C
ongenital heart diseases (CHDs) are the most
common birth defects, responsible for nearly one-third of all congenital
birth defects [1]. The birth prevalence of CHD is reported to be
8-12/1000 live births [2,3]. One-fifth of these babies have critical
heart disease requiring very early intervention. Advances in pediatric
cardiology and cardiac surgery have made it possible to repair or
palliate most of the CHDs including the complex ones. If access to
screening, early diagnosis and treatment is available, over 90% of
patients born with CHD survive to adult life with good long-term outcome
[4]. Most middle- and low-income countries lack such advanced level of
care for children with CHD. Considering a birth prevalence of 9/1000,
the estimated number of children born with CHD every year in India
approximates 2,40,000, posing a tremendous challenge for the families,
society and healthcare system. Approximately 10% of infant mortality in
India may be accounted for, by CHDs.
justification for Developing Indian Guidelines
Evidence based recommendations for management of CHD
have been published by task force members from a number of national and
international associations, but these are primarily meant for children
born in high income countries. Applicability of these guidelines to
Indian population with CHD is likely to be limited. Majority of patients
with CHD are not diagnosed in antenatal period and often present late in
the course of the disease. These patients are often underweight,
malnourished and have comorbidities such as recurrent infections and
anemia. Many of the late presenters have advanced level of pulmonary
hypertension, ventricular dysfunction, hypoxia, polycythemia, etc. The
outcome after surgery in such patients are expected to be suboptimal
with longer periods of mechanical ventilation and stay in intensive
care. Modifications in the treatment protocol may be required for
optimizing the outcomes. All these factors justify the need for separate
guidelines for management of CHDs in India, including the timing of
intervention.
A statement on "consensus on timing of intervention
for common congenital heart disease" which originated from a Meeting of
Working Group on Management of Congenital Heart Disease in India, was
published in the year 2008 [5]. This statement was revised and updated
in a subsequent National Consensus Meeting, which was held in New Delhi
after a gap of 10 years, in August 2018. In the intervening 10 years, a
number of pediatric cardiac centres have been established and overall
the numbers of interventions have increased by several folds.
Considering the growing population of post-operative patients including
those needing regular follow-up, we added guidelines and protocols for
follow-up of these patients.
Preamble
1. Every pediatrician/cardiologist/other
healthcare provider must strive to get a complete diagnosis on a
child suspected of having heart disease, with the help of a higher
centre, if needed.
2. The proposed guidelines are meant to assist
the health care provider (pediatrician, cardiologist, pediatric
cardiologist) in managing cases of congenital heart diseases in
their practice. While these may be applicable to the majority, each
case needs individualized care, and exceptions may have to be made.
Guidelines are intended to define practices, meeting the needs of
patients in most, if not all circumstances, and should not replace
clinical judgment.
3. These guidelines are in reference to current
health care scenario prevalent in India. Subsequent modifications
may be necessary in future as the pediatric cardiology practice
evolves.
4. The recommendations are classified into three
categories according to their strength of agreement:
Class I: Is recommended/is indicated. General
agreement that the given treatment or procedure is beneficial, useful
and effective.
Class II: Conflicting evidence and/or a
divergence of opinion or both about the usefulness/efficacy of the given
treatment or procedure. IIa: Should be considered. Weight of
evidence/opinion is in favour of usefulness/efficacy. IIb: May be
considered. Usefulness/efficacy is less well established.
Class III: Is not recommended. Evidence or
general agreement that the given treatment or procedure is not
useful/effective; and in some cases may be harmful.
Aims and Objectives
1. To outline the optimal timing of intervention
in common CHDs.
2. To formulate guidelines and protocols for
follow-up of patients who have undergone surgery/catheter
interventions for CHD.
Guidelines for Individual Congenital Heart Defects
Atrial Septal Defect (ASD)
Diagnostic work-up
:
Physical examination, ECG, X-ray chest, echocardiography and
cardiac catheterisation (in select cases).
Types of Atrial septal defect: Ostium secundum
(~75%); Ostium primum (15%-20%); Sinus venosus (5%-10%); and Coronary
sinus (<1%).
Patent foramen ovale: Small defect in fossa
ovalis region with a flap with no evidence of right heart volume
overload. Diagnosed on echocardiography, is a normal finding in
newborns.
Indication for closure: ASD with left-to-right
shunt associated with evidence of right ventricular volume overload
without evidence of irreversible pulmonary vascular disease (Class
I). Indications for ASD closure remain the same irrespective of the
method of closure.
Contraindications for closure: Severe pulmonary
arterial hypertension or irreversible pulmonary vascular disease
(Class III).
Ideal Age of Closure
Asymptomatic child: 2-4 years (Class I).
For sinus venosus defect surgery may be delayed to 4-5 years (Class
IIa).
Symptomatic ASD: Rarely seen in infants. Present
with congestive heart failure, pulmonary arterial hypertension. Early
closure is recommended (Class I) after ruling out associated
lesions such as left ventricular inflow obstruction, aortopulmonary
window, total anomalous pulmonary venous drainage, etc.
If presenting beyond ideal age: Elective closure
irrespective of age as long as there is left-to-right shunt with right
heart volume overload and pulmonary vascular resistance is within
operable range (Class I).
Method of Closure
Surgical: Established mode (Class I).
Device: For secundum ASDs with adequate rims and
weight of child >15kg (Class I).
Recommendations for Follow-up
Follow-up after surgical closure: Clinical and
echo in the first year only. No further follow-up required if no
residual disease, no pulmonary hypertension or arrhythmia.
Patient/guardians should be explained about reporting to hospital in
case of any cardiac symptoms, or symptoms suggestive of arrhythmias.
Follow-up after device closure: (a)
Anti-platelet agents for total duration of 6 months (b)
Echocardiography: - At discharge, 1 month, 6 months, 1 year, then every
3-5 years.
IE prophylaxis: It is recommended for 6 months
after device or surgical closure. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
Isolated Ventricular Septal Defect (VSD)
Diagnostic Work-up
:
Physical examination, ECG, X-ray chest, echocardiography and cardiac
catheterisation (in select cases).
Classification of Ventricular Septal Defect
Perimembranous: 80%; Outlet or sub-pulmonary
(doubly committed): 5%-7%; Inlet: 5%-8%; and muscular: 5%-20%, these
could be central (mid muscular), apical, marginal (anterior, septal-free
wall area) or multiple, "swiss cheese" type.
Indications and Timing of Closure (All Class I
recommendations)
Small VSD: (No symptoms, normal PA pressure,
normal left heart chambers, no cusp prolapse): (a) Annual
follow-up till 10 years of age, then every 2-3 years; (b) Closure
indicated if patient has an episode of endocarditis or develops cusp
prolapse with aortic regurgitation or develops progressive significant
right ventricular outflow tract obstruction.
Moderate VSD: (a) Asymptomatic (normal
pulmonary artery pressure with left heart dilation): Closure of VSD by
2-5 years of age; (b) Symptomatic: If controlled with
medications, VSD closure by 1-2 years of age;
Large VSD: (a) Poor growth/congestive
heart failure not controlled with medications (furosemide/spironolactone
or enalapril +/- digoxin): As soon as possible; (b) Controlled
heart failure: By 6 months of age.
