OBJECTIVE:Our goal was to use three dimensional (3D) strain analysis to evaluate myocardial function and ascending aorta elasticity changes in juvenile dermatomyositis (JDM). METHODS:Between 2019 and 2021, 23 JDM patients and 20 healthy volunteers participated. Both groups underwent 2D and 3D strain analysis, assessing aortic stiffness using aortic distensibility, stiffness index, strain, and elastic modulus. RESULTS:JDM patients had a median age of 13.3 ± 5.2 years, while controls had a median age of 13.8 ± 4.76 years. 3D strain analysis revealed significantly lower global longitudinal (GLS) and circumferential strain (GCS) in JDM patients compared to controls. Specifically, 3D GLS was notably reduced in patients (-28.1% vs. -31%, p = .047) compared to controls, and 3D GCS was also lower in patients (-27.5% vs. -30.5%, p = .019) compared to controls. Aortic strain and elastic modulus were significantly lower in JDM patients, while aortic stiffness index and distensibility showed no significant differences. Correlation analyses within the JDM group revealed a negative correlation between 3D GLS and age at diagnosis (r = -.561, p = .04), as well as a positive correlation between 3D GLS and both aortic strain (r = .514, p = .0001) and elastic modulus (r = .320, p = .03) in JDM patients. CONCLUSION:Our study demonstrated a trend towards lower ejection fraction and strain in patients with JDM, along with increased aortic stiffness using 3D echocardiography. These findings suggest potential cardiovascular involvement in juvenile dermatomyositis, emphasizing the importance of comprehensive cardiac assessments in these patients.

Early detection of cardiac involvement in Juvenile Dermatomyositis (JDM) is difficult due to the absence of clinical signs and symptoms, with systolic dysfunction often emerging in late stages and associated with a poor prognosis. This study aimed to employ two-dimensional speckle-tracking echocardiography (STE) for subclinical assessment of left ventricular (LV) systolic failure in JDM and explore potential associations between impaired LV systolic function (LV-GLS) and disease activity. A prospective study enrolled 20 healthy volunteers and 26 JDM patients (< 18 years old) without cardiac symptoms. Clinical data were collected from medical records, and echocardiograms were conducted by a pediatric cardiologist. Our study cohort demonstrated similar age to controls (13.5 ± .6 vs. 13.8 ± 4.7; p = 0.465). Median illness duration at echocardiography was 5 (1.5–17.5) years, and conventional echocardiography indicated normal LV ejection fraction (> 55

Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on current perinatal outcomes of EA/TVD in our center and to investigate clinical and fetal echocardiographic predictors of perinatal mortality. We performed a retrospective study among fetuses diagnosed from January 2014 to December 2023. Clinical and echocardiographic data were obtained from hospital records of Research and Education Hospital. The primary outcome was perinatal mortality. Of 21 fetuses diagnosed, there were 1 lost to follow-up, 1 termination, and 7 demises. In the live-born cohort of 12 live-born patients, 2 died before discharge, yielding an overall perinatal mortality of 50

Left atrial (LA) dilatation is a powerful predictor of cardiovascular morbidity and mortality. LA longitudinal strain parameters evaluating reservoir, conduit and contractile strain can represent LA functions. We investigated the association between LA strain and left ventricular (LV) functions using speckle-tracking echocardiography in children with mitral regurgitation in 45 asymptomatic mitral regurgitation patients graded as mild, moderate and severe. LV strain was lower in the moderate group (–17.8±3.7%) and further reduced in the severe regurgitation group (-16±2.14%) in comparison with the reference group (-20.5±6.2%). Regarding LA functions, reservoir strain was higher in the severe and moderate mitral regurgitation groups (38±12.9% and 40.7±9.5%, respectively) in comparison with the controls (40.0±7.2%; p<0.0001). A milder depression of contraction was observed in the severe group (12.2%) than in the moderate group (13±7.2%). In the moderate and severe groups, LV strain correlated significantly with the vena contracta (r=–0.858; p=0.04). Vena contracta was the only independent association for LV longitudinal strain. ROC curve analysis predicted LV longitudinal strain as shown by vena contracta, with a cut-off value >5.65 mm. Vena contracta had a positive predictive value of 75% for predicting LV function. We propose the use of LA and ventricular strain during the follow-up of asymptomatic children with mitral regurgitation. Also, a vena contracta diameter of >5.6 mm can be used as a threshold for LV function impairment.

