Pei-Ni Jone

Tissue Doppler Imaging Predicts Adverse Outcome in Children with Idiopathic Pulmonary Arterial Hypertension

OBJECTIVE To evaluate the clinical utility of tissue Doppler imaging (TDI) in assessment of disease severity and prognostic value in children with idiopathic pulmonary arterial hypertension (PAH). STUDY DESIGN A prospective study was performed to evaluate TDI velocities (systolic myocardial velocity, early diastolic myocardial relaxation velocity [Em], late diastolic myocardial velocity associated with atrial contraction), brain natriuretic peptide, New York Heart Association (NYHA) functional class, and hemodynamics in 51 children (mean age; 11.6 years) with idiopathic PAH. Fifty-one healthy children with comparable demographics served as controls. RESULTS Em, Em/late diastolic myocardial velocity associated with atrial contraction ratio, and systolic myocardial velocity at mitral annulus, septum, and tricuspid annulus in PAH were significantly reduced compared with controls. Tricuspid Em had significant inverse correlations with plasma brain natriuretic peptide levels (r = -0.60, P < .001), right ventricular end-diastolic pressure (r = -0.79, P < .001), and mean pulmonary arterial pressure (r = -0.67, P < .001). Statistically significant differences were observed in tricuspid Em between NYHA functional class II vs combined III and IV (mean and SD; 11.9 ± 4.2 cm/s vs 8.2 ± 3.6 cm/s, respectively, P = .002). Cumulative event-free survival rate was significantly lower when tricuspid Em was ≤8 cm/s (log-rank test, P < .001) CONCLUSIONS Tricuspid Em velocity correlated with NYHA functional class as disease severity and may serve as a useful prognostic marker in children with idiopathic PAH. The present study is the initial report to evaluate TDI velocities against midterm outcome variables in a relatively large pediatric PAH population.

Echocardiography in Pediatric Pulmonary Hypertension

Pulmonary hypertension (PH) can be a rapidly progressive and fatal disease. Although right heart catheterization remains the gold standard in evaluation of PH, echocardiography remains an important tool in screening, diagnosing, evaluating, and following these patients. In this article, we will review the important echocardiographic parameters of the right heart in evaluating its anatomy, hemodynamic assessment, systolic, and diastolic function in children with PH.

Prenatal diagnosis of congenital heart disease.

This article presents advancements in the field of fetal echocardiography and the significant impact of these within the fields of pediatric cardiology, perinatology, and neonatology. A prenatal diagnosis of congenital heart disease allows for improved counseling of the parents, guides the timing and optimal location of delivery, and allows appropriate planning and consultation between the cardiologist and neonatologist. It also facilitates accurate diagnosis and management of fetal arrhythmias, identifies potential candidates for in utero cardiac intervention, and serves as the imaging guidance technique for these procedures. The goals, indications, advantages, limitations, and spectrum of congenital heart disease that can be diagnosed are reviewed.

Concurrent Respiratory Viruses and Kawasaki Disease

BACKGROUND: The diagnosis of Kawasaki disease (KD) remains challenging without a definitive diagnostic test and currently is guided by using clinical patient characteristics and supported by laboratory data. The role of respiratory viruses in the pathogenesis of KD is not fully understood. METHODS: Charts of patients with KD admitted to Children’s Hospital Colorado from January 2009 to May 2013 were retrospectively reviewed. Patients with KD who had a nasopharyngeal wash submitted for multiplex polymerase chain reaction (PCR) viral testing were included. Clinical characteristics, laboratory data, and outcomes of patients with and without positive respiratory viral PCR results were compared. RESULTS: Of 222 patients with KD admitted to the hospital, 192 (86%) had a respiratory viral PCR test performed on or shortly after admission. Ninety-three (41.9%) of the 192 patients with KD had a positive respiratory viral PCR, and the majority were positive for rhinovirus/enterovirus. No statistically significant differences were found in the clinical characteristics and laboratory values between the groups with and without positive respiratory viral PCR findings. Both groups had the same frequency of upper respiratory and gastrointestinal symptoms and had the same incidence of admission to the PICU, intravenous immunoglobulin–resistant disease, and coronary artery lesions. CONCLUSIONS: No differences in clinical presentations or outcomes in children with KD stratified according to positive or negative respiratory viral PCR testing were observed. A positive respiratory viral PCR or presence of respiratory symptoms at the time of presentation should not be used to exclude a diagnosis of KD.

