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.