Cardiology in the Young (2014), 24, 935–937
© Cambridge University Press, 2013
doi:10.1017/S1047951113001510
Brief Report Anomalous right coronary artery from the pulmonary artery (ARCAPA), and aberrant right subclavian artery in a 2-month infant with heart failure Mohammad Mahdavi,1 Koorosh Vahidshahi,1 Ramin Baghai Tehrani,2 Hamidreza Poor Ali-Akbar,3 Mohammad Rad Godarzi,4 Department of Pediatric Cardiology; 2Department of Cardiovascular Surgery; 3Department of Radiology, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran; 4Department of Pediatrics, Golestan University of Medical Science, Golestan, Iran 1
Abstract Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital heart anomaly. Most of the cases are asymptomatic during infancy and childhood. We report ARCAPA associated with aberrant right subclavian artery in a 2-month male infant presenting with heart failure. We used computed tomography angiography for confirming the diagnosis and also for post-operative follow-up. Keyword: Anomalous origin of the right coronary artery from the pulmonary artery; infant; heart failure Received: 01 August 2013; Accepted: 28 August 2013; First published online: 29 October 2013
A
NOMALOUS ORIGIN OF THE RIGHT CORONARY
artery from the pulmonary artery is a very rare congenital heart anomaly. Most of the cases are asymptomatic during infancy and childhood, and thus the anomaly is more frequently detected in late childhood, adolescence, and adulthood.1 We report ARCAPA and aberrant right subclavian in a 2-month male infant.
The case A 7-week-old male infant was referred to our centre because of heart failure. Manifestations of the patient had begun gradually since the third week of life with intermittent poor feeding, and restlessness. After an ambulatory visit, the patient was admitted in another centre because of increasing clinical manifestations at the age of 5 weeks. Sepsis work-up and relevant evaluations were performed, and the patient was finally referred to our centre for evaluation and management of heart failure at 7 weeks of age. Correspondence to: Dr M. Mahdavi, Department of Peadiatric Cardiology, Rajaie Cardiovascular Medical and Research Center, Vali-asr Ave., Niyayesh Blvd., 199691-1151 Tehran, Iran. E-mail: [email protected]
The medical history was uneventful, with a normal vaginal delivery. At presentation to our centre, the patient was underweight, with clinical manifestations of heart failure. Chest X-ray demonstrated increased cardio-thoracic ratio and pulmonary vascular marking. Echocardiography revealed enlargement of the left atrium, left and right ventricles, moderate tricuspid regurgitation, relatively decreased ejection fraction (40%), and an abnormal diastolic flow in the main pulmonary artery. Computed tomographic angiography revealed abnormal origin of the right coronary artery from the pulmonary artery, also aberrant subclavian artery (Fig 1). Digoxin, captopril and furosemide were initiated for the patient; corrective surgery was also planned. Surgical operation was performed on the seventh day of admission, using direct reimplantation of right coronary artery on the aorta. The post-operative course was uneventful. Computed tomographic angiography after operation revealed normal origin of the right coronary artery from the aorta and no kinking (Fig 2); in echocardiography, the ejection fraction was increased. The patient was discharged from the hospital on the eighth day after surgery with good general condition, good feeding, and no respiratory distress.
936
Cardiology in the Young
Figure 1. Computed tomographic angiography before corrective surgery.
October 2014
There are few reports of ARCAPA diagnosed at infancy.6–9 Except for a 6-week infant with ARCAPA,10 our patient is one of the youngest diagnosed case of the anomaly. Heart failure may be a feature of presentation, as in our patient. The associated structural heart defects – more frequently aortopulmonary window, tetralogy of Fallot, ventricular septal defect, and aortic stenosis – were found in more than one-third of the patients.1,11 With regard to the method of confirming the diagnosis, historically, angiography alone, in addition to echocardiography, has been used, whereas in the recent years non-invasive imaging methods with excellent accuracy – computed tomographic angiography, magnetic resonance imaging – have been recommended for the diagnosis of coronary artery origin anomaly.12–15 In this way, we used computed tomographic angiography for confirming the diagnosis, and also for the post-operative follow-up. We used the technique of direct implantation of the anomalous right coronary artery from the pulmonary artery to the aorta, as a recommended method for corrective surgery.16–18 Post-operative clinical and para-clinical findings of our patients indicated good right coronary artery flow. This case highlights the special importance of evaluation and appropriate management of correctable causes of heart failure in infancy, including ARCAPA, as well as the presentation of ARCAPA in infancy.
