Pulmonary Hypertension after PE – CTEPH

Pulmonary hypertension affects up to 4 % of PE patient and typically occurs within the first 2 years after a PE.  It is defined as a mean pulmonary artery pressure of ≥ 25 mm Hg by right heart catheterization, with normal pulmonary capillary wedge pressure, and elevated pulmonary vascular resistance.  Pulmonary hypertension occurring after an episode of PE or due to asymptomatic recurrent thrombosis is referred to as Chronic Thrombo-Embolic Pulmonary Hypertension (CTEPH).  As it eventually leads to right heart failure and death, the condition is to be taken seriously and some patients with previous PE should be evaluated for it.  Risk factors for its development are larger perfusion defects (i.e. larger PEs), recurrent PE, female gender, younger age.  Progressive dyspnea with exercise is the classic presentation.  As the disease progresses, additional symptoms may be present: chest pain, light-headedness, syncope, and eventually symptoms of right heart failure.

Whom to screen?

The following patients should be evaluated for pulmonary HTN after they had a PE:  the patient with (a) history of large PE, (b) shortness of breath, limited recovery of exercise tolerance after the PE episode and, (c) symptoms or signs of right heart failure after a PE.  Typically, patients improve from their PE in the weeks to months following the acute PE, and within 6, 9 or 12 months a steady state is reached.  Certainly, there is some variability from patient to patient.  However, if there has been no significant improvement at 6-9 months or improvement has reached a plateau at an impaired functional level, then evaluation for pulmonary HTN is appropriate.

How to screen?

The following tests are appropriate:

1. Pulse oximetry at rest and after climbing stairs in clinic (or formal 6 minute walk test in a pulmonary function laboratory);
2. Cardiac echo with focus on the right heart and estimation of pulmonary artery pressure;
3. VQ scan to look for chronic/previous/old PE.  A CTA (CT angiogram) of the chest is NOT sensitive for chronic PE screening;
4. If any of the these screening test raise the suspicion for presence of pulmonary hypertension, then:

• Referral to a pulmonologist with expertise and interest in pulmonary hypertension, and
• Right heart catheterization with pulmonary artery pressure measurements and pulmonary arteriography (if the V/Q scan is abnormal).

Treatment

• Indefinite anticoagulation should be given in the absence of contraindications in the patient with pulmonary HTN due to PE (i.e.CTEPH) [ref 1].
•  Pulmonary endarterectomy in a specialized center should be considered.
• Other pulmonary arterial HTN medications (Bosentan, etc) may be considered by the pulmonologist for patients with CTEPH who are not surgical candidates or who have residual pulmonary HTN after endarterectomy [ref 1]

Professional Guidelines

1. American Heart Association (AHA) Scientific Statement [ref 1]
2. AHA, ACCP (American College of Chest Physicians) and PHA (Pulmonary Hypertension Association) Consensus Document 2009 [ref 2]
3. European Guideline [ref 3]

Support

1. Clot Connect Support Forum
2. The Pulmonary Hypertension Association (PHA)

References

1. Jaff MR et al. Management of massive and submassive pulmonary embolism, iliofemoral deep vein thrombosis, and chronic pulmonary hypertension. Circulation 2011;123:1788-1830.
2. McLaughlin VV et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009 Apr 28;53(17):1573-619.
3. Galie N et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J.  2009 Oct;30(20):2493-537. Epub 2009 Aug 27.

Acknowledgement:  This blog entry was written in collaboration with Dr. Hubert (Jimmy) Ford, Assistant Professor, Division of Pulmonary , Critical Care and Sleep Medicine, UNC School of Medicine, Chapel Hill, NC. Dr. Ford directs the UNC Pulmonary Hypertension Clinic.

For Patients:  This same information, written for patients and other non-health care professionals, can be found here.

Disclosure: The authors have no financial conflict of interest relevant to this educational post.

Last updated:  June 29th, 2011

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