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Trilogy of Fallot

Fallot Trilogy

Trilogy of Fallot is comprised of pulmonary stenosis, hypertrophy of the right ventricle and an atrial septal defect, and is a distinct clinical entity from Tetralogy of Fallot, one of the most common congenital heart diseases in clinical practice. Very few reports describe this type of heart disease, and the diagnosis can be made only through imaging studies, principally echocardiography.


Presentation

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
  • Trilogy of Fallot is comprised of pulmonary stenosis, hypertrophy of the right ventricle and an atrial septal defect, and is a distinct clinical entity from Tetralogy of Fallot, one of the most common congenital heart diseases in clinical practice.[symptoma.com]
  • Fallot, Étienne-Louis A., French physician, 1850-1911. pentalogy of Fallot - Fallot tetralogy with, in addition, a patent foramen ovale or atrial septal defect. trilogy of Fallot - atrial septal defect associated with pulmonic stenosis and right ventricular[medical-dictionary.thefreedictionary.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]
Jaundice
  • Case Presentation A 24-year-old man was admitted to our hospital due to diarrhea, progressive jaundice, light stool, fatigue and excessive sweating for 18 days.[bmcgastroenterol.biomedcentral.com]
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]
  • Trilogy of Fallot Classification and external resources Specialty medical genetics ICD - 10 Q21.3 ICD9 746.09 , 746.8 ( CDC/BPA 746.84) MeSH D014286 [ edit on Wikidata ] The trilogy of Fallot is a congenital heart disease consisting of the following defects[en.wikipedia.org]
  • 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]
  • And also done a fellowship in structural heart disease at Royal Brompton Hospital, UK and training in Intravascular Ultrasound at Cleveland Clinic, USA PDF HTML[omicsonline.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]
  • Another common sign of tetralogy of Fallot is a heart murmur . A heart murmur is an extra or unusual sound that doctors may hear while listening to the heart. The sound occurs because the heart defect causes abnormal blood flow through the heart.[pediatricct.surgery.ucsf.edu]

Workup

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]

Treatment

  • (September 2017) Treatment [ edit ] Thoracotomy is used to surgically correct this pathology. [1] History [ edit ] It is named in honor of its discoverer: Etienne Fallot . [2] References [ edit ] Wang YQ, Chen RK, Ye WW, et al. (1999).[en.wikipedia.org]
  • 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]
  • A comprehensive account of the pathology, natural history, and treatment of both congenital and acquired heart disease is given for both the adult and pediatric age groups.This valuable reference volume is designed for medical students, interns, residents[books.google.de]
  • 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]

Prognosis

  • 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]

Etiology

  • Q000139] ; classification [Q000145] ; complications [Q000150] ; diagnosis [Q000175] ; diet therapy [Q000178] ; drug therapy [Q000188] ; economics [Q000191] ; embryology [Q000196] ; enzymology [Q000201] ; epidemiology [Q000453] ; ethnology [Q000208] ; etiology[slicksurface.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]

Epidemiology

  • […] cerebrospinal fluid [Q000134] ; chemically induced [Q000139] ; classification [Q000145] ; complications [Q000150] ; diagnosis [Q000175] ; diet therapy [Q000178] ; drug therapy [Q000188] ; economics [Q000191] ; embryology [Q000196] ; enzymology [Q000201] ; epidemiology[slicksurface.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]
Sex distribution
Age distribution

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]
  • 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]

Prevention

  • […] etiology [Q000209] ; genetics [Q000235] ; history [Q000266] ; immunology [Q000276] ; metabolism [Q000378] ; microbiology [Q000382] ; mortality [Q000401] ; nursing [Q000451] ; parasitology [Q000469] ; pathology [Q000473] ; physiopathology [Q000503] ; prevention[slicksurface.com]
  • 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]
  • 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]
  • Regular check-ups can help detect any problems early on and prevent long-term damage to the heart.[achaheart.org]

References

Article

  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.

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Last updated: 2018-06-22 06:26