Circulation. 1999;100:II-162-II-166
(Circulation. 1999;100:II-162.)
© 1999 American Heart Association, Inc.
Surgery for Congenital Heart Disease |
Early Results of the Ross Procedure in Simple and Complex Left Heart Disease
Bradley S. Marino, MD, MPP;
Gil Wernovsky, MD;
Jack Rychik, MD;
John R. Bockoven, MD;
Rodolfo I. Godinez, MD, PhD;
Thomas L. Spray, MD
From the Divisions of Cardiology, Cardiothoracic Surgery, and Critical
Care Medicine, Children’s Hospital of Philadelphia, and the Departments
of Pediatrics, Surgery, and Anesthesiology, University of Pennsylvania School
of Medicine, Philadelphia, Pa, and Dayton Children’s Cardiology,
Children’s Medical Center, Wright State University School of Medicine,
Dayton, Ohio (J.R.B.).
Correspondence to Thomas L. Spray, MD, The Cardiac Center at the Children’s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104.
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Abstract
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Background—The Ross procedure
has been used increasingly
to treat aortic valve disease in children
and young adults.
Benefits include the lack of anticoagulation after
surgery and
the potential growth and durability of the autograft. The
purpose
of this study was to review our institutional experience with
the
Ross procedure and to compare early outcome in simple aortic
valve
disease and complex left heart disease.
Methods and Results—Between January 1995 and October 1998, 66
patients (median age, 10.8 years; range, 6 days to 34.8 years)
underwent the Ross procedure. The primary indication for surgery was
isolated valvular disease in 41 patients: aortic
stenosis (AS; n=3), aortic insufficiency (AI; n=11), and AS/AI
(n=27). The remaining 25 patients had multiple levels of left
ventricular outflow tract obstruction, 12 of whom had at
least moderate AI. Additional left heart disease in the complex group
included subaortic stenosis (n=20), arch obstruction (n=7),
mitral valve disease (n=5), apical aortic conduit stenosis or
insufficiency (n=3), and supravalvar AS (n=2). There were 123 prior
interventions performed in 51 patients, including aortic
valvotomy/valvuloplasty (n=56), coarctation repair (n=21), subaortic
stenosis resection/Konno procedure (n=10),
ventricular septal defect closure (n=8), apical aortic
conduit placement (n=3), aortic valve replacement (n=3), and other
(n=22). An isolated Ross procedure was performed in 41 patients, 10 of
whom required concurrent aortic annulus enlargement procedure to
accommodate the larger pulmonary autograft. In the remaining 25
patients, 49 concurrent procedures were performed, including the Konno
procedure (n=17), aortic annulus enlargement (n=2), subaortic membrane
resection (n=9), arch augmentation (n=5), mitral valvuloplasty (n=5),
ventricular septal defect closure (n=4), apicoaortic
conduit division (n=3), and other (n=4). One patient (1.5%) died 3
days after a Ross-Konno procedure, which included arch reconstruction,
from presumed arrhythmia. There were no other early deaths. One
patient required ECMO (extracorporeal membrane
oxygenation) for 3 days after a
ventricular tachycardia (VT)–related cardiac
arrest. Transient complete heart block was seen in 4 patients; the
duration was <5 days. No patient had left ventricular
outflow tract obstruction on discharge
echocardiography. Neo-AI was graded as none (n=5),
trivial-mild (n=57), or moderate (n=3). All 3 patients with moderate
neo-AI at discharge had abnormal pulmonary valves before
surgery. Perioperative VT was noted in 18 patients
(27.2%), 2 of whom were discharged on antiarrhythmic medication.
Conclusions—The Ross procedure can be performed in isolation or
in combination with other complex procedures with low mortality (1.5%)
and acceptable short-term results, even in patients with complex left
heart disease and multiple prior interventions. Postoperative VT is
common. Anatomic abnormalities of the pulmonary valve preclude
its use as an autograft.
Key Words: aorta • valves • heart defects, congenital • pediatrics
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Introduction
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The Ross procedure, first described in
1967,
1 involves replacement
of the diseased aortic valve
with a pulmonary autograft and
placement of a pulmonary
or aortic homograft between the right
ventricle and the main
pulmonary artery. After the Ross procedure,
patients do not
require anticoagulation, and the autograft has
been shown to be durable
and to grow in proportion to somatic
growth.
2 3 Because of
these attributes, the pulmonary autograft
is an attractive
alternative to mechanical, porcine, and homograft
valves in the
treatment of aortic valve disease in children
and young adults.
