Evaluation of atrial electromechanical conduction delay in case of hemodynamically insignificant rheumatic heart disease: A tissue Doppler study

Background: Atrial electromechanical delay (AEMD) that reflects delayed conduction may show us the clinical reflection of pathological changes in the atria. The main objective of the present study is to investigate AEMD in patients who had previous rheumatic carditis but without hemodynamically significant valvular disease. Methods: A total of 40 patients, previously diagnosed as rheumatic carditis but without significant valvular stenosis/regurgitation and atrial enlargement; and 39 age- and-sex matched controls were enrolled for the present study. Parameters of AEMD (lateral mitral annulus electromechanical delay, septal mitral annulus electromechanical delay and lateral tricuspid annulus electromechanical delay) were measured with tissue Doppler echocardiography and left intra-atrial and inter-atrial conduction times were calculated accordingly. A 24h ambulatory Holter monitoring was used in both groups to detect atrial fibrillation episodes and quantify atrial extrasystoles. Results: Parameters of AEMD, including left intra-atrial and inter-atrial conduction times of subjects in the study group were longer compared to the control group (23.7 ± 7.0 vs. 18.3 ± 6.2). Conclusions: Increased AEMD is observed in patients with previous rheumatic carditis and no significant valvular stenosis/regurgitation and atrial enlargement, which may partly explain the increased incidence of atrial fibrillation observed in these patients

Yenidoğan döneminde dilate kardiyomiyopatinin nadir bir nedeni: ALCAPA sendromu

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. ALCAPA is asymptomatic in many cases in newborn periode. It is mostly diagnosed in the first few months. In this case report, we present a newborn with ALCAPA who admitted to our clinic with heart failure occurred at an earlier age than expected. The electrocardiography showed deep wide Q waves in D1 and aVL, ST elevation in leads V1-V6. Echocardiography revealed a dilated cardiomyopathy and left main coronary artery originates from pulmonary artery. Diagnosis was confirmed by coronary angiography. In this report, we emphasized that ALCAPA may cause dilated cardiomyopathy also in newborn period and we aimed that enhanced awareness of this disease. (Turk Arch Ped 2011; 46:256-8

A rare cause of dilated cardiomyopathy in the newborn period: ALCAPA syndrome

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. ALCAPA is asymptomatic in many cases in newborn periode. It is mostly diagnosed in the first few months. In this case report, we present a newborn with ALCAPA who admitted to our clinic with heart failure occurred at an earlier age than expected. The electrocardiography showed deep wide Q waves in D1 and aVL, ST elevation in leads V1-V6. Echocardiography revealed a dilated cardiomyopathy and left main coronary artery originates from pulmonary artery. Diagnosis was confirmed by coronary angiography. In this report, we emphasized that ALCAPA may cause dilated cardiomyopathy also in newborn period and we aimed that enhanced awareness of this disease. (Turk Arch Ped 2011; 46:256-8

Scimitar Syndrome: A Complex Form of Anomalous Pulmonary Venous Return

Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital anomaly, in which all the right pulmonary veins drain into the inferior vena cava. In this study, we review the diagnostic features, clinical management, and surgical strategy in the Scimitar syndrome and discuss the significance of new generation diagnostic imaging methods for this rare anomaly. doi: 10.1111/j.1540-8191.2011.01309.x (J Card Surg 2011;26:529-534

Ventricular Septal Defect Closure in a Patient with VACTERL Syndrome: Anticipating Sequelae in a Rare Genetic Disorder

Noncardiac components of genetic disorders can complicate the operative and postoperative courses of pediatric cardiac surgery patients. Prolonged hospital stay, increased treatment cost, morbidity, and death are more likely in this subgroup of patients

Echocardiographic follow-up of children with isolated discrete subaortic stenosis

