Ongoing vascular inflammation evaluated by 18F-fluorodeoxyglucose positron emission tomography in patients long after Kawasaki disease.

BACKGROUND: This study aimed to determine whether ongoing vascular inflammation presents in patients who had coronary artery aneurysms (CAAs) caused by Kawasaki disease (KD). METHODS: Subjects were 26 patients with a history of KD; 15 had giant CAA (gCAA) ≥ 8.0 mm and 11 had smaller CAA (smCAA) < 8 mm in the acute phase. They underwent X-ray computed tomography and 18F-fluorodeoxyglucose positron emission tomography. We determined the maximum coronary target-to-background ratio (CaTBR) and the mean thoracic aorta TBR (TaTBR) in each patient. They were compared between groups, and their correlation with various variables was determined. RESULTS: CaTBR and TaTBR were significantly higher in gCAA than in smCAA (P < .005 for both values) and were significantly higher even in patients without any metabolic risk factor (P < .05 for both values). The CAA size in acute phase significantly positively correlated with CaTBR (R2 = 0.32) as well as TaTBR (R2 = 0.28). Also, TaTBR significantly positively correlated with CaTBR (R2 = 0.32) as well as cumulative number of metabolic risk factors (trend, P = .03). CONCLUSIONS: Ongoing vascular inflammation may present long after KD, especially in patients with severe inflammation expressed as gCAA in the acute phase.

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.

Intergenerational differences in the effects of transcatheter closure of atrial septal defects on cardiac function.

BACKGROUND: Little is known regarding intergenerational differences in the effects of atrial sepal defect (ASD) closure on the left heart. We therefore analyzed age-related serial changes in the left heart following ASD closure. METHODS: We studied 50 patients with an isolated ASD who underwent successful transcatheter closure using Amplatzer septal occluders (St. Jude Medical, Little Canada, MN, USA) between June 2007 and June 2013. Patients were divided into three age groups: young patients aged ≤17 years; middle-aged patients aged 18-50 years; and older patients aged >50 years. Multi-modal echocardiographic studies with different views were performed before and at 1 day, 1-3 months, and 6-12 months after ASD closure. Echocardiographic variables were compared among the groups at different time points after closure. RESULTS: Left ventricular end-diastolic and end-systolic volume indices (EDVI and ESVI) in the older group were significantly smaller than those in the other groups before closure. EDVI and ESVI increased with time after closure in all groups with stable ejection fractions. However, EDVI and ESVI remained significantly smaller in the older group compared with the other groups. There was a significant interaction among the age groups only in terms of left atrial volume index (LAVI). LAVI increased significantly with time in the older group, but did not change in the other groups. CONCLUSION: Although the left ventricle enlarged with time after ASD closure in all age groups, left ventricular size in older patients never reached that in younger patients. In addition to this inadequate enlargement of the left ventricle, diastolic dysfunction might also result in late left atrial enlargement in older patients following ASD closure.

Transpulmonary echocardiography to guide stent implantation into coarctation of the aorta.

Although stent implantation into aortic coarctation has been performed solely under fluoroscopy, we successfully applied intracardiac echocardiography (ICE) to guide this procedure in a 13-year-old patient. Placing an intracardiac echocardiographic catheter in the left pulmonary artery facing upward, we readily visualized the precise anatomy of coarctation, measured the pressure gradient, and monitored the stent inflation process. This report suggests a new application of ICE for intervention with structural and vascular diseases other than interatrial septum.

Successful diuretics treatment of protein-losing enteropathy in Noonan syndrome.

There are few reports on successful high-dose spironolactone treatment of refractory protein-losing enteropathy (PLE) caused by Fontan procedure. We report successful diuretics treatment with spironolactone and furosemide at standard dose, of refractory PLE in a patient with Noonan syndrome and repaired congenital heart disease. This is the first successful application of diuretics treatment in a patient with refractory PLE without Fontan procedure. This case illustrates that diuretics treatment can be the first-line treatment of PLE regardless of the causative physiology, and can be effective in refractory PLE with Noonan syndrome.

Trans-pulmonary echocardiography as a guide for device closure of patent ductus arteriosus.

OBJECTIVES: The aim of this study was to develop trans-pulmonary echocardiography (TPE) to guide device closure of patent ductus arteriosus (DC-PDA). BACKGROUND: Aortography requires a large amount of contrast yet may give us an inadequate image to evaluate anatomy or residual shunt in patients with large PDA or dilated vessels and is precluded in patients with renal dysfunction. Practically, there is no imaging modality to monitor the entire procedure except for trans-esophageal echocardiography that requires general anesthesia. METHODS: Subjects were seven patients with ages ranged from 6- to 77-years old and body weight > 15 kg. The size of the PDA ranged from 1.8 to 6.3 mm with pulmonary to systemic flow ratios from 1.2 to 2.2. During DC-PDA using Ampaltzer Duct Occluder or coil, an intra-cardiac echocardiographic (ICE) catheter was advanced into pulmonary arteries and standard views were developed to guide DC-PDA. RESULTS: We have developed two standard views; the main pulmonary artery view (MPA view) and the left pulmonary artery view (LPA view). The MPA view provided aortic short axis view equivalent to that seen by trans-thoracic echocardiography in children. The LPA view, obtained by the echo probe in the LPA and turned it up upside down, provided long axis view of the PDA allowing more precise anatomical evaluation. TPE allowed us to monitor the entire procedure and determine residual shunts. CONCLUSIONS: TPE in the MPA and LPA view can be an effective guide for DC-PDA. This report leads to new application of this imaging device.

