Acyanotic congenital heart disease

Congenital heart diseases have varied presentations depending on the age of presentation. Regression of neonatal pulmonary hypertension and the timing of establishment of left to right shunt determines the onset of symptoms. Pre-tricuspid shunts generally remain asymptomatic during the childhood while large post-tricuspid shunts present with heart failure in late neonatal or early infancy period. Admixture lesions have pathophysiology similar to large post tricuspid shunts with additional small right to left shunt causing mild systemic desaturation. Murmurs are prominent in valvular heart diseases. Careful clinical assessment of features of high pulmonary blood flow, presence of absence of systemic desaturation, status of second heart sound and murmur would help to arrive at a reasonable bedside diagnosis. • Zulfikar Ahamed M, Sajan Ahmad Z, Abhilash TG. Approach to infants and children with cyanotic congenital heart disease. Kerala Heart J. 2015;5:30–5. • Tandon R. Bedside approach in the diagnosis of congenital heart diseases. New Delhi: Bi publications Pvt ltd. India; 1999. • Somerville W. Bedside diagnosis of congenital heart disease. Postgrad Med J. 1956;32:62–8. • Rao PS. Diagnosis and management of acyanotic heart disease: part II – left to right shunt lesions. Indian J Pediatr. 2005;72:503–12. • Lee JY. Clinical presentations of critical cardiac defects in the newborn: decision making and initial management. Korean J Pediatr. 2010;53:669–79. • Rao PS. Diagnosis and...

Cyanotic congenital heart disease Cyanotic congenital heart disease (CCHD) is a condition present at birth. CCHD causes low levels of oxygen in the blood. A common symptom is a bluish tint to the skin, called cyanosis. Several birth defects can cause this type of heart disease, including: • issues with the heart valves, which are the flaps in the heart that make sure the blood flows through in the right direction • an interruption in the aorta, which is the largest artery in the body • abnormalities in the large blood vessels leading to or from the heart In many cases, if only one defect is present, there’s no cyanosis. Often more than one defect is present in CCHD. Doctors use imaging tests to confirm the presence of defects that lead to CCHD. These include In many cases, an infant will be born with this disease in association with a genetic factor. An infant is more at risk for CCHD when there’s a family history of congenital heart diseases. Certain genetic syndromes can be accompanied by defects that cause CCHD. These include: • • • • Noonan syndrome In some instances, outside factors can cause this disease. If a pregnant woman is exposed to toxic chemicals or certain drugs, her infant may have a higher risk of developing heart defects. Infections during pregnancy are also a factor. Poorly controlled gestational diabetes can also lead to a higher risk of the infant developing CCHD. Many physical defects in the heart can cause CCHD. Some infants may be born with several ...

Acyanotic congenital heart disease comprises numerous etiologies, which can be divided into those with increased pulmonary vascularity ( • increased pulmonary vascularity • • • • • other less common • • • ruptured • • • normal pulmonary vascularity • small shunts (see above) • • • Note that certain congenital defects may initially cause acyanotic congenital heart disease and later There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorized, it is helpful to have a working understanding of • • subtypes • • • • • normal relationship between chambers and valves • • • • • • • • • stenosis • atrioventricular valves • • • • outflow tract • • • • • • • great vessels • • • venous inflow • • • hypoplasia • • • anomalous valves • • abnormal relationship of chambers and valves • atrioventricular abnormality • when associated with a univentricular heart • • • • great vessel connection abnormality • • transposition of the great arteries • • • • • • • • • • • • • • • • • • • • • • • • • •

