Marfan Patient Research Articles

Cluster analysis of 100 Marfan patients based on aortic 4D flow MRI and Z-score: insights into disease heterogeneity and stratification of subgroups.

4D flow MRI-derived variables from Marfan patients are highly heterogeneous. Our aim was to identify distinct Marfan patient subgroups based on aortic 4D flow MRI and Z-score for stratification of distinct hemodynamic profiles and clinical features by means of hierarchical cluster analysis. One hundred Marfan patients underwent baseline aortic 4D flow MRI at 3 T. Z-scores, degree of helical and vortical flow, wall shear stress, flow displacement, and peak velocity were determined in the ascending aorta. Sex, age, BMI, antihypertensive medication, and dural ectasia were recorded. Hierarchical cluster analysis was performed using 4D flow MRI variables and Z-scores as input. Cluster analysis resulted in three distinct clusters characterized by different Z-scores (mean ± SD); cluster 1: 0.4 ± 1.1 vs. cluster 2: 3.1 ± 1.1 vs. cluster 3: 3.6 ± 1.9. The three clusters delivered differences in helical and vortical flow patterns (global p = 0.003 and p < 0.001, respectively), wall shear stress (0.49 ± 0.11 vs. 0.44 ± 0.12 vs. 0.37 ± 0.09 N/m2, global p < 0.001), flow displacement (0.11 ± 0.05 vs. 0.16 ± 0.08 vs. 0.15 ± 0.07, global p = 0.006), and peak velocity (76.3 ± 9.0 vs. 60.1 ± 7.3 vs. 56.0 ± 7.8 cm/s, global p < 0.001). Patients in cluster 1 and 2 were relevantly younger than in cluster 3 (32.3 ± 13.8 vs. 32.8 ± 12.6 vs. 40.2 ± 15.0 years, all pairwise ∆p < 0.0297). Hierarchical cluster analysis based on aortic 4D flow MRI and Z-score revealed three distinct subgroups of Marfan patients, each characterized by specific hemodynamic profiles and clinical features. Follow-up of our patients is warranted to assess if 4D flow MRI- and Z-score-based stratification can predict future aortic diameter growth and ultimately improve outcomes. A combination of Z-score and 4D flow MRI-derived parameters may help identify subgroups of Marfan patients representing different stages or phenotypes of aortic disease, which require specific management strategies. Four-dimensional (4D) flow MRI-derived variables of Marfan patients are highly heterogeneous across varying Z-scores. Cluster analysis based on 4D flow MRI and Z-score revealed three distinct subgroups of Marfan patients. A combination of Z-score and 4D flow MRI-derived parameters may identify different stages of aortic disease in Marfan patients.

Endovascular Treatment with a Branched Arch Endograft of an Aneurysmal Dissection of Innominate Artery in a Marfan Patient

Here we will discuss a 38-year-old Caucasian patient, presenting with Marfan disease, integrated in a family history, who benefited from endovascular surgical management of her innominate artery (IA) dissection with aneurysmal progression.

Case Report: Yamane Fixation of a Light Adjustable Lens in a Patient with Marfan Syndrome

This paper aims to discuss a case of a Light Adjustable Lens (LAL) implanted with the Yamane technique in a Marfan patient undergoing complex cataract surgery. A 60-year-old female with a history of Marfan syndrome presented in May 2020 with complaint of worsening vision due to bilateral subluxed cataracts. Bilateral LALs were planned to be implanted in the capsular bag with scleral fixated Ahmed segments using 5-0 prolene due to zonular instability. The procedure was complicated by an anterior capsular tear at 6 O’clock due to a very friable capsular bag. This tear extended posteriorly with no vitreous loss. Given inadequate capsular support, the decision was then made to scleral fixate the LAL using a Yamane technique. The first attempt at scleral fixation resulted in the LAL haptic breaking off into the 27G needle during the scleral pass. The LAL with remaining haptic was cut in half and explanted. A new LAL was successfully fixated with the Yamane technique. A scleral fixated Ahmed segments using 5-0 prolene was done successfully for the right eye. Final adjustment and locking occurred 10 weeks after right eye procedure. The final refraction of the right eye was measured to be plano, + 0.5 cyl, 85 axis with uncorrected distance visual acuity of 20/20 and corrected distance visual acuity of 20/20. The final refraction of the left eye was measured to be -2.75 D, 0 cyl, 0 axis with uncorrected distance visual acuity of 20/200, uncorrected near visual acuity of J1+ and corrected distance visual acuity of 20/20. When choosing a lens to Yamane, LALs are uniquely capable of correcting a residual refractive error without an additional surgery. LALs are an excellent option for patients that desire a refractive outcome with limited or no capsular support.

