Adel K. Younoszai

Recommendations for Quantification Methods During the Performance of a Pediatric Echocardiogram: A Report From the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council

n Society of Echocardiography designates this educational activity for of 1.5 AMA PRA Category 1 Credits . Physicians should only claim credit te with the extent of their participation in the activity. CCI recognize the ASE’s certificates and have agreed to honor the credit their registry requirements for sonographers. n Society of Echocardiography is committed to ensuring that its educan and all sponsored educational programs are not influenced by the special y corporation or individual, and itsmandate is to retain only those authors ial interests can be effectively resolved to maintain the goals and educaty of the activity. Although amonetary or professional affiliationwith a cors not necessarily influence an author’s presentation, the Essential Areas and e ACCME require that any relationships that could possibly conflict with al value of the activity be resolved prior to publication and disclosed to . Disclosures of faculty and commercial support relationships, if any, dicated. ience: is designed for all cardiovascular physicians and cardiac sonographers with erest and knowledge base in the field of echocardiography; in addition, reschers, clinicians, intensivists, and other medical professionals with a spein cardiac ultrasound will find this activity beneficial.

Clinical Utility of Echocardiography for the Diagnosis and Management of Pulmonary Vascular Disease in Young Children With Chronic Lung Disease

OBJECTIVE. The goal was to determine the clinical utility of Doppler echocardiography in predicting the presence and severity of pulmonary hypertension in patients with chronic lung disease who subsequently underwent cardiac catheterization. METHODS. A retrospective review of data for all patients <2 years of age with a diagnosis of bronchopulmonary dysplasia, congenital diaphragmatic hernia, or lung hypoplasia who underwent echocardiography and subsequently underwent cardiac catheterization for evaluation of pulmonary hypertension was performed. The accuracy of echocardiography in diagnosing pulmonary hypertension, on the basis of estimated systolic pulmonary artery pressure, was compared with the detection of pulmonary hypertension with the standard method of cardiac catheterization. RESULTS. Thirty-one linked measurements for 25 children were analyzed. Systolic pulmonary artery pressure could be estimated in 61% of studies, but there was poor correlation between echocardiography and cardiac catheterization measures of systolic pulmonary artery pressure in these infants. Compared with cardiac catheterization measurements, echocardiographic estimates of systolic pulmonary artery pressure diagnosed correctly the presence or absence of pulmonary hypertension in 79% of the studies in which systolic pulmonary artery pressure was estimated but determined the severity of pulmonary hypertension (severe pulmonary hypertension was defined as pulmonary/systemic pressure ratio of ≥0.67) correctly in only 47% of those studies. Seven (58%) of 12 children without estimated systolic pulmonary artery pressure demonstrated pulmonary hypertension during subsequent cardiac catheterization. In the absence of estimated systolic pulmonary artery pressure, qualitative echocardiographic findings, either alone or in combination, had worse predictive value for the diagnosis of pulmonary hypertension. CONCLUSION. As used in clinical practice, echocardiography often identifies pulmonary hypertension in young children with chronic lung disease; however, estimates of systolic pulmonary artery pressure were not obtained consistently and were not reliable for determining the severity of pulmonary hypertension.

Early Pulmonary Vascular Disease in Preterm Infants at Risk for Bronchopulmonary Dysplasia

RATIONALE Pulmonary hypertension (PH) is associated with poor outcomes among preterm infants with bronchopulmonary dysplasia (BPD), but whether early signs of pulmonary vascular disease are associated with the subsequent development of BPD or PH at 36 weeks post-menstrual age (PMA) is unknown. OBJECTIVES To prospectively evaluate the relationship of early echocardiogram signs of pulmonary vascular disease in preterm infants to the subsequent development of BPD and late PH (at 36 wk PMA). METHODS Prospectively enrolled preterm infants with birthweights 500-1,250 g underwent echocardiogram evaluations at 7 days of age (early) and 36 weeks PMA (late). Clinical and echocardiographic data were analyzed to identify early risk factors for BPD and late PH. MEASUREMENTS AND MAIN RESULTS A total of 277 preterm infants completed echocardiogram and BPD assessments at 36 weeks PMA. The median gestational age at birth and birthweight of the infants were 27 weeks and 909 g, respectively. Early PH was identified in 42% of infants, and 14% were diagnosed with late PH. Early PH was a risk factor for increased BPD severity (relative risk, 1.12; 95% confidence interval, 1.03-1.23) and late PH (relative risk, 2.85; 95% confidence interval, 1.28-6.33). Infants with late PH had greater duration of oxygen therapy and increased mortality in the first year of life (P < 0.05). CONCLUSIONS Early pulmonary vascular disease is associated with the development of BPD and with late PH in preterm infants. Echocardiograms at 7 days of age may be a useful tool to identify infants at high risk for BPD and PH.

