PeaceHealth Southwest curates collaboration with specialty treatments for pulmonary hypertension patients - clarkcountytoday.com

PeaceHealth Southwest curates collaboration with specialty treatments for pulmonary hypertension patients - clarkcountytoday.com


PeaceHealth Southwest curates collaboration with specialty treatments for pulmonary hypertension patients - clarkcountytoday.com

Posted: 29 Jul 2020 06:33 PM PDT


Cardiologist and pulmonologist work together to treat nuanced illness

VANCOUVER — It's rare, but deadly; nuanced, but treatable. Pulmonary hypertension (PH) is an illness affecting a wide range of people, but most often those with several larger conditions that are the actual cause.

Across the country, many hospitals and medical centers have PH clinics. They are typically run by the cardiology or pulmonology departments, with 70 percent in the case of pulmonologists. At PeaceHealth Southwest Medical Center, they've opted for collaboration.

Dr. Josh Grant, a cardiologist at PeaceHealth Southwest Medical Center, demonstrates how PH occurs in and affects the heart using a model. Photo by Jacob Granneman
Dr. Josh Grant, a cardiologist at PeaceHealth Southwest Medical Center, demonstrates how PH occurs in and affects the heart using a model. Photo by Jacob Granneman

Doctors Josh Grant and Bhanu Patibandla, a cardiologist and pulmonologist respectively, form the base of the program at PeaceHealth. Together, they work to treat patients with PH and explain the effects the illness has on the heart and lungs.

Dr. Josh Grant
Dr. Josh Grant

"We're very driven by patient care, and we really want to see people do well," Grant said. "Having this clinic allows us to take that multi-faceted approach so that we can see the interplay between both the heart and the lungs in this disease. We see many patients with really several causes for their pulmonary hypertension, and it's through treatment of all of these causes that really allows us to better patient care, to improve symptoms and to hopefully provide quality of life."

At its simplest, PH is an illness that negatively affects the arteries in the lungs and the right side of the heart. The arteries can be obstructed by blood vessels which are not functioning properly and are narrowed, blocked or damaged, or the left side of the heart can be compromised making blood flow irregular.

As Patibandla explains it, there are two sides in the heart. The left side of the heart pumps blood to the whole body except the lungs. After delivering oxygen to the body, this blood returns to the right side of the heart, which is responsible for pumping blood into the lungs, where it gets oxygen and returns to the left side of the heart to be pumped forward again to the rest of the body. This process is how normal blood flow happens. 

When this process is disrupted in any way, it can lead to PH.

Dr. Bhanu Patibandla
Dr. Bhanu Patibandla

"Any condition that compromises this blood flow can cause pulmonary hypertension," Patibandla said. "Many of the symptoms of PH are also similar to what patients experience with heart and lung problems, such as heart failure, or atrial fibrillation or any valvular disease, or lung problems such as COPD or asthma. That's one of the things that makes the diagnosis of pulmonary hypertension very challenging."

With a difficult diagnosis process, PH is often under or misdiagnosed, Grant said. The process is thorough, and begins with a screening and patient exam. A special type of heart ultrasound known as an echocardiogram is the primary method for then observing the pressures in the heart.

The PeaceHealth PH clinic has been open for about a year, and has seen many patients and administered a number of treatments. Jane Nelson is one of those patients, and traveled all the way from Mossyrock, WA to be treated by the clinic.

"They're both in the room at the same time, you know, asking questions, and so it's great," Nelson said. "It's one co-pay, and it's great. I'm fine. I'm good. They've done everything that they should do, I guess, got me on the right medicine and I'm feeling great."

A special kind of ultrasound machine, known as an echocardiogram, is seen here. It is the primary tool for preliminary examinations for PH. Photo by Mike Schultz
A special kind of ultrasound machine, known as an echocardiogram, is seen here. It is the primary tool for preliminary examinations for PH. Photo by Mike Schultz

Prior to being treated, Nelson was experiencing significant breathing issues, and at one point checked herself into the emergency room. Now she is on two specialty medications that help regulate the causes of her PH.

Virginia Christianson will be 84 this November, and thanks to the treatments she receives for her PH at the PeaceHealth clinic, she is confident she has many more years. After struggling with breathing during sleep, she went to the hospital and was later connected with Grant and Patibandla.

