RECOMMENDATIONS

Indian Guidelines for Indications and Timing of Intervention for Common

Congenital Heart Diseases: Revised and Updated Consensus Statement of
the Working Group on Management of Congenital Heart Diseases.
Abridged Secondary Publication
WORKING GROUP ON MANAGEMENT OF CONGENITAL HEART DISEASE IN INDIA; ANITA SAXENA1, JAY RELAN1, RAVI
AGARWAL2, NEERAJ AWASTHY3, SUSHIL AZAD4, MANISHA CHAKRABARTY5, KULBHUSHAN S DAGAR3, VELAYOUDAM
DEVAGOUROU1, BAIJU S DHARAN6, SAURABH K GUPTA1, KRISHNA S IYER4, M JAYRANGANATH7, RAJA JOSHI8, BRJ
KANNAN9, ASHISH KATEWA10, VIKAS KOHLI5, NAGESWARA RAO KONETI11, SHYAM S KOTHARI1, KM
KRISHNAMOORTHY6, SNEHAL KULKARNI12, ROHIT MANOJ KUMAR13, RAMAN KRISHNA KUMAR14, SUNITA
MAHESHWARI15, KRISHNA MANOHAR16, ASHUTOSH MARWAH17, SMITA MISHRA17, SMRUTI R MOHANTY12, KONA
SAMBA MURTHY, PV SURESH19, S RADHAKRISHNAN4, PALLETI RAJASHEKAR1, SIVASUBRAMANIAN RAMAKRISHNAN1,
NITIN RAO20, SURESH G RAO12, CHINNASWAMY HM REDDY15, RAJESH SHARMA17, KRISHNANAIK SHIVAPRAKASHA21,
RAGHAVAN SUBRAMANYAN22, R SURESH KUMAR23, SACHIN TALWAR1, MUNESH TOMAR24, SUDEEP VERMA25 AND
VIJAYAKUMAR RAJU26
From 1All India Institute of Medical Sciences, New Delhi; 2Madras Medical Mission; Chennai; 3Max Super Speciality Hospital,
New Delhi; 4Fortis Escorts Heart Institute, New Delhi; 5Apollo Hospitals, New Delhi; 6Sree Chitra Tirunal Institute for Medical
Sciences and Technology, Trivandrum, Kerala; 7Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore,
Karnataka; 8Sir Ganga Ram Hospital, New Delhi; 9Vadamalayan Hospitals, Madurai, Tamil Nadu; 10Sri Sathya Sai Sanjeevani
Hospital, Raipur, Chhattisgarh; 11Care Hospital, Hyderabad, Telangana; 12Kokilaben Dhirubhai Ambani Hospital, Mumbai,
Maharashtra; 13Postgraduate Institute of Medical Education and Research, Chandigarh; 14Amrita Institute of Medical Sciences,
Kochi, Kerala; 15Narayana Institute of Cardiac Sciences, Bangalore, Karnataka; 16Sri Sathya Sai Sanjeevani International Centre
for Child Heart Care and Research, Palwal, Haryana; 17Jaypee Hospital, Noida, Uttar Pradesh; 18Innova Children’s Heart
Hospital, Hyderabad, Telangana; 19Narayana Hrudayalaya, Bangalore, Karnataka; 20Star Hospital, Hyderabad, Telangana,
India; 21HN Reliance Hospital, Mumbai, Maharashtra; 22Frontier Lifeline Hospital, Chennai; 23Believers International Heart
Centre, Thiruvalla, Kerala; 24Nutema Hospital, Meerut, Uttar Pradesh; 25Krishna Institute of Medical Sciences, Secunderabad,
Telangana; 26GKNM Hospital, Coimbatore, Tamil Nadu; India.
Correspondence to: Dr Anita Saxena, DM (Cardiology), Professor, Department of Cardiology, All India Institute of Medical
Sciences, New Delhi 110 029, India. [email protected]
(This is abridged secondary publication of a primary publication in Annals of Pediatric Cardiology: Saxena A, Relan J, Agarwal R, Awasthy
N, Azad S, Chakrabarty M, et al. Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised
and updated consensus statement of the Working group on management of congenital heart diseases. Ann Pediatr Cardiol. 2019;12:254-
86)

Justification: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However,
these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when
recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may
have co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the 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. The meeting was supported by Children’s HeartLink, a non-governmental organization based in Minnesota, USA.
Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart
diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.
Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenital
heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus
arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart
diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein
anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

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C
ongenital heart diseases (CHDs) are the most patients including those needing regular follow-up, we
common birth defects, responsible for nearly added guidelines and protocols for follow-up of these
one-third of all congenital birth defects [1]. patients.
The birth prevalence of CHD is reported to be
PREAMBLE
8-12/1000 live births [2,3]. One-fifth of these babies have
critical heart disease requiring very early intervention. 1. Every pediatrician/cardiologist/other healthcare
Advances in pediatric cardiology and cardiac surgery provider must strive to get a complete diagnosis on a
have made it possible to repair or palliate most of the child suspected of having heart disease, with the help
CHDs including the complex ones. If access to screening, of a higher centre, if needed.
early diagnosis and treatment is available, over 90% of 2. The proposed guidelines are meant to assist the health
patients born with CHD survive to adult life with good care provider (pediatrician, cardiologist, pediatric car-
long-term outcome [4]. Most middle- and low-income diologist) in managing cases of congenital heart dis-
countries lack such advanced level of care for children eases in their practice. While these may be applicable to
with CHD. Considering a birth prevalence of 9/1000, the the majority, each case needs individualized care, and
estimated number of children born with CHD every year exceptions may have to be made. Guidelines are in-
in India approximates 2,40,000, posing a tremendous tended to define practices, meeting the needs of patients
challenge for the families, society and healthcare system. in most, if not all circumstances, and should not replace
Approximately 10% of infant mortality in India may be clinical judgment.
accounted for, by CHDs.
3. These guidelines are in reference to current health care
JUSTIFICATION FOR DEVELOPING INDIAN scenario prevalent in India. Subsequent modifications
GUIDELINES may be necessary in future as the pediatric cardiology
practice evolves.
Evidence based recommendations for management of
4. The recommendations are classified into three
CHD have been published by task force members from a
categories according to their strength of agreement:
number of national and international associations, but
these are primarily meant for children born in high income Class I: Is recommended/is indicated. General agreement
countries. Applicability of these guidelines to Indian that the given treatment or procedure is beneficial, useful
population with CHD is likely to be limited. Majority of and effective.
patients with CHD are not diagnosed in antenatal period
Class II: Conflicting evidence and/or a divergence of
and often present late in the course of the disease. These
opinion or both about the usefulness/efficacy of the given
patients are often underweight, malnourished and have
treatment or procedure. IIa: Should be considered.
comorbidities such as recurrent infections and anemia.
Weight of evidence/opinion is in favour of usefulness/
Many of the late presenters have advanced level of
efficacy. IIb: May be considered. Usefulness/efficacy is
pulmonary hypertension, ventricular dysfunction,
less well established.
hypoxia, polycythemia, etc. The outcome after surgery in
such patients are expected to be suboptimal with longer Class III: Is not recommended. Evidence or general
periods of mechanical ventilation and stay in intensive agreement that the given treatment or procedure is not
care. Modifications in the treatment protocol may be useful/effective; and in some cases may be harmful.
required for optimizing the outcomes. All these factors AIMS AND OBJECTIVES
justify the need for separate guidelines for management of
CHDs in India, including the timing of intervention. 1. To outline the optimal timing of intervention in
common CHDs.
A statement on “consensus on timing of intervention 2. To formulate guidelines and protocols for follow-up of
for common congenital heart disease” which originated patients who have undergone surgery/catheter
from a Meeting of Working Group on Management of interventions for CHD.
Congenital Heart Disease in India, was published in the
year 2008 [5]. This statement was revised and updated in GUIDELINES FOR INDIVIDUAL CONGENITAL
a subsequent National Consensus Meeting, which was HEART DEFECTS
held in New Delhi after a gap of 10 years, in August 2018.
Atrial Septal Defect (ASD)
In the intervening 10 years, a number of pediatric cardiac
centres have been established and overall the numbers of Diagnostic work-up: Physical examination, ECG, X-ray
interventions have increased by several folds. chest, echocardiography and cardiac catheterisation (in
Considering the growing population of post-operative select cases).

