Tricuspid atresia overview: Difference between revisions
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== Classification | == Classification== | ||
Tricuspid atresia is classified according to connection between ventricles with great arteries(aorta, pulmonary) into two subgroups: | Tricuspid atresia is classified according to connection between ventricles with great arteries(aorta, pulmonary) into two subgroups: | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
Tricuspid atresia is an uncommon form of [[congenital heart disease]] that affects about 1.2 per 10000 live births | Tricuspid atresia is an uncommon form of [[congenital heart disease]] that affects about 1.2 per 10000 live births. Twenty percent of patients with this condition will also have other heart problems. | ||
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* severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent ductus arteriosus .After physiologic PDA closure , the cyanosis will be aggravated. | * severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent ductus arteriosus .After physiologic PDA closure , the cyanosis will be aggravated. | ||
* In patients with normal pulmonary blood flow, complications of heart failure occur. | * In patients with normal pulmonary blood flow, complications of heart failure occur. | ||
* Prognosis is generally poor with out surgery and 90% of patients will die before 10 year old | * Prognosis is generally poor with out surgery and 90% of patients will die before 10 year old. The 15 year survival of patients with fontan procedure is approximately %92 according to Merry et al. | ||
== Diagnosis == | == Diagnosis == | ||
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:* Rapid breathing <br /> | :* Rapid breathing <br /> | ||
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: Symptoms of longstanding cyanosis and | : Symptoms of longstanding cyanosis and hyperviscosity syndrome as a result of secondary erythrocytosis in older children include the following: | ||
:* Headache | :* Headache | ||
:* Alter mentation | :* Alter mentation | ||
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* Effective measures for the primary prevention of tricuspid atresia include fetal echocardiography and sonography in 18th weeks of pregnancy for prenatal screening. | * Effective measures for the primary prevention of tricuspid atresia include fetal echocardiography and sonography in 18th weeks of pregnancy for prenatal screening. | ||
* Once diagnosed and surgically treated ,post operated patients with fontan palliative shunt should be evaluated by cardiac MRI. follow up may be about the complications of fontan shunt including | * Once diagnosed and surgically treated ,post operated patients with fontan palliative shunt should be evaluated by cardiac MRI. follow up may be about the complications of fontan shunt including: | ||
Exercise intolerance as the result of ventricular failure, | Exercise intolerance as the result of ventricular failure, | ||
Revision as of 05:20, 29 June 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor-In-Chief:; Sara Zand,MD. Keri Shafer, M.D. [2] Priyamvada Singh, MBBS [3]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [4]
Overview
Tricuspid atresia is the third most common cyanotic congenital heart disease whether the non oxygenated blood can not flows from right atrium to right ventricle due to nondevelopment or agenesia of tricuspid valve. Right ventricle is small and pulmonary artery in some cases is hypoplastic. ASD or PFO are necessary for passing the blood from right atrium to left system and without them the infants will not survive.Majority of infants with die without palliative surgery.
Historical perspective
Tricuspid atresia was first discovered by friedrich ludwig kreysig in 1817, a German physician who found the obstruction between right atrium and right ventricle in autopsy of cyanotic infants. . The classic term of tricuspid atresia was used firstly by schuberg in 1861.
Pathophysiology
In tricuspid atresia, there is no continuity between the right atrium and right ventricle.Inferior vena cava and superior vena cava collect venous non oxygenated blood into right atrium. Through ASD blood reaches into left atrium and finally flows off left ventricle and via aorta artery is transferred the rest of body. This blood is the mixture of saturated and unsaturated O2. If there is a VSD, this mixed blood in left ventricle come into right ventricle via VSD , then via pulmonary artery flows out pulmonary bed and becomes oxygenated ,then returns back into left atrium. In deminished pulmonary blood flow whether the flow is dependent on PDA, the mixed blood in aorta flows from this passage into pulmonary artery and pulmonary bed.In the presence of normal positioning of great arteries cyanosis is more prominent and is affected by the size of VSD .TGA and subaortic stenosis are others associated anomalies.
Classification
Tricuspid atresia is classified according to connection between ventricles with great arteries(aorta, pulmonary) into two subgroups:
- · Normal connection between ventricles and aorta and pulmonary artery . this type is much more common and consistence 70%-80% of cases.Most patients are cyanotic.
- · Aorta originated from small right ventricle and pulmonary artery comes from left ventricle. Heart failure and pulmonary hypertension are common and patients are not cyanotic. Flow in aorta is dependent on VSD size . Subaortic stenosis and aortic arch anomalies are common.
Differentiating tricuspid atresia from other Diseases
- Tricuspid atresia must be differentiated from other diseases that cause lung olygemia and cyanosis ,such as:
- TS
- PS
- ASD
- TOF
Epidemiology and Demographics
Tricuspid atresia is an uncommon form of congenital heart disease that affects about 1.2 per 10000 live births. Twenty percent of patients with this condition will also have other heart problems.
Age
- Tricuspid atresia is more commonly observed among infant less than one year old.[1]
Gender
- Tricuspid atresia affects male and female equally.
Race
- There is no racial predilection for tricuspid atresia
Risk Factors
- There is no specific risk factors for occurrence of tricuspid atresia during pregnancy .
