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The Pediatric Heart Congenital Heart Disease CHD is a type of defect or malformation in one or more structures of the heart or blood vessels that occur.

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Presentation on theme: "The Pediatric Heart Congenital Heart Disease CHD is a type of defect or malformation in one or more structures of the heart or blood vessels that occur."— Presentation transcript:



3 The Pediatric Heart

4 Congenital Heart Disease CHD is a type of defect or malformation in one or more structures of the heart or blood vessels that occur before birth CHD affects 8-10 out of every 1,000 children Approximately 500,000 adults in the U.S. have congenital heart disease

5 Congenital Heart Disease Symptoms in Adults – Shortness of breath – Limited ability to exercise Symptoms in Children – Can be detected prenatally – Usually detected by the presence of a heart murmur, but not all CHD has a murmur, and not all murmurs are pathological!!!

6 The Evaluation of the Heart History Physical Examination Electrocardiogram Chest radiograph Echocardiogram and further evaluation by a cardiologist (cardiopulmonary stress test, 24- hour Holter monitor, ambulatory EKG, cardiac catherization, etc

7 The History Ask about timing (at rest or activity-related), onset, and termination (gradual versus sudden), precipitating and relieving factors Infants: – Cyanosis, squatting, loss of consciousness, tachypnea, diaphoresis, poor weight gain Older children: – Dizziness, syncope, exercise intolerance, dyspnea on exertion, diaphoresis

8 Acrocyanosis vs. Cyanosis

9 The Physical Exam Visual assessment of activity (agitation or lethargy) Skin perfusion and skin color Vitals: heart rate, respiratory rate, blood pressure (in all four extremities), and oxygen saturation Dysmorphic features Cardiovascular exam

10 Cardiac Defects in Syndromes Down syndrome: AVSD Turner syndrome:Bicupsid aortic valve, coarctation Marfan syndrome:MVP, MR, dilated aortic root Fetal alcohol syndrome:VSD, ASD Maternal rubella:PDA, PPS

11 Down Syndrome

12 Marfan Syndrome

13 Cardiovascular Exam Inspection & Palpation – Chest conformation (left precordial bulge from longstanding cardiomegaly) – Increased precordial activity – A diffuse point of maximal impulse – A precordial or suprasternal notch thrill – A palpable pulmonary (P2) at upper LSB

14 Cardiovascular Exam: Points of Auscultation

15 Cardiovascular Exam Auscultation – The first heart sound (S1) is the sound of atrioventricular (AV) valve closure is best heard the lower left sternal border – The second heart sound (S2) is the sound of semilunar valve closure is best heard at the upper left sternal border. – S2 has a variable split (A2 and P2), that varies with respiration, non-variable can be cardiac disease

16 Cardiovascular Exam Auscultation – The third heart sound (S3) is the sound of rapid filling of the left ventricle, it occurs in early diastole, after S2, and is medium- to low-pitched, usually only heard in the supine position. – The fourth heart sound (S4) is associated with atrial contraction and increased atrial pressure, its low-pitched, prior to S1 and usually not audible

17 Cardiovascular Exam Auscultation – Ejection clicks are high-pitched, related to dilated great vessels or valve abnormalities Occur anytime during systole: early, mid, or late Early clicks at the mid LSB are from the pulmonic valve Aortic clicks are best heard at the apex

18 Cardiovascular Exam Auscultation – Murmurs should described by the following characteristics Location and radiation Relationship to cardiac cycle and duration Intensity Quality Variation with position

19 Cardiovascular Exam Location of pathology – RUSB Aortic valve stenosis – LUSB ASD Pulmonary valve stenosis – LLSB VSD inc AVSD – Apex MR/MS/MVPS Aortic stenosis

20 Cardiovascular Exam Relationship to the cardiac cycle and duration – Systolic Crescendo/decrescendo – Pansystolic Throughout systole or the same intensity – Diastolic – Continuous Systolic Ejection – ASD – Coarctation Pansystolic – VSD – MR/TR Diastolic – MS/TS Continuous – PDS/AVM

21 Cardiovascular Exam Intensity of murmur – Grade 1: soft, heard with difficulty – Grade 2: soft, but easily heard – Grade 3: loud without a thrill – Grade 4: loud with a precordial thrill – Grade 5: loud with a thrill heard with edge of stethoscope – Grade 6: loud with thrill and stethoscope off chest

22 Cardiovascular Exam Quality of murmur: harsh, musical, or rough; high, medium, or low in pitch Variation with position: audible when the patient is supine, sitting, standing, or squatting

23 Extracardiac Exam Arterial Pulse: Rate and Rhythm Quality and Amplitude of Pulse Arterial Blood Pressure Extremities Abdomen

24 Extracardiac Exam Rate and Rhythm Rhythm should be regular, or with a phasic variation with respiration (sinus arrhythmia) is normal Resting Heart Rates <1 month months months years years40-90