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Types of Atrial septal defect: Ostium secundum Isolated Ventricular Septal Defect (VSD)
(~75%); Ostium primum (15%-20%); Sinus venosus
Diagnostic Work-up: Physical examination, ECG, X-ray
(5%-10%); and Coronary sinus (<1%).
chest, echocardiography and cardiac catheterisation (in
Patent foramen ovale: Small defect in fossa ovalis select cases).
region with a flap with no evidence of right heart volume
overload. Diagnosed on echocardiography, is a normal Classification of Ventricular Septal Defect
finding in newborns. Perimembranous: 80%; Outlet or sub-pulmonary
Indication for closure: ASD with left-to-right shunt (doubly committed): 5%-7%; Inlet: 5%-8%; and
associated with evidence of right ventricular volume muscular: 5%-20%, these could be central (mid
overload without evidence of irreversible pulmonary muscular), apical, marginal (anterior, septal-free wall
vascular disease (Class I). Indications for ASD closure area) or multiple, “swiss cheese” type.
remain the same irrespective of the method of closure. Indications and Timing of Closure (All Class I
Contraindications for closure: Severe pulmonary recommendations)
arterial hypertension or irreversible pulmonary vascular Small VSD: (No symptoms, normal PA pressure, normal
disease (Class III). left heart chambers, no cusp prolapse): (a) Annual
Ideal Age of Closure follow-up till 10 years of age, then every 2-3 years; (b)
Closure indicated if patient has an episode of endocarditis
Asymptomatic child: 2-4 years (Class I). For sinus or develops cusp prolapse with aortic regurgitation or
venosus defect surgery may be delayed to 4-5 years develops progressive significant right ventricular outflow
(Class IIa). tract obstruction.
Symptomatic ASD: Rarely seen in infants. Present with
Moderate VSD: (a) Asymptomatic (normal pulmonary
congestive heart failure, pulmonary arterial hypertension.
artery pressure with left heart dilation): Closure of VSD
Early closure is recommended (Class I) after ruling out
by 2-5 years of age; (b) Symptomatic: If controlled with
associated lesions such as left ventricular inflow
medications, VSD closure by 1-2 years of age;
obstruction, aortopulmonary window, total anomalous
pulmonary venous drainage, etc. Large VSD: (a) Poor growth/congestive heart failure not
If presenting beyond ideal age: Elective closure controlled with medications (furosemide/spironolactone
irrespective of age as long as there is left-to-right shunt or enalapril +/- digoxin): As soon as possible; (b)
with right heart volume overload and pulmonary vascular Controlled heart failure: By 6 months of age.
resistance is within operable range (Class I). VSD with aortic cusp prolapse: Any VSD with cusp
Method of Closure prolapse and directly related aortic regurgitation that is
more than trivial: Surgery whenever aortic regurgitation
Surgical: Established mode (Class I). is detected.
Device: For secundum ASDs with adequate rims and Contraindications for Closure: Severe pulmonary
weight of child >15kg (Class I). arterial hypertension with irreversible pulmonary
Recommendations for Follow-up vascular disease (Class III).

Follow-up after surgical closure: Clinical and echo in the Method of Closure
first year only. No further follow-up required if no Surgery: Conventionally patch closure is done.
residual disease, no pulmonary hypertension or Pulmonary artery banding to be considered for patients
arrhythmia. Patient/guardians should be explained about with multiple VSDs, inaccessible VSDs and those with
reporting to hospital in case of any cardiac symptoms, or contraindications for cardio-pulmonary bypass.
symptoms suggestive of arrhythmias.
Device closure: For VSDs with adequate rims around
Follow-up after device closure: (a) Anti-platelet agents for defect and weight of child >8kg.
total duration of 6 months (b) Echocardiography: - At
discharge, 1 month, 6 months, 1 year, then every 3-5 years. Recommendations for Follow-up
IE prophylaxis: It is recommended for 6 months after Follow-up after surgery: Clinical, ECG and echo in the
device or surgical closure. However, all patients are first year only. No further follow-up required if no
advised to maintain good oro-dental hygiene after this residual defect or pulmonary hypertension. Patient/
period also. guardians should be explained about reporting to hospital

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in case of any cardiac symptoms, or symptoms suggestive regurgitation may necessitate early surgery in partial or
of arrhythmias. intermediate forms.
Follow-up protocol for device closure: Anti-platelet IV. Pulmonary artery banding is reserved for complex
agents for total duration of 6 months. cases and patients with contraindications for cardio-
pulmonary bypass (Class IIb).
IE prophylaxis: It is recommended for 6 months after
V. Surgery for moderate to severe left atrioventricular
device or surgical closure. However, all patients are
valve regurgitation is recommended as per the
advised to maintain good oro-dental hygiene after this
guidelines for mitral regurgitation, discussed later
period also.
(Class I).
Atrioventricular Septal Defect (AVSD)
Recommendations for Follow-up
Diagnostic Work-up: Physical examination, ECG, X-ray
I. Lifelong follow-up is required.
chest, echocardiography and cardiac catheterisation (in
select cases). II. In patients with no significant residual abnormality,
annual follow-up is required till 10 years of age
Types of AVSD followed by 2-3 yearly follow-up.
I. Complete AVSD: Large septal defect with an atrial III. IE prophylaxis recommended for 6 months after
component (ostium primum defect) and ventricular surgical closure. However, all patients are advised to
component (inlet septal defect), common maintain good oro-dental hygiene after this period
atrioventricular valve ring and common also.
atrioventricular valve. Generally associated with large
Patent Ductus Arteriosus (PDA)
left-to-right shunt, pulmonary arterial hypertension
and congestive heart failure. Diagnostic Work-up: Clinical assessment, X-ray chest,
II. Partial AVSD: Two separate atrioventricular valves ECG, Echocardiography. Cardiac catheterisation is
and primum atrial septal defect. Cleft of the anterior usually performed for device closure.
leaflet of atrioventricular valve is common with Ideal Age of Closure
variable degree of regurgitation.
I. Large/moderate PDA (significant left heart volume
III. Intermediate AVSD: Two separate atrioventricular overload, congestive heart failure, pulmonary arterial
valves with primum atrial septal defect and small hypertension): Early closure (by 3 months) (Class I).
restrictive inlet ventricular septal defect.
II. Moderate PDA (Some degree of left heart overload,
IV. Unbalanced AVSD: One of the ventricles is mild to moderate pulmonary arterial hypertension, no/
hypoplastic. This form is usually associated with mild congestive heart failure): 6 months-1 year (Class
complex congenital heart defects such as heterotaxy I). If failure to thrive, closure can be accomplished
syndrome (isomerism). earlier (Class IIa).
Varying degree of atrioventricular valve regurgitation III. Small PDA (Minimal or no left heart overload. No
may be associated with AVSD. pulmonary hypertension or congestive heart failure):
Ideal Age of Surgery Between 12-18 months (Class I).
IV. Silent PDA (Diagnosed only on echo Doppler.
I. Complete AVSD
Hemodynamically insignificant, produce no murmur
(a) Uncontrolled heart failure: Complete surgical and there is no pulmonary hypertension): Closure not
repair as soon as possible (Class I). recommended (Class III).
(b) Controlled heart failure: Complete surgical Contraindication for closure: PDA associated with
repair by 3 months of age (Class I). severe pulmonary arterial hypertension with irreversible
pulmonary vascular disease, and silent PDA (Class III).
(c) Pulmonary artery banding: May be considered in
select patients under 3 months of age (Class IIb). Method of Closure: Surgical: Established mode (Class
I). Device closure: Preferred for children >6kg as less
II. Partial or intermediate AVSD, stable and with normal
invasive (Class I).
pulmonary artery pressures: Surgical repair at 2-3
years of age (Class I). Recommendations for Follow-up
III. Associated moderate or severe atrioventricular valve I. Clinical assessment, ECG and echo at one-year post

