Heart & Lung 43 (2014) 476e480

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Case Studies in Critical Care

Surviving sudden cardiac death secondary to anomalous left coronary artery from the pulmonary artery: A case report and literature review Osama Alsara, MD a, *, Jagadeesh K. Kalavakunta, MD b, Victor Hajjar, MD b, Ahmad Alsarah, MD a, Nam Cho, DO b, Gaurav Dhar, MD b a b

Department of Internal Medicine, Division of General Internal Medicine, Michigan State University, East Lansing, MI, USA Department of Internal Medicine, Division of Cardiovascular Diseases, Michigan State University, East Lansing, MI, USA

a r t i c l e i n f o

a b s t r a c t

Article history: Received 1 March 2014 Received in revised form 4 June 2014 Accepted 4 June 2014 Available online 8 July 2014

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon type of congenital coronary anomaly. In adults, the clinical picture of ALCAPA varies widely between being asymptomatic, presenting similarly to other heart diseases, or as malignant arrhythmias. Patients who have cardiac arrest are usually young and do not exhibit prior warning symptoms. In this article we describe a case of a 22-year-old healthy male, who experienced cardiac arrest during exertion. He was then diagnosed with ALCAPA, which was surgically corrected. Ó 2014 Elsevier Inc. All rights reserved.

Keywords: Sudden cardiac death Adult Congenital coronary anomaly Resuscitation

Introduction Coronary artery anomalies are congenital abnormalities in number, origin, course, or termination of the coronary arteries. These conditions are found in about 1% of the population.1,2 Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly associated with high mortality in infants, and is an uncommon diagnosis in adults. Most cases of ALCAPA in adulthood are diagnosed incidentally or after complaints of angina, dyspnea, palpitations, fatigue, syncope, arrhythmia, or cardiac arrest. In this report, we describe the case of a 22-year-old male who survived cardiac arrest secondary to undetected ALCAPA. Case presentation A 22-year-old Caucasian male was brought to the emergency department (ED) after an episode of cardiac arrest. He was seen Abbreviations: ALCAPA, anomalous origin of the left coronary artery from the pulmonary; ICD, implantable cardioverter defibrillator; VF, ventricular fibrillation; LVEF, left ventricular ejection fraction. Disclosures: Drs. Alsara, Kalavakunta, Hajjar, Alsarah, and Cho have no relationships to disclose. Dr. Dhar is a speaker for Astra Zeneca. * Corresponding author. 788 Service Road, Room B301, East Lansing, MI 48824, USA. Tel.: þ1 517 353 5100; fax: þ1 517 432 2759. E-mail addresses: [email protected], [email protected] (O. Alsara). 0147-9563/$ e see front matter Ó 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.hrtlng.2014.06.048

riding his bike when he suddenly collapsed, became unresponsive, and pulseless. Cardiopulmonary resuscitation was started immediately by a witness and emergency medical services were contacted. When paramedics arrived 5 min later, he was found to be in ventricular fibrillation (VF). After defibrillation at the scene, the patient returned to sinus rhythm and was transported to the hospital. Upon arrival to ED, he was unconscious, hemodynamically stable, however ventricular fibrillation was again noted and was converted to sinus rhythm by electrical defibrillation. Subsequently, he was intubated and started on an amiodarone drip as well as a dopamine drip. Further history provided by his mother was significant for a cardiac murmur during his childhood, which was evaluated at that time, however, no heart disease was detected. She reported that he occasionally drinks alcohol, but denied any history of smoking, drugs use, or taking medications. Family history of heart diseases or sudden cardiac arrest was negative as well. Electrocardiogram (ECG) in the ED showed ST elevation in the inferior and lateral precordial leads and ST depression in the anterior precordial leads (Fig. 1). Therefore, the patient was taken to cardiac catheterization laboratory emergently. During the coronary angiography, the left coronary system was unengageable, and a left ventriculogram showed a mildly reduced left ventricular ejection fraction (LVEF) at 40%. When a right coronary angiography was performed, it revealed a dominant right coronary along with large conus branch supplying extensive right-to-left collaterals to the left

