Recent Surgical Outcomes ofCongenital Heart Disease according toKorea Heart Foundation Data

Background and Objectives@#This study presents an update of the surgical outcomes ofcongenital heart disease (CHD) according to Korea Heart Foundation (KHF) data. @*Methods@#We investigated the data of the 7,305 patients who were economically supportedby KHF in 2000–2014. Of them, we analyzed surgical outcomes of the 6,599 patients whounderwent CHD surgery. @*Results@#The median patient age was 1.9 years (range, 0–71.5 years). Of the 6,599 patients,5,616 (85.1%) underwent biventricular repair and 983 (14.9%) underwent palliativeprocedures. The mean Basic Aristotle Score was 6.6±2.2. A complex procedure (definedas Basic Aristotle Score above 6) was performed in 3,368 patients (51.0%). The earlymortality rate was 3.8%, while the late mortality rate was 1.8%. Previous reports of the KHF(1984–1999) showed that the early surgical and late mortality rates were 8.6%, and 5.3%,respectively. There were 491 neonates (7.4%); among them, the early mortality rate was 12.2%and late mortality rate was 3.7%. There were 2,617 infants (40.0%); among them, the earlymortality rate was 6.0% and the late mortality rate was 2.3%. A total of 591 patients from 30countries were helped by the KHF. @*Conclusions@#More neonatal surgeries (491 vs. 74 patients) were performed than those inthe past (1984–1999). The surgical outcomes were much better than before. Our surgicaloutcomes revealed that the Republic of Korea has been transformed from a country receivinghelp to a country that helps other low socioeconomic status countries.

Dasatinib Inhibits Lyn and Fyn Src-Family Kinases in Mast Cells to Suppress Type I Hypersensitivity in Mice

Mast cells (MCs) are systemically distributed and secrete several allergic mediators such as histamine and leukotrienes to cause type I hypersensitivity. Dasatinib is a type of anti-cancer agent and it has also been reported to inhibit human basophils. However, dasatinib has not been reported for its inhibitory effects on MCs or type I hypersensitivity in mice. In this study, we examined the inhibitory effect of dasatinib on MCs and MC-mediated allergic response in vitro and in vivo. In vitro, dasatinib inhibited the degranulation of MCs by antigen stimulation in a dose-dependent manner (IC 50 , ~34 nM for RBL-2H3 cells; ~52 nM for BMMCs) without any cytotoxicity. It also suppressed the secretion of inflammatory cytokines IL-4 and TNF-α by antigen stimulation. Furthermore, dasatinib inhibited MC-mediated passive cutaneous anaphylaxis (PCA) in mice (ED 50 , ~29 mg/kg). Notably, dasatinib significantly suppressed the degranulation of MCs in the ear tissue. As the mechanism of its effect, dasatinib inhibited the activation of Syk and Syk-mediated downstream signaling proteins, LAT, PLCγ1, and three typical MAP kinases (Erk1/2, JNK, and p38), which are essential for the activation of MCs. Interestingly, in vitro tyrosine kinase assay, dasatinib directly inhibited the activities of Lyn and Fyn, the upstream tyrosine kinases of Syk in MCs. Taken together, dasatinib suppresses MCs and PCA in vitro and in vivo through the inhibition of Lyn and Fyn Src-family kinases. Therefore, we suggest the possibility of repositioning the anti-cancer drug dasatinib as a treatment for various MC-mediated type I hypersensitive diseases.

Chylothorax after Surgery for Congenital Cardiac Disease: A Prevention and Management Protocol

Background@#Chylothorax after congenital heart surgery is not an uncommon complication, and it is associated with significant morbidity. However, consensus treatment guidelines are lacking. To improve the treatment outcomes of patients with postoperative chylothorax, we implemented a standardized management protocol at Severance Hospital in September 2014. @*Methods@#A retrospective review of patients treated at a single center was done. All corrective and palliative operations for congenital heart disease performed at our institution between January 2008 and April 2018 were reviewed. The incidence and treatment outcomes of postoperative chylothorax were analyzed. @*Results@#The incidence of chylothorax was 1.9%. Sixty-one percent of the patients could be managed with a low-fat diet, while 28% of the patients required complete restriction of enteral feeding. Thoracic duct embolization was performed in 2 patients and chest tube drainage decreased immediately after the procedure. No patient required thoracic duct ligation or pleurodesis. After implementation of the institutional management protocol, the number of chest tube drainage days decreased (median, 24 vs. 14 days; p=0.45). @*Conclusion@#Implementing a strategy to reduce postoperative chylothorax resulted in an acceptable incidence of postoperative chylothorax. Instituting a clinical practice protocol helped to curtail the treatment duration and to decrease the requirement for surgical treatment. Image-guided embolization of the thoracic duct is an effective treatment for postoperative chylothorax.

