CPB IN PATIENTS WITH SICKLE CELL DISEASE (1980-2003)
1: J Extra Corpor Technol 2001 Dec;33(4):243-4
Cardiopulmonary bypass with deep hypothermic circulatory arrest for a patient
with sickle cell anemia: a case report.
Vocelka CR, Lindley GG, Mulligan MS.
Division of Cardiothoracic Surgery, Department of Surgery, University of
Washington, Seattle 98195, USA. vocelka@u.washington.edu
A 36-year-old sickle cell anemia patient undergoing a pulmonary
thromboendarterectomy required the use of cardiopulmonary bypass incorporating
deep hypothermic circulatory arrest. Being aware of reported incidences of
sickling crises, a team of the surgeon, anesthesiologist, hematologist, and
perfusionist met to devise a plan of treatment. Treatment included preoperative
and intraoperative exchange transfusion, optimal blood gas management, and
increased blood flows during bypass. The surgical procedure was performed and
was successful in reducing pulmonary hypertension, incorporating a team approach
and utilizing these techniques. No incidence of adverse sickling events was
observed during this procedure.
PMID: 11806437 [PubMed - indexed for MEDLINE]
2: Anaesthesia 2001 Jul;56(7):667-9
Coronary artery bypass graft surgery in a patient with haemoglobin SC disease.
Marchant WA, Wright S, Porter JB.
The Middlesex Hospital, Mortimer Street, London W1N 8AA, UK.
Patients with sickle cell disease who undergo surgery are generally considered
to be at greater risk of peri-operative complications than otherwise healthy
patients. We report a case of a woman with haemoglobin SC disease undergoing
coronary artery bypass grafting. She was successfully managed with pre-operative
exchange transfusion and normothermic cardiopulmonary bypass.
PMID: 11437768 [PubMed - indexed for MEDLINE]
3: Anaesthesia 2001 Mar;56(3):286-7
Comment on:
Anaesthesia. 2000 Oct;55(10):1034.
Aortic cross-clamping in sickle cell disease.
Marchant WA, Wright S.
Publication Types:
Comment
Letter
PMID: 11251455 [PubMed - indexed for MEDLINE]
4: Tex Heart Inst J 1999;26(4):283-8
Sickle cell disease and aortic valve replacement: use of cardiopulmonary bypass,
partial exchange transfusion, platelet sequestration, and continuous
hemofiltration.
Sutton SW, Hunley EK, Duncan MA, Rodriguez R, Meyers TP.
Department of Thoracic and Cardiovascular Surgery, Baylor University Medical
Center, Dallas, Texas 75246, USA.
Sickle cell disease in patients undergoing open heart procedures presents a
multitude of challenges to the medical staff. With improved techniques of
cardiopulmonary bypass, surgery, and anesthesia for treating patients with
sickle cell disease, perfusionists will likely encounter patients with this
genetic disorder on a more frequent basis. A 40-year-old black woman was
admitted to our institution with recurrent Staphylococcus epidermidis and
sepsis. She underwent transesophageal echocardiography and cardiac
catheterization and was subsequently diagnosed with severe aortic insufficiency.
The aortic valve was replaced. Herein, we report our experience in the
preoperative, perioperative, and postoperative management of this patient. We
present a concise update on the current literature and techniques used by others
in similar cases, and we provide a brief section on future considerations to
assist fellow practitioners in recognizing this disease and meeting the
accompanying challenges.
PMID: 10653257 [PubMed - indexed for MEDLINE]
5: Anesth Analg 1999 Sep;89(3):598-603
Comment in:
Anesth Analg. 2000 Feb;90(2):503.
Fast-track cardiac anesthesia in patients with sickle cell abnormalities.
Djaiani GN, Cheng DC, Carroll JA, Yudin M, Karski JM.
Department of Anesthesia, The Toronto Hospital, University of Toronto, Ontario,
Canada.
