The new Coronavirus infection, which was first seen in China in late December 2019 and eventually became a worldwide pandemic, poses a serious threat to public health. After a high spike in the number of new COVID-19 infection cases following increase in overall daily death toll in Turkey, Turkish Ministry of Health has taken immediate precautions to postpone elective surgeries in order to reduce the burden to the healthcare system which might be challenged. Whereas different areas of medicine were able to suspend their operative procedures during this period, this was not completely possible in pediatric cardiovascular surgery due to the severity and urgency of congenital heart disease patients requiring operation. Based on the guideline that was published by the Turkish Pediatric Cardiology and Cardiac Surgery Association, in which the patients requiring surgical intervention during the Covid-19 pandemic period are ranked according to the priority, directions were given regarding the operations that hereby, be delayed, we report our experience in 29 cases retrospectively, regarding the preoperative evaluation of these patients, makings of an emergency operation decision, and strategies taken about intraoperative and postoperative management and arrangements during the pandemic period. In this article, we present crucial precautions that was applied in pediatric cardiovascular surgery and extensive list of cases in order to deliver highest level of the patient safety and protection for the surgical team.
Minimal right vertical infra-axillary thoracotomy can be performed with favorable cosmetic and clinical results for atrial septal defects closure. Infra-axillary thoracotomy provides a good alternative to standard median sternotomy for patients with atrial septal defects.
O chronosis is a rare metabolic disorder associated with a homogentisate 1,2-dioxygenase deficiency.1 The transmission is autosomal recessive, and homogentisic acid (HGA) is the end product of the tyrosine and phenylalanine metabolism. Accumulated HGA is observed in connective tissue and in eliminated urine.1 The usual clinical signs include arthropathy; manifestations affecting the ears, eyes, and skin; and genitourinary and cardiovascular complications. The accumulation of HGA can involve cardiac structures, especially the aorta, coronary vessels, and heart valves. In the elderly, the pericardium can be involved.2 Pathologic examinations of the aortic valve and ascending aorta have revealed intracellular and extracellular deposits of ochronotic pigments. The extracellular deposits are, in part, degenerated cells that resemble valvular calculus.3 Few reports have described ochronotic patients with severe cardiac involvement. We present the case of a man in whom ochronosis of the aortic and mitral valves was diagnosed intraoperatively.
Case ReportIn May 2013, a 72-year-old man who had severe aortic stenosis and mitral insufficiency presented at our clinic with dyspnea. He had no history of endocarditis or rheumatic fever. His medical history included progressive degenerative arthritis affecting his knees and spine, confirmed by means of musculoskeletal examination. Results of routine laboratory tests were normal. Echocardiograms revealed severely calcified aortic valve leaflets and a mean aortic valve gradient of 55 mmHg. The calculated aortic valve area was 0.5 cm 2 . Mitral valve leaflet malcoaptation and grade 4/4 mitral insufficiency were detected. The patient's left ventricular function was normal. Cardiac catheterization yielded normal coronary arteries.The patient underwent surgery to replace his aortic and mitral valves. A dark-green sternum was initially noted. The aortic and mitral valve leaflets, intima of the ascending aorta, and left ventricular outflow tract all contained black pigment (Fig. 1). Alkaptonuria was suspected. We replaced the native valves with a 25-mm mechanical aortic prosthesis and a 29-mm mechanical mitral prosthesis (Medtronic, Inc.; Minneapolis, Minn). The patient recovered uneventfully and was discharged from the hospital on postoperative day 10.After surgery, the patient revealed that he had noticed dark discoloration of his urine since early childhood. Results of a general examination included black pigmentation of the sclera of both eyes and the cartilage of the outer ears (Fig. 2). Radiographs of the knees and spine showed calcification and severe degeneration of the vertebral discs.Pathologic examination of the explanted native aortic and mitral leaflets revealed ochronosis (Fig. 3), and high HGA levels in the patient's urine confirmed the diagno-
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