Severe cardiac failure and type Ib pseudohypoparathyroidism.

The authors report the case of a 28 year old woman with acute left ventricu lar failure associated with severe hypocalcaemia (1.7 mmol/l) without chron ic renal failure or hypoproteinaemia. The echocardiographic appearances wer e those of dilated and globally hypokinetic cardiomyopathy with a severely depressed left ventricular ejection fraction (23%). Haemodynamic improvemen t was only obtained by the association of calcium supplements and Vitamin D derivatives (Un-Alfa) to conventional treatment. A low serum calcium associated with hyperphosphotaemia, hypocalciuria, hypo phosphaturia and, above all, a high parathormone concentration, provided th e diagnosis of a sporadic form of type Ib pseudohypoparathyroidism. Seconda ry cardiac failure to the hypocalcaemia is mainly observed in children and young adults in the context of chronic renal failure or true hypoparathyroi dism. Pseudohypoparathyroidism is a very rare condition and systolic LV dysfuncti on for which hypocalcaemia is responsible, would seem to be totally reversi ble after calcium supplementation.