Congenital 1-100 Flashcards
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
- frequent pulmonary infections
- fatigue
- exercise intolerance
- palpitations.
-
Atrial arrhythmias:
- atrial flutter
- atrial fibrillation
- sick sinus syndrome
- Flow-related PAH accompanies large left-to-right shunts
- 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:
- 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
- Simple - Below the Aorta
- Taussig - Bing - below the pulmonic
- Doubly committed VSD
- 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
- Tetralogy of Fallot
- Tricuspid atresia or Ebstein’s anomaly
- Pulmonic atresia or stenosis
Ductal independent cyanotic congenital heart lesions
- Truncus arteriosus
- Transposition of the great arteries
- Total anomalous PVR
- 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
80%
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)
25cc/kg
Types and prevalence of ASD
- Secundum (80%)
- sinus venosus (10%)
- Primum (10%)
- Coronary sinus (10%)
potential complications of ASD ?
Bleeding
Residual ASD
Sinus node dysfunction
Baffle occlusion
Patient with TAPVR
medical support
Low oxygen setting
High PEEP (decrease pulmonary blood flow / edema)
Inotropes
Avoid - Prostaglandins and pulmonary vasodilators
what to look for on Echo for TAPVR
- Anatomic variant
- Supracardiac
- Cardiac
- Infracardiac
- Presence and location of the shunt
- Survival is dependent on RàL shunt
- Almost always due to non-restrictive PFO
- Evidence of RV Fluid overload
Medical / preop management of TAPVR
- Patients requiring resuscitation should be intubated
- kept on low oxygen settings and high PEEP
- decrease pulmonary blood flow and limit pulmonary edema.
- Inotropes should be used as necessary.
- AVOID: Prostaglandins and pulmonary vasodilators should be avoided.
- TAPVR with obstruction: surgical emergency
- TAPVR without obstruction: may optimize with diuretics and supplemental oxygen, proceed with early elective repair
- 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.
- Obtain an echo (transthoracic) to evaluate the right sided pulmonary venous return.
- Consider contrast enhanced MRI, CTA of the heart, or possibly catheterization to look for obstruction.
- May need surgical revision with sutureless technique (see above).
Shone complex
Shone complex
four obstructive left heart lesions
- supravalvular mitral membrane
- parachute mitral valve
- muscular or membranous subvalvular aortic stenosis
- coarctation of aorta.
when is coarc significant
> 50% stenosis
vent settings to prevent pulm overcirculation with coarctation of the aorta
- Ventilation (prevent pulm over circ)
- Low FiO2
- pCO2 > 45
Indications for ASD repair in children
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
Adults:
- Pulmonary hypertension: ASD should be fixed to prevent
- If PVR < 10 u/m2, good outcomes.
- IF PVR > 15 à high mortality
