Pediatric Cardiology Snippets and Board Review Thomas Burklow

Pediatric Cardiology Snippets and Board Review Thomas Burklow, MD National Capital Military Children’s

Attempting the impossible… • Ch. 23: Recognition of cardiovascular disorders • Ch. 24: Congenital heart disease • Ch. 25: Acquired heart disease • Ch. 26: Arrhythmias and conduction disorders ational Capital Military Children’s Center National

Ground rules • You already know this stuff (at least it seems vaguely familiar • Material reflects materials covered by ABP examination (and not necessarily clinical practice) ational Capital Military Children’s Center National

Recognition of Cardiovascular Disorders National Capital Military Children’s

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Vena Cavae (VC) Right Atrium (RA) Right Ventricle (RV) Pulmonary Artery (PA) ational Capital Military Children’s Center National Pulmonary Veins (PV) Left Atrium (LA) Left Ventricle (LV) Aorta (Ao)

Normal newborn cardiopulmonary physiology • Anatomically… – – • PV Ductus arteriosus Foramen ovale Physiologically… – VC RA Normal physiology • • Left-right intra-atrial shunting Left-right ductal shunting RV LV PA Ao

At birth • Initial physiology: high pulmonary vascular resistance VC PV RA RV LV PA Ao

Several hours after birth… Pulmonary vascular resistance typically falls VC PV RA RV LV PA Ao

Quiz: Murmurs, the benign ones • Healthy one month old with a systolic murmur noted in both axillae and back – Bilateral pulmonary artery branch stenosis • 4 year old with a newly noted murmur, best heard along LSB, louder supine, attenuates when stands. “Vibratory” in character – Still’s murmur, or ‘normal flow murmur’ • 3 year old with a continuous murmur noted primarily at the RUSB – Venous hum • Neonate with mild persistent tachypnea with a murmur best heard at the lower sternal border, systolic regurgitant (“holosystolic”) which attentuates over the next few days – Tricuspid regurgitational Capital Military Children’s Center National

Newborn murmurs • In the newborn, systolic murmurs can be caused by all but: – Patent ductus arteriosus – Peripheral pulmonary stenosis – Pulmonary outflow tract murmur – Pulmonary insufficiency from congenital dysplastic pulmonary valve – Benign left ventricular outflow tract murmur ational Capital Military Children’s Center National (pulmonary insufficiency is a diastolic murmur)

Murmur referrals? • Which of the following is cause of appropriate referral to cardiology? – Holosystolic murmur – Late systolic murmur – Murmur with a thrill – Continuous murmur (that is not a venous hum) – Diastolic murmur ational Capital Military Children’s Center National

Syndromes and heart disease What is the incidence and typical heart disease associated with the following? • Down syndrome – 50%; Ventricular septal defect • Noonan syndrome – 50%; Pulmonary stenosis, HCM • Turner syndrome – 33%; Coarctation, aortic valve disease, PAPVR • Williams syndrome – 33%; supravalvar aortic stenosis • Di. George syndrome – 35%; conotruncal abnormalities (i. AA type B, TA, To. F) ational Capital Military Children’s Center National

Chest pain in Children • Identify the false statement – Most chest pain is musculoskeletal or idiopathic in origin – An echocardiogram is a reasonable tool in the assessment of most chest pain – A history of structural heart disease or previous Kawasaki disease should raise concern – EKG abnormalities are common in children with chest pain ational Capital Military Children’s Center National

Neonatal cyanosis • What is the hyperoxia challenge test? • Below what value of pa. O 2 suggests congenital heart disease as cause for cyanosis? – 150 mm. Hg • Which parameter on the ABG is strongly suggestive of pulmonary disease? – Elevation in pa. CO 2 ational Capital Military Children’s Center National

Congestive heart failure • Which of the following are the best tests for determining if CHF is present? – Echocardiogram – Electrocardiogram – History – Serum sodium and creatinine – Physical examination – Chest roentgenogram ational Capital Military Children’s Center National

CHF and shock • A 4 day infant presents to the emergency room tachypnea, tachycardia, perfusing poorly, and showing signs of obtundation. • Physical examination shows a quiet precordium except for tachycardia, clear lungs, poor distal pulses and capillary refill, and marked hepatomegaly. • What type of structural heart diseases could this presentation be consistent with? ational Capital Military Children’s Center National

Ductal dependent left-sided obstruction • Initial physiology VC PV RA RV LV PA Ao Hypoplastic Left Heart Syndrome

Ductal dependent left-sided obstruction • The ductus closes…. VC PV RA RV LV Critical Aortic Stenosis Critical Coarctation PA Ao

CHF and shock • A 1 month infant presents to your clinic with parental concerns of poor feeding, diaphoresis and tachypnea. • Physical examination shows a grade III harsh systolic ejection murmur, tachypnea to 70 bpm, normal distal pulses and capillary refill, and marked hepatomegaly. • What structural heart diseases could this presentation be consistent with? ational Capital Military Children’s Center National

