LETTERS TO THE EDITOR
may be to correct vitamin D deficiency.2 New lipid-lowering drugs may also prove useful in reducing the risk of SIM altogether.6 Statin discontinuation (or reducing statin dosage) is a very relevant clinical issue because it may increase the risk of cardiovascular events.7 Therefore, we need to continue our efforts to provide optimal treatment of hyperlipidemia while avoiding (or appropriately treating) SIM. Maciej Banach, MD Medical University of Lodz Lodz, Poland
Dimitri P. Mikhailidis, MD University College London London, United Kingdom
Type A Aortic Dissection in Fibromuscular Dysplasia To the Editor: Fibromuscular dysplasia (FMD) is a rare, noninflammatory vascular disease characterized by arterial stenosis and aneurysm or dissection most commonly affecting renal, carotid, and vertebral arteries.1 Although multiple arterial involvement has been described in the literature,2-5 no surveillance strategies are in place, which often results in catastrophic consequences. Report of a Case. A 70-year-old woman presented with severe, nonexertional substernal chest pain radiating to the right shoulder. She had
a history of FMD, subarachnoid hemorrhage in 2001, ischemic stroke in 2011, and well-controlled hypertension. Transesophageal echocardiography performed in 2011 for stroke work-up had revealed a borderline dilatation of the ascending aorta measuring 3.5 cm. Her pertinent physical examination results included blood pressure of 136/76 mm Hg, equivalent in both arms, and normal S1 and S2 heart sounds without murmur. Electrocardiography revealed ST-segment depression and new T-wave flattening in the lateral leads. Echocardiography illustrated normal left ventricular function with a dilated aortic root (4.3 cm). Urgent cardiac catheterization and
On behalf of the Lipid and Blood Pressure Meta-analysis Collaboration (LBPMC) Group
1. Banach M, Serban C, Sahebkar A, et al; Lipid and Blood Pressure Meta-analysis Collaboration Group. Effects of coenzyme Q10 on statininduced myopathy: a meta-analysis of randomized controlled trials. Mayo Clin Proc. 2015; 90(1):24-34. 2. Michalska-Kasiczak M, Sahebkar A, Mikhailidis DP, et al; Lipid and Blood Pressure Meta-analysis Collaboration (LBPMC) Group. Analysis of vitamin D levels in patients with and without statin-associated myalgiada systematic review and meta-analysis of 7 studies with 2420 patients. Int J Cardiol. 2015;178: 111-116. 3. Vrablik M, Zlatohlavek L, Stulc T, et al. Statin-associated myopathy: from genetic predisposition to clinical management. Physiol Res. 2014;63(suppl 3): S327-S334. 4. Garrido-Maraver J, Cordero MD, Oropesa-Ávila M, et al. Coenzyme Q10 therapy. Mol Syndromol. 2014; 5(3-4):187-197. 5. Cannon CP. IMPROVE-IT Trial: a comparison of ezetimibe/simvastatin versus simvastatin monotherapy on cardiovascular outcomes after acute coronary syndromes. Paper presented at: American Heart Association 2014 Scientific Sessions; November 17, 2014; Chicago, IL. 6. Dragan S, Serban MC, Banach M. Proprotein convertase subtilisin/kexin 9 inhibitors: an emerging lipid-lowering therapy [published online ahead of print June 17, 2014]? J Cardiovasc Pharmacol Ther. http://dx.doi.org/10.1177/1074248414539562. 7. Tziomalos K, Athyros VG, Mikhailidis DP. Statin discontinuation: an underestimated risk? Curr Med Res Opin. 2008;24(11):3059-3062.
