Cyanotic Heart Lesions

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Dr. Kalpana MallaMBBS MD (Pediatrics)

Manipal Teaching Hospital

Cyanotic Heart Lesions

• The 5 Ts– Tetralogy of Fallot– Transposition of the Great Arteries– Truncus Arteriosus (Persistent)– Tricuspid Atresia– Total Anomalous Pulmonary Venous Return

(TAPVR)

Cyanotic Heart Lesions

• Hypoplastic left heart syndrome (HLH)• Pulmonary atresia (PA) / critical PS• Double outlet right ventricle (DORV)• Ebstein anomaly• Single ventricle

R L

R L with pulmonary stenosis • TOF• Tricuspid atresia• Ebstein’s anomaly

Evaluation possible congenital heart

Exam: rate, rhythm, impulse, murmur, pulses (brachial and femoral)

• Oxygen saturation - Hyperoxia test • ABG• Chest X- ray• Echocardiogram• Cardiac catheterization

Tetralogy of Fallot (TOF):

• Most common (75% )cyanotic CHD in >2yrs

• ~ 10% of all CHD

Tetralogy of Fallot• TOF = consists– Ventricular septal defect– Rt ventricular outflow obstruction – infundibular or

infundibular + pulmonary valve stenosis – Aorta position is shifted to the right and over-rides

the VSD

– Hypertrophy of the right ventricle

Essential components: VSD Pulmonary stenosisOther components : overriding of Aorta RVH

Pentalogy of Fallot: all above + ASD

Hemodynamics:

• Large, non-restrictive VSD, perimembranous type, extending upto right ventricular outlet allows equalisation of pressures in two ventricles VSD is silent

• Pulmonary stenosis Shunting of blood from R L ventricle mixing of oxygenated & deoxygenated blood in left ventricle circulated to whole body

• Severity depends upon degree of pulmonary stenosis• Pulmonary stenosis causes concentric rt ventricular

hypertrophy without cardiac enlargement & ↑rt vent pressure

• Flow from Rt vent to pul artery across pul stenosis produce ejection systolic murmur

• If obstruction small, RL shunt minimal or absent

(pink or acyanotic TOF)

• P2 Delayed & reduced in intensity due to rt vent outflow obstruction reduced PA pressure

• S2 single and A2 audible• Severity of cyanosis directly proportional to

severity of pul stenosis but intensity of systolic murmur inversely related to severity of pulmonic stenosis

Clinical features:

• May become symptomatic any time after birth – usually 2nd half of 1st yr

• Anoxic spells (synonyms- hypoxic, hypercyanotic, blue, tet ) – paroxysmal attack of dyspnea

• Common symptoms – dyspnea on exertion,exercise intolerance

• Cyanosis

• H/O squatting during dyspneaic episodes

Anoxic spells

• Occur predominantly after waking up or following exertion

• Most commonly start around 4 to 6 months of age and are charcterized by

1.Sudden crying2.Sudden onset or deepening of cyanosis3.Sudden onset of dyspnea

Anoxic spells

4. Alterations of consciousness5. Convulsions6.Decrease in intensity of systolic murmurFrequency varies from once in a few days

to numerous attacks every day

• Mild outflow obstruction: cyanosis in later part of 1st year

• Severe outflow obstruction: cyanosis immediately after birth (as ductus starts to close)

CCF unusual in children with TOF except in: Severe anemia Valvular regurgitation Infective endocarditis Systemic hypertension Coincidental myocardial diseases

Physical examination:

• Cyanosis• Clubbing• Polycythemia• Normal sized heart• Mild parastrnal impulse

Auscultation

• S1 –normal• S2 – Single, (A2 heard,P2 soft &delayed• Murmur –• Shunt – absent• Flow - Loud short pulmonary ejection systolic

murmur grade 3-5/6 at 3rd ICS in left side

Diagnosis:• Blood: polycythemia• CXR: 1. Upturned apex (RVH)- Small boot shaped

heart 2. Oligemic lung fields3. Absence or concavity of pulmonary artery

segment gives the shape described as cor- en sabot

4. Right aortic arch ~25 -30%

• Diagnosis:

• ECG: RAD, RVH with tall peaked p waves

• Echo: overriding aorta,RVH,outflow obstruction

• Cardiac catheterisation

Complications:

• Cerebral thrombosis

• Brain abscess

• Bleeding diathesis

• Infective endocarditis

• CCF-in acyanotic or pink TOF

Complications:

• CNS - Embolism to CNS - sluggish circulation from polycythemia

• Hemiplegia - infarction in CNS during anoxic spell

Management:

• Management of Tet spells: - knee-chest position

- humidified O2 inhalation

- morphine 0.1 mg/kg s/c/ iv

Management of Tet spells: - IV fluids - Correct metabolic acidosis- Na-bicarbonate - Propanolol – 0.1mg/kg/iv during spell (0.5-1

mg/kg PO 6 hrly

Management:

