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Although elevated right ventricular pressure and left ventricular diastolic dysfunction measured by echocardiogram are independent predictors of death in adults with sickle cell disease (SCD), the utility of routine echocardiographic screening in the pediatric population is controversial. We performed a 3-year retrospective review of children ≥ 10 years of age with SCD who underwent an outpatient transthoracic echocardiogram as part of a screening program. Of 172 patients referred for screening, 105 (61%) had a measurable tricuspid regurgitation jet velocity (TRV): median 2.4 m/s (IQR 2.3–2.5). Elevated right ventricular (RV) pressure (TRV ≥ 2.5 m/s, 25 mmHg), documented in 30% (32/105), was significantly associated with chronic transfusion therapy and elevated lactate dehydrogenase. Left ventricle (LV) dilation, documented in 25% (44/172), was significantly associated with lower hemoglobin, and higher reticulocyte count, lactate dehydrogenase level, and bilirubin level. There was no association between elevated right ventricular pressure or left ventricle dilation and indices of biventricular systolic or diastolic function. The one death in the cohort during the study period had normal echocardiographic findings. In conclusion, mild RV pressure elevation and LV dilation in children with SCD is associated with abnormal laboratory markers of disease severity, but not with ventricular dysfunction over the 3-year study period. [ABSTRACT FROM AUTHOR]

Previous studies have demonstrated a correlation between E:Ea and ventricular filling pressure in adults after heart transplantation. We sought to determine if E:Ea correlates with filling pressure after heart transplantation in children. A prospective analysis of children who have undergone heart transplantation was performed. Inflow and myocardial velocities were recorded and compared to catheter-derived filling pressures and rejection status. We performed 61 studies in 49 subjects. No correlation was found between septal E:Ea and PCWP (r = 0.14, p = 0.28); or between lateral tricuspid E:Ea and mean RAp (r = 0.04, p = 0.79). However, the mean PCWP was higher among subjects with elevated septal E:Ea (>12) compared to normal E:Ea (12.3 ± 2.8 mmHg vs. 10.1 ± 2.9 mmHg, p = 0.02). Similarly, mean RAp was higher among subjects with an elevated lateral tricuspid E:Ea (>10) compared to normal lateral tricuspid E:Ea (7.7 ± 2.1 mmHg vs. 6.0 ± 2.4 mmHg, p = 0.04). Elevated septal E:Ea was also associated with high-grade cellular rejection (OR = 17.3 [95% CI 1.4-221], p = 0.028). In children following heart transplant, E:Ea does not correlate well with the range of filling pressures seen after pediatric heart transplantation. However, a septal E:Ea > 12 is associated with elevated PCWP and high grade cellular rejection and a lateral tricuspid E:Ea > 10 is associated with elevated mean RAp. [ABSTRACT FROM AUTHOR]

Hall EK, Glatz AC, Quartermain MD, Ravishankar C, Kaufman B, Cohen MS, Hanna BD, Goldberg DJ. Brain-type natriuretic peptide correlates with right heart pressures in a cross section of pediatric heart transplant patients. Pediatr Transplantation 2011: 15:70-74. © 2010 John Wiley & Sons A/S. Serum brain-type natriuretic peptide level (BNP) correlates with hemodynamic parameters measured during cardiac catheterization in adult patients with heart failure. We sought to describe the relationship of BNP with invasive hemodynamic measurements and cellular rejection in children following OHT. Children undergoing catheterization for OHT surveillance had simultaneous measurement of BNP. A total of 62 subjects were studied. The median BNP was 171 pg/mL (range 19-1130). There were significant positive correlations between BNP and mean PAP (R = 0.33, p = 0.009), RVSP (R = 0.25, p = 0.05), RVEDP (R = 0.29, p = 0.02), and mean RAP (R = 0.39, p = 0.002). Rejection grade varied from 0 to 3A (58 patients < ISHLT 3A and four patients ≥ ISHLT 3A). There was no significant difference in BNP based on cellular rejection grade. In a cohort of pediatric patients after heart transplantation, BNP correlates with direct measurements of right-sided pressures, but not with other hemodynamic measurements, time from transplant or rejection grade. This suggests that BNP may have a complimentary role in the monitoring of children following heart transplantation. [ABSTRACT FROM AUTHOR]

Pathologies in both the respiratory and cardiovascular systems frequently coexist and impact on each other. This manuscript introduces an approach to the interpretation of this complex relationship. Pulmonary hypertension can be a significant consequence of many respiratory diseases. This in turn can lead to right ventricular dysfunction and cor pulmonale. Many childhood illnesses can result in cor pulmonale and can be conveniently grouped into three categories: idiopathic pulmonary hypertension, neonatal lung diseases, and lung disease beyond the neonatal period. When considering the impact of cardiac disease on the lung, one must consider two main pathologies: compression of the pediatric airway and increased lung water. In conclusion, thorough attention must be given to the interpretation of the complex relationship between cardiac and respiratory diseases. Pulmonary hypertension is a complication to consider in respiratory illness at all ages. In addition, when dealing with the complexities of congenital heart disease, one must always be aware of the risks of pulmonary complications whether parenchymal or airway. Ongoing improvements in ventilation strategies, vasodilator therapy, and surgical interventions continue to improve the outlook for these complex cases. [ABSTRACT FROM AUTHOR]

Background: Altered cardiac autonomic control may play a role in the morbidity and mortality suffered by neonates who undergo surgery for complex congenital heart disease (CHD). The purpose of this study was to evaluate cardiac autonomic activity, as measured by spectral indices of heart rate variability (HRV), prior to and early after infant surgery for CHD and attempt to correlate HRV indices with clinical outcome. In addition, we assessed the hypothesis that single-ventricle physiology and surgical interruption of the great arteries negatively affects HRV. Methods: Sixty neonates prospectively wore 24-hour Holter monitors at three time points: before and early after CHD surgery, and at 3- to 6-month follow-up. Standard spectral indices of HRV were measured. Results: In the early postoperative time point, patients with single-ventricle physiology had lower low-frequency power (LF) compared to patients with two ventricles (P = 0.040). Surgical interruption of the great arteries did not affect HRV in this cohort. For the entire cohort, LF (P = 0.004) and high-frequency power (HF) (P < 0.001) increased over the three time points, while LF/HF (P = 0.119) did not significantly change. In the multivariable linear regression model, significant predictors of longer postoperative hospital stay included longer total support time (P = < 0.001), longer duration of inotrope support (P = 0.012), elevated mean heart rate at postoperative time point (P = 0.002), and lower LF/HF ratio at the postoperative time point (P = 0.014). Conclusion: Patients with single-ventricle physiology have a significant physiologic reduction in LF in the early postoperative period compared to patients with two ventricles. Diminished cardiac autonomic control is associated with longer hospitalization following neonatal cardiac surgery. [ABSTRACT FROM AUTHOR]