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Tricuspid Valve Atresia (TVA)

Tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) (TVA) is a congenital Congenital Chorioretinitis heart defect (CHD) causing an absent or rudimentary tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy (TV) associated with an interatrial or ventricular septal defect Ventricular Septal Defect Tetralogy of Fallot. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination at birth due to blood mixing between the right atrium (RA) and right ventricle (RV). Diagnosis can be made in utero or confirmed after birth with an echocardiogram Echocardiogram Transposition of the Great Vessels. Definitive management involves a staged surgical procedure beginning in the neonatal period.

Last updated: 18 Feb, 2021

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Overview

Definition

Tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) (TVA) is a congenital Congenital Chorioretinitis heart defect (CHD) featuring the complete absence or agenesis Agenesis Teratogenic Birth Defects of the tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy (TV) resulting in a lack of communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence between the right atrium (RA) and the right ventricle (RV). Tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) (TVA) is considered a cyanotic congenital Congenital Chorioretinitis heart disease (CCHD) as most patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination at birth.

Anatomical variation

Epidemiology

  • 3rd most common CCHD
  • Prevalence Prevalence The total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time. Measures of Disease Frequency: 1 per 10,000 live births
  • M:F ratio 1:1
  • No familial forms

Etiology

  • Pathogenesis unknown but postulated to be:
    • Abnormal development of valves from endocardial cushions
    • Abnormal delamination of ventricular myocardium Myocardium The muscle tissue of the heart. It is composed of striated, involuntary muscle cells connected to form the contractile pump to generate blood flow. Heart: Anatomy
  • Genetic predisposition is noted and seen with syndromes such as:
    • Trisomy 21 Trisomy 21 Down syndrome, or trisomy 21, is the most common chromosomal aberration and the most frequent genetic cause of developmental delay. Both boys and girls are affected and have characteristic craniofacial and musculoskeletal features, as well as multiple medical anomalies involving the cardiac, gastrointestinal, ocular, and auditory systems. Down syndrome (Trisomy 21) ( Down syndrome Down syndrome Down syndrome, or trisomy 21, is the most common chromosomal aberration and the most frequent genetic cause of developmental delay. Both boys and girls are affected and have characteristic craniofacial and musculoskeletal features, as well as multiple medical anomalies involving the cardiac, gastrointestinal, ocular, and auditory systems. Down syndrome (Trisomy 21))
    • Trisomy 18 Trisomy 18 Edwards syndrome, or trisomy 18, is a genetic syndrome caused by the presence of an extra chromosome 18. The extra chromosome is either from 3 full copies of chromosome 18 or an additional segment of chromosome 18. As the 2nd most common trisomy, Edwards syndrome is seen in 1 out of every 5,500 live births. Edwards Syndrome (Trisomy 18) ( Edwards syndrome Edwards syndrome Edwards syndrome, or trisomy 18, is a genetic syndrome caused by the presence of an extra chromosome 18. The extra chromosome is either from 3 full copies of chromosome 18 or an additional segment of chromosome 18. As the 2nd most common trisomy, Edwards syndrome is seen in 1 out of every 5,500 live births. Edwards Syndrome (Trisomy 18))
    • Vertebral, anal atresia Anal Atresia Esophageal Atresia and Tracheoesophageal Fistula, cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR), trachea Trachea The trachea is a tubular structure that forms part of the lower respiratory tract. The trachea is continuous superiorly with the larynx and inferiorly becomes the bronchial tree within the lungs. The trachea consists of a support frame of semicircular, or C-shaped, rings made out of hyaline cartilage and reinforced by collagenous connective tissue. Trachea: Anatomy, esophageal, renal, and limb defect (VACTERL) association
Tricuspid atresia

Tricuspid atresia Atresia Hypoplastic Left Heart Syndrome (HLHS):
Note the thick muscular floor base of the RA in place of the TV in the cadaver sample. The image indicates no communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence between the RA and the RV.

Image: “The new concept of univentricular heart” by Frescura C, Thiene G. License: CC BY 3.0, edited by Lecturio.

