The impact of cardiovascular comorbidities associated with risk for left heart disease on idiopathic pulmonary arterial hypertension: Data from the Hellenic Pulmonary Hypertension Registry (HOPE).

Whereas younger female patients were diagnosed with idiopathic pulmonary arterial hypertension (IPAH) in 1980s, it is now frequently encountered in elderly patients with cardiovascular comorbidities (CVCs) associated with increased risk for left heart disease. We present data until November 2019 regarding specific features and clinical outcomes of IPAH population from the Hellenic Pulmonary Hypertension Registry (HOPE). Patients were divided into two groups based on the presence of ≥ or <3 CVCs, arterial hypertension, diabetes mellitus, obesity, presence of coronary artery disease, or atrial fibrillation. Overall, 77 patients with IPAH (55.1 [interquartile range, IQR: 24.1] years, 62.8% women) have been recorded. Fifteen patients (19.2%) had ≥3 CVCs, while 25 (32%) were over 65 years old. Patients with ≥3 CVCs were older, presented an almost equal female to male ratio, walked less in 6-min walk test, and had lower mean arterial pulmonary pressure and pulmonary vascular resistance at baseline than patients with less CVCs. Fewer patients with ≥3 CVCs received PAH-specific treatment compared to patients with less comorbidities (n = 11 [73.3%] versus n = 58 [95.5%], p = 0.02). During a median follow-up period of 3.8 (IQR: 2.7) years, 18 patients died (all-cause mortality 24.3%). Male sex and older age were independent predictors of mortality and/or lung transplantation, while CVCs did not have a significant impact on clinical outcomes. In this nationwide, register-based study, the epidemiology of IPAH involves older patients with CVCs, who seem to have less hemodynamic compromise, but worse functional impairment and are treated less aggressively with PAH pharmacotherapy.

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

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).

Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE).

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

Nocturnal hypertension: poor correlation with office blood pressure but strong prognostic factor for target organ damage.

INTRODUCTION: We investigated the correlation between systolic and diastolic nocturnal blood pressure (BP) values and office BP values, as well as parameters of 24-hour ambulatory BP monitoring, in patients with hypertension. In addition, we compared nocturnal hypertensives with nocturnal normotensives regarding their demographic, clinical, and laboratory characteristics, as well as other data from 24-hour BP monitoring. METHODS: The study included 182 consecutive patients who had newly diagnosed, never treated, uncomplicated arterial hypertension. Blood samples were obtained from all patients for the determination of glycaemic and lipidaemic profiles. All underwent a complete echocardiographic examination, including tissue Doppler imaging, measurement of carotid intima-media thickness, measurement of carotid-femoral pulse wave velocity, and determination of the augmentation index of reflected waves (Aix@75), as well as 24-hour ambulatory BP monitoring. The population was divided into nocturnal normotensives (NN, n=77) and nocturnal hypertensives (NH, n=105, nocturnal BP >120/70 mmHg). RESULTS: Although the NH did not differ from the NN as regards the classical cardiovascular risk factors, they showed an excessive inotropic response to exercise (61.9% vs. 22.7%, p=0.028), higher levels of serum uric acid (5.5 ± 1.56 mg/dl vs. 4.7 ± 1.36 mg/dl, p=0.003), as well as greater arterial stiffness, as expressed by a higher carotid-femoral pulse wave velocity (8.6 ± 1.6 m/s vs. 7.9 ± 1.4 m/s, p=0.009), and a greater carotid intima-media thickness (0.74 ± 0.17 mm vs. 0.68 ± 0.15 mm, p=0.007). In addition, although the two groups did not differ significantly as regards office BP values and did not show strong correlations between nocturnal and office BP, both nocturnal diastolic and, especially, systolic BP showed strong correlations with levels of serum uric acid and with subclinical lesions in the heart, central aorta, peripheral vessels, and renal vasculature. CONCLUSIONS: Nocturnal BP is poorly correlated with office BP values. However, the presence of nocturnal hypertension is associated with morphological and functional disturbances of the cardiovascular net. 24-hour ambulatory BP monitoring is an essential tool for revealing this subgroup of hypertensive patients who are at increased cardiovascular risk.