- Research article
- Open Access
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The mid-term outcome of primary open valvotomy for critical aortic stenosis in early infancy - a retrospective single center study over 18 years
© The Author(s). 2016
- Received: 7 October 2015
- Accepted: 26 July 2016
- Published: 2 August 2016
The objective of this study was to examine early and long-term results of surgical aortic valvotomy in neonates and infants aged less than four months and to identify predictors of outcome.
Between August 1994 and April 2012, 83 consecutive patients younger than 4 months of age underwent open heart valvotomy for critical aortic stenosis in our institution. Median age was 17 days (range 0-111 days). We examined clinical records to establish determinants of outcome and illustrate long-term results.
Fifty-six patients (67 %) were neonates. Associated cardiac malformations were found in 24 patients (29 %), including multilevel left heart obstruction in 5. The median follow-up was 4.2 years. The time-related survival rate was 87 and 85 % at 5 and 15 years, respectively. The time-related survival without reintervention was respectively 51, 35 and 18 % at 5, 10 and 15 years. The time-related survival without aortic valve replacement was respectively 67, 54 and 39 % at 5, 10 and 15 years. Ventricular dysfunction (p = 0.04), delayed sternal closure (p = 0.007), endocardial fibroelastosis (p = 0.02) and low z-score of the aortic annulus (p = 0.04) were found predictors of global mortality. Ventricular dysfunction (p = 0.01) and endocardial fibroelastosis (p = 0.04) were found predictors of reintervention.
The experience, in our center, on the management of critical aortic stenosis, shows a low early and late mortality, but the aortic valvotomy is a palliative procedure and we see unfortunately a high rate of reintervention among which the aortic valve replacement. These results suggest to reconsider the use of aortic balloon valvotomy, and particularly for the neonates with a low cardiac output in order to avoid the myocardial stress and the neurological injury due to the cardiopulmonary bypass.
- Aortic Valve Replacement
- Aortic Regurgitation
- Hypoplastic Left Heart Syndrome
- Aortic Annulus
- Ross Procedure
Management of critical aortic stenosis in infants is still a controversial issue [1, 2]. When the patient is considered suitable for a biventricular repair, several options are available: conservative surgery including open heart valvotomy or, in some centers, closed transventricular aortic valvotomy ; or transcatheter balloon dilation.
During surgical open heart valvotomy, the surgeon has direct access to the morphology of the aortic valve and can adapt surgical strategy to individual valvular anatomy. In addition to dividing the zones of commissural fusion, thick and dysplastic leaflets can be thinned, improving valvular mobility. After surgery there is generally a low risk of aortic regurgitation with moderate residual gradient .
In contrast, transcatheter balloon dilation does not permit direct vision of the valve and tears aortic leaflets in a random way, usually at the weakest part of the valve and not necessarily at the level of the fused commissures. Despite its “blind” nature, transcatheter balloon dilation is now favored by most teams in the neonate and young infant as a first-choice procedure, because of its greater facility to carry out. After percutaneous balloon valvuloplasty, there is a higher risk of significant aortic regurgitation but generally a lower risk of residual stenosis. Progress in catheter procedures using new generation balloons of different sizes more adapted to the individual anatomic situation of the infant makes the difference between surgical commissurotomy and balloon valvuloplasty not evident [5–8].
Valvotomy of any kind is a palliative procedure, and reinterventions remain frequent .
In our institution, the approach to the neonate and young infant with critical aortic stenosis eligible for biventricular repair (size of aortic annulus not less than five millimeters) was almost exclusively open surgical valvotomy since 1994. Some percutaneous balloon valvuloplasties were performed from 1986 to 1993 and abandoned because of occurrence of acute aortic insufficiency.
The purpose of this study was to evaluate the results of this deliberate surgical approach, by analyzing the mid-term outcome after surgical valvotomy in young infants less than four months of age. In the current area of the percutaneous dilation of the aortic valve, we would like to know if we made the best choice of management of critical aortic stenosis.
Between August 1994 and April 2012, 83 consecutive patients younger than 4 months of age (19 girls, 64 boys) underwent open heart valvotomy for critical aortic stenosis in our institution. Median age was 17 days (range 0–111 days). Fifty-six patients (67 %) were neonates. Median weight at operation was 3.5 kg (range 1.8–6.9 kg).
Definition of study group
Critical aortic stenosis was an isolated lesion in 59 patients (71 %). Associated cardiac malformations were found in 24 patients (29 %), including multilevel left heart obstruction in 5, aortic coarctation in 8, atrial septal defect in 5, interruption of the aortic arch in 1 (IAA), ventricular septal defect in 2, mitral stenosis in 2, a mild form of hypoplastic left heart syndrome (HLHS) in 1, and supra valvular aortic stenosis with associated stenosis of the right pulmonary artery in 1.
The surgical threshold of intervention is a mean gradient ≥ 50 mmHg or less, if the patient presents a severe left ventricular dysfunction and/or an associated cardiac malformation requiring a concomitant surgery.
