Congenital: Aortic Valve
Valve-sparing root replacement after the Ross procedure
The Ross procedure is a preferred treatment for infants and children with aortic valve disease. Progressive neoaortic root dilation and neoaortic insufficiency can occur after the Ross procedure, and because of the young age of these patients, valve-sparing aortic root replacement procedures have advantages compared with the Bentall procedure. The aim of this study is to describe our experience with different techniques of aortic valve-sparing root replacement in this unique cohort of patients.Ross procedure in neonates and infants: A valuable operation with defined limits
The Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome.Long-term outcomes of primary aortic valve repair for isolated congenital aortic stenosis in children
We aimed to assess the long-term outcomes of children with isolated congenital aortic stenosis who underwent primary aortic valve repair.Outcomes of aortic valve repair in children resulting in bicuspid anatomy: Is there a need for tricuspidization?
We aimed to assess outcomes after aortic valve repair leading to bicuspid valve anatomy in children.The Ross/Ross-Konno procedure in infancy is a safe and durable solution for aortic stenosis
The objective of this study was to characterize early and midterm outcomes after the Ross/Ross-Konno procedure performed in infancy for severe aortic valve disease.Ross procedure or complex aortic valve repair using pericardium in children: A real dilemma
Difficult to repair aortic valve lesions, requiring the use of a valve substitute, remain controversial in the face of the Ross procedure, despite undeniable technical advances. This study was undertaken to compare midterm outcomes of children treated using the Ross procedure or aortic valvuloplasty for complex aortic valve lesions.Aortic valve repair in children without use of a patch
We aimed to assess the long-term outcomes of children in whom the aortic valve could be repaired without the use of patch material. We hypothesized that if the aortic valve is of sufficiently good quality to perform repair without patches, a durable repair could be achieved.Aortic valve surgery in children
The ideal aortic valve surgery in children must result in an unobstructed, competent aortic valve that is also durable and accommodates growth of the child. Intuitively, aortic valve surgery in children must emulate native aortic valve anatomy and physiology as close as possible, preferably using autologous tissues with growth potential. The optimal approach to aortic valve surgery in children is yet to be defined. The purpose of this focused review is to highlight recent key publications on the topic.Congenital aortic and truncal valve reconstruction using the Ozaki technique: Short-term clinical results
Aortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease.Commentary: Continue to imitate the nature: We are getting closer
A sustained long-term treatment strategy for the treatment of aortic valve disease in the pediatric population is inevitable. Thus, this strategy should involve the entire current armamentarium of available treatment options that need to be applied individually in each and every case. The requirements for the ideal valve substitute are well known, whereas such a valve is still not available.1 A myriad of factors influence the decision-making in this population that starts with the anatomy of the valve, concomitant cardiac lesions, previous interventions, patient needs and choice, and particularly the expertise of the surgical team.Commentary: Unicorns and leprechauns
Stackhouse and colleagues1 report on the cohort of patients with aortic atresia (read: hypoplastic left heart syndrome [HLHS]) enrolled from 1994 to 2000 in the Congenital Heart Surgeons' Society study comparing outcomes of different initial management strategies. The focus of this manuscript is on the 2 most common initial interventions—surgical palliation and listing for primary cardiac transplantation. The authors discovered that survival with primary transplantation, including waitlist mortality, was superior to surgical palliation.Commentary: No justification for transplantation in place of standard surgical palliation
In their review of CHSS data from 1994 to 2000, Stackhouse and colleagues1 make clear their point that a strategy of surgical palliation for treatment of aortic atresia during an earlier generation carried a greater early risk of death than primary transplantation. This observation led them to conclude that it may be reasonable to encourage full utilization of primary pediatric transplantation, including extension to more aggressive use of primary transplantation in patients with aortic atresia today.Commentary: Aortic valve reconstruction and the Ozaki procedure in children—Finding the best fit
Aortic valve disease continues to be a challenging problem in pediatric patients. We continuously search for new innovations, as our best options are imperfect. In this issue of the Journal, Wiggins and colleagues1 from Great Ormond Street present their short-term results using the Ozaki procedure (neotricuspidization) and other valve reconstruction techniques in children and young adults. This is currently the largest retrospective single-center review in children and young adults, comprising 58 patients from 2015 to 2019.The utility of aortic valve leaflet reconstruction techniques in children and young adults
The treatment of aortic valve disease in children and adolescents requires an individualized approach to provide a long-term solution with optimal hemodynamic profile. The role of aortic leaflet reconstruction techniques is evolving.Surgical outcomes for anomalous left coronary artery from the pulmonary artery: Influence of late presentation
Although surgical outcomes for anomalous left coronary artery from the pulmonary artery (ALCAPA) are excellent in the modern era with the coronary reimplantantion technique, mortality remains high in Chinese population. This study was undertaken to review the surgical management for ALCAPA in our center and assess the midterm outcomes.Commentary: Are pediatric aortic leaflet reconstructions a long run for a short slide or a home run?
