 through May 2020, 3D models from three patients with ApHCM were generated using the end-diastolic cardiac CT phase image. After computer-aided designing of measures to prevent structural deformation during silicone injection into molding, 3D printing was performed to reproduce anatomic details and molds for the left ventricular (LV) myocardial mass. We compared the myocardial thickness of each cardiac segment and the LV myocardial mass and cavity volumes between the myocardial model images and cardiac CT images. The surgeon performed mock surgery, and we compared the volume and weight of the resected silicone and myocardium. Results: During the mock surgery, the surgeon could determine an ideal site for the incision and the optimal extent of myocardial resection. The mean differences in the measured myocardial thickness of the model (0.3, 1.0, 6.9, and 7.3 mm in the basal, midventricular, apical segments, and apex, respectively) and volume of the LV myocardial mass and chamber (36.9 mL and 14.8 mL, 2.9 mL and -9.4 mL, and 6.0 mL and -3.0 mL in basal, mid-ventricular and apical segments, respectively) were consistent with cardiac CT. The volume and weight of the resected silicone were similar to those of the resected myocardium (6 mL [6.2 g] of silicone and 5 mL [5.3 g] of the myocardium in patient 2; 12 mL [12.5 g] of silicone and 11.2 mL [11.8 g] of the myocardium in patient 3). Conclusion: Our 3D model created using hybrid 3D printing and silicone molding may be useful for determining the extent of surgery and planning surgery guided by a rehearsal platform for ApHCM.

• Journal of Cardiovascular Imaging
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• 제31권2호
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• pp.85-95
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• 2023

BACKGROUND: The prognostic utility of follow-up transthoracic echocardiography (FU-TTE) in patients with hypertrophic cardiomyopathy (HCM) is unclear, specifically in terms of whether changes in echocardiographic parameters in routine FU-TTE parameters are associated with cardiovascular outcomes. METHODS: From 2010 to 2017, 162 patients with HCM were retrospectively enrolled in this study. Using echocardiography, HCM was diagnosed based on morphological criteria. Patients with other diseases that cause cardiac hypertrophy were excluded. TTE parameters at baseline and FU were analyzed. FU-TTE was designated as the last recorded value in patients who did not develop any cardiovascular event or the latest exam before event development. Clinical outcomes were acute heart failure, cardiac death, arrhythmia, ischemic stroke, and cardiogenic syncope. RESULTS: Median interval between the baseline TTE and FU-TTE was 3.3 years. Median clinical FU duration was 4.7 years. Septal trans-mitral velocity/mitral annular tissue Doppler velocity (E/e'), tricuspid regurgitation velocity, left ventricular ejection fraction (LVEF), and left atrial volume index (LAVI) at baseline were recorded. LVEF, LAVI, and E/e' values were associated with poor outcomes. However, no delta values predicted HCM-related cardiovascular outcomes. Logistic regression models incorporating changes in TTE parameters had no significant findings. Baseline LAVI was the best predictor of a poor prognosis. In survival analysis, an already enlarged or increased size LAVI was associated with poorer clinical outcomes. CONCLUSIONS: Changes in echocardiographic parameters extracted from TTE did not assist in predicting clinical outcomes. Cross-sectionally evaluated TTE parameters were superior to changes in TTE parameters between baseline and FU at predicting cardiovascular events.

• Journal of Chest Surgery
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• 제27권4호
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• pp.313-317
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• 1994

Surgical treatment is possible for the obstructive form of hypertrophic cardiomyopathy and transaortic left ventricular septal myectomy and myotomy has been the procedure of choice. If coexisting intrinsic mitral valve disease exists, mitral valve replacement has been performed. But abnormal systolic anterior motion of anterior mitral leaflet[SAM] with intrinsic normal mitral valve disease is the typical feature of IHSS and we prefer not to replace mitral valve. 3 patients underwent transaortic myotomy and myectomy for IHSS with mitral regurgitation. 2 patients of them have coexisting intrinsic mitral valve diseases such as mitral valve vegetation and chorda rupture. Concomittent mitral valve replacement were performed. 1 patient shows SAM of mitral anterior leaflet but has intrinsic normal mitral valve morphologically and transepicardial echocardiogram and direct monitoring of pressure gradient during the operative procedure gives better information for subsided mitral regurgitation. Post operative course during the 12 months follow-up was uneventful.

