Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Surgical Outcomes and Long-Term Follow-Up of Patients with Large Ventricular Septal Defects with Severe Pulmonary Arterial Hypertension: Single-Center Experience(2023) Orgun, Ali; Tokel, Kursad; Erdogan, Ilkay; Varan, Birgul; Ozkan, Murat; Aslamaci, SaitPurpose: In this retrospective study, pediatric patients who underwent surgery for large ventricular septal defect (VSD) with severe pulmonary arterial hypertension (PAH) were evaluated in detail in terms of clinical characteristics, and long-term results, and reinterpreted with the current surgical criteria.Materials and Methods: Twenty-four patients who underwent surgery for large VSD due to PAH between 1994 and 2005 were included in the study. The patients were divided into two groups according to their pulmonary vascular resistance index (PVRI) and compared (group 1 with PVRI <6 WU.m(2) and group 2 with PVRI >= 6 WU.m(2)). The patients with PVRI 6-8 WU.m(2) were accepted as being in the gray zone and were evaluated in detail.Results: Age at diagnosis ranged from 5 months to 17 years (median: 5 years). PVRI ranged between 3 and 18.4 WU.m(2) (median PVRI was 5 WU.m(2).) According to the PVRI, 13 patients were accepted as group 1, and 11 patients were accepted as group 2. mPAP, PVR/SVR, and last PAP values were found statistically significantly lower in group 1 than in group 2. The median postoperative follow-up time was 6 years (range, 1 month-18 years). PAH was seen in 15/24 (62.5%) patients. Severe PAH was observed in seven of these 15 patients and all were in group 2 (four had PVRI >= 8WU.m(2) and three had PVRI 6- 8 WU.m(2)).Conclusion: Patients with large VSD with severe PAH should be carefully evaluated before surgery to more accurately identify suitable candidates.Item Embolization of Pulmonary Sequestration with Onyx: An Unusual Application(2014) Gursu, Alper Hazim; Boyvat, Fatih; Varan, Birgul; Erdogan, Ilkay; https://orcid.org/0000-0002-0707-2678; https://orcid.org/0000-0002-6719-8563; https://orcid.org/0000-0001-6887-3033; 24643150; AHI-4502-2022; F-4230-2011; ABB-1767-2021; ABB-2220-2021We report a baby with intralobar pulmonary sequestration who was successfully treated with a new embolization agent, Onyx. A 1.5-month-old female infant was admitted to our hospital with sweating and fatigue. Telecardiography showed cardiomegaly, dextrocardia, and increased pulmonary vascular markings. In thoracic computerized tomography, pulmonary sequestration, right pulmonary hypoplasia, and large collateral arteries were seen. The collateral arteries were originating from the celiac trunk and aorta. Echocardiography revealed enlargement of the left atrium and ventricle and left ventricle systolic dysfunction. Angiography revealed a large feeding artery and three branches originating from the aorta and another feeding artery originating from the celiac trunk. We performed embolization of the feeding arteries and their branches, with coils and Onyx. The procedure was performed without complications, and all feeding arteries were completely occluded. The infant started to gain weight. One year later, the infant's body weight had increased and she had no respiratory problems or signs of congestive heart failure. In this case report, we suggest that embolization with Onyx is a reliable alternative method to surgery for infants with pulmonary sequestration. With future studies, pulmonary sequestration embolization with Onyx may become an acceptable and easy treatment option in pediatric patients.Item The Roberts Syndrome: A Case Report of an Infant with Valvular Aortic Stenosis and Mutation in ESCO2(2014) Dogan, Mustafa; Firinci, Fatih; Balci, Yasemin Isik; Zeybek, Selcan; Ozgurler, Funda; Erdogan, Ilkay; Varan, Birgul; Semerci, Cavidan Nur; https://orcid.org/0000-0002-6719-8563; 24864645; ABB-1767-2021Roberts syndrome, which is inherited as an autosomal recessive group of disorders, is a rare syndrome characterized with symmetrical extremity defects, craniofacial abnormalities, and prenatal and