01Approach to the Critical Patient/attcp36

Immediate stabilization

›Airway and oxygenation priorities

›Supplemental oxygen — target SpO2 >=90% to reduce pulmonary vasoconstriction

›COPD patients target SpO2 88–92% to avoid hypercapnic drive suppression

›High-flow nasal cannula if refractory hypoxemia

›Avoid intubation if possible — positive pressure ventilation increases RV afterload

›Prefer NIV (BiPAP) for hypercapnic respiratory failure

›If intubation required, minimize PEEP; use lung-protective tidal volumes

›Escalate to resuscitation bay for hemodynamic instability or acute RV failure

Hemodynamic assessment

›RV failure recognition

›Hypotension with elevated JVP — classic RV cardiogenic shock pattern

›Cool extremities, oliguria, rising lactate

›Tachycardia disproportionate to clinical state

›Avoid large-volume IV fluid resuscitation

›RV is preload-sensitive but easily volume-overloaded

›Boluses >250 mL worsen RV dilatation and interventricular septal shift

›Vasopressors for hypotension — norepinephrine preferred over volume

›Norepinephrine 0.01–0.5 mcg/kg/min titrated to MAP >=65 mmHg

›Vasopressin as adjunct to reduce norepinephrine requirements

Critical interventions

›Inotrope initiation for low cardiac output

›Dobutamine 2–20 mcg/kg/min for RV systolic dysfunction

›Titrate to improved cardiac output and urine output

›Caution: may cause hypotension via systemic vasodilation

›Milrinone 0.125–0.75 mcg/kg/min as alternative PDE-III inhibitor

›Reduces RV afterload via pulmonary vasodilation

›Avoid in hypotension — profound systemic vasodilation risk

02History/hx54

Presenting symptoms

›Dyspnea

›Initially exertional, progressing to rest in advanced disease

›Most common presenting symptom

›Symptoms often exceed degree of lung function impairment

›Orthopnea and PND — more suggestive of left heart failure; can overlap

›Peripheral edema

›Progressive bilateral pitting leg edema, worse at end of day

›Presacral edema in bedridden patients

›Distinguish from RAAS activation edema in COPD without true RV failure

›Exercise intolerance and fatigue

›Often disproportionate to pulmonary function test results

›Exertional syncope or presyncope — indicates critically reduced cardiac output

›Alarm feature requiring urgent evaluation

Symptom context

›Chest pain

›RV ischemia from pressure overload

›Typically non-pleuritic, exertional

›Pleuritic pain — consider acute PE as precipitant

›Abdominal symptoms from hepatic congestion

›Early satiety, nausea, RUQ discomfort

›Abdominal distension from ascites

›Acute vs. chronic onset

›Acute presentation — sudden dyspnea, hemodynamic instability

›Massive PE causing acute cor pulmonale

›ARDS with acute RV failure

›Chronic insidious presentation — progressive dyspnea, edema over months to years

›COPD, ILD, PAH etiologies

03Physical Exam/exam40

Vital signs and general

›Hemodynamic status

›Tachycardia — compensatory in reduced cardiac output

›Hypotension — MAP <65 mmHg indicates decompensated RV failure

›Tachypnea — respiratory rate elevation with hypoxemia

›SpO2 — hypoxemia (<90%) almost universal in significant cor pulmonale

›Cyanosis — central (lips, tongue) in severe hypoxemia

›Clubbing — suggests chronic hypoxemia or underlying ILD

Jugular venous assessment

›JVP elevation

›Most sensitive clinical sign of right heart failure

›Prominent V wave — tricuspid regurgitation

›Prominent A wave — RV diastolic dysfunction, reduced compliance

›Kussmaul sign — JVP rise with inspiration

›Suggests constrictive pericarditis in differential but can occur in severe RV failure

