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A new and evolving health struggle for Heart failure patients: COVID-19

It’s safe to say we are not living in normal times.  This is Heart Failure (HF) in the time of the coronavirus disease-2019 (COVID-19). Patients with COVID-19 and preexisting cardiovascular disease (CVD) are at an increased risk of severe disease and death. Moreover, infection has also been associated with cardiac injury such as acute myocardial infarction (AMI), myocarditis, and stress-induced cardiomyopathy leading to subsequent cardiogenic shock (CS) requiring advanced heart failure therapies. There is a bidirectional relationship between viral upper respiratory traction infection(URI) and worsening HF with an increase in hospital re-admission rate as previously noted with influenza. Patients with HF are especially susceptible to influenza-related complications, including acute decompensated HF and secondary pneumonia. Furthermore, HF is associated with greater in-hospital mortality and adverse clinical outcomes. With around 1 million confirmed COVID-19 cases and counting in the US, one would expect an increase in heart failure admissions. Over the past several weeks as the number of COVID-19 admissions increase, the number of patients admitted with heart failure admissions have been at their lowest, which raises the following question: Where are all the HF patients?

We can speculate that people are terrified at home so they are not showing up to the emergency departments. Patients could be slowly accumulating fluids and getting into a decompensated state. On the other hand, being less active, they could also have been experiencing less symptoms. First it was influenza season now overlapping with a COVID-19 pandemic. It would be expected to see an increased number of HF admissions.  It is suggested that we might be experiencing the calm before the storm when it comes to HF decompensation requiring hospitalization. The alternative is that social distancing is the remedy that we have long been waiting for to help decrease heart failure exacerbation and hospital re-admissions rates.

On one bright note, during a telehealth cardiology visit follow up with a long-term patient with chronic systolic heart failure known to have been admitted several times during the past year secondary to medication non-adherence, who admits that he has been feeling great. He takes all his medications religiously now, including his diuretics. He states that the fact that he stays home, he doesn’t have to worry about going to the bathroom to urinate so often when he gets out of the house, therefore he doesn’t miss any of his diuretic doses. He is also compliant with diet as he doesn’t eat out as often as he is used to. He admits that he stopped going out to fast food places. This is one very small sample. On the other hand, on another telehealth visit, there is a patient with newly diagnosed Non-Ischemic Cardiomyopathy and HF with reduced ejection fraction, who is been followed for up-titration of guideline directed medical therapy. It was a challenge to safely increase the dose of his medications without vital signs and avoiding to have the patient physically get to a laboratory to get blood work done. As of now, no major changes were made in the patient current management. Of note, patient did ask about holding angiotensin-converting enzyme (ACE) inhibitors because of what he heard from another source. Once more, no changes were made to the medical regimen and it was explained that it has been recommended based on different society guidelines and expert consensus report, to continue with ACE inhibitors1.

COVID-19 times are dynamic and medical information is constantly being updated. This is an ongoing discussion as the clinical data comes in. As the pandemic evolves and more telehealth visit under our belts, we will continue to find out more. Although as our health care system is currently fighting the COVID-19; we must brace ourselves for the aftermath whether our patients are dying at home, or slowly decompensating. Only time will tell.  As we are flattening to curve with social distancing, our patients with chronic conditions like HF are waiting at home with so much uncertainties surrounding their current and future medical care. “When life gives you lemon, make lemonade”.

The following suggestions can be useful when taking care of heart failure patients during these unprecedented times. (Figure 1) With COVID-19, we should let our HF patients know although social distancing is essential, they are a higher risk population for a complicated course if infected. It is important to inform them on when to seek medical care, whether it’s to contact a health care provider, call emergency medical services, or go to the emergency department. Although, prevention remains the best medicine. They should take the extra step in precautions and follow the latest recommendations from their local department of public health as we should always remind them of what those recommendations consist of via our telehealth visits.  From a cardiologist stand point, it is important to remain available whether it is via email, pager and/or more frequent telehealth visit if possible.  If they don’t have a scale and/or automatic blood pressure machines, it should be suggested to obtain them along with a thermometer from their local pharmacies. With a phone camera, it is feasible to assess Jugular Venous Distention, pitting edema. In addition, with weight trends, blood pressure and heart rate, clinical decisions could be made.  If available, assessment of data via CardioMEMS can also be very helpful in making medical decisions. Desperate times call for desperate measures.  This is too shall pass. If this is the calm before the storm for our heart failure patients, we should be ready when it hits remembering the sun always shines after a storm.

