Coding PPHN

PPHN, PFO and PDA are not supposed to be coded together per the Excludes 1 note. We have many babies with these diagnoses. When PPHN is present in a newborn along with a PFO and/or PDA, I code the PPHN because it is going to be the focus of treatment. If the PDA requires treatment for closure I do go ahead and code it with the PPHN.

On the other hand, our coders will always code the PFO and PDA instead of the PPHN. I feel this is incorrect.

I would love to have feedback from others as to how you code the record when there is PPHN with a PFO and/or PDA?

Thank you!


Mary Birch Hospital for Women & Newborns


  • Sarah,

    I work in a facility with a level III NICU. I have been doing NICU CDI for a bit over a year and find it difficult to find resources for this group (also OB patients). I am having similar issues with PPHN. Coding will go to the PFO/PDA because of the echo reading even when the providers documenting PPHN is being treated, the issue being the connection of the two conditions. There has been ongoing discussion on how this should be documented to capture because the excludes note does not allow the 2 conditions to be coded together unless they are not related. I feel like this is a work in progress and I would appreciate any discussion.

    I know this information may not help but may allow for collaboration on this topic.


  • Sarah and Kim,

    This has been an ongoing topic here at DCH as well. I would like to cite a NIH article below...

    W/ 10% being idiopathic and the remaining d/t certain neonatal pulmonary diseases...that tells me they are not related to PDA or PFO. The shunting happens across the PDA/PFO d/t the PVR.

    I think a question to the Coding Clinic might shine some light on this...but from a clinical perspective (w/ this article to support), you can code all of them. I would also ask if you are looking for respiratory failure and/or heart failure with the moderate to severe cases of PPHN because not all PPHN require significant support like some of our higher acuity patients do.

    I'd love to see what other colleagues are thinking!!




    Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome characterized by sustained elevation of pulmonary vascular resistance (PVR) and is often associated with normal or low systemic vascular resistance (SVR). This leads to extrapulmonary shunting from right to left across persistent fetal channels (patent ductus arteriosus, PDA and patent foramen ovale, PFO) leading to labile hypoxemia. This disorder was previously referred to as persistent fetal circulation (PFC) and is often secondary to an unsuccessful pulmonary transition at birth. This article gives a brief overview of fetal circulation and transition at birth and focuses on mechanisms of PPHN in various neonatal respiratory disorders and postnatal management of an infant with PPHN.

    Etiology and pathophysiology of PPHN

    Failure of the pulmonary circulation to undergo the normal transition after birth leads to PPHN, which is characterized by an elevated PVR/SVR ratio resulting from either vasoconstriction, structural remodeling of the pulmonary vasculature, intravascular obstruction or lung hypoplasia. There is right-to-left shunting of blood across the foramen ovale and ductus arteriosus, resulting in hypoxemia and labile oxygen saturations (figure 3).

    PPHN may be idiopathic (10%) or secondary to certain neonatal pulmonary diseases which lead to delayed relaxation of the pulmonary vascular bed. Common pulmonary conditions such as congenital diaphragmatic hernia (CDH), respiratory distress syndrome (RDS), pneumonia, meconium aspiration syndrome (MAS) and transient tachypnea of the newborn (TTN) may be associated with PPHN. Some of the rare causes of severe and intractable PPHN include alveolar capillary dysplasia 27, hyaline membrane disease caused by mutations in surfactant protein B (SP-B) gene 28 and respiratory failure due to ATP binding cassette protein member A3 (ABCA3) deficiency.29 Recently, Byers et al noted a genetic association. PPHN was significantly associated with genetic variants in corticotropin-releasing hormone (cRh) receptor 1, CRHR1 and cRh-binding protein, CRHBP.30

  • Kim and Jorde,

    Thank you both for your thoughtful comments. I have a feeling this is an ongoing issue in other institutions as well. Probably the best way to settle the coding quandary is to do as you suggested Jorde, and submit a question to coding clinic.


  • Sarah,

    Did you send a question to Coding Clinic on this topic? I am planning to work with our coding department and send it in the next few weeks and would welcome any discussion with you. Please feel free to reach out to me via email.


  • See also AHA CC 4Q 2017 pg 20 and Official Coding Guideline I.C.17. If a manifestation is not inherent/integral to the underlying condition, the manifestation can be coded. Also, although the Excludes 1 has been changed to an Excludes 2 for FY 2022, you may use that knowledge to review prior denials and state how the coding of both codes would fall under the 'exception' for Excludes 1 note.

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