Dursun Alehan

Evaluation of Coronary Artery Disease by Computed Tomography Angiography in Patients Treated for Childhood Hodgkin's Lymphoma

PURPOSE To detect pathologies in coronary arteries by computed tomography angiography (CTA) in survivors of childhood Hodgkins lymphoma who have been treated with radiotherapy and/or cardiotoxic agents. PATIENTS AND METHODS Patients with HL who have been in remission for at least 2 years after completion of therapy were included. CBC, lipid profile, urine analysis, brain natriuretic peptide, troponin-T, creatinine kinase-myocardial band, ECG, telecardiography, echocardiography, and CTA of the patients were performed. Cardiac vascular abnormalities were noted. Results A total of 119 patients were included in the study. In 19 patients (16%), we found coronary artery abnormalities. There was a significant difference between the patients who received mediastinal radiotherapy and those who did not (P = .02). By multivariate analysis, in patients receiving mediastinal radiotherapy the risk of developing a coronary artery abnormality was found to increase 6.8 times compared with patients who did not receive mediastinal radiotherapy (P = .009). Stent implantation was performed in a 28-year-old patient because of critical stenosis in right coronary. In two patients some irregularities were detected both in CTA and conventional angiography, and they remained in close follow-up. A 22-year-old patient whose CTA showed critical stenosis in his left anterior descending artery refused the conventional angiography. CONCLUSION To our knowledge, this is the first large study using CTA for detection of coronary abnormalities in patients treated for HL in pediatric age group. Coronary CTA is a minimally invasive tool for early diagnosis of coronary artery disease in patients who were treated with mediastinal radiotherapy and/or cardiotoxic chemotherapy.

HEAD-UP TILT TEST : A HIGHLY SENSITIVE, SPECIFIC TEST FOR CHILDREN WITH UNEXPLAINED SYNCOPE

Unexplained syncope may cause diagnostic and therapeutic problems in children. The head-up tilt test has been shown to be a useful tool for investigating unexplained syncope, especially for diagnosis of neurally mediated syncope. In this study 20 patients aged 9–18 years (12.0±2.5 years) with syncope of unknown origin and 10 healthy age-matched children were evaluated by head-up tilt to 60° for 25 minutes. The test was considered positive if syncope or presyncope developed in association with hypotension, bradycardia, or both. If tilting alone did not induce symptoms (syncope or presyncope), isoproterenol infusion was administered with increasing doses (0.02–0.08 μg/kg per minute). During the tilt test, symptoms were elicited in 15 (75%) of the patients with unexplained syncope but in only one (10%) of the control group (p<0.001). The sensitivity of the test was 75% and its specificity 90%. Three patterns of response to upright tilt were observed in symptomatic patients: vasodepressor pattern with an abrupt fall in blood pressure in 67%; cardioinhibitory pattern with profound bradycardia in 6%; and mixed pattern in 27%. In patients with positive head-up tilt, there were sudden decreases in systolic blood pressure (from 130±15 to 61±33 mmHg) and in mean heart rate (from 147±26 to 90±38 beats per minute) (p<0.001) during symptoms. Treatments with atenolol 25 mg/day has shown complete suppression of syncope in positive responders during a mean follow-up period of 18±6 months. The head-up tilt test is a noninvasive, sensitive, specific diagnostic tool for evaluating children with unexplained syncope.

Comparison of sensitivity and specificity of tilt protocols with and without isoproterenol in children with unexplained syncope.

Head‐up tilt testing with or without isoproterenol is extensively used in the evaluation of patients with unexplained syncope. However, sensitivity and specificity of tilt protocols with and without isoproterenol have not been clarified in children, due to lack of age matched control subjects. This study was designed to assess and to compare the sensitivity and specificity of tilting alone and tilting in conjunction with isoproterenol. Thirty children with unexplained syncope (group I) and 15 age‐matched control subjects (control group I) underwent successive 60° head‐up tilts for 10 minutes during infusions of 0.02, 0.04, and 0.06 μg/kg/min of isoproterenol, after a baseline tilt to 60° for 25 minutes. Also, 35 children (group II) with unexplained syncope and 15 healthy control subjects (control group II) were evaluated by head‐up tilt to 60° for 45 minutes without an infusion of isoproterenol. In response to tilt protocol with graded isoproterenol, 23 (76.6%) of the patients in group I and 2 of the 25 (13.3%) control subjects developed syncope. Accordingly, the sensitivity of tilt testing with isoproterenol was 76.6%, and its specificity was 86.7%. Tilt testing without isoproterenol was positive in 17 (48.5%) of the patients in group II but in only 1 of the 15 (6.6%) control subjects. Thus, sensitivity and specificity of tilt testing without isoproterenol were 48.5% and 93.4%, respectively. The mean heart rate and systolic blood pressure decreased significantly (P < 0.001) in all tilt positive patients during syncope. In conclusion, the head‐up tilt test is a valuable diagnostic test in the evaluation of children with unexplained syncope, and isoproterenol is likely to increase the sensitivity of the test without decreasing its specificity.

