Long-term results of one-and-a-half ventricle repair in complex cardiac anomalies
Keywords : One-and-a-half-ventricle repair, Right-tricular venhypoplasia, Right-ventricular dysfunction.
One-and-a-half ventricle repair is a surgical
option for complex cardiac anomalies characterized by
right-ventricle hypoplasia or dysfunction. The long-term
result analyses or large clinical reviews are rare. The aim
of this study is to evaluate the long-term functional results
of this surgical procedure.
Methods
The 29 patients, who underwent one-and-a-half
ventricle repair from June 1993 to June 2007, at our Institution,
were included. The median age was 26 months
(range 6 months to 26 years). One-and-a-half-ventricle repair
was performed for volume unloading the small right
ventricle (group A, n = 18), for work unloading in patients
with chronic right-ventricle dysfunction (group B, n = 9),
and with the acute postoperative right-ventricular dysfunction
(group C, n = 2). The mean Z value of the tricuspid
valve in group A was -3.6 ± 0.7 (range -2.6 to -4.8). The
median follow-up duration of hospital survivors was 82
months (range 3 months to 16 years).
Results
There were four early deaths (two in group A and
C, respectively) and no late cardiac death. During followup,
no patient had superior vena cava (SVC) hypertension
or chronic atrial arrhythmia. There was one patient with
protein-losing enteropathy. Functional status was New
York Heart Association Functional Class I in 21 patients
and class II in three patients. Arterial oxygen saturation
increased significantly after operation, compared with thepreoperative saturation (86.6 ± 9.7-96.8 ± 4.0%, p < 0.01).
Two patients in group B needed medications related to the
cardiac function. Four patients underwent reoperation.
The 10-year freedoms from late reoperation were 80.0 ±
12.6% in group A and 51.4 ± 20.4% in group B.
Conclusion
The patients with one-and-a-half ventricle
repair resulted in favorable late survival in this series. During
the follow-up period, most surviving patients showed
good functional status without common late complications
of the Fontan procedure such as, recurrent cyanosis,
pulmonary arteriovenous fistulas, chronic arrhythmias,
and SVC syndrome. This procedure appears to be a valid
alternative to Fontan and biventricular repairs in patients
with right-ventricular dysfunction or hypoplasia.
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