25 August 2020

LIVE UPDATES from around the world: Pulmonary hypertension and COVID-19

This page is being updated regularly with the latest information on the COVID-19 pandemic with a relation to our clinics across the world. Details of how you are able to contribute to this page are found at the bottom of the article.

Webinars

WHF Webinar: Beyond Doom and Gloom: The future of CVD & NCD management in the current and post COVID-19 era

Posted 9 September 2020

The World Heart Federation (WHF) is inviting you to join their upcoming webinar exploring how the future of CVD & NCD management in the current and post COVID-19 era.

"Since the start of the COVID-19 pandemic, we have been hearing stories about overstretched health systems and learning about the increased vulnerability of those living with cardiovascular and other circulatory diseases. At the same time, the pandemic has also led to increased interest in innovative care and prevention models, as well as recognition for frontline health workers and those working to save the lives of patients with chronic diseases.

The urgent need to address both shortages in the health workforce and limitations in access to care for circulatory diseases is becoming increasingly evident. Join us in 8 October for the inaugural webinar of Heart Talks: A Series of the 5th Global Summit on Circulatory Health, which will explore the future of circulatory health management following one of the most tumultuous years for global health in recent history. The conversation will bring together speakers from international organizations, academia and civil society, as well as the voices of health workers and patients on the ground, to address both present challenges and future opportunities for circulatory health."

Join the last of the WHF webinar series

Posted 13 July 2020

The World Heart Federation (WHF) is inviting you to join their upcoming webinar series, which will be held on 16 & 30 June, and 14 July 2020.

Presented by the Global Coalition for Circulatory Health (GCCH), which brings together 32 international, regional and national stakeholders in circulatory health to drive the urgent action needed to combat heart disease and stroke, the webinar topics will be as follows:

16 June // Circulatory risk factors for Covid-19
30 June // Covid-19 and circulatory conditions: mitigating poor outcomes
14 July // Supporting the healthcare workforce: Covid-19 and public health in the circulatory space.

NCDs & COVID-19: Learning lessons, building back better for the future

Posted 2 July 2020

WHO will join the Norwegian Ministry of Foreign Affairs and NCD Alliance in hosting a virtual event on July 13 called 'NCDs & COVID-19: Learning lessons, building back better for the future'.

In his address to the 73rd World Health Assembly on 18 May 2020, WHO Director-General Dr Tedros Adhanom Ghebreyesus said “COVID-19 is not just a global health emergency, it is a vivid demonstration of the fact that there is no health security without resilient health systems, or without addressing the social, economic, commercial and environmental determinants of health.”

This virtual high-level event will convene global thought leaders to:

  • Explore the collision of NCDs and COVID-19 to demonstrate the need to prioritise NCDs in policy responses to health emergencies and humanitarian responses;
  • Position investment in NCD prevention and treatment as an essential foundation for human security and preparedness for health emergencies;
  • Inform ‘build back better’ agenda for resilience, recovery and rebuilding, including the opportunity of Universal Health Coverage (UHC) and health systems strengthening to break down silos across global health, at global and national levels and catalysing a holistic approach to health and the Sustainable Development Goals (SDGs).

WHO webinar series: NCD Voices in the decade of action

Posted 19 June 2020

The World Health Organization has launched a new webinar series called NCD Voices in the Decade of Action.

The first took place on 18 June 2020 and was entitled:  The value of linking COVID-19 and NCDs to 'build back better'. 

Articles

Sex-derived attributes contributing to SARS-CoV-2 mortality

Posted 6 August 2020

Neha Chanana, Tsering Palmo, Kavita Sharma, Rahul Kumar, Brian B. Graham, and Qadar Pasha

Abstract

Epidemiological data in COVID-19 mortality indicate that men are more prone to die of SARS-CoV2 infection than women, but biologic causes for this sexual dimorphism are unknown. We discuss the prospective behavioral and biological differences between the sexes that could be attributed to this gender-based differentiation. The female sex hormones and the immune stimulatory genes including toll-like receptors, interleukins, micro-RNAs present on X-chromosome may impart lesser infectivity and mortality of the SARS-CoV-2 in females over males. The sex hormone estrogen interacts with the Renin-Angiotensin-Aldosterone System, one of the most critical pathways in COVID-19 infectivity, and modulate the vasomotor homeostasis. Testosterone on the contrary enhances the levels of the two most critical molecules angiotensin converting enzyme 2 (ACE2) and the transmembrane protease, serine-type 2 (TMPRSS2), transcriptionally and post-translationally, thereby increasing viral load and delaying viral clearance in men as compared to women. We propose that modulating sex hormones, either by increasing estrogen or anti-androgen, may be a therapeutic option to reduce mortality from SARS-CoV-2.

