tricuspid valve annulus diameter could not be measured in cases showing muscular tricuspid atresia and double-inlet LV. The initial RV dimensions quantified by CT were significantly lower for the hypoplastic RV group than for the control group (p < 0.001). The types of final surgery performed in the hypoplastic RV group were univentricular repair in 46 patients, biventricular repair in 4 patients, or an indeterminate surgery in 7 patients.CONCLUSION: Initial RV dimensions in infants with CHD and a hypoplastic RV can be quantified by CT and are substantially smaller than those in infants with tetralogy of Fallot.]]>
Follow-Up Studies ; Heart Defects, Congenital ; Heart Ventricles ; Humans ; Infant ; Tetralogy of Fallot ; Tricuspid Atresia ; Tricuspid Valve

An Ebstein anomaly is a rare congenital heart defect defined by an inferior displacement of the septal and posterior leaflets of the tricuspid valve from the tricuspid annulus. This anomaly shows various ultrasonographic manifestations, thus making the prenatal diagnosis sometimes difficult. We here report a rare case of an Ebstein anomaly which was prenatally suspected as the absence of the tricuspid valve with functional pulmonary atresia because of non-visible tricuspid leaflets on an echocardiograph at 24 weeks of gestation. An emergency cesarean section was performed at 35 weeks of gestation as fetal hydrops were seen on a follow-up scan. Postnatal surgery confirmed Ebstein anomaly type-D which demonstrates an almost complete atrialization of the right ventricle with the exception of a small and infundibular component. Because of its rarity, prenatal findings of a type-D Ebstein anomaly have not been reported previously. We suggest from this first such case report that this anomaly should be considered as a possible diagnosis when the tricuspid leaflets are not well visualized.
Cesarean Section ; Diagnosis ; Ebstein Anomaly* ; Echocardiography ; Emergencies ; Female ; Fetal Heart ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Ventricles ; Hydrops Fetalis ; Pregnancy ; Prenatal Diagnosis ; Pulmonary Atresia ; Tricuspid Valve

OBJECTIVETo analyze 68 pediatric cases with functional univentricle heart who underwent bidirectional Glenn procedure during from April 1998 to December 2005.

METHODSThere were 47 males and 21 females in this group, aged from 5 months to 14 years old and weighed from 6.7 to 30.0 kg. Among them, 39 cases were received bidirectional Glenn procedure on the right side, 13 cases on the left side and 16 cases on both sides. Three cases had the pulmonary artery banded; one case had the pulmonary artery ligated;one case had the original A-P shunt cut off; six cases had the PDA ligated; four cases had the MAPCAs cut off; one case had TAPVC corrected contemporarily; two cases of PAPVC were also corrected; four cases had the atrial-ventricular valve repaired.

RESULTSThree cases died. The mortality was 4.4%. The mean post-operative pressure of super vena cava was (15.9 +/- 2.4) mm Hg (1 mm Hg = 0.133 kPa), higher than the pre-operative one (8.3 +/- 1.8) mm Hg (P < 0.01). The mean post operative SpO(2) was (89.3 +/- 4.2)%, higher than the pre-operative one (78.4 +/- 6.0)% (P < 0.01).

CONCLUSIONSBidirectional Glenn procedure is of satisfied effect on surgical treatment for functional univentricle heart. The persistent forward flow from pulmonary artery should be reserved in bidirectional Glenn procedure.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Fontan Procedure ; methods ; Heart Defects, Congenital ; surgery ; Humans ; Infant ; Male ; Retrospective Studies ; Tricuspid Atresia ; surgery

The status of pulmonary circulation is regarded with utmost importance for the successful Fontan operation. The absence of unilateral pulmonary artery leads to decreased pulmonary vascular bed and elevated pulmonary vascular resistance which are the risk factors for Fontan operation. A 9-year-8-month-old female patient diagnosed as tricuspid atresia, pulmonary atresia with absent left pulmonary artery flow, received extracardiac conduit Fontan operation using 18 mm Gore-Tex graft. She was discharged on twenty sixth postoperative day with arterial O2 saturation of 70% on room air. On last follow up at 14 months after the operation, she was clinically well with O2 saturation of 91%.
Female ; Follow-Up Studies ; Fontan Procedure* ; Humans ; Polytetrafluoroethylene ; Pulmonary Artery* ; Pulmonary Atresia ; Pulmonary Circulation ; Risk Factors ; Transplants ; Tricuspid Atresia ; Vascular Resistance

BACKGROUND: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. MATERIAL AND METHOD: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. RESULT: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8+/-1.50 (-3.2~1.2) and -2.5+/-0.5 (-3~-2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. CONCLUSION: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.
Blalock-Taussig Procedure ; Echocardiography ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Mortality ; Pulmonary Atresia* ; Tricuspid Valve ; Ventricular Function, Left ; Ventricular Function, Right ; Ventricular Septum*

