562969 research-article2014
ACC0010.1177/2048872614562969European Heart Journal: Acute Cardiovascular CarePachon et al.
EUROPEAN SOCIETY OF CARDIOLOGY ®
Clinical practice/education
Sudden cardiac death as a presentation of anomalous origin of the left coronary artery from pulmonary artery in a young adult
European Heart Journal: Acute Cardiovascular Care 1–2 © The European Society of Cardiology 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/2048872614562969 acc.sagepub.com
Ronald Pachon1, Claudio Bravo2 and Mark Niemiera3
Abstract Sudden cardiac death in 5–10% of cases is explained by patients with congenital abnormalities that include coronary artery malformations such as anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). We report a case of sudden cardiac death as the first presentation of ALCAPA in a young female with no history of hypertrophic cardiomyopathy. Keywords Sudden cardiac death, syncope, ALCAPA, exercise intolerance, chest pain, dyspnea, congenital anomaly Date received: 22 September 2014; accepted: 16 November 2014
Clinical case An 18-year-old apparently healthy and physically active female, who was last seen competitively running 4 min prior, was found to be unresponsive. A paramedic at the site, who found her pulseless, performed cardiopulmonary resuscitation (CPR) until the emergency medical services (EMS) team cardioverted and intubated her on arrival 5 min later. In the emergency department vital signs revealed: blood pressure: 126/100 mmHg; heart rate: 172 beats/min; respiratory rate: 20/min; T: 98.7°F. Her family reported a flu-like illness one week prior to admission but denied any episodes of cyanosis, palpitation, joint swelling or skin lesions in the remote past. They denied any history of trauma, smoking, drugs, alcohol, medication use or any family history of sudden cardiac death. On examination the patient was intubated and appeared pale without cyanosis, jugular venous distention or carotid bruit; she had regular heart sounds with no murmur, rub or gallop. The rest of the physical examination was unremarkable. Electrocardiogram (ECG) showed sinus tachycardia with Q waves in leads I, aVL, V1–V3. Chest X-ray showed no evidence of pneumothorax, enlarged mediastinum or pleural effusion. Chest computed tomography (CT) angiogram did not reveal any evidence of pulmonary embolism. Head CT
scan showed no evidence of hemorrhage. Urine drug and pregnancy screens were negative. Complete blood count, metabolic panel and complement levels were within normal limits while serial troponin I (0.36, 7.39 ng/ml) and creatinine kinase MB (5.69, 216 units/l) were elevated. Echocardiography showed severely reduced ejection fraction (20%) without any evidence of significant valvular or hypertrophic heart disease. She was immediately transferred to a tertiary center for left heart catheterization, which showed anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)1–3 (see Figure 1). Figure 1(a) represents the early injection phase 1Cardiovascular
Research Institute, Department of Cell Biology and Molecular Medicine, Rutgers-New Jersey Medical School, Newark, USA 2Internal Medicine Department, Bridgeport Hospital/Yale New Haven Health System, Bridgeport, USA 3Raritan Bay Medical Center, Perth Amboy, USA Corresponding author: Ronald Pachon, Cardiovascular Research Institute, NJ Medical School, Rutgers University, Medical Science Building, Room G669, 185 South Orange Avenue, Newark, NJ 07101-1709, USA. Email: [email protected]
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2
European Heart Journal: Acute Cardiovascular Care
Figure 1. Left cardiac catheterization: (a) early injection phase, (b) late injection phase. Large ectatic right coronary artery (RCA) with collateral to the left system with origin of the left system from the pulmonary artery. There is a right coronary branch (Bx) that gives a collateral to the proximal left anterior descending coronary artery (LAD). No significant obstructive coronary disease. No left circulation is found from the aorta or coronary cusps. LM: left main coronary artery
showing a large ectatic right coronary artery (RCA) with a branch (Bx) that gives a collateral to the proximal left anterior descending coronary artery (LAD). Figure 1(b) represents the late injection phase with multiple collaterals to the left system with origin of the left system from the pulmonary artery. Unfortunately the patient had irreversible brain damage due to anoxic encephalopathy and no further intervention was pursued. The patient had collateral circulation from her RCA to LAD, which likely enabled her to be physically active without any symptoms until the day of presentation.
Conclusion This patient illustrates the need for early consideration and recognition of ALCAPA in an otherwise healthy young adult individual who presents with sudden cardiac death.
Conflict of interest The authors declare that there is no conflict of interest.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Chugh SS, Jui J, Gunson K, et al. Current burden of sudden cardiac death: Multiple source surveillance versus retrospective death certificate-based review in a large U.S. community. J Am Coll Cardiol 2004; 44: 1268–1275. 2. Huikuri HV, Castellanos A and Myerburg RJ. Sudden death due to cardiac arrhythmias. N Eng J Med 2001; 345: 1473–1482. 3. Chugh SS, Kelly KL and Titus JL. Sudden cardiac death with apparently normal heart. Circulation 2000; 102: 649–654.
Downloaded from acc.sagepub.com at VITERBO UNIV on January 16, 2015
ACC0010.1177/2048872614562969European Heart Journal: Acute Cardiovascular CarePachon et al.
