PBLD Submission for 2013 Winter Scientific Meeting in Las Vegas, Nevada IMPROVING SAFETY IN THE PEDIATRIC CARDIAC CATH LAB

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PBLD Submission for 2013 Winter Scientific Meeting in Las Vegas, Nevada Title: Moderators: IMPROVING SAFETY IN THE PEDIATRIC CARDIAC CATH LAB Joyce Phillips, MD, FAAP and Jennifer Dillow, MD, FAAP The Division of Pediatric Anesthesia The Children s Hospital of New Mexico University of New Mexico Goals: At the completion of the discussion the learner will be able to: 1. Identify factors which contribute in making the pediatric cardiac cath lab a high risk anesthetizing environment. 2. Describe three anesthetic/sedation techniques for the child undergoing cardiac catheterization 3. Discuss which anesthetic technique might be best for a. Child with single ventricle undergoing diagnostic cardiac cath pre-fontan b. Child undergoing device closure of ASD c. Child undergoing myocardial biopsy 4. Review the management of local anesthetic toxicity in children 5. Identify and treat cardiac tamponade Description: Adverse events occur more commonly during cardiac catheterization than during pediatric anesthesia in general. 1 We discuss two cases where untoward events occurred in the pediatric cardiac cath lab. In both cases, understanding of environment, identification of roles and good communication in addition to medical knowledge are essential to improving outcomes. Case # 1: 19 month old female s/p repair of TOF with pulmonary atresia/mapcas, brought to the cath lab for diagnostic cardiac catheterization to evaluate increasing right ventricular outflow obstruction. During the catheterization done under precede/ketamine sedation a generalized tonic clonic seizure occurred following a toxic injection of lidocaine into the aorta.

Discussion Outline: 1. The Environment: Performing anesthesia for children in the cardiac catheterization lab comes with increased risks and unique challenges. - What is the system in your institution to provide anesthesia for the pediatric cardiac catheterization lab? Who provides the anesthesia care? - How should the patients be evaluated pre-operatively? Does that differ from what actually occurs in your institution? - What are your safety concerns? What are the most common complications minor and major- associated with cardiac catheterization? How common is cardiac arrest? 2. The Case: You are evaluating a 19-month old female with a history of tetralogy of fallot with pulmonary atresia and MAPCAS (major aortopulmonary collateral arteries) s/p repair who will be having a diagnostic cardiac catheterization tomorrow. The cardiology nurse has told you she is having increasing right ventricular outflow obstruction. She had a RV to PA conduit placed at 1 month of age during the repair. - What details do you want to ask about her history? What things are you looking for on physical exam? - What diagnostic studies are necessary to plan her anesthetic? 3. History, cont.: After taking a thorough history, you find she s having fatigue, shortness of breath with playing, and intermittent blue spells. She has also lost some weight over the last few months, dropping from over 8 kg to her current weight of 7.5 kg. Her O2 saturation is 93% in RA. Her echocardiogram shows: S/p repair of ToF/PA/MAPCAs with 11 mm conduit, with increasing obstruction. Normal LV function, moderately dilated RA and RV, moderate pulmonary (conduit) regurgitation. A CBC/T&S is ordered. Her nervous parents ask what kind of anesthetic she will have - Based on the information you have gathered, what is your anesthetic plan? - Are there any advantages/disadvantages to using sedation vs. GETA vs. GA/LMA in this patient? Are you worried about using ketamine in patients with pulmonary hypertension? - What are your hemodynamic goals for a patient with a fixed pulmonary obstruction? - Which anesthetic technique may be best for the below patients and why? Is there any role for regional anesthesia or a caudal? o Child with single ventricle undergoing diagnostic cardiac cath pre- Fontan o Child undergoing device closure of an ASD o Child undergoing myocardial biopsy with TTE? TEE?

4. To the cath lab! You bring the patient to the cath lab, an inhalation induction is performed and a PIV is secured (after 3 attempts) with blood sent for type and screen. You then start a dexmedetomidine infusion at 2 mcg/kg/hr, give a ketamine bolus, and place a nasal canula on the patient who is spontaneously ventilating. The procedure is proceeding smoothly (with loud 80 s rock blaring), when oxygen saturation drops from 94% to 85%...75% abruptly. - What are you thinking? What do you do? 5. The unlabeled syringe: You look down at your patient who appears to be having a tonic clonic seizure. You administer 100% O2 via mask and ask the cardiologist if he did anything differently. He looks down at an empty syringe with a ripped label in his hand and remarks that he thought he injected saline into the catheter sitting in the LV, but it could have been Lidocaine. You proceed to administer propofol and intubate the patient. The EKG shows sinus tachycardia with a HR of 140. - What are the signs and progression of local anesthetic toxicity? - How do you treat local anesthetic toxicity? When would you start using intralipid? At what dose? - Does cardiac resuscitation differ when treating local anesthetic toxicity versus another etiology? How? 6. Recovery: The catheterization is completed and the results conclude the patient s restrictive RV to PA conduit will need to be replaced surgically in the very near future. The patient is spontaneously breathing, awakens, and is extubated. As you are walking to the recovery area, you pass the MRI suite and wonder - Could the same information obtained via cardiac catheterization today be gathered from performing a cardiac MRI instead? What are the potential advantages and disadvantages of a cardiac MRI for this patient? For a patient with known pulmonary branch stenosis? Which would you prefer to perform the anesthesia for? - In your institution, where will this patient recover? Be admitted to?

