Summary
Background
Long-term survival and risk of reoperation in “non-Marfan syndrome” patients with a long life expectancy who undergo emergency surgery for acute type A aortic dissection (aTAAD) are not well known.
Aim
To analyse survival, risk of reoperation and quality of life in this population.
Methods
From 1990 to 2010, all patients aged ≤ 50 years and not affected by Marfan syndrome, who underwent emergency surgery for aTAAD at two institutions, were included in this analysis. Patients were categorized into four groups according to the extension of the aortic replacement: SUPRACORONARY, ROOT, ARCH and EXTENSIVE.
Results
Sixty-six patients (mean age 45 ± 4 years; range 34–50 years) were considered eligible for this analysis. Overall in-hospital mortality was 24% (16/66 patients); and 25%, 23%, 20.5% and 43% in the SUPRACORONARY, ROOT, ARCH and EXTENSIVE groups, respectively. Mean follow-up among survivors was 10.5 ± 7.2 years (range: 0.1–24.7 years). Overall 10-year survival was 55 ± 6%; and 75 ± 12%, 69 ± 13%, 47 ± 8% and 28 ± 17% in the SUPRACORONARY, ROOT, ARCH and EXTENSIVE groups, respectively. Overall freedom from reoperation on the aorta was 73 ± 7.5%; and 40 ± 20%, 75 ± 21%, 78 ± 8% and 100% in the SUPRACORONARY, ROOT, ARCH and EXTENSIVE groups, respectively.
Conclusions
In our experience, patients who underwent isolated supracoronary ascending aorta or root replacement showed the most satisfactory late survival. However, because the risk of reoperation is low when the replacement is extended to the root, our data suggest that root replacement could represent a good compromise between operative mortality and long-term survival.
Résumé
Contexte
Les résultats à très long terme après chirurgie pour dissection aortique aiguë de type A chez le patient avec une longue espérance de vie non atteint du syndrome de Marfan sont peu connus.
Objectifs
Nous analysons la survie et le risque de réintervention dans cette population.
Méthodes
Nous avons analysé rétrospectivement tous les patients âgés de moins de 50 ans, non atteints du syndrome de Marfan et opérés d’une dissection aortique aiguë de type A dans deux hôpitaux, entre 1990 et 2010. Les patients ont été repartis en 4 groupes selon l’extension du remplacement aortique : SUS-CORONAIRE, RACINE (étendu que à la racine), ARCHE (étendu que à l’arche) et ETENDU (de la racine à l’arche).
Résultats
Soixante-six patients (âge moyen : 45 ± 4 ans; de 34 à 50 ans) ont été inclus dans cette analyse. La mortalité hospitalière globale était de 24 % (16/66 patients) et de 25 %, 23 %, 20,5 % et 43 % dans les groupes SUS-CORONAIRE, RACINE, ARCHE et ETENDU, respectivement. Le suivi moyen parmi les survivants était de 10,5 ± 7,2 ans (de 0,1 à 24,7 ans). La survie à 10 ans (mortalité hospitalière incluse) était de 55 ± 6 %, 75 ± 12 %, 69 ± 13 %, 47 ± 8 % et 28 ± 17 %, dans les groupes SUS-CORONAIRE, RACINE, ARCHE et ETENDU, respectivement. La liberté d’une réintervention sur l’aorte à 10 ans était de 73 ± 7,5 %, 40 ± 20 %, 75 ± 21 %, 78 ± 8 %, dans les groupes SUS-CORONAIRE, RACINE, ARCHE et ETENDU, respectivement.
Conclusion
Dans notre série, les patients qui ont bénéficié d’un remplacement de l’aorte sus-coronaire ou d’un remplacement de la racine aortique présentent la meilleure survie au long terme. Toutefois, puisque le risque de réintervention est faible après remplacement de la racine aortique, nos données suggèrent que le remplacement de la racine aortique semble être un bon compromis entre la mortalité hospitalière et la survie au long terme.
Background
Long-term outcomes after surgery for acute type A aortic dissection (aTAAD) in patients with a long life expectancy are unclear. While survival after surgical repair has been thoroughly investigated, studies reporting long-term follow-up in the young (mean follow-up > 5 years) are anecdotal, and included a variable proportion of patients affected by Marfan syndrome .
