Increased plasma type B natriuretic peptide in the acute phase of Kawasaki disease.

BACKGROUND: The aim of this study was to identify possible factors associated with type-B natriuretic peptide (BNP) production in the acute phase of Kawasaki disease (KD). METHODS: Subjects were 54 patients with KD (KD group [KDG]) and 18 age-matched controls (control group [CG]). We evaluated left ventricular function using multi-modal echocardiography and determined blood chemistry including BNP, white blood cell count, C-reactive protein (CRP), and interleukin (IL)-6 in the KDG. We compared echocardiographic parameters between the KDG and the CG and determined the correlation between log (BNP) and echocardiographic parameters, white blood cell count, CRP, and IL-6 in the KDG. RESULTS: The KDG showed high BNP (169.6 ± 529.6 pg/ml) despite preserved left ventricular function indicated by no significant difference in left ventricular ejection fraction (72.2 ± 9.2 vs 71.2 ± 7.8 %), z-score of left ventricular diastolic dimension (0.8 ± 1.3 vs 0.9 ± 0.8 SD), and Tei index (0.29 ± 0.09 vs 0.30 ± 0.06) between the KDG and the CG. However, left ventricular ejection fraction (r =-0.44, P= .001) and left ventricular end-diastolic dimension (r = 0.30, P < .05) significantly correlated with log (BNP). On the other hand, the KDG showed high CRP (89.7 ± 55.6 mg/l) and high IL-6 (242.2 ± 243.5 pg/ml), and CRP (r = 0.60, P < 0.0001) and IL-6 (r = 0.78, P < 0.0001) significantly correlated with log (BNP). Multiple stepwise regression analysis identified IL-6 (r = 0.77, P < 0.0001) most significantly correlated with log (BNP). CONCLUSIONS: In acute KD, BNP significantly increases, despite well-preserved global left ventricular function, and inflammation might be associated with this increased BNP.

Portal-systemic encephalopathy after Fontan-type operation in patient with polysplenia syndrome.

An 18-year-old patient, who had polysplenia and single ventricle, presented with altered mental status 9 years after a Fontan-type operation and pacemaker implantation. He underwent replacement of common atrioventricular valve and aortic valve plasty 1 year previously and has been placed on multiple medications including beta-blocker for his poor ventricular function. Blood chemistry revealed hyperammonemia of 2420 microg/l as a cause of this altered mental status disturbance. Superior mesenteric arteriography revealed large portal-systemic shunts in venous phase as a cause of hyperammonemia. To control blood ammonia level, we placed him on low protein diet, oral polymixin B, and lactulose instead of closing shunt with device. This case illustrates that portal-systemic shunt may result in hyperammonemia leading to altered mental status long after a Fontan-type operation.

[New diagnostic methods and modalities for congenital heart disease].

Ultrasound imaging of cardiovascular structures has progressed rapidly from standard surface techniques to real-time 3-dimensional echocardiography and intracardiac echocardiography. Especially, 3-dimensional echocardiography is having a significant impact on the evaluation of congenital heart diseases (CHD). However, the present system of prenatal screening for CHD in Japan is not as efficient as it is in some Western countries. For this reason, the number of patient referrals for fetal echocardiography and the fetal diagnosis of CHD increased year after year. Gestational age at referral and at the diagnosis of CHD has increased rapidly in recent years. To improve the patient's prognosis, we should form more simple prenatal primary screening programs for CHD. In addition, we require the progression of the diagnostic modalities and familiarize these new diagnostic methods and modalities.

Transcatheter placement of a low-profile biodegradable pulmonary valve made of small intestinal submucosa: a long-term study in a swine model.

OBJECTIVE: We sought to investigate a placement of a percutaneous low-profile prosthetic valve constructed of small intestinal submucosa in the pulmonary position in a swine model. METHODS: Twelve female farm pigs were stented at the native pulmonary valve to induce pulmonary insufficiency. Once right ventricular dilation occurred, the small intestinal submucosa valve was implanted. The pigs were followed up with transthoracic echocardiographic Doppler scanning. One animal died of heart failure before valve replacement. Animals were euthanized at 1 day, 1 month, 3 months, 6 months, and 12 months after valve implantation. RESULTS: The small intestinal submucosa pulmonary valve showed effective reversal of pulmonary regurgitation. There were no misplacements during deployment. There were no embolizations. One-year echocardiographic follow-up showed minimal regurgitation and no stenosis for a valve/vessel ratio of 0.78 or greater. Histologic examination demonstrated intensive remodeling of the small intestinal submucosal valve. Within 1 month, the surface was covered by endothelium, and fibroblasts invaded the interior. Over the following months, the small intestinal submucosal valve remodeled without apparent graft rejection. CONCLUSION: The small intestinal submucosa valve has the potential for graft longevity without the need for anticoagulation or immunosuppression. Histologic remodeling of the valve tissue provides a replacement capable of resembling a native valve that can be placed percutaneously with low-profile delivery systems.

Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study.

BACKGROUND: The purpose of this study was to assess the sequential follow-up results of catheter intervention in Kawasaki disease by use of quantitative coronary angiography (QCA) and intravascular ultrasound imaging. METHODS AND RESULTS: Catheter intervention was performed on 23 stenotic lesions in 22 patients (aged 2 to 24 years). Percutaneous balloon angioplasty (PBA) was performed in 4 patients, stent implantation in 7, percutaneous transluminal coronary rotational ablation (PTCRA) in 10, and a combination of PTCRA with stent implantation in 2. A total of 21 lesions (91%) were successfully dilated by catheter intervention without major or minor complications. One patient immediately underwent coronary artery bypass grafting (CABG) surgery because stent implantation failed to resolve his lesion. At 4 to 6 months after catheter intervention, 2 restenotic lesions (9%) were detected by QCA in 2 patients who had undergone PBA, and these patients subsequently underwent CABG surgery. In 6 months to 3 years after catheter intervention, no patients showed evidence of ischemic findings. At 3 to 4 years after catheter intervention, QCA and intravascular ultrasound studies were performed on 15 lesions in 14 patients. Two restenotic lesions (13%) were detected by QCA in 2 patients. One of the 2 had stent implantation and underwent CABG surgery, and the other had undergone PTCRA and underwent re-PTCRA. Thirteen patients demonstrated no ischemic findings at 3 to 8 years after catheter intervention. CONCLUSION: Catheter intervention for Kawasaki disease can be accomplished and can be effective in the short term, but the long-term efficacy should be verified by further study.