IPAH, Poor Heart Function Linked to Early Death After Transplant, Study Finds - Pulmonary Hypertension News

IPAH, Poor Heart Function Linked to Early Death After Transplant, Study Finds - Pulmonary Hypertension News
Pediatric PAH Diagnosis and Management: A Clinical Roundtable - Pulmonology Advisor
Describing and Managing Severe Pain - Pulmonary Hypertension News

Posted: 29 Apr 2019 02:30 AM PDT

Certain parameters of poor heart function are risk factors for early death among idiopathic pulmonary arterial hypertension (IPAH) patients who undergo lung transplant, a small Chinese study suggests.

The study, "Risk Analysis of Perioperative Death in Lung Transplant Patients With Severe Idiopathic Pulmonary Hypertension," was published in the journal Transplantation Proceedings.

Lung transplant remains the most effective treatment to improve an IPAH patient's survival. However, the death rate associated with the procedure is high: 2015 estimates from the International Society for Heart and Lung Transplantation Registry show that 23% of patients with IPAH die within three months of their lung transplants.

Researchers in the study decided to investigate the risk factors associated with an early death among IPAH patients undergoing lung transplants.

The study enrolled 22 critically ill IPAH patients who underwent lung transplants at the Affiliated Wuxi People's Hospital of Nanjing Medical University, between November 2007 and October 2016.

The mortality rate for these patients was 22.99% three months after their transplants. The researchers then divided patients into two groups: an early death group that included five patients who died within three months following transplant, and a control group with the 17 remaining patients.

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When comparing potential risk factors between both groups, the researchers saw that patients who died within three months of the transplant had a significantly higher frequency of syncope — a sudden transient loss of consciousness (fainting) — and lower salt (sodium) concentration of 116.80 mmol/L in their blood (a condition called hyponatremia), when compared with the control group, which had a sodium concentration of 129.88 mmol/L.

Syncope frequency is a symptom that can arise from several underlying causes, including cardiac problems.

The researchers then analyzed patients' cardiac characteristics before surgery. The cardiac index, which measures the amount of blood pumped from the left side of the heart to the whole body and is proportional to heart performance, was lower in the early death group (1.30 L/min/m2) compared with the control group (1.58 L/min/m2).

Also, an echocardiogram of the heart showed that the right ventricle (the heart's right chamber) was enlarged in the early death group, which indicates a lower ability of the heart to pump out blood, often leading to blood supply complications. The heart's left ventricle was also smaller in the early death group.

The results suggest that "lung transplant patients with severe idiopathic pulmonary hypertension are at higher risk of perioperative mortality," and that "high frequencies of syncope, hyponatremia, lower CI [cardiac index], inner diameter of the LV [left ventricle], and upward RV/LV [right ventricle/LV]" are risk factors for an early death in this patient population, the researchers wrote.

According to the team, the data "support the need for a careful assessment of cardiovascular risks before lung transplant to improve IPAH patients' outcomes," they concluded.

Pediatric PAH Diagnosis and Management: A Clinical Roundtable - Pulmonology Advisor

Posted: 03 Apr 2019 12:00 AM PDT

Because of the low incidence of pediatric pulmonary arterial hypertension (PAH; 0.48-0.70 per million children per year), high-quality studies in this population have been scarce.¹ The severity and complexity of the disease requires management by a multidisciplinary team at specialty centers, with suggested follow-up ≥3 months following diagnosis and twice annually once the patient is considered stable.¹

Findings from the Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry have shown that the most common symptoms in pediatric PH are dyspnea on exertion and fatigue, and syncope affects up to 31% of patients.¹ The optimal treatment approach for PAH depends upon the severity and underlying etiology of the disease. Study results point to idiopathic PAH (IPAH), heritable PAH, and PAH associated with congenital heart disease as the most common causes of PAH in children; these 3 diagnoses represented 93% of patients in the TOPP registry.^1,2

However, this "may be explained by a diagnostic approach that has not excluded all possible forms of PAH and overdiagnosed IPAH," noted the authors of a review published in Expert Review of Cardiovascular Therapy.¹ PAH may also be associated with connective tissue disease, systemic disease, or infectious disease, as well as certain toxins and drugs (eg, serotonin reuptake inhibitors in persistent pulmonary hypertension of the newborn).

To learn more about the specifics of pediatric PAH, Pulmonology Advisor interviewed the following clinicians: Sharon A. McGrath-Morrow, MD, MBA, professor of pediatrics at Johns Hopkins' Children's Center and program director of the Pediatric Pulmonary Fellowship Program at Johns' Hopkins Hospital in Baltimore; Catherine Avitabile, MD, an attending cardiologist in the Cardiac Center at the Children's Hospital of Philadelphia, and assistant professor of pediatrics at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia; and John C. Carl, MD, chair of the Center for Pediatric Pulmonary Medicine at Cleveland Clinic Children's in Cleveland.

