Congenital Heart Disease

Ductus venosus, oval foramen, ductus arteriosus

What are the 3 structures that are the most important in the transition from fetal cardiovascular system to the neonatal cardiovascular system?

placenta → umbilical vein → ductus venous → IVC → RA → oval foramen → LA → LV → Aorta (ductus arteriosus) → systemic blood flow

Describe the route of blood flood for the fetus starting at the placenta

directs blood flow from RA through oval foramen into LA

What is the purpose of the crista dividens?

10% of the blood passes into the RV → pulmonary vessels → lungs

How do the fetal lungs get their oxygen

sphincter in the ductus venosus constricts, increased pressure in LA closes the oval foramen, flow in the DA reverses due to increased pulmonary vascular resistance, DA closes due to oxygen and prostaglandins, umbilical arteries constrict

What changes occur at birth to transition the cardiovascular system as the neonate’s lung expand and begin to function

foramen ovale closes in 1 hour, DA in 2-3 days (decrease in prostaglandin), left side becomes dominant in 6-8 weeks

When do the changes after birth occur?

bicuspid aortic valve

What is the most common congenital heart disease?

prematurity (2-3x), family hx (3x), genetic syndromes, maternal factors, assisted reproductive technology (ART), in utero infection (TORCH)

Risk factors for congenital heart disease

cyanotic congenital heart disease

What includes lesions that allow circulation of deoxygenated blood in the systemic circulation via intracardiac/extracardiac shunting

left to right shunts

What is characterized by when oxygenated blood from the left heart/aorta shunts to the right side of the heart/pulmonary artery through communication between the 2 sides?

obstructive lesions

What occurs when blood flow is obstructed, causing a pressure gradient across the obstruction; pressure overload proximal may result in hypertrophy and HF

ASD (atrial septal defect)

What is characterized by an opening in the atrial septum allowing the flow of blood between the 2 atria

ostium secundum (most common), ostium primum, sinus venosus, coronary sinus

Types of atrial septal defect

other congenital heart defects, family hx, thalidomide/EtOH/smoking exposure in utero, maternal age 35 yr+

Risk factors for ASD

Holt-Oram, Ellis-van Creveld, VACTERL syndrome, Noonan syndrome

What conditions are often associated with ASDs

asymptomatic in childhood, found on routine physical

What are some characteristics of small ASDs?

right-sided HF, recurrent respiratory infections, failure to thrive

What are some characteristics of a large ASD

age 40 (atrial arrhythmias, exercise intolerance, dyspnea, fatigue)

In uncorrected ASDs, when do folks become symptomatic

Echo (transthoracic doppler, maybe transesophageal)

3 y/o male presents to the clinic for a routine physical. While conducting a physical you note the child is small for his age. You also note a fixed, widely split S2, RV heave, a palpable pulmonary pulse at LUSB, and an S4. What diagnostic test do you want to order?

systolic ejection murmur, low-pitched diastolic rumble, diastolic murmur, systolic murmur

Other than the fixed, wide S2 split, what other murmurs might you see with ASD?

RAD, RAE, RV conduction delay, Q in V1, RBBB, crochetage pattern

What might you see on EKG for ASD?

close follow up, most (75%) spontaneously close

What is the treatment plan for small secundum ASDs (less than 8mm)

8 mm+, primum and sinus venosus, coronary sinus

Which ASDs require surgical closure?

treat a fib like a fib (anticoag and cardioversion, rate control) treat HF like HF (diurectics, oxygen, digoxin, etc), pulmonary vasodilator therapy

1st line medication treatment for ASD

Recommended before dental or invasive procedures for 6 months after closure, and longer if there's residual shunting near a prosthetic device; Not given for unrepaired/isolated lesions

When do we give antibiotics (amoxicillin, azithromycin) for ASD

defect 8+mm in kids older than 2, any size in child 5+ with related symptoms

When is surgical closure of ASD indicated in children?

Right side heart enlargement, pulmonary systemic flow ratio 2:1 (if under 21 than 1.5:1), documented shortness of breath when standing (playpnea), orthodeoxia, paradoxical embolism

When is surgical closure via percutaneous transcatheter device of ASD indicated in adults?

irreversible severe pulmonary HTN (shunt is keeping them alive), no symptoms, small asymptomatic secundums

When are we NOT closing an ASD?

VSD (ventricular septal defect)

Second most common congenital heart defect that is characterized by a defect of the interventricular septum that allows communication of blood between the LV and RV (also occurs as a complication of AMI)

Eisenmenger Complex

What happens with a VSD over time as the RV gets swole and the flow reverses becoming a right to left shunt?

family hx, prematurity, prenatal exposure to weed, ibuprofen, organic solvents, febril illness

Risk factors of a congenital VSD

1st MI, HTN, 1st week after MI, anterior MI

Risk factors of a VSD as a result of a MI

membranous (most common 70%), muscular, AV canal type, supracristal

Types of congenital VSD

Cardiac Cath (gold standard), echo,

1 month old infant reports to the clinic for failure to thrive. On a physical exam you note tachypnea and tachycardia. You auscultate a harsh holosystolic murmur at LLSB with a thrill, diastolic rumble at apex, and an increased intensity of P2. What diagnostic test do you want to order

harsh holosystolic murmur at LLSB detected at 4-8 weeks

What might you hear with a small VSD?

holosystolic murmur throughout the precordium with diastolic rumble at apex with precordial bulge and hyperactivity (maybe no murmur), if CHF than tachycardia, tachypnea, hepatomegaly

What might you find with a large VSD?

cyanosis, clubbing, holosystolic murmur throughout the precordium with diastolic rumble at apex with precordial bulge and hyperactivity (maybe no murmur)

What might you find with a large VSD with Eisenmeneger’s complex

12 lead (LVH, LAE, RVH), CXR (haziness in the lungs, cardiomegaly), Echo, Color flow doppler (direction of flow, estimated RV pressure)

What other tests could you use for VSD?

