Pediatric Cardiology Centers Research Articles

Closure of patent ductus arteriosus in children, small infants, and premature babies with Amplatzer duct occluder II additional sizes: Multicenter study

To evaluate safety and efficacy of closure of patent ductus arteriosus (PDA) with Amplatzer duct occluder II Additional Sizes (ADO II AS) and to report early and midterm results of the device in children and very young symptomatic infants. Retrospective analysis of angiographic data of 60 children from four pediatric cardiology centers. The median patient age and weight were 6.5 (0.5-168) months and 6.8 (1.19-57) kg, respectively. In the study, 26 children had a body weight of ≤ 6 kg. Of these 26 children, 9 had a body weight of ≤ 3 kg. The median narrowest diameter of PDA was 2 (1.2-4) mm. Ductal anatomy was Type A in 29, Type B in 2, Type C in 11, Type D in 1, and Type E in 16 patients, and a residual PDA after surgery in 1 patient. Closure with ADO II AS was achieved in 58 (96.6%) of 60 attempted cases. In two infants, the device was not released because of significant residual shunt. ADO II was used in one, and the other was sent to surgery. Complete closure was observed in all ADO II AS deployed children by the next day on echocardiography. Median follow-up was 12 (1-18) months. Neither death nor any major complications occurred. Our study shows that closure of medium and small sized PDA by using ADO II AS device is effective and safe in children. The use of the device will expand the field of application of PDA closure in small infants.

Report Report from the 12th Conference of the Pediatric Cardiology Section of the Polish Cardiac Society (Zabrze 7–8.06.2013) – “Progress in interventional treatment of congenital heart diseases”

Report Report from the 12th Conference of the Pediatric Cardiology Section of the Polish Cardiac Society (Zabrze 7–8.06.2013) – “Progress in interventional treatment of congenital heart diseases”

Clinical Characteristics and Prognostic Factors of Primary Pulmonary Vein Stenosis or Atresia in Children

Primary pulmonary vein stenosis or atresia (PVS/A) is a rare entity with a high mortality rate. The aim of this study was to elucidate the clinical characteristics, progression, and prognostic factors of primary PVS/A in children. We reviewed the medical records of patients who had primary PVS/A with normally connected pulmonary veins (PVs) at five pediatric cardiology centers in Korea between 1995 and 2010. A total of 34 cases were identified. The median age at diagnosis was 12.0 months. During the follow-up period (median, 18 months; range, 2 to 185 months), PVS/A progressed to previously uninvolved veins in 9 patients. Surgical interventions were performed on 29 patients (venoplasty on 25 and pneumonectomy on 4). Nineteen of the patients who underwent venoplasty had restenosis after a median of 2 months. The sutureless technique did not reduce the rate of restenosis, progression of the disease to previously uninvolved PVs, or mortality rate. The mortality rate was 46.7%, the median age of death was 10.8 months, and the median interval between diagnosis and death was 3.0 months. In univariate analysis, predictors of death included involvement of at least three PVs, bilateral PV involvement, infancy-onset PVS/A, restenosis after surgery, and progression to previously uninvolved PVs. In multivariate analysis, significant risk factors for death were involvement of at least three PVs (hazard ratio, 8.8; p < 0.0001) and progression to uninvolved PVs (hazard ratio, 4.2; p = 0.014). Primary PVS/A may carry a significant risk of recurrent and progressive PV obstruction or death even after surgical venoplasty.

Congenital Heart Defects in Adults : A Field Guide for Cardiologists

Advances in cardiology and cardiac surgery allow a large proportion of patients with congenital heart defects to survive into adulthood. These patients frequently develop complications characteristic of the defect or its treatment. Consequently, adult cardiologists participating in the care of these patients need a working knowledge of the more common defects. Occasionally, patients with congenital heart defects such as atrial septal defect, Ebstein anomaly or physiologically corrected transposition of the great arteries present for the first time in adulthood. More often patients previously treated in pediatric cardiology centers have transitioned to adult congenital heart disease centers for ongoing care. Some of the more important defects in this category are tetralogy of Fallot, transposition of the great arteries, functionally single ventricle defects, and coarctation. Through this field guide, we provide an overview of the anatomy of selected defects commonly seen in an adult congenital practice using pathology specimens and clinical imaging studies. In addition, we describe the physiology, clinical presentation to the adult cardiologist, possible complications, treatment options, and outcomes.

