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Tropical endomyocardial fibrosis (FET) is a leading cause of heart failure and the most common restrictive cardiomyopathy worldwide. Extensive fibrosis of the ventricular endocardium causing architectural distortion, impaired filling and valvular insufficiency define the disease. Confined to peculiar and limited geographical areas, the aetiology remains blurred and it carries a grim prognosis. The changing burden reported recently in some endemic areas and the refinement of diagnostic tools have emphasized alternative routes for understanding and treatment of the disease.

Background Access to cardiac surgery is limited in low-income settings, and data on patient outcomes are scarce. Aims To assess characteristics, surgical procedures and outcomes in patients undergoing open-heart surgery in low-income settings. Methods This was a cohort study (2001–2011) in two low-income countries, Cambodia and Mozambique, where cardiac surgery had been promoted by visiting non-governmental organizations. Results In Cambodia and Mozambique, respectively, 1332 and 767 consecutive patients were included; 547 (41.16%) and 385 (50.20%) were men; median age at first surgery was 11 years (interquartile range [IQR] 4–14) and 11 years (IQR 3–18); rheumatic heart disease affected 490 (36.79%) and 268 (34.94%) patients; congenital heart disease (CHD) affected 834 (62.61%) and 390 (50.85%) patients, with increasingly more CHD patients over time (P

In-plane phase-contrast (PC) imaging is now a routine component of MRI of regional blood flow in the heart and great vessels. In-plane PC MRI provides a volumetric, isotropic, time-resolved cine sequence that enables three-directional velocity encoding, a technique known as four-dimensional (4D) flow MRI. Recent advances in 4D flow MRI have shortened imaging times, while progress in big-data processing has improved dataset pre- and postprocessing, thereby increasing the feasibility of 4D flow MRI in clinical practice. Important technical issues include selection of the optimal velocity-encoding sensitivity before acquisition and preprocessing of the raw data for phase-offset corrections. Four-dimensional flow MRI provides unprecedented capabilities for comprehensive analysis of complex blood flow patterns using new visualization tools such as streamlines and velocity vectors. Retrospective multiplanar navigation enables flexible retrospective flow quantification through any plane across the volume with good accuracy. Current flow parameters include forward flow, reverse flow, regurgitation fraction, and peak velocity. Four-dimensional flow MRI also supplies advanced flow parameters of use for research, such as wall shear stress. The vigorous burgeoning of new applications indicates that 4D flow MRI is becoming an important imaging modality for cardiovascular disorders. This article reviews the main technical issues of 4D flow MRI and the different parameters provided by it and describes the main applications in cardiovascular diseases, including congenital heart disease, cardiac valvular disease, aortic disease, and pulmonary hypertension.

Tropical endomyocardial fibrosis (EMF) is a neglected disease of poverty that afflicts rural populations in tropical low-income countries, with some certain high-prevalence areas. Tropical EMF is characterized by the deposition of fibrous tissue in the endomyocardium, leading to restrictive physiology. Since the first descriptions in Uganda in 1948, high-frequency areas for EMF have included Africa, Asia, and South America. Although there is no clear consensus on a unified hypothesis, it seems likely that dietary, environmental, and infectious factors may combine in a susceptible individual to give rise to an inflammatory process leading to endomyocardial damage and scar formation. The natural history of EMF includes an active phase with recurrent flare-ups of inflammation evolving to a chronic phase leading to restrictive heart failure. In the chronic phase, biventricular involvement is the most common presentation, followed by isolated right-sided heart disease. Marked ascites out of proportion to peripheral edema usually develops as a typical feature of EMF. EMF carries a very poor prognosis. In addition to medical management of heart failure, early open heart surgery (endocardectomy and valve repair/replacement) appears to improve outcomes to some extent; however, surgery is technically challenging and not available in most endemic areas. Increased awareness among health workers and policy makers is the need of the hour for the unhindered development of efficient preventive and therapeutic strategies.

Coronary sequelae of Kawasaki disease, post-surgical coronary lesions and cardiac allograft vasculopathy are the main causes of acquired coronary pathology in childhood. Surveillance and timely recognition of coronary problems in children who are at risk of ischemic events are imperative and noninvasive imaging is increasingly utilized for these purposes. Herein, we summarize the causes of acquired coronary disease in children and discuss the role of various imaging techniques that are available to establish the diagnosis and guide management.

Acute rheumatic fever develops after an inadequate immune response to throat streptococcal infection that induces the production of antibodies reacting against cardiac endothelial cells. Valve damage may lead to irreversible cardiac valve sequela (rheumatic heart disease) with further evolution towards severe valve dysfunction and heart failure. The disease has been almost eradicated in Western countries with the development of living conditions and prevention policies, including primary prevention (treatment of sore throats) and secondary prevention (long term administration of antibiotics). However, rheumatic heart disease remains a major health problem in developing countries. Recently, echocardiography identified children with mild features of the disease, thereby allowing early treatment.

Portable echocardiography has emerged as a potential tool to detect rheumatic heart disease (RHD) early. Complex echocardiographic criteria used in recent epidemiological studies may be difficult to translate into daily practice in areas where the burden of RHD is greatest and skilled practitioners are lacking. The aim of this study was to evaluate a simplified echo approach for RHD screening among children in low-income countries.Retrospective analysis of data from a cross-sectional echocardiography-based study carried out in 2005 through the examination of 2170 school children in Maputo, Mozambique. We aimed to evaluate the value of a reference set of criteria (defined as a combination of Doppler and morphological rheumatic features of the aortic and/or mitral valves) compared with an easy-to-use single mitral regurgitation jet-length criterion (simplified set of criteria). All suspected lesions (according to reference or simplified criteria) detected in the field by a portable echo machine were reassessed by non-portable echocardiography and then read by three independent experts. Definite RHD cases in both groups were finally ascertained according to the reference criteria. Portable echocardiography detected valve regurgitation in 208 children. According to the reference criteria, 18 children were detected with suspected RHD on site. Of these, 15 children (83%) were considered to have definite RHD, giving a prevalence of 6.9 per 1000 (95% CI: 3.9-11.4). The simplified mitral regurgitation jet-length criteria detected 12 children at school, 11 of whom were subsequently confirmed to have definite RHD, giving an estimated prevalence of 5.1 per 1000 (95% CI: 2.5-9.1) (P = 0.12, exact McNemar test). When compared with the reference criteria, the simplified approach yields a maximum sensitivity of 73% for case detection, with a positive predictive value of 92%.Simplified echocardiography-based screening for RHD appears feasible, allowing rapid and appropriate detection of a significant number of RHD cases on site.

