Congenital heart disease 2013
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Transcript of Congenital heart disease 2013
Congenital heart disease(Formulation of the problem)
Antonio [email protected]
Médico coordenadorUnidade de Medicina Intensiva PediátricaUnidade de Medicina Intensiva Neonatal
Hospital Padre Albino
Professor de Pediatria nível II Faculdades Integradas Padre Albino
Catanduva / SP
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Congenital heart diseases are a dynamic group of anomalies that originate in fetal life and change considerably during postnatal development.
Routine neonatal examination fails to detect more than half of babies with
heart disease; examination at 6 weeksmisses one third.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Early recognition, urgent identification and timelyreferral to a pediatric cardiologist and timely
intervention has great implications in prognosis,
is the key in reducing mortalityand morbidity .
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Congenital heart disease in thenewborn requiring
early intervention ????
Life threatening heart diseases may not have obviousevidence early after birth, the diagnosis is difficultsometimes and always a great concern to pediatricians.
High index of suspicion is essentialto decision making.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical Presentation of CHD in the Neonate
• Fetal Diagnosis• Cyanosis• CHF/Shock/Circulatory Collapse• Arrhythmia• Asymptomatic Heart Murmur
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Formulation of the problem
?a great concern to
pediatricians
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Classification of CHD ( clinical point of view)
1.Life-threatening CHD
-Cardiovascular collapse is likely and compromised if nottreated early
Transposition of the great arteries (TGA), criticalpulmonary and aortic valvular stenosis/atresia,
hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous
return (TAPVR).
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Classification of CHD ( clinical point of view)
2. Clinically significant CHD-Cardiac malformations that have effects on heart function but where
the collapse is unlikely to be need early intervention.
Ventricular septal defect (VSD), complete atrioventricular septal defect (AVSD), atrial septal defect (ASD) andtetralogy of Fallot (TOF) with good pulmonary artery
anatomy.
3. Clinically non-significant CHD-No functional and clinical significance. Small VSD, atrial septal defect (ASD), mild pulmonary stenosis (PS).
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
• Total Anomalous Pulmonary Veins
• Tetrology of Fallot
• Tricuspid Atresia
• Transposition• Truncus Arteriosus
5 5 ““ TT’’ss””Most common cyanotic lesions of the newborn
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Cardiac malformations - 10% of infant mortality
Most common lethal diagnosis:
Left ventricular outflow tract obstruction
•Hypoplastic left heart syndrome•Coarctation of aorta•Aortic stenosis
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
How do we improve the detection of babies with congenital heart disease ?
•Understanding normal changes at birth
•Simple way to think about congenital heart disease
•Systematic examination of the cardiovascular system
•Simple additional screening
•Robust care pathways
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Pulse Oximetry
•easy to use, harmless when done correctly
•accuracy of 2% in the range of 70 to 100%
•consider cyanotic when Sat <94% at 24 hours of age
•should be obtained prior to discharge from nursery
= Policy at Sanford Children’s
Measure sat in foot
If <95%, gets evaluation
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
FLUID DYNAMICS
The function of the human heart is that of a mechanicalpump that receives the low pressure blood from the venoussystem and ejects it with higher pressure into the arterial system.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
DiagnosisApplied clinical logic
Heart and circulationPerfect harmony between structure and function
Logical thoughtGross morphology / physiologic derangements
Clinical manifestation
Accurate observation + Correct inferences
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Logical thoughtGross morphology / physiologic derangements
-Right sideStart proximally (vena cavae) andend distally (pulmonary arteries)
-Left sideStart proximally (pulmonary veins) andend distally (Aorta)
-What is the level of shunt?Acyanotic (left to right)Cyanotic (right to left)Atrial, ventricular, great artery
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
General Approach to CHD Patient
1. Define cardiovascular pathology
2. Predict pathophysiology
3. Determine hemodynamic goals
4. Anticipate emergency treatments
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Rosen: “any neonate in shock that does not respond to fluids or pressors has LV outflow obstructionuntil proven otherwise”
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Evaluation for and treatment of presumptive sepsis should be
undertaken simultaneously with evaluation for cardiac and pulmonary
disease.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
What Are The Odds?