Pulmonology Advisor: What are some of the diagnostic and treatment challenges in pediatric PAH?

Dr McGrath-Morrow: Pediatric PH is a rare disease and most of the drug trials for PH are conducted in adults and not children, so most of the PH-specific therapies used in children have been based on adult trials. In addition, most studies performed in children with PH are not randomized controlled trials.

Since PH due to chronic lung disease is a common etiology of PH in children, recognition and correction of factors contributing to the chronic lung disease and lung injury may be instrumental in treating the underlying PH. For example, infants with chronic lung disease of prematurity may develop PH due to factors that limit lung growth, and treating conditions that limit lung growth — such as aspiration, poor nutrition, and inadequate gas exchange — may improve PH outcomes in this population.

Since PH is so rare in children, these patients should be referred to tertiary pediatric centers with expertise in the treatment of pediatric PH.

The etiology of pediatric PH in children is often different than that in adults, and diagnosis and treatment of children with PH may be delayed because of its rarity. Children may present with nonspecific symptoms such as failure to thrive, irritability, exercise intolerance, syncope, and cyanosis.

Causes of pediatric PH commonly include idiopathic PH and PH associated with congenital heart disease, congenital diaphragmatic hernias, and underlying chronic lung disease, including severe bronchopulmonary dysplasia (BPD), whereas adult patients with PH are more likely to have PH associated with connective tissue diseases, HIV, and thromboembolic diseases.

Dr Avitabile: Pediatric PH is different from adult PH in several ways. The etiologies of pediatric PH are extremely diverse. Many originate in prenatal or perinatal vascular disease. The causes of pediatric PH include, but are not limited to, developmental heart, lung, and vascular problems such congenital diaphragmatic hernia and BPD; congenital heart defects (with and without left to right shunts); PH in the setting of complex genetic syndromes; and isolated PAH, which may be idiopathic or inherited in some cases.

Infants and children may present with PH at any time in childhood, depending upon the etiology. Comprehensive diagnosis includes birth and perinatal history, family history, electrocardiogram, chest X-ray, echocardiogram, exercise testing (6-minute walk test [6MWT] and cardiopulmonary exercise testing), computed tomography (CT) angiogram, ventilation-perfusion (VQ) scan, cardiac catheterization, and cardiac magnetic resonance imaging (MRI) in some cases. These tests may be tailored based upon age at presentation and a patient's stability.

While cardiac catheterization with acute vasoreactivity testing (AVT) is the gold standard in adult PH, we cannot always catheterize critically ill neonates such as those with congenital diaphragmatic hernia or BPD. Therefore, in many cases, drug treatments are started without catheterization data, relying instead on noninvasive data from echocardiograms.

However, cardiac catheterization is still essential to obtain hemodynamic data, rule out structural problems — such as patent ductus arteriosus, other shunts, or pulmonary vein stenosis — that may complicate PH treatment in infants and neonates and to understand the contribution of left ventricular heart disease to the PH. The role of AVT testing is not clear in all forms of pediatric PH (eg, the population with BPD) and the correct criteria for defining responders is also unclear, as these may not be the same as those used to define adult responders to calcium channel blockers.

Medical treatment of pediatric PH includes right ventricular inotropic support (with dopamine or milrinone in some cases) and pulmonary vasodilator therapies. We use inhaled nitric oxide, as well as phosphodiesterase 5 inhibitors, endothelin receptor antagonists, and prostacyclins, but few drugs are approved for pediatric indications. We need more clinical trials with innovative designs that will test drug efficacy in a rare disease with a reasonable timeline.

One surgical treatment that has promise is the Potts shunt, a central shunt originally designed to increase pulmonary blood flow in congenital heart defects in which there is pulmonary atresia or severe stenosis.^3,4 In PH, a surgically placed Potts shunt can act in the reverse way, like a ductus arteriosus, and allow some blood to bypass the lungs, potentially unloading the right ventricle at the expense of lower extremity cyanosis.

Dr Carl: The incidence and pathophysiology of PH in pediatric patients is very different than in adult patients. Pediatric PH with cardiac disease affects far more children than idiopathic PH. Current definitions of BPD are based on center-specific preferences for using oxygen; there are fundamental shortcomings in defining disease by management without an association with underlying pathophysiology, disease progression, or phenotype variability.⁵

Diagnostic limitations in pediatrics include:
Pulmonary function limitations: younger patients are unable to perform standard pulmonary function tests, reproducible infant pulmonary function testing is difficult in more critically ill children, and the 6MWT is limited^.1
Echocardiography, particularly in neonates, is much easier to obtain serial measures, but it is much less sensitive that catheterization data.
Serial measurement of pharmacologic outcomes of therapies is more difficult.

