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Introduction Sudden cardiac death is the most common cause of mortality in childhood onset hypertrophic cardiomyopathy. Identifying individuals at highest risk is therefore an essential part of clinical care but remains challenging. The 12 lead electrocardiogram (ECG) has been proposed as a useful tool for risk stratification and an ECG risk score has been proposed. However, this has not been independently validated and the ECG phenotype of childhood HCM has not been previously described. The aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods Participants with an available baseline resting 12-lead ECG were identified from a large, international, multi-centre, retrospective cohort of patients aged less than 16 years fulfilling the diagnostic criteria for HCM (n=1029). Resting baseline ECG was evaluated and ECG variables were extracted. In addition, the ECG risk score based on 8 parameters (deviation in QRS axis, pathological T-wave inversion in limb or precordial leads, ST-segment depression, dominant S-wave in V4, limb-lead amplitude sum, 12-lead amplitude duration product and QTc) was calculated as previously described. The primary study endpoint was a composite outcome of major cardiac events (MACE) defined as SCD, resuscitated cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia (VT) with haemodynamic compromise. The discriminatory performance of using an ECG risk score >5 to identify patients at increased risk of MACE at 5 years was determined using Harrell’s C-index. Results Of 356 patients with childhood HCM (68.9% male, mean age at presentation 10.1 ± 4.5 years), 347 (97.5%) had baseline ECG abnormalities such as: repolarization abnormalities (n=277, 77.8%), left ventricular hypertrophy (n=240. 67.6%), abnormal QRS axis (n=126, 35.4%) or QT prolongation (n=131, 36.8%). Over a median follow up of 3.9 years (IQR 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year MACE on univariable or multivariable Cox regression analysis. Of the 164 participants with an ECG score >5, 153 (93.3%) did not have a MACE within 5 years. Harrell’s C-index (the probability of correctly distinguishing between high and low risk patients using an ECG risk score threshold of >5) was 0.60 (95% CI 0.484-0.715) at 5 years. The corresponding positive and negative predictive values were 6.7% (95% CI 4.7 – 9.4%) and 96.9% (95% CI 94.2 – 98.4%). Conclusions In a large, international, multi-centre cohort of children with HCM, ECG abnormalities are common. No ECG characteristic, either in isolation or combined in the ECG risk score, was associated with 5-year MACE risk. This suggests that the role of the baseline ECG phenotype in improving risk stratification in childhood HCM is limited. Conflict of Interest None

Aims The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. Methods and results Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0–7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93–2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484–0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7% Conclusion In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

Background Irbesartan, a long acting selective angiotensin-1 receptor inhibitor, in Marfan syndrome might reduce aortic dilatation, which is associated with dissection and rupture. We aimed to determine the effects of irbesartan on the rate of aortic dilatation in children and adults with Marfan syndrome. Methods We did a placebo-controlled, double-blind randomised trial at 22 centres in the UK. Individuals aged 6–40 years with clinically confirmed Marfan syndrome were eligible for inclusion. Study participants were all given 75 mg open label irbesartan once daily, then randomly assigned to 150 mg of irbesartan (increased to 300 mg as tolerated) or matching placebo. Aortic diameter was measured by echocardiography at baseline and then annually. All images were analysed by a core laboratory blinded to treatment allocation. The primary endpoint was the rate of aortic root dilatation. This trial is registered with ISRCTN, number ISRCTN90011794. Findings Between March 14, 2012, and May 1, 2015, 192 participants were recruited and randomly assigned to irbesartan (n=104) or placebo (n=88), and all were followed for up to 5 years. Median age at recruitment was 18 years (IQR 12–28), 99 (52%) were female, mean blood pressure was 110/65 mm Hg (SDs 16 and 12), and 108 (56%) were taking β blockers. Mean baseline aortic root diameter was 34·4 mm in the irbesartan group (SD 5·8) and placebo group (5·5). The mean rate of aortic root dilatation was 0·53 mm per year (95% CI 0·39 to 0·67) in the irbesartan group compared with 0·74 mm per year (0·60 to 0·89) in the placebo group, with a difference in means of −0·22 mm per year (−0·41 to −0·02, p=0·030). The rate of change in aortic Z score was also reduced by irbesartan (difference in means −0·10 per year, 95% CI −0·19 to −0·01, p=0·035). Irbesartan was well tolerated with no observed differences in rates of serious adverse events. Interpretation Irbesartan is associated with a reduction in the rate of aortic dilatation in children and young adults with Marfan syndrome and could reduce the incidence of aortic complications. Funding British Heart Foundation, the UK Marfan Trust, the UK Marfan Association.

