Congenital: Aortic Valve
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.
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.
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.
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”.
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.
Commentary: Innovation in pediatrics: Aortic valve ringIt 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 aneurysmA 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).
Commentary: A tale of the left ventricleThe 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.
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.1
Aortic 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.
Commentary: My best was never good enough, Bruce SpringsteenJegatheeswaran 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.
Commentary: Moving beyond survival: The long-term psychosocial impact of congenital heart diseaseWhat 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).
Pediatric valve-sparing aortic root replacement: Approaching perfectionOver 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 replacementWith 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 repairIn 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.