The Mustard procedure was developed in 1963 by Dr. William Mustard at the Hospital for Sick Children in

Toronto Toronto ( ; or ) is the capital city of the Canadian province of Ontario. With a recorded population of 2,794,356 in 2021, it is the most populous city in Canada and the fourth most populous city in North America. The city is the ancho ...

, Ontario, Canada. Dr. Mustard, with support from the

Heart and Stroke Foundation of Canada The Heart and Stroke Foundation of Canada is a Canadian charity dedicated to advocacy, education, and the funding of research surrounding heart disease and stroke. Nomenclature In November 2016, the organization re-branded as ''Heart & Strok ...

, developed an alternative and simplified technique to the

Senning procedure The Senning procedure is an atrial switch heart operation performed to treat transposition of the great arteries. It is named after its inventor, the Swedish cardiac surgeon Åke Senning (1915–2000), also known for implanting the first permanent ...

which was used to correct a congenital heart defect that produced “ blue babies”. The technique was adopted by other surgeons and became the standard operation for d-TGA. In his autobiography,

South Africa South Africa, officially the Republic of South Africa (RSA), is the southernmost country in Africa. It is bounded to the south by of coastline that stretch along the South Atlantic and Indian Oceans; to the north by the neighbouring countri ...

n cardiac surgeon

Christiaan Barnard Christiaan Neethling Barnard (8 November 1922 – 2 September 2001) was a South African cardiac surgeon who performed the world's first human-to-human heart transplant operation. On 3 December 1967, Barnard transplanted the heart of accident-v ...

claims to have been the first to perform the operation, with Mustard only following 'several years later'.

Background

The defect is called

transposition of the great vessels Transposition of the great vessels (TGV) is a group of congenital heart defects involving an abnormal spatial arrangement of any of the great vessels: superior and/or inferior venae cavae, pulmonary artery, pulmonary veins, and aorta. Congenit ...

, or transposition of the great arteries (TGV or TGA). Until the late 1950s, and Senning's operation, the condition was commonly fatal. The defect causes blood from the

lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...

s to flow back to the lungs and blood from the body to flow back to the body. This occurs because the

aorta The aorta ( ) is the main and largest artery in the human body, originating from the left ventricle of the heart and extending down to the abdomen, where it splits into two smaller arteries (the common iliac arteries). The aorta distributes ...

and the

pulmonary artery A pulmonary artery is an artery in the pulmonary circulation that carries deoxygenated blood from the right side of the heart to the lungs. The largest pulmonary artery is the ''main pulmonary artery'' or ''pulmonary trunk'' from the heart, and t ...

, the two major arteries coming out of the heart, are connected to the wrong chambers. The babies look blue because there is insufficient oxygen circulating in their bodies.

Procedure

The Mustard Procedure allows total correction of transposition of the great vessels. The procedure employs a baffle to redirect caval blood flow to the left atrium which then pumps blood to the left ventricle which then pumps the deoxygenated blood to the lungs. In a normal heart, de-oxygenated blood is pumped into the lungs via the right ventricle. Then it is distributed throughout the body via the left ventricle. In the Mustard procedure, blood is pumped to the lungs via the left ventricle and disseminated throughout the body via the right ventricle.

Superseded by Arterial Switch

The Mustard procedure was largely replaced in the late 1980s by the Jatene procedure (arterial switch), in which the native arteries were switched back to normal flow, so that the RV (right ventricle) would be connected to the pulmonary artery and the LV (left ventricle) would be connected to the aorta. This surgery had not been possible prior to 1975 because of difficulty with re-implanting coronary arteries which perfuse the actual heart muscle itself (myocardium), and even after it was first performed the excellent results from the Mustard operation meant that it was a long time before the Jatene procedure took over.

Long-term survival

The Mustard procedure improved an 80%

mortality rate Mortality rate, or death rate, is a measure of the number of deaths (in general, or due to a specific cause) in a particular population, scaled to the size of that population, per unit of time. Mortality rate is typically expressed in units of de ...

in the first year of life to an 80% survival at age 20. Long-term follow-up studies now extend to more than 40 years post-operation and there are numerous patients thriving in their 50s. A Facebook group, Mustard or Senning Survivors, gathers several hundred global survivors in their 20s to 50s into a single community, supporting adults born with TGA that have had a Mustard, Senning, Rastelli or Nikaidoh heart procedure.

References

{{Cardiac surgery procedures Cardiac surgery

Background

Procedure

Superseded by Arterial Switch

Long-term survival

See also

References