25 Extracardiac Exam Quality and Amplitude of Pulse – Pulses of upper and lower extremities should be equal – Bounding pulses are from run-off lesion, including PDA, aortic regurgitation, AVM, or any condition with low diastolic pressure (fever, anemia, or septic shock) – Narrow or thready pulses are from reduced cardiac output such as cardiomyopathy, myocarditis, pericardial tamponade, or severe aortic stenosis

26 Extracardiac Exam Arterial blood pressure – Should be obtained in the upper and lower extremities – Systolic pressure in the lower extremities should be greater than or equal to that in the upper extremities Extremities – Cyanosis, the mucous membranes – Clubbing – Edema, face and sacrum in the infant and young child

27 Extracardiac Exam Abdomen – Hepatomegaly is the cardinal sign of right heart failure in the infant and child

28 Functional Murmurs Murmurs are the most common reason for cardiology referral Innocent or functional murmurs are common and 40-45% of children will have a functional murmur at some time during childhood

29 Functional Murmurs Are always systolic murmurs, usually less than 3/6 Often position-dependent, from supine to upright tend to disappear Never associated with an ejection click or thrill Are never evaluated when a child has a fever The child is otherwise healthy and has a normal exam

30 Functional Murmurs Newborn murmur Peripheral arterial pulmonary stenosis Still murmur Pulmonary ejection murmur Venous hum Innominate or carotid bruit

31 Functional Murmurs Location of functional or innocent murmurs – RUSB Venous hum – LUSB Pulmonary flow Peripheral pulmonary stenosis – LLSB Still murmur – Apex Still murmur

32 Newborn Murmur Heard in the first few days of life Left lower sternal border, without significant radiation Soft, short, vibratory grade 1-2/6 quality which subside with pressure to the abdomen Disappears by age 2-3 weeks

33 PPS Peripheral arterial pulmonary stenosis (PPS) Heard with equal intensity at upper LSB, at the back, and in both axillae Soft, short, high pitched, grade 1-2/6 systolic ejection murmur Disappears by age 2

34 Still Murmur Most common innocent murmur in young child Heard between 2 and 7 years of age Loudest between apex and LLSB Musical or vibratory, short, high-pitched, grade 1-3 early systolic murmur Loudest when patient is supine, diminishes or disappears with inspiration or when patient stands

35 Pulmonary Ejection Murmur Most common innocent murmur in the older child 3 years of age and older Soft, systolic ejection murmur, grade 1-2/6 Localized to the upper left sternal border Louder when patient is supine

36 Venous Hum Usually occurs after 2 years of age Located in the infraclavicular areas and is usually louder on the right Continuous musical hum of grade 1-2 intensity, maybe accentuated in diastole and with inspiration Turning the childs neck, placing the child supine, and compressing the jugular resolves the murmur

37 Innominate or Carotid Bruit More common in older children and adolescents Heard in the right supraclavicular area Long systolic ejection murmur, harsh and of grade 2-3/6 intensity Can be accentuated by light pressure on the carotid artery

38 Heart Sounds Normal Heart SoundInnocent Heart Murmur Split 2Venous Hum VSD

39 Aquired Heart Disease Kawasaki Disease Rheumatic Heart Disease Cardiomyopathy

40 Kawasaki Disease First described in Japan in 1967 Used to be called mucocutaneous lymph node syndrome The etiology is unknown No specific diagnostic test Leading cause of acquired heart disease in children in the US

41 Kawasaki Disease Median age at diagnosis is 2 years of age – 80% of patients are < 5 years of age Male to female ratio is 1.5:1

42 Kawasaki Disease Diagnostic criteria – Fever for more than 5 days and at least four of: Bilateral painless, nonexudative conjunctivitis Lip or oral cavity changes (eg, lip cracking and fissuring, strawberry tongue, and/or inflammation of the oral mucosa Cervical lymphadenopathy (> 1.5 cm in diameter and usually unilateral Polymorphous exanthem Extremity changes (redness and swelling of hands and feet with subsequent desquamation)

43 Kawasaki Disease


45 Other non-diagnostic findings – GI Vomiting diarrhea, gallbladder hydrops, elevated transaminases – Blood Elevated ESR or CRP, leukocytosis, hypoalbuminemia, mild anemia in acute phase and thrombocytosis in subacute phase (usually second or third week of illness) – Renal Sterile pyuria, proteinuria

46 Kawasaki Disease Other non-diagnostic findings, cont. – Respiratory Cough, rhinorrhea, infiltrate on chest radiograph – Joint Arthralgia and arthritis – Neurologic Mononuclear pleocytosis of cerebrospinal fluid, irritability, facial palsy

47 Kawasaki Disease Cardiovascular complications – Myocarditis – Pericarditis – Valvular heart disease (usually mitral or aortic regurgitation) – Coronary arteritis (range from mild transient dilation to large aneurysm of coronary arteries)

