Management of Dilated RV with Pulmonary Hypertension

Initial Assessment and Hemodynamic Monitoring

The American College of Cardiology recommends obtaining invasive hemodynamic assessment with pulmonary artery catheterization to accurately measure pulmonary vascular resistance, cardiac output, and right atrial pressure 1, 2
Echocardiography should be performed to assess RV size, function, ventricular interdependence, and tricuspid regurgitation severity 1
Measurement of BNP levels is recommended as elevated values predict poor prognosis 2
Assessment of functional capacity with 6-minute walk test or cardiopulmonary exercise testing is advised 2

The American College of Cardiology recommends targeting euvolemia with CVP 8-12 mmHg, not aggressive volume loading 1
Diuretics should be used to reduce ventricular dilation and improve biventricular coupling when volume overloaded 1

Inhaled selective pulmonary vasodilators are preferred over systemic agents to avoid hypotension 3, 4
Inhaled nitric oxide (5-10 ppm) reduces pulmonary vascular resistance without systemic hypotension 3, 4, 5
Inhaled prostacyclin (20-30 ng/kg/min) has comparable efficacy to nitric oxide 3, 4
Sildenafil (PDE-5 inhibitor) improves exercise capacity and hemodynamics in PAH 2

The American College of Cardiology recommends maintaining systemic vascular resistance greater than pulmonary vascular resistance (SVR > PVR) to preserve RV coronary perfusion 5
Target systolic systemic arterial pressure should be higher than systolic pulmonary arterial pressure 5
Dobutamine is preferred over milrinone due to shorter half-life when hypotension risk exists 5
Low-dose norepinephrine is effective and has neutral or beneficial effects on PVR 5

Optimize PEEP to avoid both lung derecruitment and overdistension, as both worsen RV afterload 3, 4
PEEP above 15 cmH₂O promotes overdistension and worsens RV systolic dysfunction 3, 4
Avoid driving pressure ≥18 cmH₂O and PaCO₂ ≥48 mmHg as these are risk factors for RV failure 3, 4

For persistent isolated RV failure refractory to medical therapy, RV mechanical support may be indicated 1
Venoarterial ECMO is preferred when the primary lesion is pulmonary vasculature 1, 6

Pulmonary valve replacement is indicated in patients with congenital heart disease (repaired pulmonary stenosis, tetralogy of Fallot) with symptomatic moderate or greater pulmonary regurgitation AND RV dilation or dysfunction (Class I recommendation) 7, 8
Asymptomatic patients with progressive RV dilation and/or dysfunction may also benefit from pulmonary valve replacement (Class IIb recommendation) 7, 8