Titledrpauthor: WTBKLast, A SubdrpJob

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ASD and VSD in Adults Identification and
Indications for Intervention Martha Grogan, MD
No disclosures
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Atrial Septal Defects
SVC
SV
SEC
P
RV
CS
IVC
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Secundum ASD
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Secundum ASD - TEENormal Pulmonary Veins
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ASD Closure
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Secundum ASD
WDE

• Usually isolated abnormality, 5 with APVC
• Intervention for ? right heart, exclude PHT
• Device closure feasible in most

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Device ASD Closure

• Alternative to surgery for secundum ASD
• Largest device 38 mm
• 3-5 mm septal rim
• TEE or IC echo
• Monitor placement
• Surgery TR, AF, APV
• multiple ASDs

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Secundum Atrial Septal Defect

• ? survival with surgery/intervention
• Konstandinides et al - NEJM 1995
• Patel et al - J Interv Cardiol 2007
• Excellent prognosis after closure
• Spies et al - Clin Res Cardiol 2007
• PHT rare with ASD
• Persistent risk of AF with ? age at op
• Murphy et al - NEJM 1990

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ASD - AFib/Flutter
Murphy et al NEJM 1990
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ASD - Familial
Holt-Oram Syndrome Familial ASD with AV
conduction defect
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Primum ASD
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Primum ASD Partial AV Defect

• ECG - left axis deviation, first degree AV block
• Associations - MV and TV cleft, VSD, LVOT
  obstruction

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Partial AV Septal Defect

• Partial AV canal repair in pt 40 years - low
  risk
• Long-term survival good - ? in symptoms
• Bergin ML et al - J Am Coll Cardiol 1995
• Reoperation for left AV valve malfunction, or
  relief of LVOT obstruction necessary in gt10
• El-Najdawi et al - J Th CV Surg 2000

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28 -Year-Old FemaleSevere MR

• Primum ASD closure and repair mitral cleft (age 3
  yrs)
• No Residual shunt
• Severe MR - residual cleft
• Mild LVE, EF 70, mild LA enlargement
• Asymptomatic, but sedentary

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28 -Year-Old FemaleSevere MR due to residual
cleft

• You recommend
• Mitral Valve Repair
• Mitral Replacement Bioprosthesis
• Observation
• Mitral Replacement - Mechanical

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Partial AV Septal DefectCleft Mitral Valve
Lesser chance of MV repair especially if
previous attempt
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55-Year-Old Female w/Breast CancerTTE for Chemo
Right Heart Enlargement
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55-Year-Old FemaleTEE - Sinus Venosus ASD
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TEE - Sinus Venosus ASD Anomalous right upper
pulmonary vein
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Sinous Venosus ASDBreast Cancer

• Central venous catheter placed six months ago
• Received 4 cycles of chemo
• Can she proceed with radiation ?
• Should the ASD be closed now

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Sinus Venosus ASD

• Associations - anomalous PV connection (gt90)
• Surgery - ? morbidity, excellent postop survival
• Attenhofer Jost CA et al - Circulation 2005

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NormalPulmonary VenousConnections
IV
SVC
Ao
LPA
RPA
LPVs
RPVs
LA
Ammash JACC, 1997
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TEE
LU
RU
RL
LL
RPV
LPV
LA
70
110
90
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AnomalousRUPVShort Axis View
AscAo
RUPV
MPA
SVC
RPA
LPA
LPV
LA
RLPV
Esophagus
Ammash JACC, 1997
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Anomalous Right Upper Pulmonary Vein
SVC
RPA
CM881749-25
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Anomalous Right Upper Pulmonary Vein
CM881749-26
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Coronary Sinus ASDLeft-sided SVC
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Coronary Sinus ASDLeft-sided SVC
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Coronary Sinus ASD

• Often difficult to diagnose
• Isolated, with LSVC or complex CHD
• Amenable to surgical closure
• Attenhofer Jost CA et al Cardiol Young 2007

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VSD Classification Scheme STS and European
Association CT Surgery

• Outlet 6 (33 Asia) 2) Membranous 80
• 3) Muscular 10 (20 infants) 4) Inlet 4

Jacobs JP et al Ann Thorac Surg 2000
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Muscular VSD

• TTE diagnostic in most patients
• Identifies number, location, hemodynamics
• chamber size and function

