Review of the off-label use of recombinant activated factor VII in pediatric cardiac surgery patients.

In recent years the off-label use of recombinant activated factor VII (rFVIIa) has markedly increased, particularly in pediatric cardiac surgery patients, and practitioners differ widely in their usage of the drug. In 2009, the Congenital Cardiac Anesthesia Society (CCAS) assembled a task force to review the literature on rFVIIa administration to pediatric cardiac surgery patients. The goal of the CCAS Task Force was to assess current practices and make recommendations about rFVIIa therapy to enhance quality of care, improve patient outcomes, reduce costs, and develop future research. In this review we summarized the important topics on current administration of rFVIIa to pediatric cardiac surgery patients including indications for use, efficacy, safety, dosing, and monitoring. All pediatric and pertinent adult literature regarding the administration of rFVIIa to cardiac surgical patients and published since 2000 were selected and studied. Of the 40 pediatric publications reviewed for this report, only 1 was a prospective randomized controlled trial thus making determinations of efficacy difficult. There is no substantive evidence to support the efficacy of rFVIIa as prophylactic or routine therapy during pediatric cardiac surgery. It may prove reasonable as rescue therapy because current observational evidence suggests that potential benefits of rFVIIa for this indication might outweigh the risks. Rescue therapy is appropriate for bleeding that is massive, potentially life-threatening, and refractory to conventional therapy. Nevertheless, extreme caution is advised when considering the administration of rFVIIa to patients who are at risk for thromboembolic complications because rates for clinical and subclinical thrombosis secondary to rFVIIa therapy are unknown at this time. This review is designed to aid practitioners in deciding when and how to administer rFVIIa to pediatric cardiac surgery patients; it is not intended to determine standard-of-care or practice guidelines. There are insufficient data to make evidence-based recommendations. Randomized controlled trials are needed to assess the efficacy of rFVIIa as prophylactic, routine, or rescue therapy and to determine the drug's safety profile particularly with regard to thrombosis. The CCAS rFVIIa Task Force will continue to monitor the literature, gather data, and make updates as more information becomes available.

Segmental wall-motion abnormalities after an arterial switch operation indicate ischemia.

We prospectively studied 29 consecutive neonates undergoing an arterial switch operation to determine if segmental wall motion abnormalities (SWMA) represented myocardial ischemia. Intraoperative transesophageal echocardiogram was recorded at baseline and twice after cardiopulmonary bypass. Cardiac troponin I (cTnI) levels were measured before sternal incision and 3, 6, 12, 24, 48, and 72 h after removal of the aortic cross-clamp. Immediate postoperative Holter and 15-lead electrocardiograms (ECG) were evaluated for ischemia. Transthoracic echocardiograms were obtained before hospital discharge. At bypass termination, immediately after protamine administration, segmental wall motion was normal in nine neonates and abnormal in 20. SWMA were transient in five and present at the time of chest closure in 15 neonates. Neonates in whom SWMA were present at chest closure had more segments involved than those in whom SWMA were transient (P > 0.001). Neonates with SWMA at chest closure had higher cTnI levels postoperatively versus neonates with normal wall motion (P = 0.02). Postoperative ECG data were available in 26 neonates. There was ECG evidence of myocardial ischemia in two of eight neonates with normal wall motion, one of five with transient SWMA, and nine of 13 with SWMA at chest closure. CTnI levels at 12, 24, and 48 h and intraoperative SWMA were predictive of postoperative SWMA. We believe these data indicate that SWMA, which persist at the completion of an arterial switch operation, and which are present in multiple myocardial segments, correlate with myocardial ischemia. Further follow-up of these patients is needed to determine if increased intraoperative myocardial ischemia correlates with long-term outcomes.

The role of transesophageal echocardiography in rapid diagnosis and treatment of migratory tumor embolus.

Transesophageal echocardiography (TEE) is sometimes used in renal cell carcinoma excision for evaluating the extension of tumor in the inferior vena cava (IVC), characterizing the tumor anatomy, monitoring the tumor during surgical mobilization, and assessing cardiac function. Although the risk for embolization is small, when embolization does occur, its consequences can be catastrophic. In this case report, we describe the crucial role of TEE in diagnosing an intraoperative migratory embolus from the IVC to the pulmonary artery and also provide both single-frame photographs and Internet-accessible videos of the event. Our case illustrates the key role that TEE played in the intraoperative management of a patient with renal cell carcinoma undergoing surgical excision of tumor. TEE aided in accurately defining the cephalad extent of the thrombus, provided continuous monitoring of the thrombus during surgical manipulation, and allowed immediate identification of its embolization and proper notification of the surgeons. This case illustrates the crucial role TEE played in the management of a migratory tumor embolus and argues for its routine use during excision of renal cell carcinomas invading the IVC.

Use of recombinant factor VIIa as a rescue treatment for intractable bleeding following repeat aortic arch repair.

Hemorrhage, refractory to aggressive conventional therapy, at a rate of 16 L/hr following separation from cardiopulmonary bypass for aortic arch repair, was controlled with a dose of 90 microg/kg of recombinant factor VIIa, repeated once after 2 hours.

Anesthetic management of the adult patient with congenital heart disease.

As the number of CHD repairs in adults continues to increase, these operations will be performed in a wider variety of institutions and systems. Unfortunately, not all of these centers will have an optimal environment for correcting CHD in adults. This type of surgery is best accomplished in a facility specifically designed for treating adults with CHD. Optimal care of these patients is provided by cardiologists who are trained and experienced in pediatric and adult cardiology, by surgeons who are trained and experienced in treating CHD, and by anesthesiologists who are experienced in caring for adults with CHD. Whatever the setting, cardiac anesthesiologists involved in these cases must be thoroughly aware of the anesthetic implications for the unique pathophysiology of each patient, and they must not rely on their "usual" expectations of either true pediatric CHD or acquired adult heart disease.