Structural Measurements and Adjustments for Growth

A Validated Model for Sudden Cardiac Death Risk Prediction in Pediatric Hypertrophic Cardiomyopathy

“…This was a multicenter retrospective observational cohort study of pediatric patients with clinically diagnosed childhood-onset HCM. Eligibility criteria included clinical diagnosis of HCM, LV posterior wall or septal thickness z score ≥2, 29 , 30 age <18 years at the time of diagnosis, absence of a SCD event before diagnosis, and at least 1 follow-up assessment after diagnosis. Genotype-positive subjects who did not have echocardiographic evidence of LV hypertrophy and patients with known or suspected secondary causes of HCM, ie, clinical syndromes like RASopathies (Noonan syndrome, Noonan syndrome with multiple lentigines, Costello syndrome, Cardiofaciocutaneous syndrome, and Neurofibromatosis type 1), endocrine, metabolic, mitochondrial, or neuromuscular disorders, hypertension, and structural heart defects were excluded.…”

“… 4 Echocardiographic measurements of wall thickness and chamber sizes were converted to z scores using the Boston z score calculator. 29 , 30 The risk factors included age at diagnosis, family history of SCD, history of recent unexplained syncope within 6 months before the diagnosis, documented nonsustained VT (defined as ≥3 beats at ≥120 bpm on ambulatory ECG), interventricular septal diameter (IVSD) z score, LV posterior wall diameter (LVPWD) z score, left atrial (LA) diameter z score, and peak resting LV outflow tract (LVOT) gradient on echocardiography. Genetic results from clinical testing were captured from medical records and, through research sequencing, in a subset of patients.…”

The impact of fetal endoscopic tracheal occlusion in isolated left‐sided congenital diaphragmatic hernia on left‐sided cardiac dimensions

“…with expertise in echocardiographic imaging who were blinded to the study hypothesis, treatment assignment, and postnatal outcomes. Fetal echocardiogram measurements were indexed for gestational age, while postnatal echocardiogram measurements were indexed for body surface area; both reported as Z scores using available normative data collected from fetuses without CDH . The following fetal cardiac measurements were evaluated: aortic valve, mitral valve, LV long‐axis dimension (LVLAD), LV short‐axis dimension (LVSAD), right ventricular myocardial performance index (RV MPI), left ventricular myocardial performance index (LV MPI), and indexed cardiac outputs for all cardiac valves.…”

Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes

“…All two‐dimensional echocardiographic measurements were collected at the time of the study by one of two observers (pediatric cardiologist or sonographer) with expertise in fetal cardiovascular imaging who were blinded to patient outcomes. These values were indexed to gestational age and body surface area and reported as z‐scores using available normative data collected from fetuses unaffected by CDH . In cases where multiple fetal and postnatal echocardiograms were performed, the earliest fetal echocardiogram and earliest postnatal (pre‐CDH repair) echocardiogram was used.…”

“…These values were indexed to gestational age and body surface area and reported as z-scores using available normative data collected from fetuses unaffected by CDH. [40][41][42] In cases where multiple fetal and postnatal echocardiograms were performed, the earliest fetal echocardiogram and earliest postnatal (pre-CDH repair) echocardiogram was used. Every effort was made to use postnatal echocardiographic data performed when the neonate's hemodynamics were not significantly altered by external devices (i.e.…”