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Fig. 1. 12 lead electrocardiogram showing the ST elevation in the inferior and lateral precordial leads along with ST depression in the anterior precordial leads.

coronary artery suggestive of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) (Fig. 2A, B). During cardiac catheterization, hypothermia protocol was initiated, and a brain magnetic resonance imaging (MRI) was ordered that suggested an anoxic brain injury. However, primary neurological evaluations in the intensive care unit (ICU) predicted a good prognosis. The patient started to be more responsive and

followed commands by the third day in ICU, but his recovery was complicated by aspiration pneumonia which was treated with IV Vancomycin and PiperacillineTazobactam. When he was extubated 7 days later, he was fully oriented, exhibited mild memory difficulties, and required minimal assistance for walking. While in the ICU, additional tests were performed in order to confirm the diagnosis of ALCAPA including a two dimensional (2D)

Fig. 2. A) Right coronary angiogram showing large right coronary artery (RCA) viewed in both left and right anterior oblique (LAO and RAO) projections. B) Right coronary angiogram showing extensive collaterals from right coronary artery (RCA) and conus branch to the left coronary arterial system (LCA). PA: pulmonary artery.

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Fig. 3. Parasternal short axis view at aortic level showing the dilated right coronary ostium (arrow) with color flow. AO: aorta, LA: left atrium, RA: right atrium, RCA: right coronary artery, RV: right ventricle. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)

echocardiogram which demonstrated global hypokinesis with LVEF of 35%, a mildly dilated left and right ventricles, as well as trace tricuspid regurgitation. In the parasternal short axis aortic view, dilated ostium of the right coronary artery was noted (Fig. 3). A computerized tomography (CT) coronary angiogram was performed which confirmed an anomalous left coronary artery arising from the main pulmonary artery, with multiple collaterals from the dilated right coronary (Fig. 4). Treatments for ALCAPA, including surgery, implantable cardioverter defibrillator (ICD), and anti-arrhythmic drugs were discussed with the patient and his family. A decision was made to switch his medications to oral amiodarone, and to utilize a wearable cardioverter defibrillator until corrective surgery could be performed. Patient was then transferred to the inpatient rehabilitation unit to achieve better recovery after spending 12 days in the ICU.

Fig. 4. Computed tomography coronary angiogram showing the origin of left coronary artery (arrow) from the pulmonary artery (PA). AO: aorta.

Corrective surgery was performed twenty days after the cardiac arrest, where the anomalous left coronary artery was detached and re-implanted into the ascending aorta without complications. Moreover, his postsurgical course was uneventful and unremarkable. A 2D echocardiogram was repeated which demonstrated an anterograde flow in the left coronary artery, normal left ventricular size, and improved LVEF of 60%. Neither ICD implantation nor prolonged antiarrhythmic medications were used. At his follow up four months after surgery he was asymptomatic and in reasonably good health. Discussion ALCAPA is a congenital coronary anomaly reported in about 1/ 300 000 live births.3 The first clinical description of this syndrome was by Bland, White, and Garland in 1933.4 It is usually found either as an isolated anomaly or in combination with other coronary defects. Embryologically, it occurs as a result of either a defective septation of the conotruncus into the aorta and pulmonary artery or due to the combination of persistence of the pulmonary buds and faulty involution of the aortic buds. At birth, ALCAPA usually is well tolerated, due to the elevated pressure in pulmonary artery in newborns, which provides an anterograde perfusion of myocardium through the left coronary artery. When pulmonary artery pressure drops so does the pulmonary vascular resistance in the first months of life, blood flow reverses from the left coronary artery to pulmonary artery, leading to a steal phenomenon. As a result of inadequate perfusion of the myocardium, infants with ALCAPA, develop symptoms of myocardial ischemia, heart failure, and mitral regurgitation.5 Without treatment, these complications are life threatening and only 10% of those patients, who are fortunate enough to have a sufficient collateral circulation from the right coronary artery to the left coronary circulation, survive beyond infancy.6 In 14% of reported cases in adults, ALCAPA was asymptomatic and diagnosed either incidentally or by autopsy.7 However, ALCAPA typically presents with symptoms of angina, dyspnea, palpitations, or fatigue. Life threatening arrhythmias, syncope and sudden cardiac death were reported in about 17% of cases due to the

