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Aborted Sudden Cardiac Death in a 14-Year-Old Athlete: The Anomalous Coronary Artery
DEPARTMENT
Case Study—Acute and Specialty Care
Aborted Sudden Cardiac Death in a 14-Year-Old Athlete: The Anomalous Coronary Artery Megan Trahan, MS, CPNP-AC, & Shari Simone, DNP, CPNP-AC
KEY WORDS Sudden cardiac death, anomalous coronary artery, athlete
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Megan Trahan, Pediatric Critical Care Nurse Practitioner, Department of Pediatrics, University of Maryland Medical Center, Baltimore, MD. Shari Simone, Senior NP Clinical Program Manager, Women’s and Children’s Services, University of Maryland Medical Center, Baltimore, MD. Conflicts of interest: None to report. Correspondence: Megan Trahan, MS, CPNP-AC, Department of Pediatrics, University of Maryland Medical Center, 110 South Paca St, 8th Floor, Baltimore, MD 21201; e-mail: mtrahan@peds. umaryland.edu. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.02.001
www.jpedhc.org
A 14-year old female athlete with no significant medical history was admitted to the pediatric intensive care unit (PICU) after sudden cardiac arrest while playing field hockey. On the day of presentation, she experienced chest pain during a field hockey game and became unresponsive. Cardiopulmonary resuscitation (CPR) was performed by a bystander, and emergency medical services was called. Upon evaluation by paramedics, the initial cardiac rhythm was interpreted as torsades de pointes (Figure 1), and she successfully converted to normal sinus rhythm with defibrillation and administration of intravenous lidocaine. The patient was transported to a local emergency department, where she was intubated for respiratory distress and transferred to the PICU. According to her family, in the past few months the patient had reported chest pain with shortness of breath upon exertion, but it resolved with rest. Results of her last sports physical were normal. MEDICAL HISTORY The patient was previously healthy. Her family history was noncontributory; there were no unexplained deaths in the family or a history of collagen vascular disorders or asthma. The patient was not taking any medications, and the family denied a history of drug, alcohol, or substance abuse. INITIAL PHYSICAL EXAMINATION AND LABORATORY FINDINGS Upon presentation to the PICU, the following vital signs were obtained: temperature, 36.1 C; pulse, 100 beats per minute; blood pressure, 130/80 mmHg; respiratory -/- 2014
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FIGURE 1. An example of an electrocardiogram pattern of torsades de pointes ventricular tachycardia. This figure appears in color online at www.jpedhc.org.
rate, 15 breaths per minute; and oxygen saturation, 100% ventilated with 60% oxygen. Her growth parameters were age appropriate. Except for a cervical collar in place and pale lips, findings of her head and neck examination were unremarkable. A respiratory examination revealed coarse lung sounds bilaterally with crackles noted in the bases. The patient’s respiratory effort was synchronous with the ventilator. A cardiovascular examination demonstrated marginal perfusion with 1+ distal pulses and a capillary refill time of 3 to 4 seconds. A normal S1S2 was auscultated with no murmurs, rubs, or gallops. An abdominal examination was unremarkable, and no hepatosplenomegaly was noted. The patient was sedated but able to follow simple commands. Her pupils were equal and reactive to light. No rashes, lesions, or signs of trauma were noted.
Initial laboratory results from the outlying emergency department, presented in the Table, were significant for hyperglycemia and elevated liver enzymes. In addition, arterial blood gas values revealed a mixed respiratory and metabolic acidosis with a pH of 7.12 (normal, 7.35-7.45); carbon dioxide, 55 mmHg (normal, 35-45 mmHg); oxygen, 160 mmHg (normal, 80-100 mmHg); bicarbonate, 17 mEq/L (normal, 21-24 mEq/L); base deficit, 12 mmol/L (normal, 1 to +1); and oxygen saturation, 98.1%. PICU COURSE An initial diagnosis of prolonged QT syndrome was made given the initial rhythm noted by emergency medical services, although her corrected QT interval was within normal limits on subsequent
TABLE. Laboratory data Parameter
Patient’s value
Normal value
White blood cell count Hemoglobin Hematocrit Platelets Sodium Potassium Chloride Carbon dioxide Blood urea nitrogen Creatinine Glucose Total calcium Ionized calcium Magnesium Phosphorus Aspartate aminotransferase Alanine aminotransferase Alkaline phosphatase Total bilirubin Total protein Albumin
17,800/mL 14 g/dL 40.7% 212,000/mL 142 mEq/L 3.8 mEq/L 109 mg/dL 23 mEq/L 13 mg/dL 0.9 mg/dL 241 mg/dL 7.3 mg/dL 1.19 mmol/L 1.9 mEq/L 5.1 mg/dL 338 U/L 315 U/L 298 U/L 0.3 mg/dL 6 mg/dL 3.7 mg/dL
4,500-13,500/mL 12-16 g/dL 37-45% 150-350/mL 135-147 mEq/L 3.5-5.1 mEq/L 97-107 mEq/L 22-26 mEq/L 6-20 mg/dL 0.5-1.0 mg/dL 60-100 mg/dL 8.4-10.2 mg/dL 1.18-1.32 mmol/L 1.26-2.1 mg/dL 2.4-4.4 mg/dL 13-45 U/L 5-30 U/L 100-320 U/L <1.5 mg/dL 6.0-8.0 g/dL 3.6-5.2 g/dL
Note. Normal values are expressed in conventional units. Data from Tschudy, M. M., & Arcara, K. A. (2012). The Harriet Lane handbook (19th ed.). Philadelphia, PA: Elsevier.
