Pediatric Cardiology

• -weight and height (growth)
• -vital signs
• -cyanosis
• -clubbing
• -pulses compared in upper and lower extremeties
• -palpate chest
• -auscultate

• -heaves, thrills
• -brachiofemoral delay
• -abnormal S1 or S2
• -abnormal splitting
• -extra heart sounds
• -ejection click
• -opening snap
• -pericardial rub
• -murmurs (harsh/loud/blowing, doesn't change intensity relative to patient position)

-not clinically evident until absolute concentration of deoxygenated hemoglobin is at least 3.5g/dL

• Evident Sooner Under Certain Conditions:
• -high Hb concentration
• -decreased pH
• -increased PCO2
• -increased temperature
• -increased ratio of adult:fetal Hb

• Acrocyanosis:
• -blueness of extremities only
• -caused by peripheral vasoconstriction (normal during 24-48hrs)

• Origins of Cyanosis:
• -cardiac
• -pulmonary
• -neurologic
• -hematologic

• Preductal Saturation:
• -right upper extremity

• Postductal Saturation:
• -lower extremity

• Differential Cyanosis:
• -preductal saturation > postductal saturation
• -Persistent pulmonary HTN of newborn (PPHN)
• -LV outflow tract obstructive lesions (coarctation of the aorta, aortic stenosis)

• Reverse Differential Cyanosis:
• -preductal saturation < postductal saturation
• -transposition of great arteries with PPHN or coarctation of the aorta

• -baseline preductal ABG on room air (FIO2 = 0.21)
• -repeat on 100% O2 (FIO2 = 1.00)

-PaO2 > 250 mmHg on 100% O2 essentially rules out cardiac disease (→ pulmonary cause of stenosis)

• DUCTAL-INDEPENDENT MIXING LESIONS
•      1. Truncus Arteriosis
•      2. D-Transposition of Great Arteries
•      3. Total Anomalous Pulmonary Venous Connection (TAPVR)

• LESIONS WITH DUCTAL-DEPENDENT PULMONARY BLOOD FLOW:
•      1. Tricuspid atresia
•      2. Tetralogy of fallot
•      3. Ebstein anomaly

• LESIONS WITH DUCTAL-DEPENDENT SYSTEMIC BLOOD FLOW:
•      1. Hypoplastic Left Heart Syndrome
•      2. Interrupted Aortic Arch

• 
• EPIDEMIOLOGY:
• -rare
• -associated with 22q11 microdeletion (DiGeorge)

• PATHOPHYSIOLOGY:
• -single arterial vessel arising from the base of the heart
• -number of valve leaflets varies from 2-6
• -valve may be insufficient or stenotic
• -VSD always present
• *complete mixing of systemic and pulmonary venous blood

• CLINICAL MANIFESTATION:
• -varies depending on the amount of pulmonary blood flow
• -non-specific murmur/minimal cyanosis may be present at birth
• -CHF develops in weeks (PVR falls and pulmonary blood flow increases at the expense of systemic blood flow)
• -Murmur: SEM at LSB, loud ejection click and single heart sound S2
• -widened pulse pressure
• -bounding arterial pulses
• -CXR: mild cardiomegaly, increased pulmonary vasculature, 30% R aortic arch
• -EKG: biventricular hypertrophy

• TREATMENT:
• -surgery in neonatal period

• 
• EPIDEMIOLOGY:
• -5% of CHD
• -most common form of CHD presenting in first 24 HOL
• -3:1 male predominance

• PATHOPHYSIOLOGY:
• -aorta arises from RV, PA arises from LV
• -pulmonary and systemic circuits in parallel
• -required PFO

• CLINICAL MANIFESTATION:
• -cyanosis present from birth
• -tachypnea
• -Murmur: loud single S2, systolic murmur (VSD)
• -CXR: mild cardiomegaly ("egg-shaped"), increased pulmonary vascular markings
• -EKG: RAD, RVH

• TREATMENT:
• -PGE1
• -balloon atrial septostomy (Rashkind procedure)
• -arterial switch procedure during first week of life

• 
• EPIDEMIOLOGY:
• -rare (1-2% of CHD)

• PATHOPHYSIOLOGY:
• -pulmonary veins not connected to LA (confluence behind LA then drain into RA)
• 1. Supracardiac (SVC or innominate vien)
• 2. Cardiac (coronary sinus or RA)
• 3. Infradiaphragmatic (portal or hepatic vein)
• 4. Mixed
• -with obstruction when vein enters at acute angle
• *ASD or PFO is required

