Angioplasty For Chronic Thromboembolic Pulmonary Hypertension Research Articles

Collagen Triple Helix Repeat-Containing Protein 1 Is a Novel Biomarker of Right Ventricular Involvement in Pulmonary Hypertension

BackgroundPulmonary hypertension leads to right ventricular failure, which is a major determinant of prognosis. Circulating biomarkers for right ventricular function are poorly explored in pulmonary hypertension. This study aimed to clarify the significance of collagen triple helix repeat-containing protein 1 (CTHRC1) as a biomarker of right ventricular failure in pulmonary hypertension. MethodsA monocrotaline-induced pulmonary hypertension rat model was used to evaluate right ventricular CTHRC1 expression and its relationship with fibrosis. Next, human plasma CTHRC1 levels were measured in controls (n = 20), pulmonary arterial hypertension (n = 46), and patients with chronic thromboembolic pulmonary hypertension (CTEPH) (n = 64) before the first and after the final balloon pulmonary angioplasty. ResultsCTHRC1 expression was higher in the right ventricles of rats with monocrotaline-induced pulmonary hypertension than in those of controls. CTHRC1 was colocalized with vimentin and associated with fibrosis in the right ventricles. Plasma CTHRC1 levels were higher in human patients with pulmonary arterial hypertension (P = 0.006) and CTEPH (P = 0.011) than in controls. Plasma CTHRC levels were correlated with B-type natriuretic peptide (R = 0.355, P < 0.001), tricuspid lateral annular peak systolic velocity (R = –0.213, P = 0.029), and right ventricular fractional area change (R = –0.225, P = 0.017). Finally, plasma CTHRC1 levels were decreased after the final balloon pulmonary angioplasty (P < 0.001) in CTEPH. ConclusionsCTHRC1 can be a circulating biomarker associated with right ventricular function and fibrosis in pulmonary hypertension and might reflect the therapeutic efficacy of balloon pulmonary angioplasty in CTEPH.

Real-world, very long-term follow up survival of incident patients with pulmonary hypertension

Abstract Background Pulmonary hypertension (PH) is a clinical syndrome characterized by an increase in pulmonary artery pressure. Among the five groups of PH, pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) stand out due to their ominous prognosis without specific treatment. However, very long-term outcomes data are scarce. Purpose We aimed to assess the very long-term survival of PAH and CTEPH patients followed in a Portuguese PH referral center. Methods Between January 2009 and January of 2020, all incident PH cases were consecutively enrolled in a prospective cohort study. A total of 177 patients were followed up for a median of 5.0 [interquartile range 2.3–8.7] years. Kaplan-Meier survival analysis was used to estimate 1-, 5- and 9-year survival and multivariate regression was used to predict independent prognostic factors. Results Mean age was 49±20 years with a clear female preponderance (67%). The most common PH subgroups were congenital heart disease (PAH-CHD) (n=62; 35%), followed by CTEPH (n=52; 29,4%), connective tissue disease (PAH-CTD) (n=31; 17.5%), idiopathic/hereditary PAH (I/HPAH (n=22; 12.4%) and portopulmonary hypertension (PoPH) (n=8; 4.5%) (Table 1). PAH-specific drugs were used in 91% of the patients, dual combination therapy in 47.5%, and triple combination in 12.4%. The remaining 9% successfully received non-pharmacological treatment, namely cardiac surgery in PAH-CHD (n=7) and endarterectomy or angioplasty in CTEPH (n=9). Survival rates at 1-, 5- and 9-years were 97%, 80% and 66%, respectively. Age (hazard ratio [HR] 1.02; 95% CI 1.01–1.04; P=0.049), BNP [HR 2.04 (1.16–3.60); P=0.01], admission for decompensation of heart failure (HF) [HR 3.15 (1.71–5.83); P&amp;lt;0.001] and PH type [P=0.01] were predictors of all-cause mortality. PAH-CHD had the better long-term survival (9-year survival of 83%), whereas PAH-CTD and PoPH were associated with a worse prognosis (9-year survival of 24% and 28%, respectively) (Figure 1). Regarding admissions for decompensated right HF, BNP was an independent predictor [HR 3.39 (2.12– 5.43); P&amp;lt;0.001] and no difference was found between PH etiologies. Conclusions In this cohort of incident PH patients, the overall 9-year survival rate was 66%. PAH-CHD patients had better overall prognosis, while patients with PAH-CTD and PoPH had the worst prognosis. Additionally, older age, higher BNP and admission for HF were associated with higher mortality. Funding Acknowledgement Type of funding sources: None.