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An overview of Total Anomalous Pulmonary Venous Return: pathyphysiology, evaluation, treatment.

Laura D

on 23 June 2014

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Transcript of TAPVR

Relatively small left side of heart
ASD/PFO necessary for survival
Level of desaturation is proportional to pulmonary blood flow
Elevated pulmonary venous pressure freely transmitted to pulmonary capillary bed
Laura D'Addese, M.D.
Total Anomalous Pulmonary Venous Return
Northern Great Plains Registry of Congenital Heart Disease
Clinical Manifestations
Name that Lesion
39 week old male born via C/S for breech presentation to a 24 yo G1P0 female, serologies negative
Apgars 8, 9 at 1 and 5 mins, respectively
By 10 mins: respiratory distress --> oxihood --> intubation
PaO2 by ABG in 40s!!!
The Case
Differential Diagnosis
Cyanotic Heart Lesions
Transposition of the great arteries
Tetrology of Fallot
Truncus arteriosis
Tricuspid atresia
Hypoplastic left heart
Pulmonary atresia
Pulmonary stenosis (critical)
Patent foramen ovale with pure right-to-left flow.
No significant valvar dysfunction.
Systemic to supra-systemic RV pressure by septal position.
Small PDA with an aneurysmally dilated ampulla at the aortic end. Low velocity bidirectional flow (right-to-left in systole).
Qualitatively mild-to-moderate right ventricular systolic dysfunction.
Qualitatively good left ventricular systolic function.
No significant pericardial effusion.
Bilateral pleural effusions and an umbilical line in the descending aorta.
Infradiaphragmatic total anomalous pulmonary venous return with obstruction.
Drainage of the pulmonary circulation into the systemic circulation
Characterized by a common pulmonary vein (CPV)
Occurs in 0.6 – 1.2 in 1,000 live births
Prevalence: 1% of all congenital heart diseases
Fifth most common cyanotic heart lesion
Baltimore-Washington Infant Study showed a possible association w/ exposure to pesticides, lead, and paint/paint-stripping chemicals
Monogenic pattern of inheritance suggested from family cases
Associated w/ asplenia, polysplenia, cat's-eye syndrome
What's the differential???
What's the next step?
Define Total Anomalous Pulmonary Venous Return (TAPVR)
Review the anatomy
Explain the physiology of TAPVR
Appreciate clinical manifestations
Understand the management, treatment, and prognosis of TAPVR
CPV drains into SVC via left inominate; OR via azygous vein or persistent LSVC
CPV drains into coronary sinus
4:1 male predominance
2. Cardiac (20%)
3. Infracardiac (20%)
4. Mixed (10%)
CPV drains into portal vein, ductus venosus, hepatic vein, or IVC
1. Supracardiac (50%)
Combination of other types
Basic Principles
between LPA and left main bronchus; 50% of cases
stenosis at mouth of coronary sinus
diaphragm, ductus venosus, liver parenchyma
tortuous/stenotic/atretic pulmonary veins and vertical veins OR restrictive atrial septum
Microscopic Anatomy
Common pulmonary vein origins:
evagination in sinoatrial region
confluence of vessels from pulmonary plexus
confluence of capillaries b/w lung buds and heart that grows into mesocardium
Sites of Obstruction
Restrictive ASD
survival proportional to size of ASD
Pulmonary flow 3-5x systemic flow
O2 sats in RA may be > 90%
O2 sats in P/S circulation are equal
RV dilation and hypertrophy
PA dilation may occur
PAp slightly elevated to systemic
Sherman & Bauersfeld
adventitial fibrosis; focal medial fibrosis
Anomalous vessels
Left Atrium
a) w/o obstruction
b) w/ obstruction
atrophy (narrow muscle fibers w/ scant cytoplasm)
unobstructed veins: wall atrophy OR hypertrophy of intima, media-adventitia, or both
obstructed veins: media-advential thickening + intimal proliferation
prominent medial hypertrophy of muscular arteries/arterioles
medial hypertrophy of extrapulmonary veins + small lung veins
prominent dilation of interlobular and subpleural lymphatics
prominent medial hypertrophy of arteries/arterioles
Pulmonary edema; prevented by:
increased lymphatic flow
