Pulmonary Hypertension in Myelofibrosis: Risks and Treatments - www.oncnursingnews.com/

Pulmonary Hypertension in Myelofibrosis: Risks and Treatments - www.oncnursingnews.com/

Pulmonary Hypertension in Myelofibrosis: Risks and Treatments - www.oncnursingnews.com/

Posted: 19 Mar 2020 08:53 AM PDT

There may be certain factors that put a patient with myelofibrosis at a higher risk for pulmonary hypertension, according to Juan C. Lopez-Mattei, MD, FACC, FASE, FSCCT, FSCMR.

Lopez-Mattei, a multimodality imaging cardiologist and associate professor of cardiology and diagnostic imaging at The University of Texas MD Anderson Cancer Center, was the lead author of an analysis that examined 143 patients with myelofibrosis who received an echocardiograph. Fourteen percent had findings that were consistent with pulmonary hypertension.

Patients at Higher Risk


Findings showed that females and those with N-terminal prohormone brain natriuretic peptide (BNP) were significant predictors of hypertension. Lopez-Mattei explained that there is an underestimation of pulmonary hypertension due to left-side heart disease, so evaluating respiratory symptoms and elevated N-terminal prohormone BNP should prompt an echocardiogram.

"We did a deep dive looking at the diastolic parameters, or the relaxation parameters, by echocardiogram to asses whether these patients could have some sort of pulmonary hypertension or secondary to left heart disease (LHD) because of the risk factors of the age range that these patients tend to have," Lopez-Mattei said in an interview with OncLive, a sister publication of Oncology Nursing News.

"We found that the most common cause of pulmonary hypertension was secondary to LHD or diastolic dysfunction permanently, which is not a surprise based on the comorbidities of the patients in our study."

Patient characteristics that were associated with pulmonary hypertension included:

  • Older age
  • Coronary artery disease
  • Larger left atrium

There were similar risk factors between patients with heart failure who have preserved ejection fraction and pulmonary hypertension.

"However," Lopez-Mattei added, "they also have the parameters that are the surrogates of the diastolic function that are abnormal in the majority of patients with pulmonary hypertension. If you put all of that into context, then you can understand why we have to look at patient risk factors regardless of whether or not they have myelofibrosis, because sometimes they need better blood pressure control."

Pulmonary Hypertension May Affect Outcomes


Lopez-Mattei said that there is a higher mortality signal in patients with myelofibrosis who also have pulmonary hypertension.
But having the comorbidity does not usually affect the treatment that these patients receive for myelofibrosis.

"In some patients with MPNs, there are some drugs that could cause some cardiovascular adverse events. We did not find that any [drugs] in pulmonary hypertension were tied to worsening outcomes," Lopez-Mattei said.

It is important that the oncology team works with a cardiology team to best treat patients with myelofibrosis and pulmonary hypertension.

"We have a close collaboration with pulmonologists because we both take care of this patient. Our [collaborative] approach has a lot of success because we have varied perspectives," Lopez-Mattei said.

A version of this article was originally published on OncLive as, "Multidisciplinary Approach Required for Pulmonary Hypertension in Myelofibrosis."

4 Pulmonary Hypertension Programs Receive PHA's PHCC Accreditation - Benzinga

Posted: 19 Mar 2020 02:50 PM PDT

Tufts Medical Center, Cleveland Clinic, University of Nebraska Medical Center and National Jewish Health have received accreditation as PHA Centers of Comprehensive Care (CCC) as part of the Pulmonary Hypertension Care Centers (PHCC) program, the Pulmonary Hypertension Association announces.

SILVER SPRING, Md. (PRWEB) March 19, 2020

The Pulmonary Hypertension Association (PHA) announces that Tufts Medical Center in Boston; Cleveland Clinic in Cleveland; University of Nebraska Medical Center in Omaha, Nebraska; and National Jewish Health in Denver have received accreditation as PHA Centers of Comprehensive Care (CCC) as part of its Pulmonary Hypertension Care Centers (PHCC) program. They received accreditation from PHA after demonstrating their ability to deliver quality care to patients living with pulmonary hypertension (PH), a complex, often misdiagnosed, life-threatening disease.

Since PHA's accreditation program launched in 2014, 80 programs in 32 states have received PHCC accreditation. The program includes 59 adult and eight pediatric CCCs along with 13 adult Regional Clinical Programs (RCP).

The complex disease is thought to affect about 25 million adults and children worldwide and can occur among those with left-heart disease, along with sleep apnea, chronic obstructive pulmonary disease (COPD), scleroderma and other associated diseases. PH is significantly underdiagnosed and misunderstood. Symptoms, including shortness of breath, fatigue and chest pain, are not necessarily specific to PH and can lead to dangerous delays in a correct diagnosis.