Hybrid aortobrachial bypass for a giant subclavian and axillary artery aneurysm in a Marfan patient

Hybrid aortobrachial bypass for a giant subclavian and axillary artery aneurysm in a Marfan patient

Ropy zonules in a Marfan's patient: A modified Shaffer sign

Ropy zonules in a Marfan's patient: A modified Shaffer sign

Abnormal cardiac magnetic resonance-derived ascending aortic area strain demonstrates altered ventriculo-vascular function in Marfan syndrome

Abstract Funding Acknowledgements Type of funding sources: None. Background Surveillance strategies for aortic disease based on the current guidelines support the utilization of maximum aortic diameters to evaluate the risk of aortic dissection, however there remains a need for improved imaging markers for this risk stratification. Purpose This study aimed to compare cardiac magnetic resonance (CMR)-derived ventriculo-vascular properties in patients with Marfan syndrome against normal controls, and to determine the relationship between aortic area strain and other clinical variables in this population. Methods Ascending (AAAS) and descending aortic area (DAAS) strain and left ventricular (LV) longitudinal (LS) and circumferential (CS) strain were retrospectively obtained from 20 CMR studies from 17 patients with Marfan syndrome. Aortic area strain was estimated using maximal and minimal luminal areas obtained from cross-sectional aortic images at the level of the pulmonary artery and was calculated as follows: (100 × (Areamax – Areamin)/Areamin). Additionally, LV mass (LVM), end-diastolic volumes (LVEDV) and ejection fraction (LVEF) were measured. Ventriculo-vascular properties were compared to a cohort of CMR studies from 25 healthy controls. Continuous variables were expressed as mean ± standard deviation (SD) or median (interquartile range). Relationships between aortic area strain and clinical variables were investigated using Spearman’s correlation coefficient. Results Mean Marfan patient age at CMR was 16.7 [10.1, 32.9] years. Twelve patients underwent genetic testing and had a Fibrillin-1 (FBN-1) mutation, other patients met the diagnostic criteria for Marfan syndrome. Four patients (4 studies) had undergone surgical aortic root replacement prior to CMR examination. When compared with the healthy controls, LVLS and LVCS were lower in Marfan (−16.0 ± 4.9 vs −19.7 ± 4.8, p = 0.015 and −18.5 ± 3.8 vs −27.0 ± 4.1, p &amp;lt;0.001; respectively) while AAAS was significantly decreased (19.4 [14.3, 25.1] vs 46.1 ± 11.3, p &amp;lt;0.001). LV EDV indexed to body surface area was significantly greater in Marfan (126.0 ± 45.1 vs 78.4 ± 11.9, p &amp;lt;0.001). No significant differences where observed in LVEF (59.3 [55.4, 56.9] vs. 60.1 ± 3.5, p = 0.440) or DAAS (30.3 [14.6, 44.8] vs 41.7 ± 6.4, p = 0.400). Both AAAS and DAAS did not show any significant correlations to LVLS, LVM, LVEF or LVEDV. Correlation analyses demonstrated that increasing age was associated with lower aortic strain (AAAS, R= −0.57, p = 0.010 and DAAS, R = -0.58, p = 0.008; respectively). Conclusions Ascending aortic area strain was reduced in Marfan syndrome compared to normal healthy controls, indicating altered vascular function. Both LVLS and LVCS were lower in Marfan compared to controls. AAAS shows promise as an aortic imaging marker in Marfan, and larger studies examining it in combination with standard aortic dimension measurements are needed to fully ascertain clinical value.

PE-454609-2 MARFAN SYNDROME AND VENTRICULAR ARRHYTHMIAS IN PEDIATRICS: MITRAL ANNULAR DISJUNCTION, MITRAL VALVE PROLAPSE OR BOTH – WHICH IS THE REAL CULPRIT?