VSD with aortic cusp prolapse: Any VSD with cusp
prolapse and directly related aortic regurgitation that is more than
trivial: Surgery whenever aortic regurgitation is detected.
Contraindications for Closure: Severe pulmonary
arterial hypertension with irreversible pulmonary vascular disease
(Class III).
Method of Closure
Surgery: Conventionally patch closure is
done. Pulmonary artery banding to be considered for patients with
multiple VSDs, inaccessible VSDs and those with contraindications for
cardio-pulmonary bypass.
Device closure: For VSDs with adequate
rims around defect and weight of child >8kg.
Recommendations for Follow-up
Follow-up after surgery: Clinical, ECG and echo
in the first year only. No further follow-up required if no residual
defect or pulmonary hypertension. Patient/guardians should be explained
about reporting to hospital in case of any cardiac symptoms, or symptoms
suggestive of arrhythmias.
Follow-up protocol for device closure:
Anti-platelet agents for total duration of 6 months.
IE prophylaxis: It is recommended for 6 months after
device or surgical closure. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
Atrioventricular Septal Defect (AVSD)
Diagnostic Work-up:
Physical examination, ECG, X-ray chest, echocardiography and
cardiac catheterisation (in select cases).
Types of AVSD
I. Complete AVSD: Large septal defect with
an atrial component (ostium primum defect) and ventricular component
(inlet septal defect), common atrioventricular valve ring and common
atrioventricular valve. Generally associated with large
left-to-right shunt, pulmonary arterial hypertension and congestive
heart failure.
II. Partial AVSD: Two separate
atrioventricular valves and primum atrial septal defect. Cleft of
the anterior leaflet of atrioventricular valve is common with
variable degree of regurgitation.
III. Intermediate AVSD: Two separate
atrioventricular valves with primum atrial septal defect and small
restrictive inlet ventricular septal defect.
IV. Unbalanced AVSD: One of the ventricles
is hypoplastic. This form is usually associated with complex
congenital heart defects such as heterotaxy syndrome (isomerism).
Varying degree of atrioventricular valve
regurgitation may be associated with AVSD.
Ideal Age of Surgery
I. Complete AVSD
(a) Uncontrolled heart failure:
Complete surgical repair as soon as possible (Class I).
(b) Controlled heart failure: Complete
surgical repair by 3 months of age (Class I).
(c) Pulmonary artery banding: May be
considered in select patients under 3 months of age (Class
IIb).
II. Partial or intermediate AVSD, stable and with
normal pulmonary artery pressures: Surgical repair at 2-3 years of
age (Class I).
III. Associated moderate or severe
atrioventricular valve regurgitation may necessitate early surgery
in partial or intermediate forms.
IV. Pulmonary artery banding is reserved for
complex cases and patients with contraindications for
cardio-pulmonary bypass (Class IIb).
V. Surgery for moderate to severe left
atrioventricular valve regurgitation is recommended as per the
guidelines for mitral regurgitation, discussed later (Class I).
Recommendations for Follow-up
I. Lifelong follow-up is required.
II. In patients with no significant residual
abnormality, annual follow-up is required till 10 years of age
followed by 2-3 yearly follow-up.
III. IE prophylaxis recommended for 6 months
after surgical closure. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
Patent Ductus Arteriosus (PDA)
Diagnostic Work-up
:
Clinical assessment, X-ray chest, ECG, Echocardiography. Cardiac
catheterisation is usually performed for device closure.
Ideal Age of Closure
I. Large/moderate PDA (significant left heart
volume overload, congestive heart failure, pulmonary arterial
hypertension): Early closure (by 3 months) (Class I).
II. Moderate PDA (Some degree of left heart
overload, mild to moderate pulmonary arterial hypertension, no/mild
congestive heart failure): 6 months-1 year (Class I). If
failure to thrive, closure can be accomplished earlier (Class
IIa).
III. Small PDA (Minimal or no left heart
overload. No pulmonary hypertension or congestive heart failure):
Between 12-18 months (Class I).
IV. Silent PDA (Diagnosed only on echo Doppler.
Hemodynamically insignificant, produce no murmur and there is no
pulmonary hypertension): Closure not recommended (Class III).
Contraindication for closure: PDA associated with
severe pulmonary arterial hypertension with irreversible pulmonary
vascular disease, and silent PDA (Class III).
Method of Closure: Surgical: Established mode
(Class I). Device closure: Preferred for children >6kg as less
invasive (Class I).
Recommendations for Follow-up
I. Clinical assessment, ECG and echo at one-year
post intervention. No further follow-up required if no residual
defect or pulmonary hypertension. Patient/guardians should be
explained about reporting to a hospital in case of any cardiac
symptoms.
II. IE prophylaxis recommended for 6 months after
device or surgical closure. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
PDA in a Preterm Baby (Gestational age <37 weeks)
I. Intervene if baby is in heart failure (small
PDAs may close spontaneously).
II. Approved drugs –
Indomethacin/Ibuprofen/Paracetamol (if no contraindication)
(Class I).
III. Mode of drug administration – Intravenous or
oral. At least 2 courses of drug therapy should be tried before
considering surgical intervention (Class I).
IV. Surgical ligation, if above drugs fail or are
contraindicated (Class I).
Prophylactic Indomethacin or Ibuprofen therapy: Not
recommended (Class III).
Aortopulmonary Window
Diagnostic Work-up:
Clinical assessment, X-ray chest, ECG, Echocardiography, Cardiac
catheterisation and CT Angiography (select cases).
Ideal Age of Closure
I. Uncontrolled heart failure: Surgical repair as
soon as possible (Class I).
II. Controlled heart failure: Elective surgical
repair by 3 months of age (Class I).
III. In patients with associated anomalies,
single stage repair of all defects is preferred (Class I).
Contraindication for closure: Severe
pulmonary arterial hypertension with irreversible pulmonary vascular
disease (Class III).
Method of Closure: Surgical patch
repair (Class I), transcatheter device closure in select cases
with a restrictive defect.
Recommendations for Follow-up
I. Clinical evaluation, ECG and echo annually
till 5 years. No further follow-up required if no residual defect or
pulmonary hypertension. Patient/guardians should be explained about
reporting to hospital in case of any cardiac symptoms.
II. IE prophylaxis recommended for 6 months after
surgical or device closure. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
Coarctation of Aorta (CoA)
Diagnostic work up:
Clinical assessment, X-ray chest, ECG, Echocardiography, CT
angiography/cardiac MRI (in select cases when anatomy is unclear, and
for follow-up in adults), cardiac catheterisation (if intervention is
planned).
Indications for intervention
I. Patients with CoA gradient
³20mmHg (Class
I).
II. Patients of CoA presenting with left
ventricular dysfunction, even though the gradient across is <20mmHg,
where left ventricular dysfunction is considered to be due to tight
CoA (Class I).
III. Patients with gradient <20mmHg but having
upper limb hypertension, left ventricular hypertrophy or significant
collateral formation (Class IIa).
IV. Patients with hypertension who have >50%
narrowing at the site of CoA, relative to aortic diameter at
diaphragm on CTA/cMRI/angiography, irrespective of pressure gradient
(Class IIa).
V. Intervention is not indicated if Doppler
gradient across coarctation segment is <20mmHg with normal left
ventricular function and no upper limb hypertension (Class III).
