Cone Repair in Adult Patients with Ebstein Anomaly

Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes.In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.

Incomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.

Incomplete Form of Shone Complex in an Adult Congenital Heart Disease Patient / 대한흉부외과학회지

Shone complex is a rare congenital disorder that involves 4 obstructive lesions of the left heart, as follows: parachute mitral valve, supravalvular mitral ring, subaortic stenosis, and coarctation of the aorta. Incomplete forms with 2 or 3 of these lesions in adult patients have been rarely reported in the literature, meaning that insufficient general data exist concerning the surgical strategy and clinical follow-up. Herein, we report the case of a 31-year-old woman with a diagnosis of incomplete form of Shone complex with parachute mitral valve and coarctation of the aorta who underwent successful single-stage surgical repair.

Effects of Escherichia Coli-derived Recombinant Human Bone Morphogenetic Protein-2 Loaded Porous Hydroxyaptite-based Ceramics on Calvarial Defect in Rabbits

BACKGROUND: Recombinant human bone morphogenetic proteins (rhBMPs) have been widely used in regenerative therapies to promote bone formation. The production of rhBMPs using bacterial systems such as Escherichia coli (E. coli) is estimated to facilitate clinical applications by lowering the cost without compromising biological activity. In clinical practice, rhBMP-2 and osteoconductive carriers (e.g., hydroxyapatite [HA] and bovine bone xenograft) are used together. This study examined the effect of E. coli-derived rhBMP-2 combined with porous HA-based ceramics on calvarial defect in rabbits. METHODS: Six adult male New Zealand white rabbits were used in this study. The experimental groups were divided into the following 4 groups: untreated (NC), bovine bone graft (BO), porous HA (HA) and porous HA with rhBMP-2 (HA-BMP). Four transosseous defects of 8 mm in diameter were prepared using stainless steel trephine bur in the frontal and parietal bones. Histological and histomorphometric analyses at 4 weeks after surgery revealed significant new bone formation by porous HA alone. RESULTS: HA-BMP showed significantly higher degree of bone formation compared with BO and HA group (P<0.05). The average new bone formation % (new bone area per total defect area) of NC, BO, HA, and HA-BMP at 4-week after surgery were 12.65±5.89%, 29.63±6.99%, 28.86±6.17% and 49.56±8.23%, respectively. However, there was no statistical difference in the bone formation between HA and BO groups. CONCLUSIONS: HA-BMP promoted more bone formation than NC, BO and HA alone. Thus, using E. coli-derived rhBMP-2 combined with porous HA-based ceramics can promote new bone formation.

Spontaneous renal artery dissection in Ehlers-Danlos syndrome / 영남의대학술지

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

A Case of Acute Pancreatitis Caused by Bortezomib in a Patient with Multiple Myeloma / 대한췌담도학회지

Bortezomib is a proteasome inhibitor used for the treatment of patients with multiple myeloma. Recently, several case reports about acute pancreatitis caused by Bortezomib were published in the international literature. But Bortezomib induced pancreatitis case was not reported in Korea. Herein, we report a case of acute pancreatitis caused by Bortezomib therapy in a 76-year-old female with multiple myeloma. On three months after the first administration of Bortezomib, the patient visited the hospital with symptoms of acute pancreatitis. The common etiological factors for acute pancreatitis were all excluded. Then the patient was diagnosed as Bortezomib-induced pancreatitis. After cessation of Bortezomib, she showed clinical and laboratory improvement.

Effect of High Dose Rosuvastatin Loading before Percutaneous Coronary Intervention on Contrast-Induced Nephropathy

BACKGROUND AND OBJECTIVES: Contrast-induced nephropathy (CIN) is associated with increased morbidity and mortality. This observational, non-randomized study evaluated the effect of rosuvastatin loading before percutaneous coronary intervention (PCI) on the incidence of CIN in patients with acute coronary syndrome (ACS). SUBJECTS AND METHODS: A total of 824 patients who underwent PCI for ACS were studied (408 patients in the statin group=40 mg rosuvastatin loading before PCI; 416 patients of control group=no statin pretreatment). Serum creatinine concentrations were measured before and 24 and 48 hours after PCI. The primary endpoint was development of CIN defined as an increase in serum creatinine concentration of > or =0.5 mg/dL or > or =25% above baseline within 72 hours after PCI. RESULTS: The incidence of CIN was significantly lower in the statin group than that in the control group (18.8% vs. 13.5%, p=0.040). The maximum percent changes in serum creatinine and estimated glomerular filtration rate in the statin group within 48 hours were significantly lower than those in the control group (5.84+/-22.59% vs. 2.43+/-24.49%, p=0.038; -11.44+/-14.00 vs. -9.51+/-13.89, p=0.048, respectively). The effect of rosuvastatin on preventing CIN was greater in the subgroups of patients with diabetes, high-dose contrast medium, multivessel stents, high baseline C-reactive protein, and myocardial infarction. A multivariate analysis revealed that rosuvastatin loading was independently associated with a decreased risk for CIN (odds ratio, 0.64; 95% confidence interval, 0.43-0.95, p=0.026). CONCLUSION: High-dose rosuvastatin loading before PCI was associated with a significantly lower incidence of CIN in patients with ACS.