Mitral Annular Disjunction (MAD) and Mitral Valve Prolapse (MVP) have been associated with sudden death and ventricular arrhythmias (VA) in adults with connective tissue disease (CTD). While rare, there has been a case report of sudden cardiac death due to VA in a pediatric Marfan patient. However, risk factors have not been well described in this population. Our aim is to assess the association of MAD and MVP with VA in pediatric patients with Marfan syndrome. A retrospective single center cohort study was conducted and included all patients (≤ 21yrs) with clinical (based on Ghent’s criteria) and/or genetic diagnosis of Marfan syndrome. Clinical data including ambulatory cardiac monitoring and echocardiograms were reviewed prior to any mitral valve intervention, if applicable. 32 patients (38% female) with Marfan syndrome (66% with fibrillin 1 mutation) were included. The mean age was 13.5 ± 4.5 yrs. The results revealed MAD in 8 pts (25%), isolated MVP in 5 pts (16%) and bileaflet MVP in 14 pts (44%). Anterior MVP was seen in 16 pts (50%) and posterior MVP in 17 pts (53%). The mean MAD distance was 6.7 ± 2.3 mm. There was normal LV systolic function in 31 pts (97%). The median duration of cardiac monitoring was 58 hrs (IQR: 33-191). 26 pts (81%) had isolated ventricular ectopy (VE), 27 pts (84%) had complex VE (defined as couplets, triplets or NSVT), 4 pts (12.5%) had NSVT and none had sustained VT. The median VE per hour was 0.2 (IQR: 0-1). There was no R on T seen but short coupled VE (<350ms) was seen in 46% of isolated, 46% of couplets and 67% of triplets. Monomorphic ectopy was seen in 28% of isolated and 100% of couplets, triplets and NSVT. There were 5 total episodes of NSVT (Figure 1) with a mean HR of 159 ± 29 bpm, an absolute minimum HR of 94 bpm and maximum HR of 222 bpm. MAD was not associated with the presence of, or complexity in, ventricular arrhythmias. Anterior MVP was only associated with complexity in VE (p= 0.043), but posterior MVP and bileaflet MVP were not. MAD was associated with MVP (anterior, posterior and bileaflet; p= 0.026). There was a high incidence of complex VE and NSVT in this cohort of pediatric Marfan patients despite low overall ectopy burden. Anterior MVP but not MAD was associated with complexity in VE. However, MAD was associated with MVP. Larger studies are needed to better characterize MAD, including distance and circumferential extent, and MVP and their association with VA in pediatric patients with CTD.

Generation of Marfan patient specific iPSCs (ICSSUi001-A) carrying a novel heterozygous mutation in FBN1 gene

Marfan syndrome (MFS) is an autosomal genetic disorder caused by mutation in FBN1 gene, encoding the extracellular matrix protein fibrillin-1. Here, a MFS patient specific iPSC carrying a novel heterozygous mutation (c.7897 T > G) in FBN1 gene was generated. This iPSC line exhibited normal morphology and karyotype, and could differentiate into three germ layers in vivo and in vitro. Thus, the established iPSC line provided a precise platform for elucidating the molecular pathogenesis and personalized drug screening of MFS.

Modification of Frozen Elephant Trunk Technique for Acute Type-A Aortic Dissection - A Case Report

Acute type-A aortic dissection (ATAAD) is a dreadfully fatal cardiovascular emergency. The mortality rate among medically treated patients is 50% within two days. Surgery is the gold standard and urgent surgical treatment is indicated for all patients, except for those who are moribund or severely comorbid. However, early mortality after operation remains high (9–25%). We hereby present a case of 22 years young, male, Marfan patient with a family history of ATAAD treated successfully by us at Square Hospital Ltd by modified Bentall’s procedure with total arch replacement with Frozen Elephant Trunk (FET) technique.&#x0D; University Heart Journal 2022; 18(1): 65-70

Parsonage-Turner Syndrome and Closed-Incision Negative-Pressure Wound Therapy After Total Hip Arthroplasty in a Case of Marfan Syndrome.

Negative pressure wound therapy (NPWT) is a postoperative wound care method, which has recently become an ongoing field of research in hip and knee arthroplasty. We report the successful management of wound dehiscence and infection after THA in a case of Marfan syndrome by closed-incision negative-pressure wound therapy (ciNPWT). Our patient also developed a rare postoperative neurologic complication, that is, Parsonage-Turner syndrome (PTS). To our knowledge, this is the first report of PTS and ciNPWT use for SSI after THA in a Marfan patient. As wound dehiscence and infection can occur after THA in Marfan patients, we propose ciNPWT as an option to treat or even prevent (prophylactic use) such complications in this rare group of patients.

