Intravenous prostanoids in systemic sclerosis-associated pulmonary arterial hypertension: a single-centre experience.

OBJECTIVES: The current study evaluates survival rates among SSc-associated pulmonary arterial hypertension (SSc-PAH) patients on i.v. prostanoids, and short-term impact of i.v. prostanoids on clinical and haemodynamic parameters. METHODS: Baseline demographics, invasive and non-invasive data, European Society of Cardiology (ESC) score and REVEAL score of 81 SSc-PAH patients (median age 61 years, interquartile range 54-67 years, 84% females) were prospectively recorded, from November 2006 till November 2020, before initiation of i.v. prostanoids, and at first formal reassessment. Survival data were retrieved from National Health Service Spine and hospital databases. RESULTS: Significant improvements in clinical and haemodynamic parameters in response to i.v. prostanoid therapy were documented. Functional class (FC) (16.6% improved by 1FC, P =0.041), mean pulmonary arterial pressure (-6.5 mmHg, P =0.036), pulmonary vascular resistance (-2.6 WU, P =0.012), cardiac index (Q/m2) (+0.7 l/min/m2, P =0.003) and mixed venous oxygen saturation (SvO2) (+3%, P =0.036) improved. Estimated survival for CTD-PAH patients on i.v. prostanoids was 64%, 31% and 18%, at 1 year, 3 years and 5 years, respectively. Independent baseline predictors of mortality were older age (HR: 1.043, 95% CI: 1.011-1.075, P =0.007), higher N-terminal pro-brain natriuretic peptide levels (HR: 2.191, 95% CI: 1.131-4.243, P =0.020), and lower SvO2 levels (HR: 0.962, 95% CI: 0.926-0.998, P =0.039). High ESC risk or high and very high REVEAL score was associated with significantly worse survival compared with patients with lower risk scores, both at baseline and when reassessed after a median of 6.5 months. CONCLUSIONS: Survival among SSc-PAH patients on i.v. prostanoids remains poor, risk scoring at baseline and after 6.5 months of therapy improves prognostication.

COVID-19 and the Heart: A Systematic Review of Cardiac Autopsies.

Importance: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated cardiac injury has been postulated secondary to several mechanisms. While tissue diagnosis is limited during the acute illness, postmortem studies can help boost our understanding and guide management. Objective: To report the cardiac tissue autopsy findings in coronavirus disease 2019 (COVID-19) decedents. Evidence Review: Articles published in PubMed and Embase reporting postmortem cardiac pathology of COVID-19 decedents till September 2020. We included adult studies excluding preprints. The Joanna Briggs Institute Critical Appraisal Checklist for Case Reports was used to assess quality. We extracted gross and histology data as well as the incidence of myocarditis, cardiac ischemia, thrombosis, and dilatation. We also looked at the reported cause of death (PROSPERO registration CRD42020190898). Findings: Forty-one relevant studies identified including 316 cases. The deceased were mostly male (62%) and elderly (median age, 75; range, 22-97 years). The most common comorbidities were hypertension (48%) and coronary artery disease (33%). Cardiac pathologies contributed to the death of 15 cases. Besides chronic cardiac pathologies, postmortem examination demonstrated cardiac dilatation (20%), acute ischemia (8%), intracardiac thrombi (2.5%), pericardial effusion (2.5%), and myocarditis (1.5%). SARS-CoV-2 was detected within the myocardium of 47% of studied hearts. Conclusions and Relevance: SARS-CoV-2 can invade the heart, but a minority of cases were found to have myocarditis. Cardiac dilatation, ischemia, mural, and microthrombi were the most frequent findings. The systematic review was limited by the small number of cases and the quality of the studies, and there is a need to standardize the cardiac postmortem protocols.

Pulmonary Hypertension Associated with Connective Tissue Disease.

Pulmonary hypertension (PH) is common in most forms of connective tissue disease (CTD); the prevalent type of PH depends on the particular CTD. Thus, pulmonary arterial hypertension (PAH) is dominantly associated with scleroderma, while postcapillary PH is most common in rheumatoid arthritis and lung disease-associated PH is typically found in myositis and sarcoidosis.Considerable expertise is required to identify, diagnose, and manage CTD-PH, as the primary physicians providing the majority of care for this population, rheumatologists, need a good working knowledge of CTD-PH, its rather subtle presentation, and how to access the necessary investigations to screen for and identify patients with PH. The role of the rheumatologist does not stop at diagnosis; in some conditions such as lupus, optimizing immunosuppression is key to the management of PH, and unlike simple idiopathic PAH, the natural history of CTD-PH is often punctuated by complications of the CTD rather than just events due to progression of PH or therapy-related adverse events.The aim of this article is to provide an overview of all forms of CTD-PH, and to provide an easy reference source on current best practice.