Ideal Age for Intervention
I. With left ventricular dysfunction/congestive
heart failure or severe upper limb hypertension (for age): Immediate
intervention (Class I).
II. Normal left ventricular function, no
congestive heart failure and mild upper limb hypertension:
Intervention beyond 3-6 months of age (Class I).
III. No hypertension, no heart failure, normal
ventricular function: Intervention at 1-2 years of age (Class I).
Mode of Intervention
I. Neonatal presentation: Surgery (Class I).
Aortic arch hypoplasia, if associated, should also be repaired.
II. Critically ill neonate who are considered
high risk for surgery (shock like syndrome, severe left ventricular
dysfunction): Balloon angioplasty to tide over the crisis (Class
IIa).
III. Infants with native coarctation: Surgery
(Class I) or Balloon angioplasty (Class IIa).
IV. Infants with re-coarctation: Balloon
angioplasty (Class I).
V. Children <25 kg with native coarctation:
Balloon angioplasty (Class I) or Surgery (Class IIa).
VI. Children <25 kg with re-coarctation: Balloon
angioplasty ± stenting (Class I).
VII. Children >25 kg and adults with native
coarctation: Catheter based stenting (Class IIa).
VIII. Children >25 kg and adults with
re-coarctation: Catheter based stenting (Class I).
IX. Elective endovascular stenting of aorta is
contra-indicated in children < 10 years of age (Class III).
Follow-up Recommendations
I. Lifelong follow-up is required. Annual
follow-up initially; later every 2-3 years if no residual lesions.
Follow-up should include clinical assessment (upper and lower limb
blood pressure) and echocardiography. Beyond 5 years of age, cMRI or
CT angiography may be required.
II. Beta-blockers are the preferred drugs for
control of hypertension.
III. IE prophylaxis is needed for 6 months after
surgery and intervention. However, all patients are advised to
maintain good oro-dental hygiene after this period also.
Aortic Stenosis (AS)
Diagnostic Work-up
:
Clinical assessment, X-ray chest, ECG, Echocardiography, CT
Angiography/cardiac MRI (in select cases), cardiac catheterisation
(primarily for therapeutic balloon valvuloplasty for valvar AS),
Exercise test (in select cases).
Indications and Timing of Treatment
Valvar Aortic Stenosis
I. Immediate intervention required for:
(a) Newborns with severe AS who are
duct dependent (balloon dilation or surgical valvotomy)
(Class I).
(b) Infants or children with left
ventricular dysfunction due to severe AS, regardless of the
valve gradient (Class I).
II. Elective balloon dilation for:
(a) Asymptomatic or symptomatic
patients with AS having gradient by echo-Doppler of >64mmHg peak
or >40mmHg mean or peak to peak gradient of
³50mmHg,
measured invasively at cardiac catheterization (Class I).
(b) Patients with symptoms due to AS
(angina, exercise intolerance) or ECG showing ST seg-ment
changes at rest or during exercise: balloon dilation should be
considered for lower gradients (invasively measured) of
³ 40mmHg
(Class I).
(c) Asymptomatic child or adolescent
with a peak systolic valve gradient (invasively measured) of
³
40mmHg but without ST–T-wave changes, if the patient wants to
participate in strenuous competitive sports (Class IIb).
III. Intervention not indicated in asymptomatic
children with normal ECG and AS gradient < 64 mmHg peak or < 40 mmHg
mean, by echo-Doppler (Class III).
Subvalvar AS due to discrete membrane
Surgical intervention indicated in
I. Patients with a peak instantaneous gradient of
³50 mmHg
(Class I).
II. Patients with a peak instantaneous gradient
of <50 mmHg associated with aortic regurgitation of more than mild
severity (Class I).
III. Patients with a peak instantaneous gradient
between 30 and 50 mmHg (Class IIb).
IV. Symptomatic patients with a peak
instantaneous gradient < 50 mmHg in the following situations:
(a) Presence of left ventricular
dysfunction attributable to obstruction (Class I).
(b) When pregnancy is being planned
(Class IIa).
(c) When the patient plans to engage
in strenuous/competitive sports (Class IIa).
V. Intervention not indicated for asymptomatic
patients with gradient of < 30 mmHg with no or trivial aortic
regurgitation (Class III).
Supravalvar AS
Surgical intervention indicated in:
I. Symptomatic patients with peak instantaneous
gradient
³ 64
mmHg and/or mean gradient ³
50mmHg on echo-Doppler (Class I).
II. Patients with mean Doppler gradient <50 mmHg, if
they have any of the following (Class I):
(a) symptoms attributable to
obstruction (exertional dyspnea, angina, syncope)
(b) left ventricular systolic
dysfunction attributable to obstruction.
(c) severe left ventricular
hypertrophy attributable to obstruction
(d) evidence of myocardial ischemia
due to coronary ostial involvement
III. Asymptomatic patients with mean Doppler
gradient
³50mmHg
may be considered for surgery when the surgical risk is low
(Class IIb).
All patients with AS must be advised to maintain good
oro-dental hygiene.
Recommendations for Follow-up
I. All patients with AS require life-long
follow-up irrespective of the type of intervention.
II. Clinical assessment, ECG and echo are
required; the interval depending on the severity of stenosis.
III. Patients who have significant AS and are
planned for an intervention should refrain from any sporting
activity. Those with asymptomatic moderate stenosis can participate
in low- or moderate-intensity sports. Patients with mild degree of
stenosis can participate in all sports.
IV. IE prophylaxis is recommended in patients
with a prosthetic valve.
Pulmonic Stenosis (PS)
Diagnostic Work-up
:
Clinical assessment, X-ray chest, ECG, Echocardiography, cardiac
catheterisation and angiography (primarily for therapeutic balloon
valvuloplasty), CT Angiography/cardiac MRI (for peripheral pulmonic
stenosis).
Indications and Timing of Treatment
Valvar pulmonic stenosis
I. Immediate intervention required for:
(a) Newborns with severe PS with duct
dependent pulmonary blood flow (Class I).
(b) Infants or children with right
ventricular dysfunction due to severe PS, regardless of the
valve gradient (Class I).
II. Elective balloon dilation for:
(a) Asymptomatic or symptomatic
patients with valvar PS having peak instantaneous gradient by
echo-Doppler of >64mmHg (Class I).
(b) Neonates and infants with any
degree of PS who have mild hypoxia due to mild hypoplasia of
right ventricle, even if right ventricular function is normal
(Class IIa).
(c) Patients with valvar pulmonic
stenosis due to dysplastic valve, who meet the above criteria
(Class IIa).
Mode of intervention: Balloon dilatation
(Class I); surgical intervention reserved for: subvalvar or
supra-valvar PS with indications same as in valvar stenosis,
Noonan syndrome (dysplastic valve) with hypoplastic annulus and
failed balloon dilatation (Class I).
Recommendations for Follow-up
I. All patients with PS require life-long
follow-up.
II. Clinical assessment, ECG and echo is required
at each visit; the interval depending on the severity of stenosis.
III. IE prophylaxis is recommended in patients
with a prosthetic valve. However, all patients with PS are advised
to maintain good oro-dental hygiene.