Congenital heart defects are classified into two broad categories: acyanotic and cyanotic lesions. The most common acyanotic lesions are ventricular septal defect, atrial septal defect, atrioventricular canal, pulmonary stenosis, patent ductus arteriosus, aortic stenosis and coarctation of the aorta. Congestive heart failure is the primary concern in infants with acyanotic lesions. The most common cyanotic lesions are tetralogy of Fallot and transposition of the great arteries. In infants with cyanotic lesions, hypoxia is more of a problem than congestive heart failure. Suspicion of a congenital heart defect should be raised by the presence of feeding difficulties in association with tachypnea, sweating and subcostal recession, or severe growth impairment. Follow-up of infants with congenital heart disease should follow the schedule of routine care for healthy babies with some modifications, such as administration of influenza and pneumococcal vaccines. More frequent follow-up is required if congestive heart failure is present. Family psychosocial issues should also be addressed. One of the main roles for the family physician is to help the parents put the diagnosis in perspective by clarifying expectations and misconceptions, and answering specific questions. One third of infants born with CHD develop life-threatening symptoms within the first few days of life. The infant mortality rate in these cases is as high as 90 percent. 3 gallop and pulmonary rales. Failure to reco...

Overview What is cyanotic heart disease? In congenital heart disease, one or more abnormal heart structures (defects) are present at birth. There are two types: • Cyanotic congenital heart disease: Cyanotic congenital heart disease (CCHD) involves heart defects that reduce the amount of oxygen delivered to the rest of your body. It’s sometimes called critical congenital heart disease. When a baby is born with CCHD, their skin has a bluish tint, called cyanosis. • Acyanotic congenital heart disease: Acyanotic congenital heart disease involves defects that don’t interfere with the amount of oxygen delivered to the rest of your body. How does cyanotic heart disease affect my body? Your body must have a steady supply of oxygen to function properly. Cyanotic heart disease prevents your body from getting the oxygen it needs. Structural abnormalities in your heart can cause severe complications and even death. How common is cyanotic heart disease? What kinds of defects can happen with CCHD? There are three different types of cyanotic heart disease: Left heart obstructive lesions These congenital heart defects reduce blood flow between your heart and the rest of your body (systemic blood flow). Specific conditions include: • Hypoplastic left heart syndrome: • Interrupted aortic arch: In Right heart obstructive lesions These congenital heart defects reduce blood flow between your heart and lungs (pulmonary flow). Specific conditions include: • Pulmonary atresia: The pulmonary valve...

Patient Presentation An 8-year-old male was referred to the nephrology clinic of a regional children’s hospital for hypertension. The patient had been seen at a mobile free medical clinic and was noted to have an elevated blood pressure when taken several times with measurements in the 140s-150s systolic and 100-110s diastolic. The past medical history showed he had little medical care previously because he was relatively well per his parents, and he was partially vaccinated. The family history was negative for cardiac or renal disease. There was diabetes and possibly a stroke in two paternal family members. The social history revealed a pleasant immigrant family with limited educational and economic resources. The review of systems found no frequent infections, dyspnea, fatigue, lethargy, facial or extremity swelling, cyanosis, or failure to thrive (although the physicians suspected food insecurity). The patient did complain of intermittent headache but the family could not provide additional details. The pertinent physical exam showed a thin male with height and weight in the 10%. Parents were also small individuals. Blood pressure was 158/112 in right upper extremity, 134/98 in left upper extremity, 138/104 in right lower extremity, and 128/96 in left lower extremity. HEENT was normal but it was difficult to see the optic discs. His cardiac examination had no murmurs, bruits or jugulovenous distension. His abdominal examination had no masses or hepatosplenomegaly. There...

Acyanotic congenital heart disease comprises numerous etiologies, which can be divided into those with increased pulmonary vascularity ( • increased pulmonary vascularity • • • • • other less common • • • ruptured • • • normal pulmonary vascularity • small shunts (see above) • • • Note that certain congenital defects may initially cause acyanotic congenital heart disease and later There is more than one way to present the variety of congenital heart diseases. Whichever way they are categorized, it is helpful to have a working understanding of • • subtypes • • • • • normal relationship between chambers and valves • • • • • • • • • stenosis • atrioventricular valves • • • • outflow tract • • • • • • • great vessels • • • venous inflow • • • hypoplasia • • • anomalous valves • • abnormal relationship of chambers and valves • atrioventricular abnormality • when associated with a univentricular heart • • • • great vessel connection abnormality • • transposition of the great arteries • • • • • • • • • • • • • • • • • • • • • • • • • •