• CLINICAL MANIFESTATIONS W/O OBSTRUCTION:
• -RV heave
• -wide, fixed split S2 with loud pulmonary component
• -Murmur: systolic ejection murmur at LUSB
• -CXR: cardiomegaly ("snowman"), increased pulmonary vascularity
• -EKG: RAD, RVH

• CLINICAL MANIFESTATIONS W/ OBSTRUCTION:
• -marked cyanosis and respiratory distress, tachypnea
• -loud, single S2
• -CXR: normal heart size, markedly increased pulmonary vascularity, diffuse PE
• -EKG: RVH

• TREATMENT:
• -surgery as newborn if obstruction is present

• 
• EPIDEMIOLOGY:
• -rare (<1% CHD)

• PATHOPHYSIOLOGY:
• -complete atresia of tricuspid valve
• -leads to severe hypoplasia or absence of RV
• 1. With normally related great arteries (NRGA)
• 2. With transposition of great arteries (TGA)
• -90% have VSD
• -L heart handles both systemic and pulmonary venous return
• -NRGA usually have pulmonary stenosis

• CLINICAL MANIFESTATIONS:
• -depend on degree of pulmonary stenosis (most have significant)
• -progressive cyanosis, poor feeding, tachypnea (in first 2 weeks)
• -Murmur: holosystolic (VSD), continuous (PDA)
• -CXR: normal heart size, decreased pulmonary vascular markings
• -EKG: LAD, RAH, LVH
• *with TGA: shock when DA closes

• TREATMENT:
• -PGE1 to maintain PDA
• -Blalock-Taussig shunt (maintain pulmonary blood flow)
• -hemi-Fontan/bidirectional Glenn (cavopulmonary anastomosis)
• -Fontan: IVC and hepatic vein flow into pulmonary circulation

• 
• EPIDEMIOLOGY:
• -most common CHD in childhood (10%)
• -22q11 microdeletion

• PATHOPHYSIOLOGY:
• 1. VSD
• 2. Pulmonary valve stenosis
• 3. RVH
• 4. "overriding" aorta
• -R to L shunt across  VSD → cyanosis

• CLINICAL MANIFESTATION:
• -degree of pulmonic stenosis determines the timing and severity of cyanosis (hours after birth to later infancy)
• -RV heave
• -Murmur: loud systolic ejection murmur at LUSB
• -CXR: normal heart size ("bootshaped"), decreased pulmonary vascular markings
• -EKG: RAD, RVH

• "tet spells":
• -cyanosis, rapid breathing, agitation
• -caused by increased RV outflow resistance
• -last mins to hrs
• -may resolve spontaneously or lead to hypoxia/metabolic acidosis/death

• TREATMENT:
• 1. Increase SVR
• 2. Decrease PVR
• 3. Increase Preload (squatting)
• -calm patient, vagal manuevers
• -supplemental O2 (pulmonary vasodilator)
• -morphine sulfate (minimize O2 consumption)
• -volume expansion
• -vasoconstrictors
• -β-blockers (decrease infudibular spasm)
• -sodium bicarb (reduce acidosis, decrease PVR)
• -surgery: 3-6 months or after first tet spell

• 
• EPIDEMIOLOGY:
• -extremely rare
• -associated with maternal Li use

• PATHOPHYSIOLOGY:
• -septal leaflet of tricuspid valve displaced inferiorly into RV, and anterior leaflet is "sail-like" and redundant
• -RV into RA → functional hypoplasia of RV and tricuspid regurgitation
• *majority of pulmonary blood flow from PDA
• *PFO in 80% (R to L shunt at atrial level)
• -dilated RA may lead to SVT (WPW)

• CLINICAL MANIFESTATIONS:
• -cyanosis and CHF in first few DOL
• -widely fixed split S2, gallop rhythm
• -Murmur: holosystolic murmur at LLSB
• -CXR: extreme cardiomegaly with RAH, decreased pulmonary vascular markings
• -EKG: RBBB, RAH

• TREATMENT:
• -PGE1 infusion
• -avoid surgical intervention (tricuspid surgery has poor results)
• -heart transplant

• 
• EPIDEMIOLOGY:
• -second most common CHD presenting in first week of life
• -most common cause of death from CHD in first month of life