Management of Cardiovascular Disease Patients With Confirmed or Suspected COVID-19 in Limited Resource Settings

Posted 29 July 2020

Dorairaj Prabhakaran , Pablo Perel, Ambuj Roy, Kavita Singh, Lana Raspail, José Rocha Faria-Neto, Samuel S. Gidding, Dike Ojji, Ferdous Hakim, L. Kristin Newby, Janina Stępińska, Carolyn S.P. Lam, Modou Jobe, Sarah Kraus, Eduardo Chuquiure-Valenzuela, Daniel Piñeiro, Kay-Tee Khaw, Ehete Bahiru, Amitava Banerjee, Jagat Narula, Karen Sliwa

Abstract

In this paper, we provide recommendations on the management of cardiovascular disease (CVD) among patients with confirmed or suspected coronavirus disease (COVID-19) to facilitate the decision making of healthcare professionals in low resource settings. The emergence of novel coronavirus disease, also known as Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2), has presented an unprecedented global challenge for the healthcare community. The ability of SARS-CoV-2 to get transmitted during the asymptomatic phase and its high infectivity have led to the rapid transmission of COVID-19 beyond geographic regions, leading to a pandemic. There is concern that COVID-19 is cardiotropic, and it interacts with the cardiovascular system on multiple levels. Individuals with established CVD are more susceptible to severe COVID-19. Through a consensus approach involving an international group this WHF statement summarizes the links between cardiovascular disease and COVID-19 and present some practical recommendations for the management of hypertension and diabetes, acute coronary syndrome, heart failure, rheumatic heart disease, Chagas disease, and myocardial injury for patients with COVID-19 in low-resource settings. This document is not a clinical guideline and it is not intended to replace national clinical guidelines or recommendations. Given the rapidly growing burden posed by COVID-19 illness and the associated severe prognostic implication of CVD involvement, further research is required to understand the potential mechanisms linking COVID-19 and CVD, clinical presentation, and outcomes of various cardiovascular manifestations in COVID-19 patients.

 

Pulmonary vascular resistance and clinical outcomes in patients with pulmonary hypertension: a retrospective cohort study

Posted 28 July 2020

Bradley A Maron MD, Evan L Brittan MD, Edward Hess MS, Stephen W Waldo MD, Prof Anna E Barón PhD, Shi Huang PhD

Abstract

In pulmonary hypertension subgroups, elevated pulmonary vascular resistance (PVR) of 3·0 Wood units or more is associated with poor prognosis. However, the spectrum of PVR risk in pulmonary hypertension is not known. To address this area of uncertainty, we aimed to analyse the relationship between PVR and adverse clinical outcomes in pulmonary hypertension.

In pulmonary hypertension subgroups, elevated pulmonary vascular resistance (PVR) of 3·0 Wood units or more is associated with poor prognosis. However, the spectrum of PVR risk in pulmonary hypertension is not known. To address this area of uncertainty, we aimed to analyse the relationship between PVR and adverse clinical outcomes in pulmonary hypertension.

We did a retrospective cohort study of all patients undergoing right heart catheterisation (RHC) in the US Veterans Affairs health-care system (Oct 1, 2007–Sep 30, 2016). Patients were included in the analyses if data from a complete RHC and at least 1 year of follow-up were available. Both inpatients and outpatients were included, but individuals with missing mean pulmonary artery pressure (mPAP), pulmonary artery wedge pressure, or cardiac output were excluded. The primary outcome measure was time to all-cause mortality assessed by the Veteran Affairs vital status file. Cox proportional hazards models were used to assess the association between PVR and outcomes, and the mortality hazard ratio was validated in a RHC cohort from Vanderbilt University Medical Center (Sept 24, 1998–June 1, 2016).

Impact of SARS-CoV-2 pandemic on pulmonary hypertension out-patient clinics in Germany: a multi-centre study

Posted 28 July 2020

Athiththan Yogeswaran, Henning Gall, Khodr Tello, Ekkehard Grünig, Panagiota Xanthouli, Ralf Ewert, Jan C. Kamp, Karen M. Olsson, Max Wißmüller, Stephan Rosenkranz, Hans Klose, Lars Harbaum, Tobias J. Lange, Christian F. Opitz, Andrea Waelde, Katrin Milger, Natascha Sommer, Werner Seeger, Hossein Ardeschir Ghofrani, Manuel J. Richter

Abstract

Pulmonary hypertension is frequently under-diagnosed, and referral is delayed with subsequent impact on outcomes. During the SARS-CoV-2 pandemic, restrictions on daily life and changes in hospitals' daily routine care were introduced in Germany. This multi-centre study provides evidence for a negative influence of these restrictions on patient care in pulmonary hypertension expert referral centres.

