[ANMCO/SIC Consensus statement on pulmonary arterial hypertension]. / Documento di consenso ANMCO/SIC sull'ipertensione arteriosa polmonare.

Pulmonary hypertension (PH) is a frequent pathological condition worldwide, mainly secondary to cardiovascular and respiratory diseases, with a poor prognosis. Pulmonary arterial hypertension (PAH) is a rare form that affects the arterial pulmonary vasculature. PH and PAH are characterized by non-specific symptoms and a progressive increase of pulmonary vascular resistance that results in progressive, sometimes irreversible, right ventricular dysfunction. In recent years, a growing medical and social commitment on this disease allowed more accurate diagnosis in shorter times. However, the gap between guidelines and clinical practice remains a challenge for all medical doctors involved in the disease management. Considering the needs to share and describe diagnostic and therapeutic pathways, to measure the results obtained and to address the economical and organizational problems of this disease, all involved figures should collaborate to improve its prognostic impact and health expenses. In this consensus document, the PH experts of the Italian Association of Hospital Cardiologists (ANMCO) together with those of the Italian Society of Cardiology (SIC), address 1) definition, classification and unmet needs of PH and PAH; 2) classification and characteristics of centers involved in the diagnosis and treatment of the disease; 3) proposal of organization of a diagnostic-therapeutic pathway, based on robust and recent scientific evidence.

Relevance of comorbidities on initial combination therapy in pulmonary arterial hypertension.

Rationale: Demographic characteristics of pulmonary arterial hypertension (PAH) patients have changed over time, but the effects of cardiovascular risk factors on risk status and pulmonary vascular resistance (PVR) reduction with initial oral combination therapy are not known. Therefore, we tested the relevance of cardiovascular comorbidities in this setting. Methods: The study enrolled 181 treatment-naive PAH patients with a 6-month (IQR 144-363 days) right heart catheterisation and risk assessment after initial oral combination therapy. Results: Group A included 96 (53.0%) patients without cardiac comorbidities; Group B included 54 (29.8%) patients with one cardiac comorbidity; Group C included 31 (17.1%) patients with two cardiac comorbidities or more. Group C patients were older with a balanced sex distribution. There was a significant difference in PVR reduction moving from the absence to one or at least two cardiac comorbidities, respectively: median -45.0%, -30.3%, -24.3%. A European Respiratory Society/European Society of Cardiology low-risk status was present at first follow-up in 50 (52.0%) patients in Group A, 19 (35.1%) in Group B and 9 (29.0%) in Group C; a REVEAL 2.0 low-risk status was present at first follow-up in 41 (42.0%) patients in Group A, 15 (27.7%) in Group B and 7 (22.6%) in Group C. Group A patients were 2.3 times more likely to achieve/maintain a low-risk status compared with Group B and C (OR 2.27, 95% CI 1.15-4.54, p=0.02). No significant difference was observed between patients with non-cardiac comorbidities and those without comorbidities. Conclusion: Initial oral combination therapy seems associated with a less effective response for patients with cardiovascular comorbidities compared with the others, related to the magnitude of treatment-induced decrease in PVR.

Removal of an active fixation coronary sinus pacing lead 5 years postimplant: A case report.

Active fixation for a lead in the coronary sinus may be essential to select the optimal left ventricular pacing site, maximize the effectiveness of cardiac resynchronization therapy (CRT) and avoid dislodgement. The Medtronic Attain Stability lead allows fixation through a side helix concentric with the lead body. Although electrical performance of such a lead is well known, evidence of extractability remains poor especially in the long term. We describe the removal of an Attain Stability lead 63 months after implantation which, to the best of our knowledge, is the longest implant duration that has ever been reported, in an 81-year-old male patient. It was successfully achieved using simple traction and rotation maneuvers, demonstrating the long-term removal feasibility of such device.

Leadless transcatheter pacemaker: Indications, implantation technique and peri-procedural patient management in the Italian clinical practice.

