Epidemiological, clinical, and echocardiographic features, and outcome of dogs with Ebstein's anomaly: 32 cases (2002–2016)

Ebstein's anomaly (EA) is a congenital heart disease characterized by apical displacement of the tricuspid valve leaflets in the right ventricle. The objective of this retrospective study was to investigate the signalment, clinical features, echocardiographic findings, and outcome of dogs with EA. Medical records of 40 dogs with EA were reviewed. Echocardiographic variables used to assess EA severity in human pediatrics were also evaluated (e.g. displacement index, Celermajer index, Carpentier class, and apex–mitral annulus:apex–tricuspid annulus distance ratio). Labrador retriever was the most commonly recruited breed (24 of the 40 dogs, 60%). Eight of the 40 dogs with EA had hemodynamically compromising concurrent heart (n = 7) or respiratory diseases (n = 1). A right apical systolic heart murmur (median grade = 5/6) was detected in the remaining 32 dogs, without any other clinical sign related to EA in 19 of the 32 dogs (59%). Median (interquartile range) values of the displacement index and Celermajer index were 17.4 mm/m2 (12.0–21.9) and 100% (50–130), respectively. Median time to all-cause death was 74 months, and 72% dogs (95% confidence interval, 50–86%) had not succumbed to cardiac death (CD) 160 months after diagnosis. Univariate analyses showed that the time from diagnosis to CD was associated with the presence of clinical signs, ascites, severe right atrial dilation, palpable thrill, and a Celermajer index ≥100%. Right atrial enlargement is significantly associated with decreased survival time of dogs with EA although most may live for years and may not die from CD. These results support medium to long-term survival for most dogs with EA.