Management of hospitalized patients with pulmonary arterial hypertension and COVID-19 infection

Posted 20 July 2020

Sandeep Sahay, Harrison W. Farber

Abstract

The novel coronavirus SARS-CoV2 that causes coronavirus disease 2019 (COVID-19), has approximately afflicted over 2 million people worldwide, including approximately a million cases with over 50,000 deaths in the United States as of 27 April 2020. Pulmonary arterial hypertension (PAH), a chronic, progressively fatal condition, requires complicated medical management. Patients with PAH are very sensitive to the changes in their cardiopulmonary status and any disruption in treatment or development of additional cardiac or pulmonary pathology can trigger a rapid course of decline leading to death. Furthermore, all-cause hospitalization itself is a risk factor for disease progression in PAH. A recently published small case series suggested that 86% of critically ill COVID-19-infected patients had heart failure and chronic kidney disease as the most common underlying medical conditions. These conditions are commonly seen in patients with PAH and increase the risk for PAH patients to develop severe disease. Many PAH patients are on complicated regimens, including oral, inhaled or continuous intravenous therapies, some of which may be difficult to deliver during an acute illness. In this brief communication, we will highlight the in-patient management of COVID-19-infected PAH patients.

COVID-19 and the pulmonary vasculature

Posted 20 July 2020

Steeve Provencher, François Potus, Sébastien Bonnet

Abstract

Patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-related disease (COVID-19) present in a number of ways, from asymptomatic carriers to respiratory failure with acute respiratory distress syndrome (ARDS)-like features. Early observational studies documented that virtually all hospitalized patients had parenchymal abnormalities on computed chest tomography. Interestingly, vascular thickening was also shown to be a predominant imaging finding in COVID-19 compared to non-COVID-19 pneumonia (observed in 59% vs. 22%, p < 0.001), implying a potential tropism of the virus for the pulmonary vasculature. This is not surprising since its functional receptor from the host cells, the angiotensin-converting enzyme 2 receptor, is largely present on the surface of pulmonary vascular cells. Accordingly, diffuse endothelial inflammation, dysfunction and apoptosis resulting from direct viral infection of the endothelial cells have been reported within the lungs and other organs. Several lines of evidence also suggest that COVID-19 impacts the pulmonary circulation clinically. For example, patients with severe COVID-19 commonly present an atypical form of ARDS with significant dissociation between relatively well-preserved lung mechanics and severe hypoxemia for which the loss of hypoxic vasoconstriction lung perfusion regulation has been proposed as a possible explanation.

Rethinking COVID-19 ‘pneumonia’ – is this primarily a vaso-occlusive disease, and can early anticoagulation save the ventilator famine?

Posted 20 July 2020

Ting-Ting Low, Robin Cherian, Shir Lynn Lim, Bharatendu Chandra, Moon Ley Tung, Shoban Krishna Kumar, Priscillia Lye, Amanda Chin Xin Yi, Lynette Teo, Edgar Lik-Wui Tay

Abstract

As the COVID-19 pandemic rampages around the globe, it remains an enigma as to how a fraction of those infected can turn critically ill with severe hypoxemia – the scale of this problem so massive that hospitals in seriously affected cities see their ventilator capacity overwhelmed. As of 25 April 2020, there are approximately 2.6 million infections with more than 180,000 deaths worldwide. The main driver of mortality and morbidity with COVID-19 has been the acute respiratory syndrome that occurs in 12–32% of patients24 after the initial upper respiratory tract symptoms. While it is widely suspected that an abnormal host response such as a ‘cytokine storm’ is the driving force in those precariously ill,5 there are peculiarities in radiological findings and ventilator mechanics that are atypical of the usual viral pneumonia and acute respiratory distress syndrome (ARDS). Increasingly, published data and anecdotal observations indicate that the pathogenesis may lie primarily in the pulmonary vasculature with the newly observed tendency for thrombi formation.

In the eye of the storm: the right ventricle in COVID-19

Posted 20 July 2020

John F. Park, Somanshu Banerjee, Soban Umar

Abstract

The corona virus disease of 2019 pandemic caused by the SARS-CoV-2 virus continues to inflict significant morbidity and mortality around the globe. A variety of cardiovascular presentations of SARS-CoV-2 infection have been described so far. However, the impact of SARS-CoV-2 on the right ventricle is largely unknown. Due to its pathophysiologic relevance, the right ventricle finds itself in the eye of the storm of corona virus disease of 2019, placing it at higher risk of failure. Increased afterload from acute respiratory distress syndrome and pulmonary embolism, negative inotropic effects of cytokines, and direct angiotensin converting enzyme 2-mediated cardiac injury from SARS-CoV-2 are potential mechanisms of right ventricle dysfunction in corona virus disease of 2019. Early detection and treatment of right ventricle dysfunction may lead to decreased mortality and improved patient outcomes in corona virus disease of 2019.

