Balloon pulmonary angioplasty after pulmonary thromboendarterectomy

.Pulmonary thromboendarterectomy (PTE) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH) as it can remove the chronic, fibrotic, flow-limiting organized thrombi within the pulmonary arterial bed, addressing the primum movens of the disease (1). Despite significant improvement in all haemodynamic parameters, residual pulmonary hypertension (PH) is frequent after PTE, ranging from 17% to 31% (2,3). There is no clear definition of residual PH after PTE, and the actual incidence of this condition has been difficult to quantify. Usually, moderate residual PH is well tolerated by patients and, as shown by data from the United Kingdom cohort, clinically relevant residual PH after PTE mainly occur when the mean pulmonary arterial pressure (mPAP) is greater than 30–35 mmHg (3). The risk of persistent/recurrent PH in the long-term underlines the importance of a systematic patient follow-up, even after PTE. Balloon pulmonary angioplasty (BPA) has been developed as a compassionate procedure for symptomatic patients with CTEPH who are ineligible for surgery or with persistent/recurrent PH after PTE. BPA is not able to remove clots as PTE, but it is able to restore the flow by fragmenting the thrombotic and fibrotic material, resulting in hemodynamic and clinical improvement. Selection of good candidates for BPA, especially after PTE, includes a complete re-assessment of the patient with persistent symptomatic PH after PTE at least four to six months after surgery using high quality imaging techniques such as computed tomography pulmonary angiography (CTPA), selective pulmonary angiography (to provide fine details) and right heart catheterization (RHC) to assess the hemodynamic impairment. However, these imaging techniques are not widely available and require expertise

A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines

open12noTo optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk status. Previous analyses of registries made progress in using risk stratification approaches, however, the focus is often on patients with a low-risk prognosis, whereas most PAH patients are in intermediate-risk or high-risk categories. Using only six parameters with high prognostic relevance, we aimed to demonstrate a pragmatic approach to individual patient risk assessment to discriminate between patients at low risk, intermediate risk and high risk of death.This work was supported by Department of Experimental, Diagnostic and Specialty Medicine-DIMES, University of Bologna, Bologna, Italy and by the National Institute of Biostructures and Biosystems, Rome, Italy.openDardi, Fabio; Manes, Alessandra; Guarino, Daniele; Zuffa, Elisa; De Lorenzis, Alessandro; Magnani, Ilenia; Rotunno, Mariangela; Ballerini, Alberto; Lo Russo, Gerardo Vito; Nardi, Elena; Galiè, Nazzareno; Palazzini, MassimilianoDardi, Fabio; Manes, Alessandra; Guarino, Daniele; Zuffa, Elisa; De Lorenzis, Alessandro; Magnani, Ilenia; Rotunno, Mariangela; Ballerini, Alberto; Lo Russo, Gerardo Vito; Nardi, Elena; Galiè, Nazzareno; Palazzini, Massimilian

Therapeutic alternatives in chronic thromboembolic pulmonary hypertension: from pulmonary endarterectomy to balloon pulmonary angioplasty to medical therapy. State of the art from a multidisciplinary team

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with a very complex pathophysiology differing from other causes of pulmonary hypertension (PH). It is an infrequent consequence of acute pulmonary embolism that is frequently misdiagnosed. Pathogenesis has been related to coagulation abnormalities, infection or inflammation, although these disturbances can be absent in many cases. The hallmarks of CTEPH are thrombotic occlusion of pulmonary vessels, variable degree of ventricular dysfunction and secondary microvascular arteriopathy. The definition of CTEPH also includes an increase in mean pulmonary arterial pressure of more than 25 mmHg with a normal pulmonary capillary wedge of less than 15 mmHg. It is classified as World Health Organization group 4 PH, and is the only type that can be surgically cured by pulmonary endarterectomy (PEA). This operation needs to be carried out by a team with strong expertise, from the diagnostic and decisional pathway to the operation itself. However, because the disease has a very heterogeneous phenotype in terms of anatomy, degree of PH and the lack of a standard patient profile, not all cases of CTEPH can be treated by PEA. As a result, PH-directed medical therapy traditionally used for the other types of PH has been proposed and is utilized in CTEPH patients. Since 2015, we have been witnessing the rebirth of balloon pulmonary angioplasty, a technique first performed in 2001 but has since fallen out fashion due to major complications. The refinement of such techniques has allowed its safe utilization as a salvage therapy in inoperable patients. In the present keynote lecture, we will describe these therapeutic approaches and results