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Muscular VSD

• Spontaneous closure common
• Large defect close
• Operation
• Device available

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What is the Diagnosis?
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Membranous VSD

• Associations endocarditis, AV and TV
  regurgitation
• Management depends on size and associations

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What is the Diagnosis?
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What is the Diagnosis?
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Outlet VSDInfundibular/Supracristal/Doubly
Committed

• Associations AV regurgitation often
  progressive
• Management surgery to prevent worsening AV
  regurg

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Outlet Ventricular Septal DefectDilation and
Distortion of the Right Sinus
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Indications for VSD ClosureOther than Outlet VSD

• QpQs gt1.51
• No associated severe PHT
• Progressive TR or AR
• Recurrent endocarditis
• Left-sided chamber enlargement

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49-Year-Old with Eisenmenger VSD
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Eisenmenger Syndrome

• Long-standing L-R shunt
• ? pulmonary blood flow
• Pulm vascular disease
• Reversal of shunt
• Cyanosis and clubbing

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56 Year-Old Female Eisenmenger PDA

• Diagnosis age 19
• Parents told life expectancy 25 yrs
• Finished college , Married
• Phlebotomy - 30 yrs
• Hg as low as 10 gm/dl
• Self administered iron
• One episode hemoptysis
• SVT - Verapamil

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56 Year-Old Eisenmenger Referred for Transplant

• Recurrent pulmonary infections
• DOE - worse recently, but improving
• 6 min walk 573 m (1882 ft)
• O2 TMET 4.8 min
• 9.1 ml/kg/min
• Hg 18.8 g/dl, Hct 57.8

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Eisenmenger Syndrome
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Eisenmenger Syndrome
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Eisenmenger Syndrome
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Pulmonary Artery Pressure
PR end diastolic 3.6 51 mmHg
TR 5.8 m/sec 135 mmHg
Estimated PA 145/61 mmHg Systemic
90/60 mmHg
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Pulmonary Artery Pressure
PR end diastolic 3.6 51 mmHg
TR 5.8 m/sec 135 mmHg
Estimated PA 145/61 mmHg Systemic
90/60 mmHg
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56 Year-Old Eisenmenger Hgb 19 gm/dl, HCT 58PA
145/61 mmHg
You would consider starting pulmonary
vasodilators and

• Stop phlebotomy
• Stop phelbotomy and start Iron
• Continue phlebotomy
• Continue phlebotomy and evaluate for transplant

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56 Year-Old Female HG 18.7 gm/dl, HCT 57.8PA
145/61 mmHg
You would consider starting pulmonary
vasodilators and

• Stop phlebotomy
• Stop phelbotomy and start Iron
• Continue phlebotomy and list for transplant

Iron 12 ug/dl (nl 35-340), Iron saturation 8
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Eisenmenger SyndromeConservative Management

• Survival gt PPH
• Avoid phlebotomy
• Caution with procedures
• Specialized care
• Promising role of vasodilator Rx

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Eisenmenger SyndromeHematologic Issues
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Iron Deficiency Anemia
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Management Strategies
Avoid
High altitude
Dehydration
Pregnancy
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Management Strategies
Caution
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Eisenmenger Syndrome

• Rosenzweig et al Circulation 1999
• Long-term prostacyclin improves hemodynamics and
  quality of life
• Christensen et al Am J Cardiol 2004
• Bosentan - nonselective ET antagonist
• 9 Eisenmenger pt - bosentan 125 mg twice daily
• ? NYHA class (p 0.03), ? O2 sat (p 0.03)

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Eisenmenger Syndrome

• Galie et al Circulation 2006
• Placebo-controlled trial in pt with Eisenmenger
• Bosentan - well tolerated, ? exercise capacity
  and hemodynamics without compromising oxygen sats

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Eisenmenger SydromeNow 60-Year-Old

• Hg 24 gm/dl
• Sildenafil
• Feels great
• Breaks record for 6 min walk
• 40th Wedding Anniversary

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ASD and VSD Nomenclature and Examples

• ASD
• TTE diagnosis in majority
• Look for associated defects
• Indications for intervention
• VSD
• TTE diagnosis in majority
• ? LA and LV, valve dysfunction
• Indications for intervention

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Congenital Heart DiseaseLargely Pattern
Recognition
Male or Female Seagull?