A healthy 8 year old boy was referred to our hospital because of cardiomegaly on the chest X ray. No cardiac murmur was detected on chest examination and normal sinus rhythm was checked by electrocardiogram. The 2-D echocardiogram showed a huge dilated right atrium and a normal tricuspid valve and right ventricle. The findings of cardiac angiogram and MRI was compatible with the isolated giant right atrial aneurysm. For the prevention of atrial arrhythmia and thromboembolism, he underwent successful surgical reduction of the right atrium and cryoablation and the follow-up X ray showed no cardiomegaly.
Aneurysm* ; Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cryosurgery ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Murmurs ; Heart Ventricles ; Humans ; Magnetic Resonance Imaging ; Male ; Thorax ; Thromboembolism ; Tricuspid Valve

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the right lung, and right lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the right pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.
Arteriovenous Fistula* ; Cyanosis ; Dyspnea ; Embolization, Therapeutic ; Fontan Procedure ; Hepatopulmonary Syndrome ; Humans ; Lung ; Middle Aged* ; Perfusion ; Pulmonary Artery ; Tetralogy of Fallot*

Cardiac resynchronization therapy (CRT) is a new treatment for refractory heart failure. However, most patients with heart failure treated with CRT are adults, middle-aged or older with idiopathic or ischemic dilated cardiomyopathy. We treated a 12-year-old boy, who was transferred after cardiac arrest, with dilated cardiomyopathy, left bundle-branch block, and ventricular tachycardia. We performed cardiac resynchronization therapy with a defibrillator (CRT-D). After CRT-D, left ventricular ejection fraction improved from 22% to 44% assessed by echocardiogram 1 year postoperatively. On electrocardiogram, QRS duration was shortened from 206 to 144 ms. The patient's clinical symptoms also improved. For pediatric patients with refractory heart failure and ventricular arrhythmia, CRT-D could be indicated as an effective therapeutic option.
Adult ; Arrhythmias, Cardiac* ; Bundle-Branch Block ; Cardiac Resynchronization Therapy* ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Child* ; Defibrillators ; Electrocardiography ; Heart Arrest ; Heart Failure* ; Heart* ; Humans ; Male ; Stroke Volume ; Tachycardia, Ventricular

The patient was an eight-year-old female. She was diagnosed as dilated cardiomyopathy. She was supported with bi-ventricular assist because of heart failure for 15 days. After 7 days, she was suffered from prerenal type ARF and support with continuous veno-veno hemodyalisis (CVVHD). And then heart transplantation was performed, heart donor's blood type was A. Immune suppressants were used after due consideration for renal toxicity. ARF was resolved on post operative 14th day. She was discharged on post operative 52nd day without any specific post operative complication. She has been followed up without any immune rejection reaction upto 14 months.
Acute Kidney Injury ; Cardiomyopathy, Dilated ; Female ; Heart Failure ; Heart Transplantation* ; Heart* ; Heart-Assist Devices ; Humans

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.
Arrhythmias, Cardiac ; Cesarean Section ; Delivery, Obstetric ; Ebstein Anomaly ; Female ; Fontan Procedure* ; Gestational Age ; Heart Failure ; Heart Ventricles* ; Humans ; Korea ; Neonatology ; Pregnancy* ; Risk Assessment

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.
Arrhythmias, Cardiac ; Cesarean Section ; Delivery, Obstetric ; Ebstein Anomaly ; Female ; Fontan Procedure* ; Gestational Age ; Heart Failure ; Heart Ventricles* ; Humans ; Korea ; Neonatology ; Pregnancy* ; Risk Assessment

Persistent fifth aortic arch (PFAA) is a rare congenital anomaly of the aortic arch frequently associated with other cardiovascular anomalies, such as tetralogy of Fallot and aortic arch coarctation or interruption. We report the case of a neonate with PFAA with coarctation who successfully underwent surgical repair.
Aorta, Thoracic* ; Humans ; Infant, Newborn ; Tetralogy of Fallot

One of the complications of permanent pacemaker implantation is unintended phrenic nerve stimulation. A 15-year-old boy with a permanent pacemaker presented with chest discomfort due to synchronous chest wall contraction with pacing beats. Even after reprogramming of the pacemaker, diaphragmatic stimulation persisted. Therefore, we performed thoracoscopic phrenic nerve insulation using a Gore-Tex patch to insulate the phrenic nerve from the wire. A minimally invasive approach using a thoracoscope is a feasible option for retractable phrenic nerve stimulation after pacemaker implantation.
Adolescent ; Humans ; Male ; Minimally Invasive Surgical Procedures ; Pacemaker, Artificial ; Phrenic Nerve* ; Polytetrafluoroethylene ; Thoracic Wall ; Thoracoscopes ; Thoracoscopy ; Thorax

A 12-day-old female baby underwent a Norwood procedure for hypoplastic left heart syndrome. The left superior vena cava (LSVC), which was found incidentally during the operation, was divided to facilitate surgical exposure. After the operation, she developed signs of low cardiac output and died 7 hours afterward. Autopsy findings showed that the coronary sinus was atretic at the orifice without unroofing into both atria, rendering the LSVC the sole route of coronary sinus drainage. In patients with incidentally-found LSVC during surgery, special care should be taken to leave the LSVC intact because the LSVC may be the exclusive drainage vein of the coronary venous system.
Autopsy ; Cardiac Output, Low ; Coronary Sinus ; Drainage ; Female ; Humans ; Hypoplastic Left Heart Syndrome ; Norwood Procedures ; Veins ; Vena Cava, Superior

A seventeen-month-old male baby, who had received conventional biventricular repair for congenitally corrected transposition of the great arteries, underwent excision of supratricuspid ring. Although tricuspid valve annulus was marginally small on direct inspection in the operating theater, circumferential excision of supratricuspid ring alone completely relieved the right ventricular inflow obstruction.
Arteries ; Constriction, Pathologic ; Humans ; Male ; Transposition of Great Vessels ; Tricuspid Valve