Conservative Management and Postoperative Rehabilitation of Chronic Lateral Ankle Instability / 대한족부족관절학회지

As a result of the increased participation in recreational and competitive sports activity, the incidence of chronic lateral instability has risen. Because chronic ankle instability can cause significant comorbidity and increase the social cost, the interest in this issue is growing. Although there are well-documented and effective surgical treatments for managing this condition, conservative treatments are a viable first option in selected patients. Through conservative treatment, surgical treatments can be reduced or delayed without necessarily affecting the overall outcome, but the failure of conservative treatment results in the need for surgical treatment to reduce the long term complications. Appropriate postoperative rehabilitation is essential for adequate functional recovery, even when surgical treatment is required.

An Anti-Cancer Drug Candidate CYC116 Suppresses Type I Hypersensitive Immune Responses through the Inhibition of Fyn Kinase in Mast Cells

Mast cells are the most prominent effector cells of Type 1 hypersensitivity immune responses. CYC116 [4-(2-amino-4-methyl-1,3-thiazol-5-yl)-N-[4-(morpholin-4-yl)phenyl] pyrimidin-2-amine] is under development to be used as an anti-cancer drug, but the inhibitory effects of CYC116 on the activation of mast cells and related allergy diseases have not reported as of yet. In this study, we demonstrated, for the first time, that CYC116 inhibited the degranulation of mast cells by antigen stimulation (IC₅₀, ∼1.42 µM). CYC116 also inhibited the secretion of pro-inflammatory cytokines including TNF-α (IC₅₀, ∼1.10 µM), and IL-6 (IC₅₀, ∼1.24 µM). CYC116 inhibited the mast cell-mediated allergic responses, passive cutaneous anaphylaxis (ED50, ∼22.5 mg/kg), and passive systemic anaphylaxis in a dose-dependent manner in laboratory experiments performed on mice. Specifically, CYC116 inhibited the activity of Fyn in mast cells and inhibited the activation of Syk and Syk-dependent signaling proteins including LAT, PLCγ, Akt, and MAP kinases. Our results suggest that CYC116 could be used as an alternative therapeutic medication for mast cell-mediated allergic disorders, such as atopic dermatitis and allergic rhinitis.

Pediatric Ventricular Assist Device

There have been great advances in ventricular assist device (VAD) treatment for pediatric patients with advanced heart failure. VAD support provides more time for the patient in the heart transplant waiting list. Augmented cardiac output improves heart failure symptoms, end-organ function, and general condition, and consequently provides beneficial effects on post-transplant outcomes. Miniaturized continuous flow devices are more widely adopted for pediatric patient with promising results. For infants and small children, still paracorporeal pulsatile device is the only option for long-term support. Younger age, congenital heart disease, biventricular support, patient's status and end-organ dysfunction at the time of implantation are risks for poor outcomes. Patient selection, timing of implantation, and selection of device for each patient are critical for optimal clinical outcomes.

Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair / 대한흉부외과학회지

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

Pediatric Ventricular Assist Device

There have been great advances in ventricular assist device (VAD) treatment for pediatric patients with advanced heart failure. VAD support provides more time for the patient in the heart transplant waiting list. Augmented cardiac output improves heart failure symptoms, end-organ function, and general condition, and consequently provides beneficial effects on post-transplant outcomes. Miniaturized continuous flow devices are more widely adopted for pediatric patient with promising results. For infants and small children, still paracorporeal pulsatile device is the only option for long-term support. Younger age, congenital heart disease, biventricular support, patient's status and end-organ dysfunction at the time of implantation are risks for poor outcomes. Patient selection, timing of implantation, and selection of device for each patient are critical for optimal clinical outcomes.

Emergency Pulmonary Artery–to-Systemic Artery Shunt to Break the Positive Feedback Loop of a Pulmonary Hypertensive Crisis after Neonatal Coarctation Repair

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

Current Updates in Treatment of Osteochondral Lesions of the Talus / 대한족부족관절학회지

Despite the increasing number of osteochondral lesions of the talus, there are a lack of definite evidence-based treatment protocols. Several types of treatments are available, each having their advantages and disadvantages. First-line therapy consists of well-conducted conservative treatment. Surgical treatment is the second choice. Treatments are chosen based on the size of the lesion, location, chronicity, and the condition of the neighboring cartilage. This article reviews the current updates in the treatment of osteochondral lesions of the talus to help clinicians use the available treatment strategies more efficiently.

Concomitant Right Ventricular Outflow Tract Cryoablation during Pulmonary Valve Replacement in a Patient with Tetralogy of Fallot

A 38-year-old female patient with a history of tetralogy of Fallot repair at 10 years of age underwent pulmonary valve replacement with a mechanical prosthesis, tricuspid annuloplasty, and right ventricular outflow tract cryoablation due to pulmonary regurgitation, tricuspid regurgitation, and multiple premature ventricular contractions with sustained ventricular tachycardia. After surgery, she had an uneventful postoperative course with arrhythmia monitoring. She was discharged without incident, and a follow-up Holter examination showed a decrease in the number of ventricular ectopic beats from 702 to 41.

