pulmonary hypertension Archives

Lung transplant expert Michael Tsifansky, M.D., F.A.A.P., joins Children’s

December 14, 2019

Michael Tsifansky

Earlier this year Michael Tsifansky, M.D., F.A.A.P., joined Children’s National Hospital as an attending physician in the Cardiac Intensive Care Unit and in the Division of Pulmonology and Sleep Medicine. He brings to Children’s National a unique mix of expertise in critical care and pulmonary medicine. That passion for these two subspecialties has also made him one of the country’s leading experts in lung transplant procedures and the recovery from them.

Dr. Tsifansky shared more information about caring for patients with complex lung diseases, especially those with end-stage lung disease. He outlines the patient population for pediatric lung transplants and the arduous process patients endure while waiting for a transplant, undergoing this major procedure, and then recovering from it.

What types of patients undergo lung transplant surgeries?

Lung transplantation in children is indicated when the following criteria are met:

End-stage lung disease
No reasonable alternative to the established diagnosis
No medical or surgical alternative to the current course of treatment
No other organ failure
Stable social environment

Could you describe the surgery process?

Pediatric lung transplantation may be performed on cardiopulmonary bypass, on extracorporeal membrane oxygenation (ECMO) or off extracorporeal cardiopulmonary support (ECS). The donor’s lungs are kept chilled prior to transplantation and should be transplanted within six to eight hours after removal from the donor. The donor’s main-stem bronchi and pulmonary arteries are connected to those of the recipient, and the donor’s pulmonary venous drainage is connected to the recipient’s left atrium using the donor’s left atrial roof tissue. This procedure typically takes six to eight hours.

Could you describe the recovery process?

Typically, pediatric lung transplant recipients are extubated and encouraged to sit up four to six hours after the transplant procedure and walk soon afterward. It is important that they be out of bed and moving as soon as possible, and our colleague from Rehabilitation Services (physical and occupational therapists and rehabilitation physicians) will be working with the children toward these goals. After transplantation, pediatric patients will be given discharge instructions with individualized guidelines for a healthy lifestyle. Patients should return to near-normal life approximately three to six months after transplantation.

How long does the recovery process take?

The patient will remain hospitalized for 11-14 days following surgery for acute rehab, titration of antirejection meds and initial healing.

You’ve mentioned that it’s important for transplant patients to get moving as part of recovery. When can a patient begin walking again?

Lung recipients will be assisted into a chair soon after the transplant. Within the first 24-36 hours, the patient is encouraged to take short walks, increasing the distance each day. A physical therapist will work with the patient during their hospitalization to meet their goals. We also encourage patients to exercise on the treadmill regularly while hospitalized. By the time the patient is ready to go home, he or she will be able to easily move around by themselves and do most of their care without assistance. They feel so much better than before transplant and have so much energy that we almost always have to gently limit their activity for a short while to allow their chest incision to heal properly.

What do you see as the next step in pulmonary care for end stage lung disease at Children’s National Hospital?

The development of a pediatric-specific lung transplant and respiratory failure program is the natural extension of the hospital’s cystic fibrosis program, heart transplant program and programs in pulmonary hypertension, bronchopulmonary dysplasia and extracorporeal membrane oxygenation for respiratory failure.

At present, there is no local option for a pediatric-specific program that can perform the transplant and provide the necessary comprehensive wrap-around services for patients in infancy up to age 18. As a top children’s hospital, Children’s National is uniquely positioned to provide the highest level of pediatric-specific care to this patient population and allow patients and their families to spend more time at home while undergoing this and other lifesaving treatments.

Dr. Tsifansky hopes to launch a comprehensive pediatric lung transplant and respiratory failure program at Children’s National in the very near future. Stay tuned for future developments from this area.

Coming together as a team for the good of the baby

March 12, 2019

Children’s National has a new program to care for children who have severe bronchopulmonary dysplasia, a serious complication of preterm birth.

Around the 1-year-old’s crib is a tight circle of smiling adults, and at the foot of his bed is a menagerie of plush animals, each a different color and texture and shape to spark his curiosity and sharpen his intellect.

