Robust Effect of Hepatic Arterial Infusion Chemotherapy and Radiation Therapy on Hepatocellular Carcinoma Arising from Fontan-associated Liver Disease.

Fontan-associated liver disease (FALD) caused by long-term systemic venous congestion following the Fontan procedure may eventually lead to hepatocellular carcinoma (HCC). Treatment strategies for HCC due to FALD (FALD-HCC) remain unclear. We herein report a 35-year-old man with FALD-HCC that was well controlled by 3 cycles of continuous infusion of 5-fluorouracil and low-dose cisplatin (low-dose FP therapy) combined with 60 Gy of radiation therapy. However, the patient ultimately died of extrahepatic metastases. A pathological autopsy revealed more than 90% necrosis in the primary HCC lesion. This case suggests that low-dose FP therapy might be effective in FALD-HCC.

Intracardiac Echocardiography as a Guide for Transcatheter Closure of Patent Ductus Arteriosus.

BACKGROUND: Transcatheter closure of patent ductus arteriosus (TC-PDA), conventionally guided by aortography, has become the standard treatment of this disease. The purposes of this study were to evaluate whether intracardiac echocardiography (ICE) may be used for measuring PDA size and be used as a guide for TC-PDA. METHODS: This study had 2 phases. In phase 1, we compared the measurements of PDA size: pulmonary artery side diameter (PA-D), length, and aortic side diameter (Ao-D) of PDA, as measured by ICE with those measured by aortography or cardiac computed tomography (AoG/CCT) in 23 patients who underwent TC-PDA. In phase 2, we compared the demographics, fluoroscopic time, contrast volume, and complications of the TC-PDAs between 10 adult patients with ICE guidance and 16 without it. RESULTS: In phase 1, we found great correlation and agreement between ICE and AoG/CCT in PA-D (r = 0.985, bias -0.077 to 0.224), but moderate to poor correlation and agreement in length (r = 0.653, bias -0.491 to 3.065) and Ao-D (r = 0.704, bias 0.738 to 4.732), respectively. Nevertheless, all patients underwent successful TC-PDA with ICE guidance that allowed us to continuously monitor the whole process. In phase 2, TC-PDA required a significantly lower contrast volume with ICE guidance than without it, and there was no significant difference in the remaining variables between the 2 groups. CONCLUSION: ICE is comparable to AoG/CCT in providing accurate PA-D of the PDA and may be a safe alternative to guide TC-PDA as compared to conventional aortography.

Growth differentiation factor 15 as a useful biomarker of heart failure in young patients with unrepaired congenital heart disease of left to right shunt.

BACKGROUND: Growth differentiation factor 15 (GDF 15) is a member of the transforming growth factor-beta superfamily and is considered to be a useful biomarker for severity of heart failure (HF) in repaired congenital heart disease (CHD). The aim of this study was to determine the clinical implication of GDF 15 in children with unrepaired CHD. METHODS: Subjects included 69 patients (≤14 years old) who had unrepaired CHD with left to right shunt and underwent cardiac catheterization. Demographic and hemodynamic data, including oxygen demand-supply relationship, were collected from medical records. Severity of HF was evaluated using modified Ross score. Serum GDF 15 levels were determined using enzyme-linked immunosorbent assay and correlated with patients' demographics, hemodynamic data, and blood chemistry data. RESULTS: Subjects had median age of 71 (range 1-173) months and simple acyanotic CHDs with mean pulmonary to systemic flow ratio of 2.0 (1.0-5.6), median N-terminal pro type Brain natriuretic peptide (NT-pro-BNP) of 162.8 (17.1-8789) pg/mL, and median GDF 15 of 242.1 (13.6-1116.7) pg/mL. GDF 15 significantly positively correlated with the modified Ross score, mean pulmonary artery pressure, oxygen extraction rate (OER), and Ln NT-pro-BNP, but negatively correlated with age, oxygen delivery and its components, and estimated glomerular filtration rate (eGFR). Multiple linear regression analysis revealed significant correlation of GDF 15 levels with the modified Ross score, OER, and eGFR. CONCLUSIONS: GDF 15 mainly reflects oxygen demand-supply relationship and can be used as a diagnostic marker of HF in unrepaired CHD with left to right shunt for a wide range of age and diagnoses.

Long-term prognosis of patients with Kawasaki disease complicated by giant coronary aneurysms: a single-institution experience.