References
Figure 2. Computed tomographic angiography after corrective surgery.
Discussion Since the first report of abnormal origin of the coronary artery from the pulmonary artery in 1885 by Brooks,2 99 cases of anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) have been reported.3 ARCAPA is a very rare congenital heart anomaly estimated to occur in about 0.002% of the population.4 The anomaly may originate embryologically from malrotation of the dividing septum of the trunk or malposition of the coronary buds.3 ARCAPA is the second most common type of anomalous origin of coronary artery from the pulmonary artery, described by Soloff.5 The patients are usually asymptomatic during infancy and early childhood, and thus the anomaly is diagnosed commonly at later age.1
1. Kuhn A, Kasnar-Samprec J, Schreiber C, Vogt M. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA). Int J Cardiol 2010; 39: e27–e28. 2. Brooks HJS. Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery with some remarks apon the effect of this anomaly in producing crisoid dilation of the vessels. J Anat Physiol 1885; 20: 26–29. 3. Kumar Kuba P, Sharma J, Sharma A. Successful surgical treatment of a septuagenarian with anomalous right coronary artery from the pulmonary artery with an eleven year follow-up. Sultan Qabus Univ Med J 2013; 13: 169–174. 4. Jordon RA, Dry TJ, Edwards JE. Anomalous origin of the right coronary artery from pulmonary trunk. Mayo Clin Proc 1950; 25: 673–678. 5. Soloff LA. Anomalous coronary arteries arising from the pulmonary artery. Am Heart J 1992; 24: 118–127. 6. Brouwer MHJ, Beaufort-Krol GCM, Talsma MD. Aortopulmonary window associated with an anomalous origin of the right coronary artery. Int J Cardiol 1990; 28: 384–386. 7. Donaldson RM, Raphael M, Radley-Smith R, Yacoub M. Angiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Br J Radiol 1983; 56: 17–19. 8. Luisi SV, Ashraf MH, Gula G, Radley-Smith R, Yacoub M. Anomalous origin of the right coronary artery with aortopulmonary window: functional and surgical consideration. Thorax 1980; 35: 446–448.
Vol. 24, No. 5 Mahdavi et al: ARCAPA, and aberrant right subclavian artery in a 2-month infant with heart failure 937 9. Tosorsky RJ. Anomalni odstup prave venctte tepnyz plicntice. Rozhl Chir 1993; 72: 199–200. 10. Willians IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of literature. Am Heart J 2006; 152: 1004 e9–1004 e17. 11. Bossert T, Walter T, Doll N, Gummert JF, Kostelka M, Mohr FW. Anomalous origin of the right coronary artery from the pulmonary artery combined with aortic valve stenosis. Ann Thorac Surg 2005; 79: 347–348. 12. Tedeschi C, Briguori C, De Rosa R, et al. Right coronary artery arising from pulmonary trunk: assessment with conventional coronary angiography and multislice computed tomography coronary angiography. Cardiovasc Med 2009; 10: 178–182. 13. Gilmour J, Hafka H, Ropchan G, Johri AM. Case report, anomalous right coronary artery: a multimodality hunt for the origin. Cardiology 2011, doi:10.1155/2011/286598. 14. Bleumke DA, Achenbach S, Budoff M, et al. Noninvasive coronary artery imaging: magnetic resonance angiography and multidetector computed tomography angiography: a scientific statement from
15.
16.
17.
18.
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 2008; 118: 586–909. Su JT, Krishnamurthy R, Churg T, Vick GW III, Kovalchin JP. Anomalous right coronary from the pulmonary artery: non-invasive diagnosis and serial evaluation. J Cardiovasc Magn Reson 2007; 9: 57–61. Qing-yu W, Zhong hua X. Surgical treatment of anomalous origin of coronary artery from the pulmonary artery. Chinese Med J 2008; 121: 721–729. Ogiwara M, Hojo H, Ozaki M, Kyo S. Off-pump coronary artery bypass grafting for a patent with anomalous origin of the right coronary artery from the pulmonary artery. Ann Thorac Cardiovasc Surg 2006; 12: 432–434. Xu JP, Guo HW, Hu SS, Sun LZ, Song YH, Sun HS. Results of surgical correction in patients with anomalous origin of right coronary artery from the pulmonary artery. Zhonghua Wai Ke Za Zhi 2006; 44: 1525–1528.
