Eisenmenger syndrome, a shunt-related congenital heart defect which leads to pulmonary hypertension, and resulting cyanosis.  Because of the nature of the disruption caused to the circulatory system and the systemic-to-pulmonary communication, the use of circulatory medication needs special attention.  Sildenafil is one example of this; it has been shown to be both effective and safe for idiopathic pulmonary arterial hypertension, and for pulmonary arterial hypertension due to connective tissue disease.  The picture for those suffering from Eisenmenger syndrome was unclear, until now.

In a study published by the journal Heart today, researchers based in China led by Professor Zhi-Cheng Jing of Tongji University School of Medicine (Shanghai) report that over a 12 month period, sildenafil treatment (oral administration) for those with Eisenmenger syndrome was both well tolerated and appeared to improve key performance measures.  These include systemic arterial oxygen saturation, exercise capacity, and haemodynamic parameters.

The open-label prospective multicentre study was conducted in four high-blood pressure centres in China, and involved the recruitment of 84 patients exhibiting Eisenmenger syndrome (class II-IV).  Through three administrations a day of 20 mg sildenafil over a period of twelve months, the effects were assessed.

To determine whether the patients were improving, they were given a six minute walk distance test, a test of resting systemic arterial blood oxygen saturation under standard room air, as well as undergoing right heart catherisation to measure haemodynamic parameters.

The researchers found improvements in the six minute walk distance test, the resting systemic arterial blood oxygen saturation, mean pulmonary arterial pressure, and pulmonary vascular resistance.  Toleration of Sildenafil was good, and most adverse affects with both transient and mild, limited to the first two weeks of treatment.

http://heart.bmj.com/content/early/2011/09/21/heartjnl-2011-300344.full.pdf