Caroline Ovaert

Outcome of acute fulminant myocarditis in children

Objectives: To highlight clinical features and outcome of acute fulminant myocarditis (AFM) in children. Methods: Diagnostic criteria were (1) the presence of severe and acute heart failure; (2) left ventricular dysfunction on echocardiography; (3) recent history of viral illness; and (4) no history of cardiomyopathy. Results: Eleven children were included between 1998 and 2003, at a median age of 1 (0 to 9) year. Their mean left ventricular ejection fraction (LVEF) was 22 (SD 9)% at presentation. A virus was identified in five patients: human parvovirus B19 (n u200a=u200a 2), Epstein–Barr (n u200a=u200a 1), varicella zoster (n u200a=u200a 1), and coxsackie (nu200a=u200a1). The median intensive care unit course was 13 (2–34) days. Intravenous inotropic support was required by nine patients and eight were mechanically ventilated. All patients received corticosteroid, associated with intravenous immunoglobulin in seven. Five patients experienced cardiocirculatory arrest that was successfully resuscitated in four. At a median follow up of 58.7 (33.8–83.1) months, the 10 survivors are asymptomatic with normalised LVEF. Conclusion: Despite a severe presentation, the outcome of AFM is favourable. Aggressive symptomatic management is warranted and heart transplantation should be considered only when maximal supportive therapy does not lead to improvement.

Growth of the right ventricle after successful transcatheter pulmonary valvotomy in neonates and infants with pulmonary atresia and intact ventricular septum

OBJECTIVESnSince 1990, transcatheter pulmonary valvotomy has become an alternative to surgical valvotomy in the management of neonates and infants with pulmonary atresia and intact ventricular septum. We sought to determine whether right ventricular growth after transcatheter pulmonary valvotomy is commensurate with body growth.nnnMETHODSnLaser or radiofrequency-assisted balloon valvotomy was attempted in 12 neonates and infants with pulmonary atresia and intact ventricular septum. Tricuspid and mitral valve dimensions were measured retrospectively on the cross-sectional echocardiograms performed before the procedure and during follow-up. Z-values were used to standardize tricuspid valve dimensions with body size.nnnRESULTSnThe atretic pulmonary valve was successfully perforated and dilated in nine of 12 patients. Five of these nine patients required additional transcatheter or surgical procedures to augment the pulmonary blood flow. Of six survivors, five are regularly followed up with a median follow-up of 60 months (range 37 to 68 months). All five have two-ventricle circulation, two of the five patients requiring surgical enlargement of the right ventricular outflow tract with or without closure of the atrial septal defect. Echocardiographic tricuspid valve dimensions and Z-values before transcatheter valvotomy tended to be smaller in the patients who died than in the survivors. In the survivors, the absolute tricuspid valve dimensions increased after valvotomy but the Z-values tended to decrease or stayed constant.nnnCONCLUSIONSnTranscatheter valvotomy is a good alternative to surgical valvotomy in patients with pulmonary atresia and intact ventricular septum. Two-ventricle circulation can be achieved despite subnormal right ventricular growth.

Transcatheter treatment of coarctation of the aorta: a review.

Coarctation of the aorta (CoA), is a discrete stenosis in the proximal thoracic aorta [55], first described by Morgagni at autopsy in 1760 [2]. Although at first glance a straightforward obstructive lesion, the anatomy, physiology, clinical presentation, and natural history vary substantially and have challenged the ingenuity of the cardiologist and surgeon with diagnostic dilemmas and management controversies. The first surgical repair was performed by Crafoord in 1944 [32]. Since that description was published, a plethora of surgical techniques has been described, each building upon the other to improve immediate results and long-term outcomes. The pivotal role played by arterial duct tissue in the pathogenesis of CoA in the newborn was underscored [54, 105], when in the 1970s, prostaglandin E1 administration was added to the management armamentarium for neonates, further contributing to improved survival rates. In 1982, transcatheter balloon dilation was first described as a potential alternative to surgery [147]. Since that time, the literature has become replete with studies of surgical and balloon dilation results and attempted comparisons between techniques. Despite more than 5 decades of experience with numerous treatment modalities of this seemingly ‘‘simple’’ lesion, there continues to be considerable discussion on what is the ‘‘best’’ therapeutic approach for the pediatric and adult patient. The flames of such controversies are fanned not only by the interpretation of immediate and medium-term hemodynamic results and procedural risks, but also by the way the treatment, modulated by its ‘‘natural’’ history [43, 44, 69, 76, 77, 79], affects long-term results, and most significantly, by personal bias.

