Neonatal Cardiac Interventions

Single-Ventricle and Hypoplastic Left Heart Syndrome (HLHS) Procedures

• Norwood operation: It is the first stage of palliation for Hypoplastic Left Heart Syndrome and related single-ventricle lesions. The main pulmonary artery is incorporated into the aorta to create a neo-aorta, the atrial septum is opened widely, and pulmonary blood flow is supplied either through a systemic-to-pulmonary shunt or an RV-to-PA conduit.

  • Sano Modification (RV-to-PA Conduit)

    • A modification of the Norwood procedure in which pulmonary blood flow is supplied through a valveless conduit from the right ventricle to the pulmonary arteries instead of a modified Blalock-Taussig shunt. This approach may improve coronary perfusion and reduce diastolic runoff.

  • Hybrid Stage I Palliation

    • An alternative to the Norwood procedure for selected high-risk neonates with HLHS. It combines bilateral pulmonary artery banding, ductal stenting, and atrial septostomy while avoiding cardiopulmonary bypass in the neonatal period.

• Damus-Kaye-Stansel (DKS) Procedure: The proximal pulmonary artery is connected to the ascending aorta to bypass severe left ventricular outflow tract obstruction. It is commonly used in complex single-ventricle physiology and is usually combined with a source of pulmonary blood flow.

• Kawashima Procedure: A bidirectional Glenn (superior cavopulmonary connection) performed in patients with interrupted inferior vena cava (IVC) and azygos continuation, most commonly in single-ventricle physiology. Because lower body venous return travels through the azygos vein into the superior vena cava, the Glenn circulation effectively directs nearly all systemic venous return to the pulmonary arteries, with the exception of hepatic venous flow, which remains connected to the heart until Fontan completion.

• Bidirectional Glenn / Superior Cavopulmonary Anastomosis: Second-stage single-ventricle palliation in which the superior vena cava is directly connected to the pulmonary arteries, allowing passive pulmonary blood flow while reducing ventricular volume load and improving systemic oxygenation. Although most commonly performed at 4–6 months of age, the procedure may occasionally be undertaken earlier in selected neonates or young infants with unstable physiology or unsuitable pulmonary blood flow.

• Fontan Procedure: Final stage of single-ventricle palliation in which systemic venous return is directed entirely to the pulmonary arteries without passage through a subpulmonary ventricle. The procedure connects the inferior vena cava to the pulmonary circulation, typically using an extracardiac conduit or lateral tunnel, completing separation of systemic and pulmonary circulations following prior staged palliation (e.g., Norwood and bidirectional Glenn procedures). Successful Fontan physiology depends on low pulmonary vascular resistance, adequate ventricular function, competent atrioventricular valves, and unobstructed pulmonary venous return.

• Staged Single-Ventricle Palliation: Refers to the sequence of surgeries used in single-ventricle physiology, typically beginning with neonatal palliation (Norwood, pulmonary artery banding, or shunt placement), followed later by bidirectional Glenn and Fontan completion.

• One-and-a-Half Ventricle Repair: Hybrid circulatory strategy used in patients with a small, dysfunctional, or volume-overloaded right ventricle that is inadequate to support the entire pulmonary circulation but sufficient to contribute partially to forward flow. The procedure combines a bidirectional Glenn (superior cavopulmonary connection) with preservation of antegrade flow from the right ventricle to the pulmonary arteries, thereby reducing right ventricular volume load while maintaining partial biventricular physiology. Common indications include severe Ebstein anomaly, pulmonary atresia with hypoplastic right ventricle, unbalanced ventricles, or complex congenital heart disease with borderline right ventricular capacity.

Transposition and Conotruncal Repairs

• Arterial Switch Operation (ASO / Jatene Procedure): The definitive anatomical repair for Transposition of the Great Arteries. The great arteries are transected and reconnected to the appropriate ventricles, and the coronary arteries are transferred to the neo-aorta.

• Aortic Arch Reconstruction with Arterial Switch: A combined neonatal repair for d-TGA or double-outlet right ventricle associated with coarctation or interrupted aortic arch. The operation combines arterial switch with reconstruction of the aortic arch.

• Mustard Procedure: A historical atrial switch operation for d-TGA in which systemic and pulmonary venous return are redirected at the atrial level using a synthetic baffle. The morphologic right ventricle remains the systemic ventricle.

• Senning Procedure: Another atrial switch operation for d-TGA using native atrial tissue rather than prosthetic material to redirect venous return. It has largely been replaced by the arterial switch operation.