Castleman disease and SLE in a G6PD-deficient Marfan patient: a case report and literature review

IntroductionMarfan syndrome, G6PD deficiency, systemic lupus erythematosus (SLE), and Castleman disease are four distinctive, thoroughly investigated entities whose coincidence was never reported. However, occurrence in pairs was sporadically mentioned in literature.Case presentationWe report a 15-year-old Caucasian G6PD deficient Marfan male patient, who presented with tonic–clonic seizures, fever, a hemolytic episode, and general symptoms. After the discovery of hepatosplenomegaly, malar rash, and painless lymphadenopathy, further testing diagnosed a multifocal Castleman disease of the hyaline vascular subtype and systemic lupus erythematosus with lupus nephritis that got 35 points on the 2019 EULAR/ACR criteria. G6PD deficiency, SLE & Castleman disease, and seizures were handled medically with eventual improvement in the patient’s condition.Discussion and conclusionIt is extremely rare to discover the gathering of these four diseases in the same patient. Marfan syndrome and G6PD deficiency were proven by respective clinical and laboratory examinations. Castleman disease that tends to occur in older age groups was confirmed via pathological study of a lymph node biopsy, which was compatible with the HHV-8 negative type reported in Asian countries. SLE is part of the differential diagnosis for Castleman disease, yet the newest evidence strongly supports its presence as a distinct entity. However, no concrete proof is available to suggest a causative relationship between the four of them, rather than a coincidental occurrence.

TEVAR for a Marfan patient during COVID‐19 pandemic

TEVAR for a Marfan patient during COVID‐19 pandemic

Complex Endovascular Aortic Repair with a Branch for an Intercostal Artery in Marfan Patient

Introduction: Spinal cord ischemia (SCI) caused by intercostal artery occlusion in combination with procedural factors such as hypotension is the Achilles heel of open and endovascular TAAA-repair report a case of branched endovascular aortic repair including a branch for a large intercostal artery as a protective method in a patient at risk for spinal cord ischemia (SCI). We report a case of branched EVAR (bEVAR) in a Marfan patient with a 6cm Type IV TAAA including a branch for a large previously re-implanted intercostal artery. Methods: A 43-year-old patient with known Marfan syndrome presented with a type IV thoraco-abdominal aortic aneurysm (TAAA) and history of multiple previous cardiac and aortic operations over the past 28 years. The maximum diameter of the aneurysm was 60mm. The patient had two right renal arteries and two re-implanted segmental arteries. With the goal to preserve both right renal arteries and one large intercostal artery a 6-branched custom-made stent-graft was planned and manufactured. A custom-made stent-graft (Cook Medical, Bjaeverskov, Denmark) was planned. The branched component was planned to land in the mid/distal descending thoracic aorta with antegrade branches for the CA, SMA and renal arteries and a retrograde branch on the opposite left side for the intercostal artery. The retrograde branch for the intercostal artery was positioned at 5:00 o'clock in order to allow a spiral course of the bridging covered stent to the origin of the vessel at 9:30 o'clock with a lower risk of kinking. Bilateral femoral and right brachial artery access was used. The intercostal artery was catheterized and connected to the retrograde branch from femoral access successfully. Results: Selective angiography of the target vessels and final angiography demonstrated good sealing and unimpeded branch-perfusion, without endoleak. The patient was discharged from hospital to home on postoperative day 10 without complications. Six-month-follow up CTA demonstrated unchanged well-fitting endo-graft-position with good sealing and patency of all six branches including the two branches on the right renal artery and the branch to the intercostal artery (Figure. 5). The TAAA-diameter had significantly decreased from 60mm to 42mm and no signs of endoleak were present. Conclusion: Branched endovascular aortic repair with a branch to a large intercostal artery was technically feasible and clinically successful. Disclosure: Tilo Kölbel has intellectual property with Cook Medical, receives royalties, research, travel and educational grants, speaking fees and is consultant and proctor with Cook Medical. Nikolaos Tsilimparis receives travel and educational grants, speaking fees and is proctor with Cook Medical.

