Ventricular Septal Defect Double Chamber RV (DCRV) Double Chamber Right Ventricle as a Late Complication of Ventricular Septal Defect Efstathia Andrikopoulou, Marc G. Cribbs University of Alabama Medical Center, Birmingham, Alabama USA Children’s of Alabama Hospital, Birmingham, Alabama USA Ventricular Septal Defect BACKGROUND Double Chamber RV (DCRV) PATIENT COURSE Ventricular septal defects (VSD) are the second most common type of congenital heart disease Though considered to be of little hemodynamic consequence, even tiny perimembranous VSDs can be associated with significant late complications These include Aortic Insufficiency and Double Chamber Right Ventricle (DCRV) Surgical resection of the RV muscle bands, patch closure of the VSD (visualized intra-op), and tricuspid valvuloplasty performed Post-operatively, patient experienced: Symptomatic relief Normalization of RV pressures Normalization of RV thickness CASE CONCLUSIONS HPI A 24-year-old female with a history of a small unrepaired perimembranous VSD — lost to follow-up for 16 years— presented with progressive exertional dyspnea. Physical Exam Harsh 3/6 holosystolic murmur over the left mid sternal border Echocardiography (TTE) Mid-RV cavity muscular narrowing suggestive of DCRV Peak TR gradient 105mmHg (no evidence of pulmonary stenosis) Severe RV hypertrophy & severe right atrial dilation Moderate tricuspid valve insufficiency No evidence of VSD or aortic insufficiency This case highlights: A late complication of a seemingly benign defect The importance of follow-up in patients with congenital heart disease Pre-Operative TEE REFERENCES Loukas M, Housman B, Blaak C et al. Double-chambered right ventricle: a review. Cardiovasc Pathol.2013 Nov-Dec;22(6):417-23. Said S, Burkhart HM, Dearani JA et al. Outcomes of surgical repair of double-chambered right ventricle. Ann Thorac Surg. 2012 Jan;93(1):197-200. Post-Operative TTE DECISION MAKING DISCLOSURES DCRV can develop in up to 10% of unrepaired perimembranous VSDs Surgical resection is the mainstay of treatment with: + Symptoms Peak intracavitary gradient >64mmHg, regardless of symptoms Regular post-operative follow-up and TTE monitoring is recommended The authors have nothing to disclose Contact Efstathia Andrikopoulou: eandrikopoulou@uabmc.edu Adult Cardiology Fellow Marc Cribbs: mgcribbs@uabmc.edu Director, Alabama Adult Congenital Heart Program Director, UAB Comprehensive Pregnancy & Heart Program