De Bono Procedure Research Articles

Aortic valve replacement using a polytetrafluoroethylene leaflet valve in patients with a history of the Bentall–De Bono procedure: a case series

Objective: To analyze outcomes of 4 transcatheter aortic valve replacements using a polytetrafluoroethylene leaflet valve in patients with a history of the Bentall–De Bono procedure.Methods: We retrospectively analyzed a series of 4 cases. From February 2019 to March 2022 in Federal Center for Cardiovascular Surgery (Penza, Russia), patients with a history of the Bentall–De Bono procedure underwent transaortic aortic valve implantation of the MedLAB-CT balloon-expandable valve under cardiopulmonary bypass and cardioplegia. Valve No. 25 was implanted in 3 patients, and 1 patient received valve No. 23.Results: According to echocardiography, valve hemodynamic performance improved in the postoperative period: mean and peak pressure gradients were 11.5 and 24 mm Hg, respectively; the median value of effective orifice area was 1.9 cm2, while mitral regurgitation in all cases did not exceed grade 1. Median cardiopulmonary bypass and myocardial ischemia times were 59 and 31 minutes, respectively. There was no immediate and remote mortality. No complications were reported in the postoperative period.Conclusion: The proposed replacement of the valved conduit’s compromised part after the Bentall–De Bono procedure can significantly reduce the extent of surgery, cardiopulmonary bypass and myocardial ischemia times, as well as simplify the procedure. Received 1 December 2022. Revised 10 February 2023. Accepted 16 February 2023. Informed consent: The patient’s informed consent to use the records for medical purposes is obtained. Funding: The study did not have sponsorship. Conflict of interests: The authors declare no conflict of interest. Contribution of the authorsLiterature review: V.A. Karnakhin, I.D. PotopalskiyDrafting the article: V.V. Bazylev, A.B. Voevodin, I.D. Potopalskiy, V.A. KarnakhinCritical revision of the article: A.A. Kuznetsova, M.P. PatelSurgical treatment: V.V. Bazylev, A.B. VoevodinFinal approval of the version to be published: V.V. Bazylev, A.B. Voevodin, I.D. Potopalskiy, V.A. Karnakhin, M.P. Patel, A.A. Kuznetsova

Comparative Analysis of the Effectiveness of Different Options for the Administration of Cardioplegic Solution during Surgical Correction of an Aortic Aneurysm Combined with Coronary Artery Lesions

The aim. To determine the effectiveness of myocardial protection in various variants of administration of cardioplegic solution in patients with aortic aneurysms combined with coronary artery lesions.
 Materials and methods. We analyzed 111 operations for aortic aneurysms combined with lesions of the coronary arteries, which were performed from 2011 to 2020 at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine. The subjects were divided into four groups: group 1 where all three methods of cardioplegic solution administration (antegradely, retrogradely and into alloshunts) were used (60 [54.1%] patients), group 2 with administration of cardioplegic solution retrogradely and into alloshunts (48 [43.2%] patients), group 3 with antegrade and retrograde administration (2 [1.8%] patients), and group 4 with only retrograde administration of cardioplegic solution (1 [0.9%] patient). Of these operations, 19 (17.1%) were performed for acute type A aortic dissection, 4 (53.6%) for chronic type A aortic dissection, 88 (79.3%) for aortic aneurysm without dissection. The most common surgical interventions performed were: Robicsek procedure (41 [36.9%] interventions), Bentall – de Bono procedure (40 [36.0%] interventions), supracoronary ascending aortic replacement (22 [19.8%] operations), Wheat procedure (7 [6.3%] interventions).
 Results. According to our results, the aortic cross-clamp time was longer in group 2 (p < 0.05). The average time of extubation in groups 1 and 2 did not differ significantly (p > 0.05). At the same time, the average time of stay of patients in the intensive care unit differed significantly (p < 0.05) in patients of groups 1 (168.92 ± 121.54 h) and 2 (199.35 ± 214.42 h), which indicates faster recovery of patients of group 1. We diagnosed a total of 34 (30.6%) complications in 111 operated patients. A significant number of complications, namely 20 (33.3%) cases, were observed in group 1. The most frequent complications in this group were atrial fibrillation which occurred in 4 (6.7%) cases and hemorrhagic complications which were observed in 3 (5.0%) cases; of these, 1 (1.7%) case was with hemothorax and hemopericardium (this patient underwent rethoracotomy), 1 (1.7%) with hemopericardium and tamponade, and 1 (1.7%) with hemothorax. Of the 111 operations analyzed, 5 (4.5%) cases turned out to be fatal.
 Conclusions. The most effective method of introducing a cardioplegic solution in surgical interventions for aortic aneurysms combined with coronary artery lesions is the administration of cardioplegic solution antegradely, retrogradely and into alloshunts, after anastomosing thereof. This technique allows for the most effective protection of the myocardium by reducing the period of ischemia.
 In other cases, when it is impossible to introduce a cardioplegic solution antegradely (due to dissection of coronary arteries) or retrogradely (due to the presence of a persistent left superior vena cava), it is advisable to use all available alternative methods of protection and, if possible, to reduce the aortic cross-clamp time.