Management of Cardiovascular Disease Patients With Confirmed or Suspected COVID-19 in Limited Resource Settings

Posted 17 July 2020

Dorairaj Prabhakaran , Pablo Perel, Ambuj Roy, Kavita Singh, Lana Raspail, José Rocha Faria-Neto, Samuel S. Gidding, Dike Ojji, Ferdous Hakim, L. Kristin Newby, Janina Stępińska, Carolyn S.P. Lam, Modou Jobe, Sarah Kraus, Eduardo Chuquiure-Valenzuela, Daniel Piñeiro, Kay-Tee Khaw, Ehete Bahiru, Amitava Banerjee, Jagat Narula, Karen Sliwa

Abstract

In this paper, we provide recommendations on the management of cardiovascular disease (CVD) among patients with confirmed or suspected coronavirus disease (COVID-19) to facilitate the decision making of healthcare professionals in low resource settings. The emergence of novel coronavirus disease, also known as Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2), has presented an unprecedented global challenge for the healthcare community. The ability of SARS-CoV-2 to get transmitted during the asymptomatic phase and its high infectivity have led to the rapid transmission of COVID-19 beyond geographic regions, leading to a pandemic. There is concern that COVID-19 is cardiotropic, and it interacts with the cardiovascular system on multiple levels. Individuals with established CVD are more susceptible to severe COVID-19. Through a consensus approach involving an international group this WHF statement summarizes the links between cardiovascular disease and COVID-19 and present some practical recommendations for the management of hypertension and diabetes, acute coronary syndrome, heart failure, rheumatic heart disease, Chagas disease, and myocardial injury for patients with COVID-19 in low-resource settings. This document is not a clinical guideline and it is not intended to replace national clinical guidelines or recommendations. Given the rapidly growing burden posed by COVID-19 illness and the associated severe prognostic implication of CVD involvement, further research is required to understand the potential mechanisms linking COVID-19 and CVD, clinical presentation, and outcomes of various cardiovascular manifestations in COVID-19 patients.

Outpatient inhaled nitric oxide in a patient with vasoreactive idiopathic pulmonary arterial hypertension and COVID-19 infection

Posted 17 July 2020

Roham T. Zamanian, Charles V. Pollack, Jr., Michael A. Gentile, Moira Rashid, John Christian Fox, Kenneth W. Mahaffey, Vinicio de Jesus Perez, Vera Moulton Wall

Abstract

Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the cause of coronavirus disease COVID-19), is associated with significant pulmonary morbidity and acute respiratory distress syndrome (ARDS)-like illness. The
unprecedented global COVID-19 pandemic is impacting the wellbeing of vulnerable patients, particularly the elderly and those with underlying cardiopulmonary diseases (2). Because no specific antiviral therapy is currently approved for COVID-19, treatment is supportive (at times intensive) and has severely stretched global hospital staffing and equipment capacity. Here, we report on outpatient management of a patient with concomitant idiopathic pulmonary arterial hypertension (iPAH) and COVID-19 using inhaled nitric oxide (iNO).

The course of COVID-19 in a 55-year-old patient diagnosed with severe idiopathic pulmonary arterial hypertension

Posted 17 July 2020

Diana Mandler, Mona Lichtblau, Silvia Ulrich

Abstract

A 55-year old never-smoking woman was diagnosed with severe idiopathic pulmonaryarterial hypertension by right heart catheterization in fall 2019 with the following hemodynamic measurments: mean pulmonary artery pressure 71 mmHg, pulmonary artery wedge pressure 13 mmHg, cardiac index 1.4 l/min/m2, pulmonary vascular resistance 30 WU. The diagnosis was suspected due to progressive dyspnea for several years and echocardiography, which revealed severe right heart failure with a trans-tricuspid pressure gradient of 100 mmHg, enlarged right sided chambers, D-shaping of the left ventricle, normal ejection fraction and normal size of the left atrium. Chronic thromboembolic diseases was excluded by CT-pulmonary angiography. The patient was in dyspnea NYHA III, her 6-minute walk distance was 465m and the NT-proBNP was 4700 ng/l. The patient was treated for mild hypertension with an angiotensin-receptor blocker and with thyroid replacement therapy for hypothyreosis, and had no other comorbidities. The patient declined continuous intravenous prostaglandin therapy and thus a combination of macitentan (10mg daily) and tadalafil (20mg daily, increased to 40mg daily after 14 days) combined with diuretic therapy was initiated.The patient herewith improved subjectively to NYHA II, the 6-minute walk distance increased to 570 m and antihypertensive therapy was stopped in regard of low-normal blood pressure.

The patient went to visit her family in Guatemala over Christmas time. At the end of January 2020, she felt well in dyspnea NYHA I, the NT-pro BNP decreased to 421 ng/l, the transtricuspid-pressure gradient to 35 mmHg, ergospirometry revealed a peak oxygen uptake of 14.3 ml/min/kg (57% predicted) and the patient was able to go back to work.

On the 3rd of March 2020 the patient called our pulmonary hypertension unit due to symptoms of a common cold, stuffy nose, muscle- and headache, slightly elevated body temperature of 37.9°C and general weakness. She denied cough and resting dyspnea. Symptomatic therapy was recommended at that time.

