Mitral and tricuspid valve surgery in homozygous sickle cell disease: perioperative considerations for a successful outcome

Homozygous sickle cell disease (SCD) presents a multitude of challenges in patients undergoing cardiac surgery with cardiopulmonary bypass. Special consideration must be made in such patients and routine practice modified to prevent hypoxia, hypothermia, acidaemia and low-flow states which may potentially trigger a fatal sickling crisis perioperatively. We discuss several perioperative management strategies including a preoperative exchange transfusion, high flow normothermic bypass and warm blood cardioplegia that was utilized in a woman with homozygous SCD who underwent a successful double valve procedure.     

Acute respiratory insufficiency in sickle cell disease]

Two cases are reported of acute respiratory failure occurring during sickling crises. In the first one, the crisis was characterised by priapism, and in the other one, by abdominal pain. The different causes of these respiratory effects are discussed: infection, fat embolism, pulmonary infarct, haemodynamic pulmonary oedema, as was probably the case in the first patient, or non haemodynamic pulmonary oedema due to sickling, as during conventional treatment of a sickling crisis (oxygen, antibiotics, blood transfusion, cytapheresis). Invasive investigations may contribute to keeping up the clinical picture, because of hypoxic sickling. The water equilibrium of these patients must be monitored with great care. Worsening of the patient's condition despite 48 h of correct treatment must lead to the search for a specific cause.     

Stage 1 palliation for hypoplastic left heart syndrome without the use of allogeneic tissue, with reduced allogeneic blood product exposure: a case report

In the 30 years since Norwood described the palliative procedure for hypoplastic left heart syndrome (HLHS), many modifications have been described which have increased the survival rate of children born with this lesion. We describe further modifications which result in reduced cardiopulmonary bypass time, no cooling or circulatory arrest time, and decreased banked blood exposure. A 16-day-old infant with HLHS undiagnosed during pregnancy presented for stage 1 palliation incorporating the Mee modification, Sano right ventricle to pulmonary artery conduit, dual arterial cannulation of the innominate artery and descending aorta, single venous cannulation of the right atrium, and a bypass prime volume of 130 mL. Anticoagulation and hemostasis were monitored with the Hepcon HMS Plus Hemostasis Management System (Medtronic USA, Minneapolis, MN). Bypass commenced at normothermia. A 5.0 Gore-Tex shunt was placed for the Sano Shunt, and the aortic arch was repaired without use of homologous tissue or synthetic material using a modification of the Mee technique. Bypass time was 92 minutes with a 10 minutes cardiac ischemic time. Modified ultrafiltration (MUF) was performed for 12 minutes and heparinization was reversed with protamine. There was no significant bleeding and no indication to transfuse clotting factors. The patient's only allogeneic donor exposure was 350 mL of red blood cells during bypass necessary to achieve a post MUF hematocrit of 50% per our current institution policy for cyanotic infants. Using modified surgical and perfusion techniques along with low prime bypass circuits can result in reduced cross clamp and bypass times as well as a decrease in blood donor exposure. Hypothetical benefits include reduced operating room, ventilation, intensive care unit, and hospital times, improved neurodevelopmental outcomes, and an overall reduction in the cost of care for infants with HLHS.     

Aortic valve replacement for a patient with essential thrombocythemia: a case report

A 51-year-old male patient with 3+ aortic insufficiency, hepatitis C, osteomyelitis right foot, and a preoperative platelet count 1.3 million/mm3 required cardiopulmonary bypass (CPB). Essential thrombocythemia is a relatively unknown entity with the utilization of CPB. After consulting with the surgeon, an anesthesiologist and another perfusionist, a team approach was used to discuss treatment for this patient during CPB. The treatment consisted of exchange transfusions, autotransfusion techniques, and current protocol for blood gas management. No incidence of hypercoagulability was observed during this procedure or postoperatively. Based on current knowledge of pathophysiology and review of the literature, plateletpheresis should be the current management of essential thrombocythemia.     

A case of pulmonary artery bypass surgery for a patient with isolated Takayasu pulmonary arteritis and a review of the literature.

