Pierre-Emmanuel Séguéla

Congenital malformations of the mitral valve

Congenital malformations of the mitral valve may be encountered in isolation or in association with other congenital heart defects. Each level of the mitral valve complex may be affected, according to the embryological development, explaining the fact that these lesions are sometimes associated with each other. As a perfect preoperative assessment is of importance, good knowledge of both normal and abnormal anatomy is required in order to guide the surgeon accurately. This review presents the different embryological, anatomical and echocardiographic aspects of the congenital mitral anomalies.

Usefulness of three-dimensional transthoracic echocardiography for the classification of congenital bicuspid aortic valve in children

AIMSnBecause the classification of congenital bicuspid aortic valve (BAV) is of importance to predict a possible valvular dysfunction, we sought to assess the feasibility, the reproducibility, and the accuracy of three-dimensional transthoracic echocardiography (3D-TTE) to accurately depict the morphology of the leaflets in a BAV.nnnMETHODS AND RESULTSnSeventy-two consecutive children, who were suspected of having a BAV on two-dimensional transthoracic echocardiography (2D-TTE), were included in this prospective study. 2D-TTE and 3D-TTE views of a BAV were recorded by the same investigator, and then were analysed separately by two confirmed paediatric cardiologists. For each of these two imaging techniques, the spatial position of cusps and raphes was noted for each patient. Intra-observer concordance and inter-observer concordance were evaluated to assess the reproducibility of the techniques. Feasibility of 3D-TTE was 100%. Median acquisition time of 3D-TTE was 117 (98.5-176.8) s. Image quality seemed to be better with 3D-TTE compared with 2D-TTE. When using 3D-TTE, the diagnosis was reconsidered for 12 patients (17%). Only 44.4% of uncertain BAV cases identified by 2D-TTE were confirmed by 3D-TTE. Furthermore, 3D-TTE seems to provide a better visualization of the leaflet morphology, leading to reclassification for 34.4% (95% CI 22.9-47.3) of the patients. Agreement for the BAV classification between 2D-TTE and 3D-TTE was therefore moderate (κ = 0.46). Both inter-observer concordance and intra-observer concordance were good (κ = 0.91 and κ = 0.93, respectively) for 3D-TTE.nnnCONCLUSIONn3D-TTE is feasible and provides accurate description of a BAV in children. Compared with 2D-TTE, 3D-TTE seems to enable a better visualization of the structural geometry of the leaflets.

Assessment of valvular surfaces in children with a congenital bicuspid aortic valve: preliminary three-dimensional echocardiographic study.

BACKGROUNDnCongenital bicuspid aortic valve (BAV) is the most common congenital heart defect and may be responsible for aortic stenosis early in life. However, its pathogenesis remains unclear. A relationship between the severity of aortic stenosis and valvular surfaces has not been reported in the paediatric population.nnnAIMSnTo assess the feasibility of three-dimensional transthoracic echocardiographic planimetry in congenital BAV in children and to evaluate the influence of valvular asymmetry and aortic valve area (AVA) on stenosis severity.nnnMETHODSnSeventy consecutive children with BAV were included in this prospective single-centre study. Using the multiplanar review mode, surfaces were measured by planimetry (in systole for AVA and diastole for cusp surfaces). The degree of stenosis was assessed by instantaneous aortic Doppler. Results are expressed as medians and first and third quartiles.nnnRESULTSnMedian age was 5.6 years (2.2-11.5). Feasibility was 97%. Intra- and interobserver concordances were excellent for the measurement of cusp surfaces and AVA. Among the 70 children, 25 had aortic stenosis. The small/large cusp ratio was strongly associated with aortic stenosis (P<0.001). The area under the receiver operating characteristic curve was 0.89 (95% confidence interval 0.82-0.97). The best cut-off value for differentiating stenotic from non-stenotic valve was 0.75, with 84% sensitivity and 83% specificity. When indexed for body surface area, AVA was significantly smaller (P=0.031) in case of stenotic BAV (1.51cm(2) [0.99-2.28]) compared with non-stenotic BAV (1.99cm(2) [1.57-2.52]).nnnCONCLUSIONSnThree-dimensional echocardiographic planimetry is a feasible and reproducible method for assessing aortic surfaces in congenital BAV. Aortic stenosis seems to strongly depend on the asymmetry of the valve.