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intervention. No further follow-up required if no II. IE prophylaxis recommended for 6 months after
residual defect or pulmonary hypertension. Patient/ surgical or device closure. However, all patients are
guardians should be explained about reporting to a advised to maintain good oro-dental hygiene after this
hospital in case of any cardiac symptoms. period also.
II. IE prophylaxis recommended for 6 months after Coarctation of Aorta (CoA)
device or surgical closure. However, all patients are
Diagnostic work up: Clinical assessment, X-ray chest,
advised to maintain good oro-dental hygiene after this
ECG, Echocardiography, CT angiography/cardiac MRI
period also.
(in select cases when anatomy is unclear, and for follow-
PDA in a Preterm Baby (Gestational age <37 up in adults), cardiac catheterisation (if intervention is
weeks) planned).
I. Intervene if baby is in heart failure (small PDAs may Indications for intervention
close spontaneously). I. Patients with CoA gradient ≥20mmHg (Class I).
II. Approved drugs – Indomethacin/Ibuprofen/ II. Patients of CoA presenting with left ventricular
Paracetamol (if no contraindication) (Class I). dysfunction, even though the gradient across is
III. Mode of drug administration – Intravenous or oral. At <20mmHg, where left ventricular dysfunction is
least 2 courses of drug therapy should be tried before considered to be due to tight CoA (Class I).
considering surgical intervention (Class I). III. Patients with gradient <20mmHg but having upper
IV. Surgical ligation, if above drugs fail or are limb hypertension, left ventricular hypertrophy or
contraindicated (Class I). significant collateral formation (Class IIa).

Prophylactic Indomethacin or Ibuprofen therapy: Not IV. Patients with hypertension who have >50% narrowing
recommended (Class III). at the site of CoA, relative to aortic diameter at
diaphragm on CTA/cMRI/angiography, irrespective of
Aortopulmonary Window pressure gradient (Class IIa).
Diagnostic Work-up: Clinical assessment, X-ray chest, V. Intervention is not indicated if Doppler gradient across
ECG, Echocardiography, Cardiac catheterisation and CT coarctation segment is <20mmHg with normal left
Angiography (select cases). ventricular function and no upper limb hypertension
Ideal Age of Closure (Class III).

I. Uncontrolled heart failure: Surgical repair as soon as Ideal Age for Intervention
possible (Class I). I. With left ventricular dysfunction/congestive heart
II. Controlled heart failure: Elective surgical repair by 3 failure or severe upper limb hypertension (for age):
months of age (Class I). Immediate intervention (Class I).
II. Normal left ventricular function, no congestive heart
III. In patients with associated anomalies, single stage
failure and mild upper limb hypertension: Intervention
repair of all defects is preferred (Class I).
beyond 3-6 months of age (Class I).
Contraindication for closure: Severe pulmonary arterial
III. No hypertension, no heart failure, normal ventricular
hypertension with irreversible pulmonary vascular
function: Intervention at 1-2 years of age (Class I).
disease (Class III).
Mode of Intervention
Method of Closure: Surgical patch repair (Class I),
transcatheter device closure in select cases with a I. Neonatal presentation: Surgery (Class I). Aortic arch
restrictive defect. hypoplasia, if associated, should also be repaired.
Recommendations for Follow-up II. Critically ill neonate who are considered high risk for
surgery (shock like syndrome, severe left ventricular
I. Clinical evaluation, ECG and echo annually till 5
dysfunction): Balloon angioplasty to tide over the
years. No further follow-up required if no residual
crisis (Class IIa).
defect or pulmonary hypertension. Patient/guardians
should be explained about reporting to hospital in case III. Infants with native coarctation: Surgery (Class I) or
of any cardiac symptoms. Balloon angioplasty (Class IIa).

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IV. Infants with re-coarctation: Balloon angioplasty (b) Patients with symptoms due to AS (angina,
(Class I). exercise intolerance) or ECG showing ST seg-
ment changes at rest or during exercise: balloon
V. Children <25 kg with native coarctation: Balloon
dilation should be considered for lower gradients
angioplasty (Class I) or Surgery (Class IIa).
(invasively measured) of ≥ 40mmHg (Class I).
VI. Children <25 kg with re-coarctation: Balloon
(c) Asymptomatic child or adolescent with a peak
angioplasty ± stenting (Class I).
systolic valve gradient (invasively measured) of
VII. Children >25 kg and adults with native coarctation: ≥ 40mmHg but without ST–T-wave changes, if
Catheter based stenting (Class IIa). the patient wants to participate in strenuous
VIII. Children >25 kg and adults with re-coarctation: competitive sports (Class IIb).
Catheter based stenting (Class I). III. Intervention not indicated in asymptomatic children
IX. Elective endovascular stenting of aorta is contra- with normal ECG and AS gradient < 64 mmHg peak or
indicated in children < 10 years of age (Class III). < 40 mmHg mean, by echo-Doppler (Class III).

