A standard echocardiographic and tissue Doppler study of morphological and functional findings in children with hypertrophic cardiomyopathy compared to those with left ventricular hypertrophy in the setting of Noonan and LEOPARD syndromes

2008 ◽  
Vol 18 (6) ◽  
pp. 575-580 ◽  
Author(s):  
Fabiana Cerrato ◽  
Giuseppe Pacileo ◽  
Giuseppe Limongelli ◽  
Maria Giulia Gagliardi ◽  
Giuseppe Santoro ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Hypertrophy ◽  
Right Ventricular ◽  
Surface Area ◽  
Coronary Arteries ◽  
Body Surface ◽  
Ventricular Hypertrophy ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract

AbstractBackgroundSeveral clinical and echocardiographic studies describe morphological and functional findings in patients with hypertrophic cardiomyopathy. Less is known regarding morphological and functional characteristics of the left ventricular hypertrophy found in the setting of the Noonan and LEOPARD syndromes.ObjectiveTo compare non-invasively the morphological and functional findings potentially affecting symptoms and clinical outcome in children with hypertrophic cardiomyopathy as opposed to Noonan and LEOPARD syndromes.Patients and methodsWe studied by echo-Doppler 62 children with left ventricular hypertrophy, dividing them into two subgroups matched for age and body surface area. The first group, of 45 patients with a mean age of 7.5 ± 5.2 years and body surface area of 0.9 ± 0.44 mq, had idiopathic hypertrophic cardiomyopathy. The second group, of 17 patients, all had left ventricular hypertrophy in the setting of Noonan or LEOPARD syndromes. Their mean age was 6.6 ± 5 years, and body surface area was 0.8 ± 0.36 mq. In all patients, we assessed the left ventricular maximal mural thickness, expressed as a Z-score, along with any obstructions in the left and right ventricular outflow tracts. In addition, to define left ventricular diastolic function, we used mitral flow and pulsed Tissue Doppler to record the Ea, Aa, Ea/Aa, E/Ea indexes in the apical 4-chamber view at the lateral corner of the mitral annulus. We also measured the diameters of the coronary arteries in the diastolic frame.ResultsCompared to those with hypertrophic cardiomyopathy, those with syndromic left ventricular hypertrophy showed a significantly increased Z-score for mural thickness, and a higher prevalence of obstruction in the left ventricular outflow tract. In addition, the patients with Noonan or LEOPARD syndromes showed a significantly decrease of Ea and increase of Aa, with a decreased Ea/Aa ratio, all suggestive of left ventricular abnormal relaxation. Moreover, the E/Ea ratio was significantly increased in these patients. The presence of right ventricular hypertrophy, mainly associated with dynamic obstruction in the outflow tract, was detected in only 5 of the 17 patients with Noonan or LEOPARD syndromes, as was dilation of the coronary arteries.ConclusionsCompared to children with hypertrophic cardiomyopathy, those with left ventricular hypertrophy in the setting of Noonan or LEOPARD syndromes show more ventricular hypertrophy and diastolic dysfunction, due to both abnormal relaxation and reduced compliance. They also exhibit an increased prevalence of obstruction of the left ventricular outflow tract, along with dynamic obstruction of the right ventricular outflow tract and dilated coronary arteries. These morphological and functional findings could explain the different symptoms and clinical events, and potentially define the more appropriate therapeutic options in children with left ventricular hypertrophy of different aetiology.

scholarly journals Lean body mass is the strongest anthropometric predictor of left ventricular mass in the obese paediatric population

2020 ◽  
Vol 30 (4) ◽  
pp. 476-481
Author(s):  
James R. Shea ◽  
Melissa H. Henshaw ◽  
Janet Carter ◽  
Shahryar M. Chowdhury
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Surface Area ◽  
Left Ventricular Mass ◽  
Lean Body Mass ◽  
Body Mass ◽  
Body Surface ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Obese Children ◽  
Ventricular Mass

AbstractBackground:Indexing left ventricular mass to body surface area or height2.7 leads to inaccuracies in diagnosing left ventricular hypertrophy in obese children. Lean body mass predictive equations provide the opportunity to determine the utility of lean body mass in indexing left ventricular mass. Our objectives were to compare the diagnostic accuracy of predicted lean body mass, body surface area, and height in detecting abnormal left ventricle mass in obese children.Methods:Obese non-hypertensive patients aged 4–21 years were recruited prospectively. Dual-energy X-ray absorptiometry was used to measure lean body mass. Height, weight, sex, race, and body mass index z-score were used to calculate predicted lean body mass.Results:We enrolled 328 patients. Average age was 12.6 ± 3.8 years. Measured lean body mass had the strongest relationship with left ventricular mass (R2 = 0.84, p < 0.01) compared to predicted lean body mass (R2 = 0.82, p < 0.01), body surface area (R2 = 0.80, p < 0.01), and height2.7 (R2 = 0.65, p < 0.01). Of the clinically derived variables, predicted lean body mass was the only measure to have an independent association with left ventricular mass (β = 0.90, p < 0.01). Predicted lean body mass was the most accurate scaling variable in detecting left ventricular hypertrophy (positive predictive value = 88%, negative predictive value = 99%).Conclusions:Lean body mass is the strongest predictor of left ventricular mass in obese children. Predicted lean body mass is the most accurate anthropometric scaling variable for left ventricular mass in left ventricular hypertrophy detection. Predicted lean body mass should be considered for clinical use as the body size correcting variable for left ventricular mass in obese children.

