The Trilogy of Fallot is a very rare form of congenital heart disease (CHD) composed of three defects [1] [2]:

• Atrial septal defect (ASD) - Interatrial connection caused by an opening in the atrial septum causes right-to-left shunting of blood.
• Pulmonary valve stenosis - A common feature shared with Tetralogy of Fallot, one of the most frequently encountered CHDs.
• Hypertrophy of the right ventricle - As a result of the increased effort needed to propel the blood through the stenotic pulmonary valve, hypertrophy of this cardiac chamber occurs and completes the trilogy of Fallot.

Although earlier reports suggested that Trilogy of Fallot is not an uncommon CHD [2], its reports are quite scarce [1] [3]. Clinical signs and symptoms seem to appear from the congestion of blood in the right heart and the portal system, and congestive heart failure may develop without early recognition [1] [2]. Similarly to other CHDs, manifestations of cyanosis, clubbing of fingers, and abnormal auscultatory findings during the physical examination are typically encountered in patients suffering from Trilogy of Fallot [2] [4].

• Atrial Septal Defect

  […] applied to concurrent pulmonic stenosis, atrial septal defect and right ventricular hypertrophy. [medical-dictionary.thefreedictionary.com]

  The Trilogy of Fallot is a very rare form of congenital heart disease (CHD) composed of three defects: Atrial septal defect (ASD) - Interatrial connection caused by an opening in the atrial septum causes right-to-left shunting of blood. [symptoma.com]

  However, Tetralogy of Fallot is associated with a ventricular septal defect instead of an atrial septal defect, and it also involves an overriding aorta. [wikidoc.org]

  septal defect (ASD) or patent foramen ovale (PFO). [scitechnol.com]

  However, the tetralogy has a ventricular septal defect instead of an atrial one, and it also involves an overriding aorta. [en.wikipedia.org]

• Heart Disease

  Very few reports describe this type of heart disease, and the diagnosis can be made only through imaging studies, principally echocardiography. [symptoma.com]

  Clinical Diagnosis of Congenital Heart Disease is the latest edition of this comprehensive, highly illustrated guide to the diagnosis of different forms of congenital heart disease. [books.google.de]

  Disease and Heart Disease in Pregnancy, Kaiser Foundation Hospitals, Panorama City, California, USA [books.google.com]

  Some examples of cardiovascular diseases include ischemic heart disease, heart valve disease, cardiomyopathies, peripheral artery disease, congenital heart disease, and vascular disease of the kidneys. This study is located in Bethesda, Maryland. [nhlbi.nih.gov]

  The trilogy of Fallot is a congenital heart disease consisting of the following defects: pulmonary valve stenosis, right ventricular hypertrophy and atrial septal defect. This disease is 1.6-1.8% of all congenital heart defects. [en.wikipedia.org]

• Heart Murmur

  In many patients, a palpable thrill can be detected, and a heart murmur, either at the left base or in the projection of the pulmonary valve, is readily noted. [symptoma.com]

  Infants come to attention because of a heart murmur or cyanosis. Symptoms can be appreciable when... References 1. Joly F, Carlotti J, Sicot PA Jr (1950) Congenital heart disease. II. Fallot’s trilogies. [link.springer.com]

  The sound occurs because the heart defect causes abnormal blood flow through the heart. However, not all heart murmurs are signs of congenital heart defects. Many healthy children have heart murmurs. [pediatricct.surgery.ucsf.edu]

Clinical suspicion toward any CHD can only be raised with a properly obtained patient history and a meticulous physical examination. The onset and progression of symptoms should be noted, whereas cardiac auscultation is perhaps the vital component of the diagnostic workup. In many patients, a palpable thrill can be detected, and a heart murmur, either at the left base or in the projection of the pulmonary valve, is readily noted [1] [2] [4]. If cardiac-related manifestations are present and support the finding obtained during the physical examination toward CHD as a possible diagnosis, electrocardiography (ECG) and imaging studies should be employed promptly [4] [5] [6]. Echocardiography is the primary method for detecting CHDs, including Trilogy of Fallot, and the use of standard 2D and Doppler techniques, as well as the increasingly available 3D imaging, are sufficient methods for confirming the diagnosis [4] [5] [6]. The benefit of US is that it is able to make the diagnosis prenatally as well, which allows enough time to consider the optimal therapeutic approach.

