An endovascular repair of an abdominal aortic aneurysm (AAA) is technically less invasive than open surgery, but gives results as effective as open surgery if the anatomy is adequate. Unfortunately, 20-30% of AAA patients are not suitable for endovascular repair because they lack a sufficient proximal landing zone. In an effort to broaden the applicability of endovascular repair, the chimney technique has been introduced. This refers to deployment of a covered or bare-metal stent parallel to the main aortic endograft within the aneurysm, creating a conduit that runs outside the main aortic endograft into the aortic branches. We report the case of a 75-year-old male with a juxtarenal abdominal aortic aneurysm and multiple comorbidities who was treated successfully with an endovascular aneurysm repair using a chimney graft.
Aged ; Aneurysm ; Aortic Aneurysm, Abdominal* ; Comorbidity ; Endovascular Procedures ; Humans ; Male ; Stents ; Transplants*

An endovascular repair of an abdominal aortic aneurysm (AAA) is technically less invasive than open surgery, but gives results as effective as open surgery if the anatomy is adequate. Unfortunately, 20-30% of AAA patients are not suitable for endovascular repair because they lack a sufficient proximal landing zone. In an effort to broaden the applicability of endovascular repair, the chimney technique has been introduced. This refers to deployment of a covered or bare-metal stent parallel to the main aortic endograft within the aneurysm, creating a conduit that runs outside the main aortic endograft into the aortic branches. We report the case of a 75-year-old male with a juxtarenal abdominal aortic aneurysm and multiple comorbidities who was treated successfully with an endovascular aneurysm repair using a chimney graft.
Aged ; Aneurysm ; Aortic Aneurysm, Abdominal* ; Comorbidity ; Endovascular Procedures ; Humans ; Male ; Stents ; Transplants*

Mowat-Wilson syndrome is an extremely rare genetic disease that is characterized by intellectual disability, facial dysmorphism, Hirschsprung's disease, and other congenital anomalies. This disorder is caused by heterozygous mutations or deletions in the zinc finger E-box-binding homeobox-2 gene (ZEB2). Thus far, approximately 200 cases of Mowat-Wilson syndrome have been reported worldwide. In Korea, only one case with a 2q22 deletion, which also affects ZEB2, has been previously reported. Here, we describe a patient with Mowat-Wilson syndrome who presented with developmental delays, typical facial dysmorphism, and Hirschsprung's disease. Molecular analysis of ZEB2 identified a novel heterozygous mutation at c.190dup (p.S64Kfs*6). To our knowledge, this is the second report of a Korean patient with Mowat-Wilson syndrome that has been confirmed genetically.
Hirschsprung Disease* ; Humans ; Intellectual Disability ; Korea ; Zinc Fingers

A foreign body in heart is rare, but it is more frequently encountered than the past as iatrogenic causes are increasing. Clinicians should be aware that foreign body could be mistaken for normal structure of heart. In order for accurate diagnosis, multi-imaging modalities should be used for information of exact location, mobility and hemodynamic effects. A decision to intervene should be made based on potential harms harbored by foreign bodies. Endovascular retrieval should be considered as an option. However, when fatal complications occur or when foreign bodies are embedded deeply, a surgical removal should be attempted.
Cardiac Surgical Procedures/*methods ; Echocardiography ; Female ; Foreign Bodies/*radiography/*surgery ; Foreign-Body Migration/radiography ; Heart Injuries/diagnosis/etiology/*surgery ; Humans ; Iatrogenic Disease ; Middle Aged ; *Needles ; Treatment Outcome

A foreign body in heart is rare, but it is more frequently encountered than the past as iatrogenic causes are increasing. Clinicians should be aware that foreign body could be mistaken for normal structure of heart. In order for accurate diagnosis, multi-imaging modalities should be used for information of exact location, mobility and hemodynamic effects. A decision to intervene should be made based on potential harms harbored by foreign bodies. Endovascular retrieval should be considered as an option. However, when fatal complications occur or when foreign bodies are embedded deeply, a surgical removal should be attempted.
Cardiac Surgical Procedures/*methods ; Echocardiography ; Female ; Foreign Bodies/*radiography/*surgery ; Foreign-Body Migration/radiography ; Heart Injuries/diagnosis/etiology/*surgery ; Humans ; Iatrogenic Disease ; Middle Aged ; *Needles ; Treatment Outcome