Válvula aórtica quadricúspide – Casuística de 10 anos e revisão da literatura

Introduction Quadricuspid aortic valve is a rare malformation, with an estimated incidence of 0.003 to 0.043% of all congenital heart disease. It usually appears as an isolated congenital anomaly, but may also be associated with other malformations, the most common being coronary artery anomalies. Current technology enables noninvasive diagnosis in most cases. This entity's natural history is progression to valve regurgitation, which is rare before adulthood. Objective Case review of quadricuspid aortic valve patients diagnosed in the last 10 years in a tertiary pediatric cardiology center. Methods Retrospective chart review of patients diagnosed with quadricuspid aortic valve between January 2000 and December 2009. Results Over the past 10 years, four cases of quadricuspid aortic valve were diagnosed in children aged between 6 months and 8 years, two male. In three cases, the four leaflets were of similar size, which is the most common finding. Two of the valves functioned normally and two had minimal regurgitation. All patients had associated cardiac malformations (one atrial and two ventricular septal defects, one supravalvular aortic stenosis and one quadricuspid pulmonary valve). One patient was also diagnosed with Williams syndrome. During a median follow-up of 2 years (0-9), all patients remained asymptomatic and none required medical or surgical treatment of the aortic valve. Conclusion Diagnosis of quadricuspid aortic valve is rare, especially in children, since most patients are asymptomatic and have normally functioning valves. In this study, half the patients had minimal aortic regurgitation. Contrary to what is described in the literature, all patients had concomitant cardiac malformations. We provide the first description of this entity's association with Williams syndrome. Clinical follow-up should be maintained in these patients in order to promptly detect the onset or worsening of functional alterations and to enable appropriate therapeutic intervention.

Quadricuspid aortic valve – 10-year case series and literature review

IntroductionQuadricuspid aortic valve is a rare malformation, with an estimated incidence of 0.003 to 0.043% of all congenital heart disease. It usually appears as an isolated congenital anomaly, but may also be associated with other malformations, the most common being coronary artery anomalies. Current technology enables noninvasive diagnosis in most cases. This entity's natural history is progression to valve regurgitation, which is rare before adulthood. ObjectiveCase review of quadricuspid aortic valve patients diagnosed in the last 10 years in a tertiary pediatric cardiology center. MethodsRetrospective chart review of patients diagnosed with quadricuspid aortic valve between January 2000 and December 2009. ResultsOver the past 10 years, four cases of quadricuspid aortic valve were diagnosed in children aged between 6 months and 8 years, two male. In three cases, the four leaflets were of similar size, which is the most common finding. Two of the valves functioned normally and two had minimal regurgitation. All patients had associated cardiac malformations (one atrial and two ventricular septal defects, one supravalvular aortic stenosis and one quadricuspid pulmonary valve). One patient was also diagnosed with Williams syndrome. During a median follow-up of 2 years (0-9), all patients remained asymptomatic and none required medical or surgical treatment of the aortic valve. ConclusionDiagnosis of quadricuspid aortic valve is rare, especially in children, since most patients are asymptomatic and have normally functioning valves. In this study, half the patients had minimal aortic regurgitation. Contrary to what is described in the literature, all patients had concomitant cardiac malformations. We provide the first description of this entity's association with Williams syndrome. Clinical follow-up should be maintained in these patients in order to promptly detect the onset or worsening of functional alterations and to enable appropriate therapeutic intervention.

