Ayten Imamoglu

Plasma endothelin-1 levels in patients with left-to-right shunt with or without pulmonary hypertension

The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmonary hypertension (PH) secondary to congenital heart disease with left-to-right shunt. Twenty-three children (12 male, 11 female) aged 0.58-13 years were enrolled the study. Blood samples were drawn from superior vena cava, right atrium, right ventricle, pulmonary artery and pulmonary wedge or pulmonary vein during cardiac catheterization. Plasma ET-1 levels were assayed by ELISA. Patients were divided into two groups according to the presence or absence of PH. Plasma ET-1 levels of the study group were compared to the peripheral venous and arterial ET-1 levels of 11 healthy infants and children (aged 0.75-13 years). Plasma ET-1 levels in patients with left-to-right shunt were found significantly higher than those of controls. However, plasma ET-1 levels were similar between the two groups of the patients. Pulmonary venous ET-1 levels were higher than the levels of superior vena cava, this suggested an increased production of ET-1 in pulmonary vascular bed in patients with PH. No correlations were found between plasma ET-1 levels and pulmonary arterial pressure, pulmonary vascular resistance and pulmonary blood flow in the patients. Plasma ET-1 levels of the patients with left-to-right shunt were increased independently from pulmonary arterial pressure and pulmonary vascular resistance. This increase was related to the production of ET-1 in pulmonary vascular bed in patients with PH. ET-1 could not be found to be directly related to the development of PH in the patients with left-to-right shunt.

Isolated recurrent pericarditis in a patient with familial Mediterranean fever.

Familial Mediterranean fever (FMF) should be kept in mind in the differential diagnosis of recurrent pericarditis and mutation analysis should be considered, especially in patients of Mediterranean origin.

Prevalence of Idiopathic Long QT Syndrome in Children with Congenital Deafness

Abstract.Long QT syndrome (LQTS) is characterized by prolongation of the QT interval associated with a high risk for syncope and sudden death. Jervell and Lange-Nielsen initially described LQTS in association with congenital sensorineural deafness. We have investigated the prevalence of this syndrome in a school for deaf children, evaluating by ECG 350 congenitally deaf children with an age range of 6–19 years. The corrected QT interval (QTc) was calculated by Bazetts formula. Eight children with a QTc interval >440 ms were further studied by cardiac examination, repeat ECGs (three times), Holter monitoring, echocardiography, and exercise testing. The families were assessed for a history of syncope and deafness and underwent ECG evaluations regarding lengthened QTc interval. Among these eight children only two girls aged 14 and 15 years were diagnosed as having LQTS according to Schwartzs criteria (0.57% of the 350 deaf children; 95% confidence intervals 0, ≤p≤ 0.013). The first patient had two major criteria (QTc 483 ms and a history of syncopal attack) and one minor criteron (congenital deafness); the second also had two major criteria (QTc 613 ms and family members with LQTS) and one minor criterion (congenital deafness). Prophylactic β-blocker therapy was started in these two patients to prevent sudden death. In conclusion, this syndrome should be sought in children with syncope or a family history of it, especially those with congenital deafness.

Critical tricuspid regurgitation secondary to ruptured chordae tendineae mimicking a mass on the tricuspid valve in a newborn

Tricuspid regurgitation (TR) due to chordal rupture is a rere condition in newborns. We present a newborn with critical TR. Cross-sectional echocardiography demonstrated a dense, mobile mass localized at the tricuspid valve and resembling an atrial thrombus or myxoma. At operation, ruptured chordae tendineae of the tricuspid valve were found, forming a tangled mass. Histopathologic examination revealed collagen degeneration and calcification in the ruptured chordae, producing the hyperechoic image. It should be emphasized that ruptured chordae of the tricuspid valve might mimic a tricuspid valve myxoma or a thrombus on cross-sectional echocardiography.

Echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract: possible role in the pathogenesis of discrete subaortic stenosis.

