Double outlet right ventricle, a complex of congenital cardiac anomalies in which both great arteries arise wholly or in large part from the morphologic right ventricle presents diverse clinical manifestations according to the morphological
characteristics.
Eighty cases with DORV were diagnosed and operated at Yonsei Cardiovascular Center from 1988 to 1992. The analysis of the morphological characteristics of this anomaly and comparative study of operative methods and mortality according to the
morphological classification led to the following results:
1) The location of VSD varied to be subaortic in 40 cases, subpulmonic in 15, doubly committed in 6 and noncommitted in 19 cases. The great arteries were interrelated in D-malposition in 43 cases and L-malposition in 18 and 26 cases among above
mentioned 61 cases revealed the side-by-side relationship. Ten of the cases showed normal position and the rest 9, A-malposition.
2) Pulmonary stenosis was found in 60 cases, and when associated with subpulmonic VSD, occurring less frequently. PDA and ASD secundum were associated in successional order and only 3 cases were verified to carry aortic arch anomalies such as
coarctation and interruption, all with subpulmonic VSD.
3) Intraventricular tunnel repair was possible in every case associated with subaortic VSD and the postoperative prognosis was excellent. In comparison, most of the cases associated with other types of VSD in which only palliative surgery or
various
types of intraventricular repair were performed, the general outcome was poor. But total cavopulmonary connection, one of the physiological corrective methods, showing a better postoperative prognosis is being carefully considered for its broad
adoptation in future.
In conclusion, double outlet right ventricle, a pathophysiological complex of various anomalies, should be thoroughly evaluated for the morphological characteristics to decide the most appropriate types of operation and for consequent improvement
of
prognosis.
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