BACKGROUND: Some patients with Kawasaki disease develop giant coronary aneurysms and coronary stenosis, leading to ischemic heart disease. The aim of this study was to determine the long-term prognosis of patients with Kawasaki disease with giant aneurysms. METHODS AND RESULTS: From our institutional database, 76 patients (57 men and 19 women) who developed giant aneurysms after January 1, 1972, were identified. Information on patient demographics, catheter and surgical interventions, and most recent status was collected from medical charts and patients' contacts. From these data, we calculated the survival rate and cumulative coronary intervention rate. The average age at onset was 2.9±2.9 years, and the median observational period was 19 years. During this period, 7 patients died and 1 patient underwent a heart transplantation, resulting in 95%, 88%, and 88% survival rates at 10, 20, and 30 years after the onset of KD, respectively. On the other hand, catheter and surgical coronary interventions (median, 1 intervention; range, 1 to 7 interventions) were performed to alleviate coronary ischemia in 46 patients (61%) at 1 month to 21 years (mode at 1 month) after onset, resulting in 28%, 43%, and 59% cumulative coronary intervention rates at 5, 15, and 25 years after onset, respectively. CONCLUSIONS: The long-term survival of patients with Kawasaki disease complicated by giant coronary aneurysms is moderately good with multiple catheter and surgical interventions. Further research should focus on the prevention of coronary vascular remodeling and on the indications for and effectiveness of percutaneous and surgical coronary interventions.

Impact of intravenous immunoglobulin infusion on longitudinal left ventricular performance in patients with acute Kawasaki disease of usual course.

PURPOSE: To determine the acute change in cardiac performance after intravenous immunoglobulin infusion (IVIG) in patients with acute Kawasaki disease (KD). MATERIALS AND METHODS: Subjects were 33 patients with KD who were treated with IVIG 2 g/kg and recovered without coronary artery lesion and 27 controls. Subjects underwent combined two-dimensional, Doppler, and tissue Doppler echocardiographic (TDI) studies. In KD, these echocardiographic studies were performed before IVIG, 48 h after IVIG, and in convalescence. Echocardiographic variables were compared between KD and controls as well as among 3 time points in KD. RESULTS: Before IVIG, KD showed significantly higher peak aortic velocity and shorter aortic ejection time as results of tachycardia and significantly lower E' (p<0.04) but significantly higher E/E' (p<0.02). After IVIG, patients with KD became afebrile and showed significantly lower TDI indices such as S', E', and, A' and isovolumic acceleration (IVA) (163+/-56 vs. 208+/-70 cm/s(2), p<0.01) with higher TDI-derived Tei index (0.50+/-0.10 vs. 0.44+/-0.06, p<0.02) than controls. These differences tended to disappear in convalescence. In analysis of repeated measurements, except for hemodynamic changes associated with tachycardia, S' (7.9+/-1.3 vs. 7.0+/-1.1 vs. 7.4+/-0.9 cm/s, p<0.001), IVA (227+/-72 vs. 163+/-56 vs. 180+/-63, p<0.05), and A' (7.7+/-3.0 vs. 5.6+/-1.3 vs. 6.7+/-2.3 cm/s, p<0.001) were significantly different among these time points. CONCLUSIONS: In patients with acute KD with usual course, IVIG induced transient sub-clinical longitudinal left ventricular dysfunction.

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.

Benign infantile convulsions associated with mild gastroenteritis: a retrospective study of 39 cases including virological tests and efficacy of anticonvulsants.

Benign convulsions associated with mild gastroenteritis (CwG) are a commonly observed disorder in Asia, especially in infants and seniors. Here, we describe a retrospective study about the clinical features of CwG in 62 children hospitalized at St. Mary's Hospital (Kurume City, Japan) between January 1, 2000 and March 31, 2006, and further evaluate the efficacies of various anticonvulsant treatments for patients with CwG due to either rotavirus or norovirus. Causative diarrheal viruses were detected in 71% of the fecal specimens tested; 30 patients were positive for rotavirus, nine patients were positive for norovirus, two patients were positive for sapovirus, two patients were positive for adenovirus, and one patient was positive for coxackievirus A4. The age of onset for patients with norovirus-positive CwG (16.7+/-2.7 months) was significantly lower than that of patients with rotavirus-positive CwG (23.0+/-8.7 months). The duration of the seizures due to norovirus infection (11.8+/-12.0 h) was significantly longer than that due to rotavirus infection (4.9+/-5.7 h). There were no significant differences between the two groups with regard to the results of blood chemistry analysis, including the concentrations of serum electrolytes, blood glucose levels, and liver function tests. In this preliminary study, the duration of seizures in patients with CwG due to norovirus that was treated with carbamazepine was significantly shorter than the duration of seizures in the patients treated with another anticonvulsant (phenobarbital). Further randomized controlled studies are required to clarify the efficacies of the various anticonvulsants for patients with CwG.