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EPIDEMIOLOGIC ANTECEDENTS OF PERSISTENT PULMONARY HYPERTENSION OF THE NEWBORN

Despite the clinical and public health significance of Persistent Pulmonary Hypertension of the Newborn (PPHN), little is known about the syndrome's epidemiologic origins. Between July 1985 and April 1989 we interviewed a total of 103 mothers of case and 309 mothers of control infants who were hospitalized at two Harvard-affiliated neonatal intensive care units. A priori hypotheses focused on events linked to three potential pathophysiologic events: 1) inhibition of prostaglandin synthesis (i.E., maternal intake of aspirin and nonsteroidal anti-inflammatory agents [NSAIDs]), 2) fetal hypoxemia (i.E., maternal cigarette and marijuana smoking), and 3) vasospasm (i.E., maternal cocaine abuse). Two of our principal hypotheses were supported: odds ratios [OR] (95% confidence intervals [CI]) for the variables of greatest interest were: smoking=2.0 (1.2, 3.4), NSAIDs=3.6 (1.3, 10.3), aspirin products=2.1 (.89, 5.1), and illicit drugs=2.6 (.88, 7.7). Very low prevalence of confessed illicit drug use among our subjects precluded drug-specific analyses. We have tested a number of multivariate models using logistic regression to control for factors used as measures of socioeconomic status. In each model, maternal use of aspirin products and/or NSAIDs during pregnancy was linked with significantly increased risk of her infant developing PPHN, minimum OR (9 5%CI)=2,1 (1.3, 3.4). These data may have important implicat ions regarding future prevention of PPHN.


Severe Pulmonary Hypertension: What To Know

When the mean blood pressure in the pulmonary arteries is over 70 millimeters of mercury (mmHg), someone has severe pulmonary hypertension. Without prompt medical intervention, it can lead to heart and lung dysfunction.

Individuals with pulmonary hypertension may notice shortness of breath, chest pain, intense fatigue, and severe dizziness. However, these symptoms could worsen when the disease progresses to severe pulmonary arterial hypertension.

Early diagnosis and treatment are critical to improving the outlook for someone with severe pulmonary hypertension.

This article discusses severe pulmonary hypertension, its causes, stages, and treatment options.

Doctors define pulmonary hypertension as an elevated blood pressure in the pulmonary arteries. Typically, a doctor will diagnose pulmonary hypertension when a person's average systolic arterial pressure reaches 35 mmHg or higher. The systolic pressure is the pressure in your arteries when your heart beats.

Other clinical symptoms may appear too.

Pulmonary hypertension is a progressive disorder. Without prompt intervention, it can gradually worsen and become severe.

Severe pulmonary hypertension occurs when the mean resting blood pressure in the pulmonary arteries is above 70 mmHg. Severe pulmonary hypertension is rare but may occur in people with chronic medical disorders, such as chronic obstructive pulmonary disease (COPD).

Learn more about COPD and high blood pressure.

Severe pulmonary hypertension has associations with increased vascular resistance, a condition that can result in right-sided heart failure. Reduced blood supply and the heart working harder are likely the reasons for the heart failure.

Consequently, individuals with severe pulmonary hypertension may present with any of the following symptoms:

Severe pulmonary hypertension is life threatening, so a person with these symptoms should seek urgent medical attention.

Learn more about symptoms, causes, and diagnosis of PAH.

Several factors, such as genetics, environmental toxins, and underlying chronic conditions, can predispose someone to severe pulmonary hypertension.

The World Health Organisation (WHO) classifies the causes of severe pulmonary hypertension into different groups, including:

Group 1: Pulmonary arterial hypertension (PAH)

Causes include:

Group 2: Pulmonary hypertension due to left-sided cardiac disease

Causes are:

  • left-sided valvular heart valve disease, such as aortic or mitral valve disease
  • left ventricular failure
  • other congenital cardiomyopathies
  • Group 3: Pulmonary hypertension due to lung disease

    Examples include:

    Group 4: Pulmonary hypertension due to chronic blood clots

    Group 4 pulmonary hypertension can only occur as a result of a condition called chronic thromboembolic pulmonary hypertension (CTEPH). Chronic blood clots in the lungs cause CTEPH.

