Diagnosis and treatment of post-transplantation lymphoproliferative disorder in pediatric heart transplant patients.

PTLD is a severe complication in transplant recipients. Detection of increased EBV load in the peripheral blood acts as a surrogate marker for increased risk of PTLD development. We analyzed the time course of the disease, its severity, the organs involved, and mortality rates in our institutional experience of pediatric heart transplantation. This paper identifies risk factors for PTLD and describes the different ways of diagnosing and treating the disease. PTLD was screened for in 146 pediatric heart transplant patients using a retrospective analysis in patients who received transplantation before 1998. Prospective determination was performed in 72/146 patients transplanted after 1998 within the post-transplant follow-up. The occurrence of PTLD with all interventions, including tapering of immunosuppression, surgery, viral monitoring, and antiviral interventions, was recorded. PTLD was diagnosed in 12/147 (8.2%) children at a mean age of 7.2 +/- 3.3 yr after a mean post-transplant period of 3.2 +/- 2.2 yr. PTLD manifested in: lymph nodes (n = 4), intestine (n = 3), tonsils and adenoids (n = 2), eye (n = 2), and lung (n = 1). It was diagnosed in 7/12 as a monomorphic B-cell lymphoma and in four patients as a monomorphic Burkitt lymphoma, a polymorphic B-cell lymphoma, a T-cell rich or angiocentric lymphoma (Liebow) and as reactive plasmacytic hyperplasia (early lesion), respectively. Histology was not possible in one patient with ocular manifestation. EBV association was 83%. Risk factors in the comparison with patients without PTLD were age at time of Tx, primary EBV infection after Tx, use of Azathioprine and >or=3 doses of ATG. CMV mismatch and CMV infection, rejection episodes and steroids were not risk factors. Despite reduction of immunosuppression, treatment consisted of surgical procedures to remove tumor masses (n = 6), Rituximab (n = 5), polychemotherapy (n = 3), antiviral (n = 1) and autologous T-cell therapy (n = 1). All patients demonstrated full remission without death related to PTLD or treatment at 3.9 (1.3-6.2) yr median follow-up time. The manifestation of PTLD in pediatric heart transplant recipients is associated with EBV infection and is predominantly in the form of a B-cell lymphoma. A tight and specific follow-up including early assessment of immunity status and specific therapeutic intervention to improve cellular immunity is warranted and may contribute to a significant reduction of PTLD-related morbidity and mortality.

[Evaluation of abnormal myocardial wall motions in patients with univentricular heart by tissue Doppler echocardiography]. / Evaluation abnormer Myokardwandgeschwindigkeiten des univentrikulären Herzens mittels Gewebedoppler-Echokardiographie.

INTRODUCTION: Assessment of systolic and diastolic ventricular function in children and adults with morphologically and functionally univentricular heart is difficult using the conventional echocardiographic methods. Quantitative assessment of systolic and diastolic wall motion by TDE may provide information on abnormal systolic and diastolic ventricular function. Thus, the object of this study was to analyze the patterns of anterior and posterior wall motions in children with univentricular heart after palliative Fontan operation in comparison to normal subjects. PATIENTS AND METHODS: We investigated 21 patients of an average age of 10.1 years (range 4.2 to 32 years) with the primary diagnosis of univentricular heart and tricuspid atresia after a median period of 4.3 (range 1.2 to 8) years after cavo-pulmonary anastomosis (Fontan procedure) and in comparison to a normal collective of children without cardiovascular malformations. For investigation we used the novel tissue Doppler echocardiography (TDE) (EchoPack 6.3.6, Vingmed, Norway). Thereby we chose the standardized apical view and evaluated the left and right annular systolic (S(T)), early diastolic (E(T)) and atrial (A(T)) motion. We also registered the acceleration and deceleration time of each obtained curve. RESULTS: In comparison to the posterior myocardial wall the velocities at the anterior wall of the rudimentary ventricle were significantly reduced in all patients with tricuspid atresia and univentricular heart (p<0.0001). Particularly the annular systolic and diastolic wall motions of the rudimentary ventricle as well as their deceleration and acceleration time in patients with univentricular hearts differed significantly from those in healthy persons. CONCLUSION: Abnormal myocardial wall motion is detectable in children and adults with univentricular heart after palliative cardiac procedures using tissue Doppler echocardiography. The hemodynamic value of the measured abnormal wall motions, however, need further comparative studies.