postnatal growth retardation. Here, we present a case of Roberts Syndrome brought to the clinic with diarrhoea and multiple abnormalities, that had tetra phocomelia, growth and developmental retardation, abnormality of complete cleft lip-palate accompanied with Aortic stenosis and PDA, and in which cytogenetic analysis identified premature centromere separation. Mutation analysis of ESCO2 revealed a splice site mutation [c.1131+1G>A] in intron 6 in homozygous status in the patient and heterozygous status in the parents. Our case is the first Robert- Syndrome with valvular aortic stenosis in the literature, to the best of our knowledge.Item Multiple Giant Aneurysms and Stenoses of the Coronary and Systemic Arteries in an Infant with Kawasaki Disease at the Early Stage of Convalescent Period(2014) Ekici, Filiz; Varan, Birgul; Kocabas, Abdullah; Erdogan, Ilkay; Eminoglu, Sancar; Aktas, Dogukan; https://orcid.org/0000-0002-6719-8563; 24528198; ABB-1767-2021; AAD-2644-2019; AAJ-2305-2021Myocardial infarction and systemic arterial aneurysms are rarely seen during the course of the Kawasaki disease (KD). Herein, we report the case of a 4-month-old Turkish infant who was diagnosed with KD on the 17th day of the illness. On admission, echocardiogram showed multiple coronary arterial aneurysms (CAAs) and massive pericardial effusion. He was given intravenous immunoglobulin, aspirin and anticoagulant drugs. However, the aneurysms progressed to super giant CAAs, multiple huge coronary arterial thromboses developed recurrently and caused myocardial ischemia. Furthermore, the conventional angiography revealed multiple giant aneurysms and stenoses in the subclavian, celiac, and iliac arteries, besides CAAs. Mini-Abstract We report the case of a 4-month-old Turkish infant who was diagnosed with Kawasaki disease associated with multiple super giant coronary aneurysms, huge coronary thromboses, and massive pericardial effusion. Multiple systemic arterial aneurysms and stenoses were also detected in subclavian, axillary, renal, and iliac arteries as well as coronary arteries at the beginning of convalescent period.Item Use of Oral Budesonide in The Management of Protein-Losing Enteropathy Due to Restrictive Cardiomyopathy(2014) Gursu, Hazim A.; Varan, Birgul; Erdogan, Ilkay; https://orcid.org/0000-0002-0707-2678; https://orcid.org/0000-0002-6719-8563; https://orcid.org/0000-0001-6887-3033; 24029379; AHI-4502-2022; ABB-1767-2021; ABB-2220-2021A 7-year-old male patient who had abdominal swelling and eyelid oedema was diagnosed with restrictive cardiomyopathy. His serum albumin level was 2.3 g/dl. Protein-losing enteropathy due to restrictive cardiomyopathy was diagnosed and oral budesonide was started. His serum albumin level began to rise and ascites and peripheric oedema disappeared. The patient underwent a successful cardiac transplantation and budesonide was stopped. After the heart transplantation, the albumin level decreased to 2.3 g/dl, and therefore it was restarted. When the serum albumin level increased, the budesonide dose was tapered and stopped in 1 month. Budesonide may be an effective drug in patients with protein-losing enteropathy due to heart failure.Item Transposition of The Great Arteries and Cor Triatriatum: A Rare Combination(2014) Gursu, Hazim A.; Varan, Birgul; Erdogan, Ilkay; Oktay, Ayla; https://orcid.org/0000-0002-0707-2678; https://orcid.org/0000-0002-6719-8563; https://orcid.org/0000-0001-6887-3033; 24018011; AHI-4502-2022; ABB-1767-2021; AAJ-2305-2021In this case report, we present a 5-month-old girl diagnosed with a unique combination of transposition of the great arteries and cor triatriatum sinistra. A 1-day-old female patient presented to our hospital with cyanosis since the early neonatal period. We confirmed transposition of the great arteries by echocardiography. The patient underwent arterial switch operation on day 8 and was discharged on day 35. After 5 months of the operation, the patient had a lower respiratory tract infection and was unable to gain weight. Echocardiography