Sailing between scylla and charybdis—anticoagulation dilemma in a patient with calciphylaxis and mechanical cardiac valve replacement: a case report and literature review

Figures & data

Figure 1. Progression of the cutaneous lesion (the metatarsal bone, the third toe, and the little toe suffered the most and presented with ulcers within one week, accompanied by pain and progressive cutaneous darkening and sclerosis. The skin temperature was low at the initial stage. In addition, the patient had weakened bilateral dorsalis pedis pulses, especially on the right side).

Figure 2. Echocardiography manifestations [A-B: the aortic and mitral mechanical valves (red arrows) were well placed with good activity of the valve leaflets and normal perivalvular surroundings with iso-echogenicity attached. C: There was no significant thickening of the tricuspid valve leaflet. Further, the valve showed good opening but poor closing].

Figure 3. Vascular ultrasound of the lower extremities (there was no evident abnormality in the diameter of the bilateral lower limb artery. However, the walls were rough with multiple hyperechoic and hypoechoic plaques. Extensive calcification of the arterial media was detected. A: right femoral artery; B: right posterior tibial artery; C: right dorsalis pedis artery; red arrows show calcification areas of the arterial media).

Table 1. A Summary of reported cases of calciphylaxis and prosthetic cardiac valve replacement treated with warfarin.

Studies	Age	Sex	Duration of Hemodialysis	Replaced Valves	Duration of Anticoagulation	Symptoms	Treatment and Outcomes
Cadavid et al. 2011 [13]	37	M	4 years	Mechanic mitral and aortic valves replacement.	10 months	Painful necrotic ulcer on right calf. Metastatic pulmonary calcification and calciphylaxis on radiography.	Significant clinical improvement following Cinacalcet and STS after dialysis. Warfarin was continued.
Disbrow et al. 2015 [14]	37	F	Peritoneal dialysis since 2009	Mechanic mitral and aortic valves replacement.	Several months	Epistaxis, pain in the left lower extremity, and nonhealing left leg ulcers.	She was transitioned to daily haemodialysis, and took STS infusions during daily hemodialysis. The phosphate-binder therapy was optimised. Symptomatic relief was reported after five months of changing the anticoagulant into heparin from warfarin.
Berry et al. 2018 [15]	72	M	Not documented	Mechanic mitral and aortic valves replacement.	More than 20 years	Left thigh erythema with skin induration.	Warfarin was continued. The patient received STS infusions during each dialysis, but expired due to septic shock one year later.
Sohal et al. 2020 [16]	53	M	Since 2003	Biomitral valve replacement	Not documented	Nonhealing right lower extremity cellulitis.	Warfarin was continued, and STS infusions were administered during dialysis. The patient died 64 days later.
Diasty et al. 2021 [17]	39	F	At least 8 years.	Mechanic mitral valve replacement.	3 days	Rapidly worsening ulcerative lesions in the inner aspects of thighs and calves.	Warfarin was continued. The patient died due to wound infection and sepsis 45 days after surgery.
Hirooka et al. 2021 [18]	64	M	4 years	Mechanic aortic valve replacement.	7 years	Necrotic lesions on the legs and penile ulceration.	Warfarin was discontinued due to an excessive anticoagulant state. The patient developed gas gangrene in the right leg and died due to septic shock.
Smith et al. 2021 [19]	NR	F	25 years	Mechanic mitral and aortic valves replacement.	Over 10 years	Severe burning pain under the breast for a month.	Warfarin was changed to enoxaparin, and STS infusions were administered during dialysis. The painful purpura showed improvement.
Nyembo et al. 2022 [20]	54	M	8 years	Mechanic mitral and aortic valves replacement.	3 days	Painful necrotic ulcers on bilateral lower extremities.	The patient was switched from warfarin to unfractionated heparin. Therapy with STS, cinacalcet and sevelamer was initiated. The patient died due to a worsening wound infection.
Current Case	72	F	5 years	Mechanic mitral and aortic valves replacement.	22 years	Intense resting pain in the right foot.	Warfarin was discontinued due to an excessive anticoagulant state. Therapy with STS was initiated. The patient died a month later due to sepsis.

STS: sodium thiosulfate.

Cadavid JC, DiVietro ML, Torres EA, et al. Warfarin-induced pulmonary metastatic calcification and calciphylaxis in a patient with end-stage renal disease. Chest. 2011;139(6):1503–1506. doi:10.1378/chest.10-1322.

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Disbrow MB, Qaqish I, Kransdorf M, et al. Calcific uraemic arteriolopathy. BMJ Case Rep. 2015;2015:bcr2014207935. doi:10.1136/bcr-2014-207935.

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Berry A, Degheim G, Saba S. Arteriolar vs. valvular thrombosis: pick your evil!. Thromb J. 2018;16:23. doi:10.1186/s12959-018-0175-3.

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Sohal R, George T. Sodium-Thiosulfate induced Life-Threatening metabolic acidosis limiting treatment of calciphylaxis. Am J Case Rep. 2020;21:e919926. doi:10.12659/AJCR.919926.

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Diasty ME, Cuenca J. New-onset acute rapidly deteriorating case of calciphylaxis after open heart surgery: a case report. Eur Heart J Case Rep. 2021;5(3):ytab060. doi:10.1093/ehjcr/ytab060.

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Hirooka K, Anju K, Moriyama Y, et al. Calciphylaxis after aortic valve replacement in a patient with End-Stage renal disease. Ann Vasc Dis. 2021;14(4):376–379. doi:10.3400/avd.cr.21-00040.

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Smith MC, Gleaves E, Singh A, et al. Anticoagulation in a patient with mechanical prosthetic valves and calcific uremic arteriolopathy on warfarin. Cureus. 2021;13(3):e14196. doi:10.7759/cureus.14196.

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Nyembo PF, Eidman KE, Simegn M, et al. Rapidly progressive and catastrophic calciphylaxis after mechanical valve replacement: an anticoagulation dilemma. JACC Case Rep. 2022;4(19):1319–1323. doi:10.1016/j.jaccas.2022.07.034.

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