Cardiotoxicity evaluation in pediatric patients with acute lymphoblastic leukemia - results of prospective study.

AIM: The chemotherapy protocol for acute lymphoblastic leukemia (ALL) uses low doses of anthracyclines (AC), generally associated with subclinical cardiotoxicity. The aim of our study was to evaluate the serum biomarkers and echocardiography parameters in children with ALL treated with AC in order to determine the most useful element for early detection of cardiotoxicity. MATERIAL AND METHODS: In this prospective study, troponin I (TnI) and heart-type fatty acid binding protein (HFABP) were assessed five times during the first year after the onset of ALL. Serial Tissue Doppler Imaging and conventional cardiac echography were performed by two pediatric cardiologists (intraclass correlation coefficient over 0.85 for all measurements) in three periods during the study protocol. RESULTS: We evaluated 48 children with ALL. TnI increased during therapy, without returning to baseline values one year after diagnosis. HFABP did not show significant changes during the study protocol. Left ventricle outflow tract time-velocity integral and peak systolic septal mitral annulus velocity decreased during chemotherapy and returned to baseline levels at one year after diagnosis, while peak systolic tricuspid annulus velocity and excursion, maintained a descending tendency. Early filling transmitral flow velocity and E/A ratio were also transiently influenced by chemotherapy. CONCLUSIONS: The study showed signs of transient cardiotoxicity in the left ventricle and diastolic parameters after chemotherapy, compared to right ventricle parameters which maintained low values even one year after diagnosis. TnI proved to be directly proportional to chemotherapy doses but HFABP was not useful in this setting.

Complex Ebstein's malformation: defining preoperative cardiac anatomy and function.

INTRODUCTION: Ebstein's malformation is a congenital malformation of the tricuspid valve and right ventricle, with a highly variable morphology, and clinical presentation, accounting for less than 1% of all congenital heart diseases, and about 40% of congenital malformations of the tricuspid valve. METHOD: Systematic review of English language literature regarding the morphogenesis and progress of imaging techniques, especially echocardiography and cardiac magnetic resonance imaging, in the preoperative planning of Ebstein's malformation, using the MEDLINE database with the PubMed Entre interface. RESULTS: Technological developments of the recent years, with 3D echocardiographic and cardiac magnetic resonance imaging evolution, have resulted in better understanding of the malformed tricuspid valve and right ventricle in vivo anatomy. Through a better understanding of Ebstein's malformation, there is a continuous trend of surgical techniques favoring tricuspid valve sparing procedures, with a constant decrease in early and late postoperative mortality. CONCLUSIONS: Although imaging methods to investigate Ebstein's malformation have continuously evolved, standardization of assessment protocols by 3D echocardiographic imaging, speckle tracking imaging, and cardiac magnetic resonance imaging is required.

Cardiac involvement in myasthenia gravis--is there a specific pattern?

Myasthenia gravis is an autoimmune neuromuscular disorder characterized by skeletal muscle involvement, causing muscle weakness and fatigue. The prevalence of the disease is approximately 1:7500 with a maximal prevalence during the second and third decade in women and the fifth and sixth decade in men, although it may appear at any age. The disease has a slight female preponderance, with a sex ratio of 3:2. Cardiac involvement in myasthenia gravis may take several forms, ranging from asymptomatic ECG changes to ventricular tachycardia, myocarditis, conduction disorders, heart failure and sudden death. We hereby report two cases of patients with myasthenia gravis who developed signs and symptoms of cardiovascular involvement, requiring admission in a cardiology ward for further investigation and treatment. The particular characteristics of the first case may be summarized by the symptomatic conduction disturbances with frequent episodes of syncope in a patient with myasthenia gravis who necessitated permanent pacing and the difficulties we encountered in the establishment of conduction disturbancies etiology (due to the disease or due to the treatment with acetylcolinesterase inhibitors). The second case shows a different kind of cardiac involvement in myasthenia gravis--the ECG changes (giant diffuse T waves in a patient with cardiovascular risk factors) which needed further investigation and long term surveillance.