Jpn J Radiol DOI 10.1007/s11604-015-0404-7
CASE REPORT
Rare case of truncus arteriosus with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and unilateral left pulmonary artery agenesis Kartik Mittal · Amit K. Dey · Rohit Gadewar · Rajaram Sharma · Nilesh Pandit · Priya Rajput · Priya Hira
Received: 10 October 2014 / Accepted: 16 February 2015 © Japan Radiological Society 2015
Abstract The incidence of congenital heart disease (CHD) is 2.4–3.8/1000 live births. Up to 70.7 % of all cases of CHD are reported to be benign; complex heart anomalies are extremely rare. Our case is extremely rare, as we report three very rare findings—truncus arteriosus, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and unilateral left pulmonary artery agenesis—in a single patient. Congenital complex cardiac abnormalities are very rare, and two-dimensional echocardiography screening should be supported by cardiac computed tomography (CT). We report a case of truncus arteriosus associated with ARCAPA and left pulmonary artery agenesis diagnosed by cardiac computed tomography; we believe that such an unusual case with all three of these entities has never been reported before. Keywords Unilateral pulmonary artery agenesis · Truncus arteriosus · Congenital heart disease
Introduction The incidence of congenital heart disease is 2.4–3.8/1000 live births. Up to 70.7 % of all cases of congenital heart disease are reported to be benign; complex heart anomalies are extremely rare [1]. Truncus arteriosus, also known as common arterial trunk, is an uncommon (1–3 % of all cases of congenital
K. Mittal (*) · A. K. Dey · R. Gadewar · R. Sharma · N. Pandit · P. Rajput · P. Hira Department of Radiology, Seth G.S. Medical College and K.E.M. Hospital, Room No. 107, Main Boys Hostel, Acharya Donde Marg, Parel, Mumbai 400012, Maharashtra, India e-mail: [email protected]
heart disease) congenital cardiac defect that presents at birth [2]. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) typically presents during infancy, but anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare coronary anomaly that is incidentally diagnosed and is associated with sudden cardiac death [3]. Unilateral pulmonary artery agenesis is a rare congenital anomaly which can occur as a single disorder or may be associated with complex congenital cardiac abnormalities. It usually presents at adolescence, although late diagnosis after an abnormal chest radiograph is not unknown [4, 11]. The incidence of unilateral pulmonary artery agenesis is about 1 in 200,000. Right pulmonary artery agenesis is twice as common as left pulmonary artery agenesis [4, 11]. Here, we highlight a case of ARCAPA with pulmonary artery arising from the common arterial trunk and left pulmonary artery agenesis. Such a case has not previously been reported in the literature to the best of our knowledge, given that all three of these entities are so rare.
Case report A 10-month-old infant presented to our hospital with progressive dyspnea and cyanosis. A murmur on examination was also discerned. The patient was admitted to the hospital, where a series of investigations were performed. Chest X-ray revealed mild cardiomegaly, and the patient was referred for two-dimensional echocardiography, which revealed an overriding aorta, a subaortic ventricular septal defect, and nonvisualization of the pulmonary artery and associated major aortopulmonary collateral arteries
13
Jpn J Radiol
Fig. 1 Two-dimensional echocardiography shows a ventricular septal defect (asterix) with the aorta (Ao) overriding the ventricular septal defect. LV left ventricle. RV right ventricle. LA left atrium
(MAPCAs) (Fig. 1). A provisional diagnosis of tetralogy of Fallot was given. To look for the pulmonary artery and check whether its anatomy is confluent/nonconfluent, cardiac computed tomography was performed. Proper written informed consent was obtained from the patient’s guardian after explaining to them the risks and benefits of the examination. The study was performed using a Philips 64-slice computed tomography unit. The patient was sedated with 50 mg/kg of oral administered chloral hydrate. Computed tomography data were obtained using the following parameters: collimation 0.67 mm; reconstruction interval 0.5 mm. A computed tomography protocol (120 kVp, 100 mA, 4.5 mSv) was used. Scanning was performed from the thoracic inlet level to the L1 level. A contrast-enhanced scan was carried out, and computed tomography images were acquired in the arterial and venous phases. Nonionic contrast agent (2 ml/kg) was injected along the right median cubital vein via the power injector. Contrast injection was followed by a saline chase of 10 cc. The injection rate was 1 ml/s. The scan delay was determined with an automatic bolus tracking system. A threshold level of 100 Hounsfield units was set before starting the scan. No contrast reaction was encountered. The following post-processing techniques were used: multiplanar reformation, maximum intensity projection, volume rendering, and cine imaging. Cardiac computed tomography was done, and this showed a common arterial trunk with a large subaortic ventricular septal defect (Fig. 2) that had been misdiagnosed as an overriding aorta with an absent main pulmonary artery on two-dimensional echocardiography (Table 1). There was an associated anomalous right coronary arising from the main pulmonary artery and unilateral left pulmonary artery agenesis (Fig. 3a–c). Multiple MAPCAs were