OBJECTIVE:The timing of surgical patent ductus arteriosus ligation in preterm infants remains controversial. Early ligation (<2 or 3 weeks of life) benefits preterm infants with a lower incidence of bronchopulmonary dysplasia and necrotizing enterocolitis. We present patent ductus arteriosus ligation experience in premature infants who had been transported for closure to an outside centre other than the hospital they were hospitalized. MATERIALS AND METHODS:We retrospectively evaluated 17 consecutive patients who had undergone surgery of premature infant patent ductus arteriosus closure during the period of March 2009-December 2020. Patent ductus arteriosus had been clipped in 17 patients. RESULTS:The median birth weight and age were 930 g and 28 gestation weeks, respectively. The birth age of the sub-groups were A: ≤28 weeks and B: >28 weeks and birthweight were group I: ≤800 g and group II: >800 g. The median day of PDA ligation wasa 20 days, and patients with birthweight ≤800 g were ligated later than patients weighing >800 g. Two patients had intracranial hemorrhage, 6 had bronchopulmonary dysplasia, and 2 were dead. We found that exposure to large patent ductus arteriosus and low birth age in preterm babies was associated with longer hospitalization duration, preoperative mechanical ventilation time, and sepsis. CONCLUSION:Infants exposed to moderate-to-large patent ductus arteriosus requiring intubation and resistant to medical therapy for more than 2 weeks should have surgical ligation as soon as possible.

Objective:We aim to examine the prevalence and characteristics of ascending aorta (AA) dilatation in pediatric patients, specifically focusing on its relationship with the bicuspid aortic valve (BAV) and aortic valve stenosis (AS).We also seek to identify potential risk factors and clinical implications of AA dilatation within this population. Materials and Methods:In this retrospective cross-sectional study, 71 pediatric patients who underwent echocardiography between 2010 and 2022 were analyzed.AA dilatation is seen in connective tissue disorders and in BAV.Our objective was to look at the potential diagnoses for pediatric AA dilatation, focusing on associated aortic valve diseases.Patients with genetic syndromes, congenital heart defects, or other cardiac conditions were excluded.Aortic measurements were taken using standard techniques, and z-scores were calculated.Statistical analyses were performed to evaluate associations between aortic dilatation, valvular conditions, and other variables. Results:The median age of 71 patients was 9 years (1 day-18 years).AA dilation was common in patients with AS (36%) and BAV (47%).Isolated aortic root dilation was observed in 49% of cases.AA z-scores were statistically higher in BAV patients (p=0.034),but no difference was found in aortic root z-scores between BAV and non-BAV patients (p=0.117).AA z-scores correlated with aortic root z-scores and BAV presence (p=0.037,p=0.033).Patients with AS had smaller aortic root z-scores compared to those without AS (p=0.003).Smaller aortic root z-scores were also found in patients with both AS and BAV compared to those without either condition (p=0.0001).We could not find a statistically significant difference in AA dilatation in patients with and without AS. Conclusion:AA dilatation is a significant concern in pediatric patients with BAV, especially when AS is present.Routine echocardiography is essential for monitoring and early detection of aortic dilatation.Further studies are needed to understand the long-term implications and to optimize diagnostic approaches.

