eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Pulmonary Hypertension, Persistent-Newborn: Follow-up

Author: Robin H Steinhorn, MD, Raymond and Hazel Speck Berry Professor of Pediatrics, Division Head of Neonatology, Associate Chair of Pediatrics, Northwestern University School of Medicine
Contributor Information and Disclosures

Updated: Sep 22, 2009

Follow-up

Further Inpatient Care

  • Neurologic evaluation of persistent pulmonary hypertension of the newborn (PPHN)
    • After recovery, consider evaluation for CNS injury by performing brain CT or MRI.
    • Advise complete examination by a neurologist or a developmental pediatrician after discharge, as the incidence of significant neurodevelopmental impairment is 25%.
    • Because the prevalence of hearing loss is high, order an automated hearing test before discharging the patient.
  • Feeding
    • Newborns recovering from persistent pulmonary hypertension of the newborn often feed poorly for several days or weeks.
    • Nasogastric (NG) feeding is frequently required to support the newborn until oral feeding is established.
    • Speech therapists may be helpful in reestablishing normal patterns of feeding.

Further Outpatient Care

  • Because of the high risk of neurodevelopmental impairment and sensorineural hearing loss, infants should be monitored closely for the first 2 years of life, preferably in a specialty follow-up clinic, for developmental follow-up care.
  • Recommend complete screening before pediatric patients enter school to determine if they have any subtle deficits that may predispose them to learning disabilities.
  • Reassess the infant's hearing when he or she is aged 6 months and again as the results indicate. Late sensorineural hearing loss has been reported in a high percentage of patients.

Transfer

  • Guidelines for transfer to an extracorporeal membrane oxygenation (ECMO) center for consultation are published on the Extracorporeal Life Support Organization (ELSO) Web site. Individual centers may have modified guidelines. Therefore, an ongoing relationship with the closest ECMO center is needed to provide optimal care.
  • Baseline criteria for consideration for ECMO include an evaluation for risk factors because of the invasive nature of the therapy and a need for heparinization.
  • Baseline criteria for newborns considered for ECMO are generally as follows:
    • Gestation of more than 34 weeks
    • Weight more than 2000 g
    • No major intracranial hemorrhage on cranial sonograms (ie, larger than a grade II hemorrhage)
    • Reversible lung disease or mechanical ventilation for 7-14 days
    • No evidence of lethal congenital anomalies or inoperable cardiac disease
  • The timing of a referral to an ECMO center is often a difficult decision. However, referral and transfer should occur before refractory hypoxemia develops. Early consultation and discussion with clinicians at the ECMO center is strongly recommended.

Prognosis

  • Pulmonary recovery
    • Overall, the survival rate for newborns with persistent pulmonary hypertension of the newborn is greater than 90% when all resources, including ECMO, are provided.
    • Pulmonary recovery is typically complete, and survivors do not have residual pulmonary disease.
  • Neurologic sequelae
    • Although most surviving newborns with persistent pulmonary hypertension of the newborn have normal neurodevelopmental outcomes, as many as 25% have significant neurodevelopmental sequelae.
    • Prolonged hyperventilation is associated with an increased prevalence of neurodevelopmental sequelae, especially sensorineural hearing loss.

Miscellaneous

Medicolegal Pitfalls

  • The main pitfall in the treatment of persistent pulmonary hypertension of the newborn is in recognizing its existence and severity. Although inhaled nitric oxide (iNO) is an effective pulmonary vasodilator, extracorporeal membrane oxygenation (ECMO) remains the only therapy that has been proven to be life-saving for persistent pulmonary hypertension of the newborn. Timely transfer to an ECMO center is life saving for newborns with severe persistent pulmonary hypertension of the newborn.
  • Identifying and maintaining communication with clinicians at an ECMO center is especially important given the widespread availability of iNO therapy. Continuous delivery of NO is required during transport. The referring center is responsible for determining what transport capabilities are available in order to administer a successful therapeutic iNO program.
 


More on Pulmonary Hypertension, Persistent-Newborn

Overview: Pulmonary Hypertension, Persistent-Newborn
Differential Diagnoses & Workup: Pulmonary Hypertension, Persistent-Newborn
Treatment & Medication: Pulmonary Hypertension, Persistent-Newborn
Follow-up: Pulmonary Hypertension, Persistent-Newborn
Multimedia: Pulmonary Hypertension, Persistent-Newborn
References
[ CLOSE WINDOW ]

References

  1. UK Collaborative ECMO Trial Group. UK collaborative randomised trial of neonatal extracorporeal membrane oxygenation. UK Collaborative ECMO Trail Group. Lancet. Jul 13 1996;348(9020):75-82. [Medline].

