Approach to congenital cyanotic heart diseases
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Transcript of Approach to congenital cyanotic heart diseases
Approach to a patient with Congenital Cyanotic Heart Disease
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Speakers:Dr. Debasis MaityDr.Md.Samim Shikari
Chittaranjan Sishu Sadan,Kolkata
Nightmare:
• It’s 2A.M• Posted in NICU • Your 1st night therein• You get a callbook from C.S OT• You rush to the spot• See a just delivered “Blue Baby”.• Take all reversible measures to resuscitate• Excluded all causes ,remaining only cardiac causes left• Baby dies• Postmortem done to find out the cause• And yes you were right ,it was a case of TGA with Intact septum.
Congenital Heart Disease
• Prevalence• 0.8% of live births• 3-4% of stillborns, 10-25% of spontaneous
abortuses• 2% of premature infants (Excluding PDA)
• Leading cause of death among children with congenital malformations
Cyanotic Heart Disease
• Patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation.
• Can be caused by right-to-left or bidirectional shunting, or malposition of the great arteries.
Acyanotic or Cyanotic
• Normal saturations are ³ 95%• Visual diagnosis below 85%• Pulse oximetry useful in cases with SpO2
between 85% and 94%• Important to measure pre and post ductal
saturations and po2
Common Cyanotic LesionsDecreased Pulmonary Blood Flow
Increased Pulmonary Blood Flow
Tetralogy of FallotTricuspid AtresiaPulmonary Atresia with intact SeptumDouble Outlet Right Ventricle with PSTGA with PSEbstein Anomaly
Transposition of the Great ArteriesTotal Anomalous Pulmonary Venous ReturnHypoplastic Left Heart SyndromePersistent Truncus Arteriosus
History
• Development and weight gain• Poor feeding• Cyanosis and cyanotic spells• Squatting• Tachypnea, dyspnea• Frequent respiratory infections• Exercise intolerance• Chest pain, syncope, palpitations• Neurological Symptoms
Antenatal and Family History
• Maternal Infections– Rubella, CMV, Herpes, Coxsackie, HIV
• Maternal Medications, Alcohol and Smoking– Amphetamines, Lithium, Valproate
• Maternal Conditions– Diabetes, SLE
• Hereditary Disease– Marfan, Long QT syndrome, Holt Oram Syndrome
Physical Examination
• General appearance, weight and nutrition• Association with chromosomal syndromes and
other systemic malformations• Colour• Vital Signs– Pulse, BP, respiration and temperature
AssociationsSyndrome Associations
Trisomy 21 (Down syndrome) Endocardial cushion defect, VSD, ASD
X0 (Turner Syndrome) Bicuspid Aortic Valve, Coarctation of Aorta
Trisomy 18, Trisomy 13 VSD, ASD, PDA, coarctation of aorta, bicuspid aortic or pulmonary valve
Fragile X Mitral valve prolapse, aortic root dilatation
Deletion 5p (cri du chat syndrome) VSD, PDA, ASD
CHARGE association (coloboma, heart, atresia choanae,retardation, genital, and ear anomalies)
VSD, ASD, PDA, TOF, endocardial cushion defect
AssociationsSyndrome Associations
DiGeorge sequence, CATCH 22 (cardiac defects, abnormal facies, thymic aplasia, cleft palate, and hypocalcemia)
Aortic arch anomalies, conotruncal anomalies
Asplenia syndrome Complex cyanotic heart lesions with decreased PBF, TGA, TAPVR
Polysplenia syndrome Acyanotic lesions with increased PBF, PAPVR, dextrocardia, single ventricle
Congenital rubella PDA, peripheral pulmonic stenosis
Fetal hydantoin syndrome VSD, PDA, ASD
Fetal Alcohol Syndrome ASD, VSD
Maternal Diabetes Hypertrophic Cardiomyopathy, VSD, TGA
Cyanosis• Cyanosis: Bluish discoloration of skin and mucous
membrane due to reduced Hb concn more than 5gm/1ooml in cutaneous veins
• Central Cyanosis – Right to left shunts– Respiratory or CNS Pathology
• Peripheral Cyanosis - Exposure to cold, congestive heart failure, polycythemia, shock
• Differential Cyanosis-Hands RED ,feet BLUE-as in PDA
• Reverse differential cyanosis-Hands BLUE,feet RED-as in Transposition of the great vessels (TGA) + PDA + Pulmonary hypertension
• Intermittent Cyanosis-Ebstain’s anomalies.• Cyanosis is recognized at higher level of
spo2(80-85%)in polycythemia and at a lower level of spo2(45-50%) in severe anaemia.
