Curious case of calciphylaxis leading to acute mitral regurgitation

Abstract

Calciphylaxis is uncommon and typically seen in patients with end-stage renal disease. It has been defined as a vasculopathic disorder characterised by cutaneous ischaemia and necrosis due to calcification, intimal fibroplasia and thrombosis of pannicular arterioles. We present the case of a 74-year-old woman with chronic kidney disease stage III who developed calciphylaxis leading to mitral valve calcification, chordae tendineae rupture and acute mitral regurgitation. Although an alternative explanation can typically be found for non-uraemic calciphylaxis, her evaluation did not reveal any usual non-uraemic causes including elevated calcium–phosphorus product, hyperparathyroidism, or evidence of connective tissue disease. Her wounds improved with sodium thiosulfate, pamidronate, penicillin and hyperbaric oxygen therapies but she ultimately decompensated with the onset of acute mitral regurgitation attributed to rupture of a previously calcified chordae tendineae. This case highlights an unusual case of calciphylaxis without clear precipitant as well as a novel manifestation of the disease.

Sodium thiosulfate and pamidronate for treatment of calciphylaxis: case report

“…Combination therapy with sodium thiosulfate and bisphosphonates is an uncommon treatment strategy and no many published literature 9 , 10 ; only one case of calciphylaxis unsuccessfully treated with the combination of intravenous thiosulfate and pamidronate has been reported 11 . However, due to the patient's lack of control of calcium levels (a factor that may contribute to the development of calciphylaxis) and the worsening of the lesions, we decided to use them simultaneously in order to reduce vascular calcification.…”

“…El uso de una terapia combinada de tiosulfato de sodio y bifosfonato es una estrategia poco utilizada y con poca bibliografía publicada 9 , 10 ; solamente se ha descrito un caso de calcifilaxis tratada sin éxito con la combinación de tiosulfato intravenoso y pamidronato 11 . Sin embargo debido a la falta de control de los niveles de calcio (factor que puede contribuir al desarrollo de calcifilaxis) en la paciente y al empeoramiento de las lesiones se decidió su utilización simultánea con el fin de reducir las calcificaciones vasculares.…”

Calciphylaxis: when a bird is not a duck

“…A previous case reported a similar consequence of calciphylaxis causing mitral valve calcification with chordae tendineae rupture. 5 We would encourage all clinicians to consider calciphylaxis as a differential for AION in ESRD patients, particularly those post-transplant or on dialysis, and in the presence of typical cutaneous ulcers and vision worsening despite steroids. Fig.…”

“…In 1987, Atlas et al noted WE as a cause of INO. 5 Similarly, a 2005 article noted 2 out of 410 patients with INO caused by probable Wernicke syndrome. 6 In a 1990 report of 5 patients with WE, Gallucci et al noted 1 patient with INO, though no further details were provided.…”

“…11 As it runs through the pterygopalatine fossa, this artery is at risk to damage when the maxillary tuberosity is separated from the pterygoid plate with an osteotome or during a downfracture procedure. 5,11 Classically, these patients present with a bruit or a pulsatile expanding mass. These findings, however, may not be evident to the observer as the vessels are located deep within the bony structures of the face.…”