THIS MONTH'S FOCUS:

PEDIATRICS
PULMONARY HYPERTENSION

(PDF VERSION)

Many thanks to Martin Schweiger & Amresh Raina for coordinating the content for this month's issue.




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VINCENT'S TURKEY SENSE

Starting In the Spotlight are the halftime reports from our President, Maryl Johnson, and our Program Chair, Jeff Teuteberg, who provide us important and insightful summaries of our vibrant, active and refining ISHLT for the better as we approach the 37th Annual Meeting and Scientific Sessions in San Diego. Next we are pleased to announce the launch of the New ISHLT Online Learning Platform. To get going with Pediatrics, Shriprasad Deshpande from Emory compels us to keep the right heart from being left out as we tend to the congenital heart disease population and Stephanie Handler shunts us around from the past to the present by providing us an historical account of the Pott's shunt from 70 years ago and how it is applied today. For Pulmonary Hypertension, it's Sophia Airhart and Amresh Raina of Allegheny Hospital in Pittsburgh describe the results of a multicenter trial of patients with pulmonary hypertension unburdened from the stigma of carrying a continuous infusion pump with the novel strategy of a totally implantable prostacyclin system. Then, in keeping with Thanksgiving, Ryan Davey delivers us from a famine, a feast of medical strategies for patients with pulmonary arterial hypertension before Kathy Tang and Guatam Ramani remind us of the fable from Aesop on the benefit of slow and steady over haste and waste with nearly a quarter of century of prostacyclin for pulmonary arterial hypertension. As Special Interest pieces, Lori Bowser shares her perspective with us of her father's journey with an LVAD, comparing him to Superman as he goes deer hunting as a hospice patient. Next, Maryanne Chrisant enlightens us from her recent interview with an atheist and his experiential perspective as a palliative care specialist from New York on pain and suffering before your Editor-in-Chief closes this issue on keeping the Arts in mind as we care for our patients with pain and suffering. Your dedication and devotion to your patients will always make a difference.

Happy Thanksgiving!

Vincent Valentine, MD
Links Editor-in-Chief



IN THE SPOTLIGHT:


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Your President's Halftime Report

Maryl R. Johnson, MD

It doesn't seem possible that I have reached the midpoint of my ISHLT presidency! During my first 6 months, I have continued to be impressed by the dedication of our members and the commitment of our leaders and staff to move the ISHLT forward. In late September, Amanda Rowe and I had a series of conference calls with the Council Chairs and Board Liaisons during which we learned of the challenges faced by them and tried to provide suggestions as to how important initiatives might be moved forward. Last weekend we further opened lines of communication when the ISHLT Board and Council chairs met at Normandy Farms Inn outside Philadelphia for a retreat and Board meeting. This was felt to be a grand success by all and hopefully will serve as a firm foundation for ISHLT leaders to work together more closely in the future. Read more →



Update from the 2017 Program Chair

Jeffrey Teuteberg, MD

The end of October brings colder weather, changing leaves, Halloween and the ISHLT abstract deadline. The 2017 Program Committee had a busy summer and fall preparing for the meeting in San Diego, but the final program is now complete and available online as a PDF or flip book. Once again I would like to thank the membership for all of their fantastic submissions, the Program Committee for all their time and diligence, and the ISHLT staff for their support and guidance. This year's Plenary Sessions will have more featured abstracts than in past years and the plenary presentations should have a little something for everyone, from stem cells, to social media, to novel means of funding drug development. Read more →



ISHLT NEWS AND ANNOUNCEMENTS:



New ISHLT Online Learning Platform Launched

links imageIn April 2016, the ISHLT Board approved a new Strategic Framework to guide the work of the Society over the next 5 years (add small version of attached graphic to LINKS). One of the Strategic Imperatives of this new framework is Enhance Membership Value. In this era of waning participation in membership organizations, ISHLT is fortunate to be experiencing membership growth. We want continue to grow the Society's membership, but we also want to retain our current members whose engagement and participation are highly valued. The aim of this strategic imperative is to make the current ISHLT programs and services more valuable to our members and to increase the number of programs and services available only to ISHLT members. As such, one of the Goals for delivering this Strategic Imperative is to Provide Access to Education Using Innovative Platforms. And one of the Objectives to achieve that goal is to Offer Access to ISHLT Educational Opportunities Throughout The Year. Read more →




FOCUSING ON PEDIATRICS:



High PVR and Pediatric Heart Transplantation: Right Heart Should Not Be Left Out!

