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Left Atrial Stenosis Induced Pulmonary Venous Arterialization and Group 2 Pulmonary Hypertension in Rat
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Published on: November 18, 2018

Pulmonary hypertension in congenital diaphragmatic hernia

Hadi Mohseni-Bod1, Desmond Bohn

  • 1Department of Critical Care Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.

Seminars in Pediatric Surgery
|April 28, 2007
PubMed

Insights

Congenital diaphragmatic hernia (CDH) in newborns often causes pulmonary hypertension (PHTN), leading to heart and lung issues. Early intervention with PGE1 or iNO and supportive care improves outcomes for affected infants.

Area of Science:

  • Neonatal Medicine
  • Pediatric Cardiology
  • Pulmonology

Background:

  • Congenital diaphragmatic hernia (CDH) frequently presents with clinically significant pulmonary hypertension (PHTN) in neonates.
  • This PHTN can cause right-to-left shunting, hypoxemia, and acute right heart failure, even in infants without obvious ductal shunting.
  • Pulmonary vascular resistance and right ventricular pressures are often elevated in CDH, potentially persisting postnatally, with abnormal pulmonary vascular beds.

Purpose of the Study:

  • To review the pathophysiology and management of pulmonary hypertension in infants with congenital diaphragmatic hernia.
  • To highlight the impact of modern management strategies on mortality and morbidity.
  • To discuss therapeutic interventions and long-term follow-up for CDH patients.

Main Methods:

  • Review of clinical findings and diagnostic approaches for PHTN in CDH.
  • Discussion of established and emerging treatment modalities for PHTN in this population.
  • Emphasis on multidisciplinary care and long-term follow-up strategies.

Main Results:

  • "Gentle" ventilation, delayed surgery, and improved peri-operative care have significantly reduced mortality over the past two decades.
  • Prostaglandin E1 (PGE1) is recommended for hemodynamically significant PHTN with right ventricular dysfunction and restrictive patent ductus arteriosus (PDA).
  • Inhaled nitric oxide (iNO) may be beneficial, with response confirmation via echocardiography.

Conclusions:

  • Pulmonary hypertension is a critical issue in congenital diaphragmatic hernia requiring prompt recognition and management.
  • Pharmacological interventions like PGE1 and iNO play a role in managing PHTN, alongside optimized ventilation and surgical timing.
  • Long-term multidisciplinary follow-up is essential for managing chronic morbidities in survivors of CDH.

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