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*

No abstract available.
Cardiomyopathy, Hypertrophic ; Mitral Valve Insufficiency ; Mitral Valve

Erdheim-Chester disease (ECD), also known as non-Langerhans cell histiocytosis, is a multi-systemic disease with unclear pathogenesis. Based on a small number of case studies, pegylated interferon-α (PEG-IFN-α) has been used as the front-line treatment option. However, there are limited data regarding administration of ropegylated-interferon α-2b (ROPEG-IFN-α 2b) for ECD patients. Herein, we report two cases of severe ECD treated with two types of PEG-IFN-α. One patient with heart and skeleton involvement and BRAF V600E mutation was treated with weekly PEG-IFN-α 2a. Another patient with bone involvement and no BRAF V600E mutation was administered monthly ROPEG-IFN-α 2b. The two types of PEG-IFN-α showed excellent disease control, excellent survival outcomes, and manageable toxicities in ECD patients. These results suggest that ROPEG-IFN-α 2b could be used equivalently to PEG-IFN-α 2a for management of advanced ECD.

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

BACKGROUND: Atrial fibrillation (AF) is a well-established risk factor for stroke. Interestingly, ischemic stroke increases risk of incident AF in patients without prior diagnosed AF. For better risk stratification for post-stroke AF, we studied left atrial (LA) size and mechanical function using two-dimensional (2D) speckle tracking imaging in patients with acute ischemic stroke. METHODS: A total of 227 patients (132 males, age 67 ± 12) with acute ischemic stroke without a history of AF underwent 2D transthoracic echocardiography and speckle tracking imaging for the assessment of LA volume index and global LA longitudinal strain (LALS). From clinical variables, the CHA2DS2-VASc score and National Institute of Health Stroke Scale (NIHSS) were calculated in each patient. Post-stroke AF was defined as newly diagnosed AF during the course after ischemic stroke. RESULTS: Post-stroke AF occurred in 25 patients (11%). Patients with post-stroke AF were older and showed a higher tendency of CHA2DS2-VASc score, significantly higher log NIHSS, larger LA volume index and lower global LALS than those without. In multivariate analysis, global LALS was an independent predictor for post-stroke AF (hazard ratio 0.90, 95% confidence interval 0.83.0.97, p < 0.01) after controlling for confounding factors. Furthermore, global LALS provided incremental predictive value for post-stroke AF over the CHA2DS2-VASc score, NIHSS, and LA volume index. The global LALS < 14.5% better distinguished post-stroke AF (area under the curve 0.837, sensitivity 60%, specificity 95%, p < 0.01) than CHA2DS2-VASc score. CONCLUSION: Global LALS as a marker of LA mechanical function has incremental predictive value for post-stroke AF in patients with acute ischemic stroke.
Atrial Fibrillation* ; Echocardiography ; Heart Atria ; Humans ; Male ; Multivariate Analysis ; Risk Factors ; Sensitivity and Specificity ; Stroke*