Pathophysiology and Clinical Presentation

Pathophysiology

  • No communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence between RA and RV
  • Drainage of RA is impeded → RA dilation (and hypoplastic RV)
  • Interatrial communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence ( PFO PFO A patent foramen ovale (PFO) is an abnormal communication between the atria that persists after birth. The condition results from incomplete closure of the foramen ovale. Small, isolated, and asymptomatic pfos are a common incidental finding on echocardiography and require no treatment. Patent Foramen Ovale) allows flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure to left atrium (LA).
  • Allows venous return to mix with oxygenated blood in LA → left ventricle (LV) → systemic circulation Systemic circulation Circulation is the movement of blood throughout the body through one continuous circuit of blood vessels. Different organs have unique functions and, therefore, have different requirements, circulatory patterns, and regulatory mechanisms. Systemic and Special Circulations causing cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination
  • Blood flows into RV; pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment depends on presence of VSD:
    • If VSD is present and large, left-right (L-R) shunt allows mixed blood to enter RV from LV and into pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment.
    • Absent VSD or very small VSD Small VSD Ventricular Septal Defect (VSD) patent ductus arteriosus Patent ductus arteriosus The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA) ( PDA PDA The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA)) allows pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment
  • LV acts as single chamber pumping blood into both systemic and pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment.
  • If patient has concomitant pulmonary atresia Atresia Hypoplastic Left Heart Syndrome (HLHS), patient has no pulmonary blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure and presents in shock Shock Shock is a life-threatening condition associated with impaired circulation that results in tissue hypoxia. The different types of shock are based on the underlying cause: distributive (↑ cardiac output (CO), ↓ systemic vascular resistance (SVR)), cardiogenic (↓ CO, ↑ SVR), hypovolemic (↓ CO, ↑ SVR), obstructive (↓ CO), and mixed. Types of Shock once PDA PDA The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA) closes.
Tricuspid atresia cardial blood flow

Cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure in tricuspid atresia Atresia Hypoplastic Left Heart Syndrome (HLHS):
Image of blood flow Flow Blood flows through the heart, arteries, capillaries, and veins in a closed, continuous circuit. Flow is the movement of volume per unit of time. Flow is affected by the pressure gradient and the resistance fluid encounters between 2 points. Vascular resistance is the opposition to flow, which is caused primarily by blood friction against vessel walls. Vascular Resistance, Flow, and Mean Arterial Pressure through a normal heart vs. a heart with TVA. In the heart with TVA, deoxygenated blood enters the RA and flows into the LA by way of interatrial communication Communication The exchange or transmission of ideas, attitudes, or beliefs between individuals or groups. Decision-making Capacity and Legal Competence. The LV acts like a single chamber pumping blood into the systemic and pulmonary circulation Circulation The movement of the blood as it is pumped through the cardiovascular system. ABCDE Assessment via a VSD.

Image by Lecturio.

Clinical presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor

  • 50% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present within 1st day of life.
  • 80% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present within 1st month of life.
  • Presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor includes:
    • Cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination
    • Labored breathing
    • Hypoxic spells

Related videos

Diagnosis

Physical examination

  • Central cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination
  • Pulse oximetry: decreased oxygen saturation Oxygen Saturation Basic Procedures (Sp02)
  • Signs of respiratory distress: 
  • Auscultation:
    • Single second heart sound ( S2 S2 Heart Sounds)
    • Holosystolic murmur of VSD (left lower sternal border)
  • Additional signs based on associated cardiac Cardiac Total Anomalous Pulmonary Venous Return (TAPVR) lesions:
    • Pulmonary atresia Atresia Hypoplastic Left Heart Syndrome (HLHS) with PDA PDA The ductus arteriosus (DA) allows blood to bypass pulmonary circulation. After birth, the DA remains open for up to 72 hours and then constricts and involutes, becoming the ligamentum arteriosum. Failure of this process to occur results in patent ductus arteriosus (PDA), a condition that causes up to 10% of congenital heart defects. Patent Ductus Arteriosus (PDA) → continuous murmur
    • Restrictive PFO PFO A patent foramen ovale (PFO) is an abnormal communication between the atria that persists after birth. The condition results from incomplete closure of the foramen ovale. Small, isolated, and asymptomatic pfos are a common incidental finding on echocardiography and require no treatment. Patent Foramen Ovale or ASD ASD Autism spectrum disorder (ASD) is a neurodevelopmental disorder marked by poor social skills, restricted interests/social interactions, and repetitive/stereotyped behaviors. The condition is termed a “spectrum” because of the wide variability in the severity of symptoms exhibited. Autism Spectrum Disorder → hepatomegaly
    • Coarctation of aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy → diminished pulses in lower extremities