• Congenital Heart Disease 8/1000 live births
• “Critical” CHD 3/1000 live births
• In the USA:~ 32,000 children born/year with CHD~ 11,000/year with “Critical” CHD
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Cardiac malformations - 10% of infant mortality
Most common lethal diagnosis:
Left ventricular outflow tract obstruction
•Hypoplastic left heart syndrome•Coarctation of aorta•Aortic stenosis
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Left Ventricular Outflow Tract Obstruction
Major source of neonatal M&M from CHD
•Accounts for ~ 12% of congenital cardiac disease in infancy•~ 75% discharged from hospital w/o diagnosis•~ 65% - normal newborn screen examination•6% died before diagnosis•96% symptoms by 3 wks of life
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Relative Frequency of Lesions %
• Ventricular septal defect 25-30 • Atrial septal defect 6-8 • Patent ductus arteriosus 6-8 • Coarctation of aorta* 5-7 • Tetralogy of Fallot 5-7 • Pulmonary valve stenosis 5-7 • Aortic valve stenosis * 4-7 • Transposition of great arteries 3-5 • Hypoplastic left ventricle * 1-3 • Hypoplastic right ventricle 1-3 • Truncus arteriosus 1-2 • Total anomalous pulm venous return 1-2 • Tricuspid atresia 1-2 • Double-outlet right ventricle 1-2 • Others 5-10
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
•The neonatal myocardium has fewer myofibrils in a disordered pattern, making the myocardium stiffer .
•The neonatal heart follows the Frank e Starlingrelationship but with a limited increase in strokevolume for a given increase in ventricular fillingvolume.
•The neonatal myocardium is dependenton heart rate to increase cardiac output.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
•Near peak of Starling curve•Stroke volume relatively fixed•C.O. relatively heart rate dependent
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Ductus Arteriosus
•Shunt between the descending aorta to the leftpulmonary artery
•Open because low PaO2 andcirculating prostaglandins (PGE2)
•Ductus closes within the firstdays (24/48 h) of life in theterm infant
•Permanent closure due to fibrosistakes 4-6 weeks
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Ductus Arteriosus
When patent ductus arteriosus (PDA) is opened widely, many serious
malformations may not be noticed easily in the early life.
Most of anomalies compatible with six months of intrauterine life permit live offspring at term (Fetal circulation)
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Ductal-dependent Heart Disease ?
Inadequate systemic oxgenation / pulmonary blood flow due to heart disease
• Inadequate pulmonary blood flow• Inadequate systemic delivery of oxygenated blood• Inadequate mixing
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Right sided obstruction
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Left sided obstruction
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Inadequate Mixing
Survival Depends Upon Mixing Between Systemic and Pulmonary Circuits
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Ductus Arteriosus
•Congenital heart disease in which either pulmonary or systemicblood flow is dependent on shunting through the ductus arteriosus.
•Postnatally closure of the ductus arteriosus would be fatal, progressas severe acidosis/shock/cyanosis.
•Prostaglandin E1 (PGE1 or Alprosdatil™) allow stabilization.
•PGE1 must be started immediately afterdelivery.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Prostaglandin E 1
•Always given as continous IV infusion.
•Start at 0.05-0.1µg/kg/min, can be reduced to 0.005 -0.01µg/kg/min once duct is opened
•Efficacy ↓ with ↑ age, less effective after 2 weeks of life, not effective after 4 weeks
•Continous cardiorespiratory monitoring
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Ductus Arteriosus
•Before anatomic closure of the ductus arteriosus andforamen ovale, certain stresses can cause the newbornto revert to fetal circulation
•Increased pulmonary vascular reactivity, raised PVR (Pulmonary Hypertension) and right-to-left shunting at thePFO and PDA, the clinical result is cyanosis.
Hypothermia, hypercarbia, acidosis, hypoxia and sepsis can all cause a reversion to fetal circulation .
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
CyanosisChronically adapted to the hypoxia in the uterine life, newborn infants are able to tolerate some degree of cyanosis than older infants or children
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Typically, 2 g/dL of reduced hemoglobin5g/dL of reduced Hb � clinical cyanosis
35%
65%
25%
75%
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Cyanosis
Category of cyanotic CHD
decreased pulmonary flow with right to left shunting lesions (PA, TA with shunting at the atrial or ventricular level)
poor mixing lesions (transposition physiology)
right to left shunt with intra cardiac mixing lesio ns(TAPVR, single ventriclular physiology, truncusarteriosus).
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Formulation of the problem
Basic questions
1. Is the patient acyanotic or cyanotic?2.How is body/pulmonary arterial blood flow ?
3. Does the malformation originate in the left or ri ght side of the heart?
4. Which is the dominant ventricule?5. Is pulmonary hypertension present or not?
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
• Commonly divided into acyanotic and cyanotic• 9 common conditions
ACYANOTIC
LEFT ���� RIGHT SHUNTSVentricular septal defect (30%)Patent ductus arteriosus (12%)Atrial septal defect (7%)
OUTFLOW OBSTRUCTIONPulmonary stenosis (7%)Aortic stenosis (5%)Coarctation of the aorta (5%)
CYANOTIC
Tetralogy of Fallot (5%)Transposition of the great arteries (5%)Atrioventricular septal defect –complete (2%)
Other complex – 20%
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
• Total Anomalous Pulmonary Veins
• Tetrology of Fallot
• Tricuspid Atresia
• Transposition• Truncus Arteriosus
5 5 ““ TT’’ss””Most common cyanotic lesions of the newborn
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
-The clinical sign in the neonate may be vague
For pediatricians:
-identify the newborn “not doing well”
•Persistent central cyanosis, unexplained acidosis, tachypnea withoutlung problems, etc.
