Congenital Heart Diseases

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  1. Acyanotic Defects
    • Ventricular Septal Defect
    • Atrial Septal Defect
    • Patent Ductus Arteriosus
    • Pulmonary Stenosis
    • Aortic Stenosis
    • Coarctation of the aorta
    • Atrioventricular septal defect
  2. Cyanotic Defects
    • Tetralogy of Fallot
    • Transposition of the Great Vessels
    • Tructus arteriosus
    • Tricuspid Atresia
    • Total Anomalous Defect
  3. CATCH-22
    • Cardiac defects
    • Abnormal Facies
    • Thymic hypoplasia
    • Cleft Palate
    • Hypocalcemia
    • Chromosome 22 Defects
  4. Common Problems Associated with congenital Heart Disease
    • Infective Endocarditis
    • Cardiac dysrhythmias
    • complete heart block
    • HTN (systemic or pulmonary)
    • Erythrocytosis
    • Thromboembolism
    • Coagulapathy
    • Brain Abscess
    • Increased plasma uric acid concentration
    • Sudden Death
  5. Acyanotic Heart Disease Characteristics
    • Left to right shunt
    • Increased pulmonary blood flow with pulmonary HTN
    • RV hypertrophy
    • Eventual congestive heart failure
  6. Atrial Septal Defect
    • Often undetected 
    • Increased pulmonary flow could dilute IV drugs
    • Avoid prolonged increases in SVR (favors L-R shunt)
    • Decreased SVR (volatiles) or increased pulmonary vascular resistance (PPV: positive pressure ventilation)
    • No Abx prophylaxis unless a concomitant valvular abnormality is present
  7. Ventricular Septal Defect (VSD)
    • Holosystolic murmur at the left sternal border
    • Abx prophylaxis for Infective endocarditits 
    • Pharmacokinetics of inhaled and IV drugs not significantly altered. 
    • Avoid increases in SVR or decreased pulmonary vascular resistance
    • Volatiles (decreased SVR) and PPV (increased PVR) are well tolerated
    • RV infundibular hypertrophy may be exaggerated by increased contractility or hypovolemia and must be minimized: use volatiles
    • ECG: Right axis shift, RA&RV enlargement
  8. Patent Ductus Arteriosus (PDA)
    • Continuous systolic and diastolic murmur
    • Inhibition of PG synthesis with non-selective COX inhibitors (indomethacin)
    • Abx Prophylaxis Recommended 
    • Volatiles and PPV may improve systemic blood flow
    • ECG: LV hypertrophy
  9. Aortic Stenosis
    • Systolic Murmur over the 2nd Right Intercostal Space
    • ECG: LV hypertrophy, ST depression during exercise (pressures over 50mm Hg)
    • Syncope when pressures across the gradient exceed 50mm Hg
    • Increased myocardial oxygen requirements
  10. Pulmonic Stenosis
    • Supravavular often seen with other congenital cardiac abnormalities (ASD, VSD, PDA, tetralogy of Fallot)
    • Subvalvular often seen with VSD
    • Loud systolic ejection murmur at the left intercostal space
    • Anesthesia: avoid increased RV oxygen requirement (increases in HR and contractility), changes in pulmonary vascular resistance are NOT significant due to the fixed obstruction of the pulmonic valve, decreases in systemic BP should be promptly treated with sympathomimetics
  11. Coarctation of Aorta
    • Harsh systolic ejection murmur along the left sternal border and in the back
    • ECG: LV hypertrophy
    • Abx prophylaxis prior to dental or surgical procedures 
    • Anesthesia: Monitor above and below the coarctation. Maintain a MAP above 40mm Hg for blood flow to the kidneys and spinal cord
  12. Cyanotic Disease Characteristics
    • Right to left intracardiac shunt with associated decreased pulmonary flow and arterial hypoxemia
    • Erythrocytosis secondary to hypoxemia increases risk of thromboembolism
    • Secondary erythrocytosis may cause coagulation defects due to Vitamin K deficiencies and defective platelet aggregation
  13. Tetralogy of Fallot
    • 1. Large Single VSD, 2. Over-riding aorta (RV&LV), 3. RV outflow obstruction, and 4. RV hypertrophy
    • R-L Intra-cardiac shunting due to increased resistance to flow in the RV outflow
    • Ejection murmur heard along the left sternal border due to blood flow across the stenotic pulmonary valve
    • ECG: RV hypertrophy and right axis deviation
    • Oxygen desaturation is present even while breathing 100% O2 (PaCO2 usually <50mmHg)
    • PaCO2 and arterial pH are usually normal
    • Hypercyanotic Attacks: arterial hypoxemia, cyanosis, tachypnea, LOC, seizures, cerebrovascular accidents, and death. Tx with B-blockers (esmolol or propanaolol). Sympathomimetics w/ B-agonists are contraindicated since they may cause spasm of the infundibular cardiac muscle. 
    • Cerebrovascular Accident: Often Hb over 20g/dL
    • Cerebral Abscess: abrupt onset of headache, fever, and lethargy followed by emesis/ seizure
    • Infective endocarditis: Abx prophylaxis for these patients. 
