RESUMO
The Noonan syndrome is a rare disorder, one of whose major complications is cardiovascular involvement. A wide spectrum of congenital heart diseases has been observed in this syndrome. The most common cardiac disorder is pulmonary valve stenosis, which has a progressive nature. Hypertrophic cardiomyopathy is less common, but its morbidity and mortality rates are high. We herein introduce a 12-year-old boy with the typical findings of the Noonan syndrome. His symptoms began from infancy, and there was a gradual exacerbation in his respiratory and cardiac manifestations with age. The cardiac involvement included right ventricular outflow tract and pulmonary valve stenosis, hypertrophic cardiomyopathy, and subaortic valve stenosis. Due to the progressive course of the disease, surgical repair was done. Although the patient had a difficult postoperative period, his general condition improved and he was discharged. At 3 months' follow-up, his symptoms showed improvement. Additionally, there was a reduction in the echocardiographic parameters of the outflow tract stenosis gradient as well as a significant improvement in the cardiac hemodynamic indices.
RESUMO
BACKGROUND: Perimembranous Ventricular Septal Defect (PMVSD) is the most common subtype of ventricular septal defects. Transcatheter closure of PMVSD is a challenging procedure in management of moderate or large defects. OBJECTIVES: The purpose of this study was to show that transcatheter closure of perimembranous ventricular septal defect with Amplatzer Ductal Occluder (ADO) is an effective and safe method. PATIENTS AND METHODS: Between April 2012 and April 2013, 28 patients underwent percutaneous closure of PMVSD using ADO. After obtaining the size of VSD from the ventriculogram a device at least 2 mm larger than the narrowest diameter of VSD at right ventricular side was chosen. The device deployed after confirmation of its good position by echocardiography and left ventriculography. Follow up evaluations were done 1 month, 6 months, 12 months and yearly after discharge with transthoracic echocardiography and 12 lead electrocardiography. RESULTS: The mean age of patients at procedure was 4.7 ± 6.3 (range 2 to 14) years, mean weight 14.7 ± 10.5 (range 10 to 40) kg. The mean defect size of the right ventricular side was 4.5 ± 1.6 mm. The average device size used was 7.3 ± 3.2mm (range 4 to 12 mm). The ADOs were successfully implanted in all patients. The VSD occlusion rate was 65.7% at completion of the procedure, rising up to 79.5% at discharge and 96.4% during follow-up. Small residual shunts were seen at completion of the procedure, but they disappeared during follow-up in all but one patient. The mean follow-up period was 8.3 ± 3.6 months (range 1 to 18 months). Complete atrioventricular block (CAVB), major complication or death was not observed in our study. CONCLUSIONS: Transcatheter closure of PMVSD with ADO in children is a safe and effective treatment associated with excellent success and closure rates, but long-term follow-up in a large number of patients would be warranted.
RESUMO
BACKGROUND: The ventricular septal defect (VSD) is the most common form of congenital heart defects. The purpose of this study was to evaluate the results of the early complications and mid-term follow-up of the transcatheter closure of the VSD using the Amplatzer VSD Occluder. METHODS: Between April 2012 and October 2013, 110 patients underwent the percutaneous closure of the perimembranous VSD. During the procedure, the size and type of the VSD were obtained via ventriculography. A device at least 2 mm larger than the VSD diameter measured via ventriculography was deployed. The size of the VSD, size of the Amplatzer, and device-size to VSD-size ratio were calculated. After the confirmation of the suitable position of the device via echocardiography and left ventriculography, the device was released. Follow-up evaluations were done at discharge as well as at 1, 6, and 12 months and yearly thereafter for the VSD occlusion and complete heart block. RESULTS: The study population comprised 62 females and 48 males. The mean age and weight of the patients at procedure were 4.3 ± 5.6 years (range: 2 to 14) and 14.9 ± 10.8 kg (range: 10 to 43). The average device size was 7.0 ± 2.5 mm (range: 4 to 14). The VSD occlusion rate was 72.8% at the completion of the procedure and rose up to 99.0% during the follow-up. The most serious significant complication was complete atrioventricular block, which was seen in 2 patients. The mean follow-up duration was 10.9 ± 3.6 months. CONCLUSION: The transcatheter closure of the perimembranous VSD was a safe and effective treatment with excellent closure rates in our study population. This procedure had neither mortality nor serious complications.
RESUMO
BACKGROUND: Thrombosis is the most common complication during cardiac catheterization via femoral artery access. Alongside heparinization, fibrinolytic therapy is recommended if there are signs of ischemia in the lower extremity. OBJECTIVES: Given the paucity of data in the existing literature on streptokinase (SK) therapy in pediatrics, we designed this study to assess the efficacy of SK in pediatric patients with diagnosed femoral artery thrombosis following cardiac catheterization. PATIENTS AND METHODS: The study population initially consisted of 1788 pediatric patients who underwent cardiac catheterization via the femoral artery access. Diminished or absent pulses in the lower extremity were detected in 123 patients, 45 of whom (2.5% of 1788) required treatment and were therefore considered for the next stage of study. Treatment was comprised of post-procedural intravenous heparin, either 50 U/kg/Q4h or 10 - 20 U/kg/h continuously. After heparinization for 24 hours, if the pulse of the affected extremity was not palpable, heparin therapy was continued (heparin-treated group, n = 28), and if the symptoms of femoral artery ischemia were persistent, heparin was discontinued and intravenous SK with a loading dose of 2000 U/kg over 20 - 30 minutes was commenced (SK-treated group, n =17). RESULTS: In the presence of pulselessness in the lower extremity, a maintenance dose of SK (1000 U/kg/h, during 1 - 24 hours) was intravenously administered. Regarding the return of the pulses post-therapeutically, normal and weak/absent pulses were detected in seven (25.2%) and 21 (74.8%) of the 28 patients, respectively, in the heparin-treated group (P value < 0.001), whereas normal and weak/absent pulses were detected in 15 (88.2%) and two (11.8%) of the 17 patients, respectively, in the SK-treated group (P value < 0.001). CONCLUSIONS: Our findings demonstrated a high success rate and a low complication rate for systemic SK therapy in femoral artery thrombosis after catheterization.
RESUMO
BACKGROUND: The ductus arteriosus connects the main pulmonary trunk to the descending aorta. The incidence of isolated patent ductus arteriosus (PDA) in full-term infants is about 1 in 2000. The Amplatzer Ductal Occluder (ADO) is recommended for PDAs with sizes larger than 2 mm. In this procedure, we must confirm the ADO position in PDA by aortogram from the arterial line. The purpose of this study was to determine the optimal release time of the ADO in the PDA closure procedure, especially in the absence of an arterial line for post-PDA aortography. METHODS: This study recruited all patients scheduled to undergo PDA transcatheter closure with the ADO between September 2009 and September 2012 in our center. Age, weight, PDA diameter, systolic and diastolic pulmonic pressures, fluoroscopy time, and total angiographic time were studied. Major complications such as mortality and vascular complications were considered. RESULTS: We studied 237 patients in our investigation. We had 130 female and 107 male patients at a mean age of 34.3 ± 40.6 months and mean weight of 14.2 ±7.8 kg. PDA sizes ranged from 2.1 to 6.2 mm and its mean was 3.7 ± 1.8 mm. Mean of fluoroscopy time was 11.4 ± 9.7 min and mean of total angiographic time was 42.0 ± 12.3 min. There were no significant complications. CONCLUSION: We herein describe a new sign, which proved extremely helpful during our PDA closure procedures with the ADO. By considering the angle between the ADO and the cable during the procedure, the operator can release the ADO safely.
