S. Hascoet

Comparison of two- and three-dimensional transthoracic echocardiography for measurement of aortic annulus diameter in children

BACKGROUND Accurate evaluation of aortic root geometry is necessary in congenital aortic valve lesions in children, to guide surgical or angiographical intervention. AIM To compare aortic annulus diameters measured by two- and three-dimensional transthoracic echocardiography (2D- and 3D-TTE), to determine the feasibility and reproducibility of 3D imaging and assess the dynamic changes during the cardiac cycle. METHODS Thirty children without heart disease were prospectively included. Two orthogonal aortic annulus diameters were measured offline using multiplanar reconstruction in diastole and in systole and were compared with the measurement of the aortic annulus diameter by 2D-TTE. RESULTS Mean age was 11±3.6 years. Feasibility of 3D imaging was 100%. The coefficients of intra- and interobserver variability were 3.5% and 6%, respectively. The 2D mean diameter was significantly smaller than the 3D maximum diameter in systole (1.94 vs. 2.01mm; p=0.005). 2D and 3D measurements were well correlated (p<0.0001). The maximum and minimum diameters in 3D were significantly different both in systole and in diastole (p<0.001) underlining an aortic annulus eccentricity. The mean aortic annulus diameters were not significantly different between systole and diastole, with important individual variability during the cardiac cycle. CONCLUSION This study demonstrated the feasibility and reproducibility of 3D-TTE for the assessment of the aortic annulus diameter in a normal paediatric population. Because of an underestimation of the maximum diameter by 2D-TTE and the asymmetry of the aortic annulus, 3D measurements could be important before percutaneous aortic valvuloplasty or surgical replacement.

The medical history of adults with complex congenital heart disease affects their social development and professional activity

BACKGROUND In recent decades, advances in surgery and therapeutic catheterization have steadily increased the life expectancy and prevalence of adults with congenital heart disease (CHD). AIMS We assessed medical and psychosocial variables of adults with CHD, according to the disease complexity. METHODS We included, from a single-centre observational cohort study, 135 consecutive adults with CHD (median age of 40 years, interquartile range: 28.0-51.0) followed in our cardiology unit, who answered a questionnaire assessing daily activity and psychosocial functioning. Disease complexity was classified according to the Bethesda conference. RESULTS Cardiac malformation complexity was simple in 61 (45.2%), moderate in 50 (37.0%) and complex in 24 (17.8%) patients. Cardiac surgery had been performed in 86.5% of moderate and complex patients. Complications (such as heart failure, arrhythmia and pulmonary hypertension) were mainly observed in the complex group (P=0.003). Physical activity was lower in the complex group (no activity in 58.8%, but sport previously contraindicated in 50% of these; P=0.03). Education level tended to be lower in the complex and moderate groups than in the simple group (respectively, 31.2% and 33.3% vs. 45.7% had passed the Baccalaureate; P=0.47). The pass rate was lower in patients with complications (P=0.037) or more than one cardiac surgery (P=0.03). In the complex group, 56.3% of patients were unemployed (P=0.048). CONCLUSIONS Complexity of heart disease and medical history affect education level and employment of adults with CHD. Academic education of children with a complex defect and career counselling are important to prevent unemployment among adults with CHD.

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

BACKGROUND Congenital 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. AIMS To 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. METHODS Seventy 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. RESULTS Median 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]). CONCLUSIONS Three-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.

3D transthoracic echocardiography to assess pulmonary valve morphology and annulus size in patients with Tetralogy of Fallot.

BACKGROUND Accurate evaluation of the pulmonary valve (PV) is crucial before surgical repair of Tetralogy of Fallot (TOF). AIMS To assess PV and pulmonary annulus (PA) morphology using three-dimensional (3D) transthoracic echocardiography (TTE) in infants referred for surgical repair of TOF. Also, to compare PA measurements obtained by 3D TTE with those from other imaging modalities, including two-dimensional (2D) TTE and computed tomography (CT), with reference to surgical measurements. METHODS 3D zoom mode was used to assess PV morphology. 2D TTE and CT PA diameters were compared to both vertical and horizontal diameters obtained from 3D datasets. Surgical PA diameters were measured using Hegars dilators. RESULTS A total of 29 patients with TOF (median [range] age 6 [3-24] months) were included and all successfully underwent 2D and 3D TTE; 22 also underwent CT. The number of pulmonary leaflets could be visualized in 24 patients (82.8%), with complete concordance with surgical findings. Vertical diameter was significantly longer than horizontal diameter (P<0.001)-underlying PA eccentricity-and was more important in bicuspid than tricuspid valves. Correlations between 2D and 3D TTE diameters were good. Surgical diameter was better correlated with 2D and 3D diameters than with CT diameter. 3D minimum, 2D and CT diameters were significantly lower than surgical diameters, but 3D mean and maximum diameters were not. CONCLUSION 3D TTE is accurate to assess PV morphology and PA size in patients with TOF. 2D TTE and CT underestimate PA diameter with reference to surgical diameter, however 3D mean and maximum diameters did not differ significantly.