Although no cure exists, early diagnosis and proper treatment can extend and improve a patient's quality of life. In addition, advances in treatment and a better understanding of PH have led to effective therapies and improved survival for many types of PH, once diagnosed.

"We are thrilled to see the PHCC network continue to grow. This program is an important step toward providing quality care for PH patients and offering scientists and clinicians opportunities to advance clinical research," says Elizabeth Joseloff, Ph.D., vice president, Quality Care & Research, for PHA.

PHA-accredited sites are eligible for participation in the PHA registry (PHAR). PHAR collects data from individuals with pulmonary arterial hypertension (PAH), pediatric PH due to developmental lung disease and chronic thromboembolic pulmonary hypertension (CTEPH) who are starting evaluation and/or treatment at PHCCs across the United States. Over time, PHAR will help researchers evaluate trends and practice patterns to determine the treatments that work best and support PH research.

About the Pulmonary Hypertension Association

Headquartered in Silver Spring, Maryland, the Pulmonary Hypertension Association (PHA) is the oldest and largest nonprofit patient association dedicated to the pulmonary hypertension (PH) community. Pulmonary hypertension is a rare, chronic and life-threatening disease of the lungs for which no cure currently exists. PHA's mission is to extend and improve the lives of those affected by PH. To achieve this mission, PHA engages people with PH and their families, caregivers, health care providers, and researchers worldwide who work together to advocate for the PH community, provide support to patients, caregivers and families, offer up-to-date education and information on PH, improve quality patient care, and fund and promote research. For more information, visit the PHA website.

For the original version on PRWeb visit: https://www.prweb.com/releases/4_pulmonary_hypertension_programs_receive_phas_phcc_accreditation/prweb16993831.htm

Aria Raises $31M for Planned Study of Balloon Implant to Treat PAH - Pulmonary Hypertension News

Posted: 02 Mar 2020 12:00 AM PST

Aria CV announced the end of a funding round that will be used to finance a feasibility study of its medical device, the Pulmonary Hypertension System (Aria CV PH System), in people with pulmonary arterial hypertension (PAH) in the U.S.

The series B round, which raised $31 million, was led by Xeraya Capital.

"Aria CV is now well positioned to drive our clinical research forward," Dan Gladney, the company's chief executive officer and president, said in a press release.

"We are grateful for this validation by our investors and remain committed to bringing this innovative treatment option to PAH patients," Gladney added.

Pulmonary hypertension (PH) is marked by pressure in the pulmonary arteries — the blood vessels that transport blood from the heart to the lungs — rising to levels that do damage and risk heart failure. Heart muscles have to work harder to pump blood into the lungs under high pressure, and become weaker over time.

The Aria CV PH System is designed to restore pulmonary artery health and elasticity, reducing the workload on heart muscles and increasing blood flow. The device consists of a balloon surgically placed into the pulmonary artery, and inflates and deflates according to the heart cycle.

Aria is planning to open a feasibility study accessing the long-term use of the implanted device in PAH patients.

"There is an unmet clinical need with patients suffering from pulmonary arterial hypertension, and we are impressed with the promising results the Aria CV PH System has demonstrated," Jason Rushton, a partner at Xeraya Capital, said. "We look forward to being a supportive partner."

Aria's medical device recently was designated a breakthrough device by the U. S. Food and Drug Administration (FDA). The designation helps to expedite the review and potential regulatory approval of medical devices that aim to treat life-threatening or irreversibly debilitating diseases. It also offers benefits to devices' manufacturers that include additional support and feedback from the FDA.

The company expects at its device will effectively address PH symptoms, with fewer side effects than other therapies.

The device also won the Transcatheter Cardiovascular Therapeutics (TCT) 2018 Shark Tank Competition for its novelty and potential to bring change to the field of cardiovascular medicine.

Other participants in the financing round included Longview Ventures, Catalyst Health Ventures, BioStar Ventures, Cedar Point Capital, Frontcourt Group, and three strategic investors.

  • Author Details
Margarida graduated with a BS in Health Sciences from the University of Lisbon and a MSc in Biotechnology from Instituto Superior Técnico (IST-UL). She worked as a molecular biologist research associate at a Cambridge UK-based biotech company that discovers and develops therapeutic, fully human monoclonal antibodies.
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Joana holds a BSc in Biology, a MSc in Evolutionary and Developmental Biology and a PhD in Biomedical Sciences from Universidade de Lisboa, Portugal. Her work has been focused on the impact of non-canonical Wnt signaling in the collective behavior of endothelial cells — cells that made up the lining of blood vessels — found in the umbilical cord of newborns.
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Margarida graduated with a BS in Health Sciences from the University of Lisbon and a MSc in Biotechnology from Instituto Superior Técnico (IST-UL). She worked as a molecular biologist research associate at a Cambridge UK-based biotech company that discovers and develops therapeutic, fully human monoclonal antibodies.
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