Congenital heart defects are classified into two broad categories: acyanotic and cyanotic lesions. The most common acyanotic lesions are ventricular septal defect, atrial septal defect, atrioventricular canal, pulmonary stenosis, patent ductus arteriosus, aortic stenosis and coarctation of the aorta. Congestive heart failure is the primary concern in infants with acyanotic lesions. The most common cyanotic lesions are tetralogy of Fallot and transposition of the great arteries. In infants with cyanotic lesions, hypoxia is more of a problem than congestive heart failure. Suspicion of a congenital heart defect should be raised by the presence of feeding difficulties in association with tachypnea, sweating and subcostal recession, or severe growth impairment. Follow-up of infants with congenital heart disease should follow the schedule of routine care for healthy babies with some modifications, such as administration of influenza and pneumococcal vaccines. More frequent follow-up is required if congestive heart failure is present. Family psychosocial issues should also be addressed. One of the main roles for the family physician is to help the parents put the diagnosis in perspective by clarifying expectations and misconceptions, and answering specific questions. One third of infants born with CHD develop life-threatening symptoms within the first few days of life. The infant mortality rate in these cases is as high as 90 percent. 3 gallop and pulmonary rales. Failure to reco...

Overview What is cyanotic heart disease? In congenital heart disease, one or more abnormal heart structures (defects) are present at birth. There are two types: • Cyanotic congenital heart disease: Cyanotic congenital heart disease (CCHD) involves heart defects that reduce the amount of oxygen delivered to the rest of your body. It’s sometimes called critical congenital heart disease. When a baby is born with CCHD, their skin has a bluish tint, called cyanosis. • Acyanotic congenital heart disease: Acyanotic congenital heart disease involves defects that don’t interfere with the amount of oxygen delivered to the rest of your body. How does cyanotic heart disease affect my body? Your body must have a steady supply of oxygen to function properly. Cyanotic heart disease prevents your body from getting the oxygen it needs. Structural abnormalities in your heart can cause severe complications and even death. How common is cyanotic heart disease? What kinds of defects can happen with CCHD? There are three different types of cyanotic heart disease: Left heart obstructive lesions These congenital heart defects reduce blood flow between your heart and the rest of your body (systemic blood flow). Specific conditions include: • Hypoplastic left heart syndrome: • Interrupted aortic arch: In Right heart obstructive lesions These congenital heart defects reduce blood flow between your heart and lungs (pulmonary flow). Specific conditions include: • Pulmonary atresia: The pulmonary valve...

Cyanotic congenital heart disease Cyanotic congenital heart disease (CCHD) is a condition present at birth. CCHD causes low levels of oxygen in the blood. A common symptom is a bluish tint to the skin, called cyanosis. Several birth defects can cause this type of heart disease, including: • issues with the heart valves, which are the flaps in the heart that make sure the blood flows through in the right direction • an interruption in the aorta, which is the largest artery in the body • abnormalities in the large blood vessels leading to or from the heart In many cases, if only one defect is present, there’s no cyanosis. Often more than one defect is present in CCHD. Doctors use imaging tests to confirm the presence of defects that lead to CCHD. These include In many cases, an infant will be born with this disease in association with a genetic factor. An infant is more at risk for CCHD when there’s a family history of congenital heart diseases. Certain genetic syndromes can be accompanied by defects that cause CCHD. These include: • • • • Noonan syndrome In some instances, outside factors can cause this disease. If a pregnant woman is exposed to toxic chemicals or certain drugs, her infant may have a higher risk of developing heart defects. Infections during pregnancy are also a factor. Poorly controlled gestational diabetes can also lead to a higher risk of the infant developing CCHD. Many physical defects in the heart can cause CCHD. Some infants may be born with several ...