Surmounting the summit: tackling cerebral malperfusion in AMDS-treated acute deBakey I dissections. A case report.

Background: Acute Type A aortic dissection (ATAAD) with supra-aortic branch (SAB) malperfusion remains a formidable clinical challenge, often resulting in high mortality and complex treatment dilemmas. The introduction of the AMDS represents a significant innovation, designed to stabilize the aortic arch, and manage malperfusion effectively. Methods: This case study evaluates the utility of AMDS in the treatment of a 63-year-old male with hypertension, who presented with severe, acute chest pain. Diagnosed with a DeBakey type I ATAAD involving SAB, the patient underwent cardiopulmonary bypass, aortic root replacement, aortic arch repair with AMDS implantation, and subsequent endovascular stenting for severe left common carotid artery malperfusion that developed postoperatively. The AMDS was instrumental in facilitating crucial aortic arch reconstruction and addressing the initial severe malperfusion. Despite postoperative cerebral malperfusion, targeted endovascular stenting resulted in a rapid and substantial neurological recovery. The patient was discharged to a rehabilitation facility on postoperative day 20, free of neurological deficits. Conclusions: The use of AMDS in managing ATAAD with SAB involvement is transformative, enabling less invasive surgical techniques and offering immediate, effective correction of malperfusion. This case underscores the essential role of integrating advanced endovascular strategies to enhance outcomes in high-risk aortic surgeries, marking a pivotal advancement in the therapeutic approach to complex aortic dissections.

The Fate of the Aorta after Coarctation Repair: Open Surgical Replacement of Descending Aorta in a High-Volume Unit.

Objectives: Complications after aortic coarctation repair are associated with high mortality and require surgical or endovascular reintervention. For patients unsuitable for endovascular therapies, reoperation remains the only therapeutic option. However, surgical experience and up-to-date follow-up data concerning this overall rare entity in the spectrum of aortic reoperations are still highly limited. Thus, the aim of this study was to analyze the short-term outcomes and long-term survival of patients undergoing surgical descending aorta repair after previous coarctation repair in a high-volume unit. Methods: We present a retrospective single-center analysis of 25 patients who underwent open descending aorta replacement after initial coarctation repair. The surgical history, concomitant cardiovascular malformations, and preoperative characteristics as well as postoperative complications and long-term survival were analyzed. Results: The mean age at operation was 45.4 ± 12.8 years. A proportion of 68% (n = 17) of the patients were male. The most common complication necessitating reoperation after coarctation repair was aneurysm formation (68%) and re-stenosis (16%). The average time between initial repair and reoperation was 26.3 ± 9.9 years. Technical success was achieved in all the operations, while recurrent nerve damage (24%) and bleeding requiring rethoracotomy (20%) were identified as the most common perioperative complications. The one-year mortality was 0% and the overall long-term survival was 88% at 15 years. Conclusions: Open surgical descending aorta replacement can be performed safely and with excellent survival outcomes even in the challenging subgroup of patients after previous coarctation repair. Thus, reoperation should be considered a feasible approach for patients who are unsuitable for endovascular therapies. Nonetheless, concomitant cardiovascular anomalies and frequent preoperations may complicate the redo operation in this patient population.

Omentoplasty for Cervical Lymphocele after Aortic Arch Replacement.

Lymphocele formation is a rare complication after surgical procedures involving the mediastinum. While uncomplicated lymphoceles show high rates of spontaneous closure and are usually treated conservatively, surgical treatment might be required in cases with persistent or recurrent lymphoceles. We present the case of a 53-year-old male with reoccurring cervical swelling after two surgeries of the thoracic aorta. After 1.5 years, the swelling occurred for the first time and appeared for the next 2 years repeatedly without clinical or laboratory signs of infection. A cervical lymphocele was suspected, and the decision for surgical revision was made. Fibrin glue was applied to the potential leakage of the thoracic duct, and the cavity was filled with a free omental flap. This resulted in a complete regression of the swelling.

Sutureless Aortic Valve Replacement with Perceval Bioprosthesis Superior to Transcatheter Aortic Valve Implantation: A Promising Option for the Gray-Zone of Aortic Valve Replacement Procedures-A State-of-the-Art Systematic Review, Meta-Analysis, and Future Directions.