We conducted a retrospective review of 10 patients with sickle cell trait (SCT)
and 30 patients (cohort control) without SCT undergoing first-time coronary
artery bypass graft surgery with cardiopulmonary bypass. Demographic,
perioperative management, and outcome data were collected. Both groups were
matched according to age, weight, duration of surgery, and preoperative
hemoglobin (Hb) concentration. Distribution of gender, medical conditions,
pharmacological treatment, and preoperative left ventricular function were
similar between the groups. The comparisons were analyzed in respect to
postoperative blood loss and transfusion rates, as well as duration of
intubation, intensive care unit, and hospital length of stay (LOS). All patients
underwent fast-track cardiac anesthesia. A combination of cold crystalloid and
blood cardioplegia was used. The lowest nasopharyngeal temperature was 33
degrees C. There were no episodes of significant hypoxemia, hypercarbia, or
acidosis. None of the patients had sickling crisis during the perioperative
period. The postoperative blood loss was 687 +/- 135 vs 585 +/-220 mL in the SCT
and control groups, respectively. The trigger for blood transfusion during
cardiopulmonary bypass was hematocrit <20% and Hb <75 g/L postoperatively. Three
SCT patients (30%) and 10 control patients (33%) received a blood transfusion.
Median extubation time was 4.0 vs 3.9 h; intensive care unit LOS was 27 vs 28 h;
and hospital LOS was 6.0 vs 5.5 days in the SCT and control groups,
respectively. There were no intraoperative deaths. One patient in the SCT group
died from multiorgan failure 2 mo after surgery. IMPLICATIONS: Fast-track
cardiac anesthesia can be used safely in patients with sickle cell trait
undergoing first-time coronary artery bypass graft surgery. Extubation time and
intensive care unit and hospital length of stay are comparable to those of
matched controls, and blood loss and transfusion requirements are not increased.
A hematocrit of 20% seems to be a safe transfusion trigger during
cardiopulmonary bypass in these patients.
Publication Types:
Clinical Trial
PMID: 10475287 [PubMed - indexed for MEDLINE]
6: J Card Surg 1998 Jan;13(1):48-50
Mitral valve replacement in Sickle C disease using intraoperative exchange
transfusion.
Madan AK, Hartz RS, Major C, McKee P, Flint L.
Department of Surgery, Tulane Medical Center, New Orleans, Louisiana, USA.
Sickle C (SC) disease is a relatively uncommon hematologic disorder that poses
special challenges when the patient requires a major surgical procedure. In
particular, those who have a history of hemolytic crises require some type of
intervention, usually homologous transfusion, to decrease the level of
circulating hemoglobin S (HbS) and prevent intraoperative sickle crisis. We
describe a 25-year-old man with SC disease and a history of multiple sickle cell
crises who underwent mitral valve replacement using intraoperative exchange
transfusion to decrease his HbS level from 53% to 7%.
PMID: 9892486 [PubMed - indexed for MEDLINE]
7: Eur J Cardiothorac Surg 1998 Nov;14(5):527-9
Cardiopulmonary bypass in sickle cell anaemia without exchange transfusion.
Frimpong-Boateng K, Amoah AG, Barwasser HM, Kallen C.
National Cardiothoracic Centre, Korle Bu Teaching Hospital, Accra, Ghana.
ctu@africaonline.com.gh
To minimize sickling during cardiopulmonary bypass, exchange transfusion is
frequently recommended peri-operatively for patients with homozygous sickle cell
anaemia to reduce the circulating concentration of HbS. We report the successful
management of two children both aged 12 years with sickle cell anaemia
(homozygous SS), several sickle cell crises and multiple blood transfusions who
underwent cardiopulmonary bypass for mitral valve replacement. No pre- or
intra-operative exchange transfusions were employed in the two cases.
Furthermore, moderate hypothermia in both cases was not associated with adverse
sequelae.
PMID: 9860213 [PubMed - indexed for MEDLINE]
8: Am J Respir Crit Care Med 1998 May;157(5 Pt 1):1690-3
Successful pulmonary thromboendarterectomy in two patients with sickle cell
disease.
Yung GL, Channick RN, Fedullo PF, Auger WR, Kerr KM, Jamieson SW, Kapelanski DP,
Moser KM.