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Table 1 Review of reported cases of patients who presented with cardiac arrest secondary to anomalous origin of the left coronary artery from the pulmonary artery and were successfully resuscitated. Author (year)

Age/gender

Previous complaints

Initial rhythm

Surgical treatment

ICD

F/U

Complications

Viskin et al8 (1992) Kreutzer et al9 (1998)

26 Y/F 47 Y/F

VF VF

Ligation of LCA and CABG Ligation of LCA and CABG

No No

17 m 14 m

No No

Weiss et al10 (1999)

31 Y/M

Cardiac murmur at birth Progressive dyspnea, and MR since childhood. No

?

No

?

?

Frapier et al11 (1999)

38 Y/F

No

VT/VF

No

13 m

No

Frapier et al11 (1999) Kristensen et al12 2008

47 Y/F 39 F

VT VF

No Yes

11 m ?

No ?

Aslanger et al13 (2009) Parizek et al14 (2010) Parizek et al14 (2010) Sahin et al15 (2012)

22 23 33 18

Ventricular premature beats Mild impaired left ventricular function at childhood thought to be due to endocardial fibroelastosis. No No No No

Re-implantation of the anomalous left coronary artery Closure of the left main and mammary artery anastomosis to LAD Direct aortic reimplantation Aortic re-implantation of the left coronary artery.

No Yes No Yes

6m 10.5 Y 4.5 Y ?

No No No ?

Safaa et al16 (2012)

43 Y/F

No

?

Yes

1m

No

30 Y/M

No

VF

Yes

6m

No

Jujjavarapu et al

17

(2013)

Y/F Y/M Y/F Y/M

VF WCT/VF VF ?

Ligation of LCA and CABG Ligation LCA and CABG Ligation LCA and CABG LAD was detached from the pulmonary artery and connected to the aorta, and the left internal mammarian artery was anastomosed to the LAD LCA ligation and CABG using a saphenous vein graft Off-pump total arterial revascularization and ligation of the left main stem of anomalous origin.

Y: year(s); m: months; F/U: follow up; ICD: implantable cardioverter defibrillator; F: female; VF: ventricular fibrillation; VT: ventricular tachycardia; WCT: wide complex tachycardia; LAD: left anterior descending; LCA: left coronary artery; CABG: coronary artery bypass graft; MR: mitral regurgitation. The reports of Kreutzer et al and Weiss et al are in German.

inadequate perfusion which triggers arrhythmias either during an acute ischemic event or by producing a scar tissue that works as an arrhythmogenic center.7 We are aware of twelve cases of adult patients (4 males and 8 females) who were successfully resuscitated of cardiac arrest secondary to ALCAPA. All of them were young (ages ranged between 18 and 47 years, average age was 33.1) and presented mostly with cardiac arrest as the first symptom of this anomaly (Table 1).8e17 Askanazi et al reported on a case of an 18year-old girl with a known symptomatic ALCAPA who died a few hours after suffering a cardiac arrest during exertion.18 Historically, coronary angiogram, 2D echocardiogram with color Doppler, or autopsy was used to establish the diagnosis of ALCAPA. CT angiogram and MRI have emerged recently as noninvasive tools that provide a direct visualization of the vessels’ anatomy and allows a more comprehensive evaluation of the communication site between the left coronary artery and the pulmonary artery.5 Surgery is the gold standard for the treatment of ALCAPA, and has shown excellent results in infants including a survival rate of about 95% after 20-years following surgery.19 The available data on ALCAPA in adult patients who survived sudden cardiac death and treated surgically seem promising as well (Table 1). The use of ICD to prevent sudden cardiac arrest in these patients is still controversial, but well acceptable in patients with high risk of developing VF such as patients with ischemic cardiomyopathy and low ejection fraction.14 Although prognosis of ALCAPA in adults is considered to be good after treatment, no randomized control trials are yet available to evaluate long-term outcomes of different managements. In conclusion, anomalous origin of the left coronary artery from the pulmonary artery is one of the rare coronary anomalies reported in adults. Moreover, it is associated with an increased risk of malignant arrhythmias due to inadequate myocardial perfusion. It is important to consider ALCAPA in our differential diagnosis of cardiac arrest in healthy adults in order to provide proper management.