Successful Endovascular Management of Intraoperative Graft Limb Occlusion and Iliac Artery Rupture Occurred during Endovascular Abdominal Aortic Aneurysm Repair

For high-risk patients, endovascular aortic aneurysm repair (EVAR) is a good option but may lead to serious complications, which should be addressed immediately. A 75-year-old man with a history of abdominal surgery underwent EVAR for an aneurysm of the abdominal aorta and iliac arteries. During EVAR, iliac artery rupture and graft limb occlusion occurred, and they were successfully managed by the additional deployment of an iliac stent graft and balloon thrombectomy, respectively. We, herein, report a rare case of the simultaneous development of the two fatal complications treated by the endovascular technique.

Percutaneous Extracorporeal Membrane Oxygenation for Graft Dysfunction after Heart Transplantation

BACKGROUND: We evaluated the safety and efficacy of percutaneous extracorporeal membrane oxygenation (ECMO) in patients with primary graft dysfunction after heart transplantation. METHODS: Of 65 patients (44 males and 21 females) who underwent heart transplantation from January 2006 to December 2012, 13 patients (group I) needed peripheral ECMO support due to difficulty in weaning from cardiopulmonary bypass (CPB) and 52 patients (group II) were weaned from CPB without mechanical support. The mean age of the patients at the time of operation was 54.4+/-13.6 years. There were no differences in the preoperative characteristics of the two groups. Multivariable analysis was performed to identify the risk factors for ECMO therapy. RESULTS: All group I patients were successfully weaned from ECMO after 53+/-9 hours of circulatory support. Early mortality occurred in four patients (1 [7.7%] in group I and 3 [5.8%] in group II, p>0.999). There were no differences in the postoperative complications between the two groups, with the exception of reoperation for bleeding. A greater number of group I patients underwent reoperation for bleeding (5 [38.5%] in group I vs. 6 [11.5%] in group II, p=0.035). In multivariable analysis, preoperative mechanical support (ECMO and intra-aortic balloon pump) and longer CPB time were the risk factors of ECMO therapy for graft dysfunction (odds ratio, 6.377; 95% confidence interval, 1.519 to 26.77; p=0.011 and odds ratio, 1.010; 95% confidence interval, 1.001 to 1.019; p=0.033). CONCLUSION: Percutaneous ECMO support could be a viable option for rescuing patients when graft dysfunction refractory to medical management develops after heart transplantation.

Banked Vena Caval Homograft Replacement of the Inferior Vena Cava for Primary Leiomyocsarcoma

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor. Herein, we report the case of a 52-year-old male patient who had postprandial abdominal distension and right upper quadrant abdominal pain. The abdominal computed tomography (CT) angiogram showed an IVC mass extending from the infrahepatic to the suprarenal inferior vena cava. The radiologic findings were suggestive of an IVC leiomyosarcoma. Surgical resection and reconstruction with a cryopreserved homograft were performed. The follow-up abdominal CT angiogram revealed the patient to be disease-free 6 months after surgery with patency of the IVC and renal vein.

Surgical Treatment of Mediastinal Aspergilloma in a Immunocompetent Patient

Aspergillus is a common saprophytic fungi of the human airways and causes a broad spectrum of diseases, ranging from aspergilloma to invasive aspergillosis. There are few reports on mediastinal aspergilloma without any underlying pulmonary disease or immunocompromise. Herein, we report a case of mediastinal aspergilloma that we experienced and treated by thoracoscopic resection and oral antifungal medication.

Extramedullary Hematopoiesis at the Posterior Mediastinum in Patient with Hereditary Spherocytosis: A Case Report

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.

Expression of Claudin-1, p53 and E-cadherin in Pseudoepitheliomatous Hyperplasia and Squamous Cell Carcinoma of the Head and Neck

BACKGROUND: Pseudoepitheliomatous hyperplasia (PEH) is a reactive proliferation of surface epithelium and can be confused with invasive squamous cell carcinoma (SCC) in head and neck biopsy specimens. To distinguish PEH from invasive SCC, immunohistochemical staining for claudin-1, E-cadherin and p53 was performed. METHODS: Eighteen cases of PEH and 29 invasive SCC from head and neck lesions were immunostained and examined. RESULTS: The invasive SCC showed increased staining of claudin-1 (p<0.001) and p53 (p<0.001) and decreased staining of E-cadherin (p=0.005) compared to the PEH specimens. The combined score calculated by adding the positive sum of claudin-1 and p53 and subtracting E-cadherin was useful for the differentiation of SCC from PEH (89.7% sensitivity and 88.9% specificity, p<0.001). CONCLUSION: The combined immunostaining for claudin-1, p53 and E-cadherin may help differentiate PEH from invasive SCC. The results of this study suggest that the increased expression of claudin-1 and p53 and the decreased expression of E-cadherin maybe markers for the aggressive growth of invasive SCC.