RV stroke work in children with pulmonary arterial hypertension: estimation based on invasive haemodynamic assessment and correlation with outcomes

Background RV performance is an important determinant of outcomes in children with pulmonary arterial hypertension (PAH). RV stroke work (RVSW), the product of mean pulmonary artery pressure and stroke volume, integrates contractility, afterload and ventricular-vascular coupling. RVSW has not been evaluated in children with PAH. We tested the hypothesis that RVSW would be a predictor of outcomes in children with PAH. Methods Patients in the Childrens Hospital Colorado PAH database were evaluated retrospectively, and those with idiopathic PAH and those with minor or repaired congenital heart disease were included. Haemodynamic data were obtained by catheterisation and echocardiography, performed within 3 months. RVSW was calculated: mean pulmonary arterial pressure × stroke volume, and indexed to body surface area. Statistics included Kruskal–Wallis, Wilcoxon rank sum, and Spearman correlation. Results Fifty patients were included. Median age of the cohort was 9.5 (6.0, 15.7) years, with a median indexed pulmonary vascular resistance (PVRi) of 6.5 (3.7, 11.6) WU m2. RVSW had a significant association with PVRi (r=0.6, p<0.0001), tricuspid annular systolic plane excursion (r=0.55, p=0.0001), and RV fractional area change (r=−0.4, p=0.005). Grouped by WHO class, there was a significant difference in RVSW (p=0.04). Need for atrial septostomy and death were associated with higher RVSW (p=0.04 and p=0.03, respectively). Conclusions RVSW can be estimated in children with PAH, and is significantly associated with abnormal WHO class, the need for septostomy, as well as mortality. Indices accounting for RV performance as well as ventricular-vascular coupling may be useful in the prognosis and, hence, management of children with PAH.

Left ventricular strain and strain rates are decreased in children with normal fractional shortening after exposure to anthracycline chemotherapy.

BACKGROUND Anthracycline chemotherapeutic agents carry the well-recognised risk of cardiotoxicity. Previous methods to evaluate cardiac function are useful, but have significant limitations. We sought to determine the left ventricular strain and strain rate of paediatric cancer patients with normal fractional shortening treated with anthracyclines using the latest ultrasound feature-tracking technology. PATIENTS AND METHODS Echocardiograms on cancer patients before anthracycline exposure and following completion of treatment were retrospectively analysed using Velocity Vector Imaging software in the circumferential and longitudinal planes. The same analysis was performed on matched controls. Only patients with a fractional shortening ≥28% were included. RESULTS In all, 71 patients were identified with an age at diagnosis of 10.5 ± 4.7 years. The time from diagnosis to follow-up was 3.9 ± 4.0 years and the cumulative anthracycline dose was 356 ± 106 mg/m². Following anthracycline exposure, paediatric cancer patients had a higher heart rate and a lower longitudinal strain, longitudinal diastolic strain rate, circumferential strain, and circumferential systolic and diastolic strain rate when compared with controls. Diastolic strain rate showed the greatest percent difference following anthracycline exposure versus controls. CONCLUSION Despite having a normal fractional shortening, children exposed to anthracyclines have subclinical derangement of their left ventricular deformation as measured by decreases in strain and strain rate in both the circumferential and longitudinal axis. In particular, there was a profound decrease in diastolic strain rate following anthracycline exposure compared with controls. Whether the decline of strain or strain rate can predict future risk of developing cardiomyopathy requires further investigation.

Impact of Pulmonary Hemodynamics and Ventricular Interdependence on Left Ventricular Diastolic Function in Children With Pulmonary Hypertension.