• Liver Biopsy

  Liver biopsy showed intrahepatic cholestasis, mild inflammatory infiltrates, as well as significant fibrosis, indicating both acute and chronic liver injuries. [ncbi.nlm.nih.gov]

  The liver biopsy revealed characteristic features of intrahepatic cholestasis with mild inflammatory infiltrates indicative of acute liver injury. [bmcgastroenterol.biomedcentral.com]

Thiamazole is a widely used antithyroid agent that has been approved for the treatment of hyperthyroidism. Although thiamazole-induced hepatotoxicity is a main side effect, it may progress to liver failure in a very few cases. [ncbi.nlm.nih.gov]

Treatment 1. Surgical repair Patients are usually diagnosed and treated during childhood. [doctorslounge.com]

Neither thyroid nor liver function had been regularly monitored during the treatment period or in the following 4 years. [bmcgastroenterol.biomedcentral.com]

For discussion of diagnosis and treatment, see tetralogy of Fallot. tril·o·gy of Fal·lot ( tril'ŏ-jē fah-lō' ) A set of congenital defects including pulmonic stenosis, atrial septal defect, and right ventricular hypertrophy. [medical-dictionary.thefreedictionary.com]

Some people put many of their children’s health difficulties down to the heart defect and treatment alone, so their children can miss out on help they need with other illnesses caused by a syndrome. [chfed.org.uk]

Treatment and prognosis Approximately 90% of untreated tetralogy of Fallot patients succumb by the age of 10 years 6. Over the years many surgical approaches were performed until the current primary repair was developed. [radiopaedia.org]

Adults with recalcitrant arrhythmias and right heart failure have the worst prognosis. Sudden death from ventricular arrhythmias has been reported in 1-5% of patients at a later stage in life, and the cause remains unknown. [emedicine.medscape.com]

We sought for etiologies of liver injury based on clinical course and suggestive changes in liver histology. [bmcgastroenterol.biomedcentral.com]

[…] and Pathophysiology Pathophysiology in pediatric patients with tetralogy of Fallot The cause(s) of most congenital heart diseases (CHDs) are unknown, although genetic studies suggest a multifactorial etiology. [emedicine.medscape.com]

Adult congenital heart disease in a nationwide population 2000-2014: epidemiological trends, arrhythmia, and standardized mortality ratio. J Am Heart Assoc. 2018 Feb 8. 7 (4): [Medline]. [emedicine.medscape.com]

Pathophysiology in pediatric patients with tetralogy of Fallot The cause(s) of most congenital heart diseases (CHDs) are unknown, although genetic studies suggest a multifactorial etiology. [emedicine.medscape.com]

Pathophysiology of congenital heart disease in the adult: part III: Complex congenital heart disease. Circulation 2008;117:1340-50. 2. Hamamoto M, Morifuji K. Surgery for an adult with tetralogy of Fallot and acquired heart disease. [j-pcs.org]

Endocarditis Prevention People with tetralogy of Fallot are at increased risk for developing endocarditis. People with unrepaired and partially repaired tetralogy will need antibiotics to prevent endocarditis before certain dental procedures. [heart.org]

It is feasible to do BPV followed by ASD device closure as this prevents an increased left to right shunt and at the same time the device placement is not disturbed by arrhythmias and ectopics as we pre-dilate the PV. [omicsonline.org]

Regular check-ups can help detect any problems early on and prevent long-term damage to the heart. [achaheart.org]

Lowering your baby's anxiety or stress can help prevent tet spells and save the baby's energy. For example, slowly picking up your baby and speaking in a soothing voice can avoid startling him or her, which may prevent or reduce crying. [pediatricct.surgery.ucsf.edu]

1. Shen C, Zhao C-Y, Liu F, Wang Y-D, Yu J. Acute-on-chronic liver failure due to thiamazole in a patient with hyperthyroidism and trilogy of Fallot: case report. BMC Gastroenterol. 2010;10:93.
2. SWAN H, MARCHIORO T, KINARD S, BLOUNT SG. Trilogy of Fallot. Experience with twenty-two surgical cases. Arch Surg. 1960;81:291-298.
3. Wang YQ, Chen RK, Ye WW, et al. Open-heart surgery in 48 patients via a small right anterolateral thoracotomy. Tex Heart Inst J. 1999;26(2):124-128.
4. Porter RS, Kaplan JL. Merck Manual of Diagnosis and Therapy. 19th Edition. Merck Sharp & Dohme Corp. Whitehouse Station, N.J; 2011.
5. Bailliard F, Anderson RH. Tetralogy of Fallot. Orphanet J Rare Dis. 2009;4:2.
6. Villafañe J, Feinstein JA, Jenkins KJ, et al. Hot topics in tetralogy of Fallot. J Am Coll Cardiol. 2013;62(23):2155-2166.