Porcelain Heart: Rapid Progression of Cardiac Calcification in a Patient with Hemodialysis
A 34-year-old female patient with ESRD caused by hypertension was admitted to our hospital for hemodialysis to be replaced with peritoneal dialysis due to decreased adequacy. On admission, she presented with chest discomfort, exertional dyspnea of New York Heart Association class II and general weakness. In the patient's past medical history, the patient began peritoneal dialysis 10 years ago and changed into hemodialysis because of frequent dialysis catheter infections 6 years ago. The patient visited our emergency department presenting with cardiac arrest due to hyperkalemia and received an echocardiography 4 years ago. There were no unusual findings except moderate left ventricular hypertrophy (LVH) in the echocardiograph. Two years ago, the patient visited our emergency department again presenting with chest pain and had a coronary angiography performed. The coronary angiography revealed the right coronary artery (RCA) with 50% stenosis. Laboratory data showed hyperphosphatemia but was left untreated. She was discharged from the hospital after medical treatments and then had hemodialysis in a local hospital.\nThe patient's vital signs on the day of admission revealed a blood pressure of 150/70 mmHg, heart rate 110 bpm, respiration rate 20 times per minute. From physical examination, a diastolic murmur was noted at cardiac apex. A chest X-ray demonstrated mild cardiomegaly. Review of serial chest radiographs revealed progressive cardiac enlargement. An electrocardiogram showed LVH, tall-T wave in V1-4 leads. Laboratory data showed serum calcium 9.2 mg/dL (normal 8.5-10.5 mg/dL), serum phosphate 9.4 mg/dL (normal 2.7-4.5 mg/dL), serum blood urea nitrogen 135.7 mg/dL (normal 5-23 mg/dL), serum creatinine 13.5 mg/dL (normal 0.4-1.2 mg/dL), serum potassium 6.7 mg/dL (normal 3.5-5.5 mg/dL), immuno-reactive parathyroid hormone (iPTH) 2959 pg/mL (normal 10-65 pg/mL). The echocardiogram showed extensive myocardial calcification, severe mitral stenosis with a mitral valve area of 0.99 cm2 by planimetry and mean pressure gradient 24.3 mmHg. The mitral valve was severely calcified (). The aortic valve was thickened and had mild calcification. The left ventricular ejection fraction was estimated to be 61% and diastolic dysfunction showed as an impaired relaxation pattern. Coronary computed tomography (CT) showed severe calcification of the coronary artery and left ventricular myocardium and an increased calcium score in the coronary artery (). A follow-up coronary angiography was performed, and revealed remnant RCA stenosis, and left anterior descending artery with 50% stenosis. We suspected 'porcelain heart' cardiomyopathy secondary to hyperparathyroidism of ESRD. The patient started a phosphate restricted diet. A thyroid sonogram showed enlarged parathyroid glands (right lower lobe 2.2 cm size, left lower lobe 1 cm size) and the patient underwent surgical parathyroidectomy. Microscopic analysis of the parathyroid tissue showed diffuse hyperplasia of chief cells (). Post-operation laboratory data showed serum calcium 8.2 mg/dL (normal 8.5-10.5 mg/dL), serum phosphate 3.6 mg/dL (normal 2.7-4.5 mg/dL), iPTH 357 pg/mL (normal 10-65 pg/mL). After surgery, test results have shown improvement of Calcium, Phosphate, and iPTH levels (). However, the patient's cardiac symptoms remained. In the future, we will consider mitral valve replacement.