e-Herz: Case study

Herz 2012 · 37:231–233 D.M. Konstantinou1 · Y.S. Chatzizisis1 · G. Farmakis2 · I. Styliadis1 · G.D. Giannoglou1 DOI 10.1007/s00059-011-3442-7 1 1st Department, AHEPA University Hospital, Received: 10 February 2011 Aristotle University Medical School, Thessaloniki Accepted: 28 February 2011 2 Published online: 14 May 2011 Klinik für Nuklearmedizin, Universitätsklinikum des Saarlandes, Homburg/Saar © Urban & Vogel 2011 embolization e-Herz syndrome following during acute myocardial

Introduction therapy, β-blocker, , and transder- the aortic plaques. To confirm the cho- mal nitrates. lesterol embolization, a deep cutaneous The cholesterol embolization syndrome Clinical examination was unremark- of the fifth digit of the right foot (CES) is the result of atherosclerot- able. Of note, peripheral pulsation was was carried out which revealed choles- ic plaque erosion and subsequent dis- normal without any evident bruits. Chest terol crystals in the lumen of the small lodgement of cholesterol crystals from X-ray and ECG revealed normal find- diameter of the skin (. Fig. 1d). the core of the plaque to the peripheral ings. The laboratory examinations on The skin lesions and the renal func- arteries. The source of emboli is usual- admission showed an increased eryth- tion were progressively deteriorated and ly located in the , whereas the most rocyte sedimentation rate (88 mm/h), the patient had two of his toes amputated commonly affected organs are the skin moderately increased count of eosino- due to . After 30 days of hospi- and the kidneys. CES has gained increas- phils (472 per mm3) and elevated serum talization, the patient had a second infe- ing interest as it is considered a compli- and levels (77 mg/dl and rior ST-elevation myocardial infarction cation of invasive vascular procedures. 2.7 mg/dl, respectively). followed by cardiac arrest and death. In a large series in 1,786 consecutive pa- On the basis of clinical presentation, tients who underwent left cardiac cath- the patient was admitted with the diag- Discussion eterization the incidence of post-proce- nosis of lower extremities embolization dural CES was 1.4% [1]. syndrome. To further investigate the Patients with CES have usually complex, source of emboli the patient underwent unstable atherosclerotic lesions over the Case presentation a transesophageal echocardiography entire thoracic aorta [2] Male gender, ad- which revealed an overall normal cardi- vanced age, acute coronary syndromes, A 69-year-old male, obese patient pre- ac anatomy and preserved left ventricu- multivessel coronary disease, hy- sented to the emergency department of lar function. However, diffuse, ulcerat- pertension, smoking, cerebrovascular our center with bilateral painful, cya- ed atherosclerotic lesions in the course disease and aortic have been notic discoloration of his toes which de- of descending thoracic aorta were vi- recognized as major risk factors for CES veloped gradually over the past 20 days sualized. An enhanced magnetic reso- [1, 3]. A triggering event for the clinical (. Fig. 1a). No symptoms of intermit- nance aortography using gadolinium- development of the syndrome includes tent were mentioned. The DTPA further confirmed the echocar- invasive procedures of the aorta or large patient was an ex-smoker and had a his- diographic findings revealing the pres- arteries, such as , angioplas- tory of essential , dyslipid- ence of multiple, ulcerated atheroscle- ty or peripheral vascular procedures, an- emia, and chronic renal insufficiency. rotic plaques in the descending aor- ticoagulation therapy, and thrombolysis About 1 month ago the patient had an ta (. Fig. 1b,c). No obstructing lesions [3, 4]. In our case, male gender, advanced inferior ST-elevation myocardial infarc- were found between the aorta and the age, and a history of hypertension, dys- tion treated with thrombolysis. No car- site of distal embolization. These find- lipidemia, and smoking were present, diac catheterization was performed at ings led to the clinical suspicion of em- whereas the patient underwent throm- that time and the patient was discharged bolization of the of the toes bolysis 1 month previously. Both trans- from the hospital on dual antiplatelet from cholesterol crystals derived from esophageal echocardiography and mag-

Herz 2 · 2012 | 231 e-Herz: Case study

a b c

Fig. 1 9 a Blue toes, b gad- olinium-enhanced mag- netic resonance aortogra- phy showing irregular walls and atherosclerotic degen- eration of the thoracic aor- ta (arrow) without any ob- structing lesions between e d the aorta and the site of embolization, c cross-sec- 8 tional view of the athero- sclerotic aorta at the sec-

