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

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Examine the patient:
Physical signs that reflect severity of PH
❑ Loud pulmonary second heart sound (P2)
❑ Systolic murmur suggestive of tricuspid regurgitation
❑ Raised jugular venous pressure (JVP)
❑ Early systolic click
❑ Left parasternal heave
❑ Right ventricular S₄

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Physical signs suggestive of moderate to severe PH
❑ Holosystolic murmur that increases with inspiration
❑ Increased jugular v waves
❑ Pulsatile liver
❑ Diastolic murmur
❑ Hepatojugular relux

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Physical signs suggestive of advanced PH with right ventricular failure
❑ Right ventricular S₃
❑ Distension of jugular veins
❑ Hepatomegaly
❑ Peripheral edema
❑ Ascites
❑ Low BP, cool extremities

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Physical signs suggestive of possible underlying causes
❑ Central cyanosis - Abnormal V/Q, shunt
❑ Clubbing - Congenital heart disease
❑ Cardiac auscultatory findings - Congenital or acquired heart disease
❑ Rales/decreased breath sounds/dullness - Pulmonary congestion
❑ Fine rales, acc. muscle use, wheezing, protracted respiration, cough - Pulmonary parenchymal disease
❑ Obesity, kyphoscoliosis, enlarged tonsils - Disordered ventilation
❑ Sclerodactyly, arthritis, telengiectasia, Raynaud phenomenon, rash - Connective tissue disorder
❑ Peripheral venous insufficiency - Possible venous thrombosis
❑ Venous stasis ulcers - Possible sickle cell disease
❑ Pulmonary vascular bruits - Chronic thromboembolic PH
❑ Splenomegaly, spider angiomata, palmar erythema, icterus, caput medusa - portal hypertension

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

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.