Management of aTAAD in Marfan syndrome patients is unanimous. Extensive aortic replacement has been suggested to improve survival and minimize the risk of reoperation at late follow-up. However, survival of non-Marfan syndrome patients with long life expectancy has not been analysed extensively. For these patients, it is still unclear whether a more extensive aortic replacement is justified to improve overall survival.
We reviewed our 20-year experience in the management of aTAAD, with the aim of analysing the impact of surgical aggressiveness on very-long-term survival and risk of reoperation in non-Marfan syndrome patients with a long life expectancy.
Methods
From January 1990 to December 2010, 554 patients underwent emergency surgery for aTAAD at the “Dupuytren” University Hospital (Limoges, France) and at the “San Martino” University Hospital (Genova, Italy). All patients aged ≤ 50 years were screened initially; among these, patients affected by Marfan syndrome were excluded. The diagnosis of Marfan syndrome was based on clinical criteria (the Ghent nosology). None of the patients underwent DNA testing at our institution for FBN1 mutation.
The Institutional Ethics Committee approved the study protocol, and authorized its conduction and follow-up. Individual patient consent was obtained for the study.
Data collection
Data on patient demographics, medical and surgical treatment details and in-hospital patient outcomes were collected by physicians, and were obtained by review of hospital records ( Appendix 1 ). Yearly follow-up data were obtained by clinical examination or by telephone interview. No patient was lost at the last follow-up (01 January 2015). Data were forwarded to the coordinating physician (A.P.); forms were then reviewed and validated before statistical analysis.
Data analysis
Data analysis was performed using the JMP statistical analysis software (SAS Institute, Cary, NC, USA). Continuous variables are presented as means ± standard deviations and categorical variables are expressed as frequencies.
For the purpose of this analysis, we categorized patients into four groups, according to the extension of the aortic replacement. The SUPRACORONARY group included all patients who underwent isolated supracoronary ascending aorta replacement; in these patients the distal anastomosis was realized without removing the aortic clamp. The ROOT group included all patients in whom the replacement supracoronary ascending aorta was extended to the root; in these patients the distal anastomosis was realized without removing the aortic clamp. The ARCH group included all patients in whom the replacement of the supracoronary ascending aorta was extended to the concavity of the arch or to the proximal hemiarch or to the whole aortic arch. The EXTENSIVE group included all patients who underwent replacement of the aorta from the root to the concavity of the arch or to the proximal hemiarch or to the whole aortic arch.
Continuous variables were compared using Student’s t -test or the Wilcoxon rank-sum test. Categorical variables were compared using the χ 2 test or Fisher’s exact test (two-tailed test) if the expected count in any cell was < 5.
For the multivariable analysis, all variables that reached a P value ≤ 0.2 in the univariate analysis were then included in a stepwise logistic regression, provided they were present in ≥ 2% of the samples. Retention of risk variables was determined by using the likelihood ratio test. We considered a P value < 0.05 to be significant.
Survival was determined by the Kaplan–Meier method, and is expressed as the proportion ± standard error.
Results
Study population
During the study period, 654 patients underwent surgery for aTAAD at the participating centres; among these, 79 (12%) were aged ≤ 50 years, and 66 patients (10%) were considered eligible for this analysis. Table 1 details demographics, history and clinical presentation for our patient population.
