APVD

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APVD/APVR-anomolous pulmonary venous drainage/return

types:

  1. PAPVD(partial anomolous pulmonary venous drainage) failure of 1-3 pulmonary veins to drain into LA where alternatively drains into SVC/lt SVC(PLSVC)/innominate vein /IVC
  2. TAPVD(total anomolous pulmonary venous drainage) failure aii the pulmonary veins to drain into LA where alternatively drains into systemic circulation

 

SYMPTOMS:

  • asymptomatic
  • effort intollerance
  • growth retardation

SIGN:

  • S1+splitting  prominent S2(due to systolic flow through PV) +Ext0+Add0+Murmur (systolic flow murmur @ lt 2nd ICS)

SHUNTING: lt to Rt

if there is ASD -more LR shunt

may lead to RVH, TR, SVT

 

 

TAPVD

 

SYMPTOMS:

  • asymptomatic
  • effort intollerance
  • growth retardation

SIGN:

  • Acyanotic(cyanosis If pulmonary venous obstruction)
  • S1+splitting  prominent S2(due to systolic flow through PV) +Ext0+Add0+Murmur (systolic flow murmur @ lt 2nd ICS)

SHUNTING: lt to Rt

but aRL shunt must be needed for survival

if there is ASD -more LR shunt

may lead to RVH, TR, SVT

 

dIAGNOSIS:

  1. ABG(acidosis hypoxia)
  2. CXR

i.no obstruction: only increased vascular marking

ii.if vascular obstruction:

  • congestion
  • prominent pulmonary artery
  • rt atrial silhouette
  • in supra cardiac type-upper mediastinal silhouette(snowman/figure of eight sign)

3.echo

4.cardiac cath

5.MRI

 

RX:

medical  by PGE2 (to make keep the ducts patent)

catheter stenting

surgical

TAPVC-ligation of vertical vein and re routing to LA /venous cut down

FAQ:

  1. how compensatory shunting develops in TAPVD?
  • by ASD
  • PFO
  • PDA
  • VSD

TAPVC with compensatory ASD (25.6 x 17.6mm), vertical vein length 50.3mm and dia -17.2mm, confluence length61.2mm and diameter 17.0mm. four pulmonary veins are forming a confluence superior to left atrium from this confluence 1 vewrtical vein has been formed on the left side and draining into the bracheocephalic vein and thence into the right atrium via SVC

2.how venous obstruction develops?

  • hypertrophy of media
  • intimal fibrosis
  • lymphangiectasis

3.when cyanosis develops in APVD

PAPVD-

  • ASD
  • pulmonary hypertension
  • eissenmenger’s syndrome

TAPVD:

  • when there is venous obstruction
  • compression of superior vertical vein between left pulmonary artery and left bronchus(in supra cardiac variety)
  • compression of inferior vertical vein betweenpulmonary venous confluence and systemic vein(in infra cardiac variety)

4.types of TAPVD

 

1.supra cardiac(45-55%)all veins confluent to form superior vertical vein and drain in to SVC/PLSVC/innominate vein

2.intra cardiac(15-20%)all drains into coronary sinus/RA

3.infra cardiac(15-25%))all veins confluent to form inferior/descending vertical vein and drain in to portal vein/ductas venosus/IVC

4.mixed(5-10%)

  • bilateral asymmetric (46%) 3 drains @ common site and 1 @ a remote site
  • bilateral symmetric(29%)
  • all forms confluent and drain @ a common site and the confluent itself drainto another site

 

5.CXR findings

 

i.no obstruction: only increased vascular marking

ii.if vascular obstruction:

  • congestion
  • prominent pulmonary artery
  • rt atrial silhouette
  • in supra cardiac type-upper mediastinal silhouette(snowman/figure of eight sign)

 

 

figure of eight appearance(TAPVD)

 

 

 

 

case study:

case-1(TAPVC)

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