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.

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.

Microembolic signals measured by transcranial Doppler during transcatheter closure of atrial septal defect using the Amplatzer septal occluder.

PURPOSE: To determine the frequency and factors associated with increase in microembolic signals during transcatheter closure of atrial septal defect using the Amplatzer septal occluder. METHODS: During the procedure in 16 patients, we measured microembolic signals using transcranial Doppler. Procedure time was divided into five periods: right cardiac catheterisation; left cardiac catheterisation; left cardiac angiocardiography; sizing and long sheath placement; device placement and release. We compared numbers of microembolic signals among the five periods and identified factors associated with them. RESULTS: Mean size of septal occluder was 16 millimetres in diameter. Total number of microembolic signals was a median of 31.5, ranging from 3 to 113. Microembolic signals in three periods, left cardiac catheterisation; sizing, and long sheath placement; and device placement and release, were not significantly different from one another, but were significantly higher than those in the remaining two periods, right cardiac catheterisation and left cardiac angiocardiography (median was 9 in left cardiac catheterisation; 6 in sizing and long sheath placement; 6.5 in device placement and release, versus 0 in right cardiac catheterisation and 1 in left cardiac angiocardiography, p less than 0.05, respectively). Importantly, the time for device manipulation positively correlated with total number of microembolic signals (r equals 0.77, p less than 0.001), although fluoroscopic time, age, or size of septal occluder did not. CONCLUSIONS: Transcatheter closure of atrial septal defect using the Amplatzer septal occluder produces microemboli, especially during device placement. To minimise the risk of systemic embolism, we must decrease the time for device manipulation.

Stanford type B aortic dissection associated with pregnancy in patients with Marfan syndrome-A case report and review of the literature.

A 36-year-old female patient known to have Marfan syndrome (MFS) presented with Stanford type B aortic dissection (type B-AD) 3 days after delivery although she had taken oral ß-blocker and underwent prophylactic cesarean section at 34 weeks when she showed 42 mm of the ascending aorta. She was successfully treated medically without further progression of the dissection. A review of the literature revealed an additional 19 patients with MFS who suffered from type B-AD associated with pregnancy. Of 20 patients, 1 (5%) died but the remaining 19 patients were successfully treated either medically (n = 9) or surgically (n = 10). Of 13 patients whose aortic diameter was known, 5 showed <40 mm of the ascending aorta. Pregnancy in MFS can be complicated by type B-AD with a peak around term delivery irrespective of the size of ascending aorta and even with ß-blocker.

Health-related quality of life in adolescents and young adults with a history of Kawasaki disease.

OBJECTIVE: To investigate the health-related quality of life (HRQOL) in adolescents and young adults with Kawasaki disease (KD). STUDY DESIGN: We conducted a cross-sectional observational study. Patients were eligible for this study when they were > or =16 years of age and had received a diagnosis of KD > or =5 years before the start of this study. The patients were divided in 3 groups according to their coronary status: normal, aneurysms, and giant aneurysms/ischemia. A self-administered questionnaire (Medical Outcome Study Short Form 36) was mailed to the patients to evaluate the HRQOL. RESULTS: Of 624 total questionnaires mailed, 377 were delivered, and 250 were returned. The response rates of the normal, aneurysm, and giant aneurysms/ischemia groups were 33%, 62%, and 72% of all eligible patients, respectively. All subscale scoress of HRQOL, except vitality and role-emotional (limitations in the usual role activities because of emotional problems) in patients with KD were significantly higher than scores from the national norms. CONCLUSIONS: The HRQOL of adolescents and young adults with a history of KD is favorable. However, long-term follow-up is necessary, because the general health perceptions of older patients tended to be lower.

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.

Warfarin therapy for giant aneurysm prevents myocardial infarction in Kawasaki disease.

We retrospectively investigated the effect of warfarin therapy in improving the clinical outcome of Kawasaki disease (KD) patients with giant coronary aneurysms (GAs). We followed 2350 KD patients from 1973 to 2004. The GAs (> or =8 mm in diameter) were diagnosed by coronary angiography. Sixty-eight patients (54 males and 14 females) were retrospectively studied. Patients were divided into two groups. One group consisted of 19 patients with 33 branches treated with a combination of low-dose aspirin and warfarin (target international normalized ratio: 1.5 - 2.5 IU). The second group consisted of 49 patients with 102 branches treated with low-dose aspirin without warfarin. The incidence of myocardial infarction was significantly less in the combination therapy group than in patients treated with aspirin without warfarin (1 patient vs. 16 patients, p < 0.05). Sudden death occurred in seven patients taking aspirin without warfarin, but none of the patients receiving warfarin died. No major bleeding events occurred in either group. Combination therapy of warfarin with aspirin is associated with a decreased risk of myocardial infarction in KD patients with GAs.