It depends on age, maturity of newborn, weight of baby and the size of PDA.

Preterm Babies:

  • Preterm babies especially less than 1500 grams (1.5 Kg) can present as signs of heart failure, multiple episodes of apnea, dependency on oxygen/ventilator etc.
  • Large PDA can lead to heart failure, decrease in blood pressure (diastolic blood pressure) that can leads to poor blood perfusion in various organs of the body.
  • Large PDA babies are prone for the development of pulmonary vascular disease which is irreversible to treat PDA.
  • Hypoperfusion may lead to renal impairment, necrotizing enterocolitis, feeding intolerance, and hemorrhage in brain.

Term Babies:

  • Newborns that are mature and having good weight at birth are generally not having any symptoms due to PDA itself.
  • In small sized PDA, child is generally asymptomatic and comes to know by a pediatrician by hearing an abnormal heart sound (murmur) on routine examination.
  • Moderate to large sized PDA are generally asymptomatic in first few days of life.
  • Around 1-2 months of age, child can present as feeding difficulty, sweating over forehead, suck-rest-suck cycle on feeding, poor weight gain etc. Sometimes, child if not treated, can have signs of pneumonia including hospital admission and occasional requirement of ventilator.

PDA treatment in babies depends on maturity level and weight of babies.

Preterm babies needs conservative followed by drug treatment and if these measures are not successful then surgical ligation of PDA or if suitable the PDA can be closed in cath lab by using a device (without surgery).

PDA closure in cath lab (without surgery)

  • For closing of PDA by using device, size of PDA as well as weight of child has to be taken in consideration.
  • PDA is closed by using blood vessels in thigh. These vessels are connected to the heart. We pass catheters and wires through these vessels for crossing the PDA.
  • We put an umbrella shaped device across the PDA.
  • Within six months, device become endothelized and become a part of heart for life long.
  • There is no need of any medicine after this procedure (THIS IS NOT A SURGERY). Child can be discharged next day and can attend schools as well.

Is this procedure safe?

In today’s modern era, this procedure is extremely safe.

Coarctation of aorta can be managed either by drug treatment or by balloon dilatation/stenting in cath lab or by surgery.

  1. Drug therapy is meant only for controlling the high blood pressure.
  2. In newborn period, surgery is the treatment of choice.
  3. But in neonates, if left ventricle pumping is poor, balloon dilatation of COA in cath lab is advised (by angiography).
  4. Balloon dilatation is a procedure of choice in childhood and adolescent.
  5. If weight of adolescent is more than 25-30 kg then instead of ballooning, we can put a stent across the narrowed segment with good result.
  6. Stenting is better than balloon as there is almost no chance of re-coarctation of aorta by stent.
  7. This procedure is generally done in conscious sedation. Patient can be discharged on next day and can perform daily activities without any risk.
  8. After ballooning/stenting, there is no need of any medication.
  9. These procedures are safe now a day.

There is a great vessel in heart known as aorta which supplies pure blood to different parts of the body.

Aorta is having a valve at its origin.

When there is an obstruction either at the level of valve, below or above the valve, it is known as valvular, subvalvular or supravalvular aortic stenosis respectively.

The most common etiology of aortic valve obstruction in neonates and is bicuspid aortic valve.

Child presentation depends on age of the child and severity of the obstruction.

If newborn babies are having severe variety of aortic stenosis then they are generally in shock due to low perfusion to the body.

In mild form, children are asymptomatic and illness is detected on routine examination by a child doctor by hearing an abnormal heart sound in the form of murmur on clinical examination.

As severity of obstruction increases, child presents with early tiredness, decrease exercise tolerance, chest pain, loss of consciousness (syncope) and in later stages with shortness of breath (dyspnea due to heart failure) and rarely sudden cardiac death.

Best modality for detection of aortic stenosis is by ECHOCARDIOGRAPHY.

