Cardiac Prevention and Rehabilitation "3.0": From acute to chronic phase. Position Paper of the ltalian Association for Cardiovascular Prevention and Rehabilitation (GICR-IACPR).

Cardiac rehabilitation (CR) is the subspecialty of clinical cardiology dedicated to the treatment of cardiac patients, early and in the long term after an acute event. The aim of CR is to improve both quality of life and prognosis through prognostic stratification, clinical stabilization and optimization of therapy (pharmacological and non), management of comorbidities, treatment of disability, as well as through the provision and reinforcement of secondary prevention interventions and maintenaince of adherence to treatment. The mission of CR has changed over time. Once centered on the acute phase, aimed primarily at short-term survival, the healthcare of cardiac patients now increasingly involves the chronic phase where the challenge is to guarantee continuity and quality of care in the medium and long-term. The aim of the present position paper is to provide the state-of-the-art of CR in Italy, discussing its trengths and weaknesses as well as future perspectives.

ANMCO Position Paper: long-term follow-up of patients with pulmonary thromboembolism.

Venous thromboembolism (VTE), including pulmonary embolism and deep venous thrombosis, is the third most common cause of cardiovascular death. The management of the acute phase of VTE has already been described in several guidelines. However, the management of the follow-up (FU) of these patients has been poorly defined. This consensus document, created by the Italian cardiologists, wants to clarify this issue using the currently available evidence in VTE. Clinical and instrumental data acquired during the acute phase of the disease are the cornerstone for planning the FU. Acquired or congenital thrombophilic disorders could be identified in apparently unprovoked VTE during the FU. In other cases, an occult cancer could be discovered after a VTE. The main targets of the post-acute management are to prevent recurrence of VTE and to identify the patients who can develop a chronic thromboembolic pulmonary hypertension. Knowledge of pathophysiology and therapeutic approaches is fundamental to decide the most appropriate long-term treatment. Moreover, prognostic stratification during the FU should be constantly updated on the basis of the new evidence acquired. Currently, the cornerstone of VTE treatment is represented by both the oral and the parenteral anticoagulation. Novel oral anticoagulants should be an interesting alternative in the long-term treatment.

[Unexpected early obstruction of a biological aortic valve prosthesis]. / Un'inattesa ostruzione precoce di protesi biologica aortica.

A 64 years old male was submitted to the surgical substitution of a deteriorated biological aortic valve prosthesis with a new Hancock II biological prosthesis. The implantation was not followed by an anticoagulation or antiaggregation therapy. Two months later he was checked at our Institution because he complained symptoms and developed echocardiographic indexes suggestive of an aortic prosthesis obstruction by a clot. Both symptoms and the echocardiographic indexes of prosthesis obstruction faded away after giving warfarin; they arose again when the anticoagulation therapy was stopped and was replaced by aspirin. The following permanent use of warfarin normalized both clinic and echocardiographic aspects. The present case report underlines the utility of early controls after a biological prosthesis, yet aortic, implantation, when it is not followed by an anticoagulant therapy, also in subjects free from thrombosis high risk factors.

Biofeedback assisted control of respiratory sinus arrhythmia as a biobehavioral intervention for depressive symptoms in patients after cardiac surgery: a preliminary study.

The current study investigated whether biofeedback training aimed at increasing respiratory sinus arrhythmia (RSA), a measure of cardiac vagal modulation, can reduce depressive symptoms in patients after cardiac surgery. This randomized controlled study enrolled 26 patients after first-time cardiac surgery. The patients were randomly assigned to an RSA-biofeedback group (N = 13) or to a treatment as usual group (N = 13). The biofeedback training consisted of five 45 min sessions designed to increase RSA. The outcome was assessed as changes in RSA and in the Centre for Epidemiologic Studies of Depression (CES-D) values from pre- to post-training. Both groups were comparable for demographic and biomedical characteristics. RSA increased significantly in patients who underwent RSA-biofeedback compared to controls. Moreover, the CES-D scores were reduced significantly from pre- to post-training in the RSA-biofeedback group compared to the controls. Changes in RSA were inversely related to changes in CES-D scores from pre- to post-training. These findings extend the effectiveness of RSA-biofeedback for increasing vagal modulation as well as for reducing depressive symptoms in post-surgical patients. Overall, the current study also suggests that this biobehavioral intervention may add to the efficacy of postoperative risk reduction programs and rehabilitation protocols in cardiac surgery patients.