• PATHOPHYSIOLOGY:
• -hypoplasia of LV
• -aortic valve stenosis or atresia
• -mitral valve stenosis or atresia
• -hypoplasia of ascending aorta
• *Atrial defect
• -systemic blood flow is completely ductal dependent

• CLINICAL MANIFESTATIONS:
• -shock when DA closes
• -RV heave
• -single S2
• -Murmur: continuous (PDA)
• -CXR: pulmonary edema and progressive cardiac enlargement
• -EKG: RVH, poor R wave progression across precordial lead

• TREATMENT:
• -PGE1
• -palliative procedure only, no corrective surgery
• -Stage I: Norwood (combine PA and Ao, atrial septectomy, Blalock-Taussig shunt)
• -Stage II: Bidirectional Glenn/Hemi-Fontan (cavopulmonary anastomosis, at 3-6 mos)
• -Stage III: modified Fontan (2-5 yrs)
• -Heart transplant

• 
• EPIDEMIOLOGY:
• -associated with 22q11 microdeletion

• PATHOPHYSIOLOGY:
• -extreme form of coarctation of aorta
• -Type A: after L subclavian artery
• -Type B: between L subclavian and L common carotid
• -Type C: between L common carotid and brachiocephalic artery
• -systemic flow depends on PDA

• CLINICAL MANIFESTATION:
• -circulatory collapse as the ductus closes (similar to HLHS)

• TREATMENT:
• -PGE1 therapy
• -Surgical end-to-end anastomosis of the aortic segments

• INCREASED PULMONARY BLOOD FLOW (L to R)
•      1. ASD
•      2. VSD
•      3. PDA
•      4. Common AV canal

• PULMONARY VENOUS HTN
•      1. Coarctation of aorta
•      2. Aortic valve stenosis

• NORMAL/DECREASED PULMONARY BLOOD FLOW:
•      1. Pulmonary valve stenosis

• 
• EPIDEMIOLOGY:
• -8% of CHD
• -2:1 female predominance

• PATHOPHYSIOLOGY:
• 1. Ostium Secundum (2°, midportion, most common)
• 2. Ostium Primum (1°, lower portion)
• 3. Sinus Venosus (junction of RA and SVC or IVC)
• -degree of shunting depends on size of ASD and compliance of ventricles in diastole
• -L to R shunting → R heart enlargement and increased pulmonary blood flow

• CLINICAL MANIFESTATIONS:
• -usually asymptomatic
• -exercise intolerance
• -paradoxical embolism
• -SVT (atrial enlargement)
• -RV heave
• -loud S1
• -widely "fixed" S2 split
• -Murmur: systolic ejection murmur at LUSB, Mid-diastolic rumble at LRSB
• -CXR: enlarged heart and main PA, increased pulmonary vasculature
• -EKG: Secundum (RAD), Primum (extreme LAD)

• TREATMENT:
• -spontaneous closure of small secundum ASDs often occurs in first year
• -transcatheter device closure (after 2 yrs)
• -ostium primum and sinus venosus ASDs require surgery
• -pericardial patch or suture closure
• -Subacute bacterial endocarditis prophylaxis

• 
• EPIDEMIOLOGY:
• -most common congenital heart defect (25% of CHD)

• PATHOPHYSIOLOGY:
• 1. Muscular
•      -most common
•      -single or multiple
• 2. Inlet
•      -endocardial cushion defect
•      -beneath septal leaflets of TC valve
• 3. Conoseptal Hypoplasia
•      -at outflow tract of RV beneath PV
•      -membranous ventricular septum
• 4. Conoventricular
•      -most common type with muscular
• 5. Malalignment
•      -malalignment of infundibular septum
•      -anterior malalignment → TOF
•      -posterior malalignment → AS
• -Restrictive (small): L to R flow, Normal PVR
• -Nonrestrictive (large): LV = RV pressure, PVR and SVR determine shunt flow
• -may lead to Eisenmenger syndrome

• CLINICAL MANIFESTATIONS:
• -depends on the size of the shunt
• -large shunt: CHF, growth failure
• -the smaller the defect the louder the murmur
• -Murmur: harsh systolic murmur at mid to Lower LSB
• -Eisenmenger: RV heave, PV ejection click, SEM, diastolic murmur, loud single S2

• TREATMENT:
• -most small VSDs close without intervention (40% by 3 years, 75% by 10 years)
• -surgery unnecessary in small VSDs
• -VSDs with large shunts require surgical closure before pulmonary vascular changes are irreversible
• -Dacron patch closure
• -transcatheter device placement
• -SBE prophylaxis