Tetralogy of Fallot (TOF)
Diagnostic Work-up:
Clinical assessment, pulse oximetry, ECG, X-ray chest, echocardiography,
lab investigations (Hemoglobin/Packed cell volume, Fluorescence in situ
hybridization for 22q11 deletion in some cases). CT Angiography, cardiac
catheterization is performed prior to surgery in select cases.
Medical management (Class I): Maintain Hb
>14 g/dL (by oral iron or blood transfusion). Beta blockers to be given
in highest tolerated doses (usual dose 1-4 mg/kg/day in 2 to 3 divided
doses). Prostaglandin infusion for neonates with significant
cyanosis.
Management of cyanotic spell: Oxygen
administration, knee-chest position, intravenous fluid bolus of normal
saline at the rate of 10-20 mL/kg, Morphine (0.1-0.2 mg/kg IV), IV
Metoprolol (0.1 mg/kg over 5 minutes, can be repeated every 5 minutes
provided no hypotension or bradycardia) or short acting Esmolol infusion
(50-200 mg/kg/min), sodium bicarbonate 1-2 mEq/kg given IV, blood
transfusion if required. For refractory spells, Phenylephrine infusion
(2-5 µg/kg/min), IV Ketamine (0.25-1.0 mg/kg bolus dose), general
anaesthesia may be needed. Severe refractory cyanotic spell is an
indication for emergency surgery/intervention.
Timing of Surgery
I. Stable, minimally cyanosed: Total repair at
6-12 months of age or earlier according to the institutional policy
(Class I).
II. Symptomatic children of <6 months of age with
significant cyanosis or history of spells despite therapy:
Palliation (by systemic to pulmonary artery shunt or stenting of the
ductus arteriosus/right ventricular outflow tract, or pulmonary
valve balloon valvuloplasty) or total repair depending on anatomy
and centre’s experience (Class I).
III. Patients having TOF with absent pulmonary
valve who are stable: Medical management till 1 year of age followed
by total correction with repair of pulmonary artery branch
dilation/aneurysm (Class I).
IV. Patients with anomalous left anterior
descending artery from right coronary artery crossing the right
ventricular outflow tract, who are likely to need right ventricle to
pulmonary artery conduit (Class I):
(a) <10 kg weight with significant
cyanosis: Aorto-pulmonary shunt
(b) >10 kg weight: Total repair using
conduit, or double barrel approach after two years of age, when
the child weighs >10 kg.
Recommendations for Follow-up
I. Asymptomatic patients with no residual lesion
but with free pulmonary regurgitation, not requiring inter-vention,
should be followed up 1-2 yearly, life long.
II. Clinical assessment, ECG and echocardiogram
is to be done at each visit. Holter monitoring is indicated in
patients suspected to have arrhythmia.
III. Cardiac catheterization should be performed
if any residual lesion is suspected. It may also be required for
percutaneous intervention such as stenting of pulmonary artery
branch for stenosis.
IV. Cardiac MRI is an important investigation for
follow-up of these patients. In asymptomatic patients, baseline
study should be performed 10 years after surgery with periodic
follow-up.
V. Infective endocarditis prophylaxis is
indicated in non-corrected patients, patients after surgical repair
for 6 months, and patients with percutaneous or surgical pulmonary
valve replacement. However, all patients with TOF are advised to
maintain good oro-dental hygiene even after 6 months of surgical
repair.
Ventricular Septal Defect with Pulmonary Atresia
(VSD-PA)
Anatomical Types
Type A- Short segment valvar atresia, pulmonary
arteries confluent and good sized, supplied by a PDA.
Type B- Long segment pulmonary atresia with absent
main pulmonary artery. Branch pulmonary arteries confluent and good
sized, supplied by a PDA.
Type C- Long segment pulmonary atresia with absent
main pulmonary artery. Branch pulmonary arteries confluent but pulmonary
blood flow dependent predominantly on MAPCAs.
Type D- Long segment pulmonary atresia with absent
main pulmonary artery. Non-confluent branch pulmonary arteries with
MAPCA dependent pulmonary blood flow.
Diagnostic Work-up: Clinical assessment, pulse
oximetry, ECG, X-ray chest, echocardiography. Additional imaging in the
form of cardiac catheterization, CT angiography/cardiac MRI or a
combination of these is essential for planning definitive repair. Lab
investigations (Hemoglobin/Packed cell volume, Fluorescence in situ
hybridization for 22q11 deletion) in some cases.
Medical management same as outlined in section on
Tetralogy of Fallot.
Indications and timing of intervention
Management depends on the type of VSD-PA, the
institutional experience and the clinical presentation. Generally, this
lesion requires a multistage management.
Type A (short segment VSD-PA with PDA):
I. Presentation with significant cyanosis at <1
year of age: Aorto-pulmonary shunt (Class I) or PDA stenting
(Class IIa) depending on the institutional preference and
feasibility.
II. After 1st intervention or those presenting at
³1 year of
age: Total correction at about 1 year of age, since a right
ventricle (RV) to pulmonary artery (PA) conduit is not required
(Class I).
Type B (Long segment pulmonary atresia with PDA):
I. Presentation with significant cyanosis at < 1
year of age: Aorto-pulmonary shunt (Class I) or PDA stenting
(Class IIa) depending on the institutional preference and
feasibility.
II. After 1st intervention or in those presenting
at
³1 year
of age (Class I):
(a) Optimal pulmonary blood flow with
good sized PAs – Total repair with RV to PA conduit at 3-4
years.
(b) Suboptimal pulmonary blood flow
with small PAs – Additional shunt followed by total repair with
RV to PA conduit at 3-4 years.
(c) Increased pulmonary blood flow
with large PAs –Total repair with RV to PA conduit by 1 year.
Type C (Long segment pulmonary atresia with confluent
branch pulmonary arteries supplied by MAPCAs) (Class I):
(a) Neonatal presentation –
Aorto-pulmonary shunt ± Unifocalisation of MAPCAs.
(b) After 1st intervention or late
presentation: Total repair with RV to PA conduit and VSD closure
at 3-4 years of age.
Type D (Long segment pulmonary atresia with
non-confluent branch pulmonary arteries supplied by MAPCAs) (Class
IIa): Aorto-pulmonary shunt + Unifocalisation of MAPCAs, followed by
total repair with RV to PA conduit and VSD closure at 3-4 years of age.
Recommendations for Follow-up
I. All patients with VSD-PA require life-long
follow-up. Clinical assessment, ECG and echocardiogram is required;
the interval depending on the nature of repair, residual or
additional lesions, symptoms and functional status.
II. Palliated patients need to be seen more
frequently if their oxygen saturation is low and to decide for the
next intervention.
III. Infective endocarditis prophylaxis is
indicated in non-corrected or palliated patients with cyanosis,
patients after surgical repair for 6 months, and patients with
conduits and pulmonary valve replacement. All patients are advised
to maintain good oro-dental hygiene even after 6 months of surgical
repair.
Indications for pulmonary valve replacement are same
as in Tetralogy of Fallot [6].
Transposition of Great Arteries (TGA)
Diagnostic Work-up:
Clinical assessment, pulse oximetry, ECG, X-ray chest,
echocardiography, cardiac catheterization (for balloon atrial septostomy
or assessment of adequacy of left ventricle for an ASO or to assess
pulmonary vascular resistance in late presenters), CT angiography and
cardiac MRI (rarely required).
