In-Sook Park

Visibility of the origin and proximal course of coronary arteries on non-ECG-gated heart CT in patients with congenital heart disease

Background: There is little information on the ability of non-ECG-gated cardiac CT to demonstrate the coronary arteries of children. Objective: To evaluate the visibility of the origin and proximal course of coronary arteries on non-ECG-gated cardiac CT, in which the coronary artery was not of primary diagnostic concern, in children with congenital heart disease. Materials and methods: From December 2002 to March 2004, 126 cardiac CT examinations from 104 children (median age 11xa0months; age range 1xa0day to 15xa0years) were evaluated. All patients had ventriculo-arterial concordance and no malformations of the great arteries; those with coronary artery anomalies were excluded. Contrast-enhanced 16-slice spiral CT was performed without ECG-gating and multiplanar images for coronary arteries were obtained. The visibility of coronary artery origins was graded on a three-point scale, while nine segments of the arteries were graded on a four-point scale. CT images in which it was possible to trace the coronary arteries were considered diagnostic. The visibility of each whole coronary artery and the origins and proximal four segments of coronary arteries were calculated. The visibility of coronary arteries was also correlated with patient age. Results: The percentage of CT images of diagnostic quality was 49.3% for the whole coronary artery and 81.7% for the origins and proximal four segments. There was a significant positive correlation between the visibility of coronary arteries and age. Conclusions: Non-ECG-gated cardiac CT, in which the coronary artery is not of primary diagnostic concern, is frequently able to visualize the origin and proximal course of coronary arteries and may be helpful in detecting coronary artery anomalies in children with congenital heart disease.

Analyses of Left Ventricular Myocardial Deformation by Speckle-Tracking Imaging During the Acute Phase of Kawasaki Disease

This study aimed to investigate left ventricular myocardial deformation in children with Kawasaki disease during the acute phase of their illness. A total of 50 patients and 35 normal control subjects were assessed. Data were obtained from the patients during the acute and convalescent phases of Kawasaki disease. Analyses of myocardial deformation [strain (ε), strain rate (SR)] was performed using two-dimensional speckle-tracking imaging in three directions (longitudinal, circumferential, and radial) at the basal and mid levels of the left ventricular myocardium. Basal longitudinal ε (Pxa0<xa00.001) and midlongitudinal ε (Pxa0<xa00.0001) were lower during the acute phase of the disease than in the control subjects and associated with serum albumin level and left ventricular mass index (LVMI). Midlongitudinal SR (Pxa0<xa00.0001) was lower during the acute phase of Kawasaki disease than in the control subjects and associated with LVMI. Decreased systolic SR was not detected in any direction. In conclusion, left ventricular longitudinal systolic ε was significantly decreased during the acute phase of Kawasaki disease. This may be a result of myocardial swelling from myocarditis during the acute phase of the disease.

Pulmonary vascular compliance and pleural effusion duration after the Fontan procedure

BACKGROUNDnPreoperative risk analysis for Fontan candidates is still less than optimal in that patients with apparently low risks may have a poor outcome, such as prolonged pleural drainage, protein-losing enteropathy, pulmonary thromboembolism and death. We hypothesized that low pulmonary vascular compliance (PVC) is a risk factor for persistent pleural effusion after the Fontan operation.nnnMETHODSnA retrospective review of 85 patients who underwent the extracardiac Fontan procedures (median age: 3.87 years) was performed. Fontan risk score (FRS) was calculated from 12 categorized preoperative anatomical and physiological variables. PVC (mm(2)/m(2) x mmHg) was defined as pulmonary artery index (mm(2)/m(2)) divided by total pulmonary resistance (Wood Unit x m(2)) and pulmonary blood flow (L/min/m(2)), based on the electrical circuit analogy of the pulmonary circulation. Chest tube indwelling time was log-transformed (log indwelling time, LIT) to fit normal distribution, and the relationship between perioperative predictors and LIT was analyzed by multiple linear regression.nnnRESULTSnPreoperative PVC, chest tube indwelling time and LIT ranged from 6 to 94.8 mm(2)/mmHg/m(2) (median: 24.8), 3 to 268 days (median: 20 days), and 1.1 to 5.6 (mean: 2.9, standard deviation: 0.8), respectively. FRS, PVC, cardiopulmonary bypass time (CPB) and central venous pressure at postoperative 12 h were correlated with LIT by univariable analyses. By multiple linear regression, PVC (p=0.002) and CPB (p=0.003) independently predicted LIT, explaining 22% of the variation. The regression equation was LIT=2.744-0.016 PVC+0.007 CPB.nnnCONCLUSIONnLow pulmonary vascular compliance is an important risk factor for prolonged pleural effusion drainage after the extracardiac Fontan procedure.