In patients with single ventricle physiology, Fontan circulation is considered as the optimal surgical approach, although it entails a growing incidence of late complications. It has been speculated that the association of bidirectional cavopulmonary shunt (BCPS) and additional pulmonary blood flow (APBF) might provide long-lasting palliation. The present study was undertaken to assess the long-term outcome of this strategy.A group of 70 patients with single ventricle physiology, who underwent BCPS with APBF between 1990 and 2000, were reviewed retrospectively. Median age at operation was 2 years (range: 0.1-27 years). Unilateral BCPS was performed in 60 patients (86%), bilateral BCPS in 9 and the Kawashima procedure in 1. APBF was provided through antegrade pulmonary outflow tract in 43 patients (61%) and by aortopulmonary shunt in 27 (39%). Two patients died early and three were lost to follow-up. Mean follow-up of the 65 remaining patients was 13.5 ± 4 years. End-points were death, need for heart transplantation (HTx) or Fontan completion and functional outcome.Five patients died (two after HTx, three from ventricular failure); overall actuarial survival was 89 ± 4% at 15 years. Six patients underwent HTx (one after Fontan completion) with two early deaths and no late mortality. Fifty-one patients underwent Fontan completion (11 with additional palliative procedures before completion); there was no early or late mortality following Fontan completion; one patient underwent HTx. Among the remaining six patients with BCPS and APBF, two were not suitable for Fontan completion and four remained suitable. Overall, clinical failure (mortality, HTx, unsuitability for Fontan completion) occurred in 13 patients (19%). Risk factors for clinical failure were older age at BCPS (P = 0.01) and postoperative complications after BCPS (P = 0.001). Considering late mortality, HTx and Fontan completion as strategic failures, the actuarial freedom from these events was 20 ± 5% at 10 years.BCPS with APBF approach: (i) fails as a strategy for definitive palliation, (ii) provides a high survival rate, (iii) does not preclude a successful Fontan completion and (iv) may delay the long-term deleterious consequences of Fontan circulation. Palliation by BCPS with APBF should be achieved early in life.

By combining exome sequencing in conjunction with genetic mapping, we have identified the first mutation in large mitochondrial ribosomal protein MRPL3 in a family of four sibs with hypertrophic cardiomyopathy, psychomotor retardation, and multiple respiratory chain deficiency. Affected sibs were compound heterozygotes for a missense MRPL3 mutation (P317R) and a large-scale deletion, inherited from the mother and the father, respectively. These mutations were shown to alter ribosome assembly and cause a mitochondrial translation deficiency in cultured skin fibroblasts resulting in an abnormal assembly of several complexes of the respiratory chain. This observation gives support to the view that exome sequencing combined with genetic mapping is a powerful approach for the identification of new genes of mitochondrial disorders. Hum Mutat 32:1225–1231, 2011. ©2011 Wiley Periodicals, Inc.

SummaryBackgroundImprovements in the treatment of children with congenital heart disease have led to most of these patients reaching adulthood. Despite the increase in lifespan, very little is known about their quality of life – in particular, their ability to obtain a mortgage or consumer loan.AimTo investigate the outcome of mortgage and loan applications made by adults with differential severities of congenital heart disease.MethodsFour hundred and seventy-six patients were invited to participate in a questionnaire-based interview by phone. Of these patients, one hundred and forty-two responded. Respondents were classified into three categories (‘significant’, ‘complex’ and ‘mild’) based on congenital heart disease severity according to the Bethesda conference.ResultsNinety patients (64%) had applied for loans; 17 (16.5%) did not report their heart disease to the insurance company, 13 were refused insurance and 39 were asked to pay surplus fees. The imposed fees concerned patients classified in the ‘significant’ and ‘complex’ groups (P

Because of their high incidence, cardiac disorders in children are now a public health issue. These children require multidisciplinary management, but surgery is sometimes unavoidable. The two most frequent types of cardiac disorder in children are congenital defects and rheumatic valve disease. La Chaine de l'Espoir (Chain of Hope) is present in more than 30 countries in Africa, Asia and the Middle East. Due to the socio-economic context and lack of technical resources, pediatric heart surgery has several particular characteristics in developing countries. Infants rarely undergo cardiac surgery, given the complexity of their management and particularly the need for intensive care. Another specificity is the need to limit treatment to reparatory or conservative methods. Surgery is rarely attempted in terminally ill patients. Costs must be kept to a minimum, and this is why we have optedfor local manufacture of mitral rings, led by Daniel Roux. After 20 years of practice we have found that regional hospitals are the smallest structures that can offer acceptable results in terms of patient care and professional training

RESUME Le tri naturel et le choix de l’efficacite limitent les malformations cardiaques dont il faut s’occuper dans les pays pauvres. La grande majorite des patients etudies ici ont, soit un shunt gauche-droite par une communication arterielle, atriale et surtout ventriculaire, soit un shunt droite-gauche par obstacle pulmonaire associe a une communication inter-ventriculaire (Tetralogie de Fallot) ou inter-auriculaire (Trilogie de Fallot), soit enfin un obstacle pulmonaire ou aortique. L’expose insiste sur la necessite d’une detection precoce des cardiopathies qui ne peut etre efficace que par une nouvelle politique de sante publique, organisee autour d’infirmiers ou de techniciens de sante, formes a l’utilisation des appareils recents peu couteux d’Echographie Doppler couleur portables. Elle precise les modalites simples de surveillance et de traitement medicaux de ces cardiopathies communes et « ren-tables »et insistesur la necessite de pouvoir disposer d’une structure (nationale ou regionale) pour offrir une possibilite de chirurgie (cœur ferme et si possible cœur ouvert) et de KT pour traiter sur placea temps et eviter les deteriorations et les transferts par les ONG dans les pays industrialises.Lefermeture chirurgicaleacœurouvertd’uneCIVchezlenourrissonetantlebut a atteindre et le test d’efficacite de nos actions de formation de ces equipes locales.

Introduction: While in developed countries prenatal diagnosis is currently used to detect Congenital Heart Disease before (CHD) before birth, in developing countries only a minority of children with CHD is detected and few benefit from surgical treatment. Having created a referral unit for diagnosis and treatment of cardiovascular diseases in a resource-deprived country we designed a study aiming at describing the spectrum and characteristics of CHD and discuss the challenges of its management. Population and methods: We studied retrospectively all patients assisted at a referral unit between 2001 and 2007, collecting epidemiological, clinical, echocardiographic and surgical data from hospital files. Results: We studied 534 patients with median age at diagnosis of 4 years (range 0-79); 296 were females (55.4%). Only 282 (52.8%) patients were diagnosed under the age of two years, and complications were present in 155 (29.0%) at time of diagnosis. We operated 196 patients with mean age of 8+/-10 years. Only 29 of the 111 complex defects were considered for surgery. The 30-days post-operative mortality was 8/196 (4.0%). The most important post-surgical complications were post pericardectomy syndrome (22). Discussion: A pattern of late presentation accompanied by high rate of complications was found. In resource-deprived settings the approach to the management of CHD emphasizes the treatment of "curable" malformations. Surgery for CHD in these settings must be linked to early detection and a referral system in which general practitioners, pediatricians, obstetricians and cardiologists interact in the design and implementation protocols for diagnosis, management and follow-up of patients. (C) 2009 Elsevier Ireland Ltd. All rights reserved.

