Indian J Pediatr (June 2016) 83(6):611–613 DOI 10.1007/s12098-016-2045-y
SCIENTIFIC LETTER
Anomalous Left Coronary Artery Arising from Right Pulmonary Artery and Giant Left Atrium Dongxu Li 1 & Yun Xia 1 & Mengsi Li 1 & Yabo Wang 1 & Yuan Feng 2 & Qi An 1
Received: 26 November 2015 / Accepted: 14 January 2016 / Published online: 26 February 2016 # Dr. K C Chaudhuri Foundation 2016
To the Editor: A 5-y-old girl presenting with limited exercise tolerance was admitted to our hospital. She had a diagnosis of endocardial fibroelastosis in an another center beforehand. Electrocardiograph was normal (Fig.1). Transthoracic echocardiography demonstrated severe mitral regurgitation (MR) and found anomalous coronary blood flow (Fig. 2a, b). Chest radiography revealed an enlarged heart and pulmonary overflow (Fig. 2c). Meanwhile the cardiac magnetic resonance imaging (MRI) showed there was mild ischemia but with acceptable left ventricular function (Table 1). Finally, computed tomography (CT) confirmed the anomalous left coronary artery arising from the pulmonary artery with 88 × 76 mm left atrium (Fig. 2d, e and f). Surgery was performed. The patient underwent coronary reimplantation to aorta, mitral valvuloplasty and left atrium reduction under cardiopulmonary bypass through median sternotomy. Her postoperative course was uneventful with normal coronary flow, laminar mitral inflow, trivial MR, and good left ventricular function. Origin of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly. The majority of cases present between 4 and 12 wk of age as the pulmonary artery resistance progressively
decreases, leading to increased coronary steal and chronic myocardial ischemia [1]. Meanwhile it is easily to be diagnosed as endocardial fibroelastosis (47.4 %) and dilated cardiomyopathy (15.8 %) [2]. Different diagnosis leads to different treatments. And it is very unusual for these patients to present with isolated MR. Any degree of MR that is seen in these patients is generally secondary to papillary muscle ischemia or due to left ventricular dilatation secondary to ischemia [3]. And the secondary MR further and gradually overloads the left atrium. Giant left atrium (GLA) is also a rare disease which is commonly associated with rheumatic mitral valve disease, mostly MR in aged patients. Left atrial enlargement is thought to be due not only to MR, but also to intrinsic characteristics of the left atrial wall [4]. ALCAPA often causes myocardial ischemia and infarction within the first few weeks or months after birth, subsequently leading to left ventricular dysfunction, MR, and congestive heart failure. Thus surgical repair appears to provide the only viable hope for extended survival [5].
Dongxu Li and Yun Xia are both co-first authors to this manuscript. * Qi An [email protected]
1
Department of Cardiovascular Surgery, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, Chengdu, Sichuan 610041, People’s Republic of China
2
Department of Cardiology, West China Hospital, Sichuan University, Sichuan, People’s Republic of China
Fig. 1 Electrocardiograph showing the sinus rhythm and non-ST segment changes
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Indian J Pediatr (June 2016) 83(6):611–613
Fig. 2 Echocardiography showing a severe mitral regurgitation (MR) and b anomalous backward blue-color flow (Arrow) which hinted LCA maybe did not arise from aorta; c Chest radiograph revealing an enlarged heart and pulmonary overflow; d CT demonstrating ALCAPA (Arrow) and GLA; e 3-D reconstruction revealing LCA arising from the RPA; f CT measuring the maximum diameter of GLA. LV Left ventricle; LA Left atrium; Ao Aorta; LCA Left coronary artery; PA Pulmonary artery; RPA Right pulmonary artery; GLA Giant left atrium; ALCAPA Anomalous left coronary artery arising from the pulmonary artery
Table 1 Cardiac MRI data showing the ventricular function
Left ventricular volume results
Right ventricular volume results
Myocardial mass Mass index ED volume ED volume index ES volume ES volume index Stroke volume Stroke volume index Cardiac output
75.35 g 106.47 g/m2 153.85 ml 217.40 ml/m2 66.99 ml 94.66 ml/m2 86.86 ml 122.74 ml/m2 11.07 l/min
48.31 ml 68.27 ml/m2 36.74 ml 51.92 ml/m2 11.57 ml 16.36 ml/m2 1.48 l/min
Cardiac output index
15.64 l/(min/m2)
Ejection fraction
56.46 %
2.08 l/(min/m2) 23.96 %
Compliance with Ethical Standards
References
Conflict of Interest None.
1.
Source of Funding None.
Rathinam S, Stümper O, Brawn WJ, et al. Cleft mitral valve in association with anomalous left coronary artery arising from pulmonary artery. Ann Thorac Surg. 2005;80:1111–3.
Indian J Pediatr (June 2016) 83(6):611–613 2.
3.
Zheng J, Ding W, Xiao Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery in children: 15 years experience. Pediatr Cardiol. 2011;32:24–31. 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;74:946–55.
613 4.
5.
Chick JF, Sheehan SE, Miller JD, et al. Giant left atrium in rheumatic heart disease: the classic signs of left atrial enlargement. J Emerg Med. 2013;44:e393–4. Alexi-Meskishvili V, Nasseri BA, Nordmeyer S, et al. Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children. J Thorac Cardiovasc Surg. 2011;142:868–74.
