Kommerell diverticulum is a rare congenital anomaly of the aortic arch characterized by dilation at the proximal descending aorta, which gives rise to an aberrant subclavian artery. Kommerell diverticulum is usually asymptomatic, but can also be associated with symptoms due to compression of the esophagus or trachea, and can rarely be fatal due to dissection or rupture of the diverticulum. Here, we report a rare case of dysphagia caused by compression of the esophagus by Kommerell diverticulum originating from the right-sided aortic arch.

BACKGROUND: Some controversy still exists concerning the operative indications of coronary fistulas. Nevertheless, a short-term and long-term outcomes are excellent with surgical interventions. In this study, we assessed our surgical results on this disease entity during the last 20 years. Anatomic diversity was described as well. MATERIAL AND METHOD: From April 1986 to March 2005, 20 patients with coronary fistulas underwent surgical correction in Seoul National University Children's Hospital. Their medical records were reviewed retrospectively. RESULT: Twelve patients (60%) were asymptomatic prior to surgery. All had electrocardiogram and echocardiogram and all but 3 had coronary angiogram preoperatively. Anatomically, none of them had two or more coronary fistulas. The sites of origin were left coronary system in 11 patients and right in 9. The draining sites were right ventricle in 11, right atrium in 3, left ventricle in 3, main pulmonary artery in 2, and superior vena cavae in 1. All of the involved, the coronary arteries were dilated or aneurismal. In 1 case, there was atherosclerotic change but no ischemic evidence in preoperative electrocardiogram. Operative techniques included external obliteration (13), internal obliteration (5), and both (2). External obliteration was done by ligation of the fistulous tract only in 7 patients, by fistula ligation plus plication in 3 and by plication or patch closure via fistulotomy in 3. There was no operative mortality. All of postoperative morbidities including transient sinus arrhythmia (2), complete atrioventricular block (1), decreased left ventricular function (2), ventricular tachycardia (1), pericarditis (1), and seizure (1) improved on discharge. The mean follow-up was 55.1+/-50.2 months (4.0 months~18.0 years) and there were no recurrences of fistula. There was 1 second operation for aortic root aneurysm, which developed after external patch closure of right coronary fistula. CONCLUSION: We demonstrated here that coronary fistulas can be cured with excellent clinical outcome and low operative risk under precise diagnosis. Understanding the anatomic diversity will help to construct surgical plans.
Aneurysm ; Arrhythmia, Sinus ; Atrioventricular Block ; Coronary Vessels ; Diagnosis ; Electrocardiography ; Fistula* ; Follow-Up Studies ; Heart Atria ; Heart Ventricles ; Humans ; Ligation ; Medical Records ; Mortality ; Pericarditis ; Pulmonary Artery ; Recurrence ; Retrospective Studies ; Seizures ; Seoul ; Tachycardia, Ventricular ; Vena Cava, Superior ; Ventricular Function, Left

Modified Norwood procedure with maintaining cardiac beat was done in a 30-day-old neonate. Procedure was done with regional perfusion of innominate and coronary artery. Postoperative course was uneventful. Second-stage operation (bi-directional cavopulmonary shunt) was done 4 months later. The diameter of ascending aorta was more than 5 mm, Norwood procedure can be done in beating hearts.
Aorta ; Coronary Vessels ; Heart Defects, Congenital ; Heart* ; Humans ; Infant, Newborn ; Norwood Procedures* ; Perfusion

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.
Aorta, Thoracic ; Fruit* ; Heart Defects, Congenital ; Humans ; Infant, Newborn* ; Pulmonary Artery

Berry syndrome (interrupted aortic arch, aortopulmonary window, and aortic origin of right pulmonary artery with intact interventricular septum) is a very rare and complex cardiac malformation. We report a successful one-stage repair in a 14-day-old neonate without circulatory arrest.
Aorta, Thoracic ; Fruit* ; Heart Defects, Congenital ; Humans ; Infant, Newborn* ; Pulmonary Artery