A 45-year-old female was presented with progressive dyspnea and bilateral leg edema. Pulmonary angiography revealed total occlusion of the right pulmonary artery and significant stenosis of the left pulmonary artery. The inferior lobar artery as well as the segmental arteries were well patent. No pathology was detected elsewhere at the aorta and its branches. The diagnosis of chronic pulmonary arterial occlusion by isolated Takayasu arteritis was made because of the characteristic pattern of angiographic findings and the presence of unusual shunt formation from the coronary artery to the peripheral portion of the pulmonary artery, as well as a characteristic presentation of HLA typing in blood analysis, which strongly suggested the diagnosis of Takayasu arteritis. To restore the pulmonary blood flow, we employed reconstructive surgery by means of bypass procedure, using PTFE graft. Postoperatively there was marked improvement in cardiopulmonary function and the quality of life of the patient. The graft was proved to be patent at long-term follow-up study. An extremely rare case of chronic occlusive pulmonary arteritis, which was surgically treated by means of bypass procedure, is reported herein, and a brief review of previous reports on this subject was attempted.     

Anaesthetic management of a patient with the intracardiac extension of Wilms' tumour

Summary Wilms' tumour (nephroblastoma) is known to invade the inferior vena cava and extend to the intracardiac chambers. We describe the treatment and surgical removal of the intracardiac extension of a Wilms' tumour using cardiopulmonary bypass in a 4-year-old girl. Techniques to avoid paradoxical embolism in the presence of a patent foramen ovale and to deal with excessive hepatic venous blood flow using the Pringle manoevre (hepatic inflour occlusion) are described. Good communication between anaesthesiologists, surgeons and perfusionists was indispensable. The anaesthesiologist is an important member of the team during performance of a complicated procedure.     

Cardiopulmonary bypass reduction of bronchial blood flow: a potential mechanism for lung injury in a neonatal pig model

BACKGROUND: During total cardiopulmonary bypass, blood flow to the lungs is limited to flow through the bronchial arteries. We tested the hypothesis that bronchial blood flow during cardiopulmonary bypass is insufficient to prevent ischemia of the lung and that perfusion of the pulmonary arteries with oxygenated blood during bypass would reduce lung injury. METHODS: Eighteen piglets (5.0 +/- 0.5 kg) were subjected to 120 minutes of normothermic total cardiopulmonary bypass, followed by 60 minutes of postbypass perfusion. Nine of them received continuous pulmonary perfusion with oxygenated blood during bypass. Six additional piglets served as a control group and were mechanically ventilated after sternotomy for 180 minutes only. We quantitated bronchial arterial blood flow, tissue lactate content, and alveolar septal thickness and surface area. We also obtained bronchioalveolar lavage fluid samples. RESULTS: With the beginning of cardiopulmonary bypass, bronchial arterial blood flow decreased to 13% of baseline (42.1 +/- 10.4 to 5.6 +/- 1.0 mL/min). It remained decreased until the end of bypass and returned to starting levels 60 minutes after bypass. The decrease in bronchial blood flow was associated with a 3-fold increase in tissue lactate content. At the end of reperfusion there was a 2-fold increase in alveolar septal thickness and significant accumulations relative to control in the bronchoalveolar lavage fluid of albumin, lactate dehydrogenase, neutrophils, and elastase. Controlled pulmonary perfusion significantly ameliorated all the observed changes. CONCLUSION: Cardiopulmonary bypass caused a reduction in bronchial arterial blood flow, which was associated with injury of the lung. Controlled pulmonary perfusion reduced injury to the lung during bypass. The inflammatory response, as evidenced by bronchoalveolar lavage fluid, may be caused by ischemia.     

The effect of body temperature on leukocyte kinetics during cardiopulmonary bypass

Leukocyte kinetics were investigated in 22 patients undergoing cardiopulmonary bypass to determine the nature of the changes in the white blood cell count associated with this procedure. Both polymorphonuclear leukocytes and lymphocytes were taken up by the lung as pulmonary blood flow was lowered, but only polymorphonuclear leukocytes were taken up as blood flow was restored. The complement 3a level was increased approximately twice the control value within 2 minutes of going on bypass and remained elevated throughout the procedure. The peripheral white blood cell count doubled during the bypass procedure owing to a release of polymorphonuclear leukocytes and their precursors from the bone marrow. The increase in polymorphonuclear leukocytes was prevented by hyperthermia but reappeared quickly when the body temperature was restored to 36 degrees to 37 degrees C.     