| Variables | Overall ( n = 79) | Marfan ( n = 13) | Non-Marfan ( n = 66) | P |
|---|---|---|---|---|
| Demographics | ||||
| Age (years) | 43.5 ± 6 (22.5–50) | 34.5 ± 4.5 (22.5–43) | 45 ± 4 (34–50) | < 0.0001 |
| Age ≤ 40 years | 24 (30.5) | 12 (92) | 12 (18) | < 0.0001 |
| Men | 59 (74.5) | 11 (84.5) | 48 (72.5) | 0.35 |
| Patient history | ||||
| Hypertension | 69 (87.5) | 7 (54) | 62 (94) | < 0.0001 |
| Obesity | 6 (7.5) | 0 | 6 (9) | 0.13 |
| Diabetes | 3 (4) | 0 | 3 (4.5) | 0.29 |
| Smoker | 23 (29) | 5 (38.5) | 18 (27.5) | 0.43 |
| Peripheral vascular disease | 0 | 0 | 0 | |
| Coronary artery disease | 0 | 0 | 0 | |
| COPD | 0 | 0 | 0 | |
| Previous cardiac surgery | 4 (5) | 0 | 4 (6) | 0.22 |
| Clinical presentation | ||||
| Complicated dissection | 14 (17.5) | 2 (15.5) | 12 (18) | 0.81 |
| All neurologic deficits | 5 (6) | 0 | 5 (7.5) | 0.17 |
| Coma | 1 (1) | 0 | 1 (1.5) | 0.55 |
| Stroke | 4 (5) | 0 | 4 (6) | 0.22 |
| Paraplegia | 1 (1) | 0 | 1 (1.5) | 0.55 |
| Haemodynamic compromise | 7 (9) | 2 (15.5) | 5 (7.5) | 0.40 |
| Mesenteric ischaemia | 0 | 0 | 0 | |
| Cardiopulmonary resuscitation | 0 | 0 | 0 | |
| Aortic replacement | ||||
| SUPRACORONARY group | ||||
| Isolated ascending aorta | 14 (17.5) | 2 (15.5) | 12 (18) | 0.81 |
| ROOT group | ||||
| Ascending aorta + root | 18 (22.5) | 5 (38.5) | 13 (19.5) | 0.16 |
| ARCH group | ||||
| Ascending aorta + open distal anastomosis | 27 (34) | 3 (23) | 24 (38) | 0.29 |
| Ascending aorta + arch | 11 (14) | 1 (7.5) | 10 (15) | 0.45 |
| EXTENSIVE group | ||||
| Ascending aorta + root + open distal anastomosis | 4 (3.5) | 1 (7.5) | 3 (4.5) | 0.65 |
| Ascending aorta + root + arch | 5 (6) | 1 (7.5) | 4 (6) | 0.83 |
| Operative data | ||||
| Femoral artery cannulation | 63 (80) | 10 (77) | 53 (80) | 0.78 |
| Aortic arch cannulation | 2 (2.5) | 0 | 2 (3) | 0.39 |
| Right subclavian artery cannulation | 14 (17.5) | 3 (23) | 11 (16.5) | 0.59 |
| Cardiocirculatory arrest | 47 (59.5) | 6 (46) | 41 (62) | 0.29 |
| Cross-clamping time (minutes) | 111 ± 66 | 120 ± 48 | 110 ± 45 | 0.54 |
| CPB time (minutes) | 199 ± 80 | 208 ± 68 | 198 ± 82 | 0.74 |
| Associated procedure | 13 (16.5) | 3 (23) | 10 (15) | 0.50 |
| Aortic valve replacement | 7 (9) | 1 (7.5) | 6 (9) | 0.87 |
| CABG | 7 (9) | 2 (15.5) | 5 (7.5) | 0.40 |
| Postoperative complications | ||||
| All complications | 37 (47) | 5 (38.5) | 32 (48.5) | 0.51 |
| Haemodiafiltration | 2 (2.5) | 0 | 2 (3) | 0.39 |
| Re-exploration for bleeding | 18 (23) | 2 (15.5) | 16 (24) | 0.47 |
| Neurological | 10 (12.5) | 0 | 10 (15) | 0.0491 |
| Cardiac arrest | 7 (9) | 0 | 7 (10.5) | 0.10 |
| ECMO | 4 (5) | 1 (7.5) | 3 (4.5) | 0.65 |
| Sepsis | 13 (16.5) | 3 (23) | 10 (15) | 0.50 |
| Tracheotomy | 1 (1) | 0 | 1 (1.5) | 0.55 |
| MOF | 4 (5) | 0 | 4 (6) | 0.22 |
| In-hospital mortality | 17 (21.5) | 1 (7.5) | 16 (24) | 0.18 |
The mean age was 45 ± 4 years (range 34–50 years); 12 patients (18%) were young. The majority of patients had a history of hypertension, and a third of patients were active smokers. Four patients (6%) had a history of previous cardiac surgery (aortic valve replacement in two patients, mitral valve replacement in one patient and atrial septal defect closure in one patient), at least 3 years before the acute aortic dissection.
