Open-heart correction of tetralogy of Fallot in an acyanotic dog

Tetralogy of Fallot was diagnosed in an acyanotic 11-month-old dog. Predicted pressure gradient across the pulmonic valve, as assessed by use of continuous wave Doppler echocardiography, was 94.5 mm Hg. Bidirectional shunting was identified by means of selective angiography. Open-heart correction was performed, using a transatrial approach with limited ventriculotomy and cardiopulmonary bypass. The hypertrophied infundibulum was resected, the ventricular septal defect was closed primarily, and a transannular pericardial patch graft was applied. Pressure gradients across the pulmonic valve were 52.9 and 22.8 mm Hg 2 weeks and 4 months after surgery, respectively. Advances in cardiopulmonary bypass, anesthetic management, and use of the transatrial approach may improve the success of open-heart correction of tetralogy of Fallot in dogs.     

Endothelial dysfunction in cerebral microcirculation during hypothermic cardiopulmonary bypass in newborn lambs

OBJECTIVES: Inflammatory stimuli or mechanical stresses associated with hypothermic cardiopulmonary bypass could potentially impair cerebrovascular function, resulting in inadequate cerebral perfusion. We hypothesize that hypothermic cardiopulmonary bypass is associated with endothelial or vascular smooth muscle dysfunction and associated cerebral hypoperfusion. Therefore we studied the cerebrovascular response to endothelium-dependent vasodilator, acetylcholine, endothelium-independent nitric oxide donor, sodium nitroprusside, and vasoactive amine, serotonin, in newborn lambs undergoing hypothermic cardiopulmonary bypass (nasopharygeal temperature = 18 degrees C). METHODS: Studies were performed on 13 newborn lambs equipped with a closed cranial window, allowing for direct visualization of surface pial arterioles. Six animals were studied while undergoing hypothermic cardiopulmonary bypass, whereas seven served as nonbypass, warm (37 degrees C) controls. Pial arteriolar caliber (range = 111 to 316 microm diameter) was monitored using video microscopy. RESULTS: Topical application of acetylcholine caused a dose-dependent increase in arteriolar diameter in the control group that was absent in animals undergoing hypothermic cardiopulmonary bypass. Hypothermic cardiopulmonary bypass did not alter the vasodilation in response to sodium nitroprusside. Furthermore, the contractile response to serotonin was fully expressed during hypothermic cardiopulmonary bypass. CONCLUSIONS: The specific loss of acetylcholine-induced vasodilation suggests endothelial cell dysfunction rather than impaired ability of vascular smooth muscle to respond to nitric oxide. It is speculated that loss of endothelium-dependent regulatory factors in the cerebral microcirculation during hypothermic cardiopulmonary bypass may enhance vasoconstriction, and impaired cerebrovascular function may be a basis for associated neurologic injury during or after hypothermic cardiopulmonary bypass.     

Anesthetic management of a patient with Duchenne's muscular dystrophy undergoing radical repair for tetralogy of Fallot

This report describes a 13-month-old-girl with Duchenne's muscular dystrophy (DMD) who had radical repair for tetralogy of Fallot safely. Patients with DMD are considered to be at risk of malignant hyperthermia (MH). Drugs for induction and maintenance were chosen from a list of agents rarely associated with MH. To wash out the inhalation anesthetics from the equipment, oxygen was circulated continuously for 24 hours. Dantrolene sodium was kept readily available in case of MH occurrence. Differential diagnosis during surgery is difficult in term of the episodes of MH and complications of cardiac surgery, as cardiac surgery is also associated with tachycardia, tachyarrhythmias, metabolic asidosis and red colored urine, which are frequently accompanied by MH. Although increased levels of CK, GOT, LDH and myoglobin strongly support the diagnosis of MH, such evidence can only be confirmed after operation. Fortunately, these factors recovered to the normal range without treatment by dantrolene sodium. During the cardiac surgery, treatment of MH may be delayed due to its late confirmation.     

