A COLLABORATIVE

APPROACH TO
PULMONARY ATRESIA
WITH INTACT
VENTRICULAR
SEPTUM
Kelli Griffiths
Undergraduate Student
University of Florida

Redmond P. Burke MD
Chief, Division of Cardiovascular Surgery
The Congenital Heart Institute
Miami Childrens Hospital 2014
Danny Monroe, RN Danielle Madril, MD
RICHARD LAGUERUELA, MD FRANCISCO ALONSO, RNFA
CHRISTOPHER
TIROTTA MD
PULMONARY ATRESIA WITH INTACT
VENTRICULAR SEPTUM: PA/IVS
INCIDENCE
Represents 1-1.5% of all congenital heart disease
PA/IVS is a uniformly fatal form of structural cardiac disease. Outcomes of
surgical interventions are improving with a five-year survival rate of
approximately 80 percent
MORPHOLOGY
Marked morphologic heterogeneity is the hallmark of this lesion
Heterogeneity plus rarity produces uncertainty in therapeutic planning
Pulmonary Valve
Plate-like valve
Long segment stenosis
Patent Foramen Ovale
True ASD in 20%
5-10% restrictive
Right Atrium
Dilated and proportional to the amount of TR

MORPHOLOGY
Tricuspid valve
Usually smaller than normal but
ranges from Ebsteins anomaly
(5-10%) with enlarged or dilated
annulus to extremely stenotic
valve
Median Z values around -2
Correlates with size of RV
Varying types of dysplasia are
seen
MORPHOLOGY
Right Ventricle
Hypertrophied with reduced cavity in 90% and of these, 60% are severe
Can be
Unipartite: inlet part only
Bipartite: inlet and outlet
Tripartite: inlet, outlet, trabecular
Fibrosis can be present and sometimes severe in both ventricles
MORPHOLOGY
Coronary arteries
Fistulae between RV sinusoids and coronary
arteries are seen in 8-55% of small
hypertensive RVs
Correlates with TV diameter, RV size and
degree of TR
More often LAD and LCA than RCA
20% have RV dependent coronary circulation
RVDCC
MORPHOLOGY
Sinusoids
Communications between
coronary artery system and the RV
through endothelial lined blind
channels
Path of flow is from the RV
through the intramyocardial
sinusoids to the coronary arteries
to coronary veins through the
coronary sinus to the RA and back
to the RV
Coronary flow can be easily
compromised if RV systolic
pressure is reduced by any means
IN 2009, WE REVIEWED OUR MIDTERM
RESULTS FOR PA/IVS AT MCH
Retrospective study from 1996 to 2007
24 Patients
Mean age at first intervention 4.5 days
Mean follow-up of 6.05 years (range 1.9 to 12.7 years)

Ann Thorac Surg. 2009 Apr;87(4):1227-33.
Midterm results for collaborative treatment of pulmonary atresia with
intact ventricular septum.
Hannan RL
1
, Zabinsky JA, Stanfill RM, Ventura RA, Rossi AF, Nykanen
DG, Zahn EM, Burke RP.

PA/IVS TREATMENT PLANS ARE BASED ON ECHO AND
CATHETERIZATION, FOLLOWED BY PRESENTATION AT TEAM
CONFERENCE
Historically, a wide range of surgical options have been used for this
heterogeneous group of patients.
We have adopted a combined surgical and catheter-based approach
Initial treatment is dictated by the size and morphologic
development of the RV, the RVOT, the TV and by the presence or
absence of coronary abnormalities.

TRICUSPID VALVE Z-SCORE IS A
CRITICAL DETERMINANT
CORONARY ARTERY ANATOMY IS
ANOTHER CRITICAL DETERMINANT
63% OF OUR PATIENTS HAD SEVERE TV OR RV HYPOPLASIA OR
RVDCC, AND WERE PLACED IN THE SINGLE VENTRICLE PATHWAY
Strategy to reduce cumulative trauma
We try to avoid bypass and ischemic arrest
Complete palliation in cath lab
Balloon Atrial septostomy and Ductal stent
Challenges:
Incomplete septostomy
Tortuous duct
Hemodynamic instability with wire across TV
Arrhythmia and TR