Imaging

  • Chest X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests: cardiomegaly Cardiomegaly Enlargement of the heart, usually indicated by a cardiothoracic ratio above 0. 50. Heart enlargement may involve the right, the left, or both heart ventricles or heart atria. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (heart failure) or several forms of cardiomyopathies. Ebstein’s Anomaly in presence of large VSD Large VSD Ventricular Septal Defect (VSD)
  • Electrocardiogram Electrocardiogram An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG) ( ECG ECG An electrocardiogram (ECG) is a graphic representation of the electrical activity of the heart plotted against time. Adhesive electrodes are affixed to the skin surface allowing measurement of cardiac impulses from many angles. The ECG provides 3-dimensional information about the conduction system of the heart, the myocardium, and other cardiac structures. Electrocardiogram (ECG)):
    • Tall p waves
    • Left axis Axis The second cervical vertebra. Vertebral Column: Anatomy deviation 
    • LV hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation
  • Echocardiography

Management

Management

Management is medical stabilization followed by a staged surgical repair. 

Prognosis Prognosis A prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations. Non-Hodgkin Lymphomas

  • Without intervention, 75% of patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship die in early childhood.
  • 80% survival rate (>10 years) once all 3 stages completed

Differential Diagnosis

Additional CCHD include:

  • Tetralogy of Fallot Tetralogy of Fallot Tetralogy of Fallot is the most common cyanotic congenital heart disease. The disease is the confluence of 4 pathologic cardiac features: overriding aorta, ventricular septal defect, right ventricular outflow obstruction, and right ventricular hypertrophy. Tetralogy of Fallot (TOF): congenital Congenital Chorioretinitis heart disease (CHD) characterized by the occurrence of 4 key cardinal features: overriding aorta Overriding Aorta Tetralogy of Fallot, VSD, right ventricular outflow tract obstruction (RVOTO), and right ventricular hypertrophy Hypertrophy General increase in bulk of a part or organ due to cell enlargement and accumulation of fluids and secretions, not due to tumor formation, nor to an increase in the number of cells (hyperplasia). Cellular Adaptation (RVH). Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship present with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination and a history of tet spells Tet Spells Tetralogy of Fallot. Diagnosis confirmed by echocardiogram Echocardiogram Transposition of the Great Vessels and management includes surgery.
  • Transposition of the great vessels Transposition of the Great Vessels Transposition of the great vessels (TGV) is a cyanotic congenital heart disease characterized by “switching” of the great arteries. There are 2 presentations: the dextro (D)- and levo (L)-looped forms. The L-looped form is rare and congenitally corrected, as the ventricles are also switched. Transposition of the Great Vessels (TGV): congenital Congenital Chorioretinitis heart defect (CHD) characterized by a switching of great vessels; the aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy originates from the RV and the PA originates from the LV. The patient presents with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination, tachypnea Tachypnea Increased respiratory rate. Pulmonary Examination, heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR), and hypoxemia Hypoxemia Neonatal Respiratory Distress Syndrome unresponsive to supplemental oxygen Supplemental Oxygen Respiratory Failure. Diagnosis is confirmed by echocardiogram Echocardiogram Transposition of the Great Vessels and management includes surgery.
  • Truncus arteriosus Truncus arteriosus Truncus arteriosus (TA) is a congenital heart defect characterized by the persistence of a common cardiac arterial trunk tract that fails to divide into the pulmonary artery and aorta during embryonic development. Truncus arteriosus is a rare congenital malformation with a high mortality rate within the 1st 5 weeks of life if not managed promptly. Truncus Arteriosus ( TA TA Thyrotoxicosis and Hyperthyroidism): The aorta Aorta The main trunk of the systemic arteries. Mediastinum and Great Vessels: Anatomy and pulmonary artery Pulmonary artery The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. Lungs: Anatomy emerge from a common trunk overriding the VSD. The patient is symptomatic from the 1st day of life. Symptoms include cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination, respiratory distress, heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR), poor feeding, and excessive sweating. Diagnosis is confirmed by echocardiogram Echocardiogram Transposition of the Great Vessels and management includes surgery.
  • Total anomalous pulmonary venous return (TAPVR): Characterized by wide-split S2 S2 Heart Sounds, TAPVR is a rare congenital Congenital Chorioretinitis cardiopathy in which pulmonary veins Veins Veins are tubular collections of cells, which transport deoxygenated blood and waste from the capillary beds back to the heart. Veins are classified into 3 types: small veins/venules, medium veins, and large veins. Each type contains 3 primary layers: tunica intima, tunica media, and tunica adventitia. Veins: Histology drain to sites other than the LA. The patient presents with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination from birth, heart failure Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (ventricular dysfunction), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as myocardial infarction. Total Anomalous Pulmonary Venous Return (TAPVR), and respiratory distress. Diagnosis is confirmed by echocardiogram Echocardiogram Transposition of the Great Vessels and management includes surgery.
  • Ebstein’s anomaly: congenital Congenital Chorioretinitis heart defect (CHD) with downward displacement Displacement The process by which an emotional or behavioral response that is appropriate for one situation appears in another situation for which it is inappropriate. Defense Mechanisms of tricuspid valve Tricuspid valve The valve consisting of three cusps situated between the right atrium and right ventricle of the heart. Heart: Anatomy leaflets causing RVOTO. The patient presents with cyanosis Cyanosis A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule. Pulmonary Examination, arrhythmias, and failure to thrive Failure to Thrive Failure to thrive (FTT), or faltering growth, describes suboptimal weight gain and growth in children. The majority of cases are due to inadequate caloric intake; however, genetic, infectious, and oncological etiologies are also common. Failure to Thrive. Diagnosis is confirmed by echocardiogram Echocardiogram Transposition of the Great Vessels and management includes surgery.