• Vasopressors- methoxamine IM or IV drip penylephrine

• Correct anemia• Consider surgery

• General measures: - Correction of iron deficiency anemia - Adequate hydration - Antibiotics for infection - Prophylaxis with propranolol

Surgery

• Palliative surgery: - Blalock-Taussig shunt – subclavian artery to

pulm artery - Pott’s shunt-descending aorta to PA

- Waterson operation – ascending aorta to Rt pulm artery-Modified Blalock-Taussig shunt

• Corrective surgery: open heart surgery for – closer of VSD

- resecting the infundibular obstruction PS Surgery can be performed at any ageSuccess – 85-90%

Complications of surgery

• Complete heart block• RBBB• Residual VSD & Pulm stenosis• Pulm regurgitation

Tricuspid atresia

• Cong absence of Tricuspid valve

• Rt ven hypoplastic• Absent inflow portion

• 2% of CHD

Hemodynamics

• No communication between Rt atrium rt ventricle (hypoplastic)

• Blood from Rt atrium lt atrium through patent foramen ovale or ASD.mixing of oxygenated + deoxygenated blood to lt ventricle aorta

• Lt vent rt vent there is VSD pul artery ( lt .ventricle maintains both systemic & pulmonary circulation saturation of blood is identicle in pulm artery and aorta

Clinical features

• Depends on state of pulmonary flow • 90 % are with diminished blood flow• Features :As TOF • Differentiating points :1.Cyanosis from birth2.More sicker than TOF3.Lt ventricular type of apical impulse4.Enlarged liver with presystolic pulsations5.ECG- LAD,LVH

Diagnosis

• Blood: polycythemia• CXR: 1.Oligemic lung fields2.Left ventricular configuration3.Prominent SVC shadow• ECG: Rt & Lt atrial hypertrophy, LAD,LVH

• Echo: large single ventricular cavity

Tricuspid Atresia

Repair not usually performed in neonatal period- over a series of procedures– Systemic to PA shunt– SVC to PA shunt (followed by ligation of first

shunt)– Glenn Shunt– IVC to PA shunt– completion Fontan

Ebsteins Anomaly

• Rare CCHD • Post and septal leaflet of TV – displaced downwards

–• The upper part of the right ventricle is part of the

right atrium - atrialized rt ventricle• Rt ventricle is too small and Rt. atrium is too large. • Leaflets – malformed and fused – obstruction of

flow to rt ventricle

Ebsteins Anomaly

• Often Associated with other heart lesions–ASD–Pulmonary Stenosis–Pulmonary Atresia

Hemodynamics :

Abnormal leaflets obstruction to forward flow & regurgitation from Rt ven to Rt atrium atrium dilates Patent FO / ASD allows R L shunt( cyanosis) Lt atrium (enlarged) Lt ventricle (enlarged & hypertrophied)

Clinical picture

• Cyanosis• Effort intolerance• Fatigue• Paroxysmal attack of tachycardia• Clubbing • Lt ventricular apical impulse • Systolic thrill may be palpable LSB

Auscultation

• S1- normal• S2 – widely split but variable• Rt ventricular 3rd soundor rt atrial 4th sound

audible – triple/quadruple sound usually heard

• Murmur-midsystolic ejection or pansystolic• Short tricuspid delayed diastolic M

Investigations

• CXR cardiomegaly –square shaped Lung – oligemicECG- ‘p’ pulmonale ‘p’mitrale,RBBBWolff Parkinson white type conducton defect

maybe seenECHO- displaced tricuspid valve

Treatment

• Surgical – obliteration of atrialised portion of rt.ventricle and repairof tricuspid valve

Fallot’s physiology

• Presence of large VSD with PS • Useful for bedside identification of group of

condition with similar clinical findings• Defects with Fallot’s physiology:1. Complete TGA with VSD & PS2. DORV with PS & large VSD (subaortic)3. Tricuspid atresia with diminished pul flow4.Single ventricle with PS5. corrected TGA with VSD & PS

Transposition of the Great Arteries

• Most common cyanotic condition that requires hospitalization in first 2 weeks of life

• Aorta arises from RV • Pulmonary artery originates in the left

ventricle

• Oxygenated pulmonary venous blood recirculates in lungs and systemic venous blood recirculates in systemic circulation

Transposition of the Great Vessels

• A PDA,ASD,VSD, is necessary for these infants to survive until they can have corrective surgery

• More common in infants of diabetic mothers

Classification

1. Complete variety2. Physiologically corrected type

Complete variety

• Rt atrium →Rt ventricle →aorta• Lt atrium →Lt ventricle →pulmonary artery• Systemic & pulmonary circulation separate

→survival possible only if there is ASD,VSD,PDA

Classification A) With intact ventricular septum – mixing site is

atrial communication PFO B) with VSD with/without pul stenosis

Physiologically corrected type

• Rt atrium → morphologically inverted left ventricle →pulmonary artery

• Lt atrium → morphologically inverted Rt ventricle →aorta

• Route of blood flow is normal

C /F with intact VS

• Cyanotic at birth• Interatrial mixing poor (PFO) – rapid

breathing ,congestive failure due to hypoxia within 1st wk of life

• CCF • S1 –normal• S2- single• Ejection systolic murmur grade 1-2/6• CXR – egg on side appearance,plethoric lung field

