Reoperación para reemplazo valvular mitral en un niño Testigo de Jehová: realidad de la cirugía cardíaca pediátrica sin sangre

ROBERTO L. DE ROSSI, ERNESTO M. JUANEDA, GUSTAVO J. CAMPOS, HECTOR O. DIAZ, CLAUDIO C. MENESES

Unidad Cardiovascular Pediátrica. Sanatorio Allende. Córdoba. Dirección postal : Roberto De Rossi. Fragueiro 2754. 5001 Córdoba. Argentina. e-mail: robderossi@aol.com

Summary

Un niño, Testigo de Jehová, de 8 años, con antecedente de cirugía correctora de canal auriculoventricular completo fue admitido en nuestro Servicio con diagnóstico de insuficiencia mitral severa. La cirugía, realizada con hemodilución normovolémica, reemplazó la válvula mitral por una prótesis mecánica, sin utilizar sangre o sus derivados. El protocolo utilizado consistió en: tratamiento preoperatorio con eritropoyetina recombinante humana, uso de aprotinina durante la cirugía, cuidadosa hemostasia durante la apertura, disección de las adherencias y cierre del tórax, minimización del circuito extracorpóreo y colocación de un catéter venoso triple lumen a través del cual se extrajeron 450 cc de sangre que quedaron en contacto con el paciente por el lumen utilizado. La evolución postoperatoria fue muy buena y el niño fue dado de alta al sexto día con una hemoglobina de 11 g y anticoagulación oral.
Rev. Fed. Arg. Cardiol. 2005; 34: 407-408

Las personas pertenecientes a la religión Testigos de Jehová no aceptan la transfusión de sangre o sus derivados (Génesis 9; Levítico 17).
En estos pacientes la cirugía cardiovascular con circulación extracorpórea representa un desafío para el equipo médico-quirúrgico que debe tratarlos, especialmente en caso de reoperaciones, ya que hay mayor tendencia al sangrado intra y postoperatorio.

Presentamos una reoperación exitosa, para reemplazo de la válvula mitral sin utilización de sangre en un niño de 8 años, Testigo de Jehová, que a los 8 meses de vida había sido sometido a corrección total de canal auriculoventricular completo.

CASO CLINICO Un niño de 8 años de edad fue remitido a nuestro Servicio con diagnóstico de insuficiencia mitral severa, para considerar su tratamiento quirúrgico. Como antecedente más importante, el niño había sido operado a los 8 meses de edad por presentar canal auriculoventricular completo, quedando sin insuficiencia mitral. La evolución fue muy buena y se mantuvo sin medicación hasta 6 meses antes de nuestra intervención, momento en que se le diagnosticó una insuficiencia mitral que fue progresando e hizo necesario el uso de medicación para controlar la sintomatología.

Los estudios que realizamos mostraron insuficiencia mitral severa con conservación de la contractilidad y moderada elevación de las presiones pulmonares.

Como el paciente pertenecía a la religión Testigos de Jehová, luego de hablar con los padres de las diferentes opciones decidimos colocarlo dentro del protocolo de cirugía cardíaca sin sangre que desarrollamos en el Hospital de Niños de Córdoba.

Con un control inicial de laboratorio de 35% de hematocrito (Hto) y 9g de hemoglobina (Hb), fue tratado durante 3 semanas con eritropoyetina recombinante humana, una dosis de 4.000 unidades subcutáneas semanales, hierro por vía oral a 5 mg/kg/día, ácido fólico y complejo B­ 12.
El día previo a la cirugía el Hto era del 40% y la Hb 13g. El recuento de reticulocitos fue del 1,3% y las plaquetas 295.000.

