Biagio Castaldi

Two-dimensional strain to assess regional left and right ventricular longitudinal function in 100 normal foetuses

AIMSnPrevious reports have demonstrated that myocardial velocities are not sufficiently sensitive in foetal heart studies. Strain (S) imaging is a new non-invasive ultrasonic technique able to quantify regional myocardial deformation properties. Strain imaging has a superior sensitivity than myocardial velocity for non-invasive assessment of ventricular function. However, Doppler-derived strain imaging has been used to quantify myocardial deformation properties in the foetal heart with rather limited results, because of angle dependency, sensitivity to extracardiac movement, the need for good-quality images, long and time-consuming post-processing and the low reproducibility of Doppler-derived strain. Recently, a novel method for motion estimation based on two-dimensional (2D) tissue tracking strain (2D-S) echocardiography using time-domain processing has been developed, providing rapid assessment of regional myocardial strain that is independent of both cardiac translation and angle dependency, with a very good reproducibility. However, no information on 2D-S in human foetuses has so far been provided.nnnMETHODSnWe studied 100 consecutive normal foetuses (gestation range: 20-32 weeks; no evidence of structural cardiovascular disease by 2D echo and Doppler study) using 2D-S imaging. Left ventricle (LV) and right ventricle (RV) peak myocardial negative strain values were obtained.nnnRESULTSnStrain data were obtained from all the studied subjects, the duration of post processing was 3 +/- 2 min for each patient dataset. Peak longitudinal deformation parameters were homogeneous in all the three studied walls (strain: septum = -25 +/- 5%; lateral wall = -25 +/- 4%; RV free wall = -24 +/- 4%; P = NS). There were significant correlations between gestational age and peak longitudinal strain (P < 0.001; R: -0.73). Inter and intra-observer variability for strain was good, <3 and <6%, respectively.nnnCONCLUSIONnThis study demonstrated that 2D-S is a feasible and reproducible approach to assess regional ventricular function in the foetal heart, ready for the clinical application.

Abnormal regional myocardial deformation properties and increased aortic stiffness in normotensive patients with aortic coarctation despite successful correction: an ABPM, standard echocardiography and strain rate imaging study

The long-term follow-up data subsequent to a successful repair of AoC (aortic coarctation) show that life expectancy remains reduced. Previous standard echocardiographic studies have demonstrated normal or increased systolic cardiac function in patients following successful repair of AoC. SR (strain rate) imaging is a new technique able to detect subclinical myocardial abnormalities. In the present study we investigated whether young patients (without hypertension, as assessed using ambulatory blood pressure monitoring and an exercise test) following successful AoC repair already have abnormal myocardial deformation properties, and the relationship of the deformation properties with aortic stiffness. We studied 166 subjects, 83 AoC non-hypertensive patients (mean age 12+/-4 years) a number of years after successful repair of AoC and 83 age- and sex-matched subjects as controls. Peak systolic SR (1/s) for both regional longitudinal and radial function was assessed. The aortic stiffness index was calculated from the echocardiographically derived thoracic aortic diameters, and the measurement of blood pressure was obtained by cuff sphygmomanometry. The LV (left ventricular) ejection fraction was significantly increased in AoC patients, whereas regional longitudinal SRs were significantly reduced (-1.1+/-0.9 compared with -2+/-0.5, P<0.0001) in patients. The aortic stiffness index was significantly increased in AoC patients (12+/-9, P<0.0001). At multilinear regression analysis, age at repair (P=0.005; coefficient, -0.201; S.E.M., 0.027) and the aortic stiffness index (P=0.0029; coefficient, 0.334; S.E.M., 0.423) predicted longitudinal SR. Despite the presence of a successful repair for AoC, in the absence of hypertension, longitudinal deformation properties were significantly impaired. Moreover, the degree of longitudinal SR impairment was correlated with age at repair and aortic stiffness. Early repair can delay the onset of hypertension in postcoarctectomy patients, but cannot prevent the innate structural and functional abnormalities of the aorta and their deleterious effect on myocardial deformation properties.

Two-dimensional strain and atrial function: a study on patients after percutaneous closure of atrial septal defect.