6 tion indicated with the black line in b (asterisk de- notes the plaque and 4 dashed lines the lumen), d the skin biopsy specimen 2 contained cholesterol crys- tals within the occluded serum creatinine (mg/dL) 0 small vessel (arrows: cho- 1 2 3 4 5 6 7 8 9 1011 12131415 161718 192021 lesterol crystals), e in-hos- hospital stay (days) pital course of serum creat- inine levels

netic resonance aortography demon- cluding conservative, invasive or surgi- ered as a candidate for any invasive or strated a complex, ulcerated, atheroscle- cal measures; however, none of them has surgical therapeutic procedure because rotic plaque in the course of descending been tested in the clinical setting. Most of the diffuse distribution of atheroscle- thoracic aorta. authors agree that withdrawal of anti- rotic plaques along the course of the de- The most common clinical findings coagulants is beneficial [5]. Corticoste- scending aorta. of the syndrome are cutaneous lesions, roids have been also used in many cases such as , blue toe syn- either as monotherapy [6] or in combi- Conclusion drome or digital gangrene more often af- nation with [5] or cyclophospha- fecting lower extremities, renal failure, mide therapy aiming to reduce the in- CES is an under-diagnosed clinical enti- and [3]. Considering renal flammatory burden of the affected cap- ty which is often related with increased involvement three types of atheroem- illaries [7]. Statins are also considered in-hospital mortality. Since treatment bolic renal disease have been described as a key therapeutic measure. The ra- options are limited without proven ef- [4]. Our patient fulfilled the criterion of tionale behind statin treatment is that ficacy, increased awareness by the clini- skin lesions and mild eosinophilia. Al- these agents apart from LDL-lowering cians is needed. Except for statins, treat- though the patient’s baseline serum cre- properties demonstrated the ability of ment is mainly supportive and close atinine levels were already elevated due stabilizing vulnerable plaques and de- monitoring of renal function is required. to preexisting chronic renal insuffi- creasing plaque [1]. Our ciency, during the course of the hospi- patient was initially treated with ator- Corresponding address tal stay, a further deterioration in renal vastatin 40 mg which subsequently ti- D.M. Konstantinou function occurred (. Fig. 1e). Accord- trated to 80 mg daily. For patients hav- 1st Cardiology Department, AHEPA University Hospital, ing to the pattern of renal impairment, ing focal stenoses, angioplasty and stent Aristotle University Medical School our case matches the second type of ath- placement has been reported to be effec- 1 St. Kyriakidi Str., 54636 Thessaloniki eroembolic renal disease which is char- tive with a good mid-term prognosis [8]. Greece acterized by a subacute time course with Filter-assisted angioplasty has been also [email protected] a gradual deterioration of renal func- applied in order to ensure that no fur- tion and with longer latent period of few ther embolization will take place during Conflict of interest. The corresponding author states that there are no conflicts of interest. weeks from the triggering event. the procedure [9]. Surgical repair of the Currently, there is no widely accept- atheromatous aorta by passing the dis- able treatment for CES. Various thera- eased area has also been described [10]. peutic strategies have been proposed in- However, our patient was not consid-

