Rationale and objectives for ECG screening in infancy

Abstract

Electrocardiographic (ECG) screening in infants and children who may be at risk of sudden cardiac death (SCD) is controversial, and both rational and emotional arguments have often been given equal weight. We all have direct experience in this field, but have different backgrounds and have expressed divergent views on this topic. We attempted to build consensus among ourselves on the basis of the available facts, in the hope of providing an unbiased review of the relevant science and policy issues in favor of or against ECG screening in infants and children. This report presents our shared view on this medically and societally important topic. Long QT syndrome (LQTS) satisfies several criteria that may make ECG screening worthwhile: it is not rare (~1 in 2000 births); ECG diagnosis is feasible and can be used to trigger appropriate genetic testing; it causes approximately 10% of cases of sudden infant death syndrome (SIDS) as well as deaths in childhood and later in life, and effective treatments are available. By stimulating cascade screening in family members, diagnosis of affected infants may also prompt identification of asymptomatic but affected individuals. Neonatal screening is cost-effective by conventional criteria, and with a corrected QT (QTc) cutoff of 460 ms in 2 different ECGs, the number of false positives is estimated to be low (~1 in 1000 births). It is our conclusion that parents of newborn children should be informed about LQTS, a life-threatening but treatable disease of significant prevalence that may be diagnosed by a simple ECG.

Keywords: ECG screening; Infants; Long QT syndrome; Sudden cardiac death.

Figure 1

Kaplan-Meier cumulative survival curves showing time interval between birth and first cardiac event (syncope, resuscitated cardiac arrest, sudden death). Of note, figure and study do not include asymptomatic patients. LQT1 vs LQT2, p<0.0001. LQT1 vs LQT3, p=0.0001. LQT3 vs LQT2, p=NS. (From ref. )

Figure 2

Distribution of 43,080 Caucasian neonates among 5 subgroups (absolute numbers and percentage), according to QTc duration on the screening ECG. Neonates positive at the genetic analysis are also reported (From ref. ).

Figure 3

Diagram for the steps which should follow neonatal ECG screening based on the QTc observed with the second ECG performed after that the first one had showed a QTc >450 ms.