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Flashcards in Congenital 1-100 Deck (29)
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What anomaly is associated with WPW ? 

Ebstein's Anomaly 


Physiologic effect of ASD with shunt 

Left  to right shunt 

RV failure 

Pulmonary over circulation 


Sequelae of ASD 

symptoms from excess pulmonary blood flow and right-sided heart failure

  1. frequent pulmonary infections 
  2. fatigue
  3. exercise intolerance 
  4. palpitations.
  5. Atrial arrhythmias: 
    • atrial flutter
    • atrial fibrillation
    • sick sinus syndrome
  6. Flow-related PAH accompanies large left-to-right shunts
  7. Paradoxical embolism


indication for closure of smaller ASD 

Small ASDs with a diameter of less than 5 mm and no evidence of RV volume overload do not impact the natural history of the individual and thus may not require closure

unless associated with paradoxical embolism.


Class I indications for the closure of an ASD 

Closure of an ASD either percutaneously or surgically is indicated for:

  1. right atrial and RV enlargement with or without symptoms. (Level of Evidence: B)


PAP and indications for the closure of an ASD 

Closure of an ASD, either percutaneously or surgically, may be considered in the presence of:

net left-to-right shunting,

pulmonary artery pressure < 2/3 systemic levels,

PVR < 2/3 SVR 

or when responsive to either pulmonary vasodilator therapy or test occlusion of the defect (patients should be treated in conjunction with providers who have expertise in the management of pulmonary hypertensive syndromes). (Level of Evidence: C)


Variants of DORV 

Based on the location of the phenotypic large VSD 

  1. Simple - Below the Aorta 
  2. Taussig - Bing - below the pulmonic 
  3. Doubly committed VSD 
  4. Non-committed - remote to the great arteries 


Taussig - Bing heart 

DORV with VSD below the pulmonary artery 


Ductal dependent  cyanotic  congenital  heart diseases 

Ductal-dependent lesions

  1. Tetralogy of Fallot
  2. Tricuspid atresia or Ebstein’s anomaly
  3. Pulmonic atresia or stenosis



Ductal independent cyanotic congenital heart lesions 

  1. Truncus arteriosus 
  2. Transposition of the great arteries 
  3. Total anomalous PVR 
  4. HLHS 


Teleologic reason for prematurity and PDA 

respiratory distress results in elevation of prostaglandin levels that may lead to persistence of the PDA 


Indomethacin treatment of PDA 

0.2mg/kg over 20 minutes at 12 and 24 hours 


what % of premature  infants will have a successful closure of a PDA with inodmethacin 



Indications for the closure of a PDA in adult patients 

signs of LV volume overload 

PAH but PAP <2/3 of systemic  pressure or PVR <2/3 of SVR 

Device closure is the method of choice where technically suitable  PDA closure should be considered in patients with PAH and PAP >2/3 of systemic pressure or PVR >2/3 of SV but still net L–R shunt (Qp:Qs >1.5) or when testing (preferably with nitric oxide) or treatment demonstrates pulmonary vascular reactivity 


III C PDA closure should be avoided in silent duct (very small, no murmur)  PDA closure must be avoided in PDA Eisenmenger and patients with exercise-induced lower limb desaturation 


Criteria for a "hemodynamically significant" pda

two or more of the following signs are present:

-increased pulse volume or widened pulse pressure

-hyperactive precordium

-increased pulmonary vascular markings on chest radiograph



should all PDA in adults be closed 

Yes, endocarditis 


What are the complications of PDA ligation (surgical)

Chyle leak (<2) 

Vagus / recurrent laryngeal nerve injury (2-9%) 

Hemorrhage (<1%) 

Recanaliztion (@) 


pediatric cardioplegia dose (for  at least ASD repair) 



Types and prevalence of ASD 

  1. Secundum (80%) 
  2. sinus venosus (10%) 
  3. Primum (10%) 
  4. Coronary sinus (10%) 


potential complications of ASD ? 


Residual ASD 

Sinus node dysfunction 

Baffle occlusion 


Patient with TAPVR 

medical support 

Low oxygen setting 

High PEEP (decrease pulmonary blood flow / edema) 


Avoid - Prostaglandins and pulmonary vasodilators 


what to look for on Echo for TAPVR 

  1. Anatomic variant
    1. Supracardiac
    2. Cardiac
    3. Infracardiac
  2. Presence and location of the shunt
    1. Survival is dependent on RàL shunt
    2. Almost always due to non-restrictive PFO
  3. Evidence of RV Fluid overload


Medical / preop management of TAPVR 

  1. Patients requiring resuscitation should be intubated
    1. kept on low oxygen settings and high PEEP
    2. decrease pulmonary blood flow and limit pulmonary edema.
  2. Inotropes should be used as necessary.
  3. AVOID: Prostaglandins and pulmonary vasodilators should be avoided.
  4. TAPVR with obstruction: surgical emergency
  5. TAPVR without obstruction:  may optimize with diuretics and supplemental oxygen, proceed with early elective repair



  1. After TAPVR -, the baby has difficulty weaning from ventilator, CXR shows right pleural effusion and congestion.  

 likely has an obstruction at the anastomosis of the right veins.

  1. Obtain an echo (transthoracic) to evaluate the right sided pulmonary venous return.
  2. Consider contrast enhanced MRI, CTA of the heart, or possibly catheterization to look for obstruction.
  3. May need surgical revision with sutureless technique (see above).


Shone complex


Shone complex

four obstructive left heart lesions

  1. supravalvular mitral membrane
  2. parachute mitral valve
  3. muscular or membranous subvalvular aortic stenosis
  4. coarctation of aorta.


when is coarc significant 

> 50% stenosis 


vent settings to prevent pulm overcirculation with coarctation of the aorta 

  1. Ventilation (prevent pulm over circ)
    1. Low FiO2
    2. pCO2 > 45


Indications for ASD repair in children


  • Large shunt, FTT
  • Evidence of RV / LV volume overload
  • Secundum – should be corrected at 3-5 years
  • TIA/stroke


Indications for ASD repair in adults 


  • Pulmonary hypertension: ASD should be fixed to prevent
  • If PVR < 10 u/m2, good outcomes.
  • IF PVR > 15 à high mortality