THIS MONTH'S FOCUS:

PEDIATRICS
PULMONARY HYPERTENSION
MECHANICAL CIRCULATORY SUPPORT

(PDF VERSION)

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




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PRE-REGISTRATION DEADLINE

If you are planning to attend the ISHLT 38th Annual Meeting and Scientific Sessions, or one of the 2018 Academies, please keep in mind that the deadline to pre-register is Monday, March 12, at 11:59 PM EDT. Registrations received/postmarked after March 12 will not be processed and such registrants must go to Onsite Registration to register for the meeting. Higher onsite registration fees will apply. Fees for onsite Annual Meeting registration will be $300.00 more than the early bird fees, and fees for onsite Academy registration will be $100.00 more than the early bird fees. Avoid long lines and additional costs by registering NOW.

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VINCENT'S FLU SENSE

From about half a millennium to a century to today, we have yet to change our response and preparedness to threatening situations. Consider this -

When in danger, when In doubt -Run in circles, scream and shout.

This month, we reflect on the 20th century anniversary of the 1918 flu pandemic by offering an agitprop that stirs our emotional and physical responses. With contributions from Jaime-Jürgen Eulert-Grehn, Heather T. Henderson and Evgenij Potapov, we are motivated to explore educational and professional opportunities to prepare ourselves for holistic reactions. Nathan Verlinden takes on multiple perspectives in his article, "Generic Medications in Pulmonary Arterial Hypertension - the Good, the Bad, and the Ugly," and Michael A. McCulloch forecasts the future in his article, "Chronic Lung Disease Associated Pulmonary Hypertension- The Next Epidemic?" Martin Schweiger discusses "Cerebral Strokes in Pediatrics on Intra-Corporeal LAVDs," as Melissa Smallfield gives us an update on the "Management of Pulmonary Hypertension in LVAD Patients." Amresh Raina immunizes us with her article, "Pulmonary Veno-Occlusive Disease - From Clinical Ambiguity to Molecular Diagnostics," while Amy Sherman and Stephanie Pouch help me recap the progression of influenza over the past 100 years. Finally, Becca Holt and yours truly reiterate Shakespeare's prediction of immoral judgement in society.

We all know ring around the Rosie, how many recall:

There was a little girl, and she had a little bird,
And she called it by the pretty name of Enza;
But one day it flew away, but it didn't go to stay,
For when she raised the window, in-flu-Enza.

Vincent Valentine, MD
Links Editor-in-Chief



IN THE SPOTLIGHT:


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2018 Recipients of the ISHLT Leach-Abramson-Imhoff Links Travel Awards

Over the past year, the ISHLT again had a productive year from over 100 writers contributing to the ISHLT Links Newsletter. To deter us from analysis paralysis, we must decide, sometimes with "Big Decisions" but not always the right decision. From power dynamics and groupthink to group polarization, our decisions are subjected to biases resulting in decisions that could be hazardous to someone's life. A grid and a checklist will foster collaborative decision making to reduce hazards as we have decided on this year's Links' Writers of the Year. Congratulations from such big decisions and with what were you doing in 1996 to Pediatric Cardiac Prehab. Of course, the forever important and necessary discussions of difficult news with our pediatric transplant recipients shed light on our sensibilities about caring and compassion. In the end, simply showing up proves that it is all worthwhile. Read more →



FOCUSING ON PEDIATRICS:



Chronic Lung Disease Associated Pulmonary Hypertension- The Next Epidemic?

Michael A. McCulloch, MD

Advancements in neonatal intensive care unit management have improved average discharge rates to 55% following 24 week gestations and 92% for 28 week gestation [1]. However, as many as 68% of these 'NICU graduates' are diagnosed with bronchopulmonary dysplasia (BPD) and between 18 to 43% of these develop pulmonary hypertension [1,2,3]. This population's vulnerability becomes abundantly clear in mid-term follow up papers demonstrating 2-year mortality rates up to 40% [4,5]. A myriad of reasons exists for this sobering data. Echocardiographic assessments of right ventricular function and pressures are commonly inaccurate, complicating the diagnosis of right ventricular dysfunction and pulmonary hypertension [6]. Aspiration of oral or gastric contents are a frequent source of recurrent lung injury and oftentimes clinically silent [7]. Left ventricular diastolic dysfunction and pulmonary vein stenosis can significantly complicate the care of BPD patients, but are less common and often go unrecognized [8,9]. Read more →



