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136 ª 2023 by The Society of Thoracic Surgeons 0003-4975/$36.

00
Published by Elsevier Inc. https://doi.org/10.1016/j.athoracsur.2022.08.018

Congenital & Pediatric: Research

Incidence of Reoperation After Surgical


Procedure for Left Ventricular Outflow Tract
Obstruction in Children and Young Adults
Benish Fatima, MD, Hartzell V. Schaff, MD, Elizabeth H. Stephens, MD, PhD,
Katherine S. King, MS, Frank Cetta, MD, and Joseph A. Dearani, MD

Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota; Division of Clinical Trials and Biostatistics,
Department of Quantitative Health Sciences, Mayo Clinic, Rochester, Minnesota; and Division of Pediatric Cardiology,
Mayo Clinic, Rochester, Minnesota

ABSTRACT

BACKGROUND The common causes of subaortic left ventricular outflow tract obstruction (LVOTO) are hypertrophic
cardiomyopathy (HCM) and membranous/tunnel subaortic stenosis (SAS). Reoperation after corrective surgery may be
due to recurrent disease, associated congenital defects, or complications of the initial procedure. This study compares
the late outcomes of young patients with HCM and SAS.

METHODS We studied clinical, echocardiographic, and operative data of patients £21 years of age at the time of
surgery for LVOTO between August 1963 and August 2018. We stratified patients into HCM (n [ 152) and congenital
SAS (n [ 63) groups and compared survival and cumulative incidence of reoperation.

RESULTS At initial repair, patients with HCM were older than patients with SAS (median [interquartile range] age, 15
[10-19] years vs 8 [5-13] years; P < .001), and patients with HCM were more symptomatic with dyspnea (P < .001), chest
pain (P [ .002), and presyncope/syncope (P [ .005). Thirty-day mortality was 1.3% vs 0% for HCM and SAS groups.
During a median follow-up of 13.1 years, survival was similar through the first 10 years; but during the second decade,
patients with HCM had poorer survival (survival at 20 years, 80% vs 91% for patients with SAS; P [ .007). Ten years after
repair, reoperation for recurrent LVOTO was performed in 5% of patients with HCM vs 31% in those with SAS (P < .001).

CONCLUSIONS In this surgical cohort, patients with HCM were more symptomatic preoperatively than those with SAS.
Late survival of patients with SAS was superior to that of patients with HCM despite a greater need for reoperation.
(Ann Thorac Surg 2023;115:136-43)
ª 2023 by The Society of Thoracic Surgeons

H ypertrophic cardiomyopathy (HCM)


congenital subaortic stenosis (SAS) are 2 com-
mon causes of left ventricular outflow tract
obstruction (LVOTO) in young patients. During repair
and have risks of recurrence, but mechanisms appear to be
different.2 In HCM, common mechanisms for recurrence
include limited myectomy at the initial operation,
unrecognized or late-developing midventricular
of these conditions in children, the limited surgical field obstruction, anomalies of papillary muscles, and ven-
may increase the risk of injury to the aortic and mitral tricular remodeling.3 In patients with SAS, risk factors
valves as well as the risk of creating a ventricular septal for recurrent obstruction include female sex, younger
defect. Furthermore, correction of LVOTO in childhood age at the time of surgery, and failure to perform
may have the late complication of recurrent obstruction
due to incomplete resection during initial surgical pro-
The Supplemental Tables can be viewed in the online version of this
cedure or tissue regrowth.1
article [10.1016/j.athoracsur.2022.08.018] on http://www.
There are conflicting data on outcomes of operations annalsthoracicsurgery.org.
for HCM and SAS in young patients; both conditions

Accepted for publication Aug 8, 2022.


Presented as an ePoster at the Fifty-seventh Annual Meeting of The Society of Thoracic Surgeons, Virtual Meeting, Jan 29-31, 2021.
Address correspondence to Dr Schaff, Department of Cardiovascular Surgery, Mayo Clinic, 200 First St SW, Rochester, MN 55905; email: schaff@mayo.edu.

