Froukje M. Beynen

First case of X-linked dystonia-parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation

“Lubag” or X‐linked dystonia‐parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45‐year‐old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw‐opening dystonia. At 1‐year follow‐up, his Burke‐Fahn‐Marsden dystonia score and motor Unified Parkinsons Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms.

The incidence of artery puncture with central venous cannulation using a modified technique for detection and prevention of arterial cannulation

BACKGROUND Cannulation of the central circulation is essential for management of patients who require major surgery, and for patients who are critically ill. Arterial puncture is the most frequent complication associated with central venous cannulation, and is potentially fatal. Detection of arterial puncture can be problematic, especially in patients with cyanotic congenital heart disease. METHODS One thousand eleven consecutive cardiothoracic and vascular surgical patients who required central venous cannulation were studied using a new technique for detection of arterial puncture and prevention of arterial cannulation. This technique involves continuous pressure transduction of the steel introducer needle. Central venous cannulation was attempted in all patients. The sites of attempted catheterizations, number of arterial punctures and cannulations, and the number of successful catheterizations were noted. All patients were treated in accordance with standard anesthetic and surgical techniques in the institution. RESULTS One thousand one hundred seventy-two central venous catheters were placed. The overall success rate was 99.6%. The incidence of arterial puncture was 9.3% for central venous cannulation attempts of the internal jugular, subclavian, and femoral veins. No arterial cannulation occurred, and none of the patients had significant complications. Congenital heart disease patients had a higher incidence of arterial puncture (14.1%) and a lower rate (96.8%) of successful cannulation. CONCLUSION Pressure transduction of the steel needle is a useful technique for detecting arterial puncture and preventing arterial cannulation during attempts to achieve central venous cannulation.

Autologous platelet-rich plasma does not reduce transfusion of homologous blood products in patients undergoing repeat valvular surgery

Background:Patients undergoing cardiac surgery employing cardiopulmonary bypass frequently require transfusion of homologous blood products and, therefore, are exposed to the risk of transfusions. Autologous platelet-rich plasma administration may reduce homologous transfusion and attendant risks. Methods:In a blinded, randomized fashion, patients undergoing repeat sternotomy and valvular surgery received either a sham product (n = 28) or autologous platelet-rich plasma (n = 28) at the conclusion of cardiopulmonary bypass. Perioperative blood loss, coagulation profiles, and transfusion requirements were compared between the two groups. Results:In the first 24 h postoperatively, both the plateletrich plasma and sham groups received a median of 10.5 units of homologous blood products. Total median perioperative homologous transfusion requirements were 13 and 11.5 units for the platelet-rich plasma and sham groups, respectively. Conclusions:Autologous platelet-rich plasma did not reduce perioperative bleeding or transfusion requirements in repeat valvular surgery.

The modified fontan procedure: Physiology and anesthetic implications

The modified Fontan operation has gained wide acceptance as a functional corrective procedure for patients with CHD with single ventricle physiology. Long-term survival and palliation of symptoms are excellent with most patients able to lead normal lives. The absence of a pulmonary contractile ventricle means that the single ventricle is responsible for perfusion of both the pulmonary and systemic circulations. Elevated systemic venous pressure is required to overcome PVR and this state of systemic venous hypertension has a significant impact on the anesthetic and postoperative care of these patients.

Intraoperative measurement of activated partial thromboplastin time and prothrombin time by a portable laser photometer in patients following cardiopulmonary bypass

Intraoperative capabilities to rapidly assess coagulation status following cardiopulmonary bypass (CPB) may be of benefit in providing optimal hemostasis and transfusion management, because CPB causes abnormalities in coagulation that may increase morbidity and mortality. The Ciba Corning 512 coagulation monitor (Ciba Corning, Medfield, MA) is a compact and portable device that rapidly determines the prothrombin time (PT) and activated partial thromboplastin time (APTT) in whole blood samples. One hundred patients requiring CPB had APTT and PT determined in whole blood specimens by the 512 coagulation monitor and in plasma specimens by the hospital laboratory from the same arterial blood sample obtained after protamine administration. Correlation coefficients of 0.95 and 0.77 were obtained for the paired APTT and PT tests, respectively (P < 0.01). A bias of 12.6 seconds and 0.77 seconds was determined for the APTT and PT, respectively. The 95% confidence intervals of the bias of the APTT and PT were 9.7 seconds to 15.5 seconds and 0.3 seconds to 1.16 seconds, respectively. The 512 coagulation monitor provided APTT and PT results in less than 3 minutes compared to approximately 45 minutes for the hospital laboratory. A reduction in accuracy was associated with the 512 coagulation monitor PT and APTT when different sampling sites were used. The 512 coagulation monitor accuracy was not affected by a variation of hemoglobin concentration or platelet count between 6 and 12 gm/dL and 15 to 300 x 10(9)/L, respectively. In conclusion, the 512 coagulation monitor provided a rapid APTT and PT result, but the APTT was less accurate. Speeding access to hospital laboratory results would be even more efficacious and accurate.

A Prospective Evaluation of Femoral Artery Monitoring in Pediatric Patients

151 malades pediatriques de chirurgie cardiaque. 165 catheterismes femoraux ont ete etudies

A comparison of washed red blood cells versus packed red blood cells (AS-1) for cardiopulmonary bypass prime and their effects on blood glucose concentration in children.

The effects on blood glucose concentrations of packed red blood cells (AS-1) (group I) versus washed red blood cells (group II) for cardiopulmonary bypass prime were compared in 20 infants weighing less than 10 kg undergoing cardiac surgical procedures. All patients were anesthetized with N2O/O2/isoflurane/fentanyl and received lactated Ringers solution prior to bypass. Blood glucose concentrations prior to bypass were 85 +/- 15 mg/dl (mean +/- SD) in group I and 81 +/- 14 mg/dl in group II. Blood glucose concentrations were 210 +/- 21 mg/dl versus 78 +/- 14 mg/dl (P less than 0.001) 10 min after initiation of bypass, 241 +/- 48 mg/dl versus 107 +/- 28 mg/dl (P less than 0.001) prior to separation from bypass, and 214 +/- 52 mg/dl versus 97 +/- 19 mg/dl (P less than 0.001) after protamine administration in group I and group II, respectively. The use of washed red blood cells for cardiopulmonary bypass priming solution in infants significantly attenuates the increase in blood glucose concentration otherwise observed during cardiopulmonary bypass.

Right ventricular outflow construction for complex congenital cardiac malformations without cardiopulmonary bypass: Management and outcome of 44 patients

A review of surgical and anesthetic techniques used in 44 consecutive patients to establish continuity between the right ventricle and pulmonary circulation, without extracorporeal circulation, as a first-stage repair in a variety of selected complex congenital cardiac lesions with right ventricular outflow obstruction, is presented. The overall operative mortality rate was 9% (four deaths), but no deaths occurred in the last 24 patients. Eleven patients (27.5%) have subsequently undergone complete repair and one patient (2.5%) underwent a Fontan repair. The advantages, anesthetic concerns, complications, and outcome are discussed.