Shriprasad Deshpande, MD, MS

links imageElevated of pulmonary vascular resistance is considered a relative contraindication for heart transplantation. As is true with other circumstances such as ABO incompatibility or very high PRAs, elevated PVR continues to be a moving target. The elevation of PVR is thought to be a remodeling response to chronic elevation of LV end-diastolic pressure and low cardiac output. In patients with congenital heart disease, additional factors such as Fontan physiology, abnormal pulmonary vasculature and chronic exposure to left-to-right shunts contributions to high PVR. Impact of elevated PVR especially on the right ventricle post heart transplantation is a critical area of concern that is under investigated. Read more →



What's Old is New Again: Palliative Potts Shunt in Children with PAH

Stephanie Handler, MD

Despite advances in medical therapy and continued approval of new drugs to treat WHO Group 1 pulmonary arterial hypertension (PAH) in adults, 5-year survival for pediatric patients is still only 74% [1]. Even with initial symptomatic improvement in patients on prostacyclin therapy, many experience a decline in right ventricular systolic function associated with worsening functional class due to disease progression. In those patients, lung transplantation has been the only option. The Potts shunt (direct anastomosis of left pulmonary artery to descending aorta) was first performed in 1946 at Children's Memorial hospital in Chicago by Dr Willis J Potts as a "blue baby" operation to provide pulmonary blood flow for cyanotic congenital heart disease. Palliative 'reverse' Potts shunt in which flow is directed from the pulmonary artery to the aorta was first described in 2 children with PAH in the New England Journal of Medicine in 2004 by Dr Blanc and colleagues [2]. Read more →




FOCUSING ON PULMONARY HYPERTENSION:



Reducing the Burden of Parenteral Prostacyclin Therapy: The Totally Implantable Prostacyclin System

Sophia Airhart, MD
Amresh Raina, MD, FACC

As clinicians caring for patients with pulmonary arterial hypertension (PAH) we have all experienced the challenging situation in which a patient should ideally be treated with a parenteral prostacyclin for advanced symptoms but was not felt to be an appropriate candidate or declined to start prostacyclin therapy. The reasons for this are varied, including a history of non-compliance, an inability or unwillingness to maintain an access site and long-term infusion pump, or an unstable social situation. In addition, in many patients, particularly young, active patients such as children and adolescents, there is sometimes a social stigma associated with carrying an infusion pump on a daily basis, and managing a parenteral prostacyclin can impact a patient's ability to do their daily activities such as playing sports, showering and swimming. Read more →



Switching Therapies for Pulmonary Arterial Hypertension and Challenges in Going from "Famine to Feast"

Ryan Davey, MD, FRCPC, FACC

Who would have thought that even a few short years ago we, as practitioners, would be relatively spoilt for choice in deciding which pulmonary vasodilator therapy we should select for our pulmonary hypertension patients? We certainly are far from the days of "calcium antagonism or bust" but recent years have seen the PH treatment paradigm further moved on to pressing and relevant questions such as whether to use upfront combination therapy and the how to integrate oral prostacyclin agents into our practices. Perhaps one of the agents that can cause many PH care providers considerable pause is when, how and in which PAH patients to use the soluble guanylate cyclase (sGC) stimulator, riociguat. By means of introduction for the uninitiated, riociguat acts to induce vasodilation in smooth muscle by both sensitizing the sGC enzyme to endogenous nitric oxide (NO) and also by direct stimulation of the enzyme. Read more →