• Rastelli Procedure: Used in selected patients with double-outlet right ventricle or transposition with VSD and pulmonary stenosis. A baffle directs left ventricular blood through the VSD to the aorta, while a conduit connects the right ventricle to the pulmonary arteries.

• REV Procedure (Réparation à l’Étage Ventriculaire): An alternative to the Rastelli operation used for transposition physiology with ventricular septal defect and left ventricular outflow tract obstruction. The procedure involves resection of the conal (outlet) septum to create an unobstructed left ventricular–to–aorta pathway, intraventricular baffling of the left ventricle to the aorta, and the Lecompte maneuver with direct right ventricle–to–pulmonary artery reconstruction, typically without the use of a valved conduit (often using a pericardial hood).

• Nikaidoh Procedure: An aortic root translocation procedure used in complex transposition physiology with left ventricular outflow obstruction. The aortic root is moved posteriorly onto the left ventricle, and the RVOT is reconstructed.

• Yasui Procedure: A complex biventricular repair used in neonates with interrupted aortic arch, VSD, and severe left ventricular outflow tract obstruction. It combines arch reconstruction with a Damus-Kaye-Stansel connection and RV-to-PA conduit placement.

• Truncus Arteriosus Repair: The pulmonary arteries are separated from the common arterial trunk, the VSD is closed to route left ventricular output to the truncal root, and an RV-to-PA conduit is placed to establish pulmonary blood flow.

• Unifocalization: A reconstructive procedure for pulmonary atresia with major aortopulmonary collateral arteries (MAPCAs). Collateral vessels are detached from the aorta and unified into a central pulmonary artery system.

• Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) Repair: Re-implantation of the anomalous left coronary artery directly into the aorta to establish a dual coronary artery system and restore normal myocardial perfusion. When direct re-implantation is anatomically not feasible, a Takeuchi intrapulmonary baffle may be created to route oxygenated blood from an aortopulmonary window to the coronary ostium. 

• Aortopulmonary Window Repair: Surgical division and patch closure of the abnormal communication between the ascending aorta and the main pulmonary artery. This separates the systemic and pulmonary circulations to prevent severe neonatal pulmonary overcirculation and the rapid development of pulmonary vascular disease.

• Double Switch Operation: Complex biventricular repair for congenitally corrected transposition of the great arteries (cc-TGA) designed to restore the morphologic left ventricle as the systemic ventricle. The operation combines an atrial switch procedure (Mustard or Senning) with an arterial switch or Rastelli-type repair, depending on anatomy. In patients with an underprepared left ventricle, pulmonary artery banding may first be performed in infancy or the neonatal period to retrain the left ventricle for systemic workload. The double switch itself is typically not a neonatal operation — it is usually performed in later infancy or childhood after LV retraining. Only the preparatory PA banding is neonatal.

• Lecompte Maneuver: Anterior translocation of the pulmonary artery bifurcation over the ascending aorta without division of the branch pulmonary arteries. Commonly performed during the arterial switch operation and REV procedure to reduce tension on the pulmonary arteries and optimize right ventricular outflow tract geometry. 

Aortic Arch and Left Ventricular Outflow Procedures

• Coarctation Repair (Extended End-to-End Anastomosis): The current standard repair for neonatal coarctation of the aorta. The narrowed segment is excised and the descending aorta is connected to the transverse arch with extension into the lesser curvature to address associated arch hypoplasia.

• Thoracotomy Coarctation Repair: A traditional left thoracotomy approach used for isolated coarctation without major intracardiac lesions. It avoids cardiopulmonary bypass.

• Sternotomy Arch Repair: A median sternotomy approach used for coarctation associated with significant arch hypoplasia or intracardiac defects. It permits complete arch reconstruction under cardiopulmonary bypass.

• Interrupted Aortic Arch Repair: Restoration of continuity between the ascending and descending aorta, usually combined with VSD closure. This is typically performed as a single-stage neonatal repair.

• Aortic Arch Reconstruction: Comprehensive reconstruction of the hypoplastic or interrupted aortic arch using direct anastomosis and/or patch augmentation.

• Subclavian Flap Aortoplasty (Waldhausen Procedure): A historical repair technique in which the proximal left subclavian artery is used as a flap to enlarge the coarcted aortic segment.

• Reverse/Modified Subclavian Flap Aortoplasty: A modification of the standard subclavian flap technique designed to reduce anastomotic tension and improve arch geometry.

