Koch A, Hofbeck M, Dörr H G, Singer H
Klinik und Poliklinik für Kinder und Jugendliche Universität Erlangen-Nürnberg.
Z Kardiol. 1999 Jan;88(1):10-3. doi: 10.1007/s003920050257.
Hypocalcemia is a relatively uncommon reversible cause of congestive heart failure. There are a few reports of hypocalcemic children who developed congestive heart failure associated with hypoparathyroidism. In all these patients, however, cardiac failure did not occur before the age of nine years. In addition, other striking noncardial manifestations of hypoparathyroidism, e.g., convulsive seizures, had been present prior to cardiac symptoms. We report on a 3.7 year old girl with mitral insufficiency and severe cardiac failure due to hypocalcemia secondary to familial hypoparathyroidism. The infant's mother was suffering from idiopathic hypoparathyroidism, but her own history lacked any evidence for parathyroid hormone deficiency. On admission, she presented with fatigue, dyspnea, and pedal edema. Liver edge was palpable 4 cm below the right costal margin, and a 3/6 systolic murmur was heard. A chest x-ray showed cardiac enlargement; electrocardiogram demonstrated a prolonged QTc interval of 0.46 s. The echocardiography revealed a cleft in the mitral valve with mitral insufficiency and markedly reduced contractility of the left ventricle. Laboratory studies demonstrated a low total serum calcium level of 1.3 mmol/l; serum magnesium level was slightly decreased (0.5 mmol/l), and parathyroid hormone level was not detectable. Partial monosomy of chromosome 22 was excluded. Ophthalmological examination, audiometry, and renal ultrasonogram were normal. Oral calcium supplementation and anticongestive therapy with metildigoxin, furosemid, and captopril was initiated but no improvement of the heart failure occurred. However, normalization of serum calcium level by calcium infusions caused prompt clearing of the clinical symptoms, complete normalization of liver size, reduction of cardiac enlargement (thoracic ratio decreased from 0.68 to 0.57), and marked improvement in contractility (left ventricular shortening fraction increased from 21% to 34%). The QTc interval decreased to 0.39 s. The successful treatment following normalization of serum calcium level proved the superiority of hypocalcemia over mitral valve insufficiency in the etiology of the cardiac failure. To our knowledge, this is the first report of congestive heart failure due to hypocalcemia as the first manifestation of hypoparathyroidism in childhood. Hypocalcemia should be kept in mind in any congestive heart failure in children with or without underlying cardiac malformation.
低钙血症是充血性心力衰竭相对少见的可逆病因。有一些关于患有充血性心力衰竭且与甲状旁腺功能减退相关的低钙血症儿童的报道。然而，在所有这些患者中，心力衰竭均未在9岁之前出现。此外，甲状旁腺功能减退的其他显著非心脏表现，如惊厥发作，在心脏症状出现之前就已存在。我们报告了一名3.7岁女孩，因家族性甲状旁腺功能减退继发低钙血症导致二尖瓣关闭不全和严重心力衰竭。患儿母亲患有特发性甲状旁腺功能减退，但她自己的病史中没有任何甲状旁腺激素缺乏的证据。入院时，她表现为疲劳、呼吸困难和足部水肿。肝脏边缘在右肋缘下4厘米处可触及，可闻及3/6级收缩期杂音。胸部X线显示心脏扩大；心电图显示QTc间期延长至0.46秒。超声心动图显示二尖瓣有裂隙伴二尖瓣关闭不全，左心室收缩力明显降低。实验室检查显示血清总钙水平低至1.3 mmol/l；血清镁水平略有降低（0.5 mmol/l），甲状旁腺激素水平检测不到。排除了22号染色体部分单体性。眼科检查、听力测定和肾脏超声检查均正常。开始口服补钙及使用甲地高辛、呋塞米和卡托普利进行抗充血治疗，但心力衰竭无改善。然而，通过静脉输注钙剂使血清钙水平恢复正常后，临床症状迅速缓解，肝脏大小完全恢复正常，心脏扩大减轻（心胸比率从0.68降至0.57），收缩力明显改善（左心室缩短分数从21%增至34%）。QTc间期降至0.39秒。血清钙水平恢复正常后的成功治疗证明了低钙血症在心力衰竭病因中比二尖瓣关闭不全更具优势。据我们所知，这是儿童期甲状旁腺功能减退以低钙血症作为首发表现导致充血性心力衰竭的首例报道。对于任何患有或不患有潜在心脏畸形的儿童充血性心力衰竭，都应考虑到低钙血症的可能。
