Hypertension in Pregnancy: Diagnosis, Blood Pressure Goals, and Pharmacotherapy: A Scientific Statement From the American Heart Association

Children With PH Say They Can Stay Active If Exercise Is Made Fun, Social

Children with pulmonary hypertension (PH), characterized by high blood pressure in the lungs that can make exercising difficult, are more likely to be active if physical activity is fun, allows them to be with friends, and puts them in a safe place where they feel included, a study has found.

Barriers to physical activity include fear, anxiety, self-consciousness, and lack of interest. Children are less motivated by health benefits and more by external rewards, like video game time or money. Programs that consider these motivations may help them stay active, findings suggest.

The interview-based study, "Barriers to and facilitators of physical activity in pediatric pulmonary hypertension," was published in the journal Pulmonary Circulation by a team of researchers from the University of Pennsylvania and the Children's Hospital of Philadelphia.

Children with PH don't usually engage in physical activity, even though supervised exercise programs have been recommended for adult patients to reduce blood pressure in the pulmonary arteries and improve quality of life.

In PH, reduced blood flow through the pulmonary arteries can overtax the heart and cause symptoms such as shortness of breath. "While physical limitations may prevent children with PH from participating in physical activity," other reasons for not exercising as much are not well-known, the team noted.

30 children interviewed about their independence, confidence, belief in ability

To understand what children with PH think about physical activity, the researchers interviewed 30 patients (17 girls, 13 boys) with mild to moderate PH, median age 14, along with their caregivers, about their independence, confidence, and belief in their ability to be active.

Children were more likely to be active if they enjoyed the type of physical activity. One child said their ideal exercise program would include "rock climbing," while another caregiver mentioned biking, "but not necessarily [a] stationary bike." Another child said, "If I find it interesting, I'll do it on my own time."

Caregivers noted children were more likely to stick with physical activity they already knew and were comfortable with. One caregiver mentioned how their child avoided sports because they realized "he's not like those other kids."

Yet, caregivers saw value in blending familiar types of physical activity with new ones to build confidence. "We're going to do something like strength training or we're going to do something like yoga or we're going to do something like boxing," said one caregiver, seeking to push their child out of their comfort zone and be spontaneous.

Many children enjoyed physical activity more when it involved friends or peers, that is, social engagement encouraged them to be more active. The "social part [of exercising] is huge," one caregiver said. Another caregiver said their child was more likely to participate when "there's a little healthy peer pressure."

Seeing children safely push their limits brought joy to their caregivers

Seeing their child safely push their limits brought joy to caregivers. One caregiver felt "a tremendous amount of … gratitude" when seeing their child exercise beyond expectations.

Others shared similar feelings when their children tried their best: "It makes me feel good as a mom… he is pushing to his limits."

Self-motivation for physical activity was less about health for many children and more about external rewards. One child was motivated by seeing progress, such as "building up a little more stamina." However, others were driven by rewards like "play video games" or "money or computer time."

Both children and caregivers valued environments where they didn't feel singled out because of PH. They wanted to blend in while still receiving medical attention when needed. Adults should allow "[a] child to stop exercising when they said they needed to stop," the researchers wrote.

Most children said their caregivers or doctors did not stop them from being active. More often, children avoided physical activity due to fear, anxiety, or a lack of interest. For example, one child described feeling scared when short of breath: "I got super out of breath… it [felt] like something was gonna happen."

Not all children were interested in physical activity, which acted as a barrier. Feeling self-conscious also limited physical activity. One child said, "If somebody is watching me, maybe it makes me a little uncomfortable."

Most patients were positive about a regular exercise program, but preferences varied. Some preferred scheduled routines, while others wanted flexibility. For example, one child liked exercise when "I'm feeling it."

For the researchers, "integrating these rich qualitative data on barriers to and facilitators of activity in pediatric PH are critical to informing future exercise interventions," which they wrote should "focus on patients' interests, cultivate confidence, respect limitations, and acknowledge the need for extrinsic incentivization." 

'My Son Has Rare Life-limiting Hypertension - But People Don't Get It Because They Can't See It'

Sarah Williams and her son Archie, who has pulmonary hypertension (

Image: Pulmonary Hypertension Association)

A mother is calling for a greater recognition of hidden disabilities after her nine-year-old suffered several challenges relating to his condition.

