Ebstein Anomaly, Tricuspid Valve Dysplasia, and Right Atrial Anomalies

Background: Congenital cardiac diseases are a common cause of death in puppies. Tricuspid valve dysplasia is characterized by thickening and displacement of the leaflets of the tricuspid valve, agenesis of the valves, and incomplete separation of valve components. Papillary muscles may fuse and display shortened or absent chordae tendineae that contribute to tricuspid regurgitation. Diagnostic features of tricuspid valve dysplasia include cardiomegaly with massive right atrium enlargement on thoracic radiography and tricuspid insufficiency on an ultrasound. We aimed to describe clinicopathological findings in a dog (Canis familiaris) with tricuspid dysplasia.Case: We aimed to describe tricuspid valve dysplasia in a dog referred for necropsy at the Anatomical Pathology Sector of The Rural Federal University of Rio de Janeiro, Brazil, with a clinical history of abdominal swelling, dyspnea, cyanosis, ascites, and prostration. Echocardiography and abdominal ultrasound revealed right ventricular enlargement, hepatomegaly, and splenomegaly. Examination of the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver was enlarged, with a nutmeg pattern, and foci of clotting and fibrin adhesions in the lateral right lobule.Discussion: Epidemiological, clinical, and pathological findings were consistent with tricuspid valve dysplasia. Although structural abnormalities of the tricuspid and mitral valves are well known in fetuses and neonates, congenital and secondary tricuspid malformations are rare in dogs. The survival rate is associated with the severity of heart lesions. Tricuspid valve dysplasia is mostly observed in large-breed dogs (>20 kg), particularly in Labrador Retrievers, Boxers, and German Shepherds. Regardless, most dogs with tricuspid valve dysplasia are of a pure-breed, which differs from our findings because our dog was a mongrel. Our dog displayed signs of dyspnea, cyanosis, abdominal swelling, prostration, and enlarged liver and spleen on ultrasound examination. Tricuspid valve dysplasia led to heart enlargement and right congestive heart failure, with consequent ascites, abdominal swelling, weakness, lethargy, jugular venous distension, and hepatomegaly. Overall, the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver had a nutmeg pattern. Tricuspid valve dysplasia is characterized by malformation of the tricuspid valve leaflets, chord tendineae, or papillary muscles. Malformed tricuspid valves are known to result in variable degrees of regurgitation, leading to right atrial overflow and ventricular eccentric hypertrophy. Differential diagnosis includes myocarditis, tricuspid valve endocarditis, tricuspid endocardiosis, tricuspid valve prolapse and right ventricular dysplasia, right ventricular enlargement with tricuspidal regurgitation due to pulmonary insufficiency, and arrhythmogenic right ventricular cardiomyopathy. Signs of heart murmurs (irregular sounds of the heart) on clinical examination may indicate an irregular blood flow pattern, and imaging tests may be necessary for assessing the presence and severity of any lesions. The epidemiologic, clinical, and pathological findings were consistent with those of tricuspid valve dysplasia.

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Impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in patients with Ebstein anomaly and tricuspid valve dysplasia

Cardiology in the Young ◽

10.1017/s1047951121004935 ◽

2022 ◽

pp. 1-12

Author(s):

Min Bao ◽

Edgar Jaeggi ◽

Liqun Sun ◽

Fu-Tsuen Lee ◽

Renee Sananes ◽

...

Keyword(s):

Tricuspid Valve ◽

Motor Skills ◽

Umbilical Vein ◽

Brain Mri ◽

Ascending Aorta ◽

Neurodevelopmental Outcomes ◽

Ebstein Anomaly ◽

Descending Aorta ◽

Neurodevelopmental Delay

Abstract Objectives: To evaluate the impact of fetal haemodynamics on surgical and neurodevelopmental outcomes in severe Ebstein anomaly and tricuspid valve dysplasia. Methods: Thirty-four fetuses with Ebstein anomaly/tricuspid valve dysplasia were referred from 2013 to 2019 for fetal echocardiography and clinical management. Nineteen fetuses with Ebstein anomaly/tricuspid valve dysplasia and 30 controls underwent cardiovascular magnetic resonance to quantify the fetal blood flow and to calculate cerebral oxygen delivery (cDO2) and consumption (cVO2). The 3D steady-state free precession acquisition was used to measure fetal brain volume. Surgical outcome, brain MRI, and neurodevelopmental follow-up were reviewed. Results: Twenty-six fetuses were live born (76%) and survival (65%) at a mean follow-up of 4 years. Nine fetuses had a brain MRI before discharge, and all had clinically silent injuries and volume loss. At 18 months, five single-ventricle patients had a neurodevelopmental delay in cognition and language (mean percentile: 11th), with gross-motor skills more affected than fine-motor skills (mean percentiles: 4th and 34th). Fetuses with Ebstein anomaly/tricuspid valve dysplasia had smaller brains, lower combined ventricular output, ascending aorta, superior caval vien and umbilical vein flows, lower oxygen saturation in ascending aorta and superior caval vien, lower cDO2 and cVO2 (p < 0.05). Superior caval vien/combined ventricular output and descending aorta/combined ventricular output ratios were lower in fetuses with circular shunt (p < 0.05). Fetuses requiring the Starnes procedure tended to have smaller brains, lower combined ventricular output, superior caval vien, descending aorta, and umbilical vein flows. Conclusions: All patients with Ebstein anomaly/tricuspid valve dysplasia are at high risk of neurodevelopmental delay and warrant follow-up. Fetal cardiovascular magnetic resonance revealed impaired brain growth with diminished cerebral blood flow and cDO2, the extenting dependent on the severity of the haemodynamic compromise.

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