Effect of Limb Salvage by Excimer Laser Angioplasty Plus Low-Pressure Balloon Inflation in Chronic Limb-Threatening Ischemia Patients with Infrapopliteal Vessel Disease.

Background: Patients with chronic limb-threatening ischemia (CLTI) often exhibit long, diffuse, totally occluded and heavily calcified infrapopliteal (IP) lesions. This study evaluated limb salvage after peripheral excimer laser atherectomy (PELA) plus low-pressure balloon inflation (LPBI) without stent deployment in CLTI patients with severe IP disease. Methods: We retrospectively evaluated 70 consecutive patients with 109 IP vessels who underwent PELA plus LPBI from 2010 to 2013. Technical success was defined as at least one IP straight-line flow being achieved below the malleolus. Binary logistic regression was performed to identify factors associated with 6-month limb salvage. Results: Of the 109 IP vessels, 100 (91.7%) were totally occluded, and none of the patients received a stent. Of the 70 patients, 20% were octogenarians, and 85.8% had a Rutherford-Becker class 5 and 6. The technical success rate was 87.1% and 6-month limb salvage rate was 78.6%. Rutherford score was negatively correlated with clinical success (adjusted odds ratio 0.24; p = 0.028). No immediate major cardiovascular events were recorded during admission. Conclusions: PELA plus LPBI may be a treatment option for complex IP lesions in patients with CLTI. Higher Rutherford class was correlated with a lower 6-month limb salvage rate. However, a large-scale study with a control group is needed to clarify our results.

Relation of early post-stress left ventricular dyssynchrony and the extent of angiographic coronary artery disease.

BACKGROUND: Previous studies showed different dyssynchrony patterns between ischemic and normal myocardium at early post-stress using Tl-201 gated SPECT myocardial perfusion imaging (MPI). The aim of this study was to assess the relation of stress-induced dyssynchrony and the extent of angiographic coronary artery disease (CAD). METHODS AND RESULTS: The MPI images of 144 patients were retrospectively analyzed. With ≥70% stenosis as the criteria of CAD, 57 had no CAD, 32 had 1-vessel disease, 36 had 2-vessel disease, and 19 had 3-vessel disease, respectively. LV global and territorial dyssynchrony parameters were measured by the phase analysis from stress/rest Tl-201 SPECT MPI and compared between stress and rest among the patient groups. The patients with multi-vessel CAD had significantly more global dyssynchrony than the patients without ≥70% stenosis at stress. The patients with multi-vessel CAD showed significantly more global and territorial dyssynchrony on stress images than on rest. More patients with 3-vessel CAD were correctly classified as multi-vessel disease, when combining both visual interpretation and dyssynchrony assessment. CONCLUSION: The patients with multi-vessel CAD had significantly more global and territorial dyssynchrony at early post-stress than at rest. Such quantitative measures of myocardial stunning may assist in the diagnosis of multi-vessel CAD.

Short-Term Safety and Efficacy of Femoral Vascular Closure after Percutaneous Coronary Intervention with Combination of the Boomerang(TM) Device and Intravenous Protamine Sulfate.

