Approach to congenital cyanotic heart diseases

77
Approach to a patient with Congenital Cyanotic Heart Disease - Speakers:Dr. Debasis Maity Dr.Md.Samim Shikari Chittaranjan Sishu Sadan,Kolkata

description

 

Transcript of Approach to congenital cyanotic heart diseases

Page 1: Approach to congenital cyanotic heart diseases

Approach to a patient with Congenital Cyanotic Heart Disease

-

Speakers:Dr. Debasis MaityDr.Md.Samim Shikari

Chittaranjan Sishu Sadan,Kolkata

Page 2: Approach to congenital cyanotic heart diseases

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.

Page 3: Approach to congenital cyanotic heart diseases

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

Page 4: Approach to congenital cyanotic heart diseases

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.

Page 5: Approach to congenital cyanotic heart diseases

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

Page 6: Approach to congenital cyanotic heart diseases

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

Page 7: Approach to congenital cyanotic heart diseases

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

Page 8: Approach to congenital cyanotic heart diseases

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

Page 9: Approach to congenital cyanotic heart diseases

Physical Examination

• General appearance, weight and nutrition• Association with chromosomal syndromes and

other systemic malformations• Colour• Vital Signs– Pulse, BP, respiration and temperature

Page 10: Approach to congenital cyanotic heart diseases

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

Page 11: Approach to congenital cyanotic heart diseases

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

Page 12: Approach to congenital cyanotic heart diseases

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

Page 13: Approach to congenital cyanotic heart diseases

• 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.

Page 14: Approach to congenital cyanotic heart diseases

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

Page 15: Approach to congenital cyanotic heart diseases

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

Page 16: Approach to congenital cyanotic heart diseases

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

Page 17: Approach to congenital cyanotic heart diseases

Chest Radiography

• Cardiac size(CTR to be measured) and silhouette

• Cardiac chambers and great vessels• Pulmonary vascular markings• Differentiate with pulmonary disease

Page 18: Approach to congenital cyanotic heart diseases

Normal Cardiac Silhouette

Page 19: Approach to congenital cyanotic heart diseases

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….

Page 20: Approach to congenital cyanotic heart diseases

• 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)

Page 21: Approach to congenital cyanotic heart diseases

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

Page 22: Approach to congenital cyanotic heart diseases

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

Page 23: Approach to congenital cyanotic heart diseases

CHD – Age of Presentation

Page 24: Approach to congenital cyanotic heart diseases

Common Congenital Cyanotic Heart Diseases

Page 25: Approach to 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????????

Page 26: Approach to congenital cyanotic heart diseases

Xray chest

Page 27: Approach to congenital cyanotic heart diseases

Tetralogy Of Fallot

• High VSD– Large enough to equalize

pressure

• Pulmonic stenosis– Usually infundibular,

sometimes valvular

• Overriding of the aorta• Right ventricular

hypertrophy

Page 28: Approach to congenital cyanotic heart diseases

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

Page 29: Approach to congenital cyanotic heart diseases

TOF – Critical component

Degree of pulmonary stenosis – Determines degree of R L shunt(proportional

relation)– Determines duration and grade of murmur(inverse

relation)

Page 30: Approach to congenital cyanotic heart diseases

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”.

Page 31: Approach to congenital cyanotic heart diseases

TOF - Investigations

• ECG – Right axis deviation and

RVH

• CXR – Decreased pulmonary

vasculature– Concave PA segment– Right aortic arch, right

atrial enlargement

Page 32: Approach to congenital cyanotic heart diseases

Echocardiography

Parasternal long axis view Parasternal short axis view

Page 33: Approach to congenital cyanotic heart diseases

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

Page 34: Approach to congenital cyanotic heart diseases

Cyanotic spells - Pathophysiology

Page 35: Approach to congenital cyanotic heart diseases

• 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.

Page 36: Approach to congenital cyanotic heart diseases

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

Page 37: Approach to congenital cyanotic heart diseases

• 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).

Page 38: Approach to congenital cyanotic heart diseases

• 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.

Page 39: Approach to congenital cyanotic heart diseases

• 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.

Page 40: Approach to congenital cyanotic heart diseases

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

Page 41: Approach to congenital cyanotic heart diseases

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.

Page 42: Approach to congenital cyanotic heart diseases

Surgery - TOF

Page 43: Approach to congenital cyanotic heart diseases

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??????

Page 44: Approach to congenital cyanotic heart diseases

CXR of that infant

Page 45: Approach to congenital cyanotic heart diseases

Dignosed:

• TGA with VSD in Cngestive failure

Page 46: Approach to congenital cyanotic heart diseases

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

Page 47: Approach to congenital cyanotic heart diseases

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

Page 48: Approach to congenital cyanotic heart diseases

TGA - Variants

• TGA with intact Interventricular septum

• TGA with VSD• TGA with VSD and LVOT

obstruction (Pulmonary stenosis)

Page 49: Approach to congenital cyanotic heart diseases

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.

Page 50: Approach to congenital cyanotic heart diseases

TGA - Echo

Page 51: Approach to congenital cyanotic heart diseases

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

Page 52: Approach to congenital cyanotic heart diseases

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?????

Page 53: Approach to congenital cyanotic heart diseases

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)

Page 54: Approach to congenital cyanotic heart diseases

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%

Page 55: Approach to congenital cyanotic heart diseases

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

Page 56: Approach to congenital cyanotic heart diseases

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

Page 57: Approach to congenital cyanotic heart diseases

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

Page 58: Approach to congenital cyanotic heart diseases

Dignosis?

Page 59: Approach to congenital cyanotic heart diseases

Total Anomalous Pulmonary Venous Return

• Pulmonary veins drain anomalously into RA or systemic venous tributaries– Supracardiac – 50%– Cardiac – 20%– Infracardiac - 20%

Page 60: Approach to congenital cyanotic heart diseases

TAPVR

• Presentation and severity depends on pulmonary venous obstruction

• ASD or PFO essential for survival

Page 61: Approach to congenital cyanotic heart diseases

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

Page 62: Approach to congenital cyanotic heart diseases

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

Page 63: Approach to congenital cyanotic heart diseases

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

Page 64: Approach to congenital cyanotic heart diseases

Dignosis?

Page 65: Approach to congenital cyanotic heart diseases

Diagnosis?

• EBSTEIN’S anomaly with• WPW syndrome

Page 66: Approach to congenital cyanotic heart diseases

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

Page 67: Approach to congenital cyanotic heart diseases

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

Page 68: Approach to congenital cyanotic heart diseases

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

Page 69: Approach to congenital cyanotic heart diseases

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

Page 70: Approach to congenital cyanotic heart diseases

Fetal Echo

• May be done by transabdominal or transvaginal approach

• 4 chamber, 5 chamber and 3 vessel views

Page 71: Approach to congenital cyanotic heart diseases

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

Page 72: Approach to congenital cyanotic heart diseases

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

Page 73: Approach to congenital cyanotic heart diseases

A BREIF DISCUSSION

Page 74: Approach to congenital cyanotic heart diseases

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.

Page 75: Approach to congenital cyanotic heart diseases

• 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.

Page 76: Approach to congenital cyanotic heart diseases

Knee chest positions

Page 77: Approach to congenital cyanotic heart diseases

Thank You