EUROPEAN SOCIETY OF CARDIOLOGY ®
Clinical practice/education
Sudden cardiac death as a presentation of anomalous origin of the left coronary artery from pulmonary artery in a young adult
European Heart Journal: Acute Cardiovascular Care 1–2 © The European Society of Cardiology 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/2048872614562969 acc.sagepub.com
Ronald Pachon1, Claudio Bravo2 and Mark Niemiera3
Abstract Sudden cardiac death in 5–10% of cases is explained by patients with congenital abnormalities that include coronary artery malformations such as anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). We report a case of sudden cardiac death as the first presentation of ALCAPA in a young female with no history of hypertrophic cardiomyopathy. Keywords Sudden cardiac death, syncope, ALCAPA, exercise intolerance, chest pain, dyspnea, congenital anomaly Date received: 22 September 2014; accepted: 16 November 2014
Clinical case An 18-year-old apparently healthy and physically active female, who was last seen competitively running 4 min prior, was found to be unresponsive. A paramedic at the site, who found her pulseless, performed cardiopulmonary resuscitation (CPR) until the emergency medical services (EMS) team cardioverted and intubated her on arrival 5 min later. In the emergency department vital signs revealed: blood pressure: 126/100 mmHg; heart rate: 172 beats/min; respiratory rate: 20/min; T: 98.7°F. Her family reported a flu-like illness one week prior to admission but denied any episodes of cyanosis, palpitation, joint swelling or skin lesions in the remote past. They denied any history of trauma, smoking, drugs, alcohol, medication use or any family history of sudden cardiac death. On examination the patient was intubated and appeared pale without cyanosis, jugular venous distention or carotid bruit; she had regular heart sounds with no murmur, rub or gallop. The rest of the physical examination was unremarkable. Electrocardiogram (ECG) showed sinus tachycardia with Q waves in leads I, aVL, V1–V3. Chest X-ray showed no evidence of pneumothorax, enlarged mediastinum or pleural effusion. Chest computed tomography (CT) angiogram did not reveal any evidence of pulmonary embolism. Head CT
scan showed no evidence of hemorrhage. Urine drug and pregnancy screens were negative. Complete blood count, metabolic panel and complement levels were within normal limits while serial troponin I (0.36, 7.39 ng/ml) and creatinine kinase MB (5.69, 216 units/l) were elevated. Echocardiography showed severely reduced ejection fraction (20%) without any evidence of significant valvular or hypertrophic heart disease. She was immediately transferred to a tertiary center for left heart catheterization, which showed anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)1–3 (see Figure 1). Figure 1(a) represents the early injection phase 1Cardiovascular
Research Institute, Department of Cell Biology and Molecular Medicine, Rutgers-New Jersey Medical School, Newark, USA 2Internal Medicine Department, Bridgeport Hospital/Yale New Haven Health System, Bridgeport, USA 3Raritan Bay Medical Center, Perth Amboy, USA Corresponding author: Ronald Pachon, Cardiovascular Research Institute, NJ Medical School, Rutgers University, Medical Science Building, Room G669, 185 South Orange Avenue, Newark, NJ 07101-1709, USA. Email: [email protected]
Downloaded from acc.sagepub.com at VITERBO UNIV on January 16, 2015
2
European Heart Journal: Acute Cardiovascular Care
Figure 1. Left cardiac catheterization: (a) early injection phase, (b) late injection phase. Large ectatic right coronary artery (RCA) with collateral to the left system with origin of the left system from the pulmonary artery. There is a right coronary branch (Bx) that gives a collateral to the proximal left anterior descending coronary artery (LAD). No significant obstructive coronary disease. No left circulation is found from the aorta or coronary cusps. LM: left main coronary artery
showing a large ectatic right coronary artery (RCA) with a branch (Bx) that gives a collateral to the proximal left anterior descending coronary artery (LAD). Figure 1(b) represents the late injection phase with multiple collaterals to the left system with origin of the left system from the pulmonary artery. Unfortunately the patient had irreversible brain damage due to anoxic encephalopathy and no further intervention was pursued. The patient had collateral circulation from her RCA to LAD, which likely enabled her to be physically active without any symptoms until the day of presentation.
Conclusion This patient illustrates the need for early consideration and recognition of ALCAPA in an otherwise healthy young adult individual who presents with sudden cardiac death.
Conflict of interest The authors declare that there is no conflict of interest.
Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Chugh SS, Jui J, Gunson K, et al. Current burden of sudden cardiac death: Multiple source surveillance versus retrospective death certificate-based review in a large U.S. community. J Am Coll Cardiol 2004; 44: 1268–1275. 2. Huikuri HV, Castellanos A and Myerburg RJ. Sudden death due to cardiac arrhythmias. N Eng J Med 2001; 345: 1473–1482. 3. Chugh SS, Kelly KL and Titus JL. Sudden cardiac death with apparently normal heart. Circulation 2000; 102: 649–654.
Downloaded from acc.sagepub.com at VITERBO UNIV on January 16, 2015
