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 Association Between Aortic Valve Leaflet Morphology and Patterns of Aortic Dilation in Patients With Bicuspid Aortic Valves.

BACKGROUND: Dilation of the aorta is a frequent complication in patients with bicuspid aortic valves. The aim of this study was to determine the relationship between the subtype of leaflet fusion, right and noncoronary leaflet (R/N) fusion versus right and left leaflet (R/L) fusion, and the patterns of aortic dilation and valve dysfunction in young patients with bicuspid aortic valves. METHODS: We performed a retrospective review of 642 patients who presented with bicuspid aortic valves between 1994 and 2014. Of these patients, 210 (33%) had aortic dilation (z score >3) by echocardiogram. For each patient, the most recent study or the last study before intervention was reviewed. RESULTS: Median patient age was 15 years (range, 0 to 40 years) with patients with R/N fusion being younger. The most prevalent subtype was R/N fusion (R/N, n = 114, 54% versus R/L, n = 96, 46%). Dilation of the ascending aorta was seen more often in patients with R/N fusion (R/N, 88% versus R/L, 68%; p = 0.004), whereas the prevalence of dilation of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 46% versus R/N, 20%; p = 0.01). The magnitude of dilation differed as well. The z value of the sinuses of Valsalva was significantly higher in patients with R/L fusion (R/L, 2.03 versus R/N, 1.2; p = 0.003), whereas the z values of the ascending aorta and sinotubular junction were similar between the groups. Patients with R/N fusion were more likely to have aortic stenosis, and within the R/N group, patients with aortic insufficiency had a greater degree of ascending aorta dilation (p = 0.04). CONCLUSIONS: Our study suggests that in young patients with bicuspid aortic valves and aortic dilation, aortic valve morphology may be associated with the patterns of aortic dilation and valve dysfunction. Patients with R/N fusion were more likely to have ascending aorta dilation, whereas patients with R/L fusion were more likely to have dilation of the aortic root. In addition, patients with R/N fusion presented at a younger age and were more likely to have aortic stenosis. Recognition of these differences may eventually be helpful for patient counseling and the planning of follow-up.

Does the Homograft for RVOT Reconstruction in Ross: Patients Fare Better than for Non-Ross Patients? A Single-Center Experience.

BACKGROUND AND AIM OF THE STUDY: In patients undergoing the Ross procedure, the right ventricular outflow tract (RVOT) conduit is inserted in an orthotopic position rather than in the more heterotopic position used in the repair of complex congenital RVOT obstruction. The study aim was to compare the authors' institutional mid-term experience of large-sized homografts (>19 mm) in patients with Ross and non-Ross RVOT reconstructions. METHODS: The outcome was reviewed of all homografts implanted for Ross (n = 72) or non-Ross (n = 64) RVOT reconstruction at a single center between 1993 and 2012. Echocardiographic data were reviewed to evaluate valve performance. Homograft dysfunction was defined as RVOT obstruction with peak echo-Doppler gradient >40 mmHg and/or grade >III/IV conduit valve regurgitation. Homograft failure was defined as the need for conduit replacement or catheter or surgical reintervention. RESULTS: The age, body weight, conduit diameter and previous surgery were significantly higher in patients with Ross compared to the non-Ross group (p = 0.002, 0.003 and <0.001, respectively). The mean follow up duration was similar in both groups. There were 35 re-interventions (Ross, n = 17; non-Ross, n = 18). The data acquired showed actuarial survival, freedom from conduit dysfunction and conduit failure to be similar in both cohorts. Freedom from any type of reoperation was worse for the Ross group (58%) than for the non-Ross group (72%) (p = 0.05). CONCLUSION: During the first 15 years after Ross or non-Ross pulmonary homograft implantation, the survival rate, freedom from failure and dysfunction, and RVOT gradient were statistically similar. Freedom from any type of reoperation was significantly higher in the non-Ross group, however.

The Ross-Konno is a high-risk procedure when compared with the Ross operation in children.

BACKGROUND: For children who require aortic valve replacement, the pulmonary autograft (Ross procedure) may be the ideal substitute. However, performing a modified Konno procedure at the time of autograft implantation (Ross-Konno) may be associated with significant morbidity and mortality. A retrospective study was undertaken to compare the outcomes of Ross-Konno (RK) and the Ross (R) procedures including the need for reinterventions and long-term survival. METHODS: Between 1993 and 2011, 78 children (mean age, 11.1 ± 5.6 years; range, 1 week to 18 years) underwent the Ross procedure. Modified Konno-type enlargement of the left ventricular outflow tract was performed in 18 of those patients. RESULTS: There was no statistically significant difference between the groups with respect to pathologic process, sex, concomitant procedures, and aortic gradient. Our data demonstrate that mean age (R, 12.9 years versus RK, 5.3 years; p < 0.001), mean size of allograft (R, 23.3 mm versus RK, 20.1 mm; p < 0.001), previous surgery (R, 51% versus RK, 83%; p = 0.05), and postoperative morbidity (R, 3% versus RK, 28%; p = 0.003) were significantly different between the groups. There were 3 hospital deaths (all RK with mitral valve anomalies). Actuarial survival at 10 years was significantly better for Ross patients than Ross-Konno (R, 96% versus RK, 72%; p = 0.001). Freedom from autograft, right ventricular outflow tract obstruction, and cumulative reoperations at 10 years were not significantly different between groups. CONCLUSIONS: The risk of death and postoperative complications after the Ross-Konno procedure is higher than for the Ross procedure. Preoperative complexity (including mitral valve anomalies) is associated with significantly higher morbidity and mortality. Autograft insufficiency and right ventricular outflow tract obstruction are common postoperative complications, requiring reoperation in one quarter of patients, but these were not significantly different between the groups.

