Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Background and Objectives: This study aimed to analyze the outcomes of Fontan surgery in the Republic of Korea, as there were only a few studies from Asian countries. Methods: The medical records of 1,732 patients who underwent Fontan surgery in 10 cardiac centers were reviewed. Results: Among them, 1,040 (58.8%) were men. The mean age at Fontan surgery was 4.3±4.2 years, and 395 (22.8%) patients presented with heterotaxy syndrome. According to the types of Fontan surgery, 157 patients underwent atriopulmonary (AP) type; 303, lateral tunnel (LT) type; and 1,266, extracardiac conduit (ECC) type. The overall survival rates were 91.7%, 87.1%, and 74.4% at 10, 20, and 30 years, respectively. The risk factors of early mortality were male, heterotaxy syndrome, AP-type Fontan surgery, high mean pulmonary artery pressure (mPAP) in pre-Fontan cardiac catheterization, and early Fontan surgery year. The risk factors of late mortality were heterotaxy syndrome, genetic disorder, significant atrioventricular valve regurgitation (AVVR) before Fontan surgery, high mPAP in pre-Fontan cardiac catheterization, and no fenestration. Conclusions In Asian population with a high incidence of heterotaxy syndrome, the heterotaxy syndrome was identified as the poor prognostic factors for Fontan surgery. The preoperative low mPAP and less AVVR are associated with better early and long-term outcomes of Fontan surgery.

Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis has been reported in Graves' disease patients treated with propylthiouracil (PTU). In most cases, it's renal involvements has been known as crescentic glomerulonephritis. A 41-year-old female patient with hyperthyroidism has been treated with PTU for 3 years. The patient had developed isolated hematuria and polyarthralgia with p-ANCA positivity, 6 months and 10 months after PTU treatment, respectively. She had been continuously treated with PTU until she was admitted at our hospital. Three months before admission, polyarthralgia was aggravated and purpura in both lower legs and hands was developed. Urinalysis revealed hematuria, proteinuria. Serologic evaluation showed p-ANCA positive. Skin biopy showed leukocytoclastic vasculitis and renal biopsy showed focal segmental glomerulosclerosis (FSGS). She was diagnosed as PTU-associated vasculitis with FSGS. Polyarthralgia and purpura were improved after discontinuing the PTU with prednisolone treatment but hematuria, proteinuria were not changed. We suggest that progression of PTU-associated focal segmental necrotizing glomerulonephritis to FSGS over two years might be due to continued PTU medication.
Adult ; Antibodies, Antineutrophil Cytoplasmic* ; Arthralgia ; Biopsy ; Cytoplasm ; Female ; Glomerulonephritis ; Glomerulosclerosis, Focal Segmental* ; Graves Disease ; Hand ; Hematuria ; Humans ; Hyperthyroidism ; Leg ; Prednisolone ; Propylthiouracil ; Proteinuria ; Purpura ; Skin ; Urinalysis ; Vasculitis*

Human chorionic gonadotropin (HCG) is one of the glycoproteins families synthesized by the placenta, and consists of 2 noncovalently joined subunits, namely, alpha and beta. The alpha and beta-subunits have a structural homology with the alpha and beta-subunits of TSH and LH. The thyrotropic action of HCG results from its structural similarity to TSH, so beta-HCG can bind to the TSH receptor in the thyroid gland. A high level of HCG, accompanied by an increased thyroid hormone level, can be observed in gestational trophoblastic diseases (GTD), such as a hydatidiform mole or a choriocarcinoma. However, the clinical symptoms of hyperthyroidism in GTD are rarely observed. A 27-years-old woman, admitted due to an amenorrhea of 11 weeks duration, with thyrotoxic symptoms, such as weight loss, palpitation, sweating, tremor, heat intolerance and anxiety, was evaluated. Her serum free T4 level was 8 times higher than normal, and her serum beta-HCG level was over 1,000,000IU/L. She had a curettage operation, with the pathological findings of a complete hydatidiform mole. These thyrotoxic symptoms developed due to a hydatidiform mole, and were accompanied with a highly increased serum beta-HCG level. After evacuation of the molar tissue, the thyroid hormone and thyrotoxic symptoms normalized. Here, this case is reported, with brief review of the literature.
Amenorrhea ; Anxiety ; Choriocarcinoma ; Chorionic Gonadotropin ; Curettage ; Female ; Gestational Trophoblastic Disease ; Glycoproteins ; Hot Temperature ; Humans ; Hydatidiform Mole* ; Hyperthyroidism ; Molar ; Placenta ; Pregnancy ; Receptors, Thyrotropin ; Sweat ; Sweating ; Thyroid Gland ; Thyrotoxicosis* ; Tremor ; Weight Loss