AIMSnTo assess the value of two-dimensional (2D) strain in assessing regional myocardial function along the atrial wall.nnnMETHODS AND RESULTSnWe studied 20 patients late after successful percutaneous atrial septal defect (ASD) closure. The analysis was performed for atrial longitudinal peak systolic strain on the interatrial septum, in correspondence of the device, and on the lateral wall of the left atrium. The speckle tracking indexes demonstrated almost the absence of any deformation on the Amplatzer ASD occluder, a bulky non-contractile element, passively moved by global heart motion. This study in a simple clinical model demonstrates that 2D strain is not influenced by global heart motion and tethering from adjacent segments and can also be used to study the regional atrial function. Moreover, both acquisition and post-processing times of 2D strain were very short, and the reproducibility was very good.nnnCONCLUSIONnAll these above-mentioned characteristics make the 2D strain a tool fully compatible with the clinical scanning, able to provide additional clinical information.

Early Left Ventricular Abnormalities in Children with Heterozygous Familial Hypercholesterolemia

BACKGROUNDnFew data are available on cardiac morphology and function in children with heterozygous familial hypercholesterolemia (FH). Such patients represent a unique clinical model to assess the effect of pure hypercholesterolemia on cardiac morphology and function, excluding the effect of comorbidities. Speckle-tracking echocardiography, a relatively new echocardiographic modality, allows the assessment of myocardial deformation properties. The aim of this study was to define in children with FH the preclinical effects of isolated hypercholesterolemia on the cardiovascular system by examining left ventricular (LV) function using speckle-tracking echocardiography.nnnMETHODSnNinety children (45 with FH and 45 controls; mean age, 11 ± 3 years) were prospectively studied.nnnRESULTSnChildren with FH showed thicker LV walls and significantly higher LV mass indexed for height(2.7) (Pxa0= .0008) and for body surface area (P < .0001). LV ejection fractions were similar in both groups. Assessment of diastolic function demonstrated longer deceleration times (P < .0001), reduced early diastolic mitral annular velocities (P < .0001), and higher transmitral early/early diastolic mitral annular velocity ratios (Pxa0= .0003) in children with FH. Longitudinal and circumferential myocardial deformation of the left ventricle were significantly reduced (P < .0001) whereas radial deformation was increased in children with FH (Pxa0= .04) compared with controls.nnnCONCLUSIONSnThis study demonstrates that hypercholesterolemia is associated with significant LV morphologic and functional alterations during childhood. The findings also suggest that reductions in longitudinal and circumferential deformation are compensated for by increasing radial strain in children with FH with normal LV ejection fractions. This study raises the questions of the clinical importance of these findings and the opportunity for cholesterol-lowering therapy. The potential benefits and risks of such treatment at a young age need to be addressed in larger long-term studies.

Natural History and Clinical Outcome of ''Uncorrected'' Scimitar Syndrome Patients: a Multicenter Study of the Italian Society of Pediatric Cardiology

INTRODUCTION AND OBJECTIVESnTo analyze the clinical status of patients with uncorrected scimitar syndrome in a multicenter Italian study.nnnMETHODSnThe natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers).nnnRESULTSnThe median age at diagnosis was 1.05 years (range, 1 day-41 years). Thirty-three patients (75%) had an isolated form; 11 patients (25%) had associated congenital heart diseases. Twenty-two patients (50%) were symptomatic at diagnosis, including respiratory symptoms (n=20) and congestive heart failure (n=6). Patients with associated congenital heart defects had a higher prevalence of congestive heart failure (4 of 11 [36.4%] vs 2 of 33 [6.1%]; P=.027), pulmonary arterial hypertension (7 of 11 [63.6%] vs 2 of 33 [6.1%]; P=.027) than patients with isolated forms. Ten patients (22.7%) underwent correction of associated cardiac defects, leaving the anomalous pulmonary venous drainage intact. The median length of follow-up after diagnosis was 6.4 years (range, 0.2-27.5 years). Two patients died, both with associated cardiac defects and severe pulmonary arterial hypertension. Of 42 survivors, 39 (92.8%) were asymptomatic at the last follow-up visit; 3 patients still complained respiratory symptoms. There was no difference between isolated and associated forms of the disease.nnnCONCLUSIONSnIn most patients, scimitar syndrome presented as an isolated lesion with a benign outcome. Nonetheless, when associated with other cardiac defects and pulmonary arterial hypertension, there was an increased risk of congestive heart failure and mortality. Correction of associated cardiac defects (transforming associated into isolated forms), together with the therapeutic occlusion of anomalous arterial supply to the lung, led to a benign outcome comparable to that in primarily isolated forms.