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electrocardiograms (ECGs). The patient was scheduled to have surgery for an implantable cardioverter/defibrillator (AICD). Continuing evaluation of the arrest included an echocardiogram, a cardiac magnetic resonance imaging (MRI) scan, and a gated cardiac computed tomography (CT) scan, although none was diagnostic of the origin of the coronary arteries as a result of pulmonary hemorrhage from CPR. The patient was extubated on hospital day three, and the echocardiogram and gated cardiac CT scan were repeated the day before the scheduled AICD surgery. On both repeat diagnostic tests, findings included an
anomalous left coronary artery arising from the right coronary sinus (Figure 2) with a short intramural course, in which the coronary artery travels within the aortic wall and the ostium (orifice) has a slitlike appearance rather than the normal round appearance. The AICD placement was cancelled, and the patient was taken to the operating room for unroofing of the left coronary artery with reimplantation of the ostium into the left coronary sinus (Figure 3). The patient had no major postoperative complications and is awaiting her scheduled 3-month follow up with a stress test, echocardiogram, and coronary CT scan.
CASE STUDY QUESTIONS 1. 2. 3. 4.
What is the differential diagnosis of sudden cardiac arrest in the healthy adolescent athlete? What is the pathophysiology of an anomalous coronary artery arising from the opposite sinus of Valsalva? What is the appropriate diagnostic workup for a suspected anomalous coronary artery? What is the current management strategy and expected outcomes for patients with an anomalous coronary artery? 5. What are some primary and secondary strategies for prevention of sudden cardiac death from anomalous coronary arteries and other causes? CASE STUDY ANSWERS 1. What is the differential diagnosis of sudden cardiac arrest in the healthy adolescent athlete? The overwhelming majority of fatalities from sudden cardiac arrest in a healthy athlete occur as a result of cardiovascular causes, mainly structural defects (Maron, Doerer, Haas, Tierney, & Mueller, 2009). The prevailing cause of sudden cardiac death in young athletes is hypertrophic cardiomyopathy and ‘‘possible hypertrophic cardiomyopathy,’’ in which the heart has a noticeable increase in mass without the clinical features of hypertrophic cardiomyopathy. Other cardiovascular causes include myocarditis, aortic aneurysms, arrhythmogenic right ventricle, Wolf-Parkinson-White disorder, and long QT syndrome (Angelini, 2007; Berger, Kugler, Thomas & Friedberg, 2004). Among noncardiovascular causes of sudden cardiac death, blunt chest trauma precipitating commotio cordis is the most common phenomenon. This phenomenon occurs when a low-impact blow to the chest precipitates a life-threatening dysrhythmia—usually ventricular fibrillation. Commotio cordis is most commonly seen in the sports of baseball, softball, hockey, and lacrosse (Maron, Gohman, Kyle, Estes, & Link, 2002). 2. What is the pathophysiology of an anomalous coronary arising from the opposite sinus of Valsalva? The second most common cause of sudden cardiac arrest after hypertrophic cardiomyopathy is aberrant coronary arteries, with the anomalous left coronary www.jpedhc.org
artery arising from the right sinus of Valsalva as the primary lethal defect (Maron, 2003). Anomalous coronary artery from the opposite facing sinus of Valsalva (ACAOS) is a congenital heart defect in which a coronary artery arises from the opposite sinus and has an anomalous course (Angelini, Villason, Chan, & Diez, 1999). The two most common forms of ACAOS are anomalous left coronary artery arising from the right coronary sinus and anomalous right coronary artery arising from the left coronary sinus. Anomalous left coronary arteries are more commonly known to cause sudden cardiac death than their counterpart (Eckart, Jones, Shry, Garrett, & Scoville, 2006). Of those, both right and left, that cause sudden cardiac death, only approximately 37% to 52% of the patients were symptomatic in the months prior to their death (Basso, Maron, Corrado, & Thiene, 2000; Mainwaring et al., 2011). Symptoms of anomalous coronary arteries are a result of cardiac ischemia and include chest pain and syncope (Mainwaring et al., 2011). Some controversy exists with regard to the cause of ischemic symptoms in patients with anomalous coronary arteries. Initially, the common thought was that both the pulmonary artery and the aorta exerted pressure on the coronary artery in a hyperdynamic state (i.e., with exercise), causing poor perfusion to the distal portion of the vessel. This idea has lost favor, because the pulmonary artery does not create enough pressure, even during exercise, to create such an effect. Other theories include a narrowed ostium, an acute angulation of the artery at the point of entry at -/- 2014
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FIGURE 2. Normal and anomalous left coronary artery from the right coronary cusp with intramural course. L = left coronary cusp; P = posterior non-coronary cusp; PA = pulmonary artery; R = right coronary cusp. This figure appears in color online at www.jpedhc.org.
the aorta, or coronary spasm from endothelial damage (Kaushal et al., 2011). Current opinion purports that the most likely cause of ischemia and related symp-
toms is the length of the anomalous artery that runs tangential to and shares a common wall with the aorta. The pressure of the aorta increases during exercise,
FIGURE 3. Unroofing and reimplantation of the left coronary artery. A, The normally positioned right coronary artery orifice and the anomalous left coronary artery orifice both arising from the right sinus and an intramural segment of the left coronary. B, The intramural segment of the coronary is unroofed to create a neo-orifice in the left sinus. C, The left coronary artery is reimplanted into a normal position. (Reprinted from Pediatric Clinics of North America, 51, Jaquiss, R.D.B., Tweddell, J.S., & Litwin, S.B., Surgical therapy for sudden cardiac death in children, 1394, Copyright (2004) with permission from Elsevier.) This figure appears in color online at www.jpedhc.org.
