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.

Necrotizing sarcoid granulomatosis (NSG), now referred to as “sarcoidosis with NSG pattern,” is an uncommon variant of sarcoidosis. NSG is characterized by a trio of features: sarcoid granulomas, vasculitis, and extensive areas of necrosis. Symptoms can include cough, fever, chest pain, and dyspnea, typically presenting as either solitary or multiple lung nodules or masses. In this report, we describe a case of NSG accompanied by a persistent low-grade fever. Unlike the dominant presentation of NSG with single or multiple nodules, our case demonstrated diffuse micronodules with combined perilymphatic and random distribution on CT.Histological examination revealed widespread necrotizing granulomas surrounded by anthracotic pigmentation, alongside necrosis and vasculitis, diverging from the classic presentation of sarcoidosis. The diagnosis of NSG was established through a multidisciplinary discussion. The patient was administered oral prednisolone that led to noticeable clinical and radiological improvement within three months.

Necrotizing sarcoid granulomatosis (NSG), now referred to as “sarcoidosis with NSG pattern,” is an uncommon variant of sarcoidosis. NSG is characterized by a trio of features: sarcoid granulomas, vasculitis, and extensive areas of necrosis. Symptoms can include cough, fever, chest pain, and dyspnea, typically presenting as either solitary or multiple lung nodules or masses. In this report, we describe a case of NSG accompanied by a persistent low-grade fever. Unlike the dominant presentation of NSG with single or multiple nodules, our case demonstrated diffuse micronodules with combined perilymphatic and random distribution on CT.Histological examination revealed widespread necrotizing granulomas surrounded by anthracotic pigmentation, alongside necrosis and vasculitis, diverging from the classic presentation of sarcoidosis. The diagnosis of NSG was established through a multidisciplinary discussion. The patient was administered oral prednisolone that led to noticeable clinical and radiological improvement within three months.

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.

Necrotizing sarcoid granulomatosis (NSG), now referred to as “sarcoidosis with NSG pattern,” is an uncommon variant of sarcoidosis. NSG is characterized by a trio of features: sarcoid granulomas, vasculitis, and extensive areas of necrosis. Symptoms can include cough, fever, chest pain, and dyspnea, typically presenting as either solitary or multiple lung nodules or masses. In this report, we describe a case of NSG accompanied by a persistent low-grade fever. Unlike the dominant presentation of NSG with single or multiple nodules, our case demonstrated diffuse micronodules with combined perilymphatic and random distribution on CT.Histological examination revealed widespread necrotizing granulomas surrounded by anthracotic pigmentation, alongside necrosis and vasculitis, diverging from the classic presentation of sarcoidosis. The diagnosis of NSG was established through a multidisciplinary discussion. The patient was administered oral prednisolone that led to noticeable clinical and radiological improvement within three months.