Aortic Root Surgery in Adults: An Unsolved Problem

Nowadays, despite the rapid advancements in interventional cardiology, open surgery still deals with aortic root diseases, to assure the best "ad hoc" treatment. In case of middle-aged adult patients, the optimal operation still represents a matter of debate. A review of the last 10-year literature was conducted, focusing on patients below 65 to 70 years of age. Because of the small sample and the heterogeneity of the papers, no metanalysis was possible. Bentall-de Bono procedure, valve sparing, and Ross operations are the surgical options currently available. The main issues in the Bentall - de Bono operation are lifelong anticoagulation therapy and cavitation in case of mechanical prosthesis implantation and structural valve degeneration in case of biological Bentall. As transcatheter procedures are currently performed as valve in valve, biological prosthesis may be preferable, if the diameter may prevent postoperative high gradients. Conservative techniques, such as remodeling and reimplantation, preferred in the young, guarantee physiologic aortic root dynamics and impose surgical analysis of the aortic root structures to get a durable result. The Ross operation, which shows excellent performance, involves autologous pulmonary valve implantation and is performed only in experienced and high-volume centers. Due to its technical difficulty, it requires a steep learning curve and presents some limitations in specific aortic valve diseases. All three have advantages and downsides, and no ideal solution has still been reported.

Aortic homograft for valved conduit infection in a patient with severe heart failure after previous Bentall—De Bono procedure

Aortic homograft for valved conduit infection in a patient with severe heart failure after previous Bentall—De Bono procedure

Aortic surgery in a patient with Marfan's syndrome and pectus excavatum (shoemaker's chest)

Patients with Marfan syndrome often have severe life-threatening cardiovascular complications, including aortic aneurysmal dilatation or aortic dissection. Approximately two-thirds of people who have this syndrome suffer from chest wall deformities such as pectus excavatum (cobbler's chest) or pectus carinatum (bird's chest). When a patient with cobbler's chest requires aortic root surgery, the operator may face a major clinical challenge in selecting the optimal surgical access and approach. We present a case of a patient with Marfan syndrome and severe pectus excavatum who underwent aortic valve replacement, aortic root and ascending aorta reconstruction, coronary artery reimplantation (Bentall de Bono procedure ) via upper partial L-sternotomy and anterior left thoracotomy.

