Introduction:
The incidence of the left ventricular diverticulum is reported to be
0.05% of all congenital heart malformations. It is currently believed
that it occurs as a result of impaired development of the endocardial
tube during the 4th week of embryologic development. Although it may
present as an isolated disorder, this entity is often associated with
other cardiac abnormalities or with Cantrell pentalogy in around 70% of
cases (1,2). Cantrell’s Pentalogy is a rare syndrome with an estimated
incidence of 5.5 per 1 million live births. It was defined by Cantrell
et al. in 1958 as a condition with different degrees of five defects:
supraumbilical abdominal wall defect, lower sternal cleft, defect of the
diaphragm in the central tendon, pericardium defect, and various
intracardiac anomalies. They considered the intracardiac defects as an
integral of this association and further described the frequency as
100% for ventricular septal defect, 53% for atrial septal defect, 33%
for pulmonary stenosis (either valvular or infundibular), 20% for left
ventricular diverticulum, and described Tetralogy of Fallot as the only
established cardiac syndrome to be accompanying this syndrome with a
frequency of 20% (3). In 1972, Toyama et al. proposed a classification
of this syndrome into three subtypes. The first subtype is the complete
syndrome with all five defects. The second subtype is the probable
syndrome with four defects, including intracardiac and ventral wall
anomalies. The third subtype is the incomplete syndrome with various
combinations of defects sternal abnormality (4).
We describe a 6-year-old male patient with a pulsatile mass extending
from the lower chest to the upper abdomen. The IRB approval, consent
statement and clinical trial registration were waived.