Although mechanical valves provide a satisfactory
hemodynamic
result, they frequently require replacement
during somatic growth
and lifelong anticoagulation. Thromboembolism and
hemorrhage
remain important complications, especially in the
pediatric
population.
4 Porcine bioprostheses, which do not
require anticoagulation,
deteriorate rapidly in young patients, and
have limited durability.
5 Although aortic valve homograft
placement generally results
in excellent postoperative
hemodynamics, does not require anticoagulation,
and is
associated with a low incidence of thromboembolic phenomena,
these
homografts also have limited durability in the pediatric
population and
do not grow with the child.
6 7 8
Several reports have documented the effective use of the Ross procedure
for isolated aortic valve disease in children.9 10 11 12 Recent
reports have extended the pulmonary autograft to children with
complex left ventricular outflow tract
obstruction13 14 15 16 and to neonates and
infants.13 17
The purpose of this study was to review our institutional experience
with the Ross procedure and to compare the early outcome in simple
aortic valve disease to complex left heart disease.
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Methods
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Study Design
This study is a case series focusing on early surgical results.
All
patients at the Children’s Hospital of Philadelphia who
underwent
the Ross procedure in isolation or in combination
with other
interventions between January 1995 and October 1998
were included in
this investigation. Charts were reviewed for
age at surgery, sex,
original anatomic diagnoses, and prior
interventions. Prospective data
were obtained during the hospital
stay. Operative notes were reviewed
for additional surgical
procedures and the duration of
cardiopulmonary bypass, myocardial
ischemia, and
circulatory arrest. The perioperative course was
reviewed
for conduction abnormalities and arrhythmias, duration
of ventilation,
neurological sequelae, and other postoperative issues.
Early
morbidity was defined as events occurring <30 days from
operation.
Echocardiography at discharge was
reviewed to assess for neo-aortic
insufficiency, pulmonic
insufficiency, pericardial effusion,
and the presence of left or right
ventricular outflow tract
obstruction.
Patient Population
There were 66 patients, 45 male and 21 female patients, included
in the study. The median age of the group was 10.8 years at the time of
surgery (range, 6 days to 34.5 years). The frequency distribution of
age at Ross procedure shown in Figure 1
reveals that most patients underwent surgery between 5 and 18 years of
age.
At the time of the pulmonary autograft, patients were
stratified into simple and complex groups. Patients were defined as
having simple aortic valve disease if they had aortic stenosis,
insufficiency, or both with no other structural abnormalities
present. Patients were defined as having complex left heart disease
if they had aortic stenosis, insufficiency, or both and
multiple levels of obstruction or additional
hemodynamic abnormalities that required surgical
intervention.
Simple Aortic Valve Disease
There were 41 patients identified with simple aortic valve
disease. The median age for the group was 14.6 years (range, 1.3 to
26.5 years). In this group, isolated aortic stenosis or
insufficiency was the predominant initial diagnosis. For 10 of these
patients, the pulmonary autograft was their first intervention.
Prior procedures (n=46) were performed in 31 of the 41 patients (Table 1).
Complex Left Heart Disease
There were 25 patients with complex left heart disease. The
median age for this group was 6.5 years (range, 6 days to 34.8 years).
For 5 of the patients in this group, the pulmonary autograft
was their first intervention. Seventy-seven prior procedures were
performed in the other 20 patients with complex left heart disease
(Table 2).
Surgical Indications
Most patients had a combination of aortic stenosis and
aortic insufficiency at the time of the pulmonary autograft
(Figure 2). At least moderate aortic
insufficiency was present in 53 of 66 patients (80%) at the time
of surgery. Additional left heart disease in the complex group is
described in Table 3.
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Figure 2. Hemodynamic indications for
surgery (n=66) comparing type of aortic valve disease within and
between simple and complex groups. Values are expressed as percentage
of total patients in groups with aortic stenosis (AS), aortic
insufficiency (AI), or both (AS/AI).
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Surgical Technique
The Ross procedure, as previously described,18 was
performed in 41 patients with isolated aortic valve disease, 10 of whom
required a concurrent aortic annulus enlargement procedure to
accommodate the size of the larger pulmonary autograft.
Twenty-five patients with complex left heart disease underwent the Ross
procedure and 49 concurrent procedures (Table 4). In all 3 patients with apical-aortic
conduits, a median sternotomy and left thoracotomy were used for
takedown of the apical aortic conduit. Valved pulmonary
homograft was used in all patients to reconstruct the right
ventricular outflow tract.
Statistical Analysis
Summary statistics are expressed as medians and ranges.
