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Senning procedure
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| Senning procedure | |
|---|---|
| Specialty | Congenital cardiac surgery |
The Senning procedure is an atrial switch heart operation performed to treat transposition of the great arteries. It is named after its inventor, the Swedish cardiac surgeon Åke Senning (1915–2000), also known for implanting the first permanent cardiac pacemaker in 1958.
Brief history
[edit]This procedure, a form of atrial switch, was developed and first performed by Senning in 1957 as a treatment for d-TGA (dextro-Transposition of the great arteries) before improvements in cardiopulmonary bypass made more curative surgical techniques feasible.[1] In this congenital heart defect, the venous circulation drains into the right ventricle but from this chamber, blood is directed towards the systemic circulation through the aorta. This is also expressed by the term ventriculoarterial discordance, that is the ventricles are connected to the wrong great artery (the right ventricle to the aorta, thus pumping blood from the systemic venous back into the systemic arterial circulation). Thus, d-TGA is not to be confused with l-TGA, where there is both atrioventricular and ventriculoarterial discordance.[citation needed]
In the absence of a shunt, patients with d-TGA could not survive because there would be no flow of oxygenated blood (coming from the pulmonary veins) to the rest of the body after the normal prenatal shunts physiologically close a few weeks after birth. This congenital heart defect caused babies to "turn blue" due to the lack of oxygen flowing through the blood.[2][3] Before this technique became available, in 1950, two cardiac surgeons, Blalock and Hanlon, had developed a palliative procedure that consisted of opening the atrial septum.[1] Since, in TGA, the atrial septum prevents oxygenated blood from reaching the systemic circulation, this simpler procedure leads to improvements in systemic arterial O2 saturation.[citation needed]
Technical aspects
[edit]With the Senning surgical repair, a baffle – or conduit - is created within the atria that reroutes the deoxygenated blood coming from the inferior and superior vena cavae to the mitral valve and therefore to the pulmonary circulation [4] This is accomplished by creating a systemic venous conduit that channels deoxygenated blood from the superior and inferior vena cava towards the mitral valve. After this complex plastic reconstruction using flaps from the right atrial tissue and the interatrial septum, it lets the oxygenated pulmonary venous blood flow to the tricuspid valve and from there to the systemic circulation. The anatomic left ventricle continues to pump into the pulmonary circulation and the anatomic right ventricle will work as the systemic pump, in other words, the ventriculo-arterial mismatch is left unrepaired. In the Senning's operation, atrial tissue is used to create the baffle. No prosthetic material is introduced. A complex work of incising and refolding the native atrial tissue - which is so technically complex that has been referred to as "origami", is necessary to build the venous baffle. Indeed, the Senning technique was difficult to reproduce and was not widely embraced.[citation needed]
In 1963, Mustard described an alternative technique, the Mustard procedure, in which the atrial septum is excised, and the atrial baffle is created by the placement of a single elephant trunk-shaped patch made of pericardial tissue. This technique then became the standard operation for TGA as it was technically less demanding.
Alternative surgical techniques
[edit]Currently, the arterial switch or Jatene procedure is the preferred surgical corrective method. In this technique, the great arteries are excised and reimplanted to the corresponding ventricles. The Brazilian surgeon Jatene performed the first procedure in 1975. The coronary arteries are also explanted from the anatomical aorta, which lies on the venous side and reattached to the systemic great vessel. Indeed, the initial difficulties that prevented an earlier adoption of this approach were mostly the inability to transfer the coronary arteries, besides problems with early forms of cardiopulmonary bypass that made cardiac surgery in early infancy less safe than in the present times [4] Some individuals with d-TGA are not candidates for an arterial switch, particularly because of late diagnosis, coexistent VSD with associated pulmonary hypertension, inadequate left ventricular function, or complex coronary abnormalities.[1] Moreover, the Senning procedure is used as part of the double switch surgical correction of l-TGA ( Senning-Rastelli procedure).