Ross operation in the adult: long-term outcomes after root replacement and inclusion techniques.

BACKGROUNDnDilatation of the pulmonary autograft is a major concern after root replacement for the Ross operation. The inclusion technique would avoid this drawback, but few data are available on the long-term results of this technique. We retrospectively analyze long-term results of both techniques.nnnMETHODSnOf 218 patients undergoing the Ross operation between 1991 and 2006, 148 (68%) had root replacement and 70 (32%) underwent the inclusion technique. The mean age of the patients was 40 +/- 10 years (range, 16 to 64). Mean follow-up was 94 +/- 44 months (range, 13 to 196). Echocardiographic controls were available in 197 patients. Proximal aorta dilatation was defined as diameter > 40 mm.nnnRESULTSnIn the root and inclusion groups, 10-year overall survival was 94% +/- 4% and 97% +/- 4%, respectively. Freedom from autograft reoperation was 81% +/- 10% and 84% +/- 13%, respectively. Main cause of reoperation was autograft dilatation in the root group (13 of 16) and valve prolapse in the inclusion group (5 of 6). Freedom from proximal aorta dilatation was 57% +/- 12% and 80% +/- 15%, respectively. In the root group, dilatations (n = 48) affected systematically the autograft sinuses or sinotubular junction, whereas in the inclusion group, dilatations (n = 10) affected principally the ascending aorta (8 of 10). Freedom from severe autograft regurgitation was 86% +/- 9% and 83% +/- 13%, respectively. Root technique, follow-up length, and preoperative aortic valve regurgitation were predictors of proximal aorta dilatation.nnnCONCLUSIONSnIn the long term, both techniques showed excellent survival and similar rates of autograft failure. For root replacement, autograft dilatation was the main cause of failure. For the inclusion technique, the autograft, but not the ascending aorta, was protected against dilatation and autograft valve prolapse was the main cause of failure.

Balloon Angioplasty of Native Coarctation: Clinical Outcomes and Predictors of Success

OBJECTIVESnWe sought to investigate the clinical impact of balloon angioplasty for native coarctation of the aorta (CoA) and determine predictors of outcome.nnnBACKGROUNDnBalloon dilation of native CoA remains controversial and more information on its long-term impact is required.nnnMETHODSnHemodynamic, angiographic and follow-up data on 69 children who underwent balloon angioplasty of native CoA between 1988 and 1996 were reviewed. Stretch, recoil and gain of CoA circumference and area were calculated and related to outcomes.nnnRESULTSnInitial systolic gradients (mean +/- SD, 31+/-12 mm Hg) fell by -74+/-27% (p < 0.001), with an increase in mean CoA diameters of 128+/-128% in the left anterior oblique and 124+/-87% in the lateral views (p < 0.001). Two deaths occurred, one at the time of the procedure and one 23 months later, both as a result of an associated cardiomyopathy. Seven patients had residual gradients of >20 mm Hg. One patient developed an aneurysm, stable in follow-up, and four patients had mild dilation at the site of the angioplasty. Freedom from reintervention was 90% at one year and 87% at five years with follow-up ranging to 8.5 years. Factors significantly associated with decreased time to reintervention included: a higher gradient before dilation, a smaller percentage change in gradient after dilation, a small transverse arch and a greater stretch and gain, but not recoil.nnnCONCLUSIONnBalloon dilation is a safe and efficient treatment of native CoA in children. Greater stretch and gain are factors significantly associated with reintervention, possibly related to altered elastic properties and vessel scarring.

Endovascular stent implantation for the management of postoperative right ventricular outflow tract obstruction: Clinical efficacy

OBJECTIVEnExtracardiac conduits between the right ventricle and pulmonary arteries commit patients to multiple reoperations. We reviewed our experience with stent implantation in obstructed conduits.nnnMETHODSnBetween 1990 and 1997, stents were implanted across 43 conduits. The median age at procedure was 6 years (0.5-17 years), and the median interval between conduit insertion and stent implantation was 2.4 years (0.3-14 years).nnnRESULTSnMean systolic right ventricular pressures and gradients, respectively, decreased from 71 +/- 18 mm Hg and 48 +/- 19 mm Hg before to 48 +/- 15 mm Hg and 19 +/- 13 mm Hg after stent placement. Mean percentage of predicted valve area for body surface area increased from 26% +/- 12% to 48% +/- 17% after stent placement. Fifteen patients underwent a second transcatheter intervention (dilation or additional stent), and 2 patients, a third, allowing further postponement of surgery in 8 patients. One sudden death occurred 2.8 years after stent placement. Surgical conduit replacement has occurred in 20 patients. Body growth was maintained during follow-up. Freedom from surgical reintervention was 86% at 1 year, 72% at 2 years, and 47% at 4 years. Higher right ventricular pressure and gradient before and after stent placement and lower percentage of predicted valve area for body surface area after stent placement were associated with shorter palliation.nnnCONCLUSIONnEndovascular stent placement across obstructed conduits is a safe and effective palliation that allows for normal body growth.