Statistical differences were assessed by the Wilcoxon rank-sum
test.
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Results
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Mortality
There was 1 death in the series (1.5%), in a 61/2-year-old
male
patient born with aortic stenosis and arch obstruction who
had
had 2 previous repairs of aortic coarctation and severe residual
severe
arch obstruction, aortic stenosis, and aortic
insufficiency.
The child also had left ventricular
hypertrophy with a posterior
wall thickness of 2 cm and
marked coronary dilation (1 cm) at
the time of surgery. Despite
an apparently successful Ross-Konno
procedure with arch reconstruction,
the child had a sudden cardiac
arrest on postoperative day 2 and could
not be resuscitated.
Morbidity
In the simple group, 1 patient had a neo-aortic valve
commissuroplasty, 1 had transient complete heart block, and 1 had a
transient brachial plexus injury. Three patients were readmitted after
discharge for pericardial effusion, 2 of whom required
pericardiocentesis.
Perioperative morbidity was more frequent in the
complex group. Three patients had reoperation for bleeding; 3 had
transient complete heart block, none of whom required permanent
pacemaker placement; and 2 had delayed sternal closure. One patient had
a hyperkalemic cardiac arrest on postoperative day 2 and was
successfully resuscitated with extracorporeal membrane
oxygenation; the child sustained a cerebrovascular
accident and has residual neurological deficits. One patient with
interrupted aortic arch and aortic valvar and subvalvar
stenosis who had surgical repair at an outside institution
underwent stent placement in the right pulmonary artery because
of compression of the right pulmonary artery by the ascending
aorta. One patient with complex left heart disease was readmitted for
pericardial effusion and had a pericardiocentesis performed.
Conduction abnormalities and arrhythmias for both simple and
complex groups are summarized in Table 5.
Eighteen patients had ventricular tachycardia
in the postoperative period. Of the 18 patients, 16 had nonsustained
ventricular tachycardia limited to the first 24
hours. Two patients had nonsustained ventricular
tachycardia after postoperative day 1 and were discharged
on antiarrhythmic medication. The perioperative
conduction and rhythm disturbances have previously been
summarized.19
The duration of mechanical ventilation, cardiac intensive care unit
length of stay, and total hospital length of stay were significantly
less in the simple group (Table 6).
Notably, the median period to extubation in the complex group was 17
hours, and the median total hospital length of stay was 6 days.
Perfusion Data
The patients in this study had the entire operation performed,
including the right ventricle to pulmonary artery homograft
reconstruction, during a single period of aortic cross clamping. The
median cross-clamp and cardiopulmonary bypass times were
significantly shorter in the simple compared with the complex group.
Circulatory arrest was used during arch reconstruction in 7 patients,
with a median circulatory arrest time of 19 minutes (range, 10 to 32
minutes; Table 7). Of the 7 patients who
had circulatory arrest, 5 had arch augmentation, whereas 2 neonates
required circulatory arrest to permit reconstruction of the aortic
arch.
Discharge Echocardiography
All patients underwent an echocardiogram before discharge. Greater
than trivial-mild neo-aortic insufficiency was found in 3 of 65
patients at discharge. The grade distribution of neo-aortic
insufficiency at discharge is shown in Table 8. The 3 patients who had moderate
neo-aortic insufficiency at discharge all had structurally abnormal
pulmonary valves at the time of the Ross procedure. One patient
had supravalvar stenosis with 2 large pulmonic valve leaflets
and 1 hypoplastic leaflet; 1 patient had previous pulmonary
artery band placement, which resulted in pulmonary valve
distortion; and 1 patient was noted to have a gap between 2 of the
pulmonary valve commissures. No patient had residual left
ventricular outflow tract or arch obstruction at discharge.
Greater than trivial-mild neo-pulmonic insufficiency was found in 2 of
65 patients at discharge. The grade distribution of neo-pulmonic
insufficiency at discharge is shown in Table 9. In addition, 1 patient had a pressure
gradient of 20 mm Hg across the right ventricular
outflow tract at discharge.
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Discussion
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This study found that the Ross procedure can be performed with
a
low mortality risk, 1.5%, in both simple and complex left
heart
disease. Compared with the simple group, the duration
of mechanical
ventilation and hospital length of stay was longer
in patients with
complex left heart disease. Neo-aortic insufficiency
greater than
trivial-mild was uncommon at discharge.
The mortality rate in this series (1.5%) is equivalent to that in
prior reports.9 10 12 13 14 15 20 21 Moreover, the mortality
rate found in this series is the same as or less than those found for
other series using mechanical aortic valve replacement4 or
aortic homograft placement7 8 22 for aortic valve disease
in children.