Follow-up Recommendations Subvalvar AS due to discrete membrane

Surgical intervention indicated in
I. Lifelong follow-up is required. Annual follow-up
initially; later every 2-3 years if no residual lesions. I. Patients with a peak instantaneous gradient of ≥50
Follow-up should include clinical assessment (upper mmHg (Class I).
and lower limb blood pressure) and echocardiography. II. Patients with a peak instantaneous gradient of
Beyond 5 years of age, cMRI or CT angiography may <50 mmHg associated with aortic regurgitation of
be required. more than mild severity (Class I).
II. Beta-blockers are the preferred drugs for control of III. Patients with a peak instantaneous gradient between 30
hypertension. and 50 mmHg (Class IIb).
III. IE prophylaxis is needed for 6 months after surgery and IV. Symptomatic patients with a peak instantaneous
intervention. However, all patients are advised to gradient < 50 mmHg in the following situations:
maintain good oro-dental hygiene after this period also.
(a) Presence of left ventricular dysfunction
Aortic Stenosis (AS) attributable to obstruction (Class I).
Diagnostic Work-up: Clinical assessment, X-ray chest, (b) When pregnancy is being planned (Class IIa).
ECG, Echocardiography, CT Angiography/cardiac MRI
(in select cases), cardiac catheterisation (primarily for (c) When the patient plans to engage in strenuous/
therapeutic balloon valvuloplasty for valvar AS), competitive sports (Class IIa).
Exercise test (in select cases). V. Intervention not indicated for asymptomatic patients
Indications and Timing of Treatment with gradient of < 30 mmHg with no or trivial aortic
regurgitation (Class III).
Valvar Aortic Stenosis
Supravalvar AS
I. Immediate intervention required for:
Surgical intervention indicated in:
(a) Newborns with severe AS who are duct
dependent (balloon dilation or surgical I. Symptomatic patients with peak instantaneous
valvotomy) (Class I). gradient ≥ 64 mmHg and/or mean gradient ≥ 50mmHg on
echo-Doppler (Class I).
(b) Infants or children with left ventricular
dysfunction due to severe AS, regardless of the II. Patients with mean Doppler gradient <50
valve gradient (Class I). mmHg, if they have any of the following (Class I):
(a) symptoms attributable to obstruction (exertional
II. Elective balloon dilation for:
dyspnea, angina, syncope)
(a) Asymptomatic or symptomatic patients with AS
(b) left ventricular systolic dysfunction attributable
having gradient by echo-Doppler of >64mmHg
to obstruction.
peak or >40mmHg mean or peak to peak gradient
of ≥50mmHg, measured invasively at cardiac (c) severe left ventricular hypertrophy attributable to
catheterization (Class I). obstruction

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(d) evidence of myocardial ischemia due to coronary Mode of intervention: Balloon dilatation (Class I);
ostial involvement surgical intervention reserved for: subvalvar or supra-
valvar PS with indications same as in valvar stenosis,
III. Asymptomatic patients with mean Doppler gradient
Noonan syndrome (dysplastic valve) with hypoplastic
≥50mmHg may be considered for surgery when the
annulus and failed balloon dilatation (Class I).
surgical risk is low (Class IIb).
All patients with AS must be advised to maintain good Recommendations for Follow-up
oro-dental hygiene. I. All patients with PS require life-long follow-up.
Recommendations for Follow-up II. Clinical assessment, ECG and echo is required at each
I. All patients with AS require life-long follow-up visit; the interval depending on the severity of stenosis.
irrespective of the type of intervention. III. IE prophylaxis is recommended in patients with a
II. Clinical assessment, ECG and echo are required; the prosthetic valve. However, all patients with PS are
interval depending on the severity of stenosis. advised to maintain good oro-dental hygiene.
III. Patients who have significant AS and are planned for Tetralogy of Fallot (TOF)
an intervention should refrain from any sporting
activity. Those with asymptomatic moderate stenosis Diagnostic Work-up: Clinical assessment, pulse
can participate in low- or moderate-intensity sports. oximetry, ECG, X-ray chest, echocardiography, lab
Patients with mild degree of stenosis can participate in investigations (Hemoglobin/Packed cell volume,
all sports. Fluorescence in situ hybridization for 22q11 deletion in
some cases). CT Angiography, cardiac catheterization is
IV. IE prophylaxis is recommended in patients with a performed prior to surgery in select cases.
prosthetic valve.
Medical management (Class I): Maintain Hb >14 g/dL
Pulmonic Stenosis (PS) (by oral iron or blood transfusion). Beta blockers to be
Diagnostic Work-up: Clinical assessment, X-ray chest, given in highest tolerated doses (usual dose 1-4 mg/kg/
ECG, Echocardiography, cardiac catheterisation and day in 2 to 3 divided doses). Prostaglandin infusion for
angiography (primarily for therapeutic balloon neonates with significant cyanosis.
valvuloplasty), CT Angiography/cardiac MRI (for Management of cyanotic spell: Oxygen administration,
peripheral pulmonic stenosis). knee-chest position, intravenous fluid bolus of normal
Indications and Timing of Treatment 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
Valvar pulmonic stenosis
repeated every 5 minutes provided no hypotension or
I. Immediate intervention required for: bradycardia) or short acting Esmolol infusion (50-200
mg/kg/min), sodium bicarbonate 1-2 mEq/kg given IV,
(a) Newborns with severe PS with duct dependent
blood transfusion if required. For refractory spells,
pulmonary blood flow (Class I).
Phenylephrine infusion (2-5 µg/kg/min), IV Ketamine
(b) Infants or children with right ventricular (0.25-1.0 mg/kg bolus dose), general anaesthesia may be
dysfunction due to severe PS, regardless of the needed. Severe refractory cyanotic spell is an indication
valve gradient (Class I). for emergency surgery/intervention.
II. Elective balloon dilation for: Timing of Surgery
(a) Asymptomatic or symptomatic patients with I. Stable, minimally cyanosed: Total repair at 6-12
valvar PS having peak instantaneous gradient by months of age or earlier according to the institutional
echo-Doppler of >64mmHg (Class I). policy (Class I).
(b) Neonates and infants with any degree of PS who II. Symptomatic children of <6 months of age with
have mild hypoxia due to mild hypoplasia of significant cyanosis or history of spells despite
right ventricle, even if right ventricular function therapy: Palliation (by systemic to pulmonary artery
is normal (Class IIa). shunt or stenting of the ductus arteriosus/right
(c) Patients with valvar pulmonic stenosis due to ventricular outflow tract, or pulmonary valve balloon
dysplastic valve, who meet the above criteria valvuloplasty) or total repair depending on anatomy
(Class IIa). and centre’s experience (Class I).

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III. Patients having TOF with absent pulmonary valve who Type D- Long segment pulmonary atresia with absent
are stable: Medical management till 1 year of age main pulmonary artery. Non-confluent branch pulmonary
followed by total correction with repair of pulmonary arteries with MAPCA dependent pulmonary blood flow.
artery branch dilation/aneurysm (Class I).
Diagnostic Work-up: Clinical assessment, pulse
IV. Patients with anomalous left anterior descending oximetry, ECG, X-ray chest, echocardiography.
artery from right coronary artery crossing the right Additional imaging in the form of cardiac catheterization,
ventricular outflow tract, who are likely to need right CT angiography/cardiac MRI or a combination of these is
ventricle to pulmonary artery conduit (Class I): essential for planning definitive repair. Lab investigations
(Hemoglobin/Packed cell volume, Fluorescence in situ
(a) <10 kg weight with significant cyanosis: Aorto-
hybridization for 22q11 deletion) in some cases.
pulmonary shunt
Medical management same as outlined in section on
(b) >10 kg weight: Total repair using conduit, or Tetralogy of Fallot.
double barrel approach after two years of age,
when the child weighs >10 kg. Indications and timing of intervention

Recommendations for Follow-up Management depends on the type of VSD-PA, the

institutional experience and the clinical presentation.
I. Asymptomatic patients with no residual lesion but with Generally, this lesion requires a multistage management.
free pulmonary regurgitation, not requiring inter-
vention, should be followed up 1-2 yearly, life long. Type A (short segment VSD-PA with PDA):

