BIOMED - Pathophysiology

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©️ Cincinnati Children's

Single Ventricle Anomalies

Single ventricle anomalies refer to a collection of cardiac defects affecting one of the two ventricles in the heart, requiring reconstructive surgery to develop a fontan circulation. These single ventricle defects are not limited to:

☞ Tricuspid atresia
☞ Hypoplastic left heart syndrome
☞ Double inlet left ventricle
☞ Many heterotaxy defects
☞ Some variations of double outright right ventricle

Diagnosis
Echocardiogram to determine the defect present.

Cardiac catheterisation is only necessary in new boards with a single ventricle abnormality if the echo cannot identify it. CT scans may be used to add details and information about the abnormality.

Treatment
Patients with single ventricle anomalies will undergo the fontan circulation series of reconstructive palliative procedures, such as was discussed in the chapter for Hypoplastic Heart Syndrome.

Note, the end circulation that is achieved at the end of these three procedures is called the fontan circulation, as it is not a normal anatomical circulation.

Management
As I imagine you may suspect, patients with this history will require life-long specialist cardiology follow up. These patients only have one pumping chamber.

Many Fontan patients will have abnormal heart rhythms that requires treatment. Some will experience low blood-oxygen levels limiting physical activity tolerance. Some may have weakening of their pumping chamber, or leakage from one or more of the valves, and 10% of patients will develop protein losing enteropathy that requires careful management.

Fontan patients may also experience liver or kidney function impairments or problems requiring monitoring.

Women who wish to become pregnant should be assessed and counselled before becoming pregnant, and if pregnancy occurs, this should be managed by specialised medical teams skilled in managing pregnancy in women with cardiac defects.

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