title: Left ventricular remodeling, mechanics, and tissue characterization in congenital aortic stenosis.
abstract: BACKGROUND
  As the response of the myocardium to pressure overload is age-dependent, this study was designed to examine left ventricular (LV) remodeling, mechanics, and tissue characterization in children with moderate congenital aortic stenosis.
  
  
  METHODS
  We studied by echocardiography Doppler 22 patients (mean age 12.4 +/- 5.6 years) with peak and mean transvalvular gradient of 63 +/- 6 and 32 +/- 4 mm Hg, respectively. In addition, 30 age- and body surface area-matched participants with structurally normal hearts were used as a control group. Sex- and age-specific cut-off levels for LV mass/height(2.7) and relative wall thickness were defined to assess LV geometry. As a load-independent index of myocardial contractility, the relation between the rate-corrected velocity of circumferential fiber shortening both at endocardium and midwall, and meridional end-systolic stress was assessed. In addition, LV diastolic function was also evaluated by the mitral flow indexes. Finally, ultrasonic tissue characterization of the LV myocardium was performed by calculating the magnitude of cyclic variation, which reflects the intramural contractile function, and the averaged myocardial intensity of integrated backscatter, which is directly related to the myocardium collagen content.
  
  
  RESULTS
  The endocardial velocity of circumferential fiber shortening endocardium and meridional end-systolic stress relationship was within the normal range (mean +/- 2SD) in 18 of 22 patients (81.8%), and midwall velocity of circumferential fiber shortening at endocardium and meridional end-systolic stress was normal in all 22 patients. No mitral flow index of LV diastolic function was significantly different between aortic stenosis group and normal participants. In our study population, 16 of 22 patients (72.7%) showed normal LV geometry, 3 (13.6%) had a pattern of concentric remodeling, and 3 (13.6%) concentric hypertrophy. LV hypertrophy was not marked (left ventricular mass index [LVMI] < 51 g/m(2.7)) in any patient. Finally, compared with control participants our study population showed, both at interventricular septum and posterior wall, comparable values of cyclic variation integrated backscatter, but significantly higher values of averaged myocardial integrated backscatter intensity (P <.01).
  
  
  CONCLUSIONS
  In children with moderate congenital aortic stenosis, the total amount of myocardial collagen was increased despite normal LV myocardial contractility and diastolic function. Furthermore, LV remodeling was abnormal in only about a quarter of our patients and none had more than mild hypertrophy. Although the majority of these patients do not have markers now recognized to predict higher risk of cardiovascular events, the long-term significance of myocardial fibrosis and its response to treatment remain to be investigated.
authors.affiliation: Pediatric Cardiology Division, 2 Degrees University, Monaldi Hospital. gpacile@tin.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Pacileo
authors.forename: Paolo
authors.initials: P
authors.lastname: Calabrò
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Limongelli
authors.forename: Maria Giovanna
authors.initials: MG
authors.lastname: Russo
authors.forename: Carlo
authors.initials: C
authors.lastname: Pisacane
authors.forename: Berardo
authors.initials: B
authors.lastname: Sarubbi
authors.forename: Raffaele
authors.initials: R
authors.lastname: Calabrò
mesh_terms: D000293:Adolescent; D001021:Aortic Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002648:Child; D002669:Child Welfare; D015331:Cohort Studies; D003971:Diastole / Q000502:physiology; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006346:Heart Septum / Q000000981:diagnostic imaging / Q000503:physiopathology; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging / Q000503:physiopathology; D007558:Italy / Q000453:epidemiology; D008297:Male; D008943:Mitral Valve / Q000000981:diagnostic imaging / Q000503:physiopathology; D009200:Myocardial Contraction / Q000502:physiology; D012720:Severity of Illness Index; D013318:Stroke Volume / Q000502:physiology; D016277:Ventricular Function, Left / Q000502:physiology; D020257:Ventricular Remodeling / Q000502:physiology*