Aortic valve sparing root surgery for Marfan syndrome

Matalanis, George; Perera, Nisal K.

doi:10.21037/acs.2017.11.05

Cited by 8 publications

(3 citation statements)

References 19 publications

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“…Different repair techniques like subcommissural plication, stabilizing of the cusps, or cusp extension are now an essential part of the armamentarium of surgeons specialized in valvular surgery (16). All these techniques can now be applied in the increasing number of ACHD presenting with aortic root dilation with or without aortic regurgitation, as well as for patients with the pulmonary root in aortic position (17,18).…”

Section: Valve Sparing Aortic Root Replacementmentioning

confidence: 99%

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

Hörer

2018

Cardiovasc. Diagn. Ther

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Today, more than two thirds of patients with congenital heart disease (CHD) are adults. Cardiac surgery plays an essential role in restoring and maintaining cardiac function, aside from evolving medical treatment and catheter-based interventions. The aim of the present publication was to describe the spectrum of operations performed on adults with CHD (ACHD) by reviewing current literature. Currently, surgery for ACHD is predominantly valve surgery, since valvular pathologies are often either a part of the basic heart defect or develop as sequelae of corrective or palliative surgery. Surgical techniques for valve repair, established in patients with acquired heart disease (non-ACHD), can often be transferred to ACHD. New valve substitutes may help to reduce the number of redo operations. Most of valve operations yield good results in terms of survival and quality of life, with the precondition that the ventricular function is preserved. Heart failure due to end-stage CHD is the most frequent cause of mortality in ACHD. However, surgical treatment by means of mechanical circulatory support (MCS) is still uncommon and the mortality exceeds the one following other operations in ACHD. Currently, different devices are used and new technical developments are in progress. However, there still is no ideal assist device available. Therefore, heart transplantation remains the only valid option for end-stage CHD. Despite higher early mortality following heart transplantation in ACHD compared to non-ACHD, the long-term survival compares favorably to non-ACHD. There is room for improvement by refining the indications, the time of listing, and the perioperative care of ACHD transplant patients. Sudden death is the second most frequent cause of mortality in ACHD. Ventricular tachycardia is the most frequent cause of sudden death followed by coronary artery anomaly. Due to the increasing awareness of physicians and the improved imaging techniques, coronary artery anomalies are coming more into the focus of cardiac surgeons. However, the reported experience is limited and it is currently difficult to provide a standardized and generally applicable recommendation for the indication and the adequate surgical technique. With the increasing age and complexity of ACHD, treatment of rhythm disturbances by surgical ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation and resynchronisation gains importance. A risk score specifically designed for surgery in ACHD is among the newest developments in predicting the outcome of surgical treatment of ACHD. This evidence-based score, derived from and validated with data from the Society of Thoracic Surgeons Congenital Heart Surgery Database, enables comparison of risk-adjusted performance of the whole spectrum of procedures performed in ACHD and helps in understanding the differences in surgical outcomes. The score is thus a powerful tool for quality control and quality improvement. In conclusion, new developments in surgery for ACHD are currently made with regard to ...

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Section: Valve Sparing Aortic Root Replacementmentioning

confidence: 99%

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

Hörer

2018

Cardiovasc. Diagn. Ther

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“…The vast majority (80%) of patients with MFS experience root dilatation in the form of annuloaortic ectasia (characterized by a pear-shaped proximal aorta) [19]. Aortic root dilatation, both STJ and aortic annulus level, contribute to valve malfunction by reduction of the coaptation zone [20]. Gradual dilatation at the level of the STJ may also cause compensatory stretching and elongation of the free edge of the cusps.…”

Section: Discussionmentioning

confidence: 99%

Multicentre experience with valve-sparing aortic root replacement by means of combined remodelling and external aortic ring annuloplasty in patients with Marfan syndrome