Therapeutic strategy in patients with pulmonary arterial hypertension at intermediate-risk

Le attuali linee guida stratificano il rischio dei pazienti con ipertensione arteriosa polmonare (IAP) in basso, intermedio e alto (rispettivamente con mortalità a 1 anno 10%). La maggior parte dei pazienti è però classificata nella categoria intermedia. Per stratificare ulteriormente questi pazienti, abbiamo valutato il ruolo prognostico dello stroke volume index (SVI) misurato al cateterismo cardiaco destro (CCDx) in 725 pazienti naïve da terapia con IAP idiopatica/ereditaria, associata a malattie del tessuto connettivo o cardiopatie congenite. I pazienti sono stati valutati al basale e 3-4 mesi dopo l'inizio della terapia (1° F-UP) con CCDx, livelli plasmatici di peptide natriuretico cerebrale (BNP), test dei 6 minuti (T6M) e classe funzionale OMS. Abbiamo applicato una tabella di rischio semplificata utilizzando i criteri: classe funzionale OMS, T6M, pressione atriale destra o livelli plasmatici di BNP e indice cardiaco (IC) o saturazione di ossigeno venoso misto (SvO2). Le classi di rischio sono state definite come: basso= almeno 3 criteri a basso rischio e nessun criterio ad alto rischio; alto= almeno 2 criteri ad alto rischio inclusi IC o SvO2; intermedio= tutti gli altri casi. Lo SVI, mediante la regressione di Cox, stratifica la prognosi dei pazienti a rischio intermedio al 1° F-UP [p=0.008] ma non al basale [p=0.085]. Considerandone l’ottimale cut-off predittivo (38 ml/m2) i pazienti a rischio intermedio sono ulteriormente classificabili in intermedio-basso e intermedio-alto. Considerando l'effetto dei 3 principali farmaci che agiscono sulla via della prostaciclina in aggiunta alla duplice terapia di combinazione con inibitori della fosfodiesterasi-5 e antagonisti dell'endotelina, i pazienti trattati con epoprostenolo e.v. hanno ottenuto un maggiore miglioramento rispetto ai pazienti trattati con selexipag; col treprostinil s.c. vi è stata una risposta intermedia. Abbiamo quindi proposto un algoritmo di terapia con selexipag in pazienti a rischio intermedio-basso e con prostanoidi parenterali in pazienti a rischio intermedio-alto.Current guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) in low, intermediate and high-risk categories (respectively 1-year mortality 10%). Most patients anyway are classified in the intermediate risk category. In order to further stratify this group of patients we evaluate the prognostic role of stroke volume index (SVI) measured during right heart catheterization (RHC) in 725 treatment naïve patients with idiopathic/heritable PAH and PAH associated with connective tissue disease and congenital heart disease. All patients were assessed at baseline and 3-4 months after starting PAH-specific therapy (1st F-UP) with RHC, brain natriuretic peptide (BNP) plasma levels, 6-min walking distance (6MWD) and WHO functional class. We applied a simplified risk assessment strategy using the following criteria: WHO functional class, 6MWD, right atrial pressure or BNP plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. SVI, according to Cox regression analysis, can stratify the prognosis of intermediate-risk patients at 1st F-UP [p=0.008] but not at baseline [p=0.085]. According to the best predictive cut-off value (38 ml/m2) intermediate risk patients were further stratified in intermediate-low and intermediate-high. Considering the effect of the 3 main drugs acting on the prostacyclin pathway on top of the dual combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists patients treated with i.v. Epoprostenol obtained a higher improvement than the patients treated with Selexipag while the patients treated with s.c. Treprostinil presented an intermediate response. According to these results we propose a therapeutic approach with Selexipag in intermediate-low risk patients and with parenteral prostanoids in intermediate-high risk patients

