Do we really need a new classification for cor triatriatum
sinister?
We have read with great interest the manuscript written by Mashadi and
colleagues,
”Cor
Triatriatum Sinister: Long-term Surgical Outcomes in Children and a
Proposal for a New Classification. (1)” Cor triatriatum sinistrum (CTS)
is a rare cardiac congenital malformation and represents around
0.1-0.4% of all congenital heart defects, with a very limited number of
cases reported in the literature (2, 3). CTS describes an anomaly where
the left atrium is subdivided by a thin membrane resulting in two
chambers. Many classifications have been used to describe CTS; the most
commonly used are the Loeffler’s, Lam’s, and Lucas’s, which are mainly
anatomical.
Loffler’s classification was developed in 1949, and it depends on the
size and number of fenestrations communicating the two atrial chambers.
In Loffler’s classification, group 1 has no connection between the two
chambers, and group 3 has free communication between the two chambers
(4). Later in 1962, Lam’s classification came to elaborate more on the
morphological features of CTS, including pulmonary venous drainage and
the site of the atrial septal defect (5). Lucas’ classification added
further anatomical details and described the association between CTS and
anomalous drainage of the pulmonary veins, which the Society of Thoracic
Surgeons project adapted for Congenital Heart Surgery Nomenclature and
Database (6). Other uncommon classifications described the anatomical
features of the accessory left atrial chamber and membrane (7) or the
entry of the pulmonary veins into the accessory chamber (8).
The current classifications do not describe the complex nature and
possible presentation of CTS that may affect long-term outcomes. Lam’s
classification takes into consideration the location of drainage of the
pulmonary veins and if there is an atrial septal defect (ASD) or not.
However, it does not consider the number and size of communication
between the two chambers (9, 10). CTS can be associated with other
cardiac anomalies in up to 80% of the cases; the most commonly
associated cardiac anomalies are
atrial
septal defect (ASD) in around 60% of patients (11, 12) and partial or
total anomalous
pulmonary venous
return, which has been reported in nearly one-third of the patients
with CTS (13). Several studies showed the association of CTS with other
congenital anomalies, such as bicuspid aortic valve, atrioventricular
canal, tetralogy of Fallot and ventricular septal defect (1, 14). The
early and long-term outcomes may vary according to the associated
congenital cardiac defects, and in the previously mentioned
classifications, a full range of associated congenital heart defects was
not taken into consideration.
Additionally, Nagao and colleagues found better survival in CTS patients
who had biventricular repair compared to the univentricular pathway
(15). Obviously, the univentricular heart was not considered in the
previous CTS classifications despite the reported worse outcomes in this
category. Furthermore, the exact surgical approach is chosen primarily
based on the presence of associated lesions and the size of the atria.
The proposed classification mainly depends on the possible presentations
and potentially addresses the long-term outcome (1). Mashadi and
colleagues divided the patients into three groups: isolated without
other congenital heart defects, in association with single ventricle
physiology, and CTS concomitant with other congenital heart defects.
This proposed classification addresses the anatomical factors previously
presented in older classifications, in addition to the associated heart
defects and single ventricle pathology. The outcomes could differ in
each group; therefore, this proposed classification could have a
prognostic value and can anticipate the possible outcomes for each
group.
This classification seems beneficial in underlining the anatomical and
pathophysiological presentation of CTS; however, its value is still
limited, pending a multicenter study or a pooled analysis with a larger
sample size to correlate the outcomes of CTS with each classification
category.