VD10 FROZEN ELEPHANT TRUNK AND DAVID PROCEDURE BY USING THORAFLEX AND GORE HYBRID PROSTHESES IN A MARFAN PATIENT WITH ACUTE TYPE A AORTIC DISSECTION

We present the case of a 19-year-old Marfan patient with 53 mm ascending aorta aneurysm complicated by acute type A aortic dissection. Cardiopulmonary bypass was established by cannulation of right femoral artery and right atrium. Moderate (25°C) hypotermic circulatory arrest was used and the aortic root prepared for reimplantation technique. At 25°C, the aortic arch was opened and the antegrade selective cerebral perfusion was established through cannulation of brachiocephalic trunk and left carotid artery. A 22/24 mm Thoraflex hybrid graft was used and distal anstomosis performed in arch zone 2. During the rewarming, the supraaortic vessels were separately reimplanted, using also a Gore 8 mm prosthesis for the left subclavian artery. The aortic valve was reimplanted in a 30 mm Valsalva conduit. The procedure was completed with the suture of the two vascular conduits. Post-operative echocardiogram showed a well-functioning aortic valve and angio CT scan showed a good surgical result.

Betaglycan (TGFBR3) up-regulation correlates with increased TGF-β signaling in Marfan patient fibroblasts in vitro.

Marfan syndrome (MFS), a congenital connective tissue disorder leading to aortic aneurysm development, is caused by fibrillin-1 (FBN1) gene mutations. Transforming growth factor beta (TGF-β) might play a role in the pathogenesis. It is still a matter of discussion if and how TGF-β up-regulates the intracellular downstream pathway, although TGF-β receptor 3 (TGFBR3 or Betaglycan) is thought to be involved. We aimed to elucidate the role of TGFBR3 protein in TGF-β signaling in Marfan patients. Dermal fibroblasts of MFS patients with haploinsufficient (HI; n=9) or dominant negative (DN; n=4) FBN1 gene mutations, leading to insufficient or malfunctioning fibrillin-1, respectively, were used. Control cells (n=10) were from healthy volunteers. We quantified TGFBR3 protein expression by immunofluorescence microscopy and gene expression of FBN1, TGFB1, its receptors, and downstream transcriptional target genes by quantitative polymerase chain reaction. Betaglycan protein expression in FBN1 mutants pooled was higher than in controls (P=.004) and in DN higher than in HI (P=.015). In DN, significantly higher mRNA expression of FBN1 (P=.014), SMAD7 (P=.019), HSP47 (P=.023), and SERPINE1 (P=.008), but a lower HSPA5 expression (P=.029), was observed than in HI. A pattern of higher expression was noted for TGFB1 (P=.059), FN1 (P=.089), and COL1A1 (P=.089) in DN as compared to HI. TGFBR3 protein expression in cells, both presence in the endoplasmic reticulum and amount of vesicles per cell, correlated positively with TGFB1 mRNA expression (Rs=0.60, P=.017; Rs=0.55, P=.029; respectively). TGFBR3 gene expression did not differ between groups. We demonstrated that activation of TGF-β signaling is higher in patients with a DN than an HI FBN1 gene mutation. Also, TGFBR3 protein expression is increased in the DN group and correlates positively with TGFB1 expression in groups pooled. We suggest that TGFBR3 protein expression is involved in up-regulated TGF-β signaling in MFS patients with a DN FBN1 gene mutation.

MANAGEMENT OF AN ABERRANT SUBCLAVIAN ARTERY DURING THORACIC AORTIC SURGERY IN THE ERA OF THORACIC ENDOGRAFTS

Aberrant subclavian artery (ASCA) is rare in adults undergoing thoracic aortic surgery. Presence of an ASCA complicates operative management of thoracic diseases. Thoracic endografts (TEVAR) have expanded the treatment options in the field of thoracic aortic surgery. The present study evaluates the techniques and outcome of patients requiring management of an ASCA during surgery of the thoracic aorta in the TEVAR era. Since 2004, 22 adult patients were operated for the thoracic aorta and required management of an ASCA. Patients were managed and followed prospectively by a dedicated thoracic aortic team (mean follow-up: 3.1±2.4years). Table depicts patient characteristics and operative management. One and five year survival were respectively 94.7% and 82.9%. Three patients died at follow-up: one elderly patient whom required coil embolization of the ASCA developed an ASCA-esophageal fistula, a 30 yr old with traumatic rupture of the ASCA died of an aorto-esophageal fistula 3 months following TEVAR implantation and a 37 yr old Marfan patient required reoperation 4.6 yrs post-op for mitral disease and died of postoperative complications. Freedom from aortic related events at one and 5 years were respectively 84.2% and 61.8%. Six patients developed aortic related complications: two patients with esophageal fistula died, 2 patients are followed for distal type I endoleaks, one patient necessitated an open conversion for TEVAR infection and one patient required TEVAR re-lining for a type IV endoleak. Presence of an ASCA complicates operative management of thoracic diseases. In presence of compression symptoms (dysphagia lusoria), an open approach should be favoured to relieve the obstruction in patients with acceptable open operative risks. TEVAR is a good option in complex aortic cases to exclude the proximal portion of the ASCA. Amplatz occluder inserted proximal to the vertebral artery offers a more reliable distal ASCA exclusion than the ‘classic’ coil embolization. TEVAR requires lifelong imaging surveillance to identify complications at an early stage and implement appropriate treatment.