Figure 1. Heart Failure Care Suggestions During COVID-19

“The views, opinions and positions expressed within this blog are those of the author(s) alone and do not represent those of the American Heart Association. The accuracy, completeness and validity of any statements made within this article are not guaranteed. We accept no liability for any errors, omissions or representations. The copyright of this content belongs to the author and any liability with regards to infringement of intellectual property rights remains with them. The Early Career Voice blog is not intended to provide medical advice or treatment. Only your healthcare provider can provide that. The American Heart Association recommends that you consult your healthcare provider regarding your personal health matters. If you think you are having a heart attack, stroke or another emergency, please call 911 immediately.”

 

 

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Another (Louder) Call to Improve the Care We Provide Heart Failure Patients

I am always taken aback when I recommend a switch to sacubitril/valsartan in a patient with heart failure with reduced ejection fraction (HFrEF) and the response is “my patient feels fine”. This is a common response and certainly not a good enough reason to not optimize guideline directed medical therapy (GDMT) in patients with HFrEF. Optimization of GDMT in HFrEF, known to improve morbidity and mortality (1,2), is dismal. The Change the Management of Patients with Heart Failure (CHAMP-HF) registry included patients in the United States with chronic HFrEF receiving at least one oral medication for management of HF and showed >25% of eligible patients are not prescribed angiotensin converting enzyme inhibitor/angiotensin receptor blocker/angiotensin receptor neprilysin inhibitor, >33% are not prescribed a beta blocker, >50% are not prescribed a mineralocorticoid receptor antagonist. Remarkably, even among those receiving GDMT fewer than 25% are prescribed target doses and only 1% of eligible patients are simultaneously on target doses of all 3 classes of GDMT (3,4).

The mechanisms for suboptimal prescription of GDMT in HFrEF are complex and undertreatment is even more evident among women, minority patient populations, and patients from economically disadvantaged backgrounds, among others. Cost is certainly an issue, especially with more novel HF therapies and co-pay assistance programs are not always available to our most vulnerable patients. There are not enough HF cardiologists to take care of the continuously increasing population of HF patients and therefore, optimization of GDMT needs to be done by general cardiologists and primary care clinicians as well. We should also become creative and use telemedicine to optimize GDMT more efficiently. We do our patients a disservice by not optimizing GDMT that improves HF morbidity and mortality.

And just as optimization of GDMT is not ideal, neither is our evaluation of etiology of HF. Optimization of GDMT and determination of etiology of HF whose management may change disease trajectory should be undertaken in all patients with new-onset HF. This begins with a fundamental understanding of the various etiologies of HF, the laboratory and imaging testing needed, and the best treatment strategy for the underlying etiology discovered- if any (cue, “idiopathic” cardiomyopathy). O’Connor and colleagues’ observational cohort study from the Get With The Guidelines- Heart Failure (GWTG-HF) registry demonstrates the need to improve the testing we perform to exclude coronary artery disease (CAD) as the underlying etiology of new-onset HF.4

Why is this important? Well, of course for treatment, which involves deciding whether medical therapy (aspirin, statins) or revascularization (surgical or percutaneous) is a more optimal strategy. And most important to improve disease trajectory as continued ischemia will lead to worsening HF. O’Connor and colleagues found that the majority of  17,185 patients hospitalized for new-onset HF did not receive testing for CAD either during the hospitalization or in the 90 days before and after, despite data demonstrating that 60% (!!!) of HF patients have concomitant significant CAD.4 And consistent with disparities I mentioned earlier regarding the undertreatment of women with GDMT, men were more likely to be tested for CAD.

Diagnosing and treating CAD provides an opportunity to discuss risk factor modification with patients such as smoking cessation, diabetes control, exercise, healthy diets etc.… to further mitigate future risk. The importance of optimization of GDMT in patients with HFrEF cannot be understated and analogous to this, is the importance of examining the underlying etiology of HF in patients with new-onset HF with preserved, borderline, or reduced EF to improve disease trajectory. Furthermore, inequities in both aspects of the care of HF patients in terms of identification of etiology and optimization of GDMT, must be addressed on a national level. We have plenty of data illustrating suboptimal optimization of GDMT in those with established HFrEF and suboptimal testing for CAD in those with new-onset HF. The next steps are understanding the mechanisms and implementing strategies to improve care. The need for this is critical to reduce morbidity and mortality in all HF patients.

