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Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause that leads to respiratory failure and death within few years of diagnosis. Pulmonary hypertension (PH) is a common complication in IPF, where it is strongly associated with increased morbidity and mortality. Patients with IPF and PH have particularly poor prognosis, despite current best medical therapies and the anti-fibrotic therapy with pirfenidone or nintedanib. The aim of our study was to assess the clinical and prognostic impact of PH in patients affected by IPF, already treated with pirfenidone or nintedanib. Seventy-four consecutive outpatients with a diagnosis of IPF, in therapy with pirfenidone or nintedanib, were prospectively enrolled in the study. All patients underwent pulmonary and cardiology assessment by clinical exam, spirometry, DLCO test, chest CT, 6MWT and echocardiography performed by a cardiologist experienced in PH in an ambulatory setting under resting conditions. GAP index has been determinate for all patients. During follow-up, all patients were evaluated every 6 months, or less if necessary. Data about mortality were then collected in a 3-year follow-up. Of the seventy-four patients enrolled, 38 were treated with pirfenidone and 36 with nintedanib. The two groups were comparable for age, gender, FVC, DLCO and PAPS. The patients were also divided in four groups, based on presence of mild/moderate/severe PH by echocardiography at baseline. Significant differences were found for DLCO and the GAP index. Severity of PH was significantly associated with a reduction of DLCO and with an increased GAP index. Survival was directly correlated with 6MWT (R = 0.48), DLCO (R = 0.29, p

A patient with bicuspid aortic valve was evaluated in our echocardiography laboratory over serial follow-up controls. Echocardiography showed trivial mitral valve regurgitation and the already known bicuspid aortic valve (Fig. 1a) with a mild regurgitation. An abnormal origin of the subclavian right artery, deriving from the distal aortic arch (Fig. 1b), was unexpectedly found. The patient underwent CT imaging of the thoracic aorta and supraaortic vessels, which confirmed the right subclavian originating from a lusory artery after a retroesophageal course (Fig. 1c–f). No further vascular abnormalities were found. The caliber of the thoracic aorta was normal as well. Lusory artery is an uncommon congenital vascular abnormality, whose incidence varies between 0.2–2.5 % [1], resulting from interruption of the fourth right aortic arch between the common carotid artery and subclavian artery and the persistence of the retroesophageal aortic arch [2]. The right subclavian artery thus originates as the last branch of the aortic arch. The condition is usually asymptomatic, even if it may cause dysphagia due to esophageal compression [1]. Complications are described, ranging from atherosclerotic disease and thrombosis [3], potentially eased by the abnormal retroesophageal course, to aneurysma [4] and arterioesophageal fistulae, and other possible serious events usually precipitated by the placement of nasogastric tubes in patients where the erosion of the esophagus was already present [5]. Despite the potentially dangerous implications, this anatomical variant does not usually require surgery, although restoring a normal course of the right subclavian artery by aortic arch repair is feasible and often recommended [1]. The lusory artery is known to be associated with other congenital abnormalities, especially Down syndrome [2]. The association with bicuspid aortic valve appears new. One of the most consistent findings in bicuspid aortic valve is dilatation of the ascending aorta, even in the absence of clinically significant valvular dysfunction. Dilatation of the ascending aorta represents a key risk factor for dissection and rupture. So, all patients with a bicuspid valve and dilatation of the ascending aorta should have evaluation of the aorta with an MRI or CT angiography. Cardiac MRI is preferred because it also provides the added benefit of information regarding LV function, LV dimensions, and assessment of valve stenosis/regurgitation severity. For those with no aortic dilatation, we tend to use only echocardiography for follow-up. To our knowledge, there is no any association between lusory artery and persistent branchial cleft cysts. Further attention should be placed in screening of paucisymptomatic vascular abnormalities in patients found to bear bicuspid aortic valve, so that the need for repair can be determined, according to the risk of potential complications and clinical conditions. L. D. Martino M. Correale (&) D. Cocco M. D. Biase N. D. Brunetti Department of Cardiology, ‘‘Ospedali Riuniti’’ OO.RR, University of Foggia, viale L Pinto, 1, 71100 Foggia, Italy e-mail: opsfco@tin.it

The application of tissue Doppler imaging (TDI) has shown remarkable growth in clinical practice during the past few years, especially, in risk stratification of patients with coronary heart disease or heart failure (systolic and diastolic). Myocardial performance index (MPI) is a Doppler echocardiographic parameter defined as the sum of the isovolemic contraction and relaxation times divided by the ejection time. It is considered as a reliable parameter to assess global left ventricular function. Cardiac time intervals and MPI have also been found as new applications in diagnosing cardiotoxicity from chemotherapy, COPD, valvular heart disease, pulmonary hypertension and endocrinopathies.

Tremendous advances have been made in understanding the pathophysiology and treatment of congestive heart failure (CHF). However, diagnosis still remains difficult, even with a comprehensive physical examination. Symptoms such as dyspnea are non-specific and poorly sensitive indicators for early CHF that can be largely undetected. The discovery of natriuretic peptides (BNP) as diagnostic biomarkers has been one of the most critical advances for heart failure diagnosis. Therefore, both B-type and N-terminal pro-B-type have potential role in the diagnosis of heart failure, as well as in prognostic risk assessment. A single determination of BNP at any time during the progression of chronic HF provides a clinically useful tool for risk stratification. The hypothesis that repeated measurements might carry prognostic information beyond a single measure was confirmed in different settings. One of the main interests is given to the values of repeated determinations for monitoring progression of disease, and for the evaluation of the clinical effects of medical therapy. Nevertheless, despite thousands of papers describing their potential utility, current guidelines have not endorsed the highest level of recommendation for their use, in part, because the application in clinical practice is often limited for the absence of well codified cut off. Recently, European guidelines emphasized the role of natriuretic peptides as potential laboratory markers. In the near future, algorithm building will take into consideration clinical and echocardiographic parameters as well as NP measurements, and this may lead to a correct diagnosis and identification of patients at high risk. The purpose of this review is to discuss the clinical approaches and future applications of natriuretic peptides in heart failure and coronary disease.

We read with great interest the article by Fassio et al. [1] which provided a detailed review on Kounis syndrome (allergic acute coronary syndrome). The authors approached the issue both from the cardiologist’s and the allergist’s view. Authors report that initial descriptions of Kounis syndrome were focused on patients with ‘‘angiographycally’’ normal coronary arteries and acute release of allergic mediators (histamine or leukotrienes); the prognosis of these patients is good, with the left ventricular function normalizing over 3 days to several weeks [2]. However, we recently reported a case (a 70-year-old woman) of transient apical ballooning (Tako-Tsubo syndrome) [3], with mild ST-elevation in anterior leads and increased troponin-I levels [1.09 ng/ml (n.v. \ 0.03)], triggered by administration of antibiotics (cephalosporin) in a patient with history of asthma and allergy. Tako-Tsubo syndrome is characterized by acute onset of chest symptoms, ECG changes with elevated cardiac markers mimicking acute myocardial infarction, left ventricular wall motion abnormalities in the apical region with preserved function of base, and normal coronary arteries [4]. Those affected are typically older women presenting after a stressful trigger, either emotional or physical. According to authors’ opinion, could there be a link between Kounis syndrome’s variant with ‘‘angiographycally’’ normal coronary arteries and Tako-Tsubo syndrome triggered by administration of drugs in patient with history of asthma and allergy?