Results from a retrospective cohort study presented at the American Thoracic Society (ATS) 2015 International Conference in Denver earlier this year show the incidence of pulmonary hypertension (PH) among patients who experienced pulmonary embolism (PE) was twice as high as expected based on previous studies, and that many patients do not receive necessary follow-up tests.1,2,3
“These data indicate that, despite the risk of pulmonary hypertension among pulmonary embolism patients, the use of tests necessary to identify PH-and that determine the specific type of PH-is lacking,” said Dario Mirski, MD, vice president and head, U.S. Medical Affairs, Bayer HealthCare Pharmaceuticals.
The study, which investigated the incidence of PH diagnoses following acute PE in the U.S., concluded the two-year cumulative incidence of PH is 7.6%. The study also evaluated routine disease monitoring practice in PE patients and found nearly half (45%) did not undergo imaging tests that could identify PH within 24 months of their PE diagnosis-even though 87% of these patients showed signs and symptoms suggestive of the disease.4
“The need for improved monitoring of these patients is especially important, as the various types of PH are more prevalent than previously thought,” said Richard Channick, MD, director of Massachusetts General Hospital’s Pulmonary Hypertension and Thromboendarterectomy Program.
Life-Threatening Disorders
PH is a severe, progressive, life-changing and life-threatening disorder of the heart and lungs in which the blood pressure in the pulmonary arteries is above normal, and which can lead to heart failure and death.5,6,7 The most common symptoms of PH include shortness of breath, fatigue, dizziness and fainting, all of which are worsened by exertion.8,9 As the symptoms of PH are non-specific, diagnosis can be delayed by as much as two years.10,11
Another type of PH, chronic thromboembolic pulmonary hypertension (CTEPH), is a progressive and potentially life-threatening disease as well. With CTEPH, it is believed that thromboembolic occlusion (organized blood clots) of pulmonary vessels gradually lead to an increased blood pressure in the pulmonary arteries, resulting in an overload of the right heart. CTEPH is a rare disease and is comparable in terms of population size to PH, though there are fewer diagnoses made so far. CTEPH may evolve after prior episodes of acute pulmonary embolism, but the pathogenesis is not yet completely understood.
The standard and potentially curative treatment for CTEPH is pulmonary endarterectomy (PEA), a surgical procedure in which the blood vessels of the lungs are cleared of clot and scar material. However, a considerable number of patients with CTEPH (20%-40%) are not operable; and in up to 35% of patients, the disease persists or reoccurs after PEA.
VQ Scan
A diagnostic test known as the lung ventilation/perfusion, or VQ scan, an imaging test that measures air and blood flow in the lungs, was created in response to the need for improved monitoring of patients with PH.12 The VQ scan is recommended as the preferred screening diagnostic CTEPH.13
CTEPH has symptoms are similar to those of other more common conditions, such as asthma and chronic obstructive pulmonary disease, as well as other types of PH. However, unlike other types of PH, CTEPH is potentially curable with pulmonary endarterectomy surgery.14 In part because of the nonspecific symptoms, CTEPH is underdiagnosed and often misdiagnosed as another disease.15
“These findings are important because the progression of PH can be slowed with effective treatment,” said Victor Tapson, MD, director of the Venous Thromboembolism and Pulmonary Vascular Disease Research Program at Cedars-Sinai Medical Center in Los Angeles. “Consistent use of accurate PH imaging diagnostics is critical to effective treatment.”
1. Channick R, Joish V, Platt D, et al. Estimating the Incidence of Pulmonary Hypertension in Newly Diagnosed Pulmonary Embolism Patients in the United States. Presented at American Thoracic Society 2015 International Conference; May 2015; Denver, Colorado.
2. Tapson V, Joish V, Platt D, et al. Evaluating Routine Disease Monitoring Practice in Incident Pulmonary Embolism Patients in the United States. Presented at American Thoracic Society 2015 International Conference; May 2015; Denver, Colorado.
3. Channick R, Joish V, Platt D, et al. Estimating the Incidence of Pulmonary Hypertension in Newly Diagnosed Pulmonary Embolism Patients in the United States. Presented at American Thoracic Society 2015 International Conference; May 2015; Denver, Colorado.
4. Tapson V, Joish V, Platt D, et al. Evaluating Routine Disease Monitoring Practice in Incident Pulmonary Embolism Patients in the United States. Presented at American Thoracic Society 2015 International Conference; May 2015; Denver.
5. Rosenkranz, S. Pulmonary hypertension: current diagnosis and treatment. Clin Res Cardiol 2007;96:527-541.
6. Macchia, A et al. A meta-analysis of trials of pulmonary hypertension: A clinical condition looking for drugs and research methodology. Am Heart J 2007;153:1037-1047
7. PHA website. Available from: http://www.phassociation.org/AboutPH Last accessed: July 2013
8. McKenna, S et al. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): A measure of health-related quality of life and quality of life for patients with pulmonary hypertension. Qual Life Res 2006;15:103-115.
9. PHA website. Available from: http://www.phassociation.org/AboutPH Last accessed: July 2013
10. Armstrong, I et al. The trajectory to diagnosis with pulmonary arterial hypertension: a qualitative study. BMJ Open 2012; 2:e000806.
11. Peacock, JA. Treatment of pulmonary hypertension. BMJ 2003; 326:835-836.j
12. NHLBI. What is a Lung Ventilation/Perfusion Scan? Available at: https://www.nhlbi.nih.gov/health/health-topics/topics/lvq. Accessed March 26, 2015.
13. Piazza G and Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351-360.
14. Kim et al. J Am Coll Cardiol 2013; 62: D92-9
15. Tapson V et al. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006;3:564-7.
Source: Bayer HealthCare Pharmaceuticals Inc. is the U.S.-based pharmaceuticals business of Bayer HealthCare LLC, a subsidiary of Bayer AG. www.bayer.com.
