These results indicate that in patients with Kawasaki disease the coronary disease accompanying impaired reactivity to nitroglycerin is present at the sites of regressed aneurysms as well as in angiographically normal coronary segments. We suggest that these sites with morphologic and functional abnormalities are related to the development of significant stenosis.

This study determined the long-term outcome for patients after myocardial infarction (MI) due to Kawasaki disease (KD). Retrospective analysis was performed for 60 patients who had experienced MI between 1976 and 2007. Their ages at the initial MI ranged from 3 months to 33 years (median, 2 years). The maximum follow-up period after the initial MI was 33 years (median, 16 years). Coronary angiography, left ventriculography, and radioisotope myocardial perfusion imaging (MPI) had been performed for 56 patients more than 2 months after MI when all were in stable condition. The survival rate and ventricular tachycardia (VT)-free survival rate were calculated after the initial MI by the Kaplan-Meier method. Both sustained and nonsustained VT were included. Furthermore, the Cox proportional hazards model was used to analyze which factors influenced the post-MI outcome and which influenced the appearance of VT. The 30-year survival rate was 62.7% (95% confidence interval [CI], 44.6-77.9%), and the 25-year VT-free survival rate after MI was 28.5% (95% CI 15.4-46.5%). The postinfarction left ventricular ejection fraction (LVEF) was related to the outcome in this population (hazard ratio 0.86; 95% CI 0.75-0.95; P = 0.002), whereas the development of VT was related to the post-LVEF and to perfusion abnormalities in MPI (P = 0.0002). The 30-year survival rate after MI was poor for the patients with a low LVEF. With aging, the existence of nonviable myocardium in the infarct area can induce fatal ventricular arrhythmia more than 10 years after the original MI.

The endothelial function of children with and without vascular disease, consisting of 41 controls, 24 with Kawasaki disease (KD), and 46 with diabetes mellitus (DM), was examined. Age at examination ranged from 3 to 23 years (mean, 12.0 +/- 4.7). The flow-mediated dilatation (FMD) and intima-media complex in the common carotid artery were measured. In controls age at examination was not associated with FMD or intima-media complex. FMD significantly decreased in children with KD and DM compared with the control group (control vs KD or DM: 11.7 +/- 14.7 vs 3.0 +/- 11.0 or 6.4 +/- 8.5%, respectively; p < 0.05). However, there was no significant difference for intima-media complex among the groups. Furthermore, FMD in KD patients with coronary arterial aneurysm was lower than that in KD patients without aneurysm (-0.5 +/- 9.2 vs 8.3 +/- 9.1%, p < 0.05). In DM patients, FMD in the high HbA1c group (HbA1c = 7%) was lower than that in the normal HbA1c group (HbA1c < 7%) (4.8 +/- 8.1 vs 11.4 +/- 7.8%, p < 0.05). In conclusion, FMD detected endothelial impairment in children with KD or type 1 DM regardless of overt vascular complications, and FMD impairment occurs prior to intima-media complex thickening. By measuring both FMD and intima-media complex, useful information for predicting vascular complications may be obtained.

Objectives: To evaluate the effectiveness and long term outcomes of catheter intervention for obstructive conduits between the right ventricle and pulmonary arteries. Design: Retrospective chart review. Setting: Tertiary care paediatric cardiology unit. Patients and interventions: 70 procedures in 68 children (median age at intervention 6 years, median interval after conduit insertion 3.4 years) were analysed. All children had haemodynamic indications for conduit replacement. Twenty four children underwent a second intervention (stent dilatation in 17, second stent implantation in seven). Results: Mean (SD) conduit pressure gradient decreased from 44 (18) mm Hg to 18 (12) mm Hg at the initial intervention (n = 62, p , 0.001) and from 39 (15) mm Hg to 23 (10) mm Hg at the second intervention (n = 16, p , 0.001). The percentage of the predicted right ventricular outflow area increased from 17 (9)% to 44 (22)% at the initial intervention (n = 62, p , 0.001) and from 24 (8)% to 29 (11)% at the second intervention (n = 21, p , 0.001). The conduit was subsequently replaced in 33 children. Freedom from conduit replacement from the time of stent implantation was 83%, 75%, and 47% at one, two, and five years, respectively, and from the time of the initial conduit surgery it was 87%, 64%, and 42% at five, eight, and 10 years, respectively. Body growth was maintained, no deaths were reported during follow up, and pulmonary insufficiency was well tolerated. Conclusion: A catheter treatment strategy for obstructive conduits is safe and effective in prolonging conduit function.

