The goals of early palliation of pulmonary atresia with intact ventricular septum (PA-IVS) include the relief of cyanosis and ductal dependence by providing a reliable source of pulmonary blood flow, and the relief of right ventricular outflow tract (RVOT) obstruction to encourage forward flow and growth of right-sided

1. PA IVS
ManagementDr. Sweta Mohanty
Consultant Pediatric Cardiologist
RxDx Healthcare, Rainbow Hospital,
Bangalore
Image: Plasticsurgerykey.org

2. Severely hypoplastic RV Good sized RV
Single-
ventricle
repair
Two-
ventricle
repair
Image: J Pediatr Cardiol Surg, Thoracickey

4. Factors that influence
management strategy

5. Tricuspid valve
Stenotic, hypoplastic
annulus
Dysplastic / Ebstein-
like, with TR
Image: doctorlib

6. Right ventricle
Image: JACC
Hypoplastic, unipartite RV
TV Z-score correlates with RV size
Large, thin-walled RV
Seen with profound TR

7. Nature of pulmonary atresia
Muscular atresia: Narrow
infundibulum, primitive PV
Membranous atresia: Well-formed
infundibulum, commissural fusion

8. High pressure
retrograde flow through
epicardial coronaries
Endothelial
damage
Coronary
stenoses
Intrinsic
coronary
abnormality
Lack of blood-
egress from RV
Elevated RV
pressure
Blood forced
retrograde through
thebesian veins
Coronary
sinusoids
Coronary
cameral Fistulae
Ventriculocoronary connections

9. Image: doctorlib
Dilated LAD with retrograde filling Dilated RCA with retrograde filling
PSAX view

10. Image: Giglia et al, Circulation
Potential of adverse outcome after
RV decompression:
RV to coronary
Fistula without
coronary stenosis
Potential RV steal
phenomenon
RV to coronary Fistula
with proximal ± distal
coronary stenosis
Potential RV steal/
ischemia
RV to coronary Fistula
with Coronary
occlusion/ atresia
Potential Isolation
and MI

11. Ventriculocoronary connections
 With coronary
obstructive lesions,
normal aortic
diastolic pressure
insufficient to drive
coronary blood flow
 Palliative measures
like BT shunt further
lower aortic diastolic
pressure  coronary
ischemia
 In RVDCC,
myocardial perfusion
sustained by
retrograde coronary
flow from
hypertensive RV,
during systole
 Any decrease in
RVSP by RV
decompressive
procedures 
coronary ischemia

12. RV dependent coronary
circulation (RVDCC)
Coronary artery abnormalities requiring RV
systolic events:
 Absent aorto-coronary connections
 Coronary artery interruption or stenosis
 Profound coronary cameral steal or fistula

13. Atrial septum
 PFO or OS ASD with
obligatory R- L shunt
 Oxygenated pulmonary
venous blood shunts L-
to-R across TV 
perfuses the
myocardium
 Nonrestrictive flow is
essential
Subcostal view: IAS with
aneurysmal flap, R-L shunt
Image: pedcards.com

14. Associated abnormalities
 LPA coarctation at site of PDA insertion
 Bilateral PDAs or MAPCAs when PAs non-confluent
 Endocardial fibroelastosis, ventricular dysfunction
 LV outflow tract obstruction
 Aneurysm of septum primum

15. Procedures in
newborn period
Being a condition with PDA-
dependent pulmonary
circulation, intervention is almost
always required in neonatal
period

16. Goal: To increase
pulmonary blood flow
Well-developed RV, mild/
no TR, no RVDCC

Surgical or transcatheter
pulmonary valvotomy, or
RVOT reconstruction
Goal: To enable RV to provide
pulmonary circulation
Severe RV hypoplasia,
RVDCC

PGE1, PDA stent or
systemic- PA shunt

17. Is RV size adequate to support 2-
ventricle repair?
 (i) Presence of three portions of the RV i.e.
tripartite, bipartite vs. unipartite
 (ii) TV annulus diameter and its z-score
 (iii) TV/MV diameter ratio >0.5 & TV z-score >
-2.5  possibility of 2-ventricle repair