Mortality and later health effects
[edit]The acute mortality associated with the Senning procedure is reported to be around 5-10%. Patient selection and the complexity of the congenital malformation are determinants of mortality risk. Patients who have undergone such surgical correction of the congenital transposition are exposed to long-term risks of cardiovascular events. In particular, sinus node dysfunction, atrial arrhythmias, ventricular arrhythmias including sudden cardiac arrhythmic death, heart failure due to anatomically right ventricular failure, or venous obstruction at the level of the baffle or caval anatomy have been described. The high chance of developing arrhythmias results in up to 25% of patients who have undergone a Senning or Mustard procedure having a pacemaker by adulthood.[5]
Long-term studies have disclosed that although from the functional capacity standpoint the Senning and the Mustard operation are similar, there is a higher risk of sinus node disease and arrhythmias with the latter.[6] Overall, in most studies, survival is good into the second decade post-procedure. 78% of patients were alive after 16 years in a large follow-up study from the Netherlands.[7]
Before the utilization of surgical repair, Kirklin reports that the mortality associated with unrepaired TGA was 55%, 85%, and 90% mortality rates at 1 month, 6 months, and 1 year, respectively. These numbers correspond to all types of TGA.[8] A major factor affecting long-term morbidity and mortality is the coexistence of a ventricular septal defect (VSD). Patients with a concomitant VSD may have also developed pulmonary vascular disease.
Support
[edit]A patient run Facebook group mustard and Senning survivors https://www.facebook.com/groups/mustsenn is for adults born with TGA and living with Senning repair. The group gathers well over a thousand global survivors in their 20s to 60's and even some survivors in their 70's into a single community, supporting adults born with TGA that have had a Mustard, Senning, Rastelli, Fontan repair or Nikaidoh heart procedure.
References
[edit]- ^ a b c Atrial Switch Operation: Past, Present, and Future. Konstantinov IE, Alexi-Meskishvili VV, Williams WG et al.Ann Thorac Surg 2004;77:2250 – 8
- ^ Gawande, Atul (2002). Complications A Surgeon's Notes on an Imperfect Science- Education of a Knife. New York: Picador. pp. 27. ISBN 978-0312421700.
Such children are born with their heart's outflow vessels transposed: the aorta emerges from the right side of the heart instead of the left and the artery to the lungs emerges from the left instead of the right. As a result, blood coming in is pumped right back out to the body instead of first to the lungs, where it can be oxygenated. This is unsurvivable. The babies died blue, fatigued, never knowing what it was to get enough breath.
- ^ "The Mustard and Senning Procedure for Transposition of the Great Arteries (TGA)". Congenital Heart Defects UK.
- ^ a b In the Footsteps of Senning: Lessons Learned From Atrial Repair of Transposition of the Great Arteries. Review Dodge-Khatami A, Kadner, A , Berger F, et al.Ann Thorac Surg 2005;79:1433-1444
- ^ Webb, Gary. "Transposition of the Great Arteries after Mustard/Senning Repair". Adult Congenital Heart Association. Retrieved February 24, 2017.
- ^ Surgery for congenital heart disease Long-term results of atrial correction for transposition of the great arteries: Comparison of Mustard and Senning operations. Helbing W, Hansen B, Ottenkamp I et al. J Thorac Cardiovasc Surg 1994;108:363-372
- ^ Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries. Dosl L, Teruell L, Ferreiral IJ.Heart 2005;91:652-656
- ^ Complete transposition of the great arteries. Kirklin JW, Barrat-Boyes BG. Cardiac Surgery. 2nd ed. New York: Churchill Livingstone; 1993. p. 1383–1467
Senning procedure
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Background
Transposition of the Great Arteries
D-transposition of the great arteries (d-TGA) is a cyanotic congenital heart defect characterized by ventriculoarterial discordance, in which the aorta arises entirely from the right ventricle and the pulmonary artery from the left ventricle, resulting in two parallel circulatory systems rather than the normal series circulation.[9] This anatomical abnormality prevents adequate mixing of oxygenated and deoxygenated blood, leading to systemic hypoxemia from birth.[10] d-TGA accounts for approximately 5-7% of all congenital heart defects, with an incidence of about 0.2 per 1,000 live births and a male predominance.[11] In d-TGA, the right ventricle pumps deoxygenated blood back to the body via the aorta, while the left ventricle recirculates oxygenated blood to the lungs via the pulmonary artery, creating life-threatening cyanosis unless adequate mixing occurs through associated shunts such as a patent foramen ovale (PFO), atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA).[9] The degree of mixing determines the severity of hypoxemia; in cases with an intact ventricular septum, reliance on atrial-level shunts like PFO is common, but these may restrict flow as the infant grows, exacerbating cyanosis.[12] Without intervention, the natural history is dismal, with mortality exceeding 50% by the first month of life, approximately 85% by 6 months, and nearly 90% by 1 year, primarily due to progressive heart failure and profound hypoxemia.[13] Due to these severe physiological consequences, d-TGA necessitates early surgical correction to restore physiological circulation. Associated features of d-TGA vary, but the ventricular septum is intact in about 60% of cases, classifying them as "simple" d-TGA.[14] Variations include VSDs, which occur in roughly 40% of cases and allow better mixing but complicate hemodynamics, and left ventricular outflow tract obstruction (LVOTO), seen in 5-20% of patients, often subvalvular and more frequent in those with VSD.[15] Coronary artery patterns are variable in d-TGA and must be assessed preoperatively, as certain configurations (occurring in 5-15% of cases) can complicate surgical correction.[16] Diagnosis of d-TGA is typically confirmed by echocardiography, which visualizes the transposed great vessels, assesses shunt adequacy, and identifies associated lesions.[9] Prenatal detection via fetal ultrasound is increasingly common, with rates up to 60-80% in recent series from regions with advanced screening programs as of 2024, enabling planned delivery at specialized centers.[17][18] Symptoms manifest shortly after birth and include central cyanosis, tachypnea, and poor feeding, with the severity correlating to the extent of mixing; infants with intact septa often present more acutely due to limited intercirculatory flow.[12]Rationale for Atrial Switch Operations
Atrial switch operations for dextro-transposition of the great arteries (d-TGA) aim to achieve physiological correction by redirecting blood flow at the atrial level, thereby establishing a functional series circulation without modifying the discordant ventriculo-arterial connections inherent to the defect. In d-TGA, systemic venous blood returns to the right atrium and is pumped by the right ventricle into the aorta, while pulmonary venous blood returns to the left atrium and is pumped by the left ventricle into the pulmonary artery, creating parallel, non-intersecting circulations that severely limit oxygenation. The atrial switch redirects deoxygenated systemic venous blood across the atria to the left ventricle and mitral valve for delivery to the lungs, while oxygenated pulmonary venous blood is directed to the right ventricle and tricuspid valve for systemic distribution, effectively swapping the downstream pathways of the ventricles to support survival.[19] These procedures represented a major advance over earlier palliative measures, such as the Rashkind balloon atrial septostomy introduced in 1966, which enlarged an existing or restrictive atrial septal defect to permit intercirculatory mixing but maintained parallel flows, resulting in persistent cyanosis and limited long-term viability. By contrast, atrial switch operations fully separated the systemic and pulmonary circulations, dramatically improving oxygenation and enabling most patients to survive infancy and into adulthood, with survival rates exceeding 80% in the initial postoperative years. This approach was particularly crucial in the pre-1970s era, when the arterial switch operation remained unfeasible due to formidable technical challenges in transferring the coronary arteries—essential for myocardial perfusion—from their native positions around the neoaorta, which contributed to early attempts' high mortality rates of 15-33%.[20][21] A defining physiological outcome of atrial switch operations is the reliance on the morphologic right ventricle as the systemic pump, directing blood to the aorta under high-pressure conditions for which it is not anatomically optimized. In the short term, this configuration supports adequate cardiac output, but over decades, the right ventricle experiences progressive strain from sustained systemic workload, leading to dilation, reduced contractility, and elevated risks of arrhythmias and heart failure, with dysfunction evident in a substantial proportion of patients over time. Longitudinal strain analyses reveal significantly impaired global longitudinal strain in the systemic right ventricle (approximately -12.9% versus -18.9% in normal left ventricles), underscoring the long-term hemodynamic burden.[22][23] The development of atrial switch techniques evolved from rudimentary palliation to sophisticated baffle creation, addressing the inadequacies of incomplete mixing strategies. Initial efforts in the 1950s, including surgical atrial septectomies, provided temporary relief but were supplanted by the Rashkind procedure's less invasive balloon dilation for emergent mixing. This paved the way for definitive atrial redirection, with Senning's 1959 innovation using autologous atrial flaps to form intra-atrial pathways and Mustard's 1964 synthetic baffle approach, both achieving complete physiological separation and transforming d-TGA from a largely fatal neonatal condition to one amenable to long-term management.[1][24]History
