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J Thorac Cardiovasc Surg 1999;117:622-623
© 1999 Mosby, Inc.

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TRANSANNULAR PATCH REPAIR OF DOUBLE-OUTLET RIGHT VENTRICLE, {S,D,L}, AND SINGLE RIGHT CORONARY ARTERY

From the Department of Cardiothoracic Surgery, University of Tokyo, Tokyo, Japan, and the Department of Thoracic and Cardiovascular Surgery, Kanagawa Children's Medical Center, Yokohama, Japan.

Received for publication Sept 2, 1998. Accepted for publication Oct 16, 1998. Address for reprints: Yukihiro Kaneko, MD, Department of Cardiothoracic Surgery, General Hospital, University of Wien, Währinger Gûrtel 18-20, 1090 Wien, Austria.

A heart murmur and cyanosis were noted in a newborn female baby. When she was 5 years of age, cardiac catheterization and angiography revealed double-outlet right ventricle (DORV) with bilateral conus, L-malposed great arteries (that is, {S,D,L} in van Praagh's classification), single right coronary artery (RCA), and pulmonary stenosis caused by subpulmonary hypertrophied muscle and a hypoplastic pulmonary anulus. Inasmuch as the patient was free of symptoms and surgical repair was considered to entail considerable risk, repair was postponed.

At 21 years of age, because of her intention to become pregnant, the patient was referred to us again after a second cardiac catheterization and angiography. The study revealed the aforementioned anomaly and left juxtaposition of the atrial appendages. Pulmonary valve diameter was 16 mm (Z-value –2.7). The systolic pressure gradient across the pulmonary outflow tract was 83 mm Hg. An angiogram showed sufficient space at the right side of the subpulmonary conus between the pulmonary valve and the RCA (Fig. 1). Cardiac repair with transannular patch enlargement of the pulmonary outflow was planned.

View larger version (140K): [in this window] [in a new window]   Fig. 1. Preoperative left ventriculogram showed L-malposed great arteries and bilateral conus. The pulmonary anulus (white arrows) and RCA (black dots) were relatively distant at the right side of the pulmonary outflow. Ao, Aorta; LV, left ventricle; RV, right ventricle.

View larger version (50K): [in this window] [in a new window]   Fig. 2. Drawing of the surgical view after placement of a transannular incision. The pulmonary and tricuspid valves are visualized through the incision. PA, Pulmonary artery; PV, pulmonary valve; TV, tricuspid valve; RCA, right coronary artery.

Kirklin and Barratt-Boyes ² stated that in such patients an extracardiac conduit between the right ventricle and the pulmonary artery was nearly always necessary at the time of correction. However, the location of the coronary artery in the present patient required the area of proximal conduit anastomosis to be immediately behind the sternum. Placement of either a conduit to the main pulmonary artery to the right of the aorta or a conduit to the left pulmonary artery to the left of the aorta appeared prone to conduit compression between the sternum and right ventricle. ³

The REV procedure (réparation à l'étage ventriculaire) is another option. ⁴ However, the translocated pulmonary artery might be compressed between the sternum and anteriorly displaced aorta.

Lincoln ⁵ dissected out the RCA and sutured a transannular patch underneath it to enlarge the pulmonary valve ring in this anomaly. This technique stretches the RCA and thereby predisposes to coronary insufficiency. Coronary artery bypass to the distal RCA after transection of the RCA also allows transannular enlargement. ³ Nevertheless, although coronary artery bypass is effective for augmenting already impaired coronary flow, it may not perfuse hypertrophied right ventricular muscle sufficiently after obliteration of the normal coronary artery.

In the present case, it was possible to relieve pulmonary stenosis properly by placing a transannular patch on the right side of the subpulmonary conus without jeopardizing the RCA. We relieved pulmonary outflow tract obstruction sufficiently by the same technique in another case with DORV, L-malposition, and a pulmonary valve Z-value of -3.0. Because the RCA arising from the levoposed aorta typically runs obliquely toward the right atrioventricular groove, there is a better chance of making a longer transannular incision at the right side of the pulmonary outflow tract than in the ventral surface. As suggested by the surgical experience with tetralogy of Fallot repair, a limited incision into the right ventricle greatly facilitates enlargement of the pulmonary anulus. Since DORV with L-malposition usually has bilateral conus and the coronary arteries are rarely located within the subpulmonary conus, transannular patch enlargement of the pulmonary anulus on the right side of the pulmonary outflow tract is probably feasible in the majority of patients with DORV and L-malposition, in whom the RCA is not adjoining the pulmonary anulus.

References

1. Otero Coto E, Castaneda AR, Caffarena JM, Deverall PB, Cabrena A, Quero Jimenez M. L-Malposed great arteries with situs solitus and concordant atrioventricular connection. J Cardiovasc Surg 1982;23:277-86. [Medline]
   
2. Kirklin JW, Barratt-Boyes BG. Cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. 2nd ed. New York: Churchill Livingstone; 1993. p. 1493-4.
   
3. Van Praagh R, Pérez-Trevino C, Raynolds JL, Moes CAF, Keith JD, Roy DL, et al. Double outlet right ventricle {S,D,L} with subaortic ventricular septal defect and pulmonary stenosis. Am J Cardiol 1975;35:42-53. [Medline]
   
4. Houyel L, Van Praagh R, Lacour-Gayet F, Serraf A, Petit J, Bruniaux J, et al. Transposition of the great arteries {S,D,L}. J Thorac Cardiovasc Surg 1995;110:613-24. [Abstract/Free Full Text]
   
5. Lincoln C. Total correction of D-loop double-outlet right ventricle with bilateral conus, L-transposition, and pulmonic stenosis. J Thorac Cardiovasc Surg 1972;64:435-40. [Medline]
   

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This Article Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Hideo Okabe Shinichi Takamoto Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kaneko, Y. Articles by Takamoto, S. Search for Related Content PubMed PubMed Citation Articles by Kaneko, Y. Articles by Takamoto, S.