Bicuspid Aortic Valve Disease : a 10 Year Latin-american Experience

doi:10.21203/rs.3.rs-1982047/v1

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Bicuspid Aortic Valve Disease : a 10 Year Latin-american Experience

https://doi.org/10.21203/rs.3.rs-1982047/v1

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Background

Bicuspid aortic valve is one of the most common congenital heart malformations affecting 2% of the population. There is a gap in the literature regarding the Latin-American population, the aim of this study is to analyze the demographic, clinical characteristics and the surgical outcomes of patients with bicuspid aortic valve (BAV) with a 1,5 and 10 year follow-up in a single center.

Methods

We performed a retrospective review of a prospectively collected database. All patients that underwent aortic-valve related procedures with bicuspid aortic valve finding between 2011-2021 were included. Comparison between groups were performed using paired t-test or Mann Whitney-Wilcoxon when appropriate. Statistically significant value was accepted if < 0.05.

Results: 82 patients were included. Male patients constituted 71.95% of the population. Median age was 58 years (IQR 47;65). Type 1 BAV were the most common finding (85.19%). Aortic stenosis was the most common clinical feature in 64.64% of the cases. Coronary artery disease and smoking history are related with decreased left ventricular function with statistically significant value (p 0.02 - 0.03 respectively). Mortality rate was 2.44. Overall survival rate after 1-5 and 10 years was 97.56%, 96.42%, and 80.5% respectively.

Conclusions: Patients with associated coronary artery disease and smoking history should be considered high risk patients due to the relationship with decreased left ventricular function even with an asymptomatic course. Further prospective studies are needed to prove our results.

Aortic Valve surgery

Bicuspid Aortic Valve Disease

Heart valve diseases

congenital heart disease

Bicuspid aortic valve (BAV) is one of the most common congenital heart malformations affecting 1-2% of the population [1-8] . Of the 41,000 newborns diagnosed with some type of congenital malformation that requires surgical intervention for treatment, only 41% (17,000) receive adequate and specific care, without follow-up data on the remaining population [9-12]. In South America, congenital heart abnormalities occurs in 28 cases per 10,000 live births, causing around 54,000 cases per year [11] and in Colombia, approximately 3% of live births have any congenital heart disease [11, 12] with unfavorable outcomes in childhood [12] increasing health care costs; with approximately 3.2 million annual disabilities [11].

It's considered a serious clinical condition with diversity in terms of clinical presentation, related conditions and risk factors [1,2], with a progressive clinical spectrum that could vary from the asymptomatic course in the first years of life, to the development of valvular disease, generally due to calcified stenosis, (up to 85% as described in postoperative pathological study cohorts) [1-3,9-12] with or without coexisting insufficiency in adult life [2, 3, 13]. BAV is not only related to valvular disease, there is evidence of the existing linear relationship between the course with bicuspid valve and a higher risk of developing thoracic arteriopathies such as dissection, aneurysm and coarctation [1, 3, 5, 11].

Additionally, the clinical history of BAV constitutes an identified risk factor for the development of endocarditis, although there is no clarity on the mechanism that explains this susceptibility [1, 6, 13-15], there is a described relation to the turbulent flow due to the valve arrangement, which achieves anatomical substrates to the development of the infection [1, 2, 13]. The aspects of the disease can follow a course of continuous deterioration and can even debut with heart failure and sudden death [15-20].

Nowadays there is a scarce in the literature regarding the incidence, clinical characteristics and postoperative outcomes of patients with BAV who underwent surgery [16-30], evidence is limited to multiple descriptive cohorts of pathological findings in necropsies, and relatively few prospective studies that describes the natural history of with limited follow-up in North-American and European population [28-35]. Considering that there is limited evidence in the literature regarding the Latin-American population, the aim of this retrospective study is to describe the demographic, clinical characteristics and the surgical outcomes of patients with BAV with a 10 year follow-up in a single center.

Study population

With the Institutional Review Board’s approval and following HIPAA act guidelines, a retrospective review of a prospectively collected database was conducted. All patients that underwent aortic-valve related procedures with bicuspid aortic valve finding between 2011-2021 were included. Patients with missing data, and follow-up < 1 month were excluded.

