Enrico Gotti

Pulmonary hypertension due to left heart disease: analysis of survival according to the haemodynamic classification of the 2015 ESC/ERS guidelines and insights for future changes

Pulmonary hypertension (PH) is a relevant complication of left heart disease (LHD). The 2015 ESC/ERS PH guidelines report two different haemodynamic subsets of PH due to LHD (PH‐LHD) based on levels of pulmonary vascular resistance (PVR) and diastolic pressure gradient (DPG): isolated post‐capillary PH (Ipc‐PH) and combined post‐ and pre‐capillary PH (Cpc‐PH). The objective of this study is to evaluate the prognostic value of Ipc‐PH and Cpc‐PH.

Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights

Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option. This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting. Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3–4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003). The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting. Bosentan and sildenafil combination therapy improved haemodynamics and exercise in PAH patients failing monotherapy http://ow.ly/LGrPm

Anticoagulant treatment in patients with pulmonary arterial hypertension associated with systemic sclerosis: More shadows than lights

Pulmonary arterial hypertension is a chronic and progressive disease characterized by elevated pulmonary artery pressure and pulmonary vascular resistance leading to heart failure and premature death. Pulmonary arterial hypertension is characterized by proliferative and obstructive lesions in the distal pulmonary arteries and some descriptions include also thrombotic lesions. Despite this, in an era when multiple effective pulmonary arterial hypertension therapies are available, the role of anticoagulation in the treatment of pulmonary arterial hypertension remains uncertain. In particular, anticoagulant treatment in pulmonary arterial hypertension associated with connective tissue disease seems to be associated with unfavorable risk to benefit ratio due to an increased rate of bleeding from the gastrointestinal tract. However, anticoagulation may be required in conditions with increased thrombophilia like in the presence of lupus anticoagulant phenomenon or in the presence of anticardiolipin antibodies.

Reply: Left Main Extrinsic Compression in Pulmonary Arterial Hypertension: From Identification to Percutaneous Coronary Intervention Optimization

We thank Dr. Montero-Cabezas and colleagues for the suggestions aimed to optimize the evaluation of left main coronary artery (LMCA) stenosis severity due to extrinsic compression from dilated pulmonary artery (PA) in patients with pulmonary arterial hypertension (PAH). They propose to use

Comparative survival of operable chronic thromboembolic pulmonary hypertension patients as compared to inoperable medically treated or untreated patients

Background: Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) patients. It is unclear whether the outcome of patients with inoperable CTEPH is influenced by the use of pulmonary arterial hypertension (PAH)-specific drugs. Aim: To compare the survival of operable CTEPH patients who underwent PEA (OP-PEA), inoperable CTEPH patients medically treated with PAH-specific drugs (INOP-MT) and a control group of inoperable untreated CTEPH patients (INOP-C) in a single center. Methods: Between July 1996 and February 2013 we included 261 consecutive patients with CTEPH. All patients underwent right heart catheterization and 6-minute walk test (6MWT). Kaplan-Meier curves were used to estimate the survival of the three groups. Results: The mean follow-up period was 43±37 months. One hundred and nine patients were included in the OP-PEA group, 118 in the INOP-MT group and 34 in the INOP-C group. In the INOP-MT group, 54 patients received phosphodiesterase type-5 inhibitors, 36 endothelin receptor antagonists, 5 prostanoids and 23 combination therapy. Age was 63±14, 64±17 and 57±16 years in INOP-C, INOP-MT and OP-PEA respectively (P = 0.063 and < 0.001 for OP-PEA vs INOP-C and INOP-MT respectively). Baseline 6MWT was 324±125, 354±138 and 387±123 m in INOP-C, INOP-MT and OP-PEA respectively (P = 0.042 and 0.057 for OP-PEA vs INOP-C and INOP-MT respectively). Pulmonary Vascular Resistance was 9.9±5.9, 9.8±4.9 and 9.7±0.4 WU in INOP-C, INOP-MT and OP-PEA respectively (NS). Kaplan-Meier survival of the three groups is reported in the table. View this table: Table 1 Conclusions: OP-PEA patients were younger, with a better baseline exercise capacity and had the best long term survival as compared to the inoperable groups. INOP-MT patients had an intermediate survival between OP-PEA and INOP-C groups.