13
Fig. 2 Cardiac computed tomography image in the sagittal plane showing that the common arterial trunk (CAT) originates from both the right ventricle (RV) and left ventricle (LV). A subtruncal ventricular septal defect (asterix) is seen
seen supplying the left lung parenchymal tissue (Fig. 3a). Three-dimensional reconstructed images showed a dilated common arterial trunk with the main pulmonary artery arising from it and continuing as the right pulmonary artery (Fig. 4). In addition, an associated persistent left superior vena cava and a communicating channel between the right and left superior vena cava was seen (Fig. 5). Retrospective two-dimensional echocardiography was performed, which revealed that the main pulmonary artery arose from the aortic trunk, suggestive of truncus arteriosus, which was completely missed in the previous two-dimensional echocardiography (Fig. 6a–c). Thus, a final diagnosis of type III/ A3 truncus arteriosus with left pulmonary artery agenesis, ARCAPA, and an associated left persistent superior vena cava was made. The patient was treated symptomatically in view of the pulmonary hypertension, but died due to severe congestive cardiac failure. Written informed consent for the details of this case to be included in a report was obtained from the patient’s guardian.
Discussion The common arterial trunk supplies blood to the coronary and pulmonary arteries and the systemic circulation, so pulmonary vascular resistance falls during the early hours of the neonatal period, leading to increased pulmonary circulation and frequently leads to congestive heart failure. Symptoms
Jpn J Radiol Table 1 Comparison of two-dimensional echocardiography with cardiac computed tomography in this case Parameter
Two-dimensional echocardiography
Cardiac computed tomography
Ventricular septal defect Main pulmonary artery (MPA)
Seen Atresia
Right and left pulmonary arteries
Nonconfluent with atresia of left pulmonary artery Seen
Seen Main pulmonary artery arising from aorta suggestive of truncus arteriosus Main pulmonary artery continuing as right pulmonary artery (RPA) with agenesis of left pulmonary artery (LPA) Seen
Not seen
Not seen
Tetralogy of Fallot with MPA and LPA atresia
Truncus arteriosus with left pulmonary artery agenesis with anomalous right coronary artery arising from pulmonary artery (ARCAPA) with double superior vena cava
Major aortopulmonary collateral arteries (MAPCAs) Patent ductus arteriosus (PDA) Final diagnosis
Fig. 3 Reformatted images from contrast-enhanced cardiac computed tomography showing the main pulmonary artery (MPA) arising from the common arterial trunk (CAT) and continuing as the right pulmonary artery (RPA). The right coronary artery arises from
the main pulmonary artery. Multiple major aortopulmonary collaterals (MAPCA) that arise from the descending aorta (DA) and supply the left lung are seen. The left pulmonary artery is not visualized. RA right atrium. RV right ventricle
and signs may include exhaustion, cyanosis, pale and cool skin, sweating, tachypnea, respiratory distress, tachycardia, poor feeding, failure to thrive, and hepatomegaly [5]. There are two proposed classifications for truncus arteriosus. Collett and Edwards classified truncus arteriosus into four types. Type 1 corresponds to a single pulmonary artery which arises from the truncus arteriosus just after the truncal valve. In type 2 the main pulmonary trunk is absent and the two pulmonary branches arise from the dorsal wall of the truncus. Type 3 truncus arteriosus has the pulmonary branches arising from the side of the truncus. In type 4 the pulmonary arteries are absent, so the pulmonary circulation is supplied by MAPCAs arising from the descending aorta. Van Praag classified truncus arteriosus into two types on the basis of the presence of a ventricular septal defect. Each type in this classification is then further subclassified into four subtypes. In type A1, the aorticopulmonary septum is still partially present, unlike in type A2, where the aorticopulmonary septum is completely absent, with both pulmonary arteries arising directly from the common trunk.
