Congenital Heart Disease

Dr. Muhammad Sajjad Sabir

MBBS, DCH, MCPS, FCPS

Assistant Professor of Paediatrics

VSDASDPDA

Tetralogy of Fallot(TOF)Transposition of great arties(TGA)Ebstein anomalyHypoplastic left heart syndromeTotal anomalous pulmonary venous return(TAPVR)

Aortic StenosisPulm. StenosisCoarctation of aorta

Tetralogy of Fallot

• Tetralogy of Fallot (TOF) is common cyanotic congenital heart disorders (CHD)

• Tetralogy of Fallot results in an inadequate flow of blood to lungs for oxygenation (right-to-left shunt)

• Patients with Tetralogy of Fallot initially present with cyanosis

ToF

Four anatomic malformations:

A- Pulmonary Valve Stenosis

B- Over riding of aorta

C- Ventricular Septal Defect

D- Right Ventricular Hypertrophy

Clinical PresentationBirth weight is low

Clinical presentation is directly related to the degree of pulmonary stenosis

Severe stenosis results in immediate cyanosis following birth

Mild stenosis will not present until later

Growth is retarded – insufficient oxygen and nutrients

Development and puberty may be delayed

Poor feeding

Cyanosis

Cyanosis during feeding

Anoxic spells

Dyspnea on exertion

Tachypnea

Exercise intolerance

Fussiness and agitation

Clinical Presentation

Clinical Presentation

• “Tet spells” at 2-3 yrs

• Paroxysmal attacks of dyspnea

• Anoxic spells

• Predominantly after waking up

• child becomes distressed and inconsolable, without apparent reason

“Tet Spell”

• Child cries→ Dyspnea (hyperpnea not tachypnea)→

progressively deeper Cyanosis → Loss of

consciousness → Convulsion (may experience syncope)

• During the spell diminished/absent murmur

• Frequency

once a few days to many attack everyday

“Tet Spell”

• Sitting posture – squatting

• Compensatory mechanism

• Squatting ↑ses peripheral (systemic) vascular resistance

• Which diminishes right-to-left shunt

• Increases pulmonary blood flow

• Increases oxygenation of blood

“Tet Spell”

Physical Examination• Clubbing + Cyanosis (Variable)

• Squatting position

• bulging left hemithorax

• Prominent “a” waves JVP

• Normal heart size

• Mild parasternal impulse

• Systolic trill (30%)

Investigations• CBC

- hematocrit↑

• ECG

-RVH, RAD

• EchocardiogramA- Pulmonary Valve Stenosis

B- Over riding of aorta

C- V S D

D- Right Ventricular Hypertrophy

CXR

• boot-shaped heart

secondary to uplifting of the cardiac apex from RVH

• Decreased pulmonary vascularity

• Right atrial enlargement

• Right-sided aortic arch (20-25% of patients)

Course and Complication• Each anoxic spell is potentially fatal

• Polycytemia

• Cerebral thrombosis

• Cerebral abcess

• Seizures

• Hypoxic damage

• Infective Endocarditis & vegetations

• Postoperative strokes

MANAGEMENT

Management of anoxic spell1) Calm the baby

2) increase SVR

3) Knee chest position

4) Humidified O2

5) Morphine 0.1 -0.2 mg/Kg Subcutaneous

6) Correct acidosis – Sodium Bicarb IV

6) Inj Phenyelphrine

7) Propranolol 0.1mg/kg/IV during spells

0.5 to 1.0 mg/kg/ 4-6hourly orally

7) Vasopressors

8) Correct anemia

9) PHELABOTOMY if polycythemia

Blalock Taussig Shunt• Classic BT shunt 1945…

• Lt Subclavian artery – Lt Pulmonary artery anastomosis

Modified Blalock Taussig Shunt

Rt Subclavian artery – Rt Pulmonary artery anastomosis

Potts Shunt

Side - side anastomosis of

Lt Pulmonary Artery to

Descending Aorta

Waterston shunt

Side-side anastomosis of Rt Pulmonary Artery to Ascending Aorta

Palliative Procedures

Surgical Intervention

Complete intracardiac repair

• Repair the VSD with a patch

Transcatheter patches

Open repair

• Repair of Pulm stenosis

either PA removed

or

removing the excessive muscle tissue of PA

Complications:

• Infective bacterial endocarditis

• pulmonic regurgitation

• Arrhythmias

• RBBB

• Left anterior hemiblock

VENTRICULAR VENTRICULAR

SEPTAL DEFECTSEPTAL DEFECT

(VSD)(VSD)

VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT

• most common ACHD

• 2nd most common CHD(32%)

• SYNONYMS

* Roger’s disease

* Interventricular septal defect

PATHOPHYSIOLOGYPATHOPHYSIOLOGY• Primarily depends on ----size of VSD

----status of pulm. vascular bed (rather than location)

• Small communication (less than 0.5cm`) VSD is restrictive & rt.ventricular pressure is normal – does not cause significant hemodynamic derangement (Qp:Qs =1.75:1.0)

• Moderately restrictive VSD with a moderate shunt(Qp:Qs =1.5-2.5:1.0) &poses hemodynamic burden on LV