"One of the lower parts [of my heart], it would expand, not contract," Christianson said. "So the fluids were going into my lungs. That's why I had a hard time breathing, and I had no clue of it. I thought, 'Well, maybe I'm gonna check out soon, because I'm older.' But no, I've got plenty of get up and go and I'm gonna get up and go."

Both Nelson and Christianson said they felt the collaborative nature of the clinic made them feel more confident of their diagnosis and treatment.

"I feel hundred percent taken care of here," Nelson said. "I mean, I love those two guys. They have really changed my life because I can breathe pretty darn good."

For more information on the PeaceHealth Southwest Medical Center's pulmonary hypertension clinic, visit their site here. To learn more about PeaceHealth and the collaborative programs they are using, visit their main site.

PAH Caused by Undiagnosed Systemic Sclerosis in Case Study - Pulmonary Hypertension News

Posted: 29 Jul 2020 09:00 AM PDT

A case report found a patient's pulmonary arterial hypertension (PAH) was caused by an undiagnosed case of the autoimmune disorder systemic sclerosis (SSc), highlighting the need for early and thorough diagnostic workups, researchers say.

The 77-year-old woman's PAH was also worsened by other conditions, including lung disease, sleep apnea, heart failure, and chronic anemia, which made the diagnosis difficult. 

With multiple possible causes for PAH, researchers recommend that attention be paid to undiagnosed autoimmune disorders that may play a role in the disease's development. 

"Development of severe pulmonary hypertension and [lung disease] leading to diagnosis of SSc in this case is unusual," the researchers wrote in the article "Multifactorial Pulmonary Hypertension in Systemic Sclerosis," which was published in the Cureus Journal of Medical Science

PAH is a chronic and progressive disease characterized by high blood pressure in the pulmonary arteries — the blood vessels that supply blood from the heart to the lungs.

The disease can have many potential causes, including heart abnormalities, viral infections, liver disease, connective tissue disorders such as scleroderma and lupus, and the use of certain medications, or it can be inherited. PAH without an easily identifiable cause is called idiopathic PAH.

When multiple possible causes occur at the same time, diagnosis and treatment can be challenging, as illustrated by the case of the 77-year-old female patient in the study reported by researchers at the University of Illinois College of Medicine

The patient complained of worsening shortness of breath upon physical exertion for the past nine months, which interfered with her daily activities. 

She had a medical history of genetic-related anemia (low red blood cells) and a type of inflammatory bowel disease known as Crohn's disease, and was being treated for both conditions. She reported no history of blood clots, or heart or liver disease, and no family history of connective tissue disorders. 

An initial examination found low blood oxygen levels and abnormal rattling sounds in the lungs. The patient was thus given oxygen supplementation. 

Blood tests confirmed the presence of antibodies associated with autoimmune disease, which led to a diagnosis of SSc — an autoimmune disease characterized by the buildup of scar tissue in the skin and several internal organs, including the arteries. 

Functional and imaging tests showed signs of lung disease. The patient was also diagnosed and treated for sleep apnea, in which pauses in breathing occur during sleep. 

Over the next few months, her shortness of breath during physical activity worsened, which was confirmed by a decline in lung function. A sonogram of the heart (echocardiogram) showed high blood pressure in the pulmonary arteries.

The patient was then diagnosed with PAH in a setting of SSc along with lung disease, sleep apnea, heart failure, and chronic anemia. 

She declined rapidly over the next three months and was treated for possible pneumonia. She continued to show low blood oxygen levels, and her pulmonary blood pressure had increased. 

"With continued increment in oxygen requirement, [lung function tests] and CT findings were out of proportion to the degree of pulmonary hypertension," the team wrote. 

The patient started treatment with increasing doses of the blood pressure medication nifedipine (sold under several brand names).

Following a meeting with specialists in lung disease, cardiology, and rheumatology, she was started on a triple combination of PAH therapies which included the blood vessel dilators Uptravi (selexipag) and Opsumit (macitentan), both marketed by Actelion, along with Adcirca (tadalafil, marketed by United Therapeutics). 

She also continued treatment with nifedipine, immunosuppressant steroids, and mycophenolate to treat SSc, as well as torsemide to treat fluid overload due to heart failure and high blood pressure.

After six months, her pulmonary blood pressure decreased, which was consistent with mild improvements. Her treatment regimen continued to be optimized. 

"Despite evident causes, due consideration should be given to undiagnosed autoimmune disorders playing a role in causing PAH," the researchers wrote, noting that "management in such cases remains a multidisciplinary approach."