    Group 5: Pulmonary hypertension due to other health conditions

    Causes include:

  • blood disorders, such as myeloproliferative disorders
  • inflammatory conditions such as sarcoidosis and vasculitis
  • metabolic disorders, such as glycogen storage diseases.
  • chronic kidney disease
  • mechanical obstruction from tumors compressing the pulmonary arteries
  • Learn more about the five classes of pulmonary hypertension.

    Doctors typically diagnose pulmonary hypertension using a combination of clinical findings and laboratory investigations. During the medical history, the doctor will ask questions about the presenting symptoms and conduct a physical examination.

    The doctor will typically perform a general and chest examination to identify signs of pulmonary hypertension.

    Doctors will conduct the following laboratory tests to diagnose pulmonary hypertension:

  • Cardiac catheterization: This procedure helps to measure the blood pressure in the pulmonary arteries. A doctor will diagnose severe pulmonary hypertension if a person's blood pressure exceeds 70 mmHg.
  • Echocardiography: Doctors use it to evaluate the structure and functions of the heart chambers.
  • Other supportive tests include:

    Learn more about diagnosing pulmonary hypertension.

    The treatment of severe pulmonary hypertension requires a combination of different types of treatments.

    According to the Pulmonary Hypertension Association, therapeutic interventions include:

  • Intravenous medications: Epoprostenol is a vasodilator that relaxes the pulmonary blood vessels and improves blood flow. It is typically the first-line medication for treating severe pulmonary hypertension. Doctors may also use ambrisentan, bosentan, macitentan, riociguat.
  • Oxygen therapy and ventilation support.
  • Lung transplant, especially for those with severe lung damage.
  • Interventional cardiac surgeries
  • Supportive care and other medications, such as diuretics, to reduce fluid retention in the lungs.
  • Treatment for pulmonary hypertension depends on which WHO group the person falls into and the level of their pulmonary blood pressure. For example, someone in WHO Group 1 will undertake a different treatment course than someone from Group 2.

    Learn more about treating pulmonary hypertension.

    Below are some commonly asked questions about severe pulmonary hypertension:

    What are the stages of pulmonary hypertension?

    The four stages of pulmonary hypertension are:

  • Class I: No symptoms during rest or when performing activities.
  • Class II: Presence of symptoms, such as fatigue or shortness of breath, during routine activities, except at rest.
  • Class III: Symptomatic and experiences fatigue, shortness of breath, and chest pain. People may also experience limitations in performing everyday activities.
  • Class IV: Symptoms occur at rest and during any physical activity.
  • What is the life expectancy for severe pulmonary hypertension?

    The life expectancy of severe pulmonary hypertension varies for each person. The following factors can have an impact on someone's life expectancy:

  • what WHO group the pulmonary hypertension falls into — for example, someone in group 1 with idiopathic pulmonary hypertension is likely to have a poor outlook
  • the available treatments
  • the class of symptoms a person has
  • How common is severe pulmonary hypertension?

    Generally, pulmonary hypertension is rare. However, severe pulmonary hypertension tends to be more prevalent in people with COPD.

    Severe pulmonary hypertension is a clinical condition in which the resting blood pressure in the pulmonary arteries is above 70mmHg. Individuals with severe pulmonary hypertension may notice profound shortness of breath, cyanosis, chest pain, intense fatigue, and severe dizziness.

    Genetic factors, chronic medical conditions, and exposure to environmental toxins are possible causes of severe pulmonary hypertension.

    Cardiac catheterization and echocardiography are essential tests for diagnosing severe pulmonary arterial hypertension.

    Severe pulmonary hypertension treatment includes pulmonary vasodilators, oxygen and ventilation support, and lung transplantation.

    Learn more about hypertension in our dedicated hub.


    Pregnancy More Likely To Be Favorable If Mother's PH Is Mild

    Pregnant women with mild pulmonary hypertension (PH) show a significantly lower risk of maternal and fetal complications than those with moderate-to-severe PH, according to a study from China.

