The first patient is an 18-year-old female collegiate athlete who underwent an evaluation for exertional chest pain. She was diagnosed with anomalous aortic origin of the right coronary artery from the left coronary artery (Fig 1A). The right coronary artery had an interarterial course without intramural course on cross-sectional imaging. She has a history of mitral valve prolapse and premature ventricular contractions not requiring antiarrhythmic therapy. A dobutamine stress perfusion cardiac magnetic resonance did not reveal induced perfusion abnormalities; however, invasive stress testing using instantaneous wave-free ratio showed significantly impaired coronary flow during pharmacologic stress. The patient underwent a successful surgical transection and reimplantation of the right coronary artery to the right aortic sinus. The right coronary artery was noted to share its proximal course with the aortic wall where it was narrowed. Post-surgery, the reimplanted right coronary artery appeared patent and all provocative stress test results were normal (Fig 1B-C), but a 3-beat run of ventricular tachycardia was detected on Holter monitor 3 weeks after surgery (Fig 1D). Cardiac MRI revealed mitral valve prolapse and mitral annular disjunction with a disjunction distance of 4.4 mm (Fig 1E, Video 1), which could explain the ventricular tachycardia, given no evidence of myocardial ischaemia or myocardial scar (Fig 1E). A maximal treadmill test did not reveal evidence of ischaemia or sustained arrhythmia. No antiarrhythmic therapy was initiated following discussion with the patient. The patient was released to competitive sport participation and has been doing well since.

Figure 1.

An 18-year-old female with anomalous right coronary artery (RCA) from the left main coronary artery (LMCA), mitral annular disjunction (MAD), and mitral valve prolapse (MVP). (A) selective LMCA angiogram demonstrated anomalous aortic origin of the right coronary artery (R-AAOCA); (B) post-transection and reimplantation, the RCA now arises from the correct aortic sinus with patent ostium and caliber; (C) normal postoperative dobutamine stress perfusion cardiac MRI; (D) run of ventricular tachycardia; (E) MVP and MAD (MAD distance of 4.4 mm, measured between the posterior mitral valve hinge point and left ventricular myocardium); (F) No myocardial delayed enhancement.

The second patient was evaluated and managed at another institution. He was a previously healthy, 47-year-old man who was evaluated for recurrent chest pain while playing soccer and was diagnosed with ventricular tachycardia and anomalous aortic origin of the left coronary artery from the right aortic sinus with an intraseptal course. Cardiac MRI showed no myocardial scarring or fibrosis. He was suspected to have ischaemia-induced ventricular tachycardia and underwent an uneventful transconal unroofing of the left coronary artery at that centre. He continued to experience recurrent ventricular tachycardia 3 months after surgery. The patient underwent an electrophysiology study, during which monomorphic ventricular tachycardia was induced with double stimulus testing. Sustained ventricular fibrillation was also induced requiring external defibrillation (200J x 1) with successful return to sinus rhythm. He was started on Amiodarone and subsequently received a subcutaneous implantable cardioverter defibrillator. We reviewed the record as a second opinion consultation due to concern for postoperative ischaemia-induced ventricular arrhythmia. The postoperative coronary CTA showed patent left coronary artery ostium and its intraseptal course (Fig 2A). Upon review of the cardiac MRI images, mitral annular disjunction was identified on the cine of the long axis of the left ventricular outflow tract and its distance measured 8 mm at end systole (Fig 2B, Video 2). The patient did not have mitral valve prolapse. We recommended further provocative testing with a non-invasive stress perfusion imaging study and a cardiac catheterisation using instantaneous wave-free ratio. A maximal treadmill test did not reveal any sustained arrhythmia. A nuclear perfusion stress test, using single-photon emission CT, was performed and did not reveal an induced perfusion defect. A cardiac catheterisation has not been performed to date. He remains on amiodarone and has been doing well following cardiac surgery.

Figure 2.

A 47-year-old man with intraseptal, anomalous left coronary artery (LCA) from the right aortic sinus (L-AAOCA). (A) LCA ostium and caliber appear patent postoperatively; (B) Mitral annular disjunction (MAD) without mitral valve prolapse (MAD distance of 8 mm, measured between the posterior mitral valve hinge point and left ventricular myocardium).

We believe this is the first report of patients with ventricular arrhythmia who presented with both anomalous aortic origin of a coronary artery and mitral annular disjunction, two conditions known to be associated with sudden cardiac arrest/death. ^{Reference Doan, Zea-Vera and Agrawal1–Reference Ezzeddine, Siontis and Giudicessi3} While anomalous aortic origin of the coronary artery is a leading cause of sudden cardiac arrest/death in young athletes in the United States of America, presumed preceded by ventricular arrhythmia, the prevalence of mitral annular disjunction in patients with anomalous aortic origin of the coronary artery remains unknown. In anomalous aortic origin of the coronary artery, the risk of sudden cardiac arrest varies depending on the type of anomaly and may be mitigated by surgical intervention. Surgery may be considered in select patients with anomalous aortic origin of the left coronary artery with intraseptal course and patients with anomalous aortic origin of the right coronary artery, especially in those presenting with concerns for myocardial ischaemia. ^{Reference Doan, Zea-Vera and Agrawal1,Reference Doan, Sachdeva and Bonilla-Ramirez2} While mitral annular disjunction is known to be associated with ventricular tachycardia and sudden cardiac arrest, the management of mitral annular disjunction is not yet well defined, as the pathophysiology of malignant ventricular arrhythmia in this population remains unclear. ^{Reference Ezzeddine, Siontis and Giudicessi3} Cardiac MRI is essential in the diagnosis and assessment of mitral annular disjunction and anomalous aortic origin of a coronary artery. Mitral annular disjunction can be detected on a cine steady-state free precession of the long-axis left ventricular outflow tract. The disjunction distance can be measured at end systole between the hinge point of the posterior mitral valve leaflet and left ventricular myocardium. In patients with mitral valve prolapse, the posterior mitral valve leaflet hinge point should be tracked starting in diastole, so it can be carefully identified at end systole to avoid overestimation of disjunction distance. ^{Reference Doan and Morris4} The assessment using a stress perfusion imaging study to elicit induced ischaemia is an important step for risk stratification in patients with anomalous aortic origin of a coronary artery. ^{Reference Doan, Zea-Vera and Agrawal1,Reference Doan, Sachdeva and Bonilla-Ramirez2} It may be important to review cardiac MRI or echocardiographic images to assess for evidence of mitral annular disjunction, especially in patients who presented with ventricular tachycardia and sudden cardiac arrest. ^{Reference Ezzeddine, Siontis and Giudicessi3,Reference Doan and Morris4} Additionally, a phase-sensitive inversion recovery and T1 mapping sequences before and after contrast provide a comprehensive assessment of myocardial scarring and fibrosis.

In this manuscript, we presented two patients with anomalous aortic origin of a coronary artery who had ventricular arrhythmia both before and after anomalous aortic origin of the coronary artery surgeries. Mitral annular disjunction was detected on a retrospective review of the images and should be considered as a potential cause of ventricular arrhythmia when stress tests do not reveal induced perfusion abnormalities . Future research should include the prevalence of mitral annular disjunction in anomalous aortic origin of a coronary artery and their long-term follow-up to understand their risk for malignant arrhythmias.