Procalcitonin as a marker of bacterial infection in children undergoing cardiac surgery with cardiopulmonary bypass.

BACKGROUNDnOwing to systemic inflammatory response syndrome, the diagnosis of post-operative infection after cardiopulmonary bypass is difficult to assess in children with the usual clinical and biological tools. Procalcitonin could be informative in this context.nnnMETHODSnRetrospective study in a paediatric intensive care unit. Blood samples were collected as soon as infection was clinically suspected and a second assay was performed 24 hours later. Using referenced criteria, children were retrospectively classified into two groups: infected and non-infected.nnnRESULTSnOut of the 95 children included, 14 were infected. Before the third post-operative day, procalcitonin median concentration was significantly higher in the infected group than in the non-infected group - 20.24 nanograms per millilitre with a 25th and 75th interquartile of 15.52-35.71 versus 0.72 nanograms per millilitre with a 25th and 75th interquartile of 0.28 to 5.44 (p = 0.008). The area under the receiver operating characteristic curve was 0.89 with 95% confidence intervals from 0.80 to 0.97. The best cut-off value to differentiate infected children from healthy children was 13 nanograms per millilitre with 100% sensitivity - 95% confidence intervals from 51 to 100 - and 85% specificity - 95% confidence intervals from 72 to 91. After the third post-operative day, procalcitonin was not significantly higher in infected children - 2 nanograms per millilitre with a 25th and 75th interquartile of 0.18 to 12.42 versus 0.37 nanograms per millilitre with a 25th and 75th interquartile of 0.24 to 1.32 (p = 0.26). The area under the receiver operating characteristic curve was 0.62 with 95% confidence intervals from 0.47 to 0.77. A procalcitonin value of 0.38 nanograms per millilitre provided a sensitivity of 70% with 95% confidence intervals from 39 to 89 for a specificity of 52% with 95% confidence intervals from 34 to 68. After the third post-operative day, a second assay at a 24-hour interval can improve the sensitivity of the test.nnnCONCLUSIONSnProcalcitonin seems to be a discriminating marker of bacterial infection during the post-operative days following cardiopulmonary bypass in children.

Critical stenosis of a right ventricle to coronary artery fistula seen at dual-source CT in a newborn with pulmonary atresia and intact ventricular septum

We report the case of a newborn with pulmonary atresia with intact ventricular septum and right ventricle-dependent coronary circulation. He died several weeks after a Blalock-Taussig procedure because of a progressive stenosis of the main coronary artery. We present echocardiographic and dual-source CT images of the stenosis, with autopsy correlation. To our knowledge, CT images of this quality have never been reported in a newborn. This case illustrates the extreme difficulty in prognosticating the outcome for these patients and underlines the need for a detailed neonatal coronary mapping to assess right ventricle-dependent coronary circulation.

Evolution of the QT interval in premature infants: a preliminary study.

BACKGROUNDnThe association between long QT interval and sudden infant death syndrome has been clearly established. Several studies have been conducted to determine the evolution of the QT interval in childhood from birth, but only in full-term newborns. However, data on the QT interval in pre-term infants are extremely scarce. The objective was to describe the development of the QT interval in premature infants. Material and methods In a prospective monocentric study in a neonatal intensive care unit, pre-term newborns born before 37 weeks of gestation without congenital heart disease, family history of long QT, unstable haemodynamic status, or administration of drugs inducing QT interval prolongation were included with parental consent. An electrocardiogram was recorded in similar conditions weekly until discharge in each child. The corrected QT was calculated with Bazetts formula.nnnRESULTSnIn all, 309 echocardiograms were recorded in 87 children, with gestational age ranging from 24-36 weeks. QT first increased after birth in very premature infants - less than 30 weeks of gestation - and then started to decrease, whereas it only decreased in more mature infants. When plotted against postmenstrual age, QT first increased, and then decreased after 32 weeks. Discussion Our data suggest that the QT interval varies with postmenstrual age in very premature infants, reaching a peak at 32 weeks. These developmental changes may induce specific vulnerability to QT-lengthening medications in premature infants. This study underlines the need for specific pharmacological studies in this population.

