SARS-CoV-2 pneumonia and Eisenmenger's Syndrome: doubling the challenge.

Eisenmenger's syndrome (ES) is the most severe phenotype of pulmonary arterial hypertension (PAH) secondary to congenital heart disease. In these cases, a significant systemic-to-pulmonary (left-to-right) shunting triggers the development of pulmonary vascular disease (PVD) and pulmonary hypertension. In cases of acute hypoxemic respiratory failure in patients with ES, high flow nasal cannula (HFNC) oxygen therapy should be considered as a first-line approach in order to avoid pulmonary complications and right ventricular overload related to positive pressure ventilation. Here, we report a case of HFNC use in a patient with COVID-19 infection and ES.

Significant burden of post-COVID exertional dyspnoea in a South-Italy region: knowledge of risk factors might prevent further critical overload on the healthcare system.

Background: Exertional dyspnoea in post-COVID syndrome is a debilitating manifestation, requiring appropriate comprehensive management. However, limited-resources healthcare systems might be unable to expand their healthcare-providing capacity and are expected to be overwhelmed by increasing healthcare demand. Furthermore, since post-COVID exertional dyspnoea is regarded to represent an umbrella term, encompassing several clinical conditions, stratification of patients with post-COVID exertional dyspnoea, depending on risk factors and underlying aetiologies might provide useful for healthcare optimization and potentially help relieve healthcare service from overload. Hence, we aimed to investigate the frequency, functional characterization, and predictors of post-COVID exertional dyspnoea in a large cohort of post-COVID patients in Apulia, Italy, at 3-month post-acute SARS-CoV-2 infection. Methods: A cohort of laboratory-confirmed 318 patients, both domiciliary or hospitalized, was evaluated in a post-COVID Unit outpatient setting. Post-COVID exertional dyspnoea and other post-COVID syndrome manifestations were collected by medical history. Functional characterization of post-COVID exertional dyspnoea was performed through a 6-min walking test (6-mwt). The association of post-COVID exertional dyspnoea with possible risk factors was investigated through univariate and multivariate logistic regression analysis. Results: At medical evaluation, post-COVID exertional dyspnoea was reported by as many as 190/318 patients (59.7%), showing relatively high prevalence also in domiciliary-course patients. However, functional characterization disclosed a 6-mwt-based desaturation walking drop in only 24.1% of instrumental post-COVID exertional dyspnoea patients. Multivariate analysis identified five independent predictors significantly contributing to PCED, namely post-COVID-fatigue, pre-existing respiratory co-morbidities, non-asthmatic allergy history, age, and acute-phase-dyspnoea. Sex-restricted multivariate analysis identified a differential risk pattern for males (pre-existing respiratory co-morbidities, age, acute-phase-dyspnoea) and females (post-COVID-fatigue and acute-phase-dyspnoea). Conclusion: Our findings revealed that post-COVID exertional dyspnoea is characterized by relevant clinical burden, with potential further strain on healthcare systems, already weakened by pandemic waves. Sex-based subgroup analysis reveals sex-specific dyspnoea-underlying risk profiles and pathogenic mechanisms. Knowledge of sex-specific risk-determining factors might help optimize personalized care management and healthcare resources.

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.

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.

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.).

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.

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.

[Diagnostic and therapeutic models for the management of acute pulmonary embolism: an issue of local available resources]. / Modelli diagnostici e terapeutici nel trattamento dell'embolia polmonare acuta: una questione di risorse locali.

Acute pulmonary embolism (PE) still represents the third leading cause of cardiovascular mortality in developed countries. In this regard, the last European guidelines offer important suggestions on the management of the disease in daily clinical practice but, at the same time, they do not take into account the feasibility of the recommendations according to the local available resources, including the presence or lack of adequate healthcare facilities (cardiological intensive care unit, cath-lab) or specialists (cardiologist available on a 24 h basis, interventional cardiologist, cardiac surgeon, etc.) all over the day. In the real clinical practice, those recommendations should be adapted to the local available resources. The aim of this document is to provide some suggestions regarding the diagnosis and treatment of acute PE, according to the possible available resources in different local circumstances.

