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Pulmonary Hypertension Management

Mild Pulmonary Hypertension with Normal Biventricular Function
  • Continue respiratory management, no additional cardiovascular support
  • Continue to monitor closely for clinical change and repeat ECHO as needed

Moderate or Severe Pulmonary Hypertension

Normal biventricular function

Left to right atrial shunt

Left to right PDA
  • Initiate trial of iNO at 20 ppm

Primary RV dysfunction

Right to left atrial shunt

Right to left PDA
  • Pulmonary vasodilators: Initiate trial of iNO at 20 ppm
  • Consider maintaining ductus (e.g., RV dysfunction, PDA constriction) with PGE 1 (consult Cardiology)
  • Systolic and diastolic RV support, including milrinone, epinephrine, vasopressin
  • Consider ECMO as rescue therapy

Primary LV dysfunction or Biventricular dysfunction

Left to right atrial shunt

Right to left PDA
  • Systolic and diastolic LV support, including: Dobutamine or milrinone and/or epinephrine. Epinephrine may be needed in conjunction with milrinone
  • Ensure normal calcium
  • Consider prostaglandins to maintain ductus for systemic flow
  • Avoid iNO
  • Consider ECMO as rescue therapy

Adapted from Bhombal, S., & Patel, N. (2022). Diagnosis and management of pulmonary hypertension in congenital diaphragmatic hernia. Seminars in Fetal and Neonatal Medicine, 27(4), 101383. https://doi.org/10.1016/j.siny.2022.101383

 

These pathways do not establish a standard of care to be followed in every case. It is recognized that each case is different, and those individuals involved in providing health care are expected to use their judgment in determining what is in the best interests of the patient based on the circumstances existing at the time. It is impossible to anticipate all possible situations that may exist and to prepare a pathway for each. Accordingly, these pathways should guide care with the understanding that departures from them may be required at times.

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