Indications and Timing of Surgery
Surgery is indicated for all patients with TGA except
in those with irreversible pulmonary vascular disease.
Pre-surgical stabilization (Class I):
I. Start intravenous infusion of Prostaglandin E1
(PGE1), soon after delivery, if oxygen saturation is lower than 75%
and/or lactic acidosis is present. Monitor respiration as PGE1
infusion may result in apnea. Use lowest maintenance dose once PDA
is open.
II. Balloon atrial septostomy: This procedure is
most successful in patients younger than 6 weeks, but can be tried
in older infants also if the atrial septum is thin. Indications
include:
(a) Low saturations despite PGE1
infusion and ASD is restrictive (Class I).
(b) Those presenting with low
saturation and a restrictive ASD beyond 3-4 weeks with a closed
PDA where PGE1 is likely to be ineffective (Class IIa).
(c) Patient with restrictive ASD, not
fit for immediate surgery (e.g. having sepsis or respiratory
infection) (Class IIa).
(d) Restrictive ASD in TGA patients
with large VSD or PDA: to decrease left atrial pressure and
pulmonary venous hypertension (Class IIa).
Timing and type of Surgery
I. TGA with intact ventricular septum presenting
soon after birth: Arterial switch operation (ASO) is the best option
(Class I).
Timing of surgery: 7 days to 3 weeks, earlier if baby
is unstable or has associated persistent pulmonary hypertension of the
newborn. Exact timing based on institutional preference, but is best
done before 4 weeks.
II. TGA with intact ventricular septum presenting
beyond 3-4 weeks of life with regressed left ventricle:
(a) Presenting between 1 to 2 months:
ASO; extracorporeal membrane oxygenator (ECMO) support may be
required in some cases (Class IIa).
(b) Presenting between 2 to 6 months:
ASO with ECMO support or rapid two stage ASO* or an atrial
switch (if rapid two stage or ECMO not feasible) (Class IIa).
(c) Presenting between 6 months to 2
years: Atrial switch operation (Senning or Mustard operation)
(Class IIa). Rapid two stage ASO* to be considered in select
cases after detailed evaluation (Class IIb).
*The first stage of rapid two stage ASO involves
retraining of regressed left ventricle by performing pulmonary artery
banding along with the addition of a modified aorto-pulmonary shunt as
the first stage. The same can also be achieved in select patients by
stent placement in a patent ductus arteriosus (Class IIb). It must be
noted that ASO with ECMO support and rapid two stage ASO have higher
morbidity and mortality than primary ASO.
III. TGA with a large VSD and/or a large PDA: ASO
with VSD and/or PDA closure by 6 weeks of age (Class I).
These patients develop early pulmonary vascular disease and may
become inoperable by 6 months to one year of age.
IV. TGA with VSD and coarctation of aorta: ASO
with VSD closure and arch repair as soon as possible (Class I).
It is preferable to repair all lesions in a single stage.
V. TGA with VSD and significant left ventricular
outflow obstruction (Class I):
(a) Subvalvar pulmonary obstruction
with normal or near-normal pulmonary valve and pulmonary
annulus: ASO with resection of subvalvar stenosis.
(b) If obstruction involves pulmonary
valve or is subpulmonary but not amenable to resection:
(i) Neonates and infants
presenting with significant cyanosis: The options depend on
patient’s age and surgeon’s preference:
a. Systemic to pulmonary shunt
(at any age) followed by Rastelli type repair or root
translocation (at 2-3 years of age, or when the child
weighs >10kg).
b. Réparation à l’Etage
Ventriculaire (REV) procedure (usually done at 4-6
months)
c. Pulmonary root translocation
(usually done at 6-12 months)
d. Nikaidoh procedure (usually
done beyond 6-9 months of age)
(ii) In older, stable patients,
presenting beyond 2-3 years of age: One of the following
surgeries: Rastelli type repair, Nikaidoh procedure or root
translocation surgery.
(c) If the VSD is remote and not
amenable to one of the biventricular repairs: Multistage
palliative cavo-pulmonary connection (Class IIa).
Recommendations for Follow-up
I. All patients need lifelong follow-up.
Follow-up intervals depend on age, type of surgery and residual
findings.
II. In operated patients with no residual
defects: Follow-up visits should be at 1, 3 and 6 months after
surgery, yearly after that till onset of adult life and every 2-3
years thereafter.
III. Follow-up visits should include clinical
assessment, ECG and echocardiography.
IV. Infective endocarditis prophylaxis is
recommended in patients with cyanosis, and for 6 months after
definitive surgery, and in cases with conduits or other prosthetic
material during surgery. However, all patients are advised to
maintain good oro-dental hygiene even after 6 months of definitive
surgery.
Double Outlet Right Ventricle (DORV)
Diagnostic Work-up:
Clinical presentation, ECG, X-ray chest, pulse oximetry,
echocardiography, cardiac catheterization (in select cases), CT
angiography and cardiac MRI (when anatomy unclear).
Indication and Timing of Surgery
Surgery is indicated in all patients with DORV except
in those with irreversible pulmonary vascular disease.
Timing and type of surgery depends on DORV variant
(Class I)
I. DORV with subaortic VSD and pulmonary stenosis
(TOF type DORV):
(a) Presenting with significant
cyanosis at <3-4 months: Aorto-pulmonary shunt
(b) Presenting with significant
cyanosis at >3-4 months: Total repair with closure of VSD and
infundibular resection.
(c) Stable patients with no or minimal
cyanosis: Total repair with closure of VSD and infundibular
resection by 6-12 months.
II. DORV with large subaortic VSD and pulmonary
hypertension (VSD type DORV):
(a) VSD closure by 6 months of age.
(b) Presenting beyond 6 months of age:
assess for operability and close VSD if operable.
III. DORV with subpulmonary VSD and pulmonary
hypertension (TGA type DORV):
(a) Arterial switch operation (ASO)
with VSD closure by 6 weeks of age.
(b) If presenting beyond 3 months,
should be evaluated for operability. ASO with VSD closure if
operable.
(c) If associated with aortic arch
abnormality, arch repair should be done in same sitting.
IV. DORV with subpulmonary VSD and pulmonary
stenosis:
(a) If pulmonary obstruction is
localized e.g. subvalvar fibrous membrane or ridge: ASO
with resection of subvalvar stenosis.
(b) If pulmonary obstruction is
tubular or valvar: One of the following complex surgeries
required: Rastelli type repair, REV procedure, Nikaidoh
procedure or root translocation. A systemic to pulmonary artery
shunt may be required before these procedures in those
presenting early with significant cyanosis. Please refer to
section on "TGA with VSD and left ventricular outflow tract
obstruction" for more details.
V. DORV with remote VSD or associated with other
complex anatomy: One should strive to perform biventricular repair
by intraventricular baffling of left ventricular connection to
aorta. Univentricular palliation is done in cases where
biventricular repair is not possible.
Recommendations for Follow-up
I. All patients need lifelong follow-up,
frequency to be individualized depending on the type of surgery,
presence or absence of residual lesions and functional status.
II. Follow-up visits should include clinical
assessment, ECG and echocardiography.
