What is the treatment for corpulmonale (right-sided heart failure)?

Treatment of Cor Pulmonale (Right-Sided Heart Failure)

The primary treatment for cor pulmonale is diuretics to manage fluid overload, combined with supplemental oxygen to correct hypoxemia and reduce pulmonary vascular resistance, while aggressively treating the underlying pulmonary or cardiac disease. 1, 2

Immediate Management Priorities

Oxygen Therapy

• Supplemental oxygen is essential and should be administered to maintain oxygen saturation above 90% to prevent hypoxia-induced increases in pulmonary vascular resistance 2, 3
• Hypoxia, acidosis, and hypercapnia all increase pulmonary vascular resistance and worsen right ventricular failure, so these must be corrected 1
• Avoid positive pressure ventilation when possible, as it increases RV afterload 1

Fluid Management

• Loop diuretics are first-line therapy for systemic venous congestion and peripheral edema 1, 2
• Spironolactone may be added to enhance diuresis in refractory cases 1, 2
• A critical caveat: excessive fluid administration must be avoided, as RV overdistention causes leftward septal shift that compromises left ventricular filling and worsens cardiac output 1
• Target weight loss of 0.5-1.0 kg daily during active diuresis 2

Hemodynamic Support

For Hypotension

• Vasopressors (norepinephrine or vasopressin) should be used to maintain coronary perfusion pressure 1
• Norepinephrine dosing: 0.05-3.3 mcg/kg/min 1

For Reduced Cardiac Output

• Inotropic agents improve right ventricular contractility and provide pulmonary vasodilation 1, 2
• Dobutamine: 2.5-5.0 mcg/kg/min (higher doses may compromise RV filling due to tachycardia) 1
• Milrinone: 0.25-0.75 mcg/kg/min (monitor for systemic hypotension) 1
• Digoxin has limited evidence but may be considered for refractory cases 2

Pulmonary Vasodilator Therapy

Specific interventions to decrease pulmonary vascular resistance should be used when pulmonary hypertension is the primary driver: 1, 2

• Sildenafil: 20 mg three times daily (oral or via nasogastric tube) 1
• Inhaled nitric oxide: 5-40 ppm (monitor methemoglobin levels every 6 hours; avoid abrupt discontinuation) 1
• Inhaled prostacyclin: 10-50 ng/kg/min 1
• Intravenous prostacyclin: Start at 1-2 ng/kg/min via central line (side effects include systemic hypotension, nausea, headache) 1
• Calcium channel blockers for primary pulmonary hypertension 1, 2

Important pitfall: Pulmonary vasodilators should be used with caution in cor pulmonale secondary to parenchymal lung disease, as they can worsen ventilation-perfusion mismatch and compromise gas exchange 4

Treatment of Underlying Causes

Pulmonary Embolism

• Anticoagulation, thrombolytics, or thrombectomy as indicated 1, 2

Pulmonary Infection

• Antibiotics for at least 10 days; delay surgery if possible until infection clears 1, 2

COPD and Chronic Lung Disease

• Optimize bronchodilator therapy (beta-agonists and anticholinergics) continued until day of surgery 1
• Consider short-term systemic or inhaled corticosteroids for exacerbations 1
• Treatment is primarily directed at the underlying pulmonary disorder rather than the right ventricular failure itself 3

Bacterial Endocarditis

• Appropriate antibiotic therapy when infection is the underlying cause 1, 2

Ventilatory Support

• Non-invasive positive pressure ventilation (CPAP or BiPAP) is preferred over intubation when possible 1
• Endotracheal intubation should be avoided if possible, as positive pressure ventilation increases RV afterload 1, 5
• When mechanical ventilation is required, optimize settings to minimize mean airway pressure 5

Medications with Limited or No Benefit

• Digoxin has limited evidence for efficacy in right-sided heart failure 1
• ACE inhibitors have not shown significant utility, at least acutely 4
• Routine use of corticosteroids is not justified in acute cor pulmonale 1

Long-Term Management

• Anticoagulation may decrease mortality in some patients with pulmonary hypertension and cor pulmonale 4
• Sodium restriction to 2-3 grams daily enhances diuretic effectiveness 2
• Daily weight monitoring is essential 2
• When medical therapies fail, lung or heart-lung transplantation may be considered for selected patients 3

Critical Clinical Pearls

• Right ventricular function is particularly sensitive to afterload changes; even modest increases in pulmonary artery pressure can cause steep declines in stroke volume 1, 2
• The right ventricle is preload-dependent, so patients may require careful fluid resuscitation despite appearing volume overloaded 5
• Echocardiography typically reveals a severely dilated hypokinetic right ventricle with septal deviation into the left ventricle 1
• After the initial phase of RV failure, left ventricular failure with cardiogenic pulmonary edema often becomes the prominent finding, requiring adjustment of management strategy 1