Cerebral oxygenation during cardiopulmonary bypass in children

OBJECTIVE: Previous work has found cerebral oxygen extraction to decrease during hypothermic cardiopulmonary bypass in children. To elucidate cardiopulmonary bypass factors controlling cerebral oxygen extraction, we examined the effect of perfusate temperature, pump flow rate, and hematocrit value on cerebral hemoglobin-oxygen saturation as measured by near infrared spectroscopy. METHODS: Forty children less than 7 years of age scheduled for cardiac operations with continuous cardiopulmonary bypass were randomly assigned to warm bypass, hypothermic bypass, hypothermic low-flow bypass, or hypothermic low-hematocrit bypass. For warm bypass, arterial perfusate was 37 degrees C, hematocrit value 23%, and pump flow 150 ml/kg per minute. Hypothermic bypass differed from warm bypass only in initial perfusate temperature (22 degrees C); hypothermic low-flow bypass and low-hematocrit bypass differed from hypothermic bypass only in pump flow (75 ml/kg per minute) and hematocrit value (16%), respectively. Cerebral oxygen saturation was recorded before bypass (baseline), during bypass, and for 15 minutes after bypass had been discontinued. RESULTS: In the warm bypass group, cerebral oxygen saturation remained at baseline levels during and after bypass. In the hypothermic bypass group, cerebral oxygen saturation increased 20% +/- 2% during bypass cooling (p < 0.001), returned to baseline during bypass rewarming, and remained at baseline after bypass. In the hypothermic low-flow and hypothermic low-hematocrit bypass groups, cerebral oxygen saturation remained at baseline levels during bypass but increased 6% +/- 2% (p = 0.05) and 10% +/- 2% (p < 0.03), respectively, after bypass was discontinued. CONCLUSIONS: In children, cortical oxygen extraction is maintained during warm cardiopulmonary bypass at full flow and moderate hemodilution. Bypass cooling can decrease cortical oxygen extraction but requires a certain pump flow and hematocrit value to do so. Low-hematocrit hypothermic bypass and low-flow hypothermic bypass can also alter cortical oxygen extraction after discontinuation of cardiopulmonary bypass.     

Phacotrabeculectomy with "smile" incision, punch, and single releasable suture

A 66-year-old female had ischemic heart disease due to left main lesion complicated with calcified ascending aorta, right pelvic carcinoma and liver cirrhosis. The combined operations with coronary artery bypass by left internal thoracic artery under the hypothermic ventricular fibrillation with cardiopulmonary bypass and right nephrectomy were performed successfully. Postoperatively Treadmill exercise test and scintigram revealed no ischemihc change. She is doing well. The aortocoronary bypass to left anterior descending artery using a left internal thoracic artery under hypothermic ventricular fibrillation with cardiopulmonary bypass might be one of surgical options for high risk patient.     

Cerebrovascular relaxation responses to endothelium-dependent and -independent vasodilators after normothermic and hypothermic cardiopulmonary bypass in the rabbit

BACKGROUND: Cardiopulmonary bypass causes activation of leukocytes and increased concentrations of proinflammatory mediators, which may result in endothelial dysfunction. Because hypothermia attenuates many inflammatory processes, the authors hypothesized that hypothermic cardiopulmonary bypass would be associated with better endothelial function than normothermic cardiopulmonary bypass. METHODS: Isoflurane-anesthetized New Zealand White rabbits were randomized to undergo 90 min of either normothermic (37 degrees C, n=9) or hypothermic (27 degrees C, n=9) cardiopulmonary bypass with terminal rewarming. A third group served as anesthetized normothermic non-cardiopulmonary bypass surgical controls (n=8). Basilar artery and descending thoracic aorta were isolated from each animal. In vitro vessel relaxation responses to increasing concentrations of acetylcholine (which induces endothelial release of nitric oxide) and nitroprusside (which provides exogenous nitric oxide) were measured in phenylephrine-precontracted vessel rings. RESULTS: There were no differences in vessel relaxation responses between normothermic and hypothermic cardiopulmonary bypass groups in basilar artery or aorta. In contrast, basilar arteries from non-cardiopulmonary bypass controls had increased relaxation responses to both acetylcholine (P=0.004) and nitroprusside (P=0.031) compared with the pooled cardiopulmonary bypass animal data. CONCLUSIONS: The authors observed no differences in endothelial or vascular smooth muscle function between normothermic and hypothermic cardiopulmonary bypass groups. Compared with non-cardiopulmonary bypass controls, cardiopulmonary bypass appeared to decrease basilar artery smooth muscle relaxation in response to endogenous and exogenous nitric oxide.     