Objective: Mid-term evaluation of an aggressive surgical management of isolated congenitally corrected transposition of the great arteries (ccTGA) by pulmonary artery (PA) bandingin early infancy.Methods:Between 2001 and 2009, 11 asymptomatic patients (seven neonates and four infants) underwenta dilatable, partially adjustable, homemade PA banding for ccTGAwith intact ventricular septum. PA band circumference was correlated to body weight (22 mm + 1 mm kg 1 ) and ideally adjusted to obtainflat septal geometry. Mean age at operation was 1.5 1.4 months. Results: There was no hospital mortality. Mean ventilation time and intensive care unit (ICU) stay were 20 9 h and 2.6 1.5 days, respectively. Five patients required postoperative inotropic support. One late death occurred suddenly at 4 months; normal biventricular function and no tricuspid regurgitation were noted at last follow-up, 1 week before death. Mean follow-up was 21.5 26 months. Mean band velocity increased over time from 2.65 0.7 m s 1 postoperatively to 3.7 0.3 at 6 months and 4.5 0.4 m s 1 at 2 years. Tricuspid regurgitation remained stable in seven patients, decreased in three and worsened in one. Flat septal geometry was obtained in all patients after the third postoperative month. One patient underwent a double-switch procedure at 7 years due to suprasystemic morphologically left ventricular pressure. The postoperative course was uneventful. Conclusions: In neonates with isolated ccTGA, prophylactic PA banding is safe and carries a low morbidity. At mid-term evaluation, tricuspid valve function is stabilised or improved and systemic competence of the left ventricle is maintained, thus allowing double switch if indicated. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective: To evaluate the impact of coronary patterns with intramural arteries on the outcome of arterial switch operation (ASO) in neonates with transposition of the great arteries (TGA). Methods: Between 1987 and 2008, 919 neonates underwent ASO for TGA. Forty-six (5.0%) had intramural coronary arteries. Intramural course involved the left main coronary artery in 28 of the 46 cases (61%), the left anterior descending artery in 12 patients (26%), the right coronary artery in three and both right and left coronary arteries in three cases. Various techniques were used to manage the coronary arteries: ASO without coronary relocation in one, ASO with coronary transfer as a single coronary button in nine and ASO with coronary transfer as two separate buttons in 36 patients (additional pericardial patches were implanted to orientate the coronary buttonin ninecases or enlarge thecoronaryostium inthree cases).Theintramural coursewas unroofed in mostcases(after 1995).Results:There were 13 deaths (28%): two intra-operative, nine before discharge from the hospital and two after discharge; during the same period, overall mortality in the 873 neonates with other coronary patterns was 3.9%. Actuarial survival at 10 years was 71 7%. Most deaths (11/13, i.e., 85%) were related to coronary complications. No time—trend effect was noted regarding mortality. Non-fatal coronary lesions were detected in eight patients (three with clinical evidence of myocardial infarction and five without). Five patients underwent re-operation for coronary revascularisation. Actuarial freedom from coronary events at 10 years was 46 10%. After a mean follow-up of 8.3 4.8 years, left ventricular function was normal in 97% of the survivors; minor ischaemic sequelae were present in two patients. Conclusions: Coronary patterns with intramuralarteriesremainassociatedwithhighcoronarymortalityandmorbidityfollowingneonatalASO,eveninthecurrentera.Theassociation of slit-like deformation of the ostium, stenosis of the intramural course and abnormal angle of take-off might explain the difficulty in coronary transfer. The technique of coronary transfer should be individually adapted to each anatomical situation. The place of patch ostioplasty of the intramural artery remains to be determined. # 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Objective Left ventricular mechanical dyssynchrony has been poorly studied in the pediatric population with dilated cardiomyopathy. We investigated the degree of dyssynchrony in children with dilated cardiomyopathy using tissue Doppler imaging and speckle tracking strain. Methods Twenty-five children with dilated cardiomyopathy were compared with healthy subjects. Left ventricular mechanical dyssynchrony was assessed by speckle tracking strain and tissue Doppler imaging. Both radial and longitudinal dyssynchrony were analyzed. Left ventricular end-diastolic diameter was measured to assess the relation between dyssynchrony and ventricular function and remodeling. Results Radial and longitudinal dyssynchrony parameters were significantly higher in the dilated cardiomyopathy group and correlated with Z-score left ventricular end-diastolic diameter. A logarithmic correlation between left ventricular ejection fraction and left ventricular end-diastolic diameter parameters was found. Conclusion In children with dilated cardiomyopathy, tissue Doppler imaging and speckle tracking strain allowed the detection of dyssynchrony, which correlates with the severity of left ventricular function.

Background— Early case detection is vital in rheumatic heart disease (RHD) in children to minimize the risk of advanced valvular heart disease by preventive measures. The currently utilized World Health Organization (WHO) criteria for echocardiographic diagnosis of subclinical RHD emphasize the presence of pathological valve regurgitation but do not include valves with morphological features of RHD without pathological regurgitation. We hypothesized that adding morphological features to diagnostic criteria might have significant consequences in terms of case detection rates. Methods and Results— We screened 2170 randomly selected school children aged 6 to 17 years in Maputo, Mozambique, clinically and by a portable ultrasound system. Two different echocardiographic sets of criteria for RHD were assessed: “WHO” (exclusively Doppler-based) and “combined” (Doppler and morphology-based) criteria. Independent investigators reviewed all suspected RHD cases using a higher-resolution, nonportable ultrasound system. On-site echocardiography identified 18 and 124 children with suspected RHD according to WHO and combined criteria, respectively. After consensus review, 17 were finally considered to have definite RHD according to WHO criteria, and 66 had definite RHD according to combined criteria, giving prevalence rates of 7.8 (95% confidence interval, 4.6 to 12.5) and 30.4 (95% confidence interval, 23.6 to 38.5) per 1000 children, respectively ( P Conclusions— Important consideration should be given to echocardiographic criteria for detecting subclinical RHD because the number of cases detected may differ importantly according to the diagnostic criteria utilized. Currently recommended WHO criteria risk missing up to three quarters of cases of subclinically affected and therefore potentially treatable children with RHD.

