Letter by Hoeper and Gali\ue8 Regarding Article, "hemodynamic, Functional, and Clinical Responses to Pulmonary Artery Denervation in Patients with Pulmonary Arterial Hypertension of Different Causes: Phase II Results from the Pulmonary Artery Denervation-1 Study"

Comments on "Hemodynamic, Functional, and Clinical Responses to Pulmonary Artery Denervation in Patients with Pulmonary Arterial Hypertension of Different Causes: Phase II Results from the Pulmonary Artery Denervation-1 Study

Ipertensione polmonare: Necessit\ue0 di chiarezza tra definizione emodinamica-fisiopatologica, stima ecocardiografica e diagnosi clinica finale. Pulmonary hypertension and the relationships between hemodynamic-pathophysiologic definitions, echocardiographic estimate and final clinical diagnosis: clarification is needed.

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Pulmonary hypertension and pulmonary arterial hypertension: a clarification is needed.

[No abstract available

Current therapeutic approaches to pulmonary arterial hypertension.

Pulmonary hypertension is a heterogeneous hemodynamic and pathophysiological state that is observed in a number of clinical conditions, which have been divided into six diagnostic groups. Although the increase in pulmonary pressure observed in these clinical groups may be similar, underlying disease mechanisms, diagnostic methods, and prognostic and therapeutic consequences are completely different. Pulmonary arterial hypertension is associated with several rare conditions that have comparable clinical and hemodynamic characteristics and exhibit virtually identical anatomical and pathological alterations in the lung microcirculation. These conditions include idiopathic and familial forms of the disease and disease forms associated with connective tissue disease, congenital heart defects involving systemic-to-pulmonary arterial shunts, portal hypertension, and HIV infection. It has been shown that treatment with specific drugs (e.g. prostanoids, endothelin-receptor antagonists and phosphodiesterase type-5 inhibitors) is effective in these patients and that these drugs can also be administered in various combinations. An evidence-based treatment algorithm has been developed for these patients. In patients with pulmonary hypertension due to left heart disease or lung disease, treatment focuses on the underlying condition and there is no convincing evidence that agents approved for pulmonary arterial hypertension are effective. For patients with chronic thromboembolic pulmonary hypertension, the treatment of choice is pulmonary endarterectomy. However, drugs intended specifically for the treatment of pulmonary arterial hypertension may be considered in inoperable cases or after suboptimal surger

PATENT PLUS: A blinded, randomised and extension study of riociguat plus sildenafil in pulmonary arterial hypertension

Abstract PATENT PLUS evaluated the safety and efficacy of riociguat in combination with sildenafil in pulmonary arterial hypertension patients. Patients receiving sildenafil (20\u2005mg three times daily) were randomised to placebo or riociguat (up to 2.5\u2005mg three times daily) for 12\u2005weeks. The primary outcome was maximum change in supine systolic blood pressure (SBP) from baseline within 4\u2005h of dosing. Secondary objectives comprised additional blood pressure, heart rate and exploratory efficacy variables, and safety. Patients could enter a long-term extension (LTE), where all patients received riociguat plus sildenafil. There was no difference in maximum change in supine SBP from baseline within 4\u2005h between the riociguat (n=12) (mean\ub1sd baseline: -20.2\ub115.3\u2005mmHg; week 12: -20.7\ub118.0\u2005mmHg) and placebo groups (n=6) (-7.6\ub13.9 and -20.2\ub112.9\u2005mmHg, respectively). Changes in standing SBP and supine or standing diastolic blood pressure were also not different. Combination therapy showed no favourable effects on exploratory clinical parameters, including haemodynamics and exercise capacity. In the LTE, there were high rates of discontinuation due to hypotension and three (18%) deaths (not considered study drug-related by the investigator). There were potentially unfavourable safety signals with sildenafil plus riociguat and no evidence of a positive benefit/risk ratio. Concomitant use of riociguat with phosphodiesterase-5 inhibitors is therefore contraindicated

Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups.

Abstract AimsThis study compared the clinical, functional, and haemodynamic characteristics and current era survival of subgroups of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD): Eisenmenger syndrome (ES); PAH-CHD associated with systemic-to-pulmonary shunts (SPs); PAH with small defects (SDs); and PAH after defect correction (CDs).Methods and resultsData from consecutive PAH-CHD patients referred to our centre from 1 January 1998 to 31 May 2011 were collected. A contemporary group of idiopathic PAH patients was utilized for comparison. Treatment was per PAH guidelines, including combination therapy, with approved PAH-specific drugs. Survival was assessed with Kaplan-Meier analysis from the first invasive haemodynamic confirmation of PAH and compared across subgroups by log-rank test. Of 192 patients (mean age 41 \ub1 17 years; 61% female), 90 had ES (aged 41 \ub1 16 years); 48 SP (aged 47 \ub1 18 years); 10 SD (aged 25 \ub1 21 years); and 44 CD (aged 36 \ub1 17 years). Patients with ES had the highest baseline pulmonary vascular resistance and the lowest exercise capacity. Seventy-eight per cent were treated with approved PAH-specific drugs, and 44% were treated with combination therapy. Kaplan-Meier survival estimates (95% confidence interval) at 20 years for ES, SP, and CD were 87% (77-93%), 86% (60-96%), and 36% (12-72%, P = 0.0001 vs. ES; P = 0.004 vs. SP), respectively, and at 15 years for SD was 66% (16-91%, P = 0.015 vs. ES; P = 0.016 vs. SP). The survival of the 278 patients with idiopathic PAH appeared to be worse when compared with the PAH-CHD subgroups.ConclusionRelevant clinical, functional, haemodynamic, and survival differences were observed among subgroups. In particular, patients with CD and SD had the worst survival. These findings should be considered when planning medical or interventional treatment strategies in PAH-CHD patients