Ventricular Septal Defect

What is Ventricular Septal Defect (VSD)?

VSD is a hole in heart that presents at birth, i.e. child born with a hole in heart. This hole is present between 2 lower chambers of heart, known as right and left ventricles in ventricular septum. This septum separates right and left ventricles.

What are the types of VSDs?

There are different types of defect noted in ventricular septum:
1. Peri-membranous VSD: most common type
2. Muscular VSD
3. Outlet VSD
4. Sub-pulmonic VSD etc

Clinical presentation of VSD?

It depends on size of VSD.

If size of VSD is small, child is generally asymptomatic, and only Systolic murmur will be there on examination by a pediatrician or physician.

If size of VSD is moderate (neither small nor big), child generally have history of poor weight gain, cough and cold etc.

In large sized VSD, child generally presents at 1-2 months of age with complaints of cough, cold, difficulty in breathing, poor weight gain, sweating over forehead.

Diagnosis of VSD?
For diagnosing VSD, Echocardiography of child needs to be done. Echo is a non-invasive test that is without pain. Echo is done from chest. Generally in small child, echo needs to be done when child sleeps.
Timing of closure of VSD?

After confirming the size of VSD and degree of dilatation of left heart chambers on Echocardiography, we can plan further treatment.

If size of VSD is small with no dilatation of left heart chambers then we do follow-up of child at periodic interval. Generally, small VSD closed spontaneously by 4-5 years.

If moderate sized VSD,then it generally associated with left heart dilatation, then we should close the hole between 6 months - 1 year of age.

In large sized VSD: we close between 3-6 months of age.

Can we close VSD by medications?

Medicine is not available for closing the VSD. Medicines are used for controlling symptoms due to heart failure, LRTI etc.

What are the options for closure of VSD?

Either angiographically (without surgery) or by surgery.

Which method is suitable for closing VSD?
It depends on size of VSD, location of VSD as well as on weight of the child. Small sized VSD: If needs closure and is suitable for closure by using a device in cath lab (without surgery), then can be closed in cath lab by using a device. Moderate sized VSD: Closing by method is decided by size & location of VSD and weight of child. Majority can be closed by device method in cath lab by angiography. Large VSD: by open heart surgery only.
Advantages of VSD closure by using adevice (Non-surgical method)?

1. Short hospital stay (2-3 days)
2. No surgical scar on the body
3. Only one tablet once a day for 6 months needs to be taken after the procedure.
4. Can attend school from next day of discharge
5. Can participate in all outdoor activities from very next day
6. Very less risk as compared to surgery, although very safe procedure
7. No requirement of ventilator in most cases

Surgical options?
Only by open heart surgery on bypass machine.
How we should do follow-up after device closure or after surgery?

After Device closure by angiographically: After one month then 3, 6, 12 months after the procedure.

After surgery: after 3 days for wound dressing, then 1,3,6,12 months after surgery.

How are VSDs closed in the cath lab?

By cardiac angiography from thigh blood vessels that are connected with the heart. We close VSDin a beating heart.

This procedure is generally done under conscious sedation. Within six months, device become endothelized and become a part of heart for life long.

Tetralogy of Fallot

What is Tetralogy of Fallots’s (TOF)?

TOF is a cyanotic heart disease that is present at birth, i.e. child born with heart disease.

This consists primarily of a hole known as Ventricular Septal Defect (VSD) and narrowing or obstruction of blood flow to the lungs (Pulmonary Stenosis, PS).

Clinical presentation of TOF?

It depends on severity of PS.

If PS is severe, child is generally blue (cyanosed) and also have an irritable behavior.

If PS is mild with large VSD, child generally have history of poor weight gain, frequent cough and cold, feeding difficulty etc. as it will behave like a large VSD.

Diagnosis of TOF?

For diagnosing TOF, Echocardiography of child needs to be done. Echo is a non-invasive test that is without pain. Echo is done from chest.Generally in small child, echo needs to be done when child sleeps.

Are other Investigation required?
Yes, sometimes we need to do “CT angiography” or “cardiac cath angiography” of heart.
Can we treat TOF by medications?

Medicine is not available for closing VSD and relief of PS. Medicines are used for controlling symptoms due to bluishness, and if child is not operated on time, then medical treatment is advised for increased hemoglobin (polycythemia) if symptomatic.

What are the options for treatment of TOF?

Mainstay of treatment is only by open heart surgery (Intracardiac Repair of TOF).

If child is very blue in early infancy, then surgical options in the form of BT shunt or if suitable then stent can be placed in right ventricular outflow tract angiographically (RVOT stenting) as a palliative procedure. By doing this, child can be less blue for few months. When child grows well, then Intracrdiac repair can be carried out.

Timing of surgery (TOF)?

If child is very blue in early infancy, then as mentioned above BT shunt or RVOT stenting can be advised as an initial treatment.

If child is blue and size of pulmonary arteries (vessels of lung) are small even in more than 5-6 months of age, then again a palliative surgery in the form of BT shunt is advised.

If child is not having any signs of bluishness and growing well with adequate sized branch pulmonary arteries then Intracradiac repair of TOF is done after 5-6 months of age.

Can we delay surgery if child is not having any problems due to TOF?

Ideally, TOF should be operated before child attains one year of age as best outcome will be there in this period.

How we should do follow-up after surgical repair of TOF?

At regular intervals: After 3 days for wound dressing, then 1, 3, 6, 12 months after surgery.