SCIENTIFIC LETTER
Anomalous Left Coronary Artery Arising from Right Pulmonary Artery and Giant Left Atrium Dongxu Li 1 & Yun Xia 1 & Mengsi Li 1 & Yabo Wang 1 & Yuan Feng 2 & Qi An 1
Received: 26 November 2015 / Accepted: 14 January 2016 / Published online: 26 February 2016 # Dr. K C Chaudhuri Foundation 2016
To the Editor: A 5-y-old girl presenting with limited exercise tolerance was admitted to our hospital. She had a diagnosis of endocardial fibroelastosis in an another center beforehand. Electrocardiograph was normal (Fig.1). Transthoracic echocardiography demonstrated severe mitral regurgitation (MR) and found anomalous coronary blood flow (Fig. 2a, b). Chest radiography revealed an enlarged heart and pulmonary overflow (Fig. 2c). Meanwhile the cardiac magnetic resonance imaging (MRI) showed there was mild ischemia but with acceptable left ventricular function (Table 1). Finally, computed tomography (CT) confirmed the anomalous left coronary artery arising from the pulmonary artery with 88 × 76 mm left atrium (Fig. 2d, e and f). Surgery was performed. The patient underwent coronary reimplantation to aorta, mitral valvuloplasty and left atrium reduction under cardiopulmonary bypass through median sternotomy. Her postoperative course was uneventful with normal coronary flow, laminar mitral inflow, trivial MR, and good left ventricular function. Origin of an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly. The majority of cases present between 4 and 12 wk of age as the pulmonary artery resistance progressively
decreases, leading to increased coronary steal and chronic myocardial ischemia [1]. Meanwhile it is easily to be diagnosed as endocardial fibroelastosis (47.4 %) and dilated cardiomyopathy (15.8 %) [2]. Different diagnosis leads to different treatments. And it is very unusual for these patients to present with isolated MR. Any degree of MR that is seen in these patients is generally secondary to papillary muscle ischemia or due to left ventricular dilatation secondary to ischemia [3]. And the secondary MR further and gradually overloads the left atrium. Giant left atrium (GLA) is also a rare disease which is commonly associated with rheumatic mitral valve disease, mostly MR in aged patients. Left atrial enlargement is thought to be due not only to MR, but also to intrinsic characteristics of the left atrial wall [4]. ALCAPA often causes myocardial ischemia and infarction within the first few weeks or months after birth, subsequently leading to left ventricular dysfunction, MR, and congestive heart failure. Thus surgical repair appears to provide the only viable hope for extended survival [5].
Dongxu Li and Yun Xia are both co-first authors to this manuscript. * Qi An [email protected]
1
Department of Cardiovascular Surgery, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, Chengdu, Sichuan 610041, People’s Republic of China
2
Department of Cardiology, West China Hospital, Sichuan University, Sichuan, People’s Republic of China
Fig. 1 Electrocardiograph showing the sinus rhythm and non-ST segment changes
612
Indian J Pediatr (June 2016) 83(6):611–613
Fig. 2 Echocardiography showing a severe mitral regurgitation (MR) and b anomalous backward blue-color flow (Arrow) which hinted LCA maybe did not arise from aorta; c Chest radiograph revealing an enlarged heart and pulmonary overflow; d CT demonstrating ALCAPA (Arrow) and GLA; e 3-D reconstruction revealing LCA arising from the RPA; f CT measuring the maximum diameter of GLA. LV Left ventricle; LA Left atrium; Ao Aorta; LCA Left coronary artery; PA Pulmonary artery; RPA Right pulmonary artery; GLA Giant left atrium; ALCAPA Anomalous left coronary artery arising from the pulmonary artery
Table 1 Cardiac MRI data showing the ventricular function
Left ventricular volume results
Right ventricular volume results
Myocardial mass Mass index ED volume ED volume index ES volume ES volume index Stroke volume Stroke volume index Cardiac output
75.35 g 106.47 g/m2 153.85 ml 217.40 ml/m2 66.99 ml 94.66 ml/m2 86.86 ml 122.74 ml/m2 11.07 l/min
48.31 ml 68.27 ml/m2 36.74 ml 51.92 ml/m2 11.57 ml 16.36 ml/m2 1.48 l/min
Cardiac output index
15.64 l/(min/m2)
Ejection fraction
56.46 %
2.08 l/(min/m2) 23.96 %
Compliance with Ethical Standards
References
Conflict of Interest None.
1.
Source of Funding None.
Rathinam S, Stümper O, Brawn WJ, et al. Cleft mitral valve in association with anomalous left coronary artery arising from pulmonary artery. Ann Thorac Surg. 2005;80:1111–3.
Indian J Pediatr (June 2016) 83(6):611–613 2.
3.
Zheng J, Ding W, Xiao Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery in children: 15 years experience. Pediatr Cardiol. 2011;32:24–31. 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;74:946–55.
613 4.
5.
Chick JF, Sheehan SE, Miller JD, et al. Giant left atrium in rheumatic heart disease: the classic signs of left atrial enlargement. J Emerg Med. 2013;44:e393–4. Alexi-Meskishvili V, Nasseri BA, Nordmeyer S, et al. Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants and children. J Thorac Cardiovasc Surg. 2011;142:868–74.