Early Palliative Interventions
In the mid-20th century, neonates with dextro-transposition of the great arteries (d-TGA) faced high mortality due to inadequate mixing of systemic and pulmonary venous blood, prompting the development of palliative interventions to improve oxygenation before definitive repairs became feasible.[19] The first such approach was the Blalock-Hanlon procedure, introduced in 1950 by surgeons Alfred Blalock and C. Rollins Hanlon at Johns Hopkins Hospital.[19] This closed-heart operation involved creating an atrial septal defect through a right thoracotomy using specialized clamps to excise a portion of the atrial septum, allowing interatrial shunting and partial mixing of oxygenated and deoxygenated blood.[19] Performed under hypothermia without cardiopulmonary bypass, it represented a significant advancement in the era of emerging congenital heart surgery, building on Blalock's prior work with the Blalock-Taussig shunt for tetralogy of Fallot.[19] Despite its palliative intent, the Blalock-Hanlon procedure had notable limitations, including incomplete atrial mixing that often resulted in persistent cyanosis, heart failure, and excessive pulmonary blood flow from a predominant right-to-left shunt.[25] These shortcomings were particularly evident in younger infants, where the procedure's efficacy diminished, leading to accelerated pulmonary vascular disease or inadequate systemic oxygenation.[19] Reported perioperative mortality for the Blalock-Hanlon operation varied widely, ranging from 22% to 57% across early series, reflecting the technical challenges and lack of supportive care in the 1950s.[26] Overall survival remained poor; without further intervention, approximately 50% of untreated d-TGA infants survived to one year, but palliative septectomy improved short-term prospects only modestly, underscoring the need for more effective strategies amid broader advancements in cardiac catheterization and extracorporeal circulation during the 1950s and 1960s.[27] A major refinement came in 1966 with the Rashkind balloon atrial septostomy, developed by cardiologist William J. Rashkind and associate William W. Miller at Children's Hospital of Philadelphia.[28] This minimally invasive, catheter-based technique enlarged an existing or restrictive atrial septal defect by inflating a balloon across the septum and forcefully withdrawing it to tear the tissue, thereby enhancing mixing without open surgery.[28] Performed via the femoral or umbilical vein under fluoroscopic guidance, it rapidly increased systemic oxygen saturation in cyanotic neonates, often stabilizing them for subsequent repairs and reducing the urgency for immediate surgical palliation.[28] While the Rashkind procedure offered short-term oxygenation improvements and lower procedural risks compared to surgical alternatives— with complication rates around 11% in initial cohorts—it was not curative and failed to address the underlying anatomical mismatch in d-TGA.[28] High long-term failure rates, including ongoing cyanosis and heart strain, contributed to overall palliative mortality exceeding 50% by one year in many early series, driving the pursuit of anatomical corrections like atrial switch operations in the late 1950s and 1960s.[28]Development and First Applications
The Senning procedure was invented by Åke Senning, a Swedish cardiac surgeon born in 1915 and deceased in 2000, during the 1950s while he was working at Karolinska Hospital in Stockholm under the mentorship of Clarence Crafoord.[29][3] Senning's development of the procedure was part of the broader advancements in open-heart surgery during that era, drawing inspiration from pioneering techniques such as those employed by C. Walton Lillehei in the United States, which emphasized direct visualization and repair inside the heart using early perfusion methods.[3] His work focused on creating a physiological correction for transposition of the great arteries (TGA) through atrial redirection, building on experimental animal models and the limited clinical successes of prior palliative approaches. Senning performed the first successful Senning procedure in 1957 on a patient with TGA at Karolinska Hospital, marking a significant milestone in the surgical management of this congenital defect.[19] This initial operation faced substantial challenges due to the rudimentary state of cardiopulmonary bypass technology in the mid-1950s, which provided only short durations of safe perfusion and carried high risks of hemolysis, clotting, and inadequate oxygenation.