Mitral valve cleft (MVC) is the most common cause of congenital mitral regurgitation (MR). MVC may be located on the anterior or posterior leaflets. We evaluated children with moderate-to-severe MR using 3D transthoracic echocardiography (3DTTE) to diagnose MVC and determine the location, shape and size of MVC. Twenty-one patients under 18 years of age with moderate-to-severe MR without symptoms who were suspected of having MVC were included in the study. The patients’ history and clinical data were obtained from the medical records. 2D and 3D imaging were performed with a high-quality machine (EPIQ CVx). A vena contracta (VC) of colour Doppler regurgitated jet 3–7 and ≥ 7 mm defined moderate-to-severe regurgitation. An isolated anterior leaflet cleft (ALC) was detected in four patients, an isolated posterior leaflet cleft (PLC) in 12 patients, and both an ALC and PLC in five patients. VC was larger in patients with ALCs than PLCs (8.85 mm vs. 6.64 mm). Global LV longitudinal strain was better in the ALC group than in the PLC and both-posterior-and anterior MVC groups (− 24.7, − 24.3, and − 24

Backround: Coronary artery fistula (CAF) is an abnormal precapillary connection between a coronary artery and an adjacent structure. The incidence of CAF in children as estimated from echocardiography is 0.06-0.2%. We aimed to establish the follow up results of clinically silent CAF found incidentally by colour Doppler echocardiography. Methods: The study included patients with abnormal fistulous flow in the pulmonary artery who had been studied between 2008 and 2020. Patient demographics, clinical findings, indication for echocardiography, electrocardiographic findings at presentation, follow-up times and any fistula progress were obtained from the recorded files. Results: Among the 78,000 patients who had had an echocardiographic examination, 118 had been found by colour Doppler flow mapping to have a clinically silent CAF. The exit point of CAF was clearly visualized with colour Doppler in all patients. In most patients the CAF was located around the pulmonary valve and the bifurcation; in 76 patients, it was on the aortic side of the main pulmonary artery, 26 patients had fistulous flow through the anterior wall of the main pulmonary artery, two had the right ventricular outflow tract (RVOT) as the exit site, for 12, it was the right pulmonary artery, and for another two, the left pulmonary artery. The 118 patients had ongoing follow-up to mean 41 months. There was no spontaneous resolution of fistula. The patients were asymptomatic without intervention and with ongoing echocardiographic evidence of small CAF at the last follow-up. Conclusion: We recommend the treatment strategy for paediatric patients should be individualized according to fistula origin, size, chamber enlargement, draining site, age of the patient and cost of imaging during follow up.

Objective Alström syndrome (ALMS) is a rare autosomal recessive genetic disorder that is caused by homozygous or compound heterozygous mutation in the ALMS1 gene. Dilated cardiomyopathy (DCM) is one of the well-recognized features of the syndrome ranging from sudden-onset infantile DCM to adult-onset cardiomyopathy, sometimes of the restrictive hypertrophic form with a poor prognosis. We aimed to evaluate severe cardiomyopathy in Alström syndrome in infancy and display susceptible specific mutations of the disease, which may be linked to severe DCM. Secondarily we reviewed published mutations in ALMS1 with cardiomyopathies in the literature. Method We represent new mutagenic alleles related to severe cardiomyopathy and cardiac outcome in this patient cohort. We evaluated echocardiographic studies of nine Turkish patients diagnosed with Alström syndrome (between 2014 and 2020, at age two weeks to twenty years). Thus, we examined the cardiac manifestations of a single-centre prospective series of nine children with specific ALMS mutations and multisystem involvement. All patients underwent genetic and biochemical testing, electrocardiograms, and echocardiographic imaging to evaluate systolic strain with speckle tracking. Results Four of the patients died from cardiomyopathy. Three patients (including three of the four fatalities) with the same mutation (c.7911dupC [p.Asn2638Glnfs*24]) had cardiomyopathy with intra-familial variability in the severity of cardiomyopathy. Global longitudinal strain, a measure of systolic contractile function, was abnormal in all patients that can be measured. Conclusion Cardiac function in ALMS patients with infantile cardiomyopathy appears to have different clinical spectrums depending on the mutagenic allele. The c.7911dupC (p. Asn2638Glnfs*24) mutation can be related to severe cardiomyopathy. Parents can be informed and consulted about the progression of severe cardiomyopathy in a child carrying this mutagenic allele.