II. Clinical assessment, ECG and echocardiogram is to be I. Presentation with significant cyanosis at <1 year of
done at each visit. Holter monitoring is indicated in age: Aorto-pulmonary shunt (Class I) or PDA stenting
patients suspected to have arrhythmia. (Class IIa) depending on the institutional preference
and feasibility.
III. Cardiac catheterization should be performed if any
residual lesion is suspected. It may also be required for II. After 1st intervention or those presenting at ≥1 year of
percutaneous intervention such as stenting of age: Total correction at about 1 year of age, since a
pulmonary artery branch for stenosis. right ventricle (RV) to pulmonary artery (PA) conduit
is not required (Class I).
IV. Cardiac MRI is an important investigation for follow-
Type B (Long segment pulmonary atresia with PDA):
up of these patients. In asymptomatic patients, baseline
study should be performed 10 years after surgery with I. Presentation with significant cyanosis at < 1 year of
periodic follow-up. age: Aorto-pulmonary shunt (Class I) or PDA stenting
(Class IIa) depending on the institutional preference
V. Infective endocarditis prophylaxis is indicated in non-
and feasibility.
corrected patients, patients after surgical repair for 6
months, and patients with percutaneous or surgical II. After 1st intervention or in those presenting at ≥1 year
pulmonary valve replacement. However, all patients of age (Class I):
with TOF are advised to maintain good oro-dental (a) Optimal pulmonary blood flow with good sized
hygiene even after 6 months of surgical repair. PAs – Total repair with RV to PA conduit at 3-4
Ventricular Septal Defect with Pulmonary years.
Atresia (VSD-PA) (b) Suboptimal pulmonary blood flow with small
Anatomical Types PAs – Additional shunt followed by total repair
with RV to PA conduit at 3-4 years.
Type A- Short segment valvar atresia, pulmonary arteries
confluent and good sized, supplied by a PDA. (c) Increased pulmonary blood flow with large PAs –
Total repair with RV to PA conduit by 1 year.
Type B- Long segment pulmonary atresia with absent
main pulmonary artery. Branch pulmonary arteries Type C (Long segment pulmonary atresia with
confluent and good sized, supplied by a PDA. confluent branch pulmonary arteries supplied by
MAPCAs) (Class I):
Type C- Long segment pulmonary atresia with absent
main pulmonary artery. Branch pulmonary arteries (a) Neonatal presentation – Aorto-pulmonary shunt
confluent but pulmonary blood flow dependent ± Unifocalisation of MAPCAs.
predominantly on MAPCAs. (b) After 1st intervention or late presentation: Total

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repair with RV to PA conduit and VSD closure at (b) Those presenting with low saturation and a
3-4 years of age. restrictive ASD beyond 3-4 weeks with a closed
PDA where PGE1 is likely to be ineffective
Type D (Long segment pulmonary atresia with non-
(Class IIa).
confluent branch pulmonary arteries supplied by
MAPCAs) (Class IIa): Aorto-pulmonary shunt + (c) Patient with restrictive ASD, not fit for
Unifocalisation of MAPCAs, followed by total repair with immediate surgery (e.g. having sepsis or
RV to PA conduit and VSD closure at 3-4 years of age. respiratory infection) (Class IIa).
Recommendations for Follow-up (d) Restrictive ASD in TGA patients with large VSD
or PDA: to decrease left atrial pressure and
I. All patients with VSD-PA require life-long follow-up.
pulmonary venous hypertension (Class IIa).
Clinical assessment, ECG and echocardiogram is
required; the interval depending on the nature of Timing and type of Surgery
repair, residual or additional lesions, symptoms and
I. TGA with intact ventricular septum presenting soon
functional status.
after birth: Arterial switch operation (ASO) is the best
II. Palliated patients need to be seen more frequently if option (Class I).
their oxygen saturation is low and to decide for the
Timing of surgery: 7 days to 3 weeks, earlier if baby is
next intervention.
unstable or has associated persistent pulmonary
III. Infective endocarditis prophylaxis is indicated in non- hypertension of the newborn. Exact timing based on
corrected or palliated patients with cyanosis, patients institutional preference, but is best done before 4 weeks.
after surgical repair for 6 months, and patients with
II. TGA with intact ventricular septum presenting beyond
conduits and pulmonary valve replacement. All
3-4 weeks of life with regressed left ventricle:
patients are advised to maintain good oro-dental
hygiene even after 6 months of surgical repair. (a) Presenting between 1 to 2 months: ASO;
extracorporeal membrane oxygenator (ECMO)
Indications for pulmonary valve replacement are same
support may be required in some cases (Class
as in Tetralogy of Fallot [6].
IIa).
Transposition of Great Arteries (TGA)
(b) Presenting between 2 to 6 months: ASO with
Diagnostic Work-up: Clinical assessment, pulse ECMO support or rapid two stage ASO* or an
oximetry, ECG, X-ray chest, echocardiography, cardiac atrial switch (if rapid two stage or ECMO not
catheterization (for balloon atrial septostomy or feasible) (Class IIa).
assessment of adequacy of left ventricle for an ASO or to
(c) Presenting between 6 months to 2 years: Atrial
assess pulmonary vascular resistance in late presenters),
switch operation (Senning or Mustard operation)
CT angiography and cardiac MRI (rarely required).
(Class IIa). Rapid two stage ASO* to be
Indications and Timing of Surgery considered in select cases after detailed
evaluation (Class IIb).
Surgery is indicated for all patients with TGA except in
those with irreversible pulmonary vascular disease. *The first stage of rapid two stage ASO involves retraining of
regressed left ventricle by performing pulmonary artery banding along
Pre-surgical stabilization (Class I): with the addition of a modified aorto-pulmonary shunt as the first
stage. The same can also be achieved in select patients by stent
I. Start intravenous infusion of Prostaglandin E1 (PGE1), placement in a patent ductus arteriosus (Class IIb). It must be noted
soon after delivery, if oxygen saturation is lower than that ASO with ECMO support and rapid two stage ASO have higher
75% and/or lactic acidosis is present. Monitor morbidity and mortality than primary ASO.
respiration as PGE1 infusion may result in apnea. Use III. TGA with a large VSD and/or a large PDA: ASO with
lowest maintenance dose once PDA is open. VSD and/or PDA closure by 6 weeks of age (Class I).
II. Balloon atrial septostomy: This procedure is most These patients develop early pulmonary vascular
successful in patients younger than 6 weeks, but can be disease and may become inoperable by 6 months to
tried in older infants also if the atrial septum is thin. one year of age.
Indications include: IV. TGA with VSD and coarctation of aorta: ASO with
(a) Low saturations despite PGE1 infusion and ASD VSD closure and arch repair as soon as possible (Class
is restrictive (Class I). I). It is preferable to repair all lesions in a single stage.