BACKGROUND: The Cardiva Boomerang(TM) is a device used to perform femoral vascular closure. It facilitates passive hemostasis at the arteriotomy site, leaving no residual foreign body. METHODS: We performed a controlled, randomized study of 60 patients undergoing percutaneous coronary intervention. Patients were randomized into two groups (30 per group) to undergo vascular closure with the Boomerang(TM) or the Perclose(TM) suture-based device after the intravenous administration of protamine sulfate. We compared overall success rates, patient-reported pain, length of time to achieve hemostasis and mobilization of the patient, and the frequency of complications in the two groups. RESULTS: Overall success rates using the Boomerang(TM) and Perclose(TM) devices were similarly high, at 93% and 97%, respectively. The Boomerang(TM) was significantly quicker to deploy than the PercloseTM, device deployment time, median (Q1-Q3), [2.00 (1.33-2.75) vs. 3.84 (2.75-4.38) mins, p < 0.001)]. The pain score was significantly lower in the Boomerang(TM) group (1.1 ± 1.7 vs. 6.4 ± 2.9, p < 0.001). The time the device remained in the artery and manual compression time were significantly longer with the Boomerang(TM) (p < 0.001), as well as the time taken to achieve hemostasis and time to ambulation. There were no major complications in either group and no significant differences between the groups in the frequency of minor complications. CONCLUSIONS: We conclude that when used in combination with intravenous protamine sulfate, the Boomerang(TM) device is as safe and effective as the Perclose(TM) device for femoral vascular closure, but quicker to deploy and less painful to patients. KEY WORDS: Boomerang; Percutaneous intervention; Vascular closure device.

Noninvasive methods of accurately diagnosing in children anomalous origin of the left coronary artery from the pulmonary trunk.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary trunk is a rare congenital heart defect. Cardiac catheterization remains the standard means of diagnosis. Our purpose in this study is to emphasize the importance of assessing the electrocardiogram when making the diagnosis, in addition to taking note of transthoracic echocardiographic findings. We also analyzed the sensitivity of each parameter under investigation. METHODS AND RESULTS: Between June, 1999, and March, 2007, we studied 9 patients, 6 males and 3 females, with a mean age of 3.02 years, in whom anomalous origin of the left coronary artery from the pulmonary trunk was suspected subsequent to transthoracic echocardiographic examination. We examined their electrocardiograms, and undertook cardiac catheterization. In all patients, the transthoracic echocardiogram had shown retrograde flow into the pulmonary trunk, with the left coronary artery arising from pulmonary trunk, along with a dilated right coronary artery, or intercoronary collateral vessels. In 8 patients, the electrocardiogram showed deep Q wave in leads I and aVL, with depression of the ST segments over lead V4 through 6, or inversion of the T waves in leads I, II, and aVL. In the remaining patient, the electrocardiogram showed incomplete right bundle branch block. Later, cardiac catheterization confirmed the diagnosis in 8 patients, but the other patient was shown to have the right coronary artery arising from the pulmonary trunk. CONCLUSIONS: By combining transthoracic echocardiography with study of the electrocardiogram, it is possible to provide accurate evaluation of anomalous origin of the left coronary artery from the pulmonary trunk.

Significant relationship between serum high-sensitivity C-reactive protein, high-density lipoprotein cholesterol levels and children with Kawasaki disease and coronary artery lesions.

BACKGROUND/PURPOSE: The aim of this study was to understand the association between Kawasaki disease (KD) in children and serum high-sensitivity C-reactive protein (hs-CRP), which is a sensitive indicator of inflammation, lipid profiles and coronary artery lesions. METHODS: Between July 2005 and December 2007, 119 children with KD at least 1 year after diagnosis were recruited. The children were classified into one of two groups: Group I comprised 55 children with KD and coronary aneurysms; Group 2 comprised 64 children with KD and normal coronary arteries. The relationship between hs-CRP concentration and high-density lipoprotein cholesterol (HDL-C) and the size of arterial lesions was investigated in Group I 1 year after onset of KD. RESULTS: Serum hs-CRP levels in Group I (mean, 0.251 mg/dL) were significantly greater than those in Group II (mean, 0.162 mg/dL; p = 0.011). However, plasma HDL-C levels in Group I (mean 42.51 mg/dL) were significantly lower than those in Group II (mean, 44.34 mg/dL; p = 0.037). In Group I, there was a positive association between hs-CRP and the size of coronary artery lesions (r = 0.672, p = 0.035), but no association between lipid profiles, including HDL-C, and coronary artery lesions (all p > 0.05). CONCLUSION: Our results support the possibility of ongoing low-grade inflammation late after the acute phase of KD in children with coronary aneurysms. Serum hs-CRP and HDL-C levels are associated with coronary artery lesions in children with KD.