References

  1. Yancy CW, Jessup M, Bozkurt B et al. 2017 ACC/AHA/HFSA Focused Update of the 2013 ACCF/AHA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Failure Society of America. Circulation 2017;137.
  2. Yancy CW, Januzzi JL, Allen LA et al. 2017 ACC Expert Consensus Decision Pathway for Optimization of Heart Failure Treatment: Answers to 10 Pivotal Issues About Heart Failure With Reduced Ejection Fraction. Journal of the American College of Cardiology 2017.
  3. Greene SJ, Butler J, Albert NM et al. Contemporary Utilization and Dosing of Guideline-Directed Medical Therapy for Heart Failure with Reduced Ejection Fraction: From the CHAMP-HF Registry. Journal of the American College of Cardiology 2018.
  4. O’Connor, Kyle D., et al. “Testing for Coronary Artery Disease in Older Patients With New-Onset Heart Failure.” Circulation: Heart Failure, vol. 13, no. 4, 2020, doi:10.1161/circheartfailure.120.006963.

“The views, opinions and positions expressed within this blog are those of the author(s) alone and do not represent those of the American Heart Association. The accuracy, completeness and validity of any statements made within this article are not guaranteed. We accept no liability for any errors, omissions or representations. The copyright of this content belongs to the author and any liability with regards to infringement of intellectual property rights remains with them. The Early Career Voice blog is not intended to provide medical advice or treatment. Only your healthcare provider can provide that. The American Heart Association recommends that you consult your healthcare provider regarding your personal health matters. If you think you are having a heart attack, stroke or another emergency, please call 911 immediately.”

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COVID-19; Patients with Congenital Heart Disease (CHD)

This week, the ACHA (American Congenital Heart Association) hosted a webinar in regards to Coronavirus aka SARS-CoV-2, and the illness it causes, COVID-19, 3,000 attended (view the recording here.) CHD patients, parents of CHD patients and CHD providers had the same question, how does this virus affect this special population? Unfortunately, the data is lacking on coronavirus those with CHD and there is a lot we don’t know.

A recent study1 in China looked at 2143 pediatric patients with COVID-19, the majority (94.1%) were asymptomatic or mild, more severe cases, were seen in infants (<1 year old) than older children, and there was only one death. This study lacks details, such as what other medical conditions they may have. Severe cases were 5.9% compared to 18.5% in adult population studies. Although reassuring for the general pediatric population, we still don’t know how this applies to pediatric patients in the United States and those with CHD.

What we do know.

There is a trend toward overgeneralization of “heart disease,” particularly in the media. It has been noted that the COVID-19 affects older adults and those with “heart disease,” meaning cardiovascular disease(CVD), such as coronary artery disease and hypertension, more severely.2 This does not include Congenital Heart Disease.

The virus may also cause myocardial injury, with reports of myocarditis and arrhythmias in those with severe cases.2-4 The effect is thought to be related to Angiotensin-converting enzyme 2(ACE2), which, in animal studies, has a role in the cardiovascular, and immune system and has been identified as a functional receptor for coronaviruses.2,3

Many patients with CVD and CHD take a medication known as ACE inhibitors or an Angiotensin Receptor Blockers (ARBs). The use of these medications is common in both populations, but for different indications, as their “heart disease” is not the same. There are trials assessing the use of these medications and effect on COVID19 in adults, and varying theories on whether they are protective or not, with that said, the HFSA/ACC/AHA currently recommends continuing these medications as prescribed.5

Are patients with CHD considered high risk?

The answer is we don’t know. With a wide range of congenital heart disease, from repaired/“normal” hearts, to those with altered blood flow, lung abnormalities, and arrhythmias. As  mentioned, the CDC places those with “heart disease,” meaning those with CVD, and older adults, at high risk of severe illness,6 this does not include CHD, however, CHD patients aren’t immune to CVD and if a patient has CVD and also CHD they are considered high risk.

With data lacking in many populations, it is important for those considered at high risk for other viruses, like influenza, such as CHD, asthma and those who are immunocompromised, to take appropriate precautions. It is better to be over prepared and over cautious.

Follow up and Communication.

CHD patients should keep in close contact with their medical team and stay updated with recommendations of their team and the CDC (found in detail here), like social distancing, good hand hygiene and staying home if you are sick. Concerning symptoms that require further evaluation include shortness of breath (or fast breathing in infants), chest pain, and palpitations.

 As far as visiting your doctor, you will likely be asked to either re-schedule or have a telephone visit. You can ask your medical team about this option and even anticipate it for the next few months. Elective procedures, catheterizations and imaging will likely be delayed. If one good thing comes out of this pandemic, it may be better options and availability for telemedicine in the future.

Keep your Mind Healthy

Use this time to support your mental health— pay attention to the news and social media, but set timers so you don’t over-saturate yourself. Find the book you’ve had on your shelves that you’ve been too busy for and set aside time every day to read, call or FaceTime friends, and maybe even fill up your bathtub and relax!