revealed mild neopulmonary regurgitation, minimal neoaortic regurgitation, and pulmonary arterial hypertension. In addition, a fibrous membrane was also seen dividing the left atrium. The patient was diagnosed with cor triatriatum and underwent successful resection of the membrane.Item A Rare Variation in Drainage of Total Anomalous Pulmonary Venous Return(2016) Gursu, Alper Hazim; Varan, Birgul; Ozkan, Murat; 0000-0002-6719-8563; 0000-0002-0707-2678; 26536995; ABB-1767-2021; AHI-4502-2022Item A Rare Cardiovascular Finding in Two Cases with Williams Syndrome: Recurrent Coarctation of Aorta(2015) Ari, Mehmet Emre; Erdogan, Ilkay; Varan, Birgul; Ozkan, Murat; 0000-0002-6719-8563; 0000-0001-6887-3033; 0000-0001-7932-1074; ABB-1767-2021; ABB-2220-2021; W-1590-2017Williams syndrome is a genetic disorder caused by multiple gene deletions on chromosome 7. The majority of the cases is sporadic and has typical facial appearance, cardiac anomalies and mental retardation. Cardiovascular anomalies are present in about 80% of the cases, most frequently supravalvular aortic stenosis and pulmonary arterial stenosis. In this article, we report two pediatric cases with a rapidly progressive form of aortic coarctation, each of whom required two surgeries and two percutaneous balloon dilatations within the first five months of life.Item Rituximab Therapy for Rejection in Pediatric Heart Transplant(2018) Erdogan, Ilkay; Varan, Birgul; Sezgin, Atilla; Pirat, Arash; Zeyneloglu, Pinar; 0000-0002-6719-8563; 0000-0003-2312-9942; 0000-0001-6887-3033; 27210774; ABB-1767-2021; C-3736-2018; ABB-2220-2021Objectives: Humoral rejection is the B-cell-mediated production of immunoglobulin G antibody against the transplanted heart. Antibody-mediated rejection may be resistant to standard immunosuppressive therapy and is associated with high mortality and graft loss. Rituximab can be used to treat antibodymediated rejection in heart transplant recipients. This retrospective study describes our experience with rituximab treatment in children with heart transplants. Materials and Methods: We present 7 pediatric patients with antibody-mediated rejection who were treated with plasma exchange and rituximab therapy. Rituximab was given at a dose of 375 mg/m2 by slow infusion in the intensive care unit after 5 days of plasmapheresis, in addition to a conventional regimen consisting of steroids, mycophenolate mofetil, and tacrolimus. The peripheral blood count and sodium, potassium, serum urea nitrogen, creatinine, aspartate aminotransferase, and alanine aminotransferase levels were measured in all patients before and after treatment. Results: Seven patients were treated with plasma exchange and rituximab. We repeated this therapy in 5 patients because of refractoriness or recurrent rejection. After diagnoses of antibody-mediated rejection, 4 patients died within 6 months (mortality rate of 57.1%). We did not observe any adverse effects or complications related to rituximab. Conclusions: Rituximab can be used in humoral rejection after pediatric heart transplant. However, the success of the treatment is controversial, and further study is needed to find an effective treatment for antibody-mediated rejection and steroid-resistant cellular rejection in children.Item A Rare Cause of Cyanosis in Newborns: Arteriovenous Fistula Between the Right Pulmonary Artery and the Left Atrium and Its Treatment(2018) Yakut, Kahraman; Varan, Birgul; Ozkan, Murat; 0000-0002-6719-8563; 29362005; ABB-1767-2021The formation of a fistula between the right pulmonary artery and the left atrium via a sac is a very rare cyanotic congenital cardiopulmonary defect. A fistula between the pulmonary artery and left atrium may cause cardiac failure in utero. It can safely be treated surgically and in selected cases closure can be performed with transcatheter insertion of a device. In this article, we present a case with a fistula between the right pulmonary artery and the left atrium that was considered unsuitable for transcatheter closure and was safely treated surgically.