Junctional ectopic tachycardia may be a spectrum of injury to the AV node in which

partial injury may be associated with increased automaticity and more complete injury with AVB. Although to date there has been no report documenting an association between congenital Silmitasertib solubility dmso junctional ectopic tachycardia in the absence of AVB and maternal autoantibodies, given the lack of symptoms among otherwise healthy women with infants who have complete AVB and/or maternal autoimmune-mediated cardiomyopathy, prospective serological evaluation of the mothers of affected infants should be considered. A spectrum of structural heart disease has been reported among foetuses and infants with maternal autoimmune-mediated cardiovascular disease. In children with maternal autoimmune-mediated learn more congenital AVB, structural congenital heart disease has been reported in 16–42% [22, 38]. These lesions have included persistent ductus arteriosus most of which have required intervention, and atrial and ventricular septal defects. Of greater interest, semilunar and atrioventricular valve abnormalities have also been described in association with AVB, including

stenosis, regurgitation and dysplasia without functional changes (Fig. 4) [22, 38, 54]. Inflammation and fibrosis as well as haemodynamic changes could potentially contribute to the evolution of at least some of these lesions, as suggested in one case of acute chordal rupture with moderate mitral insufficiency in 7-week old infant with echocardiographic evidence of EFE involving left ventricular papillary muscles and chordae [54]. The incidence of structural and even functional heart disease among infants of mothers

with autoantibodies in the absence of AVB is still not certain. In one study that assessed structural abnormalities in a series of 165 pregnancies with autoimmune disease and anti-Ro antibodies, four offspring had structural heart disease suggesting a potential incidence of 2.8%, which represents an increase over that of the general Calpain population [51]. Maternal autoimmune-mediated pathology could be aetiological in the evolution of other forms of congenital heart disease, as suggested in a recent case of prenatally diagnosed hypoplastic left heart syndrome [55]. Further prospective longitudinal investigations of pregnancies (and offspring) in women with anti-Ro and anti-La antibodies with and without autoimmune disease are necessary at this time to determine the true incidence of congenital structural, functional and rhythm-related cardiovascular disease associated with maternal autoantibodies.