Tara Bharucha

Multiplanar review analysis of three-dimensional echocardiographic datasets gives new insights into the morphology of subaortic stenosis

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 review 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 degrees , vs. normal subjects, mean 153 +/- 6 degrees , 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.

Impact of Multiplanar Review of Three-Dimensional Echocardiographic Data on Management of Congenital Heart Disease

BACKGROUND In patients with congenital cardiac malformations, accurate diagnosis is critical in diagnosis and management. The multiplanar review mode (MPR) allows the operator to cut three-dimensional (3D) echocardiographic data sets in infinite planes, and to review the moving image in three simultaneous orthogonal planes. We sought to describe the clinical utility of MPR of 3D echocardiography for analysis of congenitally malformed hearts. METHODS Cross-sectional and 3D MPR echocardiography was performed in 300 patients with congenitally malformed hearts. RESULTS Analysis in multiplanar mode was possible in all patients. New, clinically important information, which altered management or changed the principal diagnosis, was obtained in 32 (11%) cases. This determined suitability for biventricular repair in 11 patients, clarified the morphology of atrioventricular valves in 7, helped in assessment of aortic, mitral, or prosthetic valvar disease in 13, and identified a vascular ring in the other patient. CONCLUSIONS 3D MPR is feasible in the setting of the congenitally malformed heart, permitting focused and in-depth analysis. This substantially improves the understanding of functional morphology, above the information derived from cross-sectional echocardiography. We recommend the use of the 3D format with MPR for patients with complex congenital cardiac disease.

Multiplanar review of three-dimensional echocardiography gives new insights into the morphology of Ebstein's malformation

INTRODUCTION We aimed to assess the ability of the multiplanar review modality of three-dimensional echocardiography to examine the dynamic morphology and the functional characteristics of malformed tricuspid valves in patients previously identified as having Ebsteins malformation. Based on these characteristics, we attempted to differentiate Ebsteins malformation from tricuspid valvar dysplasia. METHODS Using three-dimensional multiplanar review, analysed with either Qlab 6.0 or Tomtech Image Arena 3.0, we studied 23 patients, aged from 1 day to 70 years, previously diagnosed using cross-sectional echocardiography as having Ebsteins malformation. RESULTS Using the features of rotational abnormality, and the orientation, of the effective tricuspid valvar orifice as diagnostic features of Ebsteins malformation, we reclassified 11 patients (48 per cent) as exhibiting tricuspid valvar dysplasia. In addition, we studied the dynamic morphology as well as the function of the tricuspid valve. Surgical treatment was undertaken on 10 patients, revealing good correlation with the findings obtained using three-dimensional multiplanar review. In those with Ebsteins malformation, we found varying degrees of rotation, with the effective valvar orifice always directed towards the right ventricular outflow tract. The opening of the orifice of dysplastic tricuspid valves, in contrast, was towards the apex of the right ventricle. The degree of delamination, and abnormalities of subcordal apparatus, were similar in the two groups. DISCUSSION Three-dimensional multiplanar review permits accurate definition of the dynamic morphology of Ebsteins malformation, permitting clear differentiation from tricuspid valvar dysplasia.

Neonatal diabetes mellitus: Insulin pump as an alternative management strategy

Abstract:  Neonatal diabetes mellitus (hyperglycaemia within the first month of life, with an insulin requirement) may be transient or permanent. Management is complex, due to lack of subcutaneous fat and the need for small doses of insulin, and may be complicated by additional medical problems. Three cases are presented, the first of which was treated with conventional insulin therapy. The latter two were successfully treated with subcutaneous insulin pump therapy. We present suggested guidelines for treatment of neonatal diabetes, using this novel approach to management.

Sudden Death in Childhood Cardiomyopathy: Results From a Long-Term National Population-Based Study

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 determine the incidence and risk factors for SCD in children with varying CM phenotypes from a long-term population-based study of childhood CM. METHODS The NACCS (National Australian Childhood Cardiomyopathy Study) is an ongoing longitudinal cohort study including all children in Australia with primary CM who were diagnosed between January 1, 1987, and December 31, 1996, and were <10 years of age. The cumulative incidence and risk factors for SCD within individual CM phenotypes were explored using survival analysis. RESULTS Of 289 eligible patients, 16 (5.5%) experienced SCD over a median follow-up of 11.9 years (interquartile range: 1.7 to 15.4). The risk of SCD varied according to CM phenotype (p=0.007). The cumulative incidence of SCD at 15 years was 5% for dilated cardiomyopathy (DCM), 6% for hypertrophic cardiomyopathy (HCM), 12% for restrictive cardiomyopathy, and 23% for left ventricular (LV) noncompaction. Older age at diagnosis, positive family history of CM, and severity of LV dysfunction were related to increased risk of SCD in patients with DCM, and a higher posterior wall thickness Z-score was the sole risk factor identified for patients with HCM. CONCLUSIONS Predictors of SCD include CM phenotype, family history of CM (DCM), severity of systolic dysfunction (DCM), and extent of LV hypertrophy (HCM). Continuing follow-up of this cohort into adulthood is likely to reveal an ongoing risk of SCD.

Management of newborn lymphangiectasia and chylothorax after cardiac surgery with octreotide infusion

Postoperative chylothorax compromises nutrition, immune function, coagulation, and fluid status. We report rapid short-term suppression of chylothorax by octreotide in an infant after surgery for complex congenital heart disease complicated by lymphangiectasia.

The angle of the components of the common atrioventricular valve predicts the outcome of surgical correction in patients with atrioventricular septal defect and common atrioventricular junction

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 degrees vs 83 +/- 9 degrees in patients with no or mild regurgitation; P = .002). Angles < or = 59 degrees 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.

Right ventricular mechanical dyssynchrony and asymmetric contraction in hypoplastic heart syndrome are associated with tricuspid regurgitation.

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.

Measurement of Effective Aortic Valve Area Using Three‐Dimensional Echocardiography in Children Undergoing Aortic Balloon Valvuloplasty for Aortic Stenosis

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)

Recent advances in pediatric echocardiography.

Echocardiography is the fundamental tool in the management of children with congenital heart disease (CHD), and cross-sectional echocardiography is still the main technique used for diagnosis and therapeutic planning. Recent advances in pediatric echocardiography include 3D echocardiography and functional imaging. The recent development of specific pediatric probes allows imaging of pediatric hearts with high temporal and spatial resolution. Lesions are often anatomically complex, and 3D echocardiography allows increased appreciation of complex spatial relationships and can thereby be valuable in understanding functional anatomy and planning interventions. Assessment of pediatric myocardial function can be difficult, with highly variable ventricular morphology. Assessment of right ventricular function and function of the single ventricle are current challenges in CHD. The introduction of myocardial tissue Doppler velocities and deformation imaging (strain and strain-rate quantification) facilitates the quantification of myocardial function independent of underlying morphology. These techniques offer new insights into the mechanics of CHD.