•Assessment of saturation monitoring, status of perfusion (blood gasanalysis) and pulses/blood pressures in all extremities.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Maternal Risk Factors
• Congenital heart disease• Cardiac teratogen exposure
– Lithium – Amphetamines– Alcohol– Anticonvulsants: phenytoin, valproic acid,
carbamazepine, and trimethadione– Isotretinoin
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Maternal Risk Factors
• Diabetes mellitus• PKU• Hyperthyroidism• Lupus, collagen vascular disease• Rubella, CMV, Coxsackie, Parvovirus
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Fetal Risk Factors
• Trisomies, Turner’s syndrome, abnormal karyotype• Congenital malformations: duodenal atresia, TEF,
omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus
• Fetal arrhythmias• IUGR• Nonimmune hydrops• ?2 vessel cord
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
Dyspnea
• Lung or heart problems?
• Large shunt lesions:dyspnea, tachypnea, feedingdifficulty, irritability and distress.
• Ventilator weaning can be difficult in premature infantswith large left to right cardiac shunts.
Cyanosis with markedly reduced pulmonaryblood flow usually leads to "quiet tachypnea”,
without significant respiratory distress.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
Sign of poor perfusion
• Ductus dependent systemic circulatory ?
• Progressive dyspnea, cold, clammy mottled skin, whichindicates poor perfusion and acidosis, shock, oliguria
• Cardiovascular collapse at the time of ductal closure
• Shock in newborn ?
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
Cyanosis
•Central cyanosis
•noted in the trunk, tongue, mucous membranes
•due to reduced oxygen saturation
•Peripheral cyanosis
•noted in the hands and feet, around mouth
•due to reduced local blood flow
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
central peripheral
CAUSE ARTERIAL BLOOD DESATURATION OR ABNORMAL Hb
CUTANEOUS VASOCONSTRICTION DUE TO LOW CO
CONDITIONS Seen in R-L shunt, impaired pulmonary function, abnormal Hb
exposure to cold air or water and abnormally greater extraction ofO2 from normally saturated blood
SITES conjunctiva,palate,tongue,inner side of lips& cheeks
limited to ears,nose,cheeks outer side of lips hands feet&digits
certainly central if associated with clubbing and polycythemia,
clubbing is absent
probably central if it deepens on effort
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
Cyanosis
• Pulmonary X cardiac problems ?
• Persistent hypoxia refractory to 100% oxygen supply would indicate cyanotic CHD rather than pulmonary problems.
• Hyperoxia test
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical manifestations
Hyperoxia testarterial blood gas analysis while 100% oxygen
• PaO2 > 220 mm Hg would suggest respiratory disease
• PaO2 100‒220 mm Hg would require evaluation for cyanotic CHD
• PaO2 < 100 mm Hg would suggest cyanotic CHD
• PaO2 < 40‒50 mm Hg would be likely to have a poormixing disease such as TGA
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
HYPEROXIA TEST
GIVE 100% O2ASSES PO2
PO2>200 PO2<150
NO CCHD CCHD
PASS FAIL150-200
?CCHD WITH PBF OR PPHN
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Additional screeningAdditional screening
•• Pulse Pulse oximetryoximetry–– Post Post ductalductal
saturationssaturations
–– < 95% warning < 95% warning signsign
–– PULSOX studyPULSOX study
DUCTDUCT
R R handhand
L Hand and L Hand and both feetboth feet
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
What information do we require?
– 4 extremity BP’s– H & P
• Murmurs• Organomegaly• Pulses• ECG • Labs, CXR findings, saturations
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
The “Noncardiac” Cardiac Exam
• Vital signs, growth percentiles• UE/LE blood pressure & pulse oximetry• Color - cyanosis, pallor, mottling• Lungs - work of breathing, rate, equality, crackles• Abdomen - hepatomegaly, situs• Extremities - pulses, capillary refill time• Dysmorphic features, other organ system abnormalities
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Initial evaluation of child’s heart
•Listen to heart first when/if infant quiet•First concentrate on S1 and especially S2
•Louder than normal?•Split normally?