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V. TGA with VSD and significant left ventricular outflow catheterization (in select cases), CT angiography and
obstruction (Class I): cardiac MRI (when anatomy unclear).
(a) Subvalvar pulmonary obstruction with normal or Indication and Timing of Surgery
near-normal pulmonary valve and pulmonary
Surgery is indicated in all patients with DORV except in
annulus: ASO with resection of subvalvar
those with irreversible pulmonary vascular disease.
stenosis.
Timing and type of surgery depends on DORV variant
(b) If obstruction involves pulmonary valve or is
(Class I)
subpulmonary but not amenable to resection:
I. DORV with subaortic VSD and pulmonary stenosis
(i) Neonates and infants presenting with
(TOF type DORV):
significant cyanosis: The options depend on
patient’s age and surgeon’s preference: (a) Presenting with significant cyanosis at <3-4
a. Systemic to pulmonary shunt (at any age) months: Aorto-pulmonary shunt
followed by Rastelli type repair or root (b) Presenting with significant cyanosis at >3-4
translocation (at 2-3 years of age, or months: Total repair with closure of VSD and
when the child weighs >10kg). infundibular resection.
b. Réparation à l’Etage Ventriculaire (REV) (c) Stable patients with no or minimal cyanosis:
procedure (usually done at 4-6 months) Total repair with closure of VSD and
c. Pulmonary root translocation (usually infundibular resection by 6-12 months.
done at 6-12 months) II. DORV with large subaortic VSD and pulmonary
d. Nikaidoh procedure (usually done hypertension (VSD type DORV):
beyond 6-9 months of age) (a) VSD closure by 6 months of age.
(ii) In older, stable patients, presenting beyond 2- (b) Presenting beyond 6 months of age: assess for
3 years of age: One of the following operability and close VSD if operable.
surgeries: Rastelli type repair, Nikaidoh
procedure or root translocation surgery. III. DORV with subpulmonary VSD and pulmonary
hypertension (TGA type DORV):
(c) If the VSD is remote and not amenable to one of
the biventricular repairs: Multistage palliative (a) Arterial switch operation (ASO) with VSD
cavo-pulmonary connection (Class IIa). closure by 6 weeks of age.
(b) If presenting beyond 3 months, should be
Recommendations for Follow-up
evaluated for operability. ASO with VSD closure
I. All patients need lifelong follow-up. Follow-up if operable.
intervals depend on age, type of surgery and residual
(c) If associated with aortic arch abnormality, arch
findings.
repair should be done in same sitting.
II. In operated patients with no residual defects: Follow-
up visits should be at 1, 3 and 6 months after surgery, IV. DORV with subpulmonary VSD and pulmonary
yearly after that till onset of adult life and every 2-3 stenosis:
years thereafter. (a) If pulmonary obstruction is localized e.g.
subvalvar fibrous membrane or ridge: ASO with
III. Follow-up visits should include clinical assessment,
resection of subvalvar stenosis.
ECG and echocardiography.
(b) If pulmonary obstruction is tubular or valvar:
IV. Infective endocarditis prophylaxis is recommended in
One of the following complex surgeries required:
patients with cyanosis, and for 6 months after
Rastelli type repair, REV procedure, Nikaidoh
definitive surgery, and in cases with conduits or other
procedure or root translocation. A systemic to
prosthetic material during surgery. However, all
pulmonary artery shunt may be required before
patients are advised to maintain good oro-dental
these procedures in those presenting early with
hygiene even after 6 months of definitive surgery.
significant cyanosis. Please refer to section on
Double Outlet Right Ventricle (DORV) “TGA with VSD and left ventricular outflow tract
Diagnostic Work-up: Clinical presentation, ECG, X-ray obstruction” for more details.
chest, pulse oximetry, echocardiography, cardiac
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V. DORV with remote VSD or associated with other arterial switch) (Class I).
complex anatomy: One should strive to perform
(b) >6 months: Double switch (atrial plus arterial
biventricular repair by intraventricular baffling of left
switch), provided that patient has not developed
ventricular connection to aorta. Univentricular
irreversible pulmonary vascular disease
palliation is done in cases where biventricular repair is
(Class I).
not possible.
(c) 3-6 months: Pulmonary artery banding followed
Recommendations for Follow-up
by double switch operation or direct double
I. All patients need lifelong follow-up, frequency to be switch operation depending on institutional
individualized depending on the type of surgery, policy (Class IIa).
presence or absence of residual lesions and functional
III. Associated with Large VSD and left ventricular
status.
outflow obstruction (pulmonary stenosis): Double
II. Follow-up visits should include clinical assessment, switch (atrial switch plus Rastelli) (Class I) or
ECG and echocardiography. univentricular repair pathway (Class IIa). If the
III. Infective endocarditis prophylaxis recommended in saturation is good, medical follow-up may be
patients with cyanosis, and in cases with conduits or considered after discussion with the family.
other prosthetic material in the heart. Prophylaxis is IV. Associated with complete heart block: Permanent,
also required for 6 months after definitive surgery. dual chamber pacemaker implantation (Class I).
However, all patients with DORV are advised to
maintain good oro-dental hygiene even after 6 months Recommendations for Follow-up
of definitive surgery. I. All patients with ccTGA require lifelong follow-up,
Congenitally Corrected Transposition of Great usually every year.
Arteries (ccTGA) II. Infective endocarditis prophylaxis is recommended
Diagnostic Work-up: Clinical assessment, pulse for all patients with cyanosis and in cases with conduits
oximetry, ECG, X-ray chest, echocardiography, cardiac or other prosthetic material in the heart. It is also
catheterization (in select cases), cardiac MRI (in adults or advised for 6 months after a definitive surgery.
after surgery), electrophysiological testing (selected However, all patients with ccTGA are advised to
patients, who have arrhythmias/blocks). maintain good oro-dental hygiene.

Indications and Timing of Surgery [7,8] Univentricular Hearts (Single ventricles)

General recommendations: Diagnostic Work-up: Clinical assessment, ECG, X-ray

chest, pulse oximetry, echocardiography, cardiac
I. Tricuspid valve (systemic atrioventricular valve) catheterisation, CT angiography, cardiac MRI.
surgery for severe regurgitation should be considered
before systemic ventricular failure (ejection fraction Timing and Type of Intervention
<45%) sets in (Class IIa). Preamble: Surgery for univentricular heart is a palliative
II. Anatomic repair (double switch operation - atrial procedure. The life expectancy is less than normal (exact
switch plus arterial switch or Rastelli) may be age cannot be predicted), and is interposed by
considered when left ventricle is functioning at interventions over these years [9]. Treating physician
systemic pressure and when such surgery is feasible must inform and discuss the details with the parent/
(Class IIa). guardian prior to surgery.
Indications and Timing for Specific Groups of The timing and type of intervention depends on age at
ccTGA presentation and presence or absence of obstruction to
pulmonary blood flow.
I. No associated anomalies: Medical follow-up to look
for any development of tricuspid regurgitation or right I. Those presenting in neonatal period, or within 2-3
ventricular dysfunction (Class I). Neonatal double months of life (Class I):
switch operation may be considered (Class IIb). (a) With increased pulmonary blood flow:
II. Associated with large VSD (i) Type of surgery: Pulmonary artery banding at
(a) <3 months: Pulmonary artery banding followed 4-6 weeks of age, preferably before 3
later by double switch operation (atrial plus months.

