Dexrazoxane and Long-Term Heart Function in Survivors of Childhood Cancer.

PURPOSE: For survivors of childhood cancer treated with doxorubicin, dexrazoxane is cardioprotective for at least 5 years. However, longer-term data are lacking. METHODS: Within the Children's Oncology Group and the Dana Farber Cancer Institute's Childhood Acute Lymphoblastic Leukemia Consortium, we evaluated four randomized trials of children with acute lymphoblastic leukemia or Hodgkin lymphoma, who received doxorubicin with or without dexrazoxane, and a nonrandomized trial of patients with osteosarcoma who all received doxorubicin with dexrazoxane. Cumulative doxorubicin doses ranged from 100 to 600 mg/m2 across these five trials, and dexrazoxane was administered uniformly (10:1 mg/m2 ratio) as an intravenous bolus before doxorubicin. Cardiac function was prospectively assessed in survivors from these trials, plus a matched group of survivors of osteosarcoma treated with doxorubicin without dexrazoxane. Two-dimensional echocardiograms and blood biomarkers were analyzed centrally in blinded fashion. Multivariate analyses adjusted for demographic characteristics, cumulative doxorubicin dose, and chest radiotherapy determined the differences and associations by dexrazoxane status. RESULTS: From 49 participating institutions, 195 participants were assessed at 18.1 ± 2.7 years since cancer diagnosis (51% dexrazoxane-exposed; cumulative doxorubicin dose 297 ± 91 mg/m2). Dexrazoxane administration was associated with superior left ventricular fractional shortening (absolute difference, +1.4% [95% CI, 0.3 to 2.5]) and ejection fraction (absolute difference, +1.6% [95% CI, 0.0 to 3.2]), and lower myocardial stress per B-type natriuretic peptide (-6.7 pg/mL [95% CI, -10.6 to -2.8]). Dexrazoxane was associated with a reduced risk of having lower left ventricular function (fractional shortening < 30% or ejection fraction < 50%; odds ratio, 0.24 [95% CI, 0.07 to 0.81]). This protective association was primarily seen in those treated with cumulative doxorubicin doses ≥ 250 mg/m2. CONCLUSION: Among young adult-aged survivors of childhood cancer, dexrazoxane was associated with a cardioprotective effect nearly 20 years after initial anthracycline exposure.

The Epidemiology and Outcome of Pericardial Effusion in Hospitalized Children: A National Database Analysis.

OBJECTIVES: To describe the epidemiology of pericardial effusion in hospitalized children and evaluate risk factors associated with the drainage of pericardial effusion and hospital mortality. STUDY DESIGN: A retrospective study of a national pediatric discharge database. RESULTS: We analyzed hospitalized pediatric patients from the neonatal age through 20 years in the Kids' Inpatient Database 2016, extracting the cases of pericardial effusion. Of the 6 266 285 discharged patients recorded, 6417 (0.1%) were diagnosed with pericardial effusion, with the highest prevalence of 2153 patients in teens (13-20 years of age). Pericardial effusion was drained in 792 (12.3%), and the adjusted risk of pericardial drainage was statistically low with rheumatologic diagnosis (OR, 0.485; 95% CI, 0.358-0.657, P < .001). The overall mortality in children with pericardial effusion was 6.8% and 10.9% of those who required pericardial effusion drainage (P < .001). The adjusted risk of mortality was statistically high with solid organ tumor (OR, 1.538; 95% CI, 1.056-2.239, P = .025) and pericardial drainage (OR, 1.430; 95% CI, 1.067-1.915, P = .017) and low in all other age groups compared with neonates, those with cardiac structural diagnosis (OR, 0.322; 95% CI, 0.212-0.489, P < .001), and those with rheumatologic diagnosis (OR, 0.531; 95% CI, 0.334-0.846, P = .008). CONCLUSION: The risk of mortality in hospitalized children with pericardial effusion was higher in younger children with solid organ tumors and those who required pericardial effusion drainage. In contrast, it was lower in older children with cardiac or rheumatologic diagnoses.

Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Postrenal biopsy AVM leading to severe hypertension and dilated cardiomyopathy.

A 3-year-old girl with Alport syndrome presented with decompensated heart failure from hypertension-induced cardiomyopathy 6 months following renal biopsy. Selective renal angiography revealed a large left renal arteriovenous fistula (AVF) with poor perfusion to the left renal parenchyma. The AVF was treated by transcatheter embolization using an Amplatzer vascular plug. Her blood pressure normalized after embolization, and her cardiac function normalized over the following 4 months.