Although the clinical features and natural course of discrete subaortic stenosis (DSS) are well defined, the etiology remains speculative. The purpose of this study was to identify the echocardiographic, morphologic, and geometric variations of the left ventricular outflow tract associated with DSS in children and to determine whether these variations have a role in the pathogenesis of DSS. The aortoseptal angle (ASA), mitral-aortic valve separation (MAS), and the size of the aortic annulus were determined in two groups of children. Group 1 comprised 11 patients with isolated DSS, who were compared with an age- and body surface area- (BSA) matched healthy children (Group 1A, n: 20). Group 2 comprised 10 patients with DSS and ventricular septal defect (VSD). Group 2 was compared with an age- and BSA-matched patients with isolated perimembranous VSD (Group 2A, n: 22). Measurements were carried out from previously recorded echocar diographic studies. The ASA was steeper (119.3 ±6.1° vs 137.5 ±5.6°, p < 0.001), and the MAS was wider (6.1 ±1.6 vs 3.2 ±0.7 mm, p < 0.001) in patients with isolated DSS than in healthy control subjects. Similar differences were found between patients in Group 2 and Group 2A; the ASA was steeper (122.2 ±6.5° vs 141.3 ±5.0°, p< 0.001), and the MAS was wider (5.8 ±1.5 vs 3.8 ±1.1 mm, p < 0.001). The size of the aortic annulus was not different among the four study groups. Although the MAS was significantly wider in patients with DSS, there was significant overlap in MAS between patients and controls. However, if an ASA ≤ 130° was chosen as a predictive variable, it was found to be a highly sensitive, specific, and positive predictive marker for the development of DSS. This study demonstrates that DSS is associated with a steeper ASA, and a wider MAS, in patients with or without associated VSD. These morphologic abnormalities, especially a steeper ASA, may be risk factors for the development of DSS.

VALUE OF THE ECHOCARDIOGRAPHIC FINDINGS OF ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA WITH LEFT VENTRICULAR INVOLVEMENT IN A CHILD

A 9-year-old girl was admitted because she had had palpitations for 2 years. Her electrocardiogram revealed multifocal ectopic beats with left bundle branch morphology, sinoatrial block, and first- and second-degree atrioventricular blocks. Cross-sectional echocardiography demonstrated an enlarged left ventricle and filling defects and sacculations of the posterior left ventricular wall, apex, and septum. The diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) was confirmed by endomyocardial biopsy and thallium 201 scintigraphy. We present this case because of the rarity of left ventricular involvement associated with ARVD. We also emphasize the value of echocardiography in the early diagnosis of ARVD.

Mitral Valve and Left Ventricular Thrombi in an Infant with Acquired Protein C Deficiency A Case Report

The authors present an infant with left ventricular and mitral valve thrombi diagnosed by cross-sectional echocardiography. Thrombosis was due to acquired transient protein C deficiency, which was caused by impaired liver function due to sepsis. Because the thombi were very large and mobile, urgent surgery was performed. Eight weeks later, the patients protein C level returned to normal ranges. The authors suggest that in all cases with intracardiac thrombosis, protein C deficiency should be investigated.

Quantitation of Doppler color flow jet areas for mitral regurgitation in children: angiographic correlation

Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 years (mean age +/-SD, 12.69+/-3.47 years) were analyzed with color Doppler imaging. Sixteen patients were performed cardiac catheterization within 24 h. Jets were classified as eccentric and central. Regurgitant jet area and its ratio to left atrial area and body surface area were measured by Doppler color flow imaging. Regurgitant volume and regurgitant fractions were calculated with angiography. There was a good correlation between regurgitant jet area and angiographic grade of mitral regurgitation (P<0.01). The correlation between regurgitant jet area/left atrial area ratios and angiographic grade of mitral regurgitation was limited (P<0.01). There was excellent correlation between regurgitant jet area/body surface area and angiographic regurgitant fraction (r = 0.85; P<0.001). There was also a good correlation between regurgitant jet area and regurgitant fraction (r = 0.82; P<0.001). However, the relation of regurgitant jet area/left atrial area to regurgitant fraction was weak (r = 0.72; P<0.01). In conclusion, the measurement of regurgitant fraction and its ratios to left atrial area and body surface area by color Doppler flow imaging can predict the angiographic severity in children who have even eccentric regurgitant jets.

Relation of mass/volume ratio to ECG abnormalities and symptoms in children with aortic stenosis/insufficiency

In this study, to determine whether symptoms and ECG abnormalities relate to left ventricular (LV) mass, volume, and mass/volume (M/V) ratio in children with aortic stenosis (AS) and/or insufficiency (AI), the authors examined 23 patients with echocar diography. LV volumes and mass were calculated with echocardiography. Also, the peak and mean gradients across the aortic valve and left ventricular meridional wall stress (ESWS) were determined by use of echo Doppler techniques. Fourteen patients (mean age 5.84 ±3.49 years) had AS alone. Of these, 14 had symptoms and seven had abnormal-appearing ECGs. Nine patients (mean age 6.91 ±4.35 years) had AS/AI. Of these nine, four had symptoms and two had ECG abnormalities. The authors observed that the incidences of symptoms and ECG abnormalities were higher in the patients with AS than in those with AS/AI. There was no significant correlation between symptoms and ECG abnormalities with peak gradient, mean gradient, valve area, LV mass, volume, and mass/volume ratio in patients with AS/AI. However, significant correlation was found between symptoms and ECG abnormalities and ESWS in AS/AI patients. In conclu sion, ECG abnormalities and symptoms do not always indicate the severity of AS and AS/AI.