Transcatheter stenting of arterial duct in duct-dependent congenital heart disease

Introduction. Critical congenital heart diseases (CHD) are mostly duct-dependent and require stable systemic-pulmonary communication. In order to maintain patency of the ductus arteriosus (DA), the first line treatment is Prostaglandin E1 and the second step is the surgical creation of aortic-pulmonary shunt. To reduce surgical risk in neonates with the critical CHD, transcatheter stenting of DA can be performed in selected cases. Case Outline. A four-month old infant was diagnosed with the pulmonary artery atresia with ventricular septal defect (PAA/VSD). The left pulmonary artery was perfused from DA, and the right lung through three major aortopulmonary collaterals (MAPCAs). A coronary stent was placed in the long and critically stenotic DA, with final arterial duct diameter of 3.5 mm, and significantly increased blood supply to the left lung. After the procedure, the infant’s status was improved with regard to arterial oxygen saturation, feeding and weight gain. During the follow-up, one year later, aortography revealed in-stent stenosis. The left pulmonary artery, as well as the branches, was well-developed and the decision was made to proceed with further surgical correction. Conclusion. Stenting of DA can be an effective alternative to primary surgical correction in selected patients with duct-dependent CHD

Proteinuria in frasier syndrome

Introduction. Frasier syndrome (FS) is a genetic form of glomerulopathy, which results from mutations in the Wilms’ tumour suppressor gene (WT1). Proteinuria in FS has been traditionally considered unresponsive to any medication and FS inevitably progresses to end stage renal failure. Case Outline. We present a patient with FS who had atypical clinical manifestation and unusual beneficial antiproteinuric response to renin-angiotensin system (RAS) inhibitors given in combination with indomethacin. After 13 years of follow-up, the patient is now 17-year old with normal renal functions and no proteinuria. Conclusion. RAS inhibitors combined with indomethacin showed beneficial effect in our patient. Thus, this combination might be the initial treatment of patients with FS. If this treatment strategy was not satisfied for at least 3 months, then CsA would be considered to be administered taking account of the nephrotoxicity and the increased risk of malignancy. Further prospective study is required to clarify this issue. [Projekat Ministarstva nauke Republike Srbije, br. OI175079

Oral propranolol for infantile hemangiomas: a prospective study on the role of 48-hour Holter monitoring in additional safety assessment

Purpose: Oral propranolol has been recently approved for infantile hemangiomas (IHs), but potential side effects stay a challenge. We sought to make an additional assessment on oral propranolol safety for this indication. Materials and methods: Prospective study included 108 infants consecutively treated for IHs at the University Children’s Hospital Tirsova, Belgrade from January 2010 to December 2013. Propranolol was administered orally at a daily dose of 0.5 mg/kg and doubled every 48 hours in the absence of side effects until reaching the maximum dose of 2 mg/kg daily. Systolic and diastolic blood pressure and heart rate were measured every 48 hours with clinical observation. Heart rate was monitored by standard electrocardiogram (ECG) and 48-hour Holter ECG. Results: Statistically significant, but asymptomatic decreases in systolic blood pressure and heart rate recorded by Holter ECG were observed during the first doubling of dose and then remained stable. Arrhythmias were not detected. Despite mild sleep disturbance observed in 31% of infants in the hospital milieu, Holter monitoring indicated circadian rhythm maintenance. Conclusions: Oral propranolol for IHs does not remarkably affect heart rhythm including circadian variations throughout hospital initiation. Therefore, there is no necessity for Holter monitoring in additional safety assessment

Transcatheter closure of patent ductus arteriosus using Flipper coil and Amplatzer Duct Occluder: Ten-year experience from a single center

Introduction/Objective. Transcatheter closure is a well-established procedure for treatment of patent ductus arteriosus (PDA). We aimed to make a comparison between transcatheter PDA occlusion with Flipper coil and Amplatzer Duct Occluder (ADO) and to determine the incidence and significance of procedural complications. Methods. Between November 2004 and October 2014, 148 patients were eligible for transcatheter PDA closure at the University Children’s Hospital in Belgrade, Serbia. The median age was 5.9 years (the range of 0.9 years to 17.3 years) and the median weight was 21 kg (the range of 8.8 kg to 94 kg). Follow-up evaluations with Doppler echocardiogram were performed at one day, three months, and one and two years after the PDA occlusion. Results. Median narrowest PDA diameter was 1.5 mm (the range of 0.5 mm to 5.6 mm). Flipper coil was used for PDA closure in 84 (59.2%) and ADO in 58 patients (40.8%). There was no significant difference in the rate of immediate complete closure between the coil and the ADO group (86.9% vs. 75.9%, p = 0.089), but a significantly higher rate of complete closure was achieved with ADO at one day (83.3% vs. 98.3%, p = 0.004), three months (85.7% vs. 100%, p = 0.002), and both one and two years after the implantation (91.7% vs. 100%, p = 0.041). In total, 12 complications occurred during the procedure, seven of which with coil and five with ADO occlusion of PDA. Conclusion. Transcatheter closure of PDA using both coils and ADOs is a very safe and effective procedure. ADO proved superior to coil in terms of complete closure rate as early as one day after the procedure

