TY - JOUR
T1 - Anatomic, Imaging, and Clinical Characteristics of Double-Inlet, Double-Outlet Right Ventricle
AU - Saleeb, Susan F.
AU - Juraszek, Amy
AU - Geva, Tal
N1 - Funding Information:
This study was supported by the Higgins Family Noninvasive Cardiac Imaging Research Fund, Children's Hospital Boston, Massachusetts.
PY - 2010/2/15
Y1 - 2010/2/15
N2 - The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up. Among the 22 patients with DI-DORV, 14 had clinical data, 5 had clinical and autopsy data, and 3 had only autopsy data. Abdominal and atrial situs were normal in all, and heart position was levocardia in 91%. The morphology of the atrioventricular valves was variable, with 15 patients showing neither a typical tricuspid nor mitral valve. In the remaining 7 patients, 1 or both atrioventricular valves resembled a tricuspid or a mitral valve. Myocardial architecture was characterized by atypical muscle bundles of varying degrees of hypertrophy and orientation. Most patients had bilateral conus, and 82% had either aortic or pulmonary outflow tract obstructions. The rate of overall mortality or heart transplantation was 36%. Significant differences between DI-DORV and DILV included the type of ventricular loop, the type of conus, and a higher mortality rate in patients with DI-DORV. In conclusion, DI-DORV is a distinct type of functional single ventricle congenital heart disease with variable atrioventricular valve morphology and myocardial architecture. Accurate diagnosis using noninvasive imaging techniques is of paramount importance for optimal management.
AB - The purposes of this study were to characterize the morphologic, imaging, and clinical characteristics of double-inlet, double-outlet right ventricle (DI-DORV), a rare congenital heart disease, and to compare these findings to those in patients with double-inlet left ventricle (DILV). The clinical records, imaging studies, and heart specimens of 22 consecutive patients with DI-DORV were reviewed. A comparison group of 44 patients with DILV was matched by age at latest follow-up. Among the 22 patients with DI-DORV, 14 had clinical data, 5 had clinical and autopsy data, and 3 had only autopsy data. Abdominal and atrial situs were normal in all, and heart position was levocardia in 91%. The morphology of the atrioventricular valves was variable, with 15 patients showing neither a typical tricuspid nor mitral valve. In the remaining 7 patients, 1 or both atrioventricular valves resembled a tricuspid or a mitral valve. Myocardial architecture was characterized by atypical muscle bundles of varying degrees of hypertrophy and orientation. Most patients had bilateral conus, and 82% had either aortic or pulmonary outflow tract obstructions. The rate of overall mortality or heart transplantation was 36%. Significant differences between DI-DORV and DILV included the type of ventricular loop, the type of conus, and a higher mortality rate in patients with DI-DORV. In conclusion, DI-DORV is a distinct type of functional single ventricle congenital heart disease with variable atrioventricular valve morphology and myocardial architecture. Accurate diagnosis using noninvasive imaging techniques is of paramount importance for optimal management.
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U2 - 10.1016/j.amjcard.2009.09.056
DO - 10.1016/j.amjcard.2009.09.056
M3 - Article
C2 - 20152252
AN - SCOPUS:75349096342
SN - 0002-9149
VL - 105
SP - 542
EP - 549
JO - American Journal of Cardiology
JF - American Journal of Cardiology
IS - 4
ER -