Autograft or allograft aortic root replacement in children and young adults with aortic valve disease: a single-center comparison.

BACKGROUND: We analyzed the outcome of children and young adults (younger than 40 years) with aortic valve disease who underwent allograft or autograft aortic root replacement (ARR) in our institution and evaluated whether there is a preference for either valve substitute. METHODS: One-hundred fifty patients younger than 40 years underwent ARR between January 1990 and July 2011. Forty-four patients, aged 18.8 ± 12.4 years, had ARR with allograft conduit (allograft group), whereas 106 patients, aged 17.9 ± 11 years (p = 0.63), had a Ross ARR during the same period of time (autograft group). Echocardiographic data were reviewed to evaluate valve performance. The 2 groups were similar with respect to age, gender, etiology, and previous and concomitant procedures. RESULTS: Operative deaths were 3 in the autograft group. There were 6 late deaths in the autograft group and 5 in the allograft group. Survival was 92% and 84% at 5 and 15 years, respectively, in the allograft group versus 93% and 91% in the autograft group (p = 0.42). Freedom from any type of reintervention and from reoperation on aortic valve were similar (autograft, 64% and 72% versus allograft, 66% and 66%; p = not significant) at 15 years. Freedom from explantation were significantly better for Ross patients (autograft, 82% versus allograft, 66%; p = 0.05). CONCLUSIONS: Aortic valve replacement with either the autograft or allograft provides good clinical results in children and young adults during an intermediate duration of observation. Survival early after ARR does not differ depending on the type of prosthesis. In patients with aortic valve disease, autograft and allograft ARR show comparable satisfactory early and long-term results, with the increasing reoperation risk in the second decade after operation remaining a major concern.

Decellularized versus standard cryopreserved valve allografts for right ventricular outflow tract reconstruction: a single-institution comparison.

BACKGROUND: Standard cryopreserved valved allografts (SCAs) are recognized as the benchmark for reconstruction of the right ventricular outflow tract (RVOT). However, SCAs frequently demonstrate early valve deterioration and elicit an immune response. Decellularized cryopreserved valve allografts (SynerGraft, SG) are less immunogenetic and may be more durable. This study analyzed our results of RVOT reconstruction using SGs and compared it with the SCAs used during the same period. METHODS: We reviewed the outcome of all allografts (SG and SCA) that were implanted for RVOT reconstruction at a single center from 2000 to 2005. Echocardiographic data were reviewed to evaluate valve performance. Conduit failure is defined as the need for conduit replacement or reintervention in either the catheterization laboratory or operating room. Conduit dysfunction is defined as RVOT obstruction with peak echocardiographic Doppler gradient greater than 40 mm Hg and/or grade III/IV or greater conduit valve regurgitation. Data were compared using the Wilcoxon rank sum and Fisher's exact test. RESULTS: From January 2000 to April 2005, 100 patients (mean age 18.6 ± 16.8 years) received SG (n = 39) or SCA (n = 61) conduits. The 2 retrospective nonrandomized cohorts were similar with respect to age, gender, weight, conduit indication, bypass and crossclamp time, and conduit size. Follow-up time was not significant between the 2 groups (SG, 5.7 ± 2.5 years vs SCA, 5.8 ± 2.8 years; P = .83). Early and late mortality were similar (SG, 13%; SCA, 10%; P = .75). No death was graft related. Freedom from dysfunction was superior with SG (SG, 74%, vs SCA, 52%; P = .05). Freedom from failure was also better in patients with SG (SG, 87%, vs SCA, 68%; P = .05). Freedom from explantation and more than moderate pulmonary insufficiency were significantly better for SG patients (SG, 92% and 90%, vs SCA, 78% and 68%; P = .02). CONCLUSIONS: This study suggests that the midterm performance of SGs may be superior to that of SCAs. Decellularization of the cryopreserved allografts may provide a more durable option for patients who need RVOT reconstruction. Further long-term follow-up is needed to see whether this decellularization process improves long-term allograft durability.

Repair of transposition of the great arteries with total anomalous pulmonary venous return.

A 13-year-old Haitian girl presented with complex congenital heart disease consisting of transposition of the great arteries with total anomalous venous return. The patient exhibited clinical findings of long-standing congestive heart failure and cyanosis. She underwent complete repair with resolution of her symptoms.