Watch for overload (diuretics, minimize IV fluids), ACEI, digoxin, NG feeds, treat anemias

General game plan for VSD

Complex cyanotic heart disease is present, 6 months following repair, if any residual lesions are present post surgery

When are we giving VSD peeps antibiotics prophylactically?

furosemide, spironolactone, captopril, digoxin

Medication treatments of VSD (Peds)

digoxin, diuretics

Medication treatments of VSD (grown folks)

pulmonic-systemic flow is more than 2:1, poorly controlled pulmonary circulation, infant with persistent pulmonary HTN/failure to thrive

Indication for surgical repair of VSD

Corrected with patch or repair (RBBB are common afterward)

How are VSDs surgically repaired?

Small ones may close spontaneously, Large ones causing CHF/failure to thrive need repair

Prognosis of congenital VSD

worse with anterior MI, 80-90% mortality in 1st 2 weeks

Prognosis of post-MI VSD

PDA (patent ductus arteriosus)

Persistence of the fetal communication between the descending aorta and main pulmonary descending (normally closes due to O2 and decreased prostaglandins)

Echo with doppler (transesophageal), CXR, Cardiac MRI/CT, cardiac cath

2 y/o Patient presents to the clinic for a routine check up. The mother notes that the child becomes very tired and breaths heavily when playing. While auscultating the heart you note a washing machine murmur over the left scapula, a displaced apex beat, increased JVP. What diagnostic tests do you want to order?

Qp:Qs less than 1.5:1

What is classified as a small PDA

Qp:Qs 1.5-2.2:1

What is classified as a moderate PDA

Qp:Qs more than 2.2:1

What is classified as a severe PDA

LAE, LVH, if pulmonary HTN has occurred RAD, RVH, RAE

What might you see on EKG with someone with a PDA

NSAIDs (indomethacin, ibuprofen)

Treatment of PDAs in premature infants

signs of volume overload, pulmonary HTN, audible murmur

When should PDAs be closed (DO NOT CLOSE IF EISENMEYER IS THERE)

Coarctation of the aorta (CoA)

A constricting lesion at any point along the aorta (usually congenital) that causes outflow obstruction and HYN proximal to the site with LV pressure overload, myocardial hypertrophy, and possible HF

male, turners syndrome family Hx

Risk Factors for CoA

biscuspid aortic valve, PDA, VSD, mitral valve abnormalities, turners syndrome, transposition of the great vessels, aneurysm of circle of willis

Conditions commonly associated with CoA

Echo, CXR (rib notching), EKG (RVH, LVH), BNP

3 y/o Patient presents to the ER for syncope. Mother reports that he hasn’t eating well and gets tired very easily. On a physical you note a brachial-femoral pulse delay, upper extremity HTN, prominent neck pulsations, and corkscrew tortuosity of retinal arteries. What diagnostic tests do you want to order?

Alprostadil (prostaglandins) continuous IV infusion, short term esmolol/nitroprusside, longterm beta blocker and ACEI, manage HF

What is the medication game plan for ductal dependent CoA?

surgical correction, balloon angioplasty

How is CoA repaired in neonates, infants, and small children? In older children and adolescents?

VSD, pulmonary valve stenosis, overriding aorta, RV hypertrophy

4 thangs in tetralogy of Fallot

Echo, CXR (boot heart), cardiac cath, cardiac MRI, CBC (polycythemia), ABG, EKG (RAD, RVH, RAE), exercise testing

A 3 y/o male presents to the clinic for failure to thrive. The mother notes that he has extreme dyspnea on exertion and gets into a squatting position. On a physical exam you note cyanosis of the nail beds, digital clubbing, a palpable RV impulse, systolic thrill along left sternal border, crescendo/decrescendo systolic murmur LUSB with posterior radiation. What diagnostic tests do you want?

tet spells

paroxysmal episodes of worsening cyanosis with rapid and deep breathing that occurs when there is transient increase RV outflow tract obstruction

persistent foramen ovale, ASD, pulmonary artery anomalies, Right aortic arch, left superior vena cava to coronary sinus, additional VSDs, coronary artery anomalies, aortic regurg

ToF is associated with what other cardiac defects?

O2, prostaglandins, phenylephrine (increase systemic vascular resistance), morphine (decrease pulmonary resistance), Beta blockers (decrease RV outflow tract)

Treatment plan for ToF

close VSD with patch, relieve RV outflow obstruction

Complete surgical repair of ToF

increase pulmonary blood flow, improve O2 sats to allow for pulmonary artery growth

Palliative surgery of ToF