En cirugía se colocó un catéter triple lumen 7F en la vena yugular interna derecha; por uno de los lúmenes se extrajeron 450 cc de sangre que fueron repuestos con la misma cantidad de hidroxietilalmidón (Voluven, Fresenius-Kabi) y quedaron en contacto con el paciente a través de la tubuladura. Se tomó la precaución de colocar una línea purgada (PT26) entre el acceso venoso central y el oxigenador para mantener la continuidad sanguínea.
Con el inicio de la anestesia se comenzó a infundir aprotinina, una dosis inicial de 240 mg/m2 de superficie corporal y un mantenimiento de 56 mg/m2/hora hasta finalizar la cirugía. La operación se realizó con hemodilución normovolémica e hipotermia a 25°C, se pinzó aorta y se infundió cardioplegia cristaloide, que fue aspirada al exterior.

Se abordó la válvula mitral abriendo el septum interauricular a través de la aurícula derecha; se intentó hacer plástica valvular pero, ante el fracaso de la misma por el mal estado de las valvas que estaban engrosadas y retraídas, se colocó una prótesis mecánica bivalva.

Durante el cierre de las aurículas se recalentó al paciente y, luego de purgar el aire de las cavidades izquierdas, se abrió el clamp de aorta. El corazón retomó ritmo sinusal apoyado con dopamina a bajas dosis (5µg/kg/min) y milrinona a 0,4 µg/kg/min.

El tiempo de circulación extracorpórea fue de 137 minutos y el tiempo de clampeo aórtico fue de 117 minutos, manteniéndose el Hto entre el 23% y el 27%.

No se utilizó hemofiltración porque el flujo urinario del paciente era excelente.

Durante la hemostasia se devolvió la sangre del oxigenador a través de la línea previamente preparada; asimismo se reinfundió la sangre que había sido extraída al comenzar el procedimiento.

En el postoperatorio el Hto fue del 30%, la Hb 9gr, las plaquetas 180.000 y la saturación venosa mixta fue del 72%.

La extubación endotraqueal se produjo a las 6 horas y la sedación se mantuvo durante 24 horas, con dexmedetomidina (Precedex, Abbott) en infusión continua de 0,3 a 0,5µg/kg/hora. El sangrado por tubo de drenaje fue de 0,3cc/kg/h durante las primeras 12 horas.

Al día siguiente de la cirugía el Hto era del 32%, se colocaron 4.000 unidades de eritropoyetina endovenosa, se inició el tratamiento con anticoagulantes por la prótesis y se recomenzó la administración de hierro endovenoso.

Al sexto día del postoperatorio fue dado de alta con 11gr de Hb y 205.000 plaquetas.

COMENTARIO El uso de sangre y sus derivados es un hecho común en la cirugía cardíaca, especialmente en las reoperaciones.
Con el creciente conocimiento del peligro de aparición de enfermedades y reacciones adversas inmediatas y tardías por el uso de sangre también hay en la comunidad médica un deseo de desarrollar técnicas y nuevos productos que en lo posible eviten transfusiones de sangre y sus derivados [1,2]. Al respecto abundan las publicaciones de cirugías en adultos pero es muy poca la bibliografía de cirugía cardíaca sin sangre en edad pediátrica [3-5], especialmente tratándose de reoperaciones. Con esta premisa hace dos años en el Hospital de Niños de Córdoba iniciamos un protocolo para realizar cirugía cardiovascular sin sangre [6] que está en constante revisión para ampliar sus límites.

Los pacientes que pertenecen a la religión Testigos de Jehová son un grupo especial que plantea problemas éticos al grupo médico tratante ya que rehusan totalmente el uso de sangre, incluso la extracción y conservación de sangre propia antes de la operación, aunque sí aceptan procedimientos alternativos como los utilizados en este caso [7].

CONCLUSION Con el uso de drogas específicas, el refinamiento de las técnicas quirúrgicas, de anestesia, perfusión y cuidados postoperatorios, y siguiendo líneas de trabajo ya establecidas, pudimos realizar con éxito una reoperación de cambio de válvula mitral en un niño Testigo de Jehová sin utilizar sangre, solucionando su problema cardíaco y respetando sus creencias religiosas.


