Major adverse cardiac and cerebrovascular events after the Ross procedure: a report from the German-Dutch Ross Registry.

BACKGROUND: The purpose of the study is to report major cardiac and cerebrovascular events after the Ross procedure in the large adult and pediatric population of the German-Dutch Ross registry. These data could provide an additional basis for discussions among physicians and a source of information for patients. METHODS AND RESULTS: One thousand six hundred twenty patients (1420 adults; 1211 male; mean age, 39.2±16.2 years) underwent a Ross procedure between 1988 and 2008. Follow-up was performed on an annual basis (median, 6.2 years; 10 747 patient-years). Early and late mortality were 1.2% (n=19) and 3.6% (n=58; 0.54%/patient-year), respectively. Ninety-three patients underwent 99 reinterventions on the autograft (0.92%/patient-year); 78 reinterventions in 63 patients on the pulmonary conduit were performed (0.73%/patient-year). Freedom from autograft or pulmonary conduit reoperation was 98.2%, 95.1%, and 89% at 1, 5, and 10 years, respectively. Preoperative aortic regurgitation and the root replacement technique without surgical autograft reinforcement were associated with a greater hazard for autograft reoperation. Major internal or external bleeding occurred in 17 (0.15%/patient-year), and a total of 38 patients had composite end point of thrombosis, embolism, or bleeding (0.35%/patient-year). Late endocarditis with medical (n=16) or surgical treatment (n=29) was observed in 38 patients (0.38%/patient-year). Freedom from any valve-related event was 94.9% at 1 year, 90.7% at 5 years, and 82.5% at 10 years. CONCLUSIONS: Although longer follow-up of patients who undergo Ross operation is needed, the present series confirms that the autograft procedure is a valid option to treat aortic valve disease in selected patients. The nonreinforced full root technique and preoperative aortic regurgitation are predictors for autograft failure and warrant further consideration. Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT00708409.

Autograft reinforcement to preserve autograft function after the ross procedure: a report from the german-dutch ross registry.

BACKGROUND: Autograft reinforcement interventions (R) during the Ross procedure are intended to preserve autograft function and improve durability. The aim of this study is to evaluate this hypothesis. METHODS AND RESULTS: 1335 adult patients (mean age:43.5+/-12.0 years) underwent a Ross procedure (subcoronary, SC, n=637; root replacement, Root, n=698). 592 patients received R of the annulus, sinotubular junction, or both. Regular clinical and echocardiographic follow-up was performed (mean:6.09+/-3.97, range:0.01 to 19.2 years). Longitudinal assessment of autograft function with time was performed using multilevel modeling techniques. The Root without R (Root-R) group was associated with a 6x increased reoperation rate compared to Root with R (Root+R), SC with R (SC+R), and without R (SC-R; 12.9% versus 2.3% versus 2.5%.versus 2.6%, respectively; P<0.001). SC and Root groups had similar rate of aortic regurgitation (AR) development over time. Root+R patients had no progression of AR, whereas Root-R had 6 times higher AR development compared to Root+R. In SC, R had no remarkable effect on the annual AR progression. The SC technique was associated with lower rates of autograft dilatation at all levels of the aortic root compared to the Root techniques. R did not influence autograft dilatation rates in the Root group. CONCLUSIONS: For the time period of the study surgical autograft stabilization techniques preserve autograft function and result in significantly lower reoperation rates. The nonreinforced Root was associated with significant adverse outcome. Therefore, surgical stabilization of the autograft is advisable to preserve long-term autograft function, especially in the Root Ross procedure.

Autograft regurgitation and aortic root dimensions after the Ross procedure: the German Ross Registry experience.

BACKGROUND: Autograft regurgitation and root dilatation after the Ross procedure is of major concern. We reviewed data from the German Ross Registry to document the development of autograft regurgitation and root dilatation with time and also to compare 2 different techniques of autograft implantation. METHODS AND RESULTS: Between 1990 and 2006 1014 patients (786 men, 228 women; mean age 41.2+/-15.3 years) underwent the Ross procedure using 2 different implantation techniques (subcoronary, n=521; root replacement, n=493). Clinical and serial echocardiographic follow up was performed preoperatively and thereafter annually (mean follow up 4.41+/-3.11 years, median 3.93 years, range 0 to 16.04 years; 5012 patient-years). For statistical analysis of serial echocardiograms, a hierarchical multilevel modeling technique was applied. Eight early and 28 late deaths were observed. Pulmonary autograft reoperations were required in 35 patients. Initial autograft regurgitation grade was 0.49 (root replacement 0.73, subcoronary 0.38) with an annual increase of grade 0.034 (root replacement 0.0259, subcoronary 0.0231). Annulus and sinus dimensions did not exhibit an essential increase over time in both techniques, whereas sinotubular junction diameter increased essentially by 0.5 mm per year in patients with root replacement. Patients with the subcoronary implantation technique showed nearly unchanged dimensions. Bicuspid aortic valve morphology did not have any consistent impact on root dimensions with time irrespective of the performed surgical technique. CONCLUSIONS: The present Ross series from the German Ross Registry showed favorable clinical and hemodynamic results. Development of autograft regurgitation for both techniques was small and the annual progression thereof is currently not substantial. Use of the subcoronary technique and aortic root interventions with stabilizing measures in root replacement patients seem to prevent autograft regurgitation and dilatation of the aortic root within the timeframe studied.

Re-interventions on the autograft and the homograft after the Ross operation in children.