Guidewire and catheter manipulation without coil placement to close minimal patent ductus arteriosus.

BACKGROUND: Transcatheter coil closure (TCC) is safe and effective for most patients with PDA, but coil associated complications may occasionally be encountered. For occlusion of minimal PDA (<1 mm), we employed a closure protocol with guidewire and catheter manipulation. METHODS: Between April 2000 and September 2004, 38 patients with a minimal PDA were classified into two groups according to the occlusion method at our institution. Group A consisted of 11 patients (age range 0.7 to 3.5 years; mean age 1.6 years) who underwent guidewire and catheter manipulation. Group B consisted of 27 patients (age range 0.5 to 2.7 years; mean age 1.3 years) who underwent TCC of PDA. RESULTS: In group A (a PDA 0.4-0.9 mm), 9 (82%) patients had successful closure, and two (18%) patients failed the manipulation. No patient had complications during the procedure or follow-up. In group B (a PDA 0.5-0.9 mm), 26 (96%) patients had successful closure and 1 (4%) patient failed the attempt at TCC. No adverse events of coil closure was found during follow-up. Compared to the patients in group B, those in group A differed significantly in terms of procedure time. There were no significant differences in age, sex, body weight, PDA size, fluoroscopy time, success rate, and complication rate. CONCLUSIONS: Our results indicate that the manipulation is safe and effective for patients with minimal PDA. The manipulation technique can be tried before TCC in patients with minimal PDA.

Assessment of branch pulmonary artery stenosis in children after repair of tetralogy of Fallot using lung perfusion scintigraphy comparison with echocardiography.

PURPOSE: The aim of this study was to compare the usefulness of lung perfusion scintigraphy and echocardiogram in the evaluation of the branch pulmonary arteries stenosis in children with tetralogy of Fallot (TOF). MATERIALS AND METHODS: From February 2006 to November 2008, 74 children (mean age 7.8 years, range 1­18 years) who underwent repair of TOF at ages from 10 months to 13 years were suspected to have unilateral or bilateral branch pulmonary artery stenosis. In all patients, cardiac angiography was performed to confirm the diagnosis of branch pulmonary artery stenosis. Lung perfusion scintigraphy and two-dimensional transthoracic echocardiography were performed in all patients to compare their abilities to diagnose branch pulmonary artery stenosis. RESULTS: Of the 74 patients, 51 cases were found to have branch pulmonary artery stenosis by cardiac angiography. There was agreement between the scintigraphic and angiographic findings in 44 (86%) patients and there were discrepancies in 11 (15%) patients. The positive predictive value of our lung perfusion scintigraphy in detecting the branch pulmonary artery stenosis was 92 %. The positive and negative likelihood ratios of lung perfusion scintigraphy were 4.96 and 0.17, respectively. There was conformity between the echocardiographic and angiographic findings in 40 (78%) patients with discrepancies in 16 (21%) patients. The positive predictive value of our echocardiography in detecting the branch pulmonary artery stenosis was 89%. The positive and negative likelihood ratios of echocardiography were 3.61 and 0.28, respectively. CONCLUSIONS: Lung perfusion scintigraphy is a valuable, non-invasive screening tool in the assessment of branch pulmonary artery stenosis in children after TOF.

Trends in the incidence of hepatocellular carcinoma in boys and girls in Taiwan after large-scale hepatitis B vaccination.