Objectives: We aim to characterize cardiac morphological and functional changes by echocardiography that are present in the early and late stages of Duchhenne type muscular dystrophy (DMD). Methods: Sixty-two patients with DMD and 62 healthy children were included in the study. Study group was divided into patients with systolic dysfunction group Ia and those without systolic dysfunction group Ib. Transthoracic echocardiography was performed in all individuals in the study. Left ventricular end diastolic diameter (LVEDd), left ventricular end systolic diameter (LVSd), posterior wall thickness (LVPWd), interventricular septal thickness (IVSD), ejection fraction and fractional shortening (FS), LV mass index (LVmassi), early (E) and late (A) transmitral inflow velocities, the ratio of early-to-late peak velocities (E/A) were recorded. Tissue Doppler measurements of the peak early (E') and late diastolic (A') annular velocities, Doppler measurements of pressure half time (PHT) and mitral valve area were measured. Results: DMD patients had significantly higher LVmass, LVmass index and mitral valve area (P < 0.05). Patients showed significantly lower EF values (P < 0.001), SF (P < 0.001), stroke volume (P < 0.05), cardiac output (P < 0.05), Mitral valve PHT (P < 0.001), Mitral E' (P < 0.001), Mitral A' (P < 0.001), E'/ A' ratio (P < 0.001) than the control patients. Group Ia patients had higher age, LVH and LV mass index, LVEDd (P < 0.05), LVSd (P < 0.05), IVSd (P < 0.05) and LVPWd (P < 0.05) compared to group Ib patients (P < 0.05). There was a positive linear correlation between age and LVPWd (P < 0.001, r = 0.446). Conclusions: Our study showed that diastolic functions began to deteriorate in the early phase of DMD and systolic impairment begins subclinically in early stages.

Background: Diabetes is an important disease that affects fetal development during pregnancy and causes metabolic disorders in newborns. Changes in serum glucose, ketone bodies, and somatomedin-C inhibitors in the diabetic pregnancies are responsible for the development of metabolic syndromes. The frequency of maternal hyperglycemia has reduced with careful monitoring during pregnancy of diabetic mothers. Nevertheless, morbidity in offspring of mothers with gestational diabetes mellitus (GDM) continues to be significant. We aimed to investigate the cardiac function of infants of mothers with GDM by echocardiographic (ECHO) Doppler and tissue Doppler methods and to evaluate their demographic features, somatomedin-C level and the relations them with each other. Methods: ECHO findings, laboratory data of newborns applied to our clinic between January and December 2016, and demographics of GDM mothers were examined. In the study, 108 neonates born to GDM mothers, and 100 healthy neonates (controls) were enrolled at postpartum 48th hour. Serum somatomedin-C levels were measured and detailed clinical and ECHO examination were performed. Isovolumetric relaxation (IVRT), isovolumetric contraction (IVCT), Myocardial Performance Index (MPI=ei Index) as an index that include both systolic and diastolic time intervals in expressing global systolic and diastolic ventricular functions [MPI: (IVRT + IVCT) /systolic ejection time] were calculated using both ventricular function M-mode, pulsed-wave and tissue Doppler ECHO methods. In addition, pulmonary arterial pressure (PAP) and tricuspid and mitral valves annular plan systolic excursion motions (TAPSE, MAPSE) were measured. Anamnesis and laboratory studies (fasting blood glucose [GHG] and glycosylated hemoglobin c [HbA1c]) measurements of all mothers were performed. Results: In the study group, 49 (45.4%) of infants were females and 59 were males (54.6%), the incidence of macrosomia was 4 (3.7%) in the study group and 2 (2.0%) in the control group. In comparison between the maternal groups, GDM mothers had significantly higher levels of fasting blood glucose (FBG) and HbA1c (p<0.0001). There was no significant difference in birth weight and somatomedin-C levels in neonates between two groups, but head circumference and height values were significantly higher in infants born to GDM mothers (p<0.05). In the echocardiographic examinations, small muscular ventricular septal defects were detected in 3 infants (2.77%) in study group, and 1 infant (1.0%) in control group. The M mode review revealed that diastolic end-interventricular septum thickness was significantly higher in IDMs group (p<0.001). In the evaluation of right ventricular function, MPI values were significantly higher in the infants of GDM group than control group (p<0.05). Also, IVCT, a diastolic function indicator, was significantly longer in the study group (p<0.05). There was a statistically insignificant decrease in TAPSE values in infants of GDM group (p>0.05). In evaluation of left ventricular function between groups, MPI and IVRT values were significantly higher in infants of GDM group (p<0.05), whereas MAPSE values were significantly decreased in the study group (p<0.05). Conclusion: Although infants of mothers with GDM were clinically normal in our study, we determined significant differences in diastolic parameters in addition to subclinical findings of ventricular septal thickness. We emphasize that cardiac involvement requires early diagnosis and neonatal approach even though complications in infants born to GDM mothers usually recover spontaneously. As expected, these infants are prone to various hyperglycemic adverse outcomes. Therefore, recognizing and treating hyperglycemia in time should be an important target for prevention of metabolic problems and deaths in severe cases.

