Congenital Heart Disease in Children

Volume overload and pressure overload are subsets of

acyanotic congenital heart disease

TOF Tri. atresia

Decreased PBF

Tx of TOF?

Severe: urgent need surgery asap w prostaglandins to keep the PDA open LEss severe 4-6 months you can wait still need surgery Surgery is removing obstructive bundles and patch

least common septal defect, upper seputm, abnormal return R pulmonary veins

Sinus venosus ASD

ASD (atrial septal defect) more common with

boys

PSD

girls

Pressure overload Can be from transverse arch to iliac bifurcation 98% are below the origin of left subclavian artery M:F 2:1 Turner syndrome Bicuspid aortic valve 70%

Coarctation of the aorta

Septum grows towards the cushion and divides the atria, failure leads to ASD Desaturated blood mixes in the RA w/ fully saturated blood (L -> R) Increased oxy sat in RA

ASD pathophysiology

Predominant physiological load Most common is volume load d/t L to right shunt Pressure load is also common d/t ventricular outflow obstruction What type of lesion is this?

Acyanotic congenital lesions

Critical AS:LV failure Low output and pulmonary edema Sudden death can occur in severe LV outflow obstruction (murmur is heard in these pt) Mild/moderate = early systolic ejection click, no changes w/ respiration Severe is louder longer w thrill

Aortic stenosis

Acyanotic pressure overload 5% of CHD Bicuspid aortic valve in 1.5% pt M:F 3:1 Most common is valvular stenosis of the leaflets

Aortic stenosis Others: Subvalvar AS: discrete fibromuscular shelf Shone syndrome Supravalvar AS: least common Williams syndrome to right

The type of defect that causes sudden cardiac death in athletes

Aortic valve stenosis

Acyanotic w/ volume overload D/t failure of septal growth Includes isolated secundum, sinus venosus and PFO

Atrial septal defect

Atrioventricular Septal Defect: it exists Volume overload AKA ostium primum and AV canal endocardial cushion defect Failure of the septum to fuse with endocardial cushon Common in down syndrome trisomy 21

Atrioventricular Septal Defect

Tx of aortic valve stenosis

Balloon if bad Sub AS surgery High long-term risk of restenosis

trisomy 13, 18, 21 and turner syndrome 22q11 (DiGeorge) Genetic cardiomyopathies Heritable arrhythmias

CHD

HTN Pulse and BP different in arms and legs Legs 20/10 mm higher than normal Neonates may have acidosis or heart failure Cardiomegaly and pulmonary congestion are possible

COA Tx: close it but need PEG1 to maintain systemic flow

Tricuspid atresia, tetralogy of fallot and a single ventricle are examples of what

Cyanotic Congenital Lesions:Decreased Pulmonary Flow

In cyanosis from abnormal blood mixing the most common forms are HLHS, TAPVR, truncus arteriosus TAPVR = total anomalous pulmonary venous return

Cyanotic Congenital Lesions: Increased Pulmonary Flow

No obstruction of pulmonary blood flow Cyanosis d/t VA connections or mixing Most common form is TGA (transposition of the great arteries) in the subset of connections

Cyanotic Congenital Lesions: Increased Pulmonary Flow

Subset of diseases defined by systemic venous blood and oxygenated pulmonary venous blood mixing completely so that O2 sat is the same in PA and aorta

Cyanotic Congenital Lesions: Increased Pulmonary Flow

Cyanotic, increased PBF 5% of all CHD Normal venous and A-V connections but... Aorta arises from RV, pulmonary artery from LV Blue blood ejected out aorta to body Red blood re-directed back to lungs Systemic and pulmonary circulations in "parallel" Survival by mixing at PFO, PDA 50% also have VSD (more mixing)

D-Transposition of the great Arteries 5% HD, fairly common Aorta arises from RV and the pulmonary artery is from left ventricle and messes it all up Desat blood goes out the aorta to the body again and the oxygenated pulmonary blood goes back to the lungs Become parallel circuits Survival need mixing at ovale and ductus arteriosis otherwise they get no oxygenated blood Transposition and VSD which is good because you need the mixing at some level Only way this blood can get oxygen is mixing at PFO or PDA

Hypoxemia when the ductus closes Cyanosis, tachypnea, hemodynamic compromise Low arterial PO2 even with 100% oxygen Anomalous coronaries balloon atrial septostomy

DTGA Bedside procedure, slide wire across PFO and when the tip is in left atrium shoot air and it pumps a balloon then yank the wire through PFO and it rips open and stats go right up

PS AS Coarctation

Pressure overload

What do you see on chest x-ray with DTGA

Egg on a string

Cyanotic, increased PBF Underdeveloped left heart; stenosis/atresia of MV, AV RV maintains pulm + systemic circulations by PDA Pulm venous blood thru PFO/ASD Total mixing in right heart Usually inadequate maintenance of systemic circ Diagnosed prenatally or in first few days as PDA closes 5-15% associated with syndromes: Turner, trisomies, etc. May also have small aorta or coarctation

Hypoplastic Left Heart Syndrome

Can pulmonary stenosis, aortic stenosis and coarction be overcome?

If not severe then yes w/ hypertrophy If severe then no

TGA HLHS TAPVR Truncus

Increased PBF

Tx of pulmonary valve stenosis

Mod or severe balloon Often great results though the balloon may cause leakage Mild: just watch it

What are the most common causes of increased volume load in acyanotic congenital lesions?