  2. Walsh-Sukys MC, Tyson JE, Wright LL, et al. Persistent pulmonary hypertension of the newborn in the era before nitric oxide: practice variation and outcomes. Pediatrics. Jan 2000;105(1 Pt 1):14-20. [Medline].

  3. Wung JT, James LS, Kilchevsky E, James E. Management of infants with severe respiratory failure and persistence of the fetal circulation, without hyperventilation. Pediatrics. Oct 1985;76(4):488-94. [Medline].

  4. Baquero H, Soliz A, Neira F, Venegas ME, Sola A. Oral sildenafil in infants with persistent pulmonary hypertension of the newborn: a pilot randomized blinded study. Pediatrics. Apr 2006;117(4):1077-83. [Medline].

  5. Steinhorn RH, Kinsella JP, Pierce C, Butrous G, Dilleen M, Oakes M, et al. Intravenous Sildenafil in the Treatment of Neonates With Persistent Pulmonary Hypertension of the Newborn (PPHN). J Pediatr. 2009; in press.

  6. Abman SH. Neonatal pulmonary hypertension: a physiologic approach to treatment. Pediatr Pulmonol Suppl. 2004;26:127-8. [Medline].

  7. Abman SH. New developments in the pathogenesis and treatment of neonatal pulmonary hypertension. Pediatr Pulmonol Suppl. 1999;18:201-4. [Medline].

  8. Alano MA, Ngougmna E, Ostrea EM Jr, Konduri GG. Analysis of nonsteroidal antiinflammatory drugs in meconium and its relation to persistent pulmonary hypertension of the newborn. Pediatrics. Mar 2001;107(3):519-23. [Medline].

  9. Auten RL, Notter RH, Kendig JW, Davis JM, Shapiro DL. Surfactant treatment of full-term newborns with respiratory failure. Pediatrics. Jan 1991;87(1):101-7. [Medline].

  10. Bahrami KR, Van Meurs KP. ECMO for neonatal respiratory failure. Semin Perinatol. Feb 2005;29(1):15-23. [Medline].

  11. Chambers CD, Hernandez-Diaz S, Van Marter LJ, et al. Selective serotonin-reuptake inhibitors and risk of persistent pulmonary hypertension of the newborn. N Engl J Med. Feb 9 2006;354(6):579-87. [Medline].

  12. Cheung PY, Tyebkhan JM, Peliowski A, Ainsworth W, Robertson CM. Prolonged use of pancuronium bromide and sensorineural hearing loss in childhood survivors of congenital diaphragmatic hernia. J Pediatr. Aug 1999;135(2 Pt 1):233-9. [Medline].

  13. Clark RH, Gerstmann DR. Controversies in high-frequency ventilation. Clin Perinatol. Mar 1998;25(1):113-22. [Medline].

  14. Clark RH, Kueser TJ, Walker MW, et al. Low-dose nitric oxide therapy for persistent pulmonary hypertension of the newborn. Clinical Inhaled Nitric Oxide Research Group. N Engl J Med. Feb 17 2000;342(7):469-74. [Medline].

  15. Davidson D, Barefield ES, Kattwinkel J, et al. Inhaled nitric oxide for the early treatment of persistent pulmonary hypertension of the term newborn: a randomized, double-masked, placebo-controlled, dose-response, multicenter study. The I-NO/PPHN Study Group. Pediatrics. Mar 1998;101(3 Pt 1):325-34. [Medline].

  16. Farrow KN, Fliman P, Steinhorn RH. The diseases treated with ECMO: focus on PPHN. Semin Perinatol. Feb 2005;29(1):8-14. [Medline].

  17. Findlay RD, Taeusch HW, Walther FJ. Surfactant replacement therapy for meconium aspiration syndrome. Pediatrics. Jan 1996;97(1):48-52. [Medline].

  18. Finer NN, Barrington KJ. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Rev. 2001;CD000399. [Medline].

  19. Hintz SR, Suttner DM, Sheehan AM, Rhine WD, Van Meurs KP. Decreased use of neonatal extracorporeal membrane oxygenation (ECMO): how new treatment modalities have affected ECMO utilization. Pediatrics. Dec 2000;106(6):1339-43. [Medline].

  20. Inhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failure. The Neonatal Inhaled Nitric Oxide Study Group. N Engl J Med. Feb 27 1997;336(9):597-604. [Medline].

