title: Double Outlet Right Ventricle With Intact Ventricular Septum: Avulsion or Exclusion.
abstract: Double outlet right ventricle (DORV) is almost always associated with a ventricular septal defect. The variant of DORV with intact ventricular septum is very uncommon and may be associated with fetal demise or death immediately after birth. Reports of successful palliation of these patients to the stage of superior cavopulmonary anastomosis (bidirectional Glenn shunt) are rare. We describe the case of a child with DORV with intact ventricular septum who underwent successful palliation. This condition often provides a diagnostic, interventional, and surgical challenge. Patients generally require either balloon atrial septostomy or surgical atrial septectomy for survival, with the addition of a Blalock-Tausig shunt or pulmonary artery band depending on the pulmonary blood flow. Very few patients survive to a Glenn procedure. For those who do survive, a decision must be made regarding the management of the hypertensive and hypoplastic left ventricle (LV) and associated mitral regurgitation. The LV can be excluded by either mitral valve avulsion or closure (exclusion) of the mitral valve with a patch. The choice of the technique should be carefully made and depends in part on the size of the LV. In our patient, who was successfully palliated by bidirectional Glenn shunt, the LV was managed by means of mitral valve avulsion.
authors.affiliation: Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India sabarinathmenon@yahoo.co.in sabarinath.menon@gmail.com.
authors.forename: Sabarinath
authors.initials: S
authors.lastname: Menon
authors.affiliation: Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India.
authors.forename: C J Ashok
authors.initials: CJ
authors.lastname: Kumar
authors.affiliation: Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India.
authors.forename: Thomas
authors.initials: T
authors.lastname: Mathew
authors.affiliation: Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India.
authors.forename: S
authors.initials: S
authors.lastname: Venkateshwarn
authors.affiliation: Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India.
authors.forename: K
authors.initials: K
authors.lastname: Jayakumar
authors.affiliation: Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvanathapuram, Kerala, India.
authors.forename: Baiju S
authors.initials: BS
authors.lastname: Dharan
mesh_terms: D004310:Double Outlet Right Ventricle / Q000000981:diagnostic imaging / Q000601:surgery*; D005260:Female; D018729:Fontan Procedure / Q000379:methods*; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D008943:Mitral Valve / Q000601:surgery*; D010166:Palliative Care; D011666:Pulmonary Valve Stenosis / Q000000981:diagnostic imaging / Q000601:surgery*; D054088:Ventricular Septum / Q000000981:diagnostic imaging
keywords: atrium; cardiac; congenital heart disease; right; ventricle