[Atherosclerosis of the lower extremities as a linked comorbidity in Patients Admitted for cardiac rehabilitation (THINKPAD): rationale, design, and study group]. / La survey THINKPAD (ATHerosclerosis of the lower extremities as a liNKed comorbidity in Patients Admitted for carDiac rehabilitation) di GICR-IACPR: razionale, disegno e gruppo di studio.

Peripheral arterial disease (PAD) is a frequent comorbidity among patients entering cardiac rehabilitation (CR) programmes and an important source of disability and impaired prognosis. The prevalence of PAD across the wide range of conditions for CR is poorly understood, as far as its impact on drug optimization and intervention delivered. The "ATHerosclerosis of the lower extremities as a liNKed comorbidity in Patients Admitted for carDiac rehabilitation" (THINKPAD) study was carried out by the Italian Association for Cardiovascular Prevention, Rehabilitation and Epidemiology (GICR-IACPR) in order to explore PAD both as a comorbidity and a primary indication at the entry of CR. The study was a retrospective case series. In the study period (from May 1, 2012 to June 30, 2012), data on consecutive patients discharged from 17 CR units in Northern Italy were collected. Web-based electronic case report forms (e-CRF), accessible in a dedicated section of the IACPR website (www.iacpr.it), were used for data entry, and data were transferred via web to a central database. The data collection instrument was designed with a multiple choice format, with jump menus or select boxes and obligatory items. A sample size of 1,300 subjects is expected, with first data available by the end of 2012.

[Standards and outcome measures in cardiovascular rehabilitation. Position paper GICR/IACPR]. / Requisiti e indicatori in cardiologia riabilitativa: documento di consenso del GICR/IACPR.

Despite major improvements in diagnostics and interventional therapies, cardiovascular diseases remain a major health care and socio-economic problem in Italy. Costs and resources required are increasing in close correlation to both the improved quality of care and to the population ageing. There is an overwhelming evidence of the efficacy of cardiac rehabilitation (CR) in terms of reduction in morbidity and mortality after acute cardiac events. CR services are by definition multi-factorial and comprehensive. Furthermore, systematic analysis and monitoring of the process of delivery and outcomes is of paramount importance. The aim of this position paper promoted by the Italian Association for Cardiovascular Prevention and Rehabilitation (GICR-IACPR) is to provide specific recommendations to assist CR staff in the design, evaluation and development of their care delivery organization. The position paper should also assist health care providers, insurers, policy makers and consumers in the recognition of the quality of care requirements, standards and outcome measure, quality and performance indicators, and professional competence involved in such organization and programs. The position paper i) include comprehensive CR definition and indications, ii) describes priority criteria based on the clinical risk for admission to both inpatient or outpatient CR, and iii) defines components and technological, structural and organizing requirements for inpatient or outpatient CR services, with specific indicators and standards, performance measures and required professional skills. A specific chapter is dedicated to the requirements for highly specialized CR services for patients with more advanced cardiovascular diseases.

[Treatment adherence in cardiovascular prevention]. / L'aderenza globale al trattamento nel continuum della prevenzione cardiovascolare.

Treatment adherence is a key element for (i) improving prognosis in cardiovascular and/or high-risk patients, (ii) reducing the burden of morbidity and mortality associated with cardiovascular disease at a population level, and (iii) decreasing costs due to rehospitalizations.Promotion of adherence should embrace all pharmacological and non-pharmacological interventions in cardiovascular prevention, including lifestyle and behavioral changes. In this perspective, cardiac prevention and rehabilitation programs are the most appropriate and cost-effective setting for delivering structured and multi-component interventions on patient's adherence. In this expert opinion document authored by the Italian Association for Cardiovascular Prevention and Rehabilitation, a modern reappraisal of the adherence issue is provided, together with simple, practical, and feasible suggestions to achieve this goal in the real life as well.

Prevalence and management of familial hypercholesterolemia in patients with coronary artery disease: The heredity survey.

