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critical heart disease
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FIVE CRITICAL CARDIAC PROBLEMS THAT ARE
FIVE CRITICAL CARDIAC PROBLEMS THAT AREPROBLEMS THAT ARE
IMPORTANT TO DIAGNOSEPROBLEMS THAT ARE
IMPORTANT TO DIAGNOSE
A i A d f P di iA i A d f P di iAmerican Academy of Pediatrics October 2008
American Academy of Pediatrics October 2008
Stuart Berger, MDStuart Berger, MD
Faculty Disclosure SlideFaculty Disclosure Slide
Nothing to disclose Nothing to disclose
Five Critical Cardiac ProblemsFive Critical Cardiac Problems
1. Critical left heart obstruction in the newborn2. Cyanotic congenital heart disease with minimal 1. Critical left heart obstruction in the newborn2. Cyanotic congenital heart disease with minimal y g
cyanosis 3. Dilated cardiomyopathy
y gcyanosis
3. Dilated cardiomyopathy4. Long QT syndrome and other channelopathies5. Chronic tachycardia and tachycardia-induced 4. Long QT syndrome and other channelopathies5. Chronic tachycardia and tachycardia-induced y y
cardiomyopathyy y
cardiomyopathy
Format for DiscussionFormat for Discussion
For each entity Describe anatomy and physiology
For each entity Describe anatomy and physiology Describe the presentation(s) Describe therapy/interventions and the usual Describe the presentation(s) Describe therapy/interventions and the usual
handling of the entity Describe what may happen if not diagnosed in a
timely fashion
handling of the entity Describe what may happen if not diagnosed in a
timely fashiontimely fashion Describe clues/tips to enable timely
discovery/diagnosis
timely fashion Describe clues/tips to enable timely
discovery/diagnosisdiscovery/diagnosisdiscovery/diagnosis
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Anatomy and physiology Typical lesions include hypoplastic left heart syndrome,
Anatomy and physiology Typical lesions include hypoplastic left heart syndrome,
critical coarctation of the aorta/interrupted aortic arch and critical aortic stenosis Key issue is systemic output; each entity is dependent
critical coarctation of the aorta/interrupted aortic arch and critical aortic stenosis Key issue is systemic output; each entity is dependent Key issue is systemic output; each entity is dependent
upon ductal patency which is required for systemic output
Key issue is systemic output; each entity is dependent upon ductal patency which is required for systemic output Perfusion of descending aorta (coarctation) Perfusion of both ascending and descending aorta (HLHS) Perfusion of descending aorta (coarctation) Perfusion of both ascending and descending aorta (HLHS)
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Presentation Varies with the lesion as well as with the state
Presentation Varies with the lesion as well as with the state
of the ductus All signs and symptoms are related to low
di t t t t d d t b
of the ductus All signs and symptoms are related to low
di t t t t d d t bcardiac output; accentuated as ductus becomes more restrictive With closure of the ductus which usually
cardiac output; accentuated as ductus becomes more restrictive With closure of the ductus which usuallyWith closure of the ductus, which usually
occurs within a few days after birth CARDIOGENIC SHOCK
With closure of the ductus, which usually occurs within a few days after birth CARDIOGENIC SHOCK
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Presentation Critical coarctation of the aorta/interrupted
Presentation Critical coarctation of the aorta/interruptedCritical coarctation of the aorta/interrupted
aortic arch May not have many findings while ductus is patent
Critical coarctation of the aorta/interrupted aortic arch May not have many findings while ductus is patent As ductus restricts, decrease in lower extremity
pulses, tachypneaU ll t ti l t ti i t i
As ductus restricts, decrease in lower extremity pulses, tachypneaU ll t ti l t ti i t i Usually not cyanotic unless presentation in extremis as ductus closes Usually not cyanotic unless presentation in extremis
as ductus closes
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Presentation Hypoplastic left heart syndrome
Presentation Hypoplastic left heart syndrome Many are diagnosed in-utero Though desaturation is usually present, hard to
detect cyanosis; screen with pulse oximetry?
