Use of ACE inhibitors for reduction of pulmonary artery pressure in patients with univentricular physiology

  • Dr Ashutosh Marwah, Escorts Heart Institute and Research Centre New Delhi, India
  • Dr Shrinivas Kini, Escorts Heart Institute and Research Centre New Delhi, India
  • Dr Rajesh Sharma, Escorts Heart Institute and Research Centre New Delhi, India
  • Introduction: In patients with univentricular physiology, longstanding hypoxia and altered hemodynamics leads to reduced ventricular compliance and elevated ventricular end-diastolic pressure. This causes elevation of pulmonary artery pressure and a patient may be deemed unsuitable for Fontan.
    Drawing simile from experience in adults with diastolic dysfunction it seemed logical that ACE inhibitors could also be used in patients with univentricular physiology with beneficial effects.
    Material and methods: Patients presenting for Fontan completion were enrolled into the study if they had elevated pulmonary artery pressure and ventricular end-diastolic pressure on catheterization. 3 patients were entered into the study between, April 2007 to August 2008. All three received ACE inhibitors (captopril or enalapril) for 7-11 months before a second cardiac catheterization. The study end point was considered to be reduction in PA pressure and ventricular end diastolic pressure allowing conversion to Fontan.
    Results : There were 2 females, and one male. There age ranged from 7years – 20years (mean 13.7years) and weight ranged from 24.5kg-44kg (mean 31.3 Kg). The duration of treatment with ACE inhibitors ranged from 210days – 340days. All three patients showed significant drop in mean pulmonary artery pressure from 19.6mmHg(19-21mmHg) to 13.6mHg(11-14mmHg) and drop in ventricular end-diastolic pressure allowing successful Fontan completion. Postoperative period was uneventful.
    Discussion: Patients with elevated LV end-diastolic pressure and mean pulmonary artery pressure are considered unsuitable for Fontan completion. Longterm use of ACE inhibitors helps in reducing PA pressure and improves chances of successful conversion. Conclusion: After load reduction helps in improving Fontan candidature

    age/sex diagnosis Hb/ Sat Pre PAP (mmHg) Pre LVED (mmHg) Post PAP (mmHg) Post LVED (mmHg) drainage days outcome
    7y/F TA, PS 17/82 19 21 14 12 7 discharge
    20y/M DILV, PS 16.6/78 19 21 13 11 2 discharge
    14y/F TA PS, VA discord. 18/82 21 22 11 14 1 discharge