[30] Despite these limitations, Senning's approach demonstrated feasibility, with the patient surviving the procedure and achieving improved hemodynamics, though long-term follow-up was limited by the era's diagnostic constraints. A core innovation of the Senning procedure was its exclusive use of autologous atrial tissue to fashion intracardiac baffles, thereby redirecting systemic and pulmonary venous blood flows without relying on synthetic or prosthetic materials, which reduced the risks of thrombosis and infection associated with foreign implants.[31] This tissue-based technique allowed for better growth potential in pediatric patients and more natural conduit compliance compared to earlier baffle methods. Senning first detailed this innovation in a 1959 publication reporting his initial clinical experience, which included a small series of cases and highlighted the procedure's potential as a definitive repair.[2] Early adoption of the Senning procedure was confined to a handful of specialized centers equipped with advanced perfusion capabilities, such as those in Europe and North America, due to the technical demands and the need for precise atrial reconstruction under hypothermic bypass.[3] By the early 1960s, as improvements in cardiopulmonary bypass extended safe operative times and lowered perioperative risks, the procedure transitioned from an experimental intervention to a more standardized option for TGA correction, with reported survival rates in initial series exceeding 70% in selected patients.[32] This shift paved the way for wider application throughout the decade, influencing subsequent refinements like the Mustard procedure.Surgical Technique
Procedure Overview
The Senning procedure is an atrial-level switch operation that corrects dextro-transposition of the great arteries (d-TGA) through the creation of intra-atrial baffles fashioned exclusively from the patient's native atrial walls, thereby redirecting systemic venous return to the mitral valve and left ventricle while routing pulmonary venous return to the tricuspid valve and right ventricle.[12] This approach inverts the parallel circulations characteristic of d-TGA, achieving a biventricular physiological repair in which the morphological right ventricle functions as the systemic pump.[12] The technique involves intricate folding and suturing of atrial tissue to form the baffles without the need for prosthetic materials.[33] The procedure is primarily indicated for infants with d-TGA and an intact ventricular septum or a small ventricular septal defect, where arterial switch may not be feasible due to delayed presentation or other factors.[12] It was historically performed in infancy after the neonatal period.[34] Surgically, the operation is conducted under cardiopulmonary bypass with moderate hypothermia and cardioplegic arrest to achieve a still, bloodless field for precise tissue manipulation. The total procedure duration generally ranges from 3 to 5 hours, encompassing cannulation, baffle construction, and weaning from bypass, with no reliance on synthetic patches or grafts.[7]Intraoperative Steps
The Senning procedure is performed under general anesthesia with the patient positioned supine. A median sternotomy is made to access the heart, followed by opening the pericardium to the right of midline for optimal exposure. Cardiopulmonary bypass is established via aorto-bicaval cannulation, using purse-string sutures on the ascending aorta and angled cannulae in the superior vena cava (SVC) and inferior vena cava (IVC); mild hypothermia (typically 25–28°C) is induced to facilitate myocardial protection.[2][35] The aorta is cross-clamped, and antegrade cold cardioplegia is administered through the aortic root or coronary ostia to arrest the heart, with additional doses as needed during the procedure.[35] Atrial incisions are then created to access the interatrial septum and prepare tissue flaps for baffle construction. A vertical incision is made on the right atrial free wall, parallel to the sulcus terminalis or interatrial groove, extending from near the SVC to the IVC. An inverted L-shaped incision detaches the atrial septum from the limbus of the fossa ovalis, creating a septal flap while preserving the eustachian and Thebesian valves. On the left side, a stab incision is made in the left atrium at Waterston's groove, extended superiorly into the right superior pulmonary vein and inferiorly toward the left inferior pulmonary vein to outline the pulmonary venous confluence. Any patent foramen ovale or atrial septal defect (ASD) is inspected and temporarily closed or left open for venting.[2][36][35] Baffle creation redirects venous return: the systemic venous baffle routes SVC and IVC blood to the mitral valve (left ventricle), while the pulmonary venous baffle directs pulmonary venous return to the tricuspid valve (right ventricle). The septal flap is folded and sutured to the left atrial wall and pulmonary vein orifices using continuous 5-0 or 6-0 polypropylene sutures, forming the roof of the systemic venous pathway; superficial bites are taken near the coronary sinus to avoid atrioventricular node injury. The posterior right atrial wall is mobilized and sutured to the septum and atrial remnants to complete the floor of the systemic baffle, often augmented with a temporary stent (e.g., from a chest tube) in the IVC to ensure patency. The pulmonary venous chamber is reconstructed by attaching the superior right atrial flap to the ridge between the pulmonary veins and left atrial appendage, creating a C-shaped pathway; autologous pericardium may be used if additional tissue is needed for enlargement, particularly in small atria. Suture lines are positioned to avoid the sinoatrial node and phrenic nerve.