BACKGROUND: For children who require aortic valve replacement, the pulmonary autograft may be the ideal substitute. However, re-operations for conduit exchange in the pulmonary position are inevitable. In addition, re-operations on the autograft may be necessary due to dilatation and neo-aortic insufficiency. We sought to assess predictors for re-intervention in an international Ross-operated paediatric population. METHODS: Data of 152 children below 16 years of age at the time of the Ross operation were analysed using Cox proportional hazard modelling. Mean follow-up time was 6.1+/-4.2 years. RESULTS: The median age at the time of the Ross operation was 10.1 years (range 54 days to 15 years). Early mortality was 2.6%. Survival at 5 and 10 years was 93.9+/-2.0% and 90.4+/-3.1%, respectively. Seven patients required autograft re-intervention (explantation n=6 and reconstruction n=1). Freedom from autograft re-intervention at 5 and 10 years was 99.3+/-0.7% and 95.5+/-2.7%, respectively. Prior endocarditis (p=0.061), prior aortic regurgitation (p=0.061) and longer follow-up time (p=0.036) emerged as risk factors for autograft re-intervention. Seventeen patients required 36 conduit re-interventions (replacement n=16, percutaneous valvuloplasty n=10). Freedom from conduit re-intervention at 5 and 10 years was 89.3+/-2.9% and 79.6+/-6.1%, respectively. Implantation of an aortic homograft (p=0.013), and smaller conduit size (p=0.074) emerged as risk factors for conduit re-intervention. CONCLUSIONS: There is a consistent need for conduit re-intervention following the Ross operation in children. Re-interventions on the autograft are rare within the first decade after surgery. However, the number of autograft re-interventions may increase after the first decade, since longer follow-up time is a risk factor for autograft failure.

Neoaortic root diameters and aortic regurgitation in children after the Ross operation.

BACKGROUND: For children who require aortic valve replacement, the Ross operation provides a unique advantage of growth potential of the pulmonary autograft in the aortic position. This study assessed the progression of autograft root diameters and its effect on aortic regurgitation (AR). METHODS: Neoaortic echo dimensions from 48 children (<16 years) undergoing Ross operation who had follow-up echocardiograms before age 20 were analyzed (mean follow-up, 5.1 +/- 3.3 years). RESULTS: The mean age at the time of the Ross operation was 10.0 +/- 4.3 years. Mean z values of the neoaortic annulus (1.5 +/- 0.4), sinus (2.5 +/- 0.4), and sinotubular junction (2.6 +/- 0.9) when the autograft was implanted were significantly larger compared with normal values (p < 0.001, all). The mean z values significantly increased with follow-up at the level of the sinus (0.5 +/- 0.1/year, p < 0.001) and the sinotubular junction (0.7 +/- 0.2, p < 0.001), but not at the level of the annulus (0.1 +/- 0.1, p = 0.59). AR increased with follow-up time (0.07 +/- 0.02 grade/year, p < 0.001). AR increased with sinotubular junction diameter (p = 0.028), but there was not significant evidence of an association with annulus diameter (p = 0.25) or sinus diameter (p = 0.40). CONCLUSIONS: Children undergoing Ross operation have larger neoaortic root dimensions than healthy children. Growth of the annulus matches somatic growth. The diameters of the sinus and the sinotubular junction increase significantly relative to somatic growth. The latter may explain the development of AR.

Homograft performance in children after the Ross operation.

BACKGROUND: The Ross operation may be the ideal aortic valve replacement in pediatric patients. However, reoperations for replacement of the homograft in the pulmonary position are inevitable. This study determined influencing factors for the development of homograft stenosis and regurgitation in pediatric Ross patients. METHODS: Follow-up echocardiograms of 116 children (86 boys) undergoing Ross operations at a mean age, 9.3 +/- 4.9 years were analyzed using hierarchic multilevel modeling. Mean duration of the echocardiographic follow-up was 5.3 +/- 4.2 years (609 patient-years, 398 examinations). RESULTS: Median homograft diameter z value was 0.3 (range -2.2 to +7.3). Mean homograft pressure gradient at implantation was 5.0 mm Hg with a significant increase of 4.2 mm Hg/y (p < 0.001) within the first 2 years and a steady state thereafter. Older donor age was significantly associated with lower mean pressure gradient at implantation (p = 0.037). Larger z value had no significant influence on the annual increase of pressure gradient (p = 0.87). Mean grade of regurgitation at implantation was 0.9, without significant annual increase (0.02 grade/y, (p = = 0.32). Older recipient (p = 0.005) and donor age (p < 0.0001) were significantly associated with lower mean regurgitation at implantation. Larger z value was associated with a higher annual increase of regurgitation (p = 0.014). CONCLUSIONS: Relevant midterm homograft regurgitation is rare in children after the Ross operation. However, a significant annual increase occurs in the pressure gradient that cannot be influenced by larger graft size. Homograft oversizing may lead to a higher annual increase of regurgitation.

The spectrum of subdivided left atrium: diagnosis, treatment and outcome in eight patients.

From January 1996 to April 2001, we treated eight patients with subdivided left atrium. Their ages at diagnosis ranged from newborn to 6.4 years. Prominent clinical symptoms were failure to thrive, respiratory symptoms, demand for oxygen, and congestive cardiac failure. Diagnosis was made by transthoracic echocardiography in all cases. Cardiac catheterization was necessary only in those patients who had associated cardiac anomalies, or suspected signs of pulmonary hypertension. In 7 patients, surgery was performed immediately after diagnosis, but one preterm infant died before operation due to neonatal sepsis and respiratory distress syndrome. In the postoperative period, one patient developed a severe capillary leak syndrome, and died due to irreversible congestive cardiac failure. The other 6 patients have all been followed up, with good results in the short- and intermediate-term at a mean of 34.3+/-20.2 months. The infants were thriving, had a reduction in the frequency of infections of the respiratory tract, no significant arrhythmias, and showed early recovery from pulmonary hypertension and right ventricular hypertrophy.