Department of Medicine, University of California at San Diego, USA.
Patients with sickle cell disease have been reported to have an increased risk
of thromboembolism and pulmonary hypertension. Some of these patients may
benefit from pulmonary thromboendarterectomy (PTE), a procedure that requires
profound hypothermia, cardiopulmonary bypass, and periods of circulatory arrest,
factors that may potentially increase the risk of sickling. Two patients with
sickle cell disease (sickle-thalassemia [Hb S/beta+] and Hb SS) presented to the
Pulmonary Vascular Center of UCSD Medical Center with significant shortness of
breath and limitation of daily activities. Both of these patients were found to
have surgically accessible chronic thromboembolic disease with pulmonary
hypertension. PTE was performed in both patients using exchange transfusion,
with avoidance of anemia, hypoxia, and acidosis. A successful outcome with
resolution of pulmonary hypertension was achieved in both cases. To our
knowledge this is the first report of patients with sickle cell disease who
successfully underwent PTE for chronic thromboembolic pulmonary hypertension.
PMID: 9603156 [PubMed - indexed for MEDLINE]
9: Ann Thorac Surg 1998 May;65(5):1444-6
Acute normovolemic red cell exchange for cardiopulmonary bypass in sickle cell
disease.
Shulman G, McQuitty C, Vertrees RA, Conti VR.
Department of Pathology and Laboratory Medicine (Blood Bank Division),
University of Texas Medical Branch at Galveston 77555-0717, USA.
gshulman@utmb.edu
A patient with sickle cell disease (hematocrit, 28.5%; hemoglobin S fraction,
79%), required mitral valve repair. Partial red cell removal and blood component
sequestration with an autotransfusion device before cardiopulmonary bypass
initially decreased the sickle red cell mass. This was followed by an acute
one-volume whole blood exchange transfusion performed upon the initiation of
cardiopulmonary bypass, resulting in a further reduction. Both techniques
yielded fresh autologous plasma for use; sequestration yielded a
platelet-pheresis product. Adequate postbypass hemostasis was demonstrated.
PMID: 9594885 [PubMed - indexed for MEDLINE]
10: Perfusion 1997 May;12(3):197-201
Surgical management of catheter tip thrombus: surgical therapy for right atrial
thrombus and fungal endocarditis (Candida tropicalis) complicating paediatric
sickle-cell disease.
Hollingsed MJ, Morales JM, Roughneen PT, Burch KD.
Eastern Heart Associates, Savannah, Georgia, USA. micpumper@aol.com
The use of indwelling central catheters for long-term administration of
hyperalimentation, chemotherapy or other intravenous therapies is increasing.
This unusual presentation of a catheter-induced right atrial thrombus was
complicated by fungal infection. We present a case of a paediatric sickle-cell
patient who underwent surgical removal of a right atrial thrombus secondary to
fungal (Candida tropicalis) endocarditis from an indwelling catheter. Successful
thrombus removal utilizing cardiopulmonary bypass and subsequent discharge
underscores the importance of surgical therapy in treating this important
complication.
PMID: 9226709 [PubMed - indexed for MEDLINE]
11: Masui 1996 Oct;45(10):1269-71
[Cardiac surgery using cardiopulmonary bypass in a patient with sickle-cell
trait]
[Article in Japanese]
Ohashi Y, Akamatsu T, Hirata T, Uchida O, Kuro M.
Department of Anesthesiology, National Cardiovascular Center, Suita.
The sickle-cell gene is most concentrated in West Central Africa, the northeast
corner of Saudi Arabia and East Central India. Sickle cell trait is the
heterozygous condition for Hb S gene. Thirty to fifty per cent of their
hemoglobin is Hb S and the remainder is Hb A. The sickle-cell crisis is induced
by hypoxia, hypercarbia, acidosis, low flow condition, and hypothermia, which
leads to vasoocclusion. A 39-year-old black man from Burkina Faso located in
West Africa with left ventricular rupture was admitted for operation using
cardiopulmonary bypass (CPB). He had been diagnosed as sickle-cell trait. The Hb
S concentration was 36.2 per cent before operation with hemoglobin
electrophoresis. During CPB, the minimum blood temperature was 31 degrees C and
an aortic cross-clamp was not done. Total CPB time was 1 hour 31 minutes. Use of
vasodilator and hyperventilation was effective. No neurological sequelae were
observed.