© Cambridge University Press, 2013
doi:10.1017/S1047951113001510
Brief Report Anomalous right coronary artery from the pulmonary artery (ARCAPA), and aberrant right subclavian artery in a 2-month infant with heart failure Mohammad Mahdavi,1 Koorosh Vahidshahi,1 Ramin Baghai Tehrani,2 Hamidreza Poor Ali-Akbar,3 Mohammad Rad Godarzi,4 Department of Pediatric Cardiology; 2Department of Cardiovascular Surgery; 3Department of Radiology, Rajaie Cardiovascular Medical and Research Center, Iran University of Medical Sciences, Tehran, Iran; 4Department of Pediatrics, Golestan University of Medical Science, Golestan, Iran 1
Abstract Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a very rare congenital heart anomaly. Most of the cases are asymptomatic during infancy and childhood. We report ARCAPA associated with aberrant right subclavian artery in a 2-month male infant presenting with heart failure. We used computed tomography angiography for confirming the diagnosis and also for post-operative follow-up. Keyword: Anomalous origin of the right coronary artery from the pulmonary artery; infant; heart failure Received: 01 August 2013; Accepted: 28 August 2013; First published online: 29 October 2013
A
NOMALOUS ORIGIN OF THE RIGHT CORONARY
artery from the pulmonary artery is a very rare congenital heart anomaly. Most of the cases are asymptomatic during infancy and childhood, and thus the anomaly is more frequently detected in late childhood, adolescence, and adulthood.1 We report ARCAPA and aberrant right subclavian in a 2-month male infant.
The case A 7-week-old male infant was referred to our centre because of heart failure. Manifestations of the patient had begun gradually since the third week of life with intermittent poor feeding, and restlessness. After an ambulatory visit, the patient was admitted in another centre because of increasing clinical manifestations at the age of 5 weeks. Sepsis work-up and relevant evaluations were performed, and the patient was finally referred to our centre for evaluation and management of heart failure at 7 weeks of age. Correspondence to: Dr M. Mahdavi, Department of Peadiatric Cardiology, Rajaie Cardiovascular Medical and Research Center, Vali-asr Ave., Niyayesh Blvd., 199691-1151 Tehran, Iran. E-mail: [email protected]
The medical history was uneventful, with a normal vaginal delivery. At presentation to our centre, the patient was underweight, with clinical manifestations of heart failure. Chest X-ray demonstrated increased cardio-thoracic ratio and pulmonary vascular marking. Echocardiography revealed enlargement of the left atrium, left and right ventricles, moderate tricuspid regurgitation, relatively decreased ejection fraction (40%), and an abnormal diastolic flow in the main pulmonary artery. Computed tomographic angiography revealed abnormal origin of the right coronary artery from the pulmonary artery, also aberrant subclavian artery (Fig 1). Digoxin, captopril and furosemide were initiated for the patient; corrective surgery was also planned. Surgical operation was performed on the seventh day of admission, using direct reimplantation of right coronary artery on the aorta. The post-operative course was uneventful. Computed tomographic angiography after operation revealed normal origin of the right coronary artery from the aorta and no kinking (Fig 2); in echocardiography, the ejection fraction was increased. The patient was discharged from the hospital on the eighth day after surgery with good general condition, good feeding, and no respiratory distress.
936
Cardiology in the Young
Figure 1. Computed tomographic angiography before corrective surgery.
October 2014
There are few reports of ARCAPA diagnosed at infancy.6–9 Except for a 6-week infant with ARCAPA,10 our patient is one of the youngest diagnosed case of the anomaly. Heart failure may be a feature of presentation, as in our patient. The associated structural heart defects – more frequently aortopulmonary window, tetralogy of Fallot, ventricular septal defect, and aortic stenosis – were found in more than one-third of the patients.1,11 With regard to the method of confirming the diagnosis, historically, angiography alone, in addition to echocardiography, has been used, whereas in the recent years non-invasive imaging methods with excellent accuracy – computed tomographic angiography, magnetic resonance imaging – have been recommended for the diagnosis of coronary artery origin anomaly.12–15 In this way, we used computed tomographic angiography for confirming the diagnosis, and also for the post-operative follow-up. We used the technique of direct implantation of the anomalous right coronary artery from the pulmonary artery to the aorta, as a recommended method for corrective surgery.16–18 Post-operative clinical and para-clinical findings of our patients indicated good right coronary artery flow. This case highlights the special importance of evaluation and appropriate management of correctable causes of heart failure in infancy, including ARCAPA, as well as the presentation of ARCAPA in infancy.
