Diapositiva 1

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Course in Pediatric Cardiology, Anaesthesia and
Cardiac Surgery A Project of International
Cooperation MILAN 19.01.2007
Anomalies of the Pulmonary Venous
Drainage I.BIANCA, C. MIGNOSA
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DEFINITION

• The
• Anomalous Pulmonary Venous Connection is a
  congenital heart defect in which the pulmonary
  veins drain into a systemic venous structure,
  rather than directly into the left atrium.

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B
A
From Moss and Adams Heart Disease in Infants,
Children and Adolescent 2001 Lippincott
Williams Wilkins

• The lung bads are enmesched by the vascular
  plexus of the forgut.
• There are multiple connections to the UV and
  cardinal venosus system
• B The common pulmonary vein connects the
  pulmonary venous plexus
• and the heart

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B
A
D
C
From Moss and Adams Heart Disease in Infants,
Children and Adolescent 2001 Lippincott
Williams Wilkins
C. The connections between the pulmonary plexus
and the splanchnic venous plexus involute D
The common pulmonary vein incorporates into the
LA The individual pulmonary veins connect
separately and directly to the LA
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Anomalous Pulmonary Venous Drainage

• TWO TYPES
• Partial (P-APVD)
• Mild physiologic abnormality
• Usually asymptomatic
• Total (T- APVD)
• Serious physiologic abnormalities

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From Moss and Adams Heart Disease in Infants,
Children and Adolescent 2001 Lippincott
Williams Wilkins
Partial-APVD results from a failure to estabilish
a connection between one or more of the pulmonary
veins with the common pulmonary vein, before the
connections with the splanchnic plexus system are
regressed
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From Moss and Adams Heart Disease in Infants,
Children and Adolescent 2001 Lippincott
Williams Wilkins
Total-APVD results from a failure to estabilish a
connection between the pulmonary venous plexus
and the common pulmonary vein, before the
connections with the splanchnic plexus system are
regressed
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Partial -APVD

• One / two of the four pulmonary veins drain into
  the right atrium
• Clinically well tollerated
• Associated with ASD (SV and/or S-ASD)

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Sinus Venosus -ASD
- In SV- ASD the atrial communication is located
OUTSIDE the TRUE septal structure a true septal
structure a structure that can be removed
without exiting the heart cavities - The wall
separating the SVC from the LA is formed by the
roof of RUPV entering the LA - A deficency in
this wall UNROOFS the RUPV resulting not only in
a SVC OVERRAIDING but olso in an anomalous
connection of the RUPV.
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NORMAL
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P AVPD (left lung) to SVC
L LUNG
SVC
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Return of blood from lungs is mostly to LA
One vein abnormally connected to right heart
Frequently associated with sinus venosus or
secundum ASD
RV overloaded
Partial Anomalous Pulmonary Drainage
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Partial APVDPATHOPHYSIOLOGY

• Similar to ASD
• RA dilatation
• RV volume overload (RV dilatation)
• Increased pulmonary bood flow

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Partial APVDDIAGNOSTIC

• Cardiac Catheterization
• Its indicated if
• Pulmonary hypertention is suspected
• Interventional catheterization is necessary
• occlusion of Ao-Pulmonary collaterals as in
• Scimitar Syndrome

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Partial APVDDIAGNOSTIC

• ECHOcardiography
• 2D
• Color
• TEE
• MRI
• Advantages WILDE FIELD OF VIEWS
• EXCELLENT SPATIAL ORIENTATION
• 3-D VIEWS

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SVC
RA appendage
P APVD ( RUPV draining in SVC)
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SV - ASD
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SVC
Pulmonary vein
Korean Journal of Radiology
P- APVD
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Partial APVDScimitar Syndrome

• 1-3 per 100.000 births
• Right lung HYPOPLASIA
• RPA HYPOPLASIA
• DEXTROPOSITION of the heart
• Aortic-pulmonary collaterals supplying the right
  lung

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Partial APVD


RA

IVC
Aortic-pulmonary collaterals
Scimitar Syndrome
Anomalous pulmonary vein
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Scimitar Syndrome
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Partial APVDSurgery

• Indications for surgery similar to what has been
  considered for the ASD

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Partial APVDSurgery

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Partial APVDSurgery

Ao
RA
SVC
RPVs
RL
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Partial APVDSurgery

ASD
RUPV
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Partial-APVD

• Surgery
• How to do it ?
• General Anesthesia
• Median Sternotomy / Right Thoracotomy
• Normothermic CPBP / Bi-Caval Cannulation
• Right Atriotomy

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDSurgery

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Partial APVDFOLLOW UP problems

• Arrhythmias
• Pulmonary venous obstruction
• Echocardiography
• MRI
• Angiography
• SCINTIGRAPHY

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T-APVD

• Surgery

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Total APVDPOSTOPERATIVE PROBLEMS

• Low cardiac output
• Pulmonary oedema
• Pulmonary hypertensive crises
• Rhythm disturbances
• Phrenic nerve damage
• Cerebral damage

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Total APVDPOSTOPERATIVE PROBLEMS

• Low Cardiac Output
• Non compliant LV
• Sizes and compliances of LA and LV are
  influenced by
• dilated rigt-sided structures
• Mainteinance of optimal heart rate
• LV filling pressure maintained at 15 mmHg
• Avoid overaggressive volume replacement

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Total APVDPOSTOPERATIVE PROBLEMS

• Perioperative Dysrhytmias
• 20
• Supraventricular tachydysrhythmias

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Total APVDPOSTOPERATIVE PROBLEMS

• Pulmonary Hypertension
• Significant risk of death
• in the immediate postoperative period

Nitric Oxide
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Total APVDPOSTOPERATIVE PROBLEMS

• Respitatory Insufficency
• Pulmonary edema prior to surgery
• Parlysis and sedation
• Positive End-Expiratory Pressure ( PEEP )
• ExtraCorporeal Membrane Oxygenation ( ECMO )

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Total APVDPOSTOPERATIVE PROBLEMS

• Respitatory Insufficency
• Phrenic nerve damage
• Hemidiaphragm plication

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Total APVDPOSTOPERATIVE PROBLEMS

• ANASTOMOTIC STRICTURE
• Incidence 4-18
• (mean 4.8)
• Reduced with the use of circulatory arrest

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Total APVDPOSTOPERATIVE PROBLEMS

• CEREBRAL DAMAGE
• Reduced with the use of cerebral perfusion
• NIRS monitoring

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Suggested Literature Strark-deLeval Surgery for
Congenital Heart Defects. 3th Edition W.B.
Saunders Company Wilcox-Anderson Surgical
Anatomy of the Heart Gower Medical
Publishing Moss and Adams Heart Disease in
Infants, Children and Adolescent Lippincott
Williams Wilkins 2001