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The prospect of undergoing heart surgery, even in an adult, is a frightening
prospect, but most surgical procedures for congenital heart defects have a low
risk of death or serious complications (less than 3 percent). Most pediatric
heart patients require short hospitalization and quickly return to normal
activities.
Surgery is required as a last measure when other forms of therapy either
cannot maintain adequate circulation for development or quality of life, or the
structural defect requires correction to prevent further damage to the heart or
other organs. Generally, corrective surgery for congenital heart defects is
performed during childhood, but the procedure also may occasionally be used to
treat adults or older patients.
Open heart corrective surgical procedures
In
general, there are two categories of corrective surgical procedures for
congenital heart defects -- open heart procedures and closed procedures. Both
techniques may be correctable. Both are palliative, meaning the heart is either
brought to a stable condition or is prepared to undergo a further reparative or
definitive surgical procedure.
As a principle, open heart surgery requires the use of a heart/lung machine
or cardiopulmonary bypass, which takes over the functions of the heart and
lungs, allowing the surgeon to operate on the heart while the machine sustains
circulation. Use of the heart/lung machine enables surgeons to work inside the
heart with relative impunity in correcting defects within the atrioventricular
septum and create or reconstruct obstructive or restrictive pathways.
Development of small-body perfusion techniques and highly specific management
strategies designed to meet pediatric patient requirements (very small patients
less than 1.5 kg up to patients in excess of 90 kg) have minimized the hazards
associated with pediatric heart surgery.
Challenges for open heart procedures
Open
heart surgical techniques may be relatively straightforward or extremely
complex. The degree of complexity usually reflects the degree of risk involved,
and is further heightened by risks and conditions associated with newborn
infants. Included is the inherent risk of operating on a patient with immature
organ systems, as well as associated congenital abnormalities that accompany
congenital heart disease.
It should be stressed at the outset that surgical correction of congenital
heart defects is a team effort. This involves a multidisciplinary approach with
a highly specialized and dedicated group of anesthesiologists, pediatricians,
nursing ancillary staff, pediatric cardiologists and pediatric subspecialty
consultation. The surgical skill required for pediatric open heart surgery is
very different from that for an older or larger patient population, requiring a
surgical service that is dedicated to performing surgery in very small patients.
Types of procedures
These procedures
are performed under general anesthesia. Some require the use of a heart-lung
machine, which cools the body to reduce the need for oxygen and takes over for
the heart and lungs during the procedure.
Arterial switch
Arterial switch is performed to correct transposition of the great arteries,
where the position of the pulmonary artery and the aorta are reversed. The
procedure involves connecting the aorta to the left ventricle and the pulmonary
to the right ventricle.
Fontan procedure
For tricuspid atresia and pulmonary atresia, the Fontan procedure connects
the right atrium to the pulmonary artery directly or with a conduit, and the
atrial defect is closed. Survival is more than 90 percent.
Pulmonary artery banding
Pulmonary artery banding creates a narrowing in the pulmonary artery with a
band to reduce blood flow and pressure to the lungs. It is used for ventricular
septal defect, atrioventricular canal defect and tricuspid atresia. Later, the
band can be removed and the defect corrected with open heart surgery.
Ross procedure
The Ross procedure is performed to correct aortic stenosis (narrowing). The
Ross procedure grafts the pulmonary artery to the aorta.
Shunt procedure
For Tetralogy of Fallot, or tricuspid or pulmonary atresia (absence of
normal openings), the shunt procedure creates a passage between blood vessels,
sending blood into parts of the body that need it.
Transplantation
Cardiac transplantation is a last-stage procedure when all other forms of
treatment have been unsuccessful. The Cleveland Clinic is home to the largest
cardiac transplant center in Ohio and enjoys a 1-year survival rate of more than
90 percent.
Recovery
Children tend to recover
quickly, with an average hospital stay of 5 to 7 days and a general return to
normal activities 48 hours after surgery. Once discharged, return to daily
activity is usually quite rapid with full recovery in 4 to 6 weeks. However,
strenuous activity should be avoided for at least 6 weeks following the
procedure to allow the sternal wound to heal and to allow time for the heart to
adjust to the new repair.
Follow up care
Pediatric corrective
heart surgery requires long-term follow-up care by a pediatric cardiologists as
well as routine follow up by a family practitioners, especially with children
who have undergone repair at a very early age.
Patients who are diagnosed with congenital heart disease at an older age --
know as "grown-ups with congenital heart disease or GUCH" -- require
specialized, long-term follow up care by a pediatric cardiologist, as do
children who underwent congenital heart surgery 15 to 20 years ago and are now
adults. Their problems are unique and are not those generally encountered in
standard cardiology practice. As such, pediatric cardiology patients represent a
very special challenge. Developments continue to expand the possibilities and
increase the safety margins associated with correction of these defects.
Success rates
While children tend to
do very well after open heart surgery, the risk of untoward complications is
relative to the magnitude of the operative procedure. This, in turn, relates to
the complexity of the underlying congenital heart disease.
The Cleveland Clinic has enjoyed a more than 97 percent success rate in
performing pediatric corrective heart surgery, compared to current regional
figures. This is exceptionally high success rate must be viewed in context with
the high level of complexity among cases referred to The Cleveland Clinic.
Summary:
Surgical correction of congenital heart disease is only performed under
very specific circumstances.
Surgery can be undertaken with a low risk of death or serious
complications (less than 3 percent).
Ongoing follow-up by a team who is specialized in care of these patients
is required over the long term.
Older patients who have undergone repair should continue to be followed by
this specialized team.
"Grown-ups with congenital heart disease" constitute a new
subgroup of patients that also should be followed and tracked by a
specialized team of pediatric cardiology and surgical physicians.
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