[ANMCO statement: Arterial and venous thromboembolism: what implications for clinical practice?] / Statement ANMCO: Tromboembolismo arterioso e venoso: quali implicazioni per la pratica clinica?

Acute myocardial infarction, stroke, peripheral arterial disease and pulmonary embolism share thrombosis as a common mechanism. Some well-known risk factors for arterial thromboembolism are recognized as "weak risk factors" of venous one, too. Arterial and venous thrombosis share also some pathophysiological mechanisms, including inflammation, endothelial damage, and hypercoagulability. It is likely, thus, that any disease related to arterial and venous thrombosis belong to the same "pan-vascular syndrome", that constitutes itself a chronic, recurrent inflammatory disease. According to the available data, there are elements for implementing an omni-comprehensive cardiovascular evaluation after an episode of venous thromboembolism, requiring the investigations, in addition to the known unrecognized prothrombotic conditions, also of indirect signs and risk factors for a possible arterial thromboembolic event. Large, prospective studies are needed to establish the more appropriate therapeutic strategies in this context.The aim of the present statement is to make aware all the physicians involved in the management of arterial and venous diseases and to provide some tools for evaluating the implications of related major risk factors. Thus, it could be possible to lay the foundation for a reduction of total cardiovascular risk, in terms of primary and secondary prevention of arterial and venous thromboembolism.

Practical implementation of the Endocarditis Team in 'functional' reference centres: the Italian hospital network experience and recommendations of the Italian Society of Echocardiography and Cardiovascular Imaging.

: The 2015 European Society of Cardiology (ESC) guidelines for the management of infective endocarditis recommend the use of a multidisciplinary team in the care of patients with infective endocarditis. A standardized collaborative approach should be implemented in centres with immediate access to different imaging techniques, cardiac surgery and health professionals from several specialties. This position paper has been produced by the Task Force for Management of Infective Endocarditis of Italian Society of Echocardiography and Cardiovascular Imaging (SIECVI) with the aim of providing recommendations for the implementation of the Endocarditis Team within the Italian hospital network. On the basis of the Italian hospital network with many cardiology facilities encompassing a total of 405 intensive cardiac care units (ICCUs) across the country, 224 (3.68 per million inhabitants) of which have on-site 24-h PCI capability, but with relatively few centres equipped with cardiac surgery and nuclear medicine, in the present article, the SIECVI Task Force for Management of Infective Endocarditis develops the idea of a network where 'functional' reference centres act as a link with the periphery and with 'structural' reference centres. A number of minimum characteristics are provided for these 'functional' reference centres. Outcome and cost analysis of implementing an Endocarditis Team with functional referral is expected to be derived from ongoing Italian and European registries.

Multifactorial Genesis of a Seeming Case of Pulmonary Hypertension.

Herein, we report the case of a 44-year-old female with end-stage renal disease on hemodialysis. She was admitted to our hospital to evaluate if she was eligible for a kidney transplant. Transthoracic echocardiography showed a markedly dilated coronary sinus and an unexpected finding of increased right ventriculoatrial gradient. A saline contrast echocardiography to confirm the presence of persistent left superior vena cava (PLSVC) was not performed because of arteriovenous fistula (FAV) for hemodialysis on the left forearm. Therefore, computed tomography angiography was performed, and it showed the PLSVC. We also proceeded with a transesophageal echocardiography which showed an atrial septal defect (ASD) of the sinus venous type hemodynamically significant. In this case, we identified a rare association of PLSVC with a ASD; therefore, there is a right ventricular volume overload because of the ASD hemodynamically significant and high flow FAV leading to a condition of a seeming pulmonary hypertension.