AN OVERVIEW OF CONGENITAL HEART DISEASE: BANGLADESH CHAPTER

Congenital Heart Disease (CHD) in Bangladesh

Congenital heart disease refers to structural or functional heart disease present at birth, even if first discovered later. The Incidence of CHD is 25/1000 live birth according to a study conducted in newborn in a tertiary care hospital of Dhaka.

Function of the heart

The heart functions as a pump which provides a continuous flow of blood through the body. This circulatory function can be divided into 2 parts

  1. The Right side of the heart: It collects deoxygenated blood from the whole body then pumps it to lungs for gaseous exchange
  2. The Left side of the heart: It collects oxygenated blood from the lungs and then pumps it to the whole body for tissue oxygenation. 

Causes of congenital heart disease

Though the causes of congenital heart diseases are still unknown, there are some factors which have some role in some disease. They are as follows

a. Inheritance

> HCM, ASD, Supravalvar A.S – Autosomal dominant
> Marfan’s with cardiac involvement- Autosomal dominant
> Holt – Oram syndrome with heart lesion (V.S.D) – Autosomal dominant
> Noonan’s with heart lesion – Autosomal dominant

b. Chromosomal Aberration

> 40% of Down’s syndrome have CHD (AV canal, TOF, VSD.)
> 20% of Turner’s have coarctation of the aorta or aortic stenosis

c. Maternal use of drugs during pregnancy

> Alcohol causes – TGA, VSD, PDA
> Amphetamine – TGA, VSG, PDA
> Phenytoin – PS, AS, COS, PDA
> Lithium- Ebstein anomaly
> Sex-hormones – TGA, TOF, VSD

d. Infections

> TORCH infections cause Pulmonary stenosis, Patent ductus arteriosus, Endocardial fibroelastosis.

e. Maternal conditions

> Maternal Diabetes mellitus causes – hypertrophic cardiomyopathy|
> Systemic Lupus erythematosus causes heart block in a baby.

Common congenital heart diseases in Bangladesh are

  • Ventricular septal defect (VSD)
  • Patent ductus arteriosus (PDA)
  • Atrial septal defect (ASD)
  • Aortopulmonary window (A-P window)
  • Pulmonary stenosis (PS)
  • Coarctation of Aorta (COA)
  • Aortic Stenosis (AS)
  • Tetralogy of  Fallot  (TOF)
  • Transposition of great arteries (TGA)
  • Pulmonary Atresia (PA)
  • Tricuspid Atresia (TA)
  • Truncus Atreriosus
  • Double Outlet Right Ventricle (DORV)
  • Single ventricle
  • Hypoplastic Left heart syndrome.
  • Others

Common acquired heart diseases in children

  • Rheumatic Fever and Rheumatic Heart Disease
  • Kawasaki Disease
  • Inflammatory Disease – Myocarditis, Pericarditis
  • Cardiomyopathy- Dilated cardiomyopathy, ischemic cardiomyopathy, Hypertrophic cardiomyopathy,  restrictive cardiomyopathy.
  • Infective Endocarditis
  • Tumors – Myxoma, Rhabdomyoma, Rhabdomyosarcoma.
  • Arrhythmia
  • Myocardial Involvement with systemic disease:
    • Glycogen storage disease type 1
    • Mucopolysaccharidoses: Hurler and Hunter syndrome.
    • Neuromuscular disease
    • Tuberous sclerosis.

Symptom of congenital heart diseases

In case of Neonates:
  • Feeding difficulties
  • Profuse head sweating
  • Failure to gain weight
  • Bluish discoloration of a lip, face, and nails
  • Fast breathing
  • Lethargy or shock
In the case of children
  • Failure to thrive
  • Bluish discoloration of lip, tongue, face or nails
  • Early fatigue
  • Fast breathing
  • Difficulties in breathing
  • Swelling & bluish discoloration of nails
  • A Sudden attack of a blue spell

Investigations for congenital heart diseases

With the following investigation, congenital heart diseases can be diagnosed

I.      Chest X-ray
II.    ECG
III.  Echocardiography
IV.  Cardiac catheterization

Treatments of congenital heart disease

  1. Treatment of congenital heart diseases can be divided in 3 groups ( Medical treatment, Catheter-based interventions & Surgical interventions)
  2. Medical treatment

A. For newborn

    • Management of Persistent pulmonary hypertension
    • Management of persistent fetal circulation
    • Keep the duct(PDA) open
    • Close the duct (PDA) in case of preterm baby
    • Management of heart failure
    • Management of cardiomyopathy
    • Management of arrhythmia
    • Management of shock

B. For children

    • Emergency management of cyanotic spell, SVT (Supraventricular Tachycardia), heart failure & Shock
    • Prophylaxis of cyanotic spell (blue spell)
    • Reduction of Pulmonary arterial pressure
    • Anti-heart failure treatment
    • Prophylaxis of bacterial endocarditis
    • Management of polycythemia ( partial exchange transfusion)

3. Catheter-based interventions

A. For newborn:

    • Balloon arterial septostomy
    • Stenting of Patent ductus arteriosus
    • Balloon dilatation of critical Aortic stenosis(AS), Pulmonary stenosis (PS), Coarctation of Aorta (CoA)Laser perforation of a pulmonary valve

