Pediatric Valvar Aortic Stenosis Guidelines

The American College of Cardiology (ACC) and American Heart Association (AHA) released their updated recommendations on managing valvular heart disease in December 2020. ^{[25, 26]} Key messages are outlined below.

Valvular heart disease (VHD) stages (stages A-D) in patients should be classified based on symptoms, valve anatomy, severity of valve dysfunction, and response of the ventricle and pulmonary circulation.

When evaluating patients with VHD, findings from the history and physical examination (PE) should be correlated with those from noninvasive testing (ie, electrocardiography [ECG], chest x-ray, transthoracic echocardiography [TTE]). If conflict exists between results on the PE and that of initial noninvasive studies, consider obtaining further noninvasive (computed tomography [CT], cardiac magnetic resonance imaging [CMRI], stress testing) or invasive (transesophageal echocardiography [TEE], cardiac catherization) studies to decide the optimal treatment strategy.

In the setting of VHD and atrial fibrillation (AF) (except for patients with rheumatic mitral stenosis [MS] or a mechanical prosthesis), the decision to use oral anticoagulation with either a vitamin K antagonist (VKA) or a non-VKA anticoagulant to prevent thromboembolic events should be a shared decision-making process based on the CHA2DS2-VASc score (congestive heart failure [CHF], hypertension, age ≥75 years, diabetes mellitus, previous stroke/transient ischemic attack/thromboembolic event, vascular disease, age 65-74 years, sex). Oral anticoagulation with a VKA should be given to those with rheumatic MS or a mechanical prosthesis and AF.

All those with severe VHD under consideration for valve intervention should be evaluated by a multidisciplinary team, either with a referral or in consultation with a primary or comprehensive valve center.

Treatment of severe aortic stenosis (AS) with either a transcatheter or surgical valve prosthesis should be based primarily on symptoms or reduced ventricular systolic function. Consider earlier intervention if indicated by the results of exercise testing, biomarkers, rapid progression, or the presence of very severe stenosis.

Expanded indications for transcatheter aortic valve implantation (TAVI) are a result of findings from multiple randomized trials of TAVI versus surgical aortic valve replacement (SAVR). The selection of intervention type for patients with severe AS should be a shared decision-making process that considers the lifetime risks and benefits associated with the valve type (mechanical vs bioprosthetic) and approach type (transcatheter vs surgical).

Bioprosthetic valve dysfunction may occur because of either degeneration of the valve leaflets or valve thrombosis. Catheter-based treatment for prosthetic valve dysfunction is reasonable in selected patients for bioprosthetic leaflet degeneration or paravalvular leak in the absence of active infection.

Go to 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: Developed by the Task Force on cardiac pacing and cardiac resynchronization therapy of the European Society of Cardiology (ESC) With the special contribution of the European Heart Rhythm Association (EHRA) for full details.

For more information, please go to Aortic Stenosis, Aortic Regurgitation, Mitral Stenosis, Mitral Regurgitation, and Tricuspid Regurgitation.

For more Clinical Practice Guidelines, please go to Guidelines.

Guidelines for the management of patients with valvular heart disease (VHD) were published in August 2021 by the European Society of Cardiology (ESC) and the European Association for Cardio-Thoracic Surgery (EACTS), ^[27] including the following recommendation on aortic valve disease:

Intervention is recommended for symptomatic patients with severe, high-gradient aortic stenosis or with severe low-flow, low-gradient aortic stenosis with LVEF < 50% and evidence of flow reserve, as well as for asymptomatic patients with severe stenosis who have systolic LV dysfunction (LVEF < 50%) without another cause or who have symptoms on exercise testing.

Aortic Valvuloplasty

In 2011, the American Heart Association (AHA) released guidelines for interventions in pediatric cardiac disease that included the following class I recommended indications for aortic valvuloplasty in children ^[28] :

• In newborns with isolated critical valvar aortic stenosis (AS) who are ductal dependent regardless of valve gradient
• In children with isolated valvar AS who have the following characteristics:

  • Depressed left ventricular systolic function
  • ​Resting peak systolic valve gradient (by catheter) of ≥50 mm Hg
  • Resting peak systolic valve gradient (by catheter) of ≥40 mm Hg in the presence of anginal or syncopal symptoms or ischemic ST-T-wave changes on electrocardiography at rest or with exercise

According to these guidelines, aortic valvuloplasty may be considered in an asymptomatic child or adolescent with a resting peak systolic valve gradient (by catheter) of ≥40 mm Hg or without ST–T-wave changes if pregnancy or participation in strenuous competitive sports is desired (class IIb).

Aortic valve balloon dilation is not indicated in children with isolated valvar AS who also have a degree of aortic regurgitation that warrants surgical aortic valve replacement or repair (class III).

