HFpEF With Severe Pulmonary Hypertension Linked to Increased Mortality - Pulmonology Advisor

Posted: 25 Jun 2019 12:45 AM PDT

Patients with heart failure, severe pulmonary hypertension, and a preserved ejection fraction of ≥50% had a worse prognosis when compared with other patients with heart failure and severe pulmonary hypertension, according to a study published in Heart and Lung.

Researchers of this prospective, observational study analyzed The Israeli Association for Cardiovascular Trials database for baseline characteristics, comorbidities, renal and heart status, and long-term prognosis of patients with heart failure and pulmonary hypertension.

Using conventional trans-thoracic echocardiograms, patients were categorized by left ventricular ejection fraction into reduced ejection fraction (<40% ejection fraction), mid-range ejection fraction (40%-49% ejection fraction), or preserved ejection fraction (≥50% ejection fraction). Using echocardiography, severe pulmonary hypertension was classified as an estimated systolic pulmonary arterial pressure of ≥50 mmHg. Demographics, echocardiography, and biochemical blood analysis were collected at baseline, follow-up evaluations were completed at 12 months, and mortality rates were assessed at 2 years.

Of the 372 patients included in this study, 56% were men, the mean age was 77.3 years old, and the mean duration of heart failure was 6.5 years. The reduced ejection fraction cohort consisted of 159 patients, the mid-range ejection fraction cohort consisted of 50 patients, and the preserved ejection fraction cohort consisted of 163 patients.

The reduced ejection fraction cohort was predominantly associated with smokers who had coronary artery disease and renal failure. The preserved ejection fraction cohort was predominantly associated with older women who were obese and had atrial fibrillation. The mid-range ejection fraction cohort did not show patterns in baseline characteristic.

Overall, 15% of the patients died by the 2-year mortality rate follow-up. Multivariable analysis indicated New York Heart Association functional class 3-4 (hazard ratio [HR] 2.41; 95% CI, 1.17-4.97; P =.017) and renal failure (HR 2.53; 95% CI 1.45-4.42; P =.001) were independent predictors for mortality. Kaplan-Meier survival curves indicated an association between severe pulmonary hypertension and mortality in the preserved ejection fraction cohort (adjusted HR 2.99; 95% CI, 1.29-6.91; P =.010) but not in the other cohorts.

Limitations of this study include only evaluating demographic, clinical, and echocardiographic information from a database, not assessing right heart parameters, and the potential for selection bias.

Researchers concluded that patients with heart failure with preserved ejection fraction and pulmonary hypertension "are likely to have different pathophysiology and worse prognosis" and "defining these patients as an independent subgroup may be more appropriate for their management and treatment."

Reference

Zafrir B, Carasso S, Goland S, et al. The impact of left ventricle e jection fraction on heart failure patients with pulmonary hypertension [published online June 5, 2019]. Heart Lung. doi:10.1016/j.hrtlng.2019.05.006

This article originally appeared on The Cardiology Advisor

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Pulmonary Hypertension

'My heart is getting very tired - I need a transplant' - Mother-of-two (47) hoping for 'brighter future' with her family - Independent.ie

Posted: 23 Jun 2019 06:30 PM PDT

Karen Doherty has been living with the condition for 14 years. Photo: Gerry Mooney

Everyone feels tired when a new baby arrives on the scene, particularly if there is also a toddler needing attention.

But Karen Doherty couldn't shake the constant exhaustion she felt after her daughter Rachel (now 14) was born in 2004. She put it down to sleepless nights and the general weariness of being a mum looking after two children, but when she noticed her fitness levels seemed to be at a shockingly low level, she went to the GP, who initially thought she might simply be unfit.

However, despite taking up regular exercise, her fitness didn't improve and instead got dramatically worse. So Karen returned to the doctor, who sent her for tests, which revealed she had a condition called pulmonary hypertension (PH), a chronic disease affecting the lungs and heart.

"After having Rachel, I felt permanently exhausted and put it down to looking after her and Darragh (now 16)," says Karen, who is married to Shay. "I was unnaturally tired for months, almost a year, and then one day I attempted a short run on the way back from my mother's house and I actually got sick and felt terrible pains in my chest.

"I went to see my GP, who said I was probably not fit enough. I might have had a few baby pounds still hanging around but I was never what you would call a big girl, but I decided to take up walking and started regular walks with a friend. But I couldn't keep up with her so I went back to the doctor who ordered an X-ray - but this showed nothing irregular."

Somewhat reassured by the negative X-ray, results but also still concerned that she couldn't shake off the 'tiredness', the mother-of-two tried to carry on as normal. But when things took a turn for the worse, further tests were done and she was finally diagnosed with a life-altering condition.

