Response by Alexander et al to Letters Regarding Article, “A Shocking Development in a Young Male Athlete With Chest Pain”

In Response:

We thank Dr McGorrian and colleagues for their insights on our challenging case recently presented in Circulation.¹ Eosinophilic coronary periarteritis is an intriguing potential cause of our patient’s presentation. As the authors describe, the disease entity has been characterized in patients with coronary artery vasospasm and associated coronary dissection or sudden cardiac death. As in our case, some may have a preexisting history of atopy. The most distinguishing features of eosinophilic coronary periarteritis are appreciated by histology, which reveals eosinophilic infiltration isolated to the adventitia and vasa vasorum of the epicardial coronary arteries.² Thus, this diagnosis is difficult to confirm outside of autopsy.

Some features of our case make eosinophilic coronary periarteritis less likely to be the main contributor to his presentation. On coronary angiography, there was no evidence of aneurysmal changes, which have been described for other cases of coronary periarteritis.³ Similarly, his serum C-reactive protein level, a marker of inflammation, albeit nonspecific, was normal. His case was striking for the large burden of premature coronary artery disease and potential genetic predisposition for aggressive atherosclerosis, as suggested by markedly elevated lipoprotein (a) levels in the patient and multiple first-degree relatives. Nevertheless, a recent report describes a patient with concomitant coronary artery disease and eosinophilic coronary periarteritis.⁴ Therefore, it is possible that our patient could also have had underlying periarteritis that acted synergistically to cause endothelial dysfunction and subsequent coronary vasospasm.

Increasing awareness of eosinophilic coronary periarteritis as a potential cause of coronary vasospasm is warranted, as these patients may benefit from adding immunosuppressive therapy to a standard antivasospasm regimen. Pursing further diagnostic tests in coronary vasospasm patients, such as computed tomography coronary angiography with positron emission tomography or intravascular ultrasound, may prove useful for identifying individuals with underlying periarteritis.³

We also thank Dr Bairey Merz and colleagues for their interest and thoughtful comments on our case. We agree that coronary artery vasospasm represents a wide spectrum of disease, requiring precise definitions and prognostic tools to devise optimal treatment strategies. Potentially, sex, race/ethnicity, genetic factors, and the specific cause of coronary spasm all affect prognosis and, in part, explain the variability in prognosis described in the literature. The standardized diagnostic criteria proposed by COVADIS (Coronary Vasomotor Disorders International Study Group) and recently developed clinical risk score are important initial steps.^5,6 An essential goal for future prospective studies using these tools is the ability to identify patients, like ours, who have a high risk of cardiovascular complications despite optimal medical management. Moreover, delineating indications for advanced interventions (eg, implantable cardioverter defibrillator implantation) will be imperative. We look forward to seeing future data from this study group.