Background: The management of patients with aortic valve pathologies can sometimes fall into a "gray zone", where the optimal treatment approach is not straightforward. The comparative benefits of sutureless aortic valve replacement (SUAVR) using the Perceval bioprosthesis versus transcatheter aortic valve implantation (TAVI) for the "gray zone" of aortic valve replacement procedures remain a topic of debate. To further explore this issue, we conducted a study with pairwise, single-arm, and Kaplan-Meier-based meta-analyses to compare the outcomes of SUAVR with the Perceval bioprosthesis versus TAVI, as well as to evaluate the efficacy, safety, and durability of SUAVR with the Perceval bioprosthesis over mid-term and long-term follow-up periods. Methods: The PubMed, PubMed Central, OVID Medline, Cochrane Library, Embase, and Web of Science databases were systematically searched. All study types were included, except study protocols and animal studies, without time restrictions. The final search was carried out in May 2024. Results: No statistically significant differences were observed in permanent pacemaker implantation (PPI) rates between the two groups. SUAVR showed a lower incidence of new-onset myocardial infarction but was associated with higher rates of new-onset atrial fibrillation and major bleeding. TAVI had higher rates of left bundle branch block and major vascular complications. Conclusions: Our findings show that SUAVR has a lower incidence of complications and a favorable mid-term overall survival compared to TAVI. SUAVR has more advantages compared to TAVI and can be considered a valuable and promising option for the "grey zone" of aortic valve pathologies.

Hypothermic Ventricular Fibrillation in Redo Minimally Invasive Mitral Valve Surgery: A Promising Solution for a Surgical Challenge.

Background: Minimally invasive mitral valve surgery (MIMVS) is a treatment for severe mitral valve pathologies. In redo cases, especially after coronary artery bypass grafting (CABG) surgery with patent mammary bypass grafts, establishing aortic clamping followed by antegrade cardioplegia application might be challenging. Here, we present the outcome of hypothermic ventricular fibrillation as an alternative to conventional cardioprotection. Methods: Patients who underwent MIMVS either received hypothermic ventricular fibrillation (study group, n = 48) or antegrade cardioprotection (control group, n = 840) and were observed for 30 postoperative days. Data were retrospectively analyzed and collected from January 2011 until December 2022. Results: Patients in the study group had a higher preoperative prevalence of renal insufficiency (p = 0.001), extracardiac arteriopathy (p = 0.001), insulin-dependent diabetes mellitus (p = 0.001) and chronic lung disease (p = 0.036). Furthermore, they had a longer surgery time and a lower repair rate (p < 0.001). No difference, however, was seen in postoperative incidences of stroke (p = 0.26), myocardial infarction (p = 1) and mitral valve re-operation (p = 1) as well as 30-day mortality (p = 0.1) and postoperative mitral valve insufficiency or stenosis. Conclusions: The patients who underwent redo MIMVS with hypothermic ventricular fibrillation did not have worse outcomes or more serious adverse events compared to the patients who received routine conventional cardioprotection. Therefore, the use of hypothermic ventricular fibrillation appears to be a promising cardioprotective technique in this challenging patient population requiring redo MIMVS.

Surgical Therapy of Infective Prosthesis Endocarditis following TAVI: A Single Center's Experience.

BACKGROUND/OBJECTIVES: Infective prosthesis endocarditis (IE) following transcatheter aortic valve implantation (TAVI) presents significant management challenges, marked by high mortality rates. This study reviews our center's experience with surgical interventions for IE in patients post-TAVI, focusing on outcomes, challenges, and procedural complexities, and providing an overview of the limited literature surrounding this subject. METHODS: This study was executed as a comprehensive retrospective analysis, targeting the clinical outcomes of surgical treatment in patients presenting with PVE following TAVI procedures at our institution. From July 2017 to July 2022, we identified five patients who had previously undergone transfemoral transcatheter aortic valve implantation and were later diagnosed with PVE needing surgery, strictly adhering to the modified Duke criteria. RESULTS: All surgical procedures were reported successful with no intra- or postoperative mortality. Patients were predominantly male (80%), with an average age of 76 ± 8.6 years, presenting mostly with dyspnea (NYHA Class II). The mean follow-up was between 121 and 1973 days, with outcomes showing no occurrences of stroke, myocardial infarction, or major bleeding. One patient expired from unrelated causes 3.7 years post-surgery. The operative and postoperative protocols demonstrated effective disease management with enhanced survival and minimal complications. CONCLUSIONS: The surgical treatment of IE following TAVI, though challenging, can be successfully achieved with careful patient selection and a multidisciplinary approach. The favorable outcomes suggest that surgical intervention remains a viable option for managing this high-risk patient group. Our study also highlights the scarce literature available on this topic, suggesting an urgent need for more comprehensive research to enhance understanding and improve treatment strategies. Future studies with larger cohorts are needed to further validate these findings and refine surgical strategies for this growing patient population.

