Mitral valve surgery in patients with Marfan syndrome.

OBJECTIVE: To review the pathology of the mitral valve (MV) and long-term outcomes of surgery in patients with Marfan syndrome (MFS). PATIENTS AND METHODS: From 1988 through 2020, 60 patients with MFS had surgery to correct mitral regurgitation (MR): 19 had isolated MV surgery, 32 had combined MV and aortic root surgery, and 9 had MV surgery after aortic root surgery. Follow-up was complete for a median of 16.1 years. RESULTS: MV pathology was myxomatous degeneration in all patients and of advanced degree in 78.6% with bileaflet prolapse in 65.5%, mitral annulus disjunction in 57.5%, and mitral annulus calcification in 8.2% of patients. The MV was repaired in 47 patients and replaced in 13. Kaplan-Meier estimates of cumulative mortality at 20 years 21.3% for all patients, 6.7% after MV repair, and 57.8% after replacement (P < .001). MV reoperations were performed in 5 patients: 2 after repair and 3 after replacement. The cumulative incidence rate of reoperations on the MV was 3.8% at 10 years and 11.0% at 20 years in the entire cohort. Among 47 patients who had MV repair, moderate MR developed in 11 patients and severe in 2. Both patients with severe MR underwent MV reoperation. The cumulative incidence rate of recurrent moderate or severe MR after MV repair was 20.4% at 10 years, and 36.5% at 20 years. CONCLUSIONS: MV repair was associated with better survival than MV replacement, but recurrent MR after repair occurred in approximately one-third of the patients at 20 years after surgery.

Long-term outcomes following valve-sparing root replacement with concomitant mitral repair.

OBJECTIVES: To examine the late outcomes of valve-sparing root replacement and concomitant mitral valve repair in patients who have been followed prospectively for more than 2 decades. METHODS: From 1992 to 2020, 54 consecutive patients (mean age, 47 ± 16 years; 80% men) underwent valve-sparing root replacement (45 reimplantation and 9 remodeling) with concomitant repair of the mitral valve. Patients were followed prospectively for a median of 9 years (IQR, 3-14 years). RESULTS: No patient experienced perioperative death or stroke. There were 3 late deaths and the 15-year overall survival was 96.0% (95% CI, 74.8%-99.4%), similar to the age- and sex-matched population. Over the follow-up period, 6 patients had reoperation of the aortic valve and 3 on the mitral valve. Of those, 2 had reoperation on both aortic and mitral valves for a total of 7 reoperations in this cohort. The cumulative proportion of reoperation at 10 years of either or both valves were as follows: aortic valve 11.4% (95% CI, 3.9%-33.3%), mitral valve 4.2% (95% CI, 0.6%-28.4%), and both valves 11.4% (95% CI, 3.9%-33.3%). The estimated probability of developing moderate/severe aortic insufficiency at 15 years was 18.5% (95% CI, 9.0%-34.2%). On final echocardiographic follow-up, none of the patients had developed moderate/severe mitral regurgitation. CONCLUSIONS: In this single-center series of concomitant valve-sparing root replacement and mitral valve repair, we observed excellent clinical outcomes with a low risk of death or valve-related complications. Continued surveillance of late valve function is necessary.

Valve-Sparing Aortic Root Replacement State-of-the-Art Review, Part I: Anatomy and Physiology.

Aortic valve disease is common, and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging, but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long-term results vary based on the underlying anatomy, pathology, and patient selection, as well as surgeon expertise. The first installment of this Valve-Sparing Aortic Root Replacement State-of-the-Art Review article addresses patient anatomy and physiology as it relates to candidacy for VSRR.

Valve-Sparing Aortic Root Replacement State of the Art Review Part II: Surgical Techniques.

Aortic valve disease is common and valve-preserving operations are preferred whenever possible. Valve-sparing aortic root replacement (VSRR) has become an important tool for managing aortic root pathology in children and adults. The learning curve for this operation is challenging but with increasing experience and technical modifications, early and late outcomes continue to improve. Durable long term results vary based on underlying anatomy, pathology, and patient selection, and surgeon expertise. The purpose of Part II of this VSRR State of the Art Review article is to provide technical pearls related to VSRR.

Aortic Valve-Sparing Operations.

