Ver el código de «Congenital heart disease»

==Background==
{{Congenital heart disease DDX}}

==Clinical Features==
{| class="wikitable" 
| align="center" style="background:#f0f0f0;"|'''Clinical Presentation'''
| align="center" style="background:#f0f0f0;"|'''Causative Conditions in Neonates'''
| align="center" style="background:#f0f0f0;"|'''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]], [[Hypoplastic left heart syndrome|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 DDX}}

==Evaluation==
[[File:CHD.png|thumb|Algorithm for the Evaluation and Management of Suspected Congenital Heart Disease in Neonates]]
===Cyanotic===
{| class="wikitable" 
| align="center" style="background:#f0f0f0;"|'''Cardiac Lesion'''
| align="center" style="background:#f0f0f0;"|'''Chest Radiograph'''
| 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
|-
| [[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:<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)===
{| class="wikitable" 
| align="center" style="background:#f0f0f0;"|'''Coarctation of the aorta'''
| align="center" style="background:#f0f0f0;"|'''Cardiomegaly with pulmonary edema (neonate)'''
| align="center" style="background:#f0f0f0;"|'''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)===
{| class="wikitable" 
| align="center" style="background:#f0f0f0;"|'''[[Atrial septal defect]]'''
| align="center" style="background:#f0f0f0;"|'''Cardiomegaly with increased vascular markings'''
| align="center" style="background:#f0f0f0;"|'''Right axis deviation, [[RVH]], [[RBBB]]'''
|-
| [[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
|-
| 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==
===[[Pediatric shock|Shock]] (duct-dependent lesion)===
*[[PGE1]] 0.1mcg/kg/min IV/IO 
**Side Effects: 
***Apnea ([[intubation|intubate]]) 
****[[Hypotension]]
****[[Bradycardia]]
****Flushing 
*[[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<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==
*[[Innocent Murmurs (Peds)]]
*[[Congenital Heart Defects - Surgical Procedures]]
*[http://pted.org/?id=list www.pted.org - list of congenital heart diseases, overviews, management strategies]

==References==
<references/>

[[Category:Pediatrics]] 
[[Category:Cardiology]]