The most common postoperative morbidity found in this series was
arrhythmias.19 In general, children who are
candidates for the Ross procedure have a myocardium "at
risk" because of long-standing pressure and or volume overload and
the intraoperative ischemia that results from surgical repair.
These factors lead to ventricular dysfunction and ectopy
(nonsustained ventricular tachycardia and
nonsustained supraventricular tachycardia) in
the early postoperative period. Reddy et al,13 who
reported extending the Ross procedure to children with complex left
heart disease, found isolated cases of transient atrial and
ventricular dysrhythmias. Elkins et al2
reported 1 early death secondary to a fatal arrhythmia.
Similarly, in a 20-year follow-up of a pulmonary autograft
cohort by Matsuki and colleagues,23 there was 1 early and
1 late death attributed to arrhythmia. The higher incidence of
identified perioperative ventricular
tachycardia in this study may result from our methodology,
incorporating 24-hour full-disclosure telemetry, but is also more
likely to be attributable to the complexity of this cohort of patients.
Most patients had multiple prior procedures, and many had concurrent
additional surgical procedures performed at the time of autograft
placement.
The incidence of complete heart block after the Ross procedure
varies from 0% to 6%.11 22 23 24 In the present
series, permanent, complete heart block did not occur, despite the fact
that 19 of 25 patients (76%) within the complex group had an
annulus-enlarging procedure. Although there was transient heart block
in 4 patients within this cohort, all were discharged in normal sinus
rhythm. Possible causes of complete heart block in this population
include damage to the septal perforating coronary artery during
autograft harvest, suture placements during anastomosis of the proximal
end of the autograft, resection of subaortic stenosis, and the
Konno procedure for aortic annulus and left ventricular
outflow tract enlargement.
Reoperation for mediastinal bleeding was required in 4.5% of our study
group. All 3 patients who required reoperation had complex left heart
disease. Reoperation for mediastinal bleeding was required in 8.5% of
a cohort with complex left heart obstruction followed up by Reddy et
al,13 in 4.0% of a cohort followed up by Elkins et
al,10 and in 1.2% of patients in a group followed up by
Matsuki et al.23
Both Starnes et al15 and Reddy et al13 have
reported results for the Ross procedure in patients with complex left
heart disease with durations of mechanical ventilation and hospital
length of stay similar to those of the present series. For the
complex group within our cohort, the median duration of mechanical
ventilation was 17 hours, median length of stay in the intensive care
unit was 3 days, and median total hospital length of stay was 6
days.
Similar to other pulmonary autograft10 11 13 15
and aortic homograft22 series, discharge
echocardiography revealed moderate neo-aortic
insufficiency in very few (3 of 65) patients in our series, all of whom
had abnormal pulmonary valves before the Ross procedure.
Long-term issues include autograft durability, autograft growth,
homograft durability, and the long-term significance of
perioperative arrhythmias. It is unclear
whether use of the Ross procedure at an earlier age alters the natural
history of simple or complex left ventricular outflow tract
disease. It is also not known if the risk of late reoperation on the
neo-aortic valve is higher if performed in the neonate, infant, or
child because of aortic root sinus dilation and valve distortion over
time. Similarly, given the lack of growth of the cryopreserved
homograft, late reintervention is likely with interval replacement of
the homograft. Long-term changes in left ventricular
mechanics await further study.
This study is limited in that it focuses solely on early surgical
results. Long-term follow-up is underway to determine whether there is
a significant difference in late outcome between simple and complex
left heart disease groups and between neonates/infants and
children/adolescents.
In conclusion, the Ross procedure can be used for simple aortic valve
disease and complex left heart disease with low morbidity and mortality
in children and young adults. Most patients had a short period of
mechanical ventilation, cardiac intensive care unit length of stay, and
total hospital length of stay. Significant neo-aortic insufficiency is
uncommon in the short term.
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Acknowledgments
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This work was supported in part by the Daniel M. Tabas endowed
chair
in Cardiothoracic Surgery. We acknowledge the work of William
M.
DeCampli, MD, PhD, who operated on 1 patient in this series.
We would
like to thank the medical, nursing, respiratory, and
support staffs of
the Cardiac Center at the Children’s
Hospital of Philadelphia for
their help in the care of these
patients and with data collection;
David Bush, MD, PhD, for
his help with the statistical
analysis; and Amy Oskinson, RN,
and Maritza Lozada for
assistance with data acquisition and
manuscript preparation.
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Abstract
Introduction