›Hepatojugular reflux — sustained JVP rise with 10 seconds of abdominal pressure

Precordial exam

›Left parasternal heave

›RV enlargement — palpable sustained lift along left sternal border

›Best appreciated with patient supine or in left lateral decubitus

›Indicates significant RV hypertrophy or dilatation

›Loud P2 — pulmonary hypertension marker

›Palpable P2 at left upper sternal border in severe PH

›Widely split S2 in right bundle branch block

›Auscultatory findings

›Holosystolic murmur at left lower sternal border

›Increases with inspiration (Carvallo sign) — tricuspid regurgitation

›Intensity correlates with severity of TR and RV pressure

04Differential Diagnosis/ddx32

Right heart failure mimics

›Left heart failure (HFrEF or HFpEF) — Group 2 PH, most common cause of PH overall

›ICD-10: I50.20 (HFrEF, unspecified)

›Distinguished by elevated PAWP >15 mmHg on right heart catheterization

›Orthopnea, PND, S3 gallop, pulmonary crackles more prominent

›Echocardiography — reduced LVEF or diastolic dysfunction with normal RVSP in early stages

›Constrictive pericarditis

›ICD-10: I31.1

›Kussmaul sign, pericardial knock, equalization of diastolic pressures

›Pericardial thickening and septal bounce on echocardiography

›CT shows pericardial calcification in chronic cases

Acute RV failure causes

›Acute pulmonary embolism — acute cor pulmonale

›ICD-10: I26.09 (PE without acute cor pulmonale), I26.09 variants

›Sudden onset dyspnea, pleuritic chest pain, hemodynamic instability

›Wells score, PERC rule for risk stratification

›CTPA is diagnostic gold standard; RV:LV ratio >0.9 indicates RV strain

›Cardiac tamponade

›ICD-10: I31.4

›Beck triad: hypotension, JVP elevation, muffled heart sounds

›Pulsus paradoxus >10 mmHg; equalization of diastolic pressures on echo

Other edema causes

›Restrictive cardiomyopathy — amyloid, sarcoid, hemochromatosis

›ICD-10: I42.5

›Biatrial enlargement, diastolic dysfunction, normal LVEF

›Speckled pattern on echo (amyloid); tissue Doppler abnormalities

›Nephrotic syndrome — hypoalbuminemia-driven edema

›ICD-10: N04.9

05Laboratory Tests/lt45

Cardiac biomarkers

›BNP / NT-proBNP

›Elevated in RV dysfunction and pressure overload

›Sensitive for tracking disease trajectory and treatment response

›BNP >100 pg/mL or NT-proBNP >300 pg/mL — significant RV stress

›Serial measurements guide diuresis and treatment optimization

›Declining BNP with therapy indicates favorable response

›Rising BNP despite therapy signals need for escalation

›Troponin

›RV ischemia and strain marker

›Elevated in acute RV decompensation and massive PE

›High-sensitivity troponin for serial assessment

›Persistent elevation associated with worse prognosis

Hematology

›Complete blood count

›Polycythemia — secondary to chronic hypoxemia

›Hematocrit >52% in males, >48% in females suggests secondary polycythemia

›Increases blood viscosity and PVR

›Anemia — worsens cardiopulmonary symptoms; correct if present

›Iron studies, B12/folate for reversible causes

Metabolic panel

›Basic metabolic panel

›Renal function — cardiorenal syndrome from reduced cardiac output

›Rising creatinine indicates reduced renal perfusion or diuretic excess

›Electrolytes critical with loop diuretic use: hypokalemia, hyponatremia

›Liver function tests — congestive hepatopathy

›Elevated bilirubin, transaminases, alkaline phosphatase

›Prolonged INR — synthetic dysfunction in severe congestion

06Diagnostic Tests/dt83

Scoring Systems

›WHO Functional Classification for PH

›Class I — no symptoms with ordinary activity

›Class II — slight limitation of physical activity; comfortable at rest

›Class III — marked limitation of physical activity; comfortable at rest

›Class IV — inability to carry out any physical activity; right heart failure symptoms at rest

