adult patient wit uncommon cause of RVOT stenosis

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…….
A. Left ventricle volume overload
B. Left ventricular hypertrophy.
C. Right ventricular hypertrophy.
D.Non remarkable echo findings.

12. Mid –esophageal Long axis

13. Intact IVS

16. Mid –esophageal RVOT

19. 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

21. 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.

22. 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

23. 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 .

24. 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

25. Cardiac cath

26. 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.

27. 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.

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

29. 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.

30. 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.