Three-dimensional Echocardiography of Right Ventricular Function Correlates with Severity of Pediatric Pulmonary Hypertension.

BACKGROUND Right ventricular function and biomarkers of B-type natriuretic peptide (BNP) and N-Terminal pro-BNP (NT pro-BNP) are used to determine the severity of right ventricular failure and outcomes from pulmonary hypertension. Real-time three-dimensional echocardiography (3DE) is a novel quantitative measure of the right ventricle and decreases the geometric assumptions from conventional two-dimensional echocardiography (2DE). We correlated right ventricular functional measures using 2DE and single-beat 3DE with biomarkers and hemodynamics to determine the severity of pediatric pulmonary hypertension. METHODS We retrospectively evaluated 35 patients (mean age 12.67 ± 5.78 years) with established pulmonary hypertension who had echocardiograms and biomarkers on the same day. Ten out of 35 patients had hemodynamic evaluation within 3 days. 2DE evaluation included tricuspid annular plane systolic excursion (TAPSE), right ventricular myocardial performance index from tissue Doppler imaging (RV TDI MPI), and right ventricular fractional area change (FAC). Three-dimensional echocardiography evaluation included right ventricular ejection fraction (EF), end-systolic volume, and end-diastolic volume. The quality of the 3DE was graded as good, fair, or poor. Pearson correlation coefficients were utilized to evaluate between biomarkers and echocardiographic parameters and between hemodynamics and echocardiography. RESULTS Three-dimensional echocardiography and FAC correlated significantly with BNP and NT pro-BNP. TAPSE and RV TDI MPI did not correlate significantly with biomarkers. 3D right ventricular EF correlated significantly with hemodynamics. Two-dimensional echocardiography did not correlate with hemodynamics. CONCLUSIONS Single-beat 3DE is a noninvasive, feasible tool in the quantification of right ventricular function and maybe more accurate than conventional 2DE in evaluating severity of pulmonary hypertension.

Right Atrial Deformation in Predicting Outcomes in Pediatric Pulmonary HypertensionCLINICAL PERSPECTIVE

Background— Elevated right atrial (RA) pressure is a risk factor for mortality, and RA size is prognostic of adverse outcomes in pulmonary hypertension (PH). There is limited data on phasic RA function (reservoir, conduit, and pump) in pediatric PH. We sought to evaluate (1) the RA function in pediatric PH patients compared with controls, (2) compare the RA deformation indices with Doppler indices of diastolic dysfunction, functional capacity, biomarkers, invasive hemodynamics, and right ventricular functional indices, and (3) evaluate the potential of RA deformation indices to predict clinical outcomes. Methods and Results— Sixty-six PH patients (mean age 7.9±4.7 years) were compared with 36 controls (7.7±4.4 years). RA and right ventricular deformation indices were obtained using 2-dimensional speckle tracking (2DCPA; TomTec, Germany). RA strain, strain rates, emptying fraction, and right ventricular longitudinal strain were measured. RA function was impaired in PH patients versus controls (P<0.001). There were significant associations between RA function with invasive hemodynamics (P<0.01). RA reservoir, pump function, the rate of RA filling, and atrial minimum volume predicted adverse clinical outcomes (hazard ratio [HR], 0.15; confidence interval [CI], 0.03–0.73; P<0.01; HR, 0.05; CI, 0.003–0.43; P<0.004; HR, 0.04; CI, 0.006–0.56; P<0.01; and HR, 8.6; CI, 1.6–37.2; P<0.01, respectively). Conclusions— RA deformation properties are significantly altered in pediatric PH patients. Progressive worsening of RA reservoir and conduit functions is related to changes in right ventricular diastolic dysfunction. RA reservoir function, pump function, the rate of atrial filling, and atrial minimum volume emerged as outcome predictors in pediatric PH.

Procalcitonin (PCT) and Kawasaki Disease: Does PCT Correlate With IVIG-Resistant Disease, Admission to the Intensive Care Unit, or Development of Coronary Artery Lesions?