    • R-L shunt can be increased by: 1.Decreased SVR, 2.Increased PVR, 3.Increased myocardial contractility (accentuates infundibular obstruction to RV blood ejection). 
    • Increased R-L shunting occurs due to volatiles, histamine, ganglionic blockade, alpha blockers and will increase arterial hypoxemia. PPV or PEEP may increased PVR
    • Preoperative: Avoid dehydration, B-blockers continued
    • Induction: Ketamine (1-2mg/kg IV), intubation w/ muscle relaxants, IV onset may be more rapid
    • Maintenance: N2O+Ketamine to preserve SVR. N2O may increase PulmVR, muscle paralysis w/ pancuronium, ventilation w/ limited PPV, maintain fluid levels.
  14. Eisenmenger's Syndrome
    • L-R shunt is reversed as a result of increased pulmonary vascular resistance to a level that equals or exceeds SVR (VSD > ASD)
    • The murmur associated with these defects disappears when Eisenmenger's syndrome develops 
    • ECG: RV hypertrophy
    • Anesthesia: maintain pre-op SVR and avoid sudden vasodilation. This may be achieved by an NE infusion. Minimize blood loss and prevent iatrogenic paradoxical embolization. Prophylactic phlebotomy w/ Hct above 65%. No anti platelet therapy. Opioids for analgesia. 
    • Pulmonary infarction or rupture of pulmonary a/v may lead to hemoptysis.
  15. Ebstein's Anomaly
    • Abnormality of the tricuspid valve: leaflets malformed or displaced downward in the RV
    • Commonly have an intra-atrial communication (patent foramen oval/ASD)
    • Systolic murmur on the left lower sternal border (regurgitation)
    • ECG: tall and broad P waves (resembles RBBB) and 1st degree AV block is common. 
    • Paroxysmal SVT and VT may occur
    • 20% have ventricular pre-excitation (WPW syndrome)
    • Anesthesia: Abx prophylaxis against IE and diuretics/digoxin for management of congestive heart failure. Delayed onset of pharmacological effects of IV drugs
  16. Tricuspid Atresia
    • RA blood to the LA
    • Arterial hypoxemia, small RV, and large LV
    • Anesthesia: Opioids and volatile anesthetics. Positive inotropic drugs (dopamine) and vasodilators (nitroprusside) are often required to optimize CO and maintain low pulmonary vascular resistance.
  17. Transposition of the great Vessels
    • Survival only possible with a communication between the 2 systems.
    • ECG: Right axis deviation and RV hypertrophy
    • Tx: Prostaglandin E infusion to maintain patency of ductus arteriosus. Diuretics and digoxin to tx CHF. 
    • Anesthesia: IV drugs have minimal direction and dosages may have to be decreased. Inhaled drug onset is delayed since inly small amounts reach the systemic circulation
    • Intubation: Ketamine + muscle relaxants (pancuronium vagolytic effect may be useful). 
    • Maintenance: Ketamine + opioids + benzos
    • High O2 levels (no N2O), no volatiles (negative inotrope), avoid histamine. 
    • Periopetative: avoid dehydration
  18. Trunctus arteriosus
    • Single trunk for the aorta and pulmonary a. 
    • ECG and ascultation: not predictable or diagnostic. 
    • Increased pulmonary flow: PEEP may decrease symptoms of CHF and it may be associated with myocardial ischemia. When myocardial ischemia does not respond to 1. IV phenylephrine  or fluids and 2.PEEP, temporary banding of the pulmonary a. may be needed.
  19. Partial Anomalous pulmonary venous return
    R or L Pulmonary vein returns blood to the right side of circulation.
  20. Increasing pulmonary blood flow w/ shunt
    • 1. Increased SVR (ketamine)
    • 2. Increased oxygen concentrations
  21. Increasing pulmonary vascular resistance and decreasing pulmonary blood flow
    • 1.Decrease volume of ventilation: Increased PaCO2 and decreased pH
    • 2.Positive end-expiratory pressure: increased lung volumes and further increased pulmonary vascular resistance.
  22. Mechanical Obstruction of the Trachea
    • Double Aortic Arch
    • Aberrant Left Pulmonary Artery 
    • Absent Pulmonic Valve
  23. Double Aortic Arch
    • Vascular ring producing pressure on the trachea and esophagus
    • S/S: Inspiratory stridor, difficulty mobilizing secretions, and dysphagia
  24. Aberrant Left Pulmonary Artery
    • Left pulmonary artery is absent and the arterial supply to the left lung is derived from a branch of the right pulmonary artery passing between the trachea and esophagus (vascular sling). 
    • S/S: stridor, wheezing, and arterial hypoxemia.
  25. Absent Pulmonic Valve
    • Dilation of the pulmonary artery resulting in compression of the trachea and left mainstream bronchus 
    • S/S: tracheal obstruction and occasional arterial hypoxemia and CHF.
Card Set:
Congenital Heart Diseases
2015-09-09 23:03:57
Anesthesia Heart Congenital

Anesthesia for Congenital Heart Disease
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