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The Pediatric Heart Mutter Museum historic Medical Photographs, the picture was taken approximately in the 1890’s at the Children’s Hospital, note the.

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Presentation on theme: "The Pediatric Heart Mutter Museum historic Medical Photographs, the picture was taken approximately in the 1890’s at the Children’s Hospital, note the."— Presentation transcript:

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2 The Pediatric Heart Mutter Museum historic Medical Photographs, the picture was taken approximately in the 1890’s at the Children’s Hospital, note the pronounced bulge along the edge of her sternum could be caused by hypertrophy in the right side of heart, possibly by fluid in the pericardium, or maybe even a mediastinal condition, is it aquired or congenital?

3 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

4 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!!!

5 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

6 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 The most common complaint thought to be associated with cardiac disease is chest pain, which is rarely the cause. It’s most likely to be chest wall tenderness (the palpable chest pain), reflux or heartburn (occurs mostly after meals or at night), and undiagnosed asthma.

7 Acrocyanosis vs. Cyanosis

8 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

9 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

10 Down Syndrome

11 Marfan Syndrome

12 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

13 Cardiovascular Exam: Points of Auscultation

14 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 The variable split is hardest to discern in the newborn infant, takes time, patience, and practice

15 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

16 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

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

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

19 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

20 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

21 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

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

23 Extracardiac Exam Rate and Rhythm Resting Heart Rates
Rhythm should be regular, or with a phasic variation with respiration (sinus arrhythmia) is normal <1 month 1-3 months 2-24 months 2-10 years 11-18 years

24 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

25 Extracardiac Exam Arterial blood pressure Extremities
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

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

27 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

28 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

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

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

31 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

32 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

33 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

34 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

35 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 child’s neck, placing the child supine, and compressing the jugular resolves the murmur

36 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

37 Heart Sounds Normal Heart Sound Innocent Heart Murmur Split 2
Venous Hum VSD

38 Aquired Heart Disease Kawasaki Disease Rheumatic Heart Disease
Cardiomyopathy

39 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

40 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

41 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)

42 Kawasaki Disease

43 Kawasaki Disease

44 Kawasaki Disease Other non-diagnostic findings GI Blood Renal
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

45 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

46 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)

47 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

48 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

49 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

50 Kawasaki Disease Immediate management
Referral to Pediatric Emergency Department IVIG and aspirin therapy Echocardiogram’s immediately, at 2 weeks, and weeks

51 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

52 Rheumatic Heart Disease
Group A B-hemolytic streptococcal infection of the upper respiratory tract is the trigger in predisposed individuals 30-50 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

53 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

54 Rheumatic Heart Disease: Modified Jones Criteria for Diagnosis
Major manifestations (2 major –or-) Minor Manifestatiions (1 major and 2 minor) Carditis Polyarthritis Sydenham chorea Erythema marginatum Subcutaneous nodules Plus Supporting evidence of preceding streptococcal infection must be present 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 Supporting evidence includes increased titers of antistreptolysin O or other streptococcal antibodies, positive throat culture for group A Streptococcus

55 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 Chorea can last up to 3 months and can occur months to years after acute episode

56 Rheumatic Heart Disease
Erythema Marginatum Subcutaneous nodules

57 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

58 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

59 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

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

61 References Pictures The Pediatric Heart: Cyanosis Acrocyanois: Cyanosis: Dysmorphic features Down’s syndrome: AVSD: Points of Auscultation:

62 References Pictures Kawasaki Disease little boy: strawberry tongue: syndrome picture: rash:

63 References Erythema marginatum
Subcutaneous nodules Marfan:


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