Tricuspid atresia is a congenital heart defect that affects the normal development of the heart. It is a condition where the tricuspid valve, located between the right atrium and the right ventricle of the heart, does not form properly. This leads to the absence or severe underdevelopment of the tricuspid valve, preventing proper blood flow between the right atrium and the right ventricle.
In a normal heart, the tricuspid valve opens to allow blood to flow from the right atrium into the right ventricle and then closes to prevent the backflow of blood. This helps ensure that oxygen-poor blood from the body is pumped adequately to the lungs for oxygenation before being circulated to the rest of the body.
In cases of tricuspid atresia, the valve may be completely missing or abnormally small and nonfunctional. As a result, blood cannot flow directly from the right atrium to the right ventricle. This leads to a mixing of oxygen-poor and oxygen-rich blood in the heart, which can result in decreased oxygen supply to the body.
Epidemiology
The incidence of congenital heart disease stands at approximately 81 per 10,000 live births. Among cyanotic congenital heart diseases, tricuspid atresia emerges as the third most prevalent, with an occurrence of approximately 1.2 per 10,000 live births.
This condition affects males and females equally, and its recurrence within families does not pose a significant risk. While a limited number of instances of familial recurrence for tricuspid atresia have been documented, they were thought to follow an autosomal recessive inheritance pattern.
Anatomy
Pathophysiology
The pathophysiology of tricuspid atresia involves the abnormal development of the heart structures, specifically the tricuspid valve and the chambers it separates. In a healthy heart, deoxygenated blood returns to the right atrium from the body. The tricuspid valve opens to allow this deoxygenated blood to flow into the right ventricle. When the proper ventricle contracts, the tricuspid valve closes to prevent the backflow of blood into the right atrium. The blood is pumped from the right ventricle to the lungs for oxygenation.
In cases of tricuspid atresia, the tricuspid valve fails to develop correctly. This results in a lack of a functional valve between the right atrium and the right ventricle. Consequently, blood cannot flow directly from the right atrium to the right ventricle. Without a functional tricuspid valve, two separate circulatory pathways are established: one for oxygenated blood and another for deoxygenated blood. The oxygenated blood returning from the lungs through the pulmonary vein’s mixes with the deoxygenated blood in the right atrium, leading to a lower oxygen saturation level in the overall blood supply.
The left side of the heart, which handles oxygenated blood, typically has stronger contractions and higher pressures than the right, which deals with deoxygenated blood. In tricuspid atresia, the right ventricle is underdeveloped due to its lack of use, and the left ventricle must pump blood to both the lungs and the body, resulting in increased workload and potential strain.
The body initiates adaptive mechanisms to compensate for the lack of a functional tricuspid valve and the underdeveloped right ventricle. One of the most notable adaptations is the development of alternative pathways for blood flow, which can involve other heart defects like ventricular septal defects (VSD) or patent ductus arteriosus (PDA). These adaptations help to ensure that oxygenated and deoxygenated blood continues to mix in some way, providing the body with at least partially oxygenated blood.
Etiology
The exact origins of tricuspid atresia’s development remain partially elucidated, yet it arises from the disturbance of the usual progression of atrioventricular valve formation originating from the endocardial cushion.
In most affected individuals, the tricuspid inlet presents as a concave recess within the right atrium, exhibiting a muscular manifestation. In less common variations, partial cohesion of delaminated leaflets occurs, creating structures resembling membranes, which is reminiscent of the Ebstein type.
Genetics
Prognostic Factors
The prognosis for individuals with tricuspid atresia varies based on several factors, including the severity of the condition, the presence of associated heart defects, the timing of diagnosis, and the effectiveness of medical and surgical interventions. While tricuspid atresia is a complex congenital heart defect, advancements in medical care and surgical techniques have improved the outlook for many patients. The prognosis can be highly individualized.
Some individuals may have mild forms of tricuspid atresia that allow for better oxygenation and less strain on the heart. Others may have more complex cases with additional heart defects, which can complicate treatment and affect outcomes. Many individuals with tricuspid atresia can survive into adulthood with appropriate medical care and surgical interventions. However, long-term outcomes can vary. Some individuals may require ongoing medical management and potential additional surgeries as they age.