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which compresses the coronary artery and limits coronary blood flow. 3. What is the appropriate diagnostic workup for a suspected anomalous coronary artery? An anomalous coronary artery from the opposite sinus of Valsalva can result in a diagnostic challenge, as evidenced by this case study. A simple ECG and stress test are not sufficient and have been normal prior to sudden cardiac arrest in patients with ACAOS (Eckart et al., 2006). Current practice guidelines, including those from the American Heart Association (AHA; Bluemke et al., 2008), call for specific evaluation of anomalous coronary artery with any incidence of sudden cardiac death, life-threatening dysrhythmias, and ischemic symptoms in the pediatric and young adult population. To date, several algorithms have been suggested for the evaluation of anomalous coronary arteries, although none has been included in existing practice guidelines. Angelini (2007) suggested that the evaluation should include an ECG, Holter monitor, and echocardiogram with Doppler. If the echocardiogram identifies two normal coronary ostia, no further testing is required. If, however, the echocardiogram cannot decipher two normal ostia, an MRI or CT scan is indicated. A diagnosed anomalous coronary artery also requires a baseline stress test. Current opinions differ as to the optimal modality for diagnosis of ACAOS if the echocardiogram cannot clearly delineate coronary artery origins. Although coronary angiography was considered the gold standard in the past, a gated cardiac CT scan has been shown to identify coronary anatomy when angiography yielded equivocal results (Datta et al., 2005). A cardiac CT scan, although efficient, requires use of intravenous contrast material and radiation, although current techniques have considerably limited the radiation dose. For this reason, some authors strongly recommend coronary MRI/magnetic resonance angiography (MRA; Bluemke et al., 2008). However, coronary MRA requires a relatively stable patient and is liable to a certain amount of artifact (Arrigan, Killeen, Dodd, & Torreggiani, 2011). At this point, clinical judgment and patient condition dictate which modality must be used. 4. What is the current management strategy and expected outcomes for patients with an anomalous coronary artery? Several management options exist for the treatment of ACAOS. Both nonsurgical and surgical options may be considered, depending on which coronary artery is anomalous, if there is an intramural course, and presenting symptoms (Mainwaring et al., 2011). Although several surgical options are available for a patient with ACAOS, nonoperative management is now an opwww.jpedhc.org
tion for patients with an anomalous right coronary artery if certain criteria are met. A practice guideline from the AHA states that anomalous left coronary arteries must be operated on because of the risk of sudden death, whereas with right coronary arteries, surgery is indicated if there is an intramural course, if the artery courses between the aorta and the pulmonary artery, or if the patient has ischemic symptoms (Bluemke et al., 2008). Practice guidelines from the American College of Cardiology also recommend that all patients with unrepaired anomalous coronary arteries should be totally restricted from participating in competitive sports (Graham et al., 2005). Many options are available to the pediatric cardiothoracic surgeon for operative repair of ACAOS; these options include unroofing of the intramural segment, in which the shared common wall between the ACAOS and aorta is excised, reimplantation of the anomalous coronary artery, and coronary artery bypass grafting with use of the saphenous or internal mammary artery (Figure 3). Although an explanation of surgical options is beyond the scope of this paper, it should be noted that the two techniques most frequently utilized include unroofing and reimplantation. Individual anatomy plays a large role in the selection of the preferred surgical correction. Bypass grafting has been deemed undesirable because of graft atrophy from competitive flow (Warnes et al., 2008). Whereas the diagnosis of ACAOS has been associated with adverse outcomes such as sudden cardiac death, the primary surgery to repair this defect has very few associated complications. Authors of all but one study found no complicaWhereas the tions/minimal complications or residual diagnosis of coronary ischemia in ACAOS has been the corrected anomaassociated with lous coronary artery by unroofing of the adverse outcomes intramural segment such as sudden (Frommelt, Frommelt, cardiac death, the Tweddell, & Jaquiss, 2003; Romp et al., primary surgery to 2003). Authors of one repair this defect study found no has very few residual symptoms, and 85% of patients associated were unrestricted in complications. activities, with a 63% participation rate in sports at 5-year follow-up (Mainwaring et al., 2011). Authors of only one study found evidence of ischemia in 9 of 24 patients postoperatively, utilizing three stress test modalities to evaluate for ischemia (Brothers et al., 2007). All of these patients were asymptomatic. Current practice guidelines for adults recommend a -/- 2014
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3-month waiting period after a full surgical repair before return to sports if the patient is asymptomatic and has no evidence of ischemia (Warnes et al., 2008). To date, no studies have been performed to examine the outcomes of a conservative approach to asymptomatic anomalous right coronary artery with no intramural course. 5. What are some primary and secondary strategies for prevention of sudden cardiac death from anomalous coronary arteries and other causes? Prevention of sudden cardiac death is at the crux of a debate involving appropriate athlete screening in the United States and internationally. The American Academy of Pediatrics (AAP) released a policy statement in 2012 regarding sudden cardiac arrest. Primary prevention of sudden cardiac arrest includes appropriate diagnosis and treatment of the common causes responsible for the arrest. Primary prevention includes preparticipation sports physicals by the athlete’s primary care physician with use of a formal screening tool in conjunction with a thorough cardiovascular examination. The utility of a screening ECG in the prevention of sudden cardiac arrest is addressed in the policy statement. Although screening ECGs are endorsed by the European Society of Cardiology and refuted by the AHA, the AAP (2012) does not take a stance, stating only that the issue requires ‘‘more data and debate.’’ Secondary prevention of sudden cardiac arrest includes early CPR combined with early defibrillation using an automated external defibrillator. The AHA and the AAP both advocate for emergency response preparedness and automated external defibrillator programs in schools and sports facilities. Anomalous coronary artery from the opposite sinus of Valsalva is a rare but serious congenital heart defect. Presentation can be dramatic, frequently with sudden cardiac death, and diagnosis continues to be a challenge for practitioners, as evidenced by the case presentation. Appropriate nonsurgical and surgical management lead to a favorable prognosis with few complications. REFERENCES American Academy of Pediatrics, Section on Cardiology and Cardiac Surgery. (2012). Pediatric sudden cardiac arrest. Pediatrics, 129, e1094. Angelini, P. (2007). Coronary artery anomalies: An entity in search of an identity. Circulation, 115, 1296-1305. Angelini, P., Villason, S., Chan, A., & Diez, J. (1999). Normal and anomalous coronary arteries in humans. Philadelphia, PA: Lippincott Williams & Wilkins. Arrigan, M., Killeen, R., Dodd, J., & Torreggiani, W. (2011). Imaging spectrum of sudden athlete cardiac death. Clinical Radiology, 66, 203-223.