Pulmonology Advisor: What are key differences in the screening and management of pediatric vs adult PAH patients?

Dr McGrath-Morrow: In children, screening involves identifying specific pediatric conditions that may be contributing to the development of PH, such as chronic lung disease, cardiac shunts, congenital heart disease, and congenital diaphragmatic hernias. Echocardiography is an important screening tool for PH, particularly in preterm infants with chronic lung disease.

Cardiac catheterization in pediatric PH is still the gold standard for diagnosis and risk stratification and allows for AVT. Cardiac catheterization is recommended prior to initiating vasodilator therapy. However, the risk for adverse events with cardiac catheterization in preterm infants with BPD can be high and must be weighed against the benefit of the information gained.

Unique to preterm infants is the development of PH secondary to pulmonary vein stenosis, which can be associated with poor outcomes. Biomarkers for pediatric PH are less defined than adult PH.

Management of PH in children often includes the use of targeted pharmacologic therapies including phosphodiesterase inhibitors, endothelin receptor antagonists, and prostacyclin analogues, all based on studies of adults with PH. Dual therapies may also be beneficial in children with PH.

Treatment algorithms for children have been traditionally based on adult algorithms. Pediatric-specific algorithms are currently being developed to help provide a guideline approach to the management of PH in children.

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Describing and Managing Severe Pain - Pulmonary Hypertension News

Posted: 12 Apr 2019 12:00 AM PDT

"Rate your pain on a scale of 1 to 10, 10 being the worst you ever felt."

If I have learned one thing over the past few decades, it's that pain is almost guaranteed when living with chronic illness and pulmonary hypertension. I have been through a wide range of physical pain in the past 27 years.

I deal with many "pains" while in pain. These include describing and rating my pain level, knowing the agony can get worse, staying on top of my discomfort and learning to manage it, and getting some type of control over my anger when I experience physical distress.

It's difficult to find the right words to accurately describe pain. That being the case, doctors often ask for a number to rate it. Many do not realize that rating it is even more difficult than describing it.

At age 18, when an ovarian cyst ruptured, I told the doctors, "This is the worst pain I've ever felt." I gave my pain a 10. By assessing what I have been through since that age, I would change that rating to a 6.

Now when I experience a great amount of discomfort, I find myself questioning, "Am I comparing the pain to my worst ever? Even though it's not my worst pain ever, would giving a 6 get me the same care as saying a 9 or 10?"

Another of my "pains" is that the suffering can get much worse. It can increase in severity, intensity, and duration. There was a time when I thought I wouldn't be able to physically tolerate much more. I didn't think pain could get worse … until it did.

Now I know the pains of waking up after bypass surgery and not being able to move my neck. I know the pains of getting my ovaries and fallopian tubes removed. I have felt the soreness of waking up with a PEG tube placed in my stomach. I have felt chest aches that takes my breath away.

One thing is true for all types of pain: My body adapts to different levels. At some points, I feel like it even "normalizes" the discomfort.

Agony is not something that anyone should have to get used to. When it started to become my "new normal," I had a hard time managing it and staying on top of it.

When I had severe suffering, it was difficult to take pain medications prescribed by my doctors because I feared dependency problems. I feared the stigma around pain medication, and I was stuck thinking about the dangers of addiction to these substances. I've learned the hard way that managing severe pain by taking medication as prescribed is the right thing to do.

Not only does pain impact the body and my physical state, but it affects my mental state, too. Suffering increases my anger, makes me externalize my inner struggles, and brings out the worst in me. I cry at everything and anything, take things personally, and feel as though my emotions heighten.

Knowing this about myself, I give those around me fair warning: I'm honest about my mental health while managing hurt. My close friends and family know that the "Britt in pain" is not the same as the "real Britt."

My body doesn't want to endure more physical stress than it manages every day. Fighting through chronic illness and pulmonary hypertension is enough to stick my body in a constant state of exhaustion. When pain is added to this, my body gives out warning signs that it's fighting too hard.

My "pains" have taught me to be honest with my healthcare team about how great my hurts are. I'm learning to manage it without shame and fear. It's helpful to give my family and friends a warning when I am in distress because torment changes me. I remind myself that pain is not my fault. The body, like the mind, needs a break in order to heal.

***

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Hypertension News or its parent company, BioNews Services, and are intended to spark discussion about issues pertaining to pulmonary hypertension.

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