One or more of the following tests will be conducted to help understand the heart disease.
Echocardiogram: The most important test is the Echocardiogram. This is the sonogram of the heart. The heart and its vessels can be seen by this test. A paediatric cardiologist works with a specially adapted sonography probe to study the structure of the heart from the inside. The windows to look at the heart are from in between the ribs and above and below the rib cage. Results are instant. It typically takes 15minutes or longer to do this. Your baby may need to drink a medicine to make him/her sleepy enough (Sedation) to cooperate. Most of the time we try to distract them by showing them cartoons or songs on the mobile phone.
Pulse Oximetry: this tells us about the oxygen level in the body. A clip or a wrap around patch is placed on a finger (or toe) till a stable display of the oxygen number is obtained. The result is abnormal in babies with impure blood flowing in their body.
ECG: this is the short form of electrocardiogram. It is obtained by connecting the chest to an electrical signals reader via many wires. It typically takes 5minutes to perform. It gives valuable information on the electrical activity of the heart which influences the heart rate and rhythm.
BP monitor: The oscillometric and the manual methods of BP monitoring are available for use in the clinic.
Chest X Ray: a large heart size and extra/less of blood flow to lungs may be seen on this.
CT scan/MRI: A few children need this additional test to better define vessels. It needs placement of an IV line.
Cardiac Catheterization (Diagnostic): this test needs admission. The child is sedated by giving medicines through the IV. Tiny tubes are passed inside the blood vessels leading to the heart, under live XR vision in a special lab (cath lab) by the paediatric cardiologist. Vital information about the pressure, oxygenation of blood and structure of the heart is obtained through these tubes.
3D Printed Heart Model: This is a model of the particular baby’s heart. It is made by processing information from CT scan/MRI (see above). It helps the doctors plan the treatment better in complex heart defects
Holter Monitor: this is portable form of the ECG. The leads and the device are taped to the chest and the patient gets to be at home for the duration of the test (1-3 days).
Exercise Stress Test: The child (generally over age of 12 years) is made to run on a treadmill while vital parameters and symptoms are continuously monitored by the team. This test is done to gauge the exercise capacity of the child’s heart.
6minute Walk Test: the child is asked to walk for 6minutes while some vital parameters are monitored and the distance they walk is measured at the end of 6minutes. This is a useful way to test baseline status of a child with a diseased heart and the response to treatment.
Blood tests: The hemoglobin level of the child with blue baby syndrome can give an idea of the level of impure blood in the body. The BNP or NT proBNP level can give an idea of the degree of heart failure in a child with cardiomyopathy. Cholesterol profile is performed in the overweight child with family history of high cholesterol.
Treatment:
What is the Treatment for a Heart Defect?
Only certain milder forms of holes or malformed valves can be left alone but still have to be kept under follow up. The rest of the various kinds of heart defects require treatment to repair the defect. The hole needs to be closed, the valve needs to be opened up and the wrong connection needs to be reconnected normally.
Surgery: most heart defects are repaired by surgery. The surgery (open heart surgery) involves entering the heart by making a cut in its wall after bypassing the heart with a (bypass) machine. Some surgeries can be done off pump as well. While a few require the body to be cooled and the circulation arrested. Open heart surgery has been performed since the 1950s. Successful heart surgery in a baby or child involves an extreme set of skills in the operating surgeons complimented by a vigilant team in the operation theatre. The team consists of perfusionists, nurses and anaesthesiologists. Typically such teams are comfortable handling babies with weight less than 2 kilos to a fully grown person.
Once the baby is shifted to the intensive care unit, another team takes care of his recovery. This may take anywhere from 2 days to more than 2 weeks. The baby needs multiple intravenous medicines, support for breathing, feeding and all round 24 hour care. The paediatric cardiac intensivists take care of these aspects.
Once the baby is discharged home, recovery is generally rapid. For the 5 most common heart defects that are prevalent, babies start thriving: gaining weight and catching up with their milestones and are soon on the way to good health. Most kids come off all medicines at the first follow up visit post surgery. Periodic screening at the paediatric cardiac OPD is essential though.
Cardiac catheterization: Some heart defects can be repaired without surgery. A plug or umbrella shaped device is used to close certain holes. This detachable device can be placed in the hole via a special tube (catheter) inserted in the heart from the groin vessels. The interventional paediatric cardiologist does this under live x-ray guidance. The recovery is rapid as there are no cuts on the skin/muscle etc and the child is discharged home the day after the procedure. This kind of surgery-less procedure is performed in the cardiac cath lab and has become the standard of care for certain types of holes in the heart. Other heart defects handled in the cath lab routinely include narrow valves or vessels and abnormal connections between vessels (PDA).
Medicines: medicines play a very small role in treatment of heart defects. None of the medicines offer a cure of the defect (the only exception is closure of PDA in premature babies using medicine).
Heart/Heart Lung Transplant: The heart of a brain-dead person can be donated to a needy child with a failed heart who has no other surgical option left. A recipient of a donated heart need lifelong medicine and careful follow up. This is done at very few places around the world/India. This is available at our centre.
The best interest of the child is the bottomline for the team. No matter which surgeon or cardiologist in the team you will meet with, the entire team contributes to the care of the child. The proposed treatment plan is discussed in a combined meeting, potential high risk markers are identified so that appropriate preparation can be made. Children are usually admitted in the ward (or ICU) a day prior to the procedure or surgery. Intravenous lines are put and various pre-operative laboratory tests are done. All care is taken to ensure that the child stays pain free and has the least stressful experience in the hospitalization.
Treament by Cardiac Catheterization:
Device closure of certain kinds of ASDs, VSDs, PDAs, collaterals and extra vessels and fistulae.
Balloon valvuloplasties for narrow (stenosed) valves like aortic and pulmonary valvuloplasty.
Stenting of narrow vessels (coarctation, pulmonary artery)
Stenting the PDA
Balloon atrial septostomy for TGA
EP study for RFA
Pacemaker insertion
Treatment by Paediatric Cardiac Surgery:
Patch closure of certain kinds of ASDs, most kinds of VSDs
Ligation of large PDAs.
Arterial Switch for TGA
Double Switch for ccTGA
Intracardiac Correction for TOF, TAPVC, CAVSD and all other suitable heart defects
Aortic arch repair for interrupted aortic arch or arch hypoplasia
Glenn and Fontan Surgeries for Single Ventricle spectrum
Norwood surgery for HLHS
Valve repair surgeries
Ross surgery for defective aortic valves
Mechanical Circulatory Support (ECMO, VAD)
Paediatric Heart Transplantation
Pacemaker insertion