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Basso, C., Maron, B., Corrado, D., & Thiene, G. (2000). Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. Journal of the American College of Cardiology, 36(6), 1493-1501. Berger, S., Kugler, J. D., Thomas, J. A., & Friedberg, D. Z. (2004). Sudden cardiac death in children and adolescents: Introduction and overview. Pediatric Clinics of North America, 51(5), 1201-1209. Bluemke, D., Achenbach, S., Budoff, M., Gerber, T., Gersh, B., Hillis, D., . Woodard, P. K. (2008). Noninvasive coronary artery imaging: Magnetic resonance angiography and multidetector computed tomography angiography: A scientific statement from the American Heart Association Committee on Cardiovascular Imaging and Intervention of the Council on Cardiovascular Radiology and Intervention, and the Councils on Clinical Cardiology and Cardiovascular Disease in the Young. Circulation, 118(5), 586-606. Brothers, J., McBride, M., Seliem, M., Marino, B., Tomlinson, R., Pampaloni, M. H., . Paridon, S. M. (2007). Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. Journal of the American College of Cardiology, 50, 2078-2082. Datta, J., White, C., Gilkeson, R., Meyer, C., Kansal, S., Jani, M., . Read, K. (2005). Anomalous coronary arteries in adults: Depiction at multi-detector row CT angiography. Radiology, 235, 812-818. Eckart, R., Jones, S., Shry, E., Garrett, P., & Scoville, S. (2006). Sudden death associated with anomalous coronary origin and obstructive coronary disease in the young. Cardiology in Review, 14, 161-163. Frommelt, P., Frommelt, M., Tweddell, J., & Jaquiss, R. (2003). Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course. Journal of the American College of Cardiology, 42, 148-154. Graham, T., Driscoll, D., Gersony, W., Newberger, J., Rocchini, A., & Towbin, J. (2005). Task force 2: Congenital heart disease. Journal of the American College of Cardiology, 45(8), 1326-1333. Kaushal, S., Backer, C., Popescu, A., Walker, B., Russell, H., Koenig, P., . Mavroudis, C. (2011). Intramural coronary length correlates with symptoms in patients with anomalous aortic origin of the coronary artery. Annals of Thoracic Surgery, 92, 986-992. Mainwaring, R., Reddy, M., Reinhartz, O., Petrossian, E., MacDonald, M., Nasirov, T., . Hanley, F. L. (2011). Anomalous aortic origin of a coronary artery: Medium-term results after surgical repair in 50 patients. Annals of Thoracic Surgery, 92, 691-697. Maron, B. (2003). Sudden death in young athletes. New England Journal of Medicine, 349, 1064-1075. Maron, B., Gohman, T., Kyle, S., Estes, N., & Link, M. (2002). Clinical profile and spectrum of commotio cordis. Journal of the American Medical Association, 287, 1142-1146. Maron, B., Doerer, J., Haas, T., Tierney, D., & Mueller, F. (2009). Sudden deaths in young competitive athletes: Analysis of 1866 deaths in the United States, 1980-2006. Circulation, 119, 1085-1092. Romp, R., Herlong, J. R., Landolfo, C., Sanders, S., Miller, C., . Jaggers, J. (2003). Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Annals of Thoracic Surgery, 76, 589-596. Warnes, C., Williams, R., Bashore, T., Child, J., Connolly, H., Dearani, J. A., . Yancy, C. W. (2008). ACC/AHA 2008 Guidelines for the management of adults with congenital heart disease. Circulation, 118, e714-e833.