The neonate and young infant with critical aortic stenosis were eligible for biventricular repair if the size of the aortic annulus didn't measure less than five millimeters.
Nine patients were directly admitted in the intensive care unit because of cardiorespiratory failure. Eight needed ventilation support and nine needed inotropic support. Ductal dependency was present in 14 patients who required prostaglandin E1 treatment.
The malformation had been diagnosed prenatally in 14 patients (17 %).
The pressure gradient across the aortic valve was measured by transthoracic echocardiography coupled with continuous wave Doppler technique. Preoperative peak and mean aortic valve gradient were respectively 86 mmHg (range 20 to 190 mmHg) and 53 mmHg (range 14 to 106 mmHg). Aortic regurgitation grade ¼, assessed by color-flow Doppler, was present in two patients (2,4 %). Four patients had associated subaortic stenosis (4.8 %). Three of four patients with subaortic stenosis were included in the associated cardiac malformations described in definition of study group section (one aortic coarctation and two multilevel left heart obstructions) except one who has an isolated aortic stenosis.
The median z-score of the aortic annulus was −1.9 (range −7.5 to 1.6), the median z-score of the ascending aorta was 2.1 (range −3.8 to 4.7), the median z-score of left ventricle end-diastolic diameter (LVEDD) was 0.5 (range −3.9 to 7.2) .
Caution should be used with these measures because, on one hand, it's an historic series with a downstream calculation of z-score, and, on the other hand, according to the method of calculation of the z-score, the value of the latter can change a lot.
Thirty-seven patients had left ventricular dysfunction (45 %), defined as a shortening fraction (SF) < 30 %. The median SF was 35 % (range 8 to 61 %). Endocardial fibroelastosis (EFE) was present in 31 % of patients (determined by echocardiography and confirmed by the surgeon; The extend of EFE was not detailed).
Morphology of the aortic valve assessed at surgery
The aortic valve was bicuspid in 70 patients (84 %), monocuspid in seven patients (9 %) and tricuspid in six patients (7 %).
After median sternotomy, right atrial cannulation, and aortic cannulation, all patients were placed on normothermic cardiopulmonary bypass (CPB). Myocardial protection was obtained with hyper potassic antegrade blood repeated at ten minutes intervals; Through a transverse aortotomy, a careful commissurotomy of the aortic valve was performed in order to avoid appearance of aortic regurgitation (AR). Obstructive myxomatous and fibrous nodules were removed from the leaflets. Median CPB time was 42 min (range 13–260 min), median aortic cross-clamping time was 20 min (range 6–69 min).
Concomitant surgery (18 %) included aortic coarctation repair in 11 patients, IAA repair in one patient, closure of ventricular septal defect in 1, closure of atrial septal defect in 3, resection of supra-mitral membrane in 1, mitral commissurotomy in 1 and pulmonary artery banding in 1.
The surgeon had to enlarge the sinotubular junction with a patch in 17 patients (20.5 %) without supra-aortic stenosis described pre-operatively. The decision was taken by the surgeons in view of the observations during open heart valvotomy.
Delayed sternal closure was required in 16 patients (19 %).
Hospital survivors were examined in outpatient clinics at regular intervals by their pediatric cardiologist. Two-dimensional echocardiography and Doppler studies were routinely performed before discharge from the hospital and during each outpatient visit. The transvalvular aortic peak pressure gradient was calculated using the simplified Bernoulli equation. Color-flow Doppler imaging was used to analyze the degree of AR, using jet width and end-diastolic velocity of the retrograde flow in the descending aorta. The median follow-up was 4.2 years (range 1 day to 17.7 years). Five patients were lost of follow-up. Early death was defined as death in the hospital or within 30 days after the operation.
The data were imported into Stat Statview 5.0 SAS Institut Inc software. Risk factors for mortality and reintervention were studied by univariate analysis (with contingency coefficient (CC)), using the two-tailed paired Student’s t test and U of Mann–Whitney for the continuous variables and the χ2 test for nominal variables, and by multivariate analysis using a logistic regression model (with odds ratio (OR)). The actuarial method was used to determine event-free survival curves. The level of statistical significance was set at a p value of less than 0.05.
Early mortality and functional status
There were five (6 %) early deaths. Two patients with low cardiac output died on the first postoperative day. A third patient could have been converted to Norwood procedure but died on the first postoperative day because the parents refused the intervention. The last two patients died at 44 and 56 postoperative days due to respectively a severe tricuspid regurgitation with a restrictive mitral profile, and a low cardiac output. These five patients were critically ill preoperatively with severe ventricular dysfunction (two of them needed mechanical ventilation and inotropic support) among whom three needed a long cardiopulmonary bypass (147,161 et 190 min). Four of them required delayed sternal closure.
In fact, three of them were not eligible for a biventricular repair with a diameter of the aortic annulus which was less than five millimeters.