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Ann Thorac Surg FATIMA ET AL 137
2023;115:136-43 OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION

septal myectomy in conjunction with membrane SURGICAL DATA. For patients with obstructive HCM,
resection.4,5 The purpose of this study was to compare transaortic septal myectomy was performed as previ-
the reoperation rates and survival after correction of ously described.7 Transaortic membranectomy was
LVOTO in patients 21 years of age with HCM and SAS. supplemented by shallow septal myectomy in 36
patients (57%).8 Additional procedures are detailed in
Supplemental Table 1.
PATIENTS AND METHODS
FOLLOW-UP DATA. The last clinical follow-up was
STUDY POPULATION. This investigation, approved by
collected from the medical record to assess
the institutional review board (#18-002278) in February
reoperation. Mortality data were supplemented with
2020, included patients who underwent the index
information from the national death and location
corrective operation for LVOTO between August 1963
database (Accurint). Assuming a small lag in data,
and August 2018 at Mayo Clinic, Rochester, Minnesota.
January 31, 2020, was used as the date of last vital
Patients who did not authorize their records for
status for those accurately matched to the national
research were excluded. We analyzed available clinical,
database and still assumed to be alive.
echocardiographic, operative, and postoperative data
of patients who were 21 years of age. Patients who STATISTICAL METHODS. Categorical data were compared
underwent apical myectomy or had midcavitary/ with the c2 test. Most continuous variables were not
nonobstructive HCM and those who had septal hyper- normally distributed and therefore are reported as me-
trophy secondary to any other underlying cause were dian and interquartile range, and groups were compared
excluded. In addition, we excluded patients with with Wilcoxon rank sum test. We used the Kaplan-Meier
Shone syndrome or atrioventricular canal defect method to estimate survival. We analyzed occurrence of
with concomitant SAS. Thus, the final study groups all reoperations as well as procedures related to
included 152 patients with HCM and 63 patients with recurrent subaortic LVOTO. We provided a summary of
congenital SAS. these reoperations along with estimated cumulative
incidence with death as a competing risk. Univariable
CLINICAL DATA. Clinical information included age,
proportional regression models were used to
height, weight, body surface area, and body mass
investigate the association of morphologic features,
index at the time of surgery as well as symptoms
surgical procedure type, and diagnosis with survival
such as shortness of breath, chest pain, presyncope, and
and the need for reoperation (event-specific regression
syncope. Data on preoperative dysrhythmias included
model). Because of the age differences between the
the need for implantable cardioverter-defibrillator (ICD),
groups, we reweighted the data to the empirical age
pacemaker, history of radiofrequency ablation, and
distribution of our cohort and repeated the reoperation
cardiac arrest. Postoperative need for ICD placement
analyses on the weighted data set.
within 30 days after surgical procedure was noted.
Results of any genetic testing and family history of RESULTS
HCM or SAS were obtained from the patient’s medical
history. At the time of presentation for initial repair, patients
with HCM were older than patients with SAS (15 [10-19]
IMAGING AND HEMODYNAMIC DATA. Transthoracic echo- years vs 8 [5-13] years; P < .001). Patients with HCM
cardiographic examination reports were generally had higher body mass index than SAS patients (21.4
available in patients after November 1974. Hemody- [17.3-26.1] kg/m2 vs 18.2 [15.4-23.0] kg/m2; P < .001).
namic information from cardiac catheterization was Patients with HCM were more symptomatic with dys-
included for patients treated between August 1963 and pnea (64% vs 35.6%; P < .001), chest pain (44% vs
November 1974. Maximum instantaneous left ventricu- 20.7%; P ¼ .002), and presyncope/syncope (26.7% vs
lar (LV) outflow tract gradients were obtained at rest 8.6%; P ¼ .005) compared with those with SAS (Table 1).
with continuous wave Doppler echocardiography. In Of the patients with HCM, 2% (n ¼ 3) had cardiac ar-
some patients who may not have had a large resting rest before operation, and 15% (n ¼ 23) had ICD im-
gradient, provocative maneuvers (Valsalva maneuver, plantation; none of the SAS patients had a history of
amyl nitrite inhalation, or exercise) may have been used cardiac arrest and only 2% (n ¼ 1) had an ICD. A pace-
to elicit a gradient. Preoperative ejection fraction, LV maker was placed preoperatively in 2% (n ¼ 3) of pa-
mass index, and wall thickness were determined as tients in the HCM group vs 2% (n ¼ 1) in the SAS group.
previously described.6 Information on mitral valve A family history of the disease was noted in 36% of
systolic anterior motion (SAM) was obtained from patients with HCM, and 4% of HCM patients had a
preoperative transthoracic and intraoperative family history of sudden cardiac death. In the SAS group,
transesophageal echocardiograms. 5% (n ¼ 3) had a family history consistent with HCM but