BioBentall procedure and autological materials in aortic root surgery

The BioBentall procedure has many options, but the optimal approach is still not determined. Alternative options for aortic root surgery include Ross procedure and modification of the Bentall – De Bono procedure using autologous pericardium leaflets. This literature review showed topical issues of surgical technique and the results of various modifications of the BioBentall operation, as well as methods of the aortic root repair using autologous materials. The search strategy included the analysis of international (PubMed, Scopus, Embase) and Russian’s databases (Russian Science Citation Index). The BioBentall procedure demonstrates satisfactory early and long-term results comparable to both the classic Bentall – De Bono procedure and valve-sparing techniques. Composite grafts containing frameless bioprostheses seem to be more promising and durable in comparison with stent-grafts, however, given the minimal amount of publications directly comparing these techniques, further study of this hypothesis is necessary. Total xenopericardial conduits have demonstrated higher rates of degradation and are currently not recommended for use, as demonstrated in most foreign studies. Modified Ross procedure shows excellent hemodynamic outcomes in the long-term follow-up, however, the literature data regarding its applicability in a cohort of patients with proximal aortic aneurysms are limited. The combination of the Bentall – De Bono procedure and autopericardial neocuspidization seems to be a promising and cost-effective method, however, this approach requires a clinical assessment, which results have not been yet published. Received 26 February 2022. Revised 20 April 2022. Accepted 21 April 2022. Funding: The study did not have sponsorship. Conflict of interest: Authors declare no conflict of interest. Contribution of the authorsConception and study design: R.N. Komarov, A.M. IsmailbaevDrafting the article: A.N. Dzyundzya, A.O. Danachev, O.O. Ognev, M.V. LencovetsCritical revision of the article: S.V. CherniavskiiFinal approval of the version to be published: R.N. Komarov, A.M. Ismailbaev, S.V. Cherniavskii, A.N. Dzyundzya, A.O. Danachev, O.O. Ognev, M.V. Lencovets

Long-Term Outcomes after Aortic Valve and Root Replacement in a Very High-Risk Population.

Background: Aortic valve and root replacement (AVRR) is a standardised procedure to treat patients with aortic valve and root disease. In centres with a well-established aortic valve and root repair program (valve repairs and Ross operations), only patients with very complex conditions receive AVRR; this procedure uses a mechanical or biological composite valve graft (modified Bentall–de Bono procedure). The aim of the study was to evaluate the short- and long-term results after AVRR in a high-risk population with complex pathologies. Methods: Between 2005 and 2018, a total of 273 consecutive patients (mean age 64 ± 12.8 years; 23% female) received AVRR. The indication for surgery was an acute type A aortic dissection in 18%, infective endocarditis in 36% and other pathologies in 46% patients; 39% were redo procedures. The median EuroSCORE II was 11.65% (range 1.48–95.63%). Concomitant surgery was required in 157 patients (58%). Results: The follow-up extended to 5.2 years (range 0.1–15 years) and it was complete in 96% of the patients. The 30-day mortality was 17%. The overall estimated survival at 5 and 10 years was 65% ± 3% and 49% ± 4%, respectively. Univariate and multivariate logistic regression analyses revealed the following risk factors for survival: perioperative neurological dysfunction (OR 5.45), peripheral artery disease (OR 4.4) and re-exploration for bleeding (OR 3.37). Conclusions: AVRR can be performed with acceptable short- and long-term results in a sick patient population. The Bentall–De Bono procedure may be determined to be suitable for only elderly or high-risk patients. Any other patients should receive an AV repair or the Ross procedure in well-established centres.