›Drives treatment escalation and prognosis stratification

›Class III-IV warrants PAH-specific therapy initiation

›Class IV requires urgent specialist involvement

›ESC/ERS Risk Stratification (2022 Guidelines)

›Low risk — WHO FC I–II, 6MWT >440 m, BNP <50 pg/mL, no RV dysfunction

›Intermediate-low risk — 2 of 4 low-risk criteria

›Intermediate-high risk — 3+ low-risk criteria absent

›High risk — WHO FC IV, 6MWT <165 m, BNP >300 pg/mL, pericardial effusion

›Guides initial and escalation therapy decisions

›High-risk presentation warrants early triple combination therapy

›6-Minute Walk Test (6MWT)

›Functional capacity assessment and prognosis tool

›Distance <300 m associated with high mortality risk

›Serial measurements track treatment response

›Reproducible; widely available in outpatient PH programs

ECG

›ECG findings in cor pulmonale

›P pulmonale — peaked P wave >2.5 mm in lead II (right atrial enlargement)

›Right axis deviation — QRS axis >90 degrees

›RV hypertrophy — tall R wave in V1 (R/S ratio >1), deep S in V5–V6

›RV strain pattern — ST depression and T-wave inversion in V1–V4 and inferior leads

›Right bundle branch block — rSR' pattern in V1 (complete or incomplete)

›S1Q3T3 — classic for acute cor pulmonale (PE); sensitivity only ~20%

›ECG abnormalities present in ~87% of PAH patients

›Right axis deviation in 79%; RV hypertrophy in 87%; RV strain in 74%

›Low voltage in COPD due to hyperinflation — reduces ECG sensitivity

MRI

›Cardiac MRI for RV assessment

›Gold standard for RV volume and mass quantification

›RV ejection fraction — most prognostically important parameter

›RV end-diastolic volume index >84 mL/m2 indicates dilatation

›Late gadolinium enhancement — fibrosis at RV insertion points

›Predicts adverse outcomes and treatment response

›LGE at RV-LV junction typical in pressure-overloaded RV

›4D flow MRI — pulmonary artery flow patterns and wall shear stress

›Emerging tool for PAH phenotyping and severity

›Detects pulmonary vascular remodeling before hemodynamic changes

›MRI limitations

›Not available for acute assessment; requires patient cooperation

›Contraindicated with non-MRI-compatible devices

›Gadolinium caution in severe renal impairment (GFR <30 mL/min)

CT

›CT pulmonary angiography (CTPA)

›First-line for acute PE diagnosis — sensitivity ~95%, specificity ~98%

›RV:LV diameter ratio >0.9 on CTPA indicates RV strain

›Interventricular septal bowing toward LV in massive PE

›CTPA signs of chronic PH — mosaic attenuation, enlarged pulmonary arteries

›Main pulmonary artery diameter >29 mm suggests PH

›Pulmonary artery diameter exceeding aorta diameter is 70% sensitive for PH

›High-resolution CT chest

›Identifies underlying lung parenchymal disease

›ILD patterns: UIP (honeycombing), NSIP (ground-glass predominant)

›Emphysema extent and distribution in COPD

›Mosaic attenuation — CTEPH or small vessel PH

›Engorged central pulmonary arteries with peripheral pruning

Ultrasound

›Transthoracic echocardiography — cornerstone diagnostic test

›RV size assessment — RV:LV end-diastolic area ratio

›Ratio >0.6 indicates RV dilatation; >1.0 indicates severe dilatation

›RV basal diameter >41 mm = dilated RV (ASE 2025 guidelines)

›Estimated RVSP from TR jet — Bernoulli equation + RAP

›TR jet velocity >3.4 m/s estimates RVSP >50 mmHg

›Sensitivity 79–100%, specificity 68–98% for PH detection (Cochrane 2022)

›TAPSE (tricuspid annular plane systolic excursion)