  • Treatment depends on “age of presentation” as well as on “severity of obstruction”.
  • Newborn babies with severe aortic stenosis are generally present in shock and will need urgent balloon dilatation of aortic valve after birth.
  • Child with mild to moderate obstruction, generally child is asymptomatic and needs medical follow-up at periodic intervals.
  • In severe obstruction, patient needs immediate intervention for resolving the obstruction.
  • In Valvular aortic stenosis, treatment of choice is to do ‘’Balloon dilatation of Aortic valve’’.

Balloon procedure details

  • This procedure is done in cardiac cath lab by angiographic technique without open heart surgery.
  • This procedure generally done by using femoral artery (groin vessel), but sometimes, we use axillary arteries (this is a retrograde method, in this we cross the valve from above).
  • Valve needs to be crossed by wire and catheters followed by balloon dilatation of aortic valve. In bigger children and adolescents, we use pacing catheter in right sided ventricle for stabilizing the balloon during its inflation.

Is this procedure safe?

  • Majority of these procedures are safe.
  • There is a great vessel in heart known as “pulmonary artery” which supplies blood to both lungs. Pulmonary artery has a valve.
  • When there is an obstruction at the level of valve, it is known as valvular pulmonary stenosis.
  • Valvular pulmonary stenosis can occur either as an independent lesion or can be associated with other cardiac diseases like TOF.
  • Child can be presented in various forms depending on age of the child and severity of the obstruction.
  • Newborn babies if symptomatic are having severe or critical obstruction in pulmonary valve and presented as a shock lesion with low blood supply to body parts.
  • In children, if PS is mild, then children are asymptomatic and illness is detected on routine examination by a pediatrician by hearing an abnormal heart sound in the form of murmur on examination.
  • As severity of obstruction increases, child presents with early fatigue, decrease exercise tolerance, syncope, shortness of breath rarely sudden cardiac death.

 

For diagnosing pulmonary stenosis, “echocardiogram” needs to be done.

Treatment of valvular PS depends on its severity and age of presentation.  

In Valvular pulmonary stenosis, treatment of choice is to do ’Balloon dilatation of pulmonary valve’’.

  • Newborn babies with severe/critical pulmonary stenosis: Urgent balloon dilatation of pulmonary valve is recommended.
  • Child with mild to moderate obstruction: medical follow-up at regular intervals.
  • In severe obstruction: Immediate intervention in the form of ballooning is advised.

When echo gradient is more than 60 mm Hg or in critical valvular pulmonary stenosis in neonates, ballooning is indicated.

Balloon procedure details

  • This procedure is done retrogradely in cardiac cath lab by angiographic technique without open heart surgery.
  • This procedure generally done by using femoral vein, but occasionally we use neck vein (internal jugular vein) for crossing the pulmonary valve.
  • Valve is crossed by wire and catheters followed by balloon dilatation of pulmonary valve.

Is this procedure safe?

  • Majority of these procedures are safe in modern era.

 

It is the defect in septum, situated between 2 atrias.

Types of ASD

Ostium secundum atrial septal defect (OS-ASD) is the most common type of ASD. Other types of ASDs are Ostium primum defect, coronary sinus defect, sinus venosus type of defect in atrial septum.

Presentation

Majority of children are asymptomatic. As majority is asymptomatic, ASD can be easily missed in early childhood. On careful examining the child, we can hear some abnormal sound in left upper chest area.

DIAGNOSIS OF ASD

ASD can be suspected clinically and diagnostic modalities for ASD are ECG, chest X-ray but confirmatory test is by doing echocardiography (ECHO) from chest of the child.

ECHO is a non-invasive test and it is not painful to the child. This test generally takes 5-10 minutes for completion.­

In older children, sometimes another modality of ECHO is required names as transesophageal echocardiography (TEE) that needs to be done by putting an echo probe through the food pipe.

There is no role of medicines in closing the ASD.

Ostium primum, sinus venosus or coronary sinus type of atrial septal defects can be closed only by surgery.

Only ostium secundum (OS-ASD) with good rims around the defect can be closed in cath lab by putting an umbrella device (angiographically, without surgery) or else has to be closed by surgery.