Many are diagnosed in-utero Though desaturation is usually present, hard to
detect cyanosis; screen with pulse oximetry?detect cyanosis; screen with pulse oximetry? Tachypnea is common; becomes more pronounced
as ductus restricts
detect cyanosis; screen with pulse oximetry? Tachypnea is common; becomes more pronounced
as ductus restricts Other signs of CHF: hepatomegaly, decreased
pulses (upper and lower), gallop rhythm Progression to cardiogenic shock
Other signs of CHF: hepatomegaly, decreased pulses (upper and lower), gallop rhythm Progression to cardiogenic shockg gg g
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Therapies/Intervention Immediate transfer to a tertiary care facility
Therapies/Intervention Immediate transfer to a tertiary care facilityImmediate transfer to a tertiary care facility Start prostaglandin as soon as lesion is
suspected, even if you are sure and your degree
Immediate transfer to a tertiary care facility Start prostaglandin as soon as lesion is
suspected, even if you are sure and your degree p , y y gof suspicion is high All of the lesions require an intervention in
p , y y gof suspicion is high All of the lesions require an intervention in q
order to assure adequacy of systemic outputq
order to assure adequacy of systemic output
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Optimization of detection and minimization of neonates slipping through the cracks Optimization of detection and minimization
of neonates slipping through the cracks Routine fetal echocardiography Routine discharge from nursery later Routine fetal echocardiography Routine discharge from nursery later Routine upper and lower extremity pulse
oximetry prior to discharge from newborn nursery
Routine upper and lower extremity pulse oximetry prior to discharge from newborn nurserynursery Routine upper and lower extremity blood
pressure prior to discharge
nursery Routine upper and lower extremity blood
pressure prior to dischargep p gp p g
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Optimization of detection and minimization of neonates slipping through the cracks Optimization of detection and minimization
of neonates slipping through the crackspp g g More vigilant Follow-up visit with pediatrician
pp g g More vigilant Follow-up visit with pediatricianFollow up visit with pediatrician Frequency of these entities - Does it justify
more vigilance and/or more intense screening?
Follow up visit with pediatrician Frequency of these entities - Does it justify
more vigilance and/or more intense screening?g gg g
I Critical left heart obstruction inI Critical left heart obstruction inI. Critical left heart obstruction in the newborn
I. Critical left heart obstruction in the newborn
Clues to a potential diagnosis Tachypnea and/or tachycardia
Clues to a potential diagnosis Tachypnea and/or tachycardia Active precordium +/- gallop rhythm +/-
murmur Active precordium +/- gallop rhythm +/-
murmur +/- Cyanosed appearance General decrease in pulses or perfusion
abnormalities (cool clammy poor capillary
+/- Cyanosed appearance General decrease in pulses or perfusion
abnormalities (cool clammy poor capillaryabnormalities (cool, clammy, poor capillary refill) Differential pulses or perfusion
abnormalities (cool, clammy, poor capillary refill) Differential pulses or perfusionDifferential pulses or perfusionDifferential pulses or perfusion
When in doubt, targeted BP and POX measurements indicated
II Cyanotic congenital heartII Cyanotic congenital heartII. Cyanotic congenital heart disease with minimal cyanosisII. Cyanotic congenital heart
disease with minimal cyanosis Anatomy quite variable Many lesions can fit in this category Anatomy quite variable Many lesions can fit in this categoryMany lesions can fit in this category May be ductal dependent but not all
cyanotic lesions necessarily are
Many lesions can fit in this category May be ductal dependent but not all
cyanotic lesions necessarily arecyanotic lesions necessarily arecyanotic lesions necessarily are