Atrial arrhythmias in adults after repair of tetralogy of Fallot. Correlations with clinical, exercise, and echocardiographic findings

BACKGROUND: The long-term success of intracardiac repair of tetralogy of Fallot is hampered by the occurrence of arrhythmias. Numerous studies have stressed the potential role of ventricular arrhythmias. However, the importance of other arrhythmias in the morbidity of these patients appears to be underestimated. Furthermore, most follow-up studies have been limited to children or adolescents, whereas many patients have reached adulthood after earlier repair of tetralogy of Fallot. The aim of the present study was to determine the incidence of atrial fibrillation, atrial flutter, and other supraventricular arrhythmias in adult patients after intracardiac repair for tetralogy of Fallot and their correlation with surgical and clinical findings. METHODS AND RESULTS: The study group consisted of 53 consecutive patients referred to the Thoraxcenter adult congenital heart disease clinic. They underwent repair at a mean age of 9.1 years (range, 0.7 to 55 years). The median age at the time of study was 23.2 years (range, 15 to 57 years; mean age, 26.6 years), and the mean duration of follow-up of 17.5 years (range, 1.4 to 32 years) after surgery. Records were reviewed extensively for evidence of arrhythmias. The follow-up study included routine 12-lead ECG, 24-hour continuous ambulatory monitoring, and echocardiography, and 46 patients underwent exercise testing. Sinus node dysfunction was recorded in 19 patients (36%), of whom 4 required a permanent pacemaker. Atrial fibrillation or flutter was found in 12 patients, and other supraventricular tachycardias were found in 6. The former were more frequently of older age at follow-up. Antiarrhythmic therapy and cardioversion were typically directed at control of atrial (and not ventricular) tachyarrhythmias. Ten patients (19%) showed nonsustained ventricular tachycardia; they were older at initial surgery and older at follow-up and had more intracardiac repairs and longer cardiopulmonary bypass times. CONCLUSIONS: Despite an emphasis on ventricular ectopy in past series, the main sources of morbidity in adult patients after surgical correction of tetralogy of Fallot emanated from atrial arrhythmias, which were present in one third of the patients.     

Deep hypothermia

Early correction of congenital cardiac defects has been facilitated by the use of deep hypothermia and cardiocirculatory arrest. The technique currently used by us consists of surface cooling to 24 degrees C, total hemodilution, cardiopulmonary bypass to 20 degrees C and complete cardiocirculatory arrest. Rewarming is achieved with a pump oxygenator. In over 266 small children, 67% of whom were in the first year of life, lesions corrected included transposition of the great arteries (TGA)(43%), ventricular septal defect (16%), tetralogy of Fallot (16%), and total anomalous pulmonary venous drainage (5%). Early primary correction with deep hypothermia has a risk that is comparable to or better than staged repair.     

Effects of cardiopulmonary bypass and circulatory arrest on endothelium-dependent vasodilation in the lung

Endothelial injury with failure of pulmonary endothelium-dependent vasodilatation has been proposed as a possible cause for the increased pulmonary vascular resistance observed after cardiopulmonary bypass, but the mechanisms underlying this response are not understood. An in vivo piglet model was used to investigate the role of endothelium-dependent vasodilatation in postbypass pulmonary hypertension. The pulmonary vascular responses to acetylcholine, a receptor-mediated endothelium-dependent vasodilator, and nitric oxide, an endothelium-independent vasodilator, were studied in one group of animals after preconstriction with the thromboxane A2 analog U46619 (n = 6); a second group was studied after bypass with 30 minutes of deep hypothermic circulatory arrest (n = 6). After preconstriction with U46619, both acetylcholine and nitric oxide caused significant decreases in pulmonary vascular resistance (34% +/- 6% decrease, p = 0.007, and 39% +/- 4% decrease, p = 0.001). After cardiopulmonary bypass with circulatory arrest, acetylcholine did not significantly change pulmonary vascular resistance (0% +/- 8% decrease, p = 1.0), whereas nitric oxide produced a 32% +/- 4% decrease in pulmonary vascular resistance (p = 0.007). These results demonstrate a loss of receptor-mediated endothelium-dependent vasodilatation with normal vascular smooth muscle function after circulatory arrest. Administration of the nitric oxide synthase blocker Ngamma-nitro-L-arginine-methyl-ester after circulatory arrest significantly increased pulmonary vascular resistance; thus, although endothelial cell production of nitric oxide may be diminished, it continues to be a major contributor to pulmonary vasomotor tone after cardiopulmonary bypass with deep hypothermic circulatory arrest. In summary, cardiopulmonary bypass with deep hypothermic circulatory arrest results in selective pulmonary endothelial cell dysfunction with loss of receptor-mediated endothelium-dependent vasodilatation despite preserved ability of the endothelium to produce nitric oxide and intact vascular smooth muscle function.     

