You have been treated for cor pulmonale — enlargement and strain of the right side of your heart caused by high blood pressure in your lung blood vessels
This condition requires careful ongoing management of both your lung and heart disease
Missing medications or follow-up appointments can cause rapid worsening
Daily monitoring at home
Weigh yourself every morning after urinating and before eating
Contact your doctor if weight increases more than 1 kg (2 lbs) in one day or 2 kg (5 lbs) in one week
Check your oxygen levels daily if you have a home pulse oximeter; target above 90%
Diet and fluid management
Limit sodium (salt) to less than 2 grams per day — avoid processed foods, canned soups, fast food
Limit fluids to 1.5–2 litres per day if instructed by your doctor
Avoid alcohol — worsens heart muscle function and interacts with medications
Medications — critical points
Take all medications exactly as prescribed, especially oxygen and diuretics (water pills)
Never stop PAH medications suddenly — this can cause a life-threatening crisis
If you run low on PAH medications, contact your specialist immediately; do not wait
Activity and lifestyle
Avoid strenuous exertion, heavy lifting, and Valsalva maneuvers (straining)
Supervised cardiac rehabilitation or pulmonary rehabilitation is recommended
Avoid high altitude travel without medical clearance
Defines WHO functional classification and ESC/ERS risk scoring system
AHA Scientific Statement on Right-Sided Heart Failure (2018)
Konstam MA, Kiernan MS, Bernstein D, et al. Circulation 2018
Evaluation and management framework for RV failure
Emphasizes hemodynamic phenotyping and individualized approach
ASE Guidelines for Echocardiographic Assessment of the Right Heart (2025)
Mukherjee M, Rudski LG, Addetia K, et al. JASE 2025
Updated RV size and function reference values
Guidance for PH assessment including TAPSE, TR velocity, RVSP estimation
Key trials and studies
AMBITION trial (2015) — ambrisentan + tadalafil vs. monotherapy in PAH
50% reduction in primary composite endpoint of clinical failure
Established upfront combination as standard of care for Group 1 PAH
STELLAR trial (2023) — sotatercept in PAH
Improved 6MWT by 40 m; reduced clinical worsening events
Novel activin signaling inhibitor; first new mechanism class in decades
INCREASE trial (2021) — inhaled treprostinil in ILD-associated PH
Improved 6MWT and reduced clinical worsening in Group 3 PH
Led to FDA approval of inhaled treprostinil for ILD-PH
MRC Oxygen Trial — long-term oxygen therapy in hypoxemic COPD
Demonstrated survival benefit and reduction in pulmonary artery pressure
Established LTOT as standard of care for resting hypoxemia
Landmark references
Mandoli GE et al. Cor Pulmonale: The Role of Traditional and Advanced Echocardiography. Heart Failure Reviews 2021
Comprehensive echocardiographic assessment of RV in acute and chronic cor pulmonale
Houston BA, Brittain EL, Tedford RJ. Right Ventricular Failure. NEJM 2023
Contemporary pathophysiology, diagnosis, and management review
Covers acute RV failure, hemodynamic support, and mechanical circulatory support
Wilcox SR, Kabrhel C, Channick RN. Pulmonary Hypertension and RV Failure in Emergency Medicine. Annals of Emergency Medicine 2015
Emergency medicine-focused assessment and management of acute RV failure
Practical guidance for ED resuscitation of decompensated PH
Ruopp NF, Cockrill BA. Diagnosis and Treatment of PAH. JAMA 2022
Review of PAH diagnosis, classification, and evidence-based therapy
Covers combination therapy strategies and emerging agents
Budev MM, Arroliga AC, Wiedemann HP, Matthay RA. Cor Pulmonale: An Overview. Seminars in Respiratory and Critical Care Medicine 2003
Classic comprehensive overview of etiology, diagnosis, and management
SymptomDx is an educational tool for medical professionals. It does not replace clinical judgment. Verify all clinical data and drug dosages with authoritative sources.