Journal of Pediatric Health Care
Case Study—Acute and Specialty Care
Aborted Sudden Cardiac Death in a 14-Year-Old Athlete: The Anomalous Coronary Artery Megan Trahan, MS, CPNP-AC, & Shari Simone, DNP, CPNP-AC
KEY WORDS Sudden cardiac death, anomalous coronary artery, athlete
Section Editors Karin Reuter-Rice, PhD, CPNP-AC, FCCM Corresponding Editor Duke University Durham, North Carolina Terea Giannetta, DNP, RN, CPNP California State University Children’s Hospital Central California Fresno, California Maureen A. Madden, MSN, RN, CPNP-AC, CCRN, FCCM Rutgers Robert Wood Johnson Medical School New Brunswick, New Jersey Bristol Myers Squibb Children’s Hospital New Brunswick, New Jersey Megan Trahan, Pediatric Critical Care Nurse Practitioner, Department of Pediatrics, University of Maryland Medical Center, Baltimore, MD. Shari Simone, Senior NP Clinical Program Manager, Women’s and Children’s Services, University of Maryland Medical Center, Baltimore, MD. Conflicts of interest: None to report. Correspondence: Megan Trahan, MS, CPNP-AC, Department of Pediatrics, University of Maryland Medical Center, 110 South Paca St, 8th Floor, Baltimore, MD 21201; e-mail: mtrahan@peds. umaryland.edu. J Pediatr Health Care. (2014) -, ---. 0891-5245/$36.00 Copyright Q 2014 by the National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pedhc.2014.02.001
www.jpedhc.org
A 14-year old female athlete with no significant medical history was admitted to the pediatric intensive care unit (PICU) after sudden cardiac arrest while playing field hockey. On the day of presentation, she experienced chest pain during a field hockey game and became unresponsive. Cardiopulmonary resuscitation (CPR) was performed by a bystander, and emergency medical services was called. Upon evaluation by paramedics, the initial cardiac rhythm was interpreted as torsades de pointes (Figure 1), and she successfully converted to normal sinus rhythm with defibrillation and administration of intravenous lidocaine. The patient was transported to a local emergency department, where she was intubated for respiratory distress and transferred to the PICU. According to her family, in the past few months the patient had reported chest pain with shortness of breath upon exertion, but it resolved with rest. Results of her last sports physical were normal. MEDICAL HISTORY The patient was previously healthy. Her family history was noncontributory; there were no unexplained deaths in the family or a history of collagen vascular disorders or asthma. The patient was not taking any medications, and the family denied a history of drug, alcohol, or substance abuse. INITIAL PHYSICAL EXAMINATION AND LABORATORY FINDINGS Upon presentation to the PICU, the following vital signs were obtained: temperature, 36.1 C; pulse, 100 beats per minute; blood pressure, 130/80 mmHg; respiratory -/- 2014
1
FIGURE 1. An example of an electrocardiogram pattern of torsades de pointes ventricular tachycardia. This figure appears in color online at www.jpedhc.org.
rate, 15 breaths per minute; and oxygen saturation, 100% ventilated with 60% oxygen. Her growth parameters were age appropriate. Except for a cervical collar in place and pale lips, findings of her head and neck examination were unremarkable. A respiratory examination revealed coarse lung sounds bilaterally with crackles noted in the bases. The patient’s respiratory effort was synchronous with the ventilator. A cardiovascular examination demonstrated marginal perfusion with 1+ distal pulses and a capillary refill time of 3 to 4 seconds. A normal S1S2 was auscultated with no murmurs, rubs, or gallops. An abdominal examination was unremarkable, and no hepatosplenomegaly was noted. The patient was sedated but able to follow simple commands. Her pupils were equal and reactive to light. No rashes, lesions, or signs of trauma were noted.
Initial laboratory results from the outlying emergency department, presented in the Table, were significant for hyperglycemia and elevated liver enzymes. In addition, arterial blood gas values revealed a mixed respiratory and metabolic acidosis with a pH of 7.12 (normal, 7.35-7.45); carbon dioxide, 55 mmHg (normal, 35-45 mmHg); oxygen, 160 mmHg (normal, 80-100 mmHg); bicarbonate, 17 mEq/L (normal, 21-24 mEq/L); base deficit, 12 mmol/L (normal, 1 to +1); and oxygen saturation, 98.1%. PICU COURSE An initial diagnosis of prolonged QT syndrome was made given the initial rhythm noted by emergency medical services, although her corrected QT interval was within normal limits on subsequent
TABLE. Laboratory data Parameter
Patient’s value
Normal value
White blood cell count Hemoglobin Hematocrit Platelets Sodium Potassium Chloride Carbon dioxide Blood urea nitrogen Creatinine Glucose Total calcium Ionized calcium Magnesium Phosphorus Aspartate aminotransferase Alanine aminotransferase Alkaline phosphatase Total bilirubin Total protein Albumin
17,800/mL 14 g/dL 40.7% 212,000/mL 142 mEq/L 3.8 mEq/L 109 mg/dL 23 mEq/L 13 mg/dL 0.9 mg/dL 241 mg/dL 7.3 mg/dL 1.19 mmol/L 1.9 mEq/L 5.1 mg/dL 338 U/L 315 U/L 298 U/L 0.3 mg/dL 6 mg/dL 3.7 mg/dL
4,500-13,500/mL 12-16 g/dL 37-45% 150-350/mL 135-147 mEq/L 3.5-5.1 mEq/L 97-107 mEq/L 22-26 mEq/L 6-20 mg/dL 0.5-1.0 mg/dL 60-100 mg/dL 8.4-10.2 mg/dL 1.18-1.32 mmol/L 1.26-2.1 mg/dL 2.4-4.4 mg/dL 13-45 U/L 5-30 U/L 100-320 U/L <1.5 mg/dL 6.0-8.0 g/dL 3.6-5.2 g/dL
Note. Normal values are expressed in conventional units. Data from Tschudy, M. M., & Arcara, K. A. (2012). The Harriet Lane handbook (19th ed.). Philadelphia, PA: Elsevier.
