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Background Lung transplantation (LT) still remains a treatment option for patients with pulmonary arterial hypertension (PAH) and not operable chronic thromboembolic pulmonary hypertension patients (CTEPH). Purpose The study is intended to compare the survival of transplant recipients (TR) and the survival of not-transplanted (NT) patients since listing. Methods We included all patients with PAH and not-operable CTEPH listed for LT. The survival of NT, TR and of all listed patients was evaluated starting from the date of listing (patients were censored as alive at the time of LT). The survival of TR was also evaluated starting from the date of the LT. Results 125 patients were included (112, 90% had PAH). Fifty-eight (46%) patients were transplanted, after a mean waiting time of 1.5±1.3 years. Forty-one patients (33%) died while on the list and 25 (20%) patients were alive on the list on December 2019. The survival of NT patients at 1, 3 and 5 years after listing was 74%, 42%, 33%, respectively. The survival of TR patients at 1, 3 and 5 years after listing was 90%, 70%, 63%, respectively. The survival of all patients since listing (intention to treat analysis) at 1, 3 and 5 years was 85%, 59%, 48% respectively. The survival of TR at 1, 3 and 5 years since transplantation was 63%, 61%, 59%, respectively. Conclusions Despite biases in the comparison of non-randomized groups, the data confirm a better long-term survival since listing of TR as compared with NT PAH or not-operable CTEPH patients. Funding Acknowledgement Type of funding sources: None.

SARS-CoV-2 infection determines a disease that predominantly affects lungs. However the cytokines storms, determined by the huge immune response to the infection, could affect also other organs and apparatus such as heart and vessels. Beyond the acute inflammation itself also hypercoagulative status has been linked to SARSCoV-2 infection and this surely relates to the increase seen in prevalence of pulmonary embolism and myocardial infarction. A number of cardiac abnormalities and pathologies have been observed, with special attention to cardiac arrhythmias and myocardial involvement. Furthermore, indirect damages determined by the reduction in acute and chronic cardiovascular care, results in a strong mortality and morbidity outcomes in cardiological patients. In this review we will summarise current knowledge on both direct and indirect cardiovascular damages determined by the SARS-CoV-2 pandemia.

Introduction: Atherosclerosis causes a chronic reduction of vascularization with consequent impairment of the performance of organs, like the brain or muscles, which determines the functional and cognitive decline of the elderly and their ability to respond to acute stressful condition. Therefore, our aim was to evaluate if ankle brachial index (ABI) could effectively be a determinant of in-hospital functional status and complications in elderly hospitalized patients. Methods: This is a monocentric cross-sectional study of 189 patients aged 65 years or older. The study was undertaken at the Internal Medicine ward of Niguarda Hospital in Milan. ABI (BOSO ABY-System 100) and in-hospital status (activities of daily living, ADL and instrumental activities of daily living, IADL) were collected on the second day of hospitalization. Complications (falls and delirium episodes) were also recorded during the whole hospitalization period. Results: The average age of patients was 79.3 ± 6.9 years. Among outcomes, only ADL (r = 0.192, p = 0.007) and IADL score (r = 0.200, p = 0.005) showed significant correlation with ABI. Moreover, during the subsequent logistic regression, ABI remained among the statistically significant determinants of both scores (β = 0.231, p = 0.013 and β = 0.314, p = 0.001, respectively). Conclusions: The main result of our study is the finding of ABI as a significant determinant of acute in-hospital functional impairment (evaluated as ADL and IADL scores). The continuous exposure of the brain and muscles to the reduced perfusions induced by vascular atherosclerosis, probably determined the reduced ability to respond to stressful conditions.