On the 11th of March 2020 the patient called again still suffering from rhinitis, reduced general health state and muscle aches. Upon request, she told that she suffered from dyspnea and anxiety mainly during the night, but no fever. As Sars-CoV-2 infections started to be diagnosed in Switzerland at that time, we asked the patient to be brought to the
entrance of the outpatient clinic, where she was expected by a fully protected doctor, who immediately provided her with a protective surgical mask and disinfectant for the hands. 

Medical history revealed that her symptoms started a day after her weekly visits to the church the 1st of March and that around 30 other persons that regularly visit her church suffered from symptoms of common cold, some of which were diagnosed with SARS-CoV-2. The patient confirmed that she had followed home quarantine together with her husband,
who similarly suffered from symptoms of a common cold last week. The patient’s symptoms slightly improved over the last days, but in addition to the nightly dyspnea attacks with anxiety, she also had sore throat and loss of appetite, but neither suffered from cough nor fever.

Outpatient inhaled nitric oxide in a patient with vasoreactive IPAH and COVID-19 infection

Posted 3 June 2020

Roham T. Zamanian, Charles V. Pollack, Jr., Michael A. Gentile, Moira Rashid, Kenneth W. Mahaffey, Vinicio De Jesus Perez

Abtract

Infection with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), the cause of respiratory illness COVID-19, is associated with significant pulmonary morbidity and acute respiratory distress syndrome (ARDS)-like illness(1). The unprecedented global COVID-19 pandemic is impacting the wellbeing of vulnerable patients, particularly the elderly and those with underlying cardiopulmonary diseases(2). As no specific anti-viral therapy is currently approved for COVID-19, treatment is supportive (at times intensive) and has severely stretched global hospital staffing and equipment capacity. Here we report on an outpatient management of a patient with concomitant idiopathic pulmonary
arterial hypertension (iPAH) and COVID-19 disease using inhaled nitric oxide (iNO).

COVID-19 lung injury is different from high altitude pulmonary edema

Posted 3 June 2020

Hermann Brugger,Buddha Basnyat, John Ellerton, Urs Hefti,Giacomo Strapazzon,and Ken Zafren

Abstract

We read with interest the recent article by Luks et al.(2020). Recently, emergency physicians (Solaimanzadeh, 2020; Sidell, 2020) have suggested that there are pathophysiological similarities between coronavirus disease 2019 (COVID-19) pneumonia and high altitude pulmonary edema (HAPE). They have suggested that drugs known to be effective in patients suffering from acute mountain sickness or HAPE, such as acetazolamide, nifedipine, and phosphodiesterase inhibitors, might be useful in the treatment of COVID19. In this journal, a group of high altitude researchers has
debunked this myth and expressed concerns that this misconception may adversely affect the management of COVID-19 patients (Luks et al., 2020). We members of the International Society of Mountain Medicine (ISMM), International Commission for Alpine Rescue Medical Commission (ICAR MedCom), and Medical Commission of the International Climbing and Mountaineering Federation (UIAA) support these concerns and echo their warning.

COVID-19 and pulmonary hypertension

Posted 3 June 2020

Samar Farha

Abstract

Pulmonary hypertension (PH) is a pulmonary vascular disease characterized by pulmonary arterial remodeling and vasoconstriction leading to elevated pulmonary artery pressure and, ultimately, right heart failure. So far, few cases of COVID-19 disease in patients with PH have been reported. Caution is warranted in interpreting this observation as data are evolving and several factors may influence the number of reported cases of PH and COVID-19. Social distancing and quarantine could play a role, especially for patients with chronic diseases who might be more vigilant of their potential for respiratory infection. In addition, PH is a rare disease, and because testing is not universal, we could be underestimating the number of cases. Other hypothetical factors to consider are the underlying pathophysiology of PH and the medications used to treat PH and their implications in COVID-19.

Immune mechanisms of pulmonary intravascular coagulopathy in COVID-19 pneumonia

Posted 21 May 2020

Dennis McGonagle, James S O’Donnell, Kassem Sharif, Paul Emery, Charles Bridgewood

Abstract

The lung pathology seen in patients with coronavirus disease 2019 (COVID-19) shows marked microvascular thrombosis and haemorrhage linked to extensive alveolar and interstitial inflammation that shares features with macrophage activation syndrome (MAS). We have termed the lung-restricted vascular immunopathology associated with COVID-19 as diffuse pulmonary intravascular coagulopathy, which in its early stages is distinct from disseminated intravascular coagulation. Increased circulating D-dimer concentrations (reflecting pulmonary vascular bed thrombosis with fibrinolysis) and elevated cardiac enzyme concentrations (reflecting emergent ventricular stress induced by pulmonary hypertension) in the face of normal fibrinogen and platelet levels are key early features of severe pulmonary intravascular coagulopathy related to COVID-19. Extensive immunothrombosis over a wide pulmonary vascular territory without confirmation of COVID-19 viraemia in early disease best explains the adverse impact of male sex, hypertension, obesity, and diabetes on the prognosis of patients with COVID-19. The immune mechanism underlying diffuse alveolar and pulmonary interstitial inflammation in COVID-19 involves a MAS-like state that triggers extensive immunothrombosis, which might unmask subclinical cardiovascular disease and is distinct from the MAS and disseminated intravascular coagulation that is more familiar to rheumatologists.

