DEVELOPMENT OF A DOUBLE-CHAMBERED RIGHT VENTRICLE AFTER SURGICAL CLOSURE OF A VENTRICULAR SEPTAL-DEFECT

The double-chambered right ventricle (DCRV) is a rare cardiac malforma tion, often associated with ventricular septal defect (VSD). Case repo rt: The authors describe a case of DCRV in a child who underwent surgi cal repair of perimembranous VSD at the age of 7 months because of sys temic pulmonary artery hypertension and growth retardation. At this ti me, no pressure gradient was measured within the right ventricle and n o muscle proliferation was observed on echocardiography or right ventr iculography. Postoperative follow-up revealed hypertrophy of the moder ator band accompanied by progressive development of a right midventric ular pressure gradient, which reached 60 mm Hg at the age of 3 1/2 yea rs. The child was operated after invasive confirmation of the diagnosi s of DCRV and the haemodynamic data. The child is now 6 years. old and no restenosis has been observed. Conclusion: DCRV is usually associat ed with VSD, but the course of the two diseases can be dissociated. Th e diagnosis of DCRV must be considered in any child with a history of VSD, even after surgical or spontaneous closure, when clinical examina tion reveals a systolic ejection murmur.