What is Pediatric Cardiology?
Pediatric Cardiology is the pediatric sub-speciality which deals with heart diseases in children, and congenital heart diseases in fetuses, children and adults. A pediatric cardiologist primarily treats patients with congenital heart disease (CHD), whether a child or an adult, and we also treat children with other types of heart diseases such as Rheumatic Heart Diseases, Kawasaki Disease, myocarditis and cardiomyopathies.
What is the burden of congenital heart disease in India?
In India, every year, approximately 240,000 children are born with CHD of which about one fifth would need early intervention to survive the first year of life. There are an estimated 25 million children, adolescents and adults with untreated CHD in India.
What is the difference between a Pediatric Cardiologist and a Pediatric Cardiac Surgeon?
A Pediatric Cardiologist is a medical doctor who treats children with heart disease. They will diagnose the disease, treat and stabilize the patient medically and refer the patient for cardiac surgery, if required. A lot of heart diseases may require only medical management by the pediatric cardiologist and no procedural or surgical treatment. For patients who undergo surgery, after the surgery, the pediatric cardiologist plays an important role in the ICU treatment and continues the long term medical treatment and follow up of the patient. Interventional Pediatric Cardiologists like Dr Sen also do minimally invasive procedures such as device closures and stenting in the cardiac cath-lab which are alternatives to open heart surgery.
A Pediatric Cardiac surgeon is a surgical doctor who operates on children with heart disease. They perform corrective and palliative surgeries to correct congenital defects in a child’s heart.
The roles of a pediatric cardiologist and pediatric cardiac surgeon are not interchangeable. They work as a team for the optimum care of the child with heart disease.
What are the symptoms and signs in a child where one should suspect CHD and get a cardiac evaluation?
Babies with CHD might present with difficulty in feeding with suck-rest-suck cycle, poor weight gain, excessive sweating with feeds, recurrent respiratory infections (pneumonias), fast breathing and bluish discolouration of skin. Other children might have reduced activity and energy levels and complain of getting tired easily. Children with cyanotic heart disease will have low oxygen levels when SpO2 is checked. Some patients might have loss of consciousness or seizures.
We live outside Mumbai; how can we arrange a video consultation first?
In my homepage, there is a link for booking both physical and video consults. https://www.narayanahealth.org/c/mumbai/cardiology-paediatrics/dr-supratim-sen It is easy to book a video consult from the website or the NH care app. For any queries, you can email me at: supratim@drsupratimsen.com
If my child was growing well and had normal activity till recently but is now getting excessively tired or has fast breathing, can that be due to a heart disease?
Yes, conditions such as myocarditis, cardiomyopathy and even rheumatic heart disease present with progressive symptoms of heart failure in a previously normal child. And these symptoms may be the first indication that the child has a heart problem.
If my child is diagnosed with a heart defect, what should I do next? Is surgery always necessary? Can the defect close spontaneously if I wait?
It is essential to consult a pediatric cardiologist if your child is diagnosed or even suspected to have a cardiac defect. The pediatric cardiologist does a detailed clinical and echocardiographic evaluation and makes a complete diagnosis. They will also advise you regarding the treatment options available for the diagnosed CHD, the optimum timing of treatment, and whether surgery or a minimally invasive intervention in the cath-lab is recommended.
While some CHDs do close spontaneously, it is essential that the patient is evaluated by a pediatric cardiologist, and only if they advise that spontaneous closure is likely should the parents wait. By waiting for weight gain or spontaneous closure in large defects which should have been closed early, the patient develops complications and becomes inoperable. Hence this decision always needs to be taken only after consulting a pediatric cardiologist.
Does insurance cover pediatric heart procedures like ASD/VSD device closures?
This depends on the insurance policy and the insurance company. Most corporate insurance policies from your employer will cover the child’s congenital heart defect treatment. You will need to check with our hospital once the treatment plan is finalized.
How are the outcomes if a child is diagnosed to have a congenital heart defect (CHD)?
