Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.

OBJECTIVE: Symmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. METHODS: We performed a retrospective review of patients who underwent the symmetric bicuspidizing repair at Boston Children's Hospital between December 2019 and June 2022 with a contemporary comparator group of patients who underwent other forms of bicuspidization. Survival, valve-related reoperation, and the development of moderate or greater aortic or truncal valve regurgitation were assessed. RESULTS: There were 23 patients who underwent symmetric bicuspidizing repair and 18 who underwent another form of bicuspidization. Preoperative aortic regurgitation was present in 87.0%. Patients who underwent symmetric bicuspidizing repair more commonly underwent suture annuloplasty (100% vs 55.6%; P = .002) and ascending aortoplasty (78.3% vs 27.8%; P = .004). There was 1 operative mortality (2.4%) in the entire cohort and 1 late mortality. Freedom from moderate aortic regurgitation was 87.5% at 21 months after symmetric bicuspidizing repair compared with 43.5% for patients who underwent other types of bicuspidization; P = .03. Freedom from valve-related reoperation was 100% in the symmetric bicuspidizing repair group compared with 64.4%; P = .02. CONCLUSIONS: The symmetric bicuspidizing repair may be safely extended to patients with various forms of congenital aortic and truncal valve disease. Longer term follow-up will be necessary to determine the comparative effectiveness of this technique compared with neocuspidization and the Ross procedure.

Rescue ross procedure and mitral valve repair on a low birth weight preterm neonate.

Although mid- and long-term outcomes after the Ross procedure for aortic valve disease have been increasingly improving over the years, this is still a rather challenging operation in neonates and small children. This is particularly true for patients with associated congenital heart defects and critical clinical conditions. Herein we describe the application of this procedure as a rescue operation in emergency circumstances in a low-birth-weight neonate with severe aortic stenosis, aortic regurgitation and mitral regurgitation after a previous aortic coartectomy.

Congenital unicuspid aortic valve repair without cusp patch augmentation.

Unicuspid aortic valves are rare congenital malformations. Surgical repair is feasible in aortic regurgitation, and in some cases of aortic stenosis. The standard surgical approach is a bicuspidization and symmetrization with pericardial patch augmentation of valve leaflets. Herein, we are describing our original technique for bicuspidization of a unicuspid aortic valve in adults without leaflet patch augmentation. We also address the surgical management of a commissural diastasis.

Congenitally Abnormal Aortic Valve Causing Coronary Obstruction and Cardiac Arrest in Infancy.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

Long-Term Fate of the Neoaortic Root After Neonatal Ross Operation: A Case Series.

The aim of this study is to analyze the adaptation properties of the pulmonary autograft in four infants who underwent the Ross operation before one year of life. The patients underwent serial echocardiographic assessments of the autograft diameters at short- and long-term follow-up and values were reported as the Z scores for normal aortic and pulmonary diameters. At a median follow-up time of 18.5 years (range: 18.2-19.4 years), all the patients are alive, none requiring autograft reinterventions. This series shows excellent adaptation potential of the "infant pulmonary autograph" in the long-term, during somatic growth of the patient.

[Infective Endocarditis in Right Ventricle( RV)-Pulmonary Artery( PA) Conduit Late after the Ross Procedure;Report of a Case].

Ross procedure has been found to have a lower incidence of infective endocarditis compared to other aortic replacement procedure using prosthetic valves. We report a case of 25-year-old man who underwent Ross procedure for congenital aortic stenosis and regurgitation when he was 7 years old. He presented with fever and was highly suspected of infective endocarditis. All sets of blood cultures were positive for Heamophilus parainfluenzae. Autologous pericardial pulmonary valve was severely stenotic and computed tomography (CT) scan and radio isotope (RI) scan revealed infection at the stenotic valve. We performed right ventricle (RV)-pulmonary artery (PA) conduit replacement and he was discharged after completion of intravenous antibiotic treatment. We experienced a rare case of infective endocarditis in a patient late after Ross procedure. Prophylaxis against infective endocarditis is mandatory even in patients with infection resistant Ross procedure.

Late results of the Ross procedure.