Endomyocardial fibrosis is the most common restrictive cardiomyopathy worldwide. It has no specific treatment and carries a poor prognosis, since most patients present with advanced heart failure. On the basis of clinical series, regional variations in distribution have been reported within several countries in Africa, Asia, and South America, but large-scale data are lacking on the epidemiologic features and early stages of the disease.We used transthoracic echocardiography to determine the prevalence of endomyocardial fibrosis in a rural area of Mozambique. We screened a random sample of 1063 subjects of all age groups selected by clustering. Major and minor diagnostic criteria were defined, and a severity score was developed and applied. Cases were classified according to the distribution and severity of the lesions in the heart.The estimated overall prevalence of endomyocardial fibrosis was 19.8%, or 211 of 1063 subjects (95% confidence interval [CI], 17.4 to 22.2). The prevalence was highest among persons 10 to 19 years of age (28.1%, or 73 of 260 subjects [95% CI, 22.6 to 33.6]) and was higher among male than among female subjects (23.0% vs. 17.5%, P=0.03). The most common form was biventricular endomyocardial fibrosis (a prevalence of 55.5%, or 117 of 211 subjects [95% CI, 48.8 to 62.2]), followed by right-sided endomyocardial fibrosis (a prevalence of 28.0%, or 59 of 211 subjects [95% CI, 21.9 to 34.1]). Most affected subjects had mild-to-moderate structural and functional echocardiographic abnormalities. Only 48 persons with endomyocardial fibrosis (22.7%) were symptomatic. The frequency of familial occurrence was high.Endomyocardial fibrosis is common in a rural area of Mozambique. By using echocardiography, we were able to detect early, asymptomatic stages of the disease. These findings may aid in the study of the pathogenesis of the disease and in the development of new management strategies.

Background— Infants and young children with certain types of heart disease are at increased risk for thromboses. Clopidogrel 75 mg/d is used in adults to prevent thrombotic events. The dose to achieve similar platelet inhibition in children is unknown. The objectives of the present study were (1) to determine the dose of clopidogrel needed in infants and young children to achieve a mean 30% to 50% inhibition of 5-μmol/L ADP–induced platelet aggregation (ie, inhibition similar to that observed with 75 mg in adults) and (2) to assess the safety and tolerability of clopidogrel in infants and young children. Methods and Results— We performed a prospective, multicenter, randomized, placebo-controlled trial evaluating the pharmacodynamics of clopidogrel in children (0 to 24 months) with a cardiac condition at risk for arterial thrombosis. Patients were randomized to clopidogrel versus placebo in a 3:1 ratio in 4 sequential groups (0.01, 0.10, 0.20, and 0.15 mg/kg) for ≥7 and ≤28 days. Platelet aggregation was assessed at baseline and steady state by light-transmission aggregometry. Of 116 patients enrolled, 92 (50% neonates, 50% infants/toddlers) were randomized, and 73 completed the study. A total of 79% of the randomized and treated patients were taking aspirin. Compared with placebo, clopidogrel 0.20 mg · kg −1 · d −1 resulted in a mean 49.3% (95% confidence interval 25.7% to 72.8%) inhibition of the maximum extent of platelet aggregation and a mean 43.9% (95% confidence interval 18.6% to 69.2%) inhibition of the rate of platelet aggregation. There was marked interpatient variability in the degree of platelet aggregation inhibition within each treatment-dose group and age group. No serious bleeding events occurred. Conclusions— Clopidogrel 0.20 mg · kg −1 · d −1 in children 0 to 24 months of age achieves a platelet inhibition level similar to that in adults taking 75 mg/d. Clopidogrel is well tolerated in infants and young children at this dose.

ObjectiveWe sought to investigate the mechanism whereby a particular deformity of the aortic arch, an angulated Gothic shape, might lead to hypertension late after anatomically successful repair of aortic coarctation.MethodsFifty-five normotensive patients with anatomically successful repair of aortic coarctation and either a Gothic (angulated) or a Romanesque (smooth and rounded) arch were studied with magnetic resonance angiography and flow mapping in both the ascending and descending aortas. Systolic waveforms, central aortic stiffness, and pulse velocity were measured. We hypothesized that arch angulation would result in enhanced systolic wave reflection with loss of energy across the aortic arch, as well as increased central aortic stiffness.ResultsTwenty patients were found to have a Gothic, and 35 a Romanesque, arch. Patients with a Gothic arch showed markedly augmented systolic wave reflection (12 ± 6 vs 5 ± 0.3 mL, P < .001) and greater loss of systolic wave height in the distal aorta (30% ± 16% vs 22% ± 12%, P < .01) compared with that of subjects with a Romanesque arch. Pulse wave velocity was also increased with a Gothic arch (5.6 ± 1.1 vs 4.1 ± 1 m/s, P < .0001), as well as left ventricular mass index (85 ± 15 vs 77 ± 20 g/m2). Patients with a Romanesque arch had increased aortic stiffness compared with that of control subjects (stiffness β-index, 3.9 ± 0.9 vs 2.9 ± 1; P = .03).ConclusionsAngulated Gothic aortic arch is associated with increased systolic wave reflection, as well as increased central aortic stiffness and left ventricular mass index. These findings explain (at least in part) the association between this pattern of arch geometry and late hypertension at rest and on exercise in subjects after coarctation repair.

Resume Le scanner ou tomodensitometrie est l’examen d’imagerie de reference pour la plupart des pathologies thoraciques tant chez l’adulte que l’enfant. Il a beneficie de progres technologiques extremement rapides ces dernieres annees avec l’apparition des scanners multibarette (ou multidetecteur ou multicoupe). Cette technologie permet actuellement d’obtenir une acquisition volumique du thorax d’un enfant en 2 a 5 secondes. Ces avancees technologiques sont particulierement utiles pour l’exploration des pathologies des voies aeriennes, en particulier grâce aux diverses possibilites de reconstruction ( maximum et minimum intensity projection, volume rendering , endoscopie virtuelle) qui offrent des perspectives interessantes en pediatrie. L’imagerie multiplanaire et surtout tridimensionnelle permet une analyse tout a fait originale dans l’espace des rapports parfois complexes entre les structures vasculaires et aeriques et de comprendre precisement certains mecanismes de compression bronchique extrinseque. Le present travail rappelle brievement les aspects techniques du scanner thoracique chez l’enfant et illustre la place actuelle du scanner volumique dans les pathologies thoraciques de l’enfant, au travers d’exemples cliniques tires de notre pratique quotidienne.