scholarly journals Peguero–Lo Presti criteria modified by body surface area for the electrocardiographic diagnosis of left ventricular hypertrophy in Thai patients

2021 ◽  
Vol 15 (2) ◽  
pp. 101-107
Author(s):  
Wongsakorn Luangphiphat ◽  
Atthaphon Phaisitkriengkrai ◽  
Ratikorn Methavigul ◽  
Komsing Methavigul
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Surface Area ◽  
Body Surface Area ◽  
Body Surface ◽  
Ventricular Hypertrophy ◽  
Characteristic Curve ◽  
Left Ventricular ◽  
Lv Mass ◽  
Low Sensitivity ◽  
Ecg Criteria

Abstract Background Several electrocardiographic (ECG) criteria are used to diagnose left ventricular hypertrophy (LVH); however, they have low sensitivity. Objective To assess the sensitivity of LVH diagnosis using Peguero–Lo Presti criteria modified by body surface area (BSA). Methods This study used retrospective data from 9,438 patients who attended the Central Chest Institute of Thailand from January 2017 to December 2017 with available echocardiography, and who were categorized into those with and without LVH to determine diagnostic accuracy. We randomly selected 317 patients after excluding others based on various conditions. The left ventricular mass of the 317 patients was estimated using echocardiography. Peguero–Lo Presti criteria were modified by dividing original criteria by BSA. The accuracy of the modified criteria was compared with that of the original Peguero–Lo Presti, Sokolow–Lyon, and Cornell voltage criteria. A McNemar test was used to determine the agreement of all ECG criteria examined with LV mass index. The area under a receiver operating characteristic curve (AUC) was used to assess the performance of the criteria. Results LVH was diagnosed in 164 of the 317 patients using echocardiography. The sensitivity of modified Peguero–Lo Presti criteria was 50.6% (95% confidence interval [CI] 42.7% to 58.5%), and specificity was 88.2% (95% CI 82.0% to 92.9%), with an AUC of 0.67 (95% CI 0.61–0.73). Conclusions Peguero–Lo Presti criteria modified by dividing them by BSA can improve sensitivity with acceptable specificity for the diagnosis of LVH compared with other ECG criteria examined, at least in selected Thai patients. The modified Peguero–Lo Presti criteria have accuracy similar to that for the original criteria.

Left Ventricular Hypertrophy in Adolescents with Elevated Blood Pressure: Assessment by Chest Roentgenography, Electrocardiography, and Echocardiography

PEDIATRICS ◽  
1981 ◽  
Vol 67 (2) ◽  
pp. 255-259
Author(s):  
W. Pennock Laird ◽  
David E. Fixler
Keyword(s):  
Blood Pressure ◽  
Left Ventricular Hypertrophy ◽  
Surface Area ◽  
Body Surface Area ◽  
Body Surface ◽  
Ventricular Hypertrophy ◽  
Left Ventricular ◽  
Elevated Blood ◽  
Control Subjects ◽  
Normotensive Control

The purpose of this study was to assess the prevalence of left ventricular hypertrophy in adolescents with persistently elevated blood pressures. Chest roentgenograms, electrocardiograms, and echocardiograms were performed on 50 adolescents with elevated blood pressure and 50 matched normotensive control subjects. No subject in either group demonstrated cardiomegaly on x-ray. Interpretation of the electrocardiograms indicated that similar numbers of both hypertensive (7/50) and control subjects (8/50) had ECG evidence of left ventricular hypertrophy. The echocardiograms showed that the mean left ventricular wall thickness (LVWT) in the hypertensive adolescents was 7.8 mm ± 0.1 (SE), compared with 6.5 ± 0.1 in the control subjects (P &lt; .001). When the measurements were indexed to body surface area, the difference remained highly significant. Indexed left ventricular mass (LVM)/body surface area (BSA) was also significantly greater (P &lt; .001) in the hypertensive (84.2 gm/sq m ± 2.1) than in the control subjects (72.0 ± 2.1). Using data from the normotensive control subjects, we defined the 95th percentile for both LVWT/BSA and LVM/BSA. Among hypertensive adolescents, 9/50 had LVWT/BSA and 8/50 had LVM/BSA above this level. For control subjects, only 1/50 had elevated LVWT/BSA values and 2/50 elevated LVM/BSA values. This study demonstrates that hypertensive adolescents have an increased prevalence of left ventricular hypertrophy and that echocardiography is the most useful noninvasive method to detect these changes.

Sign in / Sign up

Export Citation Format

Share Document