Background—Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that pediatric PH patients have LV diastolic dysfunction, related to adverse pulmonary hemodynamics, leftward septal shift, and prolonged right ventricular systole. Methods and Results—Echocardiography was prospectively performed at 2 institutions in 54 pediatric PH patients during cardiac catheterization and in 54 matched controls. Diastolic LV measures including myocardial deformation were assessed by echocardiography. PH patients had evidence of LV diastolic dysfunction, most consistent with impaired LV relaxation, though some features of reduced ventricular compliance were present. PH patients demonstrated the following: reduced mitral E velocity and inflow duration, mitral E′ and E′/A′, septal E′ and A′, pulmonary vein S and D wave velocities, and LV basal global early diastolic circumferential strain rate and increased mitral E deceleration time, LV isovolumic relaxation time, mitral E/E′, and pulmonary vein A wave duration. PH patients demonstrated leftward septal shift and prolonged right ventricular systole, both known to affect LV diastole. These changes were exacerbated in severe PH. There were no statistically significant differences in diastolic measures between patients with and without a shunt and minimal differences between patients with and without congenital heart disease. Multiple echocardiographic LV diastolic parameters demonstrated weak-to-moderate correlations with invasively determined PH severity, leftward septal shift, and prolonged right ventricular systole. Conclusions—Pediatric PH patients exhibit LV diastolic dysfunction most consistent with impaired relaxation and reduced myocardial deformation, related to invasive hemodynamics, leftward septal shift, and prolonged right ventricular systole.

Left Ventricular Myocardial Function in Children With Pulmonary Hypertension Relation to Right Ventricular Performance and Hemodynamics

Background— Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that LV strain/strain rate, surrogate measures of myocardial contractility, are reduced in pediatric PH and relate to invasive hemodynamics, right ventricular strain, and functional measures of PH. Methods and Results— At 2 institutions, echocardiography was prospectively performed in 54 pediatric PH patients during cardiac catheterization, and in 54 matched controls. Patients with PH had reduced LV global longitudinal strain (LS; −18.8 [−17.3 to −20.4]% versus −20.2 [−19.0 to −20.9]%; P =0.0046) predominantly because of reduced basal (−12.9 [−10.8 to −16.3]% versus −17.9 [−14.5 to −20.7]%; P <0.0001) and mid (−17.5 [−15.5 to −19.0]% versus −21.1 [−19.1 to −23.0]%; P <0.0001) septal strain. Basal global circumferential strain was reduced (−18.7 [−15.7 to −22.1]% versus −20.6 [−19.0 to −22.5]%; P =0.0098), as were septal and free-wall segments. Mid circumferential strain was reduced within the free-wall. Strain rates were reduced in similar patterns. Basal septum LS, the combined average LS of basal and mid interventricular septal segments, correlated strongly with degree of PH ( r =0.66; P <0.0001), pulmonary vascular resistance ( r =0.60; P <0.0001), and right ventricular free-wall LS ( r =0.64; P <0.0001). Brain natriuretic peptide levels correlated moderately with septal LS ( r =0.48; P =0.0038). PH functional class correlated moderately with LV free-wall LS ( r =−0.48; P =0.0051). The septum, shared between ventricles and affected by septal shift, was the most affected LV region in PH. Conclusions— Pediatric PH patients demonstrate reduced LV strain/strain rate, predominantly within the septum, with relationships to invasive hemodynamics, right ventricular strain, and functional PH measures.Background—Through ventricular interdependence, pulmonary hypertension (PH) induces left ventricular (LV) dysfunction. We hypothesized that LV strain/strain rate, surrogate measures of myocardial contractility, are reduced in pediatric PH and relate to invasive hemodynamics, right ventricular strain, and functional measures of PH. Methods and Results—At 2 institutions, echocardiography was prospectively performed in 54 pediatric PH patients during cardiac catheterization, and in 54 matched controls. Patients with PH had reduced LV global longitudinal strain (LS; −18.8 [−17.3 to −20.4]% versus −20.2 [−19.0 to −20.9]%; P=0.0046) predominantly because of reduced basal (−12.9 [−10.8 to −16.3]% versus −17.9 [−14.5 to −20.7]%; P<0.0001) and mid (−17.5 [−15.5 to −19.0]% versus −21.1 [−19.1 to −23.0]%; P<0.0001) septal strain. Basal global circumferential strain was reduced (−18.7 [−15.7 to −22.1]% versus −20.6 [−19.0 to −22.5]%; P=0.0098), as were septal and free-wall segments. Mid circumferential strain was reduced within the free-wall. Strain rates were reduced in similar patterns. Basal septum LS, the combined average LS of basal and mid interventricular septal segments, correlated strongly with degree of PH (r=0.66; P<0.0001), pulmonary vascular resistance (r=0.60; P<0.0001), and right ventricular free-wall LS (r=0.64; P<0.0001). Brain natriuretic peptide levels correlated moderately with septal LS (r=0.48; P=0.0038). PH functional class correlated moderately with LV free-wall LS (r=−0.48; P=0.0051). The septum, shared between ventricles and affected by septal shift, was the most affected LV region in PH. Conclusions—Pediatric PH patients demonstrate reduced LV strain/strain rate, predominantly within the septum, with relationships to invasive hemodynamics, right ventricular strain, and functional PH measures.