Differentiating COVID-19 Pneumonia from Acute Respiratory Distress Syndrome (ARDS) and High Altitude Pulmonary Edema (HAPE): Therapeutic Implications

Posted 21 May 2020

Running Title: Archer et al.; Impaired Oxygen Sensing in COVID-19 Pneumonia 

Abstract

COVID-19 is an acute respiratory illness caused by a droplet-borne coronavirus, SARSCoV-2. By May1st 2020 the pandemic had resulted in ~3.3 million infections, over 235,000 deaths and global disruption of trade. While 80% of people with COVID-19 suffer a minor, acute respiratory infection, the mortality ranges from 2-7%. Patients with COVID-19 pneumonia may decompensate due to hypoxemic respiratory failure. Autopsy data show inflammation, diffuse alveolar damage (DAD), alveolar fluid accumulation, and occasional hyaline membranes, consistent with acute respiratory distress syndrome (ARDS). Understanding the causes of hypoxemia in COVID-19 is complicated by a paucity of hemodynamic and autopsy data; however the presentation of COVID-19 patients is atypical of ARDS in that the hypoxemia is often profound without appropriate dyspnea, occurs despite relatively preserved lung compliance and is associated with a large intrapulmonary shunt. These traits suggest a failure of the body’s homeostatic O2-sensing system (HOSS), which includes the pulmonary circulation, carotid body, adrenomedullary cells, and neuroepithelial bodies. The HOSS optimizes oxygen uptake and systemic oxygen delivery. Hypoxic pulmonary vasoconstriction (HPV) is the pulmonary circulation’s homeostatic response to airway hypoxia in lung diseases, such as pneumonia. HPV constricts pulmonary arteries (PA) serving hypoxic lung segments, diverting blood to better ventilated alveoli, optimizing ventilation/perfusion (V/Q) matching. The carotid body senses hypoxemia, increasing respiratory drive. COVID-19 hypoxemia is variably attributed to ARDS, impaired HPV and a high altitude pulmonary edema (HAPE)-physiology (Figure 1). We propose that the best explanation is profound impairment of HPV and carotid body function, sometimes combined with virally-induced, ARDS.

A short report on health, patients and well-being

Posted 21 May 2020

Michael A. Gatzoulis in London

Abstract

Cycling to work in London now feels surreal: very few people about, a metropolis stripped of its many attractions (museums, restaurants, shops, etc.), but buildings and other material objects remain intact. It is only people who are affected. Uncertainty looms large on relaxing the draconian measures taken to slow down the spread of the disease, so we can return to some ‘normality’. Last, but not least, legitimate concerns are about the economic and psychosocial implications of the pandemic, and the consequent disruption of the societal fabric, as we know it.

And yet, there is no doubt that we will weather this, as man did weather virus pandemics and other global challenges in our long history. There is always opportunity with crises. One hopes that at the other end of this storm we will be better people, more humane, considerate, together, and appreciative of healthcare and of science, and of all other support services essential to a smooth running of a society.

This short communication is of course about health, patients, and well-being. While the frenzied efforts to combat the COVID-19 pandemic are understandable, we must not forget our primary obligation to look after our patients; their needs should not be neglected. This includes patients with life-long diseases, such as congenital heart disease (CHD), emergencies such as acute coronary syndromes, patients with suspected or newly diagnosed cancer, and many more.

Understanding the current status of patients with pulmonary hypertension during COVID-19 outbreak: a small-scale national survey from China

Posted 18 May 2020

Hongmei Zhou, Gangcheng Zhang, Xiaoxian Deng; Bowen Jin; Qiu Qiu; Menghuan Yan; Xi Wang and Xuan Zheng