A b s t r ac t Background Epidemiologic studies of the prevalence of rheumatic heart disease have used clinical screening with echocardiographic confirmation of suspected cases. We hypothesized that echocardiographic screening of all surveyed children would show a significantly higher prevalence of rheumatic heart disease. Methods Randomly selected schoolchildren from 6 through 17 years of age in Cambodia and Mozambique were screened for rheumatic heart disease according to standard clinical and echocardiographic criteria. Results Clinical examination detected rheumatic heart disease that was confirmed by echocardiography in 8 of 3677 children in Cambodia and 5 of 2170 children in Mozambique; the corresponding prevalence rates and 95% confidence intervals (CIs) were 2.2 cases per 1000 (95% CI, 0.7 to 3.7) for Cambodia and 2.3 cases per 1000 (95% CI, 0.3 to 4.3) for Mozambique. In contrast, echocardiographic screening detected 79 cases of rheumatic heart disease in Cambodia and 66 cases in Mozambique, corresponding to prevalence rates of 21.5 cases per 1000 (95% CI, 16.8 to 26.2) and 30.4 cases per 1000 (95% CI, 23.2 to 37.6), respectively. The mitral valve was involved in the great majority of cases (87.3% in Cambodia and 98.4% in Mozambique). Conclusions Systematic screening with echocardiography, as compared with clinical screening, reveals a much higher prevalence of rheumatic heart disease (approximately 10 times as great). Since rheumatic heart disease frequently has devastating clinical consequences and secondary prevention may be effective after accurate identification of early cases, these results have important public health implications.

New generation multislice CT technology has changed the approach to non-invasive assessment of congenital heart disease, in both paediatric and adult patients. This is mainly because of rapid advances in spatial and temporal resolution and in post-processing capability. At Hôpital Necker-Enfants Malades, CT with multiplanar and three-dimensional reconstruction has become a routine examination in the evaluation of congenital heart disease planning surgery, complex interventional catheterisations and for follow-up. It has proved to be an invaluable diagnostic and decision-aiding methodology in these situations, as a complement to echocardiography and, increasingly, as a substitute for diagnostic angiography (which is usually associated with higher-dose radiation and longer sedation times, as well as occasional morbidity). This review illustrates the current status of 64-slice CT in congenital heart diseases, including assessment of the aorta, the coronary arteries, the pulmonary arteries, the systemic and pulmonary veins, and other intra- and extracardiac malformations.

Objectives This study was designed to investigate the influence of aortic arch geometry on vascular remodeling after anatomically successful repair of coarctation of the aorta (CoA). Background Abnormalities of the precoarctation vasculature are known to occur after CoA repair and appear related to adverse outcomes. The influence of aortic arch geometry on such abnormalities is unknown. Methods Sixty-three postcoarctectomy subjects (age 15.9 ± 6.3 years) were compared with 63 control volunteers. Aortic arch shape was characterized on magnetic resonance imaging using both qualitative classification, identifying 3 subtypes of arch geometry (Gothic, Crenel, Romanesque), and a quantitative index, height/width ratio (H/W) of the aorta. Using ultrasound, we measured carotid artery intima-media thickness (IMT) and stiffness index and distensibility, as well as right brachial artery flow-mediated dilation (FMD) and glyceryl trinitate (GTN)-induced dilatation, to assess the precoarctation vasculature of these subjects. Results Gothic arch type was associated with higher carotid IMT and stiffness index, lower carotid distensibility (p Conclusions In young adult survivors of anatomically successful CoA repair, a gothic-type aortic arch with high H/W is associated with abnormal IMT, higher aortic stiffness index, and impaired arterial reactivity in the pre-CoA vasculature.

Acyl-CoA dehydrogenase family, member 9 (ACAD9) mutation is a frequent, usually fatal cause of early-onset cardiac hypertrophy and mitochondrial respiratory chain complex I deficiency in early childhood. We retrospectively studied a series of 20 unrelated children with cardiac hypertrophy and isolated complex I deficiency and identified compound heterozygosity for missense, splice site or frame shift ACAD9 variants in 8/20 patients (40%). Age at onset ranged from neonatal period to 9 years and 5/8 died in infancy. Heart transplantation was possible in 3/8. Two of them survived and one additional patient improved spontaneously. Importantly, the surviving patients later developed delayed-onset neurologic or muscular symptoms, namely cognitive impairment, seizures, muscle weakness and exercise intolerance. Other organ involvement included proximal tubulopathy, renal failure, secondary ovarian failure and optic atrophy. We conclude that ACAD9 mutation is the most frequent cause of cardiac hypertrophy and isolated complex I deficiency. Heart transplantation in children surviving neonatal period should be considered with caution, as delayed-onset muscle and brain involvement of various severity may occur, even if absent prior to transplantation.

ObjectiveWe sought to investigate the effect of particular deformations of aortic arch shape on blood pressure response in children who had undergone successful repair of coarctation of the aorta.MethodsWe studied normotensive patients (aged 15.1 ± 5.8 years) who had undergone coarctation surgery by means of simple (n = 43) or extended (n = 32) end-to-end anastomosis. All patients underwent systematic exercise testing during follow-up, as well as magnetic resonance imaging. Three types of aortic arch geometry were evident on magnetic resonance imaging: gothic arch with angular geometry and an increased height/width ratio of the arch, crenel arch with a rectangular shape, and normal arch with a smooth rounded shape. For each subject’s arch, the continuous variable height/width ratio was calculated.ResultsExercise-induced hypertension was more frequent in patients with gothic arch, with a prevalence of 83% at 15 years after surgical intervention (95% confidence interval, 69%-97%) versus 25% (95% confidence interval, 0%-50%) in those with crenel and 21% (95% confidence interval, 10%-46%) in those with normal arch geometry (P < .001). The cumulative incidence of exercise-induced hypertension as a function of aortic arch geometry was significantly higher in patients with gothic arch geometry throughout follow-up. On multivariate analysis, both gothic arch geometry and higher height/width ratio were significantly correlated with exercise-induced hypertension.ConclusionAn angulated gothic arch is independently associated with abnormal blood pressure response. This deformation of the aortic arch identifies a subgroup of subjects with postoperative coarctation at high risk of hypertension in young adult life.

Background The objective of this study was to review our institutional experience concerning neonatal aortic commissurotomy and to identify predictors of outcome. Methods From January 1, 1990, to January 1, 2005, 65 neonates were referred for critical aortic stenosis. Among these 36 consecutive patients underwent surgical valvotomy by the same surgeon and 16 patients had balloon valvotomy. We examined clinical records to establish determinants of outcome and illustrate long-term results. Results In the surgical group 6 patients had nonstenotic parachute mitral valve, 6 had aortic coarctation, 13 had endocardial fibroelastosis; and 15 had depressed fractional shortening. In the balloon valvotomy group most infants had associated mitral stenosis (n = 7), small aortic annulus (n = 5), multiple anomalies (n = 2), endocardial fibroelastosis (n = 9), and depressed fractional shortening (n = 13). In the surgical group global mortality was 19%; 47% in infants with depressed fractional shortening and 0% in infants with normal fractional shortening. Mortality in the balloon valvotomy group was 56%. Low fractional shortening, low mean aortic gradient, endocardial fibroelastosis, and small aortic annulus were associated with fatal outcome. In the surgical group 6 patients needed Ross operation at a median interval of 4 years from commissurotomy. In the balloon valvotomy group 3 patients needed early mitral replacement and 5 aortic valve surgery. At a median follow-up of 6 years, all children are in New York Heart Association class I, all have a normal ventricular function, 2 have aortic restenosis, and 5 have at least moderate aortic incompetence. Conclusions In a selected population of neonates with isolated aortic stenosis, surgical valvotomy offers an excellent long-term outcome to patients with a normokinetic left ventricle, while patients with a hypokinetic left ventricle have a poor outcome. Balloon valvotomy offers poor results when performed in patients with complex anomalies.