›<17 mm indicates RV systolic dysfunction

›Prognostically important in PAH

›D-sign — systolic septal flattening indicates pressure-overloaded RV

›Paradoxical septal motion in volume and pressure overload

›Eccentricity index >1.0 indicates RV pressure or volume overload

›Point-of-care ultrasound (POCUS)

›Rapid ED assessment of RV size, IVC, pericardial effusion

›IVC diameter >2.1 cm with <50% inspiratory collapse — elevated RAP

›Pericardial effusion — suggests PAH severity; tamponade physiology

›B-lines — pulmonary edema vs. dry lung in RV failure

›Right heart catheterization — gold standard

›Confirms PH: mPAP >20 mmHg at rest

›Normal mPAP 8–20 mmHg; PVR >2 Wood units defines precapillary PH

›PAWP <=15 mmHg — precapillary PH (cor pulmonale); >15 mmHg — postcapillary

›Vasoreactivity testing during RHC

›Inhaled iNO 40 ppm or inhaled epoprostenol

›Positive response: mPAP fall >=10 mmHg to <40 mmHg with maintained or increased CO

›Only positive responders benefit from calcium channel blocker therapy

07Disposition/dispo32

ICU admission criteria

›Immediate ICU indications

›Hemodynamic instability, cardiogenic shock, or vasopressor requirement

›MAP <65 mmHg despite initial therapy

›Lactate >=2 mmol/L suggesting tissue hypoperfusion

›Acute cor pulmonale from massive PE

›Hypotension with evidence of RV failure on echo

›Need for systemic thrombolysis or catheter-directed therapy

›Severe refractory hypoxemia despite supplemental oxygen

›Need for inhaled pulmonary vasodilators (iNO, inhaled epoprostenol)

›New arrhythmia with hemodynamic compromise

›Mechanical circulatory support consideration (ECMO, RVAD)

Step-down or telemetry admission

›Indications for monitored bed

›Decompensated chronic cor pulmonale requiring IV diuresis

›Anticipated response to loop diuretics within 24–48 hours

›Stable hemodynamics on oral medications not yet achieved

›New diagnosis of pulmonary hypertension requiring workup

›Echocardiography, RHC, PFTs, CTPA pending

›Significant end-organ dysfunction: cardiorenal or congestive hepatopathy

›Worsening functional status despite outpatient optimization

Discharge criteria

›Criteria for safe discharge

›Hemodynamically stable on oral medications

›Adequate oxygenation on home oxygen regimen

›Euvolemic or near-euvolemic on oral diuretics

›Stable or improving renal function

›Reliable follow-up arranged within 7 days

08Treatment/t102

Oxygenation

›Supplemental oxygen therapy

›Long-term oxygen therapy (LTOT) — only therapy proven to improve survival in COPD-related cor pulmonale

›Indication: resting PaO2 <=55 mmHg or SpO2 <=88% (Class I)

›Indication: PaO2 56–59 mmHg with polycythemia or cor pulmonale signs

›Target SpO2 >=90%; >=15 hours/day for mortality benefit

›Acute supplemental oxygen — target SpO2 >=90%

›COPD: titrate carefully to avoid hypercapnic drive suppression

›High-flow nasal cannula for refractory hypoxemia at 40–60 L/min

Diuresis

›Loop diuretics — volume overload management

›Furosemide IV 40–80 mg for acute decompensation

›Titrate to urine output 0.5–1 mL/kg/hour

›Double dose every 2–4 hours if inadequate diuresis

›Maximum furosemide 400–600 mg/day in refractory cases

›Bumetanide IV 1–3 mg as alternative loop diuretic

›Better oral bioavailability than furosemide (80% vs. 50%)

›Thiazide augmentation for diuretic resistance

›Metolazone 2.5–5 mg orally 30 minutes before loop diuretic

›Monitor electrolytes closely — profound hypokalemia risk

›Electrolyte monitoring with aggressive diuresis

›Potassium replacement to maintain K+ >=4 mmol/L

›Magnesium replacement for hypomagnesemia

PAH-specific pharmacotherapy

›Endothelin receptor antagonists (ERA)

