Anatomical variations and associated cardiac and extracardiac anomalies in patients with common arterial trunk
| Cardiac-associated anomalies . | Specification . |
|---|---|
| Aortic arch and tributaries | |
| Atresia/interruption aortic arch, usually type B | |
| Aortic hypoplasia/coarctation | |
| Right aortic arch, ± mirror image branching | |
| Double aortic arch (rare) | |
| Aberrant subclavian artery (arteria lusoria) | |
| Aortic dilatationa | |
| Arterial duct | |
| Absent | |
| Bilateral | |
| Patent (usually associated with aortic stenosis/interruption) | |
| Patent with normal aortic arch (rare) | |
| Atrial septum | |
| ASD type 2 (ostium secundum defect) | |
| ASD type 1 (ostium primum defect, partial AVSD) (rare) | |
| Common atrium | |
| AVSD | |
| AV valves and junction | |
| Mitral valve atresia/stenosis | |
| Tricuspid valve atresia/stenosis (rare) | |
| Polyvalvular disease | |
| Double inlet left ventricle | |
| Discordant AV connections | |
| Coronary arteries | |
| High ‘take-off’ from aorta/acute angulation | |
| Single coronary arteries | |
| Coronary ostium positioned near valve commissure | |
| Absent left main stem/double coronary orifices | |
| Ectopic position of coronary ostium | |
| Proximity of coronary arteries to pulmonary arteries | |
| Anomalous coronary arterial course | |
| Congenital ostium stenosis | |
| Large infundibular branching of RCA | |
| RCx origin from RPA | |
| Intramural coronary arterial course | |
| Pulmonary arteries | |
| PA hypoplasia/agenesis/atresia, unilateral absence of a PA, MAPCAs | |
| Peripheral PA stenosis/PA branch obstruction | |
| Distal ductal origin of 1 PA | |
| Crossed/malpositioned PAs (association with aortic arch interruption) | |
| Situs anomalies | |
| Discordant AV connections | |
| Heterotaxy syndrome | |
| Situs inversus | |
| Truncal valve | |
| Bicuspid (approximately 7–8%) | |
| Tricuspid (approximately 60–70%) | |
| Quadricuspid (approximately 20–30%) | |
| Unicuspid, pentacuspid and hexacuspid (extremely rare) | |
| Dysplasia/fibrosis | |
| Venous connections | |
| Abnormal pulmonary venous connections, in particular PAPVC | |
| Abnormal systemic venous connections, in particular PLSVC | |
| Ventricles | |
| Anomalous muscle bundles | |
| Anteroseptal twist | |
| Pronounced ALM | |
| Pronounced PMM | |
| Ventricular dominance, e.g. in unbalanced AVSD, extreme overriding of arterial trunk, mitral or tricuspid atresia (rare) | |
| Ventricular septum | |
| VSD | |
| Outlet subarterial with muscular posterior rim (most frequent) | |
| Outlet perimembranous (less common) | |
| Muscular ‘remote’ (uncommon) | |
| Multiple VSDs (uncommon) | |
| Non-cardiac anomaliesb | |
| Intracranial aneurysms | |
| Palatum anomaliesc | |
| Pancreas anomalies | |
| Parathyroid anomaliesd | |
| Phenylketonuria | |
| Thymus anomaliesc | |
| Tracheobronchial stenosisd |
| Cardiac-associated anomalies . | Specification . |
|---|---|
| Aortic arch and tributaries | |
| Atresia/interruption aortic arch, usually type B | |
| Aortic hypoplasia/coarctation | |
| Right aortic arch, ± mirror image branching | |
| Double aortic arch (rare) | |
| Aberrant subclavian artery (arteria lusoria) | |
| Aortic dilatationa | |
| Arterial duct | |
| Absent | |
| Bilateral | |
| Patent (usually associated with aortic stenosis/interruption) | |
| Patent with normal aortic arch (rare) | |
| Atrial septum | |
| ASD type 2 (ostium secundum defect) | |
| ASD type 1 (ostium primum defect, partial AVSD) (rare) | |
| Common atrium | |
| AVSD | |
| AV valves and junction | |
| Mitral valve atresia/stenosis | |
| Tricuspid valve atresia/stenosis (rare) | |
| Polyvalvular disease | |
| Double inlet left ventricle | |
| Discordant AV connections | |
| Coronary arteries | |
| High ‘take-off’ from aorta/acute angulation | |
| Single coronary arteries | |
| Coronary ostium positioned near valve commissure | |
| Absent left main stem/double coronary orifices | |
| Ectopic position of coronary ostium | |
| Proximity of coronary arteries to pulmonary arteries | |
| Anomalous coronary arterial course | |
| Congenital ostium stenosis | |
| Large infundibular branching of RCA | |
| RCx origin from RPA | |
| Intramural coronary arterial course | |