Purpose: The association between serum uric acid (SUA) and pulse wave velocity (PWV), has been extensively evaluated but with some discrepancies in results. A further limitation refers to the fact that only few data were analyzed taking into account the possible effects of gender. The purpose of this study was to estimate the association between SUA and arterial stiffness in general population and hypertensive patients, as a whole population and as divided by gender, by pooling results from existing studies. Materials and methods: Carotid-femoral and brachial-ankle PWV (cf- and ba-PWV) have been analyzed separately and subgroup analyses by gender are reported. Among 692 potentially relevant works, 24 articles were analyzed. Results: Seven studies referred to cf-PWV in the general population with an overall positive association at adjusted analysis for both males and females (beta regression coefficient (β): 0.07; 95%CI: 0.03; 0.11 and β: 0.06; 95%CI: 0.03; 0.09, respectively). Twelve studies referred to ba-PWV in the general population with the finding of a positive association at adjusted analysis for females (β: 0.04; 95% confidence interval (CI): 0.01;0.07), but not for males (β: 0.13; 95%CI: −0.09; 0.34). In hypertensive patients only four studies evaluated cf-PWV and one ba-PWV with only one study (with cf-PWV) finding positive association. Conclusion: The association between SUA and cf-PWV resulted significant in general population in both males and females while it was only significant for female regarding ba-PWV. Furthermore, the few available studies found no significant relationship between SUA and both cf- and ba-PWV in hypertensive subjects.

Introduction: Cardiac rehabilitation (CR) improves the functional capacity and the prognosis of patients with coronary artery disease. Aim: Our study was aimed at assessing the relationship between functional improvement (evaluated with 6-min Walk Test—6MWT) and the improvement in left ventricular ejection fraction (LVEF) after CR. Methods: We collected data from 249 patients (age 66.79 ± 11.06 years; males 81.52%) with a recent history of Acute Coronary Syndrome that performed CR. The functional improvement after CR was expressed as the Δ between distance covered at the final versus the initial 6-min Walking Test (6-MWT), while LVEF was calculated with transthoracic echocardiogram at the beginning and at the end of the CR. Results: Patients were divided accordingly to their pre-rehab LVEF (≥ 55% vs < 55%). With superimposable age and baseline 6MWT distance covered (434.58 vs 405.12 m, p = 0.08), the latter group presented higher Δ meter values at 6MWT (167.93 vs 193.97 m, p = 0.018). However, no statistically significant positive correlation between Δ meters and Δ LVEF was found. Moreover, linear regression analyses found that nor baseline LVEF nor Δ LVEF were significant determinants of Δ meters when considering the whole group, with age, basal 6MWT and peak CK-MB as additional covariates in the model. Conclusion: Although it could be expected that an increase in LVEF is related to the functional improvement after CR, no significant correlation was found in our population.

Background and purpose: Radiation Induced Heart Disease (RIHD) represents a late effect of chest irradiation, contributing in increasing mortality rate in oncological patients by affecting pericardium, myocardium, valvs and coronaries. Currently, regarding the risk of Coronary Artery Disease (CAD), a cardiological screening involving exercise stress electrocardiography after 5-10 years from radiotherapy is advised. We sought to determine the rate of ischemia at exercise stress electrocardiography in a population of patients without cardiovascular risk factors who sustained radiotherapy, using a cohort of patients presenting with at least one cardiovascular risk factor as control group. Design and methods: A population of 115 patients who sustained chest irradiation (and associated chemotherapy), presenting without classic cardiovascular risk factors or typical symptoms suggesting CAD, was evaluated with exercise stress electrocardiography. 135 patients with at least one risk factor for cardiovascular disease candidate to stress testing for primary prevention or for atypical symptoms served as control group. Results: The cohort of irradiated patients without classical cardiovascular risk factors is younger (48.7±10.1 vs 60.5±10.8 years, p

Introduction: Radiation induced heart disease (RIHD) represents a late effect of chest irradiation, contributing in augmenting mortality in oncological patients by affecting pericardium, myocardium, valves and coronaries. Currently, regarding the risk of coronary heart disease (CAD), a cardiological screening involving exercise stress electrocardiography after 5–10 years from radiotherapy is advised. Aim: We sought to determine the rate of ischemia at exercise stress electrocardiography in a population of patient without cardiovascular risk factors who sustained radiotherapy, using a cohort of high cardiovascular risk patients as control group. Methods: A population of 115 patients who sustained chest irradiation, presenting without classic cardiovascular risk factors was evaluated with exercise stress electrocardiography. 135 patients with high profile of cardiovascular risk candidate to stress testing for primary prevention or for atypical symptoms served as control group. Results: The cohort of irradiated patients without classical cardiovascular risk factors was younger (48.7 ± 10.1 vs 60.5 ± 10.8 years, p

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 (pThe 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.