Left to right shunt with falling PVR: eg. VSD at birth VC PV RA RV LV PA Ao ational Capital Military Children’s Center National

VSD physiology at one month of age VC PV RA RV LV PA Ao ational Capital Military Children’s Center National

CHF: treatment with digoxin • 4 month old on digoxin whose father can’t remember how many doses of digoxin he administered. Which is the earliest sign of digoxin toxicity? – Atrioventricular block – Sinus bradycardia – Feeding intolerance – Supraventricular arrhythmias ational Capital Military Children’s Center National

Congenital Heart Disease National Capital Military Children’s

Ventricular Septal Defect • Most common congenital heart defect • Most common in chromosomal abnormalities • Identified by location – Membranous septum – Inlet septum – Muscular septum – Supracristal ational Capital Military Children’s Center National

Board question • 6 week old with a known VSD and worsening failure. Which of the following statements is MOST appropriate? : – Oxygen therapy can be deleterious – Caloric needs are reduced – Rales often accompany congestive heart failure in infants – Afterload reduction is contraindicated – IV furosemide is deleterious ational Capital Military Children’s Center National

Patent ductus arteriosus • Presentation depends upon degree of left-toright shunting • Can cause significant volume overload of the heart, like a VSD ational Capital Military Children’s Center National VC PV RA RV LV PA Ao

Patent ductus arteriosus • Presentation depends upon degree of left-toright shunting • Can cause significant volume overload of the heart, like a VSD ational Capital Military Children’s Center National VC PV RA RV LV PA Ao

The physical finding that is the hallmark of the PDA is… • • • Fixed S 2 split S 3 gallop Continuous murmur at LUSB Diastolic murmur at the LLSB S 4 ational Capital Military Children’s Center National

Atrial septal defect • This includes any defect in the atrial septum to include: – Secundum ASD – Primum ASD – Sinus venosus ASD • More common in females • Occasionally inherited as in Holt-Oram and Klippel-Feil syndromes ational Capital Military Children’s Center National

A 14 year female is found to have a loud murmur on examination. Which of the following would NOT suggest a ASD? • A fixed S 2 split • Right ventricular enlargement on EKG • Increased pulmonary vascular markings on CXR • A diastolic murmur over the right lower sternal border ational Capital Military Children’s Center National

ASD physiology VC PV RA RV LV PA Ao ational Capital Military Children’s Center National

Coarctation of the aorta • Obstruction of the aorta just distal to the left subclavian artery in proximity of the PDA insertion site • More common in males • Most common lesion found in Turner syndrome • Presentation may range from an asymptomatic murmur to cardiogenic shock ational Capital Military Children’s Center National

A 5 year girl is being evaluated for short stature. Which of the following would NOT suggest the presence of a coarctation of the aorta? • Systolic hypertension in the arms • A absent or markedly diminished femoral pulse • Left ventricular hypertrophy on EKG • Right axis deviation on EKG • Rib notching on CXR ational Capital Military Children’s Center National

What does rib notching look like? ational Capital Military Children’s Center National

Tetralogy of Fallot • The most common cyanotic heart disease diagnosed outside of the neonatal period • Features – Components of valvar, subvalvar and supravalvar pulmonary stenosis – Large VSD – Right ventricular hypertrophy – Overriding aorta • CXR classically describes the boot-shaped heart ational Capital Military Children’s Center National

Physiology ational Capital Military Children’s Center National

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Hypercyanotic spells can be induced by all of the following except: • • • Pain Induction of anesthesia Dehydration Iron Deficiency Squatting ational Capital Military Children’s Center National

d-Transposition of the Great Arteries • The most common cyanotic heart disease identified in neonatal period • Profoundly cyanotic and acidotic • Physical finding often lack specific cardiac findings such as a murmur ational Capital Military Children’s Center National

Physiology ational Capital Military Children’s Center National

A 3 hour old infant is found profoundly cyanotic and in shock. Prostaglandin E 1 is begun. The side effects of PGE 1 include… • • • Fever Apnea Hypotension Flushing Seizures Male pattern baldness ational Capital Military Children’s Center National

Acquired Heart Disease National Capital Military Children’s

An 4 -year-old girl is seen 10 days following an upper respiratory infection. Her knees and ankles were swollen and painful, and her temperature is 104 F degrees. The cardiac examination is significant for grade 6/6 systolic murmur at the apex. These findings are consistent with which of the following: • • • Acute rheumatic fever Septic arthritis Juvenile rheumatoid arthritis Pericarditis Kawasaki disease ational Capital Military Children’s Center National

A 2 -year-old boy presents with fever of 5 days duration, swollen hands and feet, strawberry tongue, maculopapular rash, and conjunctivitis. The most likely diagnosis is: • • • Lyme disease Kawasaki disease Stevens-Johnson syndrome Mononucleosis Acute rheumatic fever ational Capital Military Children’s Center National

