Canan Ayabakan

Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever

AIM Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. METHODS AND RESULTS Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9+/-2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. CONCLUSION Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

Left ventricular myocardial velocities in healthy children: quantitative assessment by tissue Doppler echocardiography and relation to the characteristics of filling of the left ventricle.

AIM To assess the myocardial velocities of the mitral annulus, left ventricular lateral wall, and midseptum in healthy children, and to compare these parameters with transmitral and pulmonary venous velocities. METHODS AND RESULTS We examined 72 children, half being male, who had no systemic or cardiac pathologies. Their mean age was 6.73 +/- 5.10 years, with a range from 0.1 to 17.75 years, and a median age of 6.71 years. Each parameter was measured twice, at end inspiration and end expiration. The tissue velocities are similar in males and females (p > 0.05). The longitudinal velocity of the heart in early diastole has a positive correlation with age (p < 0.05; midseptum velocity r = 0.57, left ventricular lateral wall velocity r = 0.56, mitral annulus velocity r = 0.56), and the tissue velocities are not influenced by respiration (p > 0.05). The myocardial velocities of different segments of the left ventricle are not correlated with the transmitral or pulmonary venous flows (p < 0.05). When age is controlled for heart rate, age mainly affects the systolic velocity of the mitral annulus and the early diastolic velocity of the midseptum in longitudinal axis, as well as the early diastolic velocity of the midseptum in transverse axis (p > 0.05 for all, r = 0.34, 0.29, 0.30 respectively). CONCLUSION This study, which has determined reference values for tissue velocities in a large healthy group of children, will now set the scene for further studies in children with heart disease.

Evaluation of subclinical valvar disease in patients with rheumatic fever.

Carditis is the only manifestation of acute rheumatic fever that leads to permanent disability. Hence, its diagnosis is of paramount importance. Recently, it has been reported that Doppler echocardiography has disclosed subclinical valvar regurgitation in some patients with acute rheumatic fever manifested as isolated arthritis or pure chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is not clear. We aimed, therefore, prospectively to investigate the potential to diagnose patients with subclinical carditis. We examined 40 patients, aged from 7 to 16 years, with Doppler evidence of mitral and aortic regurgitation, but in the absence of any pathologic murmur. The major findings satisfying the Jones criterions were arthritis in 29 patients, chorea in 10 patients, and arthritis and erythema marginatum in one patient. Of the patients, 33 had mitral regurgitation, 6 patients had combined mitral and aortic regurgitation, and one patient had aortic regurgitation. The patients were followed over a mean period of 18.1 +/- 13.9 months, the valvar regurgitation disappearing in 23 (57.5%). No significant differences were observed in the resolution of the valvitis between those treated with acetylsalicylic acid, steroids, or those receiving no treatment. It is noteworthy, nonetheless, that patients treated with steroids were the fastest to recover from valvitis (p < 0.05). Based on our study, we suggest that subclinical valvitis demonstrated by echocardiography should now be accepted as adequate evidence for the diagnosis of carditis, and become a major diagnostic criterion for acute rheumatic fever. When managing this group of patients with subclinical disease, treatment with steroids seems to have a role in promoting early resolution of the valvitis.

Heart Rate Variability and Autonomic Nervous System Changes in Children with Vasovagal Syncope

ALEHAN, D., et al.: Heart Rate Variability and Autonomic Nervous System Changes in Children with Vasovagal Syncope. The aim of this study was to evaluate the HRV at rest and during tilt test (HUTT) in children with a history of vasovagal syncope and to link the HRV indices with the clinical results of the test. HRV indices were assessed in the supine position and during the initial 5 minutes of the 60‐degree HUTT in 49 patients (33 females, 16 males, mean age of 13 ± 2.8 years) who were evaluated for recurrent syncope. The positive to negative results of the test were 21 to 28. The normalized power of high frequency component (npHF) decreased, normalized power of low frequency component (npLF) and the LF:HF ratio increased during HUTT of tilt‐positive patients (P < 0.05 for each parameter). Parallel changes, but to a lesser degree, were observed for similar HRV parameters of tilt‐negative patients. In addition, the HF and all the time‐domain indices decreased significantly (P ≤ 0.05) during HUTT in the latter group. When the tilt‐positive and‐negative patients were compared, the npHF was lower (P = 0.002), npLF and LF:HF ratio were higher (P = 0.01 and P = 0.001, respectively) during the test in tilt‐positive patients, reflecting increased sympathetic tone in this group. A cut‐off point for LF:HF was assigned as 2.7 for differentiating tilt‐negative and tilt‐positive results. The specificity, sensitivity, and positive and negative predictive values of this cut‐off point were calculated as 93%, 52%, 85%, and 41%, respectively. Patients with vasovagal syncope show variations in vagal autonomic tone and appear to be more prone to syncope when their sympathetic tone is elevated at the beginning of the test. LF:HF > 2.7 is a specific marker (specificity 93%) and can correctly predict a positive tilt test in 85% of patients.