Meditation and exercise are also great options, and many apps offer free trials. Calm and Headspace have some free mediation content and free trials. Peloton & DailyBurn offer free day trials with a variety of classes(Tip: If you do choose a free trial, be sure to set an alarm on your calendar before the free trial is over so you can choose if it’s worth continuing for a fee or not.) There are also options to support your local gyms and studies virtually with on demand classes, just check out their websites and/or Instagram.

There is so much unknown, which causes us to worry and discomfort, but we are learning more each day. Stay informed, stay safe, wash your hands and try to keep your mental health in check.

For more on coronavirus and heart health, read Noora Aljerhi’s blog (3/9/2020) on the early career voice.

  1. Dong, Yuanyuan, et al. “Epidemiological Characteristics of 2143 Pediatric Patients with 2019 Coronavirus Disease in China.” Pediatrics, 2020, doi:10.1542/peds.2020-0702.
  2. Hui, Hui, et al. “Clinical and Radiographic Features of Cardiac Injury in Patients with 2019 Novel Coronavirus Pneumonia.” 2020, doi:10.1101/2020.02.24.20027052.
  3. Zheng, Ying-Ying, et al. “COVID-19 and the Cardiovascular System.” Nature News, Nature Publishing Group, 5 Mar. 2020, nature.com/articles/s41569-020-0360-5?code=85e25438-46d1-4753-bfdd-84496a98b564.
  4. Hu, Hongde, et al. “Coronavirus Fulminant Myocarditis Saved with Glucocorticoid and Human Immunoglobulin.” European Heart Journal, 2020, doi:10.1093/eurheartj/ehaa190.HFS/ACC/AHA statement
  5. “HFSA/ACC/AHA Statement Addresses Concerns Re: Using RAAS Antagonists in COVID-19.” American College of Cardiology, 17 Mar. 2020, acc.org/latest-in-cardiology/articles/2020/03/17/08/59/hfsa-acc-aha-statement-addresses-concerns-re-using-raas-antagonists-in-covid-19.
  6. “If You Are at Higher Risk.” Centers for Disease Control and Prevention, Centers for Disease Control and Prevention, 12 Mar. 2020, www.cdc.gov/coronavirus/2019-ncov/specific-groups/high-risk-complications.html.

“The views, opinions and positions expressed within this blog are those of the author(s) alone and do not represent those of the American Heart Association. The accuracy, completeness and validity of any statements made within this article are not guaranteed. We accept no liability for any errors, omissions or representations. The copyright of this content belongs to the author and any liability with regards to infringement of intellectual property rights remains with them. The Early Career Voice blog is not intended to provide medical advice or treatment. Only your healthcare provider can provide that. The American Heart Association recommends that you consult your healthcare provider regarding your personal health matters. If you think you are having a heart attack, stroke or another emergency, please call 911 immediately.”

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Heart Failure Management and the Challenge of Systems-Based Practice Implementation for Optimization of Guideline Directed Medical Therapy

As the population continues to age, along with the addition of lifesaving and prolonging medical therapies, the prevalence of HF will continue to increase. In this article, we will solely focus on Heart Failure with Reduced Ejection Fraction (HFrEF), and the potential solutions to the issues with the optimization of guideline directed medical therapy (GDMT) on a systems level.

Robust evidence has established a mortality benefit of GDMT for patients with left ventricular dysfunction. Although the known benefits of GDMT have continued to solidify, there remains a visible gap among patients with HFrEF and the efficacy of treatment.

The issues that exist are likely not based on the individual pharmaceutical therapies profile. Furthermore, medication intolerance and incomplete prescription data can only partially be blamed. Nevertheless, the problems are on a bigger scale, and they involve many different components of our care system.

Let’s discuss some of the barriers to the optimization of GDMT in patients with HFrEF: patient providers and the care system. Providers, including non-cardiologists, should be trained adequately to be able to initiate patients on appropriate medications for HFrEF. They should also know the threshold to discontinue the medications, their side effects, the major contraindications, and, most importantly, when to seek help. HF patients are often complex, and it is essential to know that the different providers involved in their care should be in constant communication when it comes to their medical regimen. It is not enough to start the medication. It’s of utmost importance to continue increasing the dosages as tolerated by the patient to at least the dosages used in the different studies where these medications have shown the most benefits. Education is a key aspect, and it should involve the patient, patient’s family, providers, and everybody in the care system responsible for the patient including nurses and pharmacists. I propose 2 points among many out there:

  1. Standardized education for everybody involved in the patient’s care
  2. Standardized methods of communication between the different providers involved in the patient’s care including the patient and their families.
Heart Failure Summit 2017 Overview: Improving care and outcomes in heart failure

Figure 1. Heart Failure Summit 2017 Overview: Improving care and outcomes in heart failure1.

