In heterotaxia syndrome with left atrial isomerism, the distinguishing feature is interrupted inferior vena cava with azygos continuation. We report using a transjugular approach to device closure of an atrial septal defect in an 8-year-old boy with heterotaxia syndrome. We found that device closure of the child's atrial septal defect through a jugular venous approach was safe when an inferior vena cava approach was not possible. To our knowledge, ours is the first report of the use of internal jugular vein access to close an atrial septal defect in a child.

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Citations (16)

Scimitar Sendromu ile Birlikte Aort Koarktasyonu

Turk Kardiyoloji Dernegi Arsivi-Archives of the Turkish Society of Cardiology (2000)

Osman Küçükosmanoğlu Nazan Özbarlas Sevcan Erdem Gülbin Bingöl Karakoç

Scimitar syndrome

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Two newborn babies with generalized arterial calcification of infancy, two new mutations

The Turkish Journal of Pediatrics (2016)

Hacer Yapıcıoğlu-Yıldızdaş Nazan Özbarlas Sevcan Erdem Mehmet Bertan Yılmaz Ferda Özlü

Idiopathic generalized arterial calcification of infancy-1 (GACI-1) is a rare and potentially lethal disease characterized by diffuse calcification of large and medium-sized arteries such as aorta, renal, pulmonary, cerebral and mesenteric arteries. Here we report two new mutations in two newborn babies with GACI-1 treated with bisphosphonates, and their progress in the first year of life.

Abdominal aorta

Mesenteric arteries

10.24953/turkjped.2016.04.013

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Citations (5)

[Study of bone changes in 11 cases of iron-deficiency anemia].

PubMed (1965)

M Aksoy N Camli Sevcan Erdem

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[2 cases of puerperal osteomalacia].

PubMed (1962)

A Görpe Sevcan Erdem G Onol A Arinik

Osteomalacia

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Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome

The Anatolian Journal of Cardiology (2022)

Kadir Babaoğlu Yasemin Doğan Sevcan Erdem Nazan Özbarlas Eviç Zeynep Başar

The aim of this study is to review the spectrum of the prenatally detected absent pulmonary valve syndrome and its outcome after diagnosis.Clinical data and echocardiographic findings of 37 cases with a fetal diagnosis of absent pulmonary valve syndrome between 2008 and 2020 were analyzed in this retrospective multicenter study.Median gestational age at diagnosis was 25 weeks. Three subtypes of absent pulmonary valve syndrome were observed: (1) with tetralogy of Fallot (n=30; 81.0%); (2) absent pulmonary valve syndrome with intact ventricular septum (n=5; 13.5%); (3) with complete atrioventricular septal defect (n=2; 5.4%). In contrast to 7/25 fetuses (28%) with tetralogy of Fallot-absent pulmonary valve syndrome who had a patent ductus arteriosus, all 5 fetuses with absent pulmonary valve syndrome-intact ventricular septum had a patent ductus arteriosus (P < .001). No significant difference was found between the z-scores of pulmonary artery branches in fetuses with or without patent ductus arteriosus (P > .05). The analysis did not reveal any correlation between gestational week and z-scores of pulmonary artery, pulmonary artery branches (right pulmonary artery, left pulmonary artery), and ratio of aorta/pulmonary artery ratio. The echocardiographic measurements of survivors did not differ significantly from non-survivors (P > .05). Extracardiac anomalies were observed in 8/37 fetuses (21.6%). The incidence of extracardiac anomaly was significantly higher in cases of tetralogy of Fallot-absent pulmonary valve syndrome (P < .05). Overall, 9 fetuses (24%) had genetic anomalies. All 6 fetuses (20%) with 22q11.2 microdeletion were within the tetralogy of Fallot-absent pulmonary valve syndrome group. Overall survival after initial diagnosis in the total cases was 36.6% (11/30), with 9 of 30 (30%) tetralogy of Fallot-absent pulmonary valve syndrome cases and 2 of 5 (40%) absent pulmonary valve syndrome-intact ventricular septum cases.In this largest series of absent pulmonary valve syndrome, extracardiac, and chromosomal anomalies were found to be a common occurrence. The risk of 22q11.2 microdeletion was higher in tetralogy of Fallot cases at 40%. The sizes of the pulmonary artery and its branches and the aorta had no correlation of high mortality antenatally or after birth, which were 63.4% and 47.7%, respectively.