References

  1. Sowmya B & Teresa T (2020). Tricuspid valve atresia. UpToDate. Retrieved January 7, 2021, from: https://www.uptodate.com/contents/tricuspid-valve-atresia
  2. Sadeghpour, A., & Alizadeasl, A. (2014). Tricuspid atresia. In A. Sadeghpour, M. Kyavar & A. Alizadehasl (Eds.), Comprehensive approach to adult congenital heart disease (pp. 233-235). London: Springer London. https://doi.org/10.1007/978-1-4471-6383-1_27
  3. Kliegman, R. M., M.D. et al. (2020). Cyanotic congenital heart disease: Lesions associated with decreased pulmonary blood flow. In R. M. Kliegman MD et al., Nelson textbook of pediatrics (pp. 239-2407.e1). https://www.clinicalkey.es/#!/content/3-s2.0-B9780323529501004570
  4. Rao, P. S. (2009). Diagnosis and management of cyanotic congenital heart disease: Part I. The Indian Journal of Pediatrics, 76(1), 57-70. https://link.springer.com/article/10.1007/s12098-009-0030-4
  5. Hoffman, J. I. E., & Kaplan, S. (2002). The incidence of congenital heart disease. https://www.sciencedirect.com/science/article/pii/S0735109702018867?via%3Dihub
  6. Reller, M. D., Strickland, M. J., Riehle-Colarusso, T., Mahle, W. T., & Correa, A. (2008). Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. The Journal of Pediatrics, 153(6), 807-813. https://www.jpeds.com/article/S0022-3476(08)00481-2/fulltext
  7. Freedom, R. M., Silver, M., & Miyamura, H. (1989). Tricuspid and pulmonary atresia with coarctation of the aorta: a rare combination possibly explained by persistence of the fifth aortic arch with a systemic-to-pulmonary arterial connection. International journal of cardiology, 24(2), 241–245. https://www.internationaljournalofcardiology.com/article/0167-5273(89)90314-8/pdf

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