Vanishing pulmonary hypertension in mixed connective tissue disease

A 29-year-old woman with mixed connective tissue disease presented with sig ns of progressive pulmonary hypertension. After admission to the hospital h er condition worsened rapidly and she developed a cardiac arrest resistant to cardiopulmonary resuscitation. Therefore, emergency extracorporeal assis t was performed. No pulmonary embolism was found. Right heart catheterisati on showed severe pulmonary hypertension, which was treated with nitric oxid e ventilation. She was weaned from the extracorporeal assist with high dose s of inotropic agents. Because of suspicion of exacerbation of her underlyi ng disease, which led to pulmonary hypertension, immunosuppressive treatmen t was started with high doses of corticosteroids and plasma exchange. This resulted in slow recovery over the next four weeks. Control echocardiograph y showed complete normalisation of cardiac function without signs of pulmon ary hypertension. Two months after admission she was discharged from the ho spital in good condition.