(Circulation. 1995;92:262-266.)
© 1995 American Heart Association, Inc.
Articles |
From the Departments of Cardiovascular Surgery (J.M.F., N.C., A.S., J.E.M., R.A.J.) and Cardiology (S.J.R.), Children's Hospital of Boston, and the Departments of Surgery and Pediatrics, Harvard Medical School, Boston, Mass.
Correspondence to Richard A. Jonas, MD, Department of Cardiovascular Surgery, Children's Hospital, 300 Longwood Ave, Boston, MA 02115.
| Abstract |
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Methods and Results Six surgeons participated in the care of these patients. Follow-up is 97% complete. Preoperative anatomic and physiological factors and procedural features of the stage I operation were analyzed for impact on stage I mortality, survival to stage II palliation, and actuarial survival. Hospital mortality was not significantly lower during the second half of the study period (P=.242). Operative mortality was 46.2%. Multivariate analysis revealed improved stage I operative survival in patients with mitral stenosis (MS) and aortic stenosis (AS; P=.006). Additional risk factors for stage I mortality were a lower immediately prestage I pH (P=.034) and weight <3 kg (P=.015). Overall first-year actuarial survival for MS/AS was 59%, and it was 33% for all others (P=.001). Among stage I survivors, patients with MS/AS were more likely to survive to stage II palliation (P=.031). Analysis of actuarial survival of stage I survivors showed that a smaller ascending aorta (P<.001), aortic atresia (P<.001), and mitral atresia (P=.002) were all risk factors for intermediate death.
Conclusions Preoperative anatomic and physiological state are predictors of stage I mortality. HLHS anatomic subtype also influences intermediate outcome, most notably prestage II attrition. These data may be useful in choosing initial management for patients with HLHS.
Key Words: surgery mortality hypoplastic left heart syndrome survival
| Introduction |
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| Methods |
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Operative survival was defined as survival of >30 days and the ability
to leave the hospital. Follow-up information was obtained from 111
(97%) of the 114 survivors. Follow-up was obtained directly from
the patient's family or primary cardiologist during a 1-month period
ending April 1, 1994. Time to follow-up (or late death) ranged from
0.1 to 9.7 years, with a median of 1.7 years. This constituted a total
of 267 patient-years. Intermediate results were evaluated by the
incidence of death, survival to second-stage palliation, and
actuarial survival estimates. Potential risk factors for early
mortality were analyzed in contingency tables with
2 or Fisher's exact tests.
Multivariate analyses of operative mortality,
mortality before stage II palliation, and overall intermediate
mortality among stage I survivors were performed using stepwise
logistic regression and Cox regression models. The relation of discrete
variables to survival among stage I survivors was examined with the
log-rank test. Survivorship estimates were made by the Kaplan-Meier
method. All analyses used a standard, commercially available
software package (SAS Institute, Inc).
| Anatomic Findings |
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Fig 2
displays the distribution of ascending aorta
diameters in the study population. The ascending aorta diameters in 116
patients (57%) were between 2 and 4 mm; in 18 patients (8.8%), <2
mm; and in 71 (35%), >4 mm. Ascending aorta diameter tended to be
greater in those patients with AS than in those with AA (Fig
3
).
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The presence of a discrete coarctation of the aorta was noted preoperatively in 103 patients (49%). Prestage I right ventricular function was identified as normally preserved in 127 patients (60%), mildly depressed in 40 (19%), moderately depressed in 31 (15%), and severely depressed in 11 (5%). Tricuspid regurgitation was noted to be moderate in 14 patients (7%) and severe in 2 (1%). Forty-six (22%) were noted to have restrictive atrial septal defects.
Operative Features
Various techniques were used in the
first-stage
reconstructions performed during the study period. All but 2 stage I
procedures were performed with cardiopulmonary bypass and
hypothermic circulatory arrest. The mean duration of circulatory arrest
was 62 minutes (SD, 9.9 minutes). Sixteen patients required a second
period of circulatory arrest averaging 24 minutes (SD, 5.9 minutes).
Pulmonary arterial to aortic continuity was
established in several ways. To facilitate statistical
analyses, these were classified as type 1: direct anastomosis
of the divided proximal pulmonary artery to the side of the
ascending aorta with augmentation of the neoaortic arch with homograft,
autologous pericardium, or synthetic gusset (n=105); type 2:
anastomosis of the proximal main pulmonary artery to the
underside of the aortic arch in an end-to-side fashion directly
or with a homograft, synthetic, or autologous pericardium interposition
tube graft (n=92); and type 3: other forms of palliative reconstruction
(n=15). The types of operation with materials used for neoaortic
reconstruction are shown in Table 1
. A variety of
systemic-to-pulmonary shunts were also used during the
study period. One hundred eighteen patients (56%) received 4-mm
RMBTSs, 74 (35%) had 3.5-mm RMBTSs, and 11 (5.2%) had central shunts
placed. The current reconstruction of choice at our institution is a
type 1 procedure with a 3.5-mm RMBTS. An additional technique applied
to this population is that of delayed sternal closure. The chest was
left open in 72 patients (34%), and 51 underwent delayed sternal
closure at a mean of 3.8 days (SD, 2.4 days).