Accepted manuscript version, licensed CC BY-NC-ND 4.0. Background - Right ventricular (RV) function is a major determinant of survival in hypoplastic left heart syndrome (HLHS). However, the relation of RV geometry to myocardial mechanics and their relation to transplant-free survival are incompletely characterized. Methods - We retrospectively studied 48 HLHS patients from the Hospital for Sick Children, Toronto, (median age, 2.2; interquartile range, 3.62 years) at different surgical stages. Patients were grouped by the presence (n = 23) or absence (n = 25) of RV “apical bulging” defined as a sigmoid-shaped septum with the RV leftward apical segment contiguous with the left ventricular (LV) lateral wall. Regional and global RV strain were measured using speckle-tracking echocardiography, and regional strains were analyzed for patterns and peak values. These were compared between HLHS anatomical subtypes and between patients with versus without apical bulging. We further investigated the association between RV geometry and dysfunction with the outcomes of heart failure, death, or transplant. Results - RV global (–7.3% ± 2.8% vs –11.2% ± 4.4%; P = .001), basal septal (–3.8% ± 3.2% vs –11.4% ± 5.8%; P = .0001) and apicolateral (–5.1% ± 3.5% vs –8.0% ± 5.8%, P = .001) longitudinal strain were lower in patients with versus without apical bulging, respectively. Apical bulging was equally prevalent in all HLHS anatomical variants. Twenty of 22 (91%) patients with apical bulging displayed hypertrophy of the LV apical and lateral segments. Death or transplantation were approximately equal in both groups but related to reduced RV global strain in patients with (seven of seven) and not in those without apical bulging (two of eight; P = .022). Conclusions - These results suggest that the finding of apical bulging is related to the presence of a hypertrophied hypoplastic LV, with a negative impact on regional and global RV function. Therefore, analysis of RV and LV geometry and mechanics may aid in the assessment and prognostication of this high-risk population.

Aims Sudden cardiac death (SCD) is the most common cause of death in children with hypertrophic cardiomyopathy (HCM). The European Society of Cardiology (ESC) recommends consideration of an implantable cardioverter-defibrillator (ICD) if two or more clinical risk factors (RFs) are present, but this approach to risk stratification has not been formally validated. Methods and results Four hundred and eleven paediatric HCM patients were assessed for four clinical RFs in accordance with current ESC recommendations: severe left ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia, and family history of SCD. The primary endpoint was a composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate ICD therapy, or sustained ventricular tachycardia), defined as a major arrhythmic cardiac event (MACE). Over a follow-up period of 2890 patient years (median 5.5 years), MACE occurred in 21 patients (7.5%) with 0 RFs, 19 (16.8%) with 1 RFs, and 3 (18.8%) with 2 or more RFs. Corresponding incidence rates were 1.13 [95% confidence interval (CI) 0.7–1.73], 2.07 (95% CI 1.25–3.23), and 2.52 (95% CI 0.53–7.35) per 100 patient years at risk. Patients with two or more RFs did not have a higher incidence of MACE (log-rank test P = 0.34), with a positive and negative predictive value of 19% and 90%, respectively. The C-statistic was 0.62 (95% CI 0.52–0.72) at 5 years. Conclusions The incidence of MACE is higher for patients with increasing numbers of clinical RFs. However, the current ESC guidelines have a low ability to discriminate between high- and low-risk individuals.