II Cyanotic congenital heartII Cyanotic congenital heartII. Cyanotic congenital heart disease with minimal cyanosisII. Cyanotic congenital heart
disease with minimal cyanosis Cyanosis with
obstruction to PBFT l f F ll
Cyanosis with obstruction to PBF
T l f F ll
Cyanosis because of mixing issues Cyanosis because of
mixing issues Tetralogy of Fallot Pulmonary atresia with
VSD
Tetralogy of Fallot Pulmonary atresia with
VSD
Transposition of the great arteries Truncus arteriosus
Transposition of the great arteries Truncus arteriosus Pulmonary atresia with
IVS Tricuspid atresia
Pulmonary atresia with IVS Tricuspid atresia
Truncus arteriosus Total anomalous
pulmonary venous
Truncus arteriosus Total anomalous
pulmonary venous Tricuspid atresia Critical pulmonary
stenosis
Tricuspid atresia Critical pulmonary
stenosis
p yconnectionp yconnection
Tetralogy of FallotTetralogy of Fallot Variable degree of RVOT
obstruction and R to L shunt through VSD
Variable degree of RVOT obstruction and R to L shunt through VSDshunt through VSD
Variable degree of cyanosisT d t b i
shunt through VSD Variable degree of
cyanosisT d t b i Tends to be progressive
Loud murmur present at birth
Tends to be progressive Loud murmur present at
birth May be ductal dependent
if RVOT obstruction is severe at birth; therefore
May be ductal dependent if RVOT obstruction is severe at birth; therefore ;may become quite cyanotic if PDA closes
;may become quite cyanotic if PDA closes
Pulmonary Atresia with IVSPulmonary Atresia with IVS No antegrade flow from
RV to PA Ductus is only source of
No antegrade flow from RV to PA
Ductus is only source of Ductus is only source of PBF
Cyanosis may be minimal til PDA b
Ductus is only source of PBF
Cyanosis may be minimal til PDA buntil PDA becomes
restrictive When ductus closes,
until PDA becomes restrictive
When ductus closes, presents in extremis
Murmur may be presentpresents in extremis
Murmur may be present
Transposition of the GreatTransposition of the GreatTransposition of the Great Arteries
Transposition of the Great Arteries
Variable degree of cyanosis depending on amount of mixing (ASD,
Variable degree of cyanosis depending on amount of mixing (ASD,amount of mixing (ASD, VSD, PDA)
With IVS may be extremely desaturated
amount of mixing (ASD, VSD, PDA)
With IVS may be extremely desaturatedextremely desaturated
Typically murmur absent but very active
di
extremely desaturated Typically murmur absent
but very active diprecordium
PDA will augment mixing but may require BAS
precordium PDA will augment mixing
but may require BAS
Truncus ArteriosusTruncus Arteriosus Complete mixing at level
of VSD and at the level of the great arteries
Complete mixing at level of VSD and at the level of the great arteriesthe great arteries
Cyanosis may initially be minimal, babies will become progressively
the great arteries Cyanosis may initially be
minimal, babies will become progressivelybecome progressively tachypneic and desaturated with poor growth
become progressively tachypneic and desaturated with poor growthgrowth
Murmur may be present at birth, to and fro?
growth Murmur may be present at
birth, to and fro? Not ductal dependent Not ductal dependent
Total Anomalous PulmonaryTotal Anomalous PulmonaryTotal Anomalous Pulmonary Venous Connection
Total Anomalous Pulmonary Venous Connection
Total Anomalous PulmonaryTotal Anomalous PulmonaryTotal Anomalous Pulmonary Venous Connection
Total Anomalous Pulmonary Venous Connection
Pulmonary veins do not drain normally; sites of abnormal drainage are
Pulmonary veins do not drain normally; sites of abnormal drainage areabnormal drainage are many
Veins can be obstructed in their anomalous drainage
abnormal drainage are many
Veins can be obstructed in their anomalous drainagetheir anomalous drainage
Obligate R to L shunt at atrial level cyanosistheir anomalous drainage
Obligate R to L shunt at atrial level cyanosis
If veins are obstructed, can present in extremis
This is not a ductal-
If veins are obstructed, can present in extremis
This is not a ductal-dependent lesion, PDA harmful?dependent lesion, PDA harmful?