There is unilateral pulmonary artery agenesis in type 3, and the corresponding lung is supplied by MAPCAs from the descending aorta or collateral vessels (e.g., bronchial arteries) or a pulmonary artery from a patent ductus arteriosus. Type A4 is defined by the coexistence of hypoplasia, coarctation, and atresia, or an absence of the aortic arch [6]. Chest X-ray, electrocardiogram, and cardiac catheterization provide useful clues for diagnosing truncus arteriosus. It is frequently suggested that surgical repair by closure of the ventricular septal defect with reconstruction of the aorta from the truncus should be done early during the neonatal period due to the early progression of pulmonary artery hypertension, while some centers perform surgical repair at the age of 2–3 months [7]. Early surgical repair is associated with longer survival period [8]. There are four variants of anomalous origin of a coronary artery from the pulmonary artery—anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), anomalous origin
13
Jpn J Radiol
Fig. 5 Coronal maximum intensity projection image showing the right superior vena cava (1), left superior vena cava (2), and the communicating vein between the right and left superior vena cava (3) Fig. 4 Three-dimensional reconstructed image showing the common arterial trunk giving rise to the aorta (Ao) and right pulmonary artery (RPA). RA right atrium
of an accessory coronary artery from the pulmonary artery, and anomalous origin of the entire coronary circulation from the pulmonary artery. Among these, ARCAPA is the second most common, with an incidence of 0.002 % [9]. A murmur is a common finding with chest pain or congestive heart failure in ARCAPA due to associated myocardial ischemia, infarction, and related complications such as papillary muscle dysfunction. A cardiac computed tomography coronary angiogram permits excellent visualization of coronary artery anomalies [3]. ARCAPA is managed via surgical correction to eliminate the left-to-right shunt and establish dual antegrade coronary circulation, eliminating the potential for myocardial ischemia from coronary steal. Reimplantation of the anomalous vessel is the treatment of choice [10]. Unilateral pulmonary artery agenesis has no sex predilection, and is associated with unilateral absence of a pulmonary artery branch but a normal pulmonary trunk. Fallot’s tetralogy, intracardiac septal defects, coarctation of aorta, right aortic arch, and Eisenmenger’s syndrome are associated with left pulmonary artery atresia (75 %) [11]. Symptoms may include dyspnea on exertion, recurrent pulmonary infections, hemoptysis (in 20 % of patients), chest pain, or pleural effusion [4].
13
Chest radiography and echocardiography are helpful for diagnosing unilateral pulmonary artery agenesis. However, anatomic details for the definitive diagnosis and the presence of hilar collaterals can be discerned by contrastenhanced computed tomography scanning and magnetic resonance angiography [12]. Pneumonectomy and surgical revascularization are considered in cases of recurrent hemoptysis, pulmonary infection, and pulmonary hypertension [11]. Selective embolization of bronchial or nonbronchial systemic arteries is a valid alternative for patients with massive hemoptysis that are not eligible for surgery [13]. Pharmacological treatment for pulmonary hypertension is strongly recommended for patients who are unable to undergo surgical revascularization, or in cases that do not improve after surgery [14]. The three entities described above are only very rarely found together; indeed, such a case as ours has never before been reported in the literature. We also noted the presence of a double superior vena cava in our case. ARCAPA is associated with deoxygenated blood circulation, whereas ARCAPA arising from the common arterial trunk is associated with mixed circulation, giving better coronary circulation. In our patient, reimplantation of ARCAPA was only possible if the common arterial trunk was surgically treated. This case report also highlights the importance of cardiac computed tomography in cases with complex congenital cardiac abnormalities.