Aims Pulmonary artery banding (PAB) is the first palliation in infants with complex congenital heart disease and elevated pulmonary blood flow. In older patients with corrected transposition of the great arteries, it may be used to retrain the left ventricle. To date, the only option is surgical. We report the development and the evaluation of a device for transcatheter PAB. Methods and results We intended to implant a pulmonary artery (PA) reducer percutaneously between the native pulmonic valve and the pulmonary bifurcation. Immediately following its insertion, we planned to implant a balloon expandable stent inside the restriction to calibrate the banding. Sheep were sacrificed acutely (group 1, n = 6) and after 1 month of follow-up (group 2, n = 6), the reducer was implanted successfully in all animals. It allowed the PA diameter to be reduced from 25 to 10.5 mm. Bare stents were successfully delivered inside the reducer. No paraprosthetic leak was found by injecting contrast dye. After the insertion procedure, signs of intolerance to obstruction were present in all animals and prompted us to dilate the stents from 12 to 16 mm. One animal from group 1 died before a balloon dilatation could be achieved. In the animals from group 2, the mean systolic gradient was 19 and 34.8 mmHg, respectively, at early and late evaluation. Conclusion Implantation of a PA reducer is possible in sheep, through a transcatheter approach allowing intravascular PAB. Miniaturization of the device is necessary to enlarge its use from adulthood to childhood.

Background This study was performed to measure right and left ventricular volumes in Ebstein's anomaly before and after operation. Methods Twenty-six consecutive patients were operated on with tricuspid valve repair and plication of the atrialized right ventricle, associated with a bidirectional cavopulmonary shunt in 15 patients. Right ventricular (RV) and left ventricular end-diastolic and end-systolic volume indexes were measured by x-ray computerized tomography before and after surgical repair. Left ventricular stroke index and ejection fraction were calculated. Results Before surgery, the mean stroke index of the atrialized RV was 36 ± 33 mL/m 2 , with severe reduction in 9 patients and aneurysmal aspect in 2 patients. After surgery, the atrialized RV was no longer identifiable. Both RV end-diastolic volume index and stroke index of the remaining effective RV were reduced. Bidirectional cavopulmonary shunt was a determinant factor of decrease in the effective RV end-diastolic volume index after repair. Conversely, left ventricular ejection fraction and stroke index increased significantly after surgery. Conclusions The atrialized RV with dyskinesia seems a good indication for a plication. RV end-diastolic volume index of the effective RV decreased after surgery. In severe cases, bidirectional cavopulmonary shunt was useful by decreasing RV end-diastolic volume, thus preventing further RV dilation. In all cases left ventricular ejection fraction and stroke volume index increased after surgery.

Objective True congenital ventricular diverticulum and aneurysm in children are very uncommon. We report our experience to clarify the diagnosis and outcome of these little-known entities. Methods Twenty-two patients with congenital ventricular outpouchings were identified in our database from 1973 to 2004. Morphologic characteristics (localization, connection to a ventricle, contractility), histologic findings, and cardiac and/or extracardiac abnormalities were analyzed in all 22 patients. Cardiovascular events and clinical courses were reviewed. Results Congenital ventricular diverticula (n = 16) were characterized by synchronal contractility and three myocardial layers on histologic examination. Two categories of congenital ventricular diverticulum could be identified with regard to their localization: apical and nonapical. Apical diverticula (n = 8) were always associated with midline thoracoabdominal defects and other heart malformations. Nonapical diverticula (n = 8) were always isolated defects. Congenital ventricular aneurysms (n = 6) were characterized by akinesis with paradoxical systolic motion, wide connection to the ventricle, fibrosis on histologic examination that appeared with high signal on T2 weighted magnetic resonance imaging, and absence of other heart or midline thoracoabdominal defects. The outcome was different in these two types of outpouchings: congential ventricular aneurysms were associated with adverse outcomes whereas the prognosis for congenital ventricular diverticula was good. Conclusion Congenital ventricular diverticulum and aneurysm are two distinct entities, with different histologic and morphologic characteristics and outcomes. Assessment of these differential characteristics is of importance for prenatal counseling.

To study the effects of closure of interatrial communications associated with a right to left shunt in patients with congenital heart disease (CHD) who had a biventricular repair.Retrospective study.Tertiary referral centre.15 patients with CHD with right to left shunt through an interatrial communication: three had repaired tetralogy of Fallot, five had repaired pulmonary atresia with intact ventricular septum, four had Ebstein's disease, and three had other CHDs. Two patients had had a stroke before closure of the interatrial communication.Percutaneous atrial septal defect (n = 6) or persistent foramen ovale (n = 9) closure. All patients underwent an exercise test before and after interatrial communication closure.Five patients were cyanotic at rest. During exercise, mean (SD) oxygen saturation diminished from 93.9 (3.8)% to 84.3 (4.8)% (p0.05). Interatrial communication closure led to an immediate increase of oxygen saturation from 93.9 (3.8)% to 98.6 (1.6)% (p0.05). At a median follow up of three years (range 0.5-5) all but one patient with a residual atrial septal defect had normal oxygen saturation at rest and during exercise. Maximum workload increased from 7.2 (1.9) to 9.0 (2.2) metabolic equivalents (p0.001).Percutaneous closure of interatrial communications associated with a right to left shunt allows restoration of normal oxygen saturation at rest, avoidance of desaturation during exercise, and improvement of exercise performance in patients with CHD.

Cardiac and pulmonary physiology in patients with functionally univentricular hearts is markedly different from that found in the setting of the normal circulation. The physiology of both the heart and the lungs remains abnormal after palliative procedures, such as creation of systemic-to-pulmonary shunts, banding of the pulmonary trunk, or creation of the cavo-pulmonary connections. In this brief review, I will concentrate on two main aspects of physiology, namely oxygenation and haemodynamics. I will make comparisons between the normal circulation and the functionally univentricular circulation, the latter both before and after creation of a cavo-pulmonary connection. To finish, I will discuss the physiology of the cavo-pulmonary connection itself, and consider some clinical implications.

Objectives:To identify predictive factors of complications occurring during paediatric interventional catheterisation.Background:Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated.Methods:We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation.Results:Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent).Conclusions:Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.