Improved exposure of the pulmonary arteries for thromboendarterectomy

Pulmonary thromboendarterectomy is a surgical technique for treating pulmonary hypertension caused by unresolved pulmonary embolism. It has been recommended to perform this procedure under deep hypothermic circulatory arrest. Here we describe two technical modifications: (1) improved exposure to the right pulmonary artery by division of the superior caval vein and (2) thromboendarterectomy in normothermic cardiopulmonary bypass, with beating heart or electrically induced ventricular fibrillation. These modifications allow complete endarterectomy of both pulmonary arteries under normothermic conditions, thus avoiding hypothermic circulatory arrest, which results in short cardiopulmonary bypass times and reduces the morbidity and mortality of this procedure.     

Consequences of electroencephalographic-suppressive doses of propofol in conjunction with deep hypothermic circulatory arrest

BACKGROUND: Some patients who undergo cerebral aneurysm surgery require cardiopulmonary bypass and deep hypothermic circulatory arrest. During bypass, these patients often are given large doses of a supplemental anesthetic agent in the hope that additional cerebral protection will be provided. Pharmacologic brain protection, however, has been associated with undesirable side effects. These side effects were evaluated in patients who received large doses of propofol. METHODS: Thirteen neurosurgical patients underwent cardiopulmonary bypass and deep hypothermic circulatory arrest to facilitate clip application to a giant or otherwise high-risk cerebral aneurysm. Electroencephalographic burst suppression was established before bypass with an infusion of propofol, and the infusion was continued until the end of surgery. Hemodynamic and echocardiographic measurements were made before and during the prebypass propofol infusion and again after bypass. Emergence time also was determined. RESULTS: Prebypass propofol at 243 +/- 57 micrograms.kg-1.min-1 decreased vascular resistance from 34 +/- 8 to 27 +/- 8 units without changing heart rate, arterial or filling pressures, cardiac index, stroke volume, or ejection fraction. Propofol blood concentration was 8 +/- 2 micrograms/ml. Myocardial wall motion appeared hyperdynamic at the end of cardiopulmonary bypass, and all patients were weaned therefrom without inotropic support. After bypass, vascular resistance decreased further, and cardiovascular performance was improved compared to baseline values. Nine of the 13 patients emerged from anesthesia and were able to follow commands at 3.1 +/- 1.4 h. Three others had strokes and a fourth had cerebral swelling. CONCLUSIONS: Propofol infused at a rate sufficient to suppress the electroencephalogram does not depress the heart or excessively prolong emergence from anesthesia after cardiopulmonary bypass and deep hypothermic circulatory arrest.     

Temporary extracorporeal axillofemoral venous bypass--a beneficial device in operation for superior vena caval syndrome due to intrathoracic malignancies