Cyanotic Congenital Heart DiseaseCyanotic Congenital Heart Disease
Therapy or the usual scenario for these abnormalities
TOF Ti i f i i d d d f
Therapy or the usual scenario for these abnormalities
TOF Ti i f i i d d d f TOF - Timing of intervention dependent upon degree of RVOT obstruction Pulmonary atresia - Intervention required immediately
TOF - Timing of intervention dependent upon degree of RVOT obstruction Pulmonary atresia - Intervention required immediately y q y
after birth; PGE1 needed TGA - If IVS, intervention typically required within
first few weeks of life; may require BAS; PGE1
y q yafter birth; PGE1 needed TGA - If IVS, intervention typically required within
first few weeks of life; may require BAS; PGE1first few weeks of life; may require BAS; PGE1 Truncus - Intervention typically first few weeks,
beware of interrupted aortic arch as well as DiGeorge TAPVC I t ti i bl b t ll
first few weeks of life; may require BAS; PGE1 Truncus - Intervention typically first few weeks,
beware of interrupted aortic arch as well as DiGeorge TAPVC I t ti i bl b t ll TAPVC - Intervention variable but usually sooner rather than later; if obstruction must intervene urgently TAPVC - Intervention variable but usually sooner
rather than later; if obstruction must intervene urgently
Cyanotic Congenital Heart DiseaseCyanotic Congenital Heart Disease
Optimizing detection of cyanotic congenital heart disease - Clues to diagnosis
C i if i i l b l h bi
Optimizing detection of cyanotic congenital heart disease - Clues to diagnosis
C i if i i l b l h bi Cyanosis if even minimal may be an early harbinger Presence of a murmur may be a tip-off; although
absence of a murmur not necessarily reassuring (TGA)
Cyanosis if even minimal may be an early harbinger Presence of a murmur may be a tip-off; although
absence of a murmur not necessarily reassuring (TGA)y g ( ) An active precordium is a non-specific but important
sign Tachypnea with or without poor feeding with or
y g ( ) An active precordium is a non-specific but important
sign Tachypnea with or without poor feeding with or Tachypnea with or without poor feeding, with or
without tachycardia Is there a role for routine newborn pulse oximetry?
Tachypnea with or without poor feeding, with or without tachycardia Is there a role for routine newborn pulse oximetry? Cost, cost-effectiveness, resource availability, etc. Cost, cost-effectiveness, resource availability, etc.
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Anatomy and physiology Systolic dysfunction of left ventricle (and right
Anatomy and physiology Systolic dysfunction of left ventricle (and right
ventricle) Over time the left ventricle becomes dilated and
f ti
ventricle) Over time the left ventricle becomes dilated and
f tifunction worsens Dilatation of mitral (and tricuspid) valve
annulus with concomitant AV valve
function worsens Dilatation of mitral (and tricuspid) valve
annulus with concomitant AV valveannulus with concomitant AV valve insufficiencyannulus with concomitant AV valve insufficiency
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Presentation Acute
Presentation AcuteAcute Usually quite sick and presents with signs and
symptoms of acute heart failure/low cardiac output
Acute Usually quite sick and presents with signs and
symptoms of acute heart failure/low cardiac output
Chronic More insidious presentation; also signs of heart
f il b t b ifi i ll i
Chronic More insidious presentation; also signs of heart
f il b t b ifi i ll ifailure but may be more non-specific, especially in the infantfailure but may be more non-specific, especially in the infant
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Acute Respiratory distress
Acute Respiratory distress
Chronic Tachypnea
h di
Chronic Tachypnea
h di Poor perfusion - Cool, clammy, urine output, poor capillary refill
Poor perfusion - Cool, clammy, urine output, poor capillary refill
Tachycardia FTT, irritable, poor
feeding
Tachycardia FTT, irritable, poor
feedingpoor capillary refill Hepatomegaly Active precordium +/-
poor capillary refill Hepatomegaly Active precordium +/-
g Poor perfusion Hepatomegaly
C di l
g Poor perfusion Hepatomegaly
C di lp
gallop rhythmp
gallop rhythm Cardiomegaly Active precordium, gallop rhythm,
Cardiomegaly Active precordium,
gallop rhythm, g p yregurgitant murmurg p yregurgitant murmur
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Presentation/etiologies There are many etiologies
Presentation/etiologies There are many etiologies A very common cause of DCM is myocarditis Acquired
A very common cause of DCM is myocarditis Acquired Often (but not always) preceded by a viral type
illness several weeks prior Viral etiologies are ever-changing
