Does the pattern of bicuspid aortic valve leaflet fusion determine the success of the Ross procedure?

BACKGROUND: In addition to being associated with aortopathy, a bicuspid aortic valve (BAV) has been posed to be a risk factor for the dilation of the pulmonary autograft in the aortic position. The aim of this study is to assess the association between the subtype of native aortic valve leaflet fusion (right and noncoronary leaflets [R/N] vs right and left leaflets [R/L]) and autograft dilation and valve dysfunction after the Ross procedure. METHODS: We performed a retrospective review of 43 patients with BAV who underwent a Ross procedure in our center from 1993 to 2013. Serial transthoracic echocardiography was used to measure changes in autograft and ascending aortic diameter over time. The aortic diameter was measured at four levels, and Z values were computed. Aortic dilation was defined as a Z value greater than 3. RESULTS: The mean age at the time of the Ross procedure was 13.5 ± 9.2 years. R/L was the most prevalent native aortic valve subtype (R/L, n = 26, 61% vs R/N, n = 17, 39%). PreRoss procedure, aortic dilation was more frequent in patients with R/N fusion (P = .02), whereas the initial aortic valve gradient and grade of aortic insufficiency (AI) did not differ between the subgroups. At follow-up, (mean = 9.6 ± 4.3 years) dilation of the autograft and ascending aorta was seen more often in patients with R/N leaflet fusion (P = .03). Conversely, the prevalence of more than moderate AI was significantly higher in patients with R/L leaflet fusion (P = .03). There was no significant difference between groups among numbers of late reintervention on the aortic valve or root (P = .75); however the type of intervention varied by morphologic subtype. Patients with R/L fusion underwent more aortic valve replacements (AVRs) while patients with R/N fusion underwent more valve-sparing aortic root replacements. CONCLUSIONS: After Ross procedure, both groups of patients were likely to have a combination of dilation of the aortic root and the tubular portion of the ascending aorta at follow-up. Patients with R/L fusion were more likely to have a prevalence of root dilation, while patients with R/N fusion were more likely to have tubular ascending aorta dilation. The R/L phenotype is associated with a slightly more rapid dilation at follow-up and is more likely to have postoperative autograft insufficiency. This information may serve to guide patient and procedure selection for AVR.

The World Database for Pediatric and Congenital Heart Surgery "A Call to Service for North American Congenital Heart Surgery Programs".

The World Society for Pediatric and Congenital Heart Surgery has endorsed the establishment of an international platform for the exchange of knowledge and experience for those that treat patients with a congenital heart defect. On January 1, 2017, the release of the World Database for Pediatric and Congenital Heart Surgery opened a new era in evaluation of treatment with congenital heart defects. The contribution of data from countries with established congenital surgical databases will greatly enhance the efforts to provide the most accurate measure of overall surgical outcomes across the globe.

Management of an associated ventricular septal defect at the time of coarctation repair.

BACKGROUND: Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD. METHODS: We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality. RESULTS: There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p < 0.001). Mean age at repair for group 1 (n = 286) and group 2 (n = 472) was 87.4 days and 21.6 days, respectively (p = 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p = 0.130). Discharge mortality for group 1 and group 2 was similar, at 8.7% and 9.1%, respectively (p = 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%. CONCLUSIONS: The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach.

AngioJet rheolytic thrombectomy for thrombotic myocardial injury in a 15-year-old cystic fibrosis patient during lung transplantation.

We report on an acute myocardial injury caused by thrombotic emboli during off-pump bilateral sequential lung transplantation in a 15-year-old female patient with end-stage cystic fibrosis. A few minutes after reperfusion of the right (second sequential) allograft, the patient developed hemodynamic collapse, including hypotension, acute akinesis of the anterior and lateral myocardial walls, and severe mitral regurgitation. Thrombus was noted within the left atrium and ventricle on intraoperative transesophageal echocardiogram. After emergent cannulation for cardiopulmonary bypass, the patient underwent left cardiac catheterization with AngioJet rheolytic thrombectomy and removal of the clot from the distal left anterior descending coronary artery. Reperfusion was established without the need for stent placement or further intervention. The patient required short-term life support with venoarterial extracorporeal membrane oxygenation.

Repair of anomalous origin of right pulmonary artery from ascending aorta without cardiopulmonary bypass.

Anomalous origin of a pulmonary artery from the ascending aorta (AORPA) is a rare congenital cardiac malformation that needs prompt surgical repair; otherwise it is associated with poor prognosis. We describe 3 cases of AORPA that successfully underwent complete surgical correction without extracorporeal circulation.

Truncal valve repair in neonates using pericardial leaflet extension.

Truncal valve insufficiency is a significant risk factor for post-operative mortality following repair of truncus arteriosus. The surgical management of dysplastic and insufficient truncal valves remains an operative challenge. We report the cases of two infants with type 2 truncus arteriosus and severely dysplastic and insufficient quadricuspid truncal valves. At primary repair, their truncal valves were successfully repaired using pericardial leaflet extensions. This technique may be used in neonates with truncal valve insufficiency as part of the primary repair of truncus arteriosus.

Pathophysiologic implications of the helical ventricular myocardial band: considerations for right ventricular restoration.