Causes of cyanosis
CNS Depression Irregular respiration, poor muscle toneImproves with stimulation or mechanical ventilation
Pulmonary Disease Tachypnea, respiratory distressCrepitations, decreased breath sounds, CXR findings, Improvement with O2
Cardiac disease Tachypnea without retractionsLack of respiratory findingsLittle or no improvement with O2
CXR abnormal cardiac silhouette
Pulse and BP
• Examine pulse and BP in all four limbs• Weak lower limb pulses suggestive of
coarctation • BP compared against age specific percentile
curves
Systemic Examination
system wise but mainly-• First and second heart sounds
• Loud or muffled heart sounds• Splitting
MURMUR• Location• TimingSigns of congestive cardiac failure(eg-hepatomegaly etc)
Presence of murmur not necessarily pathological BUT Absence of murmur does not rule out cardiac lesions
Chest Radiography
• Cardiac size(CTR to be measured) and silhouette
• Cardiac chambers and great vessels• Pulmonary vascular markings• Differentiate with pulmonary disease
Normal Cardiac Silhouette
Hyperoxia Test
Appreciable specificity, sensitivity when matched clinically.
• Measurements to be obtained by TCOM or by arterial po2.
• If arterial Po2>250 torr:PASSED:excludes critical structural heart disease.
• <100:FAILED: diagnostic of cyanotic heart disease(in absence of obvious lung disease)
• 100-250: may be intracardiac mixing….
• We can also check preductal(rt radial art) and post ductal(umbilical art/post.tibial artery) arterial difference in po2.Difference of more than 20 torr is to be taken as significant.(remember differential cyanosis/reverse differential cyanosis)
Arterial Blood Gas
• Confirm or reject cyanosis• Elevated pCO2 suggests respiratory pathology• Low pH in sepsis, shock or severe hypoxemia• Pre and post ductal (umbilical artery or lower
limb) useful to confirm differential saturations
Echocardiogram
• Definitive diagnositic modality for structural heart disease
• M-mode, 2D Echo and Doppler• Windows commonly used – Apical 4 Chamber,
Parasternal long and short axis, Suprasternal, Subclavicular and Subcostal
CHD – Age of Presentation
Common Congenital Cyanotic Heart Diseases
Case scenario 01:
• A 2yr old boy presented with episodes of becoming blue mostly in the morning.
• O/E-central cyanosis,clubbing.• No pallor,oedema or respiratory distress.• CVS-heart was of normal size,parasternal heave +ve,
systolic thrill palpable over lt.middle sternal border.S1-Normal,S2-only A2 is audible.
• Liver –not enlarged. • CXR given below.• ECG-Rt axis deviation. Dignosis????????
Xray chest
Tetralogy Of Fallot
• High VSD– Large enough to equalize
pressure
• Pulmonic stenosis– Usually infundibular,
sometimes valvular
• Overriding of the aorta• Right ventricular
hypertrophy
TOF – Other Anomalies
• Right aortic arch – 25%• Pulmonary atresia – 15 – 20%• Left superior vena cava• Complete AV septal defect – 2%• Tricuspid valve anomalies• Anomalous coronary arteries – Ant descending
artery from RCA; passes across RVOT – 5%• Anomalies of Pulmonary Artery and branches
TOF – Critical component
Degree of pulmonary stenosis – Determines degree of R L shunt(proportional
relation)– Determines duration and grade of murmur(inverse
relation)
TOF - Clinical Features
• Dyspnea/Dyspnea on exersion• Cyanosis with tachypnea and clubbing• Failure to thrive• Severity of PS determines age of presentation• Cyanotic spells /“tet spells”.
