A Child With Sickle Cell Disease and Anomalous Right Coronary Artery

Egrie, Glenn; Woodson, Kristina; Alphonso, Nelson; Karl, Tom R.

doi:10.1016/j.athoracsur.2007.07.002

Cited by 10 publications

(31 citation statements)

References 7 publications

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“…2,3,[5][6][7][8][9][10][11][12][13][14][15][16] On the other hand, CPB itself is also a system that facilitates exchange transfusion, and using blood for priming the circuits can reconcile the aim. 2,6,14,15 Blood transfusion is one of the major issues during any kind of surgery. Generally, autologous transfusion is preferred, but preoperative phlebotomy or the use of cell-saver systems during surgery could reduce the need for homologous blood transfusion.…”

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

“…Although there is no consensus on absolute safe values of HbS in patients undergoing surgery, it is proposed that the level of HbS should be reduced to Ͻ30% for major surgical procedures 9,11 or even 5% for cardiac surgery before or at the time of surgery. 15 Although some authors advocate against blood transfusion for hemoglobin levels Ͻ70 g/L, 10,[17][18][19] the benefits of a reduction in HbS and an increase in HbA concentrations are obvious for preventing sickling phenomena and providing adequate delivery of oxygen to the tissues. The advantages of preoperative transfusion include increased hematocrit levels and suppressed production of HbS in this particular group of anemic patients.…”

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

“…8 -11 Although the risks accompanying sickle cell pathology before high-risk surgery, including orthopedic and cholecystectomy, have been well stated, 9 the literature contains only small series or case reports on sickle cell patients having cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] It should be noted that above-mentioned predisposing conditions are more common in patients undergoing cardiac surgery. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] Especially during the operation, CPB itself, as well as aortic cross-clamping, low-flow states, topical or whole-body hypothermia, cold cardioplegia, and use of vasoconstrictive agents, may predispose to the crisis state.…”

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Such conditions lead to potassium efflux, causing formation of insoluble globin polymers. These molecules increase the viscosity of blood and lead to vasoocclusive phenomena, which include cell sickling, adherence of sickle cells to the endothelium, and vasoocclusion.…”

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

“…This can be avoided by applying "topical-only" cooling or achieving moderate hypothermia, but totally warm CPB is another valid option mentioned frequently in the literature. 2,3,[5][6][7]14,15 In coronary surgery, the endomyocardial capillaries are at particular risk of occlusion by the sickling phenomena when exposed to cold cardioplegia. This can be prevented with the initial institution of warm cardioplegia to wash out the existing blood in coronary arteries, followed by cold or designated cardioplegia according to the protocols of the operating team.…”

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

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Open Heart Surgery in Patients With Sickle Cell Hemoglobinopathy

et al. 2010

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Background— In patients with sickle cell trait or disease, reduced life expectancy and a tendency for complications are believed to negatively affect likelihood of survival after open heart surgery. The aim of this study was to review retrospectively the perioperative results of patients undergoing cardiac surgery at our institution. Methods and Results— Between January 1995 and December 2006, 47 patients with either sickle cell disease or sickle cell trait underwent open heart surgery at our institution. The average age of the 29 male and 18 female patients was 20 years. Patient outcomes were analyzed through the use of the institutional database. Clinical and echocardiographic follow-up was complete in all patients except 3, with a mean follow-up period of 46 months. Current status could be confirmed in 32 patients. The most common operations included the treatment of congenital and valvular heart diseases. There were no coronary artery bypass grafting procedures. Average weight of the patients was 45 kg. Exchange transfusion was performed both preoperatively and during surgery. Mean preoperative hemoglobin S concentration was 30.4±3.2% and decreased to 8.1±2.6% while on pump. Average on-pump hematocrit value was 25.4±3.7%; in the postoperative period, it increased to 32.7±4.9%. Mean cardiopulmonary bypass and cross-clamp times were 95 and 69 minutes, respectively. None of the patients had sickling crisis or acidosis. Postoperative complications included exploration for hemorrhage in 3 patients (6.4%), stroke in 2 patients (4.3%), renal failure in 2 patients (4.3%), and prolonged ventilation in 1 patient (2.1%). Average hospital stay was 8.3 days (range, 4 to 27 days). Early in-hospital death occurred in 1 patient (2.1%); currently, 31 patients (66%) remain alive and free of cardiac symptoms. Conclusion— Heart valve surgery and surgery for congenital heart diseases can be performed safely in patients with sickle cell disease or sickle cell trait with acceptable outcome and survival rates.

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Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

Section: Yousafzai Et Al Sickle Cell Hemoglobinopathy and Cardiac Surmentioning

confidence: 99%

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Open Heart Surgery in Patients With Sickle Cell Hemoglobinopathy

et al. 2010

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Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

et al. 2019

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Children with sickle cell disease (SCD) require specific perioperative care, and clinical practice in this area remains poorly defined. We aimed to conduct a systematic, PRISMA-based review of the literature, available clinical guidelines and practice recommendations. We also aimed to extract any valuable information for the "best of available-evidence"-based prevention of perioperative adverse events in children with SCD, and highlight the most urgent priorities in clinical research. As data sources, USWe also included institutional, consortia and expert group guidelines. Included were reports/guidelines in English, French, German, and Italian. Excluded were reports on obstetrical and fetal management. We identified 202 reports/guidelines fulfilling the criteria outlined above. A majority focused on visceral, cardiovascular and orthopedic surgery procedures, and only five were multicenter randomized controlled trials and two prospective randomized studies. After grading of the quality of the evidence, the extracted data was summarized into clinical recommendations for daily practice. Additionally, we designed a risk-grading algorithm to identify contexts likely to be associated with adverse outcomes. In conclusion, we provide a systematic PRISMA-based review of the existing literature and ancillary practice and delineate a set of clinical recommendations and priorities for research.

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