Transcatheter Aortic Valve Replacement (TAVR)

In 2017, the AHA/American College of Cardiology released a focused update of their 2014 guidelines for managing patients with valvular heart disease, including their TAVR recommendations, which are summarized below. ^{[29, 30]}

Anticoagulation with a vitamin K antagonist (VKA) is indicated for patients with rheumatic mitral stenosis (MS) and atrial fibrillation (AF).

Anticoagulation is indicated in patients with AF and a CHA2DS2-VASc score of 2 or greater with native aortic valve disease, tricuspid valve disease, or mitral regurgitation (MR).

Surgical aortic valve replacement is recommended for symptomatic patients with severe AS (stage D) and asymptomatic patients with severe AS (stage C) who meet an indication for AVR when surgical risk is low or intermediate.

TAVR is recommended for symptomatic patients with severe AS (stage D) and a prohibitive risk for surgical AVR who have a predicted post-TAVR survival greater than 12 months.

Mitral valve surgery is recommended for symptomatic patients with chronic severe primary MR (stage D) and left ventricular ejection fraction (LVEF) greater than 30%.

Mitral valve surgery is recommended for asymptomatic patients with chronic severe primary MR and LV dysfunction (LVEF 30%-60% and/or LV end-systolic diameter [LVESD] ≥40 mm, stage C2).

Mitral valve repair is recommended in preference to mitral valve replacement (MVR) when surgical treatment is indicated for patients with chronic severe primary MR limited to the posterior leaflet.

A bioprosthesis is recommended in patients of any age for whom anticoagulant therapy is contraindicated, cannot be managed appropriately, or is not desired.

Anticoagulation with a VKA and international normalized ratio (INR) monitoring is recommended in patients with a mechanical prosthetic valve.

Anticoagulation with a VKA to achieve an INR of 2.5 is recommended for patients with a mechanical bileaflet or current-generation single-tilting disc AVR and no risk factors for thromboembolism.

Anticoagulation with a VKA is indicated to achieve an INR of 3.0 in patients with a mechanical AVR and additional risk factors for thromboembolic events (AF, previous thromboembolism, LV dysfunction, or hypercoagulable conditions) or an older-generation mechanical AVR (such as ball-in-cage).

Anticoagulation with a VKA is indicated to achieve an INR of 3.0 in patients with a mechanical MVR.

Aspirin 75-100 mg daily is recommended in addition to anticoagulation with a VKA in patients with a mechanical valve prosthesis.

Continuation of VKA anticoagulation with a therapeutic INR is recommended in patients with mechanical heart valves undergoing minor procedures (eg, dental extractions or cataract removal) where bleeding is easily controlled.

Temporary interruption of VKA anticoagulation, without bridging agents while the INR is subtherapeutic, is recommended in patients with a bileaflet mechanical AVR and no other risk factors for thrombosis who are undergoing invasive or surgical procedures.

Urgent evaluation with multimodality imaging is indicated in patients with suspected mechanical prosthetic valve thrombosis to assess valvular function, leaflet motion, and the presence and extent of thrombus.

Urgent initial treatment with either slow-infusion low-dose fibrinolytic therapy or emergency surgery is recommended for patients with a thrombosed left-sided mechanical prosthetic heart valve presenting with symptoms of valve obstruction.

Repeat valve replacement is indicated for severe symptomatic prosthetic valve stenosis.

Surgery is recommended for operable patients with mechanical heart valves with intractable hemolysis or heart failure (HF) due to severe prosthetic or paraprosthetic regurgitation.

Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is indicated in patients with infective endocarditis (IE) who present with valve dysfunction resulting in symptoms of HF.

Early surgery (during initial hospitalization before completion of a full therapeutic course of antibiotics) is indicated in patients with IE who present with valve dysfunction resulting in symptoms of HF.

Early surgery is indicated in patients with left-sided IE caused by S aureus, fungal, or other highly resistant organisms.

Early surgery is indicated in patients with IE complicated by heart block, annular or aortic abscess, or destructive penetrating lesions.

Infective Endocarditis Prophylaxis

The 2011 AHA guidelines defined the following patient groups as at highest risk for adverse outcomes from IE ^[28] :

• Those with prosthetic cardiac valves or prosthetic material used for cardiac valve repair
• Those with previous IE
• Those with unrepaired cyanotic congenital heart disease (CHD), including palliative shunts and conduits
• Those with CHD and completely repaired congenital heart defect that was repaired with prosthetic material or device, regardless of placement by surgery or catheter intervention, during the first 6 months after the procedure
• Those with repaired CHD who have residual defects at the site or adjacent to the site of a prosthetic patch or prosthetic device
• Recipients of cardiac transplantation who develop cardiac valvulopathy

In a 2015 scientific statement updating its recommendations on IE in childhood, the AHA advocated for a shift in focus from antibiotic prophylaxis to an emphasis on oral hygiene and prevention of oral disease. However, the AHA recommends considering prophylactic antibiotic drugs before certain dental procedures for children in the highest-risk groups (class IIb), such as those discussed above. ^[31]