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electrocardiograms (ECGs). The patient was scheduled to have surgery for an implantable cardioverter/defibrillator (AICD). Continuing evaluation of the arrest included an echocardiogram, a cardiac magnetic resonance imaging (MRI) scan, and a gated cardiac computed tomography (CT) scan, although none was diagnostic of the origin of the coronary arteries as a result of pulmonary hemorrhage from CPR. The patient was extubated on hospital day three, and the echocardiogram and gated cardiac CT scan were repeated the day before the scheduled AICD surgery. On both repeat diagnostic tests, findings included an
anomalous left coronary artery arising from the right coronary sinus (Figure 2) with a short intramural course, in which the coronary artery travels within the aortic wall and the ostium (orifice) has a slitlike appearance rather than the normal round appearance. The AICD placement was cancelled, and the patient was taken to the operating room for unroofing of the left coronary artery with reimplantation of the ostium into the left coronary sinus (Figure 3). The patient had no major postoperative complications and is awaiting her scheduled 3-month follow up with a stress test, echocardiogram, and coronary CT scan.
CASE STUDY QUESTIONS 1. 2. 3. 4.
What is the differential diagnosis of sudden cardiac arrest in the healthy adolescent athlete? What is the pathophysiology of an anomalous coronary artery arising from the opposite sinus of Valsalva? What is the appropriate diagnostic workup for a suspected anomalous coronary artery? What is the current management strategy and expected outcomes for patients with an anomalous coronary artery? 5. What are some primary and secondary strategies for prevention of sudden cardiac death from anomalous coronary arteries and other causes? CASE STUDY ANSWERS 1. What is the differential diagnosis of sudden cardiac arrest in the healthy adolescent athlete? The overwhelming majority of fatalities from sudden cardiac arrest in a healthy athlete occur as a result of cardiovascular causes, mainly structural defects (Maron, Doerer, Haas, Tierney, & Mueller, 2009). The prevailing cause of sudden cardiac death in young athletes is hypertrophic cardiomyopathy and ‘‘possible hypertrophic cardiomyopathy,’’ in which the heart has a noticeable increase in mass without the clinical features of hypertrophic cardiomyopathy. Other cardiovascular causes include myocarditis, aortic aneurysms, arrhythmogenic right ventricle, Wolf-Parkinson-White disorder, and long QT syndrome (Angelini, 2007; Berger, Kugler, Thomas & Friedberg, 2004). Among noncardiovascular causes of sudden cardiac death, blunt chest trauma precipitating commotio cordis is the most common phenomenon. This phenomenon occurs when a low-impact blow to the chest precipitates a life-threatening dysrhythmia—usually ventricular fibrillation. Commotio cordis is most commonly seen in the sports of baseball, softball, hockey, and lacrosse (Maron, Gohman, Kyle, Estes, & Link, 2002). 2. What is the pathophysiology of an anomalous coronary arising from the opposite sinus of Valsalva? The second most common cause of sudden cardiac arrest after hypertrophic cardiomyopathy is aberrant coronary arteries, with the anomalous left coronary www.jpedhc.org
artery arising from the right sinus of Valsalva as the primary lethal defect (Maron, 2003). Anomalous coronary artery from the opposite facing sinus of Valsalva (ACAOS) is a congenital heart defect in which a coronary artery arises from the opposite sinus and has an anomalous course (Angelini, Villason, Chan, & Diez, 1999). The two most common forms of ACAOS are anomalous left coronary artery arising from the right coronary sinus and anomalous right coronary artery arising from the left coronary sinus. Anomalous left coronary arteries are more commonly known to cause sudden cardiac death than their counterpart (Eckart, Jones, Shry, Garrett, & Scoville, 2006). Of those, both right and left, that cause sudden cardiac death, only approximately 37% to 52% of the patients were symptomatic in the months prior to their death (Basso, Maron, Corrado, & Thiene, 2000; Mainwaring et al., 2011). Symptoms of anomalous coronary arteries are a result of cardiac ischemia and include chest pain and syncope (Mainwaring et al., 2011). Some controversy exists with regard to the cause of ischemic symptoms in patients with anomalous coronary arteries. Initially, the common thought was that both the pulmonary artery and the aorta exerted pressure on the coronary artery in a hyperdynamic state (i.e., with exercise), causing poor perfusion to the distal portion of the vessel. This idea has lost favor, because the pulmonary artery does not create enough pressure, even during exercise, to create such an effect. Other theories include a narrowed ostium, an acute angulation of the artery at the point of entry at -/- 2014
3
FIGURE 2. Normal and anomalous left coronary artery from the right coronary cusp with intramural course. L = left coronary cusp; P = posterior non-coronary cusp; PA = pulmonary artery; R = right coronary cusp. This figure appears in color online at www.jpedhc.org.
the aorta, or coronary spasm from endothelial damage (Kaushal et al., 2011). Current opinion purports that the most likely cause of ischemia and related symp-
toms is the length of the anomalous artery that runs tangential to and shares a common wall with the aorta. The pressure of the aorta increases during exercise,
FIGURE 3. Unroofing and reimplantation of the left coronary artery. A, The normally positioned right coronary artery orifice and the anomalous left coronary artery orifice both arising from the right sinus and an intramural segment of the left coronary. B, The intramural segment of the coronary is unroofed to create a neo-orifice in the left sinus. C, The left coronary artery is reimplanted into a normal position. (Reprinted from Pediatric Clinics of North America, 51, Jaquiss, R.D.B., Tweddell, J.S., & Litwin, S.B., Surgical therapy for sudden cardiac death in children, 1394, Copyright (2004) with permission from Elsevier.) This figure appears in color online at www.jpedhc.org.