Purpose: This study sought to evaluate, by means of a retrospective analysis, the relationship between pulmonary hypertension (PH) and fibrotic interstitial lung disease (ILD) in a population of 43 patients affected by systemic sclerosis. In addition, we assessed the role of highresolution computed tomography (HRCT) in the diagnostic and therapeutic pathway for such patients. Materials and methods: Forty-three patients affected by progressive systemic sclerosis (PSS) and PH underwent functional, haemodynamic and HRCT evaluations between November 2001 and May 2011. Functional evaluation was performed through respiratory function testing and spirometry. Haemodynamic assessment was done with transthoracic echocardiogram (ECG) and right heart catheterisation. Semiquantitative evaluation of radiological involvement was performed using different measures: visual score, Goh's score, Wells' score and Warrick's score. Results: Of 43 patients, 16 showed fibrotic ILD after a HRCT evaluation. The main pattern of ILD was identified with nonspecific interstitial pneumonia (NSIP). Our study also evaluated the relationship among radiological, functional and haemodynamic indexes; different correlations were found to be significant, in particular, Wells' and visual scores ≥40% correlated significantly with diffusion capacity for carbon monoxide (DLCO); Wells' score also correlated significantly with percentage of predicted forced vital capacity (FVC), percentage of predicted forced expiratory volume in 1 s (FEV1) and composite physiologic index (CPI). If presence of oesophagopathy evaluated with HRCT was included, this sign correlated significantly both with DLCO (p=0.03) and mean pulmonary arterial pressure (mPAP) (p=0.03), considering total population. Conclusions: Our findings confirm the diagnostic role of HRCT in evaluating the extent of lung damage in systemic sclerosis. Furthermore, a significant correlation between mPAP and esophagopathy suggests a possible positive predictive value of this sign in identifying a subphenotypic category of patients affected by systemic sclerosis in whom we can find a more severe visceral impairment and a more frequent vascular involvement

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 ± 17 years; 61% female), 90 had ES (aged 41 ± 16 years); 48 SP (aged 47 ± 18 years); 10 SD (aged 25 ± 21 years); and 44 CD (aged 36 ± 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.

Objectives To correlate the severity of lung neovascularity (Sheehan vessels) with the cause and haemodynamic severity of pulmonary arterial hypertension (PAH), pulmonary artery (PA) size and heart disease type in patients with PH associated with congenital heart diseases (PAH-CHD) and idiopathic PH (IPAH). Methods We reviewed the HRCT and CT pulmonary angiography studies of 87 patients with PAH-CHD and 111 with IPAH; all had undergone right heart catheterisation. We evaluated the PA size and severity of neovascularity on CT. Results Neovascularity, which was found in 72% of PAHCHD (56% with Eisenmenger's syndrome) and in 22%of IPAH patients, is significantly related to the severity of PH and all patients with severe neovascularity had intermediate or high PH. All PAH-CHD patients had a dilated PA with a greater risk of developing severe dilatation (diameter >5 cm). The neovascularity correlated with the PA size only in IPAH. Conclusions Neovascularity even if not pathognomonic for PAH-CHD, is significantly more common in these patients, especially in Eisenmenger's syndrome. It is often the first CT sign to indicate the severity of PH in PAH-CHD and IPAH. A neovascularity ≥5 on CT indicates a intermediate or high PH. Key Points • Large retrospective studying specific lung disorders in patients with pulmonary arterial hypertension. • Neovascularity is often the first CT sign indicating the severity of pulmonary hypertension Alterations of lung parenchyma on CT in pulmonary hypertension are described • The first study to assess the severity of pulmonary hypertension by CT • If substantiated, CT might eventually replace some cardiac catheterisation for evaluating PH

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 surgery.