Ductus arteriosus

Pulmonary valve

Fetal echocardiography

Left pulmonary artery

10.5152/anatoljcardiol.2022.1461

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Pre-Pulseless Takayasu Arteritis in a Child Represented With Prolonged Fever of Unknown Origin and Successful Management With Concomitant Mycophenolate Mofetil and Infliximab

Archives of Rheumatology (2019)

Rabia Miray Kışla Ekinci Sibel Balcı Ferhat Can Pişkin Celal Varan Sevcan Erdem

Takayasu arteritis (TA) is classified as a large vessel vasculitis of predominantly aorta and its main branches, resulting in fibrosis and stenosis. Only a minority of TA patients are diagnosed in pre-stenosis phase when constitutional symptoms including fever, arthralgia, weight loss, headache, abdominal pain, and elevated acute phase reactants are dominant insidious characteristics. In this article, we present a 12-year-old female patient, who was referred to our department with a one-year history of low-grade fever, fatigue, and myalgia. Physical examination did not reveal pulse and blood pressure discrepancies between any extremities. Acute phase reactants were markedly elevated, and autoantibodies were negative. Magnetic resonance angiography (MRA) findings have confirmed TA diagnosis with prominent vessel wall thickening in the ascendant and abdominal aorta, focal ectasias and a thoracoabdominal fusiform aneurysm. As methotrexate and methylprednisolone treatment during three months was unsuccessful, infliximab was induced. During the next 12 months, patient had clinical improvement, but worsening of MRA findings and new onset of carotidynia forced us to switch methotrexate to mycophenolate mofetil. Six months later, laboratory and radiological remission were achieved. In conclusion, we report a challenge to diagnose pre-pulseless childhood-TA (c-TA) in the state of prolonged fever with no signs of vascular stenosis, systemic hypertension, pulses and blood pressure discrepancies, bruits and claudication. Therefore, we wish to discourse the importance of early diagnosis of TA since, to our knowledge, there are no studies investigating treatment success only in the early phases of c-TA.

Arteritis

Claudication

Magnetic resonance angiography

myalgia

10.46497/archrheumatol.2020.7599

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Citations (3)

Mid-term results of patients following total surgical correction of tetralogy of Fallot.

PubMed (2013)

Sevcan Erdem Nazan Özbarlas Osman Küçükosmanoğlu Hakan Poyrazoğlu Orhan Kemal Sali̇h

The aim of this study was to evaluate the mid-term clinical status of patients following tetralogy of Fallot (TOF) repair. We performed a cross-sectional observational analysis of 57 postoperative TOF patients and 58 age-matched controls. Patients were examined with myocardial performance index (MPI), which was obtained by tissue Doppler imaging (TDI) in addition to the conventional methods. Compared with controls, patients had significantly higher right ventricular (RV) dimensions and volumes, RV MPI and left ventricular (LV) MPI, and significantly lower RV ejection fraction (EF), LV EF, exercise duration, and maximum heart rate with exercise (p<0.001 for all parameters). A positive correlation was found between RV MPI and LV MPI (r=0.541, p<0.001). There was a negative correlation between LV MPI and exercise duration (r=0.260, p=0.034). After total surgical correction of TOF, the patients had a good clinical status at the mid-term follow-up; decreased exercise capacity and impaired functions in both ventricles related to the degree of pulmonary regurgitation were found. By using MPI, impaired cardiac functions can identify such conditions before they become clinically symptomatic.

Doppler imaging

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Citations (3)