Irreversible end-stage heart failure in a young patient due to severe chronic hypocalcemia associated with primary hypoparathyroidism and celiac disease. 2010

Konstantinos Mavroudis, and Kyriakos Aloumanis, and Panagiotis Stamatis, and George Antonakoudis, and Konstantinos Kifnidis, and Chariton Antonakoudis
Department of Endocrinology, Diabetes & Metabolism, General Hospital Asklipieion, Voula, Athens, Greece.

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. METHODS 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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D006996 Hypocalcemia Reduction of the blood calcium below normal. Manifestations include hyperactive deep tendon reflexes, Chvostek's sign, muscle and abdominal cramps, and carpopedal spasm. (Dorland, 27th ed) Hypocalcemias
D007011 Hypoparathyroidism A condition caused by a deficiency of PARATHYROID HORMONE (or PTH). It is characterized by HYPOCALCEMIA and hyperphosphatemia. Hypocalcemia leads to TETANY. The acquired form is due to removal or injuries to the PARATHYROID GLANDS. The congenital form is due to mutations of genes, such as TBX1; (see DIGEORGE SYNDROME); CASR encoding CALCIUM-SENSING RECEPTOR; or PTH encoding parathyroid hormone. Idiopathic Hypoparathyroidism,Hypoparathyroidism, Idiopathic
D008297 Male Males
D002446 Celiac Disease A malabsorption syndrome that is precipitated by the ingestion of foods containing GLUTEN, such as wheat, rye, and barley. It is characterized by INFLAMMATION of the SMALL INTESTINE, loss of MICROVILLI structure, failed INTESTINAL ABSORPTION, and MALNUTRITION. Gluten Enteropathy,Sprue, Celiac,Sprue, Nontropical,Celiac Sprue,Gluten-Sensitive Enteropathy,Sprue,Disease, Celiac,Enteropathies, Gluten,Enteropathies, Gluten-Sensitive,Enteropathy, Gluten,Enteropathy, Gluten-Sensitive,Gluten Enteropathies,Gluten Sensitive Enteropathy,Gluten-Sensitive Enteropathies,Nontropical Sprue
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D004562 Electrocardiography Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY. 12-Lead ECG,12-Lead EKG,12-Lead Electrocardiography,Cardiography,ECG,EKG,Electrocardiogram,Electrocardiograph,12 Lead ECG,12 Lead EKG,12 Lead Electrocardiography,12-Lead ECGs,12-Lead EKGs,12-Lead Electrocardiographies,Cardiographies,ECG, 12-Lead,EKG, 12-Lead,Electrocardiograms,Electrocardiographies, 12-Lead,Electrocardiographs,Electrocardiography, 12-Lead
D006333 Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION. Cardiac Failure,Heart Decompensation,Congestive Heart Failure,Heart Failure, Congestive,Heart Failure, Left-Sided,Heart Failure, Right-Sided,Left-Sided Heart Failure,Myocardial Failure,Right-Sided Heart Failure,Decompensation, Heart,Heart Failure, Left Sided,Heart Failure, Right Sided,Left Sided Heart Failure,Right Sided Heart Failure
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012720 Severity of Illness Index Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder. Illness Index Severities,Illness Index Severity

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