Introduction Pericardial effusions are rare yet potentially fatal conditions in children. Azacitidine is a DNA-hypomethylating agent used in the treatment of myelodysplastic syndrome. Although seldomly described in adults, no cases of azacitidine-induced pericardial effusion have been reported in children. Case report A 7-year-old boy with myelodysplastic syndrome presented with a large pericardial effusion with risk for cardiac tamponade after his first azacitidine cycle. Management & outcome The patient was admitted to a pediatric ICU, antibiotic and steroid therapy were initiated. Pericardiocentesis was done due to hemodynamic instability. Serum and pericardial fluid complementary evaluation excluded infectious and malignant causes. The pericardial effusion did not reappear and additional pleural and ascitic slight effusions responded well to diuretics. Follow-up azacitidine cycles were administered by tapering daily dosages and using adjunctive steroid therapy, with no additional adverse events. Discussion We report the first pediatric case of large pericardial effusion secondary to azacitidine therapy in a child with MDS. This adverse reaction has not been described in pediatric patients, in which this therapeutic option has been increasingly used. We seek to raise awareness on the potential life-threatening cardiotoxicity of azacitidine in pediatric patients.
Cardiac involvement in systemic lupus erythematosus (SLE) is well documented. The pericardium, myocardium and endocardium, as well as the coronary arteries, the valves and the conduction system can all be affected. While pericarditis is common, arrythmias are less frequently described. We present a 13-year-old male, who had fatigue, anorexia, weight loss, myalgias and arthralgias for four months. On physical examination, we identified bradycardia (heart rate 31–50 bpm), oral and nasal ulcers and polyarthritis. The laboratory results showed hemolytic anemia, hypocomplementemia, antinuclear and anti-dsDNA antibodies, hematuria and non-nephrotic proteinuria. Renal function was normal. Lupus nephritis class II was diagnosed by kidney biopsy. On the transthoracic echocardiogram we identified a minimal pericardial effusion, suggesting pericarditis, and, on the electrocardiogram, we detected sinus arrest with junctional rhythm, denoting sinus node dysfunction. The patient was diagnosed with juvenile SLE with cardiac, renal, musculoskeletal and hematologic involvement. Disease remission and cardiac rhythm control were obtained with steroids and mycophenolate mofetil. Currently, the patient is asymptomatic, with normal sinus rhythm. We described an adolescent with SLE who had sinus node dysfunction upon diagnosis. Other cases have been reported in adults but none in juvenile SLE. All SLE patients should have a thorough cardiac examination to promptly diagnose and treat the innumerous cardiac manifestations of this disease.
Accelerated idioventricular rhythm is a rare but benign form of ventricular tachycardia which might be challenging to differentiate from other more worrisome forms. We present the case of a healthy newborn diagnosed with an accelerated idioventricular rhythm which is spontaneously terminated without the need for medical therapy.
An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.
Funding Acknowledgements Type of funding sources: None. Introduction This study aimed to assess systolic and diastolic heart function changes in patients with history of aortic coarctation using advanced echocardiographic imaging. Additionally, we sought to analyse which severity factors influenced these changes. Methods We performed a complete echocardiographic evaluation, with advanced functional analysis, including myocardial work analysis, to a random sample of 53 patients (age 12 to 40 years). These had a previous history of coarctation of the aorta (CoAo), which was either corrected (aortic transisthmic Doppler gradient (Dgrad) ≤20mmHg) or presented a significant residual gradient (Dgrad >20mmHg). A control group of healthy individuals, matched for age, sex and BMI, was subjected to the same evaluation. Selected dependent variables were: E/A, E’, E/E’, atrial strain parameters, biplane ejection fraction, ventricular global longitudinal strain, and global myocardial work (GMW). One-way ANOVA with appropriate post-hoc tests was done to compare the distribution of dependent variables among controls (n = 31), patients with corrected coartation (cCOAO) (n = 36), and patients with residual coartation (rCOAO) (n = 17). Multivariable linear regression was used to evaluate the association, in the 53 patients, between the dependent variables and parameters of CoAo severity: systolic blood pressure (SBP), left ventricular indexed mass (LVmass), Dgrad, and the ratio of the narrowest diameter of the aortic arch to the aorta at the diaphragm level (Aoratio). Statistical significance was established as p < 0.05. Results Patients with either cCOAO or rCOAO had lower E’ (p < 0.001), higher E/E’ (p < 0.001), lower atrial reservoir (p < 0.001) and conduit (p < 0.001) strain, when compared with controls (table 1). Patients with rCOAO had higher GMW when compared with either cCOAO or controls (p = 0.002). Multivariable regression analysis showed that both lower atrial reservoir and conduit strain were associated with a narrower aortic arch (lower Aoratio (p = 0.002 and p = 0.011, respectively); higher E/E’ with higher LVmass (p = 0.030); higher GMW with higher LVmass (p = 0.027) and Dgrad (p = 0.035). Patients subsequently submitted to an intervention for coartation treatment (n = 8) had lower atrial conduit (p = 0.007) and higher GMW (p = 0.015) when compared to all other patients (n = 45). Conclusion: Myocardial work emerged as a particularly useful tool as it was both significantly different between CoAo groups, and significantly higher in more severe patients, driven by the LV mass and residual gradient. This analysis may have a role in these patients’ clinical decision-making. Abstract Table 1
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