Minimally Invasive Approach for Replacement of the Ascending Aorta towards the Proximal Aortic Arch.

Objectives: In recent years, minimally invasive approaches have been used with increasing frequency, even for more complex aortic procedures. However, evidence on the practicability and safety of expanding minimally invasive techniques from isolated operations of the ascending aorta towards more complex operations such as the hemiarch replacement is still scarce to date. Methods: A total of 86 patients undergoing elective surgical replacement of the ascending aorta with (n = 40) or without (n = 46) concomitant proximal aortic arch replacement between 2009 and 2023 were analyzed in a retrospective single-center analysis. Groups were compared regarding operation times, intra- and postoperative complications and long-term survival. Results: Operation times and ventilation times were significantly longer in the hemiarch replacement group. Despite this, no statistically significant differences between the two groups were observed for the duration of the ICU and hospital stay and postoperative complication rates. At ten-year follow-up, overall survival was 82.6% after isolated ascending aorta replacement and 86.3% after hemiarch replacement (p = 0.441). Conclusions: Expanding the indication for minimally invasive aortic surgery towards the proximal aortic arch resulted in comparable postoperative complication rates, length of hospital stay and overall long-term survival compared to the well-established minimally invasive isolated supracommissural ascending aorta replacement.

Minimally Invasive Direct Coronary Artery Bypass Grafting: Sixteen Years of Single-Center Experience.

Background: Coronary artery disease is a major cause of death globally. Minimally invasive direct coronary artery bypass (MIDCAB), using a small left anterior thoracotomy, aims to provide a less invasive alternative to traditional procedures, potentially improving patient outcomes with reduced recovery times. Methods: This retrospective, non-randomized study analyzed 310 patients who underwent MIDCAB between July 1999 and April 2022. Data were collected on demographics, clinical characteristics, operative and postoperative outcomes, and follow-up mortality and morbidity. Statistical analysis was conducted using IBM SPSS, with survival curves generated via the Kaplan-Meier method. Results: The cohort had a mean age of 63.3 ± 10.9 years, with 30.6% females. The majority of surgeries were elective (76.1%), with an average operating time of 129.7 ± 35.3 min. The median rate of intraoperative blood transfusions was 0.0 (CI 0.0-2.0) Units. The mean in-hospital stay was 8.7 ± 5.5 days, and the median ICU stay was just one day. Early postoperative complications were minimal, with a 0.64% in-hospital mortality rate. The 6-month and 1-year mortalities were 0.97%, with a 10-year survival rate of 94.3%. There were two cases of perioperative myocardial infarction and no instances of stroke or new onset dialysis. Conclusions: The MIDCAB approach demonstrates significant benefits in terms of patient recovery and long-term outcomes, offering a viable and effective alternative for patients suitable for less invasive procedures. Our results suggest that MIDCAB is a safe option with favorable survival rates, justifying its consideration in high-volume centers focused on minimally invasive techniques.

Long-term discontinuation of warfarin in a patient with HeartMate 3 left ventricular assist device without thromboembolic events.

The gold standard therapy for end-stage heart failure is cardiac transplantation. However, in the face of a donor shortage, a mechanical assist device such as the left ventricular assist device HeartMate 3 (Abbott Laboratories, Abbott Park, IL, USA) serves as bridging therapy to transplantation and/or destination therapy. Current guidelines recommend anticoagulation with a vitamin K antagonist in combination with low-dose aspirin. We herein report a challenging anticoagulation regimen in a patient with a HeartMate 3 in whom systemic anticoagulation with warfarin was not feasible for 4 years because of low compatibility and a rare X-factor deficiency. This is a rare hematological disorder, estimated to affect approximately 1 in every 500,000 to 1,000,000 people in the general population. The patient finally received a modified anticoagulation regimen involving the combination of rivaroxaban and clopidogrel without warfarin. Under this regimen, the patient remained free of thromboembolic complications for 4 years with in situ placement of the left ventricular assist device. This case illustrates that under specific circumstances, long-term absence of warfarin therapy is feasible in patients with a HeartMate 3.

