1. Reconstruction of pulmonary pathway in congenital cardiac anomalies
Pr Abid Abdelfatteh
5th Mauritanian cardiology meeting
31st October – 1st November 2015
Nouakchott Mauritania

2. Pulmonary pathway in congenital Cardiac anomalies
Normal Abnormal
RV-PA discontinuity
Abnormal flow
Obstruction Regurgitation
RV-PA continuity
Normal flow Interruption Stenosis
Ex Aortic stenosis
T4F

3. Abnormal Pulmonary Pathway
Native congenital Repaired congenital
malformations malformations
T4F
pulmonary atresia
Truncus arteriosus.

4. Abnormal Pulmonary Pathway
Diagnosis
-Clinical symptoms
* Dyspnea
* Cyanosis
* Rigt heart failure
Chestxray
EKG
Echocardiography +++
Angioscanner
Angio MRI +++
Cardiac catheterisation +++

5. Reconstruction in obstructive pulmonary pathway
Interruption
- Common arterial trunk
Reconstructive surgery
direct
interposition of VD-PA conduit
- Pulmonary atresia
Catheter perforation
Surgical restoration of pulmonary pathway
Patch or conduit

6. Reconstruction of pulmonary pathway
In pulmonary stenosis
Infundibular stenosis
Surgical resection of fibrous tissue and muscle Patch closure

7. Reconstruction of pulmonary pathway
Valvar stenosis
- With normal pulmonary annulus
* Percutaneous catheter commissurotomy
* Surgical commissurotomy
- With small pulmonary annulus
Surgical enlargement with transannular patch

8. Reconstruction of pulmonary pathway
In pulmonary trunk stenosis
Surgical patch enlargement
Without cusp
With cusp
RV – PA Conduit

9. Reconstruction of pulmonary pathway
In distal pulmonary stenosis Surgical patch Catheter dilatation and stenting

10. Reconstruction of pulmonary pathway
Multifocal stenosis Extended enlargement

11. Reconstruction of pulmonary pathway
Hybrid procedure

12. Reconstruction of pulmonary pathway
In abnormal coronary arteries
Myomectomy
VD –PA conduit

13. Rare Abnormal Pulmonary Arteries

14. Pulmonary Regurgitation
Agenesis of the pulmonary valve
Post repair of congenital cardiac malformations
- Diagnosis
* clinical symptoms
* EKG - Holter
* echocardiography
* cardiac cath morphological informations
* MRI functional informations
Degree of pul and tricuspid regurgitation
RV function and dilatation

15. Angio MRI Pulmonary regurgitation RV dilatation RV dysfonction
MR + 3D reconstruction
Araldi
Pulmonary regurgitation
RV dilatation
RV dysfonction
Tricuspid regurgitation 15

16. Pulmonary insufficiency
Seen every time pulmonary valve was involved in initial surgical management :
commissurotomy
transannular patch
RV-PA conduit

17. Why do Tissue Valves Fail?
Most common valve failure mode is structural valve deterioration (SVD)
Calcification remains the primary cause of SVD

18. …………….severe pulmonary regurgitation alone, requiring valve insertion, is uncommon……..
World Congress of Paediatric Cardiology 1989

19. Natural History of PR
One of the reason for the lack of appreciation of the impact of PR is its very long preclinical natural history
At age 20 years, only 6% of the pt had symptoms, but the incidence increased to 29% at age 40 years
Shimazaki Y Thorac Cardiovasc Surg1984;32:257-9

20. Natural History of PR
At the time of ToF repair the RV is hypertrophied and its compliance is low; the diameters of the central PA are either hypoplastic or low-normal, and their capacitance is low.
The heart rate is relatively high, which leads to a relatively short duration of diastole
The combination of these factors
limits the degree of pulmonary regurgitation.

21. Natural History of PR
Over time the increase in RV stroke volume leads to progressive rise in the size and compliance of the central PA and to increased RV compliance
Combined with a longer duration of diastole as HR decreases with age, these changes lead to progressive increase in the degree of PR

22. The number of pts free of reinterventions for PVR decrease during the 3rd-4th decade

24. During the past 2 decades it has become apparent that
PR is a key driver
of RV failure
but
the Timing for PVR remains Controversial

25. Criteria for PV Replacement Exercise intollerance
Pt with symptoms
Exercise intollerance
Heart failure
sVT
syncope
PVR surgically or
transcatheter

26. Criteria for PV Replacement
Pt asymptomatic with PR ≥ 25-35% + at least 2 criteria
RV EDVi ≥ 150 mL/m2 or RV/LV >1.5
RV ESVi ≥ 80 mL/m2
RV volumes and function
RV EF ≤ 45%
≤ 65% of the predicted VO2 max
QRS ≥ 180 msec (better before 180 because no improvments after PVR)
TR ++
Residual VSD
RVOTO (RVP 2/3 LVP or ΔP ≥ 50 mmHg
AR ++
LV Dysfunction

27. Why timing is so important?
Why timing is so difficult?
What do we know OR DON’T know?
Which are possible future directions?

28. The Timing!
Certainly PVR should be performed when patients develop first symptoms as dyspnea, but it is not infrequent that they may have advanced RV dysfunction by the time complain of symptoms
Serial exercise testing may help to delineate subtle changes in exercise capacity before the pt becomes symptomatic.

29. Pulmonary insufficiency
Minimal or moderate: follow up
medical treatment
Severe :
Pulmonary valvulation
Surgical management
RV-AP conduit
pulmonary valve replacement
Percutanous management :
percutanous pulmonary valve

30. Surgical treatment of pulmonary regurgitation
Pulmonary valvulation
Pulmonary bioprosthesis
RV-PA conduit with valve substitute
homograft contegra conduit conduit with bioprosthesis
Redo surgery ++++

31. Percutaneous treatment of pulmonary regurgitation
Percutaneous valved conduit insertion

32. Melody® Transcatheter Pulmonary Valve
18mm Contegra modified-bovine jugular vein with valve segment
NuMed Platinum Iridium Stent
28 mm length
Crimped down to 6mm, re-expanded 18mm up to 22mm
Balloon Expandable system
SM: Added poin 2 – valves can be self expandable or self exp – so it’s important to underline it. 32

33. Melody

34. Melody® Indications Extend the functional life of the RV-PA conduit
Restore & maintain pulmonary valve competence
Relieve conduit stenosis without inducing regurgitation

35. Complications/Failures
► Homograft rupture
► Coronary compression
► Melody dislodgement
► Pulmonary artery perforation
► Tricuspid damage
► Death (coronary compression)

36. Limitations for Melody
RVOT surgical reconstruction without conduit (?)
RVOT larger than 22 mm
Unfavourable anatomy for secure stent anchorage

39. Immediate results Worlwide experience
≅ 2500 patients
Successful implants %
Complications/Failures %

40. Conclusion I
In congenital cardiac malformations, pulmonary pathway can be abnormal : obstruction or regurgitation
Technical reconstruction is to be adapted to the type of malformation
In reconstruction of pulmonay pathway it is crucial to relieve as much as possible pulmonary obstruction and to preserve pulmonary valve competence

41. Conclusion II
After repair of cardiac malformations with abnormal pulmonary pathway regular follow up is mandatory
- To detect residual or recurrent pulmonary stenosis
- To detect and evaluate pulmonary regurgitation
 appropriate type and time of management :
In pulmonary stenosis : surgery when RV-PA gradient ≥ 50 mmHg
In pulmonary regurgitation : pulmonary valvulation before severe deterioration of RV function ( surgery or percutanous treatment) to preserve the quality of the RV .

42. Thank you