Pediatric cardiac services have been available in our country since the 1990s, and over the last two decades not only have the number of dedicated pediatric cardiac centers increased, the quality of care and results have also improved dramatically, with results in the best of these centers now being comparable to European and North American centers. This gradual and steady improvement in treatment outcomes in pediatric cardiology has had a massive positive impact on the lives of patients born with CHD. Moreover, awareness of CHD among primary physicians and pediatricians has increased, and now patients are detected early and referred for treatment early, which improves outcomes. In fact, fetal echocardiography is now available in most metropolitan areas, enabling antenatal diagnosis of critical congenital heart defects, allowing these babies to receive necessary lifesaving treatment within a few hours of birth. In the current era, even a newborn baby undergoing surgery for critical congenital heart disease has more than 90-95% chance of recovery.
With technological advances in pediatric cardiac care, defects such as holes in the heart (ASD, VSD), patent vessels (PDA), blocked valves and blocked vessels can now be treated by minimally invasive techniques by transcatheter interventions such as device closures, balloon valvotomies and stent angioplasties. This means that patients as young as a few hours of life can now be treated less invasively in the cardiac catheterization lab as an alternative to invasive open-heart surgery. While device closures and balloon valvotomies are being done for the last 2 decades in India, training of pediatric cardiac specialists and an increase in the number of tertiary pediatric cardiac programs in the country means that these highly specialized techniques are now widely available with excellent long-term results.
In the last 5-10 years, transcatheter implantation of artificial valves in the cath-lab instead of sending these patients for open-heart surgery has gained popularity in developed countries, and this technology is now available in India too, although the procedure is expensive at present and out of reach of the majority of patients. Patients can thus have pulmonary valve and aortic valve implantation in the cardiac cath-lab via vessels in the groin as an alternative to undergoing open-heart surgery.
In conclusion, in the present era, if a child is detected to have a congenital heart defect, the long-term prognosis and future for the patient are excellent.
Does my child definitely need surgery for a hole in the heart?
Not all cardiac defects need surgery. A lot of defects can be treated with minimally invasive device closure and balloon valvotomies.
We have been told our child needs surgery; can we get a second opinion on whether a non-surgical (device) closure is possible?
Yes. If parents are concerned, it is always advisable to get a second opinion from another senior pediatric cardiologist. For second opinions, you can consult me (https://www.narayanahealth.org/c/mumbai/cardiology-paediatrics/dr-supratim-sen) or email me: supratim@drsupratimsen.com
How long will my child take to recover after a device closure?
We usually discharge the child home one day after the device closure. He/ she should rest at home for the next 7-10 days and can restart school after 2-3 weeks.
Will my child be able to play sports or lead a normal life after a device closure?
Yes. Device closures are curative procedures. We generally advise the child to rest at home for around 2 weeks after any device closure, after which they can resume all normal activities. They will lead a normal life and have a normal life-expectancy.
What are the complications of device closures and how often do they occur?
The known complications of device closure in general are device dislodgement or embolisation. This can happen in 1-2% of cases.
VSD device closures have a 1% risk of heart block or aortic regurgitation.
PDA device closures have a 1% risk of aortic coarctation.
If I do a device closure for my 1 year old child, does this device need to be upsized once he grows into an adult?
No. The device closure of a congenital heart defect is a one-time permanent treatment. Within 6 months of the device closure, the internal cellular layer of the heart, the endothelium, covers the device. So as the child grows, there is no risk of the defect outgrowing the device.
Are any medications required after device closure?
Yes. We give Aspirin for 6 months after any ASD or VSD device closure.
Can it be diagnosed during pregnancy?
TOF can be diagnosed with fetal echo, and can be detected as early as 16-18 weeks gestation. In all fetal echo diagnosis of TOF, we need to clearly delineate the antegrade pulmonary blood flow of the pulmonary stenosis and differentiate this from pulmonary atresia. In pulmonary atresia, there is no connection between the right ventricle and the pulmonary artery, and the pulmonary arteries are supplied by either a PDA or by major aortopulmonary collateral arteries (MAPCAs).