Steve holds a PhD in Biochemistry from the Faculty of Medicine at the University of Toronto, Canada. He worked as a medical scientist for 18 years, within both industry and academia, where his research focused on the discovery of new medicines to treat inflammatory disorders and infectious diseases. Steve recently stepped away from the lab and into science communications, where he's helping make medical science information more accessible for everyone.
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PatrĂ­cia holds her PhD in Medical Microbiology and Infectious Diseases from the Leiden University Medical Center in Leiden, The Netherlands. She has studied Applied Biology at Universidade do Minho and was a postdoctoral research fellow at Instituto de Medicina Molecular in Lisbon, Portugal. Her work has been focused on molecular genetic traits of infectious agents such as viruses and parasites.
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Steve holds a PhD in Biochemistry from the Faculty of Medicine at the University of Toronto, Canada. He worked as a medical scientist for 18 years, within both industry and academia, where his research focused on the discovery of new medicines to treat inflammatory disorders and infectious diseases. Steve recently stepped away from the lab and into science communications, where he's helping make medical science information more accessible for everyone.
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Study sheds wider light on the clinical profile of patients with pulmonary hypertension - News-Medical.Net

Posted: 28 Jul 2020 05:29 PM PDT

When most people think of high blood pressure, they think of being tested with an arm cuff at a doctor's appointment. That type of blood pressure is separate from pulmonary hypertension (PH), which is high blood pressure in the lung arteries. In a study published today in The Lancet Respiratory Medicine, investigators from Brigham and Women's Hospital took an evidence-based approach to determine the lower end of the risk spectrum for PH based on pulmonary vascular resistance (PVR), which is resistance against blood flow from the pulmonary artery to the lungs. The team presents evidence that by redefining the lower risk level for PVR, 60 percent more patients who are at risk for death due to PH can be diagnosed.

The findings of this study cast the clinical profile of patients who have PH and are at risk for major clinical events into a new and wider light. We can use this information to raise awareness among clinicians on those patients that may need reconsideration for risk factor modification, conventional treatment, and potential clinical trial enrollment."

Bradley Maron, MD, corresponding author, associate physician in the Division of Cardiovascular Medicine at the Brigham

PH affects 70 million people worldwide across a broad age, geographic and socioeconomic spectrum. Left untreated, average survival is 2 to 3 years after diagnosis. PH can cause serious problems because changes in blood pressure in the lung arteries are not tolerated well by the heart and can lead to hospitalization, heart failure and death. Despite being a grave condition, the way PH was defined in clinical practice previously was not evidence-based and did not account for physiological changes in blood pressure, such as may occur due to circumstances that are immediately reversible. Although PVR was already considered important in the prognosis of PH, the actual range of PVR that is informative of PH was not known.

The researchers analyzed the association of PVR and mortality using the Veterans Association (VA) national database, which includes comprehensive information on patients' medical histories and clinical events. The researchers acknowledge the limitations to working with a national population relative to errors in measurements. Also, the VA population is almost entirely male, so the team validated the findings in a sex-balanced cohort at Vanderbilt University Medical Center to account for both men and women. The data analysis did account for comorbidities but was not positioned to distinguish between the different stages of diseases that could affect outcome, such as the degree of lung disease in individual patients.

The results indicated that starting at a PVR of around 2.2 Wood Units (a standard unit of measurement for PVR), there is an increase in association with mortality among patients with increased pulmonary artery pressure. This 2.2 level is well below what is currently associated with the disease in clinical practice. The new, lower threshold expands the range of patients who are considered to have PH and optimizes the specificity of PH criteria used clinically.

"Pulmonary hypertension is often overlooked in clinical practice, but this study provides a specific context for clinicians and health care workers to understand the range of risk for a large group of patients," said Maron. "We have established an evidence-based way to recognize patients with PH who would have otherwise been considered to be normal, but, in fact, have a concerning profile."

From here, the team is interested in looking at populations that were not included in this study, including patients with less common forms of PH or PH that exists in the absence of heart and lung disease.

Journal reference:

Maron, B.A., et al. (2020) The Association Between Pulmonary Vascular Resistance and Clinical Outcomes in Patients with Pulmonary Hypertension: A Retrospective Cohort Study. The Lancet Respiratory Medicine. doi.org/10.1016/S2213-2600(20)30317-9.

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