9 cases of criss-cross heart anomaly, diagnosed by angiography from April, 1979 to Feb. 1985 at Seoul National University Hospital were reviewed. The results were as follows: 1. Of 9 cases, 6 cases were male and 3 cases werefemale and the age ranged from 2 months to 16 years of age. 2. Of 9 cases, 7 cases were concordant (D-loop) and 2cases were discordant (L-loop). segmental approaches are 2 cases of SDD(TGV) and each one case of SLD(TGV),SDD(DORV), SDL(DORV), SDD(DOLV), SLL(corrected TGV), and normal one. 3. Associated anomalies are small sized rightventricle (7 cases), especially inflow tract and sinus portion, pulmonary outflow tract obstruction(5 cases),VSD(9 cases), ASD( 7 cases), PDA(2 cases) and visceroatrial situs solitus was observed in all 9 cases but 1dextrocardia.
Angiography ; Crisscross Heart ; Humans ; Male ; Seoul

A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

Criss-cross heart which is a cardiac malformation caused by abnormal rotation of the ventricles early in embryonic development, is rare but a double outlet of right ventricle in criss-cross heart is very rare. We experienced a case of criss-cross heart which is situs solitus, concordant atrioventricular connection and double outlet of right ventricle with remote ventricular septal defect of perimembranous inlet type. A 4-years old female was diagnosed as a double outlet of right ventricle in criss-cross heart after echocardiography, cardiac catheterization and cardiac angiography. The surgical correction was a intraventricular reconstruction of left ventricular outflow with 3/4 circle of 20 mm Hemashield vascular graft from the ventricular septal defect to the aorta. The patient had a temporary atrioventricular block but was recovered uneventfully, and a postoperative echocardiogram showed no left ventricular outflow obstruction, no intracardiac shunt.
Angiography ; Aorta ; Atrioventricular Block ; Bays ; Cardiac Catheterization ; Cardiac Catheters ; Child, Preschool ; Crisscross Heart* ; Double Outlet Right Ventricle ; Echocardiography ; Embryonic Development ; Female ; Heart Septal Defects, Ventricular ; Heart Ventricles* ; Humans ; Pregnancy ; Transplants ; Ventricular Outflow Obstruction

Between August 1996 and August 1997, 22 patients underwent extracardiac Fontan operations. The basic diagnoses included univentricular heart of the right ventricular type (n=12); univentricular heart of the left ventricular type (n=4); tricuspid atresia (n=4); left isomerism, transposition of great arteries, ventricular septal defect and pulmonary stenosis (n=1); and criss-cross heart with uneven ventricle (n=1). The median age of the 14 men and 8 women was 29 months (range from 21 months to 26 years). Previous procedures included bidirectional cavopulmonary shunt (n=15, interval=15.6+/-3.4 months), Kawashima operation (n=4, interval=37.5+/-20 months), and classic Glenn shunt (n=1, interval=14 years). In 2 patients, extracardiac Fontan operations were done without any previous procedures. A 16-to 22-mm flexible Gore-Tex tube graft (n=18), Hemashield graft (n=3), or, alternatively, a nonvalved aortic allograft (n=1) was cut and anastomosed end-to-end between inferior vena cava and undersurface of pulmonary artery using Gore-Tex or Prolene suture in a running fashion. In risk Fontan patients (n=12), a communication between the extracardiac conduit and the right atrium was constructed. In the most 13 recent patients, the procedures were done without cross-clamping of the aorta and with a beating heart. Operative mortality was 9.1% (n=2). Complications included persistent chest tube drainage for more than 7 days (n=5), chorea (n=2), and low cardiac output (n=1). There were no late deaths. Follow-up echocardiogram (mean: 6 months) demonstrated satisfactory hemodynamic results in the surviving 20 patients. Potential advantages of this technique consist of minimization of surgical manipulation of atrial tissue, reduction or elimination of myocardial ischemia, creationof a uniform and stable inferior vena cava-to-pulmonary artery conduit, and increased flexibility and safety in certain high-risk patients such as those with increased pulmonary vascular resistance, pulmonary hypertension, and impaired ventricular function. Further investigations during a longer follow-up are needed to confirm the intermediate and long-term results, especially the reduction of late atrial arrhythmias.
Allografts ; Aorta ; Arrhythmias, Cardiac ; Arteries ; Cardiac Output, Low ; Chest Tubes ; Chorea ; Crisscross Heart ; Diagnosis ; Drainage ; Female ; Follow-Up Studies ; Fontan Procedure* ; Heart ; Heart Atria ; Heart Septal Defects, Ventricular ; Hemodynamics ; Humans ; Hypertension, Pulmonary ; Isomerism ; Male ; Mortality ; Myocardial Ischemia ; Pliability ; Polypropylenes ; Polytetrafluoroethylene ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Running ; Sutures ; Transplants ; Transposition of Great Vessels ; Tricuspid Atresia ; Vascular Resistance ; Vena Cava, Inferior ; Ventricular Function

BACKGROUND: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. MATERIAL AND METHOD: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months (13 days~38.1 months) and body weight was 5.0 kg (2.9~13.5 kg). Preoperative pressure gradients were 25.3+/-15.7 mmHg (10~60 mmHg). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6, another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. RESULT: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of 62.7+/-38.9 months (3.3~128.1 months). Kaplan-Meier estimated actuarial survival was 71.9%+/-9.3% at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. CONCLUSION: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.
Aorta ; Aorta, Thoracic ; Bays ; Body Weight ; Cardiac Catheterization ; Cardiac Catheters ; Constriction, Pathologic ; Crisscross Heart ; Double Outlet Right Ventricle ; Echocardiography ; Follow-Up Studies ; Fontan Procedure ; Freedom ; Heart ; Heart Ventricles ; Humans ; Hypertension, Pulmonary ; Mitral Valve Insufficiency ; Multivariate Analysis ; Parturition ; Pulmonary Artery ; Reoperation ; Retrospective Studies ; Risk Factors ; Vascular Resistance