Timing of intervention of common congenital heart diseases

Timing Of Intervention Of Common Congenital Heart Diseases

Timing of intervention is the most important decision a pediatric cardiologist is expected to make. It is often complex and highly individualized decision that requires careful integration of a number of variables. General principles that are involved in taking the decision to intervene are a) Hemodynamic severity of the CHD. b) Understanding the natural history of the defect. C) Newer developments in pediatric cardiac surgery, trans-catheter interventions and intensive care.

The cardiologist must balance between the predicted natural history of the underlying congenital heart disease and the current procedural outcomes. In limited resource setting like ours we also need to keep long term concerns, costs and issues with reoperations when intervening for complex congenital heart diseases. However, Early correction of congenital heart disease is desirable because it avoids adverse cardiac lesion related morbidity and neurodevelopment consequences. Early correction of a variety of congenital heart lesions is feasible and realistic with excellent results in selected Indian centers.

Ventricular septal defects(VSD)

Large VSD with pulmonary artery hypertesnion (PAH) and uncontrolled congestive cardiac failure (CHF) are to be closed as soon as possible
Large VSD with PAH but controlled CHF it recommended for closure at 3 – 6 months of age
Moderate to large VSD with moderate PAH closure before one year of age
Subpulmonic VSD with aortic valve prolapse or aortic regurgitation is an indication of VSD closure, whereas a restricive perimemnranous VSD with mild aortic regurgitation may be followed up serially for any increase in aortic regurgitation (AR) and intervened only if there is progression of AR
Small VSD with episodes of infective endocarditis or a VSD with development of RVOTO is an indication for surgical closure. Transcatheter closure of VSD – Current Recommendations – It is resonable to perform transcatheter closure of musclar VSD in infants > 5 kg, children and adults with a significant left to right shunt (>2:1). Class II a; Evidence – B.

Atrial Septum Defects 

Indications for closure for ASD are significant failure to thrive, recurrent respiratory tract infections, significant right heart enlargement and patients at high risk for a paradoxical embolism.

Timing – Ostium secndum ASD’s – Children > 3 years or > 10 Kg with a hemodynamically significant defect transcatheter closure may the most preferred choice now. Symptomatic infants with early presentation congestive right heart failure and PAH, early surgical closure is recommended.

Late presentation of ASD beyond the ideal age, closure is recommended as long as there is right heart volume overload and pulmonary vascular resistance is within the operable range. Patients with primum ASD are surgically corrected at 1-2 years of age and those with sinus venosus ASD after 2-3 years of age.

Trans catheter closure of ASD’s – Class – I – Transcatheter secundum ASD closure is indicated in patients with hemodynamically significant ASD with suitable anatomic features. (Evidence: B). Class IIa – It is reasonable to perform transcatheter secundum ASD closure in patients with transient right-to-left shunting at the atrial level who have experienced sequelae of paradoxical emboli such as stroke or recurrent transient ischemic attack (Evidence: B).

Patent ductus arteriosus

Timing :

• Symptomatic large PDA with PAH closure is recommended at 1-3 months.
• Moderate size PDA (3-4 mm) closure is recommended at 6 months of age.
• Small PDA with an audible murmur may be closed at 6-12 months. 4. Silent PDA are left alone.

Transcatheter closure:

Class I – Transcatheter PDA occlusion is indicated for the treatment of a moderate-sized or large PDA with left-to-right shunt provided the anatomy and patient size are suitable (Evidence: B). Class IIa – Transcatheter PDA occlusion is reasonable in the presence of a small left-to-right shunt with normal-sized heart chambers when the PDA is audible. (Evidence: C).

Atrio- ventricular septal defects

• Complete AV canal defects – Symptomatic children are operated after 1 month of age and in those children with controlled congestive heart failure surgical correction is done at 3- 6 months of age.
• In children with intermediate type of AV canal defects (where VSD is small) surgical correction is done after 6 months of age.