BACKGROUND AND AIMS: Familial hypercholesterolemia (FH) is a genetic disorder characterized by high levels of low density lipoprotein cholesterol (LDL-C) predisposing to premature cardiovascular disease. Its prevalence varies and has been estimated around 1 in 200-500. The Heredity survey evaluated the prevalence of potential FH and the therapeutic approaches among patients with established coronary artery disease (CAD) or peripheral artery disease (PAD) in which it is less well documented. METHODS: Data were collected in patients admitted to programs of rehabilitation and secondary prevention in Italy. Potential FH was estimated using Dutch Lipid Clinic Network (DLCN) criteria. Potential FH was defined as having a total score≥6. RESULTS: Among the 1438 consecutive patients evaluated, the prevalence of potential FH was 3.7%. The prevalence was inversely related to age, with a putative prevalence of 1:10 in those with <55yrs of age (male) and <60yrs (female). Definite FH (DLCN score>8) had the highest percentages of patients after an ACS (75% vs 52.5% in the whole study population). At discharge, most patients were on high intensity statin therapy, but despite this, potential FH group still had a higher percentage of patients with LDL-C levels not at target and having a distance from the target higher than 50%. CONCLUSIONS: Among patients with established coronary heart disease, the prevalence of potential FH is higher than in the general population; the results suggest that a correct identification of potential FH, especially in younger patients, may help to better manage their high cardiovascular risk.

[Cardiac rehabilitation "3.0": from the acute to the chronic stage. A position paper from the Italian Association for Cardiovascular Prevention and Rehabilitation (GICR-IACPR)]. / La Cardiologia Preventiva e Riabilitativa "3.0": dalle acuzie alla cronicità. Position paper del Gruppo Italiano di Cardiologia Riabilitativa e Preventiva (GICR-IACPR).

Cardiac rehabilitation (CR) represents a cardiology subspecialty that is devoted to the care of cardiac patients, early and long term after an acute event. CR aims at improving both quality of life and prognosis through risk and prognostic stratification, clinical stabilization and optimization of therapy, management of comorbidities, treatment of disability, and the provision of sustained long-term preventive and rehabilitative services.The mission of CR has changed over time. From being centred on the acute phase, health care of cardiac patients is increasingly involving the long-term chronic phase. The aim of the present position paper is to provide the state of the art of CR in Italy, discussing strengths and weaknesses as well as future perspectives.

[Heart rate as a therapeutic target after acute coronary syndrome and in chronic coronary heart disease]. / La frequenza cardiaca come target terapeutico dopo sindrome coronarica acuta e nella cardiopatia ischemica cronica.

For patients with stable coronary artery disease (SCAD), either after hospitalization for acute cardiac events or in the chronic phase, comprehensive treatment programs should be devoted to: (i) reducing mortality and major adverse cardiovascular events, (ii) reducing the ischemic burden and related symptoms, and (iii) increasing exercise capacity and quality of life.Heart rate (HR) has demonstrated to have prognostic value and patients beyond the limit of 70 bpm display increased risk of all the above adverse outcomes, even after adjustment for parameters such as the extension of myocardial infarction and the presence of heart failure. It is well known that a sustained HR elevation may contribute to the pathogenesis of SCAD, being the likelihood of developing ischemia, plaque instability, trigger for arrhythmias, increased vascular oxidative stress, and endothelial dysfunction the mechanisms resulting in this effect. Moreover, high HR could promote chronotropic incompetence, leading to functional disability and reduced quality of life.Despite the strong relationship between HR and prognosis, there is heterogeneity among current guidelines in considering HR as a formal therapeutic target for secondary prevention in SCAD, as far as the cut-off limit. This expert opinion document considered major trials and observational registries in the modern treatment era with beta-blockers and ivabradine, suggesting that an adequate HR control could represent a target for (i), (ii), and (iii) therapeutic goals in SCAD patients with systolic dysfunction (with major evidence for reduced left ventricular ejection fraction <40%), and a target for (ii) and (iii) goals in SCAD patients with preserved left ventricular ejection fraction. The defined cut-off limit is 70 bpm. To date, there is room for improvement of HR control, since in contemporary SCAD patients HR values <70 bpm are present in less than half of cases, even in the vulnerable phase after an acute coronary syndrome.

[A rare cardiomegaly]. / Una rara cardiomegalia.

We present the case of a patient who came to our attention for enlargement of the cardiac silhouette on chest X-ray. Echocardiography showed moderate diastolic overload of both ventricles with enhanced cardiac output without valvular disease or cardiac shunt that could account for this cardiomegaly. A subsequent abdominal echocardiographic exploration showed an angiomatous transformation of the liver due to diffuse lacunar enlargement of hepatic portal vein branches and arterial-venous shunts. Computed tomography and magnetic resonance imaging confirmed the echocardiographic findings. The imaging findings coupled with cutaneous and nasopharyngeal lesions were suggestive of hereditary hemorrhagic telangiectasia (HHT) and the diagnosis was confirmed after the identification of a mutation in the ACVRL1 gene on chromosome 12. HHT is a rare but underestimated vascular disease that can affect different organs, in particular the liver, leading to organ failure requiring transplantation as occurred in our patient. Echocardiography is a useful imaging tool to exclude cardiac abnormalities as a cause of cardiomegaly and to guide the correct diagnosis of a peripheral origin of high cardiac output.