Gone are the days a newborn with extremely complex medical needs like Elijah would transfer from the neonatal intensive care unit (NICU) to the pediatric intensive care unit and transition through a couple of other hospital units by the time he was discharged. Gone are the days when he’d see a variety of new physician faces at every stop. And gone are the days he’d be confined to his room, divorced from the sights and sounds and scents of the outside world, stimulation that helps little baby’s neural networks grow stronger.

Children’s National has a new program designed to meet the unique needs of children like Elijah who have severe bronchopulmonary dysplasia (BPD), a common complication of preterm birth.

“It’s more forward-thinking – and I mean thinking for the future of each individual baby, and it’s allowing the baby to have one team and one location to take advantage of a deep knowledge of and relationship with that baby and family,” says Robin Steinhorn, M.D. Dr. Steinhorn is senior vice president of the Center for Hospital-Based Specialties and one of Children’s multidisciplinary team members who visited Elijah’s bed twice weekly during his lengthy hospitalization and who continues to see him regularly during outpatient visits.

“The pulmonologist, the neonatologist, the respiratory therapist, the physical therapist, the dietitian, the cardiologist – we all come as a team to work together for the good of the baby,” Dr. Steinhorn adds. “We stick with these babies through thick and thin. We will stick with that baby with this team and this location until they are ready to go home – and beyond.”

BPD, a serious lung condition, mostly affects extremely low birthweight preterm babies whose lungs were designed to continue developing inside the womb until the pregnancy reaches full term. Often born months before their due dates, these extremely vulnerable newborns have immature organs, including the lungs, which are not ready for the task of breathing air. Children’s program targets infants who experience respiratory failure from BPD. The respiratory support required for these infants ranges from oxygen delivered through a nasal cannula to mechanical ventilators.

“It’s more forward-thinking – and I mean thinking for the future of each individual baby, and it’s allowing the baby to have one team and one location to take advantage of a deep knowledge of and relationship with that baby and family,” says Robin Steinhorn, M.D.

About 1 percent of all preterm births are extremely low birthweight, or less than 1,500 grams. Within that group, up to 40 percent will develop BPD. While they represent a small percentage of overall births, these very sick babies need comprehensive, focused care for the first few years of their lives. And some infants with severe BPD also have pulmonary hypertension which, at Children’s National, is co-managed by cardiology and pulmonary specialists.

Children’s BPD team not only focuses on the child’s survival and medical care, they focus on the neurodevelopmental and social care that a baby needs to thrive. From enhanced nutrition to occupational and physical therapy to a regular sleep cycle, the goal is to help these babies achieve their full potential.

“These babies are at tremendous risk for long-term developmental issues. Everything we do is geared to alleviate that,” adds John T. Berger III, M.D., director of Children’s Pulmonary Hypertension Program.

“Our NICU care is more focused, comprehensive and consistent,” agrees Mariam Said, M.D., a neonatologist on the team. “We’re also optimizing the timing of care and diagnostic testing that will directly impact health outcomes.”

Leaving no detail overlooked, the team also ensures that infants have age-appropriate developmental stimuli, like toys, and push for early mobility by getting children up and out of bed and into a chair or riding in a wagon.

“The standard approach is to keep the baby in a room with limited physical or occupational therapy and a lack of appropriate stimulation,” says Geovanny Perez, M.D., a pulmonologist on the team. “A normal baby interacts with their environment inside the home and outside the home. We aim to mimic that within the hospital environment.”

Dr. Steinhorn, who had long dreamed of creating this comprehensive team care approach adds that “it’s been so gratifying to see it adopted and embraced so quickly by Children’s NICU caregivers.”

PDE-5 inhibitors for pediatric hypertension

March 8, 2017

A study led by Chinwe Unegbu, M.D., indicates the benefits of PDE-5 inhibitors to treat pediatric pulmonary hypertension far outweigh potential harmful side effects.

