Pedia 2.3a Pediatric Cardiology (Summary Table)

3.1

Pediatric Cardiology (Summary Table) Ruby Ann Punongbayan, MD CONDITION

ATRIAL SEPTAL DEFECT (ASD)  Isolated anomaly in 5-10% of all CHDs VENTRICULAR SEPTAL DEFECT (VSD)  Most common form of CHD (1520%)  Perimembranous defects: Most common (70%)

PATENT DUCTUS ARTERIOSUS (PDA)  Occurs in 5-10% of all CHDs (common in premature infants)

MITRAL VALVE PROLAPSE (MVP)  Occurs in older children & adolescents

TRANSPOSITION OF THE GREAT ARTERIES (TOGA)  Occurs in about 5% of all CHDs (M>F)  Most common cyanotic heart lesion among newborns TETRALOGY OF FALLOT (TOF)  Occurs in 10% of all CHDs  Most common cyanotic heart defect beyond infancy  “PROVe”

Sept. 8, 2014

CLINICAL MANIFESTATIONS DIAGNOSTIC FINDINGS CONGENITAL HEART LESIONS ACYANOTIC CONGENITAL HEART LESION  Right sided enlargement  Systolic ejection murmur at the 2nd  ECG: RVH & RBBB in LICS  CXR: RAE & RVE & prominent PA  Widely split S2  Left sided enlargement  Biventricular hypertrophy if with Eisenmenger syndrome  Systolic regurgitant murmur at the LLSB  ECG: LVH in moderate shunts;  Loud and single S2 in pulmonary (Combined ventricular hypertrophy) CVH hypertension in large defects  CXR: LAE, LVE, increased pulmonary vascular markings, PVOD, enlarged MPA (main pulmonary artery)  Left sided enlargement  Continuous “machinery” murmur at  ECG: LVH (small to moderate shunts) the (L) infraclavicular area or ULSB  CXR: Shows prominent pulmonary  Bounding peripheral pulses with wide markings/vessels and enlarged left pulse pressure ventricle  Midsystolic click with or without a late systolic murmur best heard at the apex  Click & murmur may be brought out by held expiration, (L) decubitus position, sitting, standing, leaning forward; may disappear on inspiration CYANOTIC CONGENITAL HEART LESION

 Single & loud S2; no heart murmur is heard in infants with an intact ventricular septum

 Systolic ejection murmur at the mid and 2nd ULSB heard at the 2nd LICS

 Loud and single S2

 ECG: RVH, LVH, CVH  CXR: Egg-shaped cardiac silhouette with a narrow, superior mediastinum

 ECG: RAD, RVH  CXR: Small heart size, decreased pulmonary vascular markings, concave main PA with an upturned apex (couer en sabot or boot-shaped heart)

MANAGEMENT

 Nonsurgical closure: Occlusion devices  Surgery for Qp/Qs >1.5:1  Delayed until 3-4 years old  Treatment of CHF  Good dental hygiene and antibiotic prophylaxis against IE

 Surgery if no improvement within the 1st 6 months of life

 Nonsurgical closure: Stainless coils  Surgical closure: PVOD is a contraindication; done between 6 months & 2 years of age or when diagnosis is made in an older child

 Asymptomatic patients do not need treatment or restriction of activity

 Antibiotic prophylaxis against SBE  Chest pain may be treated with Propranolol (CI in asthmatics)

 Definitive repair: switch right- & left-sided blood at 3 levels: atrial (Senning or Mustard), ventricular (Rastelli), great artery level (Jatene); Rashkind  Knee-chest position, Morphine sulphate, NaHCO3, O2, Vasoconstrictors, Propranolol, Ketamine  Maintain good dental hygiene & antibiotic prophylaxis o Surgery: Blalock-Taussig (>3 months old) and Gore-Tex shunt (< 3 months old) o Total repair of the defect (patch closure of VSD & widening of the RVOT by resection)

