52. Combined Aortic and Mitral Valve Disease- Indications and Guidelines

Shawn Shah MD MBA, Haytham Elgharably MD
Cleveland Clinic
January 15, 2025

Abbreviations & Definitions

ACC/AHA – American College of Cardiology/American Heart Association
AI – Aortic insufficiency
AMC – Aortomitral curtain
AS – Aortic stenosis
AVR – Aortic valve replacement
EF – Ejection fraction
ESD – End systolic diameter
LHC – Left heart catheterization
LVESD – Left ventricular end systolic diameter
MAC – Mitral annular calcification
MR – Mitral regurgitation
MS – Mitral stenosis
MVR – Mitral valve replacement
MVr – Mitral valve repair
NYHA – New York Heart Association
PASP – Pulmonary artery systolic pressure
PMBC – Percutaneous mitral balloon commissurotomy
RHC – Right heart catheterization
SAVR – Surgical aortic valve replacement
TAVR – Transcatheter aortic valve replacement
TEE – Transesophageal echocardiogram
TEER – Transcatheter edge-to-edge repair
TMVR – Transcatheter mitral valve replacement
TTE – Transthoracic echocardiogram

[#] – ACC/AHA Class Recommendation

Indications & Guidelines for Management by Grade/Stage of Disease

Patients with combined aortic and mitral valve disease present a unique challenge due to their hemodynamic interdependency. The potential benefits of a complete correction of multivalvular disease must be weighed against the greater observed risks of a more complex surgery. An analysis of the STS Adult Cardiac Surgery Database from 2011-2019 demonstrated a 2-4x higher operative mortality for combined aortic and mitral valve intervention (9%) compared to isolated aortic (2.2%) or mitral valve (5%).1

The 2020 ACC/AHA Guidelines2 do not publish specific indications for mixed aortic and mitral valve disease due to a paucity of high-quality studies examining this population; however, allusions to multivalvular management are contained within the individual sections for isolated valvular disease. As with single valve disease, the decision for intervention depends on the severity of each valve lesion, patient symptoms, left ventricular function, and overall surgical risk. Importantly, all patients with mixed valve disease should be evaluated by a comprehensive heart team.

In general, a patient with combined aortic and mitral valve disease meets indications for surgery when one of their isolated lesions meets established guidelines for surgery (severe AS/AI/MS/MR + symptoms or LV dysfunction). It is impossible to determine whether symptoms can be attributed to one or both valvular lesions. Therefore, the decision to intervene on the other valve depends on the degree of dysfunction as well as the presence of identifiable pathology amenable to repair. Mild lesions are almost never intervened upon, unless it is felt that the severity is underreported. Conversely, severe concomitant lesions should almost always be addressed at the time of intervention on the other valve.

Of note, endocarditis is a common indication for double valve surgery, which is discussed in a separate chapter.

AS AI MR MS
Class I indications to intervene on isolated valve dysfunction Severe AS + symptoms, LVEF <50%, or at time of other cardiac surgery Severe AI + symptoms, LV <55%, or at time of other cardiac surgery Primary MR: Severe MR + symptoms, EF <60%, or ESD >40 mm Secondary MR: No Class I indications to intervene (GDMT only) Severe MS + symptoms
Surgical vs endovascular approach SAVR: Age <65, life expectancy >20 years TAVR: Age >80, life expectancy <10 years, prohibitive surgical risk Gray area: Age 65-80, medium risk SAVR only MVr: Gold standard for primary MR TEER: Prohibitive surgical risk patients with primary MR OR anatomically favorable secondary MR with EF 20-50%, LVESD <70 mm, and PASP <70 mmHg Rheumatic: PMBC if candidate (<2+ MR, pliable leaflets, no clots); otherwise, MVR Nonrheumatic: MVR (rarely MVr)
Considerations to intervene on concomitant valvular disease Patients undergoing AVR with concomitant: Primary MR Mild: no intervention Moderate: MVr [IIA] Severe: MVr/R [I] MVr>MVR for primary MR Secondary MR Mild: no intervention Moderate: MVr/R [IIb] Severe: MVr/R [IIA] (Class I: medical mgmt) MVr vs MVR is controversial, no clear guidelines MS No established guidelines for when to intervene on mild-moderate MS (rheumatic or nonrheumatic) at the time of AVR Barring severe MAC or other complex anatomic barriers, reasonable to pursue MVr/MVR at the time of AVR for moderate or severe MS Patients undergoing MVr/MVR with concomitant: AS Mild: no intervention Moderate: SAVR [IIA] Severe: SAVR [I] Patients with rheumatic MS may benefit from staged PMBC followed by SAVR/TAVR when criteria are met AI Mild: no intervention Moderate: SAVR [IIA] Severe: SAVR [I]

Summary Table: Management of Combined Aortic and Mitral Valve Disease (2020 ACC/AHA Guidelines).