BACKGROUND: Safety and efficacy of leadless pacemakers (L-PM) have been demonstrated in multiple clinical trials, but real-world data on patient selection, implantation technique, and peri-procedural patient management in a clinical practice setting are lacking. METHODS: Consecutive patients undergoing L-PM implantation in 14 Italian centers were followed in a prospective, multicentre, observational project. Data on baseline patient characteristics, clinical indications, implantation procedure, and peri-procedural patient management were collected. The rate and nature of device-related complications were also recorded. RESULTS: A total of 782 L-PM patients (68.4% male, 75.6 ± 12.4 years) were included in the analysis. The main patients-related reason leading to the choice of implanting a L-PM rather than a conventional PM was the high-risk of device infection (29.5% of cases). The implantation success rate was 99.2%. The median duration of the procedure was 46 min. In 90% of patients the device was implanted in the septum. Of patients on oral anticoagulant therapy (OAT) (n = 498) the implantation procedure was performed without interrupting (17.5%) or transiently interrupting OAT without heparin bridging (60.6%). During a median follow-up of 20 months major device-related complications occurred in 7 patients (0.9%): vascular access-site complications in 3 patients, device malfunction in 2 patients, pericardial effusion/cardiac tamponade in one patient, device migration in one patient. CONCLUSIONS: In the real world setting of Italian clinical practice L-PM is often reserved for patients at high-risk of infection. The implantation success rate was very high and the risk of major complications was low. Peri-procedural management of OAT was consistent with available scientific evidence.

Performance of transcatheter pacing system use in relation to patients' age.

PURPOSE: Real-world safety data on the use of transcatheter pacing systems particularly in very elderly patients is still limited. The aim of this analysis was to investigate the effect of age on the safety and efficacy of leadless pacemaker implant. METHODS: From May 2016 through July 2019, 577 patients were implanted with a leadless single-chamber pacemaker according to current pacing indication in 15 Italian cardiologic centers. The population was divided into age quartiles for evaluation, including (1) < 70 years, (2) 70-77 years, (3) 78-83 years, and (4) ≥ 83 years. Procedural data, complications, and electrical parameters were collected at baseline and during the follow-up. RESULTS: Procedural-related complication occurrence was very low (< 1.0%) and similar in the four subgroups according to age even if the older patients were more frail. No cardiac tamponade was reported. Among the groups, no difference was observed in procedural time, fluoroscopy time duration, and electrical parameters (mean pacing impedance: 750 ± 192 and 599 ± 156, mean pacing threshold: 0.7 ± 0.5 and 0.7 ± 0.6, and mean right ventricular sensing 10.7 ± 6.1 and 11.5 ± 4.8 at implant and last follow-up, respectively). CONCLUSIONS: The reported data demonstrated a high degree of safety during leadless implant across all patient ages. Procedural complications and device electrical measurements were similar among the different ages.

The paradox of pulmonary arterial hypertension in Italy in the COVID-19 era: is risk of disease progression around the corner?

OBJECTIVE: The coronavirus disease 2019 (COVID-19) outbreak has led to significant restrictions on routine medical care. We conducted a multicentre nationwide survey of patients with pulmonary arterial hypertension (PAH) to determine the consequences of governance measures on PAH management and risk of poor outcome in patients with COVID-19. MATERIALS AND METHODS: The present study, which included 25 Italian centres, considered demographic data, the number of in-person visits, 6-min walk and echocardiographic test results, brain natriuretic peptide/N-terminal pro-brain natriuretic peptide test results, World Health Organization functional class assessment, presence of elective and non-elective hospitalisation, need for treatment escalation/initiation, newly diagnosed PAH, incidence of COVID-19 and mortality rates. Data were collected, double-checked and tracked by institutional records between March 1 and May 1, 2020, to coincide with the first peak of COVID-19 and compared with the same time period in 2019. RESULTS: Among 1922 PAH patients, the incidences of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and COVID-19 were 1.0% and 0.46%, respectively, with the latter comparable to that in the overall Italian population (0.34%) but associated with 100% mortality. Less systematic activities were converted into more effective remote interfacing between clinicians and PAH patients, resulting in lower rates of hospitalisation (1.2% versus 1.9%) and related death (0.3% versus 0.5%) compared with 2019 (p<0.001). A high level of attention is needed to avoid the potential risk of disease progression related to less aggressive escalation of treatment and the reduction in new PAH diagnoses compared with 2019. CONCLUSION: A cohesive partnership between healthcare providers and regional public health officials is needed to prioritise PAH patients for remote monitoring by dedicated tools.

Authors' response to the Letter to the Editor regarding: Post severe COVID-19 infection lung damages study. The experience of early three months multidisciplinary follow-up.

To the Editor, we thank Dr Ruggeri et al. for their interest in our previously published manuscript focused on lung damages after severe respiratory COVID-19 infection...

Post severe COVID-19 infection lung damages study. The experience of early three months multidisciplinary follow-up.