SUMMARY HEART SURGERY IN A JEHOVA'S WITNESS CHILD WITHOUT THE USE OF BLOOD OR BLOOD PRODUCTS A Jehovah's Witness child, 8 years old, with a previous correction of a complete atrio ventricular canal at the age of 8 months was admitted in our unit with the diagnosis of severe mitral insufficiency. At the operation, done with normovolemic haemodilution, the mitral valve was replaced with a mechanical prosthesis, without the use of blood or blood products. The protocol used was: pre operative treatment with recombinant erythropoietin, the use of aprotinin during the operation, careful control of the haemostasia, minimization of the extracorporeal circuit, and a triple lumen venous catheter. Using one of the lumens, 450 mL of blood were obtained from the patient, and reserved in a closed circuit in continuity with the child's blood.The post operative course was uneventful, and the child was discharged home the 6 th day with haemoglobin level of 11 mg and oral anticoagulation.


Bibliografía
De Ville A: Blood saving in paediatric anaesthesia. Editorial. Paediatric Anaesthesia 1997; 7: 181-182.
de Castro R: Bloodless surgery: establishment of a program for the special medical needs of the Jehovah's Witness community. The gynaecological surgery experience at a community hospital. Am J Obstet Gynecol 1999; 6: 1491-1498.
Van Son J, Hovaguimian H, Rao I y col: Strategies for repair of congenital heart defects in infants without the use of blood. Ann Thorac Surg 1995; 59: 384-388.
Miyagi K, Hannan R, Ojito J y col: The Ross operation in a Jehovah's Witness: a paradigm for heart surgery in children without transfusion. Ann Thorac Surg 2000; 69: 935-937.
Verger L, Vich A, De Rossi R y col: Uso de eritropoyetina en cirugía cardiovascular pediátrica. Presentación de un caso. Rev Arg Transf 2004; XXX: 283-286.
Verger L, Isler M, Pereyra G y col: Cirugía cardiovascular pediátrica: propuesta de un protocolo transfusional restrictivo. Rev Arg Trans 2004; XXX: 259-263.
Rossengart T, Helm R, Klemperer J y col: Combined aprotinin and erythropoietin use for blood conservation: results with Jehovah's Witnesses. Ann Thorac Surg 1994; 58: 1397-1403.

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bloodless surgery

USC Transfusion-Free Medicine and Surgery Program

The practice of bloodless medicine involves the delivery of medical and surgical care without the transfusion of blood products. This technique has long been applied to Jehovah's Witness patients, but it is now experiencing widespread popularity with other patients due to the lessened risk of infection and immunologic complications.
Drs. Rick Selby and Nicolas Jabbour at the USC University Hospital Center for Liver Disease use a multidisciplinary approach to eliminate the need for the transfusion of blood and blood products. The strategy is to build up the body's own reserve of blood components prior to an operation and perform all surgeries in a fashion that minimizes blood loss. Of critical importance are distinct identification of anatomic planes and rapid control of even minor hemorrhage using surgical ligation and local agents that promote coagulation in the operative field. Drs. Selby and Jabbour have honed this technique during the past 10 years as liver transplant surgeons. Liver transplant surgery is a long and complicated procedure in which all efforts must be made to reduce stresses on the patient. Keeping blood loss to a minimum is of vital importance.
Early on, the two surgeons realized the patient benefits of bloodless surgery in transplantation and began applying the same technique to all abdominal surgical procedures that they performed. Regardless of whether their patients were having surgery for something as simple as a hernia or as complex as major liver or pancreatic surgery, the bloodless surgical technique was used. The results for patients were immediately obvious. The frequency of post-surgical infections was lessened, as was the patient's recuperation time. Bloodless surgical techniques are the standard practice of Drs. Rick Selby and Nicolas Jabbour at the USC University Hospital Center for Liver Disease. If you have any questions about these techniques or the USC Center for Liver Disease, call us at (323) 442-5908 or contact us by e-mail at uscliver@surgery.hsc.usc.edu.