18. Is there an infundibulum? Is MPA
in continuity with the imperforate
pulmonary valve?
 Transcatheter RF valvotomy or RVOT
transannular patch Growth of the
hypoplastic RV is stimulated
 Simultaneous PDA stenting or modified BT
shunt allows for adequate pulmonary blood
flow while RV is allowed to grow

19. Pulmonary valvotomy
 Well-developed tripartite RV
 Mild or no TR
 No significant RV-CACs
 BPV/ transcatheter wire perforation,
laser or radiofrequency assisted
OR
 Surgical valvotomy
Ductal patency may be maintained by
PGE before the procedure
Image: Thoracickey

20. Dilated – normal sized - mildly
hypoplastic RV
 RV size ≥ 2/3rd of normal
 TV Z score 0 to -2
 Consider surgical valvotomy with RVOT
reconstruction (RVOTR)
 If additional source of PBF felt necessary,
systemic-to-PA shunt may be simultaneously
placed

21. RVOT Reconstruction Surgery
For fixed obstruction at subvalvar or annulus level:
 1] Initially, RV-CACs are ligated reversibly, off CPB.
If ligation is not tolerated only shunt surgery
If no WMA or s/o injury ligation is completed
 2] Relief of RVOTO performed:
Infundibular resection + TAP or surgical valvotomy
 3] ASD is snared to achieve mild restriction - To encourage TV
flow
*If mostly RVDCC Single ventricle pathway

22. Role of Post-op. PGE1
 Post- procedure, cyanosis may persist
 Cause: Low RV compliance from myocardial
hypertrophy or fibrosis Elevated RV diastolic
pressure elevated RA pressure R-L shunt
through the foramen ovale
 Adequate PBF may not be achieved for days to
weeks
 May continue PGE1 in a low dose to maintain
ductus patency

23. RVOT Patch + arterial shunt
RVOTR + BT
Shunt
ASD and
Shunt can be
closed: 2VR
TV & RV
growth
failure:
BDGS
RV moderate
sized:
1 ½ VR

24. Low cardiac output syndrome
Following RVOTR & modified BT shunt:
 1) Due to low diastolic pressure or
unrecognized RVDCC with myocardial
ischemia
 2) Preferential pulmonary blood flow
as PVR very low and SVR high
 3) Due to “Circular shunt”: when
trans-annular patch (resulting in
pulmonary regurgitation) is combined
with BT shunt.
Image: Ultrasound Obgyn
Blood flow: LA -> LV -> Aorta -> BT
shunt -> PA -> Retrograde into RV,
across RVOT -> Retrograde into RA,
across TV -> LA, across the ASD

25. Severely Hyoplastic RV
 Pulmonary blood supply depends
entirely on systemic- pulmonary
connections
 Systemic venous return reaches RA->
PFO -> LA
 Q.1. Adequacy of PDA to sustain
pulmonary blood flow?
 Q. 2. Adequacy of ASD for
decompression of systemic venous
return to LA?
 Management option is to perform
systemic-PA shunt or PDA stent

26. Systemic-PA shunt or PDA stent
Indications:
 Severe RV hypoplasia (TV Z score < -
4.5)
 RVDCC (these patients are candidates
for eventual SVR)
Implantation of a stent in the PDA can
achieve the same end
Image rch.org.edu

27. Balloon atrial septostomy
 If systemic-to-pulmonary artery shunt
contemplated, with presumed single-ventricle
pathway
 Unrestrictive atrial communication necessary
 If restrictive PFO, BAS to be done

28. Transcatheter procedural
considerations

29. Pulmonary valve perforation

30. Image: Pediat Therapeut 2012
4F JR2- JR4- Tip placed close
to PV- AP & Lat views
Atretic PV perforated with stiff
end of regular guide wire/
CTO guide wire/ Laser/ RF
perforation wire
5 mm Gooseneck snare placed
retrograde (FA PDA MPA)
Balloon catheter advanced
across perforated PV, over wire
(± veno-arterial loop)
Serial balloon valvuloplasty (3
mm PTCA balloon to 6-8 mm
balloons)
• Femoral v. & a. access
preferred over
umbilical (easier
catheter
manipulation)
• Heparin 50-100 U/kg
(target ACT> 200 s)

