Tetralogy of Fallot – ToF

We’re now getting to a more complicated condition called the Tetralogy of Fallot – or ToF, for short. The diseases discussed until now had just one abnormality in the heart – Atrial Septal Defect (ASD) or Ventricular Septal Defect (VSD) or Patent Ductus Arteriosus (PDA).

As the name tetralogy implies, there are FOUR abnormalities combined together in this complex heart defect. ToF is also commonly called the “blue-baby” disease.

In this article, you’ll learn:

  • What are the components of ToF?
  • Why is it called “Blue Baby Disease”?
  • What exactly happens in ToF?
  • What is a “cyanotic spell”?
  • What happens if ToF is left uncorrected?
  • What are the surgical options?
  • How is a single stage intra-cardiac repair done?
  • Variations in ToF needing modified repair
  • What is an “outflow patch”?
  • When is an intracardiac repair NOT possible?
  • What are the palliative operations for ToF?
  • The Blalock-Taussig shunt
  • Other systemic-pulmonary shunt procedures
  • What is the outcome after a total correction operation?
  • What is the future course after a shunt procedure?

Basics of Tetralogy of Fallot

What are the four components of ToF ?

1. The first is a Ventricular Septal Defect – or VSD.

2. Next is a narrowing of the Pulmonary Valve, which guards the junction of the right ventricle with the pulmonary artery. This narrowing is called Pulmonary Stenosis (PS).

3. The third feature is a thickening of the wall of the right ventricle (RV). This increases the strength of the right ventricle and helps it pump blood more forcibly. This thickening is called RV Hypertrophy.

4. And the last component is an over-riding aorta.

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Hypoplastic Left Heart Syndrome – HLHS

The “Hypoplastic Left Heart Syndrome” – or more conveniently, “HLHS” – is a unique defect of the heart in many ways. Until a decade ago, the diagnosis was a virtual death sentence. Today, the revolution in cardiac surgical thinking and technique has changed the situation radically.

Whereas survival beyond the first few months of life was previously unheard of, many centers are today reporting encouraging results. And it is bound to improve further as more knowledge is gained from the early experience.

What is the HLHS ?

The heart has two upper and two lower chambers – one of each is right sided and the other left sided. The left sided chambers, with their blood vessels and valves are sometimes referred to as the LEFT HEART. ( This does not mean that the person has TWO hearts! )

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Double Outlet Right Ventricle – DORV

What is Double Outlet Right Ventricle (DORV) ?

Some of the defects I have described are “simple”, some are a little “complex” – but DORV is something else.

It is a common term that actually describes a wide spectrum of heart disease, ranging from something similar to a Ventricular Septal Defect (VSD), through Tetralogy of Fallot (ToF) to Transposition of the Great Arteries (TGA).

It is sometimes like one, at other times like another, and occasionally a mixture of some of them. So if at first you are baffled, don’t worry. I too was, and figured it out only after a long hard struggle.

What is Double Outlet Right Ventricle ?

Normally, a ventricle has just ONE outlet. For the left ventricle, this is the aorta. For the right ventricle it is the pulmonary artery. In DORV, both of these “outlet” blood vessels – aorta and pulmonary artery – arise from the RIGHT VENTRICLE, either totally or to a great extent.

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