Case presentation ,double chambered right ventricle

describing double -chambered right ventricle as a complication with VSD

1. Case presentation Is it congenital or Acquired ? By Dr. Aliaa Shaban (M D) Lecturer of Cardiovascular Medicine Tanta University

2. History A 19-year-old male, with a history of surgical closure of PM-VSD, resection of sub aortic membrane at age of 4 . Her mother said that she was told he has a residual VSD , residual subaortic membrane , with no significant hemodynamic effect. Routine Echo follow up was done annually.

3. Initially he was asymptomatic, thereafter,at age of 16 y ,He gradually developed shortness of breath , and he was told that this complain has no direct relation to his current cardiac condition & he just need infective endocarditis prophylaxis.

4. Physical cardiac Examination Physical examination was notable for an ejection systolic murmur (grade III/VI) at the left sternal border

5. ECG Sinus Rhythm RT BBB,RT axis

6. TTE What do you expect?

7. Either… …. Left ventricle volume overload Left ventricular hypertrophy. Right ventricular hypertrophy. Non remarkable echo findings.

8. Mid –esophageal Long axis

9. Intact IVS

10. Mid –esophageal RVOT

11. DCRV Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly An anomalous muscle bands that cross the RV from the septum to posterior wall . So, It divides the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. . It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect

12. Congenital or acquired ? DCRV is considered an acquired cardiac defect . While there may be a genetic predisposition for abnormal muscle band formation that contributes to this anomaly, it has not been clearly described.

13. Mechanism Various mechanisms of DCRV have been described. Superior displacement of the (moderator band ) has been proposed, particularly in association with a VSD, and flow turbulence in the RVOT. This flow turbulence may trigger abnormal hypertrophy of the moderator band leading to DCRV. This might elucidate the concomitant association between DCRV and VSD

14. Imaging of DCRV TTE is an important first line diagnostic tool in congenital heart disease, but may have limited visualization of DCRV in adults due to the retrosternal position and asymmetrical shape of the RV .

15. Imaging of DCRV TEE is an excellent supplementary tool to assist delineation of the RV abnormalities as well as assess and quantify the severity of RV cavitary obstruction

16. Cardiac cath

17. Other imaging Recently, contrast CT and CMR have been introduced in the identification of DCRV. Those diagnostic tools are now sufficiently mature to preclude the need for invasive testing.

18. Intervention Surgical intervention is indicated in symptomatic patients or in asymptomatic patients where the peak gradient exceed 40 mm Hg . B- blocker may improve symptoms.

19. Could you mentions other causes Of RVOT obstruction ??

20. Echo messages DCRV may be missed in adult patient due to obesity or COPD. DCRV should be suspected in ECHO if RV hypertrophy is present, in absence of infudibular hypertrophy or valvular pulmonary stenosis .

21. Take Home Message Understanding the natural and un natural history of the disease helps to expect complication. Multi modality cardiac imaging are often required so as to clarify all details of the described anomaly especially in CHD. follow up of patients post operatively is of utmost importance to detect .all sequels.