FIGURE. Diagnostic studies in a 70-year-old woman with severe, nonexertional substernal chest pain radiating to the right shoulder. Urgent cardiac catheterization and aortography revealed aortic root dilatation without aortic insufficiency (A, B) and a possible large ascending aortic aneurysm. Computed tomographic angiography of the thorax revealed type A aortic dissection (C, D).
http://dx.doi.org/10.1016/j.mayocp.2015.01.003 Mayo Clin Proc. n March 2015;90(3):415-422 www.mayoclinicproceedings.org
421
MAYO CLINIC PROCEEDINGS
aortography revealed a dilated aorta with a possible large ascending aortic aneurysm but no aortic insufficiency (Figure, A and B). Computed tomographic angiography of the thorax revealed a type A aortic dissection (Figure, C and D) just beyond the coronary arteries with an extension into the arch associated with hemopericardium and hemomediastinum. The patient subsequently underwent successful repair of the dissection with hemiarch reconstruction, and after a prolonged intensive care unit stay, she was discharged with a treatment regimen of aspirin and clopidogrel. Results of pathologic studies were consistent with aortic dissection as evidenced by blood cells dissecting into the middle of the elastic layers. Discussion. In clinical practice, the diagnosis of FMD is usually established via angiographic findings. Quite frequently, these findings are coincidental and can potentially be missed, resulting in delayed treatment and serious consequences. The rarity of the disease, lack of awareness in clinical practice, and vague presentations often result in misdiagnosis. In the United States Registry for Fibromuscular Dysplasia, patients with FMD had a high incidence of family history of stroke, aneurysms, and sudden deaths that may have been related to an undetected FMD.4 Fibromuscular dysplasia
422
may actually be more common than previously thought because there is strong reliance on subtle angiographic findings for diagnosis. Multiple arterial involvement is present in 1 in 4 patients with FMD,2 which is often difficult to predict. Frequently, FMD patients are not screened for multiple arterial involvement, which can lead to catastrophic presentations as occurred in our patient. In our patient, a borderline aortic dilatation was coincidentally found 3 years before the current presentation, but there are no recommendations regarding followup of such cases. Angiography of all the vascular beds is, of course, not feasible, but some options could include finding a pattern of vasculature involvement in patients observed over time, correlation of a particular histologic subtype with vessel involvement, defining co-morbidities correlating with higher risk, and close observation of aneurysms. Conclusion. We describe an extremely rare association of type A aortic dissection in a patient with FMD. In fact, aortic involvement was not reported in any of the patients in the United States Registry for Fibromuscular Dysplasia. Most of the current knowledge regarding FMD is based on case reports, and there is a dire need for a largescale case study to delineate appropriate follow-up recommendations.
Gaurang N. Vaidya, MBBS SUNY Upstate Medical University Syracuse, NY
Danish S. Siddiqui, MD Loma Linda University Health Care System Loma Linda, CA 1. Olin JW, Gornik HL, Bacharach JM, et al; American Heart Association Council on Peripheral Vascular Disease; American Heart Association Council on Clinical Cardiology; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; American Heart Association Council on Cardiovascular Disease in the Young; American Heart Association Council on Cardiovascular Radiology and Intervention; American Heart Association Council on Epidemiology and Prevention; American Heart Association Council on Functional Genomics and Translational Biology; American Heart Association Council for High Blood Pressure Research; American Heart Association Council on the Kidney in Cardiovascular Disease; American Heart Association Stroke Council. Fibromuscular dysplasia: state of the science and critical unanswered questions; a scientific statement from the American Heart Association. Circulation. 2014;129(9):1048-1078. 2. Unlü C, van den Heuvel DA, Leeuwis JW, de Vries JP. Ruptured aneurysm of the splenic artery associated with fibromuscular dysplasia. Ann Vasc Surg. 2014;28(7):1799.e15-18. 3. Escárcega RO, Mathur M, Franco JJ, et al. Nonatherosclerotic obstructive vascular diseases of the mesenteric and renal arteries. Clin Cardiol. 2014; 37(11):700-706. 4. Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012; 125(25):3182-3190. 5. Kar S, Gopaldas RR, Kumar A. Acute aortic dissection and stroke in multivessel fibromuscular dysplasia. Tex Heart Inst J. 2013;40(1):88-90.