Case #2: 12 month old, 10 kg male with RSV and new onset cardiomyopathy is brought to the cardiac cath lab for diagnostic cath and myocardial biopsy. Discussion Outline: 1. A Sick Kiddo: You evaluate your patient in the PICU. He was admitted 8 days ago with respiratory distress and RSV. His condition deteriorated 3 days ago when he was transferred to the PICU, intubated, and placed on the ventilator. The patient was started on an epinephrine drip the same day for hypotension. An echocardiogram was done which showed evidence of cardiomyopathy with poor LV function and an EF of 15%. He remains on a low-dose Epinephrine drip and is sedated and paralyzed. - Which anesthestic risks do you discuss with the parents? Written or verbal consent? - What is the best anesthetic technique for this patient undergoing a cardiac catheterization and myocardial biopsy? 2. All Done!: After using 0.5 MAC Sevo and rocuronium for his anesthetic, the catheterization and biopsy is relatively uneventful except for some episodic hypotension during catheter manipulation (with SBP s dipping from the mid- 80 s to 60 s). As everyone exits the room and the Sevo is turned off, you note the arterial line tracing begins to dampen. The SBP drops from the 85 70 50. You flush your arterial line and the cuff does not read a blood pressure. There is no response to 5, or 10, or 20 mcg boluses of epinephrine. The EKG appears to be sinus tachycardia at 170, but now there is no blood pressure. - What are the possible etiologies of this rapid decline? - What are the clinical signs of cardiac tamponade? What is seen on echocardiogram? - What are your options to treat acute cardiac tamponade? 3. Phew!: CPR is initiated. 10 mcg/kg of Epinephrine is given. An echo is performed and a large cardiac effusion is diagnosed. 100 cc of blood is aspirated via cardiocentesis and the patient s blood pressure returns to baseline. What is your scariest/craziest/wackiest experience in the peds cardiac cath lab??!!

DISCUSSION: Today there is an increasing demand for the anesthesiologist to provide sedation and/or anesthesia services in the cardiac catheterization laboratory. 1 The pediatric cardiac catheterization laboratory has evolved into an arena for both diagnostic and interventional procedures. Today both palliative procedures (balloon septostomy/valvuloplasy) and corrective procedures, such as atrial septal defect and patent ductus arteriosus occlusion are performed. Children undergoing staged surgical procedures for repair of complex congenital heart disease return to the cath lab repeatedly for both diagnostic and interventional procedures. Providing anesthesia services in the cardiac catheterization laboratory presents unique challenges to the anesthesiologist. In the cath lab, the anesthesiologist is faced with the logistical problems involved in providing anesthesia outside of the operating room. Unfamiliar location, equipment, personnel, decreased room temperature, poor lighting, radiation exposure and poor maneuverability may present difficulties. In addition, children with congenital heart disease represent a high risk population. Adverse events occur more commonly during cardiac catheterization than during pediatric anesthesia in general. Cases with highest risk are those in under 1 year olds and those including therapeutic intervention other than PDA or ASD occlusion. 2 PREOPERATIVE EVALUATION AND ANESTHETIC PLAN: In planning an anesthetic for the pediatric patient in the cardiac cath lab, the anesthesiologist must consider the patients underlying heart disease and the purpose of the planned procedure. A thorough preoperative history should be performed. A functional cardiac history and cardiac exam should be performed. The anesthesiologist should review any previous cardiac studies including the patient s most recent echocardiogram and/or catheterization report. Often children with CHD have associated syndromes which require an understanding of the implications for anesthetic management. In addition, an understanding of the anticipated procedure, approach to vascular access and hemodynamic goals for the patient are imperative in planning the anesthetic. Multiple techniques have been used successfully in providing sedation and anesthesia for the pediatric patient in the cardiac catheterization lab. Various studies have shown the effectiveness of ketamine and/or midazolam 3, dexmedetomidine-ketamine, propofol ketamine 4,dexmedetomidine in various doses 5,6, and ketamine and sevoflurane 7. Airway management as well as mode of ventilation impact oxygenation, ventilation and measurement of hemodynamic parameters. How to best manage the airway during the procedure is a judgment made by the anesthesiologist after evaluation of the patient and consultation with the cardiologist. The following variables should be considered regarding airway management: oxygen concentration, natural vs instrumented airway, spontaneous vs controlled ventilation, need for supplemental NO and TEE.

