Part I: Cutaneous manifestations of cardiovascular disease.

In this part 1 of a 2-part continuing medical education series, we review the epidemiology and pathophysiology of cardiovascular disease, its association with cutaneous symptoms, and the diagnosis and evaluation of cutaneous features of cardiovascular syndromes, including infective endocarditis, acute rheumatic fever, Kawasaki disease, cholesterol embolization syndrome, lipid disorders, cardiac amyloidosis, and cardiac myxomas. As the incidence and prevalence of cardiovascular diseases increase, dermatologists play an essential role in recognizing the cutaneous manifestations of cardiovascular diseases in order to appropriately connect patients with follow-up care.

Cholesterol embolization syndrome: An under-recognized entity in cardiovascular interventions.

Cholesterol embolization syndrome (CES) is a multi-systemic disease caused by embolization of atherosclerotic plaque contents from proximal large-caliber artery to distal small to medium arteries, occurring spontaneously or more commonly after vascular intervention. This report is a comprehensive review of the reported cases of CES found in our literature search. We discuss the risk factors, clinical manifestations, management, and prognosis of CES. The major predisposing factors for CES include older age, male sex, atherosclerotic cardiovascular risk factors, anticoagulation, and femoral access route. The composite incidence of atheroembolic renal disease was 92% and mortality 63%. Our review highlights the importance to recognize this disease entity for the cardiologist and nephrologist.

Cholesterol Crystal Embolization Exacerbates Critical Limb Ischemia.

Chronic life-threatening ischemia (CLTI), characterized by chronic severe ischemic ulcers or gangrene in the legs with arterial occlusive disease, has a high rate of amputation and mortality. However, how lower extremity artery disease (LEAD) leads to CLTI is not fully understood yet. Here, we report a 79-year-old man with resting pain and gangrene in the left first and fifth toes for a year who had undergone repetitive endovascular treatment (EVT) that temporarily improved the ischemia. Non-obstructive general angioscopy (NOGA) revealed yellow and red floating emboli at the occluded left superficial femoral artery (SFA). Although a second EVT for the reoccluded SFA was successful, amputation of the left lower knee remained necessary because of osteomyelitis of the left heel. Cholesterol crystals (CCs) associated with innate inflammation were detected in spontaneously ruptured aortic plaques (SRAPs) via aortic screening using the NOGA, in occluded SFAs, and on the surface of the muscle cross-section of the amputated legs via a polarizing microscope. Histopathological analysis demonstrated CCs in small vessels in various stages of patchy necrosis and muscle regeneration. In this case, the process of CC embolization, such as the embolic source of CCs, occlusion in arteries, small arteries, and deposition in muscles, was confirmed in CLTI. CCs are the principal trigger of IL-6 production through the innate inflammatory response in spontaneously ruptured aortic plaques. Mechanical ischemia and chronic inflammation due to embolized CCs may cause chronic limb damage. In this case, the CC embolization might exacerbate CLTI.

Cholesterol Embolization Syndrome Post Invasive Arterial Procedure: A Case Report.

Cholesterol embolization syndrome (CES) is a rare but systemic severe disease caused by the distal showering of cholesterol crystals after angiography, major surgery, thrombolysis, or anticoagulation. Here, we present a case of a 74-year-old male with a history of coronary artery disease, chronic kidney disease, peripheral vascular disease, antiphospholipid syndrome, and right internal carotid artery occlusion who developed purple discoloration and ulceration involving several toes two months after coronary artery bypass surgery. A broad differential diagnosis for blue toes was considered, and a biopsy was obtained, which revealed an arterial lumen filled with large cholesterol crystal spaces, confirming the diagnosis of CES. Treatment of CES remains a bimodal approach of supportive and prophylactic care. Although there is no direct evidence in favor of antiplatelet agents, their use seems reasonable because they have been shown to reduce the risk of other cardiovascular events in patients with extensive atherosclerosis. In this case, the patient's toe pain improved with the use of topical amitriptyline ketamine and has achieved complete resolution of pain and skin discoloration at a seven-month follow-up.

An Unusual Presentation of Cholesterol Embolization Syndrome.