Transcatheter Closure of Symptomatic Arterial Duct in Infants Younger Than 1 Year Old

Transcatheter closure is currently considered the first-choice therapeutic option in patent arterial duct (AD), although this approach remains challenging in younger patients. To evaluate feasibility, safety, and mid-term efficacy of percutaneous AD closure using different devices in symptomatic infants <12xa0months old. Among the 483 patients who underwent transcatheter AD closure at our institution from April 2000 to January 2012, 69 were <12xa0months old. All patients showed cardiac overload at echocardiography, and 72xa0% of them were on anticongestive therapy. Based on ductal morphology and local anatomy, AD closure was attempted using detachable coils in 24 cases (group A) or the Amplatzer Duct Occluder (AGA Medical, Golden Valley, MN) device in 45 cases (group B). Procedural feasibility was 95.7xa0% without any difference between groups (95.8xa0% vs. 95.6xa0%, pxa0=xa0NS) as were fluoroscopy and procedural times. AD size was 2.5xa0±xa00.9xa0mm, and QP/QS ratio was 2.2xa0±xa01.1, with both being significantly higher in group B [2.1xa0±xa00.9 vs. 2.6xa0±xa00.9xa0mm (pxa0=xa00.04) and 1.7xa0±xa00.8 vs. 2.5xa0±xa01.2 (pxa0=xa00.009), respectively]. Overall complication rate was 1.5xa0%, without any difference between groups, as was the occlusion rate at hospital discharge (86xa0%) and over midterm follow-up (55xa0±xa036xa0months) (98.5xa0%). Percutaneous closure of symptomatic AD might be considered effective and safe in very young infants by tailoring the device choice to ductal morphology and local anatomy. In this setting, the controlled-release coil option was shown to be as effective as the ADO device during midterm follow-up.

Over‐the‐wire‐technique device implantation

Transcatheter closure of cardiac defects is a mainstay of treatment in congenital and structural heart disease. Occasionally, the devices used are useful in nonstandard or difficult positions but device embolization and malposition can be complicating factors necessitating percutaneous retrieval or emergency surgery. We describe a new “over‐the‐wire” technique that allows guided safe deployment and easy retrievability if required.

Impact of Obesity on Left Ventricular Geometry and Function in Pediatric Patients after Successful Aortic Coarctation Repair

Aims: To evaluate if obesity has an additional negative impact on left ventricular (LV) geometry and function in normotensive pediatric patients >12 months after successful treatment of aortic coarctation (CoA). Methods and Results: We studied 40 CoA patients (mean age 14 ± 3 years, and male sex 70%), of them 10 were obese and 30 lean. Both groups were age and sex comparable. The entire studied sample underwent 24‐ambulatory blood pressure (BP) monitoring, standard echocardiographic evaluation, and speckle tracking study. Both office and 24‐hour diastolic BP were significantly increased in obese patients. Obese CoA patients showed increased LV mass (52 ± 13 g/m2.7 vs. 43 ± 9 g/m2.7, P = 0.02), and significant reduction in E/A compared with lean CoA patients. Myocardial deformation properties were significantly reduced in obese CoA patients in all the three studied planes (longitudinal, radial, and circumferential) compared with CoA lean patients. LV twist values showed a significant reduction in the obese CoA group (9.9°± 2.2° vs. 14.5°± 2.3°, P < 0.0001). Conclusions: Our study shows that obesity in successfully treated CoA children, has an additional negative effect on BP, LV mass, and cardiac function. These findings are of particular concern, since life expectancy in CoA patients is limited mainly by atherosclerosis, and all the obesity‐associated abnormalities found are harbingers of higher cardiovascular risk. (Echocardiography 2011;28:907‐912)