    "For patients with mild pulmonary hypertension and good cardiac function, continued pregnancy or even delivery should be considered under multidisciplinary monitoring," its researchers wrote.

    Con­tinuous disease monitoring and control throughout the preg­nancy, "multidisciplinary, individualized treatment," and planning for a preterm birth also help to further "improve maternal and fetal outcomes," they added.

    The study, "Pregnancy feasibility in women with mild pulmonary arterial hypertension: a systematic review and meta-analysis," was published in the journal BMC Pregnancy & Childbirth.

    European guidelines advise against pregnancy with pulmonary hypertension

    PH, a chronic and progressive disease, is characterized by increased blood pressure in the vessels that supply the lungs, risking heart failure. The disease also is known to put patients at a risk of pregnancy complications, and recent European Society of Cardiology guidelines advise against pregnancy for women with PH.

    Despite advances in disease treatment and pregnancy management, questions remain as to whether women with mild PH can carry a child to term or have a vaginal delivery.

    Researchers at Tongji Medical College in Wuhan conducted a systematic review and meta-analysis to compare maternal and fetal outcomes between women with mild PH and those with moderate-to-severe disease. A meta-analysis is an assessment that pools the results of several studies.

    A total of 32 studies, published between January 1990 and April 2023 and reporting outcomes involving 2,520 women (mostly Chinese, ages ranging from 21 to 39), were used for the meta-analysis.

    Maternal outcomes included mortality, the incidence (new cases) of cardiac complications, and intensive care unit (ICU) admission.

    Fifteen studies covering 1,204 women assessed maternal mortality. Findings showed lower rates in women with mild and moder­ate PH, a mean of 0.15%, compared with those having severe disease, a mean of 9%. Women with mild PH had significantly lower rates than those with moderate-to-severe PH.

    In a subgroup analysis looking at mortality rates before and after 2010, a significant drop was seen in the mild PH group: from a mean rate of 2.13% prior to 2010, to a rate of 0.08% after that year.

    In contrast, no significant difference in maternal mortality was observed in women with moderate-to-severe PH before 2010 (mean rate, 9.45%) or after that year (mean rate, 9.28%).

    Four studies assessed cardiac complications while four others reported ICU admission. Cardiac complications in pregnant women in the mild PH group were significantly lower (mean of 13%) than in women with moderate-to-severe PH (mean of 41%). Similarly, ICU admissions were a mean of 18% in mild PH patients and 55% in those with more advanced disease.

    Careful and multidisciplinary monitoring of pregnancy seen as essential

    More positive outcomes for newborns also were reported among women with milder PH relative to those with moderate-to-severe disease. Specifically, researchers found a significantly lower incidence of preterm birth in the mild PH group, 20% versus 43%; a lower rate of a smaller size for gestational age (6% mild PH vs. 40% more advanced PH); and a lower rate of low birth weight infants (18% vs. 34%), newborns with asphyxia or insufficient oxygen (7% vs. 17%), and neonatal mortality (1% vs. 6%). Gestational age refers to how far along in weeks a pregnancy has advanced since the mother's last menstrual period.

    The rate of cesarean section births was similar between the groups (80% vs. 79%), while rates of vaginal delivery were significantly higher in the mild PH group than in women with moderate-to-severe PH (16% vs. 7%).

    Researchers also assessed the proportion of women with functional class III and IV PH during pregnancy, as defined by the New York Heart Association (NYHA). Class III is characterized by marked limitations in activity due to symptoms, and class IV denotes severe limitations, with symptoms present even at rest.

    Fewer woman in the mild PH group were identified as having NYHA class III and IV PH relative to those with moderate-to-severe PH, a mean of 12% versus 55%.

    Findings show "that pregnancies with mild pulmonary hypertension had significantly better maternal and fetal outcomes than those with moderate to severe pulmonary hypertension," the investigators concluded.

    The significantly higher likelihood of complications with more advanced disease, however, makes it "necessary to evaluate pregnancy risk and terminate it on time," they added.

    Among the study's limitations were the observational nature of the included studies, with limited methodological quality; the considerable variability among some reported results; and the fact that most studies analyzed were in Chinese patients, so its overall conclusions may not be generalizable to the global PH population.






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