Paediatric Cardiology: An Outline of Congenital Heart Disease Dr. H.C. Rosenberg

Objectives u To provide an outline of congenital heart disease u List criteria for Kawasaki syndrome u Describe the common innocent murmurs of childhood

An Outline of Congenital Heart Disease u Pink (Acyanotic) u Blue (Cyanotic)

u Resistance= ?

Acyanotic Congenital Heart Disease u Normal Pulmonary Blood Flow u ↑ Pulmonary Blood Flow

Acyanotic Congenital Heart Disease u Normal Pulmonary Blood Flow u Valve Lesions u Not fundamentally different from adults

Acyanotic Congenital Heart Disease u ↑ Pulmonary Blood Flow

Shunt Lesions Atrial Level Shunt

ASD Physiology u Left to Right shunt because of greater compliance of right ventricle u Loads right ventricle and right atrium u Increased pulmonary blood flow at normal pressure u Low resistance

ASD History u Usually asymptomatic in childhood u Occasionally frequent respiratory tract infections u Presentation with murmur as pre-schooler or older

ASD Physical Examination u Right ventricular “lift” u Wide fixed S2 u Blowing SEM in pulmonic area

ASD

ASD

ASD Natural History u Generally do well through childhood u Major complication atrial fibrillation u Can develop pulmonary hypertension / RV failure but not before third or fourth decade of life

ASD Management u Device closure around three years of age or when found u Surgery for very large defects or outside fossa ovalis (eg. sinus venosus defect)

ASD

Shunt Lesions Ventricular Level Shunt

VSD Physiology u Left to Right shunt from high pressure left ventricle to low pressure right ventricle u Loads left atrium and left ventricle (right ventricle may see pressure load)

VSD History u Small defects u Presentation with murmur in newborn period u Large defects u Failure to thrive (6 wks to 3 months) u Tachypnea, poor feeding, diaphoresis

VSD Physical Examination u Active left ventricle u Small defect u Pansystolic murmur, normal split S2 u Large defect u SEM, narrow split S2, diastolic murmur at apex from high flow across mitral valve

VSD BVH

VSD

VSD Natural History u Small defect u Often close u No real significance beyond endocarditis risk u Large defect u Failure to thrive u Progression to pulmonary hypertension as early as 1 year

VSD Management u Small defect u Large defect u Semi-elective closure if growth failure or evidence of increased pulmonary hypertension u Occasionally elective closure if persistent cardiomegally beyond 3 years of age

Shunt Lesions Great Artery Level Shunt

PDA Physiology u Left to Right shunt from high pressure aorta to low pressure pulmonary artery u Loads left atrium and left ventricle (right ventricle may see pressure load)

PDA History u Premature duct u Failure to wean from ventilator +/- murmur u Older infant u Usually murmur from early infancy u Occasionally signs of heart failure

PDA Physical Examination u Active left ventricle u Hyperdynamic pulses u Premature duct u SEM with diastolic spill u Older infant u Continuous murmur

PDA Management u Premature Duct u Trial of indomethacin u Surgical ligation u Older infant u Leave till 1 year of age unless symptomatic u Coil / device closure u Rarely surgical ligation

Truncus Arterisosus

Cyanotic Congenital Heart Disease u “Blue” blood (deoxygenated hemoglobin” enters the arterial circulation u Systemic oxygen saturation is reduced u Cyanosis may or may not be clinically evident

Causes of Cyanosis u Respiratory u Cardiac u Hematologic u Polycythemia u Hemoglobins with decreased affinity u Neurologic u Decreased Respiratory drive

Cyanosis u Respiratory u Cardiac u Hyperoxic test u Place infant in 100% 02 u Lung disease should respond to 02 u Failure of saturation to rise to > 85% suggest cardiac disease

Cyanotic Congenital Heart Disease u ↓Pulmonary Blood Flow u ↑Pulmonary Blood Flow

Cyanotic Congenital Heart Disease u Decreased Pulmonary Blood Flow

Cyanotic Congenital Heart Disease u Decreased Pulmonary Blood Flow u Tetralogy of Fallot u Pulmonary Atresia

Cyanotic Congenital Heart Disease - ↓ Pulmonary Flow = RVOT Obstruction + Shunt

Tetralogy of Fallot u VSD u Over-riding aorta u Pulmonary stenosis u RVH

Tetralogy of Fallot

History u Presentation depends on severity of PS u Severe stenosis u Cyanosis shortly after birth (as duct closes) u Mild stenosis u May present as heart murmur (from shortly after birth)

Tetralogy of Fallot Physical Examination u Variable cyanosis (remember the 50g/l rule) u Right ventricular “tap” u Decreased P2 +/- ejection click u “Tearing” SEM

