Introduction
The treatment of children with pulmonary hypertension (PH) has evolved
dramatically over the last 30 years. Early data demonstrated a median
survival of less than one year whereas current estimates now predict
5-year survival rates of at least 80%. 1,2,3 This
improvement likely reflects the development of PH-specific drugs and an
increased awareness and understanding of PH pathology along with the
development of specialized centers and personnel to treat it.
Nonetheless, despite these advances, children still die from their
disease. Children with severe PH who fail aggressive medical management
have limited options. Historically, lung transplantation has been the
palliative procedure most often considered in these critically
compromised children. However, survival with pediatric lung
transplantation remains relatively limited with current median survival
of ~7 years in children transplanted for idiopathic PH
and even lower survival for children with non-idiopathic PH, a median of
~3 years.4,5 In addition, lung
transplantation involves expensive, highly technical and specialized
care that only a relatively small number of centers around the world can
afford to offer. Consequently, care providers have looked for other ways
in which to treat these medically refractory children. Ultimately, most
children with severe PH die because of right ventricular failure. Thus,
a number of interventions have developed over the years as a means to
ameliorate right ventricular dysfunction short of transplantation.
Physiologically these procedures fall into two categories: 1) creating a
pre-tricuspid valve shunt, essentially making an atrial septal defect,
or 2) creating a post-tricuspid valve shunt, typically a
pulmonary-to-systemic anastomosis at the level of the great vessels,
e.g., a Potts shunt. We will discuss these options in more detail. Other
interventions for supporting the failing right ventricle such as ECMO,
paracorporeal lung assist devices or right ventricular assist devices
are therapies that are utilized almost exclusively in the intensive care
setting and will not be covered in this article.