Ideal age of closure of primum defect is between 2-3 years of age, sinus venosus defect (SVC/IVC type): around 5 years of age.

Ostium secundum defect (OS-ASD) generally closed between 2-4 years of age.

How we close OS-ASD (ostium secundum) by using a device? 

ASD can be closed in cardiac cath lab (angiographically) by using thigh blood vessels that are connected with the heart in a beating heart.

Procedure is generally done under conscious sedation (sometimes in general anesthesia).

Patient can be discharged on next day and can perform daily activities without any fear. Within six months, device become endothelized and become a part of heart for life long.

This method of closure of ASDs does not produce scar on the chest. Patient needs to take tab aspirin for 6 months post-procedure.

Is the procedure safe?

Generally safe.

Congenital heart diseases are the heart diseases that are present at birth.

These can be in the form of holes in the heart, abnormalities in great vessels, abnormal vessel connections, coronaries anomalies, vessel obstruction, valve anomalies etc.

Types of CHDs:

Congenital heart diseases are: VSD (ventricular septal defect), ASD (atrial septal defect), PDA (patent ductus arteriosus), AV canal defect; TOF (Tetralogy of Fallot), TAPVC, Truncus arteriosus, VSD with pulmonary atresia, Ebstein’s anomaly etc.
Coronary arteries may also have congenital issues like abnormal origins that can leads to heart failure.

Other form of CHD’s is narrowing in cardiac valves like aortic or pulmonary valves (aortic or pulmonary stenosis) or narrowing in aorta (coarctation of aorta) or in pulmonary artery (PA).

Presentation:

Can present in different ways, e.g babies are blue since birth, shock like state.

In infancy, as shortness of breath, increase sweating, difficulty in feeding, swelling on the body, blueness of fingers & lips.

Older children presents as easy fatigability (tiredness), unconsciousness, chest pain, and abnormal heart beat.

Management of acyanotic congenital heart diseases (#ASD, #VSD, #PDA, #RSOV, #coronary cameral fistula) depends on the type of diseases.

Acyanotic heart diseases (those without bluish discoloration of lips & fingers) can be managed by medicines or surgery or cardiac cath interventions in cath lab.

Medical management is by giving medicines for controlling symptoms of heart failure.

If heart failure is not controllable by medicines then we need to plan closure of that cardiac hole (responsible for heart failure) either by surgery or if suitable then by putting an umbrella device across the defect so that it can be closed, this has to be done in #cath lab angiographically.

Now a day, varieties of cardiac holes like PDA, VSD, ASD, ruptured sinus of Valsalva, coronary fistulas, pulmonary artery to atrial fistula etc can be closed in cath lab (without surgery) by putting a device angiographically (Device closure procedures).

There may be an obstruction or narrowing in cardiac valves or vessels (severe aortic or pulmonary valve stenosis or coarctation of aorta; congenital heart disease).

These can be treated in cath lab by inflating a balloon across the valve or vessel so that valve or vessel can be opened known as Ballooning of Aortic or Pulmonary valves, ballooning of coarctation of aorta or pulmonary artery.

In vessel stenosis or obstruction like coarctation of aorta or pulmonary artery narrowing, instead of using balloon, a stent (coarctation of aorta stenting or pulmonary artery stenting) can be placed across the narrowed vessel for opening it.

Result of stent deployment is better as compared to balloon dilatation.

Newborns those require interventions are generally sick and require NICU care including ventilator support.

In cyanotic heart diseases with restrictive PFO with d-TGA, PFO can be opened by using a balloon (Balloon atrial septostomy, BAS).

Sometimes, instead of using balloon, we need to place a stent across the defect if not suitable for balloon (inter-atrial septal stenting, IAS stenting) in the same situation.

In duct dependent pulmonary circulation (in pulmonary atresia), patent ductus arteriosus (PDA) is the sole supply for the lungs, PDA can be palliated by putting a stent (Patent ductus arteriosus (PDA) stenting) so that blood supply to the lungs can be restored.