Tetralogy of Fallot Management u Outside the newborn period, surgical repair if symptomatic u Elective repair at 6 months u Role for beta blockers to palliate hypercyanotic spells

Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) u Episodes of profound cyanosis u Most frequently after waking up or exercise

Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) Fall in P0 2 Hyperventilation Increased Return of deeply desaturated venous blood Increased R to L shunt

Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells u Treatment u Tuck knees to chest (pinches off femoral veins) u In hospital u O2 u Bicarbonate u Phenylephrine u Morphine u IV beta blocker

Tetralogy of Fallot

u Decreased Pulmonary Blood Flow

Duct Dependent Congenital Heart Disease  Which of the following are examples of duct dependent CHD? 1. Pulmonary atresia 2. Patent ductus arteriosus 3. Transposition of the great arteries

Cyanotic Congenital Heart Disease With ↑Pulmonary Blood Flow

u Transposition of the great arteries u Total anomalous pulmonary venous drainage

d-Transposition

Normal Heart BodyRARVPA LALVAOLungs Circulation is in “series”

d-Transposition u Circulation is in “parallel” u Need for mixing

Transposition History u Presentation u Profound cyanosis shortly after birth (as duct closes) u Minimal or no murmur

Tetralogy of Fallot Physical Examination u Profound cyanosis u Right ventricular “tap” u Loud single S2 u Little or no murmur

Tetralogy of Fallot Management u Prostaglandins to maintain mixing u Balloon atrial septostomy u Arterial switch repair in first week

Total Anomalous Pulmonary Venous Return Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

Total Anomalous Pulmonary Venous Return - Supracardiac Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

Total Anomalous Pulmonary Venous Return - Infracardiac Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

TAPVD History u Presentation depends on presence or absence of obstruction to venous return u Infradiaphragmatic u Almost always obstructed u Cyanosis and respiratory distress shortly after birth u Cardiac or supracardiac u Rarely obstructed u Can present like big ASD

TAPVD Physical Examination u Variable cyanosis (again depends on obstruction) u Right ventricular “tap” u Wide split S2 u Blowing systolic ejection murmur

TAPVD

TAPVD Management u If severe cyanosis in newborn u Emergency surgical repair u Unobstructed u Semi-elective surgical repair when discovered

Coarctation of the aorta

Coarctation of the Aorta History u Presentation varies with severity u Severe coarct u Failure (shock) in early infancy u Mild coarct u Murmur (in back) u Hypertension

Coarctation Physical Examination u Absent femoral pulses u Arm leg gradient +/- hypertension u Left ventricular “tap” u Bruit over back

Coarctation Management u Newborn with CHF u Emergency surgical repair u Infant u Semi-elective repair in uncontrolled hypertension u Older child u Balloon arterioplasty u Surgery on occasion u Failure to repair prior to adolescence recipe for life long hypertension!

“Grey” Heart Disease u Critical LVOT obstruction

Left Ventricular Outflow Tract Obstruction u Critical Aortic Stenosis u “Critical” shock

Critical Aortic Stenosis Management u Prostaglandins to provide source of systemic blood flow u Balloon valvuloplasty u Rarely surgery

Hypoplastic Left Heart Syndrome u “Duct dependent “ congenital heart disease u Ductus arteriosus is the only source of systemic blood flow

Hypoplastic left heart Management u Prostaglandins u Norwood procedure

Kawasaki Syndrome u Small artery arteritis u Coronary arteries most seriously effected u Dilatation/aneurysms progressing to (normal) stenosis

Kawasaki Syndrome u 5 days of fever plus 4 of  Rash  Cervical lymphadenopathy (at least 1.5 cm in diameter)  Bilateral conjuctival injection  Oral mucosal changes u Peripheral extremity changes u Swelling u Peeling (often late)

Kawasaki Syndrome u Associated Findings u Sterile pyuria u Hydrops of the gallbladder u Irritability!!!

Kawasaki Syndrome u Epidemiology u Generally children < 5 years u Male > Female u Asian > Black > White

Kawasaki Syndrome u Management u Gamma globulin 2g/kg u 80 mg/kg ASA until afebrile then 5 mg/kg for 6 weeks

Innocent Murmurs u Characteristics u Always Grade III or less u Always systolic (or continuous) u Blowing or musical quality u Not best heard in back

Innocent Murmurs u Types u Still’s u Vibratory SEM best heard mid-left sternal border u Pulmonary Flow murmur u Blowing SEM best heard in PA u Venous Hum u Continuous murmur best heard in R infraclavicular u Decreases lying flat or occlusion of neck veins u Physiologic peripheral pulmonary artery stenosis u Blowing SEM best heard in PA radiating out to both axillae

Questions?