BACKGROUND/AIMS: Coronary vasospasms are one of the important causes of sudden cardiac death (SCD). Provocation of coronary vasospasms can be useful, though some results may lead to false positives, with patients potentially experiencing recurrent SCD despite appropriate medical treatments. We hypothesized that it is not coronary vasospasms but inherited primary arrhythmia syndromes (IPAS) that underlie the development of SCD. METHODS: We analyzed 74 consecutive patients (3.8%) who survived out-of-hospital cardiac arrest among 1,986 patients who had angiographically proven coronary vasospasms. Electrical abnormalities were evaluated in serial follow-up electrocardiograms (ECGs) during and after the index event for a 3.9 years median follow-up. Major clinical events were defined as the composite of death and recurrent SCD events. RESULTS: Forty five patients (60.8%) displayed electrocardiographic abnormalities suggesting IPAS: Brugada type patterns in six (8.2%), arrhythmogenic right ventricular dysplasia patterns in three (4.1%), long QT syndrome pattern in one (2.2%), and early repolarization in 38 (51.4%). Patients having major clinical events showed more frequent Brugada type patterns, early repolarization, and more diffuse multivessel coronary vasospasms. Brugada type pattern ECGs (adjusted hazard ratio [HR], 4.22; 95% confidence interval [CI], 1.16 to 15.99; p = 0.034), and early repolarization (HR, 2.97; 95% CI, 1.09 to 8.10; p = 0.034) were ultimately associated with an increased risk of mortality. CONCLUSIONS: Even though a number of aborted SCD survivors have coronary vasospasms, some also have IPAS, which has the potential to cause SCD. Therefore, meticulous evaluations and follow-ups for IPAS are required in those patients.
Arrhythmias, Cardiac* ; Arrhythmogenic Right Ventricular Dysplasia ; Coronary Vasospasm* ; Death, Sudden, Cardiac* ; Electrocardiography ; Follow-Up Studies ; Heart Arrest ; Humans ; Long QT Syndrome ; Masks* ; Mortality ; Out-of-Hospital Cardiac Arrest ; Survivors

OBJECTIVE: To assess the inter-tester and test-retest reliability and validity after developing of Korean version of National Institutes of Health Stroke Scale (NIHSS). METHOD: This research was conducted on 27 patents with stroke with less than 12 months since the onset of disease. Five physiatrists translated NIHSS into Korean. Video taping were used for objective scorings. Four physiatrists conducted scorings in order to seek for inter-tester reliability and one conducted scorings three weeks interval for test-retest reliability. Six physiatrists conducted scorings in order to seek for concurrent validity with the original NIHSS and four conducted scorings for validity with other impairment scale. Each score was analyzed based on Spear-man correlation coefficient. RESULTS: According to inter-tester reliability for Korean version of NIHSS, rho value reached over 0.70, with over 0.72 concerning test-retest reliability. The test on concurrent validity with the original NIHSS reached over 0.70 at rho value, with over 0.653 for MMSE, Motricity index, Brunnstrom stage. CONCLUSION: Newly developed Korean version of NIHSS showed high inter-tester and test-retest reliabilities, together with high concurrent validity with the original and other impairment scales, to be regarded to be used as primary impairment scale for patients with stroke.
Humans ; National Institutes of Health (U.S.)* ; Reproducibility of Results* ; Stroke* ; Weights and Measures

