Tricuspid atresia differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Sara Zand, M.D.[2] Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [4]; Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Overview

Patients with tricuspid atresia should be differentiated from other cardiac causes of cyanosis and lung olygemia include:Tetralogy of Fallot, Truncus arteriosus, Total anomalous pulmonary venous connection, Pulmonary atresia, Tricuspid stenosis.

Differentiating tricuspid atresia from other Diseases

Patients with tricuspid atresia should be differentiated from other cardiac causes of cyanosis and lung olygemia include:

Table below compares cyanotic heart diseases:^[1]^[2]^[3]^[4]


Disorders	Etiology	Clinical Presentation	Laboratory Findings	Electrocardiogram Findings	Echocardiography Findings	X-Ray Findings
Tetralogy of Fallot	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Cyanosis on exertion Exertional dyspnea Palpitations Fatigue	CBC: Anemia or polycythemia. Coagulation profile. Arterial blood gas: Low oxygen saturation and acidosis	Right ventricular hypertrophy Right bundle branch block Tachycardia Rate of QRS change predicts ventricular arrhythmia	Echocardiography may show: Residual VSD or ASD RV outflow tract obstruction Abnormal valvular anatomy	The boot-shaped heart appearance Normal heart size Pulmonary vascular marking may be normal or decreased
Total Anomalous Pulmonary Venous Connection	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Tachypnea Palpitations Cyanosis Failure to thrive	Arterial blood gas: Low oxygen saturation and acidosis Coagulation profile.	Right ventricular hypertrophy with a qR pattern	Right ventricular hypertrophy Right ventricular loading Paradoxical septal motion	Prominence of the pulmonary arteries Mild enlargement of heart The classic snowman sign is seen in supracardiac subtype
Tricuspid Atresia	Multifactorial Genetic polymorphisms Maternal rubella Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Respiratory difficulties as nasal flaring or muscle retractions Cyanosis Growth retradation	Arterial blood gases CBC: Polycythemia Coagulation profile	Tall P waves indicate atrial enlargement. First-degree atrioventricular block. Frontal plane QRS axis may be leftward.	Echocardiography may show Defect size Pulmonary blood flow Ventricular function Valve abnormalities	Smooth convexity of right heart border is absent Lower part of right heart border is limited to the spine Prominency of the pulmonary artery depends on their blood flow Cardiac size may be normal or enlarged
Transposition of the Great Arteries	Multifactorial Genetic polymorphisms Poor prenatal nutrition Maternal alcohol use Maternal diabetes	Prominent cyanosis within hours of birth Congestive heart failure	Arterial blood gases: Hypoxemia Hyperoxia test	Right ventricular hypertrophy Right axis deviation Varying degrees of AV block Q waves	Echocardiography may show: Relationship between great vessels Associated anatomic lesions Coronary artery origin and branches	The classic egg on string appearance Pulmonary vascular marking may be normal or increased

References

↑ Bailliard F, Anderson RH (January 2009). “Tetralogy of Fallot”. Orphanet J Rare Dis. 4: 2. doi:10.1186/1750-1172-4-2. PMC 2651859. PMID 19144126.
↑ Kao CC, Hsieh CC, Cheng PJ, Chiang CH, Huang SY (2017). “Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update”. J Med Ultrasound. 25 (3): 130–137. doi:10.1016/j.jmu.2017.08.002. PMC 6029298. PMID 30065477.
↑ ASTLEY R, OLDHAM JS, PARSONS C (July 1953). “Congenital tricuspid atresia”. Br Heart J. 15 (3): 287–97. doi:10.1136/hrt.15.3.287. PMC 479498. PMID 13059216.
↑ Unolt M, Putotto C, Silvestri LM, Marino D, Scarabotti A, Valerio M, Caiaro A, Versacci P, Marino B (June 2013). “Transposition of great arteries: new insights into the pathogenesis”. Front Pediatr. 1: 11. doi:10.3389/fped.2013.00011. PMC 3860888. PMID 24400257. Vancouver style error: initials (help)

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[pmid19144126-1] Bailliard F, Anderson RH (January 2009). “Tetralogy of Fallot”. Orphanet J Rare Dis. 4: 2. doi:10.1186/1750-1172-4-2. PMC 2651859. PMID 19144126.

[pmid30065477-2] Kao CC, Hsieh CC, Cheng PJ, Chiang CH, Huang SY (2017). “Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update”. J Med Ultrasound. 25 (3): 130–137. doi:10.1016/j.jmu.2017.08.002. PMC 6029298. PMID 30065477.

[pmid13059216-3] ASTLEY R, OLDHAM JS, PARSONS C (July 1953). “Congenital tricuspid atresia”. Br Heart J. 15 (3): 287–97. doi:10.1136/hrt.15.3.287. PMC 479498. PMID 13059216.

[pmid24400257-4] Unolt M, Putotto C, Silvestri LM, Marino D, Scarabotti A, Valerio M, Caiaro A, Versacci P, Marino B (June 2013). “Transposition of great arteries: new insights into the pathogenesis”. Front Pediatr. 1: 11. doi:10.3389/fped.2013.00011. PMC 3860888. PMID 24400257. Vancouver style error: initials (help)

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