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Subject: search.filters.subject.Congenital heart disease

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    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, Sait
    Purpose: 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.
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    Le-Compte Maneuver in Surgical Correction of Absent Pulmonary Valve. Does it Improve Severe Bronchial Compression?
    (2017) Saritas, Bulent; Ozker, Emre; Sansoy, Ozlem; Sahin, Murat; Gumus, Burcak; Ayabakan, Canan; 28094129; P-4569-2015
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    New Approach in Stage 1 Surgery for Hypoplastic Left Heart Syndrome: Preliminary Outcomes
    (2023) Celik, Mehmet; Gokdemir, Mahmut; Cindik, Nimet; Gunaydin, Asim C.; Aygun, Fatih; Ozkan, Murat; 0000-0002-5018-0068; 0000-0002-5676-2747; 36004405; AAT-2031-2021; N-4174-2014
    Objectives: We present the short-term results of an alternative method in stage 1 surgery for hypoplastic left heart syndrome. Methods: Data of 16 consecutive patients who were treated with the novel method in our clinic between February 2019 and March 2021 were analysed retrospectively. Preoperative data and postoperative follow-up were recorded. Results: Of the 16 operated patients, 12 were diagnosed with hypoplastic left heart syndrome, while four were diagnosed with hypoplastic left heart syndrome variants. Seven patients died during early postoperative period. One patient died at home waiting stage 2 surgery. Three patient underwent stage 2 surgery. Pulmonary artery reconstruction was performed in one patient due to left pulmonary artery distortion. Conclusions: We believe that our method can be an effective alternative in the surgery of hypoplastic left heart syndrome and its variants. It is hoped that with increasing number of studies and more experience better outcome will be achieved.
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    Successful anesthetic and airway management in Coffin-Siris syndrome with congenital heart disease: Case report
    (2016) Altun, Dilek; Demir, Guray; Ayhan, Asude; Turkoz, Ayda; 0000-0003-3299-6706; AAR-7467-2020; AAJ-2066-2021; AAJ-2057-2021
    Introduction: Coffin-Siris Syndrome (CSS) is a rare congenital malformation syndrome characterized with mild to severe developmental and cognitive delay, coarse facial features, fifth digit aplasia or hypoplasia associated with ectodermal, constitutional and organ-related (cardiac/neurolo gical/gastrointestinal/genitourinary...) anomalies. Here, we have reported a successful anesthetic and airway management in a case of 5-year old boy with CSS who underwent congenital heart surgery. Case report: A 5-year old male child weighing 14 kg, who was diagnosed as CSS underwent operation for the repair of partial atrioventricular septal defect and secundum atrial septal defect. This case report pertains to the successful anesthetic and airway management in the background of difficult airway and presence of various cardiac abnormalities. Although patient was anticipated to be difficult for intubation due to laryngomalacia, micrognathia, macroglossia, tracheal intubation was performed without any difficulty using fiber-optic laryngoscopy. At the end of the operation, the patient was transferred to the cardiovascular intensive care unit and was extubated when his spontaneous breathing was satisfactory 4 h later after the operation without any complication. Results and discussion: CSS often requires surgery and anesthetic intervention. The abnormal facial and airway as well as mental related features may lead intubation difficult, potentially due to short neck, large tongue and lips, poor dentition and poor communication. Thinking that the practicing anesthetist needs to have appropriate knowledge for this entity and the equipment for managing difficult airway should readily be available. One of these patients which successfully managed without any complication was described in this brief report. (C) 2016 Publishing services by Elsevier B.V. on behalf of Egyptian Society of Anesthesiologists.
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    Case report of a rarely seen long-segment middle aortic syndrome
    (2017) Erdogan, Ilkay; Yakut, Kahraman; 0000-0001-6887-3033; 28424443; AAJ-2305-2021; ABB-2220-2021
    Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture. In order to draw attention to this disease, presently described is case of high blood pressure detected during routine examination of a child who had no complaint, and discovery of long-segment stenosis in the abdominal aorta identified with echocardiography and conventional angiography.
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    Case report and the surgical treatment of two cases with pulmonary atresia in which pulmonary arteries is circulated by coronary arteries
    (2018) Tokel, Kursad; Yakut, Kahraman; Varan, Birgul; Erdogan, Ilkay; Ozkan, Murat; 0000-0002-6719-8563; 0000-0001-6887-3033; 0000-0002-6759-1795; 29664428; ABB-1767-2021; AAJ-2305-2021; ABB-2220-2021; AAF-3253-2021
    Pulmonary atresia (PA) and ventricular septal defect (VSD) can occur in a variety of ways, from simple valve atresia to a condition in which circulation to the pulmonary bed occurs through collateral arteries separated from the aorta and there are no real pulmonary arteries, or they are present but hypoplastic. The size of the pulmonary arteries and concomitant complex cardiac lesions are important in making decisions about treatment and correctional alternatives. While complete correction surgeries in the style of a correction of tetralogy of Fallot are performed in simpler cases, many very invasive procedures are also performed and the resulting quality of life is very variable. The size of the pulmonary artery and its branches and the presence of accompanying collateral vessels are determining factors in the management of the disease. In this report, 2 cases of VSD and PA, in which the circulation of the pulmonary arteries was through the coronary arteries, diagnosed as a result of echocardiography performed following murmurs heard during examination, are described. The first patient was 40 days old and the second was 2 days old. In the second case, diagnosis was confirmed by catheter angiography, and in the first case, the final diagnosis was made during surgery. The aim of this report is to emphasize the importance of pulmonary artery size, the presence of pulmonary confluence, the origin of circulation, and concomitant collateral arteries when considering treatment methods.
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    Hybrid approach: an alternative prior to corrective surgery in a patient with arch hypoplasia and complete atrioventricular septal defect
    (2018) Celik, Mehmet; Gokdemir, Mahmut; Cindik, Nimet; Ozkan, Murat; 32082782
    The hybrid approach is mostly preferred in patients with hypoplastic left heart syndrome or univentricular physiology. Here, the hybrid approach is applied as a palliative procedure prior to corrective surgery in a patient with complete atrioventricular septal defect associated with arcus hypoplasia and results are discussed according to the literature.