Related links:

USC Transfusion-Free Medicine and Surgery Program
World's first adult-to-adult LDLT without blood transfusion
BLOODLESS SURGERY: Transfusion options can reduce infection risks -- from Health Surfing
Blood Saver -- from USC Health Magazine

A Bloodless Coup

World's First Adult-to-Adult Live Donor Liver Transplant Without a Blood Transfusion

World's First Adult-to-Adult Live Donor Liver Transplant Without Blood Transfusion - June 15, 1999William Jennings, 44, a Jehovah's Witness, has made medical history as the recipient of a living-related liver transplant done without the transfusion of blood or blood products. He received the right liver lobe from his brother, Scott Jennings, 40, and both have recovered well from the surgery. More...

Some said it could not be done. Others simply refused to attempt it. But Bill Jennings needed a liver transplant and he needed the procedure to be performed without the transfusion of blood or blood products. Neither his end-stage liver disease nor his religious beliefs as a Jehovah's Witness would allow for anything less.
Jennings already had a donor lined up: his older brother Scott, who was both able and willing. The problem was finding a doctor who would give Jennings a chance at a normal and healthy life while still respecting his religious convictions.
For Jennings, then 44, the father of two and owner of a computer business in Parker, Arizona, this was yet another bump in a road that had begun 20 years earlier with a diagnosis of primary sclerosing cholangitis or PSC. This rare, debilitating disease is associated with a narrowing and inflammation of the liver's bile ducts, eventually leading to cirrhosis of the liver. No one really knows what causes PSC and its resulting cirrhosis--a lack of understanding that frustrated Jennings, who does not smoke and only drank alcohol in moderation. And the only known cure is liver transplantation.
By the beginning of January, 1999, Jennings' condition had taken a turn for the worse. His disease had progressed and he had developed severe jaundice, his light skin taking on a deeply tanned hue. He could no longer work in his computer shop, and could neither sit nor stand for long periods of time. He then contacted three liver specialists in Northern California, where his mother lived. All declined to perform a bloodless version of the transplant he so desperately needed.
Finally, a family friend from Orange County who was a member of the L.A./Orange County Hospital Liaison Committee for Jehovah's Witnesses pointed Jennings in the direction of Randy B. Henderson, manager of the USC Transfusion-Free Medicine and Surgery Program. Henderson set up an appointment for Jennings to see hepatobiliary specialists Rick Selby, M.D., and Nicolas Jabbour, M.D.
On June 15, 1999, Jennings and the USC team made history by taking part in the first-ever live donor liver transplant without a transfusion of blood or blood products.

The Power of Their Convictions

Jehovah's Witnesses are prohibited from the use of blood and blood products because of their acceptance of the Bible as "the inspired word of God," explains Henderson. "Based on several passages in both the Old and New Testament that refer to 'eating' blood and 'abstaining' from blood, they do not accept whole blood or major components-red cells, white cells, platelets or plasma."
But Jehovah's Witnesses are not the only people who are interested in, or even demand, bloodless alternatives. An increasing number of people are rejecting the use of blood for an increasing number of valid reasons. In addition, notes Jabbour, who is both medical director of the Program and associate director of abdominal organ transplantation at USC University Hospital, there are an increasing number of reasons for physicians themselves to consider bloodless alternatives, even if the patient has no objections to the use of blood products. "Blood products are useful but carry their own set of risks," says Jabbour. "Thus, whenever we can avoid using them, we should not only in Jehovah's Witness patients, but in every patient."
Bill Jennings was lucky in that he had the luxury of time. He needed the transplant, of course, and he needed it quickly. But there was time enough to build up his blood levels--in particular, his stores of red blood cells--so that his body could afford to lose some blood during what was sure to be a risky surgery.
Blood has three main cellular components: red cells, white cells and platelets. The percentage of red cells in the blood is measured by hematocrit levels: The normal hematocrit value in an adult man is around 45 percent. Jennings' had dropped to as low as 17. Before they would operate, the USC surgeons wanted to see that boosted to the 45 percent range.
Several things were done to increase his levels. First, blood tests were used sparingly. Second, Jennings was given supplemental iron and folic acid, and a synthetic medication similar to erythropoietin, a hormone-like substance produced by the kidneys that stimulates the bone marrow to produce red blood cells. Those synthetic medications, Epogen or Procrit, are like naturally produced crythropoietin and have the same effects.
The interventional radiologists, led by Michael Katz, M.D., also placed a transjugular intrahepatic portal systemic (TIPS) shunt inside Jennings' liver. Patients with advanced liver disease tend to have dangerous increases in pressure in the veins flowing into the organ; because of the increased pressure, these vessels tend to bleed. A TIPS shunt decreases the pressure in the portal vein, reducing the risk of bleeding both before and during surgery.
Used all together, these techniques were a rousing success. By the day of his surgery, Jennings' hematocrit levels had hit 45 percent. He was ready to go.