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which compresses the coronary artery and limits coronary blood flow. 3. What is the appropriate diagnostic workup for a suspected anomalous coronary artery? An anomalous coronary artery from the opposite sinus of Valsalva can result in a diagnostic challenge, as evidenced by this case study. A simple ECG and stress test are not sufficient and have been normal prior to sudden cardiac arrest in patients with ACAOS (Eckart et al., 2006). Current practice guidelines, including those from the American Heart Association (AHA; Bluemke et al., 2008), call for specific evaluation of anomalous coronary artery with any incidence of sudden cardiac death, life-threatening dysrhythmias, and ischemic symptoms in the pediatric and young adult population. To date, several algorithms have been suggested for the evaluation of anomalous coronary arteries, although none has been included in existing practice guidelines. Angelini (2007) suggested that the evaluation should include an ECG, Holter monitor, and echocardiogram with Doppler. If the echocardiogram identifies two normal coronary ostia, no further testing is required. If, however, the echocardiogram cannot decipher two normal ostia, an MRI or CT scan is indicated. A diagnosed anomalous coronary artery also requires a baseline stress test. Current opinions differ as to the optimal modality for diagnosis of ACAOS if the echocardiogram cannot clearly delineate coronary artery origins. Although coronary angiography was considered the gold standard in the past, a gated cardiac CT scan has been shown to identify coronary anatomy when angiography yielded equivocal results (Datta et al., 2005). A cardiac CT scan, although efficient, requires use of intravenous contrast material and radiation, although current techniques have considerably limited the radiation dose. For this reason, some authors strongly recommend coronary MRI/magnetic resonance angiography (MRA; Bluemke et al., 2008). However, coronary MRA requires a relatively stable patient and is liable to a certain amount of artifact (Arrigan, Killeen, Dodd, & Torreggiani, 2011). At this point, clinical judgment and patient condition dictate which modality must be used. 4. What is the current management strategy and expected outcomes for patients with an anomalous coronary artery? Several management options exist for the treatment of ACAOS. Both nonsurgical and surgical options may be considered, depending on which coronary artery is anomalous, if there is an intramural course, and presenting symptoms (Mainwaring et al., 2011). Although several surgical options are available for a patient with ACAOS, nonoperative management is now an opwww.jpedhc.org
tion for patients with an anomalous right coronary artery if certain criteria are met. A practice guideline from the AHA states that anomalous left coronary arteries must be operated on because of the risk of sudden death, whereas with right coronary arteries, surgery is indicated if there is an intramural course, if the artery courses between the aorta and the pulmonary artery, or if the patient has ischemic symptoms (Bluemke et al., 2008). Practice guidelines from the American College of Cardiology also recommend that all patients with unrepaired anomalous coronary arteries should be totally restricted from participating in competitive sports (Graham et al., 2005). Many options are available to the pediatric cardiothoracic surgeon for operative repair of ACAOS; these options include unroofing of the intramural segment, in which the shared common wall between the ACAOS and aorta is excised, reimplantation of the anomalous coronary artery, and coronary artery bypass grafting with use of the saphenous or internal mammary artery (Figure 3). Although an explanation of surgical options is beyond the scope of this paper, it should be noted that the two techniques most frequently utilized include unroofing and reimplantation. Individual anatomy plays a large role in the selection of the preferred surgical correction. Bypass grafting has been deemed undesirable because of graft atrophy from competitive flow (Warnes et al., 2008). Whereas the diagnosis of ACAOS has been associated with adverse outcomes such as sudden cardiac death, the primary surgery to repair this defect has very few associated complications. Authors of all but one study found no complicaWhereas the tions/minimal complications or residual diagnosis of coronary ischemia in ACAOS has been the corrected anomaassociated with lous coronary artery by unroofing of the adverse outcomes intramural segment such as sudden (Frommelt, Frommelt, cardiac death, the Tweddell, & Jaquiss, 2003; Romp et al., primary surgery to 2003). Authors of one repair this defect study found no has very few residual symptoms, and 85% of patients associated were unrestricted in complications. activities, with a 63% participation rate in sports at 5-year follow-up (Mainwaring et al., 2011). Authors of only one study found evidence of ischemia in 9 of 24 patients postoperatively, utilizing three stress test modalities to evaluate for ischemia (Brothers et al., 2007). All of these patients were asymptomatic. Current practice guidelines for adults recommend a -/- 2014
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3-month waiting period after a full surgical repair before return to sports if the patient is asymptomatic and has no evidence of ischemia (Warnes et al., 2008). To date, no studies have been performed to examine the outcomes of a conservative approach to asymptomatic anomalous right coronary artery with no intramural course. 5. What are some primary and secondary strategies for prevention of sudden cardiac death from anomalous coronary arteries and other causes? Prevention of sudden cardiac death is at the crux of a debate involving appropriate athlete screening in the United States and internationally. The American Academy of Pediatrics (AAP) released a policy statement in 2012 regarding sudden cardiac arrest. Primary prevention of sudden cardiac arrest includes appropriate diagnosis and treatment of the common causes responsible for the arrest. Primary prevention includes preparticipation sports physicals by the athlete’s primary care physician with use of a formal screening tool in conjunction with a thorough cardiovascular examination. The utility of a screening ECG in the prevention of sudden cardiac arrest is addressed in the policy statement. Although screening ECGs are endorsed by the European Society of Cardiology and refuted by the AHA, the AAP (2012) does not take a stance, stating only that the issue requires ‘‘more data and debate.’’ Secondary prevention of sudden cardiac arrest includes early CPR combined with early defibrillation using an automated external defibrillator. The AHA and the AAP both advocate for emergency response preparedness and automated external defibrillator programs in schools and sports facilities. Anomalous coronary artery from the opposite sinus of Valsalva is a rare but serious congenital heart defect. Presentation can be dramatic, frequently with sudden cardiac death, and diagnosis continues to be a challenge for practitioners, as evidenced by the case presentation. Appropriate nonsurgical and surgical management lead to a favorable prognosis with few complications. REFERENCES American Academy of Pediatrics, Section on Cardiology and Cardiac Surgery. (2012). Pediatric sudden cardiac arrest. Pediatrics, 129, e1094. Angelini, P. (2007). Coronary artery anomalies: An entity in search of an identity. Circulation, 115, 1296-1305. Angelini, P., Villason, S., Chan, A., & Diez, J. (1999). Normal and anomalous coronary arteries in humans. Philadelphia, PA: Lippincott Williams & Wilkins. Arrigan, M., Killeen, R., Dodd, J., & Torreggiani, W. (2011). Imaging spectrum of sudden athlete cardiac death. Clinical Radiology, 66, 203-223.