Neurogenic Pain Disorder in the Foot and Ankle: Peripheral Neuropathy / 대한정형외과학회잡지

Most common peripheral neuropathy around foot and ankle is diabetic neuropathy, but there are another cause of peripheral neuropathy, such as rheumatoid arthritis, metabolic disease, genetic disease, toxic material, and so on. The main symptom of peripheral neuropathy is pain. The disturbance of sensory and balancing, weakness of muscle, deformity of foot and neuropathic arthropathy are also the symptoms of the peripheral neuropathy. History taking is most important to identify the cause of peripheral neuropathy. Neurological exam have to include the pin prick test, vibration test, 10 g-monofilamant test and ankle reflex test. Simple radiography is essential to observe the deformities or neuropathic arthropathy at foot and ankle. The presence of peripheral neuropathy, involvement and severity can be identified from nerve conduction study. The study of occlusive arteritis is essential for diabetic neuropathy. The medical treatment of associated disease is important but the pain of peripheral neuropathy should be controlled simultaneously. Medicine include the antidepressants, anticonvulsants, opioids and topical agents. The surgical treatment of peripheral neuropathy include lengthening of Achilles tendon, correction of deformity, the total contact cast and arthrodesis. Surgical decompression of specific nerve might helpful in pain control of peripheral neuropathy.

Fractures of the Tarsal Bone

Fractures of the tarsal bone, such as the navicular, cuboid, and cuneiform, are very rare. These injuries can lead to serious walking difficulties due to pain and deformity of the foot with delayed diagnosis of tarsal bone fractures during an injury to multiple lower extremities. The diagnosis can be done on simple radiographs. Sometime weight bearing radiographs or stress radiographs may be needed for further evaluation. Computed tomography is the most widely available diagnostic tool. Navicular and cuneiform account for the medial column of the foot, whereas cuboid for the lateral column. The treatment of tarsal bone fractures is primarily conservative management, but operative treatment is recommended for intra-articular displacement, dislocation, or shortening of the medial or lateral column of the foot. The operative treatments include screw fixation, plate fixation, or external fixation. Complications include malunion, nonunion, posttraumatic arthritis, avascular necrosis, and deformity of the foot. Tarsal bone fracture has to be evaluated carefully to prevent serious complications.

Results of Extracorporeal Cardiopulmonary Resuscitation in Children

BACKGROUND: Survival of children experiencing cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) is very poor. We sought to examine current era outcomes of extracorporeal CPR (ECPR) support for refractory arrest. METHODS: Patients who were <18 years and underwent ECPR between November 2013 and January 2016 were including in this study. We retrospectively investigated patient medical records. RESULTS: Twelve children, median age 6.6 months (range, 1 day to 11.7 years), required ECPR. patients' diseases spanned several categories: congenital heart disease (n=5), myocarditis (n=2), respiratory failure (n=2), septic shock (n=1), trauma (n=1), and post-cardiotomy arrest (n=1). Cannulation sites included the neck (n=8), chest (n=3), and neck to chest conversion (n=1). Median duration of extracorporeal membrane oxygenation was five days (range, 0 to 14 days). Extracorporeal membrane oxygenation was successfully discontinued in 10 (83.3%) patients. Nine patients (75%) survived more than seven days after support discontinuation and four patients (33.3%) survived and were discharged. Causes of death included ischemic brain injury (n=4), sepsis (n=3), and gastrointestinal bleeding (n=1). CONCLUSION: ECPR plays a valuable role in children experiencing refractory cardiac arrest. The weaning rate is acceptable; however, survival is related to other organ dysfunction and the severity of ischemic brain injury. ECPR prior to the emergence of end-organ injury and prevention of neurologic injury might enhance survival.

Cytomegalovirus Myocarditis Required Extracorporeal Membrane Oxygenation Support Followed by Ganciclovir Treatment in Infant

A 7-month-old girl with no medical history was treated with mechanical circulatory support due to myocarditis. Her cardiac contractility did not improve despite more than one week of extracorporeal membrane oxygenation treatment. Thus, we planned a heart transplant. However, a high level of cytomegalovirus was found in blood laboratory results by quantitative polymerase chain reaction. The patient's heart contractility recovered to normal range four days after ganciclovir treatment. She was discharged with slightly decreased cardiac contractility with a left ventricular ejection fraction of 45%.

