3 | DISCUSSION
Compared with left atrial appendage aneurysm, RAAA is a rare cardiac
anomaly that was first reported in 2001 which thought to be caused by
dysplasia of the muscular wall of the right atrium (RA) and right atrial
appendage.7,8 Most of these aneurysms affect adults in
their third decade of life but they can also occur in children and
neonates and can also be detected prenatally.2,3
The diagnosis can be confirmed by imaging modalities, especially initial
echocardiography and chest
X-ray.9 Both TTE and TEE are very effective in
diagnosis and distinguish of the aneurysm, such as pericardial cysts,
Ebstein’s anomaly, cortriatriatum and thrombosis.10Agitated saline study with echocardiography can be performed to confirm
the diagnosis and to distinguish from pericardial effusion or cysts,
demonstrating opacification of the RAAA and RV.2 TEE
plays an important role in further preoperative evaluation and planning
surgical procedures during resection of RAAA. In this case, before the
aneurysm resection TEE presented the giant RAAA, PFO and RV compression.
During the surgery, surgeons concerned the resection of the aneurysm
might change the entire anatomical structure, leading to tricuspid valve
dysfunction. Additionally TEE played a key role in confirming valve
competency during the surgery. In this case, the postoperative TEE
confirmed that the tricuspid valve still
functioned
normally without regurgitation or insufficiency. Therefore, the patient
was not indicated to tricuspid valvuloplasty. Taken together, TEE is
very helpful for surgeons to formulate detailed surgical plan and
evaluate the effect of surgical correction during cardiac surgery.
Finally, TTE is useful for long-term follow-up on this case since there
is no report regarding postoperative recurrence or death after RAAA
resection.
Although RAAA is a very rare anomaly in pediatric patients, prenatal
diagnosis via fetal echocardiography is possible. Clinical experience of
treatment for RAAA is limited and the natural history and optimal
management strategy are still unclear. Therefore, the therapeutic
strategies of RAAA remain controversial and aneurysm resection and
anticoagulation therapy are not recommended in all
cases.3 Surgical resection is preferred for patients
with symptoms or progressive enlargement in preventing thromboembolisms
and lowering the risk of atrial arrhythmia and heart failure, which are
the most common complications of these aneurysms.2,3Oral anticoagulants is recommended to reduce the risk of thromboembolic
events in patients with contraindications to surgery.4
In conclusion,
echocardiography
plays an important role in the diagnosis and surgical resection of RAAA
as well as postoperative evaluation of surgical effect. Treatment should
be modified according to the age, presentation, imaging findings and the
follow-up results.