Structural heart disease and defects

Structural heart disease and defects

Overview

Structural heart disease and heart defects refer to a defect or abnormality in the heart's valves or vessels. Some structural heart diseases are congenital (present at birth) while others can develop with age. These defects can involve the interior walls of the heart, the valves inside the heart, and the arteries and veins that carry blood to the heart or out to the body. Congenital heart disease defects change the normal flow of blood through the heart. Some are undetectable, and their effects aren’t felt until adulthood. One in every 150 adults has some form of congenital heart disease.

The skilled team of specialists at Northwell Health’s Cardiac Services offers the latest diagnostic procedures and the most advanced treatments for patients with structural heart diseases such as the most common adult congenital heart diseases listed below:  

  • Atrial septal defect — a hole in the septum (wall) that separates the two upper (atrial) chambers of the heart
  • Ventricular septal defect — a hole in the septum that separates the two lower (ventricular) chambers of the heart
  • Patent foramen ovales — a structural heart defect that occurs when the small flap-like opening that separates the two upper (atrial) chambers of the heart prior to birth fails to seal after birth.
  • Aortic valve stenosis — a buildup of calcium deposits on the aortic valve that causes narrowing and prevents the valve from opening properly

Risks

According to the National Heart, Lung and Blood Institute, congenital heart defects are the most common type of birth defect, affecting eight out of every 1,000 newborns. Each year, more than 35,000 babies in the United States are born with congenital heart defects. It is unknown why congenital heart defects occur, but they tend to run in families.

Adult structural heart disease can be caused by several factors, including:

  • High blood pressure
  • Atherosclerosis 
  • A previous heart attack
  • Aging
  • Certain medications
  • Other infections and disorders including cardiomyopathy (diseases of the heart muscle) 
  • Rheumatic fever and endocarditis (a bacterial infection)

Symptoms

Congenital heart defects and disease are usually recognized during the first few months after birth. Symptoms include:

  • Bluish skin (cyanosis)
  • Very low blood pressure shortly after birth
  • Difficulty breathing and feeding
  • Poor weight gain

Diagnosis

Heart defects may be diagnosed with tests that include:

  • Electrocardiogram (EKG or ECG) to test the heart’s electrical activity
  • Chest X-ray
  • Echocardiogram (ultrasound film of the heart)
  • Blood tests 
  • Cardiac catheterization and angiography, where an X-ray records blood pressure and blood flow in a cardiac chamber or major blood vessel 
  • Magnetic resonance imaging (MRI)
  • A computed tomography (CT) scan, which uses X-rays to create detailed pictures of the heart and its blood vessels
  • Holter monitor that records your heart rate, worn for 24 hours or up to two weeks
  • Stress testing, in which your heart is tested during “stress” — that is, during exercise or drug stimulation

Heart defects in children may be discovered because of the symptoms described above or during a baby’s checkup, learn more here.

Treatment

The most advanced treatments for heart defects, including atrial and ventricular septal defect treatment, patent foramen ovale treatment, and severe aortic stenosis treatment are provided by the region's top structural heart disease specialists and cardiovascular surgeons in state-of-the-art catheterization labs and operating rooms at Northwell Health’s Cardiac Services.

Highlights of our treatment program include:

  • Transcatheter aortic valve replacement (TAVR)
    • High-risk patients with severe aortic stenosis can now be considered for TAVR, a less-invasive procedure. Patients with severe aortic stenosis are generally older, with multiple medical conditions that can put them at very high risk if traditional surgical procedures are performed. Previously, these patients had few or no treatment options, because traditional cardiac surgery involves an incision along the sternum (sternotomy) to access the heart. The TAVR procedure replaces a diseased aortic heart valve percutaneously (meaning through the skin) without a sternotomy and without surgical removal of the diseased valve.
       
  • Percutaneous paravalvular leak closure 
    • Sometimes after surgical implantation of a mitral or aortic valve, a leak may occur around the artificial valve. This is a rare occurrence, but can lead to serious issues such as heart failure, anemia (low production of red blood cells), and endocarditis (an infection of the inside lining of your heart and heart valves). Our specialists are experts at performing percutaneous paravalvular leak closures. During this procedure, your doctor uses catheters to access the area of leakage around the heart valve, and using different devices can close the leak.
    • A structural defect in the mitral valve, including the leaflets (flaps), can cause degenerative mitral regurgitation, a progressive disease. It can be related to age, a birth defect, or underlying heart disease. The MitraClip Delivery System® is composed of implant catheters and the MitraClip device, used to reduce mitral valve regurgitation in patients who are not candidates for mitral valve surgery, such as those who have severe degenerative mitral regurgitation. 
    • MitraClip® therapy is a minimally invasive transcatheter mitral valve repair (TMVR) procedure. The permanent device is inserted via catheter through the femoral vein, and guided into the heart. It is then positioned by grasping both leaflets of the valve, and the catheter is removed. This procedure allows the valve to close more completely, which reduces leakiness. The device is made of metal alloys covered with polyester, to allow tissue to grow. (These materials often are used in cardiovascular implants.)
    • MitraClip is not recommended for patients who have endocarditis of the mitral valve, rheumatic mitral valve disease, or a possible intracardiac, inferior vena cava (IVC) or femoral venous thrombus.
    • The use of MitraClip reduces hospitalization time, improves quality of life, and relieves symptoms in patients who have no other therapeutic option. Some patients may be treated with an anticoagulant for a short time afterward.
       
  • Left ventricular pseudoaneurysm closure
    • Left ventricular pseudoaneurysm is a rare but serious complication that may occur after a heart attack or cardiac surgery. It involves a rupture of the heart wall that is contained within the heart sac called the pericardium. Conventional treatment for this problem has been surgery. However, percutaneous closure using catheters and closure devices can be an alternative for patients who are high-risk for surgery. Our specialists have the largest reported experience of treating this problem using catheter-based techniques.
       
  • Minimally invasive direct coronary artery bypass (MIDCAB)
    • By avoidance of sternotomy and conventional cardiopulmonary bypass operations, MIDCAB is a very good option for single-vessel LAD (left anterior descending) coronary artery disease. MIDCAB doesn't require stopping the heart, using the heart-lung machine for artificial support. or cutting open the chest. It can be performed through a 3"– 5" incision between the ribs, or through several smaller incisions, to bypass one or two coronary arteries. The advantages to a MIDCAB procedure are less bleeding, less pain after surgery, a shorter hospital stay, faster recovery, and minimal scarring. MIDCAB is especially appropriate for patients who are unable to undergo traditional open heart surgery because they are too ill, their hearts are weak, or they cannot tolerate taking blood products.

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