In July 1984, large-scale hepatitis B vaccination of newborns began in Taiwan. Vaccination decreased the overall incidence of childhood hepatocellular carcinoma (HCC). We conducted this study to learn whether the vaccination program had the same effect on boys and girls. We collected liver carcinoma (including HCC and hepatoblastoma) deaths from 1974 to 1999 from the Taiwan Mortality Registry and the 1974-1999 population data from the Taiwan Ministry of Interior to calculate the liver carcinoma mortality rate. The populations ages 0-14 and ages 15-100 in each calendar year were treated as the study group and the reference group, respectively. We divided the 1974-1999 calendar years into 4-year strata and calculated the mortality rates of each 4-year period. We used the 1980-1983 mortality rate as the standard to calculate 4-year-interval mortality rate ratios. Vaccination effects by age and gender were estimated dividing the study and the reference groups into male and female subgroups. We used a double-comparison method to confirm the effects of hepatitis B vaccination: the mortality rate trend of the study group (ages 0-14) compared with the reference group (ages 15-100) in the same period (1984-1999), and the mortality rate trend of the study group (age 0-14) compared with itself in the pre- and postvaccination periods (1974-1983, 1984-1999). Liver carcinoma mortality decreased significantly among both males and females after 1984. In the study group, the male mortality rate decreased by up to 70%, and the female mortality rate decreased by up to 62% in the 1996-1999 interval compared with the 1980-1983 period. Both the male and the female study groups' mortality rate trends decreased from 1983 to 1999 compared with the 1974-1983 period or compared with the same period of the reference groups. Our results indicate hepatitis B vaccination decreases childhood HCC in both boys and girls.

Safety and efficacy of using 0.052-inch Gianturco coil for closure of large ( > or = 4mm) patent ductus arteriosus.

Transcatheter closure of patent ductus arteriosus (PDA) is now a well-established treatment alternative to surgery in many cardiology centers. Of all the methods used, transcatheter coil occlusion is the preferred therapy. For small PDA, the method using 0.038" Gianturco coils has proven safe and effective. However, this therapeutic strategy has encountered some difficulties with large PDA. This study provides an alternative strategy, using 0.052" Gianturco coil and complete closure of residual shunt with multiple coils to close large PDA. Fifteen patients underwent transcatheter coil occlusion of large ( > or = 4mm) patent ductus arteriosus. The intermediate success rate was 86.7%. There were four complications and only two patients had to be referred for surgery.

Identification of candidate genes for congenital ventricular septal defects with HSA22q11 loss of heterozygosity.

INTRODUCTION AND OBJECTIVES: Ventricular septal defect (VSD) is one of the major forms of congenital heart disease (CHD) in individuals with Homo sapiens chromosome 22q11 (HSA22q11) deletion syndrome. The objective was to identify candidate genes associated with VSD located within HSA22q11 by analyzing loss of heterozygosity (LOH) using microsatellite genotyping and by gene dosage analysis in seven candidate genes. METHODS: The study involved 82 families with CHD, which included 261 individuals (85 patients and 176 siblings and parents). All were screened for LOH in the HSA22q11 region by microsatellite (n=10) genotyping. Bioinformatic strategies were used to characterize seven candidate genes located within this region in greater detail. Quantitative polymerase chain reaction analysis was used to determine the dosages of the seven candidate genes in 16 patients with LOH of HSA22q11. RESULTS: Overall, 42 out of 85 patients (49.4%) with CHD had at least one LOH in the HSA22q11 region. Moreover, LOH of HSA22q11 was found in 17 out of 29 patients with a VSD and in three out of four families with two offspring affected by CHD. Dosage analysis of the seven candidate genes showed recurrent heterozygous deletion of HIRA, GNB1L and TUBA8 genes in 16 VSD patients with a LOH of HSA22q11. CONCLUSIONS: Microsatellite genotyping identified LOH of HSA22q11 in several types of CHD. Heterozygous deletion of HIRA, GNB1L or TUBA8 genes might play an important role in ventricular septum development. Since CHD can be a familial disease, screening the siblings of a proband for LOH of HSA22q could be valuable for early diagnosis and treatment.

Comparison of 0.052-inch coils vs amplatzer duct occluder for transcatheter closure of moderate to large patent ductus arteriosus.