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(ii) Additional procedures may be required if Persistent Truncus Arteriosus

systemic outflow obstruction is present.
Classification of truncus arteriosus (Van Praagh
(b) With decreased pulmonary blood flow and Van Praagh’s) [10]
(pulmonary stenosis group): Systemic to
I. Type A1 - Aorta and main pulmonary artery originate
pulmonary artery shunt or stenting of ductus
from a single large common trunk.
arteriosus if systemic arterial saturation is
consistently below 70%-75%. II. Type A2 - Both pulmonary arteries arise separately and
directly from the truncus.
(c) With balanced pulmonary circulation: The baby
usually maintains saturations above 80% and is III. Type A3 - One pulmonary artery arises from the
not in failure. Such infants should be followed up truncus and the other is supplied by the patent ductus
closely. Surgery if saturation falls below 70%. arteriosus or collaterals from the aorta.
(Class I).
IV. Type A4 - There is associated obstructive lesion of the
II. Those presenting later in life or have undergone first aortic arch.
surgery earlier:
Diagnostic Work-up: Clinical assessment, pulse
(a) With pulmonary hypertension and no pulmonary oximetry, X-ray chest, ECG, echocardiography, CT
stenosis: Most patients who present beyond 3-4 angiography/cardiac MRI (select cases), cardiac
months would become unsuitable for pulmonary catheterisation (when operability is in doubt).
artery banding or any definitive repair in the
Ideal Age for Surgery: Surgery indicated in all, unless
future due to irreversible increase in pulmonary
patient is inoperable.
vascular resistance.
I. Uncontrolled heart failure: Surgical repair as soon as
(b) With normal pulmonary pressure and resistance
possible (Class I).
due to pulmonary stenosis/previous pulmonary
artery banding/previous aorto-pulmonary shunt: II. Controlled heart failure: Surgical repair by 3-6 weeks
of age (Class I).
(i) Bidirectional Glenn procedure between 4-12
months of age (Class I). Type of surgery
(ii) Total cavo-pulmonary connection or Total repair using right ventricle to pulmonary artery
completion of Fontan procedure (preferably conduit. The prospects of repeat surgeries in future for
extracardiac): Between 4-7 years of age conduit obstruction should be discussed with parents.
when the child weighs 15-20 kg. Fenestration Truncal valve is repaired if it is regurgitant.
of Fontan circuit is indicated in high-risk
Contraindication for Surgery
cases.
Severe pulmonary arterial hypertension with irreversible
Recommendations for Follow-up
pulmonary vascular disease (Class III).
I. All patients with univentricular heart (operated or
Recommendations for Follow-up after Surgery
unoperated) require lifelong follow-up. Frequency
should be individualised, but should be at least once a I. Lifelong follow-up is required in view of above listed
year in stable cases. postoperative issues.
II. Drugs after surgery: Aspirin (3-5 mg/kg/day) for all II. Follow-up after surgery with clinical assessment, X-
patients. Oral anticoagulants (warfarin) and sildenafil ray chest, ECG and echocardiography at 1, 6 and 12
in select group, or as per institution policy [9]. months, and yearly thereafter in stable cases.
III. The threshold for performing cardiac catheterisation Infective endocarditis prophylaxis is recommended
during follow-up should be low as a number of after surgical repair due to presence of conduit. All
complications can be successfully treated if diagnosed patients are advised to maintain good oro-dental hygiene.
in time.
Total Anomalous Pulmonary Venous
IV. Infective endocarditis prophylaxis recommended in Connection (TAPVC)
patients with cyanosis and in cases with conduits or
Types of TAPVC
other prosthetic material in the heart. However, all
patients with univentricular heart are advised to Type I: Anomalous connection at supracardiac level (to
maintain good oro-dental hygiene. innominate vein or right superior vena cava)

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Type II: Anomalous connection at cardiac level (to deteriorating exercise capacity, cyanosis (oxygen
coronary sinus or right atrium) saturation <90%), paradoxical embolism, progressive
cardiomegaly on chest X-ray (cardiothoracic ratio >0.65),
Type III: Anomalous connection at infradiaphragmatic
progressive dilation or dysfunction of the right ventricle
level (to portal vein or inferior vena cava)
on echocardiography.
Type IV: Anomalous connection at two or more of the
Types of Surgery: Depends on the underlying anatomy
above levels.
and size of the functional ventricle. Options include
Diagnostic Work-up: Clinical assessment, pulse tricuspid valve repair (Cone repair, best done at about 2
oximetry, ECG, X-ray chest, echocardiography, cardiac years of age)/replacement (if repair not possible), and one
catheterization (Rarely performed when operability is in and a half ventricle repair.
doubt), CT angiography/cardiac MRI (select cases).
Recommendations for Follow-up
Indications and Timing of Surgery (all are Class I
I. ECG, X-ray chest and echocardiography should be
recommendations)
done at each visit. Holter, exercise testing and cardiac
I. Patients with obstructive TAPVC should undergo MRI may be required in select patients.
emergency surgery. II. Asymptomatic patients who are not candidates for
II. Surgery should be performed as early as possible in surgery can be followed up every 2-3 years.
non-obstructive TAPVC, even if they are III. Infective endocarditis prophylaxis is indicated in
asymptomatic. patients who have undergone tricuspid valve
III. Those presenting late should be evaluated for onset of replacement, have previous history of endocarditis or
pulmonary vascular disease and operated if the data have cyanosis. However, all patients with Ebstein’s
suggests operable status. anomaly are advised to maintain good oro-dental
hygiene.
Recommendations for Follow-up
Mitral and Aortic Regurgitation
I. After surgery, patients should be followed up at one
month, 6 months and then annually for 5 years if there Background: Mitral (MR) and aortic regurgitation (AR)
is no residual defect. occur most commonly secondary to acute or chronic
rheumatic heart disease, and they may co-exist in some.
II. Since arrhythmias can occur long after TAPVC Congenital MR is uncommon, however congenital AR
surgery, parents/patients should be informed to report due to a congenitally bicuspid aortic valve is not rare.
if any symptom suggestive of arrhythmia develops.
Diagnostic Work-up: Clinical assessment, ECG, X-ray
Infective endocarditis prophylaxis is indicated in non- chest, echocardiography, exercise test (in select cases),
corrected patients and in patients after surgical repair for CT angiography or cardiac MRI (in select cases).
6 months. However, all patients with TAPVC are advised
to maintain good oro-dental hygiene after this period Medical Therapy
also. I. Angiotensin converting enzyme inhibitors are
Ebstein’s Anomaly of the Tricuspid Valve indicated in patients with severe MR and severe AR.
Diuretics to be used in those with dyspnea due to heart
Diagnostic Work-up: Clinical assessment, pulse failure.
oximetry, ECG, X-ray chest, echocardiography, cardiac
II. Sodium nitroprusside infusion is recommended for
catheterization (in select cases), cardiac MRI (important
treatment of acute MR; invasive BP monitoring is
when planning surgical repair), electrophysiological
required for these cases.
studies (select cases).
III. Anticoagulants (oral) if atrial fibrillation is present.
Indications and Timing for Treatment
IV. Secondary prophylaxis, preferably with long acting
Presentation in neonatal period: Significant cyanosis: IV Benzathine penicillin injection, is required for patients
Prostaglandin infusion; Heart failure: Anti-failure who have underlying rheumatic heart disease as the
therapy including diuretics; Tachyarrhythmias: etiology of MR or AR.
Antiarrhythmic drugs; Surgery for neonates not stabilized
Indications and Timing of Surgery
with medical therapy (Class IIa).
Mitral regurgitation [11,12]
Presentation in older children and adults: Surgery is
indicated (Class I) in those with symptoms or I. Symptomatic patients with moderate to severe MR