The purpose of this article is not to re-invent the wheel. The American Heart Association Heart Failure Summit in 2017 identified opportunities to improve care and outcomes and reduce disparities for patients with HF.(Figure 1). The purpose of this article is to remind us that we should be focusing more on implementation strategies for GDMT. We already have the tools, and, as we speak, we are adding new ones. It’s not just the tools; it is how you make use of them that will be the difference.

In summary, establishing and implementing systems of care that can help increase the number of patients on GDMT with the focus on improving medication adherence will ultimately lead to better outcomes. What is certain is that we must continue to meet the challenges of the realities of GDMT and their barriers. Our patients with heart failure depend on it.

References

  1. Pamela N. Peterson. Circulation: Heart Failure. The American Heart Association Heart Failure Summit, Bethesda, April 12, 2017, Volume: 11, Issue: 10, DOI: (10.1161/CIRCHEARTFAILURE.118.004957)

The views, opinions and positions expressed within this blog are those of the author(s) alone and do not represent those of the American Heart Association. The accuracy, completeness and validity of any statements made within this article are not guaranteed. We accept no liability for any errors, omissions or representations. The copyright of this content belongs to the author and any liability with regards to infringement of intellectual property rights remains with them. The Early Career Voice blog is not intended to provide medical advice or treatment. Only your healthcare provider can provide that. The American Heart Association recommends that you consult your healthcare provider regarding your personal health matters. If you think you are having a heart attack, stroke or another emergency, please call 911 immediately.

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How My Heart Failure/Transplant Fellowship Changed Me

When I started my Advanced Heart Failure/Transplant fellowship, my program director told me this year would change my life. I thought, “Yeah okay, whatever.” Boy, did that year change my life. The way I looked at the world changed entirely. Transplant is one of the most incredible medical therapies available to patients with end-stage heart, kidney, and liver disease, amongst others. Because of the generosity of the donor and the donor’s family, someone else is given a second chance at life. I always tell my heart transplant patients that they should now be celebrating 2 birthdays every year- to commemorate the gift of life given to them a second time over.

When I say that year changed my life, it truly did, and that change is lasting. When we’re on heart donor call and we’re evaluating hearts for suitability for our recipients, they’re usually younger hearts and cause of death is almost always unexpected. The stories are tragic- suicides, car accidents, freak accidents, and unintended drug overdoses, amongst other causes of death. As I sit in my pajamas (donor heart evaluations happen in the middle of the night a lot) on my laptop making sure I look through all personal and medical details available to me, I can’t help but create an image in my mind of who this donor is, what they may have looked like, where they worked, how much pain they must have been in if their death was intentional, and most gut-wrenching is all the people they left behind. Death is never easy, but when the donors are young, when the deaths are intentional, when the deaths are completely unexpected, it makes me realize how grateful we should be for this life we are living.

That year completely changed how I look at the world. No longer was I going to “sweat the small stuff” whether they were work related or personal. Every donor call reminds me that we sometimes spend so much time, energy, and emotions on things that, in the grand scheme of life, are truly insignificant. I became a happier and more content person. This year taught me that human connections are the most important thing in this world. My family, the friends I consider family, my friends at work, my patients, and all the people I cross paths with that have an impact on my life.

And on the other side of death, after I have pictured this life lost and the family and friends they’ve left behind, I get to tell one of our patients with end-stage heart failure that a heart “has become available” to them and now their life is going to change. I can’t imagine how they feel but I’ve heard all kinds of the emotions on the other end of that phone- tears, shock, anxious smiles that can be heard through the phone, and more tears. My patients tell me it’s a very emotional experience from the time they’re listing. Some have said it feels weird to be “waiting for someone to die” so that they can live. Some have noted guilt. Some of my patients have developed relationships with their donor’s families and I can only imagine how surreal that must feel.

What I do know is that I couldn’t imagine myself doing anything else and that being a Transplant Cardiologist has truly changed my life. I am grateful to the patients who have allowed me to play a small role in their journey and forever grateful to the donors and their families for this incredible gift of life.

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Thiamine: An Important Nutrient to Consider in Treatment of Congestive Heart Failure

Thiamine deficiency is an uncommon nutritional deficiency in the developed world. The population most at risk in North America and Europe has been noted to be alcoholics with poor diets. This nutrient deficiency can manifest as several different syndromes, one of which is “beriberi.” Beriberi was first described by Dr. Wenckebach in the early 1900s who observed the presence of dependent edema, elevated venous pressures, and an enlarged heart in patients who had three or more months of a thiamine deficient diet, with recovery after thiamine administration. What followed years after were several case reports of alcoholics with signs of congestive heart failure who improved drastically with administration of thiamine.