BACKGROUND: Percutaneous device closure is now the widely accepted management technique for patent ductus arteriosus (PDA). In the present study, current practice of closing moderate to large PDA using the Amplatzer duct occluder (ADO) was compared with the previous experience using 0.052-inch coils. METHODS AND RESULTS: From August 1997 to September 2006, 76 patients were selected for either 0.052-inch coils or ADO implantation. Selection criteria were a minimal diameter of > or =2.5 mm and angiographic type A, C, D or E. In group I, 21 patients received 0.052-inch coils in a multiple coil strategy for PDA closure. In group II, 55 patients underwent ADO device implantation. The age, weight, hemodynamics, minimal PDA diameter and shunt size were insignificantly different between groups. However, group I had a higher rate of failed implantation and device embolization, and the immediate and 24-h closure rates (38.9% vs 81.8%, P<0.05; 72.3% vs 96.4%, P<0.05) favored group II. CONCLUSIONS: ADO implantation is safer and more effective than the 0.052-inch coil strategy for transcatheter closure of moderate to large PDA.

Early and late outcomes of coil embolization of pulmonary sequestration in children.

BACKGROUND: Pulmonary sequestration (PS) is characterized by non-functioning lung tissue fed from 1 or several aberrant systemic arteries. The classical therapeutic approach is surgical resection. Several case reports have shown that coil embolization is feasible, but this technique has not been evaluated in a larger series of consecutively treated patients. The purpose of our study was to assess the early and long-term outcomes of coil embolization of PS in children and to determine the risk factors of early and late major adverse cardiovascular and pulmonary events. METHODS AND RESULTS: Between March 1999 and December 2004, 6 patients (2 boys, 4 girls, mean age 4.7 +/-3.8 years) with PS were treated by coil embolization of the feeding systemic artery. Four patients were considered to have been cured and 2 patients required a second coil embolization 6 months later because of residual systemic flow seen on computed tomography. Transient ischemic change of the lower limb occurred in the youngest patient. None of the other patients had any late complications or recurrent pneumonia. CONCLUSIONS: Coil embolization of PS is safe and feasible, with a good late outcome.

Clinical results of percutaneous closure of large secundum atrial septal defects in children using the Amplatzer septal occluder.

We reviewed our experience using the Amplatzer septal occluder (AGA Medical, Golden Valley, MN, USA) to close large, secundum-type atrial septal defects (ASDs) in children. Between June 2002 and December 2005, 52 patients (mean age 13.5 +/- 8.7 years) underwent transcatheter closure of large (> or =25 mm), secundum ASDs with the use of the Amplatzer septal occluder (ASO). Groups 1 and 2 included patients with a retroaortic rim of <5 mm (n = 39) or > or =5 mm (n = 13), respectively. All procedures were performed with general anesthesia and transesophageal echocardiographic guidance except for 10 patients, which involved local anesthesia and three-dimensional transthoracic echocardiography. Successful device implantations, device sizes, approaches, complications, and closure rates were assessed. Device implantation was successful in 50 patients (96.1%), with no difference between groups (95% vs 100%, P>0.05). In 2 patients, implantation failed because of embolism or deployment failure. Device were larger in group 1 than in group 2 (29.7 +/- 4.2 vs 26.7 +/- 3.8 mm, P = 0.04). The right upper pulmonary-vein approach was more common in group 1 than in group 2 (P = 0.0001). Complications and closure rates did not differ between the groups (P > 0.05). Transcatheter closure of large, secundum ASD by using the ASO device was feasible, and complication rates were low. A deficient retroaortic rim did not preclude successful device implantation; however, a large device may be needed to close large ASD. Close long-term follow-up is necessary to determine the safety of transcatheter closure of large ASDs in children.

Balloon angioplasty of native coarctation and comparison of patients younger and older than 3 months.