PMID: 8937027 [PubMed - indexed for MEDLINE]
12: Ann Thorac Surg 1996 Jun;61(6):1841-3
Mitral valve reconstruction in sickle cell disease.
Pagani FD, Polito RJ, Bolling SF.
Department of Surgery, University of Michigan Hospitals, Ann Arbor 48109, USA.
As survival improves in patients with sickle cell anemia, the prospects of
performing cardiac surgical procedures on older patients with this genetic
defect increase. We describe the successful management of a 52-year-old patient
with sickle cell disease (homozygous for hemoglobin S) and a history of multiple
sickle crisis undergoing cardiopulmonary bypass for mitral valve repair.
Preoperative partial exchange transfusion followed by total exchange transfusion
at the time of operation was performed to reduce the level of hemoglobin S to
5.4% during bypass. Other management strategies included high-flow normothermic
bypass with aortic crossclamping, topical hypothermia, and cold crystalloid
cardioplegia.
PMID: 8651804 [PubMed - indexed for MEDLINE]
13: J Cardiothorac Vasc Anesth 1996 Apr;10(3):419-24
Case 2--1996. Anesthetic management of a patient with hemoglobin SS disease and
mitral insufficiency for mitral valve repair.
Kingsley CP, Chronister T, Cohen DJ, Parrish JM, Drew R, Bongiovanni MB.
Department of Anesthesia, Pennsylvania State University College of Medicine,
Hershey 17033, USA.
Publication Types:
Clinical Conference
PMID: 8725428 [PubMed - indexed for MEDLINE]
14: Arq Bras Cardiol 1995 May;64(5):463-4
[Preoperative erythrocytopheresis in a patient with SC hemoglobinopathy and
mitral stenosis]
[Article in Portuguese]
Tarasoutchi F, Grinberg M, Scarabucci M, Cardoso LF, Cavalheiro Filho C, Rached
RA, Pomerantzeff P, da Luz PL, Bellotti G.
Instituto do Coracao do Hospital das Clinicas - FMUSP, Sao Paulo.
Hemoglobinopathies are associated with thrombotic complications, when exposed to
cardiopulmonary bypass. A 54-year old, black woman with hemoglobinopathy SC and
severe mitral stenosis was submitted to eritrocytopheresis 48 hours before
mitral commissurotomy surgery. The therapeutic determined appearance of the
percentual hemoglobin A of 68% with reduction the hemoglobin S of 48% to 15% and
the hemoglobin C of 51% to 17%. No complications occurred during postoperative
period. To best of our knowledge, that is the first report about application the
eritrocytopheresis in the pre operative extra corporeal circulation care in
surgical treatment of patients with chronic rheumatic heart disease and
hemoglobinopathy SC.
PMID: 8526778 [PubMed - indexed for MEDLINE]
15: Ann Thorac Surg 1994 Dec;58(6):1762-4
Removal of an infected right atrial mass in a patient with sickle cell disease.
Spotnitz WD, Dent JM, Mintz PD, Erickson NL, Fechner RE, Groh MA.
Division of Cardiothoracic Surgery, University of Virginia, Charlottesville.
The use of indwelling central catheters for hyperalimentation, chemotherapy, and
long-term venous access is increasing. We report the successful removal of an
infected right atrial mass associated with the use of a central catheter in an
adult with sickle cell disease. The clinical options for the treatment of
infected atrial thrombus as well as the challenge of performing cardiopulmonary
bypass in patients with sickle cell disease are briefly discussed.
PMID: 7979756 [PubMed - indexed for MEDLINE]
16: J Cardiovasc Surg (Torino) 1991 Mar-Apr;32(2):271-4
Cardiopulmonary bypass in sickle cell anaemia. Report of five cases.
