3 - Congenital Heart Defects

acyanotic congenital heart defects

type of congenital heart defect

• left-to-right shunting

• increased pulmonary blood flow

• risk of CHF and respiratory issues

• signs: pink skin, HTN, HF

cyanotic congenital heart defects

type of congenital heart defect

• right-to-left shunting

• systemic desaturation

• signs: bluish skin, low S_pO₂

atrial septal defect (ASD)

opening between atria

• left-to-right shunt

atrial septal defect (ASD)

• manifestations

• asymptomatic

• frequent respiratory infections

• poor weight gain

• murmur: fixed split S2

atrial septal defect (ASD)

• RT role

• monitor for CHF (tachypnea, hepatomegaly)

• avoid excess O₂

• support during illness/surgery

atrial septal defect (ASD)

• types

• secundum

  • middle of atrial septum

• primum

  • low part of atrial septum

• sinus venosus

  • upper part of septum

• coronary sinus

  • missing part of septum between coronary sinus and left atrium

ventricular septal defect (VSD)

opening between ventricles

• left-to-right shunt

• pulmonary overload

ventricular septal defect (VSD)

• types

• perimembranous

• muscular

• outlet

ventricular septal defect (VSD)

• manifestations

• tachypnea

• poor feeding

• failure to thrive (FFT)

• systolic murmur

ventricular septal defect (VSD)

• RT role

• support O₂ and feeding

• monitor for decompensation

• consider MV for CHF

• wean O₂ cautiously

ventricular septal defect (VSD)

• diagnosis

• murmur

• echocardiogram

• cardiac catheterization

• pulse ox

• CXR

ventricular septal defect (VSD)

• treatment

medications

• digoxin

• diuretics

• beta blockers

surgery

• patch closure

• pulmonary artery bands

patent ductus arteriosus (PDA)

ductus arteriosus does not close by 72 hours postnatal

• left-to-right shunt

• risks: preterm, RDS, increased F_iO₂

patent ductus arteriosus (PDA)

• manifestations

• murmur

• bounding pulse

• increased WOB

patent ductus arteriosus (PDA)

• RT role

• use lowest F_iO₂ needed to reduce patency

• CPAP/NIV for WOB

• monitor for apnea and edema

• coordinate with cardiology for meds and surgical closure

patent ductus arteriosus (PDA)

• treatment

non-surgical

• NSAIDs

• low dose acetaminophen

• cardiac catheterization with coil

surgical

• PDA ligation

coarctation of aorta (COA)

narrowing of aorta near ductus arteriosus causing hypoperfused lower limbs

coarctation of aorta (COA)

• manifestations

• weak femoral pulses

• low S_pO₂ in legs

coarctation of aorta (COA)

• diagnosis

• pre- and post-ductal saturation difference

• echocardiogram

coarctation of aorta (COA)

• RT role

• monitor perfusion

• avoid hyperoxia

• administer prostaglandin E1 (PGE1) to maintain ductal patency

• prepare for intubation and CV support

tetralogy of Fallot (TOF)

4 congenital heart defects occurring simultaneously:

• VSD

• pulmonary stenosis

• overriding aorta

• right ventricular hypertrophy

right-to-left shunt, cyanosis

tetralogy of Fallot (TOF)

• manifestations

• cyanosis

• “tet spells” (sudden cyanosis while crying or feeding)

• digital clubbing

• murmur

• CXR: “boot shape”

tetralogy of Fallot (TOF)

• RT role

• O₂ (may be ineffective)

• knee-to-chest position (decreases right-to-left shunt)

• morphine and beta blockers for tet spells

• pre-op: support feeding, minimize stress, monitor S_pO₂

transposition of great arteries (TGA)

aorta and pulmonary artery switch places

• needs ASD, VSD, or PDA for survival

transposition of great arteries (TGA)

• manifestations

• early severe cyanosis

• poor O₂ therapy response

• CXR: “egg on a string”

transposition of great arteries (TGA)

• diagnosis

echocardiogram

transposition of great arteries (TGA)

• RT role

• administer PGE1 to maintain PDA

• prep for balloon septostomy

• monitor for acidosis and apnea

• avoid hyperoxia if mixing is duct-dependent

hypoplastic left heart syndrome (HLHS)

underdeveloped left ventricle, aorta, and mitral valve

• PDA needed for survival

hypoplastic left heart syndrome (HLHS)

• manifestations

• cyanosis

• weak pulses

• acidosis

• shock

• rapid decline within first 48 hours postnatal

hypoplastic left heart syndrome (HLHS)

• RT role

• administer PGE1 infusion

• avoid hyperventilation and hyperoxia

• allow permissive hypercapnia and hypoxemia (S_pO₂ 75-85%)

• coordinate with surgery (Norwood repair)

total anomalous pulmonary venous return (TAPVR)

pulmonary veins drain into right heart

• ASD or patent foramen ovale (PFO) needed for survival

total anomalous pulmonary venous return (TAPVR)

• manifestations

• cyanosis

• distress

• poor feeding

• CXR: “snowman sign” (supracardiac)

total anomalous pulmonary venous return (TAPVR)

• diagnosis

echocardiogram

total anomalous pulmonary venous return (TAPVR)

• types

• cardiac

  • pulmonary veins drain into right atrium or coronary sinus

• supracardiac

  • pulmonary veins drain into right atrium through superior vena cava

• infradiaphragmatic

  • pulmonary veins drain into right atrium through inferior vena cava and hepatic veins

• mixed

  • pulmonary veins split and drain into multiple areas

total anomalous pulmonary venous return (TAPVR)

• RT role

• use O₂ cautiously (could lower PVR)

• prep for surgery

• monitor for deterioration and poor response