Symptomatic partial and transitional atrioventricular septal defect repaired in infancy
Sylvia Krupickova1, Gareth J Morgan2, Mun Hong Cheang3
1Department of Paediatric Cardiology, Royal Brompton Hospital, London, UK.
Insights
Infants with symptomatic atrioventricular septal defects need early surgical repair. Unfavorable left atrioventricular valve anatomy is a key risk factor for reoperation in these young patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Surgery
- Pediatric Cardiac Surgery
Background:
- Symptomatic infants with partial and transitional atrioventricular septal defects (AVSD) undergoing early surgical repair are considered high-risk.
- The specific outcomes and risk factors for this patient group are not well-defined.
Purpose of the Study:
- To evaluate the outcomes of symptomatic infants undergoing early surgical repair for partial and transitional AVSD.
- To identify risk factors associated with mortality and reoperation in this cohort.
Main Methods:
- A multicenter study involving 51 infants (0-1 year) with symptomatic partial/transitional AVSD requiring biventricular repair.
- Data collected on patient demographics, surgical procedures, valve anatomy, and clinical outcomes including mortality and reoperation rates.
Main Results:
- Three in-hospital deaths (5.9%) occurred post-primary repair.
- Eleven patients (22%) required reoperation, primarily for left atrioventricular valve regurgitation/stenosis.
- Unfavorable left atrioventricular valve anatomy was the sole predictor of reoperation (31% of patients had unfavorable anatomy).
Conclusions:
- Early surgical repair of symptomatic partial/transitional AVSD in infants leads to significant morbidity and mortality.
- A high reoperation rate is associated with unfavorable left atrioventricular valve anatomy in this population.
Objectives:
Infants with symptomatic partial and transitional atrioventricular septal defect undergoing early surgical repair are thought to be at greater risk. However, the outcome and risk profile of this cohort of patients are poorly defined. The aim of this study was to investigate the outcome of symptomatic infants undergoing early repair and to identify risk factors which may predict mortality and reoperation.
Methods:
This multicentre study recruited 51 patients (24 female) in three tertiary centres between 2000 and 2015. The inclusion criteria were as follows: (1) partial and transitional atrioventricular septal defect, (2) heart failure unresponsive to treatment, (3) biventricular repair during the first year of life.
Results:
Median age at definitive surgery was 179 (range 0-357) days. Sixteen patients (31%) had unfavourable anatomy of the left atrioventricular valve: dysplastic (n=7), double orifice (n=3), severely deficient valve leaflets (n=1), hypoplastic left atrioventricular orifice and/or mural leaflet (n=3), short/poorly defined chords (n=2). There were three inhospital deaths (5.9%) after primary repair. Eleven patients (22%) were reoperated at a median interval of 40 days (4 days to 5.1 years) for severe left atrioventricular valve regurgitation and/or stenosis. One patient required mechanical replacement of the left atrioventricular valve. After median follow-up of 3.8 years (0.1-11.4 years), all patients were in New York Heart Association (NYHA) class I. In multivariable analysis, unfavourable anatomy of the left atrioventricular valve was the only risk factor associated with left atrioventricular valve reoperation.
Conclusions:
Although surgical repair is successful in the majority of the cases, patients with partial and transitional atrioventricular septal defect undergoing surgical repair during infancy experience significant morbidity and mortality. The reoperation rate is high with unfavourable left atrioventricular valve anatomy.
Related Concept Videos
Properties of Transition Metals
Socioemotional Development during Infancy
Primary Temperament Types
Phase Transitions
Phase Transitions: Melting and Freezing
Phase Transitions: Sublimation and Deposition
Mismatch Repair