In this issue of the Journal, Wiggins and colleagues1 present their work studying the utility of aortic valve leaflet reconstruction in children and young adults with aortic valve disease. Using data from a high-volume, single center, the authors studied 58 patients over a 4-year period who underwent either an aortic valve neo-tricuspidization procedure (Ozaki) or a single-leaflet reconstruction. The authors found that these 2 techniques can be performed safely with acceptable short-term hemodynamics in a population of patients that remains a significant clinical challenge.Commentary: Why the difference?
The surgeons from Shanghai Children's Medical Center have reported their outcomes in infants and children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA).1 Their series of 105 patients in the past 10 years had an overall mortality rate of 14%. The most recent Society of Thoracic Surgeons Congenital Heart Surgery database report has a total of 135 patients operated on for this diagnosis between 2015 and 2018.2 In that series, there was no mortality! That is obviously quite a large difference in outcomes.Commentary: Anomalous left coronary artery from the pulmonary artery—Time is of the essence
The cardiac surgeon learns early in her or his career that any type of coronary artery problem—congenital, acquired, or iatrogenic injury—can result in an unrecoverable situation in the operating room. Anomalous left coronary artery from the pulmonary artery (ALCAPA) is unique in that significant systolic dysfunction at the time of presentation is often present and is not responsive to conventional resuscitative maneuvers. Early surgery with establishment of a dual coronary system involving reimplantation of the left coronary artery into the aorta is the procedure of choice.Surgical palliation or primary transplantation for aortic valve atresia
The study objective was to describe the surgical pathway progression through adolescence of an inception cohort of neonates with aortic valve atresia managed initially with surgical palliation or primary transplantation, comparing survival and self-reported health-related quality of life.Commentary: Toward a biologic understanding of autograft dilatation in the Ross procedure—creating opportunities to rescue the neoaortic root
The aortic root has a sophisticated architecture that results in hemodynamic performance and longevity beyond any prosthetic replacement.2 The seminal step by Mr Ross to enlist the aortic valve's living twin as its replacement has produced outstanding results at experienced centers.3,4 Several considerations have hampered the widespread adoption of the Ross procedure, one of which has been autograft dilatation, with consequent valvular failure.4,5 A focused effort to understand the clinical drivers of this process has reduced its incidence and highlighted its independent predictors.Commentary: Prediction of pulmonary root dilatation after Ross operation—The power of protein shake?
An explosive development of global molecular assessment technology during the recent decades has resulted in the multitude of “-omic” approaches awaiting to be translated into surgical practice. To most surgeons, however, genomics, proteomics, metabolomics, comics, and other “-omics” are of very similar predictive and entertaining value. Namely, often the “-omics” do not predict anything and are somewhat mildly entertaining, at least as far as the surgical outcomes are concerned. This is the current state of affair in surgical “-omics”.Maladaptive remodeling of pulmonary artery root autografts after Ross procedure: A proteomic study
Pulmonary autograft root dilatation is the major long-term complication after Ross procedure and the leading cause for reoperation. However, the mechanisms underlying dilatation remain to be elucidated. This study analyzed the proteomic changes seen in the dilated pulmonary autograft compared with normal pulmonary artery and aorta tissues.Commentary: Aortic valvuloplasty in children…if you like it then you should've put a ring on it!