›Ambrisentan 5 mg orally daily (titrate to 10 mg if tolerated)

›Selective ETA antagonist; lower hepatotoxicity than bosentan

›Monthly LFT monitoring; teratogenic — contraception mandatory

›Macitentan 10 mg orally daily

›Tissue-penetrant dual ERA (ETA and ETB)

›SERAPHIN trial: reduced clinical worsening by 45%

›Bosentan 62.5 mg twice daily for 4 weeks then 125 mg twice daily

›Dual ERA; monthly LFT monitoring; significant drug interactions

›PDE-5 inhibitors

›Sildenafil 20 mg orally three times daily

›Increases cGMP; selective pulmonary vasodilator

›Contraindicated with nitrates — profound hypotension

›Tadalafil 40 mg orally once daily

›Once-daily dosing improves adherence

›PHIRST trial: improved 6MWT and clinical worsening

›Prostacyclin pathway agents

›Epoprostenol IV — continuous infusion via tunneled central catheter

›Initial dose 2 ng/kg/min; titrate by 1–2 ng/kg/min every 15 minutes

›Target dose variable (often 20–40 ng/kg/min); individualized

›First-line monotherapy for WHO FC IV PAH (Class I)

›Abrupt discontinuation fatal — mandatory backup supply and pump

›Treprostinil — subcutaneous or IV continuous infusion

›Initial dose 1.25 ng/kg/min; titrate slowly

›Inhaled treprostinil (Tyvaso) — FDA-approved for Group 3 PH (ILD-associated)

›9 breaths (54 mcg) four times daily; titrate by 3 breaths per session

›Selexipag — oral IP receptor agonist

›Initial 200 mcg twice daily; titrate to maximum tolerated dose (up to 1600 mcg twice daily)

›GRIPHON trial: reduced morbidity/mortality composite endpoint

›Soluble guanylate cyclase stimulator

›Riociguat 0.5 mg three times daily; titrate by 0.5 mg every 2 weeks

›Maximum 2.5 mg three times daily

›Approved for both PAH and CTEPH (inoperable or residual after surgery)

›Contraindicated with PDE-5 inhibitors and nitrates

›Activin signaling inhibitor

›Sotatercept 0.3 mg/kg subcutaneously every 3 weeks (titrate to 0.7 mg/kg)

›Novel first-in-class rebalances TGF-beta signaling

›STELLAR trial: improved 6MWT and reduced clinical worsening events

›Approved as add-on to background PAH therapy

Upfront combination therapy

›Initial combination strategy — standard of care for Group 1 PAH

›ERA + PDE-5 inhibitor as initial combination for WHO FC II-III

›AMBITION trial: ambrisentan + tadalafil superior to monotherapy

›Reduced time to clinical failure by 50%

›Triple combination with prostacyclin analogue for high-risk WHO FC III-IV

›ERA + PDE-5 inhibitor + parenteral prostacyclin

›Class I recommendation for functional class IV

Vasoactive medications for acute RV failure

›Vasopressors

›Norepinephrine 0.01–0.5 mcg/kg/min — preferred vasopressor

›Maintains RV coronary perfusion pressure

›Prevents RV ischemia from hypotension-induced coronary hypoperfusion

›Vasopressin 0.01–0.04 units/min as adjunct

›Reduces norepinephrine dose requirements

›Minimal pulmonary vasoconstriction advantage

›Inotropes

›Dobutamine 2–20 mcg/kg/min — RV systolic dysfunction

›Positive inotrope and mild pulmonary vasodilator

›Monitor for tachycardia (>110 bpm) and systemic hypotension

›Milrinone 0.125–0.75 mcg/kg/min — PDE-3 inhibitor

›Loading dose 50 mcg/kg IV over 10 minutes (may omit in hypotension)