"I was glad that the X-ray came back clear but about two weeks later, I walked up the stairs and passed out when I got to the top," she says. "I knew something was very wrong and when I went back to the doctor, I was sent straight to A&E as she thought I might have had a lung clot.

"Tests were run and the first results showed I had an abnormal swelling of the heart, so I was kept in for a few more tests to be done and it wasn't long before the doctors told me that I had pulmonary hypertension. I was devastated - all I could think about was my two children.

"It was so unfair. I had never even heard of this disease and had no idea why I developed it. Of course, then I Googled it, which is always a bad idea as the prognosis seemed absolutely dreadful - I was in shock but I was apparently also lucky that I had been diagnosed so quickly, as often this can take a long time because PH mimics so many other illnesses."

Regina Prenderville of the Pulmonary Hypertension Association agrees and says it can take years to diagnose the disease.

"Pulmonary arterial hypertension is often not diagnosed in a timely manner because its early symptoms can be confused with those of other cardio-respiratory conditions," she says. "Symptoms can initially be subtle and relatively non-specific. A process of exclusion generally makes diagnosis of PH by looking for and ruling out other diseases.

"In fact, there is often a lengthy delay between the time when patients first visit their general practitioner and the time when they receive specialist care at a hospital. The average delay is about two-and-a-half years."

Once Karen was diagnosed, she was referred to the Mater Hospital, where she underwent further tests and was put on an initial course of treatment.

"More tests were run to see what was causing the problem but after two weeks, it was discovered that it was idiopathic, which meant there was no reason other than bad luck - this was a very bitter pill to swallow," says the Kildare woman.

"I went from knowing nothing about PH to being overloaded with information about it - I was so upset. And when I met with the consultant, I cried throughout the whole appointment. Shay took charge of dealing with all of the information and the professor, who was simply lovely, put me on a course of medication to see if my symptoms could be stabilised.

"I was told not to exert myself or even lift the kids - which, of course, I had to - and to try and get on with my life."

Karen, who believed she had "only a few years to live", tried to bring some sort of normality to her life. But it became increasingly difficult as there was very little support available.

"I didn't know anyone else who had this condition and had never even heard of it before being diagnosed with it, so it was a very lonely and difficult time for me," she admits. "But the Mater staff were amazing, as were my family and friends, and because there was no support group available, one was eventually set up and I met other people going through the same thing.

"Then in 2010, I was asked if I wanted to go on a trial drug, which I agreed to and it worked very well for two years; even though I had a much slower pace of life, I was doing well.

"Then in 2012, it was decided that I needed something else so I was put on a subcutaneous drug which was delivered by a pump through my stomach. It was very harsh and when I had to change the position to another part of my tummy, the pain would be unbearable and sometimes I would be in bed on morphine tablets for up to a week."

The 47-year-old has recently been put on another drug which is delivered intravenously through a pump, which she carries with her at all times in a small bag. Three years ago she was put on the waiting list for a double lung transplant and is still waiting for this life-changing operation.

"I have been called in twice to have the transplant but on both occasions, it fell through," she says. "This has been very difficult, as you get your hopes up and then nothing happens. For the past three years my life is on hold, I can't leave the country just in case a transplant becomes a possibility because the call could come at any time.

"My heart is getting very tired and is enlarged as it is under pressure, so a transplant would really transform my life and improve my standard of living. I'm trying to stay positive but it can be difficult.

"Shay has been amazing throughout it all and has such a positive outlook and he and my kids are what keep me going - as do my family, particularly my parents, who have been fantastic and do whatever they can to help.

"Everyone has been so good over the years but no one really understands what it is like - there are so many times that I can't leave the house and it gets very lonely. But although I often feel very low, I am still hopeful that something will happen and I will have a brighter future with my family."

What is PH?

⬤ There are currently around 300 people in Ireland suffering with this rare disease.

⬤ With PH, the arteries that carry blood from the heart to the lungs narrow for reasons that are not yet entirely understood. The heart struggles to pump blood through the narrowed arteries, resulting in high blood pressure in the lungs and enlargement of the heart. Eventually, the overworked heart wears out, and heart failure and death can result.

⬤ People with pulmonary hypertension experience a range of symptoms that vary in intensity depending on the severity of their condition. The most common symptoms are:

1. Shortness of breath (dyspnoea) with no obvious cause following exertion or even at rest.

2. Excessive fatigue

3. Dizziness, especially in climbing stairs or standing up

4. Fainting, weakness upon physical exertion

5. Chest pain, especially during physical activity

6. Palpitations

7. Swollen legs and ankles

8. Cough

9. Haemoptysis (Coughing of blood)

⬤ Symptoms can occur at rest or more frequently during periods of mild exercise, or simply walking around.

⬤ For more information, visit pulmonaryhypertension.ie and materfoundation.ie

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