Şencan Özme

Double-chambered right ventricle: experience with 52 cases

The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 +/- 4.4 years). Diagnosis was confirmed in 51 patients at cardiac catheterization and in other one on operation. The majority of the patients had associated cardiac anomalies: there were 33 ventricular septal defect (63%), 21 pulmonary valve stenosis (40%), nine atrial septal defect (17%), and four double-outlet right ventricle. The electrocardiograms revealed upright T waves alone in right precordial leads suggesting right ventricular hypertrophy in 33% of the patients. At cardiac catheterization, there was a pressure gradient of 20-160 mmHg between the right ventricular inflow and outflow portions. Forty patients have had surgery and four have undergone balloon pulmonary valvuloplasty. Surgical treatment was planned for two patients and other six had no indication for treatment.

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.

Intracardiac thrombosis diagnosed by echocardiography in childhood: predisposing and etiological factors

Eleven cases of intracardiac thrombi caused by different factors including protein-C deficiency are presented for discussion of the etiology and predisposing factors of intracardiac thrombi during infancy and childhood, and to stress the importance of protein-C deficiency as an etiological factor. Thrombi were localised in the left heart in five patients and right heart in five patients. One patient had both-sided thrombi. Four of our patients had dilated cardiomyopathy, one had mitral valve hypoplasia, and one had pulmonary valvar stenosis as the predisposing factors for thrombus formation. In three patients whose cardiac anatomies were completely normal, we determined protein-C deficiency as an etiological factor of thrombus formation. One of these had congenital protein-C deficiency and the other two had acquired temporary protein-C deficiency due to sepsis. In conclusion we recommend that protein-C deficiency should be investigated as an etiological factor in all cases of intracardiac thrombi irrespective of whether or not another predisposing factor is identified.

Assessment of left ventricular systolic and diastolic functions in children with merosin-positive congenital muscular dystrophy

Cardiopathy is an expected finding in X-linked Duchenne and Becker muscular dystrophies. This holds true for some other forms such as autosomal recessive limb-girdle dystrophies. However, data on early-onset and usually severe congenital muscular dystrophies are limited. The purpose of this study was to investigate the presence of cardiac involvement in children with merosin-positive congenital muscular dystrophy. A total of 42 patients and 22 healthy subjects were evaluated by M-mode, 2D, and Doppler echocardiography. Cardiac anatomy, left ventricular dimensions, wall thickness and systolic and diastolic functions were investigated in patients and compared with those of healthy control subjects. Mean left ventricular ejection fraction and shortening fraction were significantly lower in the patient group (P<0.05 and P<0.001, respectively) and in three patients ejection fraction was below 55%. Although some impairments in left ventricular inflow indexes which were suggestive of left ventricular diastolic dysfunction were detected in patients with merosin-positive congenital muscular dystrophy they were not statistically significant. Our results suggest that left ventricular systolic abnormalities may occur in children with merosin-positive congenital muscular dystrophy.

Pulmonary artery sling.

A case of pulmonary artery sling in a 1-year-old boy, treated by resection of the left pulmonary artery (LPA) at its origin from the right pulmonary artery (RPA) and reanastomosis of the LPA to the main pulmonary artery (MPA), is reported. In this patient the LPA-MPA anastomosis was demonstrated to be patent in a follow-up pulmonary angiogram six months after the operation. Pulmonary artery sling should be kept in mine when assessing respiratory distesss syndrome in children.