Abstract
This case report discusses the management of a 15-year-old Jehovah’s Witness (JW) with DiGeorge syndrome, complex congenital heart disease (CHD), and severe neuromuscular scoliosis requiring major orthopedic surgery. Refusal of blood transfusions required preoperative optimization and advanced blood conservation strategies. The patient underwent multiple cardiac surgeries and spinal fusion. This case emphasizes multidisciplinary coordination and blood management strategies for surgical cases with religious restrictions.
Keywords: Jehovah’s Witness, congenital heart disease, orthopedic surgery, intraoperative cell salvage, patient blood management
Introduction
Surgical procedures in Jehovah’s Witness (JW) patients who refuse blood products present challenges. Managing JW patients during major surgeries highlights ongoing issues with blood supply, safety, and conservation. Advances in patient blood management (PBM) minimize blood loss and optimize outcomes while respecting religious beliefs. Key strategies include preoperative anemia management with iron supplementation and erythropoiesis-stimulating agents, perioperative techniques to reduce intraoperative blood loss (e.g., de-priming, hemodilution prevention, and volume optimization), and postoperative conservation through restrictive transfusion protocols and hemostatic agents. 1 In the context of congenital heart disease (CHD) and the underlying syndromic conditions that come with these lesions, such as 22q11.2 deletion syndrome, hemostasis and bleeding risk become even more challenging given their platelet abnormalities, including macrothrombocytopenia, reduced platelet surface expression of GPIb-V-IX, and platelet function defects. 2 This report presents a 15-year-old JW with complex CHD and a 124-degree scoliosis, requiring spinal correction and fusion. We focus on blood conservation strategies and multidisciplinary coordination. The subject’s parents granted permission to publish this case report on 09/24/2024.
Case history
A 36.6 kg 15-year-old JW male with DiGeorge syndrome and complex CHD presented with severe scoliosis, classified as Lenke type-4, with a 132-degree Cobb angle from T2-L3. At preoperative visit, he had mild thrombocytopenia, yet no hypocalcemia or recurrent infections related to his syndrome. Preoperative radiographs showed a right trunk shift of 56.59 mm, right shoulder elevation of 77.97 mm, and skeletal maturity (Reisser sign). Neurological examination was normal.
Cardiac history included Shone’s variant, bicuspid aortic valve, interrupted aortic arch, ventricular septal defect (VSD), aberrant right subclavian artery, and subaortic membrane. Previous interventions included aortic arch repair, subaortic membrane resection, and a Ross procedure. Multidisciplinary discussions deemed him candidate for a Bentall procedure for aortic root aneurysm and valve insufficiency. Preoperative hemoglobin (Hgb) was optimized with erythropoietin (EPO) 200 units/kg subcutaneously and 325 mg of oral iron 1 month prior in order to increase Hgb by 1.5 g/dL, starting at 14.2 g/dL with a target of 15.7 g/dL. Preoperatively, a multidisciplinary discussion between surgery, anesthesia, and perfusion centered on blood conservation strategies, with the perfusionist playing a key role in optimizing intraoperative cell salvage techniques acceptable to the patient’s religious beliefs.
Fourth-time redo sternotomy for a Bentall procedure included a 23-mm St Jude mechanical valve and 22-mm homograft conduit. Cardiopulmonary bypass (CPB) was initiated using a 3/8” by 3/8” tubing set and a Terumo Capiox FX15 oxygenator system. The physiologic clear prime consisted of 1L Plasmalyte-A, 150 mL 0.45% sodium chloride, and 300 mL 25% albumin. With the use of a Terumo Capiox HC11 hemoconcentrator, the final priming volume was decreased to 819cc, with no depriming performed at the time of bypass initiation. After cooling to a moderate hypothermic temperature of 25 degrees centigrade over 25 minutes, the aortic cross clamp was applied and custom, cold cardioplegia was delivered to achieve hyperkalemic and hypothermic arrest. During the operation, any residual volume was hemoconcentrated out of the circuit to increase hematocrit (Hct), in addition to delivering any intraoperative cell saver. Flow rates on bypass were maintained at 3.48 L/min, or a 2.7 L/min/m2 cardiac index.
