A 48-year old male with a history of early childhood Hodgkin's lymphoma treated with mantle field radiation therapy presented with a late-peaking systolic murmur, chest pressure and worsening dyspnea. Echocardiography revealed severe aortic stenosis (AS) with a mean transaortic gradient of 61mmHg and extensive left ventricular outflow tract (LVOT) calcifications and underdevelopment. For anatomic reasons he was found not to be an appropriate surgical or transcatheter aortic valve replacement candidate. Thus he underwent workup for potential apico-aortic conduit surgery including coronary angiography. He was found to have significant ostial left and right coronary artery stenosis and underwent percutaneous coronary intervention and stenting. Returning a few months later for definitive surgery he reported of recurrent anginal symptoms and therefore had repeat coronary angiography.
Diagnostic invasive coronary angiography proved difficult, and selective engagement of the right coronary artery (RCA) was unsuccessful despite multiple attempts. A nonselective injection revealed opacification of the RCA from the left side of the aortic root, raising the possibility of an anomalous coronary artery.
Coronary CTA was then requested which demonstrated angulation of the proximal RCA and ostial RCA stent by dense bulky calcification within the right sinus of Valsalva. The sinus of Valsalva contained a channel between the calcifications and aortic cusp which mimicked the appearance of the anomalous coronary on nonselective invasive coronary angiography. There was also evidence of in-stent restenosis. The aortic root as well as the proximal pulmonary artery were severely calcified and narrowed (i.e. "porcelain changes") consistent with radiotherapy changes.
Coronary artery disease (CAD) typically occurs 10 to 15 years after radiation therapy in patients treated with doses exceeding 30 Gray before the age of 20.1 Radiation-induced stenosis generally affects the ostia and proximal coronary arteries, which lie within the anatomic radiotherapy field. Survivors of Hodgkin's disease bear a high relative risk for coronary artery disease2 and coronary CTA is highly sensitive for the changes following thoracic radiation therapy, allowing early diagnosis of CAD.3 Dense calcifications of the ascending aorta result from deposition of calcium due to intimal or medial scarring after radiation-induced aortitis.1 Other radiation-associated complications include stenosis, occlusion or pseudoaneurysm of the great vessels, pericardial effusion, constrictive pericarditis, valvular disease, and myocardial fibrosis.
In this case, coronary CTA enabled accurate depiction of the atypical calcification of the aortic lumen, the stented origin of the RCA, and assessment of the suspected in-stent restenosis. The RCA restenosis was treated by repeat percutaneous coronary intervention. Following, the patient had successful apico-aortic conduit surgery to relieve the aortic stenosis outflow obstruction. This approach avoided the potential morbidity and mortality associated with a full sternotomy in the setting of extensive radiation associated changes, as well as the risk of manipulation of the calcified LVOT and aortic root.