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| Results |
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There were 98 operative deaths (46.2%). Fig 4
shows
hospital mortality by anatomic subtype. The results of
univariate analysis of possible risk factors for
stage I hospital mortality are shown in Table 3
.
Patients with ascending aorta diameter <2 mm were at increased risk
for operative mortality (P=.002). These patients were more
likely to receive a type 2 stage I procedure. Only 7 of the 19 patients
(37%) with ascending aorta diameters <2 mm underwent type 1
operations, whereas 95 of the remaining 186 patients (51%) underwent a
type 1 operation.
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Patients weighing <3 kg were at higher risk for hospital death (P=.024), whereas patients with an immediately preoperative pH >7.5 were more likely to survive (P=.017). There was a trend toward improved survival in those patients with MS/AS by univariate analysis (P=.06). Preoperative right ventricular or tricuspid valve function, type of stage I procedure, type of systemic-to-pulmonary shunt, operative surgeon, or year of operation did not influence operative mortality (P>.05). Independent risk factors for stage I mortality identified in multivariate analysis were weight <3 kg (P=.015) and lower immediately preoperative pH (P=.0361). MS/AS patients were at dramatically lower risk for stage I death (P=.006).
Survival to stage II palliation was also analyzed as an end
point. Table 4
summarizes the findings of
univariate analysis. Patients who underwent stage I
later in the study period (P<.001) and those with MS/AS
(P=.001) were much more likely to survive to stage II
surgery. Those who had ascending aorta diameter <2.5 mm
(P=.018) and those in whom synthetic tube grafts were used
in neoaortic reconstruction (P=.03) were less likely to
survive to a second-stage operation. Patients receiving a 3.5-mm
RMBTS (P=.025) and those undergoing delayed sternal closure
(P=.024) were more likely to survive to a stage II
procedure. There was a trend toward increased survival to stage II in
patients who underwent a type 1 reconstruction (direct
side-to-side anastomosis of main pulmonary artery to
ascending aorta with aortic arch augmentation, P=.059).
Multivariate analysis also revealed that MS/AS
patients were less likely to die (P=.031) and patients with
synthetic tube graft neoaortic reconstruction were more likely to die
(P=.033) before stage II.
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Actuarial survival of all patients by anatomic subtype is shown in Fig
5
. Overall 1-year actuarial survival was 59% in MS/AS
patients and 33% for all others (P=.001). Actuarial
survival among stage I survivors, similarly classified according to
anatomic subtype, is shown in Fig 6
. Again, MS/AS
patients showed increased intermediate survival (P=.001), as
did those with ascending aorta diameters >4.5 mm (P=.005).
In contrast, patients with either mitral atresia (P=.002) or
aortic atresia (P=.001) had lower survival. Patients who
underwent a type 1 stage I reconstruction had improved actuarial
survival (P=.004) compared with those who underwent other
types of stage I reconstruction. Multivariate
analysis with the proportional-hazards model showed that
aortic atresia was the only independent risk factor for death among
operative survivors (P<.001).
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| Discussion |
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A lower immediately preoperative pH, perhaps reflecting the inadequacy of resuscitative efforts, was also a predictor of higher stage I mortality. This confirms the observation previously made at this institution11 and suggests a beneficial role for prenatal diagnosis, by which acidosis may be largely avoided.
The intermediate results of the MS/AS patients in this series are comparable to those obtained with transplantation when one takes into account the mortality associated with the pretransplant waiting period. The most prolific HLHS transplant center has reported an overall 5-year actuarial survival of 61%.7 However, the early mortality for the majority of the patients in the study population, with atresia of one or both of the left heart valves, did not decrease significantly during the study period. This finding contrasts sharply with the results of reparative neonatal cardiac surgery at our institution.14 There was, however, an encouraging increase in survival to stage II palliation among all patients later in the study period. Of all stage I survivors in the past 5 years, 85% have undergone a second-stage palliative procedure. The vast majority of these second-stage procedures are a bidirectional cavopulmonary connection. The second-stage bidirectional cavopulmonary connection has improved survival at completion of the Fontan procedure at this institution and is the subject of a subsequent report (unpublished data).
We conclude from these data that HLHS patients with MS/AS may undergo a course of reconstructive palliative surgery with overall results similar to those currently offered by cardiac transplantation.7 Patients with other anatomic subtypes face significant early mortality but are now more likely to survive to subsequent palliative procedures. Given the short supply of organ donors, the most appropriate HLHS patients for transplantation are those with either mitral or aortic valve atresia, particularly those who weigh <3 kg and/or have an ascending aorta diameter of <2 mm. Prospective trials are necessary to confirm such speculation.
| Selected Abbreviations and Acronyms |
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| References |
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4. Norwood WI. Hypoplastic left heart syndrome. Ann Thorac Surg. 1991;52:688-695. [Abstract]
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