Original article: cardiovascularAnatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies
Section snippets
Patient population
Thirteen patients with balanced forms of complete AVSDs and associated major cardiac anomalies underwent anatomically corrective repair between January 1988 and February 2000 at Kobe Children’s Hospital. Eight of the 13 patients had atrial isomerism (right in 1, left in 7) with DORV in 5 and associated cardiac anomalies in 3. Four of the other patients had DORV with trisomy 21, and 1 had tetralogy of Fallot. Their ages at operation ranged from 5 months to 10 years (median 4.7 years). All but 1
Results
Two hospital deaths (15%) occurred only in the isomerism group. One (patient 1) had a cardiac arrest 15 hours after the operation; another (patient 6) had severe right ventricular cardiac failure and died 8 days postoperatively. Doppler echocardiography immediately after each operation revealed moderate to severe mitral incompetence in 1, moderate in 2, mild in 5, and trivial in 3.
Patients were followed up from 2 months to 12 years (average 4.4 years). There was one late death in the isomerism
Comment
Recently satisfactory results of corrective surgery for complete AVSDs with complex cardiac lesions such as DORV or atrial isomerism have been reported 1, 2, 3, 4. In Kobe Children’s Hospital, DORV has been observed in 14% of the patients who underwent corrective repair of complete AVSDs. One-third of the candidates for definitive repair among the isomerism patients received corrective surgery, the goal of which involves both anatomical and physiological correction of these complex anomalies.
References (12)
- et al.
Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies
Ann Thorac Surg
(1988) - et al.
Complete atruoventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow obstruction. A report of successful surgical treatment
Ann Thorac Surg
(1986) - et al.
Biventricular repair of cardiac isomerism with common atrioventricular canal with the aid of an endocardial cushion prosthesis
J Thorac Cardiovasc Surg
(1993) - et al.
Biventricular repair in cardiac isomerism. A report of seventeen cases
J Thorac Cardiovasc Surg
(1995) - et al.
Repair of left superior vena cava entering the left atrium
Ann Thorac Surg
(1986) - et al.
Surgical repair of subaortic stenosis in atrioventricular canal defects
J Thorac Cardiovasc Surg
(1994)
Cited by (12)
Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis
2022, Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental AnalysisLong-Term Outcome After Pulmonary Artery Banding in Children With Atrioventricular Septal Defects
2018, Annals of Thoracic SurgeryCitation Excerpt :More challenging forms of AVSD include those with bAVSD with early heart failure or additional malformations that render early repair difficult, as well as patients with uAVSD, in whom complete repair may not be feasible at all. In patients with bAVSD, the use of PAB may allow for the patient to grow with a protected pulmonary circulation, permitting later biventricular repair [9–12]. However, it has previously been unclear how successful this strategy would be, and there has been concern that PAB may contribute to progressive LAVVR [12], which is a major contributor to morbidity in patients with AVSD [13–19].
Contemporary management of right atrial isomerism: Effect of evolving therapeutic strategies
2006, Journal of Thoracic and Cardiovascular SurgeryCorrection of complete atrioventricular septal defects with two patch technique
2004, Operative Techniques in Thoracic and Cardiovascular SurgeryHearts with isomerism of the right atrial appendages - One of the worst forms of disease in 2005
2005, Cardiology in the YoungAnesthesia for Left-to-Right Shunt Lesions
2015, Anesthesia for Congenital Heart Disease: Third Edition