III. Infective endocarditis prophylaxis
recommended in patients with cyanosis, and in cases with conduits or
other prosthetic material in the heart. Prophylaxis is also required
for 6 months after definitive surgery. However, all patients with
DORV are advised to maintain good oro-dental hygiene even after 6
months of definitive surgery.
Congenitally Corrected Transposition of Great
Arteries (ccTGA)
Diagnostic Work-up:
Clinical assessment, pulse oximetry, ECG, X-ray chest,
echocardiography, cardiac catheterization (in select cases), cardiac MRI
(in adults or after surgery), electrophysiological testing (selected
patients, who have arrhythmias/blocks).
Indications and Timing of Surgery [7,8]
General recommendations:
I. Tricuspid valve (systemic atrioventricular
valve) surgery for severe regurgitation should be considered before
systemic ventricular failure (ejection fraction <45%) sets in
(Class IIa).
II. Anatomic repair (double switch operation -
atrial switch plus arterial switch or Rastelli) may be considered
when left ventricle is functioning at systemic pressure and when
such surgery is feasible (Class IIa).
Indications and Timing for Specific Groups of ccTGA
I. No associated anomalies: Medical follow-up to
look for any development of tricuspid regurgitation or right
ventricular dysfunction (Class I). Neonatal double switch
operation may be considered (Class IIb).
II. Associated with large VSD
(a) <3 months: Pulmonary artery
banding followed later by double switch operation (atrial plus
arterial switch) (Class I).
(b) >6 months: Double switch (atrial
plus arterial switch), provided that patient has not developed
irreversible pulmonary vascular disease (Class I).
(c) 3-6 months: Pulmonary artery
banding followed by double switch operation or direct double
switch operation depending on institutional policy (Class
IIa).
III. Associated with Large VSD and left
ventricular outflow obstruction (pulmonary stenosis): Double switch
(atrial switch plus Rastelli) (Class I) or univentricular
repair pathway (Class IIa). If the saturation is good,
medical follow-up may be considered after discussion with the
family.
IV. Associated with complete heart block:
Permanent, dual chamber pacemaker implantation (Class I).
Recommendations for Follow-up
I. All patients with ccTGA require lifelong
follow-up, usually every year.
II. Infective endocarditis prophylaxis is
recommended for all patients with cyanosis and in cases with
conduits or other prosthetic material in the heart. It is also
advised for 6 months after a definitive surgery. However, all
patients with ccTGA are advised to maintain good oro-dental hygiene.
Univentricular Hearts (Single ventricles)
Diagnostic Work-up:
Clinical assessment, ECG, X-ray chest, pulse oximetry,
echocardiography, cardiac catheterisation, CT angiography, cardiac MRI.
Timing and Type of Intervention
Preamble: Surgery for univentricular heart is a
palliative procedure. The life expectancy is less than normal (exact age
cannot be predicted), and is interposed by interventions over these
years [9]. Treating physician must inform and discuss the details with
the parent/guardian prior to surgery.
The timing and type of intervention depends on age at
presentation and presence or absence of obstruction to pulmonary blood
flow.
I. Those presenting in neonatal period, or within
2-3 months of life (Class I):
(a) With increased pulmonary blood
flow:
(i) Type of surgery: Pulmonary
artery banding at 4-6 weeks of age, preferably before 3
months.
(ii) Additional procedures may be
required if systemic outflow obstruction is present.
(b) With decreased pulmonary blood
flow (pulmonary stenosis group): Systemic to pulmonary artery
shunt or stenting of ductus arteriosus if systemic arterial
saturation is consistently below 70%-75%.
(c) With balanced pulmonary
circulation: The baby usually maintains saturations above 80%
and is not in failure. Such infants should be followed up
closely. Surgery if saturation falls below 70%. (Class I).
II. Those presenting later in life or have
undergone first surgery earlier:
(a) With pulmonary hypertension and no
pulmonary stenosis: Most patients who present beyond 3-4 months
would become unsuitable for pulmonary artery banding or any
definitive repair in the future due to irreversible increase in
pulmonary vascular resistance.
(b) With normal pulmonary pressure and
resistance due to pulmonary stenosis/previous pulmonary artery
banding/previous aorto-pulmonary shunt:
(i) Bidirectional Glenn procedure
between 4-12 months of age (Class I).
(ii) Total cavo-pulmonary
connection or completion of Fontan procedure (preferably
extracardiac): Between 4-7 years of age when the child
weighs 15-20 kg. Fenestration of Fontan circuit is indicated
in high-risk cases.
Recommendations for Follow-up
I. All patients with univentricular heart
(operated or unoperated) require lifelong follow-up. Frequency
should be individualised, but should be at least once a year in
stable cases.
II. Drugs after surgery: Aspirin (3-5 mg/kg/day)
for all patients. Oral anticoagulants (warfarin) and sildenafil in
select group, or as per institution policy [9].
III. The threshold for performing cardiac
catheterisation during follow-up should be low as a number of
complications can be successfully treated if diagnosed in time.
IV. Infective endocarditis prophylaxis
recommended in patients with cyanosis and in cases with conduits or
other prosthetic material in the heart. However, all patients with
univentricular heart are advised to maintain good oro-dental
hygiene.
Persistent Truncus Arteriosus
Classification of truncus arteriosus (Van Praagh and
Van Praagh’s) [10]
I. Type A1 - Aorta and main pulmonary artery
originate from a single large common trunk.
II. Type A2 - Both pulmonary arteries arise
separately and directly from the truncus.
III. Type A3 - One pulmonary artery arises from
the truncus and the other is supplied by the patent ductus
arteriosus or collaterals from the aorta.
IV. Type A4 - There is associated obstructive
lesion of the aortic arch.
Diagnostic Work-up: Clinical assessment, pulse
oximetry, X-ray chest, ECG, echocardiography, CT
angiography/cardiac MRI (select cases), cardiac catheterisation (when
operability is in doubt).
Ideal Age for Surgery: Surgery indicated in all,
unless patient is inoperable.
I. Uncontrolled heart failure: Surgical repair as
soon as possible (Class I).
II. Controlled heart failure: Surgical repair by
3-6 weeks of age (Class I).
Type of surgery
Total repair using right ventricle to pulmonary
artery conduit. The prospects of repeat surgeries in future for conduit
obstruction should be discussed with parents. Truncal valve is repaired
if it is regurgitant.
Contraindication for Surgery
Severe pulmonary arterial hypertension with
irreversible pulmonary vascular disease (Class III).
Recommendations for Follow-up after Surgery
I. Lifelong follow-up is required in view of
above listed postoperative issues.
II. Follow-up after surgery with clinical
assessment, X-ray chest, ECG and echocardiography at 1, 6 and
12 months, and yearly thereafter in stable cases.
Infective endocarditis prophylaxis is recommended
after surgical repair due to presence of conduit. All patients are
advised to maintain good oro-dental hygiene.
Total Anomalous Pulmonary Venous Connection
(TAPVC)
Types of TAPVC
Type I: Anomalous connection at supracardiac level
(to innominate vein or right superior vena cava)
Type II: Anomalous connection at cardiac level (to
coronary sinus or right atrium)
Type III: Anomalous connection at infradiaphragmatic
level (to portal vein or inferior vena cava)
Type IV: Anomalous connection at two or more of the
above levels.
