• Most common murmur in the newborn period
• Medium to High Pitch
• Grade I-II
• Soft quality, Midsystolic ejection murmur
• Heard best in axillae and back
• Increased by: Increased cardiac output
• Decreased by: Decreased cardiac output
• Appears: Birth to 1 week
• Resolves by 2-4 months
• Produced by relatively small pulmonary arteries
• CONFUSED WITH: PERIPHERAL PULMONARY STENOSIS, PDA

• Syncope/near-syncope on exertion
• Chest pain on exertion
• Excessive dyspnea or fatigue with activity
• FH of Marfan's, Cardiomyopathy, Long QT, Sudden death
• Irregular rhythm
• Weak/absent lower extremity pulses
• Hypertension
• Loud systolic murmur
• Any diastolic murmur
• Stigmata of genetic syndromes associated with CV disease

Newborns

• Left heart obstructive lesions (HLHA, AS, COA)
• Myocarditis
• Tachyarrhythmia
• Sepsis

Infants/Older Children

• Sepsis
• Myocardial Infarction
• Myocarditis

• Closure
• Oxygen
• Decreased placental prostaglandin
• Systolic and Diastolic shunt
• Bounding pulses
• Continuous "machinery" murmur
• Premature infant
• Lung disease
• Indomethacin vs. surgical ligation
• Child
• Risk of Subacute Bacterial Endocarditis (SBE)
• Coil occlusion

• Single Ventricle
• Double Inlet Left Ventricl (DILV)
• Double Outlet Right Ventricle (DORV)
• Primitive Ventricle
• Hypoplastic Right or Left Ventricle
• Total Anomalous Pulmonary Venous Return (TAPVR)
• Truncus Arteriosus

Total Anomalous Pulmonary Venous Return

(TAPVR)

• Mixing at the atrial level
• Obstructed vs Unobstructed
• "Snowman" appearance on CXR
• If obstructed: Pulmonary edema/Small heart on CXR
• Surgical timing depends on anatomy/physiology

Total Anomalous Pulmonary Venous Return

(TAPVR)

1. Pulmonic Stenosis
2. VSD
3. Overriding Aorta
4. RVH

• (+) Blood Cx (growth on 2 occasions of organisms "typical for" IE OR Persistently (+) Cx for organism "consistent with" IE
• Positive ECHO (vegetation, paravalvular adscess, or valve dehiscence after surgery)
• New valvular regurgitation

• All dental procedures that involve manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa
• Tonsillectomy and/or Adenoidectomy
• Surgical procedures or biopsy of respiratory mucosa
• Incision and drainage of infected tissues

IE Prophylaxis (Indications)

Amoxicillin

• Prosthetic heart valve
• Previous IE
• Unrepaired cyanotic CHD, incl. shunts and conduits
• Completely repaired CHD with prosthetic material within 6 months
• Repaired CHD with residual defects at site or adjacent to the site of prosthetic patch or device
• Cardiac transplant with valvulopathy

Except for the conditions above, antibiotic prophylaxis is no longer rocommended for any other form of CHD

Acute Rheumatic Fever

(Revised Jones Criteria)

MAJOR

Joints (60-85%)

  carditis (40-50%)

Nodules (2-10%)

Erythema marginatum (10%)

Sydenham's chorea (15%)

MINOR

Arthralgia

Porlonged PR interval

Fever

Previous ARF

Elevated acute phase reactants

2 major OR 1 major and 2 minor PLUS recent strep

Acute Rheumatic Fever

(Treatment)

• Benzathine Pen G (600,000 - 1.2M units IM)
• Withhold anti-inflammatories until Dx established
• ASA 100mg/kg/day in 4-6 divided doses
• For severe carditis: Prednisone 2mg/kg/day
• Bedrest during the inflammatory process

Acute Rheumatic Fever

(Secondary Prevention)

• Benzathine PCN G 1.2M units IM q 21-28 days
• PCN V 250mg PO BID
• Sulfadiazine 500-1000 mg PO QD
• PCN Allergy: Macrolide or azalide

• Continue prophylaxis through childhood (longer with carditis)

Acute Rheumatic Fever

(Duration of Secondary Prophylaxis)

• Rheumatic fever without carditis
• 5 yrs or until age 21 (whichever is longer)

• RF with carditis but no residual heart disease (no valvular disease*)
• 10 yrs or until age 21 (whichever is longer)

• RF with carditis and residual heart disease (persistent valvular disease*)
• 10 years or until age 40 (whichever is longer)
• Sometimes lifelong prophylaxis

*Clinical or echocardiographic evidence 

• GI: Diarrhea, Hydrops of gall bladder, Hepatitis
• OCULAR: Uveitis
• GU: Urethritis
• CNS: Aseptic meningitis
• MUSCULOSKELETAL: Arthralgia, Arthritis, Myositis
• LAB: Leukocytosis, Anemia, Thrombocytosis, Hyponatremia

• Chest Pain
• Fever
• Pricardial Friction Rub
• Tamponade: JVD, Tachycardia, Hypotension, Muffled Heart Tones
• Sepsis: Purulent Pericarditis

PR segment depression

ST elevation

DILATED Cardiomyopathy

(Etiology)

• Neuromuscular disorders
• Viral - Coxsackie B, Adeno, Parvo
• Familial
• Toxic/Metabolic Agents - ADRIAMYCIN, esp. w/ adjunctive radiation
• Coronary abnormalities
• Metabolic, Nutritional, Endocrine Disorders

DILATED Cardiomyopathy

(Diagnosis)

• History: Prior viral illness, Family history
• Clinical: CHF, Weakness, Fatigue
• EKG: LVH, ST-T wave changes, Left atrial enlargement
• CXR: Cardiomegaly w/ increased pulmonary venous markings
• ECHO: Dilated LV w/ diminished systolic ventricular function

DILATED Cardiomyopathy

(Management)

ACE Inhibitors

Beta Blockers: carvedilol

Diuretics ?

Digoxin ?

Anticoagulation

Transplant

Asymmetric hypertrophy, esp. septum

LVOT obstruction

Normal systolic LV function

Imparied diastolic function

AUTOSOMAL DOMINANT inheritance

HYPERTROPHIC Cardiomyopathy

(Clinical Manifestations)

• Undetected until adolescence/young adulthood
• (+) FH in 30%
• Exercise intolerance, Syncope, Arrhythmia
• Murmur: LVOT ejection murmur (Inc. standing), MR
• EKG: LVH, ST-T wave changes, Arrhythmia
• ECHO: Diagnostic - Asymmetric Hypertrophy (Septal)

HYPERTROPHIC Cardiomyopathy

(Management)

• Activity restriction
• Beta blockers, Ca channel blockers
• AVOID digoxin and positive inotropics
• AVOID tachycardia, hypovolemia and hypotension
• Septal myomectomy for obstruction
• Defibrillator for high-risk patients

Hyperlipidemia in Children

(Indications for drug therapy)

LDL >= 190

LDL >= 160 w/ FH or >= 2 risk factors

LDL >= 130 w/ diabetes