P43 CARDIAC SURGERY IN PATIENTS WITH MARFAN SYNDROME: 20–YEAR SINGLE–CENTER EXPERIENCE

Abstract Marfan syndrome is a connective tissue disease whose most fearsome complications are proximal thoracic aorta enlargement and dissection. Nonetheless, cardiac surgery has proven to be able to considerably extend average life expectancy up to approximately 70 years1. The aim of our study was to analyze which procedures had been performed on these patients. From 2000 to 2020 we retrospectively enrolled 89 consecutive patients undergoing a first–time surgical operation at our department. Overall, aortic root surgery represented the most frequent procedure (82 patients, 92.2%), with valve–sparing operation being the preferred one (53 patients, 59.6%). On the other hand, 29 patients (32.6%) underwent Bentall–De Bono procedure. Consequently, we compared these 2 groups of patients. In emergent operation, typically type A aortic dissection, Bentall–De Bono procedure was preferred in almost all cases (p–value 0.031). Few patients required the replacement of part or all of the arch, respectively 3 and 6, with no statistical difference between the two groups. However, while 2 valve–sparing patients required a mitral valve repair, 8 patients who underwent aortic root replacement had also their mitral valve replaced. Considering mean age (average 34.68 years), predictably almost all Bentall–De Bono patients had both their aortic and mitral valve replaced with a mechanical prosthesis (respectively 27 and 7 out of 29 and 8). Although there was no significant difference, replacement of the aortic root with a composite graft required less cross–clamp time, being also different the complexity of the technique. ICU stay was significantly different, since patients who had their aortic valve spared needed intensive assistance for an average of just 1.57 days, while the other group for 6.03 days (p–value 0.037). Similarly, but non significantly, hospital stay differed (13.91 and 18.17 days). Only 2 patients died during hospital recovery, both part of the Bentall–De Bono group (p–value 0.055). During the follow–up period (average 89.39 months), 6 patients died, 2 of them for noncardiac related causes, and 9 (11.3%) required another operation. In conclusion, Marfan syndrome is characterized by a dangerous proximal aorta involvement, which in some cases may be life–threatening if left untreated. Fortunately, surgery has significantly improved life quality and expectancy of these patients, who must be adequately diagnosed and then monitored by their referral centers.

Aortic valve reimplantation in patients with Marfan and other connective tissue syndromes: a success story for these patients!

After the introduction of aortic valve (AV) and root replacement (Bentall–De Bono procedure), survival changed dramatically for patients with AV and/or root pathologies, including patients with Marfan syndrome or other genetic connective tissue diseases (CTD) [1, 2]. Since the latter mentioned patients are presented for surgery at a very young age, mechanical prosthesis is generally chosen over the biological. The consequent need for life-long anticoagulation exposes the patients to the potential, sometimes severe complications accounting up to 5% per year [3]. Due to this fact, AV-sparing techniques have been evolving over the last 30 years. Especially, AV reimplantation, as described by David and Feindel in 1992, became the standard technique for valve-sparing aortic root replacement with excellent short- and long-term results, even in patients with Marfan syndrome or other CTD [2, 4, 5]. In this issue of the European Journal of Cardiothoracic Surgery, Gil et al. describe their experience with AV reimplantation (David operation) in 157 patients with Marfan syndrome (143 patients) and other CTD [6]. The presented cohort is one of the largest published yet. The early- and long-term outcomes (median of 7.3 years) are impressively good. Especially, survival at 10 and 15 years with 98% is excellent and not different to the general Spanish population for the same age and gender. Furthermore, reported freedom from greater than moderate aortic regurgitation and reintervention were 90% and 96% at 10 years, respectively. Importantly, all these patients were operated by the same cardiosurgical team. The clear message that can be derived from this article is that David operation is an excellent option for the surgical treatment of these patients. The authors also conclude these patients should be centralized in referral centres and operated by very experienced surgeons. Finally, based on these results, authors postulate the threshold for prophylactic surgery in CTD should be reviewed and lowered.

Diagnosis and Successful Surgical Treatment of Ascending Aorta Valved Conduit Rupture

Prosthetic infection is the most common complication after aortic replacement with a synthetic vascular prosthesis or a valved conduit (VC); in this case, aortic valve (AV) rupture occurs in 0.1-1.3% of patients. The cause of valve rupture is not only infective endocarditis; ascending aortic aneurysm and obvious calcification of the native AV are also known risk factors. The paper describes a clinical case of a 49-year-old patient examined and treated in Petrovsky Russian Scientific Center of Surgery. Eighteen years after primary surgical intervention for aortic stenosis, AV reprosthesis and ascending aorta VC replacement were performed according to the Bentall - De Bono procedure modified by Kouchoukos. In 2021, computed tomographic aortography has revealed that in the presence of infective endocarditis, there is aortic prosthetic rupture to form a paraprosthetic fistula and a pseudoaneurysm cavity. The results of instrumental studies are comparable with intraoperative data. The clinical case is of interest due to the rare occurrence of complications and to the significant role of multislice computed tomography during examination and postoperative monitoring.