Most common are left-to-right shunt: ASD, VSD, AVSD, PDA Atrial septal defect Venticular septal defect Aortic valve stenosis Patent ductus arteriosis

What are the most common causes of increased pressure load

Obstruction of normal flow PS, AS and coarctation Pulmonary stenosis Aortic stenosis

Cyanotic CHD that decreases pulmonary flow requires 2 things:

Obstruction to PBF Shunt from right to left (venous to systemic) PBF = TV, RV, PV?

What is the premature benign murmur in babies called?

PDA Patient ductus arteriosis

Bounding pulses Wide pulse pressure from runoff pressure from aorta to PA during diastole Distinctive continuous washing machine murmur

PDA: Manifestations, Diagnosis

Acyanotic volume overload This structure shunts blood right to left shunt from pulmonary arteries to the aorta Remains patent and forms a left to right shunt Common in premature babies

Patent Ductus Arteriosus

Deformed valve cusps cause incomplete opening Associated with Noonan, LEOPARD, Allagille syndromes Failure of development of three leaflets Or insufficient tissue resorption

Pulmonary Valve Stenosis Isolated valvar PS accounts for 7-10% of CHD May also have subvalvar or supravalvar PS

Severe causes RV failure, edema, exercise intolerance Mild causes pulmonary ejection click, RVH on ECG Moderate causes a later click, S2 and murmur peak Severe causes: Cyanosis, cardiomegaly, loud/long murmur RV systolic dysfunction

Pulmonary valve stenosis

Cyanotic CHD with increased pulmonary blood flow

TGA, TAPRV, truncus arteriosus, HLHS Total anomalous pulmonary venous return

Most common form of ASD; region of fossa ovalis Structurally normal AV valves Single or multiple; > 2cm likely symptomatic Females > males (3:1)

Secundum ASD

Asymptomatic typically Platypnea (dyspnea on standing) Found on exam: ejection click (pulmonary) Wide split and fixed S2 in all phases of respiration Systeolic ehection murmur (d/t increased outflow into pulm artery)

Secundum ASD:Clinical Manifestations

Severe obstruction causes immediate cyanosis Older pt squat to relieve the sx as it increases venous outflow Tet spells occur: Paroxysmal hypercyanotic: Infants, agitated, cyanotic, gasping, syncope, hypoxic blue spells, crying Loud and harsh murmur

TOF

Single arterial trunk supplies systemic, pulmonary, coronary circulations from both ventricles stenotic and/or regurgitant (leaky) As PVR drops, pulm circ increases, heart failure ensues Wide pulse pressure, bounding pulses Associated with DiGeorge syndrome Echo is diagnostic; good prognosis but mult surgeries

Truncus Arteriosus

Tx of VSD depends on the size of the hole: 30-50% small ones close (muscular 80% > membranous) Small usually uneventful Large ones can cause failure, lung infection, PVD and the large ones do not close probably need diuretics for the excess fluid Tx of the large ones?

Surgical closure if symptoms, FTT, PHTN, big shunt Transcatheter occlusion for muscular VSD Excellent outcomes; usually full recovery Risk of heart block

Cyanotic, decreased PBF Primary defect: anterior deviation of the infundibular septum Separates aortic/pulm outflow Obstruction to RV outflow (PS) Malalignment VSD Overriding aorta (dextroposition) Right ventricular hypertrophy

Tetralogy of Fallot

Cyanotic CHD with decreased pulmonary blood flow

Tetralogy of fallot Tricuspid atresia

Tx PDA

Usually don't close Small ones aren't too bad Large ones can cause HF Can cath or surgically close

When small asymptomatic When large: Harsh holocystolic mumur over LLSB Loud murmurs are good = small hole w/ a lot of pressure Less harsh murmur is bad S/S dyspnea, difficulty feeding, heart failure Develop over the first month of life

VSD: Clinical Manifestations

Acyanotic volume overload Most common malformation (25%) L to R at ventricle = increased oxygen in the RV ASD is the atrium, this is ventricle Increased flow Most commonly membranous in posterior position

Ventricular septal defect Can also be mid portion or apical (single or multiple towards bottom of heart)

Pulmonary veins drain to RH instead of heart = cyanosis, emergency Rare case where prostaglandin does not help TRUE SURGICAL EMERGENCY Increased PBF Total mixing

Total Anomalous Pulmonary Venous Return

How do you tx secundum ASD?

Transcatheter or surgical closure Low mortality in childhood Cath closure by occlusion best option

cyanotic, decreased pulmonary blood flow, no open connection b/t RA RV and the tricuspid is gone R -> L shunt at the PFO or ASD LV blood goes into aorta and across VSD

Tricuspid atresia

The 3 fetal shunts are

Venous system (ductus venosus) Heart (foramen ovale) Arterial system (ductus arteriosus)

What causes increased volume load

communication b/t systemic and pulmonary circulation hence pulmonary overcirculation AKA oxygenated blood goes back to the lungs

Decreased stats and pulmonary blood flow =

cyanotic

Decreased PBF and increased PBF are subsets of

cyanotic congenital heart disease

Most of RV output diverted away from lungs through (to descending thoracic aorta and placenta)

ductus arteriosus

supplies the ductus arteriosis and descending aorta to the placenta for oxygen

fetal right ventricle

Fetal heart preferentially delivers oxygen-saturated blood to metabolically active tissues

heart and brain

Sporadic, familial, holt-oram syndrome, 7% of CHD

isolated secundum asd

Causes of CHD?

multifactorial

common in infancy Usually hemodynamically insignificant 15-30% of adults; rarely requires treatment May be important with other underlying things Can increase risk of stroke if condition causing venous clotting in adult

patent foramen ovale

The most common major congenital heart defect is the

ventricular septal defect VSD 35% of lesions

ASD VSD PDA AVSD

volume overload