[The Italian Survey on Cardiac Rehabilitation - 2013 (ISYDE.13-Directory): national availability and organization of cardiac rehabilitation facilities]. / Italian Survey on Cardiac Rehabilitation (ISYDE.13-Directory): report su strutture, organizzazione e programmi di cardiologia riabilitativa in Italia.

BACKGROUND: The Italian Association for Cardiovascular Prevention, Rehabilitation and Epidemiology (GICR-IACPR) and the Italian Society of Cardiologists of Accredited Hospitals (SICOA) developed the ISYDE.13 survey with the purpose to take a detailed snapshot of number, distribution, facilities, staffing levels, organization, and program details of cardiac rehabilitation (CR) units in Italy. METHODS: The study was carried out using a web-based questionnaire running on the GICR-IACPR website for 4 weeks from September 2 to 29, 2013. RESULTS: Out of 221 CR centers existing in Italy (+14% vs 2008), 191 (86%) participated in the survey. On a national basis, there is a CR unit every 268 852 inhabitants. The majority of CR units are located in public hospitals (57.1%), the remaining 42.9% in private hospitals; 130 CR centers (68%) provide inpatients care and account for 3527 beds (5.9 per 100 000 inhabitants): of these 374 are day-hospital beds and 408 are sub-intensive beds. Forty-one of the Italian in-hospital CR centers offer also outpatient programs and 61 centers (32%) offer only outpatient CR programs; 131 of the CR units (68.6%) are linked to dedicated cardiology divisions and in 77% of cases the head is a cardiologist. Home-based programs are offered by 9 centers (4.7%) and CR programs with telecare supervision by 16 (8.4%). Long-term secondary prevention follow-up programs are provided by 94 of CR services (49.2%). During one week of activity, the 191 centers completed 1335 inpatient CR programs and 971 outpatient CR programs. According to these data, it may be assumed that in Italy approximately 100 000 patients are referred annually to CR programs. CONCLUSIONS: ISYDE.13 showed an incremental trend of CR provision in Italy, particularly in outpatient programs. However, at present, the national network of CR units covers only one third of the potential requirements defined by current secondary prevention recommendations.

[ANMCO Position paper: Recommendations for the follow-up of patients with pulmonary thromboembolism]. / Position paper ANMCO: Raccomandazioni per il follow-up del paziente con tromboembolia polmonare.

Venous thromboembolism (VTE), including deep venous thrombosis and pulmonary embolism, is the third most common cause of cardiovascular death. The management of the acute phase of VTE is well described in several papers and guidelines, whereas the management of the follow-up of the patients affected from VTE is less defined. This position paper of the Italian Association of Hospital Cardiologists (ANMCO) tries to fill the gap using currently available evidence and the opinion of the experts to suggest the most useful way to manage patients in the chronic phase.The clinical and laboratory tests acquired during the acute phase of the disease drives the decision of the following period. Acquired or congenital thrombophilic factors may be identified to explain an apparently not provoked VTE. In some patients, a not yet clinically evident cancer could be the trigger of VTE and this could lead to a different strategy. The main target of the post-acute management is to prevent relapse of the disease and to identify those patients who could worsen or develop chronic thromboembolic pulmonary hypertension. The knowledge of the etiopathogenetic ground is important to address the therapeutic approach, choosing the best antithrombotic strategy and deciding how long therapy should last. During the follow-up period, prognostic stratification should be updated on the basis of new evidences eventually acquired.Treatment of VTE is mainly based on oral or parenteral anticoagulation. Oral direct inhibitors of coagulation represent an interesting new therapy for the acute and extended period of treatment.

Pulmonary embolism: diagnostic algorithms.

