Correlation between pulmonary vascular change and hemodynamics in patients with complete atrioventricular septal defect.

BACKGROUND AND PURPOSE: The study of the pulmonary vasculature in Taiwanese with atrioventricular septal defect has not been reported previously. This study investigated the correlation between pulmonary vascular change and hemodynamics in these patients. METHODS: Ten children with complete atrioventricular defect, 8 of whom had Down syndrome, underwent open lung biopsy. Their age ranged from 8 months to 6 years (mean, 2 years 7 months). Cardiac catheterization was performed and the systolic pressures of pulmonary and systemic arteries were measured immediately before cardiopulmonary bypass (CPB) and immediately after cardiac repair in 9 patients, and expressed as a ratio of pulmonary to systemic systolic pressure (Pp/Ps). RESULTS: Medial hypertrophy and intimal proliferation were prominent in 7 patients with pulmonary hypertension. The alveolar wall muscular arteries and percent arterial medial thickness increased significantly with increasing post-repair Pp/Ps, preoperative Pp/Ps, pulmonary arteriolar resistance index and pre-CPB Pp/Ps (p < 0.02). The arteries present per 100 alveoli were inversely correlated with the above mentioned hemodynamic parameters (p < 0.02). CONCLUSIONS: The results of qualitative and quantitative analyses of the pulmonary vasculature in patients with complete atrioventricular defect correlated well with pre-and postoperative pulmonary arterial pressure and can be used to predict the postoperative pulmonary arterial pressure.

Coronary artery anatomy in anatomically corrected malposition of the great arteries and their surgical implications.

OBJECTIVES: Coronary artery (CA) anatomy of anatomically corrected malposition of the great arteries (ACM) has not been well elucidated. We studied the CA pattern and their association with the aortopulmonary rotation (APR). METHODS: CA anatomy and APR of ACM were identified by image or surgical intervention. The degree of APR was defined by the pattern of the aortic sinus of Valsalva on the lateral angiogram or computed tomography (CT). RESULTS: Four CA types were presented in 10 patients and literatures. Six patients had the same CA type as the usual pattern in transposition of the great arteries (type 1); and two had left anterior descending (LAD) from the left-hand facing sinus that also gave rise to the right CA (RCA) (type 0). One patient had LAD from the left-hand facing sinus while RCA and circumflex artery from the right-hand facing sinus (type 10); and single CA from the anterior left-hand sinus (type 3c) was identified in another. APR was left anterior in type 1 and more lateral in types 0 and 3c; and left posterior in type 10 (p=0.001). The usual pulmonary trans-annular incision can only be performed in case of type 10 CA after posterior APR (10%, 1/10). In the majority (90%, 9/10 with the CA type 1, 0, and 3c), the RCA is in front of the pulmonary annulus, and the atrioventricular groove patch plasty posterior to the RCA had to be adopted for a trans-annular patch. CONCLUSIONS: The observed CA pattern was correlated with APR. APR identified by the aortic sinus pattern could implicate the CA patterns and the surgical options in ACM.

Clinical Implication of the C Allele of the ITPKC Gene SNP rs28493229 in Kawasaki Disease: Association With Disease Susceptibility and BCG Scar Reactivation.

BACKGROUND: a functional single nucleotide polymorphism (SNP) (rs28493229) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene has been linked to the susceptibility to Kawasaki disease (KD). The implication remains unclear. SUBJECTS AND METHODS: genotyping for the ITPKC polymorphism was conducted on 280 unrelated Taiwanese children with KD and 492 healthy ethnically and gender-matched controls. The clinical manifestations and laboratory data were systemically collected. RESULTS: the GC and CC genotypes of ITPKC gene SNP rs28493229 were overrepresented in KD patients (GG:GC:CC was 236:43:1, C allele frequency: 8.04%) than those in the controls (GG:GC:CC was 454:37:1, C allele frequency: 3.96%; OR: 2.23, P = 0.001). In KD patients, those with GC or CC genotypes of SNP rs28493229 (19/44) were more likely to have reactivation at the Bacille Calmette-Guérin (BCG) inoculation site than those with GG genotypes (66/236; OR: 1.96, P = 0.044). Such association was particularly strong in patients aged <20 months (OR: 3.26, P = 0.017). The other clinical manifestations were not related to this SNP. There were 160 (57.1%) patients with coronary arterial lesions. The development and the severity of coronary arterial lesion were also not associated with this SNP. Comparison between patients with and without BCG reactivation revealed only one difference: patients with reactivation were younger. CONCLUSION: in a cohort from a population with the world's third highest incidence of KD, we demonstrated that the C-allele of ITPKC SNP rs28493229 is associated with KD susceptibility and BCG scar reactivation during the acute phase, although its frequency is lower than that in the Japanese cohort (22.6%), suggesting this SNP contributes to KD susceptibility through induced hyperimmune function reflected in the BCG reactivation.