Life-threatening complications can occur unexpectedly during general anesthesia in operations for superior vena caval (SVC) syndrome due to intrathoracic tumors. To prevent such sequelae, we have placed a temporary extracorporeal axillofemoral venous bypass graft with satisfactory results. In six patients (malignant mediastinal tumor, four; lung cancer, two), under local anesthesia before induction of general one, the cannulas, each appropriately sized in accordance with the diameter of the axillary and femoral veins, were directly introduced into the corresponding veins after systemic heparinization. The two cannulas were connected with a tube to which a side arm, which was usually clamped, was connected. The venous pressure of the internal jugular vein decreased immediately after establishment of the temporary bypass in all patients. The symptoms that accompanied SVC syndrome did not worsen and the life-threatening complications at the time of induction of general anesthesia were avoided with this procedure. In each case cardiopulmonary bypass on stand-by was unnecessary at this time. Venous bypass grafting with vascular prostheses were mainly performed under cardiopulmonary bypass, which was required for such operative procedures. In three patients the side arms were used for part of the venous drainage during cardiopulmonary bypass. The SVC syndrome instantly disappeared after operations in all patients including one in hospital death. No serious complications related to the temporary bypass procedure have been observed. This temporary bypass procedure has several advantages. It can be safely performed under local anesthesia with no special technique for the cannulation. Venous blood naturally drains from the upper part to the lower part of the body by pressure gradient, that warrants the safe induction of general anesthesia and ensuing operative procedures. The side arm is available for venous drainage during cardiopulmonary bypass. There are no serious complications related to the bypass procedure. Thus this temporary bypass is recommended as a life-saving and auxiliary device in urgent operations for acute progressive SVC syndrome with symptoms of cerebral edema and upper airway obstruction due to intrathoracic malignancies.     

Endothelin receptor blockade prevents the rise in pulmonary vascular resistance after cardiopulmonary bypass in lambs with increased pulmonary blood flow

BACKGROUND: Children with increased pulmonary blood flow may experience morbidity as the result of increased pulmonary vascular resistance after operations in which cardiopulmonary bypass is used. Plasma levels of endothelin-1, a potent vasoactive substance implicated in pulmonary hypertension, are increased after cardiopulmonary bypass. OBJECTIVES: In a lamb model of increased pulmonary blood flow after in utero placement of an aortopulmonary shunt, we characterized the changes in pulmonary vascular resistance induced by hypothermic cardiopulmonary bypass and investigated the role of endothelin-1 and endothelin-A receptor activation in postbypass pulmonary hypertension. METHODS: In eleven 1-month-old lambs, the shunt was closed, and vascular pressures and blood flows were monitored. An infusion of a selective endothelin-A receptor blocker (PD 156707; 1.0 mg/kg/h) or drug vehicle (saline solution) was then begun 30 minutes before cardiopulmonary bypass and continued for 4 hours after bypass. The hemodynamic variables were monitored, and plasma endothelin-1 concentrations were determined before, during, and for 6 hours after cardiopulmonary bypass. RESULTS: After 90 minutes of hypothermic cardiopulmonary bypass, both pulmonary arterial pressure and pulmonary vascular resistance increased significantly in saline-treated lambs during the 6-hour study period (P <.05). In lambs pretreated with PD 156707, pulmonary arterial pressure and pulmonary vascular resistance decreased (P <. 05). After bypass, plasma endothelin-1 concentrations increased in all lambs; there was a positive correlation between postbypass pulmonary vascular resistance and plasma endothelin-1 concentrations (P <.05). CONCLUSIONS: This study suggests that endothelin-A receptor-induced pulmonary vasoconstriction mediates, in part, the rise in pulmonary vascular resistance after cardiopulmonary bypass. Endothelin-A receptor antagonists may decrease morbidity in children at risk for postbypass pulmonary hypertension. This potential therapy warrants further investigation.     

Mitral valve repair in a patient with severe porcelain aorta

We repaired the mitral valve in a patient with severe porcelain aorta. Significant mitral regurgitation developed in a 66-year-old woman with heavy calcification throughout the whole aorta. At operation, cardiopulmonary bypass was properly established by combined axillary and femoral arterial cannulations for sufficient systemic flow. Likewise, the combination of a superior mitral approach and profound hypothermic fibrillatory arrest in conjunction with low-flow cardiopulmonary bypass allowed us to repair the mitral valve successfully.     