| Pulmonary arteries | |
| PA hypoplasia/agenesis/atresia, unilateral absence of a PA, MAPCAs | |
| Peripheral PA stenosis/PA branch obstruction | |
| Distal ductal origin of 1 PA | |
| Crossed/malpositioned PAs (association with aortic arch interruption) | |
| Situs anomalies | |
| Discordant AV connections | |
| Heterotaxy syndrome | |
| Situs inversus | |
| Truncal valve | |
| Bicuspid (approximately 7–8%) | |
| Tricuspid (approximately 60–70%) | |
| Quadricuspid (approximately 20–30%) | |
| Unicuspid, pentacuspid and hexacuspid (extremely rare) | |
| Dysplasia/fibrosis | |
| Venous connections | |
| Abnormal pulmonary venous connections, in particular PAPVC | |
| Abnormal systemic venous connections, in particular PLSVC | |
| Ventricles | |
| Anomalous muscle bundles | |
| Anteroseptal twist | |
| Pronounced ALM | |
| Pronounced PMM | |
| Ventricular dominance, e.g. in unbalanced AVSD, extreme overriding of arterial trunk, mitral or tricuspid atresia (rare) | |
| Ventricular septum | |
| VSD | |
| Outlet subarterial with muscular posterior rim (most frequent) | |
| Outlet perimembranous (less common) | |
| Muscular ‘remote’ (uncommon) | |
| Multiple VSDs (uncommon) | |
| Non-cardiac anomaliesb | |
| Intracranial aneurysms | |
| Palatum anomaliesc | |
| Pancreas anomalies | |
| Parathyroid anomaliesd | |
| Phenylketonuria | |
| Thymus anomaliesc | |
| Tracheobronchial stenosisd |
Data derived from multiple sources [4, 5, 31, 50–55].
Possibly secondary characteristic.
Often caused by genetic/syndromic anomalies (see ‘Genetics of common arterial trunk’).
Associated with 22q11.2 deletion syndrome.
Associated with CHARGE syndrome.
ALM: anterolateral muscle; ASD: atrial septal defect; AV: atrioventricular; AVSD: atrioventricular septal defect; MAPCAs: major aortopulmonary collateral arteries; PA: pulmonary artery; PAPVC: partial anomalous pulmonary venous connection; PLSCV: persistent left superior caval vein; PMM: posteromedial muscle; RCA: right coronary artery; RCx: ramus circumflex; RPA: right pulmonary artery; VSD: ventricular septal defect.
Anatomical variations and associated cardiac and extracardiac anomalies in patients with common arterial trunk
| Cardiac-associated anomalies . | Specification . |
|---|---|
| Aortic arch and tributaries | |
| Atresia/interruption aortic arch, usually type B | |
| Aortic hypoplasia/coarctation | |
| Right aortic arch, ± mirror image branching | |
| Double aortic arch (rare) | |
| Aberrant subclavian artery (arteria lusoria) | |
| Aortic dilatationa | |
| Arterial duct | |
| Absent | |
| Bilateral | |
| Patent (usually associated with aortic stenosis/interruption) | |
| Patent with normal aortic arch (rare) | |
| Atrial septum | |
| ASD type 2 (ostium secundum defect) | |
| ASD type 1 (ostium primum defect, partial AVSD) (rare) | |
| Common atrium | |
| AVSD | |
| AV valves and junction | |
| Mitral valve atresia/stenosis | |
| Tricuspid valve atresia/stenosis (rare) | |
| Polyvalvular disease | |
| Double inlet left ventricle | |
| Discordant AV connections | |
| Coronary arteries | |
| High ‘take-off’ from aorta/acute angulation | |
| Single coronary arteries | |
| Coronary ostium positioned near valve commissure | |
| Absent left main stem/double coronary orifices | |
| Ectopic position of coronary ostium | |
| Proximity of coronary arteries to pulmonary arteries | |
| Anomalous coronary arterial course | |
| Congenital ostium stenosis | |
| Large infundibular branching of RCA | |
| RCx origin from RPA | |
| Intramural coronary arterial course | |
| Pulmonary arteries | |
| PA hypoplasia/agenesis/atresia, unilateral absence of a PA, MAPCAs | |
| Peripheral PA stenosis/PA branch obstruction | |
| Distal ductal origin of 1 PA | |
| Crossed/malpositioned PAs (association with aortic arch interruption) | |
| Situs anomalies | |
| Discordant AV connections | |
| Heterotaxy syndrome | |
| Situs inversus | |
| Truncal valve | |
| Bicuspid (approximately 7–8%) | |
| Tricuspid (approximately 60–70%) | |
| Quadricuspid (approximately 20–30%) | |
| Unicuspid, pentacuspid and hexacuspid (extremely rare) | |
| Dysplasia/fibrosis | |
| Venous connections | |
| Abnormal pulmonary venous connections, in particular PAPVC | |