Although today beriberi heart disease is a rare diagnosis, what it does show is that thiamine is an important micronutrient for the heart, and lack of thiamine can cause symptoms of heart failure.

Given that thiamine is excreted through the urine, another population that has been deemed to be at risk for thiamine deficiency is those on high doses of diuretics such as furosemide1. Interestingly, this population includes the difficult-to-control heart failure patients that we see on the wards every day. Biochemically, one study has shown that thiamine uptake in cardiac cells can be inhibited by furosemide2.

Yet, treatment of patients with congestive heart failure on diuretics with thiamine is not currently standard of practice.

Looking at the literature, there have been only two randomized double blind placebo controlled trials on thiamine use in patients with congestive heart failure: Shimon et al 19953 and Schoenenberger et al 20124. Both of these trials showed a statistically significant increase in left ventricular ejection fraction with the use of thiamine in patients presenting with symptomatic congestive heart failure. Granted, the ejection fraction only improved by 3-4% which we could say was due to echocardiography interpretation variability. However, being that thiamine is cheap and there is evidence that points towards its use as a medication in heart failure, should we institute it into our daily practice?

What do you think?

 

References:

  1. Katta N, Balla S, Alpert MA. Does Long-Term Furosemide Therapy Cause Thiamine Deficiency in Patients with Heart Failure? A Focused Review. Am J Med. 2016;129(7):753.e7-753.e11.
  2. Zangen A, Botzer D, Zangen R, Shainberg A. Furosemide and digoxin inhibit thiamine uptake in cardiac cells. Eur J Pharmacol. 1998;361(1):151-5.
  3. Shimon I, Almog S, Vered Z, et al. Improved left ventricular function after thiamine supplementation in patients with congestive heart failure receiving long-term furosemide therapy. Am J Med. 1995;98(5):485-90.
  4. Schoenenberger AW, Schoenenberger-berzins R, Der maur CA, Suter PM, Vergopoulos A, Erne P. Thiamine supplementation in symptomatic chronic heart failure: a randomized, double-blind, placebo-controlled, cross-over pilot study. Clin Res Cardiol. 2012;101(3):159-64.

 

 

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It’s All In The Family

Our understanding of the genetic basis of adult-onset cardiomyopathy is rapidly evolving. Most of us learned to practice medicine in a paradigm that relied on detailed disease phenotyping, but now we have the ability to incorporate genetic and genomic information into routine clinical care. Even if you cannot remember the last time you thought about genes or pedigrees, here are a few modifications you can make to your practice today that will help you keep up with this exploding field of cardiovascular medicine.

 

1) Take a minimum three-generation family history in all patients with a primary cardiomyopathy.

  • The goals of taking a family history are to learn (1) whether the cardiomyopathy is familial, (2) about disease characteristics among family members, (3) if it is inherited in a specific pattern, and (4) to identify at-risk relatives.
  • Most adult-onset cardiomyopathies are inherited in an autosomal dominant pattern, but other inheritance patterns (e.g. dignetic, multigenic) are possible and need further study.
  • Importantly, an individual can have a genetic form of cardiovascular disease without having affected relatives. Most often, this is due to recessive inheritance, de novo mutations/variants, or reduced penetrance.

 

2) Use focused questions to obtain the family history.

  • The use of vague terms like “heart attack” can lead clinicians away from pursuing an inherited etiology of disease and prevent them detecting other important cardiovascular diagnoses like sudden cardiac death in family members.
  • Ask specific questions regarding heart failure symptoms (e.g. presence or absence of dyspnea at rest or on exertion, paroxysmal nocturnal dyspnea), arrhythmia symptoms (e.g. palpitations, presyncope, syncope with or without exertion), and sudden death (e.g. drowning, single-vehicle accidents) in family members.
  • Knowing about relatives’ cardiovascular procedures like arrhythmia ablation, cardiac surgery, device implantations, or heart transplantation can also be helpful.
  • If a multisystem syndrome like a laminopathy or Fabry disease is suspected, familiarize yourself with the extracardiac manifestations and include pertinent questions in your history.

 

3) Remember that diagnosing an individual with an inherited cardiovascular disease is just the first step in the process.

  • In cardiovascular genetics, a key concept is the transition of practice from individual patient-based care to family-based care. By incorporating the information you generate from your comprehensive multi-generational family history, you also gain insight into disease penetrance, expression, age of onset, and pleiotropy.
  • Generally, cardiomyopathies are considered “medically actionable” because evidence-based treatments to reduce morbidity and mortality exist.
  • Beyond just having effects on medical and device therapies, following this paradigm of family-based care also has implications for reproductive and family planning and lifestyle practices.