Bentall–De Bono procedure for aortic regurgitation and intraoperative rupture of moderately dilated ascending aorta: A case report

We herein present a clinical case of root and ascending aortic replacement in a patient with borderline enlargement of the ascending aorta and aortic valve insufficiency. A 65-year-old man was admitted to our clinic with signs of heart failure. Subsequent echocardiography and contrast-enhanced computed tomography revealed hemodynamically significant aortic insufficiency, as well as expansion of the ascending aorta. Diameter at the levels of the sinuses of Valsalva, sinotubular junction and tubular portion of the ascending aorta were 48, 47 and 44 mm, respectively. Based on the aforementioned data, indications for isolated aortic valve replacement were determined. Although the main portion of the surgery was unremarkable, at its final stage, a rupture of a section of the ascending aorta occurred. The results of intraoperative express histological examination of the enlarged aorta revealed connective tissue dysplasia and cystic median necrosis. Replacement of the ascending aorta was performed using the modified Bentall–De Bono technique. This case demonstrated that a borderline aortic dilatation of 40–50 mm at the ascending aorta was associated with pathological changes in its wall, which can cause fatal complications (rupture and dissection) and may require a more aggressive approach during surgical correction. Intraoperative express histological examination of the wall of the ascending aorta in ambiguous situations can help determine the scope of the intervention.Received 16 March 2021. Revised 14 September 2021. Accepted 15 September 2021.Funding: The study did not have sponsorship.Conflict of interest: The authors declare no conflict of interest.Contribution of the authors Literature review: S.Yu. Boldyrev, M.A. Marukyan Drafting the article: S.Yu. Boldyrev, M.A. Marukyan, V.N. Suslova Critical revision of the article: S.Yu. Boldyrev, V.A. Porkhanov Surgical treatment: S.Yu. Boldyrev, M.A. Marukyan Final approval of the version to be published: S.Yu. Boldyrev, M.A. Marukyan, V.N. Suslova, K.O. Barbukhatti, V.A. Porkhanov

Mini-Bentall: An Interesting Approach for Selected Patients.

Minimally invasive surgery through an upper hemisternotomy for aortic valve replacement has become the routine approach with excellent results. Actually, the same minimally invasive access is used for complex ascending aorta procedures only in few centers. We report our experience with minimally invasive approach for aortic valve and ascending aorta replacement using Bentall technique. From January 2010 to November 2015, a total of 238 patients received ascending aorta and aortic valve replacement using Bentall De Bono procedure at our institution. Low- and intermediate-risk patients underwent elective surgery with a minimally invasive approach. The "J"-shaped partial upper sternotomy was performed through a 6-cm skin incision from the notch to the third right intercostal space. Patients who had previous cardiac surgery or affected by active endocarditis were excluded. The study included 53 patients, 44 male (83 %) with a median age of 63 years [interquartile range (IQR), 51-73 years]. A bicuspid aortic valve was diagnosed in 27 patients (51%). A biological Bentall using a pericardial Mitroflow or Crown bioprosthesis implanted in a Valsalva graft was performed in 49 patents. The remaining four patients were treated with a traditional mechanical conduit. Median cardiopulmonary bypass time and median cross-clamp time were respectively 84 (IQR, 75-103) minutes and 73 (IQR, 64-89) minutes. Hospital mortality was zero as well as 30-day mortality. Median intensive care unit and hospital stay were 1.9 and 8 days, respectively. The study population compared with patients treated with standard full sternotomy and similar preoperative characteristics showed similar results in terms of postoperative outcomes with a slightly superiority of minimally invasive group mainly regarding operative times, incidence of atrial fibrillation, and postoperative ventilation times. A partial upper sternotomy is considered a safe option for aortic valve replacement. Our experience confirms that a minimally invasive approach using a partial upper J-shaped sternotomy can be a safe alternative approach to the standard in selected patients presenting with complex aortic root pathology.

