Operable and inoperable CTEPH similar but different
MedWire News: Patients with operable and inoperable chronic thromboembolic pulmonary hypertension (CTEPH) present with similar symptoms but differ in terms of related medical conditions, study findings indicate.
Joanna Pepke-Zaba (Papworth Hospital, Cambridge, UK) and colleagues reviewed clinical characteristics and current management of 679 patients enrolled in a prospective international CTEPH registry. They paid particular attention to the similarities and differences between patients with operable and inoperable disease.
On the basis of the surgeon's assessment, 427 (63.3%) patients were considered operable and 247 (36.6%) inoperable (five had missing data). Overall, 386 patients (56.8%) underwent surgery.
The operable group was significantly younger (median 61 vs 67 years) and included a greater proportion of men (53.4% vs 44.5%) than the inoperable group.
At diagnosis, a median of 14.1 months had passed since symptoms first appeared, and this time period was not significantly different between the two groups.
A history of acute pulmonary embolism (PE) was reported for 74.8% of patients, while 56.1% had previously had deep vein thrombosis, a finding that supports the thromboembolic cause of CTEPH, says the team.
The proportion of patients with previous PE did not differ significantly between the groups (77.5% operable, 70.0% inoperable), but significantly more patients with operable CTEPH had massive PE, compared with those with inoperable CTEPH, at 47.1% versus 29.4%.
Operable patients were also more likely to have received thrombolytic therapy with their prior PE but were less likely to have other causes of pulmonary hypertension, such as chronic obstructive pulmonary disease, than inoperable patients.
Operable patients did not differ from inoperable patients in terms of presenting symptoms (dyspnea, edema, fatigue, chest pain, syncope), New York Heart Association class, and hemodynamics.
There were significant differences, however, in the occurrence of associated medical conditions, with thrombophilic disorders more common in operable patients and splenectomy and cancer more common in inoperable patients.
Of note, the proportion of patients receiving pulmonary arterial hypertension-targeted treatment at diagnosis varied by country, from 2.2% to 88.9%. There was also wide variation in operability, with low-volume centres reporting more significantly inoperable patients than high-volume centres, at 47.1% versus 34.4%.
This shows that "the indication for pulmonary endarterectomy is not clearly defined, and is dependent on the experience of the surgical team," write Pepke-Zaba et al in the journal Circulation.
They conclude: "With surgical progress constantly extending the selection of patients who can benefit from surgery, a consensus among experts is needed to reassess the criteria for operability."
By Laura Dean