BACKGROUND: There is a high incidence of restenosis and aneurysm formation after balloon angioplasty for discrete native coarctation in neonates and young infants, and so the techniques remains controversial in this group of patients because its clinical validity, particularly in comparison with surgery, has not been well established. METHODS AND RESULTS: From January 1999 to October 2005, group A (17 patients [8 males, 9 females] <3 months old ranging from 0.2 to 2.9 months, with a body weight of 2.5-5.5 kg) and group B (11 patients [5 males, 6 females] >3 months old ranging from 5.5 months to 6.4 years, with a body weight of 7.8-21 kg) with discrete native coarctation who underwent balloon angioplasty and were included in this study. There were 13 (76%) successes in group A, and 10 (90%) successes in group B for the initial balloon angioplasty. There was no significant difference in success rate between groups A and B (p>0.05). There were 9 (69%) cases of restenosis patients in group A, and 2 (20%) in group B, a significant difference between the 2 groups (p<0.05). In group A, 1 patient showed aneurysm formation after angioplasty, 1 (5.8%) showed femoral artery obstruction and 2 (11%) showed reduced pulses. CONCLUSIONS: Balloon angioplasty of discrete native coarctation is effective in patients both younger and older than 3 months. However, thea rates of restenosis, aneurysm formation, and approach artery injury are higher in patients younger than 3 months old when compared with patients aged over 3 months. These complications should be considered when performing balloon angioplasty in patients less than 3 months of age.

A noninvasive sizing method to choose fitted Amplatzer septal occluder by transthoracic echocardiography in patients with secundum atrial septal defects.

To simplify the conventional procedure, we developed a technique for transcatheter closure of atrial septal defects (ASDs) under transthoracic echocardiographic (TTE) sizing without balloon sizing. At present, device closure of interatrial communication has become a well-established technique to adequately treat severe left-to-right shunt associated with ASDs. During the traditional procedure, fluoroscopy with the waist of a compliant balloon is used to determine the appropriate size of the closure device and defect sizing. Choice of adequate closure device using transthoracic echocardiography (TTE) has been hitherto unreported. Between December 2002 and August 2004, 40 patients (15 males, 25 females, mean age 11.7 +/- 7.8 years) with secundum ASDs underwent transcatheter closure at our institution. In group 1, 30 patients had the procedure by balloon sizing and TTE sizing. In 10 patients (group 2), TTE sizing was used as the sole tool for selecting device size and the device size was chosen to be based on the Amplatzer septal occluder (ASO) size and TTE size ratio in group 1. The procedure was performed under continuous transesophageal echocardiographic monitoring with general anesthesia. A correlation was found between TTE and stretched balloon sizing diameter SBD (y = 1.2645x - 1.4465; R2 = 0.9861), and between TTE size and ASO size (y = 1.3412x - 1.2864; R2 = 0.9929) in group 1. In group 2, a statistical correlation between TTE and ASO (y = 1.3419x - 0.1172; R2 = 0.9934) was also found. Good linear regression between TTE size and ASO chosen size was noted in group 1 and group 2 (R2 = 0.99). In group 2, successful device implantation was accomplished in all patients whose device size was chosen to be based on the ASO and TTE ratio in group 1. Transthoracic echocardiographic sizing is a safe and ideal method to measure interatrial defect and choose the occluding device, respectively. With our experience, the sizing based on TTE is generally easier than measurement from the balloon sizing.

Secondary confirmation of endotracheal tube position by ultrasound image.

OBJECTIVE: Secondary confirmation of endotracheal (ET) tube position by ultrasound image. DESIGN: Prospective, randomized study. SETTING: A medical center-based tertiary pediatric intensive care unit. PATIENTS: A total of 59 patients aged from newborn to 17 yrs old underwent ET tube insertion because of cardiopulmonary arrest or impending respiratory failure. INTERVENTION: Ultrasound imaging was performed immediately before and after the ET tube placement procedure. The most frequently used ultrasonic scanning window was the subxiphoid window at the mid-upper abdominal, just beneath the xiphoid process and the lower margin of liver. The sector angle was set as wide as possible (90 degrees) so that the bilateral diaphragm could be well scanned. MEASUREMENTS AND MAIN RESULTS: Using the ultrasound imaging method, we successfully identified all of two esophageal intubations and eight incidents of initial ET tube misplacement, which had been positioned down to the right main bronchus. Finally, we successfully identified all 59 of the correct placements of ET tubes in the trachea. CONCLUSIONS: Ultrasound imaging of diaphragm motion is a useful, quick, noninvasive, portable, and direct anatomic method for assessment of ET tube position. We think it should be considered the method of choice for the secondary confirmation of the ET tube position.