Long term follow-up after the Ross procedure (RCD code: IV-5A.O)

Aims: The aim of this study was to analyze the incidence of long-term complications observed during follow-up of patients who had undergone the Ross procedure in childhood. Methods: The study engaged a cohort of 9 patients, all of whom were between 19 to 32 years old. Patients had been in the care of the Centre for Rare Cardiovascular Diseases since the age of 18. Clinical and echocardiographic data were collected from the follow-up period. Results: 78% of patients had preserved global systolic function of the left ventricle, and 56% had dilatation of the ascending aorta. Due to the aneurysm of the ascending aorta one patient required the Bentall de Bono procedure. Another patient underwent a reoperation because of endocarditis of the pulmonary homograft with severe aortic and pulmonary regurgitation. 1/3 of the studied patients were being considered for a reoperation. 23% of patients developed severe pulmonary valve regurgitation, 33% moderate. 67% of patients developed mild to moderate pulmonary valve stenosis. Most patients were in NYHA class I-II. Conclusion: Late complications are frequent in this group of patients, and hence they require surveillance in specialized Centers for Grown-up Congenital Heart Diseases. JRCD 2016; 3 (1): 9–13

Laubry-Pezzi Syndrome With Aortic Root Dilatation Treated With a Bentall and De Bono Procedure

Laubry-Pezzi syndrome is a clinical entity in which prolapse of an aortic valve cusp into a subjacent ventricular septal defect (VSD) due to Venturi effect results in progressive aortic valve insufficiency. Aortic valve prolapse is found in over 5% of children with VSDs, most commonly in association with supracristal VSDs, and the risk of development of aortic regurgitation increases during childhood, peaking at 5 to 10 years of age. The VSD closure eliminates the low-pressure zone that is the cause of ongoing aortic valve cusp deformity and, if performed early, prevents the development of aortic regurgitation. However, the management of this rare pathology is still a matter of some debate with respect to indications, operative techniques, and timing. We report the case of a patient with Laubry-Pezzi syndrome, originally operated on at six years of age for VSD closure and plasty between the left and the right aortic cusps. However, in the follow-up the patient developed severe aortic regurgitation and aortic root dilatation. Eventually, a Bentall and De Bono procedure was performed.

Dissecting aortic aneurysm in a 23 year-old hypertensive woman

A 23 year-old woman was admitted to our hospital after loss of consciousness with chest pain in a stress situation (the woman’s wedding ceremony). The patient had been irregularly treated for mild hypertension from the age of eight. Secondary causes of hypertension were never diagnosed. There were no morphological features of Marfan syndrome in the patient. She had been operated upon for vesicoureteral reflux in childhood. The patient was admitted in a stable condition. Her physical examination revealed diastolic aortic murmur and tachycardia, blood pressure was normal. Transthoracic echocardiography showed expansion of ascending aorta to 43 mm and thoracic aorta dissection (Fig. 1). Chest computed tomography demonstrated thoracic aorta dissection, which began 15 mm above the aortic valve, expansion of ascending aorta to 44 mm, expansion of arch of aorta to 28 mm and pseudocanal of the descending aorta to 6.5 mm (Fig. 2). The dissection included brachiocephalic trunk, both common carotid arteries, left subclavian artery and abdominal aorta to the celiac trunk (Fig. 3). The patient was operated upon immediately. A dissecting aneurysm DeBakey I type was diagnosed intraoperatively. The Bentall de Bono procedure was done with implantation CompositGraft St. Jude Medical 25. The early postoperative period was complicated by bleeding, requiring rethoracotomy and low output syndrome. Because of this, the patient was treated with pressor amines. In the following days, postoperative acute kidney failure occurred and patient was subjected to dialysis. Tamponade occurred and a puncture was performed with evacuation of liquid. The antihypertensive treatment was modified and vitamin K antagonist therapy was initiated. The patient was discharged from hospital on the 26th day of treatment. Aortal dissection is one of the most life-threatening complications of hypertension. The commonest causes of aortal dissection are arterial wall abnormalities, which had not been demonstrated in histopathology in our patient. Long term, inadequately treated hypertension was the only aetiology factor of aortic dissection in this young woman.

