Discussion

Cantrell’s Pentalogy is a rare syndrome, and those with complex cardiac defects requiring surgical intervention in neonatal and infantile age may end up with a high mortality rate and poor prognosis. The long-term prognosis mainly depends on the severity of the cardiac and accompanying defects and requires multi-disciplinary management due to the involvement of multisystem anomalies. ^{Reference Aydın, Suzan, Cevik, Odemis and Erek6} In contrast with most Cantrell’s patients, our patient had only ventricular diverticulum, and referring for operation at 6 years of age might increase procedural success. The patient’s parents postponed the surgery until the age of 6. This decision might increase the risk of ventricular arrhythmia and embolic events; fortunately, the child has never experienced such complications. The syndrome is characterised by a partial sternal cleft, anterior abdominal wall defects, anterior diaphragmatic defect, and intracardiac defects. ^{Reference Vazquez-Jimenez, Muehler and Daebritz7} The steps for closure are usually chest wall closure, closure of the sternal defect, closure of the omphalocele, placement of the heart in the thorax, and repair of the intracardiac defect. ^{Reference Pius, Abubakar Ibrahim, Bello and Bashir Tahir8} Nearly one-third of the cases with this syndrome have left ventricular diverticulum, which is a life-threatening abnormality that may cause systemic embolisation, fatal ventricular arrhythmias, and sudden death due to ventricle rupture. Early detection and surgical intervention for this pathology might be lifesaving. ^{Reference Scardigno, Chieppa, Deluca and Carbonara9}

Vazquez-Jimenez et al published a literature review of 153 Cantrell’s Pentalogy cases in 1998. According to the analysis, a ventricular septal defect was the most common cardiac malformation (72%). Atrial septal defect was present in 44 (34.6%) patients, left ventricular diverticulum in 41 (32.3%), pulmonary stenosis or atresia in 40 (31.5%), Tetralogy of Fallot was present in 22 patients (17.3%), dextrocardia in 19 (15.0%), and various malformations accompanied it in lesser numbers. ^{Reference Vazquez-Jimenez, Muehler and Daebritz7}

The analysis showed three cases of Cantrell’s Pentalogy, of which the intracardiac defect is limited with left ventricular diverticulum (6%), without any accompanying intracardiac defects. First of these three cases was a 2-year-old female patient with a lower sternal defect, an umbilical hernia, and a ventral diaphragmatic defect; the second case was a 2-year-old male patient with a short sternum, an omphalocele, and a diaphragmatic defect; and the third case was a 7-month-old male patient with a huge ventral hernia, a diaphragmatic defect, and a pericardial defect coherent with the incomplete syndrome. In addition, a case of complete syndrome of which the intracardiac defect is limited to the left and right ventricle along with a bifid sternum, an omphalocele, a ventral hernia, and absent pericardium has been reported. ^{Reference Vazquez-Jimenez, Muehler and Daebritz7}

Turbendian et al reported another incomplete case of Cantrell’s Pentalogy with an intracardiac defect limited to the left ventricular diverticulum along with a Patent Ductus Arteriosus, an omphalocele, a pericardial, and a diaphragmatic defect. ^{Reference Turbendian, Carroll and Chen10}

According to the Toyama classification, our case fulfils all requirements of the complete syndrome with supraumbilical abdominal wall defect, lower sternal cleft, defect of the diaphragm in the central tendon, pericardium defect, and left ventricular diverticulum. According to the previous research, the left ventricular diverticulum is a defect related to Cantrell’s syndrome almost always accompanies other intracardiac defects. ^{Reference Halbertsma, van Oort and van der Staak11} However, the intracardiac defect is limited to the left ventricle diverticulum in our patient.

In summary, our case is interesting because of the presence of the left ventricular diverticulum alone. Also, isolated ventricular diverticulum often accompanies incomplete Cantrell’s cases, and our case is one of the rarest complete Cantrell’s syndromes with no other intracardiac defects.