1. 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):
Said S, Burkhart HM, Dearani JA et al. Outcomes of surgical repair of double-chambered right ventricle. Ann Thorac Surg Jan;93(1):
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:
Adult Cardiology Fellow
Marc Cribbs:
Director, Alabama Adult Congenital Heart Program
Director, UAB Comprehensive Pregnancy & Heart Program