232 | Herz 2 · 2012 Abstract · Zusammenfassung

References Herz 2012 · 37:231–233 DOI 10.1007/s00059-011-3442-7 © Urban & Vogel 2011 1. Fukumoto Y, Tsutsui H, Tsuchihashi M et al (2003) The incidence and risk factors of cholesterol em- D.M. Konstantinou · Y.S. Chatzizisis · G. Farmakis · I. Styliadis · G.D. Giannoglou bolization syndrome, a complication of cardi- Cholesterol embolization syndrome following ac catheterization: a prospective study. J Am Coll Cardiol 42(2):211–216 thrombolysis during acute myocardial infarction 2. Machino-Ohtsuka T, Seo Y, Ishizu T et al (2010) Combined assessment of carotid vulnerable Abstract plaque, renal insufficiency, eosinophilia, and hs- Background. Cholesterol embolization syn- plaque in the course of descending thoracic CRP for predicting risky aortic plaque of choles- drome (CES) is the result of atherosclerot- aorta, while a skin biopsy of the cyanotic toes terol crystal . Circ J 74:51–58 ic plaque erosion and subsequent dislodge- revealed cholesterol crystals in the lumen of 3. Funabiki K, Masuoka H, Shimizu H et al (2003) ment of cholesterol crystals from the core the small diameter arteries. Cholesterol crystal embolization (CCE) after car- of the plaque to the peripheral arteries. The Conclusion. Cholesterol embolizations from diac catheterization. A case report and a review source of emboli is usually located in the aor- the aorta are difficult to treat and may end of 36 cases in the Japanese literature. Jpn Heart J 44(5):767–774 ta, whereas the most commonly affected or- in renal failure. Since treatment options are 4. Scolari F, Tardanico R, Zani R et al (2000) Choles- gans are the skin and the kidneys. limited without proven efficacy, increased terol crystal embolism: a recognizable cause of Case report. The case of a 69-year-old male awareness by the clinicians is needed. renal disease. Am J Dis 36(6):1089–1109 with cyanotic painful discoloration of his 5. Matsumura T, Matsumoto A, Ohno M et al (2006) toes following thrombolysis for acute myo- Keywords A case of cholesterol embolism confirmed by cardial infarction 1 month previously is pre- Cholesterol embolization skin biopsy and successfully treated with statins sented. Both transesophageal echocardiog- syndrome · Cyanotic toes · and steroids. Am J Med Sci 331(5):280–283 6. Nakayama M, Nagata M, Hirano T et al (2006) raphy and magnetic resonance aortography Thrombolysis · Atheroembolic renal disease Low-dose prednisolone ameliorates acute renal showed a diffuse ulcerated atherosclerotic failure caused by cholesterol crystal embolism. Clin Nephrol 66(4):232–239 7. Yücel AE, Kart-Kӧseoglu H, Demirhan B, Özdemir Cholesterinkristallemboliesyndrom nach FN (2006) Cholesterol crystal embolization mim- Thrombolyse beim akuten Myokardinfarkt icking : success with corticosteroid and cyclophosphamide therapy in two cases. Rheu- Zusammenfassung matol Int 26:454–460 8. Renshaw A, McCowen T, Waltke EA et al (2002) Hintergrund. Das Cholesterinkristallembo- aus den zyanotischen Zehen Cholesterinkris- Angioplasty with stenting is effective in treat- liesyndrom (CES) ist das Ergebnis der Erosion talle im Gefäßlumen der kleinkalibrigen Arte- ing blue toe syndrome. Vasc Endovascular Surg von atherosklerotischen Plaques und der an- rien zeigte. Der Patient entwickelte eine pro- 36(2):155–159 schließenden Verschiebung von Cholesterin- grediente Niereninsuffizienz, wahrscheinlich 9. Cardaioli P, Rigatelli G, Arboit M et al (2007) kristallen vom Kern der Plaque in die periphe- aufgrund von Kristallembolien auch in das Treatment of cholesterol crystal embolisation ren Arterien. Die Quelle der Embolie ist meis- Kapillarstromgebiet der Nieren, und verstarb syndrome with filter-assisted stenting. J Cardio- tens in der Aorta lokalisiert, wobei die am nach dem 2. Infarkt. vasc Med (Hagerstown) 8(11):953–955 10. Bojar RM, Payne DD, Murphy RE et al (1996) häufigsten betroffenen Organe die Haut und Schlussfolgerung. Die Therapieoptionen bei Surgical treatment of systemic atheroembo- die Nieren sind. Kristallembolie sind eingeschränkt und oh- lism from the thoracic aorta. Ann Thorac Surg Fallbeschreibung. Ein 69-jähriger Mann ne nachgewiesene Wirksamkeit; erhöhte Auf- 61:1389–1393 stellte sich mit schmerzhaften, zyanotisch merksamkeit durch die klinischen Ärzte ist er- verfärbten Zehen 4 Wochen nach Thromboly- forderlich. se eines akuten Myokardinfarkts vor. Sowohl die transösophageale Echokardiographie als Schlüsselwörter auch die kernspintomographische Aorto- Cholesterinkristallemboliesyndrom · graphie zeigten eine diffus ulzerierte athero­ Zyanotische Zehen · Thrombolyse · sklerotische Plaque im Bereich der thorakalen Atheroembolische Nierenerkrankung Aorta descendens, während die Hautbiopsie

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