Cholesterol embolization syndrome (CES) is a rare presentation of systemic atherosclerosis, which commonly presents in patients with risk factors of coronary artery disease and usually occurs after cardiac or vascular procedures. Laboratory tests are nonspecific, and imaging studies may visualize the plaque. Management includes supportive care directed to relieve the end-organ damage. The prognosis of CES is poor, with high mortality of up to 29% if the CES resulted in atheroembolic renal disease (AERD). In our report, we present a 90-year-old Caucasian female who was diagnosed with CES and complicated with AERD. The patient did not undergo any cardiac or vascular procedures. This case highlights the importance of considering CES and AERD as a potential cause of renal failure, especially in high-risk patients, even if the patients did not have any history of cardiac or vascular intervention.

Detecting Free Cholesterol Crystals in a Patient With Spontaneous Cholesterol Embolization Syndrome.

Spontaneous cholesterol embolization is thought to be due to aortic plaque-derived shower emboli; however, flowing cholesterol crystals have not been detected. Free multilayer cholesterol crystals obtained from the femoral artery were observed using polarized light microscopy. Detection of these crystals may indicate the occurrence of spontaneous shower emboli. (Level of Difficulty: Intermediate.).

Cholesterol Embolization Syndrome After Carotid Artery Stenting Associated with Delayed Cerebral Hyperperfusion Intracerebral Hemorrhage.

BACKGROUND: The cholesterol embolization syndrome (CES) results from the distal embolization of cholesterol crystals from atheromatous plaques in large vessels such as the aorta and results in multiorgan damage. CASE DESCRIPTION: We present the case of a patient with definite CES with skin manifestations (e.g., blue toes) and renal and neurological dysfunction, including parenchymal hematoma with cytotoxic and vasogenic edema after he had undergone left carotid artery stenting for symptomatic critical left carotid artery stenosis. CONCLUSIONS: Our patient with CES had cutaneous involvement affecting the lower limbs and renal and neurological involvement. High clinical suspicion and early treatment can reduce the mortality and morbidity after endovascular procedures. The neurological symptoms had most likely resulted from delayed cerebral hyperperfusion syndrome resulting in intracerebral hemorrhage.

Contrast-induced acute kidney injury: A review of practical points.

Contrast-induced acute kidney injury (CI-AKI) is one of the most common causes of AKI in clinical practice. CI-AKI has been found to be strongly associated with morbidity and mortality of the patients. Furthermore, CI-AKI may not be always reversible and it may be associated with the development of chronic kidney disease. Pathophysiology of CI-AKI is not exactly understood and there is no consensus on the preventive strategies. CI-AKI is an active research area thus clinicians should be updated periodically about this topic. In this review, we aimed to discuss the indications of contrast-enhanced imaging, types of contrast media and their impact on nephrotoxicity, major pathophysiological mechanisms, risk factors and preventive strategies of CI-AKI and alternative non-contrast-enhanced imaging methods.

[Massive rhabdomyolysis: an uncommon complication of cholesterol embolization syndrome]. / Rhabdomyolyse massive: une complication rare de la maladie des emboles de cholestérol.

A 79-year-old man with atherosclerosis presented blue toes and livedo reticularis. The patient had eroded aortic atheromatous plaques, and cholesterol embolization syndrome was suspected. An endovascular technique to exclude sources of cholesterol emboli was however performed. The patient immediately presented with severe muscle pain and total functional disability of lower limbs, new ischemic lesions of toes, anal and genital necrosis, and a livedo extended up to the abdomen. A massive rhabdomyolysis occurred associated with acute kidney injury and hyperkaliemia treated by continuous renal replacement therapy with regional citrate anticoagulation. Steroids have been introduced and renal function improved. Cholesterol crystals were also found on a skin biopsy, performed before endovascular procedure.

Systemic cholesterol embolization syndrome in a patient positive for anti-cardiolipin antibody.

Cholesterol embolization syndrome (CES) is caused by a cholesterol crystal embolization from atherosclerotic plaques on the walls of arteries. It often occurs after an invasive vascular procedure. CES exhibits several cutaneous manifestations before systemic symptoms take place. Here, we report a case of CES, positive for an anti-cardiolipin antibody, with a history of cerebral infarction. The existence of anti-phospholipid antibody may be a risk factor for CES after an invasive vascular procedure.