1. PULMONARY ATRESIA WITH
INTACT VENTRICULAR SEPTUM
SINUSOIDAL CORONARIES:50-70%

2. HISTORICAL NOTE
Hunter
Peacock
Grant
: 1st case report in 1783 by Hunter
: Collected 7 patients report in 1839
: Coronary sinusoid & fistula recognized in 1926
Davignon : Suggest systemic-pulmonary artery shunt in 1961
Bowman
: Shunt and RV outflow operation in 1971

3. INCIDENCE
1 to 3 % of all CHD
4 to 8 per 100,000 live births
3% of critically ill infants with CHD

4. IN A CLICK
Surgical interventions are improving
with a 5-year survival rate of
approximately 80 %

5. ABBREVIATION
Pulmonary atresia with intact ventricular septum (PAIVS) is a cyanotic congenital
cardiac lesion with an incidence quoted by various sources between 0.71 and 3.1% of
all congenital heart disease. It is characterized by an imperforate pulmonary valve
with completely fused comissures, variable degrees of dysplasia and narrowing of the
pulmonic valve, variable hypoplasia of the right ventricle and tricuspid valve and a
frequent association of coronary artery fistulae and sinusoids . The pulmonary arteries
are usually normal in size and the pulmonary blood flow is supplied by a patent ductus
arteriosus (PDA). The right ventricular hypoplasia can be extensive and involve all
three components, inlet, trabecular and infundibular parts or be confined to one
area. The left sided heart is usually normal, but in severe cases the ventricular septum is
displaced into the left ventricle and its cavity may be somewhat restricted.
Occasionally, infants with PAIVS have shown signs of both right and left ventricular
ischemia, likely related to the coronary artery fistulae. Association with atretic,
hypoplastic, or obstructed central coronary arteries, called right ventriculardependent coronary circulation (RVDCC) , carries a higher risk of morbidity and
mortality

6. NATURAL HISTORY
PA/IVS is fatal
 50 % die within two weeks of birth
 85 % by six months
 PA/IVS is a ductal-dependent lesion, closure of the patent ductus arteriosus
(PDA) generally results in rapid clinical deterioration and life-threatening
consequences, including severe metabolic acidosis and hypoxemia,
seizures, cardiogenic shock, cardiac arrest, and death. Rarely, prolonged
survival can occur with pulmonary blood flow maintained by a persistent
PDA or systemic artery to pulmonary artery blood flow via one or more
collateral blood vessels

7. PATHOLOGY
Pulmonary valve atresia(muscular-25%)
Ebstenoid malformation of TV-25% and TR-25%
Hypoplastic right ventricle
1.Tripartite(Normal) RV 59 to 83 %
2.Bipatrite(RVOT is inconspicuous):15-35%
3.Monopartite(Only a detectible RV inlet):2-5%
Almost normal PA
Vertical/inverted PDA
Coronaries: Sinusoidal in 50-70%(right ventricular dependent coronary circulation
(RVDCC)
• Normal LV/MV
• PFO+
•
•
•
•
•
•
•
•
•

8. PATHOPHYSIOLOGY: FROM RV
WHERE BLOOD GOES WHEN
RVSP>200MMHG
• TR
• SINUSOIDAL EGRESS
• VIA PFO TO LA

9. PRESENTATION

10. ON EXAMINATION
Single second heart sound
Systolic murmur due to tricuspid regurgitation.
Silent precordium
 “Machinery type” murmur due to a PDA
Room air SPO2 <70% and (PaO2) levels typically 35 to 45 mmHg

11. CHEST X-RAY
Right atrial enlargement
Cardiomegaly[ significant tricuspid regurgitation]
Paucity of pulmonary vascularity

12. ECG
Right atrial enlargement based on tall p waves in leads I, II, and Avf
abnormal relative left axis deviation (QRS axis +30 degrees) due to a
decrease of right-sided forces from RV hypoplasia
 Cyanosis in a neonate with left axis deviation on ECG should prompt the
clinician to consider PA/IVS
Although the ECG does not demonstrate significant myocardial ischemia, ST
and T wave abnormalities should be ruled out on all patients with PA/IVS due
to the possibility of coronary abnormalities

13. ECHO
• TV Z-score
• Size and morphology of the RV (uni-, bi-, or tripartite)
• TR
• PA SIZE
• PDA

14. CATHETERIZATION
RV angiogram: Coronary sinusoids and fistulae
Selective injection of the coronary artery origins from the aorta :Proximal
coronary artery atresia and coronary stenosis [9%]
 It is important to determine whether coronary arterial perfusion is dependent
on circulation from the RV (ie, right ventricular dependent coronary
circulation), as decompression of the RV during surgery could lead to
coronary artery steal, ischemia, infarction, cardiac arrest, and/or death
Vertical /INVERTED PDA with PA end stenosis

15. CORONARIES

16. RV ANGIOGRAM

17. DIFFERENTIAL DIAGNOSIS
Pulmonary atresia with ventricular septal defect
Critical pulmonary stenosis
Tetralogy of Fallot
Tricuspid atresia

18. THE DETERMINANTS FOR
INTERVENTION STYLE
• TV Z-score
• Coronary artery anatomy/ perfusion
• RV size

19. Z-SCORE OF TV
TV Z-score less than -4
or with RVDCC
TV Z-score between -4
to -2, and no RVDCC
TV (Z-score ≥-2) without
RVDCC
Fontan’s palliation
1.5 ventricle repair
Biventricular repair
Z=Z score
X=value of TV annulus in your case
µ= the expected measurement
σ= standard deviation

20. TREATMENT
INITIAL STABILSATION
FOLLOWED
 Cardiorespiratory support
 Prostaglandin E1 to maintain patency of the
ductus arteriosus
 Biventricular repair (separate pulmonary
and systemic circulations with two pumping
ventricles):RV/TV size/ coronary artery
circulation
 Univentricular repair (separate pulmonary
and systemic circulation with one pumping
ventricle):Fontan’s
palliation[http://content.onlinejacc.org/arti
cle.aspx?articleid=1121698]
 1.5 ventricle repair (separate pulmonary
and systemic circulations with two pumping
ventricles, but with one source of pulmonary
blood flow from the superior vena cava)
 Cardiac transplantation

21. OFF PUMP SURGICAL PULMONARY
VALVOTOMY:RV DECOMPRESSION
FIRST RULE RVDCC

22. PBPV
Percutaneous balloon valvotomy is an effective treatment strategy for
patients with PA-IVS provided that there is a patent infundibulum and a lack of
a right ventricle–dependent coronary circulation. Despite the observation that
right heart growth does not increase with body growth in early follow-up, it
appears adequate to maintain a biventricular circulation in many patients.
http://circ.ahajournals.org/content/108/7/826.full

23. I MAY HAVE SOME