Diagnostic Work-up: Clinical assessment, pulse
oximetry, ECG, X-ray chest, echocardiography, cardiac
catheterization (Rarely performed when operability is in doubt), CT
angiography/cardiac MRI (select cases).
Indications and Timing of Surgery (all are
Class I recommendations)
I. Patients with obstructive TAPVC should undergo
emergency surgery.
II. Surgery should be performed as early as
possible in non-obstructive TAPVC, even if they are asymptomatic.
III. Those presenting late should be evaluated
for onset of pulmonary vascular disease and operated if the data
suggests operable status.
Recommendations for Follow-up
I. After surgery, patients should be followed up
at one month, 6 months and then annually for 5 years if there is no
residual defect.
II. Since arrhythmias can occur long after TAPVC
surgery, parents/patients should be informed to report if any
symptom suggestive of arrhythmia develops.
Infective endocarditis prophylaxis is indicated in
non-corrected patients and in patients after surgical repair for 6
months. However, all patients with TAPVC are advised to maintain good
oro-dental hygiene after this period also.
Ebstein’s Anomaly of the Tricuspid Valve
Diagnostic Work-up:
Clinical assessment, pulse oximetry, ECG, X-ray chest,
echocardiography, cardiac catheterization (in select cases), cardiac MRI
(important when planning surgical repair), electrophysiological studies
(select cases).
Indications and Timing for Treatment
Presentation in neonatal period: Significant
cyanosis: IV Prostaglandin infusion; Heart failure: Anti-failure therapy
including diuretics; Tachyarrhythmias: Antiarrhythmic drugs; Surgery for
neonates not stabilized with medical therapy (Class IIa).
Presentation in older children and adults:
Surgery is indicated (Class I) in those with symptoms or
deteriorating exercise capacity, cyanosis (oxygen saturation <90%),
paradoxical embolism, progressive cardiomegaly on chest X-ray
(cardiothoracic ratio >0.65), progressive dilation or dysfunction of the
right ventricle on echocardiography.
Types of Surgery: Depends on the underlying
anatomy and size of the functional ventricle. Options include tricuspid
valve repair (Cone repair, best done at about 2 years of
age)/replacement (if repair not possible), and one and a half ventricle
repair.
Recommendations for Follow-up
I. ECG, X-ray chest and echocardiography
should be done at each visit. Holter, exercise testing and cardiac
MRI may be required in select patients.
II. Asymptomatic patients who are not candidates
for surgery can be followed up every 2-3 years.
III. Infective endocarditis prophylaxis is
indicated in patients who have undergone tricuspid valve
replacement, have previous history of endocarditis or have cyanosis.
However, all patients with Ebstein’s anomaly are advised to maintain
good oro-dental hygiene.
Mitral and Aortic Regurgitation
Background:
Mitral (MR) and
aortic regurgitation (AR) occur most commonly secondary to acute or
chronic rheumatic heart disease, and they may co-exist in some.
Congenital MR is uncommon, however congenital AR due to a congenitally
bicuspid aortic valve is not rare.
Diagnostic Work-up: Clinical assessment, ECG,
X-ray chest, echocardiography, exercise test (in select cases), CT
angiography or cardiac MRI (in select cases).
Medical Therapy
I. Angiotensin converting enzyme inhibitors are
indicated in patients with severe MR and severe AR. Diuretics to be
used in those with dyspnea due to heart failure.
II. Sodium nitroprusside infusion is recommended
for treatment of acute MR; invasive BP monitoring is required for
these cases.
III. Anticoagulants (oral) if atrial fibrillation
is present.
IV. Secondary prophylaxis, preferably with long
acting Benzathine penicillin injection, is required for patients who
have underlying rheumatic heart disease as the etiology of MR or AR.
Indications and Timing of Surgery
Mitral regurgitation [11,12]
I. Symptomatic patients with moderate to severe
MR with left ventricular ejection fraction >30% (Class I).
II. Asymptomatic patients with severe MR: Surgery
indicated if any of the following present (Class IIa):
(a) Left ventricular ejection fraction
<60%.
(b) Left ventricular end systolic
dimension Z score >3 for mitral valve replacement; and >2.5 if
likelihood of mitral valve repair is >95%.
(c) Pulmonary artery systolic pressure
>50mmHg.
III. Asymptomatic patients with moderate or
severe MR undergoing cardiac surgery for another indication
(Class IIa).
Aortic regurgitation [11]
I. Symptomatic patients with moderate to severe
AR (Class I).
II. Asymptomatic patients with severe AR: Surgery
indicated if any of the following present (Class I):
(a) Left ventricular ejection fraction
<50%.
(b) Left ventricular end systolic
dimension Z score >4.
III. Asymptomatic patient with moderate or severe
AR undergoing cardiac surgery for another indication (Class I).
All patients with valvular regurgitation must be
advised to maintain good oro-dental hygiene.
Type of Valve Surgery [13]
I. Valve repairs are preferable to valve
replacements (Class I).
II. Valve replacement in those in whom valve
cannot be repaired (Class IIa):
(a) Ross procedure for young patients
with non rheumatic AR (if expertise available).
(b) Bioprosthetic valve for: female
patients planning pregnancy in future, or if compliance with
oral anticoagulation is dubious.
(c) Prosthetic metallic valve
replacements for the rest of patients.
Anticoagulation after Valve Surgery [14]
I. Oral anticoagulant drug: Warfarin or other
anticoumarin drug
(a) Desired INR (International
Normalized Ratio):
(i) After mitral valve
replacement: 3.0 (±0.5)
(ii) After aortic valve
replacement: 2.5 (±0.5)
(iii) After valve repair,
bioprosthetic valve: 2.5 (±0.5)
(b) Patients should be educated about
the importance of maintaining INR in therapeutic range, the
effect of diet, medicines, etc. on INR and the warning signs of
overdose of warfarin. These patients should be advised to avoid
contact sports; otherwise normal activities are allowed. Regular
intramuscular immunization can be given while on oral
anticoagulant drugs. Dental surgery is safe with therapeutic
levels of INR.
(c) Duration of anticoagulation:
(i) Valve repair, bioprosthetic
valve: For 3 months after surgery
(ii) Prosthetic metallic valve:
Lifelong
(d) Oral anticoagulants are also
indicated for patients with atrial fibrillation.
II. Aspirin: Dose - 3 to 5 mg/kg/day given in
addition to anticoagulation (Class I).
(a) Duration: Valve repair,
bioprosthetic valve: For 6 months after surgery
(a) Prosthetic metallic valve:
Lifelong
Recommendations for Follow-up
I. Patients with valve lesions require lifelong
follow-up.
II. Asymptomatic patients with MR or AR: Clinical
assessment, ECG and echocardiography at periodic intervals.
III. Operated patients with no residual
abnormality: Clinical assessment, ECG and echocardiography. Patients
with prosthetic metallic valve require frequent monitoring of INR
and fluoroscopy (for valve motion).
Infective endocarditis prophylaxis [14]: All
patients must be advised to maintain good oro-dental hygiene after valve
surgery. Prophylaxis is reasonable before dental procedures that involve
manipulation of gingival tissue or periapical region of teeth, or
perforation of the oral mucosa, in patients with prosthetic valve and
also in those where prosthetic material is used for valve repair (e.g.
annuloplasty rings).
