Identification of high-risk patients and outcome in chronic thromboembolic pulmonary hypertension

CTEPH is life threatening, but by means of PEA potentially a curable form of pulmonary hypertension.
We studied whether the current detection strategies are sufficient to recognize CTEPH patients, or should a more extensive systematic search be adopted. We concluded that current detection strategies appear sufficient to recognize CTEPH patients. Early recognition of CTEPH is crucial, however the clinical presentation of CTEPH is characterized by non-specific symptoms. We described a diagnostic model, based on fast, simple and non-invasive tests that can safely rule out CTEPH in patients with an episode of PE.
Using cardiac-imaging we studied RV stroke volume (RVSV) response,the extent of the restoration of the systolic and diastolic RV function and it`s time course. In CTEPH, exercise is limited due to a decreased RVSV. After a successful PEA the RVSV response to exercise improved as was the exercise capacity. RV afterload reduction caused an immediate improvement in RV dimension and overall function and resulted in the restoration of cardiac remodeling. The systolic and diastolic RV function showed a biphasic response.
Mortality and morbidity after PEA are increased in “high-risk” CTEPH patients. We studied the effects of preoperative medical treatment on pulmonary hemodynamics, functional capacity and in a subset of patients on RV remodeling and RV function. Treatment with bosentan resulted in improvements on hemodynamics and functional capacity, and in RV remodeling and function.
To predict outcome we used predetermined BNP cut-off levels, and were able to identify patients at high(er) risk for postoperative hemodynamic instability, progressive RV failure and death.