Hypertrophic cardiomyopathy (HCM/HOCM) is characterized by thickening of heart walls with no cause clinically. This can results in poor capability of the heart for pumping of blood forward to the body.

It is a genetic disease.

Symptoms

Child can be asymptomatic or may have symptoms like

  • Easy fatigue, irregular heartbeat, chest pain, fainting attacks, difficulty in breathing due to heart failure etc.
  • Child can be prone for sudden cardiac arrest as well.

Diagnosis

  • ECG
  • Echocardiogram of the child
  • Cardiac MRI
  • Genetic testing should also be done for confirmation.
  • Sometimes, stress testing is also advised.
  • Rarely cardiac catheterization

Treatment

  • Drugs like beta blockers, diuretics, disopyramide etc
  • If prone for cardiac arrest then automated implantable cardiac defibrillator(AICD) is advised for abnormal heart beats and rhythm
  • Alcohol septal ablation
  • Surgery can be done in the form of excessive muscle resection (septal myomectomy) and in later stages; the only option left is transplant of the heart.

Prognosis

HCM is having annual mortality of approximately 1% per year.

  • Infective endocarditis refers to infection of heart endocardium mainly the valves.
  • It can affect various organs of the body like kidney, brain, spleen, lungs, skin, eyes etc.
  • Usually caused by bacterial infection but can also be infected by fungus and other germs as well.
  • Streptococci or staphylococci are most commonly involved bacteria that infect the heart endocardium.
  • Infective endocarditis can affect any age group. Males are more affected than females.
  • High risk children are those who are having congenital heart disease, damaged heart valves, artificial heart valves etc.
  • Endocarditis needs to be treated aggressively otherwise can destroy heart valves and leads to life threatening condition.
  • Symptoms and signs due to infective endocarditis can be
    • Fever, malaise, easy fatigue, joint pain, shortness of breath, cardiac murmur, bleeding.
    • Septic embolism, hemorrhage in eye, brain; renal or splenic infarcts.
  • Diagnosis: Requires high index of suspicion
    • Blood culture and sensitivity
    • Echocardiograph
    • CT scan
    • Cardiac MRI
    • Clinical criteria: Modified Duke’s criteria are used for diagnosis.
      • Can be suspected in child with a heart murmur who is having fever with no focus of infection.
      • Suspicion should be very high if any of the following is present: blood cultures are positive, recent invasive procedures.

Treatment of IE

  • It is mainly by giving intravenous antibiotics depending on sensitivity of grown bacteria on blood culture.
  • Generally antibiotics are given for 4-6 weeks depending on the bacteria grown on culture.
  • Sometimes, cardiac surgery is advised when valves are severely affected.
  • Removal of source of infection is advised if found.
  • It is characterised by damage of heart structures by Rheumatic fever.
  • Generally affected cardiac structures are mitral and aortic valves. This can results in either severe narrowing or severe leak of the valves. This can be detected either as an emergency or can be detected on routine examination.
  • Mitral valve is more affected as compared to aortic valve.
  • Effect of rheumatic fever on the heart (rheumatic heart disease) can be avoided by treating or preventing rheumatic fever.
  • Children with age group between 5 years to 15 years are prone for rheumatic fever and its consequences. This is generally seen with recurrent throat infection by bacteria known as Streptococcus.

Clinical presentation

  • Symptoms with RHD vary depending on its onset whether acute or chronic.
  • If mitral and aortic valves are affected acutely then child may have shortness of breath as predominant symptoms even in resting phase as well.
  • If heart walls (myocardium) or outer covering of the heart (pericardium) are affected then children may have pain in chest.
  • Other clinical feature that child can have joint pain etc.

Diagnosing of Rheumatic Heart Disease

  • For diagnosing RHD, clinical features (symptoms and signs) can be noticed on asking history and examining the child.
  • On examination, doctor can heart an abnormal heart sound (murmur), can see affected joints etc.
  • Modified Jone’s criteria is used for diagnosing the Rheumatic Fever.
  • Lab investigations like blood test, chest x ray, ECG, throat culture are advised for diagnosis.
  • Confirmatory test is by doing “echocardiogram of the child”.

