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 Cor Triatriaturn: A Single Institution's Experience(2015) Gursu, Hazim Alper; Varan, Birgul; Ozkan, Murat; Erdogan, Ilkay; Oktay, Ayla; Aslamaci, Salt; 0000-0002-6719-8563; 0000-0002-0707-2678; 0000-0001-6887-3033; ABB-1767-2021; AHI-4502-2022; ABB-2220-2021; AAJ-2305-2021Background: This study aims to analyze the data regarding cases diagnosed with cor triatriatum sinistrum at a single pediatric center, highlighting symptoms on presentation of the anomaly, mode of diagnosis, age at the time of diagnosis, any other accompanying cardiac defects, and outcomes. Methods: Records of 21 patients (13 females, 8 males; mean age 32.4 month; range 7 days to 57 years) diagnosed with cor triatriatum sinistrum between January 1997 and January 2012 at the pediatric cardiology clinic were analyzed retrospectively. Medical records including demographic data, clinical progress, diagnostic approach, and surgical interventions were reviewed. Results: The majority of patients (81%) had accompanying cardiac defects, atrial septal defect being the most frequent (33%). Restrictive pattern was detected in five patients. Seven patients (33%) died after diagnosis. We resect the fibromuscular diaphragm in 11 of 21 patients. One patient died after operation. Most common presenting symptoms were lower respiratory tract infections, dyspnea, and early fatigue. Sixteen patients were diagnosed with echocardiography. Conclusion: Despite the diversity in clinical findings at presentation and accompanying cardiac lesions, cor triatriatum sinistrum is a treatable defect with satisfying treatment outcomes.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 Neonatal Thyrotoxicosis with Severe Supraventricular Tachycardia: Case Report and Review of the Literature(2015) Abbasoglu, Aslihan; Ecevit, Ayse; Tugcu, Ali Ulas; Erdogan, Ilkay; Kinik, Sibel Tulgar; Tarcan, Aylin; 0000-0002-2232-8117; 0000-0001-6887-3033; 25153577; AAJ-2305-2021; AAJ-4616-2021; ABB-2220-2021; ABI-2113-2020Neonatal thyrotoxicosis is a rare condition caused by the transplacental passage of thyroid stimulating immunoglobulins from mothers with Graves' disease. We report a case of neonatal thyrotoxicosis with concurrent supraventricular tachycardia (SVT). The female infant, who was born by section due to breech delivery and meconium in the amniotic fluid at 36 weeks of gestation, presented with tachycardia on day 7. Her heart rate was between 260 and 300 beats/min, and an electrocardiogram revealed ongoing SVT. Sotalol was effective after two cardioversions in maintaining sinus rhythm. Thyroid function studies revealed hyperthyroidism in the infant, and her mother was found to have Graves' disease. Since symptoms and signs can vary, especially in preterm infants with neonatal hyperthyroidism, we want to emphasize the importance of prenatal care and follow-ups of Graves' disease associated pregnancies and management of newborns after birth.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 Magnetic Resonance Tagging for Diagnosis of Focal Hypertrophic Cardiomyopathy in A Child(2015) Coban, Gokcen; Yildirim, Muge Afsar; Donmez, Fuldem Yildirim; Tarhan, Nefise Cagla; Erdogan, Ilkay; Coskun, Mehmet; 0000-0001-5630-022X; 0000-0001-6887-3033; 0000-0003-4502-106X; 0000-0002-4010-2883; 0000-0001-9877-7106; 26012424; AAM-4120-2021; AAJ-2305-2021; ABB-2220-2021; AAE-5528-2021; P-7533-2014Magnetic resonance imaging has become an important diagnostic tool in the differential diagnosis of lesions for evaluation of cardiovascular disorders. In magnetic resonance tagging (MRt), tissue elements are magnetically labeled so that their positions can be tracked as a function of time. Thus, MRt evaluates heart wall motion both qualitatively and quantitatively. We present herein the case of a 12-year-old boy who had chest pain, dyspnea on effort and murmur. On cardiac computed tomography, there was focal thickening of the left ventricular posterior wall, similar to a mass. MRt indicated active displacement and deformation of the tags at the level of the hypertrophic myocardium during systole, as with normal myocardium. Thus, the tagged images supported the diagnosis of focal hypertrophic cardiomyopathy (HCM). In view of these results, MRt should be considered as a useful technique for differentiating between a mass-like focal lesion such as neoplasm and HCM.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 Mid-Term Results of Patients with Transposition of Great Arteries Who Underwent Senning Procedure(2014) Gursu, Hazim Alper; Varan, Birgul; Ozkan, Murat; Tokel, Kursat; Erdogan, Ilkay; https://orcid.org/0000-0002-0707-2678; https://orcid.org/0000-0002-6719-8563; https://orcid.org/0000-0002-6759-1795; https://orcid.org/0000-0001-6887-3033; AHI-4502-2022; ABB-1767-2021; AAF-3253-2021; ABB-2220-2021Background: This study aims to evaluate mid-term follow-up results for patients with transposition of great arteries to whom Senning procedure was performed. Methods: Files of 95 child patients (63 boys, 22 girls; mean age 15.2 +/- 23.9 months; range 1 month to 12.5 years), who were diagnosed with transposition of great arteries and underwent atrial switch operation in our institute, were retrospectively evaluated. In the follow-ups; physical examination, electrocardiography, and echocardiography were performed. In addition, ambulatory electrocardiography monitorization was conducted on 25 patients. Results: Out of 95 patients who were performed atrial switch operation, 10 died in the postoperative period. The mean follow-up period was 33.4 +/- 43.7 months (1-16 years; mean 18 months). During the follow-ups, arrhythmia was detected in 25.8% of the patients, stenosis of pulmonary venous baffle was detected in 21.2%, systolic dysfunction of the right ventricle was detected in 19%, severe tricuspid valve insufficiency was detected in 18.9%, and baffle leak was detected in 15.3%. Of the patients, 11.7% were operated a second time, and 8.2% were reoperated due to stenosis of pulmonary venous baffle. Conclusion: Short and mid-term results of Senning procedure are not satisfactory. Patients should be monitored for possible right ventricle dysfunction, arrhythmia, systemic valve insufficiency, and stenosis of the tunnel.