Molecular cytogenetic and clinical characterization of a patient with a 5.6-Mb deletion in 7p15 including HOXA cluster†

Here, we describe the clinical features of a boy with a 5.6‐Mb deletion at chromosome 7p15.1–p15.3. He has mild facial anomalies, hand‐foot abnormalities, hypospadias, congenital heart defects, and supernumerary nipples. This deletion was detected by array comparative genomic hybridization and verified by fluorescence in situ hybridization using BACs selected from the USCS genome browser. This deletion was not found in subsequent FISH analysis of the parental chromosomes. The deleted region contains several genes, including contiguous developmental genes on the HOXA cluster, which play a role in regulating aspects of morphogenesis during normal embryonic development. The patients limb and urogenital features were similar to those observed in hand‐foot‐genital syndrome, which is caused by haploinsufficiency of HOXA13, whereas the congenital heart defect may reflect the deletion of HOXA3. We hypothesized that many clinical features of the patient were due to combined haploinsufficiency of the HOXA cluster. Our study also demonstrates the clinical usefulness of a molecular cytogenetic tool that is capable of detecting imbalances in the genome.

Tetralogy of Fallot without the infundibular septum–restricted growth of the pulmonary valve annulus after annulus preservation may render the right ventricular outflow tract obstructive

OBJECTIVEnTetralogy of Fallot (ToF) with infundibular septal deficiency (ISD) is commonly associated with postoperative right ventricular outflow tract obstruction (RVOTO), presumably caused by subpulmonary extension of ventricular septal defect (VSD) and restricted growth of the pulmonary valve annulus (PVA). We sought to determine the postoperative growth of the PVA after annulus preservation according to the presence or absence of the infundibular septum.nnnMETHODSnA retrospective review of 90 patients who underwent surgical repair of ToF between June 1997 and August 2008 was performed. Median age at operation was 9.5 months. Infundibular septum was absent in 15 (15/90, 17%). PVA was preserved in 71 patients (71/90, 79%), including 13 patients with ISD (13/15, 87%).nnnRESULTSnMedian follow-up duration was 39 months (2 months to 13 years). There was no early mortality and one late noncardiac death. Among the patients with PVA preservation, reoperation for RVOTO was performed in 4 patients (4/71, 6%), including 3 patients without the infundibular septum. ISD was identified as the only risk factor in reoperation for RVOTO after PVA preservation (RR: 21.85, P = .007). Among the patients who underwent repair with PVA preservation during infancy (n = 43), PVA (Z-score) increased postoperatively in patients with the infundibular septum (+ 0.021/month, P = .009), whereas the changes in PVA (Z-score) were nonsignificant in patients with ISD (-0.021/month, P = .306), with a marginal intergroup difference (P = .056).nnnCONCLUSIONSnPVA preservation in ToF with ISD may be associated with a higher risk for postoperative RVOTO, which can be attributed to the restricted growth of the PVA.

Effectiveness and safety of percutaneous transcatheter implantation of pulmonary arterial stent in congenital heart disease.

Background and Objectives Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. Subjects and Methods A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. Results Percent stenosis of stenotic lesions were decreased from 54.1±10.7% to 22.8±12.5% (p<0.001) and degree of decrement in affected lung perfusion was declined from 22.7±8.0% to 10.3±9.0% (p<0.001) immediately and lasts during mid-term follow up period. Complication rate relating the procedure was 12% (6 out of 12) and there was no mortality case. Conclusion This series showed immediate and short term effectiveness of pulmonary arterial stent in congenital heart defects. We concluded that percutaneous transcatheter implantation of pulmonary arterial stent was safe and effective during short and mid-term follow up period.