This study demonstrated thromboembolic pulmonary arterial hypertension to be a crucial complication in congenital portosystemic venous shunt, and this pathologic state may be latently present in patients with pulmonary arterial hypertension of unknown etiology.

We analyzed the genetic polymorphisms of vascular endothelial growth factor (VEGF) and its receptors [Fms-related tyrosine kinase-1, kinase insert domain receptor (KDR)] in Japanese patients with Kawasaki disease (KD) and normal control subjects to examine whether these genes would contribute to the KD occurrence and/or the development of coronary artery lesion (CAL) in KD. We found that the frequency of G allele of VEGF g.Ϫ634 GϾC single-nucleotide polymorphism in the promoter region was significantly higher in KD patients with CAL than in those without CAL (p ϭ 0.012) or control subjects (p ϭ 0.021) because of a significantly higher frequency of the GG genotype in KD patients with CAL. In addition, the frequency of the A1 allele with 11 AC repeats of KDR g.ϩ4422(AC)11-14 dinucleotide repeat polymorphism in intron 2 was significantly higher in KD patients with CAL than in those without CAL (p ϭ 0.013) or control subjects (p ϭ 0.040) as a result of a significantly higher frequency of the A1A1 genotype in KD with CAL patients. The multivariate analysis of clinical features and genotypes of the two polymorphisms showed that the A1A1 genotype of KDR g.ϩ4422(AC)11-14 polymorphism was an independent risk factor for the development of CAL with the highest odds ratio among several clinical parameters (odds ratio 6.76; 95% confidence interval 1. 05-43.48). Dual luciferase assay demonstrated that the A1 allele with KDR g.ϩ4422(AC)11 repeats showed a weaker silencer function than the A2 allele with 12 AC repeats. These findings suggested that VEGF and its receptor, KDR, genes contributed to the development of CAL in KD patients. Kawasaki disease (KD) is an acute febrile vasculitis of infants and children and is accompanied by the coronary artery lesions (CALs) that occur in~5-16% of patients (1,2). Even after the introduction of high-dose i.v. immunoglobulin (IVIG) therapy, CAL occurs in a small proportion of KD patients and leads to life-threatening complications, including myocardial infarction, as well as acquired heart diseases such as myocardial dysfunction, valvular diseases, and arrhythmias (3,4). Therefore, it is important to identify as early as possible KD patients who are at risk for the development of CAL.During the acute stage of KD, activation of the immune system with increased serum cytokines such as tumor necrosis factor-␣, interferon-␥, IL-1, IL-2, IL-6, and IL-8 may play important roles in the occurrence of endothelial cell injury (5-7). The pathologic findings of vascular tissues in KD patients include subendothelial edema, vascular damage, gap formation, and fenestration of endothelial cells (8,9

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Cardiac surgery contributed to increased survival rate but not the rate of discharge alive in trisomy 18 patients. Cardiac surgery could not prevent all the trisomy 18 patients from death. The indication of cardiac surgery should be carefully individualised to improve the quality of life in trisomy 18 patients and concerned surrounding people.