Mustard procedure

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Mustard procedure
ICD-9-CM 35.91

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

Contents

Dr. Mustard, with support from the Heart and Stroke Foundation of Canada, developed an alternative and simplified technique to the Senning procedure which was used to correct a congenital heart defect that produced “blue babies”. [1] The technique was adopted by other surgeons and became the standard operation for d-TGA. [2]

In his autobiography, South African cardiac surgeon Christiaan Barnard claims to have been the first to perform the operation, with Mustard only following 'several years later'. [3]

Background

The defect is called transposition of the great vessels, 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 lungs to flow back to the lungs and blood from the body to flow back to the body. This occurs because the aorta and the pulmonary artery, 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. [2]

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. [2]

Superseded by Arterial Switch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.[ citation needed ]

Long-term survival

The Mustard procedure improved an 80% mortality rate 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 [4] and there are numerous patients thriving in their 50s. A Facebook group, Mustard or Senning Survivors, [5] 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.[ citation needed ]

See also

Related Research Articles

Aortic valve Valve in the human heart between the left ventricle and the aorta

The aortic valve is a valve in the heart of humans and most other animals, located between the left ventricle and the aorta. It is one of the four valves of the heart and one of the two semilunar valves, the other being the pulmonary valve. The aortic valve normally has three cusps or leaflets, although in 1–2% of the population it is found to congenitally have two leaflets. The aortic valve is the last structure in the heart the blood travels through before stopping the flow through the systemic circulation.

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

Congenital heart defect Defect in the structure of the heart that is present at birth

A congenital heart defect (CHD), also known as a congenital heart anomaly and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure.

The Rastelli procedure is an open heart surgical procedure developed by Italian physician and cardiac surgery researcher, Giancarlo Rastelli, in 1967 at the Mayo Clinic, and involves using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary stenosis.

Transposition of the great vessels Group of congenital heart defects

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. Congenital heart diseases involving only the primary arteries belong to a sub-group called transposition of the great arteries (TGA), which is considered the most common congenital heart lesion that presents in neonates.

Pulmonary atresia Medical condition

Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close

Tricuspid atresia Medical condition

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.

Levo-Transposition of the great arteries is an acyanotic congenital heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed.

Norwood procedure

The Norwood procedure is the first of three surgeries intended to create a new functional systemic circuit in patients with hypoplastic left heart syndrome and other complex heart defects with single ventricle physiology. The first successful Norwood procedure involving the use of a cardiopulmonary bypass was reported by Dr. William Imon Norwood, Jr. and colleagues in 1981.

Double outlet right ventricle (DORV) is a form of congenital heart disease where both of the great arteries connect to the right ventricle (RV). In some cases it is found that this occurs on the left side of the heart rather than the right side.

Taussig–Bing syndrome is a cyanotic congenital heart defect in which the patient has both double outlet right ventricle (DORV) and subpulmonic ventricular septal defect (VSD).

A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles.

A baffle is a surgically-created tunnel or wall within the heart or major blood vessels used to redirect the flow of blood. They are used in some types of heart abnormalities that a child is born with known as congenital heart defects. Baffles are usually constructed, at least in part, from a person's own heart tissue, while other methods of redirecting blood using artificial material are known by the more generic term 'conduits'. Baffle does not refer to surgical techniques that redirect blood outside the heart or blood vessels such as coronary artery bypass grafting.

<span class="mw-page-title-main">Arterial switch operation</span>

Arterial switch operation (ASO) or arterial switch, is an open heart surgical procedure used to correct dextro-transposition of the great arteries (d-TGA); its development was pioneered by Canadian cardiac surgeon William Mustard and it was named for Brazilian cardiac surgeon Adib Jatene, who was the first to use it successfully. It was the first method of d-TGA repair to be attempted, but the last to be put into regular use because of technological limitations at the time of its conception.

A cardiac shunt is a pattern of blood flow in the heart that deviates from the normal circuit of the circulatory system. It may be described as right-left, left-right or bidirectional, or as systemic-to-pulmonary or pulmonary-to-systemic. The direction may be controlled by left and/or right heart pressure, a biological or artificial heart valve or both. The presence of a shunt may also affect left and/or right heart pressure either beneficially or detrimentally.

Atrial septostomy Surgical procedure on the heart

Atrial septostomy is a surgical procedure in which a small hole is created between the upper two chambers of the heart, the atria. This procedure is primarily used to palliate dextro-Transposition of the great arteries or d-TGA, a life-threatening cyanotic congenital heart defect seen in infants. It is performed prior to an arterial switch operation. Atrial septostomy has also seen limited use as a surgical treatment for pulmonary hypertension. The first atrial septostomy was developed by Vivien Thomas in a canine model and performed in humans by Alfred Blalock. The Rashkind balloon procedure, a common atrial septostomy technique, was developed in 1966 by American cardiologist William Rashkind at the Children's Hospital of Philadelphia.

Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant.

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 cardiac pacemaker in 1958.

The Damus–Kaye–Stansel (DKS) procedure is a cardiovascular surgical procedure used as part of the repair of some congenital heart defects. This procedure joins the pulmonary artery and the aorta in situations where the systemic circulation is obstructed. It is commonly used when a patient has the combination of a small left ventricle and a transposition of the great arteries (TGA); in this case, the procedure allows blood to flow from the left ventricle to the aorta.

References

  1. ↑ Ken Heiden (2009-07-01). Congenital Heart Defects, Simplified. Midwest EchoSolutions. pp. 63–. ISBN   978-0-9822709-0-5.
  2. 1 2 3 Love, Barry A; Mehta, Davendra; Fuster, Valentin F (2008). "Evaluation and management of the adult patient with transposition of the great arteries following atrial-level (Senning or Mustard) repair". Nature Clinical Practice Cardiovascular Medicine. 5 (8): 454–467. doi:10.1038/ncpcardio1252. ISSN   1743-4297. PMID   18594551. S2CID   10491740.
  3. ↑ Barnard, Christiaan N. (1969). One Life. Bantam. p. 213. ISBN   9780552659888
  4. ↑ Vejlstrup, Niels; Sørensen, Keld; Mattsson, Eva; Thilén, Ulf; Kvidal, Per; Johansson, Bengt; Iversen, Kasper; Søndergaard, Lars; Dellborg, Mikael; Eriksson, Peter (2015). "Long-Term Outcome of Mustard/Senning Correction for Transposition of the Great Arteries in Sweden and Denmark". Circulation. 132 (8): 633–638. doi:10.1161/CIRCULATIONAHA.114.010770. PMID   26185211. S2CID   37856209.
  5. ↑ "Mustard or Senning survivors". Facebook .


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