Archie, nine, has pulmonary hypertension (PH), which leaves him suffering with breathlessness, issues with mobility, and debilitating fatigue and exhaustion. PH - which is high pressure in the heart and lung blood vessels - is considered a hidden disability and to look at Archie, you would think he was a healthy nine-year-old lad.

Sarah Williams, from Greenfield in Flintshire, is taking part in an awareness day in the hopes of encouraging understanding about the condition, as well as the difficulties of living with an invisible illness. PH DAY UK takes place on Friday, November 1, and is organised by the Pulmonary Hypertension Association (PHA UK), who support individuals affected by PH. Sarah said: "People don't understand why Archie can't do what other kids do, because he looks fine."

She added: "If he was in a wheelchair, it would be obvious, or if he was wearing an oxygen mask then people would ask questions. But all they see is a pale little boy who doesn't go out much. Simply walking to school is a huge struggle for him, but no-one has ever heard of PH so they don't get how serious it is. It's taken Archie's childhood away from him, but because it's an invisible illness, people just don't get it."

Dr Iain Armstrong, chairman of the Pulmonary Hypertension Association (

Image:

Pulmonary Hypertension Association)

Sarah has a Blue Badge to park in disabled bays with her son Archie, but has been challenged by some people questioning her use of the spaces, reports North Wales Live. Archie has a 16-year-old brother and a three-year-old sister, but is unable to play with them because of his illness.

"Archie's PH has changed everything for our family, Sarah said. "We can't even all go to the park together because it's too much for him." Explaining the impact of his condition, she said: "He falls asleep at school, and he can't join in at playtime – he just can't do things that other children do, and what he should be doing. He's alive, but it doesn't always feel like he is living."

Diagnosed with pulmonary hypertension (PH) at five, Archie is treated at Great Ormond Street Children's Hospital in London, the sole UK hospital specialising in care for children with this condition. He is on two types of medication to manage his symptoms; however, there is no cure, and the condition is expected to shorten his life.

Sarah said: "The progressive side of the illness is the hardest thing to take. But despite everything, Archie just gets on with things. He's so inspiring, and such a character. I just wish people knew more about PH and understood that not all disabilities are visible."

PH affects only 8,000 people in the UK. While it can be associated with other diseases, many cases of PH develop "out of nowhere". Dr Iain Armstrong, chair of the Pulmonary Hypertension Association UK said: "It is devastating for parents to find out their child has PH. Very few people have heard of it and those with the condition often appear well from the outside. It's important for the general public to understand the challenges of living with a hidden illness, and we are grateful to Sarah for sharing her family's story."

Patent Ductus Arteriosus: Clinical Features In Pediatric And Adult Populations

Although the majority of congenital heart diseases (CHD) are diagnosed during childhood, defects can remain undiagnosed until symptoms present in adulthood or abnormalities are found incidentally on imaging.

Patent ductus arteriosus (PDA) is the most common cardiac condition in infants born before term, with the incidence of PDA inversely related to gestational age.1 Although PDAs may become evident shortly after birth in newborns with hemodynamically significant defects, prompting earlier diagnosis, it is important to consider PDA as a diagnosis in older populations as well. Evidence suggests that PDA comprises anywhere from 5% to 10% of all CHD.1  Thus, it is crucial for clinicians to recognize PDA in older children and adults in the primary care setting.

What is a PDA?

Patent ductus arteriosus is a congenital heart defect in which the ductus arteriosus (DA) does not close properly after birth. The DA, a fetal vessel that connects the pulmonary artery to the descending portion of the aorta, forms at around 8 weeks gestation.1 This structure shunts blood away from the fetal pulmonary system and towards the placenta, ensuring sufficient oxygenation of fetal blood.1

After birth, the DA is no longer needed because the infant's lungs are able to oxygenate the blood. Under normal physiologic circumstances, the DA usually closes functionally within 4 days after birth.1 If it fails to close, it is termed a patent ductus arteriosus (Figure). The origin of most complications of a PDA is the creation of a left to right shunt in the heart.1 The potential complications of a PDA in a preterm infant include intraventricular hemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis, and an increased need for respiratory support.1

Figure. Anatomic features of a normal heart (left) and patent ductus arteriosus (right). Credit: Used with permission of Mayo Foundation for Medical Education and Research, all rights reserved.   

