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Identifying newborns with critical congenital heart disease

Author
Carolyn A Altman, MD
Section Editors
David R Fulton, MD
Leonard E Weisman, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Congenital heart disease (CHD) is the most common congenital disorder in newborns [1-3]. Critical CHD, defined as requiring surgery or catheter-based intervention in the first year of life (table 1), occurs in approximately 25 percent of those with CHD [4]. Although many newborns with critical CHD are symptomatic and identified soon after birth, others are not diagnosed until after discharge from the birth hospitalization [5-8]. In infants with critical cardiac lesions, the risk of morbidity and mortality increases when there is a delay in diagnosis and timely referral to a tertiary center with expertise in treating these patients [9,10].

Factors that should lead clinicians to suspect critical congenital heart lesions in neonates will be reviewed here. Pulse oximetry screening and the diagnosis and management of cyanotic CHD are discussed separately [11]. (See "Newborn screening for critical congenital heart disease using pulse oximetry" and "Diagnosis and initial management of cyanotic heart disease in the newborn".)

TERMINOLOGY

The following terms are used to characterize CHD in this discussion:

Cyanotic CHD − Cyanotic CHD includes lesions that allow circulation of deoxygenated blood in the systemic circulation via intracardiac or extracardiac shunting (table 1). (See "Cardiac causes of cyanosis in the newborn" and "Diagnosis and initial management of cyanotic heart disease in the newborn".)

Ductal-dependent CHD − Ductal-dependent congenital heart lesions are dependent upon a patent ductus arteriosus (PDA) (figure 1) to supply pulmonary or systemic blood flow or to allow adequate mixing between parallel circulations. In critical right heart obstructive lesions, the PDA is necessary to supply blood flow to the lungs; in critical left heart lesions, the PDA supplies systemic circulation; and in parallel circulations (eg, transposition of the great arteries), bidirectional flow in the PDA allows mixing between oxygenated and deoxygenated circuits (table 1). Many, but not all, cyanotic congenital heart defects are ductal-dependent.

                         

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Literature review current through: Jul 2017. | This topic last updated: Jun 10, 2016.
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