| Abnormal systemic venous connections, in particular PLSVC | |
| Ventricles | |
| Anomalous muscle bundles | |
| Anteroseptal twist | |
| Pronounced ALM | |
| Pronounced PMM | |
| Ventricular dominance, e.g. in unbalanced AVSD, extreme overriding of arterial trunk, mitral or tricuspid atresia (rare) | |
| Ventricular septum | |
| VSD | |
| Outlet subarterial with muscular posterior rim (most frequent) | |
| Outlet perimembranous (less common) | |
| Muscular ‘remote’ (uncommon) | |
| Multiple VSDs (uncommon) | |
| Non-cardiac anomaliesb | |
| Intracranial aneurysms | |
| Palatum anomaliesc | |
| Pancreas anomalies | |
| Parathyroid anomaliesd | |
| Phenylketonuria | |
| Thymus anomaliesc | |
| Tracheobronchial stenosisd |
| Cardiac-associated anomalies . | Specification . |
|---|---|
| Aortic arch and tributaries | |
| Atresia/interruption aortic arch, usually type B | |
| Aortic hypoplasia/coarctation | |
| Right aortic arch, ± mirror image branching | |
| Double aortic arch (rare) | |
| Aberrant subclavian artery (arteria lusoria) | |
| Aortic dilatationa | |
| Arterial duct | |
| Absent | |
| Bilateral | |
| Patent (usually associated with aortic stenosis/interruption) | |
| Patent with normal aortic arch (rare) | |
| Atrial septum | |
| ASD type 2 (ostium secundum defect) | |
| ASD type 1 (ostium primum defect, partial AVSD) (rare) | |
| Common atrium | |
| AVSD | |
| AV valves and junction | |
| Mitral valve atresia/stenosis | |
| Tricuspid valve atresia/stenosis (rare) | |
| Polyvalvular disease | |
| Double inlet left ventricle | |
| Discordant AV connections | |
| Coronary arteries | |
| High ‘take-off’ from aorta/acute angulation | |
| Single coronary arteries | |
| Coronary ostium positioned near valve commissure | |
| Absent left main stem/double coronary orifices | |
| Ectopic position of coronary ostium | |
| Proximity of coronary arteries to pulmonary arteries | |
| Anomalous coronary arterial course | |
| Congenital ostium stenosis | |
| Large infundibular branching of RCA | |
| RCx origin from RPA | |
| Intramural coronary arterial course | |
| Pulmonary arteries | |
| PA hypoplasia/agenesis/atresia, unilateral absence of a PA, MAPCAs | |
| Peripheral PA stenosis/PA branch obstruction | |
| Distal ductal origin of 1 PA | |
| Crossed/malpositioned PAs (association with aortic arch interruption) | |
| Situs anomalies | |
| Discordant AV connections | |
| Heterotaxy syndrome | |
| Situs inversus | |
| Truncal valve | |
| Bicuspid (approximately 7–8%) | |
| Tricuspid (approximately 60–70%) | |
| Quadricuspid (approximately 20–30%) | |
| Unicuspid, pentacuspid and hexacuspid (extremely rare) | |
| Dysplasia/fibrosis | |
| Venous connections | |
| Abnormal pulmonary venous connections, in particular PAPVC | |
| Abnormal systemic venous connections, in particular PLSVC | |
| Ventricles | |
| Anomalous muscle bundles | |
| Anteroseptal twist | |
| Pronounced ALM | |
| Pronounced PMM | |
| Ventricular dominance, e.g. in unbalanced AVSD, extreme overriding of arterial trunk, mitral or tricuspid atresia (rare) | |
| Ventricular septum | |
| VSD | |
| Outlet subarterial with muscular posterior rim (most frequent) | |
| Outlet perimembranous (less common) | |
| Muscular ‘remote’ (uncommon) | |
| Multiple VSDs (uncommon) | |
| Non-cardiac anomaliesb | |
| Intracranial aneurysms | |
| Palatum anomaliesc | |
| Pancreas anomalies | |
| Parathyroid anomaliesd | |
| Phenylketonuria | |
| Thymus anomaliesc | |
| Tracheobronchial stenosisd |
Data derived from multiple sources [4, 5, 31, 50–55].
Possibly secondary characteristic.
Often caused by genetic/syndromic anomalies (see ‘Genetics of common arterial trunk’).
Associated with 22q11.2 deletion syndrome.
Associated with CHARGE syndrome.
ALM: anterolateral muscle; ASD: atrial septal defect; AV: atrioventricular; AVSD: atrioventricular septal defect; MAPCAs: major aortopulmonary collateral arteries; PA: pulmonary artery; PAPVC: partial anomalous pulmonary venous connection; PLSCV: persistent left superior caval vein; PMM: posteromedial muscle; RCA: right coronary artery; RCx: ramus circumflex; RPA: right pulmonary artery; VSD: ventricular septal defect.
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