5 Critical Cardiac Problems

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)



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



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



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



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



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



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



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


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


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


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


Etiologies Inflammatory/infectious

Etiologies Inflammatory/infectious Metabolic Toxic Metabolic Toxic Neuromuscular Familial Arrhythmogenic

Neuromuscular Familial Arrhythmogenic Arrhythmogenic Idiopathic Other

Arrhythmogenic Idiopathic OtherOtherOther


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


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


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


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



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)



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



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



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



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?



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



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



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.



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



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



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



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



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


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


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?

Date post:	07-Oct-2015
Category:	Documents
Upload:	b-k
View:	2 times
Download:	0 times

5 Critical Cardiac Problems

Documents