Overestimating Neuroprotection in Congenital Heart Disease: Problems With Bayley III Outcomes

Major advances in the care of children with congenital heart disease include prenatal diagnosis, prompt and often prenatal referral to pediatric cardiology centers of excellence, early stabilization of abnormal physiology with single-stage corrective repair before 6 weeks of age, and attention to medical and environmental stressors that affect the vulnerability of a child's immature central nervous system.1,–,3 In this issue of Pediatrics , Acton et al4 describe the developmental outcomes of surviving children from a large prospective Canadian cohort after early congenital surgical heart repair at a regional center of excellence. The children had surgery at a mean age of 14 days and underwent assessments at a mean age of 21 months by using 2 versions of the well-standardized Bayley scales (Bayley II and III).5,6 These scales were used to assess gross and fine motor skills and verbal and nonverbal cognitive performance during structured evaluations. First, among these critically ill … Address correspondence to Michael E. Msall, MD, Kennedy Research Center on Intellectual and Developmental Disabilities, Section of Developmental and Behavioral Pediatrics, University of Chicago Comer Children's Hospital, 950 E 61st St, SSC Room 207, Chicago, IL 60637. E-mail: mmsall{at}peds.bsd.uchicago.edu

046 Safety of Endomyocardial Biopsy in Two Pediatric Non-Surgical Centers Using the New Atc Bioptome with Variable Flexibility

BackgroundRight ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation.Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA).MethodsHealth records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination.ResultsThere was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome.ConclusionWe conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers. BackgroundRight ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation.Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA). Right ventricular (RV) endomyocardial biopsy (EMB) is the “gold standard” for surveillance and acute detection of rejection following cardiac transplantation. Several bioptomes are in use. This study examined the safety of EMB in two pediatric non-surgical cardiac centers using the new Sparrowhawk bioptome with variable flexibility (ATC Technologies, Woburn, MA, USA). MethodsHealth records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination. Health records of all the EMB performed in two non-surgical Pediatric Cardiology Centers were reviewed: Variety Children's Heart Center, Winnipeg MB, and The Alberta Children's Hospital, Calgary AB. From October 2005 to January 2011, 79 RV EMB were performed in 33 children. The main indication for biopsy was routine surveillance for rejection following heart transplantation (n = 30) and the rest as part of cardiomyopathy work-up (n = 3). All procedures were performed under general anesthesia using custom pre-shaped guiding sheaths. The median age of the patients was 8 years (0.25-18 years) and median weight 22 kg (4.4-72.3Kg). Ten procedures were performed on children under 1 year of age. Femoral vein (FV) approach was used in most of our cases and internal jugular vein in bilateral FV occlusion. Four to five EMB specimens were obtained at each session, suitable for pathological examination. ResultsThere was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome. There was no death, vascular damage or anesthetic complication. Only a small pericardial effusion in a 12 months old baby occured after the last biopsy. This complication did not require further intervention and it occurred during our early experience. There was no mechanical malfunction of the bioptome. ConclusionWe conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers. We conclude that the EMB by using the new Sparrowhawk bioptome is safe in the infants & children and can be performed in cardiac non-surgical centers.

Big right ventricle and coarctation of aorta in newborn infants

BackgroundDiagnosis of coarctation can be notoriously difficult. In the presence patent ductus arteriosus (PDA), classical signs of coarctation may not be seen on the echocardiogram. Big right ventricle should arouse...

Impact of acute cardiac transfers conducted by the west midlands neonatal transfer service

IntroductionCongenital heart disease is the commonest group of congenital malformations and accounts for approximately 10% of infant deaths.1 Affected infants need to be transferred to paediatric cardiology centres. The West...

Urođene anomalije srca u djece sa Down sindromom koja se prate u pedijatrijskim kardiološkim centrima u Bosni i Hercegovini

Objective - The research was undertaken with the aim of establishing the range and frequency of congenital heart disease (CHD) in children with Down syndrome (DS), who are monitored at paediatric cardiology centres in Bosnia and Herzegovina, its association with other anomalies, the time of diagnosis of DS and CHD, and to analyse their surgical care. Patients and methods - By a cross-sectional study, data was collected on children aged from 0 to 18 years with DS and CHD who were monitored at paediatric cardiology centres of primary, secondary and tertiary health care (Banja Luka, Bihać, Bijeljina, Mostar, Sarajevo, Tuzla and Zenica) in Bosnia and Herzegovina. Results - Data for 100 children (51 boys and 49 girls) of an average age of 6.1 (from 0 to 17.1) were collected and analysed. The most frequently diagnosed CHD was atrioventricular septal defect, which was found in 36% of children followed by ventricular septal defect in 33%. In 79% children the CHD was isolated and 21% had multiple heart anomalies. Associated anomalies of other systems were found in 26% of children. In only one case was an intrauterine suspicion of DS established. A statistically significant reduction in the age of post-natal diagnosis of DS and CNHD was registered. In 73% patients cardiosurgical treatment was indicated, 43% underwent surgery, 19% are waiting for surgery and in 11% pulmonary hypertension developed. Conclusion - The range of CHD found in children with DS in Bosnia and Herzegovina does not differ from most similar research. The results obtained show that in the period in question there was insufficient prenatal diagnostics of DS and CHD. Postnatal diagnostics, although also insufficient at the beginning of the period in question, significantly improved over time, which resulted in a reduction of the age at which the diagnosis of DS and CHD was established. Moreover there was a clear reduction in the time difference between the diagnosis of DS and the diagnosis of CHD. However, the possibilities of early cardio-surgical treatment are still limited. The results should serve in drawing up a plan of prospective monitoring of these patients in Bosnia and Herzegovina in order to define better their specific health needs.