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138 FATIMA ET AL Ann Thorac Surg
OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION 2023;115:136-43

TABLE 1 Baseline Characteristics of Patients With HCM and Congenital SAS T ABLE 2 Preoperative Echocardiographic Parameters
Undergoing Operation for Relief of LVOTO of Patients With HCM and Congenital SAS Undergoing
Operation for Relief of LVOTO
HCM SAS
Characteristic (n ¼ 152) (n ¼ 63) P Value Preoperative
Age at surgery, y 15.2 (9.6-18.8) 7.7 (5.2-13.3) <.001a Echocardiographic
Parameter HCM (n ¼ 152) SAS (n ¼ 63) P Value
Height, cm 161.3 (136.7-173.2) 122.0 (103.5-153.9) <.001a
Septal thickness, 23 (17-30) 10 (8-16) <.001a
Weight, kg 58.0 (32.4-74.0) 26.0 (18.1-52.8) <.001 a
diastole, mm
Body surface area, m 2
1.6 (1.1-1.9) 0.9 (0.7-1.5) <.001 a
N-Miss 16 20
Body mass index, kg/m2 21.4 (17.3-26.1) 18.2 (15.4-23.0) <.001a
LV wall thickness, 13 (10-17) 10 (8-12) <.001a
Dyspnea <.001 b
diastole, mm
Yes 96 (64.0) 21 (35.6) N-Miss 17 20
N-Miss 2 4 LV mass index, g/m2 215 (157-289) 108 (92-154) <.001a
Syncope/presyncope .005b N-Miss 51 30
Yes 40 (26.7) 5 (8.6) Ejection fraction, % 75 (70-79) 72 (68-75) .002a
N-Miss 2 5 N-Miss 30 22
Chest pain .002b LVOT peak 92 (77-117) 100 (76-110) .950a
Yes 66 (44.0) 12 (20.7) gradient, mm Hg
N-Miss 2 5 N-Miss 37 26
Family history of HCM 55 (36.2) 3 (4.8) <.001b SAM <.001b
Family history of sudden 6 (3.9) 1 (1.6) Yes 137 (100.0) 6 (12.2)
cardiac death No 0 (0.0) 43 (87.8)
Family history unknown 3 (2.0) 2 (3.2) N-Miss 15 14
No family history of HCM 88 (57.9) 57 (90.5)
Genetic testing .078b a
Kruskal-Wallis rank sum test; bPearson c2 test. Categorical variables are
presented as number (percentage). Continuous variables are presented as
Not performed 125 62
median (interquartile range). HCM, hypertrophic cardiomyopathy; LV, left
Positive result 21 (77.8) 0 (0.0) ventricular; LVOTO, left ventricular outflow tract obstruction; N-Miss, number of
Negative result 6 (22.2) 1 (100.0) missing values; SAM, systolic anterior motion; SAS, subaortic stenosis.