Perioperative management of sickle cell disease in paediatric cardiac surgery

In sickle cell disease, cardiopulmonary bypass may induce red cell sickling. Partial exchange transfusion reduces the circulating haemoglobin S level. We report the management of a child with sickle cell disease who required surgical closure of a ventricular septal defect. Preoperative exchange transfusion of 50% of the total blood volume was performed with fresh packed red cells over three days. Further exchange transfusion was performed as cardiopulmonary bypass commenced. The haemoglobin S level was reduced from 76% to 37%. The blood removed from the patient during the exchanges was processed allowing storage and re-infusion of the patient's plasma and platelets. Combined preoperative and intraoperative exchange transfusions, instead of a single stage 50% volume exchange, was effective and potentially avoids larger haemodynamic effects. Cardiopulmonary bypass was conducted at normothermia and cold cardioplegia was avoided (fibrillatory arrest was used during the surgical repair).     

A Simplified Single Pump Circuit for Cardiac Bypass with Autogenous Oxygenation

A simplified extracorporeal circulation (ECC) assemblage with autogenous oxygenation (AO) using a single centrifugal pump was tested in dogs. The transpulmonary gradient was obtained by increasing pressure in the right atrium through volume expansion and decreasing it in the left atrium by collecting the blood from the pulmonary veins in a reservoir placed below the level of the animal, generating a siphon effect. This arrangement dispenses with a right side pump. The heart was electrically fibrillated after perfusion was started and defibrillated at the end of the bypass. This ECC circuit allowed the maintenance of adequate hemodynamic and blood gas parameters during the bypass. The operating field and the mobility of the heart were equivalent to that of conventional cardiopulmonary bypass (CPB). We conclude that the use of a single centrifugal pump simplifies the autogenous oxygenation approach, making it a practical choice for the coronary artery bypass graft (CABG) procedure.     

Major vascular surgery in a patient with sickle cell disease

We report a patient with homozygous sickle cell disease who underwent femoropopliteal bypass for claudication on walking 10m. Isotope studies showed a blood flow in his left femoral artery of only 0.808 ml. 100ml.min-1. The potential problems of sickle cell disease combined with peripheral vascular disease were probably reduced in this patient because he had an increased fetal haemoglobin level of 13%. Despite the femoral artery being clamped for 100 min, no sickling crisis occurred.     

Combined lung and liver transplantation in a girl with cystic fibrosis

PURPOSE: To describe the anesthetic considerations of a combined lung and liver transplant in a 14-yr-old girl with cystic fibrosis. CLINICAL FEATURES: A 14 yr-old girl with cystic fibrosis presented for combined liver and lung transplantation. Anesthetic management was complex in that the pulmonary, hemodynamic, and hematological changes after cardiopulmonary bypass and lung transplantation made the management of the subsequent liver transplant unique. We used a moderate dose fentanyl and isoflurane anesthetic with invasive monitoring including a pulmonary artery catheter. Upon reperfusion of the new liver our patient exhibited severe pulmonary hypertension that was associated with a decrease in cardiac output and systemic hypotension. Utilizing a pulmonary artery catheter, this episode was treated with an increase of prostaglandin E1 (PGE1) infusion to 0.025 microg x kg(-1) x min(-1) and the initiation of 3 microg x kg(-1) x min(-1) dobutamine. The pulmonary hypertension resolved and the cardiac output and blood pressure returned to baseline levels. CONCLUSION: The anesthetic considerations for a combined lung and liver transplant are complex because of the interactions and alterations in cardiovascular, pulmonary and hemostatic systems. The use of a pulmonary artery catheter was critical to the management of our patient because it allowed us to accurately treat an episode of hypotension occurring during liver transplantation. This episode was secondary to acute pulmonary hypertension which is common after pulmonary transplantation but unusual during liver transplantation. It is also critical that a team approach is used to consider all of the concerns of the multiple services managing these complex patients.     