A 12 -year-old boy presents with a 24 hour history of sharp, pleuritic chest pain that is worse in the supine position. His fever is 101. 3 F and pericardial friction rub. The most likely diagnosis is: • • • Musculoskeletal chest pain Pericarditis Bacterial endocarditis Mycoplasma pneumonia Pulmonary embolus ational Capital Military Children’s Center National

Infective endocarditis • Causative bugs – Streptococcal viridans – Staphylococcal aureus – Staphylococcal epidermidis – Fungal pathogens – HACEK group ational Capital Military Children’s Center National

Classic physical findings • Janeway lesions • Osler nodes • Roth spots ational Capital Military Children’s Center National

Acute Rheumatic Fever • Delayed sequelae of Grp A streptococcal infection of the pharynx – Streptococcal infections of the skin and other sites do NOT lead to ARF • Not on boards: belongs to Grp A type 18 • Typically follows 1 to 5 weeks after pharyngitis ational Capital Military Children’s Center National

Jones Criteria • J oints…polyarticular migratory arthritis • O bvious…carditis • N odules…subcutaneous, painless • E rythema marginatum • S yndenham chorea Two major; one major and two minor PLUS evidence of an antecedent Streptococcal infection…ASO or throat culture ational Capital Military Children’s Center National

Kawasaki Disease • Epidemiological case definition (classic clinical criteria)* – Presence of at least 4 principal features: 1. 2. 3. 4. 5. Changes in extremities Polymorphous exanthem Conjunctival injection, bilateral Changes in lips and oral cavity Cervical lymphadenopathy – Fever persisting at least 5 days – Exclusion of other diseases with similar findings ational Capital Military Children’s Center National

Kawasaki disease • Associated findings may include – – – – Urethritis with sterile pyuria (70%) Aseptic menigitis (50%) Hepatitis (30%) Arthralgia/arthritis (10 -20%) Hydrops of the gallbladder (15%) Myocarditis (5%) Uveitis • Typical laboratory findings – – – Leukocytosis Anemia Acute phase reactants Thrombocytosis Transaminemia CSF pleocytosis ational Capital Military Children’s Center National

Coronary artery aneurysms • Occurs in approximately 20% of untreated children • Occurs in less than 5% of treated children • Echocardiography provides the gold standard diagnosis • Treatment – IVIG, 2 grams per kilogram – Aspirin, 80 -100 mg/kg/day ational Capital Military Children’s Center National

Pericarditis • Acute inflammation of the pericardium • Typically idiopathic • Other etiologies include: – Viral infection – Acute rheumatic fever – Bacteria – TB ational Capital Military Children’s Center National

Treatment of pericarditis • Generally symptomatic • Non-steroidal anti-inflammatory medications for pain, fever, and evidence of inflammation • Pericardiocentesis for evidence of pending tamponade • Infectious pericarditis is treated as appropriate ational Capital Military Children’s Center National

Disorders of Cardiac Rate and Rhythm National Capital Military Children’s

There are no 12 - or 15 -lead EKGs on the general pediatric boards National Capital Military Children’s

A 6 -week-old infant is brought to your office for a well-baby visit. A rapid heart rate is noted. EKG shows a regular narrow QRS complex tachycardia at 260 bpm. Appropriate therapy could include all of the following, except: • • Intravenous administration of adenosine Ice over the forehead Intravenous administration of verapamil Application of gentle abdominal pressure to mimic a Valsalva maneuver ational Capital Military Children’s Center National

A 30 -year-old woman who has active systemic lupus erythematosus is pregnant. Of the following abnormalities of conduction, the one most likely to occur in her infant is: • • • Atrial flutter First-degree heart block Supraventricular tachycardia Third-degree heart block Ventricular tachycardia ational Capital Military Children’s Center National

A 16 -year-old athlete has a syncopal episode immediately following a high school basketball game. The EKG shows a QTc of 0. 52 sec. Which of the following family members would you recommend have a screening EKG: • • • All first degree relatives Brothers and male first-cousins Sisters and female cousins Father and both grandfathers Mother and both grandmothers ational Capital Military Children’s Center National

Syncope • Abrupt short loss of consciousness with loss of postural tone. • Most common form is known as “vasovagal”, “neurocardiogenic”, “neurallymediated syncope”, “common faint” • SVT rarely causes syncope alone. • Consider bradycardia, VT, ARVD, long QT • History, physical examination and EKG are imperative in evaluation of syncope ational Capital Military Children’s Center National

Long QT syndrome • Associated with syncope and sudden death • Represents a family of at least 6 different loci: LQT 1, LQT 2, LQT 3, etc • Typically autosomal dominant; autosomal recessive is less common • Jervell and Lange-Nielsen syndrome is associated with bilateral neurosensory hearing loss • Patients should avoid swimming, competitive sports, and high-risk occupations; and drugs associated with QT prolongational Capital Military Children’s Center National

Questions? ? ? National Capital Military Children’s