• Patch Aortoplasty: Patch enlargement of the coarcted or hypoplastic aortic segment using synthetic or biologic material. This technique is now used less frequently because of aneurysm risk.

• Autologous Pericardial Patch Arch Reconstruction: Uses the patient’s own pericardium to augment the hypoplastic arch while minimizing foreign material exposure.

• Homograft Patch Arch Reconstruction: Cryopreserved homograft tissue is used to enlarge the aortic arch, particularly in complex neonatal arch reconstruction.

• Sliding / Heineke-Mikulicz-Type Enlargement: A longitudinal incision is closed transversely to enlarge the narrowed aortic segment while minimizing tension.

• Amato Technique: A repair strategy for long-segment distal arch hypoplasia using native tissue and subclavian flap augmentation while preserving subclavian artery perfusion.

• Ross-Konno Procedure: Combines pulmonary autograft replacement of the aortic valve (Ross procedure) with enlargement of the left ventricular outflow tract (Konno procedure). Used in severe neonatal aortic stenosis with annular hypoplasia.

• Neonatal Aortic Valvotomy / Valve Repair: Surgical relief of critical neonatal aortic stenosis through commissurotomy or valve reconstruction to preserve left ventricular function.

• Aorto-Ventricular Tunnel Repair: Closure of a rare extracardiac channel connecting the ascending aorta to a ventricle, preventing severe volume overload.

• Vascular Ring Division: Surgical division of a vascular ring, most commonly a double aortic arch or a right aortic arch with an aberrant left subclavian artery and a left ligamentum arteriosum. This is typically performed via a left thoracotomy to relieve critical neonatal tracheoesophageal compression.

• Congenital Aortic Valve Replacement: Valve replacement using mechanical, homograft, or bioprosthetic options in neonates or infants with severe unreconstructable aortic valve disease or failed valvotomy/repair. Due to technical complexity and somatic growth considerations, replacement is generally reserved for highly selected cases. 

Ebstein

• Cone Repair (Da Silva Cone Reconstruction): The cone repair is a reconstructive tricuspid valve operation used primarily for Ebstein Anomaly. The malformed tricuspid valve leaflets are extensively mobilized and rotated clockwise to create a cone-shaped valve that is reattached circumferentially at the true tricuspid annulus, restoring a more normal tricuspid valve geometry and improving right ventricular function. In neonates and infants, cone repair may be performed in selected patients with sufficient functional right ventricular size and acceptable hemodynamic stability. It aims to preserve biventricular circulation and avoid progression to single-ventricle palliation, although the operation can be technically challenging in critically ill neonates with severe Ebstein physiology. 

• Starnes Procedure: The Starnes procedure is a palliative operation used for severe neonatal Ebstein Anomaly with profound tricuspid regurgitation, right ventricular failure, or circular shunt physiology. The tricuspid valve is excluded by patch closure of the tricuspid orifice, an atrial septectomy is performed to allow unrestricted right-to-left atrial flow, and pulmonary blood flow is provided through a systemic-to-pulmonary artery shunt (typically a modified Blalock-Taussig-Thomas shunt). This operation effectively converts the circulation into a single-ventricle pathway and unloads the severely dysfunctional right ventricle. It is generally reserved for critically unstable neonates who are poor candidates for primary biventricular repair.

Atria, ventricles and septa

• Ventricular Septal Defect (VSD) Patch Closure: Standard repair using a synthetic or pericardial patch, most commonly performed through a right atrial approach.

• Transpulmonary VSD Repair: The VSD is approached through the pulmonary artery, particularly for outlet or subarterial defects.

• Perventricular (Hybrid) VSD Closure: A hybrid off-pump procedure in which a closure device is delivered directly through the right ventricular wall into a muscular VSD.

• Complete Atrioventricular Septal Defect (CAVSD) Repair: Closure of the atrial and ventricular septal components with reconstruction of the common AV valve into separate right- and left-sided valves.

• Surgical ASD Patch Closure: Open surgical repair of primum, sinus venosus, coronary sinus, or large secundum atrial septal defects using a patch under cardiopulmonary bypass.

• Transcatheter ASD Device Closure: Percutaneous device closure of secundum ASDs with suitable septal rims using occluder devices such as the Amplatzer septal occluder.

• Warden Procedure: Repair for superior sinus venosus ASD associated with anomalous pulmonary venous drainage. The superior vena cava is redirected to the right atrium while anomalous veins are baffled to the left atrium.