A large proportion of patients with congenital heart disease (CHD), in particular those with relevant systemic-to-pulmonary shunts, will develop pulmonary arterial hypertension (PAH) if left untreated. Persistent exposure of the pulmonary vasculature to increased blood flow, as well as increased pressure, may result in pulmonary obstructive arteriopathy, which leads to increased pulmonary vascular resistance that, if it approaches or exceeds systemic resistance, will result in shunt reversal. Eisenmenger's syndrome, the most advanced form of PAH associated with CHD, is defined as CHD with an initial large systemic-to-pulmonary shunt that induces severe pulmonary vascular disease and PAH, with resultant reversal of the shunt and central cyanosis. The histopathological and pathobiological changes seen in patients with PAH associated with congenital systemic-to-pulmonary shunts, such as endothelial dysfunction of the pulmonary vasculature, are considered similar to those observed in idiopathic or other associated forms of PAH. A pathological and pathophysiological classification of CHD with systemic-to-pulmonary shunt leading to PAH has been developed that includes specific characteristics, such as the type, dimensions and direction of the shunt, extracardiac abnormalities and repair status. A clinically oriented classification has also been proposed. The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 1.6 and 12.5 cases per million adults, with 25-50% of this population affected by Eisenmenger's syndrome. Clinically, Eisenmenger's syndrome presents with multiple organ involvement, with progressive deterioration of function over time. The signs and symptoms of Eisenmenger's syndrome in the advanced stages include central cyanosis, dyspnoea, fatigue, haemoptysis, syncope and right-sided heart failure. Survival of patients with Eisenmenger's syndrome is clearly less than that of the general population, but appears to be better than that of patients with idiopathic PAH in a comparable functional class. The treatment strategy for patients with PAH associated with congenital systemic-to-pulmonary shunts and, in particular, those with Eisenmenger's syndrome is based mainly on clinical experience rather than being evidence based. General measures include recommendations for physical activity, pregnancy, infections, air travel, exposure to high altitudes and elective surgery, and that psychological assistance be provided as necessary. Phlebotomies are required only when hyperviscosity of the blood is evident, usually when the haematocrit is >65%. The use of supplemental oxygen therapy is controversial and it should be used only in patients in whom it produces a consistent increase in arterial oxygen saturation. Oral anticoagulant treatment with warfarin can be initiated in patients with pulmonary artery thrombosis and absent, or only mild, haemoptysis. The following three classes of drugs targeting the correction of abnormalities in endothelial dysfunction have been approved recently for the treatment of PAH: (i) prostanoids; (ii) endothelin receptor antagonists; and (iii) phosphodiesterase-5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts, as well as in patients with Eisenmenger's syndrome. One randomized controlled trial reported favourable short- and long-term outcomes of treatment with the orally active dual endothelin receptor antagonist bosentan in patients with Eisenmenger's syndrome. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation are options for Eisenmenger's syndrome patients with a poor prognosis. A treatment algorithm based on the one used in the treatment of PAH patients is proposed for patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome.

Background: Remodelling of the heart in patients with pulmonary arterial hypertension (PAH) involves both the right and left ventricles. The objective of the present study was to analyse the dimensions of the right and left ventricles as assessed by echocardiography, and to compare this with haemodynamics and exercise capacity in patients with PAH.Materials and methods: Transthoracic echocardiogram, exercise capacity as assessed by the 6-minute walk test and right-heart catheterization were performed within 1 week in 66 consecutive patients with PAH. Univariate correlation and multiple regression analyses were performed to relate echocardiographic and clinical variables.Results: Left ventricular end-diastolic area index had the best correlation with cardiac index (r = 0.62; p < 0.001) and 6-minute walk distance (r = 0.56; p < 0.001). The ratio of the right to left ventricular area correlated with right atrial pressure (r = 0.62; p < 0.001). Right ventricular percent change in area correlated with ...