›Reduces PVR via pulmonary vasodilation; avoid if MAP <65 mmHg

Treatment of underlying disease

›COPD management

›Optimize bronchodilator therapy — LABA/LAMA combination

›Inhaled corticosteroids per GOLD guidelines if indicated

›Pulmonary rehabilitation for functional capacity

›Avoid systemic vasodilators — CCBs and ACE inhibitors worsen V/Q mismatch and lower PaO2 in COPD

›CTEPH treatment

›Pulmonary endarterectomy — curative for operable CTEPH

›Anticoagulation indefinitely — warfarin or DOAC

›Balloon pulmonary angioplasty for inoperable CTEPH

›OSA/OHS treatment

›CPAP or BPAP — corrects nocturnal hypoxemia and reduces PVR

›Weight loss in obesity hypoventilation syndrome

Mechanical circulatory support

›Advanced support for refractory RV failure

›Veno-arterial ECMO — bridge to recovery or transplant

›Consider early in massive PE with refractory shock

›Avoid isolated RVAD in severe PAH — risk of pulmonary hemorrhage

›Atrial septostomy — palliative right-to-left shunt to decompress RV

›Reserved for refractory PAH as bridge to transplant

›Lung transplantation — bilateral preferred over single

›Indication: WHO FC III–IV despite maximal medical therapy

›Median survival post-transplant ~5–7 years

09Special Populations/sp52

Pregnancy

›Pregnancy and cor pulmonale / PAH

›Maternal mortality in PAH historically 30–56%; still 10–25% with modern therapy

›WHO risk Class IV — pregnancy contraindicated in severe PAH

›Termination of pregnancy counseling at initial PAH diagnosis

›Physiologic changes worsening PH

›Increased plasma volume by 40–50% — volume overloads failing RV

›Decreased SVR from hormones — reduces coronary perfusion pressure

›Hypercoagulable state — increased VTE risk

›Management during pregnancy

›Multidisciplinary team — obstetrics, cardiology, PH specialist, anesthesia

›Delivery in center with ECMO capability

›Planned C-section at 34–36 weeks in consultation with team

›Safe medications in pregnancy

›Sildenafil — limited data, generally used as benefit outweighs risk

›Epoprostenol IV — safest prostacyclin in pregnancy; no teratogenicity data

›Anticoagulation: LMWH preferred over warfarin (teratogenic in first trimester)

›Contraindicated medications

›ERAs (bosentan, ambrisentan, macitentan) — teratogenic; Category X

›Riociguat — Category X; congenital defects in animal studies

›Selexipag — avoid in pregnancy

Geriatric

›Older adults (age >65 years)

›Higher prevalence of Group 2 PH (left heart disease) — must be excluded before PAH-specific therapy

›Diastolic dysfunction common; elevated PAWP distinguishes from cor pulmonale

›RHC essential — echocardiographic estimation less reliable in elderly

›Medication considerations

›Increased risk of hypotension from PDE-5 inhibitors — start at lower doses

10Background/b57

Epidemiology

›Prevalence and incidence

›Cor pulmonale occurs in up to 40% of patients with severe COPD (FEV1 <35% predicted)

›Estimated 6% of all cardiac disease in adult patients in the United States

›COPD affects 300 million people worldwide; cor pulmonale is major complication

›PAH prevalence: 25–50 cases per million population in Western countries

›Female predominance in idiopathic PAH — 3:1 female to male ratio

›Mean age of diagnosis idiopathic PAH: 36–50 years (historically younger women)

›Group 3 PH (lung disease) — most common worldwide cause of PH after left heart disease

›IPF complicated by PH in ~30–40% of cases; associated with worse prognosis

›OSA-related PH: 17–70% prevalence depending on severity

›Mortality

›Cor pulmonale in COPD — median survival 5 years after edema onset without oxygen

