Mustard procedure

Last updated
Mustard procedure
Specialty Cardiothoracic surgery, pediatric surgery
ICD-9-CM 35.91

The Mustard procedure was developed in 1963 by Dr. William Mustard at the Hospital for Sick Children. It is similar to the previous atrial baffle used with a Senning procedure, the primary difference being that the Mustard uses a graft made of Dacron or pericardium, while the Senning uses native heart tissue.

Contents

The procedure was developed to treat transposition of the great vessels, eponymously known as blue baby syndrome. This is a condition in which the aorta and pulmonary artery are attached to the heart in an opposite order from what is usually present at birth, resulting in the aorta being the outflow tract for the right ventricle and the pulmonary artery serving as the outgoing path for blood from the left ventricle. [1] The technique was adopted by other surgeons and became the standard operation for the dextro variant of transposition. [2]

The procedure was developed with support from the Heart and Stroke Foundation of Canada.

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

Prior to the development of the Senning procedure in the 1950s, blue baby syndrome from transposition was usually 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. As a result, the babies are blue at birth because of insufficient blood oxygen. [2]

Procedure

In a normal heart, the sequence of blood is from vena cava to right ventricle, then to the lungs and finally from lungs to the left ventricle to be circulated throughout the body. In patients with transposition, the order is from cava to left atrium and ventricle, then to the lungs and finally to the right side of the heart to be pumped out to systemic circulation. [2]

The Mustard Procedure allows total correction of transposition of the great vessels. It creates a baffle to redirect deoxygenated caval blood to the left atrium, which then pumps blood to the left ventricle, which in turn delivers this deoxygenated blood to the lungs. The effect is that the left ventricle is a functional right, and the right ventricle a functional left.

Superseded by Arterial Switch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

<span class="mw-page-title-main">Heart</span> Organ found inside most animals

The heart is a muscular organ found in most animals. This organ pumps blood through the blood vessels. Heart and blood vessels together make the circulatory system. The pumped blood carries oxygen and nutrients to the tissue, while carrying metabolic waste such as carbon dioxide to the lungs. In humans, the heart is approximately the size of a closed fist and is located between the lungs, in the middle compartment of the chest, called the mediastinum.

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.

<span class="mw-page-title-main">Atrial septal defect</span> Human heart defect present at birth

Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO). It is common in patients with a congenital atrial septal aneurysm (ASA).

<span class="mw-page-title-main">Fontan procedure</span> Surgical procedure used in children with univentricular hearts

The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries. The procedure varies for differing congenital heart pathologies. For example, in tricuspid atresia, the procedure can be done where the blood does not pass through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. By contrast, in hypoplastic left heart syndrome, the heart is more reliant on the more functional right ventricle to provide blood flow to the systemic circulation. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in July 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, presented at the Argentinean National Cardilogy meeting of that year and finally published in 1973.

<span class="mw-page-title-main">Cardiac catheterization</span> Insertion of a catheter into a chamber or vessel of the heart

Cardiac catheterization is the insertion of a catheter into a chamber or vessel of the heart. This is done both for diagnostic and interventional purposes.

<span class="mw-page-title-main">Atrium (heart)</span> Part of the human heart

The atrium is one of the two upper chambers in the heart that receives blood from the circulatory system. The blood in the atria is pumped into the heart ventricles through the atrioventricular mitral and tricuspid heart valves.

<span class="mw-page-title-main">Hypoplastic left heart syndrome</span> Type of congenital heart defect

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart. Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.

<span class="mw-page-title-main">Transposition of the great vessels</span> 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.

<span class="mw-page-title-main">Tricuspid atresia</span> Malformation where the heart lacks the tricuspid valve

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 occurs 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.

Pulmonic stenosis, is a dynamic or fixed obstruction of flow from the right ventricle of the heart to the pulmonary artery. It is usually first diagnosed in childhood.

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'.

<span class="mw-page-title-main">Arterial switch operation</span> Open heart surgical procedure

Arterial switch operation (ASO) or arterial switch, is an open heart surgical procedure used to correct dextro-transposition of the great arteries (d-TGA).

In cardiology, 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.

<span class="mw-page-title-main">Atrial septostomy</span> 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.

<span class="mw-page-title-main">Bidirectional Glenn procedure</span>

The bidirectional Glenn (BDG) shunt, or bidirectional cavopulmonary anastomosis, is a surgical technique used in pediatric cardiac surgery procedure used to temporarily improve blood oxygenation for patients with a congenital cardiac defect resulting in a single functional ventricle. Creation of a bidirectional shunt reduces the amount of blood volume that the heart needs to pump at the time of surgical repair with the Fontan procedure.

<span class="mw-page-title-main">Hypoplastic right heart syndrome</span> Type of congenital heart disease

Hypoplastic right heart syndrome (HRHS) is a congenital heart defect in which the structures on the right side of the heart, particularly the right ventricle, are underdeveloped. This defect causes inadequate blood flow to the lungs, and thus a 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.

Raghib syndrome is rare a congenital heart defect where the left superior vena cava (LSVC) is draining into the left atrium in addition to an absent coronary sinus and an atrial septal defect. This can be considered a dangerous heart condition because it puts the individual at a high risk of stroke. Other defects that are often associated with Raghib syndrome can include ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. While this is considered an extremely rare developmental complex, cases regarding a persistent left superior vena cava (PLSVC) are relatively common among congenital heart defects. It is also important to note that the PLSVC often drains into the right atrium, and only drains into the left atrium in approximately 10 to 20% of individuals with the defect.

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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