Original article
Pediatric cardiac
Variations of Atrioventricular Septal Defects Predisposing to Regurgitation and Stenosis

Presented at the Forty-sixth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 25–27, 2010.
https://doi.org/10.1016/j.athoracsur.2010.03.098Get rights and content

Background

An additional malformation of the atrioventricular valve is occasionally encountered in patients with complete atrioventricular septal defect, and this may compromise accurate correction.

Methods

We reviewed 138 patients undergoing complete repair with two-patch technique between 1992 and 2008. The mean age was 7.1 ± 8.3 months, and the mean body weight was 5.1 ± 2.1 kg. Preceding pulmonary arterial banding was performed in 23 patients.

Results

The operative record delineated additional malformations of the atrioventricular valve that posed difficulty in positioning the ventricular septal patch and in accurately approximating the cleft in 45 patients. Of them, four types (n = 40) were associated with increased incidence of postoperative left valvular problems (moderate or worse regurgitation or stenosis). These included abnormalities of the papillary muscles that accompanied hypoplastic mural leaflet or incomplete opening of one commissure in (n = 15; p = 0.0054), dense insertion of the chords of the superior leaflet that obscured the right side of the ventricle septal crest in (n = 13; p = 0.0004), double orifice valve (n = 7; p = 0.0225), and severe length disparities of the cleft that resulted from either disproportional size of superior against inferior leaflets or redundant chord supporting the left extremity of one of these leaflets (n = 5; p < 0.0001). Neither greater age at operation (more than 6 months) nor preceding pulmonary arterial band reduced the incidence of left valvular problems in the malformation group.

Conclusions

An individualized technique is required to maintain coaptation of the atrioventricular valve, but in many cases, they are not completely correctable. Deferring complete repair by placing a pulmonary arterial band did not reduce left valvular problems.

Section snippets

Patients and Methods

The study was approved by the Institutional Review Board of Sakakibara Heart Institute, and the procedures were in accordance with institutional guidelines for protection of patient confidentiality. The need for patient consent was waived.

We reviewed 138 patients with CAVSD—including 75 female patients—undergoing complete repair between 1992 and 2008. Patients with tetralogy of Fallot or double-outlet right ventricle were excluded. Five other patients having CAVSD (with distinct two ventricles)

Overall Data

The mean age at operation was 7.6 ± 10.0 months, and the mean follow-up period was 3.4 ± 3.5 years (maximum 15.5). The AVV was Rastelli type A in 80 patients and type C in 58. The AVV cleft was completely closed in 112 patients (81.2%). The reasons for not closing the cleft were the anticipation of causing left AVV stenosis in 16 and the fragile valve tissue in 10. The mean cardiopulmonary bypass and aortic cross-clamp times were 116.1 ± 31.7 minutes and 83.9 ± 22.1 minutes, respectively. The

Comment

The surgical outcome of CAVSD has improved owing to better anatomical understanding and improved techniques. Early primary repair at 3 to 6 months of age has become common practice [3], and hence irreversible pulmonary vascular obstructive disease is rarely seen. Despite low operative mortality rates, however, left AVV regurgitation or stenosis may persist after operation and compromise patients' quality of life [6, 9]. Left AVV regurgitation is the most common indication for reoperation after

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