• 4 year old female presents with fast breathing, “grunting, cough ! " days • seen # days earlier and started on steroids and bronchodilators • initial vitals$ %& '"(, )P '((*+", && -, T ./"
• ' month old with irritability, poor feeding 0fatigues1, failure to thrive, fast breathing • no fever or runny nose • physical e!am$ %& '-(, && -( with minimal respiratory distress, gallop rhythm, rales
2nfant Cardiac 3isease eading to E& Presentation • Congenital
Congestive %eart 9ailure • the physiologic state in which cardiac output is unable to meet tissue metabolic demands 0&osen1 • C: ; %& ! <= • <= dependent upon preload, afterload, contractility
C%9 > Presentation • infants$ irritable, poor feeding 0early fatigue1, failure to thrive, respiratory symptoms • always consider in patients with respiratory symptoms 7 often misdiagnosed as respiratory illness * infection
C%9 > Etiology •
2ncreased Preload 7 to & shunts 0=<3, P35, 5= fistula1 7 severe anemia
• presents at ' day 0congenital1 0 congenital1 7 P35 in premature infants
• presents in first month 0congenital1 7 %P=, aortic stenosis, coarctation, =<3 presents later
• presents later 0ac6uired1
7 myocarditis, myocarditis, cardiomyopa cardiomyopathy thy 0dilated or hypertrophi hypertrophic1, c1, <=T, severe anemia, rheumatic fever
8yocarditis • leading cause of dilated cardiomyopathy and one of the most common causes of C%9 in children • etiology$ idiopathic, viral, bacterial, parasitic • hallmarA is C%9 • failure to respond to bronchodilators in wheeBing child • treatment includes inotropes, afterload reduction, diuretics, antibiotics, antivirals
Pericarditis • sharp stabbing precordial pain • worse with supine and better leaning forward • no sensory innervation of the pericardium 7 pain referred from diaphragmatic and pleural irritation
Pericarditis • usually a benign course • virulent bacteria 0%/ flu, E/ coli1 can cause constrictive pericarditis and subse6uent constrictive tamponade 7 may need urgent pericardiocentesis • uncomplicated pericarditis usually responds to rest and anti>inflammatori anti>inflammatories es
Chest Pain • 4 of children will have a cardiac origin • remainder$ 8<@, pulmonic 0asthma, bronchitis, pneumonia1, G2 • Cardiac causes$ myocarditis, m yocarditis, pericarditis, structural abnormalities such as congenital heart disease or hypertrophic cardiomyopathy
• '4 year old male collapses at school while in class • non>responsive for one minute • feels fine in the department • 5pproachD
<yncope • #(>"( of adolescents e!perience at least one episode of syncope 7 most cases benign
• :utflow obstruction • 8yocardial 3ysfunction • cardiac syncope often precedes future sudden cardiac death
<udden Cardiac 3eath • includes those causes that directly relate to cardiovascular dysfunction • one third of all sudden deaths
<udden Cardiac 3eath • Etiology 7 7 7 7 7 7 7 7 7
myocarditis cardiomyopathy (hypertrophic) cyanotic and noncyanotic congenital heart disease valvular heart disease congenital complete heart blocA P long FT syndrome 8arfan syndrome coronary artery disease
7 anomalous coronary arteries
&isA 9actors for <erious Cause of <yncope • history of cardiac disease in patient • 9% of sudden death, cardiac disease, or deafness • recurrent episodes • recumbent episode • e!ertional • prolonged loss of consciousness • associated chest pain or palpitations • medications that can alter cardiac conduction
hat to looA for in the 3epartment$ • ong FT syndrome
E@G
7 congenital or ac6uired 7 get paro!ysmal v tach with torsades de pointes 7 congenital long FT associated with hypertrophic cardiomyopathy 7 long FT defined as corrected FT longer than (/44 s 7 T wave alternans sometimes present 7 can have normal ECG in the department 7 two clinical syndromes not associated with structural heart disease$ &omano>ard and ervell>ange> ?ielsen