Objective: Abnormal large artery function plays an important role in the pathogenesis of cardiovascular (CV) diseases. Prior studies have suggested that the principal determinants of arterial stiffening are age, Blood Pressure (BP) and others CV risk factors such as dyslipidemia and diabetes. However, scanty data are available on the role of psychological factors on arterial stiffness. Previous work have shown that arterial stiffness was associated with depression in the elderly and in healthy adolescents, but no study has focused on the role of depression and other psychological variables in hypertensive patients. The aim of the current cross-sectional study was to evaluate the association between depression, anxiety, perceived stress, Type A personality, and Type D personality and Pulse Wave Velocity (PWV) in a cohort of hypertensive patients, using baseline examination data of the TIPICO project. Design and method: A total of 259 outpatients (ages 18–80 years) followed by the Hypertension Unit of S. Gerardo Hospital (Monza, Italy) affected by essential hypertension were recruited. Aortic stiffness was evaluated by PWV between the carotid and the femoral artery of the same side with the patient in the supine position. Moreover, anamnestic data, clinical BP, and laboratory data were evaluated. Patients were asked to complete a battery of psychological questionnaires under the guidance of a psychologist. The associations between psychological variables and PWV was explored using multivariate stepwise linear regression analysis and regression coeffi cients (b) were given per 1-point increment. Results: The mean age was 55.9 ± 10.1years, SBP and DBP were 135.6 ± 17.7 and 82.5 ± 9.1mmHg and PWV was 8.6 ± 2.1m/s. The results from the multivariate stepwise linear regression analysis showed that age (beta = 0.284, p < 0.001), pulse pressure (beta = 0.369, p < 0.001), dyslipidemia (beta = 0.130, p = 0.012), family history of CV disease (beta = -0.123, p = 0.017), and depression (beta = 0.126, P = 0.014) were signifi cantly and independently associated with PWV. Conclusions: The current study shows that, among psychological factors, higher levels of depression is related to higher PWV, while anxiety, perceived stress, Type A personality, and Type D personality are not associated with arterial stiffness. Depression assessment and target intervention to reduce it are recommended in hypertensive patients.

Özet– Pulmoner hipertansiyon alanındaki önemli gelişmelerden sonra, en önemli görevlerden biri uzun dönem sonuçlarla ilişkili kinik tedavi hedeflerini belirleme gereksinimidir. Modifiye New York Kalp Cemiyeti fonksiyonel sınıf I veya II, 380 metreden fazla 6 dakika yürüme mesafesine ulaşma, ekokardiyografide sağ ventrikül boyutunun ve fonksiyonunun normalleşmesi, B-tip natriüretik peptit düzeyinin azalması ya da normalleşmesi ve sağ atriyal basıncının 8 mmHg’den düşük ve kardiyak indeksin 2,5 L/dk/m2 ’den büyük olması güncel hedeflerdir. Bununla birlikte, egzersiz kapasitesi ve sağ kalp fonksiyonu gibi uzun dönem sonuçlarla daha fazla ilişkili olan parametreleri hedefleyen daha güçlü ve net sınırlarla çizen “eşiğin” daha yüksek tutulması gerektiği açık hale gelmektedir; Özellikle, manyetik rezonans görüntüleme ve BNP/N-terminal pro B tip natriüretik peptit gibi doğru ve noninvaziv olarak sağ ventrikül fonksiyonunu belirleyen testler, temel belirleyiciler ve tedavi hedefleri olarak hizmet etmek için umut verici göstergeler olarak ortaya çıkmaktadır. Ayrıca, sonuçlar üzerine odaklı çalışmalar tek başına hiçbir testin güvenilir bir şekilde uzun vadeli prognostik belirteç olarak kullanılamayacağını ve birleşik tedavi hedeflerinin uzun dönem sonuçlarının daha iyi öngördüreceğini gösterdi. Yeniden düzenlenen tedavi hedeflerinin şunlar olduğu ileri sürülmüştür: Modifiye New York Kalp Cemiyeti fonksiyonel sınıf I veya II, ≥380 ve 440 m arasındaki 6 dakika yürüme mesafesi, kardiyopulmoner egzersiz testi-15 ml/d/kg’dan fazla tepe oksijen tüketim ölçümü ve 45 l/dak’dan az karbondioksit için solunum eşdeğeri, normale yakın BNP değeri, ile normal/normale yakın sağ ventrikül boyut ve fonksiyonlarını gösteren ekokardiyografi ve/veya kardiyak manyetik rezonans görüntüleme ve 8 mmHg’den az sağ atriyal basınçla ve 2,5-3 l/dak/m²’den fazla kardiyak indeksle birlikte sağ ventrikül fonksiyonunun normalleştiğini gösteren hemodinamik parametreler. (J Am Coll Cardiol 2013;62:D73–81) With significant therapeutic advances in the field of pulmonary arterial hypertension, the need to identify clinically relevant treatment goals that correlate with long-term outcome has emerged as 1 of the most critical tasks. Current goals include achieving modified New York Heart Association functional class I or II, 6-min walk distance >380 m, normalization of right ventricular size and function on echocardiograph, a decreasing or normalization of B-type natriuretic peptide (BNP), and hemodynamics with right atrial pressure 2.5 L/dk/m2. However, to more effectively prognosticate in the current era of complex treatments, it is becoming clear that the "bar" needs to be set higher, with more robust and clearer delineations aimed at parameters that correlate with long-term outcome; namely, exercise capacity and right heart function. Specifically, tests that accurately and noninvasively determine right ventricular function, such as cardiac magnetic resonance imaging and BNP/N-terminal pro-B-type natriuretic peptide, are emerging as promising indicators to serve as baseline predictors and treatment targets. Furthermore, studies focusing on outcomes have shown that no single test can reliably serve as a long-term prognostic marker and that composite treatment goals are more predictive of long-term outcome. It has been proposed that treatment goals be revised to include the following: modified New York Heart Association functional class I or II, 6-min walk distance 380 to 440 m, cardiopulmonary exercise test-measured peak oxygen consumption >15 ml/min/kg and ventilatory equivalent for carbon dioxide 2.5 to 3.0 l/min/m2. (J Am Coll Cardiol 2013;62:D73-81) ©2013 by the American College of Cardiology Foundation.