Clinical History
In patients with pulmonary obstruction
Individuals with tricuspid atresia and pulmonary obstruction often present with a history of cyanosis. Their medical history may include prenatal ultrasounds showing potential cardiac abnormalities or cyanosis shortly after birth. A family history of congenital heart disease may also be relevant. Individuals may experience rapid breathing, shortness of breath, and labored breathing, especially during physical activity. Insufficient oxygen supply to the body can lead to fatigue, tiredness, and difficulty with feeding, especially in infants.
Over time, the fingertips and toes might develop clubbing, where the nails and fingertips become rounded and enlarged due to chronic low oxygen levels. Infants and children with tricuspid atresia and pulmonary obstruction might have slower growth rates due to the strain on the heart and decreased oxygen supply. Fluid retention can lead to swelling in the legs, ankles, and abdomen. Frequent respiratory infections can occur due to compromised oxygen levels and weakened immune responses.
In patients without pulmonary obstruction
Individuals with a ventricular septal defect but lacking pulmonary stenosis tend to experience substantial blood flow into the pulmonary circulation. These cases may not exhibit cyanosis at birth, potentially leading to oversight. Instead, the condition might come to light when pulmonary vascular resistance decreases, resulting in excessive lung blood flow. This scenario becomes evident through the manifestation of heart failure indications, tachypnea or respiratory difficulties, suboptimal feeding, and growth concerns.
Physical Examination
During a physical examination of tricuspid atresia with pulmonary oligemia, central cyanosis would be evident, while the pulses would remain normal. The right ventricular impulse might be subdued, and a thrill could be observed in cases involving a restrictive ventricular septal defect or severe pulmonary stenosis. If a VSD is present, a holosystolic murmur might be detectable at the left lower sternal border.
The continuous murmur associated with patent ductus arteriosus (PDA) might be heard occasionally. A systolic ejection murmur could also be auscultated at the left upper sternal border, corresponding with pulmonary stenosis. In older patients with untreated TA and chronic cyanosis, clubbing of the fingers and toes might develop. Conversely, the physical examination would reveal signs such as tachypnea, tachycardia, and hepatomegaly in patients with pulmonary plethora.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
Differential Diagnoses
Atrial Septal Defect
Pulmonary Atresia
Pulmonary Stenosis
Tetralogy of Fallot
Tricuspid Stenosis
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
In patients with pulmonary obstruction
The initial phase entails ensuring sufficient pulmonary blood flow for patients facing pulmonary obstruction. This is accomplished by establishing a shunt between the systemic and pulmonary arteries, often realized through a modified Blalock-Taussig shunt, also recognized as a Blalock-Taussig-Thomas shunt. This connection usually involves employing a polytetrafluoroethylene tubular graft to link the right subclavian artery with the right pulmonary artery.
In patients without pulmonary obstruction
The initial intervention might involve inserting a pulmonary artery band for individuals with unobstructed pulmonary blood flow (type Ic). This band can help regulate blood flow to the pulmonary vascular bed, preventing an excessive influx. In a minority of cases, the ventricular septal defect might be sufficiently small to naturally restrict the volume of blood flowing to the lungs. In such instances, these patients possess a balanced circulation, obviating the necessity for pulmonary artery banding.
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Tricuspid atresia is a congenital heart defect that affects the normal development of the heart. It is a condition where the tricuspid valve, located between the right atrium and the right ventricle of the heart, does not form properly. This leads to the absence or severe underdevelopment of the tricuspid valve, preventing proper blood flow between the right atrium and the right ventricle.
In a normal heart, the tricuspid valve opens to allow blood to flow from the right atrium into the right ventricle and then closes to prevent the backflow of blood. This helps ensure that oxygen-poor blood from the body is pumped adequately to the lungs for oxygenation before being circulated to the rest of the body.
In cases of tricuspid atresia, the valve may be completely missing or abnormally small and nonfunctional. As a result, blood cannot flow directly from the right atrium to the right ventricle. This leads to a mixing of oxygen-poor and oxygen-rich blood in the heart, which can result in decreased oxygen supply to the body.
The incidence of congenital heart disease stands at approximately 81 per 10,000 live births. Among cyanotic congenital heart diseases, tricuspid atresia emerges as the third most prevalent, with an occurrence of approximately 1.2 per 10,000 live births.
This condition affects males and females equally, and its recurrence within families does not pose a significant risk. While a limited number of instances of familial recurrence for tricuspid atresia have been documented, they were thought to follow an autosomal recessive inheritance pattern.