• 
• EPIDEMIOLOGY:
• -5% of all CHD
• -most commonly seen in Down Syndrome

• PATHOPHYSIOLOGY:
• -deficiency of the endocardial cushions
• -ostium priumum ASD and inlet VSD with lack of septation of mitral and TC valves

• 1. Incomplete CAVCD
•      -CAVV leaflets attach to top of muscular ventricular septum
•      -mitral valve is cleft (regurgitation)

• 2. Complete CAVCD
•      -CAVV not attached to muscular ventricular septum
•      -large inlet VSD
•      -L to R shunting at ASD and VSD
•      -Pulmonary HTN develop over time

• CLINICAL MANIFESTATIONS:
• -same as ASD
• -Murmur: blowing systolic murmur at LLSB and apex
• -S2 is widely split and fixed
• -CXR: cardiac enlargement, increased pulmonary vascularity
• -EKG: superior axis

• TREATMENT:
• -CHF tx with digoxin, diuretics, ACEI
• -complete usually surgically repaired during infancy (ASD, VSD repaired and CAVV divided into R and L)
• -complete heart block occurs in 5% and residual mitral insufficiency is common

• 
• EPIDEMIOLOGY:
• -10% of CHD
• -higher in premature neonates

• PATHOPHYSIOLOGY:
• -connects the underside of the aorta and the left PA distal to L subclavian
• -direction of flow depends on SVR and PVR
• -nonrestrictive (large): L to R shunt

• CLINICAL MANIFESTATION:
• -small PDA: no sx
• -CHF
• -FTT
• -bounding pulses
• -continuous murmur
• -CXR: cardiomegaly, LA and LV enlargement, increased pulmonary vascular
• -EKG: L or biventricular hypertrophy

• TREATMENT:
• -Indomethacin (closes PDA in preemie)
• -coil embolization
• -device closure
• -surgical ligation

• 
• EPIDEMIOLOGY:
• -8% of CHD
• -2:1 male predominance
• -in female consider Turner syndrome

• PATHOPHYSIOLOGY:
• -obstruction usually at the descending aorta at the insertion of the ductus arteriosus
• -leads to increased LV afterload

• CLINICAL MANIFESTATION:
• -asymptomatic in 50%
• -irritability, difficulty feeding, FTT
• -may present with circulatory collapse when PDA closes
• -weak femoral pulses
• -upper extremity HTN
• -murmur: non specific ejection murmur at apex
• -CXR: enlarged aortic knob and cardiomegaly
• -EKG: RVH in neonate, LVH in older pt

• TREATMENT:
• -PGE1
• -surgery: end to end anastomosis, patch aortoplasty
• -balloon dilation angioplasty
• -restenosis commonly needed
• -β-blockers in older children for persistent HTN

• PATHOPHYSIOLOGY:
• -thickened valvular tissue, rigid, domed in systole
• -commonly bicuspid valve
• -LVH

• CLINICAL MANIFESTATIONS:
• -asymptomatic
• -critical AS can have circulatory collapse if ductus closes
• -Murmur: harsh systolic ejection murmur at RUSB, preceded by ejection click
• *murmur increases with increased stenosis
• *murmur softens when ventricular function is compromised
• -CXR: cardiomegaly, pulmonary edema
• -EKG: LVH, ischemic changes (ST depression, inverted T waves)

• TREATMENT:
• -PGE1 if ductal dependent
• -balloon valvuloplasty (may result in progressive aortic regurgitation that requires valve replacement)

• EPIDEMIOLOGY:
• -5-8% of CHD

• PATHOPHYSIOLOGY:
• - domed valve with small central opening
• -poststenotic dilatation of the main pulmonary artery
• -RVH occurs over time

Critical Pulmonic Stenosis: decrease in compliance of RV decreases RA pressure and may open FO → R to L shunt

• CLINICAL MANIFESTATIONS:
• -most asymptomatic
• -severe may cause dyspnea on exertion and angina
• -Murmur: ejection click that varies with inspiration, harsh systolic ejection murmur at LUSB
• -thrill and RV heave
• -CXR: pulmonary artery segment is enlarged
• -EKG: RVH and RAD
•  
• TREATMENT:
• -PGE1 in critical stenosis
• -surgery/catheter based intervention