The Ross-Konno procedure in neonates and infants less than 3 months of age
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Published on: April 19, 2017
Eva Sames-Dolzer1, Elisabeth Wickenhauser2, Michaela Kreuzer1
1Division of Pediatric and Congenital Heart Surgery, Children's Heart Center Linz, Kepler University Hospital, Linz, Austria.
Insights
The early Ross-Konno procedure offers complete repair for severe left ventricular outflow tract obstruction, enabling functional recovery. While generally successful, critical aortic stenosis with aortic arch hypoplasia presents the highest operative risk.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease Repair
- Cardiovascular Surgery
Background:
- Severe left ventricular outflow tract obstruction (LVOTO) often leads to impaired ventricular function and endocardial fibroelastosis.
- Borderline left ventricular (LV) size is a common challenge in neonates and infants with LVOTO.
- Early surgical intervention is crucial for functional recovery and growth in affected children.
Purpose of the Study:
- To evaluate the efficacy and outcomes of the early Ross-Konno procedure in neonates and infants with severe LVOTO.
- To identify prognostic factors influencing mortality and reoperation rates.
- To assess the long-term functional recovery and growth potential after the procedure.
Main Methods:
- Retrospective analysis of 44 early Ross-Konno procedures performed between 2008 and March 2017.
- Inclusion of neonates and infants (under 3 months) with severe LVOTO.
- Examination of in-hospital/late mortality, postoperative complications, reoperations, and prognostic factors (fetal intervention, VSD, hypoplastic arch, endocardial fibroelastosis).
Main Results:
- In-hospital mortality was 7%, with late mortality at 2%.
- The highest mortality (27%) was observed in patients with critical aortic stenosis and aortic arch repair (P=0.012).
- Mid-term survival was 87% for patients with fetal intervention; 19 of 40 survivors required reoperation over 5.9 years.
Conclusions:
- The early Ross-Konno procedure facilitates biventricular repair in borderline left ventricles, yielding good long-term outcomes.
- No reoperations were needed at the autograft or LVOT level in this cohort.
- Critical aortic stenosis combined with aortic arch hypoplasia represents the highest surgical risk.
Objectives:
Patients with severe left ventricular outflow tract obstruction often suffer from impaired left ventricular function, endocardial fibroelastosis and borderline-sized structures. The early Ross-Konno operation can offer complete repair due to outflow tract stenosis and enable the functional recovery and growth of small structures.
Methods:
Between 2008 and March 2017, 44 early Ross-Konno procedures were performed at our centre. Thirty-five patients were neonates, and 9 were infants less than 3 months of age. A retrospective single-centre investigation was carried out analysing early and late deaths, postoperative complications and reoperations during the follow-up period. Potential prognostic influence factors as previous fetal intervention, associated lesions as presence of a VSD or hypoplastic aortic arch or severe endocardial fibroelastosis were examined.
Results:
The in-hospital mortality rate was 7% (3 of 44), and the late mortality rate was 2%. There were no deaths in the group with ventricular septal defects (9 cases), and there was 1 death in the group with critical aortic stenosis without arch repair (1 of 24; 4%). The most deaths occurred in patients with critical aortic stenosis and aortic arch repair (3 of 11; 27%) (P = 0.012). Fifteen patients with foetal intervention had a mid-term survival rate of 87%. Reoperations were necessary in 19 of 40 surviving patients during a mean follow-up period of 5.9 years.
Conclusions:
The early Ross-Konno procedure can enable biventricular repair even in borderline left ventricles with good long-term outcome. Thus far, there were no reoperations at the level of the autograft or the left ventricular outflow tract in our cohort. The highest operative risk was observed in patients with critical aortic stenosis and aortic arch hypoplasia.
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