Incidental diagnosis of unicuspid aortic valve in an asymptomatic adult

A 38 year-old male with no cardiovascular history was referred for computed tomography (CT) of chest aorta due to prominence of the right cardiac contour in prophylactic chest X-ray examination required by his employer. Initially, transoesophageal echocardiography (TEE) was performed and revealed abnormal aortic valve morphology with a posteriorly positioned commissural attachment and ascending aorta aneurysm coupled by a moderate aortic regurgitation without valve orifice stenosis (Fig. 1A, B). With the suspicion of unicuspid aortic valve (UAV) and the diagnosis of ascending aorta aneurysm, he was referred for further cardiovascular evaluation. Physical examination revealed a regular heart rate with a mild diastolic murmur at the right upper sternal border, blood pressure of 125/83 mm Hg and heart rate 68 bpm. Scheduled 64-slice multi-detector CT (MDCT) confirmed dilatation of ascending aorta up to 53 mm and UAV with only one distinctive commissure (Fig. 1C). The Bentall de Bono procedure was performed and a size 29 ATS aortic valved conduit was implanted (ATS Medical, Minneapolis, MN, USA). During surgery, the valve was found to be unicommissural and unicuspid without significant calcification and thickening (Fig. 1D). There were no postoperative complications and the patient made a full recovery. UAV is mainly discovered during surgery or autopsy and can be easily mistaken for a more common bicuspid aortic valve. Morphologic similarities between unicuspid and bicuspid valves in TEE can often provide diagnostic difficulties. The incidental finding in TEE or MDCT substantially affects further management, including Bentall procedure, ascending aorta surgery and aortic valve replacement, with the need for endocarditis prophylaxis.

Pregnancy in patient with Turner syndrome, after surgery for coarctation of the aorta, with bicuspid aortic valve and ascending aortic aneurysm (RCD code: VII-I-1B.6)

A 30‑year‑old woman with Turner Syndrome was admitted to our centre in 12th week of her first pregnancy with a history o surgical correction of coarctation of aorta. She was asymptomatic with good general condition. Transthoracic echocardiography revealed a bicuspid aortic valve with moderate regurgitation and an ascending aorta aneurysm. In angio‑MR the narrowing of the isthmus of the aorta over 50% was seen with peak systolic gradient found in echocardiography approximately 41 mmHg. She was prescribed metoprolol and was closely monitored, the course of pregnancy was uneventful. In 24th week of pregnancy steroids were administered as a preparation for earlier delivery. She delivered in 34th week of pregnancy a healthy baby weighting 2100 grams. Caesarean section was performed in the cardiothoracic surgery ward and after delivery she underwent percutaneous angioplasty of coarctation of aorta with stent implantation. Six weeks after labour she underwent the Bental de Bono procedure with artificial aortic valve implantation. In six‑month observation patient was in good general condition, echocardiography showed normal ascending aorta diameter. JRCD 2013; 1 (4): 16–20

A Learning Curve in Bentall and De Bono Procedure with the Use of the Risk-Adjusted Cumulative Sum Analysis Based on the Japan SCORE