COMPLICATIONS IN THE PEDIATRIC CARDIAC CATH LAB: Adverse events occur during pediatric cardiac catheterization with increased frequency than during pediatric anesthesia in general. The event rate in children under one year of age was 13.9% compared to 6.7% for children over the age of 1. 2 The overall mortality cited in a 2005 study by Bennet was.08%. The incidence of cardiac arrest was 1 in 200. It is apparent that critical incidents occur in the cardiac cath lab and are more likely to occur in the younger child. This requires adequate staffing and expertise when assigning anesthesia personnel to the pediatric cardiac cath lab. We present 2 cases for discussion where significant adverse events occurred. The first case involves local anesthetic toxicity. A dose of 5 mg/kg of lidocaine was mistakenly flushed into the LV line of a 20 month old female s/p TOF repair undergoing diagnostic and interventional cardiac catheterization for dilation of a stenotic RV-PA conduit. This resulted in a tonic clonic seizure which changed the course of the anesthetic. The lidocaine on the field was poorly labeled and mistaken for saline. This points out the necessity of standardization in the cardiac cath lab. It is important that safety initiatives such as patient verification, time out and labeling of all medications be followed in the cath lab. Symptoms of local anesthetic toxicity include CNS excitement, CNS depression and cardiac toxicity. Treatment of local anesthetic systemic toxicity in this case involved airway management. Rhythm and hemodynamic changes did not occur. Benzodiazepines have been recommended as first line treatment of seizures related to LAST. 8 Should cardiac toxicity and cardiac arrest occur, ACLS guidelines should be followed as well as treatment with lipid emulsion. 8 The second case involves a myocardial biopsy in an infant with acute myocarditis. During the biopsy the patient developed a perforation, hemopericardium, cardiac tamponade and arrest which responded to pericardiocentesis and CPR. A restrospective review of pediatric patients undergoing myocardial biopsy places this patient at high risk for perforation. Patients less than 10 kg, on pressors undergoing biopsy for myocarditis were found to have a 33% rate of perforation. 9 This raises the question for discussion of informed consent prior to the procedure and the necessity and expediency of surgical availability. CONCLUSION: In summary, anesthesiologists are being called upon with increasing frequency to provide services for pediatric patients in the cardiac catheterization laboratory. The cardiac cath lab is a high risk environment where adverse events occur. Improving safety involves advanced planning, knowledge of the patients anatomy and

physiology, communication with the cardiologist, adherence to standards as well as situational awareness. References: 1. Verghese ST, Martin G.R., Heavy Sedation versus General Anesthesia for Pediatric Invasive Cardiology: A Grayer Shade of Blue versus a Pinker Shade of Pale? Pediatric Cardiology 2003 24: 193-194 2. Bennett D., Marcus R., Stokes M, Incidents and Complications During Pediatric Cardiac Catheterization. Pediatric Anesthesia 2005 15: 1083-1088 3. Jobeir A et al, Use of Low-Dose Ketamine and/or Midazolam for Pediatric Cardiac Catheterization: Is an Anesthesiologist Needed? Pediatric Cardiology 2003 24:236-243 4. Tosun Z., Akin A, et al, Dexmedetomidine-Ketamine and Propofol- Ketamine Combinations for Anesthesia in Spontaneously Breathing Pediatric Patients Undergoing Cardiac Catheterization. Journal of Cardiothoracic and Vascular Anesthesia, Vol 20, No 4 (August), 2006: pp515-519. 5. Munro H., Tirotta CF, et al, Initial Experience with dexmedetomidine for diagnostic and interventional cardiac catheterization in children. Pediatric Anesthesia 2007 17:109-112. 6. Fishburn S., Phillips J., Effect of High Dose Dexmedetomidine-Ketamine Sedation on Respiratory Function and PVR in Children with Congenital Heart Disease.Poster Presentation 2011, CCAS-SPA Meeting, Tampa, Florida 7. Williams GD, Philip BM et al, Ketamine Does Not Increase Pulmonary Vascular Resistance in Children with Pulmonary Hypertension Undergoing Sevoflurane Anesthesia and Spontaneous Ventilation. Anesthesia and Analgesia, Vol. 105, No 6, December 2007: 1578-1584. 8. Neal J., Bernards C. et al ASRA Practice Advisory on Local Anesthetic Toxicity. Regional Anesthesia and Pain Medicine Volume 35, Number 2, March-April 2010. 9. Pophal SG. et al, Complications of Endomyocardial Biopsy in Children. Journal of the American College of Cardiology Vol. 34, No 7, December 1999:

10. Fogel M, Pawlowski BA, et al. Cardiac Magnetic Resonance and the Need for Routine Cardiac Catheterization in Single Ventricle Patients Prior to Fontan: A Comparison of 3 Groups. Journal of the American College of Cardiology 2012; 60(12): 1094-1102. 11. Spodick, D. Acute Cardiac Tamponade. New England Journal of Medicine 2003; 349(7): 684-690.