Total anomalous pulmonary venous connection

1. TOTAL ANOMALOUS
PULMONARY VENOUS
CONNECTION
Fifth most common cause of cyanotic CHD

2. TAPVC IS CYANOTIC CHD

3. TIMELINE
• 1798 :1st described by Wilson
• 1956:1st ICR by Lewis and Varco
In 1798, the Philosophical Transactions of the Royal Society of London published “A
description of a very unusual formation of the human heart.” A 1942 review of 100
cases of anomalous pulmonary venous connections included 35 examples that were
total, a term that applies when all four pulmonary veins connect anomalously to a
systemic venous tributary of the right atrium or to the right atrium proper but have no
connection to the left atrium. The malformation is isolated in approximately two thirds
of patients so afflicted occurs in approximately four to six per 100,000 live births,57
and accounts for about 2% of deaths from congenital heart disease in the first year of
life.

4. RT3D
• http://www.youtube.com/watch?feature=player_embedded&v=RMIKq92dfHE#t=101
The Pulmonary Veins, which carry blood back to the heart after it has
circulated through the lungs, are not connected to the left atrium.
Instead they are connected to one of the veins from the main circulation
so that the blood returning from the lungs drains back to the right side of
the heart. The affected babies may be blue or show signs of heart
failure. Most of them require surgical repair in the newborn period

5. INCIDENCE
0.6 to 1.2 per 10,000 live births
0.7 and 1.5 percent of all CHD
A strong male preponderance of 3:1
Birth weight was less than 2500 g in 16.2%
Gestational age was less than 38 weeks in 18.9%
Intrauterine growth retardation occurred in 26.8%
68% of these patients were diagnosed as neonates

6. WHAT CAUSES IT
• NOT KNOWN

7. EMBRYOLOGY
NORMAL
TAPVC
 Lung buds are formed from the primitive
foregut
 Lung buds veins drain as splanchnic plexus
initially drains into the common cardinal and
umbilicovitelline venous systems
 Splanchnic plexus differentiates into the
primitive pulmonary vascular bed
 Primitive left atrium forms a primordial
evagination (common pulmonary vein) that
grows into and joins the pulmonary portion of
the splanchnic plexus
 Primitive pulmonary venous system separates
from the cardinal and umbilicovitelline veins
 Common pulmonary vein become the two
right and two left pulmonary veins, each of
which enters the LA through a separate orifice
 Failure of the left atrium to link to the
pulmonary venous plexus, which results in the
retention of connections to the primitive
cardinal and umbilicovitelline drainage
systems
 The anatomic variants of TAPVC are
dependent upon which connections are
retained
 The cardinal venous system provides
connections to the innominate vein, right
atrium, superior vena cava, or azygous vein
 Umbilicovitelline system to the portal or
hepatic vein, or inferior vena cava.

8. WHERE DOES ANOMALOUS PV GOES

9. NATURAL COURSE
Severe obstruction —Die within the first month.
Restrictive interatrial communication — mortality rate of about 80 percent in the first year
of life out of CHF/FTT/LRTI
Unobstructed —Some only mild symptoms with exertion, but most develop progressive
RHF and PAH

10. CLASSIFICATION
Classifications take into account three features: (1) the pathway by which pulmonary
venous blood reaches the right atrium; (2) the presence or absence of obstruction along
the course of the pathway; and (3) the nature of the interatrial communication. The most
widely used clinical classification recognizes supradiaphragmatic connections with or
without obstruction and infradiaphragmatic or infracardiac connections that are always
obstructed.

11. The most common classification system was originally described by Darling et al.
consists of four types
: Supracardiac
: Cardiac
: Infracardiac
:Mixed

12. SUPRACARDIAC:SNOWMAN SIGN- 49%

13. INFRADIAPHRAGMATIC
:OBSTRUCTIVE[GROUND GLASS]-25%

14. CORONARY SINUS[CARDIAC] :16%

15. MIXED:9%

16. MALPOSITION OF SEPTUM PRIMUM

17. PATHOPHYSIOLOGY
UNOBSTRUCTIVE
 Admixture of pulmonary and systemic venous
flow
 RA and RV volume loading
 RV pressure load if ASD restricted
 PBF increases
 PAH
 LA and LV under filled
 Most have PFO

OBSTRUCTIVE






Post capillary pulmonary venous congestion
increased pulmonary lymphatic flow
Reflex pulmonary arterial vasoconstriction
Increase in PVR
Decrease in PBF
A lower volume of saturated blood in the
venous mixture
 Decrease in the CO
 Worse systemic oxygen saturation

18. EQUALIZATION OF 4CHAMBERS
SATURATION
PRESSURE
NONOBSTRUCTIVE TAPVC
CONSTRICITIVE PERICARDITIS

19. LEVEL OF OBSTRUCTION
SUPRACARDIA
C
CARDIAC
 Hemodynamic Drainage to RA
vise
site obstruction
 CC to VV
junction
 SVC/AZYGOS
DRAINAGE
SITE
 Restrictive
ASD
INFRACARDIAC
Drainage to
portal or IVC

20. WHO COULD REPLACED ANGIOGRAM?
Echocardiography
 CT
MR angiography

21. ASSOCIATED
• Single ventricles
• Heterotaxy with asplenia or polysplenia
https://circ.ahajournals.org/content/122/25/2718.full

22. PHYSICAL EXAMINATION
UNOBSTRUCTED








FS S2:RV over load
right ventricular heave
ESM :^PBF
EDM :^ TV blood flow
S3 gallop
Hepatomegaly :RHF
Tachypnea
Variable degrees of cyanosis
OBSTRUCTED
[starts at birth]







P2: loud
ESM :less
Continuous murmur at obstruction
Cyanosis
Hypotension/low volume
Hepatomegaly
Respiratory distress

23. DD
Atrial Septal Defect
Hypoplastic Left Heart Syndrome
Mitral Stenosis, Congenital
Single Ventricle
Transposition of the Great Arteries
Truncus Arteriosus

24. TREATMENT
UNOBSTRUCTED
surgical redirection can be performed within the
first month of life
 PGE1
 BAS
 surgery should be undertaken emergently.

25. SURGICAL OUTCOMES
https://circ.ahajournals.org/content/122/25/2718.full

26. ONLY STUDENTS CAN SMELL
External link
https://circ.ahajournals.org/content/122/25/2718.full