Refractory secondary hyperparathyroidism (SHPT) is a common complication observed in patients with end-stage renal disease and can result in ectopic calcification. Metastatic calcification involving the heart valves and the conduction system can easily lead to arrhythmias, including atrioventricular block. This case report describes a maintenance hemodialysis patient with refractory SHPT resulting in a complete atrioventricular block (CAVB), which was eventually reversed to a first-degree atrioventricular block.

We present the case of a 31-year-old Asian female who was receiving maintenance hemodialysis because of lupus nephropathy. She developed SHPT, and an electrocardiogram revealed a first-degree atrioventricular block. Then, she underwent parathyroidectomy (PTX) with autotransplantation. Unfortunately, a few years later, she developed SHPT again, and an electrocardiogram revealed a CAVB. A few years after the second PTX surgery, the calcification of the left atrium and left ventricle improved, and her CAVB was reversed.

This case revealed that metastatic cardiac calcification can result in complete atrioventricular blockage. Following parathyroid surgery, calcification of the cardiac conduction system improved, leading to reversal of the atrioventricular block. It is important for dialysis patients to optimize intact parathyroid hormone therapy and pay attention to calcification metastasis.

To report a rare case of hypophosphatemic rickets (HR) leading to extensive cardiac complications.

We present the clinical course and autopsy findings of a patient with HR, treated with chronic phosphate-only therapy as a child, who subsequently developed tertiary hyperparathyroidism leading to extensive cardiac calcifications and complications. We also review the literature on the pathophysiology of calcifications from HR.

A 34-year-old man was diagnosed with HR at 4 years of age after presenting with growth delay and leg bowing. Family history was negative for the disease. He was initiated on high-dose phosphate therapy (2-6 g of elemental phosphorus/day) with sporadic calcitriol use between 4-18 years of age. For 6 years he received phosphate-only therapy. Subsequently, he developed nephrocalcinosis, heart valve calcifications, severe calcific coronary artery disease, heart block, and congestive heart failure. At a young age, he required an aortic valve replacement and a biventricular pacemaker that was subsequently upgraded to an implantable cardioverter defibrillator. Autopsy showed extensive endocardial, myocardial, and coronary artery calcifications.

Cardiac calcification is a known sequela of tertiary hyperparathyroidism when it occurs in patients with renal failure, but it is rarely seen in HR due to high phosphate therapy. Phosphate alone should never be used to treat HR; high doses, even with calcitriol, should be avoided. It is important to be cognizant of high-dose phosphate effects and to consider parathyroidectomy for autonomous function, if needed. This case emphasizes the importance of appropriate therapy, monitoring, and management of patients with HR.

-Nephrogenic systemic fibrosis (NSF) is a rare but serious disorder initially described as a purely dermatologic process. Isolated autopsy reports have described multiorgan involvement by this disease.

-To further illustrate the varied and systemic involvement of NSF by describing the autopsy experience at the Massachusetts General Hospital.

-We describe the findings in a series of 4 autopsy cases of patients diagnosed with NSF. This report describes the history of renal dysfunction, exposure to gadolinium-containing contrast agents, specific laboratory parameters, and the extent of systemic involvement identified by postmortem examination.

-Causes of death included systemic thromboembolic disease (n = 3) and pneumonia (n = 1). Laboratory parameters and type, dose, or timing of gadolinium-containing contrast-agent exposure did not correlate with clinical findings and outcomes. All patients demonstrated cutaneous manifestations of the disease and nephrocalcinosis, with some exhibiting calcification and fibrosis of the dura, thyroid, and heart including the cardiac conduction system, on postmortem examination. Soft tissue calcification was associated with concurrent hyperparathyroidism or high serum parathyroid hormone levels.

-Thromboembolic disease can be a significant clinical complication of NSF. Patients with NSF may also develop characteristic histologic features of fibrosis and calcification in multiple organs, with significant morbidity and mortality. This autopsy series highlights the variability of systemic manifestations of NSF.

Patients with Alport's syndrome (causing 5% of end-stage renal disease) have a higher risk of heart conduction abnormalities.

To report a case of Alport's syndrome developing complete atrioventricular block during renal transplantation.

A 21-year-old man with chronic renal failure due to Alport's syndrome was submitted to a renal transplantation under epidural anesthesia and, during the intraoperative period, a complete atrioventricular block was diagnosed and promptly treated with a transcutaneous pacemaker. This extensive sympathetic block can contribute towards disturbances in the heart conduction system, particularly in patients with chronic renal disease in hemodialysis. Even in patients with a normal preoperative electrocardiogram or no conduction system disturbances, some degree of atrioventricular block, including complete atrioventricular block, can occur. In this situation, a transcutaneous pacemaker provides rapid and effective treatment in the operating room, thereby permitting the planning of a definitive treatment.