The effect of modified ultrafiltration on the postoperative course in patients with congenital heart disease

Improvements in the technology of cardiopulmonary bypass have significantly reduced morbidity following repair of congenital cardiac defects. However, the use of cardiopulmonary bypass exposes infants to extremes of hemodilution and hyperthermia, often in association with tissue ischemia. Exposure of the blood to surfaces of the bypass circuit initiates a systemic inflammatory response that may result in organ dysfunction after cardiopulmonary bypass, especially the heart, lungs, and brain. The technique of modified ultrafiltration (MUF) was introduced by Naik and colleagues at the Hospital for Sick Children in London over 10 years ago. Since that time, multiple studies have evaluated the effects of MUF on organ function and postoperative morbidity following repair of congenital heart defects. Use of MUF after cardiopulmonary bypass reverses hemodilution and decreases tissue edema resulting in improved pulmonary function with decreased duration of postoperative ventilation, improved left ventricular function, decreased postoperative bleeding, and a decrease in the incidence and duration of pleural effusions following the Fontan procedure. Despite the increasing evidence that the use of MUF reduces postoperative morbidity, many important questions remain unresolved. The mechanisms by which MUF results in these beneficial effects requires additional investigation. In addition, further studies are necessary to identify patients most likely to benefit from MUF and to define the optimal protocols for its use. In the future, prospective randomized studies incorporating recent advances in the technology of cardiopulmonary bypass will be necessary to define the optimal utilization of ultrafiltration during and after cardiopulmonary bypass. Copyright 2003 Elsevier, Inc. All rights reserved.     

Palliation of infants with truncus arteriosus through creation of a pulmonary artery ostial stenosis.

A surgical procedure for palliation of infants with truncus arteriosus and excessive pulmonary blood flow (types I, II, and III) is described. The technique involves the production of ostial stenosis of the pulmonary artery from within the truncus using cardiopulmonary bypass. This method has advantages over pulmonary artery banding, which in general has produced disappointing results.     

Heparinless total left heart bypass with induced ventricular fibrillation

A new technic of extracorporeal total left heart bypass without anticoagulation is described. Left atrial and ventricular cannulas, coated with PPG, drain the blood into a bladder pump which returns blood into the descending thoracic aorta, thus establishing total left heart bypass. In acute experiments, ventricular fibrillation was electrically induced and total left heart bypass was continued for one hour (nine dogs) or four hours (one dog), after which the heart was successfully defibrillated. Near normal systemic circulation was maintained during bypass, although there was no active driving force through the pulmonary circulation except for right atrial contraction. In survival experiments ten dogs were subjected to the same procedure for one to three hours. After defibrillation they were allowed to survive for two to four weeks. Eight were long-term survivors. One died because of a technical error and the other of atelectasis. In four other dogs an autogenous saphenous vein was successfully implanted between the ascending aorta and the circumflex coronary artery using a nonthrombogenic left heart bypass with ventricular fibrillation technic. There was minimal blood loss due to elimination of anticoagulants, and the elimination of an artificial oxygenator simplified the extracorporeal circulation system. During total left heart bypass with ventricular fibrillation, inspiratory pressures were maintained at 10 mm Hg. This study indicates (1) that total left heart bypass can maintain normal systemic circulation in dogs for up to four hours during ventricular fibrillation, and (2) that this technic may be applicable to simplify coronary artery bypass surgery.     

Fast-track cardiac anesthesia in patients with sickle cell abnormalities.

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.     

Pulmonary sequelae of prolonged total venoarterial bypass: evaluation with a new experimental model

Total normothermic venoarterial bypass was established in 6 healthy pigs over a period of 18 hours. A heparin-coated closed extracorporeal system was used and no heparin was administered systemically. During the bypass period the main pulmonary artery was occluded and the heart was maintained in a beating state. All the animals maintained stable hemodynamics and normal blood gases during the entire period of bypass. In the postbypass period, the central hemodynamics continued to be stable while the arterial oxygen tension (inspired oxygen fraction = 0.21) decreased significantly (p less than or equal to 0.05). The total body oxygen uptake, on the other hand, remained unaltered. All the animals died within 4 hours after weaning off the venoarterial bypass circuit on account of pulmonary edema in 2 and cardiac arrest in 4. Death was preceded by progressive pulmonary hypertension and lactacidosis in all the animals. Histological examination of the lungs showed pulmonary parenchymal damage ranging from interstitial edema to intraalveolar hemorrhage and parenchymal necrosis involving more than 80% of the pulmonary parenchyma. A normothermic total venoarterial bypass of 18 hours duration or more produces pulmonary edema of varying severity, pulmonary hypertension, pulmonary parenchymal necrosis, and lactacidosis in healthy juvenile pigs, resulting uniformly in their death. Despite these sequelae the systemic arterial hypoxemia may only be mild to moderate.     