References
Figure 2. Computed tomographic angiography after corrective surgery.
Discussion Since the first report of abnormal origin of the coronary artery from the pulmonary artery in 1885 by Brooks,2 99 cases of anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) have been reported.3 ARCAPA is a very rare congenital heart anomaly estimated to occur in about 0.002% of the population.4 The anomaly may originate embryologically from malrotation of the dividing septum of the trunk or malposition of the coronary buds.3 ARCAPA is the second most common type of anomalous origin of coronary artery from the pulmonary artery, described by Soloff.5 The patients are usually asymptomatic during infancy and early childhood, and thus the anomaly is diagnosed commonly at later age.1
1. Kuhn A, Kasnar-Samprec J, Schreiber C, Vogt M. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA). Int J Cardiol 2010; 39: e27–e28. 2. Brooks HJS. Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery with some remarks apon the effect of this anomaly in producing crisoid dilation of the vessels. J Anat Physiol 1885; 20: 26–29. 3. Kumar Kuba P, Sharma J, Sharma A. Successful surgical treatment of a septuagenarian with anomalous right coronary artery from the pulmonary artery with an eleven year follow-up. Sultan Qabus Univ Med J 2013; 13: 169–174. 4. Jordon RA, Dry TJ, Edwards JE. Anomalous origin of the right coronary artery from pulmonary trunk. Mayo Clin Proc 1950; 25: 673–678. 5. Soloff LA. Anomalous coronary arteries arising from the pulmonary artery. Am Heart J 1992; 24: 118–127. 6. Brouwer MHJ, Beaufort-Krol GCM, Talsma MD. Aortopulmonary window associated with an anomalous origin of the right coronary artery. Int J Cardiol 1990; 28: 384–386. 7. Donaldson RM, Raphael M, Radley-Smith R, Yacoub M. Angiographic diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Br J Radiol 1983; 56: 17–19. 8. Luisi SV, Ashraf MH, Gula G, Radley-Smith R, Yacoub M. Anomalous origin of the right coronary artery with aortopulmonary window: functional and surgical consideration. Thorax 1980; 35: 446–448.
Vol. 24, No. 5 Mahdavi et al: ARCAPA, and aberrant right subclavian artery in a 2-month infant with heart failure 937 9. Tosorsky RJ. Anomalni odstup prave venctte tepnyz plicntice. Rozhl Chir 1993; 72: 199–200. 10. Willians IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of literature. Am Heart J 2006; 152: 1004 e9–1004 e17. 11. Bossert T, Walter T, Doll N, Gummert JF, Kostelka M, Mohr FW. Anomalous origin of the right coronary artery from the pulmonary artery combined with aortic valve stenosis. Ann Thorac Surg 2005; 79: 347–348. 12. Tedeschi C, Briguori C, De Rosa R, et al. Right coronary artery arising from pulmonary trunk: assessment with conventional coronary angiography and multislice computed tomography coronary angiography. Cardiovasc Med 2009; 10: 178–182. 13. Gilmour J, Hafka H, Ropchan G, Johri AM. Case report, anomalous right coronary artery: a multimodality hunt for the origin. Cardiology 2011, doi:10.1155/2011/286598. 14. Bleumke DA, Achenbach S, Budoff M, et al. Noninvasive coronary artery imaging: magnetic resonance angiography and multidetector computed tomography angiography: a scientific statement from
15.
16.
17.
18.
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 2008; 118: 586–909. Su JT, Krishnamurthy R, Churg T, Vick GW III, Kovalchin JP. Anomalous right coronary from the pulmonary artery: non-invasive diagnosis and serial evaluation. J Cardiovasc Magn Reson 2007; 9: 57–61. Qing-yu W, Zhong hua X. Surgical treatment of anomalous origin of coronary artery from the pulmonary artery. Chinese Med J 2008; 121: 721–729. Ogiwara M, Hojo H, Ozaki M, Kyo S. Off-pump coronary artery bypass grafting for a patent with anomalous origin of the right coronary artery from the pulmonary artery. Ann Thorac Cardiovasc Surg 2006; 12: 432–434. Xu JP, Guo HW, Hu SS, Sun LZ, Song YH, Sun HS. Results of surgical correction in patients with anomalous origin of right coronary artery from the pulmonary artery. Zhonghua Wai Ke Za Zhi 2006; 44: 1525–1528.
