J Korean Pediatr Soc.  1996 Mar;39(3):354-360.

Clinical Studies of Tetralogy of Fallot Associated with Complete Atrioventricular Septal Defect

• Song MS
• Noh CI
• Choi JY
• Yun YS
• Kim YJ

Abstract

PURPOSE
The association of the tetralogy of Fallot(TOF) with the complete form of atrioventricular septal defect(AVSD) is rare cardiac malformation.The predominant clinical findings in both groups of cases were those associated with tetralogy of Fallot, but certain features need to be emphasized, however.
METHODS
Forteen(2%) out of 659 patients with tetralogy of Fallot seen at the department of pediatrics, Seoul National University Hospital from January 1986 to June 1994, were associated with the complete form of AVSD. We reviewed clinical records, echocardiographic findings, angiographic findings and operative findings of patients. Our diagnostic criteria for TOF with complete AVSD are as follows. 1) Anterosuperior displacement of infundibular septum and RVOT obstruction. 2) Large (nonrestrictive) VSD and an overriding aorta. 3) Primum ASD with common AV valve.Goose-neck deformity of LVOT.
RESULTS
1) Only 14 cases(2%) of the 659 TOF patients were associated with complete AVSD. 2) The male to female ratio was 1:2.5. 3) The predominant features were those associated with TOF, but 9 patients(64.4%) had Down's syndrome. 4) The electrocardiogram showed left axis deviation except 2 patients. 5) Most common type among 14 patients with complete AVSD defect was Rastelli type C(78.5%). 6) Associated cardiovascular anomaly included PDA, ASD of secundum type, right aortic arch, left superior vena cava. 7) Among 12 patients who underwent operations, 9 patients are still alive and followed up.
CONCLUSIONS
TOF associated with complete AV septal defect was a rare disease. There was a clinical difference between TOF and AVSD combined groups of case. The presence of Down's syndrome or left-axis deviation with TOF increases the likelihood of associated complete AVSD.Careful preoperative evaluation of these patient is essential to their management and successful operative treatment.

Keyword

Tetralogy of Fallot; Atrioventricular septal defect; Endocardial cushion defect

MeSH Terms

Aorta
Aorta, Thoracic
Axis, Cervical Vertebra
Congenital Abnormalities
Down Syndrome
Echocardiography
Electrocardiography
Endocardial Cushion Defects
Female
Humans
Male
Pediatrics
Rare Diseases
Seoul
Tetralogy of Fallot*
Vena Cava, Superior