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with left ventricular ejection fraction >30% (Class I). (iii) After valve repair, bioprosthetic valve: 2.5
(±0.5)
II. Asymptomatic patients with severe MR: Surgery
indicated if any of the following present (Class IIa): (b) Patients should be educated about the importance
of maintaining INR in therapeutic range, the
(a) Left ventricular ejection fraction <60%.
effect of diet, medicines, etc. on INR and the
(b) Left ventricular end systolic dimension Z score warning signs of overdose of warfarin. These
>3 for mitral valve replacement; and >2.5 if patients should be advised to avoid contact
likelihood of mitral valve repair is >95%. sports; otherwise normal activities are allowed.
Regular intramuscular immunization can be
(c) Pulmonary artery systolic pressure >50mmHg.
given while on oral anticoagulant drugs. Dental
III. Asymptomatic patients with moderate or severe MR surgery is safe with therapeutic levels of INR.
undergoing cardiac surgery for another indication
(c) Duration of anticoagulation:
(Class IIa).
(i) Valve repair, bioprosthetic valve: For 3
Aortic regurgitation [11]
months after surgery
I. Symptomatic patients with moderate to severe AR
(ii) Prosthetic metallic valve: Lifelong
(Class I).
(d) Oral anticoagulants are also indicated for
II. Asymptomatic patients with severe AR: Surgery
patients with atrial fibrillation.
indicated if any of the following present (Class I):
II. Aspirin: Dose - 3 to 5 mg/kg/day given in addition to
(a) Left ventricular ejection fraction <50%.
anticoagulation (Class I).
(b) Left ventricular end systolic dimension Z score
(a) Duration: Valve repair, bioprosthetic valve: For 6
>4.
months after surgery
III. Asymptomatic patient with moderate or severe AR
(a) Prosthetic metallic valve: Lifelong
undergoing cardiac surgery for another indication
(Class I). Recommendations for Follow-up
All patients with valvular regurgitation must be advised I. Patients with valve lesions require lifelong follow-up.
to maintain good oro-dental hygiene.
II. Asymptomatic patients with MR or AR: Clinical
Type of Valve Surgery [13] assessment, ECG and echocardiography at periodic
intervals.
I. Valve repairs are preferable to valve replacements
(Class I). III. Operated patients with no residual abnormality:
Clinical assessment, ECG and echocardiography.
II. Valve replacement in those in whom valve cannot be
Patients with prosthetic metallic valve require frequent
repaired (Class IIa):
monitoring of INR and fluoroscopy (for valve motion).
(a) Ross procedure for young patients with non
Infective endocarditis prophylaxis [14]: All patients
rheumatic AR (if expertise available).
must be advised to maintain good oro-dental hygiene
(b) Bioprosthetic valve for: female patients planning after valve surgery. Prophylaxis is reasonable before
pregnancy in future, or if compliance with oral dental procedures that involve manipulation of gingival
anticoagulation is dubious. tissue or periapical region of teeth, or perforation of the
oral mucosa, in patients with prosthetic valve and also in
(c) Prosthetic metallic valve replacements for the
those where prosthetic material is used for valve repair
rest of patients.
(e.g. annuloplasty rings).
Anticoagulation after Valve Surgery [14] These guidelines originated from a National Consensus Meeting on
I. Oral anticoagulant drug: Warfarin or other “Management of Congenital Heart Diseases in India” held on 10th
and 11th of August, 2018 at the All India Institute of Medical Sciences,
anticoumarin drug
New Delhi, India.
(a) Desired INR (International Normalized Ratio): Contributors: All authors were part of the National Consensus
(i) After mitral valve replacement: 3.0 (±0.5) Meeting that formulated these Guidelines. All authors reviewed
the literature and drafted recommendations of respective
(ii) After aortic valve replacement: 2.5 (±0.5) sections assigned to them. The final document was drafted and

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SAXENA, et al. INDIAN GUIDELINES FOR MANAGEMENT OF CHDS

compiled by AS and JR. All authors provided critical inputs at Surg. 2017;154:1023-5.
every stage to finalize the draft recommendations. All authors 8. Warnes CA, Williams RG, Bashore TM, Child JS,
approved the final document. Connolly HM, Dearani JA, et al. ACC/AHA 2008
Guidelines for the Management of Adults with Congenital
Funding: None; Competing interest: None stated. Heart Disease: A report of the American College of
REFERENCES Cardiology/American Heart Association Task Force on
Practice Guidelines (writing committee to develop
1. EUROCAT Steering Committee. Congenital Heart Defect guidelines on the management of adults with congenital
in Europe; c2014. Available from: http://www.eurocat heart disease). Circulation. 2008;52(23):e143-263.
network.eu/content/EUROCAT Special Report CHD.pdf. 9. Alsaied T, Alsidawi S, Allen CC, Faircloth J, Palumbo JS,
Accessed on Mar 05, 2019. Veldtman GR. Strategies for thromboprophylaxis in Fontan
2. Hoffman JI. The global burden of congenital heart disease. circulation: A meta-analysis. Heart. 2015;101:1731-7.
Cardiovasc J Afr. 2013;24:141-5. 10. Van Praagh R, Van Praagh S. The anatomy of common
3. Bernier PL, Stefanescu A, Samoukovic G, Tchervenkov aorticopulmonary trunk (truncus arteriosus communis) and
CI. The challenge of congenital heart disease worldwide: its embryologic implications: A study of 57 necropsy cases.
Epidemiologic and demographic facts. Semin Thorac Am J Cardiol. 1965;16:406-25.
Cardiovasc Surg Pediatr Card Surg Annu. 2010;13:26-34. 11. Carapetis JR, Beaton A, Cunningham MW, Guilherme L,
4. Warnes CA. The adult with congenital heart disease: Born Karthikeyan G, Mayosi BM, et al. Acute rheumatic fever
to be bad. J Am Coll Cardiol. 2005;46:1-8. and rheumatic heart disease. Nat Rev Dis Primers.
5. Working Group on Management of Congenital Heart 2016;2:15084.
Diseases in India. Consensus on timing of intervention for 12. Johnson JT, Eckhauser AW, Pinto NM, Weng HY, Minich
common congenital heart disease. Indian Pediatr. LL, Tani LY. Indications for intervention in asymptomatic
2008;45:117-26. children with chronic mitral regurgitation. Pediatr Cardiol.
6. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg 2015;36:417-22.
CS, Colman JM, et al. 2018 AHA/ACC Guideline for the 13. Finucane K, Wilson N. Priorities in cardiac surgery for
Management of Adults With Congenital Heart Disease: rheumatic heart disease. Glob Heart. 2013;8:213-20.
Executive Summary: A Report of the American College of 14.`Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin
Cardiology/American Heart Association Task Force on JP, Fleisher LA, et al. 2017 AHA/ACC focused update of
Clinical Practice Guidelines. J Am Coll Cardiol. the 2014 AHA/ACC guideline for the management of
2019;139:e637 97. patients with valvular heart disease: A report of the
7. Tweddell JS. What do we really know about the American College of Cardiology/American Heart
management of patients with congenitally corrected Association Task Force on Clinical Practice Guidelines. J
transposition of the great arteries? J Thorac Cardiovasc Am Coll Cardiol. 2017;70:252-89.

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.

INDIAN PEDIATRICS 157 VOLUME 57__FEBRUARY 15, 2020