Term and near-term infants >34 weeks of gestational age and parents
User group
Healthcare professionals, perinatal and neonatal units, hospitals, and health services
Statement of standard
Management of newborn infants with persistent pulmonary hypertension (PPHN) in a specialised centre improves mortality and morbidity.
Rationale
Persistent pulmonary hypertension of the newborn (PPHN) is characterised by sustained elevation of pulmonary vascular resistance (PVR) after birth resulting in extrapulmonary shunting from right to left via the fetal circulatory pathways (patent ductus arteriosus and patent foramen ovale). (1) PHN leads to severe hypoxaemia that may not respond to conventional respiratory support and to avoid severe cardiorespiratory failure. (2) The management of delivery and neonatal care should be transferred to a specialised centre to ensure optimal outcomes. (3,4)
Benefits
Short-term benefits
Reduced mortality and morbidity (2,4,5)
Reduced need for extra-corporeal membrane oxygenation (ECMO) (6)
Long-term benefits
Reduced long-term morbidity (neurodevelopmental and cardiopulmonary outcome) (7)
Components of the standard
Component
Grading of evidence
Indicator of meeting the standard
For parents and family
Parents are informed by healthcare professionals about persistent pulmonary hypertension (PPHN) in the newborn infant, treatment strategies as well as short- and long-term consequences.
B (High quality)
Patient information sheet1
For healthcare professionals
A unit guideline on indications of pre- or postnatal transfer to a specialised centre, which may include facilities for ECMO, is adhered to by all healthcare professionals. (4,5) (see Birth & transfer)
A (High quality) B (High quality)
Guideline
Training on the management of PPHN is attended by all responsible healthcare professionals.
B (High quality)
Training documentation
For perinatal and neonatal unit
A unit guideline on indications of pre- or postnatal transfer to a specialised centre, which may include facilities for ECMO is available and regularly updated, to include: (4,5)
Resuscitation and early management are focused on optimal lung recruitment and ventilation, and based on neonatal resuscitation guidelines. (2)
Diagnosis is confirmed by detection of pre- and post-ductal saturation difference of 5-10%.
2D echocardiography is performed to rule out the presence of cardiac anomalies and assess right heart function; the degree of pulmonary hypertension is classified relative to systemic blood pressure. (5)
Inotropic drugs are used to support organ perfusion. (5)
In case of poor organ perfusion, treatment with inhaled nitric oxide (iNO) is started. (5,7,8)
If there is insufficient response to iNO despite optimal lung recruitment, i.v. drugs such as phosphodiesterase inhibitors (sildenafil, milrinone) or prostaglandin are considered. (3)
ECMO is considered according the extracorporeal life support organisation (ELSO) guidelines (OI>20). (4)
Local protocols are updated in regular intervals to ensure individualised therapy.
A (High quality) B (High quality)
Guideline
For hospital
Training on the management of PPHN is ensured.
B (High quality)
Training documentation
Access to 24/7 echocardiography, radiology, and laboratory support is ensured.
B (High quality)
Guideline
ECMO is only provided in designated centres.
B (High quality)
Guideline
For health service
A national guideline on indications of pre- or postnatal transfer to a specialised centre, which may include facilities for ECMO, is available and regularly updated. (see Birth & transfer)
B (High quality)
Guideline
1The indicator “patient information sheet” is an example for written, detailed information, in which digital solutions are included, such as web-based systems, apps, brochures, information leaflets, and booklets.
Where to go
Further development
Grading of evidence
For parents and family
N/A
For healthcare professionals
N/A
For perinatal and neonatal unit
N/A
For hospital
N/A
For health service
Develop research into optimal saturation targets, use of inodilators and additional drugs to treat persistent pulmonary hypertension (PPHN).
A (Low quality)
Provide long-term multidisciplinary standardised follow-up after discharge. (7)
A (Moderate quality)
Set up an European-wide database for newborn infants with PPHN.
A (Low quality)
Getting started
Initial steps
For parents and family
Parents and family are verbally informed by healthcare professionals about persistent pulmonary hypertension (PPHN), treatment strategies as well as short- and long-term consequences.
For healthcare professionals
Attend training on management of PPHN.
Raise awareness to diagnose PPHN.
For neonatal unit
Develop and implement a unit guideline on indications of pre- or postnatal transfer to a specialised centre, which may include facilities for ECMO.
Develop information material about PPHN, treatment strategies as well as short- and long-term consequences for parents.
For hospital
Support healthcare professionals to participate in training on management of PPHN and the use of licensed drugs.
Ensure facilities and devices for optimal care and follow-up care.
For health service
Develop and implement a national guideline on indications of pre- or postnatal transfer to a specialised centre, which may include facilities for ECMO.
Steinhorn RH. Advances in Neonatal Pulmonary Hypertension. Neonatology. 2016;109(4):334–44.
Nair J, Lakshminrusimha S. Update on PPHN: Mechanisms and treatment. Semin Perinatol. 2014 Mar;38(2):78–91.
Cabral JEB, Belik J. Persistent pulmonary hypertension of the newborn: recent advances in pathophysiology and treatment. J Pediatr (Rio J). 2013 Jun;89(3):226–42.
Snoek KG, Reiss IKM, Greenough A, Capolupo I, Urlesberger B, Wessel L, et al. Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus – 2015 Update. Neonatology. 2016;110(1):66–74.
The Neonatal Inhaled Nitric Oxide Study Group. Inhaled nitric oxide in full-term and nearly full-term infants with hypoxic respiratory failure. N Engl J Med. 1997;336(9):597–604.
Konduri GG, Vohr B, Robertson C, Sokol GM, Solimano A, Singer J, et al. Early Inhaled Nitric Oxide Therapy for Term and Near Term Newborn Infants with Hypoxic Respiratory Failure: Neurodevelopmental Follow-Up. J Pediatr. 2007 Mar;150(3):235–240.e1.
Barrington KJ, Finer N, Pennaforte T, Altit G. Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database Syst Rev. 2017 05;1:CD000399.
November 2018 / 1st edition / next revision: 2023
Recommended citation
EFCNI, Reiss I, Zimmermann LJI et al., European Standards of Care for Newborn Health: Management of persistent pulmonary hypertension of the newborn infant (PPHN). 2018.