We evaluated the risk of pulmonary hypertension (PH) by measuring the velocity of the tricuspid regurgitation jet velocity (TRV) on echocardiography and the associations among the TRV, ferritin, and history of splenectomy in children with β thalassemia major (TM). In total, 85 children with TM were examined with continuous Doppler flow. Patients with an abnormal TRV (>2.5 m/s) were grouped into those with a TRV of 2.5 to 2.9 m/s and TRV>2.9 m/s. A TRV of >2.5 m/s was identified in 72; 31 (36%) of these patients had a TRV of >2.9 m/s, suggesting a risk for significant PH. The ferritin concentration was significantly higher in patients with a TRV of >2.9 m/s and showed a positive correlation with a TRV. The TRV was significantly correlated with markers of diastolic function: the tricuspid peak early diastolic wave (E) was higher in patients with a TRV of >2.9 m/s and showed a significant correlation with the TRV (R=0.315). The ratio of the TRV over the velocity-time integral (VTI) at the right ventricular outflow tract (TRV/VTI RVOT), which is correlated with the pulmonary vascular resistance, was higher in patients with a TRV of >2.9 m/s. In total, 27 patients had splenectomy. Splenectomized patients had a higher TRV and splenectomy was correlated with the TRV (R=−0.221). A risk of PH as defined by a TRV of >2.9 m/s was common in our patients with TM. Screening with Doppler flow indices on echocardiography can detect PH in early stages.

Background Myocardial iron overload is the most common cause of mortality in patients with thalassemia major (TM), also known as beta-thalassemia. T2* cardiovascular magnetic resonance imaging (MRI) is the best way of monitoring cardiac iron, and new echocardiographic techniques can be used to assess cardiac function. Objectives The aim of this study was to assess the systolic and diastolic right ventricular (RV) function of patients with TM using tissue Doppler imaging (TDI) and to determine whether this echocardiographic technique is an adequate diagnostic tool for the screening and detection of subclinical cardiac dysfunction. Patients and Methods Eighty-four patients with TM were evaluated by conventional echocardiography and pulse-wave TDI. The data of the TM group (Group 1) were compared with that of 85 age- and sex-matched healthy controls (Group 2). Cardiovascular T2* MRI examinations were performed in 49 of the 85 patients. Results The patients with TM had significantly lower values for weight, height, body mass index, systolic arterial pressure, deceleration time, E’/A’, and ejection time (ET) than the controls. Group 1 also had significantly higher values for peak early diastolic velocity (E) over peak late diastolic velocity (A), peak early diastolic velocity of TDI (E’), peak late diastolic velocity of TDI (A’), E/E’, isovolumetric relaxation time, isovolumetric contraction time, and RV magnetic perfusion imaging (MPI) than Group 2. Conclusions RV diastolic dysfunction occurs before systolic deterioration in patients with TM and cannot be screened with conventional echocardiographic techniques. In routine practice, TDI measurements, MPI (for global function) and the E/E’ parameter (for diastolic function) can be used to screen and detect early RV dysfunction.

