Fetal congenital complete heart block (CAVB) is a rare cardiac conduction abnormality that is associated with high morbidity and mortality. It is considered the commonest of fetal bradyarrhymias.
The estimated prevalence of complete heart block in newborns is at ~1 in 20,000.
It results from either anatomic or electric discontinuity in the conducting tissues connecting the atria and ventricles. Congenital heart block is often classified into cases with cardiac structural abnormalities (often complex) and those with structurally normal hearts.
The etiology of congenital heart block when the heart is structurally normal is most often a fetal autoinflammatory response, caused by the binding of antibodies to the fetal cardiac conduction system.
- mothers of these fetuses can be shown to have anti-SS-A (anti-Ro) and anti-SS-B (anti-La) antibodies to ribonucleoproteins
- congenital cardiac anomalies: 35-53% 1,2
- situs anomalies: heterotaxy syndrome
On M mode ultrasound the atrial impulses can be seen in their own rate none of which are transmitted to the ventricles. The ventricular rates are often very slow at ~40-70 bpm.
Treatment and prognosis
The prognosis of complete heart block is worsened by the presence of hydrops, a ventricular rate of <55 bpm, and an associated cardiac anomaly.
- development of hydrops fetalis
- 1. Schmidt KG, Ulmer HE, Silverman NH et-al. Perinatal outcome of fetal complete atrioventricular block: a multicenter experience. J. Am. Coll. Cardiol. 1991;17 (6): 1360-6. - Pubmed citation
- 2. Maeno Y, Himeno W, Saito A et-al. Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience. Heart. 2005;91 (8): 1075-9. doi:10.1136/hrt.2003.033407 - Free text at pubmed - Pubmed citation
- 3. Berg C, Geipel A, Kohl T et-al. Atrioventricular block detected in fetal life: associated anomalies and potential prognostic markers. Ultrasound Obstet Gynecol. 2005;26 (1): 4-15. doi:10.1002/uog.1918 - Pubmed citation