Coronary artery disease in congenital single coronary artery in adults: A Dutch case series.
OBJECTIVE
To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery (SCA) in adults.


METHODS
We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013. Symptomatic patients who underwent routine diagnostic coronary angiography (CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed.


RESULTS
Fifteen (7 females) with a mean age of 58.5 ± 13.78 years (range 43-86) had a SCA. Conventional CAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients. Minimal to moderate coronary atherosclerotic changes were found in 4, and severe stenotic lesions in another 4 patients. Seven patients were free of coronary atherosclerosis. Runs of non-sustained ventricular tachycardia were documented in 2 patients, one of whom demonstrated transmural ischemic changes on presentation. Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery. Multi-slice computed tomography (MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery. Percutaneous coronary intervention was successfully performed in 3 patients. Eight patients were managed medically. Arterial bypass graft was performed in 4 patients with the squeezed SCA.


CONCLUSIONS
SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis. The availability and sophistication of MSCT facilitates the delineation of the course of a SCA. We present a Dutch case series and review of the literature.
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Salah Am
SA
Said
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Willem G
WG
de Voogt
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Suat
S
Bulut
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Jacques
J
Han
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Peter
P
Polak
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Rogier Lg
RL
Nijhuis
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Jeroen W
JW
Op den Akker
Salah AM Said, Rogier LG Nijhuis, Andries Slootweg, Department of Cardiology, Hospital Group Twente Almelo-Hengelo, 7555 DL Hengelo, The Netherlands.
Andries
A
Slootweg
Congenital heart disease; Coronary angiography; Coronary artery anomaly; Coronary artery disease; Multi-slice computed tomography; Single coronary artery