Often (but not always) preceded by a viral type illness several weeks prior Viral etiologies are ever-changingg g g
DCM can be familial; beware of the family history
g g g DCM can be familial; beware of the family
history
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Etiologies Inflammatory/infectious
Etiologies Inflammatory/infectious Metabolic Toxic Metabolic Toxic Neuromuscular Familial Arrhythmogenic
Neuromuscular Familial Arrhythmogenic Arrhythmogenic Idiopathic Other
Arrhythmogenic Idiopathic OtherOtherOther
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Importance of early recognition and therapy Typically the therapy is supportive as there is
Importance of early recognition and therapy Typically the therapy is supportive as there is
currently no specific therapy for myocarditis Generally speaking, for patients with
diti
currently no specific therapy for myocarditis Generally speaking, for patients with
ditimyocarditis: 1/3 survive with full recovery of myocardial
function
myocarditis: 1/3 survive with full recovery of myocardial
function 1/3 survive but require chronic therapy for CHF +/-
heart transplantation 1/3 die
1/3 survive but require chronic therapy for CHF +/-heart transplantation 1/3 die 1/3 die 1/3 die
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Importance of early recognition and therapy Supportive care is critical in order to buy time for
recovery
Importance of early recognition and therapy Supportive care is critical in order to buy time for
recoveryrecovery The earlier the intervention with supportive care, the
better is the chance for survival; better impact on
recovery The earlier the intervention with supportive care, the
better is the chance for survival; better impact on recovery? Diuretics, inodilators, ACE inhibitors, aldactone, beta-
blockers, mechanical ventilation, mechanical cardiac
recovery? Diuretics, inodilators, ACE inhibitors, aldactone, beta-
blockers, mechanical ventilation, mechanical cardiac , ,support The sickest patient, with worst LV function, can
potentially fully recover
, ,support The sickest patient, with worst LV function, can
potentially fully recoverpotentially fully recoverpotentially fully recover
III Dilated cardiomyopathyIII Dilated cardiomyopathyIII. Dilated cardiomyopathyIII. Dilated cardiomyopathy Clues to a potential diagnosis Clues to a potential diagnosis Any presentation with acute collapse Chronic chest pain, abdominal pain, vomiting C di l
Any presentation with acute collapse Chronic chest pain, abdominal pain, vomiting C di l Cardiomegaly Chronic tachycardia especially with other signs of
compensated low cardiac output
Cardiomegaly Chronic tachycardia especially with other signs of
compensated low cardiac output Perfusion: cool, clammy, poor cap refill
Abnormal additional physical findings: very active precordium, gallop rhythm, hepatomegaly, edema
Perfusion: cool, clammy, poor cap refill Abnormal additional physical findings: very active
precordium, gallop rhythm, hepatomegaly, edemap , g p y , p g y, Dont forget FTT, especially in the infant population
p , g p y , p g y, Dont forget FTT, especially in the infant population
Note: No discussion about BP; this is the last sign to change, it is a poor indicator and is a very, very late sign
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Symptoms or signs to beware of (or that might tip you off)
Ch i h i h d i i h d d
Symptoms or signs to beware of (or that might tip you off)
Ch i h i h d i i h d d Chronic wheezing that doesnt improve with standard therapy Chronic FTT
Chronic wheezing that doesnt improve with standard therapy Chronic FTT Abnormal cardiac exam - Active precordium in the
absence of a murmur Persistent abdominal pain/vomiting without an obvious
Abnormal cardiac exam - Active precordium in the absence of a murmur Persistent abdominal pain/vomiting without an obvious Persistent abdominal pain/vomiting without an obvious
cause Chronic tachypnea or chronic tachycardia
Persistent abdominal pain/vomiting without an obvious cause Chronic tachypnea or chronic tachycardia
When in DoubtWhen in DoubtWhen in DoubtWhen in DoubtGet a chest x-ray then
C lt di t iGet a chest x-ray then
C lt di t iConsult your pediatric cardiologist
Consult your pediatric cardiologistca d o og sca d o og s
III. Dilated cardiomyopathyIII. Dilated cardiomyopathy
Consequences of missing the diagnosis or late intervention Consequences of missing the diagnosis or late
intervention Deterioration of clinical condition with worsening of
symptoms and progression to worse cardiogenic shock Presentation in extremis and even death