BACKGROUND: Among pediatric non-Hodgkin lymphomas, there are 4 major subtypes: Burkitt lymphoma, lymphoblastic lymphoma, diffuse large B-cell lymphoma, and anaplastic large-cell lymphoma. Understanding of other rare subtypes derives only from small pediatric case series. We report our institutional experience with rare pediatric NHLs.METHODS: Thirty-six cases of rare NHL subtypes diagnosed at the Asan Medical Center from 1995 to 2015 were evaluated. We retrospectively reviewed the clinical and pathologic features and outcomes of these patients, excluding peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), and extranodal NK/T-cell lymphoma (ENKL), on which we have previously reported.RESULTS: There were 23 cases of T-cell lineage (13 PTCL, 6 ENKL, 2 subcutaneous panniculitis-like T-cell lymphoma, 1 primary cutaneous CD4+2016-11-22 small/medium sized T-cell lymphoma, 1 enteropathy-associated T-cell lymphoma) and 13 cases of B-cell lineage lymphoma (5 marginal zone lymphoma, 6 primary mediastinal large B-cell lymphoma, 2 immunoblastic and plasmablastic lymphoma). All patients were treated with chemotherapy with or without surgery, except 4 out of 5 patients with marginal zone lymphoma who received surgery only. Two patients died and 6 patients relapsed. One patient with primary mediastinal large B-cell lymphoma received autologous peripheral blood stem cell transplantation. The 5-year overall survival and event-free survival rates of rare pediatric NHL excluding PTCL, NOS, and ENKL was 80.0% and 72.0%, respectively.CONCLUSION: Children diagnosed with rare pediatric NHL other than PTCL, NOS, and ENKL showed variable incidence and treatment outcomes. Multicenter studies in larger cohorts are needed for better understanding of these rare NHL subtypes in childhood.
B-Lymphocytes ; Burkitt Lymphoma ; Child ; Chungcheongnam-do ; Cohort Studies ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Peripheral Blood Stem Cell Transplantation ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; T-Lymphocytes

PURPOSE: The present study aimed to investigate the prevalence, characteristics, and clinical significance of concomitant specific cardiomyopathies in subjects with bicuspid aortic valves (BAVs). MATERIALS AND METHODS: A total of 1186 adults with BAV (850 males, mean age 56±14 years) at a single tertiary center were comprehensively reviewed. Left ventricular non-compaction, hypertrophic cardiomyopathy, and idiopathic dilated cardiomyopathy were confirmed when patients fulfilled current clinical and echocardiographic criteria. Clinical and echocardiographic characteristics, including comorbidities, heart failure presentation, BAV morphology, function, and aorta phenotypes, in BAV subjects with or without specific cardiomyopathies were compared. RESULTS: Overall, 67 subjects (5.6%) had concomitant cardiomyopathies: 40 (3.4%) patients with left ventricular non-compaction, 17 (1.4%) with hypertrophic cardiomyopathy, and 10 (0.8%) with dilated cardiomyopathy. BAV subjects with hypertrophic cardiomyopathy had higher prevalences of diabetes mellitus and heart failure with preserved ejection fraction, and tended to have type 0 phenotype, while BAV subjects with dilated cardiomyopathy showed higher prevalences of chronic kidney disease and heart failure with reduced ejection fraction. BAV subjects with left ventricular non-compaction were significantly younger and predominantly male, and had greater BAV dysfunction and a higher prevalence of normal aorta shape. In multiple regression analysis, cardiomyopathy was independently associated with heart failure (odds ratio 2.795, 95% confidential interval 1.603–4.873, p<0.001) after controlling for confounding factors. CONCLUSION: Concomitant cardiomyopathies were observed in 5.6% of subjects with BAV. A few different clinical and echocardiographic characteristics were found. The presence of cardiomyopathy was independently associated with heart failure.
Adult ; Aorta ; Aortic Valve ; Bicuspid ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Cardiomyopathy, Hypertrophic ; Comorbidity ; Diabetes Mellitus ; Echocardiography ; Heart Failure ; Humans ; Male ; Phenotype ; Prevalence ; Renal Insufficiency, Chronic

