Pulmonary Atresia with Intact Ventricular Septum Ali Sepahdari, MD University of Illinois at...
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Pulmonary Atresia with Intact Ventricular
SeptumAli Sepahdari, MD
University of Illinois at Chicago
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Background
Rare lesion accounting for 1-1.5% of all congenital heart disease
Complex disease with commonly associated abnormalities of the tricuspid valve, RV, coronary arteries, and left heart.
Treatment options vary depending on severity of disease
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A more familiar abnormal....
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Hypoplastic right heart...
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Components of PA.IVSPulmonic atresia
Variable degree of right ventricular hypoplasia
Interatrial communication always present
Varying degrees of tricuspid valve abnormality
Coronary artery abnormalities
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Pulmonic atresia
Exact cause unknown, but occurs late in development
Inflammatory/infectious insult speculated
Subtle hemodynamic alterations causing left heart loading is another proposed mechanism
Varying degrees of abnormality
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RV Hypoplasia
Hypertrophied wall
Reduced cavity size in 90% of cases
5-10% of cases have enlarged right heart and Ebstein’s anomaly
Unipartite, bipartite, or tripartite RV (controversial)
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Tricuspid abnormality
Typically expressed as Z score
85% are -2 to -5 SD below mean
Average Z = -2.2
Correlated with RV size
Functionally regurgitant (severe in 25%)
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Interatrial connection
True secundum ASD in 20%
Patent PFO in the rest
Can be restrictive 5-10% of the time
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Coronary artery abnormalities
Persistent right ventricular sinusoids, which may have fistulae to the coronary arteries
Presence inversely related to TV diameter, RV cavity size, and degree of TI; correlates directly with RV systolic pressure
Coronary circulation can be dependent on these communications, due to coronary artery stenoses (20%)
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Pathophysiology
Natural history is hypoxia, acidosis, early death (weeks to months)
Maintenance of PDA critical initial step
LV is overloaded, and pumps mixed blood
Can have myocardial ischemia, depending on degree of coronary artery anomaly
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Management
Cardiac cath to assess coronary arteries
If complete myocardial dependence on RV, consensus to avoid decompression
Management algorithm is complicated beyond that
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Possible repairs
“two ventricle repair” – repaired RVOT, conventional physiology
“one ventricle repair” (Fontan circulation) -- passive systemic venous return to pulmonary arteries, bypassing the heart
“one and a half ventricle repair” – RVOT repair + bidirectional Glenn
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Management Strategy
• After stabilization, initial procedure will be RVOT procedure and/or BT shunt – reassess at 3-6 mo.
• Subsequent procedures may include bidirectional Glenn +/- ASD modification
• Fontan at 1-4 years if single ventricle repair is planned
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Radiology’s role
All available literature describes combination of echo and angiography in workup of PA.IVS -- CT and MR may have future role
TV annular size, coronary arteries are most important
TV regurgitant fraction, RV chamber size, morphology, growth
Post procedure flow dynamics?
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References
• Reddy VM and Hanley FL. “Pulmonary Atresia with Intact Ventricular Septum.” Glenn’s Thoracic and Cardiovascular Surgery. Baue AE (ed). Appleton and Lange, 1996: 1315-1332.
• Mi YP et al. Evolution of the management approach for pulmonary atresia with intact ventricular septum. Heart 2005; 91:657-663
• Salvin JW et al. Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum. Pediatrics 2006 118(2):e415-e420
• Bichell DP. Evaluation and management of pulmonary atresia with intact ventricular septum. Current Opinion in Cardiology. 1999; 14(1):60-66
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• Humpl T et al. Percutaneous balloon valvotomy in pulmonary atresia with intact ventricular setpum: impact on patient care. Circulation 2003; 108(7):826-832
• Jahangiri M et al. Improved results with selective management in pulmonary atresia with intact ventricular septum. J of Thoracic and Cardiovascular Surgery. 1999; 118:1046-1052
• Rychik J et al. Outcome after operations for pulmonary atresia with intact ventricular septum. J of Thoracic and Cardiovascular Surgery. 1998; 116:924-931