Aorto – Pulmonary Window

Large AP window with CHF: early correction, often in neonatal period is recommended. In children with controlled CHF, Closure is recommended at 1-3 months. Surgery is the preferred mode of intervention. In selected cases with smaller defects and suitable anatomy, trans catheter closure may be considered.

Aortic stenosis

For aortic valve stenosis, balloon aortic valvuloplasty is the first therapeutic procedure of choice; Balloon dilatation of aortic valve is indicated when are peak-to-peak systolic pressure gradient across the aortic valve is > 70 mmHg irrespective of the symptoms or a gradient ≥ 50 mmHg with either symptoms or electrocardiographic ST-T wave changes indicative of myocardial perfusion abnormality. In the presence of ventricular dysfunction balloon dilatation is recommended irrespective of gradients

In the presence of subvalvar or supravalvar severe AS, surgical intervention is recommended.

Pulmonic stenosis

Balloon pulmonary valvuloplasty (BPV) is the treatment of choice for valvar pulmonary stenosis. BPV is indicated for severe valvar pulmonic stenosis. BPV is also indicated in neonates with critical valvar pulmonary stenosis where pulmonary stenosis present at birth with cyanosis and evidence of duct dependent pulmonary circulation. In the presence of right ventricular dysfunction, gradient across the valve is not considered for intervention.

Coarctation of Aorta

Indication and Timing:

• In the presence of ventricular dysfunction or congestive cardiac failure, immediate intervention to relieve the obstruction at the earliest point of diagnosis.
• In the presence of mild upper limb hypertension but no evidence of LV dysfunction or CHF, intervention may be delayed beyond 3 – 6 months of age.
• Intervention for relief of coarctation is not indicated if the Doppler gradient across the coarctation segment is < 20 mmHg with a normal ventricular function.

Mode of Intervention:

Surgical correction for relief of coarctation in infants < 6 months of age, whereas balloon dilatation or surgery is indicated in children > 6 months of age. Stenting of coarctation is not recommended in younger children. It is reasonable to consider placement of a stent that can be expanded to an adult size for the initial treatment of native or recurrent coarctation of aorta in patients with significant gradients during cardiac catheterization with suitable anatomy.

Cyanotic Congenital heart disease

Tetralogy of Fallot (TOF)

All patients will require surgical repair for TOF. In infants < 3 -4 months of age with significant cyanosis and cyanotic spells, palliative modified BT shunt is generally preferred. In children with significant cyanosis > 4 months of age and suitable anatomy, corrective intra-cardiac repair is done. In children with pulmonary atresia, who require a RV to PA conduit for corrective repair, the corrective surgery may be deferred until 3-4 years of age with interim palliation by a Bidirectional Glenn shunt (BDGS) or a BT shunt based on the center’s preference.

Transposition of great arteries (TGA)

• In neonates with TGA and intact ventricular septum early arterial switch operation is performed. In children presenting late (>4 – 6 weeks) the left ventricle is assessed for features of regression. In the presence of a regressed LV, an atrial switch (Senning procedure) is performed at 4-6 months of age.
• In neonates with TGA with a large VSD and no pulmonic stenosis, an arterial switch operation is performed after about 3-4 weeks of age.
• In noenates with TGA – Intact ventricular septum with a restrictive ASD, an emergency balloon atrial septostomy is indicated.

Total anomalous pulmonary venous correction (TAPVC)

Neonates diagnosed with an obstructed TAPVC must undergo emergency surgical correction. In those neonates diagnosed with unobstructed TAPVC, early correction in the neonatal period is preferred.

Single Ventricle

In children diagnosed with single ventricle physiology lesions like Tricuspid atresia, Double inlet left ventricle the surgical management depends on the overall underlying physiology so as to aim for future Fontan (Total cavo-pulmonary anastomosis) completion. In children with increased pulmonary blood flow, a pulmonary artery band is done at 1-3 months. In children with decreased pulmonary blood flow, a palliative BT shunt or a PDA stenting is considered. If the infant is > 4-6 months of age and a suitable PA anatomy, a Bidirectional Glenn shunt is considered. The Fontan completion is done at 4 -6 years of age after a detailed pre-Fontan evaluation.