Following a brief introduction covering the clinical signs and symptoms of pulmonary hypertension (PH), its most recent classification into six groups, and the computed tomography (CT) features common to all forms of PH, this paper illustrates the typical patterns that can be found on chest radiography and CT in rare causes of PH. We present and compare with the existing literature our personal series of cases of rare forms of PH, found in the following diseases: veno-occlusive disease, pulmonary capillary haemangiomatosis, non-thrombotic pulmonary embolism (tumour embolism and carcinomatous lymphangitis, talcosis, hydatid disease), pulmonary artery sarcoma, neurofibromatosis, sarcoidosis, and Langerhans cell histiocytosis. Rare forms of PH show low incidence and prevalence, and are, therefore, poorly recognised. Their diagnosis is a challenge for clinicians, pathologists, and radiologists, and any additional knowledge about the CT findings may help the diagnosis in the case of patients affected by PH of unknown origin.

Treatment of pulmonary arterial hypertension (group 1 of clinical classification) has been recently characterized by important progresses, particularly in pharmacological therapy. Only until few years ago, patients with pulmonary arterial hypertension were treated with non-specific drugs, such as diuretics and digoxin for right heart failure and calcium-channel blockers in the minority of cases, responders to the acute vasoreactivity test. In addition, use of oral anticoagulant treatment was supported by uncontrolled studies. In the last 15 years (in particular in the last 8 years) different randomized controlled trials assessing the functional, clinical and hemodynamic efficacy of three classes of targeted drugs (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) with pulmonary vascular dilating and antiproliferative effects have been performed. This information has allowed the proposal of an evidence-based treatment algorithm. Treatment starts with general measures (physical activity, fertility control, respiratory tract infection, etc.) and supportive therapy (anticoagulant therapy, diuretics, oxygen, digoxin). Patients who respond to the acute vasoreactivity test (10% of idiopathic form) are treated with high doses of calcium-channel blockers, non-responders with targeted therapies either on monotherapy or combination. Usually an oral active drug is initiated and a second compound of a different class is combined in case of non-satisfactory response to the first treatment. Combination therapy should be performed only in specialized centers with large experience on use of targeted therapies and their relevant side effects. In case of failure of medical therapy, possible options are balloon atrial septostomy and/or listing for lung or heart-lung transplantation. As available treatments do not constitute a cure for pulmonary arterial hypertension, further progresses are expected in the near future.