How is right ventricular dysfunction managed?

Management of Right Ventricular Dysfunction

The management of right ventricular dysfunction remains largely uncertain with no specific medical therapy showing definitive benefit, and treatment should be directed at the underlying cause while optimizing preload, improving contractility, and reducing afterload. 1

Diagnostic Evaluation

• Imaging assessment:

  • Cardiovascular Magnetic Resonance (CMR) is the gold standard for quantifying RV function 1
  • Transthoracic echocardiography should be performed to assess:
    • RV size and function
    • Tricuspid regurgitation
    • Presence of subpulmonic obstruction
    • Venous pathway obstruction (in patients with atrial switch)
    • Baffle leaks 1

• Additional testing:

  • ECG to identify arrhythmias and conduction abnormalities
  • Holter monitoring to detect bradycardia and arrhythmias
  • Exercise testing to evaluate functional capacity 1

Medical Management

Heart Failure Medications

• Angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), and aldosterone antagonists:

  • No statistically significant improvement in RV ejection fraction, ventricular dimensions, or exercise capacity has been demonstrated 1
  • Current evidence does not support routine use for asymptomatic RV dysfunction 1

• Beta blockers:

  • Limited data to support recommendations 1
  • Caution advised due to:
    • Potential for bradycardia
    • Limited distensibility of interatrial baffle creating preload-limited physiology 1

• Inotropic support:

  • For acute RV failure with hemodynamic compromise:
    • Dobutamine (low-dose) improves RV function 2, 3
    • Milrinone (phosphodiesterase type III inhibitor) reduces pulmonary vascular resistance and improves RV function, though hypotension is a common side effect 2, 3
    • Levosimendan may provide short-term improvement in RV performance 2, 3

Pulmonary Vasodilators

• For patients with pulmonary hypertension contributing to RV failure:
  • Inhaled nitric oxide (5-10 ppm)
  • Inhaled prostacyclin (20-30 ng/kg/min) 2
  • Prefer inhaled over systemic agents to reduce side effects 3

Volume Management

• Avoid volume overload in dysfunctional right ventricles 2, 3
• Close monitoring of RV response to fluid administration is essential as volume loading may worsen RV performance 3
• Use blood products rather than crystalloids for volume resuscitation when needed 2

Vasopressor Support

• Norepinephrine (low-dose) is effective for maintaining systemic pressure and improving RV coronary perfusion 2, 3
• Vasopressin (low-dose) may be useful for resistant vasodilatory shock 3
• Maintain systemic systolic arterial pressure close to RV systolic pressure to ensure adequate RV perfusion 4

Management Based on Etiology

RV Dysfunction in Congenital Heart Disease

• Regular follow-up with ACHD cardiologists (every 6-12 months depending on severity) 1
• CMR imaging every 12-24 months to assess RV function 1
• For patients with d-TGA with atrial switch:
  • Assess for baffle leaks or obstruction
  • Monitor for arrhythmias
  • Consider ICD implantation based on full clinical presentation in consultation with electrophysiologists with ACHD expertise 1

RV Dysfunction Due to Pulmonary Hypertension

• Treat the underlying cause of pulmonary hypertension
• Consider pulmonary vasodilators as mentioned above 2, 3
• Limit positive end-expiratory pressure (PEEP) in patients with massive pulmonary embolism 2
• Use low tidal volumes (approximately 6 ml/kg) and maintain plateau pressure <30 cm H₂O 2

RV Dysfunction in Chronic Lung Disease

• Target correction of hypoxemia
• Improve pulmonary gas exchange and mechanics
• No data support the use of pulmonary hypertension-specific therapies for RV dysfunction secondary to chronic lung disease 5

Advanced Therapies for Refractory RV Failure

• Mechanical circulatory support:

  • Extracorporeal membrane oxygenation (ECMO) for severe RV failure not responding to medical therapy 2, 3
  • RV assist devices for selected patients 3

• Heart transplantation for end-stage disease 6

Monitoring and Follow-up

• Regular assessment of RV function with imaging (echocardiography or CMR)
• Holter monitoring every 12-24 months depending on clinical status 1
• Exercise testing every 12-36 months based on severity 1

Common Pitfalls and Caveats

• Overuse of volume loading can worsen RV function in patients with RV dysfunction
• Systemic hypotension from pulmonary vasodilators can compromise RV perfusion
• Bradycardia from beta blockers can be problematic, especially in patients with atrial switch repairs
• Lack of evidence-based guidelines for medical therapy of RV dysfunction necessitates individualized approaches
• Underrecognition of RV dysfunction can lead to delayed treatment and worse outcomes

Despite speculation about potential benefits in more symptomatic patients or those with larger/more dysfunctional right ventricles, current evidence does not provide conclusive support for standard heart failure medications in RV dysfunction. Management should focus on treating the underlying cause while optimizing hemodynamics.