Amac: Bu calismanin amaci izole preaurikular tragusu olan cocuklarda eslik eden bobrek ve kalp malformasyonlarinin gorulme sikligininin belirlenmesi ve buna yonelik rutin inceleme yapilmasinin gerekliliginin ortaya konmasidir. Gerec ve Yontem: 1995-2004 yillari arasinda cocuk cerrahisi poliklinigine izole preaurikular tragus yakinmasi ile basvuran ve sendromik olmayan tum cocuklar prospektif olarak calismaya dahil edilmistir. Butun cocuklara uriner sistem ultrasonografisi (USG) uygulanmis; ayrica kardiak degerlendirme yapilmistir. Bulgular: Calismaya 46 cocuk dahil olmustur. Bu cocuklarin 4’u yenidogandir. Yenidoganlar haric tutuldugunda, ortanca yas 36 aydir (dagilim, 2 ay ile 10 yil). Cocuklarin 36’si (%65) erkek, 16’si (%35) kizdi. USG ile pelvikaliseal sistemde genislemesi olan 2 (%4) hasta tespit edildi. Bunlarin birinde bulgular kendiliginden gerilerken diger hastada ureteropelvik bileske darligi tespit edilerek duzeltici ameliyat yapildi. Kardiak degerlendirme sonucunda 1 (%2) hastada atrial septal defekt tespit edildi. Dogumsal bir kalp malformasyonu olmamakla beraber, bir baska cocukta da Wolf-Parkinson-White sendromu bulundu. Sonuc: Mevcut calismada elde edilen sonuclar izole preaurikular tragusu olan cocuklarda uriner sistemin degerlendirilmesine yonelik olarak rutin USG onerilmesini desteklemektedir. Ancak, bu cocuklarda kardiak degerlendirmenin rutin olarak yapilmasini onermeden once, yapilacak daha fazla prospektif calisma ile olgu sayisinin artirilmasi gerekmektedir. Anahtar Kelimeler: Preaurikular, Trag, Tragus, Renal, Kardiak, Cocuklar

The medical records of 685 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. Patients had been followed for a mean of 3 ± 2.5 years and median 2.2 years by echocardiography. VSD was perimembranous in 65.7% (450), trabecular muscular in 30.8% (211), muscular outlet in 2.3% (16), muscular inlet in 0.7% (5), and doubly committed subarterial in 0.5% (3). Defect size was classified in 76% (517) as small, in 18% (124) as moderate, and in 6% (44) as large. VSD closed spontaneously in 27% (186 of 685 patients) by ages 40 days to 13.7 years (mean, 2.1 ± 2.2 years; median, 1.3 years). Sixty-six of 450 perimembranous defects (15%) and 120 of 211 trabecular muscular defects (57%) closed spontaneously (p <0.001). Defect size became small in 15% of patients with VSD at mean 2.9 ± 2.3 years (median, 2.3 years). Aneurysmal transformation was detected in 56% (254), left ventricular-to-right atrial shunt in 8.4% (38), subaortic ridge in 5.8% (26), aortic valve prolapse in 11.7% (53), and aortic regurgitation in 7.3% (33) of 450 patients with perimembranous defect. There was no statistical significance between the age at closure and the age of detection of aneurysmal transformation in the patients with perimembranous defect (p = 0.25).