Balloon atrial septostomy in cath lab and Central shunt or RMBTS off pump
Challenges:
Inadequate septostomy
Shunt thrombosis/bleeding/hemodynamic instability from clamps on PA
Pulmonary artery ductal stenosis
TIME FROM CATH
INTERVENTION TO SURGERY
INCOMPLETE SEPTOSTOMY
FLOPPY EDGE OF SEPTUM PRIMUM
POST SURGICAL SEPTECTOMY

TORTUOUS DUCT

TORTUOUS DUCT
CENTRAL SHUNT PROXIMAL
ANASTOMOSIS
THROMBOSED CENTRAL SHUNT
TENACIOUS THROMBUS RESISTS THROMBECTOMY
AND REQUIRES A NEW SHUNT
INNOVATIONS TO REDUCE SURGICAL
TRAUMA
Biological glues for shunt suture lines.
Veno-venous bypass support circuits
Rotational thromboelastometry (ROTEM) analysis of
postoperative coagulopathy
Echo guided surgical septostomy with endoscopic
instruments passed through the atrial wall in beating heart,
on or off bypass, in cath lab or in OR
Attempted twice, but difficult to achieve good
septostomy with current instrumentation

37% OF OUR PATIENTS WITH PA/IVS WERE
PLACED IN THE 2 VENTRICLE PATHWAY
Mild or moderate
RV hypoplasia
Plate-like
pulmonary atresia
No RVDCC
WE ATTEMPT TO USE THE LEAST
TRAUMATIC APPROACH FOR EACH PATIENT
Transcatheter RF perforation of the valve plate and ballooon
valvuloplasty with surgical standby
Hybrid approach via subxyphoid incision
Transannular right ventricular outflow tract patch, with or without
shunt




Hybrid ventricular decompression in pulmonary atresia with intact septum
Burke RP, Hannan RL, Zabinsky JA, Tirotta CF, Zahn EM.
Ann Thorac Surg. 2009 Aug;88(2):688-9.
SINGLE VENTRICLE PATH
Shunt alone and single
ventricle repair expected
Cath in 3-6 mos. with
plan of separating
circulations
Glenn done at 3-12 mos.
Warm and beating heart
Between 1-4 years
convert to a modification
of the Fontan
Total cavopulmonary
reconstruction if RV dep.
coronary circulation
Fontan is done with
heart warm and beating
TWO OR 1.5 VENTRICLE PATH
If RVOT Procedure alone or with shunt
Cath and echo in 3-6 mos to evaluate growth of RV and
TV
In cath lab, attempt temporary closure of shunt and
ASD and if adequate response, proceed to permanent
closure
If no growth, switch to single ventricle pathway
If borderline, repeat cath and echo in 1 year
MIAMI CHILDRENS EXPERIENCE
Pulmonary Valve Perforation
And Valvuloplasty (10)
BTS or Central Shunt (7)
BDCPA (3)
Alive (3)
Alive (3)
Alive (4)
2V Pathway
Group A (37%)
BTS or Central Shunt (13)
BDCPA (9)
Death (3)
Fontan (1) Alive (2) Fontan (7)
Alive (7)
Cath Septostomy (4)
Death (1)
Fontan (2)
Alive (2)
Alive (1)
BDCPA (1)
Cath Septostomy (1) BTS (4)
BDCPA (3)
1V Pathway
Group B (63%)
Alive (1)
There was no crossover between the Two ventricle repair group,
and the Single ventricle group.
SURVIVAL
PROGNOSIS
MCH
91.7% survival at 6 years (22/24)
CHSS
81% survival at 1 month
64% survival at 4 years
Mayo Clinic Adult Congenital Heart Clinic
Twenty adult patients with PA/IVS (1998 to 2009)
Median age at death was 32 years (30-37 years)
Seven patients underwent the Fontan operation, eight patients had a biventricular
repair, and five patients remained with palliative shunts
All patients required re-interventions in adulthood. Tricuspid valve (TV) (n=5),
pulmonary valve (PV)/conduit (n=6), and mitral valve (n=2) replacements were
the most frequent
Atrial arrhythmias were present in 80% of the total cohort
FUTURE DEVELOPMENTS
Fetal echo and intervention
CONCLUSIONS
An individualized, collaborative approach to PA/IVS can
produce good results.
If the right ventricle can be safely decompressed and looks
usable, the need for a shunt after valvuloplasty does not
preclude a two or 1.5 ventricle repair.
Anatomy mandating a neonatal shunt has substantial early
mortality.
Hybrid procedures may help reduce the cumulative trauma
of care for this complex lesion.
THANK YOU