[2][36][35] If a ventricular septal defect (VSD) is present, it is closed separately through a right ventriculotomy or via the tricuspid valve using a Dacron or pericardial patch with interrupted pledgeted sutures before baffle completion, ensuring alignment with the redirected pathways. Anomalous pulmonary venous drainage, such as total anomalous pulmonary venous connection, requires incorporation of the anomalous veins into the pulmonary venous baffle, often with pericardial augmentation or direct anastomosis to avoid obstruction. Intraoperative baffle leaks are assessed by filling the chambers with saline under pressure or using transesophageal echocardiography; leaks are repaired with additional sutures or patches. The ASD, if not used for venting, is closed primarily or with pericardium.[35][37] After baffle construction, caval snares are released, the heart is de-aired through the right atrial appendage and aortic vent, and pacing wires are placed on the right ventricle. The patient is rewarmed, and cardiopulmonary bypass is weaned gradually, typically with inotropic support such as low-dose dobutamine if needed. Hemostasis is confirmed, and the sternotomy is closed after protamine reversal of heparin.[2][35]Alternative Procedures
Mustard Procedure
The Mustard procedure, developed by Canadian surgeon William T. Mustard in 1963, is an atrial switch operation designed to correct dextro-transposition of the great arteries (D-TGA) by redirecting systemic and pulmonary venous blood flow at the atrial level.[38] In this technique, a baffle—typically fashioned from autologous pericardium or synthetic prosthetic material such as Dacron or expanded polytetrafluoroethylene—is constructed within the atria to route deoxygenated blood from the superior and inferior vena cavae to the left ventricle and pulmonary artery, while directing oxygenated blood from the pulmonary veins to the right ventricle and aorta.[19] This creates a physiological correction without altering the ventriculo-arterial connections, allowing the morphological right ventricle to function as the systemic pump.[39] Compared to the Senning procedure, the Mustard operation relies on prosthetic or pericardial material for baffle construction rather than the patient's native atrial walls and flaps, which simplifies the surgical technique and makes it more reproducible for surgeons.[19] However, this material dependence introduces specific risks, including a higher incidence of baffle obstruction due to thrombosis or tissue ingrowth, as well as leaks at baffle seams, potentially leading to cyanosis or right heart failure.[38] The Mustard approach also lacks the growth potential of native tissue used in the Senning, which can contribute to long-term conduit-related complications.[19] The procedure gained widespread adoption in the 1960s and 1970s as the preferred atrial switch for D-TGA, particularly in North America, with over 550 cases performed at Toronto's Hospital for Sick Children between 1963 and 1998.[38] Perioperative mortality was approximately 20% in early series, but long-term survival rates reached about 60% at 30 years, comparable to those of the Senning procedure.[6] Despite similar overall outcomes, Mustard patients often required reoperations for baffle obstructions or arrhythmias, with pacemaker implantation associated with increased mortality risk.[6] Today, the Mustard procedure has been largely supplanted by the arterial switch operation since the late 1980s, due to the latter's superior long-term hemodynamic results and avoidance of right ventricular systemic overload.[6] It is now reserved for rare complex cases, such as late-presenting infants with pulmonary vascular disease or when arterial switch is contraindicated.[38]Arterial Switch Operation