All patients received standard anesthetic induction with a combination of fentanyl 5-10 μg/kg and propofol 0.1 mg/kg. Pancuronium 0.1 mg/kg for neuromuscular blockade. Anesthesia was maintained with sevoflurane and fentanyl supplements. The patients received heparin 400 U/kg before extracorporeal circulation and the ACT was maintained above 400. Patients were cannulated with an aortic cannula and a single two-stage venous cannula. Retrograde blood cold (6°C) cardioplegia solution was administered. Systemic temperature was allowed to drift to 34 –36°C during CBP.

Ethical compliance with the Helsinki Declaration, current legislation on research Res. 008430-1993 and Res. 2378-2008 (Colombia), and the ICMJE were ensured under our IRB approval.

Data assessment

Patients' demographics and associated comorbidities were evaluated. Clinical variables include preoperative weight, duration of symptoms until surgical procedure, heart failure at the time of surgery, type of preoperative diagnosis, left ventricular function and stenosis gradient (preoperative echocardiogram). Operative variables including, type and urgency of surgical procedure, type of bicuspid aortic valve, perfusion and ischemic time were evaluated. Postoperative mortality was evaluated at 30 days follow up, and survival rate up to 1, 5 and 10 years after surgery was defined according to the national database.

Statistical analysis

Data descriptions were evaluated according to the nature and distribution of the variable. Kurtosis/Skewness tests were performed to determine the distribution of continuous variables and were summarized by means/standard deviation in cases of normal distribution and medians/interquartile ranges for cases of non-normal distribution. Frequency and proportion were provided for categorical data. Differences between groups were evaluated using a paired t-test or a Mann-Withney Willcoxon test when appropriate, fisher exact test or chi-square were used according to the nature of the variables, statistical significance were reached when p value is < 0.05. Data were analyzed using STATA 17 licensed version.

Pre-operative and clinical data.

Between 2011-2021 a total of 322 patients underwent aortic-valve related surgical procedures including valvular change, bentall procedure, and valvular change plus supracoronary aortic replacement. And a total of 82 patients with bicuspid aortic valves who underwent surgical aortic-related procedures were included.

Male patients constituted the majority of the population (71.95% n = 59), median age was 58 years (IQR 47;65), median weight was 70 kg (IQR 62;76). In terms of comorbidities, high blood pressure (HPB) was the most frequent in 86.59% (n=71), followed by smoking habit in 53.66% (n=44) and chronic obstructive pulmonary disease (COPD) in 26.83% (n=22) of the patients. (See appendix table 1). Duration of symptoms prior to surgery were evaluated with a median time of 24 months (IQR 12;48) and in most of the cases heart failure (HF) were evidenced at the time of the procedure (63.41% n = 52) and just 10.98% (n=9) of the population presented with syncope.

The most frequent preoperative diagnosis was a double aortic disease in 34.15% (n = 28) of the population followed by an aortic stenosis in 30.49% (n = 25), aortic insufficiency were evidenced in 15.85% (n= 13), aortic aneurysm were identified in 18.29% (n= 15) (See appendix table 1). In cases of stenosis pressure gradient of aortic valve were evaluated, with a mean of 73.89 ± 17.21 mmHg, left ventricular ejection function (LVEF) was assessed according to preoperative echocardiography, with a mean of 55.48 % (SD 11.23).

Surgical characteristics and outcomes

Valvular replacement was the most frequent surgery performed in 78.05% (n=64) of the patients, followed by Bentall procedure in 13.41% (n=11). Emergency procedures were performed only in 1.22% (n=1) of the cases. According to intraoperative findings, the most frequent type was fused bicuspid aortic valve in 84.15% (n = 69), followed by the “two raphes” valve in 13.41% (n =11) of the patients. Mean ischemic time was 82.73 ± 23.25 minutes; perfusion time median was 98.5 minutes (IQR 83;116). Mortality rate at 30 day follow-up was 2.44% (n=2), in one case due to cardiac tamponade and in the second one due to right heart failure. Survival rate was 97.56%, 96.42%, and 80.5% at 1, 5 and 10 years of follow up.

Clinical Features

Type 2 bicuspid aortic valve is an uncommon finding in the female population (9.09% n=1). Mean age was different across the type of the valves, for type 1 mean age was 59.04 years (SD 1.16), type 2 mean age was 26.90 years (SD 3.28) and for type 3 mean was 47 years (SD 12). Duration of symptoms were analyzed as well, for type 1 cases mean duration of symptoms was 32.43 months (SD 2.45), for type 2 patients 35.63 months (SD 10.18) and for type 3 cases 54 months (SD 18).