Variation in Anticoagulation Prophylaxis Practice After Fontan: A Call for a National Fontan Registry

To the Editor, We read with great interest the recent publication by Anderson et al. [1] on the inconsistencies in medication therapy among Fontan patients across seven pediatric heart networks in the United States. The practice in the United Kingdom appears to reflect a similar treatment dilemma. We conducted a survey of consultant pediatric cardiologists, with 20 respondents representing 13 different pediatric cardiology centers in the United Kingdom. The main variation in practice was that 80% of respondent paediatric cardiologists prescribed long-term aspirin after superior cavopulmonary anastomosis and 20% preferred not to use any treatment. In the first 6 months after completion of Fontan, warfarin was prescribed by 74% of respondents and aspirin by the remainder. However, beyond 6 months after Fontan, all the respondents provided anticoagulation prophylaxis, with 42% prescribing aspirin compared with 58% using warfarin. The issue of prophylactic anticoagulation for patients palliated with Fontan circulation is a recurring theme [2]. Compelling data show that thrombotic events are a major cause of mortality among patients after Fontan surgery [3]. Although the pathophysiology of thromboembolism in Fontan patients still is not clearly defined and we cannot reliably risk stratify each patient, the pharmacologic options for reducing thromboembolism do vary and have their own inherent risks. In the case of warfarin, a young person’s lifestyle choices also may be affected. We are awaiting data from the study on the efficacy of aspirin compared with warfarin anticoagulation in reducing the thromboembolic risk for Fontan patients [4]. This study has the advantage of primarily including patients with lateral tunnel or extracardiac total cavopulmonary anastamosis and therefore is more relevant to the current era of surgical outcomes. However, this study may not adequately quantify the safety profile of long-term aspirin or warfarin nor is it likely to assess the impact on lifestyle and the psychosocial burden on children and young adults. We envisage that the decision making may become more complex with the newer generation of anticoagulants (direct thrombin inhibitors and direct factor Xa inhibitors), which are not as yet licensed for children. Their use may begin sporadically because they require little or no monitoring. Conducting a large well-designed multicenter prospective trial will provide more robust data but may not address every concern for Fontan patients. In the United Kingdom, setting up such a trial would be a challenge considering the small number of patients (151 survivors of Fontan surgery from 2008 to 2009 in the United Kingdom) [5] and the heterogeneity of the underlying cardiac morphology. We believe that establishing a UK Fontan registry will augment data from trials, improve understanding, allow comparison of variations in practice, and thus guide our future practice.

307 Long term results of chirurgical repair of aortic coarctation in Tunisia

Coarctation of the aorta (CoA) is a stenosis usually located in the isthmus of the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and may present excellent immediate results. However, despite immediate good results, significant residual problems often persist. The aim of the study is to describe the presentation, treatment and long-term evolution of a population of 48 unselected consecutive patients with CoA in a single pediatric cardiology center. This was a retrospective study of all patients with isolated CoA associated or not to either atrial or ventricular septal defects. RESULTS: The patients (n = 48, 56,3% male) were diagnosed at a mean age of 84 ± 109 months. The clinical presentation differed between patients aged less or more than two years, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (p < 0.01). Treatment was surgical in all cases (32 end-to-end anastomosis). The mean age of patients was 94 ± 109 months. There was two late deaths, in a mean follow-up of 8,6 ± 7,7 years. Recoarctation occurred in 12 patients (25%). There are patients who currently have hypertension (17 at rest, 2 with effort), their mean age at diagnosis being older than the others (128 vs. 76 months; p < 0.05). Aortic aneurysms occurred in five patients (10,4%). Aneurysm was associated to bicuspid aortic valve in 3 cases. repaired CoA has a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment.