Supporting Evidence for Current Indications & Guidelines

The above summary table highlights the following important points:

  • Patients with mixed aortic and mitral valve disease meet the indications for surgery based on a predominant lesion involving one of the valves.
  • The severity of the second valve lesion primarily determines the decision to intervene. Mild lesions are almost never intervened upon, and severe lesions are almost always intervened upon due to their hemodynamic consequence. The decision to intervene on a moderate secondary valve lesion is nuanced and discussed further below.
  • High-risk surgical patients should be considered for transcatheter options to treat the predominant valve lesion. Subsequent staged intervention of the second valve lesion is offered in some patients.

Severe AS + moderate-severe MR in surgical candidates

This represents the most encountered combination of mixed aortic + mitral valve disease in surgical practice.

In addition to severity, the etiology of the MR influences the decision to intervene as well as the technique.

Primary MR usually does not improve after AVR alone, as the mechanism is intrinsic to the mitral valve rather than the LV loading conditions. Therefore, the ACC/AHA gives a IIA recommendation (“is reasonable”) to address moderate primary MR during aortic valve surgery. Importantly, the probability of a successful repair is a critical consideration due to the well-established superiority of MVr over MVR for degenerative MR.

Secondary MR may improve after AVR through a combination of LV remodeling and reduced LV afterload; however, secondary MR can persist in many cases. The ACC/AHA gives a IIB recommendation (“may be reasonable”) for mitral intervention in patients with moderate secondary MR.

No randomized trials exist for this population. A close analogue is the 2016 CTSN trial randomizing patients with moderate ischemic MR to CABG alone vs. CABG + MVr.3 Importantly, two-thirds of patients undergoing CABG alone experienced improvement in their MR at follow-up. While patients receiving CABG + MVr had greater resolution of their MR, there were no differences in mortality, LV remodeling parameters, or readmissions. The CABG + MVr group also experienced higher early morbidity, reflecting the incremental surgical risk. This study supports the recommendation that routine mitral intervention should not be performed on moderate secondary MR.

All patients with secondary MR should have a preoperative LHC to determine any need for revascularization.

The superiority of MVr over MVR for severe primary MR is well established. In patients with severe secondary MR, the optimal intervention is more controversial.

A landmark 2014 CTSN trial randomizing patients with severe ischemic MR to either MVr or MVR yielded no long-term differences in mortality or LV remodeling.4 However, the repair group had a 15x higher recurrence of moderate-to-severe MR at 2 years (~60% vs 4%) with higher rates of cardiovascular-related adverse events, suggesting replacement may be more durable.

Severe AS + moderate-severe MR in prohibitive risk patients

  • Minimally invasive strategies, particularly a staged approach with TAVR followed by TEER, have emerged as viable options for elderly patients with multiple comorbidities and frailty. Patients are followed with serial echos after TAVR to evaluate the hemodynamic consequence of persistent MR. In some cases, afterload reduction from TAVR alone may lead to MR improvement, eliminating the need for TEER.
  • There is limited evidence supporting TEER for degenerative MR. It is currently approved for use in secondary MR in high-risk heart failure patients based on a landmark trial:
  • The 2018 COAPT Trial randomized over 600 patients with heart failure and moderate-to-severe secondary MR who were symptomatic despite optimal medical therapy (OMT) to either TEER + OMT vs. OMT alone. The TEER + OMT group had a significant reduction in all-cause mortality and cardiovascular-related hospitalizations at two years. This trial informed the current ACC/AHA guidelines for eligibility of patients for TEER (EF 20-50%, LVEDD <70 mm, PASP <70 mmHg).
  • Moderate to severe primary MR in the setting of severe AS in a moderate-to-high risk patient should push a patient toward open SAVR and mitral intervention (repair or replacement), as benefits for TEER in primary MR have not been established.
  • Prospective registries have been created to collect data for patients with both aortic and mitral pathology undergoing TAVR.
  • A 2021 JACC study observed over 7,000 TAVRs as part of the AMTRAC Valve Registry.5 Approximately 27% of patients had moderate or severe MR that was not addressed. Of those, 44% experienced regression of their MR. Of those with persistent MR, mortality over four years was higher.
  • A second analysis of the AMTRAC Registry followed 100 patients who underwent staged TAVR followed by TEER.6 At 1 year, 90% of patients had mild or less MR, and NYHA functional class improved dramatically (85% stage I/II).
  • It is important to note that valve-in-valve TAVR and TEER cannot be performed through a mechanical valve. The decision of optimal valve replacement type is discussed elsewhere in the isolated aortic valve disease chapter.