The correct type and time of follow-up for patients affected by COVID19 ARDS is still unclear. The aim of this study was to evaluate at the survivors to COVID19 ARDS requiring non-invasive respiratory support (NRS) admitted to a Respiratory Intensive care unit (RICU) from March 8th till May 31th 2020 looking at all sequelae via a comprehensive follow up. All patients underwent a multi-disciplinary instrumental and clinical assessment within three months form admission to evaluate all infection related sequelae. Thirty-eight patients were enrolled Lung-Ultrasound (LUS) showed an outstanding discrimination ability (ROC AUC: 0.95) and a substantial agreement rate (Cohen's K: 0.74) compared to chest CT-scan detecting improvement of lung consolidations. Youden's test showed a cut-off pressure of 11 cmH2O ExpiratoryPAP-Continuous-PAP-max (EPAP-CPAP) applied at the airways during hospitalization to be significantly correlated (p value: 0.026) to the increased pulmonary artery common trunk diameter. A total of 8/38 patients (21.8%), 2 of whom during follow-up, were diagnosed with Pulmonary Emboli (PE) and started anticoagulant treatment. Patients with PE had a statistically significant shorter length of time of hospitalization, time to negative swab, CPAP/NIV duration, P/F ratio and D-dimers at follow-up compared to non PE. A comprehensive approach to patients with ARDS COVID19 requiring NRS is necessary. This study highlighted cardiopulmonary impairment related to the ARDS and to the high-EPAP-CPAP-max greater than 11mmHg provided during admission, the usefulness of LUS in monitoring post-infection recovery and the correct identification and  treatment of patients with PE during follow up.

Remote assessment of QT interval: A new perspective for implantable cardiac monitors.

BACKGROUND: There is an unmet need for simple tools for monitoring QT intervals. The feasibility of measuring the QT interval on the single­lead subcutaneous electrocardiogram (subECG) recorded and transmitted by implantable cardiac monitors (ICMs) has never been tested. METHODS: We performed a standard ECG in patients who had already been implanted with a long sensing vector ICM (BIOMONITOR, Biotronik SE&Co.) to calculate the corrected QT interval in lead II (QTc ECG). The QTc was then evaluated on the subECG provided by ICM both by using the programmer printout (QTc subECG) and the snapshot transmitted via home monitoring (QTc HM). Values were compared with Bland-Altman analyses. RESULTS: The study cohort consisted of 23 ICM recipients (age 58 ± 19 years, 35% female) implanted mainly for unexplained syncope (78%). The mean QTc ECG interval was 404 ± 31 ms. The T-wave was visible and QTc could be calculated in all patients using the ICM programmer printout and in 21 (91%) patients remotely. The QTc subECG and QTc HM were 405 ± 34 and 406 ± 32 ms. Compared to the QTc ECG, Bland-Altman analyses revealed a bias of -0.9 (95% confidence interval: -6.8/4.9) ms and 0.1 (-12.7/12.9) ms for QTc subECG and QTc HM, respectively. CONCLUSIONS: The QTc interval can be reliably estimated on in-person and remote subECG in most patients without bias compared to the ECG lead II assessment. This technology has the potential to facilitate remote QT interval monitoring.

Inadvertent left ventricular placement of ICD lead through the left subclavian artery right positioned in a patient with situs viscerum inversus and Kartagener syndrome.

Inadvertent placement of pacemaker and implantable cardioverter-defibrillator (ICD) leads in the left ventricle is a rare but well-recognized complication of device implantation. We report a case of an unicameral ICD lead inadvertently placed through the left subclavian artery right positioned, across the aortic valve into the left ventricle, in a patient with situs viscerum inversus. A transthoracic echocardiogram about a month after the procedure showed an unusual course of the lead. The lead was successfully removed without complications or sequelae. .

A Rare Case of Mitral Prosthesis Dislocation Due to a Circumferential Left Atrial Dissection.

Left atrial dissection is a rare entity mostly associated with mitral valve surgery and revealed in early post-operative period. This case report discusses a case of left atrial dissection associated with dislocation of the mechanical mitral prosthesis in the left atrium, which was peculiar in its anatomy and pathophysiology, occurred 12 years after surgery. (Level of Difficulty: Advanced.).

Dalbavancin Efficacy and Impact on Hospital Length-of-Stay and Treatment Costs in Different Gram-Positive Bacterial Infections.