In this month's edition of The Journal of Thoracic and Cardiovascular Surgery, Wolsky and colleagues present, to our knowledge, the first case report showcasing the use of a geometric ring annuloplasty to repair a dysfunctional bicuspid aortic valve in a child.1 The treatment of complex aortic valve disease in children remains a controversial topic and is muddied by numerous and disparate treatment modalities; multiple techniques of leaflet repair, neocuspidization (Ozaki technique) and valve replacement (mechanical, bioprosthetic, pulmonary autograft [Ross procedure]).Commentary: What can be learned from a case report, or the importance of a well-rounded education
Quadricuspid aortic valves (QAVs) are very rare but have been well described since the early 1970s.1 Even less recognized but documented is the association of QAVs with a potential congenital structural aortopathy. In the largest series of QAVs to date, 42% of patients had ascending aortic diameters greater than or equal to 4 cm.2 Most patients with QAVs present with aortic insufficiency, aortic stenosis, or a combination of these. In this issue of the Journal, Xu and associates3 report on a 35-year-old woman who presented with a QAV associated with a ruptured sinus of Valsalva aneurysm.Commentary: Innovation in pediatrics: Aortic valve ring
It is rare in the current era to review an operation that is truly a novel procedure for pediatric patients. The case report from Wolsky and colleagues1 is to my knowledge the first case report of a pediatric patient with bicuspid aortic valve and severe aortic valve insufficiency having a quite successful internal aortic ring annuloplasty. The bicuspid annuloplasty ring has been previously reported in adult patients but it appears that it can also be used in appropriately selected pediatric patients.Surgical repair of a quadricuspid aortic valve associated with a ruptured sinus of Valsalva aneurysm
A 35-year-old woman presented with a history of gradually worsening dyspnea on exertion. Clinical examination revealed a diastolic murmur heard loudest along the upper left sternal border, which did not radiate to the carotids or the precordium. Transthoracic echocardiography demonstrated a quadricuspid aortic valve (type A, Hurwitz and Robert's classification) with an aorta-to-right atrium fistula (sinus 2) through a ruptured right-anterior sinus of Valsalva (Figure 1, A). In addition, the coronary orifices originated from sinus 1 (right coronary artery) and sinus 4 (left main coronary artery), and the aortic regurgitation was moderate (Figure 1, A and B).Geometric ring annuloplasty for bicuspid aortic valve repair in a child
Ring annuloplasty has been used routinely for surgical repair of tricuspid and mitral valves for more than 35 years, and more recently, a similar ring has been developed for bicuspid aortic valve (BAV) repair.1 Annuloplasty rings restore normal size and geometry of the valve annulus, bring the native leaflets into better coaptation, and prevent future annular dilatation. In this article, the first case of a pediatric patient with a BAV and severe aortic insufficiency (AI) managed with internal ring annuloplasty is presented.Commentary: A tale of the left ventricle
The historical outcomes of the Ross or Ross-Konno operation in centers of excellence remain outstanding, and it remains the criterion standard surgical repair for infants, children, and adolescents with aortic stenosis or regurgitation or with left ventricular outflow tract obstruction.1-3 Despite the inherent advantages of the Ross or Ross-Konno operation, including the potential for somatic growth, most long-term outcome studies have centered on the deterioration of the aortic autograft or pulmonary homograft.Commentary: “Daddy, is patient-prosthesis mismatch real?” “Yes, Billy, it lives under your bed with the other monsters”
Aortic valve replacement with or without aortic root replacement, especially in the reoperative setting, has previously generated an endless series of questions involving what type of valve should be implanted (mechanical, bioprosthetic (stented or stentless), autograft, homograft, Osaki, transcatheter valve, etc, etc) and what size valve is appropriate. This discussion was somewhat simpler in the past, when bioprosthetic valves were primarily reserved for “old folks” because of concerns regarding multiple reoperations for bioprosthetic valve degeneration implanted in younger patients.Commentary: Who doesn't have abnormal myocardial strain?
During the past decade, systolic myocardial strain (deformation) has become a popular way to detect early left and right ventricular systolic dysfunction. Myocardial strain (with speckle-tracking echocardiography or, more recently, feature-tracking cardiac magnetic resonance imaging) can be measured locoregionally to produce a “map” of systolic activity that reproduces the complex way in which the ventricle contracts. Deviations from the “normal” pattern reflect inefficient pump action and, ipso facto, ventricular systolic dysfunction.The Konno aortoventriculoplasty after the Bentall procedure
The Bentall procedure1 is rarely performed in pediatric patients, with exceptions such as connective tissue disorder. We report a young adult patient presenting prosthesis–patient mismatch after the Bentall procedure.Ventricular interactions and electromechanical dyssynchrony after Ross and Ross-Konno operations
Ross and Ross-Konno operations are associated with the inherent risk of residual ventricular septal dysfunction and injury to the conduction system. However, comprehensive biventricular functional outcomes on magnetic resonance imaging after Ross and Ross-Konno procedures are unknown. The purpose of this study was to evaluate and compare the degree of electrical and mechanical dyssynchrony using cardiac magnetic resonance imaging in patients late after Ross and Ross-Konno operations.Aortic valve neocuspidization: A bright future in pediatric aortic valve surgery?