Aortic valve prolapse (AVP) was detected in 82 (7.5%) of 1096 patients with ventricular septal defect (VSD) (in 50 at initial echocardiographic examination and in 32 at follow-up) by echocardiography. Of 82 patients with AVP, aortic regurgitation (AR) was detected in 53 (65%) (in 28 at initial echocardiographic examination and in 25 at follow-up), resulting in an incidence of AR of 4.8% (of VSD). The percentage of AVP (20.8%) and AR (16.7%) in muscular outlet VSDs was larger than the percentage of AVP (10.6%) and AR (6.8%) in perimembranous VSDs (p <0.05). Fourty-four patients were followed medically after AVP appeared (3 months to 10.8 years; median, 2.1 years). Initially, there was no AR in 24 of these patients, trivial AR in 7, and mild AR in 13. Trivial AR developed in 6 (25%) and mild AR developed in 3 (13%) of 24 patients who had no AR (in 5 of them within 1 year and in 9 of them within 2 years). In 2 (29%) of 7 patients, trivial AR progressed to mild AR during a median of 2 years, and in 4 (31%) of 13 patients, mild AR progressed to moderate AR during a median of 1.1 years. We recommend frequent echocardiographic evaluation (every 6 months) for detecting of appearance of AR in patients with perimembranous or muscular outlet VSD after AVP develops and for evaluating the progression of AR in patients with perimembranous or muscular outlet VSD, AVP, and trivial AR. In addition, we recommend surgical intervention in patients with perimembranous or muscular outlet VSD, AVP, and mild AR because of rapid progression of mild AR to moderate AR.

Kendiliginden kapanma sol ventrikul sag atriyum santi subaortik cikinti ve aort kapak prolapsusunu saptamak icin 685 ventrikuler septal defektli hastanin kayitlari incelendi Hastalar ekokardiyografi ile 1 ay ile 10 9 yil ortalama 3±2 5 yil izlendi Ventrikuler septal defekt 450 sinde perimembranoz 65 7 5 inde muskuler giris 0 7 211 inde trabekuler muskuler 30 8 16 sinda muskuler cikis 2 3 ve 3 unde doubly committed subarterial di 0 5 685 hastanin 186 sinda ventrikuler septal defekt 40 gun ile 13 7 yas arasinda degisen yaslarda ortalama 2 1±2 22 yas ortanca 1 3 yas kendiliginden kapandi 27 450 perimembranoz defektin 68 i 15 ve 211 trabekuler muskuler defektin 118 i 56 kendiliginden kapandi p lt;0 005 Perimembranoz defektlerin kapanma yasi ortalama 2 8±2 3 yas trabekuler muskuler defektlerin kapanma yasindan ortalama 1 8±2 1 yas daha buyuktu p lt;0 003 450 perimembranoz defektli hastanin 254 unde 56 anevrizmal degisim 38 inde sol ventrikul sag atriyum sant 8 4 26 sinda subaortik cikinti 5 8 53 unde aort kapak prolapsusu 11 7 ve 33 unde aort yetersizligi 7 3 saptandi Aort kapak prolapsusu 16 muskuler cikis defektli hastanin 5 inde 31 ve 3 doubly committed subarterial defektli hastanin birinde saptandi Perimembranoz defektli hastalarda defektin kapanma yasi ile anevrizmal degisim saptanma yasi arasinda istatistiksel farklilik saptanmadi p=0 25 Anevrizmal degisim saptanma yasi sol ventrikul sag atriyum santi subaortik cikinti ve aort kapak prolapsusu saptanma yasindan daha kucuktu sirasiyla p lt;0 03 0 05 ve 0 0007 Anahtar kelimeler: Ventrikuler septal defekt subaortik cikinti aort kapak prolapsusu

Adenozin endojen bir piinn nükleosiiidir. A t r i yovcntr ıkü lcr ( A V ) düğüm iletimim deprese eder. Bu nedenle AV düğümü reeniran halka olarak kul­ lanan taşikardilerde etkili olduğu gösterilmiştir. Sııpravcntriküler tasikaı dilerin akut tedavis inde gerek erişkin, gerekse çocuk hastalarda yaygın olarak kullanılmaya başlanmıştır (1-3). Bu vakamızda adenozinin albuterol kullanımı­ na sekonder başlayan sııpravcntriküler taşikardı (SVT) atağını başarı i le sonlandırdığmı gösterdik. Literatürde bulabildiğimiz kadarı ile ikinci vaka ol ­ ması nedeniy le ilginç bulduğumuz olgumuzu sun­ mak i stedik.