Pre-bypass hematocrit (Hct) was 38%, with an expected dilutional first Hct on CPB of 30%. Intraoperative cell salvage collected collected blood (489 cc) from the surgical field was processed and reinfused to the CPB circuit using a closed loop system, raising the Hct to 33% on CPB, which was maintained until CPB completion. No blood products were administered.
For scoliosis, the patient was managed with preoperative 35-pound halo gravity traction 3 months after cardiac surgery but prior to spinal fusion, reducing the curve to 70-degrees over 3 months. After multidisciplinary review involving orthopedic surgery, cardiology, cardiovascular surgery, hematology, and cardiac anesthesia, posterior instrumented arthrodesis from T2-L3 with multilevel Ponte osteotomies (MPOs) was planned. The family consented to fibrinogen concentrate but not whole blood. They acknowledged transfusion would be required in severe bleeding with their medical liaison present. Preoperative preparation included the same EPO and iron regimen as his cardiac surgery. During surgery, the patient lost 1L of blood, managed with 500 mL cell-saver blood and 500 mL 5% albumin, without blood products. The lowest Hct was 42% throughout the procedure. 2588 mg of fibrinogen concentrate were administered throughout the procedure. He was extubated postoperatively, with stable Hgb (13.7 g/dL), and no transfusion required. Warfarin was restarted on postoperative day 2 (Figure 1).
Figure 1.
Postoperative trends of hemoglobin and hematocrit levels after cardiac and orthopedic procedures.
Discussion
This case demonstrates the complexity of managing a JW patient with DiGeorge syndrome, CHD, and severe scoliosis. Numerous blood conservation techniques, including intraoperative cell salvage and preoperative EPO, were employed to respect religious beliefs while ensuring patient safety. A continuous closed-loop circuit maintained blood continuity, as the hemoconcentrator maximized blood conservation by concentrating red blood cells and coagulation factors while removing plasma water. Unlike traditional cell savers, which discard plasma and platelets, this system retained these components. 3
Other techniques implemented to avoid blood transfusion for this case included intraoperative cell salvage and preoperative iron and EPO supplementation before surgery. MPOs using an ultrasonic bone scalpel enabled precise cortical bone incision, effective cancellous bone cauterization, and preservation of soft tissue. 4 Ultrasonic bone scalpel usage has shown a 40% reduction in blood loss compared to other scalpels. 5
Blood conservation alternatives stated by the National Institute for Health and Care Excellence (NICE) guidelines provide clarity for JW patients, enabling individualized protocols that align with their beliefs. 6 Individualized and informed blood conservation strategies can be achieved in each JW family structure. A protocol where patients and families select blood product alternatives that align with their religious beliefs is provided before surgical interventions at our center.
Preoperative halo gravity traction played a key role in reducing spinal deformity, facilitating staged correction, and minimizing surgical risks. Using a staged procedure technique decreased surgical time and reduced estimated blood loss, a critical consideration for CHD patients. 7 Managing complex cases requires coordinated multidisciplinary care. Cardiology/cardiovascular surgery, intensive care, cardiac anesthesiology, and orthopedic surgery collaborated to deliver patient-centered care in this case. Proactive engagement with the JW community at our institution has enhanced trust and increased patient volume. Performing high-risk surgeries on minors whose parents refuse blood transfusions raises ethical and legal challenges. In Texas, state law prioritizes a child’s life over parental religious beliefs in life-threatening situations unless the minor is emancipated. Written consent ensured an understanding of blood-sparing strategies and documented preferences.