http://dx.doi.org/10.1016/j.mayocp.2015.01.004
Mayo Clin Proc. n March 2015;90(3):415-422 www.mayoclinicproceedings.org
may be to correct vitamin D deficiency.2 New lipid-lowering drugs may also prove useful in reducing the risk of SIM altogether.6 Statin discontinuation (or reducing statin dosage) is a very relevant clinical issue because it may increase the risk of cardiovascular events.7 Therefore, we need to continue our efforts to provide optimal treatment of hyperlipidemia while avoiding (or appropriately treating) SIM. Maciej Banach, MD Medical University of Lodz Lodz, Poland
Dimitri P. Mikhailidis, MD University College London London, United Kingdom
Type A Aortic Dissection in Fibromuscular Dysplasia To the Editor: Fibromuscular dysplasia (FMD) is a rare, noninflammatory vascular disease characterized by arterial stenosis and aneurysm or dissection most commonly affecting renal, carotid, and vertebral arteries.1 Although multiple arterial involvement has been described in the literature,2-5 no surveillance strategies are in place, which often results in catastrophic consequences. Report of a Case. A 70-year-old woman presented with severe, nonexertional substernal chest pain radiating to the right shoulder. She had
a history of FMD, subarachnoid hemorrhage in 2001, ischemic stroke in 2011, and well-controlled hypertension. Transesophageal echocardiography performed in 2011 for stroke work-up had revealed a borderline dilatation of the ascending aorta measuring 3.5 cm. Her pertinent physical examination results included blood pressure of 136/76 mm Hg, equivalent in both arms, and normal S1 and S2 heart sounds without murmur. Electrocardiography revealed ST-segment depression and new T-wave flattening in the lateral leads. Echocardiography illustrated normal left ventricular function with a dilated aortic root (4.3 cm). Urgent cardiac catheterization and
On behalf of the Lipid and Blood Pressure Meta-analysis Collaboration (LBPMC) Group
1. Banach M, Serban C, Sahebkar A, et al; Lipid and Blood Pressure Meta-analysis Collaboration Group. Effects of coenzyme Q10 on statininduced myopathy: a meta-analysis of randomized controlled trials. Mayo Clin Proc. 2015; 90(1):24-34. 2. Michalska-Kasiczak M, Sahebkar A, Mikhailidis DP, et al; Lipid and Blood Pressure Meta-analysis Collaboration (LBPMC) Group. Analysis of vitamin D levels in patients with and without statin-associated myalgiada systematic review and meta-analysis of 7 studies with 2420 patients. Int J Cardiol. 2015;178: 111-116. 3. Vrablik M, Zlatohlavek L, Stulc T, et al. Statin-associated myopathy: from genetic predisposition to clinical management. Physiol Res. 2014;63(suppl 3): S327-S334. 4. Garrido-Maraver J, Cordero MD, Oropesa-Ávila M, et al. Coenzyme Q10 therapy. Mol Syndromol. 2014; 5(3-4):187-197. 5. Cannon CP. IMPROVE-IT Trial: a comparison of ezetimibe/simvastatin versus simvastatin monotherapy on cardiovascular outcomes after acute coronary syndromes. Paper presented at: American Heart Association 2014 Scientific Sessions; November 17, 2014; Chicago, IL. 6. Dragan S, Serban MC, Banach M. Proprotein convertase subtilisin/kexin 9 inhibitors: an emerging lipid-lowering therapy [published online ahead of print June 17, 2014]? J Cardiovasc Pharmacol Ther. http://dx.doi.org/10.1177/1074248414539562. 7. Tziomalos K, Athyros VG, Mikhailidis DP. Statin discontinuation: an underestimated risk? Curr Med Res Opin. 2008;24(11):3059-3062.