:ther dysrhythmias • P and other <=THs • 5 5= = blocA 7 usually ac6uired, rarely congenital
• <icA sinus syndrome
2diopathic %ypertrophic • aAa 2%<< Cardiomyopathy • both a fi!ed and dynamic subvalvular s ubvalvular obstruction • characteriBed by ventricular hypertrophy with principle involvement of the ventricular septum • associated with long FT • autosomal dominant • often presents with e!ertional syncope • '( year mortality is "( for children diagnosed by age '4
:ther structural cardiac diseases
• dilated cardiomyopathy
7 usually secondary to myocarditis 7 syncope and death secondary to ventricular dysrhythmias or severe myocardial dysfunction
• coronary artery anomalies 7 e!ertional syncope or sudden death 7 aberrant artery passes between aorta and pulmonary artery
• # weeA old infant brought in by parents with difficulty breathing • %& '+(, )P "(*P, && +(, T ./" • history and physical • investigations • repeat vitals$ %& (, no )P, && '# • “definitive treatment$
• 4 year old male presents with # weeAs history of cough, fast breathing, fatigue, decreased e!ercise tolerance, “puffy eyes • :n e!am$ tachypneic, moderate respiratory distress, :# sats I#, bilateral cracAles
• - month male presents with failure to thrive, fast breathing, blue lips • :n e!am tachypnea but no respiratory distress, lips and e!tremities blue, o!ygen saturations .(
Congenital %eart 3isease • 9etal to ?eonatal Circulation
5natomic ClassificationJ ClassificationJ 4 • Right to Left Shunt
groups • Stenotic
7 Tetralogy of 9allot 7 Tr Transposition ansposition of of the Great 5rteries 7 Tricuspid 5tresia
7 5ortic valve valv e stenosis 7 Pulmonic valve stenosis 7 5ortic coarctation coarctatio n • Mixing
• Left to Right Shunt
7 5<3 7 =<3 7 P35
7 Truncus 7 Total 5nomalous Pulmonary =enous &eturn 7 %ypoplastic left heart syndrome left heart
C%3 Classified as Cyanotic vs/ 5cyanotic 5cyano tic
• Cyanotic 0& to shunt and mi!ing lesions1 7 tetralogy of 9allot 7 transposition of great vessels 7 tricuspid atresia 7 total anomalous pulmonary venous return 7 truncus arteriosus 7 hypoplastic left heart syndrome
Cyanosis • Classified as central or peripheral • Central cyanosis 0always abnormal1 7 mucous membranes, trunA, e!tremities 7 classified as cardiac 0& to shunt1 or pulmonary
• Peripheral cyanosis 0acrocyanosis1 7 no involvement of mucous membranes 7 involv involves es hands, feet, circumoral area 7 common in neonates from vasomotor instability instability 7 C%9, P=3, shocA, cold e!tremities
Tetralogy of 9allot 7 the classic cyanotic• lesion &= outflow obstruction • &=% • =<3 • overriding aorta • CK& reveals boot shaped heart with decreased pulmonary blood flow
• # month old female with Anown tetralogy of 9allot brought in with #4 hour history of vomiting and diarrhea • :n e!am$ moderate dehydration • during 2= attempts patient becomes irritable and cyanotic
Treatment of Tet <pell • 6uiet, calm environment • Anee>chest or s6uatting position 7 increases afterload thus decreasing & to shunting
• :!ygen • 8orphine 7 to treat hyperpnea and decrease systemic catecholamines
• 8anual e!ternal aortic compression below level of renal arteries • Propranolol 7 to blocA beta receptors r eceptors in infundibulum therefore lessening &= outflow obstruction
Consider • consider small volume challenge 0">'( cc*Ag1 to increase preload and reduce dynamic outflow obstruction • D?a%C: for correction of acidosis • may need general anesthesia if severe and*or prolonged spell • interim prophylactic treatment with propranolol while awaiting surgery
5cyanotic Congenital %eart 3isease • to & shunts
• stenotic lesions
=<3 •Most common congenital lesion •Large VSD’s may be silent and become symptomatic in first few weeks as pulmonary resistance ↓ •SOB and diaphoresis w feeds •oor weight gain •Systolic murmur •!"# demonstrates !
