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Other Birth Defects of the Heart

By

Lee B. Beerman

, MD, Children's Hospital of Pittsburgh of the University of Pittsburgh School of Medicine

Reviewed/Revised Apr 2023
VIEW PROFESSIONAL VERSION

Most of these defects are rare. Some of these include

  • Pulmonary atresia with an intact ventricular septum

  • Single ventricle defects

  • Tricuspid atresia

Symptoms of these defects vary, depending on the specific defect and its severity. All of these defects may cause a bluish color to the skin (cyanosis) or an abnormal heart rhythm Overview of Abnormal Heart Rhythms Abnormal heart rhythms (arrhythmias) are sequences of heartbeats that are irregular, too fast, too slow, or conducted via an abnormal electrical pathway through the heart. Heart disorders are... read more Overview of Abnormal Heart Rhythms . Doctors often suspect a heart defect when they hear a heart murmur during an examination with a stethoscope, but usually they do echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography is a type of medical imaging that uses high-frequency (ultrasound) waves to produce a moving image of internal organs and other tissues. Echocardiography is ultrasonography of... read more Echocardiography and Other Ultrasound Procedures (ultrasonography of the heart) to confirm the existence of a defect.

Surgical repair of the defect is usually needed.

Pulmonary Atresia With Intact Septum

In pulmonary atresia, the pulmonic (pulmonary) valve does not form properly, so blood does not flow into the lungs and pick up oxygen. Therefore, oxygen-poor blood is circulated to the body. Pulmonary atresia frequently occurs together with other heart disorders and with abnormalities of the coronary arteries. The symptoms and surgical treatment options depend on what type of other abnormalities are also present.

Single Ventricle Defects

Single ventricle defects include any complex heart defect in which there is only one functional ventricle. Symptoms depend on the exact abnormality and whether pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more is also present. Most infants have a bluish color of the skin (cyanosis) because their blood is not receiving enough oxygen. Infants with severe pulmonic stenosis may be very blue and require a surgical procedure fairly quickly. Infants without pulmonic stenosis have excessive blood flow to their lungs and develop symptoms of heart failure (for example, rapid breathing, poor feeding, easy tiring, excessive sweating). Surgery is required to correct the heart abnormalities. Usually, multiple surgeries are required over several years.

Tricuspid Atresia

Infants with tricuspid atresia are born without a tricuspid valve and have an underdeveloped right ventricle. Other heart defects may be present, including atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more Atrial and Ventricular Septal Defects , ventricular septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more Atrial and Ventricular Septal Defects , patent ductus arteriosus Patent Ductus Arteriosus In patent ductus arteriosus (PDA), the blood vessel connecting the pulmonary artery and the aorta (ductus arteriosus) fails to close as it usually does shortly after birth. Patent ductus arteriosus... read more Patent Ductus Arteriosus , pulmonic valve stenosis Pulmonary Valve Stenosis in Children Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs. The heart valve... read more , and transposition of the great arteries Transposition of the Great Arteries Transposition of the great arteries is a reversal of the normal connections of the aorta and the pulmonary artery with the heart. The aorta and pulmonary artery are reversed, which causes oxygen-poor... read more Transposition of the Great Arteries . Infants have a bluish color of the skin (cyanosis). Other symptoms depend on what other defects are also present. Diagnosis is by echocardiography. Treatment is surgical repair.

More Information

The following English-language resources may be useful. Please note that THE MANUAL is not responsible for the content of these resources.

NOTE: This is the Consumer Version. DOCTORS: VIEW PROFESSIONAL VERSION
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