Implementation of Endoscopic Minimally Invasive Mitral Valve Replacement Surgery With Automated Suturing Technology.

In the evolving landscape of cardiac surgery, this article explores the potential of minimally invasive mitral valve replacement procedures as a viable alternative to conventional surgical techniques. Leveraging advancements in automated suturing devices and video endoscopy, our work aims to demonstrate that minimally invasive approaches can be applied across a broad spectrum of surgical scenarios. Herein we highlight preoperative diagnostics and operative techniques, with a focus on infra-axillary anterolateral minithoracotomy as the access point. Our technique utilizes technology from LSI SOLUTIONS® (Victor, NY, USA), including the RAM® Device for automated suturing, which has an ergonomic design and safety features. The device's capabilities are further enhanced by the SEW-EASY® Device, the RAM® RING, and the COR-KNOT MINI® Device, which streamline suture management and securement. This work outlines how these technological advancements can mitigate concerns about technical complexity and learning curves, thereby encouraging wider adoption of minimally invasive techniques. Clinical benefits may include reduced surgical trauma, quicker recovery, and cost-effectiveness, making it a compelling option in an era of aggressively promoted transcatheter interventions.

Risk factors, prevention, and therapy of intraluminal stent thrombosis in frozen elephant trunk prostheses-what we know so far.

Intraluminal thrombus formation (ILT) is a recently discovered and highly clinically relevant complication after frozen elephant trunk implantation in cardiovascular surgery. In this phenomenon, a thrombus forms within the lumen of the stent graft component of the frozen elephant trunk prosthesis and puts the patient at risk for downstream embolization with visceral or lower limb ischemia. Incidence of ILT reported in the currently available studies ranges from 6% to 17% of patients after frozen elephant trunk implantation. Adverse thromboembolic events include acute occlusion of the celiac and superior mesenteric arteries, both renal arteries as well as acute lower limb ischemia due to iliac or femoral artery embolization that not infrequently require interventional or open embolectomy. Therefore, the presence of ILT is associated with increased short-term mortality and morbidity. Currently proposed strategies to avoid ILT formation include a more aggressive anticoagulation management, minimization of postoperative coagulation factor application, and even technical optimizations of the stent graft portion itself. If ILT is manifested, the therapeutic strategies tested to date are long-term escalation of anticoagulation and early endovascular extension of the FET stent graft with overstenting of the intraluminal thrombus. The long-term efficiency of these prophylactic and therapeutic measures has yet to be proven. Nonetheless, all surgeons performing the frozen elephant trunk procedure must be aware of the risk of ILT formation to facilitate a timely diagnosis and therapy.

Impact of Intercostal Artery Reinsertion on Neurological Outcome after Thoracoabdominal Aortic Replacement: A 25-Year Single-Center Experience.

BACKGROUND: Intercostal artery reinsertion (ICAR) during thoracoabdominal aortic replacement remains controversial. While some groups recommend the reinsertion of as many arteries as possible, others consider the sacrifice of multiple intercostals practicable. This study investigates the impact of intercostal artery reinsertion or sacrifice on neurological outcomes and long-term survival after thoracoabdominal aortic repair. METHODS: A total of 349 consecutive patients undergoing thoracoabdominal aortic replacement at our institution between 1996 and 2021 were analyzed in a retrospective single-center study. ICAR was performed in 213 patients, while all intercostal arteries were ligated and sacrificed in the remaining cases. The neurological outcome was analyzed regarding temporary and permanent paraplegia or paraparesis. RESULTS: No statistically significant differences were observed between the ICAR and non ICAR groups regarding the cumulative endpoint of transient and permanent spinal cord-related complications (12.2% vs. 11.8%, p = 0.9). Operation, bypass, and cross-clamp times were significantly longer in the ICAR group. Likewise, prolonged mechanical ventilation was more often necessary in the ICAR group (26.4% vs. 16.9%, p = 0.03). Overall long-term survival was similar in both groups in the Kaplan-Meier analysis. CONCLUSION: Omitting ICAR during thoracoabdominal aortic replacement may reduce operation and cross-clamp times and thus minimize the duration of intraoperative spinal cord hypoperfusion.