Deterioration of clinical condition with worsening of symptoms and progression to worse cardiogenic shock Presentation in extremis and even death Presentation in extremis and even death Less likely to recover? More difficult to treat if there is a progression of the
Presentation in extremis and even death Less likely to recover? More difficult to treat if there is a progression of theMore difficult to treat if there is a progression of the
pathophysologyMore difficult to treat if there is a progression of the pathophysology
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Anatomy and physiology Varying degrees of prolongation of the QT
Anatomy and physiology Varying degrees of prolongation of the QT
interval (> 450 msec QTc) Can be familial
interval (> 450 msec QTc) Can be familial May be difficult to diagnose Varying symptomatology including patients
with no symptoms
May be difficult to diagnose Varying symptomatology including patients
with no symptomswith no symptoms Thought be secondary to abnormalities in ion
channels; can be congenital or acquired
with no symptoms Thought be secondary to abnormalities in ion
channels; can be congenital or acquiredchannels; can be congenital or acquired channels; can be congenital or acquired
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Several ion-chanellopathies have now been identified and many can be tested for Several ion-chanellopathies have now been
identified and many can be tested fory More are being discovered over time
y More are being discovered over time
Type of LQTS Chromosome Locus
Mutated Gene Ion Current Affected
LQT1 11p15.5 KVLQT1 KCNQ1 Potassium Q p Q Q(heterozygotes) (IKs)
LQT2 7q35-36 HERG, KCNH2 Potassium (IKr)
LQT3 3p21-24 SCN5A Sodium (INa)
LQT4 4q25-27 ANK2, ANKB Na, K and calciumcalcium
LQT5 21q22.1-22.2 KCNE1(heterozygotes)
Potassium (IKs)
LQT6 21q22.1-22.2 MiRP1, KNCE2 Potassium (IKr)
LQT7Andersons
17q23 KCNJ2 Potassium (IK1)
LQT8Timothys
12q13.3 CACNA1C Calcium (ICa-Lalpha)
JLN1 11p15.5 KVLQT1/KCNQ1(homozygotes)
Potassium (IKs)(homozygotes) (IKs)
JLN2 21q22.1-22.2 KCNE1 (homozygotes)
Potassium (IKs)
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Anatomy and physiology Patient at risk for R on T phenomenon
Anatomy and physiology Patient at risk for R on T phenomenonPatient at risk for R on T phenomenon Ventricular tachycardia, ventricular fibrillation
Torsades de Pointes
Patient at risk for R on T phenomenon Ventricular tachycardia, ventricular fibrillation
Torsades de Pointes Deafness Romano-Ward syndrome (no hearing loss) vs.
Deafness Romano-Ward syndrome (no hearing loss) vs. y ( g )
Jervell and Lange-Nielsen syndrome (hearing loss)y ( g )
Jervell and Lange-Nielsen syndrome (hearing loss)
Torsades de Pointes
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Presentation Resuscitated sudden cardiac arrest
Presentation Resuscitated sudden cardiac arrestResuscitated sudden cardiac arrest Ventricular tachycardia, especially Torsades de
Pointes
Resuscitated sudden cardiac arrest Ventricular tachycardia, especially Torsades de
Pointes Unusual presentation of a seizure, especially
drop-attacks Unusual presentation of a seizure, especially
drop-attacksp Bradycardia, U waves, bizarre ST-T findings Deafness with any of the above
p Bradycardia, U waves, bizarre ST-T findings Deafness with any of the aboveyy
Examples of LQTS
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Clues to diagnosis Family history of known long QT syndrome
Clues to diagnosis Family history of known long QT syndrome Family history sudden cardiac death at an early age Unusual ECG as described Family history sudden cardiac death at an early age Unusual ECG as described Any of the presentations noted SCD VT/VF
Any of the presentations noted SCD VT/VF Seizures/drop-attacks Deafness +/- the above Seizures/drop-attacks Deafness +/- the above
IV Long QT syndrome andIV Long QT syndrome andIV. Long QT syndrome and other channelopathies
IV. Long QT syndrome and other channelopathies
Importance of diagnosis as soon as possible Therapies exist
Importance of diagnosis as soon as possible Therapies existTherapies exist Beta-blockers Other medical therapies
Therapies exist Beta-blockers Other medical therapies ICD Stellectomy ICD Stellectomy
Therapies decrease the risk of sudden cardiac death significantly Therapies decrease the risk of sudden cardiac
death significantly
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
An unusual etiology of dilated cardiomyopathy An unusual etiology of dilated
cardiomyopathy Potential reversible cause of LV
dysfunction Potential reversible cause of LV
dysfunction Chronic SVT or VT can result in chronic
DCM Chronic SVT or VT can result in chronic
DCM It may be difficult to diagnose Is the tachycardia primary or secondary?