3.1 Pediatric Cardiology (Summary Table) TRICUSPID ATRESIA  Components: 1. Atretic (Missing) Tricuspid Valve 2. Hypoplastic Right Ventricle 3. Ventricular Septal Defect 4. Atrial Septal Defect 5. Pulmonic Stenosis TRUNCUS ARTERIOSUS  1.2-2.5% of all congenital heart disease  Associated with Di-George Syndrome linked to deletion of chromosome 22q11  Components: 1. Pulmonary arteries arise from aorta 2. Truncal valve (quadracuspid stenotic and/or insufficient) 3. Large VSD (always present) TOTAL ANOMALOUS PULMONARY VENOUS RETURN (TAPVR)

 Severely cyanotic neonates: Blalock-Taussig  Systolic regurgitant murmur at LLSB; systolic ejection murmur at ULSB

Hemodynamics  Pressure hypertrophy of the LV and LA with obstruction to flow from LV COARCTATION OF THE AORTA (COA)  7% of all CHD  Aortic valve: Bicuspid in more than 70%

procedure

 Between 4-8 months: Bidirectional Glenn Shunt

 1.5 -3 years old: Modified Fontan Operation

 Single S2  Systolic regurgitant murmur at LLSB  Minimal cyanosis in neonates; older children: heart failure

 CXR: Cardiomegaly (right, left, or

 Digitalis and diuretics for heart failure  Elective repair at 6 weeks old

o Surgery: Rastelli Repair o Older patients who already have pulmonary vascular obstruction, routine surgical treatment is contraindicated and heart-lung transplantation is the only option

combined ventricular hypertrophy), increased pulmonary circulation

 Without obstruction: Mild cyanosis,  CXR: Large supracardiac shadow can be tachypnea, feeding difficulties  With obstruction: Rapid progression to seen, which together with the normal dyspnea, cardiac respiratory failure cardiac shadow forms a “Snowman  Systolic murmur at LSB; murmurs may not sign” be present OBSTRUCTIVE LESIONS

PULMONARY STENOSIS (PS)  Associated with congenital rubella, Noonan and William syndrome Hemodynamics  RV pressure overload  RV pressure hypertrophy  RV failure  RV pressures maybe > systemic pressure AORTIC STENOSIS (AS)

 Hypoplastic RV

 Prostaglandin E before surgery to dilate the DV and DA

 Van Praagh surgical procedure

 ECG: RAD, RBBB, if mild RVH (pure R  Asymptomatic to severe  Systolic murmur LUSB to back; soft P2 there is radiation to back

wave and upright T wave in V1)

 CXR

o Normal or RV cardiomegaly o Normal or dilated Main Pulmonay Artery (post stenotic dilatation)

 Usually asymptomatic only detected when there is already left ventricular failure

 Harsh systolic ejection murmur at RUSB and radiates to the neck and left midsternal border

 Most children asymptomatic but can have CHF if severe  Classic signs of coarctation are diminution or absence of femoral pulses  Higher BP in the upper extremities as

 ECG: May show ischemia in severe

 Balloon Valvuloplasty  Valvotomy (Brock’s Procedure)

 SBE prophylaxis  Balloon valvuloplasty  Valve replacement (Ross Procedure)

stenosis

 ECG: LVH  CXR: Enlargement, Rib notching (children > 5 years; around 7 years old)

    

Secondary Bacterial Endocarditis (SBE) prophylaxis Hypertension tx Elective repair at 2-3 years old Aggressive management of CHF Balloon angiography (shunt placement)

3.1 Pediatric Cardiology (Summary Table)

 Seen in Turner’s Syndome Hemodynamics  Obstruction of the left ventricular outflow  pressure hypertrophy of the LV

compared to the lower extremities o Systolic ejection murmur

o Treatment of choice: Surgical repair 

Primary re-anastomosis or a patch

aortoplasty  Subclavian flap aortoplasty (Waldhausen and Nahrwold)