AimHepatocellular carcinoma (HCC) can arise from Fontan‐associated liver disease (FALD); this is known as FALD‐HCC. The clinical features of FALD‐HCC are unclear. Thus, we examined the incidence and clinical characteristics of FALD‐HCC.MethodsFrom 1972 to 2019, 122 patients developed liver disease after undergoing Fontan procedures. HCC was diagnosed in 12 (9.8%) FALD patients. We compared FALD‐HCC and non‐HCC patients.ResultsThe incidence of HCC was 0.8% and 2.9% in FALD 10 and 20 years after the Fontan procedure, respectively. The median age of patients at diagnosis of HCC was 32.5 years (range 20.6–46.1 years), and seven of the 12 patients were men. Patients with FALD‐HCC had a higher incidence of liver cirrhosis and polysplenia than non‐HCC patients. Liver tumors were detected as single nodules in eight patients, and the median diameter was 47 mm (range 11–105 mm). HCC was treated by surgical resection in two patients, transcatheter arterial chemoembolization or chemotherapy in three patients, and proton beam therapy in four patients. Three patients could not be treated because of their poor condition. Four patients died of liver/cardiac failure and HCC, and HCC was controlled in three patients. The survival rate after 25 years was significantly lower in patients with FALD‐HCC than non‐HCC patients (68.6% vs. 97.9%, respectively; P < 0.01).Conclusions: Of the 122 patients with FALD, 12 developed HCC 20 years after surgery. Because complications of HCC are associated with poor prognosis, constant surveillance for HCC should begin 10 years after surgery.

Treatment for peripheral pulmonary artery stenosis is challenging, and conventional balloon angioplasty has not proved to be universally effective. Evaluated was the efficacy of bladed balloon (BB) dilation to address vessels resistant to conventional high-pressure (10-15 atm) balloon angioplasty (BA). Thirty-one procedures were performed on 14 children with age range 1 month to 15 years. The diameter of the BB ranged from 3 to 8 mm. After BB dilation, all children had subsequent conventional BA (balloon size range, 3-10 mm). The minimal lumen diameter (MLD) before and after the procedure, whether there was a waist at initial BA, and BB diameter-to-MLD ratio before the procedure were measured. A > 50% increase in MLD was considered successful. Four children had Williams syndrome, two children Alagille syndrome, five children Fallot's tetralogy, and three miscellaneous lesions. The resistant stenosis was located in the right central pulmonary artery in 6, right branch pulmonary artery in 7, left central pulmonary artery in 6, and left branch pulmonary artery in 12 lesions. Median BB diameter was 253% (117-440%) of the MLD and increased from 2.0 +/- 0.7 to 3.2 +/- 0.8 mm (P < 0.0001), with a mean increase of 73% +/- 62%. There was an inverse relationship between the MLD before and increase after the procedure (r = 0.75; P < 0.001). The BB diameter-to-MLD ratio before procedure was significantly associated with the increase in MLD (r = 0.70; P < 0.001). After the procedure, 18 of the 31 procedures were considered successful. In all successful procedures, the BB diameter was greater than twice the MLD before the procedure. Comparing children with Williams and Alagille syndrome with the remaining eight children, there were no significant differences in the increase in MLD. A small aneurysm and thrombus were noticed in two and three children, respectively, but no fatal complications were reported. BB angioplasty is effective for resistant peripheral pulmonary artery stenosis when conventional BA fails. The diameter of the BB should be larger than twice the minimal luminal diameter of the stenotic lesion.

Objectives: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. Methods: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. Results: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 ± 3.5 years, a mean interval of 2.7 ± 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 ± 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 ± 2.8 versus 11.1 ± 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind™ occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 ± 6% to 95 ± 3% (p < 0.01). Conclusion: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.

Cases of hypoplastic left heart syndrome (HLHS) were studied angiographically in 18 patients and pathologically in 22 patients. They were divided into three subgroups according to the morphological features of the left heart: mitral atresia with aortic atresia (MA/AA), mitral stenosis with aortic atresia (MS/AA) and mitral stenosis with severe aortic stenosis (MS/AS). Patients with MS/AA had a significantly lower right ventricular end-diastolic volume index and more hypokinesis of the right ventricular posterior wall than those with MA/AA. MS/AA not only increased the thickness of the left ventricular posterior wall and interventricular septum but also increased endocardial thickness compared with MA/AA. Myocardial histology revealed more frequent abnormal findings such as myocardial necrosis, calcification and interstitial fibrosis in the mitral stenosis groups (i.e., MS/AA and MS/AS) than in MA/AA. Right ventricular function appeared to be greatly influenced by left heart structure. The presence of larger left ventricular muscle bulk and frequent myocardial damage seen in MS/AA seems disadvantageous to right ventricular end-diastolic volume and right ventricular wall motion.