What is the recommended treatment for patients with pulmonary hypertension and nonischemic dilated cardiomyopathy?

Treatment of Pulmonary Hypertension in Nonischemic Dilated Cardiomyopathy

For patients with pulmonary hypertension and nonischemic dilated cardiomyopathy, the recommended treatment is guideline-directed medical therapy (GDMT) for heart failure with reduced ejection fraction, including ACE inhibitors/ARBs, beta-blockers, and mineralocorticoid receptor antagonists, with careful optimization of volume status using diuretics. PAH-specific therapies are not recommended for this population.

Understanding the Condition

Pulmonary hypertension (PH) in nonischemic dilated cardiomyopathy (DCM) is classified as Group 2 PH (PH due to left heart disease). The presence of PH in DCM patients significantly worsens prognosis:

• PH is independently associated with increased cardiac death in DCM patients 1
• Early studies showed that PH predicted mortality and morbidity in dilated cardiomyopathy 2

Treatment Algorithm

First-Line Therapy: Optimize Heart Failure Management

1. Guideline-Directed Medical Therapy (GDMT):

   • ACE inhibitors/ARBs or preferably ARNI (Angiotensin Receptor-Neprilysin Inhibitor)
   • Beta-blockers (shown to be protective against adverse outcomes in DCM patients) 3
   • Mineralocorticoid receptor antagonists
   • SGLT2 inhibitors
   • Careful uptitration to target doses 4

2. Volume Management:

   • Diuretic therapy is indicated for patients with signs of right ventricular failure and fluid retention 4
   • Optimize volume status with careful monitoring, which may require invasive assessment in severe heart failure 4

3. Device Therapy Consideration:

   • ICD therapy is recommended for primary prevention of sudden cardiac death in selected patients with nonischemic dilated cardiomyopathy 4
   • Consider cardiac resynchronization therapy (CRT) in appropriate candidates

Additional Supportive Measures

• Continuous long-term oxygen therapy if arterial blood O₂ pressure is consistently less than 60 mmHg 4
• Immunization against influenza and pneumococcal infection 4
• Supervised exercise rehabilitation for deconditioned patients 4
• Psychosocial support 4

Important Considerations

Avoid Potentially Harmful Therapies

• PAH-specific therapies (endothelin receptor antagonists, PDE-5 inhibitors, prostacyclins) are not recommended for PH due to left heart disease 4
• Recent clinical trials have shown no benefit of PAH therapies in PH due to left heart failure 4
• Calcium channel blockers are not recommended as routine treatment in heart failure with reduced ejection fraction 4

Monitoring and Follow-up

• Regular clinical assessment including WHO functional class evaluation
• Echocardiography to assess cardiac function and pulmonary pressures
• BNP/NT-proBNP monitoring
• Consider referral to specialized centers with multidisciplinary teams for optimal management 4

Common Pitfalls to Avoid

1. Using PAH-specific therapies: Despite their effectiveness in Group 1 PH, these medications have not shown benefit and may be harmful in PH due to left heart disease.

2. Inadequate diuresis: Insufficient diuresis can lead to persistent congestion and worsening of PH.

3. Underutilization of beta-blockers: Beta-blockers have been shown to be protective in DCM patients, and their absence is associated with increased risk of adverse events 3.

4. Failure to optimize GDMT: Medications should be uptitrated in small increments to target doses or highest tolerated doses 4.

5. Overlooking the need for device therapy: ICD therapy for primary prevention should be considered in appropriate candidates with nonischemic DCM 4.

By focusing on optimizing heart failure therapy rather than targeting pulmonary hypertension directly, clinicians can improve outcomes in patients with nonischemic dilated cardiomyopathy and associated pulmonary hypertension.