The role of modern imaging techniques in the diagnosis of malposition of the branch pulmonary arteries and possible association with microdeletion 22q11.2

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The "lesser form" is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the branch pulmonary arteries is often associated with other congenital heart defects and extracardiac anomalies, as well as with 22q11.2 microdeletion. We report three infants with crossed pulmonary arteries and one adolescent with "lesser form" of the malformation. The results suggest that diagnosis of malposition of the branch pulmonary arteries could be challenging if based solely on echocardiography, whereas modern imaging technologies such as contrast computed tomography and magnetic resonance angiography provide reliable establishment of diagnosis. In addition, we performed the first molecular characterisation of the 22q11.2 region among patients with malposition of the branch pulmonary arteries and revealed a 3-megabase deletion in two out of four patients

Outcomes of surgery for total anomalous pulmonary venous drainage

Introduction. Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease and in some variants represents the only true surgical emergency in congenital heart surgery. Basic anatomical characteristic of this anomaly is an abnormal connection of pulmonary veins with systemic venous circulation. Although the results of TAPVC repair in infancy have been markedly improved in recent years, the recurrent pulmonary venous obstruction (RPVO) remains relatively frequent complication of surgical treatment. Objective. The aim of this study was a retrospective evaluation of TAPVC repair at a single institution, identifying the risk factors associated with the increased mortality and morbidity. Methods. Between January 2001 and January 2010, 43 consecutive patients underwent repair of TAPVC at the University Children’s Hospital, with median weight of 3.8 kg (1.8-13 kg). Median age at surgery varied from 5 days to 5 years. Distribution of TAPVC types was as follows: supracardiac 19 (44%), cardiac 12 (28%), infracardiac 9 (21%), and mixed 3 (7%). Eleven patients (26%) were emergencies due to obstructed drainage. Results. Early mortality was 9.30% (4/43). An average time of followup/survival for 95% interval of confidence was 101.6±6.7 months. Kaplan-Meier cumulative survival was 83.7±5.7%. Freedom from reintervention after 10 years was 87.2±0.5%. The principal reason for reintervention was RPVO. Conclusion. Preoperative obstruction is not a risk factor of early mortality and RPVO. Low body mass (below 2.5 kg) is the only identified risk factor of early mortality. Complex morphology of the confluens, particularly in a mixed type of TAPVR, is the main risk factor of RPVO development

The role of two-dimensional echocardiography in diagnostics of coarctation of the aorta in newborns

Introduction. Diagnosis of neonatal coarctation of the aorta (CoA) still presents a challenge in routine practice because of absence of reliable morphologic and functional parameters for early detection of this congenital heart defect in newborns. Objective. The aim of this study is to identify easy obtainable two-dimensional echocardiographic parameters for detection of the CoA in newborns. Methods. Echocardiographic evaluation was performed in 30 newborns with CoA and 20 healthy neonates (control group). Measurements of the proximal transverse arch (PTA), distal transverse arch (DTA), isthmus, distance between the left common carotid artery (LCCA) at the origin of the left subclavian artery (LSA), were obtained by two-dimensional echocardiography. Aortic arch hypoplasia was defined using Mouleart, Karl and Mee criteria, and Z-value. Index 1 was calculated as a ratio of DTA and distance between origins LCCA-LSA, Index 2 was calculated as a ratio of the ascending aorta and the distance between LCCA-LSA origins, and Index 3 was calculated as a ratio of PTA and distance between LCCA-LSA origins. Results. Index 1 was significantly lower in patients with CoA in comparison with control group (0.50 vs. 1.39; p≤0.01). A cutoff point at 0.39, for Index 1, showed a sensitivity of 92% and specificity of 99% for the diagnosis of neonatal CoA, while cut off points at 0.69 and 0.44, for Index 2 and Index 3, showed the highest sensitivity and specificity for the diagnosis of CoA in newborns. Conclusion. By using these echo indexes, two-dimensional echocardiographic aortic arch measurement becomes a simple, reliable noninvasive method for the evaluation of aortic coarctation in newborns and may lead to earlier diagnosis and subsequent surgical correction