This Clinician’s View opinion piece is written by Melisa Wilson, DNP, APRN, ACNP, BC, Pulmonary Hypertension Program Coordinator at AdventHealth Heart, Lung and Vascular Institute.
Categorized by the World Health Symposium for Pulmonary Hypertension (WSPH) as Group 4 pulmonary hypertension (PH), chronic thromboembolic pulmonary hypertension (CTEPH), also known as chronic thromboembolic pulmonary disease (CTEPD) with pulmonary hypertension, is a life-threatening condition. Per the 2022, ESC/ERS guidelines hemodynamically CTEPH is characterized by the following:
- A mean pulmonary artery pressure (mPAP) ≥ 20 mmHg
- A pulmonary artery occlusion pressure of < 15 mmHg
- A pulmonary vascular resistance of > 2WU
- The presence of partially or completely occlusive thrombi in the main, lobar, segmental, or sub-segmental pulmonary arteries that are present after at least 3 months of effective anticoagulation.
Although potentially curable with pulmonary thromboendarterectomy (PTE) surgery, CTEPH often goes undiagnosed because of the nonspecific nature of its symptoms.
At the AdventHealth Pulmonary Hypertension Care Center, we specialize in providing comprehensive diagnosis and treatment of all five WHO classifications of PH, including CTEPH, and are one of only four institutions in the state to earn accreditation from the Pulmonary Hypertension Association as a Center of Comprehensive Care.
It has been estimated that as many as 1 out of 25 pulmonary embolism (PE) patients previously treated with ≥3 months of anticoagulation therapy could develop CTEPH. However, not all CTEPH patients have a history of PE.
The most common CTEPH symptoms, which can mimic more common diseases like asthma, chronic obstructive pulmonary disease (COPD) and other types of PH, include the following:
- Exercise intolerance
- Chest discomfort
- Peripheral leg edema
A Collaborative and Comprehensive Approach to Diagnosing CTEPD
To help ensure timely and proper diagnosis, any patient suspected to have PH should receive a ventilation/perfusion (V/Q) lung scan to screen for CTEPD. If the V/Q scan shows mismatched perfusion defects, the next step is to perform a CT pulmonary angiography (CTPA) to look for signs of chronic, organized, fibrotic clots. Findings on imaging may demonstrate a mosaic perfusion pattern, dilatation of proximal pulmonary arteries and right heart chambers, and the presence of vascular stenosis or obstruction.
Because patients can have CTEPD with or without PH, we also perform right heart catheterization (RHC) to evaluate for the presence of PH and differentiate the diagnosis. Careful review of diagnostics is essential to making the correct diagnosis. Currently, there is no targeted treatment of CTEPD without PH, but surveillance is required.
Prior to a patient’s appointment at the AdventHealth Pulmonary Hypertension Care Center, our intake team gathers all pertinent medical records, including V/Q, RHC, pulmonary angiography (CPTA), pulmonary function test (PFT), echocardiogram, labs and images. For convenience, both our medical and surgical teams typically see the patient on the same day. Any additional testing needed is ordered after the evaluation.
Once all pertinent testing is complete, our multidisciplinary patient review committee (PRC) meets to review all the clinical data and decide about the patient’s candidacy for the various treatment options — PTE, balloon pulmonary angioplasty (BPA) or medical management. The team invites and encourages the referring physician to participate virtually in the PRC and share any additional background information on the patient with the group.
Unlike other types of PH, CTEPH can potentially be cured by PTE, also called pulmonary endarterectomy (PEA) — a complex surgical procedure used to remove obstructing lesions in the lungs and restore pulmonary hemodynamics. The international registry of incident cases of CTEPH has reported a 3-year survival of 90% in those receiving PTE versus 70% in those who did not have surgery. At AdventHealth’s Pulmonary Hypertension Care Center, our experienced cardiothoracic surgeon Robert Duane Davis, MD, performs all PTE surgeries.
Some CTEPH patients are found to have inoperable disease, typically because of distant or inaccessible lesions. For these cases, a PH-targeted medical therapy called riociguat is recommended. BPA is another potentially effective but challenging therapeutic treatment option for CTEPH, and it is also sometimes used to address residual pulmonary hypertension after PTE.
When to Refer a Patient for CTEPH Evaluation — BREATHLESS
One of our physicians, Stacy Mandras, MD, has developed the following acronym to help guide physicians in identifying patients who should be evaluated for CTEPH:
Risk Factors: (HIV 0.5%, portal hypertension 5-10%, drugs (anorexigens), congenital heart disease (5-10%)
ECG/Echo suggest PH (PASP>40, right heart enlargement)
Absence of left heart pathology
Thoracic imaging suspicious for CTEPH (CTA or V/Q)
History of family member with pulmonary arterial hypertension (PAH) or unexplained death
Lung disease, especially interstitial lung disease
Systemic sclerosis; but also other connective tissue diseases (systemic lupus erythematous, rheumatoid arthritis, mixed connective tissue disease, psoriatic arthritis/Sjögren's, sarcoidosis)
Syncope is OMINOUS, refer urgently!
As with many medical conditions, making a prompt and proper diagnosis of CTEPH can save lives. I encourage clinicians to use this BREATHLESS guideline to help identify patients who may require further evaluation.