Treatment of acute Kawasaki disease: aspirin's role in the febrile stage revisited.

OBJECTIVE: To evaluate the effect of treatment without aspirin in the acute phase of Kawasaki disease (KD) and to determine whether it is necessary to expose children to high- or medium-dose aspirin. METHODS: A total of 162 patients who fulfilled the established criteria of acute KD between 1993 and 2003 were included in this retrospective study. All patients were treated with high-dose intravenous immunoglobulin (IVIG; 2 g/kg) as a single infusion without concomitant aspirin treatment. Low-dose aspirin (3-5 mg/kg per day) was subsequently prescribed when fever subsided. Patients who had defervescence within 3 days after the completion of IVIG treatment were classified as the IVIG-responsive group, and those whose fever persisted for >3 days were classified as the IVIG-nonresponsive group. The 162 patients were divided further into 2 groups: those who were treated with IVIG before illness day 5, and those who were treated after illness day 5. We compared the response rate of IVIG therapy, duration of fever, and incidence of coronary artery abnormalities (CAAs) between these groups. RESULTS: A total of 153 patients were classified into the IVIG-responsive group, and 128 (83.66%) of them had defervescence within 24 hours after completion of IVIG therapy. Nine (5.56%) patients were classified into the IVIG nonresponsive group, and all received additional IVIG (2 g/kg) without aspirin. Six (66.67%) had defervescence within 3 days after additional therapy. Patients in the IVIG-nonresponsive group had a significantly higher incidence of CAAs than those in the IVIG-responsive group (25% vs 2.92%). In the group that was treated before illness day 5 (n = 16), all patients had defervescence within 3 days after IVIG therapy and 13 (81.25%) had defervescence within 24 hours. In the group that was treated after illness day 5 (n = 146), 137 (93.84%) patients had defervescence within 3 days and 115 (78.77%) had defervescence within 24 hours. One (6.67%) patient in the group that was treated before illness day 5 got a new onset of CAAs, as did 5 (3.85%) in the group that was treated after illness day 5. There was no statistically significant difference in the response rate of IVIG therapy, duration of fever, and incidence of CAAs between these 2 groups. CONCLUSION: The results of our study indicate that the treatment without aspirin in acute stage of KD had no effect on the response rate of IVIG therapy, duration of fever, or incidence of CAAs when children were treated with high-dose (2 g/kg) IVIG as a single infusion, despite treatment before or after day 5 of illness. We conclude that it seems unnecessary to expose children to high- or medium-dose aspirin therapy in acute KD when the available data show no appreciable benefit in preventing the failure of IVIG therapy, formation of CAAs, or shortening the duration of fever.

Use of Inoue balloon dilatation method for treatment of Cor triatriatum stenosis in a child.

An 8-year-old girl was referred for evaluation of a heart murmur and progressive dyspnea with exertion. Transthoracic echocardiogram revealed Cor triatriatum with severe stenosis at its orifice. The resting transmembrane CW Doppler velocity was 2.3 m/sec. Cardiac catheterization and hemodynamic assessment were performed and demonstrated a marked increase in pulmonary capillary wedge and pulmonary artery pressures. The stenosis was successfully dilated with an Inoue balloon using a transseptal technique. This is the first description of dilation of a Cor triatriatum using the Inoue technique. Only one previous case report of balloon dilatation for Cor triatriatum sinister has appeared in the English literature using a different technique (double balloon method).

