İlker Kemal Yücel

Diagnosis, treatment and outcomes of patients with aortopulmonary window.

BACKGROUND Aortopulmonary window (APW) is a communication between the ascending aorta and the pulmonary artery in the presence of two separate semilunar valves and is the rarest of septal defects. AIMS To present our experience with the diagnosis and outcome of APW cases. STUDY DESIGN Retrospective cohort study. METHODS Between June 2003 and October 2011, thirteen patients were diagnosed with APW. Clinical features of patients, findings of echocardiographic and angiographic examination, results of surgical intervention and follow-up were reviewed retrospectively. RESULTS Eleven children (10 days to 16 years), underwent surgical correction of APW. In a 12-month-old boy, the defect was repaired by the transcatheter approach. In addition to APW repair, closure of VSD was performed in 2 patients. APW were associated with interruption in two patients; one also had a complex pathology. None of the patients died due to complications of surgical or transcatheter procedures. After a median follow-up period of 40 months, the patients were asymptomatic and none of them required additional medication, except for the patient with complex pathology including an interrupted aortic arch, who underwent balloon angioplasty for recoarctation. CONCLUSION In any infant with the findings of congestive heart failure and failure to thrive, APW must be kept in mind as a differential diagnosis. In isolated APW cases before 6 months of age, echocardiography is often sufficient for diagnosis. In complex cases, cardiac catheterisation is performed for the comprehensive evaluation of associated defects. After 6 months, cardiac catheterisation could be utilised to perform vasoreactivity testing and, if possible, to close the defect.

Comparison of Transcatheter Atrial Septal Defect Closure in Children, Adolescents and adults: Differences, Challenges and Short-, Mid- and Long-Term Results

Background and Objectives This study aims to compare the characteristics, effectiveness and results of transcatheter closure of atrial septal defect between children, adolescents, and adults. Subjects and Methods In this study, 683 patients who underwent atrial septal defect closure in the last 10 years were divided into three groups: children (age <12), adolescents (age 12 to 16), and adults (age >16) as group 1, group 2 and group 3, respectively. Results The average defect size and incidence of complex atrial septal defect were higher in group 3 (p=0.0001 and 0.03 respectively). While the average size of the devic was higher in adults (22.6±6.4 mm vs. 18.5±4.9 mm; p=0.0001), the ratio of the device size/total septum was higher in both children and adolescents (Group 1 and 2). In the child and adolescent groups and patients with only complex atrial septal defect, the use of techniques, other than standard deployment, was similar in all three groups (p=0.86 and 0.41, respectively). The ratio of the residual shunt was similar in all three groups. Major complications were seen in 5 cases (4 cases with migration, and 1 case with dislocation) in group 3 and 1 case (migration) in group 1. Conclusion Depending on the complexity of the defect and age of the patient, transcatheter closure of atrial septal defect might have certain difficulties and complications. Patients must be evaluated in detail to avoid major complications and possible problems during the procedure.

Assessment of cardiac function in absence of congenital and acquired heart disease in patients with Down syndrome

BackgroundExtra genetic material in patients with Down syndrome (DS) may affect the function of any organ system. We evaluated cardiac functions using conventional tissue Doppler and two-dimensional speckle tracking echocardiography in patients with DS in the absence of congenital and acquired heart disease in patients.MethodsA total of 115 patients with DS between 6 and 13 years of age with clinically and anatomically normal heart and 55 healthy children were included in this cross-sectional study. DS was diagnosed by a karyotype test. Patients with mosaic type were not included in this study. Systolic and diastolic functions were evaluated by echocardiography.ResultsPulsed waved Doppler transmitral early/late inflow velocity (E/A), tissue Doppler mitral annular early/late diastolic peak velocity (Ea/Aa), transtricuspid E/A and tricuspid valve annulus Ea/Aa, pulmonary venous Doppler systolic/diastolic (S/D) wave ratio were lower in patients with Down syndrome than in the control group (P=0.04, P=0.001, P<0.05, P<0.001, P<0.001, respectively). Mitral and tricuspid annular Ea were lower in patients with DS (P<0.001). The right and left ventricular myocardial performance indexes were higher in patients with DS than in the controls (P<0.01). They had significantly higher left ventricular mass, ejection fraction, the mitral annular plane systolic excursion values. However, the Down syndrome group compared with the controls had a lower strain values examined by two-dimensional longitudinal speckle-tracking strain echocardiography.ConclusionThese findings suggest conventional tissue Doppler and two-dimensional longitudinal speckletracking strain echocardiography were useful methods of investigating ventricular function and identifying a higher incidence of biventricular dysfunction in patients with Down syndrome compared with the healthy controls.

