Congenital heart disease
Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: The 26-year Melbourne experience

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Background

To review the long-term outcomes of patients born with single-ventricle physiology and aortic arch obstruction.

Methods

Follow-up of 70 consecutive neonates undergoing single-ventricle palliation and arch repair, excluding hypoplastic left heart syndrome, between 1983 and 2008, was reviewed. Dominant arch anomalies were coarctation (n = 48), interrupted arch (n = 10), and hypoplastic arch alone (n = 12). Neonatal Damus procedure with arch repair and shunt became the dominant approach, being performed in 1 (10%) of 10 in 1983 to 1989, 9 (32%) of 28 in 1990 to 1999, and 23 (72%) of 32 in 2000 to 2008.

Results

All patients underwent an initial procedure at a median of 6 days (range, 4-12 days): pulmonary artery banding and arch repair (n = 35); Damus, arch repair, and shunt (n = 33); and other (n = 2). Twenty-six patients died before Fontan completion. Of the 34 survivors of initial banding, 17 (50%) later required a Damus and 4 (12%) required subaortic stenosis relief. Forty patients underwent Fontan completion at a median age of 5 years (range, 4-7 years). After a mean of 5 ± 6 years after Fontan, there was 1 hospital death and 1 Fontan takedown. Overall survival was similar if patients initially underwent a Damus or pulmonary artery banding (P = .3). Overall survival at 10 years was 53% (95% confidence interval, 42%-67%).

Conclusions

Patients born with single-ventricle physiology and arch obstruction have a high risk of mortality in the first years of life. Their outcomes seem excellent once they reach Fontan status. It is likely that, in patients with single-ventricle and arch obstruction, strategies to avoid systemic outflow tract obstruction should be implemented in early life, and regular monitoring of blood pressure is warranted.

CTSNet classification

20
26.1.1

Abbreviations and Acronyms

BCPS
bidirectional cavopulmonary shunt
BT
Blalock-Taussig
CI
confidence interval
DKS
Damus-Kaye-Stansel
DORV
double-outlet right ventricle
HR
hazard ratio
PA
pulmonary artery

Cited by (0)

This study was supported by the Victorian Government's Operational Infrastructure Support Program. Dr d'Udekem is a Career Development Fellow of The National Heart Foundation of Australia (CR 10M 5339). Dr Iyengar is a cofounded Postgraduate Scholar of the National Health and Medical Research Council/National Heart Foundation of Australia (APP1038802), and holds an Royal Australian College of Surgeons Catherine Marie Enright Kelly Postgraduate Scholarship.

Disclosures: Authors have nothing to disclose with regard to commercial support.