Identificación de genes candidatos en las comunicacionesinterventriculares congénitas con pérdida de heterocigosisde HSA22q11 / Identification of Candidate Genes for Congenital Ventricular Septal Defects With HSA22q11 Loss of Heterozygosity

Introducción y objetivos. La comunicación interventricular(CIV) es una de las principales formas de cardiopatíacongénita (CPC) en los individuos con el síndromede deleción del cromosoma 22q11 del Homo sapiens(HSA22q11). Con objeto de identificar los genes candidatossituados en el HSA22q11 asociados a la CIV, seanalizó la pérdida de heterocigosis (LOH) mediante determinacióndel genotipo de microsatélites y análisis dedosis de siete genes candidatos.Métodos. Se investigó a un total de 82 familias conCPC, en las que había 261 individuos (85 pacientes, 176hermanos y progenitores). Se efectuó un examen de detecciónde la LOH en la región HSA22q11 mediante determinacióndel genotipo de microsatélites (n = 10). Lasestrategias de bioinformática permitieron caracterizarmás detalladamente siete genes candidatos situados enesa región. Se aplicaron reacciones en cadena de polimerasacuantitativas para el análisis de dosis a siete genescandidatos de 16 pacientes con LOH de HSA22q11.Resultados. En 42 (49,4%) de los 85 pacientes conCPC se identificó la presencia de al menos una LOH enla región HSA22q11. Se observaron LOH de HSA22q11en 17 de 29 pacientes con CIV y en 3 de 4 familias conambos hijos afectados por CPC. El análisis de dosis realizadopara siete genes candidatos indicó una deleciónheterocigota recurrente de los genes HIRA, GNB1L y TUBA8en 16 pacientes con CIV que presentaban LOH deHSA22q11.Conclusiones. La determinación del genotipo de microsatélitesidentificó la LOH de HSA22q11 en variostipos de CPC. La deleción heterocigota de los genesHIRA, GNB1L o TUBA8 podría desempeñar un papelimportante en el desarrollo del tabique ventricular. Dadoque la CPC puede ser una enfermedad familiar, el examende detección de LOH de HSA22q en los hermanosdel caso índice será útil para un diagnóstico y un tratamientoprecoces (AU)

Introduction and objectives. Ventricular septal defect(VSD) is one of the major forms of congenital heart disease(CHD) in individuals with Homo sapiens chromosome22q11 (HSA22q11) deletion syndrome. The objective wasto identify candidate genes associated with VSD locatedwithin HSA22q11 by analyzing loss of heterozygosity(LOH) using microsatellite genotyping and by gene dosageanalysis in seven candidate genes.Methods. The study involved 82 families with CHD,which included 261 individuals (85 patients and 176siblings and parents). All were screened for LOH in theHSA22q11 region by microsatellite (n=10) genotyping.Bioinformatic strategies were used to characterize sevencandidate genes located within this region in greater detail.Quantitative polymerase chain reaction analysis was usedto determine the dosages of the seven candidate genes in16 patients with LOH of HSA22q11.Results. Overall, 42 out of 85 patients (49.4%) with CHDhad at least one LOH in the HSA22q11 region. Moreover,LOH of HSA22q11 was found in 17 out of 29 patients witha VSD and in three out of four families with two offspringaffected by CHD. Dosage analysis of the seven candidategenes showed recurrent heterozygous deletion of HIRA,GNB1L and TUBA8 genes in 16 VSD patients with a LOHof HSA22q11.Conclusions. Microsatellite genotyping identified LOHof HSA22q11 in several types of CHD. Heterozygousdeletion of HIRA, GNB1L or TUBA8 genes might play animportant role in ventricular septum development. SinceCHD can be a familial disease, screening the siblings ofa proband for LOH of HSA22q could be valuable for earlydiagnosis and treatment (AU)