Tethering of Tricuspid Valve Resulting from Aberrant Tendinous Cords Mimic Ebstein's Anomaly, Three‐Dimensional Echocardiography Approach

A neonate with refractory cyanosis, respiratory distress, and severe cardiomegaly was referred to our institution for suspicion of Ebstein’s anomaly. The electrocardiogram (Nihon Kohden, Tokyo, Japan) showed right atrial enlargement with incomplete right bundle branch block without preexcitation. Chest radiography revealed extreme cardiomegaly with decrease pulmonary vascularity. Two-dimensional echocardiography (2DE) revealed right atrial enlargement, tethering of tricuspid valve (TV), dilated TV annulus with severe regurgitation, and right to left shunt through a large atrial septal defect. We used real time three-dimensional echocardiography (RT3DE) Philips iE33 system (Andover, MA, USA), to assess more precisely the mechanism of the TV regurgitation; the region of ineffective coaptation was well visualized using the en face view. The movements of the septal and inferior leaflets were restricted by aberrant tendinous cords, (Figs. 1 and 2). The postprocessing analysis of the 3D volume dataset by multiplanar review mode (QLab 9, Philips, Andover, MA, USA) allowed visualization of the normal delamination of the septal and inferior leaflets without apical displacement of their hinge points (Fig. 3). The aberrant tendinous cords were clearly identified by sliding the planes throughout the leaflets.

Monomorphic Sustained Ventricular Tachycardia Late After Arterial Switch for d-Transposition of the Great Arteries: Ablation in the Sinus of Valsalva

We describe the case of a young boy with d-transposition of the great arteries (TGA) that was surgically corrected with an arterial switch operation, who presented with sustained ventricular tachycardia (VT) that was successfully ablated at the embryologic pulmonary artery-related sinus of Valsalva (SV). Although the VT mechanism is still uncertain, the presence of mid-diastolic potentials during VT and postsystolic potentials during sinus rhythm argue for a re-entrant mechanism.

Two-dimensional right ventricular strain by speckle tracking for assessment of longitudinal right ventricular function after paediatric congenital heart disease surgery.

BACKGROUND Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD). AIMS To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers. METHODS In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed. RESULTS Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P<0.0001 and r=0.70, P<0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P<0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P<0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P<0.0001). CONCLUSION RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD.

P3 3D Transthoracic echocardiography assessment of the pulmonary valve in patients with TOF

Background Accurate evaluation of pulmonary valve (PV) morphology and pulmonary annulus (PA) diameter is crucial before surgical correction of tetralogy of Fallot (TOF). Our aim was to assess PV morphology using three-dimensional transthoracic echocardiography (3D-TTE) in infants with TOF before surgical correction. And to compare PA diameter obtained by different imaging modalities. Methods 30 patients with TOF were prospectively included. All patients underwent 2D and 3D-TTE, 23 patients underwent CT-Scan and 7 cardiac catheterization. PA diameter was measured using 2D-TTE in parasternal short axis view as recommended. 3D dataset was acquired using zoom mode at PV. Both vertical (Dv) and horizontal (Dh) diameters of PA were measured. Mean 3D diameter (3DD) was calculated as (Dv+Dh/2). Eccentricity index (EI) of PA was calculated (Dv—Dh/Dv). These measurements were compared to CT-Scan and angiography when available and to perioperative measurements. Results Mean age 7.4 months (3-24 months), mean weight 6.6kg (4.5-13.5kg). PV was described as bicuspid in 15/30 patients by 3D-TTE from en face view, with 75% agreement between 3D-TTE and perioperative finding (20/30 patients). PA geometry was slightly asymmetric by 3D-TTE. Dv was significantly larger than Dh (8.4mm vs 7.4mm, p=0.001), and mean EI of PA was (10%). PA was more asymmetric in bicuspid valves rather than in tricuspid valves (EI 14% vs 7%, p=0.002). PA diameter didn’t differ significantly between 2D-TTE, Dh, CT scan and angiography, and were significantly lower than mean 3DD and Dv. There was a very strong correlation between 3DD and perioperative measurement. PA was conserved in 53.3% without significant residual stenosis immediately and after a 20 month of follow-up (except 1 patient requiring balloon dilatation). Conclusion 3D-TTE in patients with TOF is an accurate method to describe PV, and to measure PA size. Thus could help the surgical repair and the preservation of PV function.

Arythmies cardiaques fœtales : diagnostic et prise en charge

Resume Une arythmie cardiaque fœtale est depistee chez 1 a 2 % des grossesses. Un diagnostic precis est important pour adapter la prise en charge therapeutique, ce qui est le plus souvent possible par l’echocardiographie TM, le mode Doppler et de nouveaux outils diagnostiques. Il s’agit souvent d’extrasystoles intermittentes qui ne justifient pas de traitement. Les arythmies soutenues sont a risque d’anasarque fœtale, de sequelles neurologiques, de prematurite et de mort fœtale in utero . Les BAV complets (1/15 000–20 000 naissances) peuvent etre associes aux anticorps anti-SSA/SSB maternels ou a des cardiopathies congenitales (isomerismes gauches, double discordance) de plus mauvais pronostic. L’utilisation d’une corticotherapie maternelle dans les BAV immunologiques est controversee. Les tachycardies fœtales concernent environ 0,4–0,6 % des grossesses. Les causes les plus frequentes de tachycardie fœtale sont la tachycardie supraventriculaire (TSV) avec intervalle ventriculo-auriculaire court, liee a une reentree, et le flutter atrial. La tachycardie ventriculaire est rare chez le fœtus. Le choix de la prise en charge depend de nombreux facteurs differents : âge gestationnel, mecanisme de l’arythmie, frequence cardiaque, duree de la tachycardie, presence ou non d’insuffisance cardiaque congestive et d’anasarque. La majorite des fœtus avec tachycardie sont traites in utero avec succes par l’administration transplacentaire de medicaments antiarythmiques. La digoxine est largement acceptee comme l’antiarythmique de premiere ligne en absence d’anasarque. Le sotalol, la flecainide et l’amiodarone, ou des associations, sont utilises comme medicaments de deuxieme ligne quand la digoxine echoue a reduire la tachycardie ou en cas d’anasarque qui est le principal marqueur du risque de mort fœtale in utero .