With VSD

• Good mixing at ventricular level, large pulmonary blood flow – cyanosis milder

• CCF at 4-10 wks• Exam –cyanosis,CCF• S1- Normal• S2 – single • Murmur – ejection systolic grade 2-4/6

Diagnosis

• CXR- egg on side appearance ,cardiomegaly with narrow base, plethoric lung field

• ECG without VSD – RAD,RVH• ECG with VSD – RAD, biventricular

hypertrophy• Cardiac catheterization• Angiocardiography

Medical management

• Control CCF• Balloon atrial septotomy by cardiac catheterization -

Inter-atrial septum opened • Definitive repair – Jatene’s switch operation -

removal of aorta and pulmonary artery from their origins and re-attached to the correct ventricles

• Less preferred – atrial switch operation –mustard or senning

Corrected TGA

• Normal route of blood flow • Commonly associated with other anomalies

98% - symtoms are due associated anomalies:1.VSD with/without PS2.Lt sided Ebstein’s anomaly of tricuspid valve3.Atrioventricular conduction abnormalities

Truncus Arteriosus

• Truncus fails to divide completely during fetal life, leaving a connection between the aorta and pulmonary arteries

• Mixed oxygenated and de-oxygenated blood exits the heart and enters the systemic circulation

Truncus Arteriosus• Single artery arises from the heart, supplying both aorta

and pulmonary artery.• VSD below the truncal valve allows mixing of right and left

ventricular blood• Degree of cyanosis is variable• Presents with progressive heart failure

Truncus Arteriosus

• Medical Management– Digoxin and Diuretics

• Surgical Repair– Usually required by 2-3 months of age– VSD is closed– PA trunk is separated from truncus– Conduit created between RV and PA using a valved

graft

TAPVR

• The pulmonary veins, instead of being connected to the left atrium , are connected to the right atrium or superior vena cava, and return oxygenated blood to the right side of the heart.

Total Anomalous Venous Return

• Uncommon CCHD• Cyanosis• CCF at age 4-10 wks• Murmur : pul ejection systolic + tricuspid flow

murmur• Continuous venous hum audible at upper left

or rt sternal border or in suprasdternal notch

Diagnosis

• CXR- snowman or figure of 8 configuration• ECG – RAD,RVH,• ECHO- demonstrate abnormal course of pul

veins

Total Anomalous Venous Return

• Control of CCF, pul infections • The only accepted treatment is surgery• Surgical connection is made between pulmonary

venous confluence and the LA• Connection to systemic venous circulation is

ligated.

Hypoplastic Left Heart

• Fatal without early surgical intervention

Treatment- continued

• General procedure for cyanotic heart lesions involves a systemic to PA shunt.

• Procedure known as the Blalock-Taussig shunt– Uses a small Gore-Tex® shunt to connect either

left or right subclavian to left or right branch PA.– Allows partially desaturated blood to enter PA,

increasing pulmonary blood flow and oxygenation

CCHD with PA HTN

• This group is named – Eisenmenger syndrome – severe PA HTN resulting in R→L shunt at atrial, ventricular or pulmonary arterial level

• Eisenmenger complex – severe PA HTN with VSD resulting in R→L shunt

• Eisenmenger's syndrome named by Dr. Paul Wood after Dr. Victor Eisenmenger, who first described the condition in 1897.

Hemodynamics

• L→R shunt in the heart causes:- - increased flow through PA - High O2 saturation in PA - Hyperreactive pul vasculature → Pul

vascular obstructive Ds →PA HTN• PA HTN → causes increased pressures in the right

side of the heart and reversal of the shunt into R → L shunt

• R → L shunt with VSD & PDA → decompresses rt ventricle →RV has only concentric hypertrophy with no increase in size ( no heave)

• R → L shunt with ASD → RVH +dilatation →rt ven failure

• R → L with ASD or VSD →mixing of blood reaches ascending aorta → distributed to whole systemic circulation → equal cyanosis

• R → L with PDA → mixed blood directed downwards to descending aorta (junction is distal to lt Subclavian artery → cyanosis + clubbing of toes only (differential cyanosis)

Examination

• Cyanosis• Clubbing• Fatigue• Effort intolerance• Dyspnea• h/o recurrent chest infection

Sounds

• S1- normal• S2- ASD- wide fixed split VSD- single• PDA- normally split• Murmurs• Pulmonary regurgitation ( graham steel)• Ejection systolic

Investigations

• CXR- prominance of pul artery,heart size – normal to large• ECG – RVH• ECHO-• Cardiac catherization-bi-directional shunt

Treatment

• Heart-lung transplant is required to fully treat the syndrome

• If this option is not available - treatment is palliative-

• Anticoagulants• Pulmonary vasodilators• Antibiotic prophylaxis to prevent endocarditis• Phlebotomy to treat polycythemia• Maintaining proper fluid balance

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