Tell Me How the Wind Sounds Book By: Leslie D. Guccione Presentation By: David B. Ayscue, Jr.
Pulmonary Venous Anomalies...Musolino AM, Santoro G, Marino B, Formigari R, Guccione P, Pasquini L....
Transcript of Pulmonary Venous Anomalies...Musolino AM, Santoro G, Marino B, Formigari R, Guccione P, Pasquini L....
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Pulmonary Venous Anomalies
NHS.
Norman H Silverman MD. D Sc (Med),FACC, FASE. FAHA.Professor of Pediatrics
Stanford University Lucile Packard Children’s Hospital
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Totally Anomalous Pulmonary Venous Connections.
• Have a variety of clinical presentations from neonatal respiratory distress and pulmonary hypertension to a presentation of clinical features similar to an atrial septal defect.
• In the neonate the features may be indistinguishable from respiratory distress or pulmonary hypertension with many diagnosis made by echocardiography as a prelude to placing patients on ECMO.
• As this is an emergency cardiac presentation and can be cured surgically this diagnosis must always be considered prior to placing infants on ECMO.
• This “obstructive pattern” may also be seen in Right Isomerism ( Asplenia, or Ivermark’s Syndrome)
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Totally Anomalous Pulmonary Venous Connections.
• The lesion is classified according to the site of drainage. The sites of drainage may be:-
– Above the heart (The Commonest)– To the Heart (The Rarest)– Below the heart– There may be mixed drainage to differing sites. Commoner
than believed – Always think of this!
• Lesions may be obstructed at differing sites. Although the infradiaphragmatic site is invariably obstructed, the commonest site for obstruction is to a left vertical vein.
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Pulmonary Vein Embryology
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Pulmonary Vein Embryology
NHS.
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Totally Anoamlous Pulmonary Venous Connections.
NHS.
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NHS.
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PulmonaryVenous Anomalies
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General Features of TAPVR.A 4 Ch.
S Cost. Sag.
RVLV
P Sx
S Cost. Cor.
RV
LV
RALA
V
LVRV
RA
LA
LVRV
LV
RV
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NHS.
Supracardiac TAPVR: Large Innominate Vein.SSN. Sag.
Inn. Vn
Ao
RPADuct
D Ao
S Cl A
LCA
IA
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Supradiaphragmatic TAPVR
NHS.
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Usual Supracardiac TAPVR.
Inn Vn
SVCV Vn
S Cost. Cor..
S Cost. Cor.
SVC
Ao MPA
P Vn P Vn
Inn VnSVC
VVn
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MRI of Supradiaphragmatic TAPVR
NHS.
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The Hemodynamic Vise
NHS.
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Supracardiac TAPVR, with Obstruction.
SSN. Cor
SSN. Cor
V Vn
SVC
V Vn
SVC
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Supracardiac TAPVR, with Obstruction.
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The Hemodynamic Vise.
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Azygos Pulmonary Venous Drainage.
3/7 Asplenia
Musolino AM, Santoro G, Marino B, Formigari R, Guccione P, Pasquini L. Echocardiographic diagnosis of totally anomalous puylmonary venous connection to the azygos vein. Cardiol Young 1999;9:305-309.
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Supracardiac TAPVR: Azygos Drainage
NHS.
S Cost. Sag.
SSN. Sag.
SVC
Az Vn
PARA
RAA
Az Vn
SVC
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Supracardiac TAPVR: Drainage to R SVC
NHS.
SSN. Cor V Vn
RA
S Cost. Cor.
SVC A Ao
P Vn
P Vn
PA
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PulmonaryVeins to the Right Atrium.
4 Ch.
LALVRA
RV
P Vn
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TAPVR: to Right Atrium
NHS.
A 4 Ch.
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TAPVR: to Right Atrium
NHS.
S Cost. Sag.
SVCP Vn
IVCRA
P Sx
RA LAP Vn
RV
S Cost. Cor.
RA
P VnP Vn
LV
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Drainage to the Coronary Sinus.
NHS.
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TAPVR: to Coronary Sinus
NHS.
CS
RALV
RV
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TAPVR: to Coronary Sinus
NHS.
S Cost. Sag.
S Cost. Cor.
Ao
PA
RV RA
CSLA
D Ao
P A CS
LV
RV
P A
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TAPVR: to Coronary Sinus
CS
Liver
RA
D Ao
P Vn
P Vn
SSN. Cor
S Cost. Cor.Inn Vn
SVC
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NHS.
Infradiaphragmatic TAPVR.
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CT for TAPVR below the diaphragm
NHS.
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TAPVR: Below Diaphragm-The Descending Vein
NHS.
S Cost. Sag.
Scost. Lx
V Vn
RV
LV
PA
V Vn
V Vn
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TAPVR: Below Diaphragm
S Cost. Sag.
D AoRA
Portal VnH Vn
V Vn
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TAPVR: Below Diaphragm
Ao PA
LA
SSN . Cor.
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TAPVR: Below Diaphragm
Liver
RA
V VnLiver
RA
V Vn
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TAPVR: Below Diaphragm
Liver
V Vn
LVRV
RA
Contrast Echocardiography
Posterior view behind atrium
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TAPVR: Below Diaphragm
S Cost. Sag.
Liver
V Vn
D AoLA
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TAPVR: Below Diaphragm
V Vn
D Ao
LiverS Cost. Sag.
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TAPVR: Below Diaphragm (Surprise!)V VnInn Vn
SSN. Cor
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Mixed TAPVR
NHS.
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Partial Anomalous Pulmonary Venous Connection.
• Variable presentation from no symptoms to the symptoms associated with atrial septal defects
• Echocardiography requires systematic search for all pulmonary venous connections
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Anomalous Left Upper Pulmonary Vein.
NHS.
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Partial Anomalous Veins to Innominate Vein
NHS.
A 4 Ch.
RALA
D Ao
LVRV SSN. Cor
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L Vertical Vein.
NHS.
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NHS.
Scimitar Syndrome: Anomlaously Draining Right Pulmonary Veins to IVC.
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Scimitar: MRA
NHS.
IVCAn Vn
H Vn H Vn
S Cost. Cor.
S Cost. Cor.
Liver
An Vn
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Scimitar Syndrome: Mesocardia!.
S Cost. Cor.
RVLV
Liver
PA
Ao
Hypoplastic Right Lung
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Scimitar
NHS.
RA
RV
H Vn
IVC
H Vn
IVC RA
IVCH Vn
RA
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Scimitar Syndrome: Anomlaously Draining Right Pulmonary Veins to IVC.
47
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Partial Anomalous Veins to SVC
NHS.
A 4 Ch.
LVRV
LARA
RA
Az Vn
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Partial Anomalous Veins to Right Atrium
NHS.
S Cost. Cor.
LA
LVRV
RAAnom Vn
Liver
S Cost. Sag.
RA
SVC RPA
Anom Vn
Anom Vn
RA LA
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IbrahimM,BurwashIG,MortonB,BraisM.Directdrainageoftherightpulmonaryveinsintothecoronarysinuswithintactinteratrialseptum:acasereport.CanJCardiol.2001Jul;17(7):807-9.
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Apical 4 Chamber View
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Parasternal 4 Chamber View
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Parasternal Short Axis View
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Preoperative Study
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Post BypassEarly Late
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Post BypassEarly Late
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.
NHS.
Thank You!