OBJECTIVE: The study objective was to examine the long-term results of the Ross procedure in a cohort of patients followed prospectively for more than 2 decades. METHODS: From 1990 to 2004, 212 consecutive patients with a median age (interquartile range) of 34 years (28-41) underwent the Ross procedure; 82% had congenital aortic valve disease. The technique of aortic root replacement was used in one half of the patients. Patients have been followed prospectively for a median (interquartile range) of 18.0 (14.6-21.2) years. Valve function was assessed by echocardiography. RESULTS: Cumulative mortality at 20 years was 10.8% (95% confidence interval, 6.5-17.8). Thirty patients required Ross-related reoperations and 3 for coronary artery disease. The cumulative probability of Ross-related reoperations at 20 years was 16.8% (95% confidence interval, 11.3-24.5), on the pulmonary autograft was 11.5% (95% confidence interval, 7.2-18.0), and on the pulmonary homograft was 8.2% (4.6-14.7). The implantation technique was not associated with the cumulative incidence of reoperations on the pulmonary autograft. The development of moderate or severe aortic insufficiency and pulmonary homograft dysfunction increased with time. At 20 years, the probability of aortic insufficiency was 13% (95% confidence interval, 8.0-20.3) and of pulmonary homograft dysfunction was 19.7% (95% confidence interval, 13.9-27.2). Preoperative aortic insufficiency was associated with increased odds of postoperative aortic insufficiency. CONCLUSIONS: The long-term results of the Ross procedure are excellent regardless of the implantation technique, but there is a progressive deterioration of function of both semilunar valves.

Double semilunar valve replacement in complex congenital heart disease using decellularized homografts.

OBJECTIVES: Patients with complex congenital heart disease often require multiple reoperations, resulting in increased rates of operative morbidity and mortality. Decellularized heart valves (DHVs) have led to reduced reoperation rates compared with current other valve substitutes when used for pulmonary valve replacement and have also shown very auspicious early results in aortic valve replacement. The aim of the work was to analyse the outcome of a single-stage decellularized valve implantation in the aortic and pulmonary position. METHODS: A prospective follow-up of all patients who received a single-stage double semilunar valve replacement using DHV at our institution. RESULTS: Since 2011, 5 patients underwent combined semilunar valve replacement with DHV at our institution: two following a Ross procedure (31-year-old man and 38-year-old woman) and 3 after repair of the truncus arteriosus communis (2-year-old boy and 11-year-old and 16-year-old girls). All patients had undergone previous surgery. The Ross patients had preceding valve procedures, and the patients with truncus arteriosus communis had undergone 1 repair and subsequent operative procedures. Despite challenging operations (median bypass time 346 min, range 275-477 min; median cross-clamp time 229 min, range 140-307 min), there was no perioperative mortality or reoperations. Four of the patients were extubated within 24 h, and the other patient was extubated on postoperative day 2. During follow-up, a good semilunar valve and biventricular heart function was present in all 5 patients, and the New York Heart Association functional class was I for all the patients at the time of their latest follow-up (median 31 months, range 8-82 months). The mean echocardiographic gradient of decellularized aortic homografts was 5.4 ± 3.2 mmHg and 11.6 ± 4.2 mmHg for the decellularized pulmonary homografts. Valvular regurgitation was 0 or 0-I for all DHVs. CONCLUSIONS: A single-stage double semilunar valve replacement with DHV has shown promising early results in these 5 very complex cases, providing an additional surgical option after multiple preceding valve procedures in young patients.

A rare mechanism of aortic regurgitation in a young patient.

A 19-year-old male patient was admitted to our institute with dyspnea. His medical history had no rheumatic fever or infective endocarditis. Physical examination revealed a diastolic murmur over the aortic area, rales of bilateral lungs. Bedside transthoracic echocardiography (TTE) revealed a severe aortic regurgitation (AR) without aortic valve stenosis and a moderately dilated left ventricle accompanied by an ejection fraction of 55%. The aortic valve could not be clearly demonstrated as either bicuspid or tricuspid. Congenital AR typically occurs in conjunction with an additional cardiac abnormality or aortic valve stenosis. Furthermore, bicuspid aortic valves are observed in the majority of patients. The aortic valve is created from the truncus ridge of the truncus arteriosus while the embryological development.

Single coronary ostium in a patient with quadricuspid aortic valve combined with aneurysmal ascending aortic dilatation.

BACKGROUND: The presence of a fourth aortic valve cusp (quadricupsid aortic valve) is a rare congenital malformation and is often accompanied by other anomalies of the adjacent cardiovascular structures. Among these concomitant anomalies, simultaneous association of both a single coronary ostium and aneurysmal ascending aortic dilation in combination with the quadricupsid aortic valve has not been reported yet. CASE PRESENTATION: We experienced the case of a 56-year-old female patient presenting as aortic regurgitation resulted from malcoaptation of quadricupsid aortic valve. The patient had also accompanying aneurysmal ascending aortic dilatation and coronary ostial anomaly. Surgical correction (aortic valve replacement with mechanical devices and supracoronary aortic replacement with prosthetic graft) was performed without any complications. CONCLUSIONS: The technological development of preoperative imaging studies enable the physician to encounter the quadricuspid aortic valve with other associated malformations more often unlike previous reports. With review on the quadricuspid aortic valve, we discussed a surgical considerations for the treatment of this anomaly.