Accord,

Mecozzi,

Aalberts

et al. 2023

Interdisciplinary CardioVascular and Thoracic Surgery

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OBJECTIVES The most recent valve-sparing root replacement technique combines the advantages of the reimplantation (David) and remodelling (Yacoub) techniques. The aortic root is reconstructed according to the remodelling technique, the aortic valve is repaired according to the principle of effective height, and an external ring provides annular support. The purpose of this study was to evaluate operative and mid-term outcomes using this technique in patients with Marfan syndrome. METHODS Adult patients with Marfan syndrome who had an indication for aortic root surgery according to European Society of Cardiology guidelines and were operated on using this new root replacement technique were retrospectively evaluated. Follow-up was obtained from standard outpatient visits and included echocardiography. RESULTS The study group comprised 22 patients (mean age 36 years, 68% males). Mean follow-up was 7.5 years. There were no mortalities. Two patients required aortic valve replacement because of aortic regurgitation. In both patients, the aortic root was severely dilated (≥65 mm) preoperatively, with grade III aortic valve regurgitation and aortic valve cusps that were very fragile. Aortic regurgitation was grade ≤I on follow-up in 18 of the remaining 20 patients. CONCLUSIONS Valve-sparing root replacement using remodelling combined with aortic-ring annuloplasty is safe in patients with Marfan syndrome. The mid-term outcome is promising in patients undergoing elective valve-sparing root replacement at recommended root diameters. However, in patients with extremely dilated aortic roots and already severe aortic regurgitation, the technique should be used cautiously as aortic cusps are fragile and might not be suitable for durable repair. Clinical registration number UMCG Research registry #11208

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“…Progressive annular or STJ dilatation is known to cause leaflet malcoaptation with a central regurgitant jet ( Figure 3 ) ( 39 , 43 , 44 ). Isolated autograft sinus dilatation on the other hand, is less likely to lead to AR ( 45 ).…”

Section: Clinical Failure Mechanismsmentioning

confidence: 99%

Understanding Pulmonary Autograft Remodeling After the Ross Procedure: Stick to the Facts

Hoof

Verbrugghe

Jones

et al. 2022

Front. Cardiovasc. Med.

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The Ross, or pulmonary autograft, procedure presents a fascinating mechanobiological scenario. Due to the common embryological origin of the aortic and pulmonary root, the conotruncus, several authors have hypothesized that a pulmonary autograft has the innate potential to remodel into an aortic phenotype once exposed to systemic conditions. Most of our understanding of pulmonary autograft mechanobiology stems from the remodeling observed in the arterial wall, rather than the valve, simply because there have been many opportunities to study the walls of dilated autografts explanted at reoperation. While previous histological studies provided important clues on autograft adaptation, a comprehensive understanding of its determinants and underlying mechanisms is needed so that the Ross procedure can become a widely accepted aortic valve substitute in select patients. It is clear that protecting the autograft during the early adaptation phase is crucial to avoid initiating a sequence of pathological remodeling. External support in the freestanding Ross procedure should aim to prevent dilatation while simultaneously promoting remodeling, rather than preventing dilatation at the cost of vascular atrophy. To define the optimal mechanical properties and geometry for external support, the ideal conditions for autograft remodeling and the timeline of mechanical adaptation must be determined. We aimed to rigorously review pulmonary autograft remodeling after the Ross procedure. Starting from the developmental, microstructural and biomechanical differences between the pulmonary artery and aorta, we review autograft mechanobiology in relation to distinct clinical failure mechanisms while aiming to identify unmet clinical needs, gaps in current knowledge and areas for further research. By correlating clinical and experimental observations of autograft remodeling with established principles in cardiovascular mechanobiology, we aim to present an up-to-date overview of all factors involved in extracellular matrix remodeling, their interactions and potential underlying molecular mechanisms.

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Aortic valve sparing root surgery for Marfan syndrome

Cited by 8 publications

References 19 publications

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

Current spectrum, challenges and new developments in the surgical care of adults with congenital heart disease

Multicentre experience with valve-sparing aortic root replacement by means of combined remodelling and external aortic ring annuloplasty in patients with Marfan syndrome

Understanding Pulmonary Autograft Remodeling After the Ross Procedure: Stick to the Facts

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