Efficacy of very low-dose prostaglandin E1 in duct-dependent congenital heart disease.

AIM The present study aims to define the lowest effective prostaglandin E1 dose in patients with inadequacy of pulmonary blood flow and/or intracardiac blood mixing and those with inadequate systemic blood flow. METHODS Patients with inadequacy of both pulmonary blood flow and/or blood mixing (Group 1) and those with inadequate systemic blood flow (Group 2) were retrospectively evaluated in two separate groups with regard to the prostaglandin E1 starting dose given in the referring facility, the lowest and the highest dose administered in our centre, treatment duration, adverse effects, and administered treatment. RESULTS No difference between the groups could be detected with respect to sex or birth weight (p=0.95 and 0.42, respectively). Group 1 and Group 2 were statistically similar in aspect of prostaglandin treatment duration (9.73±0.81 days versus 11.6±1.05 days, p=0.064). When compared with Group 2, the initial, maintenance and lowest efficient doses of prostaglandin E1 treatment were significantly lower and the titrated dose of prostaglandin E1 was significantly higher in Group 1 (p=0.001 for each). CONCLUSION Our findings indicate that the infusion of prostaglandin at a very low dose (0.003-0.005 mcg/kg/minute) is sufficient to maintain the patency of the ductus arteriosus. A higher dose of prostaglandin E1 may be necessary in patients with inadequate systemic blood flow.

Cardi-O-Fix duct occluder versus amplatzer duct occluder for closure of patent ductus arteriosus

We sought to investigate the safety, efficacy, and follow‐up results of percutaneous patent ductus arteriosus (PDA) closure using the novel Cardi‐O‐Fix duct occluder (CDO), a device similar to but less expensive than the Amplatzer duct occluder (ADO). We also aimed to compare these two devices in terms of results.

Stenting of the ductus arteriosus in infants with functionally univentricular heart disease and ductal-dependent pulmonary blood flow: A single-center experience

To determine the short‐ and medium‐term outcomes of ductal stenting (DS) in patients with functionally univentricular hearts (FUHs) and ductal‐dependent pulmonary blood flow.

Echocardiographic diagnosis and transcatheter occlusion of pulmonary arteriovenous fistula in cyanotic newborn.

Pulmonary arteriovenous malformation (PAVM) is a rare cause of cyanosis in newborn. A 12-day-old male newborn (2.8 kg) was referred to our hospital with the complaints of cyanosis and respiratory distress. On two-dimensional echocardiography, the right pulmonary artery (PA) appeared larger than left PA and the left atrium, left ventricle were dilated. The right heart chambers were in normal limits. A color flow Doppler echocardiogram revealed a turbulent flow due to a PAVM originating from medium branch of right PA, and continuous wave Doppler showed continuous flow pattern. Agitated saline injection resulted in the delayed appearance of the contrast in the left-side chambers three to four heart cycles after appearance in the right-side chambers; the study was considered positive and indicative of an intrapulmonary shunt. Selective angiography of the right PA confirmed the diagnosis of a large solitary PAVM in the right middle lobe with a feeding artery. Amplatzer vascular plug I, which is designed to close abnormal vascular structures, was chosen to close the PAVM. The deployment of device performed safely and the oxygen saturation of baby increased to 95% immediately after deployment. Heart failure and respiratory distress also resolved after the procedure.

Two Cases of Transcatheter Closure of Central Aortopulmonary Shunts: One with an Amplatzer Duct Occluder II and One with an Amplatzer Vascular Plug I

When total correction is not possible in infants who have a cyanotic congenital heart disease, creation of a palliative aortopulmonary shunt is essential. A central aortopulmonary shunt is preferable, because of its technical and hemodynamic advantages. Overcirculation, thrombosis, and stenosis of the shunt are the main postoperative sequelae that necessitate urgent reintervention. Percutaneous transcatheter closure of aortopulmonary shunts can eliminate the need for reoperation and substantially decrease postoperative morbidity and mortality rates. We report our successful transcatheter closures of central aortopulmonary shunts in a 3-month-old infant and a 15-year-old girl, with use of an Amplatzer Duct Occluder II and an Amplatzer Vascular Plug I, respectively. To our knowledge, this is the first report of the transcatheter closure of central aortopulmonary shunts with these 2 devices.