Cardiac Magnetic Resonance Imaging and Multidetector Computed Tomography Scan Illustrating Damus–Kaye–Stansel Operation
The Damus–Kaye–Stansel (DKS) operation was first described in 1975 independently by 3 authors, Damus,1 Kaye,2 and Stansel,3 and was soon felt to be an effective method to relieve systemic ventricular outflow–tract obstruction in patients with ventriculoarterial discordance. Since then, the procedure has been applied to patients with several forms of complex congenital heart disease in which systemic outflow is obstructed. The operation consists of an anastomosis of the proximal end of the transected pulmonary artery to the side of the ascending aorta, ensuring unobstructed outflow to the systemic circulation through the pulmonary valve. The pulmonary circulation can be then reestablished by a right ventricle to the distal main pulmonary artery conduit in cases of biventricular repair, or by a Fontan procedure (systemic venous return to the pulmonary artery without the subpulmonary ventricle) in cases in which only a univentricular repair can be offered. We present 2 cases that illustrate the utility of cardiac magnetic resonance imaging and multidetector computed tomography in demonstrating the anatomic findings after a DKS operation.
Case 1:This 20-year-old patient was diagnosed at birth with tricuspid atresia, d-transposition of the great arteries, and coarctation of the aorta. He initially underwent a coarctation repair with a subclavian flap and pulmonary artery banding. Subsequently, he had univentricular repair consisting of a classic Fontan (right atrium to distal main pulmonary artery anastomosis) and a DKS operation to prevent future obstruction to the systemic circulation. A cardiac magnetic resonance imaging study was performed with a 1.5-T scanner (General Electric Medical Systems, Wankesha, Wis) using an 8-channel cardic phase-array coil (Toshiba Aquilon 64, Tokyo, Japan). Images by steady-state free-precession technique were obtained in multiple planes. A single sagittal oblique view (Figure 1) almost sufficed to define the entire complexity of the patient’s anatomy. A hypoplastic right ventricle was noted to communicate with a well-developed left ventricle through a nonrestrictive ventricular septal defect. The aorta was anterior and arose from the hypoplastic right ventricle through a muscular infundibulum; it was anastomosed to the proximal main pulmonary artery at 17 mm distal to the semilunar cusp’s tips. Therefore, both outflow tracts were committed to the systemic circulation. The site of the coarctation repair in the descending aorta could also be identified. There was diastolic spin dephasing upstream from the pulmonary valve, suggesting pulmonary insufficiency—one of the complications after DKS connection. Figure 2 is a corresponding echocardiogram of this patient.
Case 2:This 22-year-old patient, diagnosed at birth with a Taussig–Bing anomaly (double-outlet right ventricle with subpulmonary ventricular septal defect), straddling mitral valve, and aortic coarctation, underwent pulmonary artery banding and coarctation repair early in life. At the age of 5 years, he had a right classic Glenn operation (superior vena cava to right pulmonary artery anastomosis, with division of the right pulmonary artery at its origin). This was followed by a Fontan procedure (right atrium to distal main pulmonary artery) and a DKS operation at the age of 8 years. As a teenager, he required a stent implantation for aortic recoarctation and pacemaker insertion that would preclude magnetic resonance imaging assessment in the future.
An electrocardiography-gated cardiac computed tomography scan was performed with 64-multidetector computed tomography. Figure 3 shows a left anterior oblique view of a 3-dimensional volume-rendered image and a sagittal left anterior oblique projection of a curved, reformatted image. The images show both great arteries arising from a right-sided morphologically right ventricle, with the ventricular septal defect committed to the pulmonary valve. Similar to Case 1, the pulmonary artery is anastomosed to the ascending aorta just above the sinotubular junction. Figure 4 is the corresponding angiogram of this patient.
The online-only Data Supplement, consisting of Movies I through VI, is available with this article at http://circ.ahajournals.org/cgi/ content/full/115/18/e440/DC1.