Aortic valve-sparing operations were developed >3 decades ago and have become part of the surgical armamentarium to treat patients with aortic root and ascending aortic aneurysms. Although remodeling of the aortic root is physiologically sounder than reimplantation of the aortic valve, most surgeons seem to prefer the latter. This review summarizes the views of one of the pioneers of aortic valve-sparing operation and includes their development, the various modifications that occurred over the years, comparative outcomes with aortic root replacement with valved conduits, outcomes from several recently reported series, and the future directions. The author argues that comparisons between aortic valve-sparing operations and aortic root replacement with valved conduits based on retrospective studies are inappropriate because these 2 types of surgery are performed in patients with different aortic valve pathology. Late outcomes after aortic valve-sparing operations have not been consistent among various reports and raise the question of surgical expertise and patients' selection. Aortic valve-sparing operations are the best treatment for young patients with aortic root aneurysm and normal aortic cusps. Further studies are needed to define their role in the management of patients with diseased aortic cusps.

Management of Adults With Anomalous Aortic Origin of the Coronary Arteries: State-of-the-Art Review.

As a result of increasing adoption of imaging screening, the number of adult patients with a diagnosis of anomalous aortic origin of the coronary arteries (AAOCA) has grown in recent years. Existing guidelines provide a framework for management and treatment, but patients with AAOCA present with a wide range of anomalies and symptoms that make general recommendations of limited applicability. In particular, a large spectrum of interventions can be used for treatment, and there is no consensus on the optimal approach to be used. In this paper, a multidisciplinary group of clinical and interventional cardiologists and cardiac surgeons performed a systematic review and critical evaluation of the available evidence on the interventional treatment of AAOCA in adult patients. Using a structured Delphi process, the group agreed on expert recommendations that are intended to complement existing clinical practice guidelines.

Reimplantation of the aortic valve in patients with tricuspid aortic valve: the Toronto General Hospital experience.

Background: Aortic valve sparing operations were introduced three decades ago but controversy remains regarding its appropriateness, reproducibility and durability. This article describes the long-term outcomes of patients who had reimplantation of the aortic valve. Methods: All patients who had reimplantation of a tricuspid aortic valve at Toronto General Hospital from 1989 through 2019 were selected for this study. Patients were followed prospectively with periodical clinical assessments and imaging of the heart and aorta. Results: Four hundred and four patients were identified. The median age was 48.0 [interquartile range (IQR), 35.0-59.0] years and 310 (76.7%) were men. There were 150 patients with Marfan syndrome, 20 with Loeys-Dietz syndrome and 33 with acute or chronic aortic dissections. The median follow-up was 11.7 (IQR, 6.8-17.1) years. There were 55 patients alive and without reoperation at 20 years. The cumulative mortality at 20 years was 26.7% [95% confidence interval (CI): 20.6-34.2%], the cumulative incidence of reoperation on the aortic valve was 7.0% (95% CI: 4.0-12.2%) and the development of moderate or severe aortic insufficiency was 11.8% (95% CI: 8.5-16.5%). We could not identify variables associated with reoperation on the aortic valve or with the development of aortic insufficiency. New distal aortic dissections were common in patients with associated genetic syndromes. Conclusions: Reimplantation of the aortic valve in patients with tricuspid aortic valve provides excellent aortic valve function during the first two decades of follow-up. Distal aortic dissections are relatively common in patients with associated genetic syndromes.

Outcomes of Reimplantation of the Aortic Valve in Patients With Aortic Cusp Fenestration.

BACKGROUND: Aortic cusp fenestrations are common in patients with aortic root aneurysm, and their management during aortic valve repair remains controversial. We believe that fenestrations in the area of the commissures may rupture after reimplantation of the aortic valve because this operation increases the mechanical stress on the cusps. For this reason we have reinforced the free margin of the aortic cusp with fenestration with fine Gore-Tex sutures (WL Gore). This study examines the outcomes of reimplantation of the aortic valve in patients who had cusp fenestration reinforced with Gore-Tex sutures. METHODS: A review of all patients who had reimplantation of the aortic valve for aortic root aneurysm disclosed 111 patients who had at least 1 cusp fenestration reinforced with a double layer of a fine Gore-Tex suture. The outcomes of these patients were examined and compared with a group of patients without fenestration using propensity score analysis. All patients were followed prospectively with images of the heart. RESULTS: The median follow-up was 8.3 years. Overall the cumulative incidence of aortic valve reintervention at 15 years was 4.8% and the cumulative incidence of aortic insufficiency of moderate or severe degree was 9.2%. Comparison of outcomes of patients with and without fenestrations showed similar results up to 15 years of follow-up. CONCLUSIONS: Reinforcement of the free margins of cusps with fenestrations using Gore-Tex sutures is safe and does not seem to adversely affect the durability of reimplantation of the aortic valve.