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PAPVR: Some of pulmonary veins return to RA or its tributaries

2. ASD Morphology
Fossa ovalis (ostium secundum) - Most common
Posterior
Ostium primum
Coronary sinus
Subcaval
Confluent

3. PAPVR Morphology
Sinus venosus syndrome
RSPV to SVC
RPV to RA
RPV to IVC - Scimitar syndrome
Rare connections of RPV
Anomalous LPV connections
Bilateral PAPVC

4. Scimitar Syndrome
RPV to IVC
Anomalous vein parallel to pericardium
Enters at or below IVC - RA junction
Associated with anomalies of right lung
Associated with cardiac and diaphragmatic anomalies in about 20%

5. Cardiac Chambers in ASD
Increased RA/LA normal
Increased RV/LV normal or decreased
Mitral Valve and ASD
Mitral prolapse 20%
Mitral regurgitation 2-10%
Cleft mitral valve

6. Clinical Features
a) Symptoms - Respiratory, Cardiac, Emboli
b) Signs
Chest X-ray - PA, Arch, Sinus venosus, Scimitar
ECG
Echocardiogram
Catheterization
Others

7. Miscellaneous Features
Survival
Pulmonary hypertension
Spontaneous closure
Ventricular function
AV valves
Supraventricular arrhythmias
Systemic hypertension

8. Indications for Repair (ASD or PAPVC)
Qp/Qs > or = 2
Scimitar
PAPVR
Part of one lung; < or = 1.8 - No
Whole lung - Yes
Age
Pulmonary vascular disease
TVD, MVD

9. Special Considerations
Percutaneous closure
Cardioplegia
Direct vs patch closure
Late sinus venosus syndrome

10. Post-Operative Care and Results
Older patients - Increased LA
35 years - Coumadin
Survival/Morbidity
Complications
Recurrence

1. Introduction
a) Present in 10-15% of pts w/CHD
b) Most common congenital lesion in adults
c) First cardiac lesion successfully repaired w/extracorporeal circulation (Gibbon, 1953)

2. Anatomy
a) Defects near IVC/SVC result from regression of the interatrial fold
b) Patent foramen ovale
i) Present in 20% of normal people
ii) High RA pressures result in R to L shunting
iii) Distended atria leads to communication
iv) Should be routinely closed during other procedures (except when R ht failure expected)
c) Secundum ASD
i) Foramen ovale type (2/3)
a) Underdevelopment of the septum primum
b) 0.5-2cm wide x 1-6cm long, fenestrated atrial tissue
ii) Sinus venosus or superior vena cava type - junction of RA + SVC (10%)
a) Inferior portion is superior limbic septum - SVC orifice displaced to left
b) Almost always assoc. w/ partial anomalous connection (RUL & RML)
iii) Low or IVC type - between inferior limbic septum and IVC ( 20%)
a) Occasional RLL venous connection to RA
b) No inferior rim - avoid compromising IVC orifice
c) May be associated w/CS to LA
d) Unroofed CS (usually associated w/ LSVC) may allow RA-LA communication
iv) Common atrium (single atrium) - endocardial cushion defect if primum ASD present

3. Associated lesions (ASD may be associated w/ any congenital anomaly)
a) Pulmonary stenosis - 10%
b) Partial anomalous venous return - 7%
c) VSD - 5%
d) ASD + rheumatic mitral stenosis = Lutembacher syndrome

4. Pathophysiology (flow depends upon size and ventricular compliance)
a) Normally, RV compliance is greater than LV compliance, therefore shunting is L to R (limited for small defects)
b) Defects 1-2cm 2, atrial pressures are equal, shunting depends upon ventricular compliance
i) Newborn: compliance is roughly equal, so there is mixed shunting
ii) First weeks of life, RV compliance decreases, leading to L to R shunting
iii) Unrestricted ASD, Qp = 2-4xQs
c) Increased Pulmonary flow
i) Children: 5-8% have PA pressure 50mm Hg
ii) Age 40: 35-50%
iii) May have difficulty maintaining high systemic output during exercise
iv) Eventually shunt reverses (2° to increased PVR) causing increased cyanosis (no longer surgical candidates)
v) Incidence of arrhythmias and MV disease increase w/age
d) Life expectancy = 50 yrs untreated (progressive CHF)

5. Clinical features
a) Typically asymptomatic w/ murmur
i) 50-60% have easy fatigability
ii) “gracile” (thin) habitus
iii) M:F = 1:2
b) PE
i) Prominent RV impulse along lower left sternal border
ii) Systolic ejection murmur along L sternal border from increased pulmonary flow
iii) Mid-diastolic murmur near apex from increased TV flow
iv) Fixed splitting of 2nd heart sound
c) ECG
i) RVH, QRS axis +95 - +150
ii) Incomplete RBBB
d) CXR = cardiomegaly, increased pulmonary vascularity (CXR may be normal)
e) ECHO
f) Cath - usually not needed

6. Differential diagnosis
a) AV canal defect, VSD
b) Other causes of oxygen step-up in RA
i) Anomalous PV connection
ii) Sinus of valsalva aneurysm w/ rupture into RA
iii) Coronary artery fistula into RA
iv) Left sided obstructive lesions causing L to R shunt

7. Treatment
a) Indications for surgery
i) Significant ASD (by PE, EKG, echo)
ii) 3-5 yo
iii) Spontaneous closure extremely rare after 2 yo
iv) Infant in CHF w/failure of medical therapy
v) Adults L? R shunt 1.5-2:1
b) Contraindication to surgery: severe pulmonary vascular disease (PVR 1/2-2/3 systemic)
c) Operative management
i) Incision - median sternotomy, submammary, R thoracotomy
ii) Cardioplegia & aortic cross-clamping
iii) Primary or patch (pericardial or prosthetic) closure
iv) Attention to SA node and to tension on septal tissue
v) Sinus venosus type- pericardial baffle (leave a small, high PV draining into SVC)
vi) Air
d) Catheter-based devices

8. Results
a) <1% mortality
b) Complications
i) Air embolus (CVA, RCA MI)
ii) SVT
iii) Injury to SA or AV nodes
c) Cardiomegaly usually resolves if ASD corrected before third decade