Congenital heart disease: Difference between revisions
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| Cyanosis ||[[Transposition]] of the great arteries, [[TOF]], tricuspid atresia, [[truncus arteriosus]], total anomalous pulmonary venous return||TOF, Eisenmenger complex | | Cyanosis ||[[Transposition]] of the great arteries, [[TOF]], tricuspid atresia, [[truncus arteriosus]], total anomalous pulmonary venous return||TOF, Eisenmenger complex | ||
|- | |- | ||
| [[Cardiovascular shock]] ||Critical [[aortic stenosis]], [[coarctation of the aorta]], HLHS ||Coarctation of the aorta (infants) | | [[Cardiovascular shock]] ||Critical [[aortic stenosis]], [[coarctation of the aorta]], [[Hypoplastic left heart syndrome|HLHS]] ||Coarctation of the aorta (infants) | ||
|- | |- | ||
| [[Congestive heart failure]] ||Rare: PDA, HLHS ||PDA, VSD, [[ASD]], atrioventricular canal | | [[Congestive heart failure]] ||Rare: PDA, HLHS ||[[PDA]], [[VSD]], [[ASD]], atrioventricular canal | ||
|- | |- | ||
| [[Murmur]] ||PDA, valvular defects (AS, PS) ||VSD, [[ASD]], PDA, outflow obstructions, valvular defects (AS, PS) | | [[Murmur]] ||[[PDA]], valvular defects (AS, PS) ||[[VSD]], [[ASD]], [[PDA]], outflow obstructions, valvular defects (AS, PS) | ||
|- | |- | ||
| [[Syncope]] ||— ||AS, PS, Eisenmenger complex | | [[Syncope]] ||— ||AS, PS, Eisenmenger complex | ||
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==Evaluation== | ==Evaluation== | ||
[[File:CHD.png|thumb|Algorithm for the Evaluation and Management of Suspected Congenital Heart Disease in Neonates]] | |||
===Cyanotic=== | ===Cyanotic=== | ||
{| class="wikitable" | {| class="wikitable" | ||
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| align="center" style="background:#f0f0f0;"|'''ECG''' | | align="center" style="background:#f0f0f0;"|'''ECG''' | ||
|- | |- | ||
| Tetralogy of Fallot||Boot-shaped heart, normal-sized heart, decreased pulmonary vascular markings||Right axis deviation, right ventricular hypertrophy | | [[Tetralogy of Fallot]]||Boot-shaped heart, normal-sized heart, decreased pulmonary vascular markings||Right axis deviation, right ventricular hypertrophy | ||
|- | |- | ||
| Transposition of the great arteries||Egg-shaped heart, narrow mediastinum, increased pulmonary vascular marking||Right axis deviation, right ventricular hypertrophy | | [[Transposition of the great arteries]]||Egg-shaped heart, narrow mediastinum, increased pulmonary vascular marking||Right axis deviation, right ventricular hypertrophy | ||
|- | |- | ||
| Total anomalous pulmonary venous return||Snowman sign, significant cardiomegaly, increased pulmonary vascular markings||Right axis deviation, right ventricular hypertrophy, right atrial enlargement | | [[Total anomalous pulmonary venous return]]||Snowman sign, significant cardiomegaly, increased pulmonary vascular markings||Right axis deviation, right ventricular hypertrophy, right atrial enlargement | ||
|- | |- | ||
| Tricuspid atresia||Heart of normal to slightly increased size, decreased pulmonary vascular markings||Superior QRS axis with right atrial hypertrophy, left atrial hypertrophy, left ventricular hypertrophy | | [[Tricuspid atresia]]||Heart of normal to slightly increased size, decreased pulmonary vascular markings||Superior QRS axis with right atrial hypertrophy, left atrial hypertrophy, left ventricular hypertrophy | ||
|- | |- | ||
| Truncus arteriosus||Cardiomegaly, increased pulmonary vascular markings||Biventricular hypertrophy | | [[Truncus arteriosus]]||Cardiomegaly, increased pulmonary vascular markings||Biventricular hypertrophy | ||
|} | |} | ||
*Can use the "5 T's' mnemonic:<ref>Waldman JD, Wernly JA. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am. 1999;46(2):385-404. doi:10.1016/s0031-3955(05)70125-5</ref> | |||
**1 vessel: Truncus arteriosus | |||
**2 vessels switched: Transposition of the great vessels | |||
**3=TRIcuspid: Tricuspid atresia | |||
**4 defects: Tetralogy of Fallot | |||
**5 letters: Total anomalous pulmonary vascular return (TAPVR) | |||
===Acyanotic (duct-dependent)=== | ===Acyanotic (duct-dependent)=== | ||
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| ||Rib notching and collateral vascularity (child)||LVH (child) | | ||Rib notching and collateral vascularity (child)||LVH (child) | ||
