Guidelines for treating pulmonary hypertension in children

Pulmonary Hypertension in children

Nearly 1 in 500 babies are born with pulmonary hypertension (PH) every year.  Although rare in newborns, pulmonary hypertension is a heart and lung disease with the potential to be fatal.  Children suffering from pulmonary hypertension have blockages in their pulmonary artery.  This makes it difficult for the right ventricle in the heart to pump blood through their lungs.  Although in some cases pulmonary hypertension is caused in isolation due to circulatory problems in the lungs, more often it is due to complications brought on by other serious issues of the heart or lungs.  Congenital heart disease, congenital diaphragmatic hernia, bronchopulmonary dysplasia are some of the issues which cause pulmonary hypertension.

Newborns with congenital heart disease generally suffer from high blood pressure in their lungs.  In many cases the blood pressure normalizes after correcting the heart defect through surgery.  In cases where this does not happen, it becomes harder for the heart to pump.  Although rare, sometimes the pressure is so high that corrective surgery is not possible or is too risky.  Medications and/or oxygen are used to relax the blood vessels – both in the case of post surgery or in case s where surgery has not been possible.

Pulmonary hypertension symptoms include fainting, shortness of breath and cyanosis.  Pulmonary hypertension can lead to death from heart failure, if not diagnosed and managed correctly. Those who survive, suffer from health issues throughout their lives.

The Guidelines

For the first time, guidelines towards the diagnosis and treatment of pulmonary hypertension in children have been laid out and recently published in the journal Circulation.  There have been guideline in place for the treatment of pulmonary hypertension in adults since a long time, but the same cannot be applied to the diagnosis and treatment in children.  It needs to be understood here that the under laying causes for pulmonary hypertension differ amongst adults and children.

Based on data collected from over 600 studies, these guidelines have been created by an interdisciplinary team of 27 physicians.  A result of the collaboration between the American Thoracic Society (ATS) and the American Heart Association (AHA), the guidelines are set as an aim to provide the best medical care to children suffering from pulmonary hypertension.

Some of the key features of the guidelines cover:

  • Classification of the different types of pulmonary hypertension;
  • Proven and emerging surgical and medical therapies;
  • Approved treatments and dosages including their appropriateness;
  • Optimal care of the patient;
  • Advice on supportive care and the social aspects of care of the patient;
  • Advice on exercise and travel restrictions;
  • The role of centers specializing in the treatment of pulmonary hypertension in children.

There are gaps in our knowledge about treating pulmonary hypertension in children.  These guidelines point to these gaps; along with offering advice on issues that health care providers and parents of children suffering from pulmonary hypertension find themselves struggling with.  Areas like whether a child should receive antiplatelet blood thinning agents or anticoagulants; parents concerns with safe exercise regime for their children, concerns regarding air travel; these and much more are covered by these guidelines.

The future

The guidelines are a first major step towards developing a proper diagnosis and treatment regime for pulmonary hypertension in children.  However, it is important to remember that further research with more specific data is required to improve the care and treatment of children suffering from pulmonary hypertension.  It is equally important for parents of children suffering from this condition to search and find doctors and care centers that offer better diagnostic and surgery facilities which can include new molecular diagnostics, latest drug therapies and recently developed surgery protocols.


  1. Dunbar Ivy, M., Steven H. Abman, M., Robyn J. Barst, M., Rolf M.F. Berger, M., Damien Bonnet, M., Thomas R. Fleming, P., et al. (2013, December). Pediatric Pulmonary Hypertension. Retrieved November 02, 2015, from JACC Journals:
  2. First Ever Guidelines for Pediatric Pulmonary Hypertension . (2015, October 27). Retrieved Nvember 02, 2015, from RT Magazine:
  3. Heiner Latus, T. D. (2015, February 03). Treatment of pulmonary arterial hypertension in children. Retrieved November 02, 2015, from NATURE REVIEWS CARDIOLOGY | REVIEW:
  4. Melvyn Rubenfire, M. F. (2015, September 15). Guidelines for Diagnosis and Treatment of Pulmonary Hypertension . Retrieved November 02, 2015, from American College of Cardiology:
  5. Rosenthal, M. (2015, October). Guidelines First To Focus on Children With Pulmonary Hypertension. Retrieved November 02, 2015, from Pharmacy Practise News:

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