%$
• # weeA old infant brought in by parents with difficulty breathing • %& '+(, )P "(*P, && +(, T ./" • history and physical • investigations • repeat vitals$ %& (, no )P, && '# • “definitive treatment$
= :utflow :bstruction • 5ortic coarctation • %ypoplastic left heart syndrome • 5ortic stenosis 0presents later1 • &osen$ “any neonate in shocA that does not respond to fluids or pressors has = outflow obstruction until proven otherwise • complete obstruction incompatible with life
unless there is shunting
Coarctation of the aorta •Most often distal to L subcla&ian •!an be diagnosed anytime • 'eonates present as acutely ill( gray shocky )from D* closure+ •Systolic murmur at the back •$epatomegaly •Diminished femoral pulses •B difference difference b,t arms and legs •!"# demonstrates !$% •-reatment of !$% •rostaglandin ./
3uct 3ependant esions • 3uct needed to perfuse lungs or periphery • ungs 7 Tetralogy of 9allot, transposition of great arteries, tricuspid or pulmonary atresia • a patent ductus arteriosus results in preserved pulmonary blood flow
• Periphery
7 5ortic coarctation 0severe1 and %ypoplastic left heart
Treatment of acute decline in patients with ductal dependant lesions
• :pen the closed duct • Prostaglandin E' (/' ug*Ag*min infusion • reduce dosage as perfusion and colour return • &osen$ “any infant in the first weeA of life with decreased perfusion, hypotension, or acidosis should be considered a candidate for PGE' administration
hat do you need to Anow about PGE D • it functions by dilating vascular smooth muscle, both systemically and in the pulmonary vascular bed • itHs use in C%3 ptsH is to maintain patency of the P35, whether to maintain P)9 or to maintain systemic blood flow past a
Classification &eview • pinA child in respiratory distress suggests acyanotic chd 0 to & shunt, coarct, aortic stenosis1 • blue cyanotic child in little respiratory distress suggests & to shunt or mi!ing lesions • gray, shocAy baby suggests outflow tract obstruction
• Treatment 7 Epinephrine 7 5tropine if Anown Anown vagally mediated or heart heart blocA
Congenital )radyarrhythmias • complete 5= blocA 7 autoimmune inMury to fetal conduction system secondary to maternal autoimmune disease 7 atropine, isoproteronol, epinephrine may be tried temporarily prior to pacing
Tachyarrhythmias • <upraventr <upraventricular icular Tachycardia 7 re>entrant with accessory pathway 05= nodal or P1 7 re>entrant without accessory pathway 0re> entry occurs within sinus node or within atrium1 7 ectopic
• nonspecific presentations in infants
8urmurs • 5reas 7 aortic$ & #nd intercostal space 7 pulmonic$ #nd intercostal space 7 mitral$ ape! 7 tricuspid and =<3$ lower lower sternal border
• Pathologic 7 diastolic, holosystolic, late systolic, continuous
2nnocent %eart 8urmurs • %istory 7 normal growth and develop development, ment, normal e!ercise tolerance 7 no history of cyanosis
• Physical E!amination 7 Grade 22 or less, localiBed 7 varies with position 0decreased with upright posture1 7 normal precordium 7 normal pulses
• ab 7 normal E@G, normal CK&
innocent murmurs • <tillHs 7 short eMection systolic murmur 7 musical or vibratory 6uality 7 heard best between ape! and left sternal border
• physiologic pulmonary flow murmur 7 harsh, located at pulmonic area
• peripheral arterial stenosis 7 low>intensity low>intensity systolic eMection murmur best heard in a!illa and bacA
• + year old male presents with fever, arthralgias • mother mentions that he had a sore throat weeAs ago for a few days with spontaneous resolution • a throat swab was done and positive for G5< but patient better so did not taAe the prescribed antibiotics
5cute &heumatic 9ever • school aged children • associated with certain strains of Group 5 beta> hemolytic streptococcal infections • the streptococcal organism stimulated antibody production to host tissues 7 CT of heart, Moints, C?<, subcutaneous tissues, sAin
• carditis is an endomyocarditis with valvulitis involving mitral and aortic valves • # to - weeAs post streptococcal pharyngitis
ones Criteria • 8aMor 7 carditis • new or changing murmur • cardiomegaly, C%9 • pericarditis
• 8inor 7 fever 7 arthralgia 7 history of previous 5&9 7 elevated E<&, C&P 7 prolonged P& on E@G 7 &ising titer of antistreptococcal antibodies
E& Treatme Treatment nt • management of complicating features of carditis 0C%91 7 significant carditis or C%9 managed with glucocorticoids
• high>dose 5<5 .">'(( mg*Ag*day • pencillin • long term management of rheumatic heart disease
Pediatric E@GHs General Principles • &= 3ominance at birthJ gradually changes to = dominan dominance ce • a!is up to N'+( in normal newborn • T waves negative in right precordial leads until adolescence 0e!cept they are upright in first weeA of life1