|- | |- | ||
| Hypoplastic left heart syndrome||Cardiomegaly||Right atrial enlargement, RVH, peaked P waves | | [[Hypoplastic left heart syndrome]]||Cardiomegaly||Right atrial enlargement, RVH, peaked P waves | ||
|- | |- | ||
| Aortic stenosis||Cardiomegaly||LVH in severe cases | | Aortic stenosis||Cardiomegaly||LVH in severe cases | ||
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| align="center" style="background:#f0f0f0;"|'''Right axis deviation, [[RVH]], [[RBBB]]''' | | align="center" style="background:#f0f0f0;"|'''Right axis deviation, [[RVH]], [[RBBB]]''' | ||
|- | |- | ||
| VSD||Cardiomegaly with increased vascular markings||[[LAH]], [[LVH]], ([[RVH]] with larger VSDs) | | [[VSD]]||Cardiomegaly with increased vascular markings||[[Left atrial enlargement|LAH]], [[LVH]], ([[RVH]] with larger VSDs) | ||
|- | |- | ||
| PDA||Cardiomegaly with increased vascular markings||[[LVH]], [[RVH]] with larger PDAs | | [[PDA]]||Cardiomegaly with increased vascular markings||[[LVH]], [[RVH]] with larger PDAs | ||
|- | |- | ||
| Endocardial cushion defect||Cardiomegaly with increased vascular markings||Superior QRS axis with [[RVH]], [[RBBB]], [[LVH]], prolonged PR interval | | Endocardial cushion defect||Cardiomegaly with increased vascular markings||Superior QRS axis with [[RVH]], [[RBBB]], [[LVH]], prolonged PR interval | ||
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==Management== | ==Management== | ||
===Shock (duct-dependent lesion)=== | ===[[Pediatric shock|Shock]] (duct-dependent lesion)=== | ||
*[[PGE1]] 0.1mcg/kg/min IV/IO | *[[PGE1]] 0.1mcg/kg/min IV/IO | ||
**Side Effects: | **Side Effects: | ||
***Apnea (intubate) | ***Apnea ([[intubation|intubate]]) | ||
****Hypotension | ****[[Hypotension]] | ||
****Bradycardia | ****[[Bradycardia]] | ||
****Flushing | ****Flushing | ||
*[[NS]] 10cc/kg | *[[NS]] 10cc/kg | ||
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*O2 | *O2 | ||
*[[Morphine]] or [[NS]] to increase preload | *[[Morphine]] or [[NS]] to increase preload | ||
* | *[[Sodium bicarbonate]] 2mEq/kg IV bolus (promotes vasodilation) | ||
*[[Propranolol]] 0.2mg/kg IV (relieves infundibular spasm) | *[[Propranolol]] 0.2mg/kg IV (relieves infundibular spasm) | ||
*[[Phenylephrine]] 2-10mcg/kg/min to increased SVR | *[[Phenylephrine]] 2-10mcg/kg/min to increased SVR | ||
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*[[Dopamine]] 5-10mcg/kg/min | *[[Dopamine]] 5-10mcg/kg/min | ||
*[[Dobutamine]] 5-10mcg/kg/min | *[[Dobutamine]] 5-10mcg/kg/min | ||
===Thrombolysis for Surgical Shunt Obstruction=== | |||
*Blalock-Taussig shunt should maintain flow murmur | |||
*Loss of flow murmur alongside profound hypoxia relative to baseline saturations should prompt consideration for shunt obstruction | |||
*Definitive treatment is surgical, but systemic recombinant tPA may be considered as salvage intervention when other options are not readily available<ref>Diaz F et al. Systemic thrombolysis with recombinant tissue plasminogen activator for acute life-threatening Blalock-Taussig shunt obstruction. Indian J Crit Care Med. 2016 Jul; 20(7): 425–427.</ref> | |||
**Heparin bolus 50-100 u/kg | |||
**Notify cardiology, CT surgeon, ECMO | |||
**0.01 mg/kg bolus r-tPA, then 0.03 - 0.06 mg/kg/hr | |||
==Disposition== | |||
==See Also== | ==See Also== |
Latest revision as of 18:45, 10 December 2020
Background
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[1]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Clinical Features
Clinical Presentation | Causative Conditions in Neonates | Causative Conditions in Infants and Children |
Cyanosis | Transposition of the great arteries, TOF, tricuspid atresia, truncus arteriosus, total anomalous pulmonary venous return | TOF, Eisenmenger complex |
Cardiovascular shock | Critical aortic stenosis, coarctation of the aorta, HLHS | Coarctation of the aorta (infants) |
Congestive heart failure | Rare: PDA, HLHS | PDA, VSD, ASD, atrioventricular canal |
Murmur | PDA, valvular defects (AS, PS) | VSD, ASD, PDA, outflow obstructions, valvular defects (AS, PS) |
Syncope | — | AS, PS, Eisenmenger complex |
Hypertension | — | Coarctation of the aorta |
Arrhythmias | — | ASD, Ebstein anomaly, postsurgical complication after repair of congenital heart defect |