For patients with type 1 bicuspid aortic valve congestive heart failure is evidenced in 65.21 % (n = 45) of the cases, in type 2 cases in 54.54 % (n = 6) of the patients and in type 3 patients in 50% of the times (n = 1) . Syncope it’s only evidenced for patients with type 1 bicuspid aortic valve in 13.04% (n = 9) of the cases.

Majority of the patients with type 1 and type 2 bicuspid aortic valve present double aortic injury (23/69 vs 5/11) and aortic stenosis (22/69 vs 3/11). In cases of type 3, 100% (n = 2) of the patients suffer aortic insufficiency. Preoperative left heart function was evaluated according to the type of the valve. Type 1 cases have a mean of 54.53 % (SD 1.47), type 2 mean was 60.54% (SD 2.91) and type 3 60.5% (SD 3.5).

Regarding the risk factors, and their distribution in the different subtypes, in the three types there were patients with a history of high HPB. History of CAD, DM and CKD was limited to type 1. (Figure 2.)

Preoperative left ventricular function (LVEF)

Echocardiographic findings were evaluated according to the clinical course (symptomatic or not) and with associated comorbidities.

LVEF were higher in the asymptomatic group (Asymptomatic group mean 60.57% vs symptomatic group mean 53.12%) with statistical significance (p 0.001). Patients with HF symptoms have mean difference of 8.85% in LVEF compared with asymptomatic patients with statistical significance (p 0.002), in terms of syncope there is no relationship between the LVEF and the presence of this clinical feature (Syncope group mean 62% vs non-syncope group 54.68% p value 0.96).

In terms of comorbidities, patients with a previous history of coronary artery disease (CAD) have significant differences in terms of LVEF with a mean difference of 7.7% (CAD mean 48.91% vs non-coronary artery disease mean 56.61%) with a p value of 0.02. Non-smokers patients have a higher LVEF compared with smokers (mean 57.97% vs 53.34% respectively) with statistical significant value (p 0.03). HPB, COPD, type 2 diabetes mellitus (DM), and chronic kidney disease (CKD) do not show statistical differences in terms of LVEF.

Pressure Gradient (PG)

Asymptomatic patients have lower PG compared with patients with any clinical feature (Asymptomatic group mean 66.36 mmhg vs symptomatic group mean 78.76 mmhg) with a p value of 0.03. There is no statistical difference in terms of PG in terms of HF (Congestive heart failure group mean 76.81mmhg vs non-congestive heart failure group mean 70 mmhg p value 0.3), however patients who present syncope shows a higher GP compared with non-syncope patients with statistical significance (mean 93 mmhg vs mean 71.6 mmhg respectively p value 0.03)

In terms of comorbidities there is no statistical relationship between the variables and the pressure gradient. (See Table 3,)

Regarding the gap in the literature about the epidemiology, clinical characteristics of patients with BAV and surgical outcomes following aortic-valve related procedures in patients with this congenital disease, this study enhances the understanding of this pathology in the Latin-American population.

Aortic-related procedures in patients with BAV were more frequent in male population (71.05% n=59%), similar evidence that the one reported by Michelena et al [9,25] who report a male to female ratio of 3:1. Usually patients become symptomatic at the age of 50-60 [9,35] years in cases of aortic stenosis and younger for patients with aortic insufficiency; data that it’s comparable with our results that shows a median age of presentation at surgery of 58 years, nevertheless gender differences are exposed in the literature in some studies [36] that shows that female patients were older and with more clinical features in the general analysis compared with male population with statistical differences; our data it’s in line with this findings, in the population analyzed female patients were approximately 7 years older than men with statistical significance (Male age mean 52.62 years vs Female age mean 59.08 years p value 0.03).

In terms of clinical related conditions, our data reveals that majority of the patients presented with BAV have high blood pressure (86.59%) and with smoking habit (53.66%) this data isn’t far to the one reported by Granath et al [36] who also evidence that HBP is the most common comorbidity associated in 41.9% of the population. Interestingly, the same study shows that there is a low proportion of patients with type 2 diabetes mellitus with associated BAV in just 8.9% of the population analyzed, comparable results with our findings in which only 9.7% of the patients had type 2 diabetes mellitus.

