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Conditions & Treatments

Adult Congenital Heart Conditions

Congenital heart disease stems from an abnormality present at birth. While some conditions require immediate surgery on a newborn, it’s possible that some patients don’t know about their conditions until well into adulthood. The cardiovascular team at Einstein Healthcare Network provides best-in-class diagnosis and care for patients with congenital heart disease, and will work with you and your family to provide both traditional and innovative, minimally invasive options – not only to manage your condition, but also to thrive.

While the range of congenital heart defects varies, some common symptoms include:

  • Arrhythmia (abnormal heartbeat)
  • Cyanosis (bluish tint to the skin)
  • Dizziness/fainting
  • Shortness of breath
  • Swelling of body tissue or organs
  • Tiring quickly

Conditions

An atrial septal defect is a hole in the wall between the upper left and right chambers (atria) of the heart. The hole causes a sound described as a heart murmur. If the hole is large enough, oxygen-rich blood from the left atrium may flow back into the right atrium instead of the ventricles. Smaller atrial septal holes generally resolve on their own. Larger holes often create great strain on the heart, which can lead to other complications.

The aortic valve is a one-way passage between the heart and the aorta (the largest artery in the body). Normal valves contain three leaflets, or flaps, that open and close, preventing the flow of blood from the aorta back into the heart. In bicuspid aortic valve disease, the valve only has two flaps. The deformity causes strain on the valve, causing deterioration that begins early in life.

Total or partial anomalous pulmonary venous return (TAPVR or PAPVR) is a rare heart defect. In a normal heart, four pulmonary veins return oxygenated blood from the lungs to the left upper chamber (atrium) of the heart. Anomalous pulmonary venous return occurs when all or one of the four blood vessels do not connect to the left atrium. Instead, one or all the pulmonary veins drain into the right atrium. TAPVR usually presents in newborns and must be immediately corrected. PAPVR can go undetected into adulthood.

A ventricular septal defect is a hole in the wall between the lower left and right chambers (ventricles) of the heart. Frequently detected at birth, these holes cause a specific type of heart murmur. If the hole is small, it will generally resolve on its own as the child grows. However, larger septal defects can be dangerous. As oxygen-rich blood from the left ventricle flows back to the right side, the heart must pump harder to circulate blood. The added strain on the heart can cause complications.

Genetic mutations that cause your heart muscle to become abnormally thick can cause a condition called hypertrophic obstructive cardiomyopathy, making it difficult for your heart to pump blood. Many people with this condition do not have symptoms, and can lead normal, healthy lives. For patients with symptoms, your doctor may recommend a variety of treatment options, including medication, an implantable device that will shock your heart back into a normal rhythm if it starts beating abnormally, or a catheter-based procedure or open-heart surgery to remove excess heart tissue.  

Procedures

Our skilled team of heart surgeons are experts in procedures to treat adult congenital heart conditions, including the following:

This type of repair uses a catheter to assist surgery. A cardiovascular surgeon will cut a small incision in the right side of the chest. Maneuvering surgical tools to the sac that surrounds the heart, the surgeon will collect a small piece of pericardial tissue. That material is used to patch the hole between the left and right atria.

Open-heart surgery is usually required to repair or replace a bicuspid aortic valve. A surgeon must divide the sternum to access the heart. If the valve is otherwise functional and not damaged or narrowed, the surgeon may be able to reshape the leaflets in the valve to restore function. In the event of an aneurysm, both the valve and the damaged part of the aorta will be replaced with an aortic aneurysm resection graft.

The goal of the surgical repair of anomalous pulmonary venous return is to create an unobstructed venous flow to the left side heart chambers. For this open-heart procedure, your surgeon will reconnect all pulmonary veins to the left atrium, and then tie off the former entry points. Patients who undergo this surgery may require a long period of postoperative care while the heart and lungs recover.

Ventricular septal defects can be repaired by either traditional open-heart or minimally invasive surgical procedures. Minimally invasive surgery uses endovascular catheters inserted via blood vessels in the groin. The cardiovascular surgeon pushes the catheter to the heart, collects a small piece of tissue from the pericardium, or sac surrounding the heart, and uses that tissue to patch the hole between the right and left ventricles. Your Einstein surgical team will help you decide if a minimally invasive surgery is possible for you.

 

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Adult Congenital Heart Conditions Specialists

Louis Samuels

Chief of Cardiac Surgery for Einstein Healthcare Network

Alexandra Tuluca, MD

Associate Chief of Cardiac Surgery for Einstein Healthcare Network

Raymond L. Singer, MD

Chief of Cardiac Surgery at Einstein Medical Center Montgomery

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At Einstein, we have clinical expertise in all core domains of cardiology and are actively invested in resident education.

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