In 90% of cases the clinical suspicion of pulmonary embolism (PE) is raised by clinical signs and symptoms, while in only 10% of cases PE is suspected on the basis of electrocardiographic, arterial blood gas analysis or radiological findings. The combination of clinical signs and symptoms and the results of first-level diagnostic tests (electrocardiography, gas analysis and chest X-ray) allows a fairly accurate classification of patients with "clinical suspicion of PE" into three categories of clinical (or pre-test) probability: low, intermediate and high. The clinical diagnosis of PE is very often inaccurate making the use of additional tests, including imaging techniques, mandatory. The choice and the combination (= diagnostic algorithms) of second- and third-level diagnostic tests (D-dimer, venous ultrasound, echocardiography, lung scintigraphy, helical computed tomography and pulmonary angiography) depend primarily on the clinical conditions of patients and their pre-test probability. We propose two diagnostic algorithms: 1) a diagnostic algorithm for patients with clinically suspected PE and critical clinical conditions (unstable patients), 2) a diagnostic algorithm for patients with clinically suspected PE and non-critical clinical conditions (hemodynamically stable patients).

Depression is associated with increased vagal withdrawal during unpleasant emotional imagery after cardiac surgery.

BACKGROUND: The aim of this study was to examine the influence of depression on heart rate and heart rate variability (HRV) during emotional imagery in patients after cardiac surgery. METHODS: Based on the scores of the Center for Epidemiological Studies of Depression (CES-D) scale, 28 patients after cardiac surgery were assigned either to the group with depression (CES-D scores ≥ 16; N = 14) or the one without depression (CES-D scores<16; N = 14). Each patient completed a rest period and an emotional imagery including pleasant, neutral and unpleasant scripts. Inter-beat intervals (IBIs) and HRV were measured during the entire protocol. RESULTS: Compared to nondepressed patients, those with depression had greater reductions in high frequency expressed in normalized units (HF n.u.) during the imaging of the unpleasant script (p = .003, Cohen's d = 1.34). Moreover, HF n.u. were lower during the imaging of the unpleasant script than the pleasant one in depressed patients only (p = .020, Cohen's d = 0.55). CES-D scores were also inversely correlated with residualized changes in IBIs (r = -.38, p = .045) and HF n.u. (r = -.49, p = .008) from rest to the imaging of the unpleasant script. CONCLUSIONS: The relationship between depression and increased vagal withdrawal during unpleasant emotional imagery extends to patients after cardiac surgery. The present study suggests that increased vagal withdrawal to negative emotions in patients after cardiac surgery may mediate the conferral of cardiac risk by depression.

[Pulmonary scintigraphy in the diagnosis of pulmonary embolism]. / A proposito di scintigrafia polmonare nella diagnosi di embolia polmonare.

Pulmonary scintigraphy constitutes an important step in the non invasive diagnosis of pulmonary embolism (PE). This technique may be employed for the evaluation of the pulmonary perfusion alone, as in Italy and in the PISA-PED study, or else even for the evaluation of the pulmonary ventilation (as in Anglo-Saxon countries and in the PIOPED study). In the present study, the reasons which have prompted the ANMCO-SIC Commission for the Guidelines for The Prophylaxis, Diagnosis and Therapy of Pulmonary Thromboembolism to propose, for the diagnostic work-up of the patient with clinically suspected PE, the use of perfusion scintigraphy alone and of the classification criteria employed in the PISA-PED study instead of the more commonly utilized ventilatory-perfusion scintigraphy and of the criteria included in the PIOPED article, are discussed. Besides, the Commission's decision to consider PE as being present in case of agreement between the scintigraphic and clinical pictures, and to exclude this condition when the scintigraphic outcome is normal/almost normal regardless of the clinical probabilities, is also motivated.

[Chronic thromboembolic pulmonary hypertension]. / Ipertensione polmonare cronica tromboembolica.

Chronic thromboembolic pulmonary hypertension is due to unresolved or recurrent pulmonary embolism. In the United States the estimated prevalence is 0.1-0.5% among survived patients with pulmonary embolism. The survival rate at 5 years was 30% among patients with a mean pulmonary artery pressure > 40 mmHg at the time of diagnosis and only 10% among those with a value > 50 mmHg. The interval between the onset of disturbances and the diagnosis may be as long as 3 years. Doppler echocardiography permits to establish the diagnosis of pulmonary hypertension. Radionuclide scanning determines whether pulmonary hypertension has a thromboembolic basis. Right heart catheterization and pulmonary angiography are performed in order to establish the extension and the accessibility to surgery of thrombi and to rule out other causes. The surgical treatment is thromboendarterectomy. A dramatic reduction in the pulmonary vascular resistance can be achieved; corresponding improvements in the NYHA class--from class III or IV before surgery to class I-II after surgery--are usually observed. Patients who are not considered candidates for thromboendarterectomy may be considered candidates for lung transplantation.

Depression and reduced heart rate variability after cardiac surgery: the mediating role of emotion regulation.