Beneficial effects of 1-year captopril therapy in children with chronic aortic regurgitation who have no symptoms

OBJECTIVE This prospective study was performed to assess the effects of 1 year of angiotensin-converting enzyme inhibition with captopril in 20 children (mean age 14.3+/-2.3 years) with asymptomatic chronic aortic regurgitation. METHODS AND RESULTS At 12 months patients receiving captopril had a significant reduction in left ventricular end-diastolic and end-systolic dimensions (57+/-9.3 vs 51+/-9.5 mm, p < 0.001; 35.4+/-6.1 vs 32+/-6.8 mm, p < 0.001), end-diastolic and end-systolic volume indexes (111+/-36 vs 94+/-29 ml/m2, p < 0.001; 35+/-13 vs 30+/-12 ml/m2, p < 0.001, respectively), and mass index (138+/-37 vs 109+/-32 gm/m2, p < 0.0001) determined by two-dimensional echocardiography. Meridian (p < 0.01) and circumferential (p < 0.0001) wall stresses also decreased significantly with therapy. Significant reduction (27.8%, p < 0.0001) was achieved in regurgitant fraction with captopril. CONCLUSIONS These data show that the long-term therapy with angiotensin-converting enzyme inhibitors is able to reverse left ventricular dilation and hypertrophy and suggest that such therapy has the potential to favorably influence the natural history of the disease in children.

Right ventricular noncompaction in a neonate with complex congenital heart disease.

Ventricular noncompaction is a rare unclassified cardiomyopathy occurring because of arrest of the normal intrauterine compaction of the loose luminal component of the ventricular myocardium. There is limited data regarding its diagnosis and outcome in children. It is recognised, however, that right ventricular involvement is extremely rare. We report a case in which only the right ventricular myocardium was noncompacted, a situation which led to heart failure soon after birth.

Tissue doppler evaluation of systolic and diastolic cardiac functions in long-term survivors of Hodgkin lymphoma†

Monitoring for late adverse events is crucial in long‐term management of childhood cancer survivors. A case–control study to evaluate long‐term cardiovascular status of childhood Hodgkin lymphoma (HL) using tissue Doppler imaging (TDI) was performed.

The role of serotonin re-uptake inhibitors in preventing recurrent unexplained childhood syncope – a preliminary report

AbstractTo assess the efficacy of a serotonin re-uptake inhibitor, sertraline hydrochloride, in preventing recurrent neurocardiogenic syncope, we studied 15 patients (10 female; mean age 12.9 ± 2 years) with positive head-upright tilt test and resistant to standard pharmacotherapy, atenolol or disopyramide. The patients were given 50 mg oral sertraline hydrochloride daily for 6 weeks. Intolerance to the drug was seen in 3 patients and 2 had syncopal episodes during the therapy. A head-upright tilt table test was then repeated in 10 patients. Six were tilt negative and asymptomatic over a mean follow up period of 7 ± 3 months while four remained tilt positive: two experienced marked hypotension and bradycardia, characterized as mixed type syncope, and two had cardiac asystole, lasting >10 s, during tilting, thereby exhibiting a cardio-inhibitory response. Conclusion Sertraline hydrochloride may be useful in preventing recurrent neurocardiogenic syncope resistant to standard pharmacotherapy but careful clinical studies are essential before such a treatment strategy can be recommended since serious asystole could develop.

Causes of syncope in children: a prospective study

We prospectively evaluated 80 patients with syncope, between January 1991 and January 1992 to determine the causes of syncope in children. There were 35 male and 45 female patients, whose mean age was 10.5 years. A single syncopal attack had occurred in 30 patients and multiple attacks in 50. A cardiovascular cause was established in 22 (27.5%) patients and a noncardiovascular cause in 36 (45%). The cause remained unknown in 22 patients (27.5%). Vasovagal syncope was the leading cause of syncope in these patients with an incidence of 32.5%. These findings suggest that every patient who has even one syncopal attack should be promptly investigated since the underlying cause could be a life-threatening one.

Unguarded tricuspid orifice: a rare malformation of tricuspid valve diagnosed by echocardiography. Report of two cases and review of the literature.

In this report we present two cases with unguarded tricuspid orifice, and review the literature. The complete absence of tricuspid valvular structures is a very rare anomaly coexisting with pulmonary atresia and intact ventricular septum, and is termed unguarded tricuspid orifice. Double tricuspid orifices and isolated dextrocardia were distinguishing features of our first case. The second patient of this report, a 5-year-old boy, is the first case in the literature with this malformation of the tricuspid valve as an isolated anomaly, in addition to being the first surviving case in the literature.

Mitral and aortic insufficiency in polyarticular juvenile rheumatoid arthritis.

SummaryValvar heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), the aortic valve being most commonly affected. Reported cases with symptomatic mitral involvement are rare. We describe a 13-year-old boy with seronegative, polyarticular onset of JRA in whom mitral and aortic valve insufficiency was diagnosed by clinical and laboratory investigations. Two-dimensional and continuous-wave Doppler echocardiography confirmed mild pericardial effusion with moderate mitral and mild aortic insufficiency. Cardiac assessment and echocardiogrphic follow-up are recommended in all patients with JRA.