Twelve patients (18%) presented a complicated dissection because of a preoperative neurological deficit and/or haemodynamic compromise.
Table 2 details demographics, history and clinical presentation according to the extension of the aortic replacement. The distribution of age was similar among the groups ( P = 0.57). However, patients in the SUPRACORONARY group were younger because a third were aged < 40 years. Patients in the SUPRACORONARY and EXTENSIVE groups had no history of previous cardiac surgery. Patients in the ROOT and EXTENSIVE groups had no neurological deficit before the surgery.
| Variables | SUPRACORONARY ( n = 12) | ROOT ( n = 13) | ARCH ( n = 34) | EXTENSIVE ( n = 7) | P |
|---|---|---|---|---|---|
| Demographics | |||||
| Age (years) | 43.8 ± 4 | 45.9 ± 5 | 45.7 ± 4 | 45.2 ± 4 | 0.57 |
| Age ≤ 40 years | 4 (33) | 2 (15.5) | 5 (14.5) | 1 (14) | 0.57 |
| Men | 6 (50) | 12 (92.5) | 26 (76.5) | 4 (57) | 0.07 |
| Patient history | |||||
| Hypertension | 12 (100) | 13 (100) | 30 (88) | 7 (100) | 0.14 |
| Obesity | 1 (8.5) | 0 | 3 (9) | 2 (28.5) | 0.20 |
| Diabetes | 0 | 0 | 3 (9) | 0 | 0.25 |
| Smoker | 4 (33) | 3 (23) | 10 (29.5) | 1 (14.5) | 0.78 |
| Previous cardiac surgery | 0 | 1 (7.5) | 3 (9) | 0 | 0.42 |
| Clinical presentation | |||||
| Complicated dissection | 2 (16.6) | 2 (15.5) | 7 (20.5) | 1 (14.5) | 0.96 |
| All neurologic deficits | 1 (8.5) | 0 | 4 (11.5) | 0 | 0.27 |
| Haemodynamic compromise | 1 (8.5) | 2 (15.5) | 1 (3) | 1 (15.5) | 0.46 |
| Operative data | |||||
| Femoral artery cannulation | 12 (100) | 13 (100) | 24 (70.5) | 4 (57) | 0.0021 |
| Aortic arch cannulation | 0 | 0 | 1 (3) | 1 (14.5) | 0.37 |
| Right subclavian artery cannulation | 0 | 0 | 9 (26.5) | 2 (28.5) | 0.0081 |
| Cross-clamping time (minutes) | 102 ± 21 | 131 ± 54 | 103 ± 44 | 122 ± 55 | 0.36 |
| CPB time (minutes) | 122 ± 45 | 186 ± 80 | 209 ± 84 | 247 ± 56 | 0.0153 |
| Associated procedure | 3 (25) | 2 (15.5) | 4 (11.5) | 1 (14.5) | 0.77 |
| Aortic valve replacement | 3 (25) | 0 | 3 (9) | 0 | 0.09 |
| CABG | 1 (8.5) | 2 (15.5) | 1 (3) | 1 (14.5) | 0.46 |
| Postoperative complications | |||||
| All complications | 5 (41.5) | 7 (54) | 15 (44) | 5 (71.5) | 0.54 |
| Haemodiafiltration | 1 (8.5) | 0 | 0 | 1 (14.5) | 0.15 |
| Re-exploration for bleeding | 1 (8.5) | 2 (15.5) | 10 (29.5) | 3 (43) | 0.23 |
| Neurological | 1 (8.5) | 4 (30.5) | 4 (11.5) | 1 (14.5) | 0.42 |
| Cardiac arrest | 2 (16.5) | 1 (7.5) | 4 (11.5) | 0 | 0.54 |
| ECMO | 1 (8.5) | 2 (15.5) | 0 | 0 | 0.10 |
| Sepsis | 2 (16.5) | 1 (7.5) | 4 (11.5) | 3 (43) | 0.25 |
| Tracheotomy | 0 | 0 | 0 | 1 (14.5) | 0.20 |
| MOF | 0 | 2 (15.5) | 2 (6) | 0 | 0.28 |
| In-hospital mortality | 3 (25) | 3 (23) | 7 (20.5) | 3 (43) | 0.70 |
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