Preliminary Data on the Diagnostic Accuracy of Rubidium-82 Cardiac PET Perfusion Imaging for the Evaluation of Ischemia in a Pediatric Population

Evaluation of myocardial perfusion is sometimes necessary in children with congenital heart disease or acquired coronary artery abnormalities. Limited information is available regarding the clinical utility of myocardial perfusion imaging in children. PET imaging with rubidium-82 may provide a convenient clinical means of assessing regional circulatory compromise in pediatric patients with small hearts, due to its improved spatial resolution. Clinically indicated cardiac PET studies obtained in 22 pediatric patients were reviewed by two blinded observers and assigned myocardial perfusion scores using a standard 17-segment model. PET results were correlated with coronary angiography, available in 15 cases, to determine the accuracy of PET scanning for evaluating compromise of the myocardial circulation. Reversible defects consistent with myocardial ischemia were present in 6 of 15 (40%) PET cases. The sensitivity and specificity of cardiac PET for the detection of significant coronary artery disease were 100% and 82%, respectively. The positive predictive value of cardiac PET was 67%, while the negative predictive value was 100%. Cardiac PET imaging with rubidium-82 appears promising for the noninvasive assessment of myocardial perfusion in the pediatric population. The findings from this small series suggest that prospective study in a larger patient cohort merits consideration.

Reliability of Echocardiographic Indicators of Pulmonary Vascular Disease in Preterm Infants at Risk for Bronchopulmonary Dysplasia

Objectives To determine the assessment and inter‐rater reliability of echocardiographic evaluations of pulmonary vascular disease (PVD) in preterm infants at risk for bronchopulmonary dysplasia. Study design We prospectively studied echocardiograms from preterm infants (birthweights 500‐1250 g) at 7 days of age and 36 weeks postmenstrual age (PMA). Echocardiograms were assessed by both a cardiologist on clinical service and a single research cardiologist. Interpretations were reviewed for inclusion of determinants of PVD and assessed for inter‐rater reliability using the Prevalence Adjusted Bias Adjusted Kappa Score (PABAK). Results One hundred eighty and 188 matching research and clinical echocardiogram reports were available for the 7‐day and 36‐week PMA studies. At least one of the specific qualitative measures of PVD was missing from 54% of the clinical reports. PVD was diagnosed at 7 days in 31% and 20% of research and clinical interpretations, respectively (PABAK score of 0.54). At 36 weeks, PH was diagnosed in 15.6% and 17.8% of research and clinical interpretations, respectively (PABAK score of 0.80). Conclusions Although all qualitative variables of PVD are not consistently provided in echocardiogram reports, the inter‐rater reliability of cardiologists evaluating measures of PVD revealed strong agreement, especially at 36 weeks PMA. We speculate that establishment of a protocol for echocardiographic evaluation may improve the identification of PVD in preterm infants.

Tricuspid regurgitant jet velocity and hospitalization in Tanzanian children with sickle cell anemia

Elevated tricuspid regurgitant velocity (TRV) measured by echocardiogram predicts death in adult sickle cell anemia (SCA)[1][1]–[4][2] and occurs in children,[5][3]–[9][4] although etiology and association with survival and complications requiring hospitalization are unknown. In children,