altered capillary wall permeability
reflex arteriolar constriction
PHTN, RVH, right heart failure
Leftward shift of interventricular septum
Decreased cardiac output
Systemic to supra-systemic RV pressure by septal position.
Right ventricular systolic dysfunction.
N = 74 patients
56% symptomatic in 1st mo.; rest by 1 yr
tachypnea, feeding difficulties
FTT, recurrent respiratory infections
cardiorespiratory failure by age 6 mos.
RV heave
loud S1; systolic ejection click
widely split, fixed S2
S3 almost always present
grade II/VI systolic murmur @ LUSB
venous hum
Physical Exam
RA enlargement
right-axis deviation
RV hypertrophy
increased pulmonary blood flow
enlarged RA and RV
prominent PA segment
"figure-of-8" or "snowman" sign
N = 43 patients
72% symptomatic in 1st mo, rest by 1 yr
symptoms usually absent in first 12 hrs
age at death from 2 days to 4.5 months
cyanosis & dyspnea accentuated by swallowing
no significant RV heave
loud S1; systolic ejection click
widely split S2
grade II/VI systolic murmur @ LUSB
bibasilar moist rales
absent RA enlargement
right-axis deviation
RV hypertrophy
diffuse, stippled densities; reticular pattern branching from hila
normal/near normal heart size
prominent superior pulmonary veins
Cardiac cath
definitive diagnosis
97% sensitive; 99% specific
no longer needed
useful when ECHO fails to resolve anatomy
provides hemodynamic info for palliative procedures
defines pulmonary venous anatomy and surrounding structures
RAp > LAp by at least 2 mmHg is indicative of a restrictive inter-atrial communication
Medical management
oxygen supplementation
mechanical ventilation
inotropic support
may be needed both pre- and post-operatively
size of inter-atrial communication
presence of obstructing lesions
degree of pulmonary HTN
size of pulmonary veins (Jenkins et al.)
survival dependent on:
survival dependent on:
degree of pulmonary HTN
recurrence of pulmonary vein obstruction
10% of patients
mortality rates of 25-46%
development of arrhythmias
CHB (1998-2004) 3-yr survival rate was 85.2% (CI (81.3-88.4%)
required in 10-15% of patients
rarely required if re-stenosis does not occur within 1 year after surgical repair
Surgical repair on DOL #0
Post-operative ECHO:
Back to our patient...
- No ASD
- Trivial TR and MR
- Vertical vein remains patent
- Qualitatively normal LV function
- No pericardial effusion
POD #5 - chest tubes removed
POD #6 - extubated to NC
POD #12 - discharged home
Take Home Points
TAPVR = absent pulmonary venous connection to systemic circulation
Four anatomic variants
Right-to-left shunting produces cyanosis
Inter-atrial communication necessary for survival
Clinical presentation dependent on presence and degree of pulmonary venous obstruction
Echocardiography provides diagnosis
Surgery has >85% survival rate
Re-intervention necessary in 10-15% of patients
Special Thanks to...
Dr. Juan Carlos Muñiz
Dr. John Dykes
Batool, Salina, and Jenny
Works Cited
1. Keane, John F and Flyer, Donald C. Total Anomalous Pulmonary Venous Return. Nadas' Pediatric Cardiology
2. Geva, Tal and Van Praagh, Stella (2008) Anomalies of the Pulmonary Veins. Moss and Adams' Heart Disease in Infants, Children, and Adolescents: Including the Fetus and Young Adult.
3. Soriano, B and Fulton, DR (2013) Total Anomalous Pulmonary Venous Return. UpToDate. www.uptodate.com
4. Total Anomalous Pulmonary Venous Connection. (March 2013). American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Total-Anomalous-Pulmonary-Venous-Connection-TAPVC_UCM_307039_Article.jsp
5. Congenital Heart Disease and Defects. Children's Hospital Wisconsin. https://www.chw.org/display/PPF/DocID/34305/Nav/1/router.asp
6. Hoffman JL, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890-1900.
7. Congenital Heart Disease (September 2011). Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/data.html
- Lasix 40mg BID
- Digoxin 15mcg BID
Name the cyanotic heart lesions...
What are common sites of obstruction?
Remember our patient's ECHO?
Full transcript