The pathophysiology of tricuspid atresia involves the abnormal development of the heart structures, specifically the tricuspid valve and the chambers it separates. In a healthy heart, deoxygenated blood returns to the right atrium from the body. The tricuspid valve opens to allow this deoxygenated blood to flow into the right ventricle. When the proper ventricle contracts, the tricuspid valve closes to prevent the backflow of blood into the right atrium. The blood is pumped from the right ventricle to the lungs for oxygenation.
In cases of tricuspid atresia, the tricuspid valve fails to develop correctly. This results in a lack of a functional valve between the right atrium and the right ventricle. Consequently, blood cannot flow directly from the right atrium to the right ventricle. Without a functional tricuspid valve, two separate circulatory pathways are established: one for oxygenated blood and another for deoxygenated blood. The oxygenated blood returning from the lungs through the pulmonary vein’s mixes with the deoxygenated blood in the right atrium, leading to a lower oxygen saturation level in the overall blood supply.
The left side of the heart, which handles oxygenated blood, typically has stronger contractions and higher pressures than the right, which deals with deoxygenated blood. In tricuspid atresia, the right ventricle is underdeveloped due to its lack of use, and the left ventricle must pump blood to both the lungs and the body, resulting in increased workload and potential strain.
The body initiates adaptive mechanisms to compensate for the lack of a functional tricuspid valve and the underdeveloped right ventricle. One of the most notable adaptations is the development of alternative pathways for blood flow, which can involve other heart defects like ventricular septal defects (VSD) or patent ductus arteriosus (PDA). These adaptations help to ensure that oxygenated and deoxygenated blood continues to mix in some way, providing the body with at least partially oxygenated blood.
The exact origins of tricuspid atresia’s development remain partially elucidated, yet it arises from the disturbance of the usual progression of atrioventricular valve formation originating from the endocardial cushion.
In most affected individuals, the tricuspid inlet presents as a concave recess within the right atrium, exhibiting a muscular manifestation. In less common variations, partial cohesion of delaminated leaflets occurs, creating structures resembling membranes, which is reminiscent of the Ebstein type.
The prognosis for individuals with tricuspid atresia varies based on several factors, including the severity of the condition, the presence of associated heart defects, the timing of diagnosis, and the effectiveness of medical and surgical interventions. While tricuspid atresia is a complex congenital heart defect, advancements in medical care and surgical techniques have improved the outlook for many patients. The prognosis can be highly individualized.
Some individuals may have mild forms of tricuspid atresia that allow for better oxygenation and less strain on the heart. Others may have more complex cases with additional heart defects, which can complicate treatment and affect outcomes. Many individuals with tricuspid atresia can survive into adulthood with appropriate medical care and surgical interventions. However, long-term outcomes can vary. Some individuals may require ongoing medical management and potential additional surgeries as they age.
In patients with pulmonary obstruction
Individuals with tricuspid atresia and pulmonary obstruction often present with a history of cyanosis. Their medical history may include prenatal ultrasounds showing potential cardiac abnormalities or cyanosis shortly after birth. A family history of congenital heart disease may also be relevant. Individuals may experience rapid breathing, shortness of breath, and labored breathing, especially during physical activity. Insufficient oxygen supply to the body can lead to fatigue, tiredness, and difficulty with feeding, especially in infants.
Over time, the fingertips and toes might develop clubbing, where the nails and fingertips become rounded and enlarged due to chronic low oxygen levels. Infants and children with tricuspid atresia and pulmonary obstruction might have slower growth rates due to the strain on the heart and decreased oxygen supply. Fluid retention can lead to swelling in the legs, ankles, and abdomen. Frequent respiratory infections can occur due to compromised oxygen levels and weakened immune responses.
In patients without pulmonary obstruction
Individuals with a ventricular septal defect but lacking pulmonary stenosis tend to experience substantial blood flow into the pulmonary circulation. These cases may not exhibit cyanosis at birth, potentially leading to oversight. Instead, the condition might come to light when pulmonary vascular resistance decreases, resulting in excessive lung blood flow. This scenario becomes evident through the manifestation of heart failure indications, tachypnea or respiratory difficulties, suboptimal feeding, and growth concerns.
During a physical examination of tricuspid atresia with pulmonary oligemia, central cyanosis would be evident, while the pulses would remain normal. The right ventricular impulse might be subdued, and a thrill could be observed in cases involving a restrictive ventricular septal defect or severe pulmonary stenosis. If a VSD is present, a holosystolic murmur might be detectable at the left lower sternal border.
