Cardiac Surgery and Sickle Cell Disease

Al‐Ebrahim, Khaled E.

doi:10.1177/021849230801600610

Cited by 7 publications

(17 citation statements)

References 12 publications

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“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

Section: Resultsmentioning

confidence: 99%

“…Intraoperative monitoring varies. Per standard anesthetic care, blood pressure, cardiac rhythm and rate and oxygenation are monitored strictly to avoid hypoxia . Some groups perform more invasive systematic monitoring such as the continuous measurement of central venous pressure, arterial blood pressure, regular arterial blood gas exchange and/or urine output determination (by Foley catheterization) .…”

Section: Resultsmentioning

confidence: 99%

“…Oxygenation has to be strictly controlled and hypoxia needs to be avoided. Acidosis has to be controlled and bicarbonate is used to reverse it . Mild alkalosis induced by bicarbonate therapy has sometimes been advocated for, mostly older articles .…”

Section: Resultsmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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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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“…Special care and specific precautions are required preoperatively to minimize the surgical risk. [2][3][4] Therefore, careful review of the SCD literature was promptly initiated along with a consult of the Hematology Service; both were quite insightful. From this effort, the care pathway was divided into three phases of intensive treatment: preoperative, intraoperative and postoperative.…”

Section: Case Study and Patient Historymentioning

confidence: 99%

“…A homozygous patient will have 80%-95% HgbSS, 2%-20% fetal hemoglobin and 0% hemoglobin A, whereas a heterozygous carrier will have 35%-40% HgbSS and 60%-65% hemoglobin A. 4 The molecular causation of HgbSS is a single nucleotide polymorphism (GAG → GTG) of the β-globin gene, resulting in the substitution of glutamic acid by valine at position 6. [5][6][7] HgbSS with this point mutation can polymerize in the deoxygenated state, stiffening the erythrocyte membrane and distorting the cellular structure to the characteristic sickle shape as shown in Figure 1B.…”

Section: Introductionmentioning

confidence: 99%

Sickle cell disease and complex congenital cardiac surgery: a case report and review of the pathophysiology and perioperative management

et al. 2013

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Sickle cell anemia and thalassemia are hemoglobinopathies rarely encountered in the United States. Compounded with congenital heart disease, patients with sickle cell disease (SCD) requiring cardiopulmonary bypass and open-heart surgery represent the proverbial "needle in the haystack". As such, there is some trepidation on the part of clinicians when these patients present for complex cardiac surgery. SCD is an autosomal, recessive condition that results from a single nucleotide polymorphism in the β-globin gene. Hemoglobin SS molecules (HgbSS) with this point mutation can polymerize under the right conditions, stiffening the erythrocyte membrane and distorting the cellular structure to the characteristic sickle shape. This shape change alters cellular transit through the microvasculature. As a result, circumstances such as hypoxia, hypothermia, acidosis or diminished blood flow can lead to aggregation, vascular occlusion and thrombosis. Chronically, SCD can give rise to multiorgan damage secondary to hemolysis and vascular obstruction. This review and case study details an 11-year-old African-American male with known SCD who presented to the cardiothoracic surgical service with congenital heart disease consisting of an anomalous, intramural right coronary artery arising from the left coronary sinus for surgical consultation and subsequent surgical correction. This case report will include a review of the pathophysiology and current literature regarding preoperative, intraoperative and postoperative management of SCD patients.

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Aortic valve replacement in a patient with sickle cell disease—Are we justified to perform surgery in the TAVI era?

Ravishankar

Turkistani

Elghoneimy

et al. 2021

Clinical Case Reports

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Cardiac Surgery and Sickle Cell Disease

Cited by 7 publications

References 12 publications

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

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

Sickle cell disease and complex congenital cardiac surgery: a case report and review of the pathophysiology and perioperative management

Aortic valve replacement in a patient with sickle cell disease—Are we justified to perform surgery in the TAVI era?

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