A number of mechanisms assist in the closure of the ductus arteriosus after birth. The DA has what is described as intrinsic tone.2 The dilating factors required to keep it open throughout fetal development decrease after birth through the rise in oxygen pressure as the infant takes a breath, leading to the constriction of smooth muscle within the DA and facilitating closure.1-3 Beyond this functional mechanism leading to closure of the DA, an anatomical mechanism of closure occurs through apoptosis of the smooth muscle,3 which results in the development of a structure known as the ligamentum arteriosum.2

Although PDA is a common cardiovascular condition among preterm neonates, there is no clear consensus on whether to treat it and by what modality. Pharmacologic, surgical, and conservative management approaches are available. Conservative management consists of "watching and waiting" for spontaneous closure without the use of pharmacologic or surgical therapies.2 The 3 most common medications used for the pharmacologic treatment of PDA are indomethacin, ibuprofen, and acetaminophen. These prostaglandin inhibitors work to constrict the smooth muscle of the DA, facilitating ductal closure.3 Other, less conservative, treatment options include surgical ligation and transcatheter closure. ​​Generally, pharmacologic therapy is considered first in preterm infants, saving surgical repair for those who do not respond to medications.4 However, term infants and older patients may not respond to pharmacological treatment. Thus, if intervention is needed, these populations may benefit from surgical ligation or a transcatheter approach for definitive closure.1 

Shared Clinical Features in Pediatric and Adults

A classic sign of PDA on auscultation is a continuous, "machinery" murmur that is best heard at the left upper sternal border. A PDA may be suspected in a neonate or infant with hypoperfusion demonstrated by oximetry. Patients with PDA also may be tachypneic or have difficulty weaning off respiratory assistance. Another physical examination finding is a widened pulse pressure, such that systolic blood pressure is maintained at a normal range but diastolic pressure is decreased.5 A moderate to large PDA can cause left ventricular dysfunction or left atrial/ventricular enlargement. Additionally, renal and hepatic sequalae are possible.1,3,5

How Do Patients Present With PDA?

Preterm infants, older children, and adults with PDA all may present with the classic features described above. However, patients may remain asymptomatic until they reach adulthood when hypertension develops. Systemic hypertension creates pressure that increases the left-to-right shunting from the PDA. As a result, symptoms may manifest for the first time in adulthood.6

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Although PDA is a common cardiovascular condition among preterm neonates, there is no clear consensus on whether to treat it and by what modality. Pharmacologic, surgical, and conservative management approaches are available.

Another potential presentation of unrepaired PDA is Eisenmenger syndrome (ES), though rare in children,7 which results in severe pulmonary hypertension.8 As described earlier, a hemodynamically significant PDA usually causes a left-to-right shunt.1  However, in ES, the shunt reverses to a right-to-left shunt.8 This reversal is due to pulmonary resistance surpassing systemic resistance.1 This syndrome can be life threatening because unoxygenated blood is distributed systemically.6

Overlap exists between the symptoms of hemodynamically significant PDA and ES. However, a clinical finding that is considered pathognomonic for ES in a patient with PDA is clubbing of the toes with sparing of the fingers.1 A retrospective study conducted in China demonstrated that the risk of ES sharply increased after the age of 15 in patients with PDA that had not been repaired.7 This highlights that older children and adults may present with symptoms of ES before being diagnosed with CHD. Due to the significance of this syndrome, it would be best to prevent it by early detection and intervention for PDA and other CHD.

Importance and Primary Care Perspective

Patent ductus arteriosus may be detected early in an infant's life. However, as described earlier, it is possible for patients to not experience any symptoms or exhibit any signs until later in life. Adults with associated pulmonary hypertension can present with a diastolic decrescendo murmur or even more severe signs and symptoms of ES.1 It is important that PDA remains on the differential in adults and children alike. Although it may not be the most likely diagnosis, it cannot be ruled out in older children and adults based solely on age. This is especially true in the primary care setting when clinicians are differentiating potentially innocent murmurs from pathological murmurs. Primary care practitioners should recognize these findings and refer patients to cardiology when appropriate. To do this, each provider must diligently consider all differentials, including PDA, during patient workup.  

Conclusion

The continuous "machinery" murmur is the hallmark sound auscultated on patients with PDA. Shared clinical features in both pediatric and adult populations include widened pulse pressure and dyspnea. Older children and adults may present with physical exam features of ES due to longstanding shunting of blood in the heart and pulmonary hypertension. Treatment options include pharmacological therapy with prostaglandin inhibitors or surgical repair via ligation or transcatheter closure. Although congenital heart defects, such as PDA, often are diagnosed in infancy and childhood, it is necessary to also consider these defects in the older patients.

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