Irreversible End‐stage Heart Failure in a Young Patient due to Severe Chronic Hypocalcemia Associated with Primary Hypoparathyroidism and Celiac Disease

Abstract

Background: Chronic hypocalcemia may cause electrocardiographic (ECG) changes and mimic acute myocardial infarction. It has also been associated with reversible cardiac dysfunction. On the other hand cardiomyopathy and heart failure have been reported in patients with idiopathic hypoparathyroidism or celiac disease. Clinical Case: A 39-year-old male was admitted to the emergency room with acute retrosternal pain and dyspnea. He exhibited severe hypocalcemia and acute renal failure. High creatine kinase (CK) levels did not correlate with biomarkers of myocardial necrosis (negative troponin test, heart type creatine kinase isoenzyme (CK-MB) <1% of CK value). The ECG showed an extremely long QT interval (0.6 sec) and T-wave inversions on V 4 through V 6 . The left ventricular ejection fraction (LVEF) was as low as 25%, while coronary angiography was normal. Investigation of the hypocalcemia revealed primary hypoparathyroidism (Parathyroid hormone (PTH) <3 pg/ml) and concomitant celiac disease with positive antigliadin and endomysial antibodies. The cardiovascular episodes and the dilated heart failure were attributed to the chronic hypocalcemia since no other cause was found. The correction of hypocalcemia has not been sufficient to reverse the end-stage heart failure after more than 6 months of treatment, even though ECG abnormalities have receded, implying permanent cardiac impairment. Conclusion: This case demonstrates an unusual clinical condition where 2 calcium homeostasis disorders led to severe hypocalcemia with clinical manifestations of end-stage heart failure. The severe cardiac failure appeared to be nonreversible after calcium repletion suggesting permanent cardiac muscle dysfunction due to associated cardiomyopathy. IntroductionSevere chronic hypocalcemia may predominantly be presented with paresthesias, neuromuscular irritability, and tetany, but can sometimes manifest cardiovascular complications like hypotension, myocardial dysfunction, prolongation of the QT interval, and triggered dysrhythmias like torsades de pointes, decreased myocardial performance, and even heart failure.1 Hypocalcemia induced heart failure (or hypocalcemic heart failure) has been associated with various clinical entities like idiopathic or postsurgical hypoparathyroidism, vitamin D deficiency, and celiac disease. 2,3,4 The symptoms are often misleading and, consequently, endocrine causes of heart failure are sometimes overlooked.The case of a 39-year-old man, who upon evaluation for clinical acute myocardial syndrome and end-stage heart failure was diagnosed with primary hypoparathyroidismand celiac disease, is presented and the role of these diseases in heart function is discussed.

Use of recombinant human parathyroid hormone in hypocalcemic cardiomyopathy

“…Hypocalcemic cardiomyopathy has been described in more than 25 reports in the literature and in all age groups (3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33). We conducted a search in Pubmed using keywords 'hypocalcemia (all fields) AND heart failure (all fields)' yielding 221 entries.…”

A surprising cause of reversible dilated cardiomyopathy

“…Calcium plays a central role as initiator of electric activation and ion channel gating since increase of free calcium level in the cytoplasm is the trigger for myocardial contraction 2 10. The sarcoplasmic reticulum (SR) is an intracellular storage pool of calcium ions, but it cannot store enough calcium ions to start the contraction itself.…”

Une cause réversible de la cardiomyopathie dilatée : l’hypocalcémie

“…Toutefois l'hypocalcémie peut parfois entraîner des complications cardiaques comme l'hypotension [4], la prolongation de l'intervalle QT, les arythmies comme la torsade de pointe, la dysfonction myocardique et parfois même l'insuffisance cardiaque ou l'insuffisance cardiaque hypocalcémique [3].…”

“…Les symptômes sont souvent trompeurs ou même insidieux si l hypocalcémie est d'installation chronique et progressive, et par conséquent les causes endocriniennes de l'insuffisance cardiaque sont parfois négligées [4].…”

“…L'hypocalcémie induit la dysfonction cardiaque et devrait être évoquée chez les patients en insuffisance cardiaque avec un intervalle QT allongé [11]. La dysfonction myocardique peut survenir en cas d'hypocalcémie aiguë ou chronique, et elle a été décrite chez les enfants et les adultes [4]. Dans certains cas de la littérature, on a pu ont avoir des preuves reliant l'insuffisance cardiaque et l'hypocalcémie, mais la relation causale n'a pas encore été prouvée [3].…”