Normal patterns of flow in the superior caval, hepatic and pulmonary veins as measured using Doppler echocardiography during childhood

To date, no reference values have been provided for right and left atrial filling in normal children. The aim of our study, therefore, was to characterize measurements of superior caval, hepatic, and pulmonary venous flow using Doppler echocardiography in a large group of normal children to reflect the effects of age, body mass index, sex, heart rate and respiration. Doppler echocardiographic examinations of the superior caval, hepatic and pulmonary veins were performed during inspiration and expiration in 72 healthy children with a mean age of 6.73 +/- 5.10 years. The subjects were segregated into four age groups, namely infants < 2 years, preschool children between the ages of 2 and 7 years, children of school age between 7 and 11 years, and adolescents older than 11 years. Age has significant effect on the systolic and reverse atrial flows within the superior caval vein (p < 0.05). No change in the Doppler velocities was observed related to body mass index or sex. All peak systolic velocities decreased significantly during expiration (p < 0.05). This decrease was most prominent in the hepatic vein (26%), but less remarkable in the superior caval vein (5.7%) and the pulmonary veins (3.9%). During expiration, the peak diastolic flow in the superior caval and the hepatic veins decreased, while the reverse atrial flow in the hepatic vein increased (p < 0.05). Pulmonary venous velocities were similar in all age groups (p > 0.05). Except for the systolic pulmonary venous velocities, these parameters were not influenced by respiration (p > 0.05). The diastolic time, the interval between reverse atrial flow and ventricular systole reflected by the R wave on the electrocardiogram, and the interval between ventricular systole and diastolic flow, were negatively correlated with heart rate (p < 0.05; r = -0.35, -0.85, and -0.8 respectively), and positively correlated with age (p < 0.05; r = 0.3, 0.8, and 0.7 respectively). They were not influenced by respiration. Our study provides data of the patterns and the normal ranges of velocities of superior caval, hepatic, and pulmonary venous flow in a series of normal children. The results can now be used for comparison with the patterns found in the setting of disease.

Dysrhythmia as a cause of syncope in children without neurological or cardiac morphological abnormalities

Background : Arrhythmias are among the malignant causes of syncope. This study has been undertaken to determine the relative incidence and significance of dysrhythmia in the pathogenesis of syncope among patients referred to a pediatric cardiology unit.

A Case of Superoinferior Ventricular Heart with Situs Ambiguus, Dextroventricular Loop, and Levo Transposition of the Arteries

A unique case of superoinferior ventricles, left atrial isomerism, concordant atrioventricular connection, and discordant ventriculoarterial connection is described. The associated anomalies were azygous vein continuation, large subpulmonary and apical ventricular septal defects, and left ventricular outflow tract obstruction (pulmonary stenosis). The diagnosis was mostly made by fetal echocardiography and the superoinferior location of the ventricles was confirmed by postnatal echocardiography. Fetal and postnatal echocardiography facilitated the precise morphologic diagnosis and segmental analysis of this defect.

Constitutional Growth Delay Pattern of Growth in Velo−Cardio−Facial Syndrome: Longitudinal follow up and final height of two cases

We report two patients with velo−cardio−facial syndrome (VCFS) who were admitted to our pediatric endocrinology clinic because of short stature and followed longitudinally until attainment of final height. Both patients followed a growth pattern consistent with constitutional delay of puberty with normal and near normal final height. Case 2 also had partial growth hormone (GH) deficiency and severe short stature (height SDS −3.4 SDS), but showed spontaneous catch−up and ended up with a final height of −2 SDS. These cases suggest that short stature in children with VCFS is due to a pattern of growth similar to that observed in constitutional delay of growth and puberty. Conflict of interest:None declared.

Truncus Arteriosus with a Very Small Ventricular Septal Defect Diagnosed by Echocardiography

By definition, infundibular septal deficiency is not a necessary component of truncus arteriosus (TA); however, instances of this anomaly with intact infundibular septum are very rare [1, 3, 4]. We present a case of TA with a very small ventricular septal defect (VSD) diagnosed by echocardiography. A 6-year-old boy was diagnosed with levocardia, normal visceroatrial situs, D-ventricular loop, and a single great artery arising from both ventricles with a single semilunar valve. The atrial septum was intact. The anterior and posterior limbs of the septal band embraced the truncal valve (Fig. 1, top left). A very small VSD and a single bicuspid semilunar valve were present (Fig. 1, top right). The muscular and membranous ventricular septum were intact (Fig. 1, bottom). The patient was diagnosed with type I TA with a small ventricular communication (Fig. 2). Since the truncal septum is always absent in TA there is invariably a single semilunar valve. Cases with two semilunar valves should be considered aortopulmonary window [2]. The parietal band in TA is underdeveloped and does not fill the space above the septal band, so the truncal valve forms the roof of the VSD [2, 5]. Carr et al. [1] diagnosed a TA with intact ventricular septum at autopsy. Rosenquist et al. [4] described two cases with small conal septal defects located close to the truncal valve. In these cases the valvular tissue arose from crest of the muscular septum, partially or completely covering it. TA exhibits a spectrum in which the size of the truncal or conal defect varies. A definitive diagnosis of TA is feasible non invasively by means of echocardiography.

Double-Outlet Left Ventricle with a Superiorly Positioned and Hypoplastic Left Ventricle

AbstractDouble outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both the pulmonary artery and the aorta arise exclusively or predominantly from the morphologic left ventricle. We describe a case of DOLV with situs inversus, L-loop ventricles, d-malposition of the great arteries (I, L, D), superior–inferior ventricles, unbalanced complete atrioventricular septal defect, pulmonary stenosis, and total anomalous pulmonary venous return in which the left ventricle was also severely hypoplastic and superiorly positioned. Many morphologic variations of this malformation have been described, but to the best of our knowledge DOLV with superiorly positioned and hypoplastic left ventricle has not been reported before.