It may be difficult to diagnose Is the tachycardia primary or secondary?
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Pathophysiology Experimental data that tachycardia-induced CM
Pathophysiology Experimental data that tachycardia-induced CM
can occur and results in biventricular systolic and diastolic dysfunction C di t t i d d SVR l t d it l
can occur and results in biventricular systolic and diastolic dysfunction C di t t i d d SVR l t d it l Cardiac output is reduced, SVR elevated, mitral
regurgitation occurs all the usual accompanying signs and symptoms of CHF
Cardiac output is reduced, SVR elevated, mitral regurgitation occurs all the usual accompanying signs and symptoms of CHFp y g g y p Underlying reasons for development of DCM
not clear
p y g g y p Underlying reasons for development of DCM
not clear
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Pathophysiology - Theory Abnormal calcium handling may be responsible for the
tachycardia induced cardiomyopathy
Pathophysiology - Theory Abnormal calcium handling may be responsible for the
tachycardia induced cardiomyopathytachycardia-induced cardiomyopathy Extensive abnormalities in calcium channel activity and
sarcoplasmic reticulum transport noted in animal model as early as 24 hours after pacing; persists for up to 4 weeks after
tachycardia-induced cardiomyopathy Extensive abnormalities in calcium channel activity and
sarcoplasmic reticulum transport noted in animal model as early as 24 hours after pacing; persists for up to 4 weeks afterearly as 24 hours after pacing; persists for up to 4 weeks after pacing stopped Severity of calcium cycling abnormality correlates with the
degree of LV dysfunction
early as 24 hours after pacing; persists for up to 4 weeks after pacing stopped Severity of calcium cycling abnormality correlates with the
degree of LV dysfunctiong y Probably calcium availability to myocytes is decreased with
subsequent reduction in contractility
g y Probably calcium availability to myocytes is decreased with
subsequent reduction in contractility
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Importance of paying close attention to the rhythm of a patient with DCM, especially if Importance of paying close attention to the
rhythm of a patient with DCM, especially if suggestion that presentation is chronic FTT
suggestion that presentation is chronic FTT Less likely to present in acute collapse though
not always true Chronic symptomatology as opposed to acute
Less likely to present in acute collapse though not always true Chronic symptomatology as opposed to acute Chronic symptomatology as opposed to acute Poor feeding, chronic wheezing or cough, falling off
growth curve, etc.
Chronic symptomatology as opposed to acute Poor feeding, chronic wheezing or cough, falling off
growth curve, etc.
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Carefully review the rhythm, 12-lead ECG mandatory in all patients with Carefully review the rhythm, 12-lead ECG
mandatory in all patients with y pcardiomyopathy If rhythm in NOT sinus and the patient is
y pcardiomyopathy If rhythm in NOT sinus and the patient isIf rhythm in NOT sinus and the patient is
tachycardic, most likely will be: Atrial tachycardia
If rhythm in NOT sinus and the patient is tachycardic, most likely will be: Atrial tachycardia Other forms of SVT Ventricular tachycardia Other forms of SVT Ventricular tachycardia
IMMEDIATE CONSULTATION REQUIRED: Pediatric cardiologist and electrophysiologist
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
The rhythm should be carefully analyzed and if abnormal The rhythm should be carefully analyzed and if
abnormal Try and decide if the abnormal rhythm is primary or
secondary This may be difficult
Try and decide if the abnormal rhythm is primary or secondary This may be difficult This may be difficult It may require therapy for the rhythm and waiting (usually
weeks) of observation to give LV function some time for improvement
This may be difficult It may require therapy for the rhythm and waiting (usually
weeks) of observation to give LV function some time for improvementimprovement Ablation Medical therapy DC cardioversion
improvement Ablation Medical therapy DC cardioversion DC cardioversion DC cardioversion