31. Hybrid approach
 Sternotomy followed by placement of a purse
string suture in RV free wall, at a suitable location
aiming at the RVOT/ PV
 Advance needle via the purse-string suture,
towards PV
 Using TEE, perforate the PV
 Advance guidewire and remove needle
 Guide wire position confirmed by fluoroscopy
 4 French sheath placed over guidewire and
secured by the surgeon
 Serial balloon dilatations performed

32. Post-procedure role of PGE1
 Dynamic obstruction of RVOT secondary to strong
contraction of the hypertrophic RV infundibulum
noted immediately after successful BPV 
Continue PGE1 ±βblocker dynamic sub-PS may
resolve in a few days as RV hypertrophy resolves
 RV volume decreases compared to pre-
intervention volume by approx. 40-50%
Continue PGE1RV volume increases back to
pre-intervention volume by approx. 3 weeks

33. PDA Stenting

34. Types of PDA in PA IVS
Usual: normal origin from
proximal descending aorta and a
short straight course with
constriction at insertion onto
MPA
Less common: More proximal
origin from aortic arch, opposite
origin of LSCA, at acute angle
Image: Alwi, Ann Pediatr Cardiol. 2008

35. 4F long sheath tip
positioned near the
ampulla
Using 4F JR catheter,
0.014” guidewire
passed, Ao PDA
MPA RV RA IVC
3.5 mm-4.5mm
diameter pre-mounted
stent positioned
balloon inflated and
stent expanded
balloon deflated
Post-expansion
angiogram: Entire
length of ductus is
covered by stent; no
encroachment of origin
of branch PA
PGE1 turned off before procedure
Femoral artery cannulated with 4F sheath
Heparin 50 units/kg, repeated every 1-1 ½ hour
Image: Alwi, Ann Pediatr Cardiol. 2008

36. Definitive
Repair
Goal: Complete separation of
pulmonary and systemic
circulations

37. Severely
hypoplastic
RV, RVDCC,
failed attempt
to induce TV
growth
Good sized,
tripartite RV,
successful
attempt to
induce TV
growth
Fontan
pathway
2VR-
Valvotomy/
RVOTR-
infundibular
resection &
patch
Image: J Pediatr Cardiol Surg, Thoracickey
 

38. Image: EJCTS 2011
Group A:
TV Z-score >-2.5
Good infundibulum,
Membranous atresia
Tripartite RV
No major sinusoids
Variable TR
Valvotomy & dilation

Dilation of restenosis,
RVOTR or TV repair
Group B
TV Z-score -2.5 to -4.5
Patent Infundibulum,
Subvalvular PS
Bipartite RV
Major sinusoids ±
Variable TR
Valvotomy & dilation, PDA
stenting ± BAS
TAP + modified BT shunt

RVOTR Or
BDGS (1 ½ VR) if RV fails
to develop*
Group C
TV Z-score < -4.5
Absent infundibulum,
Muscular atresia
Unipartite RV
Major sinusoids
Usually competent TV
PDA stenting or modified
BT shunt
± BAS

BDGS
 Fontan/ Cardiac
transplantation*
*If extreme TR
Starnes approach

39. Intermediate RV size
If no fixed RVOTO (e.g. small PV annulus):
Reasonable to wait 3–4 years (with interim
valvotomy & PDA stenting) in anticipation of
RV growth.
If failure of RV growth: bidirectional Glenn
shunt and closure of ASD (1½ ventricle
repair) may be performed.
Aim: to retain the RV in the circulation if it is
capable of maintaining even part of
Pulmonary Blood Flow

40. One-and-half ventricle repair
The heart is
surgically septated (may
include ASD closure, RV-
PA conduit).
Additionally, a superior
cavopulmonary shunt is
created.
BDGS provides preload
reduction for the limited
right heart, thereby
avoiding right heart
failure.

