13 research outputs found
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 infantās
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 Wilmsā 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 Childrenās Hospital Tirsova, Belgrade from January 2010 to December 2013. Propranolol was administered orally at a daily dose of 0.5āmg/kg and doubled every 48āhours in the absence of side effects until reaching the maximum dose of 2āmg/kg daily. Systolic and diastolic blood pressure and heart rate were measured every 48āhours 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 Childrenās 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 Childrenās 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 101.6Ā±6.7 months. Kaplan-Meier cumulative survival was 83.7Ā±5.7%.
Freedom from reintervention after 10 years was 87.2Ā±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; pā¤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