Jpn J Radiol
Fig. 6 Two-dimensional echocardiography images (corresponding to the sagittal multiplanar reformation computed tomography image) performed retrospectively, showing the pulmonary artery (4) aris-
ing from the common arterial trunk (CAT) and MAPCA (2) arising from the descending aorta (1), as well as a communicating channel between the right and left SVC (3)
Acknowledgments Dr. Amit Patel, D.M. Cardiology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai.
hearts with a double outlet right ventricle: usefulness and limitations. Magn Reson Imaging. 2000;18:245–53. 6. Mittal SK, Mangal Y, Kumar S, Yadav RR. Truncus arteriosus type 1: a case report. Indian J Radiol Imaging. 2006;16:229–31. 7. Ziyaeifard M, Azarfarin R, Ferasatkish R. New aspects of anesthetic management in congenital heart disease “common arterial trunk”. J Res Med Sci. 2014;19(4):368–74. 8. Williams JM, de Leeuw M, Black MD, Freedom RM, Williams WG, McCrindle BW. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol. 1999;34:545–53. 9. Yamanaka O, Hobbs RE. Coronary artery anomalies in 1,26,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn. 1990;21(1):28–40. 10. Williams IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature. Am Heart J. 2006;152(5):1004e17–9. 11. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002;122:1471–7. 12. Lynch DA, Higgins CB. MR imaging of unilateral pulmonary artery anomalies. J Comput Assist Tomogr. 1990;14:187–91. 13. Reñé M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol. 1995;18:251–4. 14. Steiropoulos P, Trakada G, Bouros D. Current pharmacological treatment of pulmonary arterial hypertension. Curr Clin Pharmacol. 2008;3:11–9.
Conflict of interest The authors declare that they have no conflict of interest.
References 1. Jung JW, Kim NS, Lee SY, Park IS. Recent trends in the frequency of congenital heart diseases: nationwide survey in Korea (abstract). The 3rd Congress of Asia-Pacific Pediatric Cardiac Society; 2010 July 6–8. Chiba: Japan; 2010. p. 225. 2. Russell HM, Pasquali SK, Jacobs JP, Jacobs ML, O’Brien SM, Mavroudis C, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2012;93:164–9. 3. Williams IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature. Am Heart J. 2006;152(5):1004.e9–17. 4. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest. 1995;108:670–6. 5. Beekmana RP, Roest AA, Helbing WA, Hazekamp MG, Schoof PH, Bartelings MM, et al. Spin echo MRI in the evaluation of
13
CASE REPORT
Rare case of truncus arteriosus with anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) and unilateral left pulmonary artery agenesis Kartik Mittal · Amit K. Dey · Rohit Gadewar · Rajaram Sharma · Nilesh Pandit · Priya Rajput · Priya Hira
Received: 10 October 2014 / Accepted: 16 February 2015 © Japan Radiological Society 2015
Abstract The incidence of congenital heart disease (CHD) is 2.4–3.8/1000 live births. Up to 70.7 % of all cases of CHD are reported to be benign; complex heart anomalies are extremely rare. Our case is extremely rare, as we report three very rare findings—truncus arteriosus, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and unilateral left pulmonary artery agenesis—in a single patient. Congenital complex cardiac abnormalities are very rare, and two-dimensional echocardiography screening should be supported by cardiac computed tomography (CT). We report a case of truncus arteriosus associated with ARCAPA and left pulmonary artery agenesis diagnosed by cardiac computed tomography; we believe that such an unusual case with all three of these entities has never been reported before. Keywords Unilateral pulmonary artery agenesis · Truncus arteriosus · Congenital heart disease
Introduction The incidence of congenital heart disease is 2.4–3.8/1000 live births. Up to 70.7 % of all cases of congenital heart disease are reported to be benign; complex heart anomalies are extremely rare [1]. Truncus arteriosus, also known as common arterial trunk, is an uncommon (1–3 % of all cases of congenital
K. Mittal (*) · A. K. Dey · R. Gadewar · R. Sharma · N. Pandit · P. Rajput · P. Hira Department of Radiology, Seth G.S. Medical College and K.E.M. Hospital, Room No. 107, Main Boys Hostel, Acharya Donde Marg, Parel, Mumbai 400012, Maharashtra, India e-mail: [email protected]
heart disease) congenital cardiac defect that presents at birth [2]. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) typically presents during infancy, but anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare coronary anomaly that is incidentally diagnosed and is associated with sudden cardiac death [3]. Unilateral pulmonary artery agenesis is a rare congenital anomaly which can occur as a single disorder or may be associated with complex congenital cardiac abnormalities. It usually presents at adolescence, although late diagnosis after an abnormal chest radiograph is not unknown [4, 11]. The incidence of unilateral pulmonary artery agenesis is about 1 in 200,000. Right pulmonary artery agenesis is twice as common as left pulmonary artery agenesis [4, 11]. Here, we highlight a case of ARCAPA with pulmonary artery arising from the common arterial trunk and left pulmonary artery agenesis. Such a case has not previously been reported in the literature to the best of our knowledge, given that all three of these entities are so rare.