 

If you are interested in learning more about this topic, check out the 2018 update to the Heart Failure Society of America Practice Guideline on the Genetic Evaluation of Cardiomyopathy (PMID: 29567486).

 

 

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Highlights of the 1st Annual Sex and Gender Conference at AHA18

Walking into the Palmer House Hotel, the longest continuously operating hotel in the United States, you can’t help but pause in awe at the intricate décor and take in the most photographed ceiling in the world. I make my way to the Honoré Ballroom, named after Bertha Honoré Palmer, the wife of Palmer and an astute businesswoman and well-known Chicago socialite of her time, not knowing what to expect for the 1st annual Sex and Gender Influence on Cardiovascular Disease (CVD) conference.

Annabelle Volgman, medical director of the Rush Heart Center for Women, kicks off the evening by thanking the speakers and planning members, and encouraging photography and social media sharing. The many photos of the evening include Bertha Honoré’s portrait adjacent to the colorful and modern logo that, I think, will become a recognized image at future AHA Scientific Session meetings.

Dr. Annabelle Volgman welcomes attendees to the 1st Annual Sex and Gender Influences on Cardiovascular Disease at the Palmer Hotel in Chicago, IL (November 11, 2018).

 

Dr. Nanette Wenger of the Emory Women’s Heart Center starts the conversation with her presentation titled “Why is Mortality from Cardiovascular Disease Rising in Men and Women?” She flashes a graph of CVD mortality on the screen, highlighting the steep decline in the past decades, but the leveling off and reversal in recent years, particularly in women under the age of 55 years. The parallel rise in obesity and diabetes, as well as “non-traditional” CVD risk factors such as depression and perceived stress disproportionally affect women, she explains, and may be responsible for this reversal in CVD death rates. Summarizing the recent paper, “Defining the New Normal in Cardiovascular Risk Factors” by Dr. Donald Lloyd-Jones and Dr. Philip Greenland she points to a combination of health behaviors and ideal levels of total cholesterol, blood pressure, and fasting blood glucose, as key factors in achieving cardiovascular health.

“Behavior change,” she says, “is the ‘Holy Grail’ of heart health” and as “health professionals take back the role [of health educator] and address lifestyle behaviors” we will see favorable trends in biomarker targets we’re so interested in.

Later during the Q+A panel, when asked about the best way to approach behavior change with patients, she advises to first, “Give information – if your patient does not have the information, they can’t make a change. Then, let them start with what they would like to start with. Don’t give them 8-10 [health behaviors] to change – they will tune you out.” Dr. Gina Lundberg, co-director of the Emory Women’s Heart Center, chimes in that the clinician’s “approach to weight loss is similar to smoking cessation. Identify the obstacles in the patient’s way – money, time, desire – and often just identifying those hurdles will lead to improvement.”

Dr. Laxmi Mehta, director of the Women’s Cardiovascular Health Program at the Ohio State University Wexner Medical Center, adds that she includes an emotional appeal – “Where is the patient going and what do they want?” Seeing a child’s wedding or playing with their grandkids, developing rapport with patients and fitting your recommendations to their goals can start the health behavior change process, even in a 5 minute clinician-patient discussion.

 

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How the Immune System Favors Females in Pulmonary Artery Hypertension? Another Regulatory T Cell Story.

While it is commonly thought that cardiovascular disease is a man’s disease, CVD is the number one killer of women with the same number of deaths per year as cancer, diabetes and respiratory disease combined (according to 2015 statistical data from AHA). In addition, women exhibit different and more silent symptoms of heart attacks. There is a lot of interest in the difference between how males and females respond to CVD. A lot of emphasis is put on hormonal differences, but the immune system also seems to play an important role in this disparity. Females have a more robust immune system and therefore respond faster to infections providing more protection than in males. However, a more responsive immune system also means a more reactive immune system that can result in increased incidence of autoimmune diseases, such as rheumatoid arthritis and lupus.

Part of the difference in the immune system response in females can be attributed to the fact that multiple immune-related genes are expressed on the X chromosome. Since females have two alleles of the X chromosomes and males have only one, it is evident that females express more genes that regulate immune system functions. One of these genes is Foxp3, the key transcription factor for regulatory T cells, an adaptive immune cell which I have discussed before in a previous post. Regulatory T cells play an important protective role in CVD, especially in atherosclerosis and hypertension.