• Journal of Chest Surgery
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• 제43권5호
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• pp.522-524
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• 2010

지속성 좌상대정맥을 가진 환자에서의 심장 이식술은 양측상대정맥의 단일화 과정이 필요하다. 저자들은 양측상대정맥을 동반한 비후성 심근증 환자에서 양측상대정맥 문합술을 통한 상대정맥 단일화 수술 후 심장이식을 시행하여 양호한 성적을 얻었으므로 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제50권10호
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• pp.1024-1029
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• 2007

Costello 증후군은 특징적 얼굴 형태(coarse face), 발달 장애, 지능 저하, 성장 지연, 신경학적 이상, 심근증, 피부 병변, 수유 장애, 상대적 대두증, 소관절의 과신전, 고형종 발생 증가 등을 특징으로 하는 드문 증후군으로 1977년 Costello에 의해 처음으로 보고되었으며, 이후 전세계적으로 약 100례 이상이 보고되었다. 최근 Costello 증후군은 HRAS 유전자 내의 glycine 12 또는 13 codon을 침범하는 de novo mutation에 의해 발생하는 것으로 알려져 있다. 저자들은 특징적인 얼굴 형태와 지능 저하, 대두증, 손과 발의 과도한 주름, 비후성 심근증과 심방성 빈맥으로 특징지어지는 Costello 증후군으로 진단된 환아 3례를 경험하였기에 이를 보고하는 바이다.

• Journal of Genetic Medicine
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• 제13권2호
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• pp.99-104
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• 2016

$Cant{\acute{u}}$ syndrome (CS, OMIM 239850) is a very rare autosomal dominantly inherited genetic disease characterized by congenital hypertrichosis, neonatal macrosomia, a distinct facial features such as macrocephaly, and cardiac defects. Since the first description by $Cant{\acute{u}}$ et al. in 1982, about 50 cases have been reported to date. Recently, two causative genes for CS has been found by using exome sequencing analyses: ABCC9 and KCNJ8. Most cases of clinically diagnosed CS have resulted from de novo mutations in ABCC9. In this study, we report three independent Korean children with CS resulting from de novo ABCC9 mutations. Our patients had common clinical findings such as congenital hypertrichosis, distinctive facial features. One of them showed severe pulmonary hypertension and hypertrophic cardiomyopathy, which require medical treatment. And, two patients had a history of patent ductus arteriosus. Although two of our patients had shown early motor developmental delay, it was gradually improved during follow-up periods. Although CS is quite rare, there are the concerns about development of various cardiac problems in the lifetime. Therefore, an accurate diagnosis followed by appropriate management and genetic counseling should be provided to CS patients.

• Korean Journal of Radiology
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• 제23권6호
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• pp.581-597
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• 2022

Left ventricular (LV) wall thickening, or LV hypertrophy (LVH), is common and occurs in diverse conditions including hypertrophic cardiomyopathy (HCM), hypertensive heart disease, aortic valve stenosis, lysosomal storage disorders, cardiac amyloidosis, mitochondrial cardiomyopathy, sarcoidosis and athlete's heart. Cardiac magnetic resonance (CMR) imaging provides various tissue contrasts and characteristics that reflect histological changes in the myocardium, such as cellular hypertrophy, cardiomyocyte disarray, interstitial fibrosis, extracellular accumulation of insoluble proteins, intracellular accumulation of fat, and intracellular vacuolar changes. Therefore, CMR imaging may be beneficial in establishing a differential diagnosis of LVH. Although various diseases share LV wall thickening as a common feature, the histologic changes that underscore each disease are distinct. This review focuses on CMR multiparametric myocardial analysis, which may provide clues for the differentiation of thickened myocardium based on the histologic features of HCM and its phenocopies.

• Investigative Magnetic Resonance Imaging
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• 제23권2호
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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.