Regardless of patient beliefs, minimizing blood loss and employing conservative blood management strategies are critical in any surgical procedure. This is even more critical in CHD patients, due to alloimmunization risk and the potential need for a heart transplant in the future. These risks are amplified in this case, as the patient had 22q11.2 deletion syndrome, which encodes for the GP1B-V-IX complex on platelet surfaces, resulting in altered hemostasis. Furthermore, these patients are at risk of developing transfusion-associated graft-versus-host disease if non-irradiated blood is used perioperatively. 2 Advocating for practitioners to follow patient blood management (PBM) strategies allows for the standard of care to involve blood conservative techniques that could apply to any patient, regardless of their beliefs. 8
Conclusion
This case demonstrates the feasibility of major scoliosis surgery with significant curve correction in the context of severe congenital heart disease and the specific beliefs of Jehovah’s Witness patients. Preoperative optimization, innovative surgical and anesthetic techniques, and a multidisciplinary approach resulted in favorable outcomes. Minimal blood loss and conservative blood strategies should be prioritized in any surgery regardless of patient and parental religious beliefs. Evolving blood conservation techniques are critical to advancing patient care for those with such unique challenges.
Footnotes
Author contributions: Drs. Maria E. Hoyos and Mario A. O’Connor contributed to writing and preparing the manuscript. Madeline Loftin performed data collection, analysis, and drafting the case report. Dr. Brian Kaufman reviewed the manuscript draft and supervised data collection. Drs. Cynthia Keene and Hugo R. Martinez, MD oversaw manuscript development and supervised data collection, and Drs. Charles D. Fraser and Erin Gottlieb, MD, MHCM, served as the principal investigators. All authors reviewed, edited, and supervised the manuscript to ensure accuracy and clinical relevance.
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding: The author(s) received no financial support for the research, authorship, and/or publication of this article.
Ethical statement
Informed consent
Written informed consent for publication was provided by the participants’ legal guardians/next of kin.
ORCID iD
Maria E. Hoyos https://orcid.org/0009-0003-8129-6092
References
- 1.Tibi P, McClure RS, Huang J, et al. STS/SCA/AmSECT/SABM update to the clinical practice guidelines on patient blood management. Ann Thorac Surg 2021; 112(3): 981–1004. [DOI] [PubMed] [Google Scholar]
- 2.Brenner MK, Clarke S, Mahnke DK, et al. Effect of 22q11.2 deletion on bleeding and transfusion utilization in children with congenital heart disease undergoing cardiac surgery. Pediatr Res 2016; 79(2): 318–324. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Mongero L, Stammers A, Tesdahl E, et al. The effect of ultrafiltration on end-cardiopulmonary bypass hematocrit during cardiac surgery. Perfusion 2018; 33(5): 367–374. [DOI] [PubMed] [Google Scholar]
- 4.Da Broi M, Amarossi A, Spina M, et al. Posterior spine fusion in a Jehovah’s Witness patient with severe rigid idiopathic scoliosis – a case report. Brain Spine 2022; 2: 100883. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Bartley CE, Bastrom TP, Newton PO. Blood loss reduction during surgical correction of adolescent idiopathic scoliosis utilizing an ultrasonic bone scalpel. Spine Deform 2014; 2(4): 285–290. [DOI] [PubMed] [Google Scholar]
- 6.National Clinical Guideline Centre (UK) . Blood transfusion. London: National Institute for Health and Care Excellence (NICE), 2015. [cited 2024 Jul 25]. (National Institute for Health and Care Excellence: Guidelines). Available from: https://www.ncbi.nlm.nih.gov/books/NBK327570/ [PubMed] [Google Scholar]
- 7.Park DK, Braaksma B, Hammerberg KW, et al. The efficacy of preoperative halo-gravity traction in pediatric spinal deformity: the effect of traction duration. Clin Spine Surg 2013; 26(3): 146–154. [DOI] [PubMed] [Google Scholar]
- 8.Hofmann A, Spahn DR, Holtorf AP, et al. Making patient blood management the new norm(al) as experienced by implementors in diverse countries. BMC Health Serv Res 2021; 21: 634. [DOI] [PMC free article] [PubMed] [Google Scholar]