TOF - Investigations
• ECG – Right axis deviation and
RVH
• CXR – Decreased pulmonary
vasculature– Concave PA segment– Right aortic arch, right
atrial enlargement
Echocardiography
Parasternal long axis view Parasternal short axis view
TOF – Cyanotic spells
• Hyperpnea and cyanosis, progressing to limpness and syncope, might terminate in convulsions or even CVA
• Right ventricular infundibular spasm and decreased systemic vascular resistance– Increased catecholamines– Increased respiratory rate
Cyanotic spells - Pathophysiology
• The highly accepted theory justifying pathophysiology of TOF with cyanotic spells:
• kothari et al: suggests role of mechanoreceptors in right ventricular infundibulum(spasm of RVOT) due to increased catecholamine,decreased RV size and hyperpnoea which ultimately precipitates a tet spell.
• Associated reduction in SVR is also postulated.
Cyanotic spells – Treatment
• Always check ABC,vitals as and when necessary.• A) Knee chest position- To increase systemic
vascular resistance• B) Oxygen- Oxygen to improve oxygenation.• C) Morphine- Depresses respiratory center→
abolish hyperpnoea. While giving this, facilities for ventilation should be available.dose-0.2mg/kg sc/im
• E) Propanolol- 0.01-0.25 mg/kg intravenously over 5 min. Reduces dynamic RV outflow obstruction.
• Slows HR (↓ R→ L Shunting). Slight ↑ in SVR. Blocks hyperpnea response.
• F) Ketamine- 1-3mg/kg. IV over 1min→ has dual benefit causes sedation and ↑ SVR
• G) Phenylephrine Hydrochloride- 0.01 mg/kg IV (slowly) or 0.1 mg/kg SC or IM (↑ SVR – dose to be titrated to BP response).
• H) Methoxamine- 0.10mg/kg IV over 5-10 min. Leads to ↑ SVR.
• I) IV fluids- preferably initially as bolus of 10-20cc/kg which may be increased to 60cc/kg. Bolus fluid should be isotonic saline or colloid. Extra volume can be given in cyanotic spell as the physiology is not inductive to CCF and also because of a restrictive RV physiology.
• J) Inj NaHCO3-(1-2 meq/kg intravenously slowly) with 1:1 dilution with 5%D. To correct metabolic acidosis.
• K) Transfuse PRBC’s- 5-10 ml / kg IV over 5 hrs.• L) Correct Tachyarrhythmia- Improve diastolic filling
and cardiac output.• M) RSI and mechanical ventillation as and when
necessary.Some people recommend availability of atleast temporary pacing facilty before instituting beta blockers.
TOF – Surgical Management
• Complete surgical repair– As early as 3 – 4 months of age
• Palliative surgery– TOF with pulmonary atresia/hypoplastic PA– Unfavourable coronary artery anatomy– Young infants with severe cyanotic spells
Palliative surgeries in TOF
• Waterston’s shunt: between ascending aorta & right pulmonary artery.
• Blalock-Taussig shunt: between subclavian artery & pulmonary artery.
• Pott’s shunt: descending aorta and left pulmonary artery.
Surgery - TOF
CASE SCENARIO 02:
• A 2month old INFANT OF DIABETIC MOTHER(full term delivery) presented to ER with marked respiratory distress.o/e –central cyanosis+,b/l-creps,HR-180/min,RR-58/min,Liver palpable-5.5cm.H/O-repeated episodes of cough,resp.distress,fever since birth.CVS-gr 3 ESM @ lt.parasternal area,CXR-cardiomegaly with narrow base and plethoric lung fields.Also there is a H/O exacerbation of cyanosis during cry.Diagnosis??????