Válvula aórtica cuadricúspide: caso clínico y discusión / Ouadricuspid aortic valve

The quadricuspid aortic valve is a rare congenital anomaly, usually presenting as an isolated malformation causing aortic regurgitation in the faith or sixth decades of life. The first reported cases were found at autopsy or became evident by surgical findings. However, the emergence of new imaging modalities currently allows an earlier diagnosis, including in asymptomatic patients. Hereby the case of a 60-year-old woman with quadricuspid aortic valve diagnosed by echocardiography is presented. A brief discussion of the disease is included.

Pathological Investigation of Congenital Bicuspid Aortic Valve Stenosis, Compared with Atherosclerotic Tricuspid Aortic Valve Stenosis and Congenital Bicuspid Aortic Valve Regurgitation.

BACKGROUND: Congenital bicuspid aortic valve (CBAV) is the main cause of aortic stenosis (AS) in young adults. However, the histopathological features of AS in patients with CBAV have not been fully investigated. METHODS AND RESULTS: We examined specimens of aortic valve leaflets obtained from patients who had undergone aortic valve re/placement at our institution for severe AS with CBAV (n = 24, CBAV-AS group), severe AS with tricuspid aortic valve (n = 24, TAV-AS group), and severe aortic regurgitation (AR) with CBAV (n = 24, CBAV-AR group). We compared the histopathological features among the three groups. Pathological features were classified using semi-quantitative methods (graded on a scale 0 to 3) by experienced pathologists without knowledge of the patients' backgrounds. The severity of inflammation, neovascularization, and calcium and cholesterol deposition did not differ between the CBAV-AS and TAV-AS groups, and these four parameters were less marked in the CBAV-AR group than in the CBAV-AS (all p<0.01). Meanwhile, the grade of valvular fibrosis was greater in the CBAV-AS group, compared with the TAV-AS and CBAV-AR groups (both p<0.01). In AS patients, thickness of fibrotic lesions was greater on the aortic side than on the ventricular side (both p<0.01). Meanwhile, thickness of fibrotic lesions was comparable between the aortic and ventricular sides in CBAV-AR patients (p = 0.35). CONCLUSIONS: Valvular fibrosis, especially on the aortic side, was greater in patients with CBAV-AS than in those without, suggesting a difference in the pathogenesis of AS between CBAV and TAV.

Surgery for doubly committed ventricular septal defects.

OBJECTIVES: Doubly committed ventricular septal defects (VSDs) account for up to almost one-third of isolated ventricular septal defects in Asian countries, compared with only 1/20th in western populations. In our surgical experience, this type of defect accounted for almost three-quarters of our practice. To date, patch closure has been considered the gold standard for surgical treatment of these lesions. Our objectives are to evaluate the indications and examine the outcomes of surgery for doubly committed VSDs. METHODS: Between October 2013, when our service of paediatric cardiac surgery was opened, and December 2014, 24 patients were referred for surgical closure of VSDs. Among them, 17 patients (71%), with the median age of 6 years, ranging from 2 to 9 years, and with a median body weight of 19 kg, ranging from 11 to 56 kg, underwent surgical repair for doubly committed defects. In terms of size, the defect was considered moderate in 4 and large in 13. Aortic valvular regurgitation (AoVR) was present in 11 patients (65%) preoperatively, with associated malformations found in 14 (82%), with 5 patients (29%) having two or more associated defects. RESULTS: After surgery, there was trivial residual shunting in 2 patients (12%). AoVR persisted in 6 (35%), reducing to trivial in 5 (29%) and mild in 1 (6%). Mean stays in the intensive care unit and hospital were 2.6 ± 1.2 days, ranging from 2 to 7 days, and 6.8 ± 0.8 days, ranging from 6 to 9 days, respectively. The mean follow-up was 14 ± 4 months, ranging from 6 to 20 months, with no early or late deaths and without clinical deterioration. CONCLUSIONS: The incidence of doubly committed lesions is high in our experience, frequently associated with AoVR and other associated malformation. Early detection is crucial to prevent further progression of the disease. Patch closure remains the gold standard in management, not least since it allows simultaneous repair of associated intracardiac defects.

