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(Circulation. 2006;114:1807-1810.)
© 2006 American Heart Association, Inc.

Congenital Heart Disease

Phosphodiesterase-5 Inhibitor in Eisenmenger Syndrome

A Preliminary Observational Study

Saibal Mukhopadhyay, MD, DM; Manish Sharma, MD, DM; S. Ramakrishnan, MD, DM; Jamal Yusuf, MD, DM; Mohit Dayal Gupta, MD, DM; Naveen Bhamri, MD; Vijay Trehan, MD, DM; Sanjay Tyagi, MD, DM

From the Department of Cardiology, GB Pant Hospital and Maulana Azad Medical College, New Delhi, India.

Correspondence to Dr Saibal Mukhopadhyay, 123 Academic Block, GB Pant Hospital, New Delhi, India 110 002. E-mail saibalmukhopadhyay{at}yahoo.com

Received November 22, 2005; revision received August 19, 2006; accepted August 21, 2006.

Background— Phosphodiesterase-5 inhibitors produce a significant decrease in pulmonary vascular resistance in patients with idiopathic pulmonary arterial hypertension. We studied the effects of tadalafil, a phosphodiesterase-5 inhibitor, on short-term hemodynamics, tolerability, and efficacy over a 12-week period in patients of Eisenmenger syndrome having a pulmonary vascular pathology similar to idiopathic pulmonary arterial hypertension.

Methods and Results— Sixteen symptomatic Eisenmenger syndrome patients (mean age, 25±8.9 years) were assessed hemodynamically at baseline and 90 minutes after a single dose of tadalafil (1 mg/kg body weight up to a maximum of 40 mg). The same dose was then continued daily for 12 weeks, and the patients were restudied. There was a significant decrease in mean pulmonary vascular resistance immediately (24.75±8.49 to 19.22±8.23 Woods units; P<0.005) and at 12 weeks (19.22±8.23 to 17.02±6.19 Woods units; P=0.03 versus 90 minutes). Thirteen of 16 patients (81.25%) showed a 20% decrease in pulmonary vascular resistance and were defined as responders. The mean systemic oxygen saturation improved significantly both immediately (84.34±5.47% to 87.39±4.34%; P<0.005) and at 12 weeks (87.39±4.34% to 89.16±3.8%; P<0.02 versus 90 minutes) without a significant change in systemic vascular resistance. None of the patients had a fall in systemic arterial pressure, worsening of systemic oxygen saturation, or any adverse reactions to the drug. The mean World Health Organization functional class improved from 2.31±0.47 to 1.25±0.44 (P<0.0001), and the 6-minute walk distance improved from 344.56±119.06 to 387.56±117.18 m (P<0.001).

Conclusions— Preliminary evaluation of tadalafil has shown efficacy and safety in selected patients with Eisenmenger syndrome, warranting further investigation in this subgroup of patients.

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CLINICAL PERSPECTIVE

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