The 2D-echocardiography coupled with Doppler study showed a postoperative mean and maximal peak aortic valve gradient of respectively 24 mmHg (range 10 to 56 mmHg) and 37 mmHg (range 12 to 88 mmHg), significantly lower than before surgery (p‹0.0001). There were 58 % of AR grade 1-2/4 and one severe AR 3/4. Seven patients had a SF < 30 % (median 37,5 %, range 21 to 56 %), which was significantly lower than before surgery (7/77 (9 %) vs 45 %, p < 0.05).
Late mortality and reinterventions
There were six late deaths, with a global mortality of 13 % (64 % were neonates). Four of these late deaths occurred early after a second intervention: during Ross intervention in 1, on the first postoperative day after a Ross-Konno procedure because of low cardiac output in the second, five days after a Ross procedure associated with mitral valvuloplasty in the third one, and the fourth one after mitral valvotomy due to pulmonary hypertension. Two other patients died because of pulmonary hypertension : one four years after having two aortic valvotomies and a Ross procedure, and the last one less than a year after aortic valvotomy.
Restenosis in 27 patients (13 surgical valvotomies, 6 Ross procedures, 3 Ross-Konno, 5 balloon valvotomies). Concomitant surgery included 7 patches of enlargement of the sinotubular junction, 4 mitral valvotomies and 1 atrial septal defect closure.
Severe aortic regurgitation (AR grade ≥ 3) in 3 (2 Ross procedures and 1 Ross-Konno).
Association of aortic stenosis and aortic regurgitation in 7 (three AR grade 2, two AR grade 3 and two AR grade 4) with 6 Ross procedures and 1 surgical valvotomy. Concomitant interventions included 1 patch of enlargement of the sinotubular junction, 2 mitral and 1 tricuspid valvotomy and 1 resection of vegetation.
Isolated mitral stenosis in 1.
Eleven patients had a third intervention (15.5 %): 3 mitral surgical valvotomy, 1 balloon aortic valvotomy, 1 surgical aortic valvotomy with ventricular septal defect closure and resection of subvalvular aortic stenosis, 1 right ventricle to pulmonary artery Hancock conduit, 1 Ross-Konno procedure, 2 Ross procedures, 1 mechanical aortic valve replacement with enlargement of left pulmonary artery and 1 mechanical mitral valve replacement. There was no death among these patients.
Three patients had a fourth intervention (3.5 %): one left and right coronary artery bypass, one Ross procedure and one right ventricular to pulmonary artery conduit. There was no death.
There is no significant difference in survival, survival without re-intervention and survival without AVR/Ross between before and after 2008.
Risk factors for global mortality and reintervention
The risk factors studied were: aortic annulus diameter left ventricular dysfunction, SF, inotropic drugs, mechanical ventilation, neonatal period, weight, LVEDD, endocardial fibroelastosis, delayed sternal closure because of the higher risk of mediastinitis,, associated cardiac malformations and prenatal diagnosis.
The bicuspid valve was not studied as a risk factor because of its too high prevalence (84 % of infants had a bicuspid valve). Ventricular dysfunction (p = 0.04, CC = 0,2), delayed sternal closure (p = 0.007; OR = 21), endocardial fibroelastosis (p = 0.02, CC = 0,3), low z-score of the aortic annulus (p = 0.04, OR = 0,7) and prenatal diagnosis (p = 0,01; OR = 20) were found predictors of global mortality.
As regards prenatal diagnosis, the result must be interpreted with caution. The percentage of this latter was low (only 17 %) because it is an historic series and only the most severe forms were detected, what explains that the prenatal diagnosis was found predictor of global mortality.
Ventricular dysfunction (p = 0.01; OR = 4) and endocardial fibroelastosis (p = 0.04; OR = 0,3) were found predictors of re-intervention.
Long-term functional status
At the latest echocardiographic examination for the 50 patients still having their native aortic valve (69 % of survivors), aortic regurgitation was null in 10, grade 1 in 18, grade 2 in 18, grade 3 in 4. The mean and maximal peak aortic valve gradients were respectively 21 and 41 mmHg.
This is a retrospective single-center study with loss of data in the medical records.
The experience, in our center, on the management of critical aortic stenosis, shows a low early and late mortality (actuarial survival rate at 15 years: 85 %), but the aortic valvotomy is a palliative procedure and we see unfortunately a high rate of reintervention among which the aortic valve replacement. These results suggest to reconsider the use of aortic balloon valvotomy, and particularly for the neonates with a low cardiac output in order to avoid the myocardial stress and the neurological injury due to the cardiopulmonary bypass.
I would like to thank Marielle Gouton who helped me to perform a part of the statistics, and all the surgeons who worked in the Marie-Lannelongue Surgical Center: Emre Belli, Francois Lacour-Gayet, Emmanuel Lebret, Mohamed Ly, Claude Planchét, Régine Roussin and Alain Serraf.
Availability of data and material
CB collected the medical data and drafted the manuscript; AC analyzed and interpreted the patient data; LH helped to draft the manuscript; EB suggested the idea of the article; all authors read and approved the final manuscript.
The authors declare that they have no competing interests.
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