Pulmonary hypertension resident survival guide

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: , Vidit Bhargava, M.B.B.S [2]

Definition

Pulmonary hypertension (PH) is defined by mean pulmonary artery pressure > 25, pulmonary capillary wedge pressure (PCWP), left atrial pressure, or left ventricular end-diastolic pressure (LVEDP) ≤ 15 mm Hg; and a pulmonary vascular resistance (PVR) > than 3 Wood units.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

• Pulmonary embolism

Common Causes

• Congenital heart disease with left-to-right shunt (ASD, VSD, PDA)
• Congestive heart failure
• COPD
• Cor pulmonale
• Interstital lung disease
• Obstructive sleep apnea
• Thromboembolism

Management

Characterize the symptoms: ❑ Progressive dyspnea ❑ Exertional dizziness and syncope ❑ Edema of the extremities ❑ Angina ❑ Palpitations

Examine the patient: ❑ Loud pulmonary second heart sound (P2) ❑ Systolic murmur suggestive of tricuspid regurgitation ❑ Raised jugular venous pressure (JVP) ❑ Peripheral edema ❑ Ascites

Consider alternative diagnosis: ❑ Left sided heart failure ❑ Coronary artery disease ❑ Liver disease ❑ Budd-Chiari syndrome

❑ Anticoagulation +/- ❑ Diuretics +/- ❑ Oxygen therapy +/- ❑ Digoxin

Acute vasoreactivity testing

Positive

Negative

Oral calcium channel blocker (CCB)

Lower risk

Higher risk

❑ Follow closely for efficacy and safety Sustained response

❑ Endothelin receptor antagonsists (ERA's) or Phospodiesterase-5 inhibitors (PDE-5 Is) (oral) ❑ Epoprostenol or Treprostinil (IV) ❑ Iloprost (inhaled) ❑ Treprostinil (SC)

❑ Epoprostenol or Treprostinil (IV) ❑ Iloprost (inhaled) ❑ ERAs or PDE-5 Is ((Oral) ❑ Treprostinil (SC)

❑ Reassess

Continue CCB

In case of absence of response to initial monotherapy: ❑ Consider combo-therapy

In case of progress despite optimal medical treatment: ❑ Investigational protocols, OR ❑ Atrial septostomy, OR ❑ Lung transplant

The following guideline is based on Expert consensus document on pulmonary hypertension published by ACCF/AHA in 2009.

Follow up testing after etiology for pulmonary hypertension is established:

Substrate	Futher action
BMPR2 mutation 1st degree relative of patient with BMPR2 mutation or with 2 or more relatives with PH	Yearly echocardiogram, right heart catheterization if evidence of PH. Genetic counselling for BMPR2 testing, proceed as aboveif positive.
Systemic sclerosis	Yearly echocardiogram, right heart catheterization if evidence of PH.
HIV infection	Do echocardiogram if signs & symptoms are suggestive of PH. Right heart catheterization if evidence of PH on echo.
Portal hypertension	If considering orthotopic liver transplant perform echocardiogram. Right heart catheterization if evidence of PH.
CHD with shunt	Echocardiogram and right heart catheterization at the time of diagnosis. If significant defect - repair.
Recent acute pulmonary embolism	If symptomatic 3 months after event, perform ventilation perfusion scinitigraphy. Do a pulmonary angiogram if positive.
Prior appetite suppressant use (fenfluramine)	Echocardiogram only if symptomatic.
Sickle cell disease	Yearly echocardiogram, right heart catheterization if evidence of PH.

Do's

• The diagnosis of Pulmonary hypertension requires confirmation with a right heart catheterization.
• Objective assessment of treatment measures includes:

• Exercise capacity.
• Hemodynamics.
• Survival.

• Epoprostenol is the only therapy that has been shown to prolong survival in patients with pulmonary hypertension.
• Monitor liver function tests monthly in patients being treated with endothelin receptor antagonists.
• Patients presenting with advanced symptoms, right heart failure, advanced hemodynamics and those on parenteral or combination therapy must be seen every 3 months.

Don'ts

• Do not perform vasospastic testing for those with overt heart failure or hemodynamic instability.