Congenital Heart Defects I Ohio State Medical Center


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What is a congenital heart defect?

A congenital heart defect is a condition that occurs when the heart – or blood vessels near the heart – do not develop normally before birth. Congenital heart defects are one of the most common birth defects, occurring in approximately one in 100 newborns. As a result of dramatic improvements in the surgical and medical management of these conditions, it is estimated that approximately one million adults live in the United States with congenital heart disease (CHD).

Types of congenital heart defects

Interatrial Communication (ASD) – An abnormal opening between the two upper chambers of the heart causes abnormal blood flow through the heart. Some interatrial communications still require surgical repair. Certain interatrial communications are suitable for transcatheter (non-surgical) procedures to close certain types of interatrial communications.

Bicuspid Aortic Valve – Bicuspid aortic valve is a defect in the aortic valve that results in the formation of two flaps instead of the normal three flaps. It occurs in one to two percent of the general population. In many cases, this condition is not a problem. Later in life, a stenotic or regurgitating murmur may occur.

Coarctation of the aorta (CoA) – The aorta is narrowed or narrowed, obstructing blood flow to the lower part of the body and increasing blood pressure above the constriction. Usually there are no symptoms at birth, but symptoms can develop quickly depending on the degree of aortic stenosis. Treatment includes surgical repair in the infant. In unrepaired coarctation of the aorta in an adolescent or young adult, treatment may include catheter procedures.

Congenital Aortic Stenosis (AS) – This congenital heart defect involves the aortic valve (e.g. heart to pump blood to the body. Management depends on age at diagnosis, severity of the obstruction and Symptoms In children, treatment may include balloon valve or surgical valvotomy.

Ebstein’s Anomaly – This tricuspid valve defect involves the valve moving toward the apex of the heart. Symptoms depend on the degree of displacement of the tricuspid valve and the presence of associated defects, such as interatrial communication. Some patients may require repair or replacement of the tricuspid valve.

Hypoplastic Left Heart Syndrome – In this condition, the left side of the heart is underdeveloped. Babies who have this condition often appear normal at birth, but the condition will become noticeable a few days after birth as the persistent ductus arteriosus closes (essentially arterial arrest). Babies will turn ashy, have a decreased pulse in their legs, have difficulty breathing, and be unable to feed. Treatment may include complex surgery or a heart transplant.

Pulmonary valve stenosis – The pulmonary valve does not open properly. Usually, the obstruction can be corrected with a balloon valvuloplasty. Some patients may require open heart surgery.

Subvalval aortic stenosis (AS) – Patients with subvalve aortic stenosis represent the most diverse group of patients. Patients with subvalvular aortic stenosis are born with or develop a muscle or tissue ridge below the aortic valve. This obstructs the blood flow from the left ventricle to the aorta. Symptoms and associated valve disease may be an indication for surgical repair.

Tetralogy of Fallot – This is the most common defect that causes cyanosis (a condition in which blood pumped through the body contains less than normal amounts of oxygen causing a bluish tinge to the skin). The condition is characterized by four components:

  • Interventricular communication
  • Stenosis at or just below the pulmonary valve that partially blocks blood flow from the right side of the heart to the lungs Right muscular ventricle
  • The aorta lying directly above the ventricular communication.

Transposition of the large arteries – This condition involves an abnormal arrangement of the primary arteries. The aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. Full transposition is recognized early in the first day of life, presenting with a “blue baby”. Surgical intervention is necessary.

Tricuspid atresia – This defect results in the absence of the tricuspid valve, so that no blood flows from the right atrium to the right ventricle. This condition is diagnosed in early childhood due to cyanosis (a bluish tinge of the skin). A staged palliative surgical approach is needed.

Ventricular Communication (VSD) – A hole between the two lower chambers of the heart circulates blood from the left ventricle into the right ventricle. It is one of the most common congenital heart defects. It occurs in isolation and with other flaws. The first symptoms depend on the size of the ventricular port and the age of the patient.

Rare faults include:

  • Hypoplastic heart (the right or left side of the heart is incompletely formed)
  • Have only one ventricle
  • The pulmonary artery and the aorta originate from the same ventricle

Causes

In most cases, the cause of a congenital heart defect is not known. There is growing evidence that genetics play a role in certain congenital heart defects.

Why Choose Ohio State for the Treatment of Congenital Heart Defects? – the COACH program sets us apart from the competition

Adults with coronary heart disease require coordinated care at regional or national centers for adults with coronary heart disease throughout their lives. Ohio State University’s Wexner Medical Center specializes in providing lifelong care from birth to adulthood. OSU Wexner Medical Center’s COACH (Columbus Ohio Adult Congenital Heart Disease) program brings together the specialist resources needed to care for adult congenital heart patients and provides training opportunities for cardiologists and other physicians.

The COACH program focuses on:

  • Adults with congenital heart disease (CHD)
  • Pulmonary hypertension
  • Cardiovascular Connective Tissue Disorders
  • Pregnancy in women with heart disease
  • Transition of Adolescents with CHD to Adult Congenital Heart Disease (CHD) Care
  • Training of future ACHD providers

Diagnosis of congenital heart defect

Thanks to technological improvements, congenital heart defects are now detected before a baby is born by fetal ultrasound. Serious heart defects are often detected soon after a baby is born. Other forms of benign congenital heart disease may not be detected until childhood, late adolescence, or adulthood.

Tests that doctors use to diagnose heart defects include:

  • Electrocardiogram (ECG / ECG) – A test that records the electrical activity of the heart.
  • Echocardiogram (also called an echo) – This test uses sound waves to assess the function and structure of the heart muscle and valves.
  • Chest x-ray – An x-ray or image of the heart and lungs, including blood vessels, ribs, and bones in the spine.
  • Cardiac MRI (Cardiac Magnetic Resonance Imaging or CMR) – A sophisticated, non-invasive imaging procedure that uses large magnets and a computer to produce detailed images of the structure and function of the heart as it beats.

Treatments for congenital cardiovascular malformations

Medications

  • Medicines used to treat heart failure include beta blockers, diuretics, and ACE inhibitors.
  • Drugs prescribed to treat high blood pressure include diuretics, calcium channel blockers, and beta blockers.
  • Drugs prescribed to treat arrhythmia include beta blockers, calcium channel blockers, sotalol, and amiodarone.

Surgery and procedures

Some congenital heart defects can be treated with minimally invasive procedures via cardiac catheterization, for example. Defects that can now be treated with catheter-based procedures include atrial septal defects (treated by transcatheter closure) and pulmonary valve stenosis (treated by balloon valvuloplasty).

Innovations in the aftermath of catheterization have led to many developments in catheter interventions. Other congenital heart defects require open heart surgery and sometimes multiple surgeries.

In very rare cases, a baby’s heart defect is so complex that repair is not possible and a heart transplant may be necessary.

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