Pediatric and Congenital Heart - Cedar

What Is Aortic Stenosis?

Aortic stenosis is a condition that occurs when the opening of the aortic valve becomes narrowed. The aortic valve is a heart valve that helps direct blood flow from the heart to the rest of your body. When the valve becomes too narrow it limits blood flow through the heart and to the main artery (blood vessel) that supplies oxygen-rich blood to the body (the aorta). This condition can also affect the pressure within the heart's left upper chamber (the left atrium).

Some people are born with a condition known as a bicuspid aortic valve, which can lead to aortic stenosis. But for many, this condition occurs later in life, often due to aging. An estimated 2% to 9% of people over the age of 75 years experience severe aortic stenosis. For those who experience aortic stenosis later in life, calcium buildup and scarring in the blood vessels can harm the valve. This makes it harder for blood to pass through the valve.

Many people with aortic stenosis may not show any clear symptoms until the condition severely limits blood flow. For some, symptoms may not begin until after the age of 50. When symptoms of aortic stenosis do appear they can include: Chest pain or discomfort Heart palpitations Shortness of breath Fainting Dizziness Difficulty walking or exercising without feeling exhausted Swelling in the ankles or feet Trouble sleeping unless sitting up Lethargy or fatigue Chronic cough that sometimes occurs with coughing up blood Children who are born with a bicuspid aortic valve have a higher risk of developing aortic stenosis. If your child has this condition, they may experience the following symptoms: Extreme fatigue Difficulty gaining weight as expected Challenges with feeding properly Breathing difficulties that emerge within days or weeks after birth Some people are born with a defect in the aortic valve, which can eventually cause aortic stenosis. For people born with a bicuspid aortic valve, your heart valve has two flaps instead of three, which increases the risk of heart valve problems. This is the most common cause for younger people with aortic stenosis. However, if you develop this condition as you get older, several factors can contribute to the onset of symptoms. One common cause is a buildup of calcium in the valve, which can cause hard deposits in your blood vessels and limit blood from flowing effectively. Other conditions, including rare metabolic disorders, autoimmune disorders (like lupus), and end-stage kidney disease, can contribute to calcium buildup in your heart valve. Risk Factors Certain factors can cause a rapid decline in your heart function and increase your risk of developing symptoms of aortic stenosis, such as: Aortic stenosis can worsen your heart health, so getting a timely diagnosis is essential. Diagnosing aortic stenosis usually begins with a physical exam. Your healthcare provider often detects a heart murmur, a clicking sound, or other unusual heart sounds using a stethoscope. Signs such as a weak pulse, low blood pressure, or changes in the pulse's strength in the neck can also be signs of this condition. To confirm a diagnosis of aortic stenosis, your healthcare provider will likely order a specific heart imaging test known as a transthoracic echocardiogram (TTE). A TTE is a non-invasive diagnostic test that uses ultrasound waves to create images of the heart. The purpose of this scan is to measure the size and performance of the heart's four chambers. Additional tests that your provider may order include: Electrocardiogram (ECG): Records the heart's electrical activity Exercise stress testing: Assesses how the heart functions under physical stress Left cardiac catheterization: Examines the heart's chambers and blood flow Heart MRI: Provides detailed images of the heart's structure and function Transesophageal echocardiogram (TEE): Gives a closer look at the heart's valves and chambers from inside the esophagus If symptoms are mild, regular appointments with a healthcare provider may be sufficient for monitoring aortic stenosis. During these visits, your healthcare provider may review your personal medical history, conduct a physical examination, and order an echocardiogram to review your heart health and assess the severity of your condition. However, if you have severe aortic stenosis, your provider will often recommend that you limit strenuous physical activity and avoid competitive sports to prevent added strain to your heart. Healthcare providers also recommend managing high cholesterol and quitting smoking. However, some cases of aortic stenosis require treatment, which may include medications or surgical procedures. Medications Prescription medications can help manage symptoms and reduce the risk of heart failure or irregular heart rhythms, such as atrial fibrillation. Your provider may recommend one or more of the following drug types: Diuretics to reduce fluid buildup Nitrates to improve blood flow Beta-blockers to reduce heart strain Managing high blood pressure is also important, but should be approached with caution. That's because severe aortic stenosis can sometimes cause blood pressure to fall too low. Surgical Procedures For both adults and children who experience symptoms of aortic stenosis, surgery to repair or replace the affected valve is a common treatment option. Sometimes, even if the symptoms are mild, healthcare providers may suggest surgery based on the findings of your diagnostic testing. There are several surgical approaches for replacing a damaged valve, including: Mechanical valve: Involves using a valve made from durable materials Tissue valve: Uses a heart valve made from human or animal donor tissue Ross procedure: Relocates a healthy valve in your heart to replace the damaged valve and uses a new and healthy heart valve to replace the relocated heart valve Balloon valvuloplasty: Inserts a balloon through an artery in the groin and into the heart to inflate and widen the valve opening Transcatheter aortic valve replacement (TAVR): Implants an artificial valve through a catheter Aortic stenosis often results from age-related changes or congenital conditions (conditions that are present at birth) that cannot be completely prevented. While a healthy lifestyle (such as getting exercise, eating a low-cholesterol diet, and abstaining from cigarette smoking) may reduce some risk factors, unfortunately, the development of aortic stenosis itself may not always be avoidable. Several conditions can occur alongside aortic stenosis. These include: Heart failure: Your heart's pumping ability can decrease, which affects how much blood your heart can deliver to other organs in your body Pulmonary hypertension: Also known as high blood pressure in the lungs, this condition can occur due to increased pressure in the heart caused by aortic stenosis Infective endocarditis: An infection of the heart's inner lining or valves, which is generally more common among people with a bicuspid aortic valve Aortic stenosis develops when your heart's aortic valve narrows. This is often due to calcium buildup in the heart's aortic valve or from being born with a condition like a bicuspid aortic valve. While prevention may be a challenge, advancements in medical treatments can help manage this condition effectively. Early detection is key and implementing lifestyle factors like eating a low-cholesterol diet and quitting smoking can help manage your overall heart health.

