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Otto et al 2020 ACC/AHA Guideline for the Management of Valvular Heart Disease

Circulation. 2021;143:e72–e227. DOI: 10.1161/CIR.0000000000000923 February 2, 2021 e135 CLINICAL STATEMENTS AND GUIDELINES

rate) in the absence of RV dysfunction or pulmonary hypertension. 6.	 Isolated tricuspid valve surgery for severe TR historically has been performed relatively late in the natural history of the disease, when patients have become symptomatic with signs of right-sided HF. Unadjusted mortality rates for isolated tricuspid valve surgery have therefore exceeded those reported for isolated aortic or mitral valve surgery, and this trend has been even more pronounced for reoperative tricuspid surgery late after left-sided valve surgery.1,2,39 This high reoperative mortality rate is likely related to the advanced nature of RV failure encountered at the time of the second procedure, residual pulmonary hypertension, LV dysfunction, and other valve abnormalities. The hazards imposed by reoperation have influenced decision-making for initial repair of functional TR at the time of left-sided valve surgery in an attempt to prevent the development of severe TR later after the left-sided valve surgery. However, if there is no significant pulmonary hypertension or severe RV systolic dysfunction, operation for severe symptomatic isolated TR years after surgery for leftsided disease may improve symptoms of right-sided HF, if done before the onset of severe RV dysfunction or end-organ damage with either hepatic or renal dysfunction.11,18

9 PULMONIC VALVE DISEASE

See guidelines for the management of adults with congenital heart disease.1

10 MIXED VALVE DISEASE

10.1 Diagnosis of Mixed VHD

Recommendations for Diagnosis and Follow-Up of Patients With Mixed Valve Disease COR LOE Recommendations

1 C-EO 1.  For patients with mixed valve disease, TTE is recommended to assess the etiology, severity, and pathophysiological impact.

2a C-EO 2.  In patients with ambiguous symptoms that are suspected to be attributable to mixed mitral valve disease, further assessment of filling pressure by using biomarkers or invasive hemodynamic measurements at rest or with exercise is reasonable.

Synopsis

Mixed valve disease is either 1) stenosis and regurgitation of a single valve or 2) stenosis or regurgitation of 2 separate valves. Mixed valve disease presents a special diagnostic challenge to the clinician in assessing the impact of the lesions on cardiac remodeling, ventricular function, and timing of intervention.1–5 For many patients with mixed valve disease, there is a predominant valve lesion (ie, stenosis versus regurgitation; mitral versus aortic), and symptoms and pathophysiology resemble those of a pure dominant lesion. When pressure overload predominates, there is usually concentric hypertrophy, whereas volume overloads cause chamber dilation and eccentric hypertrophy; management should follow the guidelines for the predominant lesion. However, in other cases, patients present with a more balanced picture, with the mixed pathophysiology making patient management difficult. It may be that neither lesion by itself reaches Stage C as described in previous sections for pure lesions, yet the lesions may be, in combination, severe enough to impact outcome. Mixed valve disease was primarily attributable to rheumatic disease in the past, but it is now more frequently seen with degenerative disease or after prior chest radiation.5 Decision-making for patients with mixed valve disease is frequently complex and may require referral to or consultation with a Comprehensive Valve Center.

Recommendation-Specific Supportive Text

1.	 The complex nature of mixed valve disease requires a comprehensive imaging approach that involves assessing each lesion separately and then collectively judging how the lesions affect the patient’s overall presentation. TTE is the standard modality for measuring jet velocities, valve areas, regurgitant flow, and regurgitant orifice areas. TTE establishes the baseline for pathoanatomy and pathophysiology from which comparison is made as the lesions progress over time. Doppler hemodynamics have been validated for patients with single-valve disease but have not necessarily been studied in patients with multivalve disease. Limitations exist for assessment of calculations, such as those for valve areas, because of differential flows with multivalve disease.2–5 2.	 The complex nature of mixed valve disease makes it necessary to consider all available data to reach a final management decision. Although natural history data for many types of mixed valve disease are lacking, it is reasonable to assume that the onset of symptoms is a negative prognostic occurrence, as it is for all other valve lesions. The difficulty may lie in attributing such symptoms to the mixed valve disease at hand, especially if TTE demonstrates moderate but not severe mixed disease. Elevated BNP and elevated filling pressures at catheterization, either at rest or with exercise, support that cardiac disease is the cause of the patient’s symptoms and may help to further quantify lesion severity.

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