Critical Congenital Heart Disease

Clinical assessment of Coarctation of Aorta (COA) in neonates
We can assess by palpating femoral pulses, in Coarctation of AortaCOA, these are either very weakly felt or not felt at all. Also there will be no pulse trace while assessing saturation in lower limbs. We should not rely on saturations until we get a good trace on monitor. In severe Coarctation of Aorta COA, saturation number we may get but trace will not be there. Severe Coarctation of Aorta COA is a medical emergency and needs to address on priority basis.
Critical Congential Cyanotic Heart Disease (Screening in Newborn)

In first 48 hours, many of cyanotic congenital heart disease can be easily missed. To avoid that, we shall assess saturation before discharge (at least in one upper and one lower limb). In cyanotic heart disease, saturation will always be less than 95%. If difference in spo2 of upper and lower limbs is more than 5%, then we should get an echo done before discharging the newborn.

Neonates presented with shock after 48 hours of life
Whenever a neonates (especially after 48 hours) comes with shock ,suspect duct (PDA) dependent lesion newborn may need Prostaglandin infusion to keep the duct open.

Atrial Septal Defect

What are the types of ASD?

There are 4 types of defect noted in atrial septum:

  • OstiumSecundum (OS-ASD) ASD: True ASD
  • OstiumPrimum Defect
  • Sinus venosus (SVC and IVC types) Defects
  • Coronary sinus type defect

Ostiumsecundum atrial septal defect is the most common type.

Clinical presentation of isolated ASD?

Majority of children are asymptomatic with ASD. Rarely may have poor weight gain, cough, cold etc.

As majority is asymptomatic, ASD can be easily missed in day to day examination by his/her child heart specialist. Some patients are coming in late adulthood with diagnosis of ASD.

By careful examining the child, chest x-ray or ECG, we can suspect ASD.

Timing of closure of ASDs?

Ostiumsecundum ASDs are closed between 2-4 years of age.

Ostiumprimum defect: if not associated with other cardiac defects, then we can follow and can close between 2-3 years of age; and if associated with moderate to severe mitral valve regurgitation (MR) then we should close by one year of age.

Sinus venous type of defect: We generally close by 4-5 years of age.

Which method is suitable for closure of ASDs?

Ostiumsecundum(OS-ASD) ASD: There are two options: Either surgical closure or by transcatheter method if suitable for device closure.

For other varieties of ASDs: Surgery is treatment of choice.

Which method is suitable for ostiumsecundum ASDs (OS-ASD: Device ottranscatheter closure, or surgical closure?

This type of ASD can be closed either by surgery or by using a device in cath lab by angiography.

Closing by method is decided by size of ASD and weight of child as well as surrounding margins of ASD.

If size of ASD is moderate (not very large in size) with good surrounding margins with accepted weight of the child, then device closure method is the treatment of choice.

If not then surgery is treatment of choice.

Advantages of ASD closure by using Device (Non-surgical method)?
  1. Short hospital stay (2-3 days)
  2. No surgical scar on the body
  3. Only one tab needs to be taken for 6 months
  4. Can attend school from next day of discharge
  5. Can participate in all outdoor activities from very next day
  6. Very less risk as compared to surgery, although very safe procedure
  7. No requirement  of ventilator in most cases
Medicine to be used after device closure?

Only tab Aspirin to be taken for 6 months after device closure.

How we should do follow-up after device closure or after surgery?

After Device closure by angiographically: After one month then 3, 6, 12 months after the procedure.

After surgery: after 3 days for wound dressing, then 1, 3, 6, 12 months after surgery.

Patent Ductus Arteriosus (PDA)

What is PDA?

PDA is a normal communication between descending aorta and left pulmonary artery. This is normally seen in fetal stage as well.

Normally it closes within 48 hours of life in majority of kids especially in term babies. In preterm babies, depending on his gestational age and weight of baby, sometimes it may take longer time to close spontaneously.

Clinical presentation of PDA in term infants?

It depends on size of PDA and symptoms of child.

If size of PDA is small, child is generally asymptomatic, and only continuous murmur will be there on examination.

If size of PDA is moderate (neither small nor big), child generally have history of poor weight gain, cough and cold etc.

In large sized PDA, child generally presents at 1-2 months of age with complaints of cough, cold, difficulty in breathing, poor weight gain, sweating over forehead.

Timing of closure of ASDs?

If size of PDA is small with no dilatation of left heart chambers then we do follow-up of child at periodic interval. Generally small PDA is closed between 12-18 months of age.

If moderate sized PDA,then it generally associated with left heart dilatation, then we should close the hole between 6 months-1 year of age.

In large sized PDA: we close between 3-6 months of age.

Can we close PDA by medications?

Yes, but only in premature babies.

What are the other options for closure of Preterm PDA?

If premature PDAs are not getting closed either by conservative management or by medications then either ligation of PDA by surgery or now we can close preterm PDA by device method (without surgery).

Which method is suitable for closing PDA?

It depends on size of PDA as well as on weight of the child.

Small sized PDA:closed now a day by using a device in cath lab by angiography.

Moderate sized PDA:Closing by method is decided by size of ASD and weight of child. Majority can be closed by device method in cath lab by angiography.

Large PDA: by surgical ligation.

Advantages of PDA closure by using Device (Non-surgical method)?
  1. Short hospital stay (2-3 days)
  2. No surgical scar on the body
  3. No need of any medication after procedure
  4. Can attend school from next day of discharge
  5. Can participate in all outdoor activities from very next day
  6. Very less risk as compared to surgery, although very safe procedure
  7. No requirement  of ventilator in most cases
Surgical options?

From back of the child by incision method.