Antibacterial copper-filled TiO2 coating of cardiovascular implants to prevent infective endocarditis-A pilot study.

BACKGROUND: Infective endocarditis (IE) poses a significant health risk, especially in patients with prosthetic heart valves. Despite advances in treatment, mortality rates remain high. This study aims to investigate the antibacterial properties of a copper titanium dioxide (4× Cu-TiO2) coating on cardiovascular implants against Staphylococcus aureus, a common causative agent of IE. METHODS: Titanium oxide carriers functionalized with copper ions were employed as an antibacterial coating for heart and vascular prostheses. The coating's antibacterial efficacy was assessed using S. aureus ATCC 29213. Microscopic evaluations were conducted on both biological and artificial materials. Antibacterial activity was qualitatively assessed via a modified disc diffusion method and quantitatively measured through colony counts in NaCl suspensions. RESULTS: The coating process was successfully applied to all tested cardiovascular prosthetic materials. Qualitative assessments of antibacterial effectiveness revealed an absence of bacterial growth in the area directly beneath the coated valve. Quantitative evaluations showed a significant reduction in bacterial colonization on coated mechanical valves, with 2.95 × 104 CFU per valve, compared to 1.91 × 105 CFU in control valves. CONCLUSIONS: The 4× Cu-TiO2 coating demonstrated promising antibacterial properties against S. aureus, suggesting its potential as an effective strategy for reducing the risk of bacterial colonization of cardiovascular implants. Further studies are needed to assess the longevity of the coating and its efficacy against other pathogens.

Acute right atrial thrombus formation after extracorporeal life support removal during LVAD-implantation.

A 58-year-old male underwent LVAD-Implantation after ECLS explantation. After removal of ECLS (A) transesophageal echocardiography revealed thrombus in the inferior vena cava (B) and right atrium (C). The thrombus was removed with a second pump run including RVAD-Implantation. (D) The diameter of thrombus formations was 6 × 1 cm and 5 × 1.5 cm.

Right Axillary Artery Cannulation for Veno-Arterial Extracorporeal Membrane Oxygenation in Postcardiotomy Patients: A Single-Center Experience.

Background and Objectives: To analyze the patient outcome and complication rate of axillary artery cannulation for veno-arterial extracorporeal membrane oxygenation (VA-ECMO) in patients who could not be weaned from cardiopulmonary bypass after cardiothoracic surgery. Materials and Methods: We analyzed the data of 179 patients who were supported with VA-ECMO with femoral-axillary access (FA VA-ECMO) after cardiothoracic surgery between January 2014 and January 2019 in our department. Patients requiring central aortic cannulation and patients with respiratory failure requiring veno-venous ECMO were excluded. Primary outcomes were in-hospital mortality and 1-year survival rate of patients who were weaned from VA-ECMO support. Secondary outcomes were cannulation-related complications at the axillary site, VA-ECMO-related complications, and systemic complications. Results: In our cohort, 60 (33.5%) patients were female. Mean age was 67.0 ± 10.9 years. Overall, 78 (43.5%) patients were operated upon electively, 37 (20.7%) patients underwent urgent surgery, and 64 (35.8%) patients underwent emergency surgical treatment. Sixty-seven patients (37.4%) were resuscitated preoperatively. The mean duration of VA-ECMO support was 8.4 ± 5.1 days. Weaning from VA-ECMO was successful in 87 (48.6%) patients; 62 (34.6%) patients survived the hospital stay. The 1-year survival rate was 74%. Subclavian bleeding occurred in 24 (13.4%) patients, femoral bleeding in 4 (2.2%) patients, ischemia of the upper limb in 11 (6.1%) patients, intracerebral bleeding in 9 (5%) patients, and stroke in 19 (10.6%) patients. Conclusions: In patients with acute LV dysfunction after cardiothoracic surgery who cannot be weaned from cardiopulmonary bypass, right axillary artery cannulation is a safe and reliable method for VA-ECMO support with an acceptable complication rate.

Expanding the Minimally Invasive Approach towards the Ascending Aorta-A Practical Overview of the Currently Available Techniques.