BACKGROUND AND OBJECTIVES: We sought to investigate an anti-atherosclerotic and anti-inflammatory effect of sodium-glucose cotransporter-2 (SGLT-2) inhibitors in normoglycemic atherosclerotic rabbit model.METHODS: Male New Zealand white rabbits (n=26) were fed with a 1% high-cholesterol diet for 7 weeks followed by normal diet for 2 weeks. After balloon catheter injury, the rabbits were administered with the Dapagliflozin (1mg/kg/day) or control-medium for 8 weeks (n=13 for each group). All lesions were assessed with angiography, optical coherence tomography (OCT), and histological assessment.RESULTS: Atheroma burden (38.51±3.16% vs. 21.91±1.22%, p<0.01) and lipid accumulation (18.90±3.63% vs. 10.20±2.03%, p=0.047) was significantly decreased by SGLT-2 inhibitor treatment. The SGLT-2 inhibitor group showed lower macrophage infiltration (20.23±1.89% vs. 12.72±1.95%, p=0.01) as well as tumor necrosis factor (TNF)-α expression (31.17±4.40% vs. 19.47±2.10%, p=0.025). Relative area of inducible nitric oxide synthase+ macrophages was tended to be lower in the SGLT-2 inhibitor-treated group (1.00±0.16% vs. 0.71±0.10%, p=0.13), while relative proportion of Arg1⁺ macrophage was markedly increased (1.00±0.27% vs. 2.43±0.64%, p=0.04). As a result, progression of atherosclerosis was markedly attenuated in SGLT-2 inhibitor treated group (OCT area stenosis, 32.13±1.20% vs. 22.77±0.88%, p<0.01). Mechanistically, SGLT-2 treatment mitigated the inflammatory responses in macrophage. Especially, Toll-like receptor 4/nuclear factor-kappa B signaling pathway, and their downstream effectors such as interleukin-6 and TNF-α were markedly suppressed by SGLT-2 inhibitor treatment.CONCLUSIONS: These results together suggest that SGLT-2 inhibitor exerts an anti-atherosclerotic effect through favorable modulation of inflammatory response as well as macrophage characteristics in non-diabetic situation.
Angiography ; Atherosclerosis ; Catheters ; Constriction, Pathologic ; Diet ; Humans ; Interleukin-6 ; Macrophages ; Male ; Nitric Oxide ; Plaque, Atherosclerotic ; Rabbits ; Toll-Like Receptors ; Tomography, Optical Coherence ; Tumor Necrosis Factor-alpha

BACKGROUND AND OBJECTIVES: We sought to investigate an anti-atherosclerotic and anti-inflammatory effect of sodium-glucose cotransporter-2 (SGLT-2) inhibitors in normoglycemic atherosclerotic rabbit model. METHODS: Male New Zealand white rabbits (n=26) were fed with a 1% high-cholesterol diet for 7 weeks followed by normal diet for 2 weeks. After balloon catheter injury, the rabbits were administered with the Dapagliflozin (1mg/kg/day) or control-medium for 8 weeks (n=13 for each group). All lesions were assessed with angiography, optical coherence tomography (OCT), and histological assessment. RESULTS: Atheroma burden (38.51±3.16% vs. 21.91±1.22%, p<0.01) and lipid accumulation (18.90±3.63% vs. 10.20±2.03%, p=0.047) was significantly decreased by SGLT-2 inhibitor treatment. The SGLT-2 inhibitor group showed lower macrophage infiltration (20.23±1.89% vs. 12.72±1.95%, p=0.01) as well as tumor necrosis factor (TNF)-α expression (31.17±4.40% vs. 19.47±2.10%, p=0.025). Relative area of inducible nitric oxide synthase+ macrophages was tended to be lower in the SGLT-2 inhibitor-treated group (1.00±0.16% vs. 0.71±0.10%, p=0.13), while relative proportion of Arg1⁺ macrophage was markedly increased (1.00±0.27% vs. 2.43±0.64%, p=0.04). As a result, progression of atherosclerosis was markedly attenuated in SGLT-2 inhibitor treated group (OCT area stenosis, 32.13±1.20% vs. 22.77±0.88%, p<0.01). Mechanistically, SGLT-2 treatment mitigated the inflammatory responses in macrophage. Especially, Toll-like receptor 4uclear factor-kappa B signaling pathway, and their downstream effectors such as interleukin-6 and TNF-α were markedly suppressed by SGLT-2 inhibitor treatment. CONCLUSIONS These results together suggest that SGLT-2 inhibitor exerts an anti-atherosclerotic effect through favorable modulation of inflammatory response as well as macrophage characteristics in non-diabetic situation.

Background and Objectives: The Korean Organ Transplant Registry (KOTRY) provided data for this third official report on adult heart transplantation (HT), including information from 709 recipients. Methods: Data from HTs performed at seven major centers in Korea between March 2014 and December 2020 were analyzed, focusing on immunosuppression, acute rejection, cardiac allograft vasculopathy (CAV), post-transplant survival, and mechanical circulatory support (MCS) usage. Results: The median ages of the recipients and donors were 56.0 and 43.0 years, respectively.Cardiomyopathy and ischemic heart disease were the most common preceding conditions for HT. A significant portion of patients underwent HT at waiting list status 1 and 0. In the multivariate analysis, a predicted heart mass mismatch was associated with a higher risk of 1-year mortality. Patients over 70 years old had a significantly increased risk of 6-year mortality. The risk of CAV was higher for male donors and donors older than 45 years. Acute rejection was more likely in patients with panel reactive antibody levels above 80%, while statin use was associated with a reduced risk. The employment of left ventricular assist device as a bridge to transplantation increased from 2.17% to 22.4%. Pre-transplant extra-corporeal membrane oxygenation was associated with worse post-transplant survival. Conclusions In this third KOTRY report, we analyzed changes in the characteristics of adult HT recipients and donors and their impact on post-transplant outcomes. The most notable discovery was the increased use of MCS before HT and their impact on post-transplant outcomes.