Thus, TOF with pulmonary atresia is a duct dependent condition which will require delivery in a cardiac center and may require a neonatal intervention or surgery.
What advice do we give to patients if TOF is diagnosed during pregnancy?
TOF generally has a good long-term prognosis, and we tell the parents about the treatment options available. The long-term outcomes often depend on the sizes of the branch pulmonary arteries. Pulmonary atresia is a more complex disease and will require multiple surgeries in the lifetime of the patient.
After birth, what symptoms does a patient with TOF have?
Most babies born with TOF may have mild desaturation at birth. Generally, in TOF with adequate antegrade pulmonary blood flow, if the SpO2 is above 80% and the baby has no respiratory distress, no neonatal intervention is required.
What are cyanotic spells? How are these managed?
Older children with TOF, usually above the age of 2-6 months, have a baseline cyanosis, and episodic worsening of the cyanosis on crying. The worsening cyanosis can lead to hypoxia, and if it does not resolve spontaneously or with treatment, this may progress to hypoxic seizures and stroke. These episodes are known as cyanotic spells.
The first step in the treatment of cyanotic spells is to calm the baby and to put the baby in a knee-chest position. Other measures, which can be done at any local pediatric hospital include oxygen by facemask, normal saline bolus, and slow intravenous bicarbonate injection along with sedation with intravenous morphine. Intravenous Metoprolol also helps in the treatment of cyanotic spells. Frequent or refractory cyanotic spells need to be treated by intubation and ventilation, followed by early cardiac surgery.
At what age do we plan for complete surgical correction in these patients?
Studies have shown that the best outcomes of total surgical correction of TOF are seen if the child is operated between 3 months to 9 months of age. In India, we offer elective surgery to all stable patients with TOF from 5-6 months of age, but it is not uncommon for us to see late presenters who are diagnosed after 1 year of age. These patients are operated soon after diagnosis. Of late, at our center, we have started offering early Total correction of TOF even to babies as young as 3-4 weeks of age, if the cyanosis is significant, and the branch PA anatomy is otherwise good.
A syndromic child with multiple congenital anomalies or those with small hypoplastic branch pulmonary arteries are often offered a palliative surgery as the first step, with total correction planned 6 months to 1 year later.
Is it a single surgery for TOF patients, or do they need more than one surgery in their lifetime?
Children who undergo total correction of TOF with preservation of the pulmonary annulus require only a single surgery. In some patients, the pulmonary annulus is extremely hypoplastic, and needs to be enlarged with a transannular incision and patch during the surgery. In these patients, there will be severe pulmonary regurgitation after TOF surgery, and they will require a pulmonary valve replacement to achieve pulmonary valve competence and prevent severe right ventricular dilation around 10 to 15 years after the TOF surgery.
Are any minimally invasive options available for the treatment of these patients who require a pulmonary valve replacement later in life?
Over the last decade, transcatheter pulmonary valve replacement in the cardiac cath-lab has become a regular treatment option for patients who require a pulmonary valve replacement. We now have various options for transcatheter pulmonary valve implantation, and although this is still more expensive than surgery, it is slowly becoming a viable treatment alternative.
What is the long-term outcome of these patients?
Patients operated for Tetralogy of Fallot will usually have a normal quality of life into adulthood, and pregnancy is safe in operated female TOF patients. They have normal exercise tolerance and excellent long-term outcomes.
In patients with TOF with pulmonary atresia, outcomes are variable and depend on the sizes of the branch pulmonary arteries at birth. Those patients with non-confluent small branch pulmonary arteries can be offered multiple staged surgeries, but their long-term outcomes are not very good.
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Book at NH SRCC Children’s Hospital or via the link, the NH Care App
or email me on supratim@drsupratimsen.com for guidance on how to set up a pediatric cardiac video consult.
Dr. Supratim Sen | Senior Consultant Interventional Pediatric Cardiologist | NH SRCC Children’s Hospital, Mumbai.
Clinic: NH SRCC Children’s Hospital. 1, 1A Haji Ali Park. K Khadye Marg. Mahalakshmi. Mumbai- 400034