Double-orifice mitral valve assessed by two- and three-dimensional echocardiography in a newborn

MOTS CLÉS Valve mitrale ; Double-orifice mitral ; Cardiopathie A full-term female newborn with prenatal diagnosis of ventricular asymmetry was referred to our department for postnatal echocardiographic evaluation. Two-dimensional echocardiography revealed a coarctation of the aorta (Fig. 1A), a bicuspid aortic valve (Fig. 1B) and a double-orifice mitral valve (DOMV) (Fig. 2A, Video 1). Three-dimensional echocardiography showed two distinct orifices of unequal size divided by a complete fibrous bridge (Fig. 2B). The smaller orifice was situated laterally (Fig. 2A and B). There was no mitral stenosis or insufficiency. The mitral annulus was of normal size and two papillary muscles were clearly distinguished. Surgical repair of the mitral valve was not performed. The coarctation of the aorta was treated surgically at day 7 with the conventional technique of resection and end-to-end anastomosis (Crafoord’s operation). The postoperative course was uneventful. DOMV is a rare congenital anomaly, usually found in association with another congenital cardiac anomaly, such as atrioventricular septal defect (52%) or obstructive left-sided lesions (41%), but may also be isolated. A complete or partial fibrous bridge, defining the ‘bridge type‘, separates the mitral orifice into two orifices, which are usually of unequal size ; the medial orifice is usually larger. The ‘hole type’ is characterized by a secondary

Three-dimensional transthoracic echocardiographic assessment of supramitral ring in a young child.

Three-dimensional echocardiography is an increasingly used method to accurately depict congenital heart disease in children. We report the case of a 2-month-old girl with supramitral ring. Three-dimensional echocardiography allowed perfect visualisation of the fibrous shelf before surgery.

Percutaneous closure of a left superior vena cava draining directly into the left atrium in a child

MOTS CLÉS Cardiopathie congénitale ; Fermeture A 1-month-old boy was referred to our unit for discrete cyanosis during breastfeeding. There was no familial history of congenital heart disease. He was born at 34 weeks of gestation after a normal pregnancy. On examination, cardiac auscultation was normal and oxygen saturation was 97%. There were no signs of cardiac insufficiency. Two-dimensional transthoracic echocardiography revealed a left superior vena cava (LSVC) draining into the roof of the left atrium (Fig. 1A and Supplementary data, Video 1). There was a persistent foramen ovale with a discrete left-to-right shunt. The coronary sinus was not dilated. A computed tomography (CT) scan confirmed the abnormal systemic venal return (Fig. 1 B and C). The coronary sinus did not communicate with the LSVC and its drainage ostium measured 2.3 mm in diameter. The LSVC and right superior vena cava were not connected by any left innominate vein. No additional cardiac anomaly was seen. At 13 months of age, the patient was dyspnoeic and sweated at effort. The saturation rate was 91% at rest. Using a 2/10 mm plug, the percutaneous closure of

P383 - Défibrillateur automatique et myocarde non compacté : à propos d’un cas

Objectif Discuter l’interet du defibrillateur automatique implantable (DAI) en cas de troubles du rythme ventriculaires graves inauguraux chez l’enfant atteint de myocardiopathie spongieuse. Sujet La non compaction du ventricule gauche est une cardiopathie rare sous diagnostiquee. Elle peut etre isolee ou associee a d’autres anomalies cardiaques. La presentation clinique est extremement variable. Ses complications sont la dysfonction ventriculaire gauche, les troubles du rythme cardiaque, les accidents thrombo-emboliques systemiques et la mort subite. La frequence des arythmies est de 20 % chez l’enfant. Nous rapportons ici le cas d’un enfant de 33 mois, originaire du benin, ne de parents consanguins, chez qui le diagnostic de non compaction du ventricule gauche isolee a ete porte au decours de 3 arrets circulatoires sur fibrillation ventriculaire. Un DAI a ete pose en association avec un traitement par cordarone et aspirine. Ce cas s’integre dans une forme familiale (3 enfants atteints sur 5). Conclusion La pose d’un DAI n’est pas frequente chez le jeune enfant. Les troubles du rythme ventriculaires, dans le cadre du myocarde non compacte, peuvent cependant apparaitre comme une indication formelle y compris chez le jeune enfant.