Background: Children with cardiomyopathy (CM) are at risk of sudden cardiac death (SCD), but the incidence and risk factors for this outcome are not clear.Objectives: This study sought to d...

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates.Design, Setting, and Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017.Exposures: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping.Main Outcomes and Measures: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise).Results: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95%, CI 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years.Conclusions and Relevance: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Background Right ventricular (RV) dysfunction and tricuspid regurgitation (TR) are associated with adverse outcome in hypoplastic left heart syndrome (HLHS) but remain poorly understood. The aim of this study was to assess RV longitudinal strain and mechanical dyssynchrony in HLHS in relation to TR. The hypothesis was that inhomogeneous RV contraction and mechanical dyssynchrony around the tricuspid valve apparatus may be associated with TR in some patients with HLHS. Methods Echocardiograms of children aged 0 to 2 years with HLHS at all stages of surgical palliation were retrospectively reviewed for anatomic subtype and severity of TR. RV peak strain and dyssynchrony were assessed by vector velocity imaging. Results Sixty echocardiograms of patients with a median age of 0.54 years (interquartile range, 0.04–1.63 years) and a median weight 6.40 kg (interquartile range, 3.70–10.45 kg) demonstrated TR, which was absent or trivial in 25 (42%), mild in 20 (33%), moderate in five (8%), and severe in 10 (17%). The difference in peak longitudinal strain between the RV free wall and the septum or left-sided RV wall was significantly higher in patients with moderate or severe TR compared with no, trivial, or mild TR (2.93 ± 6.03% vs 0.16 ± 6.6%, P = .04). The difference in time to peak longitudinal strain between walls was significantly longer in moderate or severe TR compared with no, trivial, or mild TR (57.4 ± 145.1 vs 15.8 ± 75.9 msec, P = .04). There was a significant difference in anatomic subtype between patients with the most difference in peak strain compared with those with the least. Conclusions RV mechanical dyssynchrony and inhomogeneous contraction are worse in patients with clinically important TR and HLHS.

Cardiorespiratory collapse can have a number of causes in the neonate, and echocardiography is an important diagnostic tool in this population. While speed of diagnosis may be important, it is also critically important that the cardiologist is aware of the limitations and potential pitfalls of echocardiography. Echocardiographic evaluation must include a full and accurate assessment of anatomy and haemodynamics, and the cardiologist must also correctly interpret findings. We present a case that highlights the principles of interpretation.

Aims The multifactorial mechanisms of tricuspid valve (TV) insufficiency in patients with hypoplastic left heart syndrome (HLHS) include structural anomalies of TV leaflets and ventricular dilatation. We hypothesized that 2-D echocardiography underestimates the importance of TV structural abnormalities, whereas surgical assessment underestimates the importance of motion abnormalities, and compared echocardiographic assessment with surgical description. Methods and results Two independent experts retrospectively reviewed echocardiograms of all patients who had staged single-ventricular palliation and TV repair during January 1998–December 2008, and compared with case-matched controls who did not require TV repair. Primary and secondary mechanisms of TV insufficiency were categorized, and surgical findings ascertained from operation records. There were 32 patients with a median age of 5.9 months (0.3–140) and 32 matched controls. On echocardiographic review, an abnormality of at least one leaflet was noted in every patient (100%) vs. in only 14 controls 14 (44%) ( P < 0.001). Leaflet prolapse was described in 22 (69%), and the restriction of a leaflet in 20 (69%). Agreement between the experts was excellent ( κ = 0.64–0.88). On surgical inspection, annular dilatation was found in 17 (53%), and leaflet dysplasia in 14 (44%). Agreement between echocardiographic and surgical assessment was poor ( κ < 0.6). Conclusion Important structural abnormalities are common in patients with HLHS and TV insufficiency, some readily identified by 2-D echocardiography. However, there are significant discrepancies between echocardiographic and surgical findings. Echocardiographic assessment is sensitive to detect leaflet motion abnormalities, but not leaflet structural abnormalities. Both echocardiographers and surgeons should be aware of these limitations when planning surgical interventions.