FIGURE. Diagnostic studies in a 70-year-old woman with severe, nonexertional substernal chest pain radiating to the right shoulder. Urgent cardiac catheterization and aortography revealed aortic root dilatation without aortic insufficiency (A, B) and a possible large ascending aortic aneurysm. Computed tomographic angiography of the thorax revealed type A aortic dissection (C, D).
http://dx.doi.org/10.1016/j.mayocp.2015.01.003 Mayo Clin Proc. n March 2015;90(3):415-422 www.mayoclinicproceedings.org
421
MAYO CLINIC PROCEEDINGS
aortography revealed a dilated aorta with a possible large ascending aortic aneurysm but no aortic insufficiency (Figure, A and B). Computed tomographic angiography of the thorax revealed a type A aortic dissection (Figure, C and D) just beyond the coronary arteries with an extension into the arch associated with hemopericardium and hemomediastinum. The patient subsequently underwent successful repair of the dissection with hemiarch reconstruction, and after a prolonged intensive care unit stay, she was discharged with a treatment regimen of aspirin and clopidogrel. Results of pathologic studies were consistent with aortic dissection as evidenced by blood cells dissecting into the middle of the elastic layers. Discussion. In clinical practice, the diagnosis of FMD is usually established via angiographic findings. Quite frequently, these findings are coincidental and can potentially be missed, resulting in delayed treatment and serious consequences. The rarity of the disease, lack of awareness in clinical practice, and vague presentations often result in misdiagnosis. In the United States Registry for Fibromuscular Dysplasia, patients with FMD had a high incidence of family history of stroke, aneurysms, and sudden deaths that may have been related to an undetected FMD.4 Fibromuscular dysplasia
422
may actually be more common than previously thought because there is strong reliance on subtle angiographic findings for diagnosis. Multiple arterial involvement is present in 1 in 4 patients with FMD,2 which is often difficult to predict. Frequently, FMD patients are not screened for multiple arterial involvement, which can lead to catastrophic presentations as occurred in our patient. In our patient, a borderline aortic dilatation was coincidentally found 3 years before the current presentation, but there are no recommendations regarding followup of such cases. Angiography of all the vascular beds is, of course, not feasible, but some options could include finding a pattern of vasculature involvement in patients observed over time, correlation of a particular histologic subtype with vessel involvement, defining co-morbidities correlating with higher risk, and close observation of aneurysms. Conclusion. We describe an extremely rare association of type A aortic dissection in a patient with FMD. In fact, aortic involvement was not reported in any of the patients in the United States Registry for Fibromuscular Dysplasia. Most of the current knowledge regarding FMD is based on case reports, and there is a dire need for a largescale case study to delineate appropriate follow-up recommendations.
Gaurang N. Vaidya, MBBS SUNY Upstate Medical University Syracuse, NY
Danish S. Siddiqui, MD Loma Linda University Health Care System Loma Linda, CA 1. Olin JW, Gornik HL, Bacharach JM, et al; American Heart Association Council on Peripheral Vascular Disease; American Heart Association Council on Clinical Cardiology; American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; American Heart Association Council on Cardiovascular Disease in the Young; American Heart Association Council on Cardiovascular Radiology and Intervention; American Heart Association Council on Epidemiology and Prevention; American Heart Association Council on Functional Genomics and Translational Biology; American Heart Association Council for High Blood Pressure Research; American Heart Association Council on the Kidney in Cardiovascular Disease; American Heart Association Stroke Council. Fibromuscular dysplasia: state of the science and critical unanswered questions; a scientific statement from the American Heart Association. Circulation. 2014;129(9):1048-1078. 2. Unlü C, van den Heuvel DA, Leeuwis JW, de Vries JP. Ruptured aneurysm of the splenic artery associated with fibromuscular dysplasia. Ann Vasc Surg. 2014;28(7):1799.e15-18. 3. Escárcega RO, Mathur M, Franco JJ, et al. Nonatherosclerotic obstructive vascular diseases of the mesenteric and renal arteries. Clin Cardiol. 2014; 37(11):700-706. 4. Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012; 125(25):3182-3190. 5. Kar S, Gopaldas RR, Kumar A. Acute aortic dissection and stroke in multivessel fibromuscular dysplasia. Tex Heart Inst J. 2013;40(1):88-90.
http://dx.doi.org/10.1016/j.mayocp.2015.01.004
Mayo Clin Proc. n March 2015;90(3):415-422 www.mayoclinicproceedings.org
