PULMONARY ATRESIA WITH
INTACT VENTRICULAR SEPTUM
SINUSOIDAL CORONARIES:50-70%
HISTORICAL NOTE
Hunter
Peacock
Grant

: 1st case report in 1783 by Hunter
: Collected 7 patients report in 1839
: Coronary...
INCIDENCE
1 to 3 % of all CHD
4 to 8 per 100,000 live births

3% of critically ill infants with CHD
IN A CLICK

Surgical interventions are improving
with a 5-year survival rate of
approximately 80 %
ABBREVIATION
Pulmonary atresia with intact ventricular septum (PAIVS) is a cyanotic congenital
cardiac lesion with an inci...
NATURAL HISTORY
PA/IVS is fatal
 50 % die within two weeks of birth
 85 % by six months
 PA/IVS is a ductal-dependent ...
PATHOLOGY
Pulmonary valve atresia(muscular-25%)
Ebstenoid malformation of TV-25% and TR-25%
Hypoplastic right ventricle
1....
PATHOPHYSIOLOGY: FROM RV
WHERE BLOOD GOES WHEN
RVSP>200MMHG
• TR
• SINUSOIDAL EGRESS
• VIA PFO TO LA
PRESENTATION
ON EXAMINATION
Single second heart sound
Systolic murmur due to tricuspid regurgitation.
Silent precordium
 “Machinery...
CHEST X-RAY
Right atrial enlargement
Cardiomegaly[ significant tricuspid regurgitation]
Paucity of pulmonary vascularit...
ECG
Right atrial enlargement based on tall p waves in leads I, II, and Avf
abnormal relative left axis deviation (QRS ax...
ECHO
• TV Z-score
• Size and morphology of the RV (uni-, bi-, or tripartite)
• TR
• PA SIZE

• PDA
CATHETERIZATION
RV angiogram: Coronary sinusoids and fistulae
Selective injection of the coronary artery origins from th...
CORONARIES
RV ANGIOGRAM
DIFFERENTIAL DIAGNOSIS
Pulmonary atresia with ventricular septal defect
Critical pulmonary stenosis

Tetralogy of Fallo...
THE DETERMINANTS FOR
INTERVENTION STYLE
• TV Z-score
• Coronary artery anatomy/ perfusion
• RV size
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
R...
TREATMENT
INITIAL STABILSATION

FOLLOWED

 Cardiorespiratory support
 Prostaglandin E1 to maintain patency of the
ductus...
OFF PUMP SURGICAL PULMONARY
VALVOTOMY:RV DECOMPRESSION

FIRST RULE RVDCC
PBPV
Percutaneous balloon valvotomy is an effective treatment strategy for
patients with PA-IVS provided that there is a p...
I MAY HAVE SOME
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Pulmonary atresia with intact ventricular septum

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Transcript of "Pulmonary atresia with intact ventricular septum"

  1. 1. PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM SINUSOIDAL CORONARIES:50-70%
  2. 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. 3. INCIDENCE 1 to 3 % of all CHD 4 to 8 per 100,000 live births 3% of critically ill infants with CHD
  4. 4. IN A CLICK Surgical interventions are improving with a 5-year survival rate of approximately 80 %
  5. 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. 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. 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. 8. PATHOPHYSIOLOGY: FROM RV WHERE BLOOD GOES WHEN RVSP>200MMHG • TR • SINUSOIDAL EGRESS • VIA PFO TO LA
  9. 9. PRESENTATION
  10. 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. 11. CHEST X-RAY Right atrial enlargement Cardiomegaly[ significant tricuspid regurgitation] Paucity of pulmonary vascularity
  12. 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. 13. ECHO • TV Z-score • Size and morphology of the RV (uni-, bi-, or tripartite) • TR • PA SIZE • PDA
  14. 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. 15. CORONARIES
  16. 16. RV ANGIOGRAM
  17. 17. DIFFERENTIAL DIAGNOSIS Pulmonary atresia with ventricular septal defect Critical pulmonary stenosis Tetralogy of Fallot Tricuspid atresia
  18. 18. THE DETERMINANTS FOR INTERVENTION STYLE • TV Z-score • Coronary artery anatomy/ perfusion • RV size
  19. 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. 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. 21. OFF PUMP SURGICAL PULMONARY VALVOTOMY:RV DECOMPRESSION FIRST RULE RVDCC
  22. 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. 23. I MAY HAVE SOME
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