Risk factors for higher cost in congenital heart operations

BACKGROUND: For many congenital heart defects, hospital mortality is no longer a sensitive parameter by which to measure outcome. Although hospital survival rates are now excellent for a wide variety of lesions, many patients require expensive and extensive hospital-based services during the perioperative period to enable their convalescence. These services can substantially increase the cost of care delivery. In today's managed care environment, it would be useful if risk factors for higher cost could be identified preoperatively so that appropriate resources could be made available for the care of these patients. The focus of this retrospective investigation is to determine if risk factors for high cost for repair of congenital heart defects can be identified. METHODS: We assessed financial risk by tracking actual hospital costs (not charges) for 144 patients undergoing repair of atrial septal defect (58 patients), ventricular septal defect (48 patients), atrioventricular canals (14 patients), or tetralogy of Fallot (24 patients) at Duke University Medical Center between July 1, 1992, and September 15, 1995. Furthermore, we were able to identify where the costs occurred within the hospital. Financial risk was defined as a large (> 60% of mean costs) standard deviation, which indicated unpredictability and variability in the treatment for a group of patients. RESULTS: Cost for atrial septal defect repair was predictably consistent (low standard deviation) and was related to hospital length of stay. There were factors, however, for ventricular septal defect, atrioventricular canal, and tetralogy of Fallot repair that are identifiable preoperatively that predict low- and high-risk groups using cost as an outcome parameter. Patients undergoing ventricular septal defect repair who were younger than 6 months of age at the time of repair, who required preoperative hospital stays of longer than 7 days before surgical repair, or who had Down's syndrome had a less predictable cost picture than patients undergoing ventricular septal defect repair who were older than 2 years, who had short (< 4 days) preoperative hospitalization, or who did not have Down's syndrome ($48,252 +/- $42,539 versus $15,819 +/- $7,219; p = 0.008). Patients with atrioventricular canals who had long preoperative hospitalization (> 7 days), usually due to pneumonia (respiratory syncytial virus) with preoperative mechanical ventilation had significantly higher cost than patients with atrioventricular canals who underwent elective repair with short preoperative hospitalization ($83,324 +/- $60,138 versus $26,904 +/- $5,384; p = 0.05). Patients with tetralogy of Fallot had higher costs if they had multiple congenital anomalies, previous palliation (combining costs of both surgical procedures and hospital stays), or severe "tet" spells at the time of presentation for operation compared with patients without these risk factors ($114,202 +/- $88,524 versus $22,241 +/- $7,071; p = 0.0005). One patient (with tetralogy of Fallot) with multiple congenital anomalies died 42 days after tetralogy of Fallot repair of sepsis after a gastrointestinal operation. Otherwise, hospital mortality was 0% for all groups. CONCLUSIONS: Low mortality and good long-term outcome for surgical correction of congenital heart defects is now commonplace, but can be expensive as some patients with complex problems receive the care necessary to survive. This study demonstrates that it is possible to identify factors preoperatively that predict financial risk. This knowledge may facilitate implementation of risk adjustments for managed care contracting and for strategic resource allocation.     

Detection of optic disc changes with Glaucoma-Scope probability maps

Before use of cardiovascular surgical techniques and procedures in humans, many experiments, e.g., hypothermic circulatory arrest and cardiopulmonary bypass using the heart-lung machine, have been performed in the dog. As a consequence experimental canine cardiovascular surgery is highly developed. This has not resulted in the routine performance of open heart surgery in veterinary medicine, probably because of the high costs. Cardiovascular surgery in the dog is generally limited to interventions not depending on hypothermic circulatory arrest or cardiopulmonary bypass. The clinical cardiovascular surgery in dogs can be divided into routine and more specialized interventions. The first category includes correction of peritoneopericardial diaphragmatic hernia, pericardial fenestration in dogs with pericardial effusion, treatment of persistent right aortic arch, and patent ductus closure. The specialized interventions include dilation of pulmonic and aortic stenoses and pacemaker implantation. The diagnosis and surgical treatment of such diseases is described. New developments in cardiovascular surgical treatment that can be expected include catheter techniques for occlusion of shunts and dilations using balloons, because the financial costs of these procedures are not prohibitive.     

Generation of chimeric C5a/formyl peptide receptors: towards the identification of the human C5a receptor binding site

The surgical management of tetralogy of Fallot has undergone important changes in recent years. Earlier repair of tetralogy of Fallot is now favored by many institutions. At Stanford University Medical Center, we have performed definitive repair of tetralogy of Fallot at the time of presentation, regardless of the child's age, with few exceptions. In this report, we describe our results with early repair, and we believe these support the contention that infants who undergo early repair (< 1 year of age) have postoperative results similar to those of children who undergo repair at an older age. Complications related to shunts are prevented by the infant repairs, and, in the future, reduced ventricular ectopy may be demonstrated to be a benefit of such repairs.     