BACKGROUND: Heart rate variability (HRV), as an index of autonomic nervous system (ANS) functioning, is reduced by depression after cardiac surgery, but the underlying mechanisms of this relationship are poorly understood. Poor emotion regulation as a core symptom of depression has also been associated with altered ANS functioning. The present study aimed to examine whether emotion dysregulation could be a mediator of the depression-reduced HRV relationship observed after cardiac surgery. METHODS: Self-reported emotion regulation and four-minute HRV were measured in 25 depressed and 43 nondepressed patients after cardiac surgery. Mediation analysis was conducted to evaluate emotion regulation as a mediator of the depression-reduced HRV relationship. RESULTS: Compared to nondepressed patients, those with depression showed lower standard deviation of normal-to-normal (NN) intervals (p<.05), root mean square successive difference of NN intervals (p<.004), and number of interval differences of successive NN intervals greater than 50ms (NN50) (p<.05). Increased low frequency (LF) in normalized units (n.u.) and reduced high frequency (HF) n.u. were also found in depressed compared to nondepressed patients (p's<.01). Mediation analysis revealed that suppression of emotion-expressive behavior partially mediated the effect of depression on LF n.u. and HF n.u. CONCLUSIONS: Results confirmed previous findings showing that depression is associated with reduced HRV, especially a reduced vagal tone and a sympathovagal imbalance, after cardiac surgery. This study also provides preliminary evidence that increased trait levels of suppression of emotion-expressive behavior may mediate the depression-related sympathovagal imbalance after cardiac surgery.

Role of Cardiovascular Ultrasound in the Evaluation of Obese Subjects.

Obesity represents a worldwide increasing health problem. Obesity, through complex and not fully understood pathogenetic mechanisms, induces different structural and functional changes of left heart chambers, right heart chambers, and arteries. Ultrasound techniques are the first choice for a comprehensive assessment of the cardiovascular adaptation to obesity. This review summarizes the up-to-date literature on the topic, with particular focus on the main clinical studies, which range over different cardiovascular adaptations to obesity, namely left ventricular mass, diastolic function, right ventricle structure and function, arterial stiffness, and intima-media thickness. Also, the importance of epicardial fat and of the degree of obesity is described. Finally, the role of weight loss and bariatric surgery and the study of cardiovascular obesity-induced abnormalities in children and adolescent are discussed.

[Diagnosis and therapy of pulmonary arterial hypertension in Italy: results of the INCIPIT2 survey]. / Diagnosi e terapia dell'ipertensione arteriosa polmonare nelle Cardiologie italiane: risultati dell'indagine conoscitiva INCIPIT2.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a rare clinical condition characterized by increased pulmonary vascular resistance and premature death. It is necessary to activate a pathway from the screening of pulmonary hypertension to the diagnosis of PAH, so as to use the drugs able to improve the outcome. Nowadays, in Italy, there are no data about the management of PAH in peripheral centers and about the integration of peripheral centers with those of excellence. METHODS: In order to have a map of the actual Italian pathway for diagnosis and therapy of PAH, on behalf of the ANMCO Pulmonary Circulation Area, 923 Italian cardiology departments were asked to reply, on a special electronic file, to a few simple questions about their organization, from December 2012 to May 2013. RESULTS: 101/923 centers (48 in the North, 18 in the Middle, 35 in the South) answered correctly. 32% has no organization for PAH, 68% has a pathway for PAH diagnosis and management, and two thirds of them collaborate with excellence centers. 36 centers perform right heart catheterization with vascular reactivity (21 with nitric oxide, 8 with adenosine, 5 with epoprostenol, 2 with nitric oxide or epoprostenol). 61/101 are prescriber centers: 33 perform right heart catheterization with vascular reactivity test, 23 send their patients to the reference center for right heart catheterization, 5 perform no right heart catheterization before the prescription of specific drugs for PAH, and only 14 prescribe intravenous prostanoids. In 2011, the participating centers followed 561 patients with PAH, of whom 126 (23%) were in independent centers. With regard to the network organization of the groups, the participating centers are partly independent of the diagnostic pathway, partly refer to outside regions; in others there is a structured regional network and there are 3 Italian regions with Hub & Spoke networks that receive patients coming from other regions. CONCLUSIONS: Our results show the interest of Italian Cardiology to find a pathway for the diagnosis of PAH and a heterogeneity suggesting the need for a shareable pathway, thus improving the collaboration between peripheral cardiology departments and the excellence centers for PAH in a functional Hub & Spoke network.