Cerebral Strokes in Pediatrics on Intra-Corporeal LVADs

Martin Schweiger, MD, PD

links imageThere has been a rapid evolution of using adult designed continuous flow VADS (cf-VADs) to support pediatrics even in children with congenital heart disease. This comes with the need for care providers specialized in this field to determine optimal patient and device selection, and to improve outcomes and decrease complication rates for new innovative strategies. The Berlin Heart EXCOR® is and has been the mainstay of long term VAD support for children of all ages but its limitations, especially the risk of thromboembolic events, are well known. Data from adult experience have shown a significant decrease in neurologic dysfunction with cf-VADs compared with pulsatile VADs in adults [1]. There are very few data on outcome especially on cerebral strokes in children supported with adulted sized LVADs. Read more →




FOCUSING ON PULMONARY HYPERTENSION:



Pulmonary Veno-Occlusive Disease - From Clinical Ambiguity to Molecular Diagnostics

Amresh Raina, MD, FACC

links imageAs pulmonary hypertension (PH) clinicians, we are often faced with diagnostic challenges that force us to re-evaluate the physiology of a patient's disease. In the clinical realm, outside of randomized clinical trials, a patient's PH diagnosis can often be painted in shades of gray rather than with the clarity of black and white. Especially when patients do not respond to therapy in an expected manner, we often find ourselves reassessing the diagnosis and questioning common alternative diagnoses: I have often found myself pondering again "is it left heart disease?" "Is it chronic thromboembolic disease?" "Is it smoldering interstitial lung disease?" Consider the recent case of a 52-year-old man with an 18-month history of progressive dyspnea, extremity edema and presyncope. Read more →



Update on Management of Pulmonary Hypertension in LVAD Patients

Melissa Smallfield, MD

In the United States, there are 5.8 million people with heart failure, a small percentage of them with advanced heart failure requiring therapy with left ventricular assist devices (LVAD) and heart transplantation. A significant number of advanced heart failure patients will have secondary pulmonary hypertension due to the passive congestion of left heart disease. LVAD therapy is effective in decompressing the left ventricle, thus reducing left sided filling pressures and reducing pulmonary pressures. Unfortunately, patients may have combined pre- and post-capillary pulmonary hypertension (CpcPH) for which LVAD therapy does not entirely normalize PA pressures and pulmonary vascular resistance (PVR). CpcPH is defined as a mean pulmonary artery pressures (mPAP) > 25 mmHg, a pulmonary artery wedge pressure (PAWP) > 15 mmHg, a PVR ≥ 3 WU and a diastolic pressure gradient (DPG) ≥ 7 mmHg by hemodynamic testing. Read more →



Generic Medications in Pulmonary Arterial Hypertension - the Good, the Bad, and the Ugly

Nathan Verlinden, PharmD, BCPS

links imagePulmonary arterial hypertension (PAH) is a rare disease associated with a high burden of healthcare costs. In a recent analysis of costs in the United States, the average per-patient-per-month healthcare costs for patients with PAH were over $9,500 US dollars [1]. The average annual pharmacy costs in this study exceeded $50,000 per patient [1]. For patients requiring oral or parenteral prostacyclin therapy, medication costs can surpass $100,000 annually. The availability of generic medications to treat PAH might be expected to help reduce medication and overall healthcare costs. This article will discuss the current state of generic medications for PAH and some of the challenges for generic availability with a specific focus on issues in the US. Read more →




FOCUSING ON MECHANICAL CIRCULATORY SUPPORT:



Right is the New Left, Right?

Jaime-Jürgen Eulert-Grehn, MD

As a resident in cardiac surgery from a northern European center with a large amount of experience in mechanical circulatory support (MCS), I am especially looking forward to the ISHLT Annual Meeting in Nice and the sessions about MCS. I attended the Master Class in MCS at the last meeting in San Diego this year, and now I wish to join the ISHLT Academy session on Core Competencies in MCS one day before the annual meeting really gets started. This makes the ISHLT meeting unique for residents interested in MCS: the combination of educational and state-of-the-art scientific sessions. The next day, after recouping energy at the Junior Faculty Mentor Lunch, the next session for me will be "The devil wears Prada - The role of the RV in advanced heart failure and LVADs." Our team for implantable electronic cardiac devices faces on a daily basis many questions concerning the management of such devices in LVAD patients, and my next stop, on Thursday at 2 pm, will be "It's Electric! Electrophysiological management of the LVAD patient." Read more →