Aims: Pressure gradient is used for timing of balloon aortic valvuloplasty for aortic stenosis (AS) in children, but does not correlate well with outcome and is limited if ventricular function is poor. In adults, effective orifice area (EOA) is used to assess AS severity, but EOA by continuity equation or 2D echo is unreliable in children. Three-dimensional echocardiography (3DE) may reliably assess EOA but has not been studied in children. We assessed measurement of aortic valve EOA by 3DE in children with AS before and after balloon aortic valvuloplasty and compared results with change in aortic valve gradient. Methods: 3DE was performed at time of catheterization before and after balloon aortic valvuloplasty. Using 3DE multiplanar review mode, valve annulus diameter, area, and EOA were measured and compared with change in aortic gradient and degree of aortic insufficiency. Results: Twenty-four 3DE studies in 12 children (mean age 4.4 ± 5.0 years) were analyzed. EOA was measurable in all. Catheter peak gradient decreased from 45 ± 10 to 26 ± 17 mmHg (P = 0.0018). 3DE EOA increased after balloon aortic valvuloplasty (0.59 ± 0.52 cm2 vs 0.80 ± 0.70 cm2; P = 0.03), without change in valve diameter. EOA change correlated with change in peak (r = 0.77; P = 0.005) and mean (r = 0.60; P = 0.03) aortic valve gradient post balloon aortic valvuloplasty. Conclusion: 3DE facilitates EOA measurement in pediatric AS and correlates with change in aortic valve gradient after balloon valvuloplasty. (Echocardiography 2012;29:484-491)

Background A potential genetic basis has been identified in around 30% of all childhood dilated cardiomyopathy (DCM). Despite this, the long-term outcomes for children with familial DCM have not been well characterized. This study examines the presentation, outcomes and echocardiographic findings for children with familial DCM enrolled in the National Australian Childhood Cardiomyopathy Study. Methods NACCS is a longitudinal, population based study which includes all children aged 0–10years who were diagnosed with primary cardiomyopathy between 1987 and 1996. Details of clinical status at diagnosis, medical therapy and late outcomes were recorded. Serial echocardiographic indices of LV size, fractional shortening and ejection fraction were expressed as Z scores, based on BSA and age. Familial cardiomyopathy was defined as the presence of an affected first or second degree relative with a similar cardiomyopathy. All available echocardiograms were interpreted by a single observer. Results There were 175 subjects with DCM. Children with familial DCM (27 cases) were younger at diagnosis than those with sporadic DCM (median age 3.2 vs. 8.4months ( p =.004) and were less likely to have congestive heart failure and to be hospitalised at presentation ( p ≤.002 for both). Familial DCM subjects had a higher mortality (62.9% vs. 36.5%; p =.02) and were less likely to be free of medical therapy at latest follow-up (7.4% vs. 52.0%; p p =.006) and a lower FS Z score (−7.35 vs. −3.40; p =.002) score at latest follow-up. Conclusions Familial DCM subjects are younger and are less likely to have symptomatic heart failure at the time of diagnosis. Despite this they have a higher mortality and worse ventricular function at late follow-up. This study underlines the differing presentations and outcomes among children with familial and sporadic DCM.