B. For children and adults:

    • Device closure of ASD, VSD, PDA, AP window
    • Stenting of stenosed area like: Stenting of vessels Coarctation of Aorta, Pulmonary Arteries, Cerebral vessels, Multiple aortopulmonary collateral arteries (MAPCA)
    • Stenting of Right Ventricular outflow tract (RVOT)
    • Balloon dilatation of valve & vessels like AS, PS, CoA.
    • Valve replacement
    • Electrophysiology, pacemaker implantation

C. Structural intervention

    • PFO closure
    • Left atrial appendage closure
    • Alcohol septal ablation for Hypertrophic obstructive cardiomyopathy
    • Mitral clip for MR
    • Aortic Valve replacement
    • Pulmonary valve replacement
    • Mitral valve replacement
    • Electrophysiology – LASER ablation of various arrhythmia

     

4. Surgical interventions:

A. Palliative Surgeries like BT Shunt, PA banding, BDG, Fontan, Norwood etc

B. Corrective Surgeries like ASD, VSD, PDA closure, Arterial switch operation, Rerouting of TAPVC, TOF and DORD repair, Truncus repair etc.

Some Important Advice for patient with CHD

Lifestyle modification

  1. There is no specific restriction for any special food for congenital heart disease.
  2. A patient should have proper dental check-up routinely
  3. Should avoid over crowded area to prevent infection
  4. Avoid smoking for some of the diseases like Severe aortic stenosis, severe hypertrophic obstructive cardiomyopathy.

Immunization

  1. Normal immunization schedule should be followed in CHD.
  2. However after cardiac surgery immunization schedule should be withheld for three months.

Feeding

  1. Breastfeeding is not contraindicated in any of congenital heart disease but if there are feeding difficulties, expressed breast milk may be given with spoon or dropper.
  2. Complementary feeding should be started at 6 months of age.

Exercise

Patient with Pulmonary Hypertension, Heart Failure, Severe left to right heart obstruction should not do any exercise before corrective treatment.

Schooling

Schooling is encouraged for all children with congenital heart disease until the patient is moderately or severely ill.

Dental care

All patients with congenital heart disease even after surgery/intervention should maintain dental hygiene. They should be checked by a dental surgeon twice in a year.

Spontaneous bacterial endocarditis (SBE) prophylaxis

Prophylaxis required for Dental Procedures in following cases:

2007 Statement of the American Heart Association

·        Prosthetic cardiac valve or prosthetic material used for cardiac valve repair

·        Previous infective endocarditis in a case of CHD

·        Unrepaired cyanotic CHD, including palliative shunts and conduits

·        Completely repaired CHD with prosthetic material or device, whether placed by surgery or catheter intervention, during the 1st 6 mo after the procedure

·        Repaired CHD with residual defects at the site or adjacent to the site of a prosthetic patch, or

·        prosthetic device (which inhibit endothelialization)

·        Cardiac transplantation recipients who develop cardiac valvulopathy

Adolescent girl and women

Adolescent girls who are on Warfarin/Aspirin should consult their gynaecologist in case of menorrhagia. Those who take sildenafil for pulmonary hypertension should consult their physician for any adverse effects. Some Drugs are contraindicated in pregnancy. So pregnant women with CHD need consultation with obstetrician prior to conception.

Precaution for adult with CHD

  • Dental hygiene should be maintained regularly.
  • Smoking should be avoided
  • Adherent to regular check up to congenital heart disease clinic
  • Avoidance of pregnancy in case of Eisenmenger syndrome.
  • Regular blood test for polycythemia cases to assess the need for partial exchange transfusion.

Treatment facilities available in Bangladesh

A patient should go to pediatric cardiologist of any cardiac center where all kind of facilities is available to complete the workup of patient. In Bangladesh, pediatric cardiac services are available in the following hospital with facility for echocardiography in all with or without full range services of intervention and surgery.

  • Combined military hospital, Dhaka
  • Labaid cardiac center, Dhaka
  • National Institute of cardiovascular disease
  • National heart foundation
  • Dhaka Shishu Hospital
  • Bangabandhu Sheikh Mujib Medical University
  • Ibrahim Cardiac hospital
  • Apollo hospital, Dhaka

a.  Advance catheter interventions are available in the following hospital

  • Combined Military Hospital
  • Labaid cardiac hospital

b.  Surgical interventions are available in the following hospital

  • National Institute of cardiovascular disease
  • National heart foundation
  • Ibrahim Cardiac hospital
  • Apollo hospital
  • Bangabandhu Sheikh Mujib Medical University
  • Combined military hospital, Dhaka
  • Square Hospital, Dhaka

c.  Interventions available in CMH Dhaka and Lab aid cardiac Hospital for CHD

  • ASD, VSD, PDA device Closure.
  • Coronary Fistula & AP window device closure.
  • Stenting of vessels eg. Coarctation of the aorta, pulmonary arteries, and PDA
  • Balloon valvoplasty for Aortic stenosis, Pulmonary stenosis and Balloon Angioplasty for Coarctation of Aorta
  • RVOT stenting (TOF, DORV)
  • A neonatal Lifesaving intervention like Balloon dilatation of Coarctation of the aorta, Critical Aortic and Pulmonary stenosis
  • PDA stenting for Pulmonary Atresia, Extreme TOF
  • Balloon atrial septostomy (BAS) for D-TGA, Tricuspid atresia, Total anomalous pulmonary venous drains,
  • Melody valve replacement for dysfunctional conduit
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