Transposition of The Great Arteries

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Transposition of The Great Arteries
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Transposition of the Great Arteries

• Most common form of cyanotic congenital heart
  disease (5 to 7 of all congenital heart defects
  ) which presents in the newborn period.
• More common in males
• Babies are usually normal birth weight and size.

1. Aorta emerges from RV 5. PDA
2. PA emerges from LV
3. VSD
4. ASD

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Transposition of the Great Arteries

• Associated heart abnormalities
• Ventricular septal defect the most common
• Left ventricular outflow tract obstruction.
• Atrial septal defect
• Patent ductus arteriosus
• Physical Findings
• Cyanosis, seen in lips and under fingernail beds
  may not be present if baby has either an atrial
  septal defect and/or a patent ductus arteriosus.
  A ventricular septal defect is also compatible
  with life, but congestive heart failure will
  develop because of the extra blood flow going to
  the lungs. Sxs consistent with CHF poor
  feeding, poor weight gain, sweating, and fast or
  labored breathing

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Transposition of the Great Arteries

• Physical Findings
• Cyanosis will not improve with the application of
  oxygen if it does, then the problem is
  pulmonary, not cardiac.
• The second heart sound is loud and single
• ? heart murmur, depending if there is a VSD or
  not
• Diagnosis
• From physical findings ? Echo, which if not 100
  definitive ? heart cath

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Transposition of the Great Arteries

• The type and timing of operation depend on the
  combination of defects that accompany the primary
  problem of TGA.
• Development of early pulmonary vascular disease
  is also common. The changes associated with PVD
  can occur as early as a few weeks of life and
  tend to occur more frequently in babies who have
  ventricular septal defects in addition to TGA.
  Early corrective surgery minimizes the chances of
  development of PVD.

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Transposition of the Great Arteries

• Treatment
• Prostaglandin is administered until surgery can
  be performed to help maintain a patent ductus
  arteriosus
• A balloon atrial septostomy can be performed
  during a heart cath if the cyanosis is severe
  this involves passing a catheter into the right
  atrium and across the atrial septal defect. Once
  the catheter is in the left atrium a balloon is
  expanded and pulled back through the ASD into the
  right atrium enlarging the ASD, and allowing more
  mixing of oxygenated and deoxygentaed blood ?
  higher oxygen levels while the baby awaits
  surgery

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Transposition of the Great Arteries

• Surgical procedures
• Arterial Switch
• The aorta and pulmonary artery are divided and
  reconnected so that the pulmonary artery is
  connected to the right ventricle and supplies
  deoxygenated blood to the lungs. The aorta is
  connected to the left ventricle and supplies
  oxygenated blood to the body. The coronary
  arteries are also relocated so that they will
  receive oxygenated blood from the aorta.
• Any septal defects are closed at the same time

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Transposition of the Great Arteries

1. Deoxygenated blood redirected to LA
2. LV ? Lungs
3. Oxygenated blood from lungs redirected to RA
4. RV ? Body

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Transposition of the Great Arteries

• Surgical procedures
• Mustard Procedure
• Used very rarely now, but most commonly done if
  the baby is older at diagnosis
• Deoxygenated blood returning to the right atrium
  is redirected to the left atrium, and flows to
  the left ventricle where it is pumped through the
  pulmonary arteries to the lungs.
• Oxygenated blood returning from the lungs is
  baffled to the right atrium, and flows to the
  right ventricle where it is pumped through the
  aorta and out to the body.
• Even though the blood goes to the right location,
  the heart remains configured so the right
  ventricle (the weaker ventricle) pumps blood to
  the high pressure system of the aorta, and the
  left ventricle pumps blood to the low pressure
  system of the lungs.

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Transposition of the Great Arteries

1. Deoxygenated blood redirected to LA
2. LV ? Lungs
3. Oxygenated blood from lungs redirected to RA
4. RV ? Body

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Transposition of the Great Arteries

• Surgical procedures
• Rastelli Procedure
• Performed for TGA with left ventricular outflow
  obstruction
• The ventricular septal defect is closed in such a
  way that the left ventricle is connected to the
  aorta and the right ventricle is connected to the
  lungs using a conduit with a valve in it.
• One end of the conduit is connected to the right
  ventricle where blood exits into the pulmonary
  artery and the other end is attached to the
  pulmonary artery
• The conduit acts a bypass around the naturally
  occurring narrowing.

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Transposition of the Great Arteries

• Surgical procedures
• Rastelli Procedure
• This conduit will need to be replaced as the
  child grows. Usually this is not necessary for 3
  to 5 years after the original operation if done
  in infancy and then 1 to 2 more times throughout
  the child's life.

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Transposition of the Great Arteries

1. VSD closed with patch
2. Conduit

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Transposition of the Great Arteries

• Long-term complications of the Mustard procedure
• Systemic heart failure1,4,5,6
• Arrhythmias 1,2,3
• Venous return stasis 1
• Pulmonary edema 1
• Sudden death7
• From studies done at US and New Zealand heart
  centers, the overall survival rate at 10 years is
  807,8

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Transposition of the Great Arteries

• What happens to the pregnant pt who is status
  post a Mustard procedure in infancy?
• Numerous studies have shown that these patients
  have good outcomes, overall. But they require
  intensive and specialized follow up.1-6
• They should be closely followed throughout
  pregnancy by both their cardiologist and
  obstetrician.
• A number of these patients will develop clinical
  signs of right heart failure, but of these most
  will fully recover. 1,4,5,6
• There is a higher incidence of pre-eclampsia
  according to a study out of Germany 2

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Transposition of the Great Arteries

• What happens to the pregnant pt who is status
  post a Mustard procedure in infancy?
• They are prone to SVT 1,2,3
• All of these patients should undergo close
  hemodynamic monitoring 1,4
• From all the studies reviewed, no maternal deaths
  occurred

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Transposition of the Great Arteries

• All pictures courtesy of University of Michigan
  Congenital Heart Center website -
  http//www-umchc.pdc.med.umich.edu
• Rousseil MP, et al. Successful term pregnancy
  after Mustard operation for transposition of the
  great arteries. Eur J Obstet Gynecol Reprod Biol.
  1995 Mar59(1)111-3
• Reinecke H, et al. Pregnancy in patients with
  transposition of great vessels corrected by the
  Mustard procedure. Report of a case and review of
  reported cases. Z Kardiol. 1997 Nov86(11)945-56
• Megerian G, et al. Pregnancy outcome following
  Mustard procedure fro transposition of the great
  arteries a report of five cases and review of
  the literature. Obstet Gynecol. 1994
  Apr83(4)512-6
• Lynch-Salomon DI, et al. Pregnancy after Mustard
  repair for transposition of the great arteries.
  Obstet Gynecol. 1993 Oct82(4 Pt 2 Suppl)676-9
• Connolly HM, et al. Pregancy among women with
  congenitally corrected transposition of great
  arteries. J Am Coll Cardiol. 1999
  May33(6)1692-5
• Therrien J, et al. Outcome of pregnancy in
  patients with congenitally corrected
  transposition of the great arteries. Am J
  Cardiol. 1999 Oct 184(7)820-4
• Wilson NJ, et al. Long-term outcome after the
  Mustard repair for simple transposition of the
  great arteries. 28-year follow-up. J Am Coll
  Cardiol. 1998 Sep32(3) 758-65
• Wells WJ, et al. Intermediate outcome after
  Mustard and Senning procedurs A study by
  Congenital Heart Surgeons Society. Semin Thorac
  Cardivasc Surg Pediatr Card Surg Annu.
  20003186-197