Balasundaram S, Duran CG, al-Halees Z, Kassay M.
Department of Cardiovascular Diseases, King Faisal Specialist Hospital and
Research Centre, Riyadh, Saudi Arabia.
Five patients with sickle cell haemoglobinopathies underwent open heart surgery.
At the start of the cardiopulmonary bypass the patient's circulating blood
volume was separately drained into a bag, spun down and retransfused into the
patient at the end of the operation. Moderate hypothermia, aortic cross
clamping, topical hypothermia and cold crystalloid cardioplegia were used in all
patients. None of the patients had transfusions in the postoperative period. No
macroscopic or microscopic evidence of haemolysis were seen, nor haematuria or
other clinical evidence of sickling. There was no evidence of wound infection
and all were discharged home at a mean of 12.6 days. It is concluded that in
cases of sickle cell anaemia exchange transfusion at the beginning of
cardiopulmonary bypass followed by retransfusion of the red cell free blood can
be used safely, permitting the performance of standard open heart procedures.
PMID: 2019634 [PubMed - indexed for MEDLINE]
17: Am Surg 1991 Mar;57(3):157-60
Tracheoplasty for congenital long-segment intrathoracic tracheal stenosis.
Van Meter CH Jr, Lusk RM, Muntz H, Spray TL.
Children's Hospital, Washington University Medical Center, St. Louis, Missouri.
Congenital long-segment intrathoracic tracheal stenosis (CTS) is a rare
life-threatening obstruction in infancy and childhood. From July 1983 to March
1988 six infants aged 14 days to 14 months with CTS were identified. Symptoms
ranged from recurrent stridor and wheezing to severe respiratory compromise and
hypercarbia. Routine chest x-rays were not diagnostic. Definitive diagnosis was
made by bronchoscopy, which showed complete tracheal rings in all patients with
severely compromised tracheobronchial lumens. In three patients, pericardium was
successfully used for anterior tracheoplasty with one early death due to
fulminant sepsis in an infant with undiagnosed sickle cell disease. The other
two died late, at 3 and 9 months from problems unrelated to the repair. In three
patients a rib graft was used for repair; in one, tracheoplasty was required
after earlier repair of tetralogy of Fallot. All are late survivors with no
postoperative symptoms. After recognition of CTS, prompt surgery is warranted to
avoid the late complications of tracheostomy for long-term ventilatory support.
Rigid repair with rib cartilage is preferable to use of pericardium. Proper rib
harvesting with intact perichondrium, intraoperative bronchoscopy, oxygenation
by cardiopulmonary bypass, and meticulous graft alignment are necessary for
successful postoperative outcome.
PMID: 2003703 [PubMed - indexed for MEDLINE]
18: J Cardiothorac Anesth 1990 Jun;4(3):421
Mitral valve replacement in pediatric patients with sickle cell disease.
Gouvea F, Pereira Sde J.
Publication Types:
Letter
PMID: 2131894 [PubMed - indexed for MEDLINE]
19: J Cardiothorac Anesth 1989 Aug;3(4):477-80
The management of two pediatric patients with sickle cell trait and sickle cell
disease during cardiopulmonary bypass.
Baxter MR, Bevan JC, Esseltine DW, Bernstein M.
Department of Anesthesia, McGill University, Montreal Children's Hospital,
Quebec, Canada.
PMID: 2520923 [PubMed - indexed for MEDLINE]
20: Thorac Cardiovasc Surg 1987 Apr;35(2):129-30
Mitral valve replacement in a patient with sickle cell anaemia.
McGovern E, Otridge BW, Neligan MC.
Cardiopulmonary bypass can be expected to cause problems in a patient with
sickle cell anaemia (SCA) as such surgery is associated with various factors
which may induce sickling and subsequent sickle cell crisis. This patient is
only the second with SCA to have valve replacement performed, and is the seventh
case reported in the English literature to have cardiopulmonary bypass surgery.
PMID: 2440137 [PubMed - indexed for MEDLINE]
Last Updated: Saturday, June 7, 2003
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