›LTOT extends survival: 5-year survival 62% in HOT trial

›3-year transplant-free survival with modern PAH therapy: ~70–80% in Group 1

Pathophysiology

›Primary mechanism

›Chronic alveolar hypoxia → hypoxic pulmonary vasoconstriction

›Sustained HPV leads to vascular remodeling — smooth muscle hypertrophy and intimal proliferation

›Elevated RV afterload — increased pulmonary vascular resistance (PVR)

›RV adaptation sequence

›Compensated phase: RV hypertrophy maintains cardiac output

›Decompensated phase: RV dilatation, tricuspid regurgitation, systemic congestion

›RV-LV interaction: septal shift reduces LV compliance and cardiac output

›Vascular remodeling mechanisms

›Endothelial dysfunction — impaired prostacyclin and nitric oxide production

›Endothelin-1 overproduction — potent vasoconstrictor and smooth muscle mitogen

›BMPR2 pathway dysfunction in heritable PAH — promotes vascular smooth muscle proliferation

›Inflammation and oxidative stress

›Perivascular inflammatory infiltrates in PAH plexiform lesions

›Reactive oxygen species amplify vasoconstriction and vascular remodeling

›Classification system — WHO/ESC 2022

›Group 1: PAH — idiopathic, heritable, drug/toxin-induced, associated conditions

›Group 2: PH due to left heart disease (most common)

›Group 3: PH due to lung disease and/or hypoxia (COPD, ILD, OSA)

›Group 4: CTEPH and other pulmonary artery obstructions

›Group 5: PH with unclear and/or multifactorial mechanisms

Therapeutic Considerations

›Evidence base for treatments

›LTOT in COPD — MRC trial: reduces pulmonary artery pressure and polycythemia; improves survival

›NOCTURNAL oxygen in non-severe resting hypoxemia — no survival benefit (LOTT trial)

›Treatment of underlying lung disease does not reliably reverse established cor pulmonale

›PAH combination therapy — AMBITION trial (2015)

›Ambrisentan + tadalafil reduced primary composite endpoint by 50% vs. monotherapy

›Established upfront combination as standard of care for treatment-naive PAH

›Sotatercept STELLAR trial (2023)

›Improved 6MWT distance by 40 m vs. placebo; reduced clinical worsening risk

›Novel mechanism: rebalances activin-BMPR2 signaling

›Treatment gaps and emerging evidence

›Group 3 PH (lung disease) — macitentan failed to improve outcomes in IPF-PH (MUSIC trial)

›Inhaled treprostinil showed benefit in ILD-associated PH (INCREASE trial) — FDA approved

›No PAH-specific therapy approved for COPD-related PH; cautious use only in severe Group 3 PH at expert centers

›Right heart failure — AHA Scientific Statement 2018

›Highlights lack of RCT data specific to RV failure management

›Recommends individualized approach based on etiology and hemodynamics

›ICD-10 coding

›Cor pulmonale (chronic): I27.81

›Acute cor pulmonale: I26.09 (PE-related); I27.82 (chronic pulmonary heart disease)

›Primary pulmonary hypertension: I27.0

›Secondary pulmonary hypertension: I27.2

11Patient Discharge Instructions/pdi35

copy discharge instructions

›Discharge instructions for Cor Pulmonale

›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

›Stop smoking completely — smoking worsens lung disease and increases pulmonary pressure

›Vaccinations

›Annual influenza vaccine

›Pneumococcal vaccine (Prevnar 20 or Pneumovax 23) if not yet received

›Return to the emergency department immediately for

›Sudden worsening of shortness of breath at rest

›New or rapidly worsening leg swelling or abdominal bloating

12References/r38

Guidelines and key sources

›ESC/ERS 2022 Guidelines for Diagnosis and Treatment of Pulmonary Hypertension

›Humbert M, Kovacs G, Hoeper MM, et al. European Respiratory Journal 2023

›Comprehensive risk stratification and treatment algorithm for all PH groups

›Updated diagnostic threshold: mPAP >20 mmHg (previously >25 mmHg)

›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

Evidence & Review

Evidence & Review