Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

Abstract

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

Chronic Thromboembolic Pulmonary Hypertension: An Update

“…These data highlight the natural history of CTEPH, which appears as a potential long-term complication of one or more thromboembolic events [11][12][13][14]. However, why only a minority of patients (cumulative incidence 0.1-9.1%) may develop PH after an initial episode of PE remains to be clarified [13][14][15][16]. Some epidemiological studies have identified some predisposing factors offering some insight as to the etiology of CTEPH [17][18][19].…”

“…As aforementioned, CTEPH is a potentially treatable disease, and the treatment of choice is pulmonary endarterectomy (Figure 3) [12,13,38,39]. This procedure involves a bilateral endarterectomy of the pulmonary arteries with the removal of the obstructive thromboembolic material from the vessel wall.…”

“…As aforementioned, CTEPH is a potentially treatable disease, and the treatment of choice is pulmonary endarterectomy ( Figure 3 ) [ 12 , 13 , 38 , 39 ].…”

“…An analysis of the data from a large prospective international registry of CTEPH patients evidenced a history of acute pulmonary embolism (PE) and a history of vein thrombosis in 74.8% and 56.1% of them, respectively [10]. These data highlight the natural history of CTEPH, which appears as a potential long-term complication of one or more thromboembolic events [11][12][13][14]. However, why only a minority of patients (cumulative incidence 0.1-9.1%) may develop PH after an initial episode of PE remains to be clarified [13][14][15][16].…”

Pulmonary Hypertension: Current Diagnosis, Approach and Treatment at the Dawn of the New European Guidelines

“…Demerouti et al, provide detailed data on Greek CTEPH patients between 2015 and 2019. Ιn total, 98 patients were enrolled; differences between operable and non-operable CTEPH patients in real world are presented [ 13 ]. Finally, Karyofyllis et al, provide data from 180 BPA procedures performed in Greece.…”