The arterial switch operation (ASO), also known as the Jatene procedure, is an open-heart surgical technique that provides anatomical correction for dextro-transposition of the great arteries (d-TGA) by transposing the aorta and pulmonary artery to their proper positions at the base of the heart. Developed by Brazilian cardiac surgeon Adib D. Jatene, the procedure was first successfully performed in 1975, involving the excision of the great vessels, their reconnection in a switched orientation, and the critical reimplantation of the coronary arteries from the original aortic root to the neoaorta (formerly the pulmonary root). This approach restores normal ventriculo-arterial concordance, allowing the left ventricle to function as the systemic pump and the right ventricle as the pulmonary pump.[19] Compared to atrial switch operations like the Senning procedure, the ASO offers significant advantages, including the preservation of the left ventricle's role as the systemic ventricle, which avoids the long-term strain on the right ventricle that often leads to dysfunction in atrial repairs. It also substantially reduces the risk of late arrhythmias, baffle obstructions, and the need for lifelong monitoring of atrial pathways. As a result, the ASO has become the standard treatment for d-TGA, typically performed in neonates within the first two weeks of life to prevent irreversible right ventricular hypertrophy and optimize outcomes.[40] Key technical challenges in the ASO include the precise transfer of the coronary artery buttons, which must be harvested as small circular patches and reimplanted into the neoaortic root without kinking or tension to ensure adequate myocardial perfusion. Another critical aspect is the Lecompte maneuver, which involves mobilizing the branch pulmonary arteries anterior to the neoaorta during reconstruction, allowing for tension-free anastomosis of the pulmonary trunk and avoiding compression by the aortic arch. These steps demand meticulous surgical expertise, particularly in neonates with variable coronary anatomy.[41] Following its initial description, the ASO gained widespread adoption in the 1980s after refinements by surgeons such as William G. Williams and Aldo R. Castaneda, leading to improved perioperative techniques and myocardial protection strategies. Today, it is the preferred procedure for simple and complex d-TGA, with hospital survival rates exceeding 95% in high-volume centers and excellent long-term transplant-free survival approaching 98% at 10 years.[42]Clinical Outcomes
Perioperative Mortality and Morbidity
The perioperative mortality for the Senning procedure has historically ranged from 10% to 20% during the 1960s to 1980s, reflecting the technical challenges of the era and limited experience with the operation.[43] Early studies reported higher rates, such as 20% 30-day mortality in cohorts undergoing surgery between 1973 and 1985, often due to associated cardiac anomalies and less refined surgical techniques.[44] In experienced centers today, perioperative mortality has improved to less than 5%, with hospital mortality rates around 5.3% in series from the 1990s to 2000s, attributed to advancements in perioperative care and patient selection.[45] Common perioperative morbidities include low cardiac output syndrome and severe heart failure, often occurring within the first two postoperative days and contributing to early deaths.[45] Baffle leaks necessitating reoperation occur in approximately 4% of cases, stemming from suture line imperfections in the atrial baffles.[31] Bleeding complications arise from the intricate suturing required for baffle construction, though specific incidence rates vary by center and are mitigated by meticulous hemostasis techniques.[45] Key risk predictors for perioperative adverse outcomes include associated anomalies, such as ventricular septal defects (VSDs), which significantly elevate 30-day mortality risk in complex transposition cases requiring concomitant VSD closure.[44][43] Recent single-center analyses, including a 2025 review of Mustard repairs (a related atrial switch variant) performed in the late 1970s with follow-up into the fifth decade, report early mortality rates of approximately 7%, with improvements linked to earlier surgical timing in infancy to preserve ventricular function.[46] These findings underscore the benefits of neonatal or early infant intervention in reducing short-term risks, though the Senning procedure is now rarely performed in favor of arterial switch operations.[46]Long-Term Health Effects
The Senning procedure, an atrial switch operation for transposition of the great arteries, yields favorable long-term survival in early adulthood, though rates decline progressively due to systemic right ventricular failure. A nationwide multicenter study in Belgium reported actuarial survival rates of 91.7%, 88.6%, and 79.3% at 10, 20, and 30 years post-surgery among early survivors, with the Senning cohort demonstrating superior outcomes compared to the Mustard procedure. A Dutch cohort study similarly documented 82% survival at a median of 39.7 years after atrial correction. More recent analyses indicate a continued downward trajectory, with cumulative survival reaching 61% at 48 years in comparable atrial switch cohorts, primarily attributable to right ventricular dysfunction.