We congratulate Ozaki and colleagues1 on their study evaluating midterm results after aortic valve neocuspidization (AVNeo) with glutaraldehyde-treated autologous pericardium. In their cohort of 850 adult patients with various aortic valve diseases who underwent an aortic valve replacement during a period of 8 years, excellent results were reported in terms of actuarial freedom from death, cumulative incidence of reoperation, and recurrent moderate or greater aortic regurgitation.1Aortic valve neocuspidization: Oversized shoes for children?
For adult patients, aortic valve neocuspidization (AVNeo) with glutaraldehyde-treated autologous pericardium is a very attractive option, with neither any limitation in annulus size nor any need for anticoagulation and with favorable midterm results.1 These features are enough to draw the attention of pediatric cardiac surgeons as well. In their letter, Kalfa and colleagues2 mentioned their preliminary pediatric AVNeo cases, which had been performed on children for whom the Ross procedure was unsuitable.Commentary: Midaortic syndrome—What is the skinny?
Midaortic syndrome (MAS) is characterized by narrowing of the descending aorta between the distal aortic arch and the aortic bifurcation. The narrowing may be focal and discrete, or it may be more diffuse along the course of the descending aorta (Figure 1). Not infrequently, the disease process may also extend into visceral arterial branches, such as the celiac, mesenteric, or renal vessels. Most cases of MAS are idiopathic, and the underlying pathogenesis remains poorly understood. In select instances, a link has been established with such conditions as inflammatory arteritis, mucopolysaccharidosis, fibromuscular dysplasia, and Williams syndrome.Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons' Society Study
Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents and young adults with repaired interrupted aortic arch.Commentary: My best was never good enough, Bruce Springsteen
Jegatheeswaran and colleagues1 have provided an update on outcomes for patients in the Congenital Heart Surgeons' Society registry for interrupted aortic arch (IAA) surgery repairs done from 1987 to 1997. I applaud the focus on functional health status rather than durability of aortic repair or other surgical outcomes. Specifically, surgical outcomes have been reported,2,3 but less is available on overall health status. In an era of increasing surgical outcome transparency, it is equally important that we provide parents with a realistic sense of how life will likely be for a fetus with diagnosed with congenital heart disease.Valve-sparing aortic root replacement in children: Outcomes from 100 consecutive cases
Valve-sparing root replacement is an attractive alternative to composite mechanical or biologic prostheses for aortic root aneurysms in children. Data on outcomes in pediatric patients are limited. We present our institutional experience with 100 consecutive pediatric valve-sparing aortic root procedures.Commentary: Moving beyond survival: The long-term psychosocial impact of congenital heart disease
What is health, and how should we measure it? These seemingly innocuous questions have significant implications for the direction of our field. Health implies more than perioperative survival or even long-term optimization of cardiac care. Health is a multifactorial and multidimensional construct that is related to the holistic well-being of an individual. “Medical” health only represents a portion of the overall well-being of an individual, with the health of the family being closely interrelated with the personal health of the individual (Figure 1).Successful staged management of neonatal midaortic syndrome
Midaortic syndrome (MAS) is narrowing of the descending thoracoabdominal aorta. Symptomatic MAS is associated with high mortality in neonates and infants.1 We describe the case of a 4-month-old infant with MAS and illustrate a staged, multidisciplinary, and multimodality approach.Selection of prosthetic aortic valve and root replacement in patients younger than age 30 years
Long-term outcomes of prosthetic aortic valve/root replacement in patients aged 30 years or younger are not well understood. We report our single institutional experience in this young cohort.Pediatric valve-sparing aortic root replacement: Approaching perfection
Over the past 3 decades, aortic root replacement surgery has evolved with much attention given to preserving the aortic valve when possible. Valve-sparing operations including the reimplantation and remodeling procedures, along with modifications, have gained support with the techniques being expanded to treat aneurysmal aortic disease in children. The benefits of conserving the valve are clear: avoidance of complications associated with anticoagulation therapy. Concerns over the development of aortic valve insufficiency or a pseudoaneurysm necessitating further surgery have been the primary arguments against these operations.Continued pursuit of evidence-based indications and the optimal operation for pediatric valve-sparing aortic root replacement