Objectives The goal of this study was to develop a device for percutaneous replacement of the tricuspid valve in animals. Background Percutaneous valve replacement has recently been introduced, and early clinical experience has been reported. To date, this technique is limited to the replacement of pulmonary and aortic valves in selected patients. Methods A newly designed nitinol stent, forming two large disks separated by a cylinder with a diameter of 18 mm, was specially designed for the purpose of this study. An 18-mm bovine valve was mounted in the central part of the stent, and a polytetrafluoroethylene membrane was sutured onto the ventricular disk. Eight ewes were equally divided into two groups (group 1, acute study; group 2, killed at one month). Results Seven of eight devices were successfully delivered in the desired position. In one animal, the device was trapped in tricuspid cordae, leading to its incomplete opening. A significant paravalvular leak was noticed in one animal of group 2. Mean right atrial pressure increased from 5 to 7 mm Hg and did not change during the follow-up. At autopsy, examination confirmed the good position of devices in successfully implanted animals. Conclusions Implantation of a semi-lunar valve in the tricuspid position is possible in ewes through a transcatheter approach. A disk-based nitinol stent is needed to allow valve implantation in the atrioventricular position. These studies open new perspectives into tricuspid as well as mitral valve replacement.

Objective:Primary implantation of stents is an accepted technique for treating aortic recoarctation, albeit that the effects of stenting on pressure profiles, carotid mechanical properties, intimal medial thickness, and reflection of the pulse wave have not been systematically investigated.Methods:Over the period from 1 January, 1999, to 31 December, 2002, we implanted stents to relieve aortic recoarctation in 15 patients, with a median age of 17 years, and a range from 7 to 29 years, with a median weight of 56 kilograms, ranging from 20 to 96 kilograms. Indications were a gradient of 20 millimetres of mercury or more measured in all, systemic hypertension at rest in 8, and systemic hypertension at exercise in all. Of the patients, 5 were receiving anti-hypertensive treatment. Before implantation of the stents, and after a mean follow-up of 22 months, all patients underwent an exercise test, vascular echography, and examination of the common carotid artery so as to determine its cross sectional compliance and distensibility, and the augmentation index.Results:The stents were implanted successfully in all patients. The mean gradient was reduced from 27 to 4 millimetres of mercury (p < 0.001). Systolic blood pressure at rest diminished from 140 to 131 millimetres of mercury (p = 0.04), while hypertension at rest regressed in 4 patients. Systolic blood pressure at exercise diminished from 245 to 222 millimetres of mercury (p = 0.018), and hypertension at exercise regressed in 1 patient. Anti-hypertensive treatment is still required for 4 patients. A correlation was found between systolic blood pressure at rest and initial peak-to-peak gradient (r = 0.8), and between initial gradient and percentage reduction of systolic blood pressure at rest at follow-up (r = −0.73). Compliance and distensibility of the common carotid artery were not significantly modified, albeit that the intimal medial thickness diminished from 0.64 to 0.57 millimetres (p = 0.04), and the augmentation index decreased from 5 to −1 (p = 0.012).Conclusions:Primary implantation of stents is effective in mid-term repair of aortic recoarctation. Although there is an improvement in systemic hypertension, the tensional profile and vascular sonography are not normalized. At long term follow-up, the suppression of an early reflection site of the pulse wave could decrease the wall stress of the great elastic vessels, reducing the thickness of the arterial walls.

Resume L'utilisation de catheters veineux centraux a demeure est de pratique courante dans les maladies chroniques en pediatrie. Les complications thrombotiques reduisent progressivement le capital veineux central et compliquent la prise en charge a long terme. Observation. – Nous rapportons deux cas d'obstruction des troncs veineux superieurs et discutons des techniques employees pour repermeabiliser ces axes veineux avant la mise en place d'un nouveau catheter a demeure. Conclusion. – Le controle de la permeabilite des veines devrait etre fait avant tout retrait ou changement d'un catheter veineux central a demeure, la repermeabilisation des axes veineux centraux etant plus simple lorsque le catheter central est en place dans le vaisseau thrombose.

Aims To investigate the role of the geometry of the aortic arch in resting hypertension after successful repair of coarctation (CoA). Methods and results 105 patients (15.3 ± 6 years) with successful repair of aortic CoA underwent blood pressure (BP) measurements at rest and magnetic resonance imaging (MRI) of the aortic arch and left ventricle. Three categories of aortic arch shape were defined based on the global geometry of the aortic arch: gothic, crenel and normal. The ratio of the maximum height/transverse diameter of the aortic arch (A/T), the percentage of residual stenosis (RS), the growth index of the aortic arch segments (GIA), and the left ventricular mass index (LVMI) were calculated. Twenty-seven (25.7%) patients had hypertension (HT). HT was more frequent in patients with gothic arch geometry (20/44, 45.5%) than in crenel geometry (5/18, 27.8%) and in normal geometry (2/43, 4.6%) (P < 0.001). Resting systolic BP and LVMI were significantly higher with gothic arch geometry. In multivariate analysis, the only correlates to HT, to level of systolic BP or to LVMI were A/T and gothic arch geometry. Conclusion Gothic geometry of the aortic arch is associated with resting hypertension in patients having undergone successful repair of CoA. This raises concern about methods to harmoniously repair CoA in order to prevent or delay rest hypertension in adulthood.

Resume Certaines cardiopathies obstructives du cœur droit sont corrigees grâce a la mise en place chirurgicale d'un conduit prothetique entre le ventricule droit et l'artere pulmonaire. Apres quelques annees, ces conduits s'obstruent et doivent etre changes chirurgicalement. Le catheterisme interventionnel grâce a la mise en place d'un stent est devenu une alternative interessante permettant de differer la chirurgie. Neanmoins, la correction de l'obstruction laisse place a une insuffisance pulmonaire qui, a long terme, peut etre deletere pour la fonction ventriculaire droite. Nous avons developpe une bioprothese implantable par voie percutanee permettant de lever l'obstruction et d'implanter dans le meme temps une valve. Nous rapportons, dans cet article, notre experience dans le domaine du remplacement valvulaire pulmonaire par voie non chirurgicale chez l'animal et plus recemment chez l'homme, et discutons les perspectives futures d'une telle approche.