[47][48][46] Major long-term complications encompass arrhythmias, systemic right ventricular dysfunction, baffle obstructions, and heart failure. Arrhythmias are prevalent, with arrhythmia-free survival at 64.1% and 45.4% at 10 and 20 years post-Senning repair, and approximately 30% of patients requiring pacemaker implantation by adulthood due to sinus node dysfunction or atrioventricular block. Systemic right ventricular dysfunction develops increasingly over time, contributing to progressive heart failure that affects 20-30% of patients by age 40 and represents a leading cause of late mortality. Baffle obstructions, including superior or inferior vena cava stenoses and pulmonary venous pathway issues, necessitate reoperation in 9-30% of cases, often within the first two decades.[47][49][50][51] Research from 2022 to 2025 has advanced risk stratification, including a simplified prediction score in a large adult cohort after atrial switch operations that identifies 5-year risks of end-stage heart failure or death based on factors like tricuspid regurgitation and ventricular function. As of 2025, guidelines from major societies emphasize lifelong monitoring for arrhythmias and systemic right ventricular function in adult survivors. Senning patients exhibit higher atrial strains compared to those after Mustard repair, potentially linked to greater atrial remodeling. Transplant candidacy is rising as surviving cohorts age, with reported cases of successful heart transplantation decades post-procedure amid worsening right ventricular failure.[52][53] Despite these challenges, many patients achieve a reasonable quality of life, engaging in active lifestyles with appropriate management, though exercise intolerance is common due to reduced cardiac output and right ventricular limitations. The risk of sudden cardiac death persists at 1-2% per year in adulthood, often during exertion and accounting for up to 45% of late deaths, underscoring the need for ongoing arrhythmia surveillance.[54][55]Support and Resources
Patient Support Networks
Patients who have undergone the Senning procedure, along with their families, can access dedicated peer support through online communities such as the Mustard & Senning Survivors group on Facebook, founded in 2009 and recognized as the world's largest gathering of adult survivors from these atrial switch operations.[56] This private group fosters connections among thousands of members worldwide, enabling them to exchange practical advice and emotional encouragement tailored to life after surgery.[57] The Adult Congenital Heart Association (ACHA) provides essential resources for Senning procedure patients transitioning to adulthood, including webinars and educational Q&As that cover managing long-term health aspects like arrhythmias and baffle obstructions, as well as pregnancy risks and the shift to specialized adult care.[39][58] These offerings emphasize peer-to-peer interaction and expert-led discussions, helping survivors navigate emotional challenges and build resilience.[59] Internationally, networks like CHD-UK support congenital heart disease patients, including those post-Senning, through counseling services, online forums, and events that promote community building and information sharing.[60] Participation in these groups has seen heightened engagement since 2020, driven by the pivot to virtual platforms amid the COVID-19 pandemic, which expanded access for remote participants.[61]Follow-Up and Management Guidelines
Patients who have undergone the Senning procedure for d-transposition of the great arteries require lifelong surveillance by specialists in adult congenital heart disease (ACHD) to monitor for complications such as arrhythmias, systemic right ventricular dysfunction, and baffle-related issues.[62] According to the 2018 AHA/ACC Guidelines for the Management of Adults With Congenital Heart Disease, routine follow-up includes annual evaluations by an ACHD cardiologist, encompassing a detailed history, physical examination, and targeted testing to assess arrhythmia risk, ventricular function, and baffle patency.[62] These guidelines emphasize early detection of arrhythmias, which affect up to 50% of patients, often involving intra-atrial reentrant tachycardia or sinus node dysfunction, through serial electrocardiograms (ECGs) and ambulatory monitoring.[62] Monitoring protocols are tailored to the patient's Adult Congenital Heart Disease Anatomical and Physiological (ACHD AP) classification, with more frequent assessments for those with higher complexity (ACHD AP III or IV). The following table summarizes key recommendations from the 2018 AHA/ACC guidelines:| Test/Procedure | Frequency for Lower Complexity Patients (ACHD AP I/II) | Frequency for Higher Complexity Patients (ACHD AP III/IV) | Purpose (Class/Level of Evidence) |
|---|---|---|---|
| Outpatient ACHD Cardiology Visit | Every 12 months | Every 6-12 months | Comprehensive evaluation (I/C-EO) |
| 12-Lead ECG | Every 12 months | Every 6-12 months | Arrhythmia screening (I/C-EO) |
| Transthoracic Echocardiography (TTE) | Every 12-24 months | Every 12 months | Ventricular function, baffle leaks (I/C-EO) |
| Holter Monitor | Every 24 months | Every 12 months | Arrhythmia detection (I/C-EO) |
| Cardiac Magnetic Resonance (CMR) | Every 24-36 months | Every 12-24 months | Baffle assessment, RV function (I/B-NR) |