Pulmonary artery hypertension (PAH) is a fatal cardio-pulmonary disorder where the pulmonary arterioles narrow leading to a right ventricular fibrosis, heart failure and death. Regulatory T cells play an important role in this disease as animal models that lack regulatory T cells are more susceptible to PAH. Adding regulatory T cells back prevents the development of PAH showing the protective power of these cells. A recent study published in the journal Circulation Research, shows that in the absence of regulatory T cells, females rats are more prone to PAH than male animals due to a lower levels of PGI2, a pulmonary vasodilator, and the lack of the enzyme COX-2 that regulated PGI2. The researchers conducting the study show that by transferring regulatory T cells into these rats, these immune cells were sufficient to restore the levels of COX-2 and PGI2, as well as other immune inhibitory molecules PDL1 and IL-10. The authors suggest that regulatory T cells have both a direct and indirect effects on the arteries. The direct effects are exerted on the endothelial cells directly via COX-2 and PGI2, and the indirect effect is through the release of inhibitory molecules such as IL-10 and TGF, both of which would result in immune suppression and preventing inflammation. The results from this report suggested that females are more reliant on regulatory T cells for protection against PAH.

These new findings highlight the subtlety of immune regulation between females and males and further proves that in addition to hormonal differences, immune regulation disparities between genders that can alter the outcome of cardiovascular diseases. By understanding more about gender differences in CVD and the immune system, and figuring out ways to manipulate these subtle differences, scientists hope to achieve a more personalized and effective therapies to women versus men to combat CVD.

 

Dalia Gaddis Headshot

Dalia Gaddis is a postdoctoral fellow at the La Jolla Institute for Allergy and Immunology. She has a Ph.D. in microbiology and immunology. She is currently working on understanding the interactions between the immune system and atherosclerosis development

 

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Nursing research at Heart Failure congress 2018 in Vienna, Austria

The 26th-29th of May I went to the Heart Failure Congress 2018, Including the World Congress on Acute Heart Failure in Vienna, Austria. The congress had over 5,750 participants from over 80 countries. To see all the tweets during this congress, search for the #HeartFailure2018.

This year there were a lot of interesting talks and I would like to highlight a few in this blog.

Dr. Tiny Jaarsma gave a talk about sex and heart disease. Pointing out that heart disease can lead to sexual problems. Furthermore, patients think that sexual counseling should be part of cardiac rehabilitation, which is often not a subject that is discussed. Patients prefer to receive written material and/or individual discussions.

Dr. Cecilia Line from @karolinskaint pointed out that one third of patients with an ICD are not aware that they can deactivate their ICD. Furthermore, patients with ICDs would like to be informed especially when the life expectancy is short. And we should remember, according to Dr. Anna Strömberg, that patients with an LVAD are going through a transition in life and that there are psychosocial challenges in patients with an LVAD. See her take-home messages in the pictures below.

managing patients psychosocial distress slide

Some talks highlighted the importance of frailty in cardiac patients. Dr. Inger Ekman from @SahlgrenskaAcad discussed the importance of screening for frailty in cardiac patients and suggested existing instruments to do so (see picture below). Dr. Kentaro Kamiya’s research showed that frailty is associated with clinical outcomes in elderly patients hospitalized for heart failure, which indicates that frailty is useful for prognosis in in hospital settings.

frailty instruments slide

In the late breaking trials, the results of the HF-Wii study were discussed. This study proves that exergaming (being physical active through video gaming) has positive influence with the exercise capacity and wellbeing of patients with heart failure. See an interview about this study during the interview. In a poster presenting a sub-study of the HF-Wii showed that exergaming could also be feasible for patients with an LVAD, and patients especially liked to exergame with their grandchildren.

conclusion slide

There was also a great session on alternative ways to be physically active in patients with heart failure. One of the alternative ways was presented by Dr. Anna Strömberg: medi-yoga. Read more about medi-yoga. A second alternative way to exercise was presented by Dr. Taylor-Piliae: Tai-Chi. And a third way presented by Dr. Tiny Jaarsma was exergaming. In this session Dr. Von Haehling pointed out that testosterone therapy could increase exercise capacity. Dr. Massimo Piepoli concluded that half of the patients with heart failure are denied a highly recommended therapy and that scientific societies should strongly promote a well-recognized therapeutic tool to improve exercise capacity, quality of life and outcomes in patients with heart failure.

exergaming has physical and quality of life benefits for heart patients flier

And last but not least, I would like to congratulate Lilas Ali, who won the nursing investigator award session with her research that showed that person-centered telephone-support is effective in patients with chronic obstructive pulmonary disease and/or chronic heart failure (see picture below).

photo of women receiving award

Please save the date for next years’ Heart Failure congress 2019, 25-28th of May 2019 in Athens, Greece.

save the date for heart failure and world congress on active heart failure

Leonie Klompstra Headshot

Leonie Klompstra is a Nurse Scientist at the Linköping University in Sweden. Her primary focus is on heart failure and rehabilitations.