41. Advantages of one-and-half
repair over BDGS:
 No increase in LV volume load (as
no ASD)
 Systemic saturation is complete
 No risk of paradoxical emboli

42. Additional
procedures

43. TV repair for TS or TR
 Valvar stenosis can be improved by BPV or
open valvoplasty. By open valvoplasty,
division of fused commissures/ fused papillary
muscles can be performed
 If TV insufficiency significant-> valvar repair

44. Dysplastic TV with Marked TR / Ebstein
malformation
 Difficult to manage, high mortality
 Systemic to PA shunt may relieve cyanosis, but
combination of increased LVVO ( from shunt), and RVVO
(from TR) often induces cardiac failure
 If patient requires placement of RVOT patch when 2VR
is considered-> PR results; 2 regurgitant valves (TV and
PV) in series make adequate C.O unlikely -> repair/
replacement of TV required

45. Dysplastic TV with Marked TR /
Ebstein malformation
 Contemporary approach:
Convert to tricuspid
atresia, (Starnes)+
construct systemic-PA
shunt
 Later cavopulmonary
palliation
Image: Sakurai, JTCVS, 2018

46. Closing the ASD
If right heart structures
fail to grow, or patient
remains too cyanotic
If ASD too large, size of
ASD should be
reduced
Device closure may be
performed if trial of
balloon occlusion is
tolerated

47. Heart failure from PR
 In infants who have had surgical
opening of the RV infundibulum
and the annulus of the valve
 If PR significant  Large RV
volume overload  marked RV
enlargement & progressive right
heart failure
 With progressive RV enlargement
-> pulmonary valve replacement
necessary
Image: JUM

48. Contemporary Approaches
 Fetal treatment: in utero
valvotomies- reported at
about 28 weeks (23-32
weeks). Providing forward
flow encourages RV and TV
growth.
 Transplantation: subset of
patients where there are
significant RV-CACs and no
continuation between aorta
and coronary a.
Intrauterine BPV
Image: Ultrasound Obstet Gynecol

49. Take Home Message

50. Image: EJCTS 2011
Group A:
TV Z-score >-2.5
Good infundibulum,
Membranous atresia
Tripartite RV
No major sinusoids
Variable TR
Valvotomy & dilation

Dilation of restenosis,
RVOTR or TV repair
Group B
TV Z-score -2.5 to -4.5
Patent Infundibulum,
Subvalvular PS
Bipartite RV
Major sinusoids ±
Variable TR
Valvotomy & dilation, PDA
stenting ± BAS
TAP + modified BT shunt

RVOTR Or
BDGS (1 ½ VR) if RV fails
to develop*
Group C
TV Z-score < -4.5
Absent infundibulum,
Muscular atresia
Unipartite RV
Major sinusoids
Usually competent TV
PDA stenting or modified
BT shunt
± BAS

BDGS
 Fontan/ Cardiac
transplantation*
*If extreme TR
Starnes approach

51. References
 Nykanen DG. Pulmonary atresia and intact ventricular septum. In Ed.
Allen HD, Driscoll DJ, Shaddy RE, Feltes TF. Moss and Adam’s Heart
Disease in infants, children and adolescents. Seventh ed. Philadelphia,
Lippincott Williams and Wilkins
 Burkholder H, Balaguru D. Pulmonary Atresia with Intact Ventricular
Septum: Management Options and Decision-making. Pediat Therapeut
2012, S5
 Alwi M. Management Algorithm in Pulmonary Atresia With IVS.
Catheterization & Cardio-vascular Interventions 2006; 67:679–686
 Alwi M. Stenting the ductus arteriosus. Case selection, technique and
possible complications. Ann Pediatr Cardiol. 2008;1: 38–45.
 Chikkabyrappa SM, Loomba RS, Tretter JT. Pulmonary Atresia With an
Intact Ventricular Septum: Preoperative Physiology, Imaging, and
Management. Seminars in Cardiothoracic and Vascular Anesthesia ·
February 2018
 Foker JE et al. Treatment algorithm for PA with IVS. Progress in Pediatric
Cardiology 2010;29:61–63
 Pulmonary atresia with IVS: Long-term results of 1&1/2 ventricle repair.
Kagami et al. AnnThoracic Surgery 1995;60:1762-1764