BAV is also associated with related aortic pathologies, such as aortic stenosis, aortic insufficiency, infective endocarditis, and aneurysm formations [37]. Aortic stenosis (AS), in several studies is recognized as the most frequent clinical feature in patients with BAV who require surgical procedures, according to Granath et al [36], 66.6% of the population with BAV have aortic stenosis associated, and just 26.4% of aortic insufficiency. This data is similar to the one evidenced in our study, in which 64.64% of the patients have associated stenosis, and 15.85% have aortic insufficiency. Aortic aneurysm is also evidenced in the 50% of the population with BAV, and it’s described an 8-fold higher risk to present aortic dissection compared with the general population, and it's frequently related to connective tissue disorders.[30-37], in our population just 18.29% of the patients have an associated aneurysm who underwent surgical repair. Infected endocarditis rate in patients with BAV is high compared with general population, with a reported incidence between 2-5% and for that reason some studies recommend the use of prophylactic antibiotic regimen [4,35,38], our data report a 1.22% rate of infective endocarditis associated with BAV who required aortic valve replacement, data that similar to the world-wide literature [4,35,38].

Types of BAV are widely described, according to Michelena et al [25] in the international consensus statement about BAV nomenclature, the most frequent described type of BAV it’s the “Fused bicuspid aortic valve” (Or type 1) with 90-95% of the cases, following the “two sinus BAV” (Or type 2), in 5-7% of the population, and the “partial fusion BAV” with a underestimated incidence due to the early recognition [25]. Type 1 BAV in the Latin-american population remains to be the most common finding in 84.15% followed by type 2 in 13.41% and just 2.44% of the patients have type 3 BAV. Our study analyzed the pattern differences between the bicuspid aortic valve types; type 2 BAV its most common finding in female population; and in terms of clinical features, congestive heart failure it’s a common finding in the three types of BAV however with an increased proportion for type 1 patients (65.21%).

30-day follow up mortality in patients with BAV who underwent aortic-valve related procedures is between 0.4-2% according to some retrospective studies [36,39,40]. Our population mortality rate was 2.44% (n=2) data that it’s comparable with the one reported by Svensson et al who report a 2% of postoperative mortality in this group of patients [41]. Also, our study provides a 1-, 5- and 10-year follow-up, with survival rate of 97.56%, 96.42%, and 80.5% respectively. Regarding long-term follow up, Masri et al [35] report a 78% of freedom of re-operation and mortality after 10 years, data that it’s similar to our findings in which at 10 years, 80.5% of the patients survived with no requirement of re-operation.

Echocardiographic findings are also evaluated in several studies who evaluate clinical characteristics of patients with BAV [35]. Masri et al. [35] shows a mean left ventricular ejection fraction in the overall population of 55% (SD 8%), these results are similar to the one evidenced by our study (55.48% SD 11.23). Even though, our comparison between symptomatic vs asymptomatic groups shows that there is a difference of more than 7% of LVEF and symptoms starts with preserved left heart function according to international definitions [42] this data suggest that surgery may be performed earlier in patients with BAV even with preserved LVEF. According to our data, patients with smoking history and coronary disease have significant differences in terms of LEVF with statistical significant value and suggest this patients should be considered as high risk patients due to the association with decreased LVEF and increased risk of complications, those data it’s comparable with the one reported by Alshehri et al [43] who evidence significant alterations of ventricular functions in smoker patients.

Syncope it’s a well known clinical feature of aortic stenosis, and it’s related to a decrease in the blood flow of vertebral and carotid arteries [44], some studies evaluate the relationship between the LVEF and the presence of syncope and conclude that lower left ventricular with associated arrhythmias are related with syncopal episodes [44], in our study, LVEF do not differ between syncope/non-syncope group, nevertheless, there is a relationship between the pressure gradient and the presence of syncope with statistical relationship (p = 0.03), this data suggest that increased pressure gradients will decrease vertebral and carotid flows leading to neurologic symptoms such as syncope in patients with aortic stenosis and BAV.

Among the limitations of our study are the retrospective nature, and the single-center experience, therefore the results in terms of incidence could change. However, the strengths of our study it’s that to the best of our knowledge this is the first study that analyzes the prevalence, clinical characteristics and surgical outcomes of patients with BAV in Latin-America with a long term follow-up of 10 years.

Surgical procedures associated with BAV offer good results in short-mid and long term follow up. With limited rate of mortality and morbidity. According to our data, patients with coronary artery disease and smoking history should be considered as high risk patients due to the relationship with decreased LVEF even in asymptomatic patients. Elevated pressure gradient is related with syncope in patients with aortic stenosis. Our study successfully characterizes the preoperative clinical features and surgical outcomes with a 10 year follow up in latin america, however reflects the needing of long-term follow-up to patients with BAV in their childhood in order to secure and early treatment. Further prospective studies are needed to prove our results.