This chapter describes the structure/function relationships of the right ventricle (RV), and shows how the geometry of the helical ventricular myocardial band model defines spatial geometry of the free wall and septum that underlie dynamic action. Myocardial fiber orientation is the keynote to performance in health and disease. The transverse geometry of the RV free wall allows constriction (bellows-type motion), whereas oblique septal fiber orientation and midline septal position is essential for ventricular twisting, the vital mechanism for RV ejection against increased pulmonary vascular resistance. Therefore, the septum is considered "the lion or motor of RV performance." Distortion of such normal structure/function relationships underlies the pathophysiologic mechanisms of RV failure. Operative methods that restore normal myocardial fiber orientation are described to outline evolving surgical techniques for the surgical treatment of RV failure.

Successful management of patients with pulmonary atresia with intact ventricular septum using a three tier grading system for right ventricular hypoplasia.

BACKGROUND: We sought to validate a simple grading scheme for right ventricular hypoplasia in determining suitability for a biventricular repair. METHODS: We reviewed the medical records for 106 patients with pulmonary atresia-intact ventricular septum (PA-IVS) treated between 1982 and 2001. Over this period, children were assigned to mild (> 2/3 normal size, 23.7% of patients), moderate (1/3 to 2/3, 41.2%), or severe (1/3, 35.1%) right ventricular hypoplasia, and this grouping, along with severity of coronary anomalies (45% right ventricle to coronary fistulae, 16% with right ventricle dependent coronary circulation [RVDCC]), triaged children to eventual single ventricle (severe) or two-ventricle (mild or moderate) repair. RESULTS: Actuarial 10-year survival was 86.3% with mortality predicted by severe hypoplasia (odds ratio [OR] 12.9, p < 0.001), RVDCC (OR 15.0, p < 0.001), and non-Caucasian race (OR 10.7, p < 0.001). Multivariate analysis with a Cox proportional hazards model confirmed only RVDCC (risk ratio [RR] 10.9, p = 0.0009} and non-Caucasian race (RR 6.9, p = 0.007) as significant. Although not an independent risk factor for survival, the degree of hypoplasia was the most important determinant for definitive repair. Severe hypoplasia virtually precluded two-ventricle repair (OR 33.1, p < 0.001 by chi2 analysis) and was the strongest risk factor for a one-ventricle system (OR 78.7, p < 0.001). Actuarial survival after either repair was 91%, and no biventricular repair later converted to a Fontan system. CONCLUSIONS: Surgical management of patients based on this three tier grade for right ventricular hypoplasia results in excellent survival and correctly predicts patients destined for eventual Fontan and biventricular repair.

Seventeen-year experience with 1,083 heart transplants at a single institution.

BACKGROUND: Heart transplantation is the most accepted treatment for end-stage heart disease. A review of 1,083 consecutive transplants (1984 to 2001) was undertaken. METHODS: Adult recipients were divided into quartiles. The last 540 transplants were combined. Three eras were created from these, 1984 to 1991, 1991 to 1995, and 1995 to 2001, with three age groups: 0 to 18 years, 19 to 61 years, and 62 to 74 years. All patients have at least 1 year of follow-up time. End points were survival, rejection, and graft coronary artery disease. RESULTS: There were 1,012 patients. Donor age, graft ischemic time, and the proportion of elderly recipients and nonstandard donor hearts have increased in the current era. Actuarial 60-month survivals of recipients after 1995 were 80.7% (0 to 18 years); 75.3% (19 to 61 years); and 76.2% (>62 years). The current era children and younger adult groups demonstrated improved results when compared with previous eras (p = 0.003 and p = 0.05). Rejection episodes equal to or greater than ISHLT grade 3A per person per year improved to 0.15 in the current era (p < 0.001). During the three eras, older recipients (>62 years) demonstrated fewer episodes of rejection when compared with other adults (0.13 versus 0.58, p = 0.03). Deaths attributed to graft coronary artery disease decreased from 11% to 5% from era 2 to era 3. Regression analysis revealed a mild effect of donor age on survival and graft coronary artery disease (hazard ratio = 1.02, p = 0.001; hazard ratio = 1.039, p < 0.001, respectively). Recipient predictors of graft coronary artery disease were diagnosis of ischemic cardiomyopathy (hazard ratio = 1.6, p = 0.014) and congenital heart disease (hazard ratio = 3.41, p = 0.02). CONCLUSIONS: Improved survival in the current era may be attributed to better organ preservation, improved immunosuppression and control of infection, and less life-threatening graft coronary artery disease.

Allograft mitral valve replacement.

Allograft valves have been used in cardiac valve replacement for over 40 years. Early failures of allograft mitral valve replacement (AMVR) were attributed to inadequate allograft preservation or unsuccessful implantation techniques. Due to an improved understanding of mitral valve function gained from experience in mitral valve reconstruction, improved imaging techniques for preoperative assessment, and improved methods of allograft preservation, there has been a resurgence of interest in AMVR. The use of bioprosthetic valves in children and young adults is characterized by reduced durability and early valve failure. With the use of mechanical valves, there is a significant long-term risk of morbidity and mortality resulting from thrombosis or hemorrhagic complications from anticoagulation. Additionally, anticoagulation is often difficult to manage in children. The advantages of using allograft mitral valves include the avoidance of anticoagulation and the preservation of the subvalvar apparatus and its role in ventricular function. At our institution, successful early results with AMVR have been obtained in eight children and young adults with previous atrioventricular septal defects, previous placement of mitral valve prostheses, rheumatic valvular disease, and bacterial endocarditis. Our early results and those of others are encouraging for the continued use of AMVR in children and young adults. Long-term follow-up will be necessary to assess the durability and function of these allograft mitral valves. Copyright 1999 by W.B. Saunders Company