•Systolic murmur:•Diastolic murmur?•Widely radiating murmur?•Palpate liver•BP in arm and leg•Tongue - cyanosis
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Murmurs
• Loudness graded 1-6. Presence of thrill > 4• Timing – systolic/diastolic• Duration – ejection/mid/pansystolic• Site where loudest• Radiation
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Grading of murmurs
• Grade 1: only a cardiologist can hear• Grade 2: murmur softer than S1/S2• Grade 3: murmur louder than S1/S2• Grade 4: thrill palpable• Grade 5: murmur audible with stethoscope partially
off chest• Grade 6: murmur audible with stethoscope
completely off chest
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Diagnosis
Chest x ray
• Usually performed to rule out pulmonary disease as well as to evaluate pulmonary vascular marking andcardiomegaly.
• Some CHD has characteristic features
• Most of the serious CHD have no specific findingsexcept vague cardiomegaly, change of pulmonaryvascular marking and subtle finding of pulmonaryvenous congestion.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Diagnosis
Electrocardiography
EKG has been considered a useful tool in the diagnosis ofCHD,especially if echocardiogram is not easily available.
Ventricular maturation and associated ECG changes
• The fetal heart is right-side dominant• Right axis deviation and R wave dominance in lead V1 and S wave
dominance in lead V6. • At 3 e 6 months the classical left ventricular dominance pattern of
adulthood is established as ventricular hypertrophy occurs in response to increased systemic vascular resistance.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Diagnosis
Echocardiography
Echocardiogram is the most valuable method in thediagnosis of CHD.
• Identification of cardiac anatomy• Assessment of systolic ventricular function• Measurement of chamber dimensions and wall thickness• Assess the pressure gradients across the stenotic or regurgitation flow
through the valves• Assess abnormal cardiac physiology• Flow in the descending aorta • Estimation of pulmonary arterial pressure• Defining the direction of flow when valve regurgitation and shunt exist
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Diagnosis
Cardiac Catheterization
• The diagnostic frequency of cardiac catheterization is relatively decreasing especially in the neonate.
• It is still the key in defining certain anatomic variantsdifficult to be delineated by echocardiography alone
• Therapeutic catheterizations are considered as one ofthe life savingmodalities in some fields.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Diagnostic ladder
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
•Clinical evaluation with CXR and Hyperoxia test excludes CHD in most cases.
•Echocardiography recommended in all doubtful cases.
•Prior stabilization and a monitored transport to tertiary center ensures a optimal pre-operative state.
•Early intervention with very encouraging results is realistic for most forms of critical CHD in newborns
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Consultation: may be more cost-effective! 95% sens/spec for
discriminating CHD from innocent murmur
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Hypercyanotic spells
Cyanotic heart diseases
• Tetralogy of Fallot
• Pulmonary atresia• Transposition of great arteries• Tricuspid atresia
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
• Sudden severe episodes of intense cyanosis caused by reduction of pulmonary flow
• The level of cyanosis and onset of cyanotic spell is determined the SVR & level of PS component
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Clinical Presentation
• peak incidence age: 3 to 6 months• often in the morning, can be precipitated by crying,
feeding or defecation• severe cyanosis, hyperpnoea, metabolic acidosis• in severe cases, may lead to syncope, seizure, stroke or
death• there is a reduced intensity of systolic murmur during spell
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Management
• treat this as a medical emergency• knee-chest/squatting position:
- place the baby on the mother’s shoulder with the knees tucked up underneath.
- this provides a calming effect, reduces systemic venous return and increases systemic vascular resistance
• administer 100% oxygen
• give IV/IM/SC morphine 0.1 – 0.2 mg/kg to reduce distress and hyperpnoea
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Management
• IV Propranolol 0.05 – 0.1 mg/kg • IV Esmolol 0.5 mg/kg slow bolus over 1 min,followed by 0.05 mg/kg/min for 4 mins.• volume expander, crystalloid, 20 ml/kg rapid IV push to
increase preload• give IV sodium bicarbonate 1 mEq/kg to correct metabolic
acidosis• heavy sedation, intubation and mechanical ventilation
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
• a single episode of hypercyanotic spell is an indication for early surgical referral
(either total repair or Blalock Taussig shunt)
• oral propranolol 0.2 – 1 mg/kg/dose 8 to 12 hourly should be started soon after stabilization while waiting for surgical intervention.
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Thomas-Blalock-Taussig Shunt
Vivien Thomas, Partners of the Heart, 1998 andSomething the Lord Made - Best Made-for-TV Movie, 2004
Helen Taussig
Alfred Blalock
Vivien Thomas
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
November 29, 1944Thomas-Blalock-Tuassig
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Keep in your mind
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
•Routine neonatal examination fails to detect more thanhalf of babies with heart disease
•High index of suspicion is essential to decision making
•“not doing well”•Any neonate in shock that does not respond to fluids or pressors has LV outflow obstruction until proven otherwise
•If you think you have a ductal dependent lesionPGE1 must be started immediately(don’t be afraid of prostin)
Dr. Antonio Souto [email protected] 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Thanks a lot!!!