Case report A 10-month-old infant presented to our hospital with progressive dyspnea and cyanosis. A murmur on examination was also discerned. The patient was admitted to the hospital, where a series of investigations were performed. Chest X-ray revealed mild cardiomegaly, and the patient was referred for two-dimensional echocardiography, which revealed an overriding aorta, a subaortic ventricular septal defect, and nonvisualization of the pulmonary artery and associated major aortopulmonary collateral arteries
13
Jpn J Radiol
Fig. 1 Two-dimensional echocardiography shows a ventricular septal defect (asterix) with the aorta (Ao) overriding the ventricular septal defect. LV left ventricle. RV right ventricle. LA left atrium
(MAPCAs) (Fig. 1). A provisional diagnosis of tetralogy of Fallot was given. To look for the pulmonary artery and check whether its anatomy is confluent/nonconfluent, cardiac computed tomography was performed. Proper written informed consent was obtained from the patient’s guardian after explaining to them the risks and benefits of the examination. The study was performed using a Philips 64-slice computed tomography unit. The patient was sedated with 50 mg/kg of oral administered chloral hydrate. Computed tomography data were obtained using the following parameters: collimation 0.67 mm; reconstruction interval 0.5 mm. A computed tomography protocol (120 kVp, 100 mA, 4.5 mSv) was used. Scanning was performed from the thoracic inlet level to the L1 level. A contrast-enhanced scan was carried out, and computed tomography images were acquired in the arterial and venous phases. Nonionic contrast agent (2 ml/kg) was injected along the right median cubital vein via the power injector. Contrast injection was followed by a saline chase of 10 cc. The injection rate was 1 ml/s. The scan delay was determined with an automatic bolus tracking system. A threshold level of 100 Hounsfield units was set before starting the scan. No contrast reaction was encountered. The following post-processing techniques were used: multiplanar reformation, maximum intensity projection, volume rendering, and cine imaging. Cardiac computed tomography was done, and this showed a common arterial trunk with a large subaortic ventricular septal defect (Fig. 2) that had been misdiagnosed as an overriding aorta with an absent main pulmonary artery on two-dimensional echocardiography (Table 1). There was an associated anomalous right coronary arising from the main pulmonary artery and unilateral left pulmonary artery agenesis (Fig. 3a–c). Multiple MAPCAs were
13
Fig. 2 Cardiac computed tomography image in the sagittal plane showing that the common arterial trunk (CAT) originates from both the right ventricle (RV) and left ventricle (LV). A subtruncal ventricular septal defect (asterix) is seen
seen supplying the left lung parenchymal tissue (Fig. 3a). Three-dimensional reconstructed images showed a dilated common arterial trunk with the main pulmonary artery arising from it and continuing as the right pulmonary artery (Fig. 4). In addition, an associated persistent left superior vena cava and a communicating channel between the right and left superior vena cava was seen (Fig. 5). Retrospective two-dimensional echocardiography was performed, which revealed that the main pulmonary artery arose from the aortic trunk, suggestive of truncus arteriosus, which was completely missed in the previous two-dimensional echocardiography (Fig. 6a–c). Thus, a final diagnosis of type III/ A3 truncus arteriosus with left pulmonary artery agenesis, ARCAPA, and an associated left persistent superior vena cava was made. The patient was treated symptomatically in view of the pulmonary hypertension, but died due to severe congestive cardiac failure. Written informed consent for the details of this case to be included in a report was obtained from the patient’s guardian.
