Comparison of the Results of Cardiopulmonary Exercise Testing between Healthy Peers and Pediatric Patients with Different Echocardiographic Severity of Mitral Valve Prolapse.

Patients with mitral valve prolapse (MVP) have been reported to have exercise intolerance. However, the underlying pathophysiological mechanisms and their physical fitness remain unclear. We aimed to determine the exercise capacity of patients with MVP through the cardiopulmonary exercise test (CPET). We retrospectively collected the data of 45 patients with a diagnosis of MVP. Their CPET and echocardiogram results were compared with 76 healthy individuals as primary outcomes. No significant differences regarding the patient's baseline characteristics and echocardiographic data were found between the two groups, except for the lower body mass index (BMI) of the MVP group. Patients in the MVP group demonstrated a similar peak metabolic equivalent (MET), but a significantly lower peak rate pressure product (PRPP) (p = 0.048). Patients with MVP possessed similar exercise capacity to healthy individuals. The reduced PRPP may indicate compromised coronary perfusion and subtle left ventricular function impairment.

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.

Rapid spontaneous closure and remodelling of a medium-sized symptomatic congenital coronary artery fistula in an infant before the age of 3 months.

It is controversial to observe or close symptomatic congenital coronary artery fistula in infants. We herein describe a medium-sized symptomatic congenital coronary artery fistula that underwent rapid spontaneous closure in an infant aged <3 months.

Reappraisal of the prostaglandin E1 dose for early newborns with patent ductus arteriosus-dependent pulmonary circulation.

OBJECTIVES: The usual initial dose of prostaglandin E1 (PGE1) for ductal-dependent congenital heart disease (CHD) is 50-100 ng/kg/minute. The aim of this study was to review our experience of a low initial dose of PGE1 treatment in early newborns with congenital heart disease and patent ductus arteriosus (PDA)-dependent pulmonary flow. METHODS: We reviewed the clinical data of 33 newborns with CHD and PDA-dependent pulmonary circulation who were admitted from January 2005 to December 2010. Clinical parameters were collected, including, PGE1 dosage, oxygenation condition, vital signs, and other related clinical parameters during admission. Echocardiography was employed to assess the status of the PDA as clinically indicated. RESULTS: Thirty-three newborns, including 17 males and 16 females, with CHD and PDA-dependent pulmonary circulation were enrolled in the study. Their mean age was 2.9 ± 5.1 (within the range of 1-26) days with a median of 1.0 day. Among the 33 cases, 25 were diagnosed with pulmonary atresia and eight with critical pulmonary stenosis. Twenty-five of our patients were treated with the initial low-dosage regimen of 20.0 ± 7.4 ng/kg/minute in our neonatal intensive care unit. None of these 25 patients with had significant apnea necessitating intubation and none had hypotension, fever, convulsion or cortical hyperostosis. Three of the eight patients who were treated with high-dose PGE1 (39 ± 13.2 ng/kg/minute) before referral to our unit had apnea and intubation after PGE1 use. All patients had adequate PDA patency with a low maintenance dose of 10.5 ± 5.3 ng/kg/minute before operation under our protocol. CONCLUSION: In our experience, adequate PDA flows in early newborns with CHD and PDA-dependent pulmonary circulation could be achieved at a much lower dose than recommended in the literature. The lower dose of PGE1 also causes much fewer complications, such as apnea, fever, and hypotension. For early newborns with CHD and PDA-dependent pulmonary circulation, treatment with a lower initial dose of PGE1 of 20 ng/kg/minute and a maintenance dose of 10 ng/kg/minute is recommended.

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.

Closure of large patent ductus arteriosus in infants.

The purpose of this study was to evaluate the feasibility, clinical outcome, and resource utilization of transcatheter closure of large, symptomatic patent ductus arteriosus (PDA) in young infants, and compare the results with those of surgery. From January 2003 to December 2006, successful transcatheter implantation of the Amplatzer duct occluder was performed in 20 young infants (< or = 3 months of age) with large PDA who suffered from severe congestive heart failure despite medical treatment. The data was compared with a historical control of 18 young infants who underwent surgical ligation of PDA. Patient ages ranged from 18 to 89 days (mean 51.8) in the study group and 13 to 82 days (mean 39.9) in the control group. Patient weight ranged from 2.7 to 7.1 kg (mean 4.24) in study group and 2.5 to 5.7 kg (mean 3.79) in the control group. Between the 2 groups, there was no significant difference in the rate of complications. Patients who underwent transcatheter closure utilized significantly fewer resources (length of hospital stay and total cost) than those who underwent surgery. In conclusion, percutaneous device occlusion with Amplatzer duct occluder in small, symptomatic infants is feasible and more cost-effective compared with surgery. In experienced hands with careful preprocedural evaluation and delicate manipulation, the safety of transcatheter closure with Amplatzer ductus occluder in this age group can be assured and major complication avoided. Revision of the delivery sheath, making it kink-resistant, and PDA shape-tailored devices should further optimize the procedure.

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.

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.

A potentially fatal complication during subclavian vein catheterization in an infant with congenital heart disease-puncture to pulmonary artery directly: a case report.

The most frequent and acute complications of subclavian vein catheterization are arterial puncture to the subclavian artery and pneumothorax. We report an arterial puncture directly to the pulmonary artery in infant during subclavian vein catheterization.

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.

Supraclavicular versus infraclavicular subclavian vein catheterization in infants.

BACKGROUND: Central venous catheterization is an important procedure for infant patients for a number of different purposes, including nutritional support, surgical operation, hemodynamic monitoring, and multiple lines for critical care medications. Subclavian vein catheterization (SVC) is one of the central vein catheterization techniques. SVC can be performed from 4 different locations: right supraclavicular (RSC), left supraclavicular (LSC), right infraclavicular (RIC), and left infraclavicular (LIC). The purpose of this study was to evaluate the relative effectiveness and complication risks of these 4 SVC locations in infants. METHODS: In our pediatric intensive care unit, which is part of a tertiary medical center, a well-trained fellow doctor performed the following catheterizations: 21 RSC, 24 LSC, 24 RIC, and 22 LIC, for a total of 91 SVC operations in infants. The patients were placed in the Trendelenburg position. The site of puncture was decided by the operator. Statistical significance was analyzed according to Fisher's exact test and 2-sample t test. RESULTS: The overall success rate was 90.1% (82 out of 91 operations). No statistically significant differences were noted among these 4 groups, either in the success or complication rate. There were 6 cases of arterial puncture (5 supraclavicular and 1 infraclavicular, p = 0.09), 2 cases of pneumothorax (1 RSC and 1 RIC), and 2 cases of malpositioned catheter (1 RSC and 1 RIC). There was no mortality. CONCLUSION: In our study, we found that there was no statistically significant difference among the 4 SVC locations in effectiveness of operation or in risk of complication. There was a tendency to damage the subclavian arteries through the supraclavicular route.

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.

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.

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.

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).

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.