Relation of Aortic Valve Morphologic Characteristics to Aortic Valve Insufficiency and Residual Stenosis in Children With Congenital Aortic Stenosis Undergoing Balloon Valvuloplasty.

Aortic valve morphology has been invoked as intrinsic to outcomes of balloon aortic valvuloplasty (BAV) for congenital aortic valve stenosis. We sought to use aortic valve morphologic features to discriminate between valves that respond favorably or unfavorably to BAV, using aortic insufficiency (AI) as the primary outcome. All patients who underwent BAV at 2 large-volume pediatric centers from 2007 to 2014 were reviewed. Morphologic features assessed on pre-BAV echo included valve pattern (unicuspid, functional bicuspid, and true bicuspid), leaflet fusion length, leaflet excursion angle, and aortic valve opening area and on post-BAV echo included leaflet versus commissural tear. Primary end point was increase in AI (AI+) of ≥2°. Eighty-nine patients (median age 0.2 years) were included in the study (39 unicuspid, 41 functional bicuspid, and 9 true bicuspid valves). Unicuspid valves had a lower opening area (p <0.01) and greater fusion length (p = 0.01) compared with functional and true bicuspid valves. Valve gradient pre-BAV and post-BAV were not different among valve patterns. Of the 16 patients (18%) with AI+, 14 had leaflet tears (odds ratio 13.9, 3.8 to 50). True bicuspid valves had the highest rate (33%) of AI+. On multivariate analysis, leaflet tears were associated with AI+, with larger opening area pre-BAV and lower fusion length pre-BAV. AI+ was associated with larger pre-BAV opening area. Gradient relief was associated with reduced angle of excursion. Valve morphology influences outcomes after BAV. Valves with lesser fusion and larger valve openings have higher rates of leaflet tears which in turn are associated with AI.

Endurance Training on Congenital Valvular Regurgitation: An Athlete Case Series.

BACKGROUND: Both intense endurance training and valvular regurgitation place a volume load on the right and left ventricles, potentially leading to dilation, but their effects in combination are not well-known. PURPOSE: The purpose of this case series is to describe the combined volume load of intense endurance athletic training and regurgitant valvular disease as well as the challenging assessment of each component's cardiovascular effect. METHODS: In this article, the clinical course of three elite endurance athletes with congenital valvular disease were reviewed. RESULTS: A swimmer with aortic regurgitation, a cyclist with aortic regurgitation, and a cyclist with pulmonary regurgitation were found to have severe dilation of the associated ventricles despite continuing to train at an elite level without symptoms. CONCLUSIONS: Because of the cumulative effects of endurance training and valvular regurgitation, each athlete manifested ventricular dilation out of proportion to their valvular disease and symptoms. Although the effects of congenital valvular disease and athletic remodeling on ventricular dilation have been thoroughly studied individually, their cumulative effect is not well understood. This complicates the assessment of athletes with valvular regurgitation and underscores the need for athlete-specific recommendations for valve replacement.

Surgical repair of congenital aortic regurgitation by aortic root reduction: A finite element study.

During surgical reconstruction of the aortic valve in the child, the use of foreign graft material can limit durability of the repair due to inability of the graft to grow with the child and to accelerated structural degeneration. In this study we use computer simulation and ex vivo experiments to explore a surgical repair method that has the potential to treat a particular form of congenital aortic regurgitation without the introduction of graft material. Specifically, in an aortic valve that is regurgitant due to a congenitally undersized leaflet, we propose resecting a portion of the aortic root belonging to one of the normal leaflets in order to improve valve closure and eliminate regurgitation. We use a structural finite element model of the aortic valve to simulate the closed, pressurized valve following different strategies for surgical reduction of the aortic root (e.g., triangular versus rectangular resection). Results show that aortic root reduction can improve valve closure and eliminate regurgitation, but the effect is highly dependent on the shape and size of the resected region. Only resection strategies that reduce the size of the aortic root at the level of the annulus produce improved valve closure, and only the strategy of resecting a large rectangular portion-extending the full height of the root and reducing root diameter by approximately 12% - is able to eliminate regurgitation and produce an adequate repair. Ex vivo validation experiments in an isolated porcine aorta corroborate simulation results.

Female Adolescent With Quadricuspid Aortic Valve.

Quadricuspid aortic valve is a rare congenital heart defect. The authors present a case of this anomaly in a young, asymptomatic adolescent who presented to the echocardiography laboratory for further evaluation of a cardiac murmur detected on routine physical examination. Imaging revealed a quadricuspid aortic valve with aortic regurgitation. This case highlights the importance of auscultation in detecting cardiac abnormalities.