Pentalogy of Fallot

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Abdul-Kareem Lukan, M.D.

Overview

Pentalogy of Fallot is defined as the occurrence of Tetralogy of Fallot plus an atrial septal defect.

Department of Companion Animal Clinical Studies, Faculty of Veterinary Science, University of Pretoria, Private Bag X04, Onderstepoort 0110, South Africa. paolo.pazzi@up.ac.za.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3984729/

Historical Perspective

Classification

  • There is no established system for the classification of the pentalogy of Fallot.

Pathophysiology

It is thought that conotruncal diseases like tetralogy of fallot and pentalogy of Fallot occur as a result of:[3]

  • Neural crest migration defect.
  • Underdevelopment of right ventricular infundibulum

Causes

  • The cause of the pentalogy of Fallot has not been identified.
  • To review risk factors for the development of pentalogy of Fallot, click here.

Differentiating pentalogy of Fallot from other Diseases

  • Pentalogy of Fallot must be differentiated from other diseases that cause:[1]

Right to left shunt

Bidirectional shunt diseases

Epidemiology and Demographics

The epidemiology of the pentalogy of Fallot has not been well studied.

Risk Factors

Common risk factors in the development of pentalogy of fallot include:[4]

Screening

There is insufficient evidence to recommend routine screening for the pentalogy of Fallot.

Natural History, Complications, and Prognosis

If left untreated, patients with pentalogy of fallot may progress to develop finger clubbing, stunted growth, cardiac arrhythmias, hypoplastic pulmonary artery, paradoxical emboli and polycythemia .[3] Prognosis is generally excellent provided the VSD has been closed and the right ventricular outflow tract has been relieved.[5]

Diagnosis

Diagnostic Study of Choice

There are no established criteria for the diagnosis of pentalogy Fallot.

History and Symptoms

The hallmark of pentalogy of fallot is cyanosis. A positive history of cyanosis and easy fatigability leading to squatting during exercise is suggestive of cyanotic congenital heart defect.[5] . The most common symptoms of pentalogy of fallot include cyanosis, easy fatigabillity , and hypercyanotic spells with crying , feeding and defecating.[5]

Physical Examination

Patients with pentalogy of Fallot appear cyanotic.[3][6]

Laboratory Findings

Some patients with pentalogy of fallot may have polycythemia which is usually suggestive of worsening hypoxia and compensatory production of erythropoetin.[5]

Electrocardiogram

An ECG may be helpful in the diagnosis of pentalogy of Fallot, Findings on an ECG suggestive of pentalogy of fallot include right axis deviation and right ventricular hypertrophy.[5]

Echocardiography Research Center, Rajaie Cardiovascular, Medical and Research Center, Iran University of Medical Sciences, Tehran,https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3874376/

X-ray

An x-ray may be helpful in the diagnosis of pentalogy of Fallot. Findings on an x-ray suggestive of pentalogy of fallot include boot shaped heart or coeur en sabot.[5]

Typical preoperative chest X‐ray of a 16‐month‐old boy with tetralogy of Fallot.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5412831/

Echocardiography or Ultrasound

Echocardiography may be helpful in the diagnosis of pentalogy of fallot. Findings on an echocardiography diagnostic of pentalogy of fallot include.[5]

CT scan

Thoracic CT angiography may be helpful in the diagnosis of pentalogy of fallot. Findings on CT scan diagnostic of pentalogy of fallot include. [7]

MRI

cardiac MRI may be helpful in the diagnosis of pentalogy of fallot. Findings on MRI diagnostic of pentalogy of fallot include RVOT stenosis, VSD, ASD,Overriding aorta, right ventricular hypertrophy and hypoplastic pulmonary arteries.[7]


Other Imaging Findings

Other imaging findings for pentalogy of fallot include Right ventricular angiopraphy which demonstrates infundibular and pulmonary artery anatomy. left ventricular angiography, which demonstrates. [5]

Other Diagnostic Studies

There are no other diagnostic studies associated with pentalogy of Fallot

Treatment

Medical Therapy

The mainstay of treatment for pentalogy of fallot is Surgery.[5]

Pharmacologic medical therapy is recommended among patients with severe RVOTO and hypercyanotic spells.[5]


Pharmacologic medical therapies for pentalogy of fallot include.[5]


Patients with hypercyanotic spells are treated with,

  • oxygen
  • volume expansion
  • morphine sedation.

Surgery

Surgery is the mainstay of treatment for the pentalogy of Fallot. interventional procedures are used to relieve pulmonary obstructions and embolize collaterals. different surgical procedures can be done to treat pentalogy of fallot and it includes.[5]

Primary Prevention

There are no established measures for the primary prevention of the pentalogy of Fallot.

Secondary Prevention

There are no established measures for the secondary prevention of the pentalogy of Fallot.

References

  1. 1.0 1.1 Diaz-Frias J, Guillaume M. PMID 30020660. Missing or empty |title= (help)
  2. 2.0 2.1 Apitz, Christian; Webb, Gary D; Redington, Andrew N (2009). "Tetralogy of Fallot". The Lancet. 374 (9699): 1462–1471. doi:10.1016/S0140-6736(09)60657-7. ISSN 0140-6736.
  3. 3.0 3.1 3.2 OM, Akinlade; JA, Ogunmodede; OA, Adeyemi; WO, Yusuf; RO, Awodun; YA, Ayoola; IA, Yusuf; PM, Kolo (2020). "Adult Nigerian with Untreated Pentalogy of Fallot: A Case Report". Asploro Journal of Biomedical and Clinical Case Reports. 3 (1): 62–66. doi:10.36502/2020/ASJBCCR.6187. ISSN 2582-0370.
  4. Silva, Joana A.; Neves, Ana L.; Flor-de-Lima, Filipa; Soares, Paulo; Guimarães, Hercília (2019). "Risk Factors and Outcomes of Tetralogy of Fallot: From Fetal to Neonatal Life". Pediatric Cardiology. 41 (1): 155–164. doi:10.1007/s00246-019-02239-4. ISSN 0172-0643.
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  6. Parwani, Purvi; Phillips, Sabrina (2016). "IMPORTANCE OF PHYSICAL EXAMINATION IN ADULT PATIENTS WITH TETRALOGY OF FALLOT". Journal of the American College of Cardiology. 67 (13): 1101. doi:10.1016/S0735-1097(16)31102-0. ISSN 0735-1097.
  7. 7.0 7.1 Lapierre, C.; Dubois, J.; Rypens, F.; Raboisson, M.-J.; Déry, J. (2016). "Tetralogy of Fallot: Preoperative assessment with MR and CT imaging". Diagnostic and Interventional Imaging. 97 (5): 531–541. doi:10.1016/j.diii.2016.01.009. ISSN 2211-5684.

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