Associazione Nazionale Medici Cardiologi Ospedalieri



Odd connections

Bellin Anna Mestre (Venezia) – Cardiologia Ospedale Dell’Angelo | Cutolo Ada Mestre (Venezia) – Cardiologia Ospedale Dell’Angelo | Vio Riccardo Mestre (Venezia) – Cardiologia Ospedale Dell’Angelo | Ronco Federico Mestre (Venezia) – Cardiologia Ospedale Dell’Angelo | Themistoclakis Sakis Mestre (Venezia) – Cardiologia Ospedale Dell’Angelo

A 40 yo man was admitted to the ER due to typical oppressive chest pain at rest; he had no previous medical history and he denied any cardiovascular risk factor, including family history of cardiac disease. He was actually a sportsman, practicing martial arts. 

The basal ECG showed sinus rhythm, and an early repolarization pattern with no pathological features. Physical examination revealed a continuous cardiac murmur (2/6) along the sternal border and no other pathological signs. Three consecutive HsTnI determinations resulted negative. He was evaluated by the cardiologist, and a bedside echocardiogram resulted unremarkable. 

Due to the typical characteristics of the symptoms, an exercise ECG was performed, and the results were suspicious for inducible ischemia at high workloads.

He was then admitted to our Cardiology ward, where he underwent a coronary angiography that showed a really surprising and unexpected anatomy: numerous coronary fistulas arising from various tracts (mid-LAD, intermediate coronary artery, conal branch of RCA, and distal Cx). A CT coronary angiogram was then performed, showing some ectasic vascular malformations between different coronary arteries, between coronaries and the pulmonary artery, and also a vascular anomaly connecting a bronchial artery with the right pulmonary artery. 

We subsequently performed a full body CT angiogram and a cerebral MR angiography, that excluded the presence of extrathoracic arteriovenous malformations. 

This case shows an extremely rare coronary congenital anomaly (accounting for 0.08–0.4% of all congenital heart disease), that generally goes underdetected in the first decades of life of these subjects. In this case, the typical anginal symptoms (probably due to coronary “steal” mechanism) arose suspicion that lead to the, albeit least expected, odd diagnosis. The treatment of symptomatic coronary fistulas is percutaneous/surgical closure. The patient was referred to our Tertiary Centre GUCH department, where a multidisciplinary discussion will decide the best therapeutic strategy.