ACT = Activated coagulation time

BAV = Bicuspid aortic valve

CAD = Coronary artery disease

CKD = Chronic kidney disease

COPD = chronic obstructive pulmonary disease

DM = Diabetes Mellitus

HIPAA = Health Insurance Portability and Accountability Act

HF = Heart failure

HPB = High Blood Pressure

ICMJE = International Committee of Medical Journal Editors

IRB = Ethics and Research Institutional Committee

MG= Mean Gradient

LVEF = Left ventricular ejection

Acknowledgments

To our patients.

Ethics approval and consent to participate: Following approval of our Institutional Review Board and ethical committee (Hospital Universitario San Ignacio), all procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee, and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in the study.

Consent for publication

Does not apply.

Availability of data and materials

The datasets used and/or analyzed during the current study are available from the corresponding author upon reasonable request.

Competing interest

None of the authors declare any conflict of interest.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Author’s contributions:

P.T.G. had the research idea.

P.T.G., C.R.. participated in drafting the article and revised it critically for important intellectual content.

P.T.G., C.R, J.C, E.R. made substantial contributions to the conception and design, acquisition of data, analysis, and interpretation of data.

E.R. gave the final approval to the manuscript as principal researcher and Chief and Chairman.

Statement of conflict of interest

There is no conflict of interest of any of the listed authors.

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Table 1. Pre-operative characteristics

Variable	Result
Gender % (n)
Male	71.95 (59)
Female	28.05 (23)
Age median (IQR); years	58.5 (47;65)
Weight median (IQR); kilograms	70 (62;76)
Comorbidities % (n)
Chronic Renal Impairment	2.44 (2)
Coronary Arterial Disease (CAD)	14.63 (12)
Type 2 Diabetes Melllitus (DM)	9.76 (8)
Active Smoking habit	10.98 (9)
Chronic obstructive pulmonary disease (COPD)	26.83 (22)
History of Smoking	53.66 (44)
Arterial hypertension (HPB)	86.59 (71)
Clinical characteristics
Duration of symptoms median (IQR) Months	24 (12;48)
Congestive heart failure (HF) % ( n )	64.41 (52)
Syncope	10.98 (9)
Preoperative diagnosis % (n)
Aortic Stenosis	30.49 (25)
Aortic Insufficiency	15.85 (13)
Double aortic injury	34.15 (28)
Aortic aneurysm + Aortic Insufficiency	14.63 (12)
Aortic aneursym + Double aortic injury	3.66 (3)
Double aortic injury + endocarditis	1.22 (1)
Echocardiographic findings
Left ventricular function mean (LVEF) (SD) %	55.48 (11.23)
Pressure gradient mean (SD) mmHg	73.89 (17.21)

Table 2. Operative characteristics

Variable	Result
Type of procedure % (n)
Valvular replacement	78.05 (64)
Valvular replacement + Supracoronary aortic replacement	8.54 (7)
Bentall	13.41 (11)
Emergency procedure	1.22 (1)
Type of bicuspid aortic valve
Type 1	84.15 (69)
Type 2	13.41 (11)
Type 3	2.44 (2)
Operative characteristics
Ischemic time mean (SD) minutes	82.73 (23.25)
Perfusion time median (IQR) minutes	98.5 (83;116)
Mortality rate at 30 days-Follow up % (n)	2.44 (2)
Survival rate %
1 year follow up %	97.56
5 year follow up %	96.42
10 year follow up %	80.5%

Table 3.Paired-t test analysis

Variable/Echocardiographic findings	LVEF	MG
Comorbidities
Chronic Renal Impairment	0.08	0.56
Coronary Arterial Disease (CAD)	0.02	0.89
Type 2 Diabetes Mellitus (DM)	0.37	0.5
Active Smoking habit	0.91	0.87
Chronic obstructive pulmonary disease (COPD)	0.10	0.78
History of Smoking	0.03	0.5
Arterial hypertension (HPB)	0.61	0.96
Clinical course
Asymptomatic	0.00	0.03
Congestive heart failure (HF)	0.00	0.30
Syncope	0.96	0.03

No competing interests reported.

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Bicuspid Aortic Valve Disease : a 10 Year Latin-american Experience

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