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Background: Right ventricular (RV) fibrosis is common in patients with repaired tetralogy of Fallot (rTOF). Although accumulating evidence indicates the role of circulating biomarkers of collagen metabolism in left ventricular fibrosis, rTOF data are lacking. This study examined the expression profile and clinical relevance of circulating biomarkers of collagen type I metabolism in rTOF patients., Methods: Serum biomarkers of collagen type I synthesis (carboxy-terminal propeptide of procollagen type I, PICP), degradation (carboxy-terminal telopeptide of collagen type I, CITP), and enzymes regulating collagen degradation (matrix metalloproteinases, and type I tissue inhibitor, TIMP-1) were measured in 70 rTOF and 91 control adults. All patients had complete clinical data and received cardiovascular magnetic resonance scans with late gadolinium enhancement (LGE)., Results: Compared to the controls, rTOF patients had higher PICP levels (p<0.001), PICP:CITP ratios (p<0.001), and TIMP-1 concentrations (p<0.001). Increasing PICP levels correlated with higher RV LGE scores (r=0.427, p<0.001), lower VO2max (r=-0.428, p=0.002), and larger RV volumes. Furthermore, stepwise multivariate linear regression analysis identified RV end-diastolic volume index >150mL/m(2) (β=40.52, p=0.016), RV LGE score (β=3.94, p=0.008), and age (β=-1.77, p=0.011) as independent correlates of circulating PICP levels., Conclusions: Patients with rTOF exhibited a profibrotic state with excessive collagen type I synthesis and dysregulated degradation. Elevated circulating PICP levels might reflect RV fibrosis, and link to adverse markers of clinical outcome., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Uncorrected congenital heart defects (CHD) with severe pulmonary hypertension (sPH, systolic pulmonary artery>70% of systolic pressure) are usually considered inoperable. We are curious to know if some selected patients might benefit from palliative operation for those sPH with uncorrected CHD., Methods: Adults or adolescents with sPH associated with ventricular septal defect (VSD) with/without great artery anomalies were selected for pulmonary artery banding (PAB) to reduce sPH. The target pulmonary pressure was less than half of the systolic blood pressure after arch or great arteries reconstruction. Repeated catheterization was performed to evaluate the feasibility of defect closure., Results: Consecutively, 8 patients (age 26 ± 9 years) received PAB as a palliative procedure in the past 8 years without mortality. The pre-PAB systolic pulmonary pressure was 119 ± 9 mmHg. Additional PAB had been applied in 4 of them. All patients showed significant improvement in function class (III to I or II). The mean post-PAB pulmonary pressure decreased significantly (77.5 ± 9.2 mmHg to 42.0 ± 9.0 mmHg) and 6-minute walk test was also found to have great improvement (270 ± 86 m to 414 ± 49 m), but the saturation at rest did not show a difference. Three of them received corrective surgery to close defects over 3-5 years., Conclusion: For some selected adult sPH with uncorrected CHD, PAB can work as a palliative procedure to improve their functional class and even provide a chance of total repair., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Aims: To evaluate electron beam computed tomography (EBCT) for recognition of coronary artery patterns in children with congenital heart diseases., Methods: Institutional review board approval was obtained; informed consents were not required. A total of 226 children diagnosed with Tetralogy of Fallot (n=122), double outlet right ventricle (n=52), transposition of the great arteries (n=34), and congenitally corrected transposition (n=18) who had undergone cardiac EBCT at our institution between 1995 and 2002 were identified. Iodinated contrast medium was injected with arterial phase acquisition. The two radiologists and one pediatric cardiologist that interpreted the EBCT images and cardiac angiograms, respectively, were blinded to each other's results. Surgical and cardiac angiogram findings were compared to the EBCT results. Descriptive statistics were used to compare efficacy., Results: Numerous aberrant patterns were clearly identified on the EBCT images. Pattern IX occurred in most patients with Tetralogy of Fallot or double outlet right ventricle. Patterns I and 0 are the most common coronary artery types in transposition of the great arteries and congenitally corrected transposition, respectively. Overall diagnostic accuracy for all disease groups was 82.7%. The diagnostic accuracy of the coronary arterial anatomy by EBCT increased with older age, and was more than 90% in individuals aged over 3 months., Conclusion: EBCT is effective for identification of the coronary anatomy of children with specific congenital heart diseases, except for neonates and small infants less than 3 months of age.

From July 1, 1989 to June 30, 1996, a total of six patients, four male and two female, with double aortic arch (DAA) were registered. They aged 16 days to 6.5 years. There were two patients with isolated DAA, and four complicated with intracardiac anomalies. All six patients presented respiratory distress and feeding problem early in life. However, cyanosis, owing to decreased pulmonary blood flow, predominated the clinical picture in each patient having DAA intricated with tetralogy of Fallot (TOF) and pulmonary stenosis, TOF with absent pulmonary valve syndrome, and type II-A tricuspid atresia. DiGeorge syndrome was documented in one patient with TOF and pulmonary stenosis. Expiratory wheezing was remarkable in the patient with TOF and absent pulmonary valve syndrome. Mild cyanosis and heart failure was noted in one patient with ventricular septal defect (VSD), and bicuspid pulmonary valve with mild valvular stenosis. Barium esophagogram showed indentations on esophagus in five patients. Echocardiography was yielding in three infants. Four patients, two isolated and two complex DAA, survived the surgical division of the smaller left aortic arch. One-stage and two-stage operation was performed in patients having DAA intricated with VSD and TOF, respectively. The other two patients with complex DAA remained in close observation owing to the tolerance of symptoms related to intracardiac anomalies.

Background: The most common type of left ventricular-to-right atrial shunts are those associated with perimembranous ventricular septal defect when the defects diminish in size via aneurysmal transformation. This study is to characterize the echocardiographic features and to determine the possible mechanisms., Methods and Results: From January 1986 to December 1992, of 930 consecutive patients with isolated perimembranous ventricular septal defect, 692 showed evidence of aneurysmal transformation. Excluding those with subaortic ridge, echocardiographic analysis was based on 664 of them. Of these, 94 patients had left ventricular-to-right atrial shunts, 115 had their defects spontaneously closed and in 455 only interventricular shunt persisted. Four types of echocardiographic findings featured in the aneurysmal transformation process: type A, both the anterior and septal leaflets of tricuspid valve contributed to the process; type B was similar to type A, but the arc-like structure from the anterior leaflet is much less evident; in type C, only the septal leaflet, and in type D, other adjacent tissues, contribute to the aneurysmal transformation. Type A morphology was closely associated with the development of left ventricular-to-right atrial shunt while types C and D were most commonly seen in those spontaneously closed defects and those with only interventricular shunt. The vegetations of infective endocarditis were located at the atrial side of the tricuspid valve in patients with left ventricular-to-right atrial shunts, while they were in the right ventricle in those with only interventricular shunt., Conclusion: The morphology of the aneurysmal transformation in perimembranous ventricular septal defect can be characterized by echocardiograms. Although the septal leaflet of the tricuspid valve is usually involved in this process, it is the incorporation of the anterior leaflet that predisposes to the development of left ventricular-to-right atrial shunts.

A case of 'absent' left pulmonary vein with ventricular septal defect was diagnosed based on echocardiography, magnetic resonance imaging and cardiac catheterization at newborn stage. Pulmonary arterial banding was performed at the age of 1 month to ameliorate the pulmonary flow. At a cardiac catheterization 1 year later, the left pulmonary veins were visualized with moderate stenosis at their entrance into the left atrium. The patient has been stable after surgical repair of the ventricular septal defect and dilation of the pulmonary venous stenosis.