Congenital: Aortic Valve
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.
