Answer for BIR CoW 12 Dec 2021
Ebsteins anomaly
Findings
Right atrium is significantly enlarged Right ventricle is significantly dilated with atrialisation of right ventricle Double superior venacava Distance from the mitral annulus to the tricuspid valve annulus measures 21 mm significantly enlarged right atrium and Right ventricle with atrialisation of right ventricle , features suggestive of ebsteins anomaly
Discussion
The differential on a chest radiograph is extremely broad, particularly since the findings in Ebstein anomaly are so variable. With echocardiography and MRI, the diagnosis is usually self-evident, once the apically displaced tricuspid valve in identified. Differential on a chest radiograph includes: other congenital heart anomalies left to right shunts: enlarging right atrium pulmonary stenosis: right heart enlargement tetralogy of Fallot Uhl anomaly large pericardial effusion selective fatty infiltration of the right ventricular myocardium prominent thymus Ebstein anomaly is an uncommon congenital cardiac anomaly, characterized by a variable developmental anomaly of the tricuspid valve The anomaly accounts for only ~0.5% of congenital cardiac defects , although it is the most common cause of congenital tricuspid regurgitation. There is no recognized gender predilection, and almost all cases seem to be sporadic, although an association with maternal lithium carbonate injection has been postulated . A few familial cases have been reported chromosomal anomalies trisomy 13 trisomy 21 Turner syndrome multiple other congenital heart lesions (ASD is quite common) conduction abnormalities leading to arrhythmia (common), e.g. Wolf-Parkinson-White syndrome maternal lithium carbonate ingestion The presentation is often antenatal, with the development of hydrops fetalis and fetal tachyarrhythmias. In less severe cases, it may present at birth. Depending on the degree of atrial right-to-left shunting, the infant may or may not be cyanotic. Findings on chest radiographs largely depend on the severity of the abnormality and the degree to which the tricuspid valve is displaced downwards. There is often severe right-sided cardiomegaly due to an elongated and enlarged right atrium which may result in an elevated apex. Classically, the heart is described as having a "box shape" on a frontal chest radiograph Diagnosis relies on visualization of the septal leaflet of the tricuspid valve, typically appreciated from transthoracic parasternal and apical windows. Apical displacement the septal leaflet in excess of 8 mm per m2 (body surface area) with “sail-like” elongation is considered diagnostic 12. Further features include: tricuspid regurgitation degree of right/left ventricular dysfunction right atrial enlargement calculation of the chamber area ratio in the apical 4 chamber view at end-diastole sum of the right atrial and atrialized right ventricular areas divided by the remaining three cardiac chambers values of one or more considered markedly enlarged/severe and portend a poor prognosis CT/MRI Allows direct visualization of anatomical detail. Cine MRI can be used akin to echocardiography for functional assessment. apical displacement of the septal and posterior leaflets of the tricuspid valve as a rule of thumb: if the tricuspid septal attachment lies more than 1.5 cm "beneath" (i.e. towards the apex) than mitral septal attachment, this can be considered Ebstein anomaly (in adults, the measurement is 2 cm) some prefer a value indexed to body surface area a septal displacement below the mitral valve of >8 mm/m2 (or >0.8 mm/cm2) is the cutoff value 9 for Ebstein anomaly "atrialization" of the right ventricle tricuspid regurgitation
References
Choi YH, Park JH, Choe YH et-al. MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 1994;163 (3): 539-43. AJR Am J Roentgenol (abstract) - Pubmed citation 2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 27 (5): 1323-34. doi:10.1148/rg.275065148 - Pubmed citation
Findings
Right atrium is significantly enlarged Right ventricle is significantly dilated with atrialisation of right ventricle Double superior venacava Distance from the mitral annulus to the tricuspid valve annulus measures 21 mm significantly enlarged right atrium and Right ventricle with atrialisation of right ventricle , features suggestive of ebsteins anomaly
Discussion
The differential on a chest radiograph is extremely broad, particularly since the findings in Ebstein anomaly are so variable. With echocardiography and MRI, the diagnosis is usually self-evident, once the apically displaced tricuspid valve in identified. Differential on a chest radiograph includes: other congenital heart anomalies left to right shunts: enlarging right atrium pulmonary stenosis: right heart enlargement tetralogy of Fallot Uhl anomaly large pericardial effusion selective fatty infiltration of the right ventricular myocardium prominent thymus Ebstein anomaly is an uncommon congenital cardiac anomaly, characterized by a variable developmental anomaly of the tricuspid valve The anomaly accounts for only ~0.5% of congenital cardiac defects , although it is the most common cause of congenital tricuspid regurgitation. There is no recognized gender predilection, and almost all cases seem to be sporadic, although an association with maternal lithium carbonate injection has been postulated . A few familial cases have been reported chromosomal anomalies trisomy 13 trisomy 21 Turner syndrome multiple other congenital heart lesions (ASD is quite common) conduction abnormalities leading to arrhythmia (common), e.g. Wolf-Parkinson-White syndrome maternal lithium carbonate ingestion The presentation is often antenatal, with the development of hydrops fetalis and fetal tachyarrhythmias. In less severe cases, it may present at birth. Depending on the degree of atrial right-to-left shunting, the infant may or may not be cyanotic. Findings on chest radiographs largely depend on the severity of the abnormality and the degree to which the tricuspid valve is displaced downwards. There is often severe right-sided cardiomegaly due to an elongated and enlarged right atrium which may result in an elevated apex. Classically, the heart is described as having a "box shape" on a frontal chest radiograph Diagnosis relies on visualization of the septal leaflet of the tricuspid valve, typically appreciated from transthoracic parasternal and apical windows. Apical displacement the septal leaflet in excess of 8 mm per m2 (body surface area) with “sail-like” elongation is considered diagnostic 12. Further features include: tricuspid regurgitation degree of right/left ventricular dysfunction right atrial enlargement calculation of the chamber area ratio in the apical 4 chamber view at end-diastole sum of the right atrial and atrialized right ventricular areas divided by the remaining three cardiac chambers values of one or more considered markedly enlarged/severe and portend a poor prognosis CT/MRI Allows direct visualization of anatomical detail. Cine MRI can be used akin to echocardiography for functional assessment. apical displacement of the septal and posterior leaflets of the tricuspid valve as a rule of thumb: if the tricuspid septal attachment lies more than 1.5 cm "beneath" (i.e. towards the apex) than mitral septal attachment, this can be considered Ebstein anomaly (in adults, the measurement is 2 cm) some prefer a value indexed to body surface area a septal displacement below the mitral valve of >8 mm/m2 (or >0.8 mm/cm2) is the cutoff value 9 for Ebstein anomaly "atrialization" of the right ventricle tricuspid regurgitation
References
Choi YH, Park JH, Choe YH et-al. MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 1994;163 (3): 539-43. AJR Am J Roentgenol (abstract) - Pubmed citation 2. Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 27 (5): 1323-34. doi:10.1148/rg.275065148 - Pubmed citation
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!