Cyanotic CHD lesions with decreased pulmonary blood flow

John M. Costello*, Peter C. Laussen

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter


Infants with congenital heart defects associated with cyanosis and decreased pulmonary blood flow are a heterogenous group of patients. The complex aspects of the provision of intensive care for this patient population are largely concentrated in the newborn period, which is the focus of this chapter. An approach to the initial evaluation and stabilization of the cyanotic neonate with suspected congenital heart disease is provided. The anatomy, pathophysiology and clinical presentation, preoperative evaluation, surgical or transcatheter intervention, and postoperative care and outcome for tetralogy of Fallot with pulmonary stenosis are discussed. The concept of restrictive right ventricular physiology is covered in detail. The salient features of complex variants of tetralogy of Fallot are also noted, including pulmonary atresia, absent pulmonary valve syndrome and atrioventricular canal defect. A similar approach is used to discuss patients with pulmonary valve stenosis, pulmonary atresia with intact ventricular septum, and Ebstein’s anomaly of the tricuspid valve. With each lesion, esoteric nuances related to the physiology and perioperative care are noted that may contribute to improved outcomes for this complex group of patients.

Original languageEnglish (US)
Title of host publicationPediatric Critical Care Medicine
Subtitle of host publicationVolume 2: Respiratory, Cardiovascular and Central Nervous Systems
PublisherSpringer-Verlag London Ltd
Number of pages17
ISBN (Electronic)9781447163565
ISBN (Print)9781447163558
StatePublished - Jan 1 2014


  • Cyanosis
  • Ebstein’s anomaly
  • Hyperoxia test
  • Prostaglandin E1
  • Pulmonary atresia
  • Pulmonary stenosis
  • Tetralogy of Fallot
  • Tetralogy of Fallot with pulmonary atresia

ASJC Scopus subject areas

  • Medicine(all)

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