20 Years of Prostacyclin Therapy in PAH: Slow and Steady Progress, but Still Not at the Finish Line

Kathy Tang, PharmD
Guatam V. Ramani, MD

As we learned from the tale of the tortoise and the hare, slow and steady wins the race. Patience, hard work, and tireless commitment to innovation and progress are the keys to success. Are we winning the race when it comes to the management of pulmonary arterial hypertension (PAH)? From one perspective, the management of PAH has come a long way since the development of prostacyclin therapy in the mid-1990s. We now have14 FDA approved therapies, utilizing multiple delivery routes and targeting different molecular pathways. Yet, progress has been slow compared to other chronic conditions. For example, treatment for hepatitis C (HCV) and human immunodeficiency virus (HIV) have leapt ahead over the past few decades as a result of several breakthrough therapies. Today, HCV can be cured in most patients, and those with HIV are living for decades following their diagnosis. By comparison, the management of PAH seems to be at 3 kilometers into a 5K race. Read more →




SPECIAL INTEREST:



Living with an LVAD - My Father's Journey

Lori Bowser
Manreet Kanwar, MD

In September of 2010, at age 70, my father was diagnosed with lymphoma. But before I introduce him as a patient, let me tell you about him as a person. My parents have now been married for 55 years and my dad was born in the house he still lives in. He had always been very healthy, strong, hard-working and robust - he could fix anything and he only missed work on extremely rare occasions. My dad loves to garden, hunt, and spend most of his day outside, no matter what the weather. His doctors have a nickname for him - Superman! After the initial shock of learning about his diagnosis, we got down to business and dad went through chemotherapy treatment with good results. He insisted that the port be placed in his left arm so he could still shoot his bow. But then we got more bad news. As a result of the chemotherapy, his heart was damaged and he developed congestive heart failure. Read more →



Pain and Suffering, Without Belief

Maryanne Chrisant, MD

"So, as an atheist, how do you explain and deal with pain and suffering?" I asked my friend, Jon, for his thoughts, as part of my exploration into how we filter pain and suffering through faith and religion. I've known Jon since we were in college together, thirty-something years ago. He's a palliative care physician in New York and daily navigates his way through pain and suffering. I'd like to say we were sitting in some downtown bar, but we were both driving to our respective homes and talking via mobile. "Like, how do I justify pain and suffering? What does it mean?" He paused. "Pain and suffering just happen. I can't do anything to stop it. Let me tell you what I do every day. I recently had a patient who had been in a vegetative state for over two years. He'd been this dynamic, brilliant, young guy in law school. If this guy had lived, he would've been another John Kennedy. Until, one day...he was just in the wrong place at the wrong time: totally freak accident. Read more →




EDITOR'S CORNER:

Enlightening Us on Pain and Suffering

Vincent Valentine, MD

links imageHealth care providers in the ISHLT caring for patients suffering from various heart and lung conditions tend to and deal with many who live with disease, experience pain, suffer with medication side effects, cope with attachments to machines, and will eventually die. Protocols have been established focusing on either infection and rejection or bleeding and clotting. Another focus has been adhering to protocols and guiding patients to comply with prescribed regimens to improve survival and optimize outcomes. An important focus is the scientific study of problems ranging primary graft dysfunction, acute cellular and antibody mediated rejection, chronic allograft dysfunction, the various infections and their categorizations, malignancies and countless other known and unknown medical consequences after replacement therapies. Progress has been made through innovation with substantial improvements. Read more →





Contact

Vincent G Valentine, MD
Editor-in-Chief
vgvalent@utmb.edu


Editorial Staff


"Although the world is full of suffering, it is also full of overcoming it."
— Helen Keller

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Disclaimer: Any opinion, conclusion or recommendation published by the Links is the sole expression of the writer(s) and does not necessarily reflect the views of the ISHLT.