BACKGROUND AND OBJECTIVES: The study aimed to evaluate the impact of dalbavancin therapy on both hospital length-of-stay (LOS) and treatment-related costs, as well as to describe the clinical outcome, in a retrospective cohort of patients with diverse Gram-positive bacterial infections, hospitalized in different specialty Units. METHODS: From July 2017 to July 2019, clinical and sociodemographic data were collected for all hospitalized patients switched to dalbavancin for the treatment of Gram-positive infections. LOS and treatment-related costs were assessed and compared to a hypothetical scenario where the initial standard antimicrobial therapy would have been administered in hospital for the same duration as dalbavancin. RESULTS: A total of 50 patients were enrolled. The observed infections were: acute bacterial skin and skin structure infections (ABSSSIs, 12 patients), complicated ABSSSIs (eight patients), osteoarticular infections (18 patients), vascular graft or cardiovascular implantable electronic devices (CIED) infections (12 patients). After a median of 14 [interquartile range (IQR) 7-28] days, the in-hospital antimicrobial therapy was switched to dalbavancin 1500 mg. When appropriate, considering the site and the clinical course of the infection, 1500 mg doses were repeated every 14 days until recovery. Overall, 49/50 (98%) patients reported clinical success at the end of therapy. No relapses were observed in 37 patients for whom a median follow-up of 150 (IQR 30-180) days was available. By switching to dalbavancin, a median of €8,259 (IQR 5644-17,270) and 14 hospital days (IQR 22-47) per patient were saved. CONCLUSIONS: In this experience, the use of dalbavancin contributed to shorten LOS and treatment-related costs, especially in difficult Gram-positive infections requiring prolonged therapy.

Lung ultrasonography for long-term follow-up of COVID-19 survivors compared to chest CT scan.

BACKGROUND: While lung ultrasonography (LUS) has utility for the evaluation of the acute phase of COVID-19 related lung disease, its role in long-term follow-up of this condition has not been well described. The objective of this study is to compare LUS and chest computed tomography (CT) results in COVID-19 survivors with the intent of defining the utility of LUS for long-term follow-up of COVID-19 respiratory disease. METHODS: Prospective observational study that enrolled consecutive survivors of COVID-19 with acute hypoxemic respiratory failure (HARF) admitted to the Respiratory Intensive Care Unit. Three months following hospital discharge, patients underwent LUS, chest CT, body plethysmography and laboratory testing, the comparison of which forms the basis of this report. RESULTS: 38 patients were enrolled, with a total of 190 lobes analysed: men 27/38 (71.1%), mean age 60.6 y (SD 10.4). LUS findings and pulmonary function tests outcomes were compared between patients with and without ILD, showing a statistically significant difference in terms of LUS score (p: 0.0002), FEV1 (p: 0.0039) and FVC (p: 0.012). ROC curve both in lobe by lobe and in patient's overall analysis revealed an outstanding ILD discrimination ability of LUS (AUC: 0.94 and 0.95 respectively) with a substantial Cohen's coefficient (K: 0.74 and 0.69). CONCLUSIONS: LUS has an outstanding discrimination ability compared to CT in identifying an ILD of at least mild grade in the post COVID-19 follow-up. LUS should be considered as the first-line tool in follow-up programs, while chest CT could be performed based on LUS findings.

Multimodality imaging of a rare pulmonary artery sessile mass: a case report.

BACKGROUND: Primary pulmonary artery masses are unusual entities that mimic pulmonary embolism (PE) in clinical presentation and on imaging studies. It is necessary to perform advanced diagnostic exams, such as transesophageal echocardiography (TEE) and cardiac magnetic resonance imaging (MRI), to determine the proper diagnosis. In unclear cases, laboratory findings, morphological follow-up, and response to anticoagulant therapy can help to clarify the diagnosis. CASE SUMMARY: A 47-year-old previously healthy man with worsening effort dyspnoea underwent chest computed tomography (CT) for suspicion of PE, which showed a pedunculated eccentric mass at the origin of the pulmonary artery causing severe stenosis. The patient was started on anticoagulation therapy, but, after TEE and cardiac MRI, a neoplastic fibroelastic mass was suspected. Unexpectedly, 18fluorodeoxyglucose (FDG) positron emission tomography (PET)-CT revealed a unique area of glucose uptake in the superior lobe of the left lung and not in the pulmonary artery. The biopsy was consistent with pleomorphic high-grade lung sarcoma. After 3 months of chemotherapy, a CT scan showed progression of the lung disease with no change in the arterial mass, which was therefore confirmed as pulmonary fibroelastoma. DISCUSSION: Due to the rarity of pulmonary artery tumours, they can be initially misdiagnosed as PE or a metastasis of a lung sarcoma. Three-dimensional TEE and cardiac MRI are particularly useful in differentiating tumours from PE.

The Diagnosis of Acute Myocarditis in Emergency (DAME) score: improving diagnostics within the emergency department.