A comparison of blood and Fluosol-DA for cardiopulmonary bypass

Fluosol-DA was compared to blood as a pump prime for total cardiopulmonary bypass in the pig animal model. Nineteen pigs weighing between 14 and 22 kg were studied, nine with blood and ten with Fluosol. Metabolic and hemodynamic measurements were determined before, during and after 60 minute bypass to establish the adequacy of Fluosol to sustain perfusion as compared to blood. The measurements and subsequent calculations included blood gases, arterial and mixed venous oxygen content, oxygen extraction and consumption, cardiac output, systemic and pulmonary vascular resistance and arterial, venous, pulmonary artery and left atrial pressures. The result showed a significant decrease in hematocrit during bypass in the Fluosol group as compared to blood perfusion (20 vs. 30%). While the arterial oxygen content fell from control levels with Fluosol during bypass, in the blood prime group, oxygen content remained at pre-control levels. Whole body oxygen consumption decreased during bypass, in both groups equally, but this decrease did not lead to acidosis and was stable during recovery. Oxygen and carbon dioxide transport were adequately maintained during bypass in both Fluosol and blood groups. Systemic pressures remained stable during bypass and were lower, but stable, during recovery. Pulmonary vascular resistance was elevated in both groups during recovery which probably explains a concomitantly decreased cardiac output. There was a 40% mortality in both experimental groups secondary to postpump pulmonary hypertension. It is concluded that Fluosol is a satisfactory oxygen carrying agent to be used instead of blood during cardiopulmonary bypass, and in the pig model both blood and Fluosol were associated with a high incidence of pulmonary hypertension.     

Purulent bloody drainage after infected total hip arthroplasty: a sign of a leaking mycotic aneurysm

An infected total hip arthroplasty remains one of the most challenging problems faced by orthopaedic surgeons from diagnosis through treatment. We present the case of a 58-year-old woman who was referred to our service with an infected revision total hip arthroplasty. The hip had migrated medially, and the patient had recently noted the presence of blood discharging through a chronic sinus. Angiography revealed a mycotic aneurysm of the external iliac artery. An emergency procedure incorporating a femorofemoral bypass graft and a Girdlestone procedure was undertaken. This is the first reported case of a leaking mycotic aneurysm presenting acutely with blood discharging through a previously chronically discharging sinus tract.     

Liver Transplantation Techniques with Preservation of the Natural Venovenous Bypass: Effect on Surgical Resection of Renal Cell Carcinoma Invading the Inferior Vena Cava

Although renal tumors invading the inferior vena cava (IVC) are unusual, they represent a challenge to the surgical team because their accessibility is difficult. Liver transplantation techniques have been developed that preserve the venous collaterals, enhance the exposure, increase the safety of the resection, and avoid cardiopulmonary bypass. We describe our technique for dealing with renal tumors that have invaded the IVC, a combined experience of two centers, and the safety of the procedure and subsequent low morbidity. Between May 1997 and February 2003, a total of 45 patients (mean age 60.7 years) underwent surgical resection of a renal tumor extending into the IVC by techniques developed from liver transplantation, with the intention to avoid sternotomy and cardiopulmonary bypass. In 42 patients (93.3%) surgical resection of the tumor and thrombus was successful using the transabdominal approach while preserving the venous collaterals; 3 patients with a level IV tumor thrombus required cardiopulmonary bypass. The mean operating time was 342 minutes, and the mean estimated blood loss was 1442 cc. Postoperative ileus in one patient required laparoscopic lysis of the adhesions, and 2 patients (4.4%) died owing to multiple system organ failure and massive pulmonary embolism. The median follow-up was 36 months, during which time 6 patients developed metastatic disease and 37 were disease-free. We concluded that liver transplantation techniques enhance the surgical management of complicated urologic tumors. Patients with tumor thrombus extending to the IVC can be treated while avoiding thoracotomy and cardiopulmonary bypass.