Abstract

Pulmonary hypertension is a chronic disease developing progressively with high mortality. Pulmonary hypertension patients need persistent medical care; however, limited reports focused on them when there was an outbreak of coronavirus disease 2019 in China. This national survey was aimed to evaluate the overall condition of pulmonary hypertension patients during this period. A questionnaire regarding the living condition of pulmonary hypertension patients during coronavirus disease 2019 was designed by pulmonary hypertension diagnostic experts in Wuhan Asia Heart Hospital. Pulmonary hypertension patients and their family members were invited to participate in this survey online. One-hundred twenty pulmonary hypertension patients and 23 family members participated in the survey; 64.8% (n = 87) participants came from Hubei, and others were from 15 other provinces; 98.6% (n = 141) participants were in home quarantine; 65.8% (n = 79) were pulmonary arterial hypertension associated with congenital heart disease; and 76.7% (n = 92) patients proclaimed their heart function was well maintained at class I or II. One (0.8%) patient was confirmed severe acute respiratory syndrome coronavirus 2 infection. Two (1.7%) patients were hospitalized due to heart function worsening. Nearly 70% (n = 100) participants implied shortage in medications during coronavirus disease 2019 outbreak. A total of 24.2% (n = 29) patients indicated that medications were discontinued due to the insufficient supply. Most of the participants stayed optimistic on either coronavirus disease 2019 outbreak or their pulmonary hypertension disease, and 61.7% (n = 74) patients would go to the hospital for follow-up immediately after outbreak. These preliminary data show pulmonary hypertension patients are able to avoid severe disease when they are in home quarantine. Medication supplement is important for pulmonary hypertension patients when their heart function is well maintained. In addition, there might be increasing requirements of medical care for pulmonary hypertension patients after the outbreak

Could pulmonary arterial hypertension patients be at a lower risk from severe COVID-19?

Posted 28 April 2020

Evelyn M. Horn, Murali Chakinala, Ronald Oudiz, Elizabeth Joseloff, Erika B. Rosenzweig

Abstract

The COVID-19 pandemic now impacts over 1.2 million individuals worldwide with higher risk comorbidities including age, cardiac and pulmonary diseases. Pulmonary hypertension (PH) centers prepared for the worst for their high-risk pulmonary arterial hypertension (PAH) patients. However, providers have been surprised thus far by the paucity of hospitalized PAH–COVID-19 patients, generally tolerable symptoms in those affected, and their relatively early recovery.

NORD Opens COVID-19 Financial Aid Program for Rare Disease Community

Posted 27 April 2020

Mary Chapman 

Abstract

The National Organization for Rare Disorders (NORD) has opened a financial assistance program for people in rare disease community who are affected by the COVID-19 pandemic in the USA.

Called the NORD COVID-19 Critical Relief Program, the effort will provide up to $1,000 annually to those eligible to support critical, non-medical needs. The program is designed to help rare disease patients and their families who may be facing monetary hardships due to the outbreak.

“Providing financial assistance to help meet the unique needs of the rare disease community has been central to our mission for over 37 years,” said Jill Pollander, NORD’s director of patient services, in a press release. “The NORD COVID-19 Critical Relief Program enables us to provide desperately needed support to rare disease community members whose lives have been directly impacted by the current pandemic.”

Covid-19 general resources

NCDA’s new Solidarity Fund will support 20 NCD alliances to strengthen civil society’s response to COVID-19

Posted 13 July 2020

On 13 July 2020, the NCD Alliance is launching the first Civil Society Solidarity Fund on NCDs and COVID-19, which will support 20 national and regional NCD alliances in Africa, Asia, Europe, Eastern Mediterranean and Latin America to accelerate the response to the coronavirus pandemic. The fund totalling $300,000 will competitively award grants of up to US$15,000 to these alliances to support them in addressing the critical needs of people living with NCDs during COVID-19 via advocacy and communication activities that will promote stronger organisational stability and resilience. Activities will include advocacy and communication efforts for the continuity of essential NCD health services and inclusion of NCDs in national COVID-19 response and recovery plans and community-led awareness-raising campaigns on the linkages between NCDs and COVID-19.

NCDA Consultation - Implications of COVID-19 on NCDA members & NCD Alliances

Posted 27 April 2020

As the coronavirus pandemic (COVID-19) continues to spread across the world, multilateral agencies and governments are implementing measures to contain the spread and protect the public, health systems and economies. Emerging evidence shows many intersections between COVID-19 and NCDs as people living with NCDs and other chronic conditions are more vulnerable to the virus. Many are also likely to experience obstacles and additional strains in managing their conditions as health systems are buckling under the pressure of COVID-19, combined with the impact of physical distancing and stay-at-home measures.

The NCD Alliance invites you to participate in a short survey to better understand how COVID-19 is impacting their member civil society organisations and NCD alliances, their work, and to understand current needs, challenges and opportunities to more effectively navigate these complex times.

The survey will close on Friday 01 May 2020 at 5pm UK time.

The Johns Hopkins Covid-19 worldwide tracker.

A message from our leadership

Werner Seeger, President 2020-21

Posted 18 June 2020

Dear Friends

How the world has changed since my last President’s blog! When I addressed our membership in March 2020, the COVID-19 pandemic was just starting to take hold in Europe and we were haunted by daily devastating updates from China and Italy. Little did we know then, how fast this virus would spread across the globe, cause so many deaths and change the lives of all of us. Like myself, many of you would have been immersed with frontline care over the past few months. Some of you may have been called back to active service and for others, the routine of going to an office or research lab has completely stopped.