Severe AS + moderate-severe MS

  • This combination is seen most frequently in rheumatic heart disease. In younger patients, AS + MS may reflect a congenital syndrome (Shone’s Complex). CT imaging should be performed to rule out concomitant aortopathy.
  • Importantly, treatment of one valvulopathy can worsen the gradient across the other due to hemodynamic interdependency. Treatment of AS will increase the transmitral flow. Conversely, treatment of MS will increase LV preload and stroke volume. Therefore, substantial consideration should be given to treating moderate lesions, which may progress to the severe range if not addressed.
  • The ACC/AHA does not provide recommendations on when to treat moderate MS in patients with AS.
  • Open commissurotomy or partial decalcification can provide some relief of MS with minimal additional risk in borderline lesions.
  • Patients with moderate MS and mitral anatomy favorable for PMBC can undergo SAVR/TAVR alone with staged PMBC at a later date if the MS progresses.
  • Patients with severe MS amenable to PMBC (pliable, noncalcified, no left atrial thrombus) and severe AS should be considered for PMBC first. This is a low-risk procedure that can be staged with subsequent SAVR or TAVR to reduce the perioperative risks associated with double valve replacement.

Severe AI + moderate-severe MR

  • This combination is often seen in connective tissue disorders or in patients with endocarditis. The presence of two regurgitant lesions can cause rapid volume overload and LV dilation; therefore, timely intervention is recommended.
  • Recommendations to intervene on concomitant moderate MR with AI mirror the recommendations for patients with AS. There is an IIA recommendation to intervene on moderate primary MR and an IIB recommendation to intervene on moderate secondary MR. Notably, AI (unlike AS) is a volume overload lesion, which directly contributes to mitral annular dilation and resultant regurgitation. Therefore, AVR alone may allow LV remodeling to reduce MR without the need for intervention, though there is sparse literature directly studying this.

Severe MR/MS + moderate AI/AS

  • In cases where the mitral pathology is predominant, the surgical plan should focus on optimal management of the mitral valve. If a surgical approach is planned, there is an IIA recommendation to replace the aortic valve in moderate or worse AI or AS. This is because both forms of aortic valve disease are progressive and result in ongoing LV strain and failure.
  • Notably, TAVR in patients with an existing mitral valve prosthesis can be more difficult due to the asymmetric rigidity of the aortic valve imposed by the mitral valve sewing ring. This should be considered if a decision is made to defer intervention on moderate AS.

Ongoing Trials/Recent Publications

PRIMARY Trial: Prospective, randomized, parallel-controlled, open-label noninferiority trial enrolling patients with severe primary MR and moderate surgical risk to treatment with either surgical MVr or TEER. This will be one of the most momentous trials in cardiac surgery to inform future practice, with primary completion estimated in 2026.

APOLLO Trial: Single-arm, nonrandomized trial enrolling patients with moderate-to-severe symptomatic MR of any etiology to treatment with transcatheter mitral valve replacement (TMVR). Data collection for the primary endpoint is scheduled to be completed in 2026 and will result in an interim publication of results shortly after.

AMTRAC Registry: Comprehensive, multicenter registry designed to collect and analyze data on patients undergoing TAVR and TMVR. It captures data on patients with mixed aortic and mitral valve disease and has published a series of patients undergoing staged transcatheter intervention (TAVR followed by TEER) as well as concomitant TAVR and TEER during the same procedure.

Expert Commentary

Assessment of multivalvular disease requires integration of clinical presentation and TTE findings. Patients with LV dysfunction, RV dysfunction, and pulmonary hypertension may require RHC to better characterize hemodynamics for perioperative management.

TEE can be utilized to better assess valvular pathology and the possibility of repair. The severity of valve dysfunction should be evaluated in the context of non-physiologic loading conditions due to sedation or general anesthesia during TEE (eg, gradients may be artificially reduced).

The decision to intervene on a concomitant valve lesion (eg, 2+ MR at the time of AVR) should take into account the presence of morphologic changes amenable to repair (dilated annulus, focal prolapse, etc), as well as the patient’s age, complexity, and the probability of a successful repair with minimal additional ischemic time. Mild lesions are almost never intervened on. Conversely, severe lesions almost always require intervention. Moderate lesions require the most nuance and should be discussed in a multidisciplinary setting.