Granular cell tumor of the pituitary stalk

Erythropoietin is a hormone responsible for regulation of red blood cell production. Circulating erythropoietin values are regulated by renal oxygen supply, which is determined by hemoglobin concentration, hemoglobin oxygen saturation, and renal blood flow. Previous animal and human studies regarding erythropoietin regulation have assumed pulsatile renal blood flow. During cardiopulmonary bypass, non-pulsatile renal perfusion has been shown to result in decreased glomerular filtration rate and decreased renal blood flow in comparison to pulsatile perfusion. Repair of congenital heart disease during cardiopulmonary bypass is an attractive circumstance in which to study the effect of non-pulsatile blood flow on erythropoietin production. The hypothesis in this study was that non-pulsatile perfusion would result in increased erythropoietin production because of decreased renal oxygen supply. Fourteen children with congenital heart disease and without preoperative renal insufficiency or anemia were enrolled in the study. All patients underwent cardiopulmonary bypass with non-pulsatile flow. In addition, 10 control patients without congenital heart disease were enrolled. Six cardiopulmonary bypass patients had 1.5- to 6-fold increases in plasma erythropoietin concentrations from baseline. These patients had longer cardiopulmonary bypass times, more commonly performed under low flow deep hypothermic conditions. The remaining 8 patients with congenital heart disease, and all control patients, did not develop increased postoperative erythropoietin concentrations. The conditions under which cardiopulmonary bypass are performed appear to influence postoperative circulating erythropoietin concentrations.     

Diagnosis of familial Holt-Oram syndrome

Presented is one rare case in a family affected by a Holt- Oram-Syndrome. This syndrome is associated with an upper limb malformation and a congenital heart disease. In our case we found radiusaplasia on both sides, thenaraplasia on the left hand, a hypoplastic thumb on the right hand. The heart was malformed as a Fallot tetralogy, the left kidney was absent. Four additional affected members of the family are described. By routine ultrasound examination we could not find this malformation syndrome. In families with affected history ultrasound screening examination should be done on a center for prenatal diagnosis.     

Cerebral oxygenation during warming after cardiopulmonary bypass

OBJECTIVES: To evaluate jugular venous oxygen saturation (SjVO2), measured with a fiberoptic oximetry catheter, and brain tissue oxygen saturation, measured by near-infrared spectroscopy (NIRSO2), as monitors of cerebral oxygenation during cardiopulmonary bypass surgery. DESIGN: Prospective, clinical study. SETTING: Operating room of a Veterans Administration Hospital. PATIENTS: Nineteen patients undergoing moderate hypothermic cardiopulmonary bypass surgery. INTERVENTIONS: SjvO2 and NIRSO2 were monitored in the patients during the surgical procedure. MEASUREMENTS AND MAIN RESULTS: Moderate hypothermic cardiopulmonary bypass surgery had two distinct cerebral hemodynamic phases. While the patients were hypothermic, SjvO2 averaged 80 +/- 7% and none of the patients had an increase in cerebral lactate production. During the rewarming period, however, reductions in SjvO2 to < 50% occurred in 16 (84%) patients and increased cerebral anaerobic metabolism developed in 11 (58%) patients. SjvO2 during rewarming was dependent on mean arterial pressure, with 60 mm Hg appearing to be a critical value. Two other factors appeared to also contribute to the jugular desaturation, a low hematocrit and a rapid warming time. The SjvO2 catheter had excellent performance during the surgery. The average difference between paired measurements of SjvO2 by the catheter and in blood samples was -0.4 +/- 4.25%, and the correlation between the two measurements was highly significant (r2 = .93; p < .001). The NIRSO2 trended with the SjvO2 in most patients (r2 = .63; p < .001). CONCLUSIONS: The study confirms other studies showing that jugular venous desaturation can occur during rewarming after cardiopulmonary bypass surgery. Presently, SjvO2 appears to be a better monitor of cerebral oxygenation than NIRSO2. However, NIRSO2 has promise as a noninvasive monitor of cerebral oxygenation if future developments allow more quantitative measurements of oxygen saturation.