624 Neonatal Congenital Heart Defects: Clinical Status From Birth to Arrival at the Irish National Cardiac Centre

Aims: To evaluate immediate postnatal care of infants with congenital heart disease including postnatal care and transfer to the cardiac unit, and identify areas of weakness in service provision. Methods: All infants less than 6 weeks of age admitted to the national paediatric cardiology centre were included. Preadmission and admission data were recorded as well as results of subsequent investigations. Preterm infants transferred for closure of patent ductus arteriosus were excluded. Results: The majority of patients were admitted outside of routine hours. Most infants on prostaglandin were on low dose prostaglandin. In non-intubated infants on prostaglandin 8 had minor apnoea. No infants on prostaglandin required intubation due to apnoea during transfer (Table 1). Conclusions: The neonatal transport team operates during business hours and therefore service extension is necessary to meet the population's needs. No infants were intubated during transfer on low dose prostaglandin. This supports a local policy of not electively intubating all neonates with cardiac disease who require prostaglandin to maintain ductal patency.

1132 Model of Cardiology Service in District General Hospital

Background: Various models of cardiology service are available in district general hospitals (DGH) in UK, like Joint cardiology clinics, Paediatric telecardiology, local paediatricians with expertise in cardiology (PEC) and cardiac technicians. Objective: To assess the Paediatric Cardiology Services provided by local PEC at Frimley Park Hospital by combining good clinical evaluation, echocardiography skills and support of tertiary paediatric cardiology centre in the absence of telemedicine. PEC attends once a week cardiology clinic at tertiary centre as honorary consultants. Design and Methods: Retrospective analysis of all the referrals to tertiary paediatric cardiology centre between October 2008 to February 2010. Data from the Proforma was collated in Excel and was analysed using simple statistical methods and percentage calculations. Results: 80/92 referrals were analysed. The reasons for referral were: clinical 82.5%, family history 5%, and rhythm disturbances 11%. Acyanotic congenital heart disease (CHD) was diagnosed in 64% (of which 64% left to right, 29.4% obstructive lesions and 8% regurgitant lesions) and 8.75% of cases were diagnosed as cyanotic CHD. All cyanotic CHD were picked up immediately after birth. 10% were found to have rhythm disturbances (SVT, WPW). 2.5% of cases were classed as life threatening. 20% required interventions and 12.5% of the referral were normal and discharged. Conclusions: Cardiology service provided by PEC with support from tertiary cardiology centre is safe and effective even in the absence of telemedicine. Weekly access to tertiary paediatric cardiology service by PEC provides continuous medical education, constructive feedback and opportunity to discuss non-urgent cases thereby avoiding unnecessary referrals.

Benefits of an International Working Exchange in Pediatric Cardiology

This report describes a 1-year exchange between members of two pediatric cardiology centers: one in Canada and one in Australia. Five cardiologists participated in sequence, fully engaging in the activities of the host department. The motivation of the exchange was broadly educational including clinical experience, shared expertise, teaching, and research collaboration. Structured debriefing confirmed the value of the exchange. In addition to the experience of working in a different medical system, eight research papers were developed, with two research projects ongoing as well as subsequent exchanges of nursing and technical personnel. Interchange between two academic departments can add strength to both and allow development of new skills and research activity.