CXR of that infant
Dignosed:
• TGA with VSD in Cngestive failure
Transposition of Great Vessels
• Aorta arises from right ventricle, pulmonary artery from left ventricle(d-TGA)
• Systemic and pulmonary circulations exist as parallel circulations
• PDA and PFO enable survival; 30 - 40% have VSD
Transposition of Great Vessels
• Also known as D-TGA• Normal relation – Aorta
posterior and to the right of pulmonary artery
• In TGA aorta is anterior• Presents with cyanosis and
tachypnea within first few hours or days of life
• Severity inversely proportional to presence of shunts
TGA - Variants
• TGA with intact Interventricular septum
• TGA with VSD• TGA with VSD and LVOT
obstruction (Pulmonary stenosis)
TGA - Diagnosis
• ECG usually normal – Right dominant
• CXR shows mild cardiomegaly, narrow mediastinum, and normal to increased pulmonary blood flow,rt upper field shows maximum plethora.
• Egg on end appearance.
TGA - Echo
TGA - Treatment
• PGE1 (start with 0.05-0.1mcg/kg/min,once the desired effect is achieved the dose is gradually reduced to 0.01mics/kg/min)) to maintain PDA
• Rashkind balloon atrial septostomy in severely hypoxic or acidotic infants
• Arterial switch procedure (Jatene) performed as early as first 2 weeks of life
• Rastelli operation another alternative• Previously, atrial switch operations were done by
creating atrial baffles
Case scenario 03:
• A 1month old boy,presented with-• Bluish discoloration of extremiteis and tounge• h/o- dusky appearance since birth,poor
feeding,rapid respiration since birth.• o/e- poor weight gain,LV type of apical impulse
+ve,prominent large “a” wave in JVP, holosystolic murmur of grade 3/6 at lt lower sternal border, s1 normal S2 single.Liver-enlarged .
• ECG-LAD+LVH. Dignosis?????
Tricuspid Atresia
• Circulation through Foramen Ovale/ASD
• Associated VSD or PDA• Presentation depends
on relation between great arteries and presence or absence of PS
• Commonest – Type 1b(No TGA, small VSD, PS)
Tricuspid Atresia - Presentation
• 50% present on D1, 80% by 1st month
• Infants with pulmonary oligemia present early with cyanosis, hyperpnea, acidosis
• Infants with pulmonary plethora present with CHF
51%
18%
Diagnosis
• CXR depends on pulmonary blood flow• ECG – Right atrial enlargement, left axis
deviation, LVH• Echo –– Absence of Tricuspid valve– Relation of great arteries– RVOT– VSD, PDA
Management
• Corrective surgery – Fontan procedure, usually after 2 years of age
• Palliation –• BT shunt for oligemia• Manage CHF in infants with pulmonary plethora• Consider PA banding if medical measures fail,
remember VSD might close spontaneously in Type 1• Palliative measures meant to try to attain normal
LV function, adequate PBF at low PA pressure
Case scenario 04
• A 2.5months old MALE infant, presented with mild cyanosis since birth,
• o/e-Now underweight, mild central Cyanosis.tachypnea ,tachycardia, precordial bulge+ve,hyperactive rt ventricular impulse,cardiac impulse is highest at xiphoid process and lower lt sternal border.
• S1-normal,S2-wide split and fixed,P2 accentuated,mid-diastolic rumble +nt at lower lt sternal border
• Hepatomegaly +nt• ECG-RVH pattern(rsR´ IN v1)• Cxr-given below
Dignosis?
Total Anomalous Pulmonary Venous Return
• Pulmonary veins drain anomalously into RA or systemic venous tributaries– Supracardiac – 50%– Cardiac – 20%– Infracardiac - 20%
TAPVR
• Presentation and severity depends on pulmonary venous obstruction
• ASD or PFO essential for survival
TAPVR - Management
• Treat CHF with diuretics, digitalis• Correction of metabolic acidosis, pulmonary
edema• Corrective surgery – earlier in obstructed
TAPVR• Aim to return blood to LA and close ASD• Obstructed TAPVR – symptoms exacerbated or
precipitated by PGE1
CHD – Ductus dependent lesions
Ductus Dependent Systemic Circulation
• Hypoplastic Left Heart Syndrome
• Crictical AS• “Shone” complex variants• Coarctation of aorta• Interrupted Aortic Arch
Ductus Dependent Pulmonary Circulation
• TOF with PA• Pulmonary Atresia with intact
interventricular septum• Critical PS• Tricuspid Atresia with PS/PA• Single ventricle with PS/PA• Severe Ebstein anomaly• Complete TGA with intact IVS
Case scenario 05:
• A 2yr old child presented to OPD with-• h/o-effort intolerance,easy fatiguability,with new onset bluish
discolaration of body and tounge which is gradually increasing• Mother has manic depressive psychosis*7yrs.father puts
forward a h/o of rapid heart beats of the child occasionally which he has noticed.
