Pentalogy of Fallot

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

Overview

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

Historical Perspective

tetrallogy of fallot was first discovered by Niel Stenson, a Denish physician Anatomist, in 1671.

The association between Rigt ventriular outflow tract obstruction and a large ventricular septal defect and tetrallogy of fallot. was made in 1888 by Etinne-Louise].

In 1924, Canadian, Maude Abbott was the first to discover the association between ventricular septal defect , pulmonary stenosis, overriding aorta and right ventricular hypertrophy and the development of tetrallogy of Fallot.

Classification

There is no established system for the classification of [pentallogy of fallot ].

Pathophysiology

It is thought that [tetrallogy of fallot] is the result of anterocephalad deviation of the aortico pulmonary septum thus leading to

• ventricular septal defect
• right ventricular outflow tract obstruction
• overriding aorta
• right ventricular hypertrophy with an additional ASD( atrial septal defect) with ostium secundum type being the most common followed by ostium primum . thus making it a pentallogy of fallot. even though there are no reports on the molecular pathophysiology of pentallogy of fallot, conotruncal heart defects like tetrallogy of fallot are associated with neural crest migration defect. systemic pathophysiology of tetrallogy of fallot depends on the extent of right ventricular outflow tract obstruction, severe pulmonic stenosis leads to a significant decrease in pulmonary circulation and worsening right to left shunt leading to cyanosis.A collateral PDA makes Tetrallogy of fallot compatible with life as it preserves pulmonic circulation

Causes

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

Differentiating pentallogy of fallot from other Diseases

pentallogy of Fallot must be differentiated from other diseases that cause right to left shunt such as transposition of great arteries with pulmonic stenosis, double outlet right ventricle including Taussig-Bing anomaly, tricuspid atresia, Ebstein Anomaly, pulmonary atresia with intact ventricular septum , Respiratory diseases such as Bronchiolitis,viral and bacterial pneumonia and pneumothorax because when they are in heart failure they present with signs of respiratory distress, cyanosis and failure of thrive and Bidirectional shunt diseases such as total anomalous pulmonary venous return, hypoplastic left heart syndrome, single ventricle and truncus arteriosus

Epidemiology and Demographics

The incidence/prevalence of [tetralogy of fallot] is approximately [11-13.5%] with about 10% having ASD.

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Patients of all age groups may develop [disease name].

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The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.

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[Disease name] commonly affects individuals younger than/older than [number of years] years of age.

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[Chronic disease name] is usually first diagnosed among [age group].

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[Acute disease name] commonly affects [age group].

There is no racial predilection to [pentalogy of fallot.

[Disease name] affects men and women equally.

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[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.

The majority of [disease name] cases are reported in [geographical region].

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[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].

Risk Factors

Common risk factors in the development of pentalogy of fallot includes

• Alcoholism in mother
• Maternal Diabetes
• Advanced maternal age i.e greater than 40 years
• Malnutrition in pregnancy
• Family history of tetralogy of fallot
• Viral illness during pregnancy like Rubella.
• presence of Down syndrome or DiGeorge syndrome

Screening

There is insufficient evidence to recommend routine screening for 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 and polycythemia with relative iron deficiency. Prognosis is generally excellent provided the VSD has been closed and the right ventricular outflow tract has been relieved,

Diagnosis

Diagnostic Study of Choice

There are no established criteria for the diagnosis of [pentalogy fallot].

History and Symptoms

The majority of patients with [disease name] are asymptomatic.

OR

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 defct ]. The most common symptoms of [pentalogy of fallot] include [cyanosis], [easy fatigabillity ], and [hypercyanotic spells with crying , feeding and defecating ]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Patients with pentalogy of fallot usually appear cyanotic. Physical examination of patients with pentalogy of fallot is usually remarkable for cyanosis, systolic murmur at the left upper sternal boarder , and finger clubbing .

Laboratory Findings

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

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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.

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.

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Echocardiography or Ultrasound

Echocardiography may be helpful in the diagnosis of pentalogy of fallot. Findings on an echocardiography diagnostic of pentallogy of fallot include Ventricular septal defect, Atrial septal defect , Right ventricular outflow tract obstruction and overriding aorta

CT scan

Thoracic CT angiography may be helpful in the diagnosis of pentallogy of fallot. Findings on CT scan diagnostic of pentalogy of fallot include ventricular septal defect, right ventricular hypertrophy, pulmonary atresia and major aortopulmonary collateral arteries.

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.

Other Imaging Findings

There are no other imaging findings associated with pentalogy of Fallot.

Other Diagnostic Studies

Other diagnostic studies for pentalogy of fallot include Right ventricular angiopraphy, which demonstrates infundibular and pulmonary artery anatomy, and left ventricular angiography, which demonstrates VSD, ASD, and Overiding aorta

Treatment

Medical Therapy

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

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Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

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The majority of cases of [disease name] are self-limited and require only supportive care.

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[Disease name] is a medical emergency and requires prompt treatment.

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The mainstay of treatment for [disease name] is [therapy].

OR   The optimal therapy for [malignancy name] depends on the stage at diagnosis.

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[Therapy] is recommended among all patients who develop [disease name].

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Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

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Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

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Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgical intervention is not recommended for the management of [disease name].

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Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

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The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

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The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

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Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

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There are no available vaccines against [disease name].

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Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

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[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

References

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