With this sentinel contribution, the Johns Hopkins group has again updated the preeminent experience with valve-sparing aortic root replacement in children.1 As acknowledged in the report, the establishment of data-driven, evidence-based surgical indications remains elusive. Because of their history of leadership in the field, many will look to these authors for guidance regarding the criteria for operative intervention. In this report, they have presented their thresholds for surgery according to size, growth rate, and disease type.Coarctation, aneurysm, carotid agenesis, oh my! Complex arch anatomy requires thoughtful operative planning for safe repair
In the current issue of the Journal, Rajagopalan and colleagues1 present an interesting case report that doubles as an educational brain teaser on the surgical approach to complex arch pathology. The patient is an adult woman who presented with a symptomatic saccular arch aneurysm associated with near interruption of the transverse arch and an aberrant right subclavian artery, reminiscent of the congenital anomaly of interrupted aortic arch type B2 (Figure 1). The key factor that further confounded the operative approach was the presence of a single right carotid artery, with presumably atresia or agenesis of the left carotid artery.Aortic stenosis of the neonate: A single-center experience
Because data for neonates are limited, optimal management of critical aortic stenosis remains controversial (balloon valvotomy [BV] or open valvoplasty [OV]). In a center with balanced experience in both methods, we hypothesized that OV can provide a better individualized approach than blunt BV and better serve long-term outcomes.Leaflet-base–preserving truncal valve repair with ethanol-treated autologous pericardium
Persistent truncus arteriosus requiring truncal valve surgery is associated with poor prognosis.1 Valve replacement should be avoided for neonates or young infants; however, truncal valve repair (TrVR) sparing native leaflets has limitations because leaflet tissues are often dysplastic and very fragile. Glutaraldehyde-treated pericardium, which is commonly used in adult valve surgery, has unresolved problems in terms of calcification and toxicity, especially in children. We report a novel TrVR in which we used ethanol-treated pericardium.Coarctation of aorta aneurysm with aberrant right subclavian artery and single carotid artery: Surgical and perfusion strategies
Coarctation of aorta aneurysm with aberrant right subclavian artery and single carotid artery is a rare and complex congenital aortic anomaly. We present the surgical and perfusion strategy that we used to maintain cerebral and spinal cord perfusion during repair, thereby preventing devastating complications of stroke and paraplegia. Written patient consent was obtained for this case report.Biventricular root replacement for transcatheter pulmonary valve endocarditis after Ross operation: Double the trouble
Use of percutaneous pulmonary valve replacement (PPVR) has steadily increased during the last decade, even though increased risk of endocarditis continues to be a concern.1,2 The close proximity of the right ventricle–pulmonary artery (RV-PA) conduit to the dilated neoaorta after the Ross operation poses unique challenges for PPVR. Stent fractures, accelerated progression of neoaortic regurgitation, aortopulmonary fistula, and coronary compression are well-documented complications of PPRV after the Ross operation.Outcomes after mechanical aortic valve replacement in children and young adults with congenital heart disease
There are little recent data on the outcomes of mechanical aortic valve replacement (AVR) in children and young adults with congenital aortic valve disease. We sought to review the survival and associated thromboembolic or bleeding complications after mechanical AVR at a single center.Newer advances, newer challenges?
The ability to percutaneously implant cardiac valves represents a major step forward in the care of patients with heart disease. Transcatheter aortic valves were initially implanted in adults with prohibitive risk for surgical valve replacement. In children, transcatheter pulmonary valve replacement (TPVR) has been shown to be a low-risk procedure with high technical success rates and a favorable outcome profile in the intermediate term.1 The Melody (Medtronic, Minneapolis, Minn) valve system composed of a bovine jugular venous valve within a platinum-iridium stent frame is the most widely used transcatheter pulmonary valve.