- Few cases of tricuspid atresia with VACTERL association and chromosomal abnormalities including triosomy 13,18 and tetrasomy 22 were reported[1]
- Other potential factors include access to prenatal care and the mother's behavior during pregnancy (i.e. alcohol consumption, illicit drug use, etc).
Natural History, Complications and Prognosis
- Early clinical features in infants include cyanosis of lips and tongue, difficulty in breathing, tiring easily during feeding.
- severity of cyanosis in infants with pulmonary stenosis is dependent on the amount of pulmonary blood flow passing through patent ductus arteriosus .After physiologic PDA closure , the cyanosis will be aggravated.
- In patients with normal pulmonary blood flow, complications of heart failure occur.
- Prognosis is generally poor with out surgery and 90% of patients will die before 10 year old. The 15 year survival of patients with fontan procedure is approximately %92 according to Merry et al.
Diagnosis
Diagnostic Criteria
- The diagnosis of tricuspid atresia is made when at least of the following three diagnostic criteria are met in echocardiography:
Absent of color flow doppler in tricuspid valve region
ASD or PFO
Small right ventricle
Left atrium dilation
Left ventricle dilation
Symptoms
- Symptoms of tricuspid atresia in neonates may include the following:
- Central cyanosis in mucous membranes and tongue
- Poor feeding and growth retardation
- Difficulty in breathing
- Rapid heart beat
- Rapid breathing
- Symptoms of longstanding cyanosis and hyperviscosity syndrome as a result of secondary erythrocytosis in older children include the following:
- Headache
- Alter mentation
- Faintness
- Dizziness
- Visual disturbances
- Paresthesia
- Tinnitus
- Myalgia
Physical Examination
- Patients with pulmonary stenosis and closed PAD usually appear cyanotic after birth.
- Physical examination may be remarkable for:
- Normal pulses
- Deminished right ventricle impulse
- Thrill due to VSD or severe PS
- Holosystolic murmure in LSB due to VSD
- Continuous murmur of PDA ,occasionally
- Systolic ejection murmur in left upper sternal border due to PS
- clubbing in older patients and unrepaired disease.
- Patients with high pulmonary blood flow without stenosis in pulmonary artery and with VSD are not cyanotic at birth.
- Physical examination may be remarkable for symptoms and signs of overt heart failure:
- Tachypnea
- poor feeding
- poor growth
Laboratory Findings
In cyanotic older patients laboratory finding may be include:
- Polycythemia due to secondary erythrocytosis and hypoxia
- Elevated prothrombin time and partial thrombopastin time
- Decreased levels of factors 5,7,8 ,9: qualitative and quantitative
- Platelet disorder
- Increased fibrinolysis and paradoxical thrombotic tendency
- Proteinuria
- Hyperuricemia
- Renal failure
- Uric acid nephrolithiasis
Imaging Findings
- Echocardiography is the imaging modality of choice for tricuspid atresia.Findings include ;ASD, VSD, PDA, and aortic arch anomaly, left ventricle is larger than right ventricle and color flow doppler is absent between right atrium and right ventricle.
- On EKG, tricuspid atresia is characterized by left axis deviation, left ventricle hypertrophy, right atrium enlargement and left atrium enlargement in increment amount of of pulmonary blood flow.
- CXR may demonestrate situs solitus, left sided aortic arch ,levocardia, absent main pulmonary artery, heart size is dependent on pulmonary blood flow, occasionally pulmonary oligemia with decrease vascular markings, right aortic arch in %25 of cases.
- Catheterization may measure the gradient between left ventricle and left atrium in subaortic stenosis.
Treatment
Medical Therapy
- The mainstay of therapy for cyanotic neonate with severe PS and small VSD is using prostaglandin E1 (PGE1) for keeping patency of ductus arteriosis.
- The mainstay of therapy for heart failure symptoms is using diuretic for reloading and then starting ACEI.
Surgery
- Surgery is the mainstay of therapy for tricuspid atresia.
- · In first 8 weeks of life if there is severe cyanosis and pulmonary obstruction and normal positioning aorta and pulmonary artery , making a shunt between systemic subclavian artery to pulmonary artery is necessary which is called Blalock -taussig(BT shunt).
- · If pulmonary artery comes from left ventricle and is overflowed, PA banding is useful for lowering the pulmonary blood flow.
- · In older children, bidirection Glenn shunt which is the connection between superior vena cava to pulmonary artery will done for transferring the blood to pulmonary system.
- Fontan procedure is a coduit between inferior vena cava and pulmonary artery and transfers the systemic venous blood to pulmonary circulation in age of 2-3 year old.
- Endocarditis prophylaxy before every procedures is recommended.
Prevention
- Effective measures for the primary prevention of tricuspid atresia include fetal echocardiography and sonography in 18th weeks of pregnancy for prenatal screening.
- Once diagnosed and surgically treated ,post operated patients with fontan palliative shunt should be evaluated by cardiac MRI. follow up may be about the complications of fontan shunt including:
Exercise intolerance as the result of ventricular failure,
Pericardial and pleural effusion,chylothorax and protein losing enteropathy due to lymphatic dysfunction
Pulmonary emboli,blood clot formation in shunt
Liver failure and portal hypertention as a result of increased pressure in shunt.
leakage of anastomosis and pulmonary hypertention.
Right atrium dilation and arrhythmia