BACKGROUND Kawasaki disease (KD) remains a clinical diagnosis due to the absence of a sensitive and specific diagnostic test. There are limited published data on the usefulness of procalcitonin (PCT) as a biomarker for the diagnosis or prognosis of children with KD. We hypothesized that PCT might be useful in predicting coronary artery lesions (CALs) and intravenous immunoglobulin (IVIG) resistance. METHODS Eighty-five children with KD who were hospitalized within the first 10 days of illness were retrospectively reviewed. PCT was measured on stored serum or plasma samples obtained at the time of admission (pre-IVIG). Data were analyzed to determine whether there were statistically significant associations with PCT, erythrocyte sedimentation rate, and C-reactive protein levels and the incidence of CALs, pediatric intensive care unit admission, or IVIG-resistant disease in KD patients. RESULTS PCT values in children hospitalized with acute KD ranged from 0.1 ng/mL to 143.9 ng/mL, with a median of 0.49 ng/mL (IQR 0.23-1.29 ng/mL). There was no correlation of PCT with patient age, race, or sex, but it was correlated with day of illness. KD patients with a PCT ≥ 0.5 ng/mL had a significantly higher incidence of IVIG-resistant disease (29% versus 7%, P = .02). There was no association between PCT and development of CALs in our sample. CONCLUSIONS Additional research is needed to establish the sensitivity and specificity of PCT for the diagnosis of KD. PCT may be of value in predicting which children are at increased risk for IVIG-resistant disease.

Myocardial Strain and Strain Rate in Kawasaki Disease: Range, Recovery, and Relationship to Systemic Inflammation/Coronary Artery Dilation.

Background Kawasaki Disease (KD), a systemic vasculitis of medium sized vessels, is the most common cause of acquired heart disease among children in the developed world. Some KD patients demonstrate echocardiographic evidence of depressed myocardial mechanics. However, the incidence, etiology, and reversibility of abnormal mechanics in KD patients remain undefined. Methods and results We retrospectively studied 41 KD patients and measured myocardial strain and strain rate by velocity vector imaging from pre-treatment and convalescent echocardiograms. Pre-treatment procalcitonin, C-reactive protein (CRP), and coronary artery z-scores were obtained in all patients and compared between the groups with preserved versus depressed acute phase mechanics. The change in mechanics between the acute and convalescent phases was also assessed. Patients with initially low longitudinal strain improved by the convalescent period (mean difference - 4.0%; p<0.005) with the greatest improvement occurring in patients with the lowest initial strain (−7.3%; p<0.05). Patients with higher initial strain did not change significantly by the convalescent period. Patients with lower longitudinal and circumferential strain demonstrated higher median procalcitonin levels (1.2 vs. 0.3 ng/mL; p<0.05 and 1.8 vs. 0.4 ng/mL; p<0.05 respectively) and a trend towards higher CRP, but no difference in coronary artery z-scores. Strain rate was not associated with inflammatory markers or coronary artery z-scores. Conclusions The range of strain found in our cohort was large. Improvement in mean strain was driven primarily by patients with lower initial strain. Lower strain was associated with increased markers of systemic inflammation, but not proximal coronary artery changes.

Prenatal diagnosis and successful outcome in neonate of aorto-left ventricle tunnel.

Aorto‐left ventricular tunnel (ALVT) is an abnormal congenital communication between the ascending aorta and the left ventricle. Prenatal diagnosis is rare and can be misinterpreted as aortic insufficiency on fetal echocardiogram. We present a case of ALVT diagnosed in a fetus who underwent successful early neonatal surgical repair.

3D echocardiographic evaluation of right ventricular function and strain: a prognostic study in paediatric pulmonary hypertension

Aims To evaluate right ventricular functional indices using 3D echocardiography (3DE) between normal children and paediatric pulmonary hypertension (PH) patients, and to evaluate these indices as outcome predictors in children with PH. Methods and results Ninety-six paediatric PH patients from 2014 to 2016 were compared with 40 normal controls. All patients underwent 3DE and off-line analysis generated 3D end-diastolic volume, 3D end-systolic volume, 3D stroke volume, 3D right ventricular (RV) ejection fraction (EF), RV longitudinal strain (LS) free wall and septum, tricuspid annular plane systolic excursion (TAPSE), and fractional area change (FAC). PH patients had higher RV volumes, lower RV EF, lower free wall and septal RVLS, lower TAPSE, and lower FAC compared with normal controls (all P < 0.001). 3D RV EF, free wall RVLS, and FAC are predictors of adverse clinical outcomes [hazard ratio (confidence interval) 0.1 (0.03-0.27], P < 0.001; 0.17 (0.07-0.45), P < 0.001; 0.08 (0.03-0.22); P < 0.001, respectively). Conclusion Paediatric PH patients have impaired RV function compared with normal children. 3D RV EF, volumes, FAC, and free wall RV strain serve as outcome predictors for paediatric PH patients.