The continuous murmur associated with patent ductus arteriosus (PDA) might be heard occasionally. A systolic ejection murmur could also be auscultated at the left upper sternal border, corresponding with pulmonary stenosis. In older patients with untreated TA and chronic cyanosis, clubbing of the fingers and toes might develop. Conversely, the physical examination would reveal signs such as tachypnea, tachycardia, and hepatomegaly in patients with pulmonary plethora.
Differential Diagnoses
Atrial Septal Defect
Pulmonary Atresia
Pulmonary Stenosis
Tetralogy of Fallot
Tricuspid Stenosis
In patients with pulmonary obstruction
The initial phase entails ensuring sufficient pulmonary blood flow for patients facing pulmonary obstruction. This is accomplished by establishing a shunt between the systemic and pulmonary arteries, often realized through a modified Blalock-Taussig shunt, also recognized as a Blalock-Taussig-Thomas shunt. This connection usually involves employing a polytetrafluoroethylene tubular graft to link the right subclavian artery with the right pulmonary artery.
In patients without pulmonary obstruction
The initial intervention might involve inserting a pulmonary artery band for individuals with unobstructed pulmonary blood flow (type Ic). This band can help regulate blood flow to the pulmonary vascular bed, preventing an excessive influx. In a minority of cases, the ventricular septal defect might be sufficiently small to naturally restrict the volume of blood flowing to the lungs. In such instances, these patients possess a balanced circulation, obviating the necessity for pulmonary artery banding.
medtigo
Tricuspid atresia
Updated :
September 4, 2023
Tricuspid atresia is a congenital heart defect that affects the normal development of the heart. It is a condition where the tricuspid valve, located between the right atrium and the right ventricle of the heart, does not form properly. This leads to the absence or severe underdevelopment of the tricuspid valve, preventing proper blood flow between the right atrium and the right ventricle.
In a normal heart, the tricuspid valve opens to allow blood to flow from the right atrium into the right ventricle and then closes to prevent the backflow of blood. This helps ensure that oxygen-poor blood from the body is pumped adequately to the lungs for oxygenation before being circulated to the rest of the body.
In cases of tricuspid atresia, the valve may be completely missing or abnormally small and nonfunctional. As a result, blood cannot flow directly from the right atrium to the right ventricle. This leads to a mixing of oxygen-poor and oxygen-rich blood in the heart, which can result in decreased oxygen supply to the body.
The incidence of congenital heart disease stands at approximately 81 per 10,000 live births. Among cyanotic congenital heart diseases, tricuspid atresia emerges as the third most prevalent, with an occurrence of approximately 1.2 per 10,000 live births.
This condition affects males and females equally, and its recurrence within families does not pose a significant risk. While a limited number of instances of familial recurrence for tricuspid atresia have been documented, they were thought to follow an autosomal recessive inheritance pattern.
The pathophysiology of tricuspid atresia involves the abnormal development of the heart structures, specifically the tricuspid valve and the chambers it separates. In a healthy heart, deoxygenated blood returns to the right atrium from the body. The tricuspid valve opens to allow this deoxygenated blood to flow into the right ventricle. When the proper ventricle contracts, the tricuspid valve closes to prevent the backflow of blood into the right atrium. The blood is pumped from the right ventricle to the lungs for oxygenation.
In cases of tricuspid atresia, the tricuspid valve fails to develop correctly. This results in a lack of a functional valve between the right atrium and the right ventricle. Consequently, blood cannot flow directly from the right atrium to the right ventricle. Without a functional tricuspid valve, two separate circulatory pathways are established: one for oxygenated blood and another for deoxygenated blood. The oxygenated blood returning from the lungs through the pulmonary vein’s mixes with the deoxygenated blood in the right atrium, leading to a lower oxygen saturation level in the overall blood supply.
The left side of the heart, which handles oxygenated blood, typically has stronger contractions and higher pressures than the right, which deals with deoxygenated blood. In tricuspid atresia, the right ventricle is underdeveloped due to its lack of use, and the left ventricle must pump blood to both the lungs and the body, resulting in increased workload and potential strain.
The body initiates adaptive mechanisms to compensate for the lack of a functional tricuspid valve and the underdeveloped right ventricle. One of the most notable adaptations is the development of alternative pathways for blood flow, which can involve other heart defects like ventricular septal defects (VSD) or patent ductus arteriosus (PDA). These adaptations help to ensure that oxygenated and deoxygenated blood continues to mix in some way, providing the body with at least partially oxygenated blood.