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PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

PLAIN RADIOGRAPHIC DIAGNOSIS OF CONGENITAL HEART DISEASE

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4I-1. Idiopathic pulmonary artery dilation. (Legend.)

A. The heart is normal size with a convex prominent pulmonary artery and normal size branch pulmonary arteries. In this condition the prominent pulmonary artery resembles that of pulmonary valvular stenosis but hemodynamic studies are normal. It may not be possible to radiographically distinguish idiopathic pulmonary artery dilation, pulmonary valvular stenosis and a small atrial septal defect.

B. Catheter is coiled in dilated pulmonary artery.

Idiopathic pulmonary artery dilation

Clinically idiopathic pulmonary artery dilation is very similar to pulmonary valve stenosis with features of an ejection click, widely split second heart sound, and radiographic features of a dilated main pulmonary artery segment. Echocardiogram in these patients however demonstrates a normally appearing pulmonary valve.

Medtronic Has More Positive Transcatheter Heart Valve Data

The Evolut FX TAVR [Image courtesy of Medtronic]Medtronic (NYSE: MDT) + today announced the release of new data for its transcatheter heart valve therapy technologies.

The medtech giant presented the results at the Society for Cardiovascular Angiography & Interventions (SCAI) 2024 Scientific Sessions.

"The data presented at SCAI 2024 reinforces our commitment to providing solutions for structural heart patients with varying and complex anatomies," said Nina Goodheart, SVP and president, Structural Heart & Aortic at Medtronic. "We are seeing positive outcomes for patient groups in need of minimally invasive solutions – including meaningful insights into the benefits of the Harmony TPV system and additional evidence from the recently released SMART Trial and Optimize PRO addendum study that reinforces the Evolut TAVR system's performance in patients with severe aortic stenosis."

Positive TAVR data from Medtronic

The company shared additional one-year outcomes from the SMART trial evaluating TAVR systems from Edwards and Medtronic. The trial aims to better understand how TAVR works in patients with small aortic annuli (SAA), particularly in women. Women tend to have smaller heart valves compared to men, the company says.

SMART evaluates the Evolut and Sapien systems in individuals with aortic stenosis (AS) with SAA. Medtronic says SMART represents the largest comparative trial of TAVR. It also marks the largest TAVR trial to date to enroll primarily women (87%). SMART randomized and treated 716 patients across more than 80 sites worldwide. The company shared positive data supporting its Evolut system last month.

The latest findings highlighted consistent outcomes between the two co-primary endpoints in patients <80 years or ≥80 years of age.

"This analysis expands upon the data presented at the American College of Cardiology Annual Scientific Session last month, showing us that, no matter their age, treatment with the Evolut TAVR system provides optimal valve performance in small annulus patients," said Dr. Howard C. Herrmann, Perelman School of Medicine at the University of Pennsylvania and lead investigator of the SMART trial. "This study will be beneficial in helping clinicians apply tailored approaches to treatment and address the unique presentation of AS in the small annulus."

More TAVR data

Medtronic also shared results from 151 patients in the Optimize PRO FX addendum study. This study looked at valve performance and procedural outcomes in patients who underwent TAVR with the Evolut FX TAV system, using an "optimized" TAVR care pathway and the cusp overlap technique.

At 30 days, the rate of all-cause mortality or all stroke came in at 2.7% (all-cause mortality, 1.3%). New pacemaker implantation came in at 6.7% and median length of stay in the hospital totaled one day. Upon discharge from the hospital, 91% of patients had none or trace aortic regurgitation (AR). The remainder (9%) had mild regurgitation and no patient had more than mild regurgitation.

Dr. Hemal Gada, president of the UPMC Heart and Vascular Institute and medical director of the Structural Heart Program at UPMC, presented the results.

"Like other recent evidence shows, the FX addendum to the Optimize PRO study reveals favorable clinical and hemodynamic outcomes with Evolut TAVR," said Gada. "The use of standardized optimized care pathways and cusp overlap technique with the Evolut FX TAV system is associated with low PPI rates and no moderate or severe AR at 30 days, a finding consistent with other recently released clinical evidence, including from the SMART Trial, about the Evolut TAVR system."

Data also supports congenital therapies

Medtronic also shared mid-term results from an expanded cohort of Harmony transcatheter pulmonary patients. Harmony TPV treats patients with right ventricular outflow tract (RVOT) anomalies in native or surgically repaired patients with severe pulmonary valve regurgitation. It provides a minimally invasive treatment alternative for such patients.

Results showed an excellent safety profile. The company also reported no new deaths, thrombosis, major stent fracture or arrhythmia between 2-3 years of follow-up.

Investigators observed sustained valve function with no more than mild pulmonary regurgitation (PR) in all but one patient at three years. They saw positive effects on right ventricular (RV) remodeling, with improved RV stroke volume and RV end-diastolic volume through two years.

In total, the study implanted Harmony valves in 86 patients. All remained implanted for more than 24 hours.

"Harmony TPV continues to perform well, with positive impacts on remodeling of the heart, quality of life, and functional outcomes. We were reassured in seeing no new arrhythmia in follow-up. We will continue to track midterm and long-term outcomes associated with valve performance and longevity." said Dr. Brian Morray of the Division of Pediatric Cardiology, Seattle Children's Hospital, who also served as principal investigator.

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