This study evaluates the progression of stenosis, onset and progression of aortic regurgitation (AR), and the results of surgical outcomes in children with isolated discrete subaortic stenosis (SAS). The medical records of 108 patients (mean age, 5.5 +/- 3.8 years; range, 3 days to 18 years) with isolated discrete SAS were reviewed. Patients with lesions other than AR were excluded. Very mild stenosis was defined as Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25-49 mmHg, moderate stenosis as 50-75 mmHg, and severe stenosis as more than 75 mmHg. Seventy-eight of 108 patients were followed for 2 months to 14 years (mean, 4.8 +/- 3.7 years; median, 5 years) with medical treatment alone. In these patients, the mean PSIG at last echocardiogram was higher than the mean PSIG at initial echocardiogram (39 +/- 19 vs 31 +/- 12 mmHg, respectively; p < 0.001). Among 24 patients with very mild stenosis at initial echocardiogram, 10 had mild and 2 had moderate stenosis after a mean period of 5.6 years. Among 46 patients with mild stenosis at initial echocardiogram, 11 had moderate and 5 had severe stenosis after a mean period of 4.1 years. Only 1 patient among the 8 patients with moderate stenosis at initial echocardiogram had severe stenosis after a mean period of 2.7 years. Thirty-nine patients (50%) had AR (13% trivial, 33% mild, and 4% moderate) at initial echocardiogram. After a mean period of 4.8 years, 77% of the patients had AR (10% trivial, 53% mild, 9% mild-moderate, and 5% moderate). Twenty-four patients underwent surgery. Preoperatively, mean Doppler PSIG and AR incidence were 64 +/- 17 mmHg and 91% (22/24), respectively. The mean Doppler PSIG was 30 +/- 19 mmHg and AR was present in all of the patients a mean period of 4.1 years after surgery. Two patients underwent reoperation for recurrent SAS and AR. Patients with very mild or mild stenosis may be followed noninvasively every year. One patient of the 8 patients with moderate stenosis progressed to severe stenosis, and moderate AR developed in 2 patients after a mean of 2.7 years. We recommend that patients with moderate stenosis undergo careful evaluation to determine whether surgery is necessary due to the severity of stenosis and AR

Echocardiographic follow-up of children with supravalvular aortic stenosis

This study evaluates the course of supravalvular aortic stenosis (SVAS)-associated right ventricular outflow tract (RVOT) obstruction and the results of surgery in children. We reviewed the medical records of 24 patients diagnosed with SVAS at initial echocardiographic examination or during the following period of RVOT obstruction. Very mild SVAS was defined as a transvalvular Doppler peak systolic instantanous gradient (PSIG) less than 25 mmHg, mild stenosis as 25-49 mmHg, moderate stenosis as 50-75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 3.1 +/- 2.9 years (range, 7 days to 12.7 years), and 18 of the patients (72%) were male. Fifteen patients had Williams' syndrome. Seventeen patients (71%) were followed for a mean of 5.2 +/- 3.8 years (range, 7 months to 13.5 years). Among 17 patients with complete follow-up records, 1 (6%) had very mild, 5 (29%) mild, 3 (18%) moderate, and 3 (18%) severe aortic stenosis at initial echocardiographic examination. In a newborn patient with mild pulmonary valvular stenosis. SVAS became evident after 2 months and progressed rapidly. Supravalvular aortic stenosis was very mild in 4 patients (23%), mild in 3 (18%), moderate in 3 (18%), and severe in 7 (41%) at last echocardiographic examination. Of 17 patients who were followed, 11 (65%) had RVOT obstruction at initial echocardiographic examination. RVOT obstruction disappeared in 5 patients, regressed in 1 patient, and appeared in 1 patient over the follow-up period. Four patients underwent operation. It appears reasonable that patients with very mild and mild stenosis should be followed medically every 1 or 2 years and patients with moderate stenosis once a year. Newborns with SVAS should be followed for rapid progression of SVAS. In some patients, RVOT obstruction may disappear, and SVAS may develop in others with RVOT obstruction. Patients with RVOT obstruction (at the valvular, supravalvular, or peripheral pulmonary arterial level) should be evaluated carefully for development of SVAS at follow-up

Rare Variation in Partial Anomalous Venous Drainage in 2 Cases: Diagnosis, Assessment Methods, and Surgical Approach

Where pulmonary veins drain and their relationship with an atrial septal defect (ASD) are important. A sinus venosus (high venosum) type of defect is the most common pathology accompanying partial anomalous pulmonary venous connection. Typically, the right superior pulmonary vein and occasionally the middle pulmonary vein drain into the junction of the superior vena cava (SVC) and the right atrium (RA), and a sinus venosus type of ASD usually accompanies these anomalies. In this report, we assess a very rare pathology in which 3 right pulmonary veins (superior, middle, and inferior) drain into the SVC-RA junction with respect to diagnostic methods and in the light of 2 cases involving patients in 2 different age groups