The risk-adjusted cumulative sum analysis of an individual surgical learning curve for the Bentall and De Bono procedure on aortic root pathologies was used to monitor and enhance quality control. From April 2004 to March 2009, 20 consecutive patients were operated upon by a single surgeon for aortic root pathologies by means of the Bentall and De Bono procedure. Operative variables, mortality, and major morbidities were analyzed. The learning curve was calculated using descriptive statistics and cumulative sum failure observed minus expected failure rate derived from the Japan SCORE calculator. In this series, the Japan SCORE calculator expected 3.91% ± 5.14% of 30-day operative mortality rate and 16.74 ± 13.47% of 30-day operative mortality and morbidity. In reality, there was no operative and in-hospital death. Major postoperative morbidity rate was 15.0% (3 patients). The risk-adjusted cumulative sum analysis revealed that the learning curve of mortality was downward below the lower 95% confidence interval throughout all the patients and that of morbidity was upward till the seventh case and remained downward thereafter. The polynomial approximation coefficient analysis revealed a significant negative correlation between aortic cross-clamp time, cardiopulmonary time, and operation time and case load. The Bentall and De Bono procedure for aortic root pathologies was performed at the professionally permissive level from the beginning, even in a low-volume environment. It showed one good example of a learning curve. The risk-adjusted cumulative sum analysis based on the Japan SCORE was a very useful tool for monitoring the performance.

Marfan syndrome. Part 2: treatment and management of patients

Aortic disease is the main cause of death among patients with Marfan syndrome. Before the development of open surgery, most patients died in the fourth decade of life. Improvements in surgical techniques have facilitated prophylactic surgery and have dramatically changed the life expectancy of patients with Marfan syndrome. Valve-sparing techniques are becoming the standard surgical treatment for these patients, since the operative and long-term results are comparable with those obtained with the Bentall and De Bono procedure and their theoretical advantages over the Bentall and De Bono procedure are attractive for young patients. Distal aortic complications still cause substantial morbidity in patients who have undergone surgery. On the other hand, several medical approaches have appeared as alternatives or adjuncts to the standard treatment with beta-blockers. Mouse models of the disease have shown that the angiotensin II receptor blocker losartan can rescue the phenotype. Among female patients, pregnancy deserves special consideration. Aortic dissection occurs mainly in the third trimester of gestation and in patients with dilated aortas. As aortic dissection carries a high risk of maternal mortality and fetal demise, prophylactic aortic surgery is recommended before attempting pregnancy for those women with an aortic diameter exceeding 40 mm.

Long-Term Results of Aortic Root Replacement: 15 Years' Experience

Long-term results of aortic root replacements and the factors affecting long-term mortality were analyzed. We operated on 254 patients from June 1993 to November 2008 for aortic root replacement with Bentall de Bono procedure. Two hundred five patients were male (80.7%) and 49 patients (19.3%) were female. The mean age was 48.3 +/- 14.7 years (range, 14 to 78 years). We performed 72 concomitant procedures in 69 patients, and the most commonly performed procedure was coronary artery bypass grafting in 37 patients (14.6%). The most common indication for aortic root replacement was aneurysm in 235 patients (92.5%). Thirty-four patients (13.4%) had Marfan syndrome. Hypothermic circulatory arrest was used in 52 patients (20.5%). After removing the clamp, we had to reclamp the aorta in 26 patients (10.2%) undergoing operation. Postoperatively 30 patients (11.8%) had in-hospital mortality. The late mortality was 2.8% (7 patients). The most common reason for hospital mortality was low cardiac output (18 in 30 patients; 51.4%). Neurologic complications were seen in 16 patients (6.3%). The mean duration of hospital stay was 16.6 +/- 11.3 days (range, 5 to 77 days). Postoperative follow-up was 6.3 +/- 4.5 years (range, 0 to 15.5 years) on average. Late mortality was significantly affected by Marfan syndrome (p = 0.025) and reclamping the aorta (p = 0.036). Actuarial survival for the overall 254 patients is 88.4% +/- 2.1%, 87.4% +/- 2.2%, and 84.5% +/- 2.56% at 1, 3, and 10 years, respectively. The late-term results of aortic root replacement with the modified Bentall de Bono procedure are satisfactory. Survival is decreased in patients with Marfan syndrome and in the patients who had reclamping intraoperatively.