However, we have learnt to adapt. We are beginning to master not only the virus, but the complex digital technologies that have revolutionised our working lives. Daily zoom or Skype meetings are now the norm for most us. Although being skeptical at first, I have changed my opinion on how useful these online tools are and how they have helped us navigate through the past few months, maintaining some stability during very uncertain times.

DDS 2020 Digital

This brings me to introduce our new ‘DDS 2020 Digital’ webinar on 29th June at 15:00 BST. We sadly had to cancel the 7th Annual Drug Discovery & Development Symposium on Pulmonary Hypertension which was planned to take place in Boston, USA, but we will bring this meeting to you in a virtual format. I am grateful to all the presenters who have agreed to record their talks, which will be released from 24-28 June. The live webinar on 29th June, which will feature three keynote presentations from David Badesch, Ardi Ghofrani and Kathryn Hall, promises an event not to be missed. With nearly 200 people registered so far, our moderators Paul Hassoun, Anna Hemnes and Allan Lawrie are looking forward to the stimulating discussions.

For more information, please click here 

PVRI Digital Clinic

Continuing on the theme of ‘virtual learning’, I am delighted that our e-learning course on PVD, The Digital Clinic, has received CME accreditation from the European Accreditation Council for Continued Medical Education (EACCME) and the European Board for Accreditation in Pneumology (EBAP). The twelve patient cases are a great introductory tool for diagnosis and management of the disease and I would encourage you all to take a look. We have also entered discussions with colleagues at the American Heart Association (AHA) to explore potential opportunities.

Find out more, please click here 

IDDI

The work of our Innovative Drug Development Initiative (IDDI) that began during our Drug Symposium in Berlin has resulted in the production of four manuscripts on ‘Endpoints, Biomarkers, Clinical Trials and Repurposing Drugs’. Immense thanks to my friend and colleague Paul Corris for leading the IDDI together with Sylvia Nikkho from Bayer and Peter Fernandes from Bellerophon. All manuscripts are currently in a peer-review process. A big thanks to all authors, Roundtable members and everyone who has been involved in one of the IDDI work streams that has resulted in this great work. Finally, many thanks also to Georgie Sutton from the PVRI office, whose help with the editing has been invaluable.

Athens 2021 Digital

Having proudly announced Athens in Greece as the future location of our Annual World Congress in January 2021 during the Gala Dinner in Lima, it saddens me to inform you that we had to make the cautious decision to change this meeting to a virtual event. However, rest assured that we will deliver the same PVRI quality and focus on live discussions and debates as has become the hallmark for PVRI meetings. All the regular features of a PVRI Annual Congress will be included. To name but a few, there will be plenary sessions with novel keynote talks, live pro/con debates, a joint PVRI-ISHLT Symposium and a joint PVRI-ESC Symposium, as well as moderated abstract poster discussions. Our scientific organisers under the leadership of Stephan Rosenkranz (Cologne, Germany) and Patricia Thistlethwaite (San Diego, USA) have already produced an initial agenda which will soon be published on the PVRI website.

Global Health & GoDeep Registry

Our work on global health is continuing. We have published an article on the prevalence of PH with the American Journal of Physiology and continuing to increase our links with the World Heart Federation (WHF) and Non-Communicable Disease Alliance (NCDA). A recent publication from Brad Maron was included in the NCDA news bulletin in April 2020. Stuart Rich is actively pursuing ongoing communications with the World Health Organization (WHO) for a joint PVRI-WHO meeting in January 2023, which will also address the molecular reclassification of the disease. Martin Wilkins is heading this new Task Force and would be happy to hear from anyone who would like to get involved. Please email admin@pvrinstitute.org stating your interest. Furthermore, we are in discussion with Greg Roth from the Institute for Health Metrics and Education (IHME) in Washington on how the PVRI can become more actively involved in the Global Burden of Disease Study, which for the first time includes pulmonary hypertension.

The GoDeep Registry, which was launched during our Lima Congress in January, has attracted a lot of interest from PH centres and registries around the world. Whilst we cannot currently travel to assess the data at the specific centre locations, the work is ongoing. GoDeep already houses over 10,000 data entries and we are actively seeking participation in this registry from any PH centre. Please visit our website at: https://pvri-godeep-registry.org/web/htmlView/home.html or email me directly werner.seeger@innere.med.uni-giessen.de,if you would like to get involved.

As you can see from my update and the various items of this newsletter, the PVRI has not only adapted, but we are continuing to work hard to progress all our projects and services. As physicians, we know that during a time of crisis, the biggest strength is a dedicated team. For the PVRI, this is the same. Our strength is our united global community! Your participation and membership is vital to us and I thank you all for your ongoing support.

Please stay safe and let me close in the words of a famous British song ‘….we will meet again’.

Werner

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