Background Three-dimensional echocardiography offers new insights into valvar function in atrioventricular septal defects (AVSDs). The aim of this study was to identify a morphological marker to predict the functional outcomes of left atrioventricular valves (AVVs) following the repair of AVSDs. Methods Twenty-nine consecutive patients were evaluated preoperatively using 2-dimensional and 3-dimensional echocardiography. The angle of the AVV relative to the crux of the heart was measured in multiplanar review mode. Results The severity of postoperative left AVV regurgitation was correlated with preoperative valvar angle, being more acute in patients with moderate or severe regurgitation (mean, 57 ± 13° vs 83 ± 9° in patients with no or mild regurgitation; P = .002). Angles ≤ 59° predicted severe regurgitation with 79% specificity. Conclusions Multiplanar review of 3-dimensional data sets is valuable for the assessment of the functional morphology of AVSD valves. Using this technique, more acute AVV angles predicted increased likelihood of severe regurgitation following surgical repair.

IntroductionThe mitral valvar complex is difficult to visualise accurately in only two dimensions. Three-dimensional echocardiography gives new insight into the dynamic changes of intra-cardiac structures during the cardiac cycle. The aim of this study was to study the mitral annulus in systole and diastole in normal children using three-dimensional echocardiography, and to analyse the effect of regurgitation on annular function.Materials and methodsThree-dimensional echocardiographic datasets, acquired in 11 consecutive subjects with mitral regurgitation, and 20 normal subjects, were analysed offline using simultaneous multiplanar review.ResultsThe mitral valvar annular area decreased in diastole, and increased in systole, in both groups. The annulus in patients with mitral regurgitation is dilated compared to normal subjects, the systolic value for those with regurgitation having a mean of 6.79 plus or minus 2.55 centimetres2/metres2, and the diastolic value a mean of 5.01 plus or minus 1.78 centimetres2/metres2, as opposed to a systolic mean value of 5.28 centimetres2/metres2 plus or minus 1.68, p = 0.091, and diastolic mean value of 3.05 centimetres2/metres2 plus or minus 0.90, in normal subjects (p less than 0.0001). The proportional change in mitral valvar annular area from systole to diastole showed a trend towards being smaller in those with mitral regurgitation, although this did not reach significance (24.8% versus 41.13%, p equal to 0.249). Analysis of subgroups of patients with moderate or severe mitral regurgitation showed mitral excursion, expressed as percentage of left ventricular length, to be significantly less than in normal subjects, at 12.78 plus or minus 5.10% versus 15.84 plus or minus 4.23% (p equal to 0.012).ConclusionsMitral valvar annular area in children decreases in diastole, and increases in systole. In those with mitral regurgitation, the annulus is dilated and the dynamic annular function is depressed.

Aims Associated left ventricular structures may play a role in progression and recurrence of discrete subaortic stenosis. The availability of a new 3D echocardiography tool, multiplanar review (MPR), allows comprehensive analysis of datasets in infinite planes, and detailed examination of anatomy. We sought to evaluate the role of MPR in defining the morphology of subaortic stenosis. Methods Consecutive patients underwent detailed 2 and 3D echocardiographic examination using MPR. Results Sixteen patients aged 0.7–15.9 years (median 4.57) with diagnosis as follows: isolated subaortic stenosis in nine, additional defects in seven (coarctation of aorta, VSD, mitral, or aortic stenosis). Position and extent of subaortic stenosis was clearly described by multiplanar revew in all patients. Additional MPR findings were: abnormalities of mitral valve leaflet or chordal apparatus attachments (14 patients), abnormal ventricular muscle band (11), abnormal increased aorto-mitral separation (two). The aortoseptal angle was significantly decreased in subaortic stenosis, mean 141 ± 12°, vs. normal subjects, mean 153 ± 6°, P = 0.02. Surgical findings correlated well with MPR findings. Conclusions MPR analysis of 3D datasets is a sensitive and accurate mode for delineation of morphological details of discrete subaortic stenosis, providing additional information to 2D echocardiography.