Discussion The common arterial trunk supplies blood to the coronary and pulmonary arteries and the systemic circulation, so pulmonary vascular resistance falls during the early hours of the neonatal period, leading to increased pulmonary circulation and frequently leads to congestive heart failure. Symptoms
Jpn J Radiol Table 1 Comparison of two-dimensional echocardiography with cardiac computed tomography in this case Parameter
Two-dimensional echocardiography
Cardiac computed tomography
Ventricular septal defect Main pulmonary artery (MPA)
Seen Atresia
Right and left pulmonary arteries
Nonconfluent with atresia of left pulmonary artery Seen
Seen Main pulmonary artery arising from aorta suggestive of truncus arteriosus Main pulmonary artery continuing as right pulmonary artery (RPA) with agenesis of left pulmonary artery (LPA) Seen
Not seen
Not seen
Tetralogy of Fallot with MPA and LPA atresia
Truncus arteriosus with left pulmonary artery agenesis with anomalous right coronary artery arising from pulmonary artery (ARCAPA) with double superior vena cava
Major aortopulmonary collateral arteries (MAPCAs) Patent ductus arteriosus (PDA) Final diagnosis
Fig. 3 Reformatted images from contrast-enhanced cardiac computed tomography showing the main pulmonary artery (MPA) arising from the common arterial trunk (CAT) and continuing as the right pulmonary artery (RPA). The right coronary artery arises from
the main pulmonary artery. Multiple major aortopulmonary collaterals (MAPCA) that arise from the descending aorta (DA) and supply the left lung are seen. The left pulmonary artery is not visualized. RA right atrium. RV right ventricle
and signs may include exhaustion, cyanosis, pale and cool skin, sweating, tachypnea, respiratory distress, tachycardia, poor feeding, failure to thrive, and hepatomegaly [5]. There are two proposed classifications for truncus arteriosus. Collett and Edwards classified truncus arteriosus into four types. Type 1 corresponds to a single pulmonary artery which arises from the truncus arteriosus just after the truncal valve. In type 2 the main pulmonary trunk is absent and the two pulmonary branches arise from the dorsal wall of the truncus. Type 3 truncus arteriosus has the pulmonary branches arising from the side of the truncus. In type 4 the pulmonary arteries are absent, so the pulmonary circulation is supplied by MAPCAs arising from the descending aorta. Van Praag classified truncus arteriosus into two types on the basis of the presence of a ventricular septal defect. Each type in this classification is then further subclassified into four subtypes. In type A1, the aorticopulmonary septum is still partially present, unlike in type A2, where the aorticopulmonary septum is completely absent, with both pulmonary arteries arising directly from the common trunk.
There is unilateral pulmonary artery agenesis in type 3, and the corresponding lung is supplied by MAPCAs from the descending aorta or collateral vessels (e.g., bronchial arteries) or a pulmonary artery from a patent ductus arteriosus. Type A4 is defined by the coexistence of hypoplasia, coarctation, and atresia, or an absence of the aortic arch [6]. Chest X-ray, electrocardiogram, and cardiac catheterization provide useful clues for diagnosing truncus arteriosus. It is frequently suggested that surgical repair by closure of the ventricular septal defect with reconstruction of the aorta from the truncus should be done early during the neonatal period due to the early progression of pulmonary artery hypertension, while some centers perform surgical repair at the age of 2–3 months [7]. Early surgical repair is associated with longer survival period [8]. There are four variants of anomalous origin of a coronary artery from the pulmonary artery—anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), anomalous origin
13
Jpn J Radiol
Fig. 5 Coronal maximum intensity projection image showing the right superior vena cava (1), left superior vena cava (2), and the communicating vein between the right and left superior vena cava (3) Fig. 4 Three-dimensional reconstructed image showing the common arterial trunk giving rise to the aorta (Ao) and right pulmonary artery (RPA). RA right atrium
of an accessory coronary artery from the pulmonary artery, and anomalous origin of the entire coronary circulation from the pulmonary artery. Among these, ARCAPA is the second most common, with an incidence of 0.002 % [9]. A murmur is a common finding with chest pain or congestive heart failure in ARCAPA due to associated myocardial ischemia, infarction, and related complications such as papillary muscle dysfunction. A cardiac computed tomography coronary angiogram permits excellent visualization of coronary artery anomalies [3]. ARCAPA is managed via surgical correction to eliminate the left-to-right shunt and establish dual antegrade coronary circulation, eliminating the potential for myocardial ischemia from coronary steal. Reimplantation of the anomalous vessel is the treatment of choice [10]. Unilateral pulmonary artery agenesis has no sex predilection, and is associated with unilateral absence of a pulmonary artery branch but a normal pulmonary trunk. Fallot’s tetralogy, intracardiac septal defects, coarctation of aorta, right aortic arch, and Eisenmenger’s syndrome are associated with left pulmonary artery atresia (75 %) [11]. Symptoms may include dyspnea on exertion, recurrent pulmonary infections, hemoptysis (in 20 % of patients), chest pain, or pleural effusion [4].