Minimally invasive techniques have gained immense importance in cardiovascular surgery. While minimal access strategies for coronary and mitral valve surgery are already widely accepted and often used as standard approaches, the application of minimally invasive techniques is currently expanded towards more complex operations of the ascending aorta as well. In this new and developing field, various techniques have been established and reported ranging from upper hemisternotomy approaches, which allow even extensive operations of the ascending aorta to be performed through a minimally invasive access to sternal sparing thoracotomy strategies, which completely avoid sternal trauma during ascending aorta replacements. All of these techniques place high demands on patient selection, preoperative planning, and practical surgical implementation. Application of these strategies is currently limited to high-volume centers and highly experienced surgeons. This narrative review gives an overview of the currently available techniques with a special focus on the practical execution as well as the advantages and disadvantages of the currently available techniques. The first results demonstrate the practicability and safety of minimally invasive techniques for replacement of the ascending aorta in a well-selected patient population. With success and complication rates comparable to classic full sternotomy, the proof of concept for minimally invasive replacement of the ascending aorta is now achieved.

Hemoadsorption in LVAD Surgery: Suitable in Theory?

The incidence of patients with heart failure is growing steadily, particularly due to advancements in medical therapy [...].

Myocardial Function after Coronary Artery Bypass Grafting in Patients with Preoperative Preserved Left Ventricular Ejection Fraction-The Role of the Left Ventricular Longitudinal Strain.

Background and Objectives: The role of coronary artery bypass grafting (CABG) on postoperative left ventricular (LV) function in patients with preoperatively preserved left ventricular ejection fraction (LVEF) is still being discussed and only a few studies address this question. This study aimed to assess LV function after CABG in patients with preoperatively preserved LVEF using left ventricular longitudinal strain assessed by 2D speckle tracking imaging (STI). Materials and Methods: Fifty-nine consecutive adult patients with coronary artery disease (CAD) referred for a first-time elective CABG surgery were enrolled in the final analysis of this prospective single-center clinical study. Transthoracic echocardiography (TTE), with conventional measures and STI measures, was performed within 1 week before CABG as well as 4 months after surgery. Patients were divided into groups based on their preoperative global longitudinal strain (GLS) value. Differences in systolic and diastolic parameters between groups were analyzed. Results: Preoperative GLS was reduced (GLS < -17%) in 39% of the patients. Parameters of systolic LV function were significantly reduced in this group of patients compared to the patient group with GLS% ≥ -17%. In both groups, 4 months after CABG there was a decline in LVEF but statistically significant only in the group with GLS% ≥ -17% (p = 0.035). In patients with reduced GLS, there was a statistically significant postoperative improvement (p = 0.004). In patients with preoperative normal GLS, there was not a significant change in any strain parameters after CABG. There was an improvement in diastolic function parameters measured by Tissue Doppler Imaging (TDI) in both groups. Conclusions: There is improvement in LV systolic and diastolic function after CABG in patients with preserved preoperative LVEF measured by STI and TDI. GLS might be more sensitive and effective than LVEF for monitoring improvements in myocardial function after CABG surgery in patients with preserved LVEF.

Management of Coronary Artery Disease in CADASIL Patients: Review of Current Literature.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common heritable form of vascular dementia in adults. It is well-established that CADASIL results in neurocognitive dysfunction and mood disturbance. There is also cumulative evidence that CADASIL patients are more susceptible to ischemic heart disease. The aim of this study is to review the current literature regarding the incidence of coronary artery disease in CADASIL patients with a focus on the various management options and the clinical challenges associated with each of these treatment strategies. We conducted a literature search using Cochrane, MEDLINE, and EMBASE for papers that reported the occurrence of coronary artery disease in patients with CADASIL. We supplemented the search with a manual search in Google Scholar. Only case reports, case series, and original articles were included. The search resulted in six reports indicating the association between coronary artery disease and CADASIL and its management. Evidence suggests that extracranial manifestations of CADASIL may include coronary artery disease, presenting as a more extensive burden of disease in younger patients. Surgical and percutaneous revascularization strategies are feasible, but the incidence of peri-procedural stroke remains significant and should be weighed against the potential benefit derived from either of these strategies. A multidisciplinary approach to therapy, with perspectives from neurologists, cardiologists, and cardiac surgeons, is needed to provide the appropriate treatment to the CADASIL patient with severe coronary artery disease. Future studies should be directed toward the development of targeted therapies that may help with the early detection and prevention of disease progress in these patients.