A novel CSX/NKX2-5 mutation causes autosomal-dominant AV block: are atrial fibrillation and syncopes part of the phenotype?

The prevalence of congenital heart defects is approximately 1% of all live births. Identifying the genes responsible for cardiac malformation is the first step to understand pathogenesis. Heterozygous mutations in the CSX/NKX2-5 (NKX2E) gene have been identified to cause atrial septal defect (ASD) and/or atrioventricular (AV) conduction disturbance in some families. However, there is great variability in expressivity of the phenotype between the patients with a CSX/NKX2-5 mutation.We screened four sporadic patients and three index cases of families with ASD and/or conduction defects. In one of them, a CSX/NKX2-5 mutation was identified. This novel mutation (p.Tyr256X) was inherited in a three-generation family causing five individuals to have cardiac anomalies ranging from ASD to arrhythmias. Interestingly, all the observed AV conduction disturbances were at the nodal level, manifesting first as an AV block of the first degree and evolving toward a second-degree block. Atrial fibrillation, previously reported in three individuals with CSX/NKX2-5 mutations, was observed in three patients.

Changes in Perceived Health of Children with Congenital Heart Disease After Attending a Special Sports Camp

Sports camps for children with cardiac anomalies have existed for many years. However, no formal evaluation of the benefits of attending such camps has been undertaken heretofore. We assessed potential changes in the self-perceived health of children with congenital heart disease who attended a special sports camp. Thirty-one children with cardiac anomalies attended a 3-day multisports camp. Sixteen children, all of whom were 10 years or older, literate, and Dutch- or French-speaking, completed the Child Health Questionnaire (CHQ-CF87) before and after attending the camp. The scores of the children were compared with those of healthy peers by calculating mean standardized differences. After attendance at the sports camp, the children achieved significant improvements in the self-perception of their physical functioning, role functioning due to emotional problems, role functioning due to behavioral problems, mental health, and general behavior. The children’s self-esteem and general behavior after the camp were significantly better than that of their healthy counterparts. We conclude that children with congenital heart disease who participate in activities at special sports camps may reap benefits in terms of their subjective health status. Although further research is needed, we recommend the participation in sport activities by children with heart defects, and more specifically their participation in sports camps.

Transcatheter closure of secundum atrial defect in small children

Objective: To assess safety and efficacy of transcatheter atrial septal defect (ASD) closure in small children. Background: Percutaneous closure of ASD is a well accepted alternative to surgery. Reported experience in small children remains, however, scarce. Methods: Fifty‐two children underwent percutaneous ASD closure at a weight ≤15 kg, in four Belgian tertiary referral paediatric cardiology centers. Indication for treatment, device implantation rate, procedural details, complication rate, residual shunt on echocardiography, and impact of procedure on symptoms were retrospectively analyzed. Results: Mean age at procedure was 36 months (7–60), mean weight 13 kg (4.7–15). Associated cardiac lesions were seen in 21% of the patients and noncardiac in 32.7%. Most patients (69.2%) were symptomatic. Mean ASD size was 12 mm (range 5–20 mm). A device could be inserted in 49 patients (94%). No major complications occurred. Minor complications occurred in 8 patients (15.4%) including device embolization in 2, successfully treated with transcatheter retrieval and second device insertion. Mean follow‐up reached 27 months (0.03–61.2). Residual shunt was absent, trivial, or small in 93% (n = 42) at latest follow‐up. Clinical improvement was noted in 91.7% of the symptomatic patients. Minor complications were more frequent in the presence of large ASDs (>15 mm) but not in smaller babies (<10 kg). Conclusion: Patients ≤15 kg requiring ASD closure form a special population with high incidence of associated anomalies and medical conditions. For this particular set of patients, percutaneous closure is a valid alternative. Asymptomatic or mildly symptomatic children should be treated at a later age.

Failure of right ventricular recovery of Fallot patients after pulmonary valve replacement: Delay of reoperation or surgical technique?

We read with great interest and surprise the article published by Therrien et al. in the November issue of the Journal [(1)][1]. These results are extremely disappointing. All the physicians familiar with the treatment of patients with repaired tetralogy of Fallot share the idea that one should not