• Blalock-Hanlon Atrial Septectomy: A historical surgical atrial septectomy performed without cardiopulmonary bypass to improve atrial mixing.

• Balloon Atrial Septostomy (Rashkind Procedure): A catheter-based enlargement of the atrial septum performed urgently in d-TGA or restrictive atrial septum physiology.

• Cor Triatriatum Repair: Surgical resection of the abnormal fibromuscular membrane that subdivides the left atrium into two distinct chambers. Complete excision of this membrane relieves the obstruction to pulmonary venous return and restores normal, unobstructed flow down to the mitral valve.

Pulmonary Venous Procedures

• TAPVC / TAPVR Repair: The pulmonary venous confluence is connected to the left atrium and anomalous venous pathways are ligated. Obstructed TAPVC requires emergent neonatal surgery.

• Sutureless Pulmonary Vein Repair: A technique used in pulmonary vein stenosis or recurrent TAPVC obstruction in which the pulmonary venous confluence is marsupialized into a pericardial well to reduce restenosis risk.

• Partial Anomalous Pulmonary Venous Return (PAPVR) / Scimitar Syndrome Repair: Surgical redirection of anomalous pulmonary venous drainage to the left atrium using intracardiac baffling or direct venous reimplantation. Scimitar syndrome, characterized by anomalous right pulmonary venous return to the inferior vena cava, may present in infancy with pulmonary hypertension, heart failure, or lung hypoplasia and can require complex neonatal or early infant repair. 

• Coronary Sinus Unroofing: Technique used in cardiac-type total anomalous pulmonary venous connection (TAPVC) repair in which the roof of the coronary sinus is excised to redirect pulmonary venous blood into the left atrium, typically combined with closure of the atrial septal defect. 

Tetralogy and Right Ventricular Outflow Procedures

• Tetralogy of Fallot (TOF) Complete Repair: Includes VSD closure and relief of right ventricular outflow tract obstruction, often with muscle resection and/or transannular patch placement.

• RVOT Stenting: A catheter-based palliative intervention used to relieve severe RVOT obstruction and improve pulmonary blood flow before complete repair.

• Balloon Pulmonary Valvuloplasty: Balloon dilation of a stenotic pulmonary valve, typically used in critical pulmonary stenosis or pulmonary atresia with intact ventricular septum.

• Radiofrequency (RF) Valve Perforation: A catheter-based intervention specifically utilized for pulmonary atresia with an intact ventricular septum. A specialized catheter uses radiofrequency energy to burn a small communication through the atretic pulmonary valve plate, which is immediately followed by balloon dilation to establish antegrade right ventricular output.

• Transannular Patch Repair (for Tetralogy of Fallot): A surgical technique utilized during the complete repair of Tetralogy of Fallot when the native pulmonary valve annulus is severely hypoplastic and cannot provide an adequate right ventricular outflow tract dimension. An incision is made extending from the right ventricular infundibulum, across the narrow pulmonary valve annulus, and into the main pulmonary artery, which is then augmented with a patch (typically autologous or treated pericardium) to effectively widen the entire outflow tract. While this approach successfully relieves the critical right-sided obstruction, it disrupts the integrity of the pulmonary valve, leading to obligate, free pulmonary regurgitation that is generally well-tolerated in infancy but frequently necessitates subsequent pulmonary valve replacement later in life due to progressive right ventricular volume overload.

• Pulmonary Atresia with Intact Ventricular Septum (PA/IVS): Repair and RV Decompression Surgical or hybrid management strategy for pulmonary atresia with intact ventricular septum aimed at establishing right ventricular outflow and promoting right ventricular growth. Procedures may include surgical pulmonary valvotomy, transannular RVOT patch augmentation, or catheter-based valve perforation and balloon valvuloplasty. In neonates with right ventricle–dependent coronary circulation and coronary sinusoids, surgical decision-making is complex, as aggressive RV decompression may compromise coronary perfusion; selective sinusoid or fistula ligation and single-ventricle palliation may be required in severe forms.

Systemic-to-Pulmonary Shunts and Palliation

• Modified Blalock-Taussig-Thomas (mBTT) Shunt: A Gore-Tex graft connecting the subclavian or innominate artery to a pulmonary artery to increase pulmonary blood flow in cyanotic congenital heart disease.

• Classic Blalock-Taussig Shunt: A historical direct anastomosis between the subclavian artery and pulmonary artery.

