Congenital Heart Disease Cram the PANCE

Cyanotic conditions

Nonoxygenated blood is pumped out to heart when blood shunts from the right side of the heart to the left

Noncyanotic conditions

Blood shunts from left to right side

How to know cyanotic vs noncyanotic

Cyanotic all start with "T" for Turquoise:

Truncus arteriosius

Transposition of great vessels

Tetralogy of Fallot

Tricuspid Atresia

Total anomalous pulmonary venous return

IF IT DOESNT START WITH T ITS NOT CYANOTIC

Atrial septal defect

Noncyanotic (L to R shunt)

Abnormal opening between right and left atrium

Most common type is Secundum ASD

Less common: Ostium Primum, Coronary Sinus ASD

Clinical manifestations: nonspecific... Know physical exam

Most ASDs are small and asymptomatic until adulthood

ASD physical exam

Systolic ejection murmur (crescendo-decrescendo)

best heard at pulmonic area (upper left sternal border)

all patients eagerly take medicine

Wide fixed split S2 (pathognomonic for ASD)

ASD dx and tx

Echocardiography always!!!

Tx: surgery is definitive

Observation if small (<5mm) or asymptomatic (Most ASD close spontaneously within first year)

Ventricular Septal Defect

Defect in interventricular septum that leads to left to right shunting between ventricles

MC: Perimembranous

VSD Clinical manifestatoins

may be asymptomatic

moderate size: frequenty respiratory infection, fatigue

Large nonrestricive VSD: reversal of shunt (eisenmeigers) cyanosis!

Physical exam: High pitched holosystolic murmur at lower left sternal border

VSD dx and tx

Echo

ECG may show LVH

Tx: small - observation

Larger: surgery!)

VSD

Very Sharp Dagger "CHOPS" a hole in your ventricular septum

Common (most common congenital heart disease of childhood)

Holosystolic Murmur/High Pitched Murmur

Observation (in small)

Perimembranous (MC type)

Surgery (in moderate/large VSD)

tetralogy or fallot

Cyanotic condition

Four defects present:

1. Right ventricular outflow obstruction (or pulmonary stenosis)

2. Right ventricular hypertrophy (caused by outflow obstruction)

3. overriding aorta

4. Ventricular septal defect

Tetralogy of fallot risk factors

Down syndrome

Digeorge syndrome

alagille syndrome

Fallot clinical presentation

Cyanosis!

Tachypnea

Tet spells (transient occlusion of right ventricular outflow leading to severe episodes of cyanosis)

Pushing legs toward chest temporarily relieves symptoms

Physical exams: Harsh systolic ejection murmur best heard at left sternal border

Dx:

Echo

ECG

Boot-Shaped heart

Fallot tx

Surgical repair definitive

prostaglandins to maintain patency of ductus arteriosus to improve circulation (keep it open until they're ready to have surgery)

Mnemonic for Fallot

Patients with Tetralolgy of Fallot CRAVE oxygen

Cyanosis

RVH

Alprostadil (1st line prostaglandin)/Aorta overriding

VSD

Echocaridogram

Coarctation of the Aorta

Narrowing of the descending aorta

associated w Bicuspid aortic valve (70% of pts)

Turners syndrome

Clinical manifestations:

neonates: asymptomatic, poor feeding

Children: angina, cold extremities, claudication with physical exertion

Adults: Hypertension

NOT SPECIFIC

Coarctation of Aorta physical Exam findings

Hypertension in the upper extremities, relative hypotension in lower extremities with delayed/diminished pulse

Systolic murmur

Dx with echo

CTA/MRA (for planning out surgical resection)

Chest xray: posterior rib notching, fugure 3 sign

Coarctation tx

Surgical intervention

Prostaglandin preoperatively

Coarctation Need to know

Associated w bicuspid aortic valve and turners syndrome

Upper extremity hypertension, lower extremity hypotension

Rib notching, figure 3 sign on chest xray

Patent ductus arteriosus

condition in which the ductus arteriosus remains patent and does not clsoe after birth

Prematurrity

2:1 female predominance

Higher altitude

Congenital Rubella

Patho: too much prostaglandin

PDA clinical manifestations

Poor feeding

Frequent lower respiratory infections

weight loss

Cyanosis if eisenmenger develops

Physical exam:

Continuous machine-like murmur

Bounding peripheral pulses

Wide pulse pressure

Dx with echo

Tx for PDA

Definitive surgical treatment

1st line is NSAIDs (ibuprofen, indomethacin)

for 48 hours then redo echo

Nonresponsive to NSAIDs, surgical correction is next