Pulmonary hypertension (PH), when pressure in the blood vessels leading from the heart to the lungs is too high, is primarily a disease of adults: Patient registries suggest that the mean age of diagnosis is around age 50. However, more and more children are developing this condition, says Chinwe Unegbu, M.D., an assistant professor in the Division of Anesthesiology, Pain and Perioperative Medicine at Children’s National Health System.

Although adults with PH have several different effective treatments, Dr. Unegbu adds, children have few options. One of these is a class of medications known as phosphodiesterase type 5 (PDE-5) inhibitors, which act on molecular pathways that can open up constricted blood vessels. However, some studies have raised questions about the safety of this class of medications, particularly with long-term use of high dosages.

In a new study, Dr. Unegbu and colleagues performed a systematic review of available literature on this class of drugs evaluating their effectiveness and safety for pediatric patients. The review showed that like all medications, PDE-5 inhibitors have some risks. However, Dr. Unegbu says, the review showed that their benefits, including improved echocardiography measurements, cardiac catheterization parameters and oxygenation, far outweigh potential harmful side effects.

“Pediatricians across the nation view the rise in pediatric PH cases with growing concern because the disease can worsen, leading to right ventricular failure and death,” says Dr. Unegbu, lead author of the study. “PH can occur in newborns, infants and children who have a number of health conditions, including congenital heart disease, the most common birth defect among newborns. There are few available treatments for the growing population of children affected by this condition, so it is heartening that the evidence supports PDE-5 inhibitors for patients with PH.”

Patients with PH experience increased pressure in the pulmonary arteries, which carry blood from the heart to the lungs where it picks up oxygen that is ferried throughout the body. According to the National Institutes of Health, this leads patients to suffer from shortness of breath while doing routine tasks, chest pain and a racing heartbeat. Changes to the arteries make it progressively harder for the heart to pump blood to the lungs, which forces the heart to work even harder. Despite the heart muscle compensating by growing larger, less blood ultimately flows from the right to the left side of the heart which can compromise the kidney, liver and other organs, Dr. Unegbu says.

The study team included four researchers from Johns Hopkins University: Corina Noje, M.D., John D. Coulson, M.D., Jodi B. Segal, M.D., M.P.H., and study senior author Lewis Romer, M.D. The researchers scoured Medline, Embase, SCOPUS and the Cochrane Central Register of Controlled Trials, looking for studies that examined PDE-5 inhibitor use by pediatric patients with primary and secondary PH. Their goals included describing the nature and scale of the pediatric PH, assessing available pharmacologic therapies and conducting the systematic review of clinical studies of PDE-5 inhibitors, a mainstay of PH therapy.

They identified 1,270 studies. Twenty-one met the criteria to be included in the comprehensive review, including eight randomized controlled trials – the gold standard. The remaining 13 were observational studies in children ranging in age from extremely preterm to adolescence.

“Although there is some risk associated with PDE-5 inhibitor use by pediatric patients with PH, overwhelmingly the data indicate the benefits of using this class of drugs far outweigh the risks. When we looked at specific clinical outcomes, we see definite improvement in a number of measures including oxygenation, hemodynamics and better clinical outcomes: The patients are doing better, feeling better and their exercise capacity rises,” Dr. Unegbu says.

Because of lingering concerns about increased mortality, they also looked at toxicity data associated with this class of drugs. “With the exception of a single trial, the remaining trials included in our review did not demonstrate increased mortality in patients placed on this class of medicines, which was reassuring to us,” she says. Side effects ranged from mild to moderate, such as flushing and headaches. “We can say with a good degree of confidence that providers should feel fairly comfortable prescribing PDE-5 inhibitors.”

Ideally, researchers would like to have access to patient-specific measures that are a good fit for neonates and infants. Unlike adults, infants’ exercise capacity cannot be measured by their ability to climb stairs or use a treadmill. Another limitation, the study authors note, is the dearth of adequately powered clinical trials conducted in kids.

“Most of the studies have been conducted in adults. However, this disease unfolds in a much different fashion in children compared with adults,” Dr. Unegbu says. “We are desperately in need of high-quality studies in the form of randomized controlled trials in pediatric patients and studies that examine the full range of formulations of this class of drugs.”

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