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Basso, C., Maron, B., Corrado, D., & Thiene, G. (2000). Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. Journal of the American College of Cardiology, 36(6), 1493-1501. Berger, S., Kugler, J. D., Thomas, J. A., & Friedberg, D. Z. (2004). Sudden cardiac death in children and adolescents: Introduction and overview. Pediatric Clinics of North America, 51(5), 1201-1209. Bluemke, D., Achenbach, S., Budoff, M., Gerber, T., Gersh, B., Hillis, D., . Woodard, P. K. (2008). Noninvasive coronary artery imaging: Magnetic resonance angiography and multidetector computed tomography angiography: A scientific statement from the American Heart Association Committee on Cardiovascular Imaging and Intervention of the Council on Cardiovascular Radiology and Intervention, and the Councils on Clinical Cardiology and Cardiovascular Disease in the Young. Circulation, 118(5), 586-606. Brothers, J., McBride, M., Seliem, M., Marino, B., Tomlinson, R., Pampaloni, M. H., . Paridon, S. M. (2007). Evaluation of myocardial ischemia after surgical repair of anomalous aortic origin of a coronary artery in a series of pediatric patients. Journal of the American College of Cardiology, 50, 2078-2082. Datta, J., White, C., Gilkeson, R., Meyer, C., Kansal, S., Jani, M., . Read, K. (2005). Anomalous coronary arteries in adults: Depiction at multi-detector row CT angiography. Radiology, 235, 812-818. Eckart, R., Jones, S., Shry, E., Garrett, P., & Scoville, S. (2006). Sudden death associated with anomalous coronary origin and obstructive coronary disease in the young. Cardiology in Review, 14, 161-163. Frommelt, P., Frommelt, M., Tweddell, J., & Jaquiss, R. (2003). Prospective echocardiographic diagnosis and surgical repair of anomalous origin of a coronary artery from the opposite sinus with an interarterial course. Journal of the American College of Cardiology, 42, 148-154. Graham, T., Driscoll, D., Gersony, W., Newberger, J., Rocchini, A., & Towbin, J. (2005). Task force 2: Congenital heart disease. Journal of the American College of Cardiology, 45(8), 1326-1333. Kaushal, S., Backer, C., Popescu, A., Walker, B., Russell, H., Koenig, P., . Mavroudis, C. (2011). Intramural coronary length correlates with symptoms in patients with anomalous aortic origin of the coronary artery. Annals of Thoracic Surgery, 92, 986-992. Mainwaring, R., Reddy, M., Reinhartz, O., Petrossian, E., MacDonald, M., Nasirov, T., . Hanley, F. L. (2011). Anomalous aortic origin of a coronary artery: Medium-term results after surgical repair in 50 patients. Annals of Thoracic Surgery, 92, 691-697. Maron, B. (2003). Sudden death in young athletes. New England Journal of Medicine, 349, 1064-1075. Maron, B., Gohman, T., Kyle, S., Estes, N., & Link, M. (2002). Clinical profile and spectrum of commotio cordis. Journal of the American Medical Association, 287, 1142-1146. Maron, B., Doerer, J., Haas, T., Tierney, D., & Mueller, F. (2009). Sudden deaths in young competitive athletes: Analysis of 1866 deaths in the United States, 1980-2006. Circulation, 119, 1085-1092. Romp, R., Herlong, J. R., Landolfo, C., Sanders, S., Miller, C., . Jaggers, J. (2003). Outcome of unroofing procedure for repair of anomalous aortic origin of left or right coronary artery. Annals of Thoracic Surgery, 76, 589-596. Warnes, C., Williams, R., Bashore, T., Child, J., Connolly, H., Dearani, J. A., . Yancy, C. W. (2008). ACC/AHA 2008 Guidelines for the management of adults with congenital heart disease. Circulation, 118, e714-e833.
Journal of Pediatric Health Care