Assisting With Anesthesia

Getting Jennings' hematocrit levels up high enough was only the first step in ensuring a successful procedure. The next step was to have a series of reliable and efficient blood management techniques to deal with the inevitable bleeding during the surgery-a task that fell to a USC anesthesiology team of Earl Strum, M.D., Pajesh V. Patel, M.D., and Duraiyah Thangathurai, M.D.
One of these techniques is an approach called acute normovolemic hemodilution, in which the patient's whole blood is collected immediately prior to surgery, and the rest of the blood is diluted with non-blood products such as normal saline or other intravenous solutions. "Consequently," notes Patel, an associate professor of anesthesiology, "if the patient bleeds during surgery, he is bleeding diluted blood." Once the operation is completed, the patient receives the concentrated blood that was collected prior to surgery.
But because Jehovah's Witnesses do not donate or store their own blood, this technique needed to be tweaked in order to be used in the Jennings transplant. To address this, Strum--who serves as interim chair and associate professor in the Department of Anesthesiology, and also holds an appointment with the Department of Orthopaedics--used an innovative device that keeps the collected blood in circuit with the patient. "Since there is still a connection with the body, the collected blood can be transfused back into the patient," Strum notes. "This allows Jehovah's Witness patients to observe their religious beliefs concerning blood products."
A second useful technique involves a device called a "cell saver." A cell saver does precisely that: It collects the patient's blood as it is lost during surgery, cleans it and returns it to the patient. This is acceptable to most Jehovah's Witnesses, says Henderson, because, like the bypass procedure, it "can be viewed as an extension of their circulatory system. The belief is that since the Bible does not specifically comment on these products or equipment, it is left up to each individual to make a conscientious and informed decision."
Finally, doctors working on a bloodless procedure like Jennings’ can give the patient drugs to reduce his blood pressure. This is called hypotensive anesthesia, explains Thangathurai, a professor of anesthesiology who also holds appointments with the departments of surgery and urology. Lowering blood pressure decreases the amount of bleeding that results when blood vessels are cut. "This approach has been especially effective in urology cancer patients," notes Thangathurai.

The Careful Cut

Jennings and his brother, Scott, underwent surgery on Tuesday, June 15th, at 7:30 a.m. The transfusion-free surgical team, under the leadership of Selby, Jabbour and Yuri Genyk, M.D., first removed a segment of Scott's healthy right liver lobe along with the vessels supplying blood to that segment. Jennings' diseased liver was then removed and replaced by the donated healthy segment.
"Bloodless surgery does not require any special instrument or fancy technical changes," Jabbour explains. "All it requires is being meticulous as far as preventing blood loss when we are dissecting the tissue, and knowing how to control the bleeding decisively using sutures and electrocoagulation. Contrary to the image of the 'macho, aggressive, fearless' surgeon, one must be precise and gentle."
"We take our time when performing these major surgeries, including liver transplantation and liver resection," adds Selby. "Through a very careful, calculated approach, we can control the blood flow. Both Scott and Bill Jennings came through their surgeries beautifully. And just eight weeks later, Selby reported, both liver halves, through the organ's natural regenerative ability, had grown to normal size.
"I received excellent care from the surgical team," Jennings said in an interview following the surgery, "and was extremely impressed with their skill and willingness to treat me while respecting my religious convictions."
Related links:
HSC Weekly article, August 13, 1999 - PDF file, 156kb
Live Donor Liver Transplant Home Page
Bloodless Surgery Home Page
USC Liver Transplant Program Faculty & Staff