My Nice 2018

Evgenij Potapov, MD, PhD

links imageOur Congress in Nice is fast approaching. My high expectations of the congress have not lessened in the past 20 years, and I have been asking myself why. For me, the aspect I look forward to most is the opportunity to meet my colleagues and close friends, to chat with them personally and have some beautiful moments under the starry sky of Nice which will remain with me forever. What do I mean? At the beginning of the congress, participation in the ISHLT Academy as a faculty member is very challenging. It is not easy to squeeze all one's knowledge about a particular topic into the requisite 15 minutes, but it is even more challenging to make the presentation easily intelligible and to field the questions that follow. This day is full of vibrant presentations, fresh ideas and questions fired by nimble-minded students. Read more →




ISHLT NEWS AND ANNOUNCEMENTS:


ISHLT 2018 GRANTS & AWARDS WINNERS ANNOUNCED

Click here to see a full list of the 2018 Grants & Awards Recipients




2018 COUNCIL MEETINGS AND NETWORKING RECEPTIONS

Click here to the times and locations of all Council Meetings and Networking Receptions taking place at the 2018 ISHLT Annual Meeting



Tweeting at #ISHLT2018

links imageWant to get even more out of ISHLT 2018? Twitter can help!

Reading on Twitter about what others are learning in sessions is the easiest way to start. If you're new to Twitter, CLICK HERE for easy to understand information on how to set up a Twitter account, how to read what others are posting, and how to post your own comments.

If you want to really engage, you can share comments about what YOU are learning. Share your conference experience and use #ISHLT2018 to connect with attendees, build professional relationships, uncover ideas, spark inspiration, and help others!

A #ISHLT2018 Twitter feed will be scrolling across the bottom of the mobile meeting app home page all day every day to provide real-time commentary and information.



2018 June International Traveling Scholarship Application Now Open!

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SPECIAL INTEREST:



ISHLT Joint Session at American Heart Association Scientific Sessions

Heather T. Henderson, MD

links imageIn October 2014, I wrote of the progress in the field of pediatric heart failure and highlighted the creation of the American Heart Association's (AHA) Cardiovascular disease in the Young (CVDY) pediatric heart failure subcommittee. As previously stated, the goal of this committee is to support the mission of the CVDY council to "improve the health of children with heart failure or cardiomyopathy through research, education, prevention, advocacy and quality improvement." The committee is supported by members of the CVDY council and from other councils within the American Heart Association, including a liaison with the ISHLT. The committee focuses on education and programming dedicated to pediatric heart failure for AHA scientific sessions and other conferences. The group continues to explore opportunities to develop scientific statements and open the doors for collaboration with other pediatric heart failure groups. Read more →



Influenza - 100 Years

Amy Sherman, MD
Vincent Valentine, MD
Stephanie Pouch, MD

1918: Shifts and Drifts in Disease and History
An incomprehensible but important fact about the Spanish flu is it killed over 3% of the world population in less than two years. Nothing; no infection, no war, no famine nor any calamity has killed so many by something so small in so little time. The mortality surpassed the deaths seen in World War I, with an estimated 50-100 million people worldwide and 675,000 in the United States who succumbed to the illness [1]. Deaths were largely due to respiratory complications, such as secondary bacterial pneumonias; however, unlike prior influenza pandemics, the 1918 pandemic presented with more aggressive and fatal complications such as severe bronchopneumonia and progressive cyanosis [2]. Another surprising aspect of this pandemic was the excess mortality in otherwise healthy, young patients between 20-40 years old with a lower than expected mortality among the elderly. It also likely changed the course of history. Read more →




WORD OF THE MONTH:

Agitprop

(noun) - Political propaganda delivered through art, music, drama, or literature.




EDITOR'S CORNER:

The Beetle and the Giant

Rebecca Holt, BA
Vincent Valentine, MD

links imageHave you ever felt wedged between a rock and a hard place? If you work in healthcare, your answer is likely yes. The adage refers to a situation offering two objectionable outcomes. As clinicians, we are forced into this uncomfortable and distressing environment daily. With arduous patient diagnoses, insurance regulation and universal healthcare, we often fight with a double-edged sword. Among the sea of urgency, the physical and emotional demands are challenging enough. With physicians, patients and families are forced to make tough decisions where results could render both negative outcomes. For patients suffering from disease, it's either morbidity or mortality. Long after treatment, patients are still dealt the hand of the Joker, and at times, families have to play God. Read more →





Contact

Vincent G Valentine, MD
Editor-in-Chief
vvalentine@uabmc.edu


Editorial Staff


"The worst pandemic in modern history was the Spanish flu of 1918, which killed tens of millions of people. Today, with how interconnected the world is, it would spread faster."
— Bill Gates

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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.