Viabahn stent graft in the management of a grade 3 coronary perforation.

BACKGROUND: Coronary artery perforation during coronary intervention has high morbidity and mortality. This case describes the collaboration between interventional cardiologists and Interventional radiologists to successfully deploy a peripheral arterial stent graft in a coronary artery that demonstrated persistent extravasation after coronary specific stent graft placement. CASE PRESENTATION: An 84 year old female patient presented with acute coronary syndrome and coronary angiography identified a right coronary artery lesion. This was dilated and stented but resulted in a grade 3 coronary perforation. Conservative treatment with balloon tamponade failed, as did placement of a covered coronary-specific stent graft. A Viabahn peripheral arterial stent graft was placed within the indwelling stents and successfully sealed the endoleak. At 6 months the patient is clinically well and follow-up imaging has demonstrated stent patency. CONCLUSIONS: In the emergency setting when coronary artery perforation fails to respond to standard initial and bail out techniques, peripheral arterial techniques and devices can be extremely useful. A good relationship between interventional cardiology and radiology is paramount.

Cardiac Effects of Lightning Strikes.

Lightning strikes are a common and leading cause of morbidity and mortality. Multiple organ systems can be involved, though the effects of the electrical current on the cardiovascular system are one of the main modes leading to cardiorespiratory arrest in these patients. Cardiac effects of lightning strikes can be transient or persistent, and include benign or life-threatening arrhythmias, inappropriate therapies from cardiac implantable electronic devices, cardiac ischaemia, myocardial contusion, pericardial disease, aortic injury, as well as cardiomyopathy with associated ventricular failure. Prolonged resuscitation can lead to favourable outcomes especially in young and previously healthy victims.

Prevalence and outcomes of coronary artery perforation during percutaneous coronary intervention.

AIMS: We aimed to examine the prevalence, clinical outcomes and procedural characteristics of percutaneous coronary intervention (PCI) complicated by coronary artery perforation (CAP) in a contemporary patient population. METHODS AND RESULTS: Procedural records of 39,115 patients undergoing PCI between 2005 and 2016 were reviewed. CAP affected 149 cases (0.37%). The prevalence of CAP increased from 0.31% in 2005 to 0.45% in 2016 (p=0.03), reflecting an increase in more complex PCI (from 14% in 2005 to 21% in 2016; p<0.0001). CAP was associated with increased all-cause mortality (23.1% vs. 9.4% in those without perforation; p=0.0054) and was an independent predictor of mortality (HR 2.55; 95% CI: 1.34-4.78). In-patient mortality was 4% (6/149). In 43 of 149 (28.9%) cases, a significant pericardial effusion ensued and mortality rates were higher in this subgroup. Thirty-one patients had covered stents (CS) inserted and five did not survive to discharge. Of the 26 patients with a CS who survived to hospital discharge, six (23.1%) had definite stent thrombosis, and two (7.7%) had possible/probable stent thrombosis. CONCLUSIONS: CAP remains uncommon but the prevalence is increasing. CAP is associated with significant short- and long-term mortality, particularly when there is haemodynamic compromise necessitating pericardiocentesis. Covered stents are a valuable tool but they are associated with a high risk of stent thrombosis.

Chronic thromboembolic pulmonary arterial hypertension: a review of the literature and novel therapeutic approaches.

Chronic thromboembolic pulmonary hypertension is defined as pulmonary hypertension (PH) caused by single or recurrent pulmonary emboli and is characterized by chronic obstruction of the pulmonary arteries leading to increased vascular resistance and PH. Also, progressive remodeling may occur in occluded and nonoccluded territories. Better understanding of the underlying mechanisms and risk factors could improve diagnosis and allow appropriate interventions. Pulmonary endarterectomy is an established approach and is considered the definitive treatment for chronic PH, resulting from thromboembolic disease. Furthermore, percutaneous transluminal pulmonary angioplasty is technically feasible, especially for those with peripheral-type of the disease. In addition, several agents, including prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors, have been tested in selected patients yielding promising results. Several novel agents are under investigation, and extensive research is currently in progress aiming to resolve uncertainties in the understanding and treatment of the disease.

Simultaneous bilateral spontaneous hydropneumothorax: a rare presentation of bilateral malignant pleural mesothelioma.

This is a case of a 69-year-old man with a history of asbestos exposure who presented with acute shortness of breath. His chest x-ray showed bilateral hydropneumothorax. Further investigations including CT chest and video-assisted thoracoscopic surgery revealed bilateral pleural thickening and histology confirmed epithelioid mesothelioma. This case highlights the need for clinicians to be aware of atypical presentations of malignant pleural mesothelioma as well as the importance of considering underlying secondary causes such as malignancy in the older patient presenting with spontaneous pneumo/hydropneumothorax.