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

INTRODUCTION/OBJECTIVES: 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. ANIMALS, MATERIALS AND METHODS: 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). RESULTS: 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%. DISCUSSION: 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. CONCLUSIONS: These results support medium to long-term survival for most dogs with EA.

Ebstein's anomaly with an atrial septal defect in a jindo dog.

A 7-month-old, female jindo dog was presented with severe ascites, cyanosis, and exercise intolerance. Diagnostic imaging studies revealed Ebstein's anomaly with an atrial septal defect. The dog was managed medically with inotropics, diuretics, and vasodilators.

[Congenital malformations of the tricuspid valve in domestic carnivores: a retrospective study of 50 cases]. / Les malformations congénitales de la valve tricuspide chez les carnivores domestiques: etude rétrospective de 50 cas.

A retrospective study was done on 14 cats and 36 dogs diagnosed with right atrioventricular valve malformations (RAVM), either tricuspid dysplasia (n = 38) or Ebstein's anomaly (n = 12). Comparison with a large reference population including 85 250 animals allowed an epidemiologic analysis of these RAVM, demonstrating that Labrador, Boxer and Chartreux cats had a 35, 7 and 11-fold higher risk respectively to be affected by one of these heart diseases than other breeds, without sexual predisposition. In 93% of cases, cardiac auscultation revealed a heart murmur, whose grade was not significantly correlated with the echographic stages of the valvular disease. The echocardiographic examination confirmed the frequent association between RAVM and other congenital anomalies, particularly mitral dysplasia, in 84% and 20% of tricuspid dysplasia and Ebstein anomaly respectively. Last, the prognosis of RAVM was worsened by atrial fibrillation and right-sided heart failure.

Ebstein anomaly in a meerkat (Suricata suricatta).

An 8-yr-old male meerkat (Suricata suricatta) presented with sudden lethargy. A globular-shaped heart associated with pleural effusion on chest thoracic radiographs was consistent with congestive heart failure, and echocardiography was performed under general anesthesia. It revealed an Ebstein anomaly, with mild pericardial effusion and marked right heart enlargement. The animal was treated with imidapril chlorydrate. After 4 mo of treatment, thoracic radiographs still showed right-sided cardiomegaly; however, the animal appeared clinically normal.

Canine tricuspid valve malformation, a model of human Ebstein anomaly, maps to dog chromosome 9.

BACKGROUND: Ebstein anomaly of the tricuspid valve is a congenital cardiac malformation characterised by downward displacement of the attachment of the septal and posterior leaflets of the tricuspid valve. Canine tricuspid valve malformation (CTVM) is morphologically similar to Ebstein anomaly; familial occurrence of CTVM has been described. Several observations suggest a genetic cause but most cases appear to be sporadic. METHODS: Three purebred Labrador Retriever kindreds enriched for CTVM underwent clinical examination and echocardiography. DNA was extracted from whole blood. Genotyping was carried out using polymorphic repeat markers with an average spacing of 15 cM and polymorphic information content of 0.74. RESULTS: Pedigree analysis identified CTVM segregating as an autosomal dominant trait with reduced penetrance. Genome wide linkage analysis in one kindred identified a CTVM susceptibility locus on dog chromosome 9 (CFA9) with a maximum multipoint lod score of 3.33. The two additional kindreds showed a conserved disease haplotype. CONCLUSIONS: This study identifies a CTVM susceptibility locus on CFA9 and a founder effect in apparently unrelated Labrador Retriever kindreds. These results provide the basis for a positional candidate cloning effort to identify the CTVM disease gene. Identification of the CTVM gene will permit mutation screening of patients with Ebstein anomaly, which should provide additional insights into the genetic programmes of valve development.

Ebstein's anomaly in a beagle dog.

An intact male beagle dog aged 1 year was referred because of shortness of breath, exercise intolerance and cardiac murmur. Based on the results from electrocardiography, thoracic radiography and echocardiography, the dog was diagnosed as Ebstein's anomaly. Although the orally administered digoxin, vasodilators and diuretics partially improved congestive signs, the dog became to be refractory and died 20 months after the diagnosis. Necropsy confirmed malformation and apical displacement of the basal attachment of tricuspid valve leaflets.

Displasia de tricúspide associada à anomalia de Ebstein em cäo: relato de caso / Tricuspid dysplasia associated to Ebstein's anomaly in dog: a case report

A displasia da tricúspide, associada ou näo à anomalia de Ebstein, näo é das cardiopatias mais freqüentes em cäes (Liu; Tilley6, 1976; Eyster et al.4, 1977; Netter9, 1978; Moise8, 1994). A displasia da tricúspide caracteriza-se pelo espessamento focal ou difuso dos folhetos valvulares, subdesenvolvimento das cordoalhas tendíneas e músculos papilares, separaçäo incompleta dos componentes valvares da parede ventricular, além da agenesia do tecido valvular. Simultaneamente ou näo a esta valvulopatia congênita, pode ocorrer a inserçäo mais baixa do aparelho valvar na parede do ventrículo direito, condiçäo esta conhecida como anomalia de Ebstein (Liu, Tilley6, 1976; Eyster et al.4, 1977; Moise8, 1994). No presente relato, os autores descrevem um caso de displasia da tricúspide associada à malformaçäo de Ebstein, em cäo Weimaraner, macho, de 3 meses de idade, cujo diagnóstico clínico foi estribado no exame físico, eletrocardiográfico, radiográfico e ecocardiográfico. O animal apresentou uma sobrevida de 15 meses após a realizaçäo do diagnóstico, vindo a óbito de forma súbita e natural, e a existência das alteraçöes congênitas foram confirmadas no exame necroscópico

Ebstein's anomaly: a report of 3 cases in the dog.

Three young unrelated dogs were diagnosed as having the canine counterpart of Ebstein's anomaly, an eponymic term for a cardiac malformation in man. All of the dogs had systolic murmurs in the tricuspid area, and all had radiographic evidence of cardiac enlargement, particularly of the right atrium. Electrocardiography indicated atrial disease or right atrial enlargement and right ventricular conduction defects. The defect was confirmed by means of cardiac catheterization in all 3 dogs. Open heart surgery was performed on 2 of the dogs--plastic reconstruction of the tricuspid annulus and placement of a prosthetic valve in 1 and closure of an atrial septal defect in the other; both of these dogs died (1 day and 1 month after surgery).