• Large nonrestrictive VSDs(more than 1.0cm`) Rt & Lt ventricular pressure are equalised (Qp:Qs is >2:1)

PATHOPHYSIOLOGYPATHOPHYSIOLOGY

• Large VSDs at birth ,PVR may remain higher than normal and Lt to Rt shunt may intially limited – involution of media of small pulm.arterioles,PVR decreases—large Lt to Rt shunt ensues

• In some infants large VSDs ,pulm. arteriolar thickness never decreases –pulm. obstructive disease develops .when Qp:Qs=1:1 shunt becomes bidirectional,signs of heart failure abate &pt. becomes cyanotic. (Eisenmenger syndrome)

ANATOMICAL CLASSIFICATIONMEMBRANOUS SEPTUM

paramembranous/perimembranous defect

(or infracristal, subaortic, conoventricular)

MUSCULAR SEPTUM

inlet, trabecular, central, apical, marginal or

swiss-cheese

OUTLET SEPTUM deficient

supracristal,subpulmonary,infundibular or conoseptal

SEPTAL DEFICIFNCY

AVseptal defect (AVcanal)

CLINICAL FEATURESCLINICAL FEATURES• Race : no particular racial predilection

• Sex :no particular sex preference

• Age :infantsinfants– difficult in postnatal period,although ccf during first 6mths is frequent,X-ray&ECG are normal.

childrenchildren—after first year variable clinical picture emerges.

small VSD – asymptomatic

large VSD – symptomatic

Common Symptoms of large VSD

• Palpitation

• Breathing dificulty

• Dyspnoea on exertion

• Feeding difficulties

• Poor growth

• Frequent chest infections

PHYSICAL FINDINGSPHYSICAL FINDINGS

• Pulse pressure - wide

• hyperkinetic Precordium with systolic thrill LSB

• S1&S2 are masked by a PSM at Lt. sternal border

• Max. intensity of the murmur is best heard at 3rd,4th & 5th Lt intercostal space

• Lt. 2nd space –widely split & accentuated P2

• Maladie de RogerMaladie de Roger – small VSD presenting in older children as a loud PSM w/o other significant hemodynamic changes

INVESTIGATIONSINVESTIGATIONS

• ECHOCARDIOGRAPHYECHOCARDIOGRAPHY

two-dimensional & doppler colour flow

• CHEST RADIOGRAPHYCHEST RADIOGRAPHY

- normal

- biventricular hypertrophy

- pulmonary plethora

• ANGIOGRAPHY

(cardiac catheterization and angiography)

ECGSmall restrictive VSDs

Normal ECG

Medium-sized VSDs

Lt atrial overload- Broad, notched P wave

Signs of LV volume overload — deep Q and tall R waves with tall T waves in leads V5 and V6

Atrial fibrillation

Large VSDs Rt ventr hypertrophy - right-axis deviation

Biventricular hypertrophy - P waves notched or peaked

COMPLICATIONSCOMPLICATIONS

• Congestive cardiac failure

• Infective endocarditis on Rt. ventricular side

• Aortic insufficiency

• Complete heart block

• Delayed growth & development (FTT) in infancy

• Damage to electrical conduction system during surgery (causing arrythmias)

• Pulmonary hypertension

INTERVENTIONINTERVENTION 3 MAJOR TYPES

• SMALL SMALL

((surface area < 0.5surface area < 0.5 cm2 or <1/3or <1/3rdrd of Aortic root size) of Aortic root size)

- hemodynamically insignificant

- b/w 80-85% of all VSDs

- all close spontaneously

* 50% by 2yrs

* 90% by 6yrs

* 10% during school yrs

- muscular close sooner than membranous

• MODERATE VSDsMODERATE VSDs * surface area surface area 0.5-1cm2 or <1/2 of <1/2 of

Aortic root sizeAortic root size * least common group of children(3-

5%) * w/o evidence of ccf/ pulm.htn can be

followed until spontaneous closure occurs.

• LARGE VSDs WITH NORMAL PVRLARGE VSDs WITH NORMAL PVR

* surface area surface area >1 cm2 or ≥ of Aortic root ≥ of Aortic root sizesize

* usually requires surgery otherwise…

develop CCF & FTT by age of 3-6mths.

Conservative treatment

- Treat CCF & prevent development of pulm. vascular disease

- prevention & treatment of infective endocarditis

INDICATIONS for SURGERYINDICATIONS for SURGERY

• VSDs at any age where clinical symptoms and FTT cannot be controlled medically.

• Infants b/w 6-12mths of age with large defects ass. with PH ,even if symptoms are controlled by medication.

• Pt.s older than 24mths of age with Qp:Qs is greater than 2:1.

• Pt.s with supracristal VSD of any size, because of high risk of development of AI.

CONTRAINDICATIONCONTRAINDICATION –severe pulmonary vascular disease.

Surgical correction has

to be done before

irreversible damage to pulmonary vasculature

occurs

Operative procedureOperative procedure

Patch closure by RV approach

Percutaneous Device Closure• Muscular VSDs can typically be closed

percutaneously

Much more to comeMuch more to come

Are we all still awake?