PURPOSE: . The final diagnosis of myocarditis is challenging. The aim of our study was to provide the D.A.M.E. (Diagnosis of Acute Myocarditis in Emergency) Score for the fast identification of patients suffering from myocarditis at Emergency Department (ED). METHODS: . This was a multicenter, retrospective study involving three centers. All medical records from January 2010 to December 2014 reporting a final discharge diagnosis of myocarditis were considered. One hundred-four patients (mean age: 40.2±16.5 years) were enrolled. Clinical, biochemical and instrumental data were gathered. Data were analysed by means of logistic regression model and factorial analysis. A validation cohort from a fourth center was enrolled. RESULTS: . The final determinants of the DAME score were six: fever, chest pain, erythrocyte sedimentation rate (ESR) > 20 mm/h, C-reactive protein (hs-CRP) >3 mg/L, troponin serum levels >3 ng/L, and left ventricle ejection fraction < 50%. All of them received a specified score ranging from 0 to 4. A score > 4 was related to 75% probability of myocarditis; a final score ranging between 1 and 4 was related to 57% probability of myocarditis. ROC curve on the validation cohort (289 patients, 27 with myocarditis) demonstrated the best cut-off to be 7: AUC 0.958 (p< 0.001), sensibility: 100%, specificity: 85.11%, PPV: 40.9%, NPV: 100% (LR+: 6.72; LR-: 0.00). Logistic regression analysis revealed Odds Ratio equal to 2.83 (95% CI 1.90 - 4.20, p < 0.0001). CONCLUSIONS: . DAME score can offer a reliable tool in ED setting for the evaluation of patients suffering from suspected myocarditis.

Risk Reduction and Hemodynamics with Initial Combination Therapy in Pulmonary Arterial Hypertension.

Rationale: An initial oral combination of drugs is being recommended in pulmonary arterial hypertension (PAH), but the effects of this approach on risk reduction and pulmonary vascular resistance (PVR) are not known.Objectives: To test the hypothesis that a low-risk status would be determined by the reduction of PVR in patients with PAH treated upfront with a combination of oral drugs.Methods: The study enrolled 181 treatment-naive patients with PAH (81% idiopathic) with a follow-up right heart catheterization at 6 months (interquartile range, 144-363 d) after the initial combination of endothelin receptor antagonist + phosphodiesterase-5 inhibitor drugs and clinical evaluation and risk assessments by European guidelines and Registry to Evaluate Early and Long-Term PAH Disease Management scores.Measurements and Main Results: Initial combination therapy improved functional class and 6-minute-walk distance and decreased PVR by an average of 35% (median, 40%). One-third of the patients had a decrease in PVR <25%. This poor hemodynamic response was independently predicted by age, male sex, pulmonary artery pressure and cardiac index, and at echocardiography, a right/left ventricular surface area ratio of greater than 1 associated with low tricuspid annular plane systolic excursion of less than 18 mm. A low-risk status at 6 months was achieved or maintained in only 34.8% (Registry to Evaluate Early and Long-Term PAH Disease Management score) to 43.1% (European score) of the patients. Adding criteria of poor hemodynamic response improved prediction of a low-risk status.Conclusions: A majority of patients with PAH still insufficiently improved after 6 months of initial combinations of oral drugs is identifiable at initial evaluation by hemodynamic response criteria added to risk scores.

Usefulness of 18F-FDG PET/CT in Patients with Cardiac Implantable Electronic Device Suspected of Late Infection.

The presence of a cardiovascular implantable electronic device (CIED) can be burdened by complications such as late infections that are associated with significant morbidity and mortality and require immediate and effective treatment. The aim of this study was to evaluate the role of 18F-fluorodeoxyglucose positron-emission tomography/computed tomography (18F-FDG PET/CT) in patients with suspected CIED infection. Fifteen patients who performed a 18F-FDG PET/CT for suspicion of CIED infection were retrospectively analyzed; 15 patients, with CIED, that underwent 18F-FDG PET/CT for oncological reasons, were also evaluated. Visual qualitative analysis and semi-quantitative analysis were performed. All patients underwent standard clinical management regardless 18F-FDG PET/CT results. Sensitivity, specificity, accuracy, positive predictive value (PPV) and negative predictive value (NPV) resulted as 90.91%, 75%, 86.67%, 90.91% and 75% respectively. Maximum standardized uptake values (SUVmax) and semi-quantitative ratio (SQR) were collected and showed differences statistically significant between CIED infected patients and those who were not. Exploratory cut-off values were derived from receiver operating characteristic (ROC) curves for SUVmax (2.56) and SQR (4.15). This study suggests the clinical usefulness of 18F-FDG PET/CT in patients with CIED infection due to its high sensitivity, repeatability and non-invasiveness. It can help the clinicians in decision making, especially in patients with doubtful clinical presentation. Future large-scale and multicentric studies should be conducted to establish precise protocols about 18F-FDG PET/CT performance.