These guidelines originated from a National Consensus
Meeting on "Management of Congenital Heart Diseases in India" held on
10th and 11th of August, 2018 at the All India Institute of Medical
Sciences, New Delhi, India.
Contributors: All authors were part of the
National Consensus Meeting that formulated these Guidelines. All authors
reviewed the literature and drafted recommendations of respective
sections assigned to them. The final document was drafted and compiled
by AS and JR. All authors provided critical inputs at every stage to
finalize the draft recommendations. All authors approved the final
document.
Funding: None; Competing interest: None
stated.
ANNEXURE List of Participants with Affiliations
Anita Saxena (Convener), All India Institute of
Medical Sciences, New Delhi; Jay Relan (Writing Committee), All
India Institute of Medical Sciences, New Delhi; Ravi Agarwal,
Madras Medical Mission, Chennai; Neeraj Awasthy, Max Super
Speciality Hospital, New Delhi; Sushil Azad, Fortis Escorts Heart
Institute, New Delhi; Manisha Chakrabarty, Apollo Hospitals, New
Delhi; Kulbhushan S. Dagar, Max Super Speciality Hospital, New
Delhi; Velayoudam Devagourou, All India Institute of Medical
Sciences, New Delhi; Saurabh K. Gupta, All India Institute of
Medical Sciences, New Delhi; Krishna S. Iyer, Fortis Escorts
Heart Institute, New Delhi; M. Jayranganath, Jayadeva Institute
of Cardiovascular Sciences and Research, Bangalore; Raja Joshi,
Sir Ganga Ram Hospital, New Delhi; B.R.J. Kannan, Vadamalayan
Hospitals, Madurai; Ashish Katewa, Sri Sathya Sai Sanjeevani
Hospital, Raipur; Vikas Kohli, Apollo Hospitals, New Delhi;
Nageswara Rao Koneti, Care Hospital, Hyderabad; Shyam S. Kothari,
All India Institute of Medical Sciences, New Delhi; K.M.
Krishnamoorthy, Sree Chitra Tirunal Institute for Medical Sciences
and Technology, Trivandrum; Snehal Kulkarni, Kokilaben Dhirubhai
Ambani Hospital, Mumbai; Rohit Manoj Kumar, Postgraduate
Institute of Medical Education and Research, Chandigarh; Raman
Krishna Kumar, Amrita Institute of Medical Sciences, Kochi;
Sunita Maheshwari, Narayana Institute of Cardiac Sciences,
Bangalore; Krishna Manohar, Sri Sathya Sai Sanjeevani
International Centre for Child Heart Care and Research, Palwal;
Ashutosh Marwah, Jaypee Hospital, Noida; Smita Mishra, Jaypee
Hospital, Noida; Smruti R. Mohanty, Kokilaben Dhirubhai Ambani
Hospital, Mumbai; Kona Samba Murthy, Innova Children’s Heart
Hospital, Hyderabad; Suresh P.V., Narayana Hrudayalaya,
Bangalore; S. Radhakrishnan, Fortis Escorts Heart Institute, New
Delhi; Palleti Rajashekar, All India Institute of Medical
Sciences, New Delhi; Sivasubramanian Ramakrishnan, All India
Institute of Medical Sciences, New Delhi; Nitin Rao, Star
Hospital, Hyderabad; Suresh G. Rao, Kokilaben Dhirubhai Ambani
Hospital, Mumbai; Chinnaswamy Reddy H.M., Narayana Institute of
Cardiac Sciences, Bangalore; Rajesh Sharma, Jaypee Hospital,
Noida; Krishnanaik Shivaprakasha, H.N. Reliance Hospital, Mumbai;
Raghavan Subramanyan, Frontier Lifeline Hospital, Chennai; R.
Suresh Kumar, Believers International Heart Centre, Thiruvalla;
Sachin Talwar, All India Institute of Medical Sciences, New Delhi;
Munesh Tomar, Nutema Hospital, Meerut; Sudeep Verma,
Krishna Institute of Medical Sciences, Secunderabad; Vijayakumar Raju,
GKNM Hospital, Coimbatore.
References
1. EUROCAT Steering Committee. Congenital Heart
Defect in Europe; c2014. Available from: http://www.eurocat
network.eu/content/EUROCAT Special Report CHD.pdf. Accessed on Mar
05, 2019.
2. Hoffman JI. The global burden of congenital heart
disease. Cardiovasc J Afr. 2013;24:141-5.
3. Bernier PL, Stefanescu A, Samoukovic G,
Tchervenkov CI. The challenge of congenital heart disease worldwide:
Epidemiologic and demographic facts. Semin Thorac Cardiovasc Surg
Pediatr Card Surg Annu. 2010;13:26-34.
4. Warnes CA. The adult with congenital heart
disease: Born to be bad. J Am Coll Cardiol. 2005;46:1-8.
5. Working Group on Management of Congenital Heart
Diseases in India. Consensus on timing of intervention for common
congenital heart disease. Indian Pediatr. 2008;45:117-26.
6. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B,
Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the
Management of Adults With Congenital Heart Disease: Executive Summary: A
Report of the American College of Cardiology/American Heart Association
Task Force on Clinical Practice Guidelines. J Am Coll Cardiol.
2019;139:e637 97.
7. Tweddell JS. What do we really know about the
management of patients with congenitally corrected transposition of the
great arteries? J Thorac Cardiovasc Surg. 2017;154:1023-5.
8. Warnes CA, Williams RG, Bashore TM, Child JS,
Connolly HM, Dearani JA, et al. ACC/AHA 2008 Guidelines for the
Management of Adults with Congenital Heart Disease: A report of the
American College of Cardiology/American Heart Association Task Force on
Practice Guidelines (writing committee to develop guidelines on the
management of adults with congenital heart disease). Circulation.
2008;52(23):e143-263.
9. Alsaied T, Alsidawi S, Allen CC, Faircloth J,
Palumbo JS, Veldtman GR. Strategies for thromboprophylaxis in Fontan
circulation: A meta-analysis. Heart. 2015;101:1731-7.
10. Van Praagh R, Van Praagh S. The anatomy of common
aorticopulmonary trunk (truncus arteriosus communis) and its embryologic
implications: A study of 57 necropsy cases. Am J Cardiol.
1965;16:406-25.
11. Carapetis JR, Beaton A, Cunningham MW, Guilherme
L, Karthikeyan G, Mayosi BM, et al. Acute rheumatic fever and
rheumatic heart disease. Nat Rev Dis Primers. 2016;2:15084.
12. Johnson JT, Eckhauser AW, Pinto NM, Weng HY,
Minich LL, Tani LY. Indications for intervention in asymptomatic
children with chronic mitral regurgitation. Pediatr Cardiol.
2015;36:417-22.
13. Finucane K, Wilson N. Priorities in cardiac
surgery for rheumatic heart disease. Glob Heart. 2013;8:213-20.
14.` Nishimura RA, Otto CM, Bonow RO, Carabello BA,
Erwin JP, Fleisher LA, et al. 2017 AHA/ACC focused update of the
2014 AHA/ACC guideline for the management of patients with valvular
heart disease: A report of the American College of Cardiology/American
Heart Association Task Force on Clinical Practice Guidelines. J Am Coll
Cardiol. 2017;70:252-89.