  21. Kanto WP Jr, Bunyapen C. Extracorporeal membrane oxygenation. Controversies in selection of patients and management. Clin Perinatol. Mar 1998;25(1):123-35. [Medline].

  22. Kelly LK, Porta NF, Goodman DM, Carroll CL, Steinhorn RH. Inhaled prostacyclin for term infants with persistent pulmonary hypertension refractory to inhaled nitric oxide. J Pediatr. Dec 2002;141(6):830-2. [Medline].

  23. Kinsella JP, Abman SH. Clinical approach to inhaled nitric oxide therapy in the newborn with hypoxemia. J Pediatr. Jun 2000;136(6):717-26. [Medline].

  24. Kinsella JP, Griebel J, Schmidt JM, Abman SH. Use of inhaled nitric oxide during interhospital transport of newborns with hypoxemic respiratory failure. Pediatrics. Jan 2002;109(1):158-61. [Medline].

  25. Kinsella JP, Ivy DD, Abman SH. Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol. Apr 2005;29(2):123-8. [Medline].

  26. Konduri GG, Solimano A, Sokol GM, et al. A randomized trial of early versus standard inhaled nitric oxide therapy in term and near-term newborn infants with hypoxic respiratory failure. Pediatrics. Mar 2004;113(3 Pt 1):559-64. [Medline].

  27. Lakshminrusimha S, Russell JA, Steinhorn RH, et al. Pulmonary hemodynamics in neonatal lambs resuscitated with 21%, 50%, and 100% oxygen. Pediatr Res. Sep 2007;62(3):313-8. [Medline].

  28. Lotze A, Mitchell BR, Bulas DI, Zola EM, Shalwitz RA, Gunkel JH. Multicenter study of surfactant (beractant) use in the treatment of term infants with severe respiratory failure. Survanta in Term Infants Study Group. J Pediatr. Jan 1998;132(1):40-7. [Medline].

  29. Paranka MS, Clark RH, Yoder BA, Null DM Jr. Predictors of failure of high-frequency oscillatory ventilation in term infants with severe respiratory failure. Pediatrics. Mar 1995;95(3):400-4. [Medline].

  30. Pawlik TD, Porta NF, Steinhorn RH, Ogata E, deRegnier RA. Medical and financial impact of a neonatal extracorporeal membrane oxygenation referral center in the nitric oxide era. Pediatrics. Jan 2009;123(1):e17-24. [Medline].

  31. Steinhorn RH. Nitric oxide and beyond: new insights and therapies for pulmonary hypertension. J Perinatol. Dec 2008;28 Suppl 3:S67-71. [Medline].

  32. Tworetzky W, Bristow J, Moore P, et al. Inhaled nitric oxide in neonates with persistent pulmonary hypertension. Lancet. Jan 13 2001;357(9250):118-20. [Medline].

  33. Ziegler JW, Ivy DD, Kinsella JP, Abman SH. The role of nitric oxide, endothelin, and prostaglandins in the transition of the pulmonary circulation. Clin Perinatol. Jun 1995;22(2):387-403. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

persistent fetal circulation, PFC, persistent pulmonary hypertension in the newborn, persistent pulmonary hypertension of the newborn, PPHN, pulmonary vascular resistance, PVR, pulmonary perfusion, black lung PPHN, clear lung PPHN, pulmonary vasodilation, persistent newborn pulmonary hypertension, patent foramen ovale, patent ductus arteriosus, meconium aspiration syndrome, respiratory distress syndrome, pneumonia, congenital diaphragmatic hernia, bronchopulmonary dysplasia, hypothermia, hypoglycemia, cystic adenomatoid malformations, treatment, diagnosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Robin H Steinhorn, MD, Raymond and Hazel Speck Berry Professor of Pediatrics, Division Head of Neonatology, Associate Chair of Pediatrics, Northwestern University School of Medicine
Robin H Steinhorn, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Heart Association, American Pediatric Society, American Thoracic Society, and Society for Pediatric Research
Disclosure: Ikaria (INO Therapeutics) Consulting fee Consulting

Medical Editor

Steven M Donn, MD, Professor of Pediatrics, University of Michigan Medical School; Director, Division of Neonatal-Perinatal Medicine, Department of Pediatrics, CS Mott Children's Hospital, University of Michigan Health System
Steven M Donn, MD is a member of the following medical societies: American Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Arun K Pramanik, MD, MBBS, Professor of Pediatrics, Director of Neonatal Fellowship, Louisiana State University Health Sciences Center
Arun K Pramanik, MD, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, National Perinatal Association, and Southern Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.