• Central Shunt: A Gore-Tex graft placed directly between the ascending aorta and main pulmonary artery.

• Mee Shunt: A central shunt between the ascending aorta and main pulmonary artery origin used to promote pulmonary artery growth.

• Potts Shunt: A historical side-to-side connection between the descending aorta and left pulmonary artery.

• Waterston Shunt: A historical side-to-side shunt between the ascending aorta and right pulmonary artery.

• Pulmonary Artery Banding (PAB): Placement of a constricting band around the pulmonary artery to limit pulmonary overcirculation and protect the pulmonary vasculature.

• PDA Ligation: Surgical closure of a patent ductus arteriosus using clips or sutures, commonly performed in premature infants with significant shunting refractory to medical therapy.

• PDA Stenting: Transcatheter placement of a stent within the ductus arteriosus to maintain ductal patency in ductal-dependent congenital heart lesions.

Mechanical Support and Miscellaneous Procedures

• ECMO Cannulation: Surgical cannulation for extracorporeal membrane oxygenation support in neonates with severe cardiac or respiratory failure.

• Permanent Pacemaker Implantation: Epicardial pacemaker placement for congenital or postoperative complete heart block.

• Neonatal Cardiac Transplantation: Orthotopic heart transplantation reserved for unreconstructable congenital heart disease or severe cardiomyopathy.

• Arch Reconstruction with Selective Cerebral Perfusion: A neuroprotective perfusion strategy during neonatal arch surgery using antegrade cerebral perfusion combined with hypothermia.

• Recoarctation Repair: Repeat intervention for recurrent aortic narrowing following prior coarctation repair. Management may involve balloon angioplasty, stenting, or surgical re-repair.

• Descending Aortic Aneurysm Repair: Repair of aneurysmal dilation following previous coarctation surgery, particularly after patch aortoplasty.

• VSD Exposure via Conal Septum Resection: A surgical exposure technique used to improve visualization of difficult VSDs by resecting portions of the infundibular septum.

• Berry Syndrome Repair: Complex neonatal reconstruction for the rare combination of interrupted aortic arch, aortopulmonary window, anomalous origin of the right pulmonary artery from the ascending aorta, and an intact ventricular septum — a defining feature that distinguishes Berry syndrome from other conotruncal anomalies. Surgical repair involves restoration of aortic arch continuity, closure of the aortopulmonary window, and reimplantation of the right pulmonary artery into the main pulmonary artery.

• Cardiac Tumor Resection: Surgical excision or partial debulking of primary neonatal cardiac tumors, such as large rhabdomyomas or teratomas. This is strictly indicated in the neonatal period when the mass causes severe, life-threatening intracardiac inflow or outflow tract obstruction or intractable arrhythmias. 

• Pericardial Window / Pericardiocentesis: Surgical or catheter-based drainage of the pericardial space to relieve cardiac tamponade. In neonates, this is often a critical temporizing or definitive intervention for large pericardial effusions associated with central lines, postoperative states, or congenital conditions.

• Shone Complex Staged Repair: Management strategy for Shone complex, a spectrum of multilevel left heart obstructive lesions that may include supramitral ring, parachute mitral valve, subaortic stenosis, coarctation of the aorta, and hypoplastic left-sided structures. Surgical treatment is typically staged and tailored to the severity and progression of individual obstructive lesions.

• Supravalvar Mitral Ring Resection: Surgical excision of a congenital fibromuscular membrane located above the mitral valve that causes left ventricular inflow obstruction. Frequently associated with Shone complex and other forms of congenital left-sided obstructive disease. 

• Congenital Mitral Valve Repair: Spectrum of reconstructive procedures for congenital mitral valve abnormalities including parachute mitral valve, hammock valve, mitral arcade, and dysplastic mitral stenosis. Surgical techniques may include commissurotomy, chordal division, papillary muscle splitting, leaflet augmentation, or annuloplasty to preserve native valve function. 

• Congenital Mitral Valve Replacement: Mitral valve replacement in neonates or infants with severe congenital mitral valve disease that is not amenable to repair, including severe dysplastic mitral stenosis, failed prior repair, or complex Shone-spectrum pathology. Replacement options include mechanical prostheses or homograft techniques, though the procedure remains technically challenging because of small annular size, patient growth, risk of left ventricular outflow tract obstruction, and the need for lifelong anticoagulation in mechanical valves.

References:

• https://www.jtcvstechniques.org/