Pulmonary vascular disease due to left heart disease: How to achieve a more accurate approach beyond the haemodynamic phenotype: Reply

Pulmonary vascular disease due to left heart disease: how to achieve a more accurate approach beyond the hemodynamic phenotype - Repl

Thrombolysis in high-risk patients with acute pulmonary embolism: underuse of a life-saving treatment in the real-world setting

This editorial refers to ‘Trends in thrombolytic treatment and outcomes of acute pulmonary embolism in Germany’, by K. Keller et al., doi:10.1093/eurheartj/ehz236

Aiming at the appropriate target for the treatment of pulmonary hypertension due to left heart disease

This editorial refers to ‘Sildenafil for improving outcomes in patients with corrected valvular heart disease and persistent pulmonary hypertension’, by J. Bermejo et al. doi:10.1093/eurheartj/ehx700

Cardiologia pediatrica. Problematiche cliniche

L'ipertensione polmonare (Pulmonary hypertension - PH) \ue8 una malattia rara nei neonati, nei lattanti ed in tutta l\u2019et\ue0 pediatrica, associata ad una significativa morbidit\ue0 e mortalit\ue0. Nella maggior parte dei pazienti pediatrici, la PH \ue8 idiopatica o associata a cardiopatie congenite; raramente \ue8 associata ad altre condizioni come malattie del tessuto connettivo o cuore polmonare cronico tromboembolico. Dati di incidenza dei Paesi Bassi hanno rivelato un'incidenza annuale ed una prevalenza puntuale (espresse in casi per milione di bambini) di 0.7 e 4.4 per l\u2019ipertensione arteriosa polmonare idiopatica (Idiopathic pulmonary arterial hypertension - IPAH) e 2.2 e 15.6 per l\u2019ipertensione arteriosa polmonare (Pulmonary arterial hypertension - PAH) associata a cardiopatie congenite (congenital heart disease - CHD). La classificazione di Nizza aggiornata della PH include e classifica con maggiore precisione alcune cardiopatie congenite e, in particolare, enfatizza l'ipertensione polmonare persistente del neonato (persistent pulmonary hypertension of the newborn - PPHN) e le pneumopatie della et\ue0 evolutiva come la displasia broncopolmonare (bronchopulmonary dysplasia - BPD) e l\u2019ernia diaframmatica congenita (congenital diaphragmatic hernia - CDH). La gestione della PH in et\ue0 pediatrica rimane impegnativa poich\ue9 le decisioni terapeutiche continuano a dipendere in gran parte dai risultati degli studi clinici effettuati sugli adulti e dall'esperienza clinica degli specialisti pediatrici

Angioplastica delle arterie polmonari nel cuore polmonare cronico tromboembolico: un caso clinico paradigmatico

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex disease where organized pulmonary thrombi and progressive vascular remodeling of the pulmonary arterial tree act synergistically to increase pulmonary vascular resistance and cause pulmonary hypertension. Balloon pulmonary angioplasty (BPA) has gained a renewed interest for the treatment of patients with CTEPH who are not undergoing surgery with pulmonary endarterectomy (PEA) or with persistent/recurrent pulmonary hypertension after PEA and has shown promising results in several observational studies conducted to date. We describe the case of a 42-year-old man with inoperable CTEPH in NYHA functional class III who normalized functional capacity, hemodynamic profile and main hemodynamic parameters after three BPA sessions