In neonates with severe coarctation with left ventricle dysfunction, we need to palliate this issue by inflating a balloon across the narrowed segment (Coarctation of aorta (COA) balloon dilatation) so that afterload on left ventricle can be reduced and when the contractility of left ventricle improved, surgical repair can be taken up.

Kawasaki disease is a disease that affects skin, mouth, and lymph nodes. Most commonly affected age group is around 5 years of age.

Etiology of this disease is not known.

Kawasaki Clinical Features

Clinical features are divided into 3 groups:

Acute phase: Last up to 2 weeks approximately. Most common symptom in this phase is fever that usually last for 5 days.

Other symptoms may be:

  • Redness in the eyes
  • Red, dry, cracked lips
  • Rash on the body
  • Swollen lymph nodes
  • Swollen tongue with a white coating and big red bumps (called “strawberry tongue”)

Second Phase: Usually begins within 2 weeks of when the fever started, begins with peeling of skin. Other symptoms can be joint pain, abdominal pain, diarrhea.

Kawasaki Diagnosis

Kawasaki disease diagnosed when child with fever lasting 5 or more days and at least four of these symptoms:

  • redness in both eyes
  • a rash in the chest, stomach, or genital area
  • a large swollen lymph node in the neck
  • changes around the lips, tongue, or mouth
  • changes in the fingers and toes, such as swelling, color change, or peeling
  • red, swollen palms of hands and soles of feet

Kawasaki Treatment

Treatment should be started as soon as possible, ideally within 10 days of when the fever begins. It is by giving IV Immunoglobulin along with high dose of aspirin. Later on low dose aspirin to be continued for a long duration depending on how much coronary arteries are affected.

Most child recover completely.

Complications

If not treated properly especially not treated in first 10 days of illness, Kawasaki disease may affect heart especially coronary arteries. Can leads to vasculitis of coronary arteries.

Cardiac arrhythmia or abnormal functioning of some heart valves also can occur.

This is a rare disease in pediatric age groups.

Causes of DCMP

Anomalous origin of the left coronary artery (ALCAPA), myocarditis common causes include viral infection with coxachie, echo, HIV, measles, mumps and rubella etc.

Sometimes, cause is familial with autosomal dominant, recessive and x-linked inheritance describes.

Systemic causes: can be metabolic, endocrine, storage, mitochondrial and connective tissue disorders

Clinical Presentation

If DCMP is severe then child is generally in symptomatic heart failure. Child presents with difficulty in breathing, cough, cold, poor feeding, edema over feet etc.

Investigations

Chest x-ray shows cardiomegaly, increased pulmonary vascularity due to fluid overload in lungs alveoli, in association with liver enlargement hepatomegaly, raises doubt of DCMP.

Transthoracic echocardiography confirms the diagnosis by showing dilatation of cardiac chambers, mitral regurgitation, decreased cardiac pumping function.

In pediatric age groups, coronary arteries should be assessed very carefully as abnormalities in coronary arteries also leads to similar picture.

ECG differentiates coronary abnormalities cause from non-coronary causes of DCMP. Generally ECG shows sinus tachycardia, left atrial enlargement etc.

Treatment

Medical treatment

This gives symptomatic relief. Drugs that commonly used are diuretic, ACE inhibitors, B-blockers. Digoxin should be used very carefully in case of myocarditis.

DCMP patients are very prone for arrhythmias so anti-arrhythmic drugs are also used whenever required.

Poor LV function is always at risk for blood clot formation in cardiac chambers so anti-clot forming drugs like aspirin is also used wherever indicated.

Mechanical and surgical treatment

Left ventricular assist devices (LVAD) and biventricular assist devices, although not widely available and quite expensive will have a role at least in short term, and act as a bridge to heart transplant.

Cardiac Transplant: Indicated in end stage heart failure.

Prognosis

Death due to DCMP is highest in first year after diagnosis due to intractable heart failure. Late deaths are due to arrhythmia.