References 1. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn. 1990;21:28e40. 2. Rigatelli G, Docali G, Rossi P, Bandello A, Rigatelli G. Validation of a clinicalsignificance-based classification of coronary artery anomalies. Angiology. 2005;56:25e34. 3. Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg. 2002;74:946e955. 4. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J. 1933;8:p787e801. 5. Peña E, Nguyen ET, Merchant N, Dennie G. ALCAPA syndrome: not just a pediatric disease. Radiographics. 2009;29(2):553e565. 6. Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation. 1968;38:403e425. 7. Yau JM, Singh R, Halpern EJ, Fischman D. Anomalous origin of the left coronary artery from the pulmonary artery in adults: a comprehensive review of 151 adult cases and a new diagnosis in a 53-year-old woman. Clin Cardiol. 2011;34(4):204e210. 8. Viskin S, Belhassen B, Paredes A, Yakirevich V, Miller H, Laniado S. Anomalous origin of the left coronary artery from the pulmonary artery: report of an adult with ventricular fibrillation as the presenting symptom. Am Heart J. 1992;124: 221e222. 9. Kreutzer U, Krulls-Munch J, Angres M, Schiessler A. Successful resuscitation of a patient with ventricular fibrillation in Bland-White-Garland syndrome in adulthood. A case report. Z Kardiol. 1998;87:560e565. 10. Weiss C, Willems S, Hofmann T, Rickers C, Meinertz T. Resuscitation in ventricular fibrillation as the first manifestation of Bland-White-Garland syndrome in adulthood. Med Klin. 1997;15:447e451. 11. Frapier JM, Leclercq F, Bodino M, Chaptal PA. Malignant ventricular arrhythmias revealing anomalous origin of the left coronary artery from the pulmonary artery in two adults. Eur J Cardiothorac Surg. 1999;15(4):539e541. 12. Kristensen T, Kofoed KF, Helqvist S, Helvind M, Søndergaard L. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report. J Cardiothorac Surg. 2008;26(3):33. 13. Aslanger E, Altun I, Umman B. Sudden cardiac arrest in a patient with an anomalous left main coronary artery originating from the pulmonary artery. Acta Cardiol. 2009;64(6):835e837.

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14. Parizek P, Haman L, Harrer J, et al. Bland-White-Garland syndrome in adults: sudden cardiac death as a first symptom and long-term follow-up after successful resuscitation and surgery. Europace. 2010;12(9):1338e1340. 15. Sahin T, Bozyel S, Acar E, et al. A young patient with coronary artery anomaly, whose left anterior descending artery originated from the pulmonary artery, underwent cardiac arrest. Cardiovasc J Afr. 2012;23(8):e15ee18. 16. Safaa AM, Du LL, Batra R, Essack N. A rare case of adult type ALCAPA syndrome: presentation, diagnosis and management. Heart Lung Circ. 2013;22(6):444e446.

17. Jujjavarapu K, Salhiyyah K, Witzke H, Amrani M. Off-pump total arterial revascularization for anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg. 2013;96:2234e2236. 18. Askenazi J, Nadas AS. Anomalous left coronary artery originating from the pulmonary artery. Report of 15 cases. Circulation. 1975;51:976e987. 19. Lange R, Vogt M, Hörer J, et al. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg. 2007;83(4):1463e1471.

Surviving sudden cardiac death secondary to anomalous left coronary artery from the pulmonary artery: a case report and literature review.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon type of congenital coronary anomaly. In adults, the cli...
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