48 Kawasaki Disease Atypical Kawasaki – Patients with fever for at least 5 days, – But fewer than four of the diagnostic features, – And coronary abnormalities by echocardiography

49 Kawasaki Disease Coronary artery lesions – Range from mild transient dilation to large aneurysms of coronary arteries – Aneurysms rarely occur before day 10 of illness – Untreated patients have a 15-25% risk of developing coronary aneurysms – Those at greatest risk are males, less than 6 months of age, and not treated with IVIG

50 Kawasaki Disease Coronary artery lesions – Detected by two-dimensional echocardiography – Most resolve in 5 years – As aneurysms resolve, stenosis or obstruction can occur, up to 19 % – Giant aneurysms (>8mm) are less likely to resolve, and nearly 50% become stenotic – Acute thrombosis can occur, resulting in myocardial infarction, fatal in 20% of cases

51 Kawasaki Disease Immediate management – Referral to Pediatric Emergency Department – IVIG and aspirin therapy – Echocardiograms immediately, at 2 weeks, and 6-8 weeks

52 Kawasaki Disease Long term management – Depends on risk stratification of cardiac disease present No cardiac disease, no f/u needed after 1 year Transient ectasia, no f/u needed after 3-5 years Single small-medium aneurysm, aspirin (asa) therapy until abnormality resolves and f/u for 10 years Giant aneurysm or multiple small-medium aneurysm, long-term asa +/- warfarin and f/u for 20 years Coronary artery obstruction, long-term asa +/- warfarin +/- calcium channel blockers and f/u every 6 months

53 Rheumatic Heart Disease Group A B-hemolytic streptococcal infection of the upper respiratory tract is the trigger in predisposed individuals – new cases are seen each year – Host susceptibility via immune response genes occurs in 15% of the population – Peak age of risk in the US is 5-15 years – More common in girls and African Americans

54 Rheumatic Heart Disease The immune response – Sensitization of B lymphocytes by streptococcal antigens – Formation of antistreptococcal antibody – Formation of immune complexes that cross-react with cardiac sarcolemma antigens – Myocardial and valvular inflammatory response

55 Rheumatic Heart Disease: Modified Jones Criteria for Diagnosis Major manifestations (2 major –or-) Carditis Polyarthritis Sydenham chorea Erythema marginatum Subcutaneous nodules – Plus Supporting evidence of preceding streptococcal infection must be present Minor Manifestatiions (1 major and 2 minor) Clinical – Previous rheumatic fever or rheumatic heart disease – Polyarthralgia – Fever Laboratory – Acuter phase reaction: elevated erythrocyte sedimentation rate, C-reative protein, leukocytosis – Prolonged PR interval

56 Rheumatic Heart Disease Syndenham chorea – Emotional instablity and involuntary movements, including ataxia, slurring speed, self-limiting Polyarthritis – Large joint swelling (knees, hips, wrists, elbow, and shoulders) Erythema Marginatum Subcutaneous Nodules

57 Rheumatic Heart Disease Erythema MarginatumSubcutaneous nodules

58 Rheumatic Heart Disease Treatment of acute episode – Anti-infective therapy Benzathine penicillin or Penicillin V Erythromycin – Anti-inflammatory therapy Asa and corticosteroids in severe cases – Therapy of CHF – Bedrest If CHF is present

59 Rheumatic Heart Disease Treatment after acute phase – Prevention of reccurence 3-5 years of therapy – Penicillin G IM every 3-4 weeks or daily antibiotic therapy Rheumatic Heart Disease – Most common in the US is mitral insufficiency – Second common in the US is aortic insufficiency – Others Mitral valve stenosis seen 5-10 years after episode Aortic stenosis seen in older adults

60 Cardiomyopathy Dilated cardiomyopathy- most frequent – caused by myocarditis, long-standing untreated tachyarrhythmias, left heart obstructive lesions, congenital abnormalities of the coronary arteries, anthracycline toxicity, genetic and metabolic disease Hypertrophic cardiomyopathy – Most common cause is familial – Leading cause of sudden cardiac death!! – Diffuse point of maximal impulse!! Restrictive cardiomyopathy- Rare

61 References The Harriet Lane Handbook, A Manual for Pediatric House Officers, 18 th Edition by The Harriet Lane Service Childrens Medical and Surgical Center of The John Hopkins Hospital Current, Diagnosis and Treatment in Pediatrics, 18 th Edition by Hay Jr, et al.

62 References Pictures – The Pediatric Heart: – Cyanosis Acrocyanois: Cyanosis: – Dysmorphic features Downs syndrome: dont-add-up/ dont-add-up/ AVSD: Points of Auscultation:

63 References Pictures Kawasaki Disease little boy: strawberry tongue: syndrome-disease-diagnosis-treatment syndrome-disease-diagnosis-treatment syndrome picture: pictures/ rash:

64 References Erythema marginatum Subcutaneous nodules – Marfan:

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