OBJECTIVES: Transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO) with or without ventricular septal defect have multiple surgical treatment options. We sought to identify pre- and intraoperative factors that determine the timing of repair, procedure type and subsequent LVOT outcome. METHODS: Twenty-eight (8.2% of all TGA) patients with TGA with LVOTO (double outlet ventricle, n= 5, TGA/intact septum, n =1 ) between 2000 and 2012 were reviewed. Anatomical factors were identified by prerepair echocardiography. LVOTO complexity was characterized by the degree of obstruction (0 = none, 0.33 = mild, 0.66 moderate and 1 = severe) at various levels: pulmonary valve (PV) dysplasia/hypoplasia, posterior deviation of the infundibular septum, fibromuscular ridge, tissue tag and abnormal chordal attachment. Summation of the obstruction score, at each level, yielded the LVOT complexity score. The descriptive analysis of intraoperative decisionmaking at late repair was performed. RESULTS: Operations: early arterial switch operation (ASO) + LVOT resection (n= 9, 32%), late ASO + LVOT resection (n= 3, 10%), Nikaidoh (n= 8, 29%), Rastelli (n= 6, 21%), single-ventricle palliation (n= 2, 7%). The primary LVOT obstruction mechanism was posterior deviation of the infundibular septum (n= 16, 57%) and PV dysplasia (n= 6, 21%). The early ASO group had a lower PV complexity score (0.42 ± 0.22 vs 0.96 ± 0.55, P= 0.007), tissue tag score (0.03 ± 0.15 vs 0.26 ± 0.34, P= 0.018) and LVOT complexity score (2.11 ± 0.86 vs 3.2 ± 0.96, P= 0.006). The LVOT complexity score in the Nikaidoh group was higher than in the late ASO group (P= 0.019). Of 16 candidates for the Nikaidoh procedure, 6 patients underwent a Rastelli operation due to coronary artery patterns (single coronary, n= 3, 1RL-2Cx, n= 2 or an abnormal left anterior descending coronary artery course, n= 1). Two patients underwent single-ventricle palliation due to the interference of essential chordae. All patients survived the operation. The 3-year survival was 96%. One patient who underwent late ASO required re-LVOT resection. CONCLUSIONS: A newly developed scoring system, the LVOT complexity score, helped to quantify the LVOT complexity and was correlated with our choice of the surgical procedure of TGA with LVOTO. The current strategy achieved reasonable survival and LVOT outcome with three quarters of the patients having an anatomically aligned LVOT. The coronary anatomy pattern was the primary determinant in the decision-making between the Nikaidoh procedure and the Rastelli operation.

Ebstein's malformation is difficult to visualise, for both the echocardiographer and the surgeon. The essence of the problem in Ebstein's malformation is the deviation of the hingepoints of the leaflets towards the junctions of the inlet and apical trabecular parts of the right ventricle. Three-dimensional echocardiography offers new insights into the morphology and function of malformed valves, and allows elucidation of all the features. It allows clear visualisation of the valve leaflets, showing the precise morphology of the valve leaflets, the extent of their formation, the level of their attachment, and their degree of coaptation. Visualisation of the mechanism of regurgitation or stenosis is possible, as is more accurate quantification of the regurgitant jet or jets. Subchordal apparatus may be seen more clearly using three-dimensional echocardiography, and their functional anatomy understood. The multiplanar review modality allows examination of the three-dimensional data set even in patients with sub-optimal echocardiographic imaging. Previously, much of this information could only be well-understood at the time of surgery or post mortem, meaning that the majority of the specimens fully examined were at the poorly functioning end of the spectrum. This information is of use in furthering our understanding of this complex lesion as it functions in vivo, and demonstrating which anatomical pathology is significant in producing functional and physiological consequences. It is also of use for the clinician in selecting which patients are amenable to surgical intervention, for either single or biventricular repair, and for the surgeon in planning how to approach the operation. Correlation between three-dimensional echocardiographic findings and surgical findings has already been established, but the effect of this enhanced anatomical knowledge on surgical planning and surgical outcome requires further investigation.