The exact origins of tricuspid atresia’s development remain partially elucidated, yet it arises from the disturbance of the usual progression of atrioventricular valve formation originating from the endocardial cushion.
In most affected individuals, the tricuspid inlet presents as a concave recess within the right atrium, exhibiting a muscular manifestation. In less common variations, partial cohesion of delaminated leaflets occurs, creating structures resembling membranes, which is reminiscent of the Ebstein type.
The prognosis for individuals with tricuspid atresia varies based on several factors, including the severity of the condition, the presence of associated heart defects, the timing of diagnosis, and the effectiveness of medical and surgical interventions. While tricuspid atresia is a complex congenital heart defect, advancements in medical care and surgical techniques have improved the outlook for many patients. The prognosis can be highly individualized.
Some individuals may have mild forms of tricuspid atresia that allow for better oxygenation and less strain on the heart. Others may have more complex cases with additional heart defects, which can complicate treatment and affect outcomes. Many individuals with tricuspid atresia can survive into adulthood with appropriate medical care and surgical interventions. However, long-term outcomes can vary. Some individuals may require ongoing medical management and potential additional surgeries as they age.
In patients with pulmonary obstruction
Individuals with tricuspid atresia and pulmonary obstruction often present with a history of cyanosis. Their medical history may include prenatal ultrasounds showing potential cardiac abnormalities or cyanosis shortly after birth. A family history of congenital heart disease may also be relevant. Individuals may experience rapid breathing, shortness of breath, and labored breathing, especially during physical activity. Insufficient oxygen supply to the body can lead to fatigue, tiredness, and difficulty with feeding, especially in infants.
Over time, the fingertips and toes might develop clubbing, where the nails and fingertips become rounded and enlarged due to chronic low oxygen levels. Infants and children with tricuspid atresia and pulmonary obstruction might have slower growth rates due to the strain on the heart and decreased oxygen supply. Fluid retention can lead to swelling in the legs, ankles, and abdomen. Frequent respiratory infections can occur due to compromised oxygen levels and weakened immune responses.
In patients without pulmonary obstruction
Individuals with a ventricular septal defect but lacking pulmonary stenosis tend to experience substantial blood flow into the pulmonary circulation. These cases may not exhibit cyanosis at birth, potentially leading to oversight. Instead, the condition might come to light when pulmonary vascular resistance decreases, resulting in excessive lung blood flow. This scenario becomes evident through the manifestation of heart failure indications, tachypnea or respiratory difficulties, suboptimal feeding, and growth concerns.
During a physical examination of tricuspid atresia with pulmonary oligemia, central cyanosis would be evident, while the pulses would remain normal. The right ventricular impulse might be subdued, and a thrill could be observed in cases involving a restrictive ventricular septal defect or severe pulmonary stenosis. If a VSD is present, a holosystolic murmur might be detectable at the left lower sternal border.
The continuous murmur associated with patent ductus arteriosus (PDA) might be heard occasionally. A systolic ejection murmur could also be auscultated at the left upper sternal border, corresponding with pulmonary stenosis. In older patients with untreated TA and chronic cyanosis, clubbing of the fingers and toes might develop. Conversely, the physical examination would reveal signs such as tachypnea, tachycardia, and hepatomegaly in patients with pulmonary plethora.
Differential Diagnoses
Atrial Septal Defect
Pulmonary Atresia
Pulmonary Stenosis
Tetralogy of Fallot
Tricuspid Stenosis
In patients with pulmonary obstruction
The initial phase entails ensuring sufficient pulmonary blood flow for patients facing pulmonary obstruction. This is accomplished by establishing a shunt between the systemic and pulmonary arteries, often realized through a modified Blalock-Taussig shunt, also recognized as a Blalock-Taussig-Thomas shunt. This connection usually involves employing a polytetrafluoroethylene tubular graft to link the right subclavian artery with the right pulmonary artery.
In patients without pulmonary obstruction
The initial intervention might involve inserting a pulmonary artery band for individuals with unobstructed pulmonary blood flow (type Ic). This band can help regulate blood flow to the pulmonary vascular bed, preventing an excessive influx. In a minority of cases, the ventricular septal defect might be sufficiently small to naturally restrict the volume of blood flowing to the lungs. In such instances, these patients possess a balanced circulation, obviating the necessity for pulmonary artery banding.
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