Absent arm pulses and early-onset coronary disease
Tarkin JM 1,2 , Gopalan D 3 , Mason JC 1
1 National Heart & Lung Institute, Imperial College London
2 Division of Cardiovascular Medicine, University of Cambridge
3 Department of Radiology, Imperial College Healthcare NHS Trust
A 28-year-old woman presented with intermittent palpitations and atypical chest pain. She had been admitted to the intensive care unit at the age of 2 years with critical left arm ischemia, and subsequently developed chronic arm pain and weakness triggered by exercise. Past medical history was significant for postoperative pulmonary embolism, for which she received long-term oral anticoagulation. Her only other medication was aspirin once daily. She was a non-smoker, with no known risk factors for atherosclerotic coronary artery disease. There were no constitutional symptoms, rashes, joint pains, or jaw claudication. On examination, peripheral pulses were absent in the upper limbs, and blood pressure was un-recordable in the arms. Cardio-respiratory examination was otherwise normal.
Blood investigations showed a mildly elevated cholesterol profile (total cholesterol 4.8 mmol/L, LDL cholesterol 3.1 mmol/L, total:HDL cholesterol ratio 4.1), Lipoprotein (a) 40 nmol/L (NR <35), and C-reactive protein 2 mg/L (NR<4). Serum immunoglobulin levels were normal, and antineutrophil cytoplasmic antibody (ANCA) screen was negative. Electrocardiogram and transthoracic echocardiogram were also within normal limits. A seven-day cardiac event recorder revealed sinus rhythm throughout, with occasional atrial ectopic beats. Exercise tolerance test, stopped due to fatigue after achieving 12 METs at 10 minutes of standard Bruce protocol, demonstrated no ST-segment changes or arrhythmia. Dobutamine stress echocardiography showed no stress-induced wall motion abnormality, but did, however, provoke mild chest pain. CT coronary angiography is shown in Figures 1 and 2.
Figure 1.CT coronary angiogram images orientated in axial plane, at the level of the (a) left and (b) right coronary ostia, showing coronary arterial abnormalities within the proximal left and right coronary arteries (arrows).
Figure 2. CT coronary angiogram images in 3D multi-planar reconstruction showing (a) the origins of the right coronary artery (RCA) and left anterior descending (LAD) artery, the (b) length of the RCA and proximal-mid left circumflex artery (LCx), and (c) the proximal-mid LAD.
- Name the abnormalities demonstrated in figures 1 and 2?
- Calcified coronary artery aneurysms associated with calcified mild (<50%) LAD and RCA stenoses
- Calcified coronary artery aneurysms associated with calcified mild (<50%) LCx and RCA stenoses
- Calcified coronary artery aneurysms associated with non-calcified moderate (>50%) LAD and severe (>70%) RCA stenoses
- Non-calcified coronary artery aneurysms associated with calcified mild (<50%) LAD and RCA stenoses
- Non-calcified coronary artery aneurysms associated with non-calcified moderate (>50%) LCx and severe (>70%) RCA stenoses
- What is the most likely diagnosis?
- Early-onset atheroscosclerosis because of familial hypercholesterolaemia
- Late complications of Kawasaki’s disease
- Granulomatosis with polyangiitis
- Active large-vessel giant cell arteritis
- Takayasu’s disease in clinical remission
- What is the best course of management?
- Conservative, symptom-guided management of stable coronary artery disease
- High-intensity statins and lipid apheresis
- High-dose steroids and tocilizumab
- Dual anti-platelet therapy and percutaneous coronary intervention
- Coronary artery bypass grafting surgery
Q1, answer C. CT coronary angiography shows densely calcified coronary aneurysms involving the left main stem and RCA ostia (Figure 3a). In addition, there is a moderate non-calcified stenosis of the proximal LAD (50-70%), and a severe (>70%) RCA stenosis at the neck of the RCA aneurysm, with normal appearances of these vessels beyond. The LCx is disease free.
Q2, answer E. Takayasu’s arteritis is a rare systemic inflammatory disease of unknown aetiology, which typically affects young females and results in large-vessel vasculitis characterised by granulomatous inflammation of the aorta and great vessels, as well as the coronary and renal arteries. Arterial narrowing or occlusion occurs in up to 90% of patients, often resulting in symptoms of limb claudication. Hence, Takayasu’s arteritis is known as the ‘pulseless’ disease. Aneurysms are also common. In this patient, there was a history of acute limb ischemia in childhood, as well as symptoms of arm claudication and absent upper limb pulses. MR angiography demonstrated bilateral subclavian artery occlusions, proximal right vertebral artery occlusion, and no abnormalities of the aorta, innominate or carotid arteries (Figure 3b). While Kawasaki’s disease and granulomatosis with polyangiitis vasculitis can affect the coronary arteries, these conditions are not typically associated with subclavian artery occlusion. Granulomatosis with polyangiitis vasculitis is also an ANCA-associated vasculitis. The lack of constitutional symptoms and normal C-reactive protein level suggest disease in remission. Takayasu’s arteritis is associated with accelerated atherosclerosis; there are no features in this case to indicate a diagnosis of familial hypercholesterolemia.
Figure 3. (a) CT angiography in 3D volumetric reconstruction showing calcified bilateral coronary aneurysms (arrows); (b) MR angiography showing bilateral subclavian artery occlusions (solid arrows) and right vertebral artery occlusion (dashed arrow).
Q3, answer A. Management of coronary artery disease in patients with Takayasu’s arteritis can be challenging, particularly when there are ostial lesions. There have been no large randomised clinical trials evaluating outcomes of percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG) surgery in this setting. Small retrospective analyses indicate that CABG surgery may be associated with better long-term outcomes than PCI in patients with Takayasu’s arteritis. On the whole, coronary revascularisation is best avoided during an acute flare of the disease due to the friable nature of the inflamed vessels, and the risk of graft stenosis at the aortic insertion site. There may also be an increased risk of restenosis following PCI in patients with vasculitis-associated coronary artery disease, compared to stable atherosclerosis. When CABG surgery is needed, saphenous vein grafts are typically used because of possible inflammatory involvement of the internal thoracic arteries. In this patient, the clinical symptoms were not typical of effort-induced angina due to obstructive epicardial coronary artery disease, and non-invasive stress-imaging excluded significant inducible myocardial ischaemia that might indicate a need for coronary revascularisation. There were also no signs of active vasculitis that would require immunosuppression. The patient was managed conservatively, with a plan to monitor closely and consider further coronary investigations based on future clinical symptoms.