13
Chest radiography and echocardiography are helpful for diagnosing unilateral pulmonary artery agenesis. However, anatomic details for the definitive diagnosis and the presence of hilar collaterals can be discerned by contrastenhanced computed tomography scanning and magnetic resonance angiography [12]. Pneumonectomy and surgical revascularization are considered in cases of recurrent hemoptysis, pulmonary infection, and pulmonary hypertension [11]. Selective embolization of bronchial or nonbronchial systemic arteries is a valid alternative for patients with massive hemoptysis that are not eligible for surgery [13]. Pharmacological treatment for pulmonary hypertension is strongly recommended for patients who are unable to undergo surgical revascularization, or in cases that do not improve after surgery [14]. The three entities described above are only very rarely found together; indeed, such a case as ours has never before been reported in the literature. We also noted the presence of a double superior vena cava in our case. ARCAPA is associated with deoxygenated blood circulation, whereas ARCAPA arising from the common arterial trunk is associated with mixed circulation, giving better coronary circulation. In our patient, reimplantation of ARCAPA was only possible if the common arterial trunk was surgically treated. This case report also highlights the importance of cardiac computed tomography in cases with complex congenital cardiac abnormalities.
Jpn J Radiol
Fig. 6 Two-dimensional echocardiography images (corresponding to the sagittal multiplanar reformation computed tomography image) performed retrospectively, showing the pulmonary artery (4) aris-
ing from the common arterial trunk (CAT) and MAPCA (2) arising from the descending aorta (1), as well as a communicating channel between the right and left SVC (3)
Acknowledgments Dr. Amit Patel, D.M. Cardiology, Seth G.S. Medical College and K.E.M. Hospital, Mumbai.
hearts with a double outlet right ventricle: usefulness and limitations. Magn Reson Imaging. 2000;18:245–53. 6. Mittal SK, Mangal Y, Kumar S, Yadav RR. Truncus arteriosus type 1: a case report. Indian J Radiol Imaging. 2006;16:229–31. 7. Ziyaeifard M, Azarfarin R, Ferasatkish R. New aspects of anesthetic management in congenital heart disease “common arterial trunk”. J Res Med Sci. 2014;19(4):368–74. 8. Williams JM, de Leeuw M, Black MD, Freedom RM, Williams WG, McCrindle BW. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol. 1999;34:545–53. 9. Yamanaka O, Hobbs RE. Coronary artery anomalies in 1,26,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn. 1990;21(1):28–40. 10. Williams IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature. Am Heart J. 2006;152(5):1004e17–9. 11. Ten Harkel AD, Blom NA, Ottenkamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest. 2002;122:1471–7. 12. Lynch DA, Higgins CB. MR imaging of unilateral pulmonary artery anomalies. J Comput Assist Tomogr. 1990;14:187–91. 13. Reñé M, Sans J, Dominguez J, Sancho C, Valldeperas J. Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol. 1995;18:251–4. 14. Steiropoulos P, Trakada G, Bouros D. Current pharmacological treatment of pulmonary arterial hypertension. Curr Clin Pharmacol. 2008;3:11–9.
Conflict of interest The authors declare that they have no conflict of interest.
References 1. Jung JW, Kim NS, Lee SY, Park IS. Recent trends in the frequency of congenital heart diseases: nationwide survey in Korea (abstract). The 3rd Congress of Asia-Pacific Pediatric Cardiac Society; 2010 July 6–8. Chiba: Japan; 2010. p. 225. 2. Russell HM, Pasquali SK, Jacobs JP, Jacobs ML, O’Brien SM, Mavroudis C, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the Society of Thoracic Surgeons Congenital Heart Surgery Database. Ann Thorac Surg. 2012;93:164–9. 3. Williams IA, Gersony WM, Hellenbrand WE. Anomalous right coronary artery arising from the pulmonary artery: a report of 7 cases and a review of the literature. Am Heart J. 2006;152(5):1004.e9–17. 4. Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest. 1995;108:670–6. 5. Beekmana RP, Roest AA, Helbing WA, Hazekamp MG, Schoof PH, Bartelings MM, et al. Spin echo MRI in the evaluation of
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