Outcome of Pediatric Patients With Pulmonary Arterial Hypertension in the Era of New Medical Therapies

Little is known about the effects of "second-generation drugs" (prostanoids, endothelin receptor antagonists, 5-phosphodiesterase inhibitors) in children with pulmonary arterial hypertension (PAH). This study describes the outcome of a national cohort of children with PAH in an era when these drugs became available. From 1993 to 2008, 52 consecutive children with idiopathic PAH (n = 29) or systemic-to-pulmonary shunt-associated PAH (n = 23) underwent baseline and follow-up assessments. Treatment was initiated depending on functional class, acute pulmonary vasoreactivity response, and drug availability. Observed survival was evaluated depending on time of diagnosis in relation to second-generation drug availability and subsequently compared to calculated predicted survival. Children for whom second-generation drugs were available had improved survival compared to their predicted survival (1-, 3-, and 5-year survival rates 93%, 83%, and 66% vs 79%, 61%, and 50%, respectively). However, this improved survival was observed only in patients for whom second-generation drugs became available during their disease course. No improved survival was observed in patients for whom drugs were available already at diagnosis. Baseline variables associated with decreased survival included higher functional class, higher pulmonary-to-systemic arterial pressure ratio, lower cardiac index, and higher serum levels of N-terminal pro-brain natriuretic peptide and uric acid. After start of second-generation drugs, functional class, 6-minute walking distance, and N-terminal pro-brain natriuretic peptide improved but gradually decreased after longer follow-up. In conclusion, survival of pediatric PAH seemed improved since the introduction of second-generation drugs only in selected patients for whom these drugs became available during their disease course. Start of second-generation drugs initially induced clinical improvements, but these effects decreased after longer follow-up.

Lead removal in young patients in view of lifelong pacing

In young patients with or without a congenital heart disease, transvenous leads for pacemakers or implantable cardioverter defibrillators can cause later vascular obstruction or infection. Removal of non-functional leads is controversial as it bears the risk of vascular disrupture and embolizations. We report the data of a single centre for paediatric cardiology on efficiency and safety of transvenous lead removal. Between May 2005 and August 2009 in 22 patients with a mean age of 12.9 years (range: 3.6-29.5 years) removal of 28 transvenous leads (mean lead age: 5.1 years) was attempted. The main indications for removal were vascular obstruction, increased threshold, and lead dislocation. Commercially available retraction tools were used, if necessary. Twenty-five leads (89%) were retrieved with clinical success, of which 22 (79%) were removed with complete procedural success. In three leads the lead tips were retained, while three leads could not be retrieved. No major complications occurred. Additional interventions such as recanalization, balloon dilation, or stent implantation were performed as indicated. Procedure and X-ray times could be correlated to the implant age of the leads. Using non-electrical techniques, transvenous lead removal can be performed with a success rate of 89% in young patients. In the case of vessel obstructions, lead replacement combined with revascularization should be performed early, as the older the lead, the more prolonged and more hazardous the extraction procedure becomes. The use of new leads and precautionary implantation techniques may facilitate later lead removal.

Spectrum of congenital heart defects in Croatia

The aim of our study was to investigate the incidence of congenital defects in children born in Croatia during a period of 5 years, its association with extracardiac malformations, its treatment, and outcome. Medical information about the patients was obtained from 14 paediatric cardiology centres that cover the whole country. Diagnosis was made by clinical findings, electrocardiography, chest X-ray, echocardiography, catheterisation, or autopsy. Between October 1, 2002 and October 1, 2007, there were 205,051 live births in Croatia, 1,480 of which were patients diagnosed with congenital heart disease, accounting for 0.72% of the live-born children. The distribution was made up of 34.6% children with ventricular septal defect, 15.9% with atrial septal defect, 9.8% with patency of arterial duct, 4.9% with pulmonary valvar stenosis, 3.3% with tetralogy of Fallot, 3.3% with transposed great arteries, 3.3% with aortic stenosis, 3.2% with aortic coarctation, 4.3% with atrioventricular septal defect and common atrioventricular orifice, 2.3% with hypoplastic left heart syndrome, and 8.3% other with severe defects. The average age in the time of diagnoses is 70.41 days (SD, 188.13), with low average time of diagnoses of severe heart defects, 9.6 days (SD, 32.52). Among patients, 14.5% had chromosomal defects, syndromes, and/or other congenital major anomalies. During the study, 57 patients died because of cardiac anomalies or other related problems, 24 who died were operated. The rates of specific cardiac defects and association with extracardiac malformations are generally comparable with those reported in similar studies. In spite of all problems, mortality rate of 3.85% is low but could be improved.

Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study

Objective:To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood.Design and setting:Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and...