Treatment of RHD

  • This is in the form of medicines, cardiac interventions or by open heart surgery.

Drug Treatment:

  • After assessment, antibiotic treatment is advised as preventive measures for treating throat infection.
  • If required then anti-inflammatory medicines for treating joint related problems like pain etc.
  • Sometimes, bed rest is also advised depending on the severity of RHD.
  • If Rheumatic fever is diagnosed, then child should be in follow up with paediatric cardiologist.
  • If RHD is confirmed then child needs to take medicines for a long duration for preventing further damage to heart structures.

Cardiac Intervention:

  • If mitral valve is obstructed then cardiac intervention in the form of balloon mitral valvuloplasty (BMV) can be planned.

Open Heart Surgery:

  • If valve is not suitable for BMV, then mitral or aortic valve can be replaced by an artificial valve by open heart surgery (MVR/DVR). This is seen in Chronic RHD.
  • Long QT syndrome (LQTS) is characterized by abnormality in repolarization of the heart leading long QT interval.
  • Long QT can occur due to noise, exercise etc.
  • Long QT syndrome can be present at birth or can be seen in older children.
  • Causes: genetic, medicines, electrolyte imbalance, drugs like antimicrobials, anti-arrhythmic, antipsychotic medicines etc.
  • Classified into various types, most common is Long QT 1.

Symptoms

  • This can lead to abnormal heart beat and can lead to fainting attacks (syncope), palpitation, drowning, sudden cardiac death

Diagnosis

  • ECG
  • Holter analysis

Management

General measures

  • Avoiding aggressive exercise
  • Enough intake of potassium, calcium rich diet etc

Drug Treatment

  • Beta blockers, mexiletine

Other treatment measures

Single ventricle means that one of two ventricles (lower chambers of heart) is very small in size.

Examples of single ventricle related congenital heart diseases are:

  • Double inlet ventricle (commonly left ventricle)
  • Heterotaxy syndromes (complex cardiac anomalies)
  • HLHS (Hypoplastic left heart syndrome)
  • Tricuspid atresia
  • Few cases of double outlet right or left ventricles

Symptoms in single ventricle physiology

Symptoms due to single ventricle depend on whether there is narrowing in pulmonary artery present or not (pulmonary stenosis, PS).

  • When there is no PS, then child will have signs of cardiac failure in the form of shortness of breath, poor weight gain etc.
  • When there is severe PS, then child will have bluish discoloration of lips and fingers (cyanosis).
  • Very rarely, baby may have balanced circulation in lungs (that is limited blood supply to both lungs); these babies will not require early treatment in newborn period.

Diagnosis of Single Ventricle

  • Pediatric echocardiogram is required for diagnosing this anomaly.
  • Occasionally, CT scan is also required when echocardiogram is not conclusive.
  • Other investigations are ECG and chest x-ray.
  • Sometimes, “diagnostic cardiac catheterizationneeds to be done when echocardiogram/CT angiogram is not conclusive or when we have to determine whether child is operable or not.

Treatment of Single Ventricle

                  Treatment is by doing “Surgery” in most cases.

Mode of operation depends on type of single ventricle cardiac defect.

In first stage:

In cases of severe pulmonary stenosis/pulmonary atresia, a graft is placed between main blood vessel artery branch (branches of aorta: left subclavian or left innominate artery) to the pulmonary artery. This is known as BT shunt or we can palliate by doing stenting of patent ductus aretriosus (PDA stenting) instead of doing BT shunt.

When blood flow to the lungs is in excess then we put a band in main pulmonary artery (PA banding).

Other operations that we need to do in more complex cardiac problems like HLHS is known as Norwood Operation.

In second stage:

                  “Glenn shunt” has to be done that is a connection between superior vena cava and pulmonary artery with take down of BT shunt or PDA stent. This operation is done between 6 months to 1 year of age.

In third stage:

“Fontan operation” is recommended in which inferior vena cava is connected to pulmonary artery as well. It is done between 2-4 years of age.

 

Prognosis

Now a day, prognosis after doing third stage of operation is good as compared to previous years.

Pregnancy

Pregnancy is a relative contraindication after doing all surgeries in single ventricle as carries high risk both to mother and baby.

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.