Cyanotic CHD Lesions with Decreased Pulmonary Blood Flow

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Abstract

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.

Keywords

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

© Springer-Verlag London 2014

Authors and Affiliations

  1. 1.Division of CardiologyAnn & Robert H. Lurie Children’s Hospital of ChicagoChicagoUSA
  2. 2.Department of Critical Care MedicineThe Hospital for Sick ChildrenTorontoCanada

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