Given improvements in the diagnosis and treatment of congenital heart disease, more patients with heart disease reach adolescence or adulthood and require noncardiac surgery. Since 1971, the Fontan operation has been widely used for the management of tricuspid atresia and a variety of univentricular heart malformations. Although the life expectancy of these patients has increased, few reports are available on their anaesthetic management when a noncardiac operation is indicated. We report on the anaesthetic management of a 40-years-old male patient scheduled for permanent pacemaker insertion who underwent a modified Fontan procedure at the age of 27 years for pulmonary stenosis, common atrioventricular cushion defect, and for corrected transposition of the great arteries
Adolescent ; Adult* ; Anesthesia ; Arteries ; Diagnosis ; Fontan Procedure* ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Life Expectancy ; Male ; Pulmonary Valve Stenosis ; Tricuspid Atresia

BACKGROUND: By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. MATERIAL AND METHOD: From October 1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0+/-7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was III or IV in six patients. Paroxysmal atrial fibrillation (n=1), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). RESULT: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional tachyarrhythmia (n=1). Postoperative central venous pressure was lower than the preoperative value (17.9+/-3.5 vs. 14.9+/-1.0, p=0.049). Follow-up was complete in all patients and extended to 50.1 months (mean, 30.3+/-12.8 months). There was no late death. All patients were in NYHA class I or II. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure. PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. CONCLUSION: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.
Anastomosis, Surgical ; Atrial Fibrillation ; Central Venous Pressure ; Cyanosis ; Follow-Up Studies ; Fontan Procedure ; Heart Failure ; Hemodynamics ; Humans ; Mortality ; Pleural Effusion ; Protein-Losing Enteropathies ; Shock* ; Tachycardia ; Tachycardia, Supraventricular ; Tricuspid Atresia

PURPOSE: To investigate the teratogenic effect of gamma-ray on the heart of chick embryo. METHODS: 50 rad, 100 rad, 150 rad, 200 rad, 250 rad, and 300 rad of gamma-ray were used to irradiate three days old chick embryos. The control group was not irradiated. After three weeks, the embryos were sacrificed and examined for cardiovascular malformation. RESULTS: The survival rate of the gamma-ray irradiated group was significantly lower than that of the control group(33.3-63.3% vs 76.4%, P=0.001). The cardiac malformation rate of the experimental group was 11.0%. In the control group, no congenital cardiac malformations were observed. The experimental groups had a significantly higher malformation rate(P=0.001). The types of malformation were ventricular septal defect, tricuspid atresia, Ebstein anomaly and aortic arch anomaly. In the gamma-ray irradiated group, the cardiac malformations were : 14 small ventricular septal defects (VSDs), five large VSDs, two tricuspid atresias, and one Ebstein anomaly. The higher the dose of radiation applied, the higher the incidence of cardiac malformation was noted. CONCLUSION: Gamma-ray irradiation of 3 days old chick embryos increased the rate of death and the rate of cardiac malformation significantly.
Animals ; Aorta, Thoracic ; Chick Embryo* ; Ebstein Anomaly ; Embryonic Structures ; Heart Septal Defects, Ventricular ; Heart* ; Incidence ; Survival Rate ; Tricuspid Atresia

PURPOSE: The actual clinical examples of co-appliance of catheter intervention with surgical procedures in the treatment of pulmonary atresia with an intact ventricular septum(PA/IVS) which we have experienced in our institution are here shown, and the anatomical and hemodynamical profiles between each method is compared. METHODS: Medical records of 33 patients with PA/IVS who underwent various treatment from January, 1995 to December, 2000 were reviewed for a retrograde study. RESULTS: In three out of 10 patients who underwent percutaneous balloon pulmonary valvotomy (PPV), residual pulmonary stenosis were observed in their out patient department(OPD) follow-ups, eventually necessitatig balloon pulmonary valvuloplasty(BPV). One out of three patients exhibited deterioration of tricuspid regurgitation after BPV, requiring surgical tricuspid annuloplasty(TAP). Two out of the seven patients who received primarily surgical right ventricle outlet tract(RVOT) repair without any systemic-pulmonary shunt or intervention needed additional intervention employing cardiac catheterization after operation. Two patients received interventional catheterization before surgical RVOT repair. In five out of 11 cases of Fontan type operation, coil embolization of collateral circulation was done before total cavo-pulmonary connection(TCPC), and in three cases, interventional catheterization was needed after TCPC. CONCLUSION: Both medical and surgical treatment modalities are widely used in management of PA/IVS patients, and recent results prove that medico-surgical cooperative treatment is essential.
Cardiac Catheterization ; Cardiac Catheters ; Catheterization ; Catheters ; Collateral Circulation ; Embolization, Therapeutic ; Follow-Up Studies ; Heart Ventricles ; Humans ; Medical Records ; Pulmonary Atresia* ; Pulmonary Valve Stenosis ; Tricuspid Valve Insufficiency ; Ventricular Septum*

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus arteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstruction, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty. Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.
Angioplasty ; Critical Illness* ; Diagnosis ; Ebstein Anomaly* ; Echocardiography ; Fontan Procedure ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Infant ; Infant, Newborn* ; Male ; Physiology ; Pulmonary Atresia ; Pulmonary Circulation ; Tricuspid Valve Insufficiency