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Again..reminder of the symptomatolgy Same signs and symptoms with regard to
Again..reminder of the symptomatolgy Same signs and symptoms with regard toSame signs and symptoms with regard to
presentation of chronic dilated cardiomyopathy There is nothing specific with regard to the
Same signs and symptoms with regard to presentation of chronic dilated cardiomyopathy There is nothing specific with regard to the g p g
chronic tachycardia perhaps except that the tachycardia may be a bit out of proportion -
g p gchronic tachycardia perhaps except that the tachycardia may be a bit out of proportion -may be difficult to pick up independently 12-lead ECG
may be difficult to pick up independently 12-lead ECG
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Signs and symptoms of chronic tachycardia Tachypnea
Signs and symptoms of chronic tachycardia Tachypnea Tachycardia FTT, irritable, poor feeding Tachycardia FTT, irritable, poor feeding Poor perfusion Hepatomegaly Cardiomegaly
Poor perfusion Hepatomegaly Cardiomegaly Cardiomegaly Active precordium, gallop rhythm, regurgitant murmur Cardiomegaly Active precordium, gallop rhythm, regurgitant murmur
V Chronic tachycardia/ tachycardia-V Chronic tachycardia/ tachycardia-V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
V. Chronic tachycardia/ tachycardiainduced cardiomyopathy
Consequences of not recognizing the DCM Consequences of not
recognizing the DCM Consequences of not
recognizing that the DCM is a result of chronic
Consequences of not recognizing that the DCM is a result of chronicin a timely fashion
Discussed earlierP i t t
in a timely fashion Discussed earlier
P i t t
is a result of chronic tachycardia Treating symptoms but not
is a result of chronic tachycardia Treating symptoms but not
Progression to acute collapse Poorer prognosis
Progression to acute collapse Poorer prognosis
underlying cause Lose the ability for a cure;
can be reversible
underlying cause Lose the ability for a cure;
can be reversible Less likely to recover Less likely to recover May not improve and may
unnecessarily go on to transplant
May not improve and may unnecessarily go on to transplant
Summary and ConclusionsSummary and Conclusions
Some abnormalities may be difficult to detect especially when the signs and symptoms may be Some abnormalities may be difficult to detect
especially when the signs and symptoms may be subtle Careful exam and attentiveness to subtle findings
subtle Careful exam and attentiveness to subtle findings When the history and response to the usual
therapy doesnt make sense Look further When the history and response to the usual
therapy doesnt make sense Look further
Summary and ConclusionsSummary and Conclusions
Tip-offs in the newborn Subtle cyanosis
Tip-offs in the newborn Subtle cyanosis Heart murmur heard immediately after birth or at
discharge exam T h ( d t h di )
Heart murmur heard immediately after birth or at discharge exam T h ( d t h di ) Tachypnea (and tachycardia) An active (not quiet) precordium Diminution in pulses either in legs or globally
Tachypnea (and tachycardia) An active (not quiet) precordium Diminution in pulses either in legs or globallyDiminution in pulses either in legs or globally Focused pulse oximetry may be useful When in doubt ask for help
Diminution in pulses either in legs or globally Focused pulse oximetry may be useful When in doubt ask for helppp
Summary and ConclusionsSummary and Conclusions
Tip-offs in the older infant or child Falling off the growth curve
i h i h di d/
Tip-offs in the older infant or child Falling off the growth curve
i h i h di d/ Persistent or chronic or recurrent tachycardia and/or tachypnea Persistent wheezing that doesnt respond to usual
Persistent or chronic or recurrent tachycardia and/or tachypnea Persistent wheezing that doesnt respond to usual g p
therapy An active (non-quiet precordium) with or without a
heart murmur
g ptherapy An active (non-quiet precordium) with or without a
heart murmurheart murmur Unsual seizures or syncope (non-neurocardiogenic) When in doubt get a chest-xray
heart murmur Unsual seizures or syncope (non-neurocardiogenic) When in doubt get a chest-xray When in doubt ask for help When in doubt ask for help
THANK YOU VERY MUCHTHANK YOU VERY MUCHTHANK YOU VERY MUCHTHANK YOU VERY MUCH
Questions?Questions?