Pulmonary Venous Anomalies

NHS.

Norman H Silverman MD. D Sc (Med),FACC, FASE. FAHA.Professor of Pediatrics

Stanford University Lucile Packard Children’s Hospital

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)

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.

Pulmonary Vein Embryology

Pulmonary Vein Embryology

NHS.

Totally Anoamlous Pulmonary Venous Connections.

NHS.

NHS.

PulmonaryVenous Anomalies

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

NHS.

Supracardiac TAPVR: Large Innominate Vein.SSN. Sag.

Inn. Vn

Ao

RPADuct

D Ao

S Cl A

LCA

IA

Supradiaphragmatic TAPVR

NHS.

Usual Supracardiac TAPVR.

Inn Vn

SVCV Vn

S Cost. Cor..

S Cost. Cor.

SVC

Ao MPA

P Vn P Vn

Inn VnSVC

VVn

MRI of Supradiaphragmatic TAPVR

NHS.

The Hemodynamic Vise

NHS.

Supracardiac TAPVR, with Obstruction.

SSN. Cor

SSN. Cor

V Vn

SVC

V Vn

SVC

Supracardiac TAPVR, with Obstruction.

The Hemodynamic Vise.

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.

Supracardiac TAPVR: Azygos Drainage

NHS.

S Cost. Sag.

SSN. Sag.

SVC

Az Vn

PARA

RAA

Az Vn

SVC

Supracardiac TAPVR: Drainage to R SVC

NHS.

SSN. Cor V Vn

RA

S Cost. Cor.

SVC A Ao

P Vn

P Vn

PA

PulmonaryVeins to the Right Atrium.

4 Ch.

LALVRA

RV

P Vn

TAPVR: to Right Atrium

NHS.

A 4 Ch.

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

Drainage to the Coronary Sinus.

NHS.

TAPVR: to Coronary Sinus

NHS.

CS

RALV

RV

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

TAPVR: to Coronary Sinus

CS

Liver

RA

D Ao

P Vn

P Vn

SSN. Cor

S Cost. Cor.Inn Vn

SVC

NHS.

Infradiaphragmatic TAPVR.

CT for TAPVR below the diaphragm

NHS.

TAPVR: Below Diaphragm-The Descending Vein

NHS.

S Cost. Sag.

Scost. Lx

V Vn

RV

LV

PA

V Vn

V Vn

TAPVR: Below Diaphragm

S Cost. Sag.

D AoRA

Portal VnH Vn

V Vn

TAPVR: Below Diaphragm

Ao PA

LA

SSN . Cor.

TAPVR: Below Diaphragm

Liver

RA

V VnLiver

RA

V Vn

TAPVR: Below Diaphragm

Liver

V Vn

LVRV

RA

Contrast Echocardiography

Posterior view behind atrium

TAPVR: Below Diaphragm

S Cost. Sag.

Liver

V Vn

D AoLA

TAPVR: Below Diaphragm

V Vn

D Ao

LiverS Cost. Sag.

TAPVR: Below Diaphragm (Surprise!)V VnInn Vn

SSN. Cor

Mixed TAPVR

NHS.

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

Anomalous Left Upper Pulmonary Vein.

NHS.

Partial Anomalous Veins to Innominate Vein

NHS.

A 4 Ch.

RALA

D Ao

LVRV SSN. Cor

L Vertical Vein.

NHS.

NHS.

Scimitar Syndrome: Anomlaously Draining Right Pulmonary Veins to IVC.

Scimitar: MRA

NHS.

IVCAn Vn

H Vn H Vn

S Cost. Cor.

S Cost. Cor.

Liver

An Vn

Scimitar Syndrome: Mesocardia!.

S Cost. Cor.

RVLV

Liver

PA

Ao

Hypoplastic Right Lung

Scimitar

NHS.

RA

RV

H Vn

IVC

H Vn

IVC RA

IVCH Vn

RA

Scimitar Syndrome: Anomlaously Draining Right Pulmonary Veins to IVC.

47

Partial Anomalous Veins to SVC

NHS.

A 4 Ch.

LVRV

LARA

RA

Az Vn

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

IbrahimM,BurwashIG,MortonB,BraisM.Directdrainageoftherightpulmonaryveinsintothecoronarysinuswithintactinteratrialseptum:acasereport.CanJCardiol.2001Jul;17(7):807-9.

Apical 4 Chamber View

Parasternal 4 Chamber View

Parasternal Short Axis View

Preoperative Study

Post BypassEarly Late

Post BypassEarly Late

.

NHS.

Thank You!