A common, noninfectious etiology for valve dysfunction requiring double valve replacement is radiation heart disease. These patients are complex and often have densely calcified aortomitral curtain (AMC) requiring extensive debridement. Many institutions perform a Commando procedure to reconstruct the AMC; however, this adds significant technical complexity and associated risk. In select patients with partial destruction of the AMC and without active infection, it has been our recent institutional preference to reinforce the AMC with a bovine pericardial patch.7 This technique simplifies operative conduct and maintains the integrity and geometry of the fibrous skeleton of the heart. Early outcomes of the AMC reinforcement technique will be published by our group in a separate work.

Left-sided valve endocarditis can be localized to the leaflets, which is addressed with repair or replacement. In some cases, the infectious pathology can invade across the AMC between the two valves. Based on the degree of invasion and tissue destruction, reconstruction techniques can range from a simple patch repair for focal invasion to a more complex operation for extensive invasion, such as the Commando or Hemi-Commando.8

The use of TEER is controversial. The mechanism by which this procedure addresses mitral pathology resembles the early Alfieri stitch, which has been shown to be a poorly durable repair technique in the surgical literature. While TEER may have limited uses in the very high surgical risk group with secondary MR and heart failure, its implementation for degenerative MR is currently unmerited. The PRIMARY trial will help adjudicate the optimal role for TEER and inform our future guidelines.

Rapid-fire technical tips for multivalve operations:

  • If MVr is planned, the aorta can be left intact to enable pressurizing the LV during saline testing of the mitral valve. After MVr is complete, aortotomy and AVR are performed.
  • For combined AVR/MVR, the aortic valve should be excised and debrided first. Additional debridement of the fibrous trigones can be performed at this time through the aortotomy. Valve sizing and the decision to pursue root enlargement should be performed prior to placement of the mitral prosthesis. After sizing, the mitral valve prosthesis is implanted first, followed by the aortic valve.
  • Orientation of the mitral valve sutures that correspond to the biological mitral prosthesis struts can be checked from the aortic side after suture placement. This ensures optimal positioning and avoids obstruction of the left ventricular outflow tract.
  • During a redo case after a prior AVR, exposure of the mitral valve is limited if the aortic valve prosthesis is left in place, particularly the anterior aspect of the mitral annulus. In some cases, the aortic prosthesis may need to be removed and replaced for adequate exposure of the mitral valve.

Sources

  1. Jacobs JP, Shahian DM, Badhwar V, et al. The Society of Thoracic Surgeons 2021 Adult Cardiac Surgery Risk Models for Multiple Valve Operations. Ann Thorac Surg. 2022;113(2):511-518. doi:10.1016/j.athoracsur.2021.03.089
  2. Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease. J Am Coll Cardiol. 2021;77(4):e25-e197. doi:10.1016/j.jacc.2020.11.018
  3. Michler RE, Smith PK, Parides MK, et al. Two-Year Outcomes of Surgical Treatment of Moderate Ischemic Mitral Regurgitation. N Engl J Med. 2016;374(20):1932-1941. doi:10.1056/NEJMoa1602003
  4. Acker MA, Parides MK, Perrault LP, et al. Mitral-Valve Repair versus Replacement for Severe Ischemic Mitral Regurgitation. N Engl J Med. 2014;370(1):23-32. doi:10.1056/NEJMoa1312808
  5. Witberg G, Codner P, Landes U, et al. Effect of Transcatheter Aortic Valve Replacement on Concomitant Mitral Regurgitation and Its Impact on Mortality. JACC Cardiovasc Interv. 2021;14(11):1181-1192. doi:10.1016/j.jcin.2021.02.030
  6. Witberg G, Codner P, Landes U, et al. Transcatheter Treatment of Residual Significant Mitral Regurgitation Following TAVR. JACC Cardiovasc Interv. 2020;13(23):2782-2791. doi:10.1016/j.jcin.2020.07.014
  7. Zaki A, Witten JC, Pettersson GB, Elgharably H. Patch reconstruction of the aorto-mitral curtain without posterior extension: Alternative to the Commando procedure for double valve replacement. JTCVS Tech. 2023;22:181-184. doi:10.1016/j.xjtc.2023.08.021
  8. Navia JL, Elgharably H, Hakim AH, et al. Long-term Outcomes of Surgery for Invasive Valvular Endocarditis Involving the Aortomitral Fibrosa. Ann Thorac Surg. 2019;108(5):1314-1323. doi:10.1016/j.athoracsur.2019
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