BACKGROUND: Prophylaxis for hepatitis B virus (HBV) recurrence is essential after liver transplantation (LT) in HBV-associated recipients. We conducted real-world analysis of HBV prophylaxis after LT in the Korean population.METHODS: Korean Organ Transplantation Registry (KOTRY) database and additionally collected data (n = 326) were analyzed with special reference to types of HBV prophylaxis.RESULTS: The study cohort comprised 267 cases of living-donor LT and 59 cases of deceased-donor LT. Hepatocellular carcinoma (HCC) was diagnosed in 232 (71.2%) of these subjects. Antiviral agents were used in 255 patients (78.2%) prior to LT. HBV DNA was undetectable in 69 cases (21.2%) and detectable over wide concentrations in the other 257 patients (78.8%) prior to LT. Polymerase chain reaction analysis of the store blood samples detected HBV DNA in all patients, with 159 patients (48.9%) showing concentrations > 100 IU/mL. Post-transplant HBV regimens during the first year included combination therapy in 196 (60.1%), hepatitis B immunoglobulin (HBIG) monotherapy in 121 (37.1%), and antiviral monotherapy in 9 (2.8%). In the second post-transplant year, these regimens had changed to combination therapy in 187 (57.4%), HBIG monotherapy in 112 (34.4%), and antiviral monotherapy in 27 (8.3%). Trough antibody to hepatitis B surface antigen titers > 500 IU/mL and >1,000 IU/mL were observed in 61.7% and 25.2%, respectively. The mean simulative half-life of HBIG was 21.6 ± 4.3 days with a median 17.7 days. Up to 2-year follow-up period, HCC recurrence and HBV recurrence developed in 18 (5.5%) and 6 (1.8%), respectively. HCC recurrence developed in 3 of 6 patients with HBV recurrence.CONCLUSION: Combination therapy is the mainstay of HBV prophylaxis protocols in a majority of Korean LT centers, but HBIG was often administered excessively. Individualized optimization of HBIG treatments using SHL is necessary to adjust the HBIG infusion interval.
Antiviral Agents ; Carcinoma, Hepatocellular ; Cohort Studies ; DNA ; Follow-Up Studies ; Half-Life ; Hepatitis B Surface Antigens ; Hepatitis B virus ; Hepatitis B ; Hepatitis ; Humans ; Immunoglobulins ; Korea ; Liver Transplantation ; Liver ; Organ Transplantation ; Polymerase Chain Reaction ; Recurrence ; Transplants

BACKGROUND: Prophylaxis for hepatitis B virus (HBV) recurrence is essential after liver transplantation (LT) in HBV-associated recipients. We conducted real-world analysis of HBV prophylaxis after LT in the Korean population. METHODS: Korean Organ Transplantation Registry (KOTRY) database and additionally collected data (n = 326) were analyzed with special reference to types of HBV prophylaxis. RESULTS: The study cohort comprised 267 cases of living-donor LT and 59 cases of deceased-donor LT. Hepatocellular carcinoma (HCC) was diagnosed in 232 (71.2%) of these subjects. Antiviral agents were used in 255 patients (78.2%) prior to LT. HBV DNA was undetectable in 69 cases (21.2%) and detectable over wide concentrations in the other 257 patients (78.8%) prior to LT. Polymerase chain reaction analysis of the store blood samples detected HBV DNA in all patients, with 159 patients (48.9%) showing concentrations > 100 IU/mL. Post-transplant HBV regimens during the first year included combination therapy in 196 (60.1%), hepatitis B immunoglobulin (HBIG) monotherapy in 121 (37.1%), and antiviral monotherapy in 9 (2.8%). In the second post-transplant year, these regimens had changed to combination therapy in 187 (57.4%), HBIG monotherapy in 112 (34.4%), and antiviral monotherapy in 27 (8.3%). Trough antibody to hepatitis B surface antigen titers > 500 IU/mL and >1,000 IU/mL were observed in 61.7% and 25.2%, respectively. The mean simulative half-life of HBIG was 21.6 ± 4.3 days with a median 17.7 days. Up to 2-year follow-up period, HCC recurrence and HBV recurrence developed in 18 (5.5%) and 6 (1.8%), respectively. HCC recurrence developed in 3 of 6 patients with HBV recurrence. CONCLUSION Combination therapy is the mainstay of HBV prophylaxis protocols in a majority of Korean LT centers, but HBIG was often administered excessively. Individualized optimization of HBIG treatments using SHL is necessary to adjust the HBIG infusion interval.