Left Atrial Decompression by Percutaneous Left Atrial Venting Cannula Insertion during Venoarterial Extracorporeal Membrane Oxygenation Support

Patients with venoarterial extracorporeal membrane oxygenation (ECMO) frequently suffer from pulmonary edema due to left ventricular dysfunction that accompanies left heart dilatation, which is caused by left atrial hypertension. The problem can be resolved by left atrium (LA) decompression. We performed a successful percutaneous LA decompression with an atrial septostomy and placement of an LA venting cannula in a 38-month-old child treated with venoarterial ECMO for acute myocarditis.

Intracardiac Thrombosis Involving All Four Cardiac Chambers after Extracardiac Membranous Oxygenation Associated with MTHFR Mutations

A 4-month-old boy diagnosed with acute myocarditis was treated with extracorporeal membrane oxygenation (ECMO). Follow-up echocardiography eight hours after ECMO revealed intracardiac thrombosis involving all four heart chambers. Because of the high risk of systemic embolization due to a pedunculated thrombus of the aortic valve, we performed an emergency thrombectomy. After the operation, the patient had a minor neurologic sequela of left upper arm hypertonia, which had almost disappeared at the last outpatient clinic two months later. He was diagnosed with a major mutation in MTHFR (methylenetetrahydrofolate reductase), which is related to thrombosis.

Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension / 소아과

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

Clinicopathologic Characteristics of Superficial Gastric Cancer Diagnosed at Primary Health Care Institutions in 2011 / 대한소화기학회지

BACKGROUND/AIMS: Stomach cancer is prevalent in Korea. The purpose of this study was to evaluate the characteristics of superficial gastric cancers detected at SOK Sokpeynhan Internal Medical Network, the nationwide primary health care institutions. METHODS: We prospectively analysed the clinicopathologic and endoscopic characteristics of 218 superficial gastric cancer patients diagnosed using gastric endoscopy at SOK network from January 2011 through December 2011. RESULTS: The mean age was 58.5 years old and male to female ratio was 1.7 : 1. Asymptomatic patients were most common (45.0%). The macroscopic classification revealed that simple types (63.8%) were more common than complex types (36.2%). The most common type was IIc (28.4%) and other types were as follows; IIb (16.1%), IIb+IIc (13.3%), IIa (10.6%), III (9.2%), IIa+IIc (7.3%), IIc+IIa (6.0%), IIc+IIb (5.0%). The most commonly involved sites were the body (53.1%) and greater curvature (32.6%) of the stomach. The size of lesion was less than 1 cm (69.3%) and less than 5 mm (33.5%) in diameter. The most common pathologic type was tubular adenocarcinoma (75.7%). Helicobacter pylori infection rate was 50.2%. Fifty five percent of the cases were diagnosed via endoscopy of National Health Insurance Corporation screenings. CONCLUSIONS: Superficial gastric cancers in 2011 at primary health care SOK network were different from those of previous reports. Type IIc was most common but type IIb was more prevalent and the body and greater curvature of the stomach were the most commonly involved sites. Therefore, careful observation of the proximal gastric mucosa and mucosal color change is needed.

The Differences of Fixation Method in Proximal Chevron Osteotomy for Hallux Valgus: K-Wire, Cannulated Screw, Plate / 대한족부족관절학회지

PURPOSE: The authors evaluated the differences between K-wires and Cannulated screw, plate for fixing the proximal metatarsal chevron osteotomy of moderate and severe hallux valgus. MATERIALS AND METHODS: There were 62 patients (79 feet) who were moderate and severe degree hallux valgus according to the classification of Mann. They all got the proximal chevron osteotomy when correcting the deformity. We divided the patients into 4 groups, Two K-wire fixed group as A, one cannulated screw fixed group as B. Two cannulated screw fixed group as C, Plate fixed group as D, Group A were patients (26 feet) and Group B were patients (9 feet), Group C were patients (31 feet) and Group D were patients (13 feet). Preoperative, postoperative and follow-up hallux valgus angle (HVA) and intermetatarsal angle (IMA) were measured for each patient. ANOVA test and Student t-test were done for statistical analysis. RESULTS: Mean follow up period was 43.8 months (range: 12~82 months). Preoperative mean IMA was 16.4+/-3.5, 17.7+/-11.3, 17.3+/-5.9 and 16.6+/-2.3 degrees in respectively group A, B, C, D. Immediate postoperative mean IMA was 5.6+/-3.4, 7.3+/-4.4, 7.6+/-4.4 and 6.7+/-2.8 degrees in respectively group A, B, C, D. The final mean IMA was 8.9+/-4.5, 15.2+/-7.5, 10.3+/-4.4 and D 7.7+/-3.5 degrees in respectively group A, B, C, D. There were significant statistical increase in final mean IMA of group B and C (p<0.05). CONCLUSION: The IMA was significantly increased in the group which used one or two cannulated screw for fixation on follow up, therefore more caution should be needed when using one or two cannulated screw fixation technique after proximal chevron osteotomy.