INTRODUCTION: Severe pulmonary hypertension with no response to vasodilators on an acute hemodynamic study is a contraindication to cardiac transplantation. The development of oral pulmonary vasodilators improves the prognosis in these patients. We present the case of a patient whose admission to the waiting list for cardiac transplantation was possible after 6 months of combination therapy with Sildenafil and Bosentan. CASE REPORT: The patient was a 50-year-old man with severe dilated alcohol-induced cardiomyopathy. A pretransplantation study, including a right hemodynamic analysis, revealed irreversible pulmonary hypertension, with 59 mm Hg mean pulmonary artery pressure and 6.4 Wood IU pulmonary vascular resistance, with no response to acute vasodilators with nitric oxide or prostacyclin. Initially, heart transplantation was not possible and the patient started treatment with oral Sildenafil. After 6 months there was no improvement in echocardiographic or hemodynamic parameters, and combination therapy with Bosentan was started. With the combination therapy, the patient progressively improved clinically and hemodynamically, the pressures becoming normal at the sixth month, at which time he was included on the waiting list for a heart transplantation. Eight months later he received a graft with a good posttransplantation course, no right ventricular failure in the acute phase, and absence of pulmonary hypertension on echocardiogrphic and invasive studies. CONCLUSION: Combinations of an oral pulmonary vasodilator with diverse action mechanisms may represent an alternative for patients with irreversible pulmonary hypertension who do not respond to monotherapy.
INTRODUCTION: Severe pulmonary hypertension with no response to vasodilators on an acute hemodynamic study is a contraindication to cardiac transplantation. The development of oral pulmonary vasodilators improves the prognosis in these patients. We present the case of a patient whose admission to the waiting list for cardiac transplantation was possible after 6 months of combination therapy with Sildenafil and Bosentan. CASE REPORT: The patient was a 50-year-old man with severe dilated alcohol-induced cardiomyopathy. A pretransplantation study, including a right hemodynamic analysis, revealed irreversible pulmonary hypertension, with 59 mm Hg mean pulmonary artery pressure and 6.4 Wood IU pulmonary vascular resistance, with no response to acute vasodilators with nitric oxide or prostacyclin. Initially, heart transplantation was not possible and the patient started treatment with oral Sildenafil. After 6 months there was no improvement in echocardiographic or hemodynamic parameters, and combination therapy with Bosentan was started. With the combination therapy, the patient progressively improved clinically and hemodynamically, the pressures becoming normal at the sixth month, at which time he was included on the waiting list for a heart transplantation. Eight months later he received a graft with a good posttransplantation course, no right ventricular failure in the acute phase, and absence of pulmonary hypertension on echocardiogrphic and invasive studies. CONCLUSION: Combinations of an oral pulmonary vasodilator with diverse action mechanisms may represent an alternative for patients with irreversible pulmonary hypertension who do not respond to monotherapy.
Authors: Ryan J Tedford; Anna R Hemnes; Stuart D Russell; Ilan S Wittstein; Mobusher Mahmud; Ari L Zaiman; Stephen C Mathai; David R Thiemann; Paul M Hassoun; Reda E Girgis; Jonathan B Orens; Ashish S Shah; David Yuh; John V Conte; Hunter C Champion Journal: Circ Heart Fail Date: 2008-11 Impact factor: 8.790