• Significant history??-One child died after similar type of presentation and had sudden death.(coincidental?)
• o/e- moderate central cyanosis,dominant “v” wave in jvp,S1-normal,S2-wide split but variable,TR murmur(systolic) +nt.
• ECG- RBBB+RAH pattern and has a characteristic clinch
Dignosis?
Diagnosis?
• EBSTEIN’S anomaly with• WPW syndrome
CHD – Management Summary
Ductus dependent Pulmonary Circulation
• TOF and PA/PS represent central cyanosis• Cyanosis aggravated with closure of ductus• Aim to keep ductus open till palliative shunt
surgery; Eg. BT shunt• PGE1 infusion indicated
Ductus Dependent Systemic Circulation• Signs of poor perfusion, acidosis, mimics sepsis• Maintain ductus patency with PGE1 • Ventilatory strategies to increase pulmonary
vascular resistance, avoid pulmonary over circulation
• Ideal systemic:pulmonary flow 1:1• Adjust PEEP, insp. Rate, Tidal Volume• Avoid too much O2 , pO2 around 80%• Decrease SVR – Phenoxybenzamine, Milrinone
Medical Management
• When a congenital cyanotic heart defect or duct dependent lesion is suspected –– Start PGE1 infusion (0.05 – 0.1 mcg/kg/min)– Maintenance dose of 0.01 mcg/kg/min– May cause apnea (12%), fever (10%) and flushing.– Less commonly brady/tachyarrythmia, shock,
cardiac arrest
Antenatal Diagnosis
• Fetal echo – usually done around 18 – 24 weeks, as early as 12 weeks
• High risk approach –– Sibling/Family history of CHD– Maternal infection, autoimmune, metabolic
conditions, drug intake– Fetal chromosomal anomaly, arrythmia– Abnormal 4 Chamber view on USG
Fetal Echo
• May be done by transabdominal or transvaginal approach
• 4 chamber, 5 chamber and 3 vessel views
Practical Approach to a cyanotic patient
CYANOSIS•Rule out acrocyanosis,cyanosis d/t extracardiac causes and treat accordingly to revert .•Crying,free flow of 100% o2,warming,Rx of circulatory compromise revert those situations
CAYNOYSIS PERSISTS!!!!!
•Conduct HYPEROXIA TEST accordingly(po2 less than 100torr)• Measure pre+post ductal po2 difference ,if any(significant)
Po2<100 torr: FAILED HYPEROXIA
TEST
•Cardiological evaluation•CXR•ECHOCARDIOGRAPHY•ECG
Contd.
•By Echocardiography•Maintain ductus dependent flow with PGE1 infusion to buy time for corrective surgeries if compatible with life
RULE OUT DUCTUS DEPENDENT FLOW
•By Echocardiography•Conservative management+corrective cardiac surgeries as recommended if any + antifailure measures if needed
DUCTUS INDEPENDENT
LESION
•For opinion from cardiologists & cardiothoracic surgeons•We must not hesitate to call help from others
REFERRAL AS AND WHEN NECESSARY
A BREIF DISCUSSION
Take home messages• Maintain a high index of suspicion in neonates
and infants with respiratory or feeding difficulties, and failure to thrive and cyanosis
• Basic investigations that are regularly done give a good idea about the presence as well as type of CHD even before an Echo is available
• One should not ignore the role of ECHOCARDIOGRAPHY because pure clinics may not be enough to definitely reach at proper diagnosis.
• Early institution of treatment to prevent long term morbidity and mortality
• Seek opinion from cardiologists and cardiothoracic surgeons without hesitation
• Maintain nutrition,hydration,prevent infection,anemia .
• Train and demonstrate “knee-chest” position to mother/attendents to abolish cyanotic spells.
Knee chest positions
Thank You