Background— This study was undertaken to determine outcomes and best strategies for treatment of occluded pulmonary arteries in patients with congenital heart disease. Methods and Results— Between 1998 and 2002, occlusion of a previously patent pulmonary artery was established in 23 patients. Data were obtained retrospectively. Diagnoses were pulmonary atresia and ventricular septal defect in 11, tetralogy of Fallot in 6, and other forms of pulmonary stenosis or atresia in 6. Median age and weight at diagnosis were 9 years (range, 6 days to 43 years) and 24 kg (range, 2.6 to 60 kg). Fourteen patients had had a previous surgery. The occluded pulmonary artery was visualized at angiography by wedge injection or injection into the collateral circulation. The left pulmonary artery was occluded in 20 patients and the right pulmonary artery in 3. Criteria for reconstruction were estimated duration of occlusion 0.2. Twelve patients fulfilled these criteria and underwent pulmonary artery reconstruction at a mean interval of 2 months (range, 6 days to 6 months) from evidence of occlusion. Six patients had pericardial patch reconstruction, 3 terminoterminal anastomosis, 2 thrombectomy, and 1 a Blalock-Taussig shunt. There was 1 late death. At a median follow-up of 4 years (2 months to 5 years), all patients underwent cardiac catheterization: in 8 patients the reconstructed artery was patent, in 3 reoccluded. Hypoplasia of the occluded artery was reversed in 6 patients. Conclusions— Our data show that in selected patients, reconstruction of an occluded pulmonary artery can restore pulmonary vascularization and reverse hypoplasia. Strict surveillance is mandatory to prevent pulmonary artery loss.

The purpose of this paper was to determine the cardiac status in children 15 years or more after adriamycin therapy for a solid tumour. Of the 447 pts, 229 pts were fully studied and 218 were not. The following cardiac evaluations were proposed to all the 447 consecutive patients (pts): (1) cardiac Doppler US by one of two expert cardiologists; (2) cardiac rhythm and conduction abnormalities including 24-hour holter ECG; (3) (131)l-mlBG myocardial scintigraphy; (4) serum brain natriuretic peptide levels at rest; (5) an exercise test with VO(2) max measurement. The radiation doses delivered to 6 points in the heart were estimated for all patients who had received radiotherapy. Congestive heart failure was diagnosed in 24 of 229 (10%) evaluated pts, with a median interval of 15 years (0.3-24 years) from the first symptom after adriamycin treatment. Among the 205 remaining pts, 13 asymptomatic pts (6%) had severe (n=4) (FS

Objectives We report our initial experience with percutaneous pulmonary valve replacement in animals with large pulmonary trunks, using a modified percutaneous approach. Background Percutaneous pulmonary valve replacement has recently been introduced, and early clinical experience has been reported. This technique is presently limited to patients with a right ventricular outflow tract no bigger than 22 mm in diameter. Methods In seven animals (groups 1 and 3), we implanted a newly designed nitinol stent in the shape of a conduit with a central restriction of its diameter, containing an 18-mm bovine valve, as a one-step procedure. The animals in groups 1 and 3 were sacrificed after valve implantation and after two-month follow-up, respectively. In the second group (n = 3), we expected to percutaneously reduce the diameter of the pulmonary artery. Eight weeks later, we implanted an 18-mm valve mounted in a balloon-expandable stent. These animals were sacrificed after valve implantation. Results Eight of 10 devices were successfully delivered and were functioning perfectly at the initial evaluation and after two months. We failed to cross the tricuspid valve in two cases. The downsize mechanism allowed the pulmonary diameter to be reduced from 30 to 18 mm, without an impact on right ventricular function in any of the animals. Conclusions Non-surgical implantation of a pulmonary valve is possible in ewes with all types of pulmonary trunk, regardless of its size. A “downsize” stent is needed to allow valve implantation in a large trunk. Further refinements will make this technique feasible in humans.

OBJECTIVES We sought to highlight the clinical, morphologic, and pathogenetic features in patients with a cleft mitral valve (MV). BACKGROUND Few studies have addressed the morphologic features of cleft MV and the outcome of these patients. The pathogenetic features, including the developmental relation to an atrioventricular (AV) septal defect, remain unclear. METHODS We reviewed the patients with cleft MV that were diagnosed by echocardiography since 1980. Patients with an AV canal, ventriculo-arterial discordance, and hypoplastic ventricles were excluded. RESULTS Twenty-two patients were identified at a median age of 0.5 years (range 0 to 10.6). In three patients, no chordal attachments of the cleft to the ventricular septum were seen. Ten patients had significant mitral regurgitation (MR), and three had subaortic obstruction by the cleft. Associated cardiac lesions and extracardiac features were present in 13 and 10 patients, respectively. During the median follow-up period of 1.5 years (range 0 to 11.8), two patients died of extracardiac causes, and one neonate died of severe subaortic obstruction. Surgical repair was performed in 10 patients at a median age of 5.2 years (range 1.3 to 10.6). Multivariate analysis showed no predictors for MV surgery. One patient was re-operated for mitral stenosis associated with aortic valve stenosis. Follow-up echocardiography demonstrated moderate MR in two unoperated patients and moderate MV stenosis in two operated patients. CONCLUSIONS A cleft of the MV comprises a wide spectrum. Important morphologic differences exist with an AV septal defect, although the two lesions may be pathogenetically related. Surgical repair always seems possible. Long-term echocardiographic follow-up is warranted.

Objective After positive experimental results were obtained with the FloWatch-R-PAB (EndoArt S.A., Lausanne, Switzerland), an implantable device for pulmonary artery banding with telemetric control, it was tested in a prospective, multicenter clinical trial. Methods From June to September 2002, 6 patients with a mean age of 10.6 months (1-31 months) and a mean weight of 6.5 kg (3.5-11 kg) underwent pulmonary artery banding with the implantation of the FloWatch-R-PAB device through median sternotomy (4 patients) or left thoracotomy (2 patients). The diagnoses were the following: univentricular heart (2 patients), complete atrioventricular septal defect (2 patients), ventricular septal defect (1 patient), and multiple ventricular septal defects with double aortic arch (1 patient). The associated procedures were atrioseptostomy with cardiopulmonary bypass (2 patients), closure of patent ductus arteriosus (2 patients), and division of double aortic arch (1 patient). Results In a mean follow-up of 7 months (6-9 months), there were no early or late deaths, reoperations, or device-related complications. A mean of 5 regulations per patient (range 2-14) were required to adjust the tightening of the pulmonary artery banding, 50% (15/30) within the first postoperative week, 20% (6/30) during the second week, and 30% (9/30) within 8 months after surgery. In 70% (21/30) of the cases, the regulation was required to further narrow the pulmonary artery, and in 30% (9/30) of the cases, the regulation was required to release the pulmonary artery. Conclusions The initial trial confirmed the adequate functioning of the FloWatch-R-PAB device as telemetrically adjustable pulmonary artery banding. Repeated pulmonary artery banding adjustments, dictated by the clinical need in all patients even weeks after surgery, were accomplished without need for reoperation or invasive procedures. In children requiring pulmonary artery banding, the therapeutic strategies can be expanded by this promising technology. This device should be particularly indicated in patients with transposition of the great arteries requiring left ventricular retraining.