Differential Diagnosis
Sick Neonate
THE MISFITS [2]
- Trauma
- Heart
- Congenital heart disease
- Hypovolemia
- Endocrine
- Metabolic
- Sodium
- Calcium
- Glucose
- Inborn errors of metabolism
- Seizure
- Formula / feeding problems
- Intestinal Disasters
- Toxin
- Sepsis
Evaluation
Cyanotic
Cardiac Lesion | Chest Radiograph | ECG |
Tetralogy of Fallot | Boot-shaped heart, normal-sized heart, decreased pulmonary vascular markings | Right axis deviation, right ventricular hypertrophy |
Transposition of the great arteries | Egg-shaped heart, narrow mediastinum, increased pulmonary vascular marking | Right axis deviation, right ventricular hypertrophy |
Total anomalous pulmonary venous return | Snowman sign, significant cardiomegaly, increased pulmonary vascular markings | Right axis deviation, right ventricular hypertrophy, right atrial enlargement |
Tricuspid atresia | Heart of normal to slightly increased size, decreased pulmonary vascular markings | Superior QRS axis with right atrial hypertrophy, left atrial hypertrophy, left ventricular hypertrophy |
Truncus arteriosus | Cardiomegaly, increased pulmonary vascular markings | Biventricular hypertrophy |
- Can use the "5 T's' mnemonic:[3]
- 1 vessel: Truncus arteriosus
- 2 vessels switched: Transposition of the great vessels
- 3=TRIcuspid: Tricuspid atresia
- 4 defects: Tetralogy of Fallot
- 5 letters: Total anomalous pulmonary vascular return (TAPVR)
Acyanotic (duct-dependent)
Coarctation of the aorta | Cardiomegaly with pulmonary edema (neonate) | RVH, right bundle-branch block (neonate) |
Rib notching and collateral vascularity (child) | LVH (child) | |
Hypoplastic left heart syndrome | Cardiomegaly | Right atrial enlargement, RVH, peaked P waves |
Aortic stenosis | Cardiomegaly | LVH in severe cases |
Acyanotic non-duct dependent (i.e. CHF)
Atrial septal defect | Cardiomegaly with increased vascular markings | Right axis deviation, RVH, RBBB |
VSD | Cardiomegaly with increased vascular markings | LAH, LVH, (RVH with larger VSDs) |
PDA | Cardiomegaly with increased vascular markings | LVH, RVH with larger PDAs |
Endocardial cushion defect | Cardiomegaly with increased vascular markings | Superior QRS axis with RVH, RBBB, LVH, prolonged PR interval |
Anomalous origin of the left coronary artery | Cardiomegaly | Abnormally deep and wide Q waves with precordial ST segment changes |
Management
Shock (duct-dependent lesion)
- PGE1 0.1mcg/kg/min IV/IO
- Side Effects:
- Apnea (intubate)
- Hypotension
- Bradycardia
- Flushing
- Apnea (intubate)
- Side Effects:
- NS 10cc/kg
- Dobutamine
Tet Spell
- Knee chest position
- Increased venous return to heart, increased SVR (decreased R>L shunting)
- O2
- Morphine or NS to increase preload
- Sodium bicarbonate 2mEq/kg IV bolus (promotes vasodilation)
- Propranolol 0.2mg/kg IV (relieves infundibular spasm)
- Phenylephrine 2-10mcg/kg/min to increased SVR
CHF
- O2 (give only if SpO2 <95%)
- Furosemide 1-2mg/kg IV
- Dopamine 5-10mcg/kg/min
- Dobutamine 5-10mcg/kg/min
Thrombolysis for Surgical Shunt Obstruction
- Blalock-Taussig shunt should maintain flow murmur
- Loss of flow murmur alongside profound hypoxia relative to baseline saturations should prompt consideration for shunt obstruction
- Definitive treatment is surgical, but systemic recombinant tPA may be considered as salvage intervention when other options are not readily available[4]
- Heparin bolus 50-100 u/kg
- Notify cardiology, CT surgeon, ECMO
- 0.01 mg/kg bolus r-tPA, then 0.03 - 0.06 mg/kg/hr
Disposition
See Also
- Innocent Murmurs (Peds)
- Congenital Heart Defects - Surgical Procedures
- www.pted.org - list of congenital heart diseases, overviews, management strategies
References
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
- ↑ Brousseau T, Sharieff GQ. Newborn emergencies: the first 30 days of life. Pediatr Clin North Am. 2006 Feb;53(1):69-84, vi.
- ↑ Waldman JD, Wernly JA. Cyanotic congenital heart disease with decreased pulmonary blood flow in children. Pediatr Clin North Am. 1999;46(2):385-404. doi:10.1016/s0031-3955(05)70125-5
- ↑ Diaz F et al. Systemic thrombolysis with recombinant tissue plasminogen activator for acute life-threatening Blalock-Taussig shunt obstruction. Indian J Crit Care Med. 2016 Jul; 20(7): 425–427.