Incidental finding of coarctation of the aorta in a Marfan patient: a case report

Aneurysm of the ascending aorta with coarctation of the aorta is a rare find in the literature. We report a case of a huge ascending aorta aneurysm in a patient of Marfan’s syndrome and associated coarctation of the aorta. The patient underwent staged procedure in the form of balloon dilatation for the coarctation of the aorta and a successful Bentall procedure for the aneurysm of the aorta with a brief period of deep hypothermic circulatory arrest (DHCA). Two-staged approach is the ideal management for such cases amenable for endovascular repair.

Missed diagnosis of acute stanford Type A aortic dissection presenting with abdominal pain in the setting of acute appendicitis

Acute Stanford Type A aortic dissection (ATAAD) has a high mortality rate, particularly with delayed diagnosis and treatment. A 30-year-old male Marfan patient presented to the emergency department with acute abdominal pain and underwent laparoscopic appendectomy for acute appendicitis about 15 h later. Postoperatively, the patient developed acute pulmonary edema and respiratory failure. Transthoracic echocardiogram revealed severe aortic insufficiency, 7.5 cm root aneurysm, severe left ventricular dysfunction, and probable ATAAD. Following computed tomography angiogram confirmation of ATAAD, he underwent emergency root and ascending aortic replacement with a composite mechanical valve graft 48 h after hospitalization. The patient had Escherichia coli pericarditis at time of sternotomy likely hematogenous from appendicitis and later also developed postoperative pneumonia. At 2 years follow-up, he was doing well without any evidence of prosthetic valve conduit infection. The simultaneous presentation of two unrelated pathologies with acute abdominal pain led to the initial missed diagnosis of ATAAD, which delayed treatment and increased risks for morbidity and mortality. Therefore, Marfan syndrome patients presenting with acute abdomen should be ruled out for acute aortic dissection despite any other obvious concomitant pathology, especially in younger patients.

Reversed Frozen Elephant Trunk Technique to Treat a Type II Thoracoabdominal Aortic Aneurysm

Purpose: To describe a hybrid technique of reversed frozen elephant trunk to treat thoracoabdominal aortic aneurysms (TAAA) through an abdominal only approach. Technique: The technique is demonstrated in a 29-year-old Marfan patient with a chronic type B aortic dissection previously treated with a thoracic stent-graft who presented with a thoracoabdominal false lumen aneurysm. Through an open distal retroperitoneal approach to the abdominal aorta, a frozen elephant trunk graft was implanted over a super-stiff wire upside down with the stent-graft component in the thoracic aorta. Following deployment of the stent-graft proximally and preservation of renovisceral perfusion in a retrograde manner, the renovisceral vessels were sequentially anastomosed to the elephant trunk graft branches, thus reducing the ischemia time of the end organs. The aortic sac was then opened, and the distal part of the hybrid graft was anastomosed with a further bifurcated graft to the iliac vessels. Conclusion: The reversed frozen elephant trunk technique is feasible for hybrid treatment of TAAAs via an abdominal approach only. This has the benefit of substantially reducing the trauma of thoracic exposure, thus preserving major benefits of open thoracoabdominal surgery, such as the presence of short bypasses to the renovisceral vessels and reimplantation of lumbar arteries to reduce spinal cord ischemia.

Aneurysm of both coronary buttons in a Marfan patient after Bentall–De Bono operation

Aneurysm of both coronary buttons in a Marfan patient after Bentall–De Bono operation