Congenital: Aortic Valve
Characterizing the anatomic spectrum, surgical treatment, and long-term clinical outcomes for patients with Shone's syndromeShone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival.
Valve-sparing root replacement after the Ross procedureThe 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 limitsThe 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 childrenWe 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 stenosisThe 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 dilemmaDifficult 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 patchWe 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 childrenThe 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 resultsAortic 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 closerA 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 leprechaunsStackhouse 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 palliationIn 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 fitAortic 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 adultsThe 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 presentationAlthough 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 essenceThe 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 atresiaThe 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 rootThe 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 studyPulmonary 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 educationQuadricuspid 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.