[Severe cardiac insufficiency and type Ib pseudohypoparathyroidism]

The authors report the case of a 28 year old woman with acute left ventricular failure associated with severe hypocalcaemia (1.7 mmol/l) without chronic renal failure or hypoproteinaemia. The echocardiographic appearances were those of dilated and globally hypokinetic cardiomyopathy with a severely depressed left ventricular ejection fraction (23%). Haemodynamic improvement 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, hypophosphaturia and, above all, a high parathormone concentration, provided the diagnosis of a sporadic form of type Ib pseudohypoparathyroidism. Secondary cardiac failure to the hypocalcaemia is mainly observed in children and young adults in the context of chronic renal failure or true hypoparathyroidism. Pseudohypoparathyroidism is a very rare condition and systolic LV dysfunction for which hypocalcaemia is responsible, would seem to be totally reversible after calcium supplementation.