Pacemaker before surgery .015b


ICD 23 (15.1) 1 (1.6)
Pacemaker 3 (2.0) 1 (1.6)
No 126 (82.9) 61 (96.8) There was no operative mortality in the SAS group.
History of radiofrequency ablation 2 (1.3) 0 (0.0) .360b Two operative deaths (1.3%) in the HCM group occurred
Preoperative history of 3 (1.95) 0 (0.0) .261b in patients with severe biventricular obstruction. One
cardiac arrest
14-month-old boy died 1 week after hospitalization for
a
Wilcoxon rank sum test; Pearson c test. Categorical variables are presented as number (percentage).
b 2 biventricular myectomy in 1982, and another 4-year-old
Continuous variables are presented as median (interquartile range). HCM, hypertrophic cardiomyopathy; ICD, boy died of low cardiac output 1 day after operation
implantable cardioverter-defibrillator; LVOTO, left ventricular outflow tract obstruction; N-Miss, number of
missing values; SAS, subaortic stenosis. in 1994.
The median postoperative follow-up duration
(reverse Kaplan-Meier) was 13 years (interquartile range,
were categorized as SAS because of intraoperative evi-
dence of a discrete subaortic membrane distinct from
the septal contact lesion seen in obstructive HCM.
Seventeen percent (n ¼ 27) of patients with HCM un-
derwent genetic testing, the result of which was positive
in 78% (n ¼ 21). Only 1 patient with SAS underwent ge-
netic testing for HCM, and the result was negative.
As seen in Table 2, preoperative echocardiographic
studies demonstrated preserved ejection fraction in
both HCM and SAS groups. Septal thickness was
greater in HCM patients (23 [17-30] mm) compared
with the SAS group (10 [8-16] mm; P < .001). Also, values
for posterior wall thickness and LV mass index were
significantly greater in the HCM group. All patients with
obstructive HCM had associated SAM. On Doppler
FIGURE 1 Survival of patients with hypertrophic car-
echocardiography, the maximum instantaneous LV diomyopathy (HCM) and congenital subaortic stenosis
outflow tract gradient was 92 (77-117) mm Hg for patients (SAS) after undergoing surgical correction of left ven-
with HCM and 100 (76-110) mm Hg for the SAS group tricular outflow tract obstruction.

(P ¼ .95).

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Ann Thorac Surg FATIMA ET AL 139
2023;115:136-43 OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION

F I G U R E 2 ( A ) Ris k of r eop e r at i o n ( R e o p ) for an y c a rd ia c s u rge r y proc e d u re i n pa t ie n ts w i th h ype r tr o p h ic c ar d i o my o p at h y


( H C M ) an d c o nge n i ta l s ub ao r ti c ste n o si s (S AS ) af te r u n d e r goi ng su r g ic a l c o rr e ct i o n ( u n w ei g h t e d ) . (B ) R i s k o f r e o p er a t i o n fo r
an y c ar d i a c su r g e ry pr o c ed ur e in p a ti e n ts w i th H C M an d c o ng e n i ta l S A S a ft er u nd er g o i ng su r g i ca l c o r re c ti o n (we i g h te d ) .

1.1-20.5), and patients with SAS had better long-term for each year; 95% CI, 0.81-0.94; P < .001). This was
survival compared with those with HCM (P ¼ .007). As also true when each group was analyzed separately
seen in Figure 1 and Supplemental Table 2, survival of (HCM: HR for each year, 0.90 [95% CI, 0.82-1.00; P ¼
the 2 groups was similar through the first 10 years .041]; SAS: HR for each year, 0.84 [95% CI, 0.71-0.99;
postoperatively; but during the second decade of P ¼ .033]). To account for the association of age and
follow-up, HCM patients had poorer survival (20-year reoperation and the significant age difference between
survival of 80% for HCM vs 91% for SAS). In the uni- groups, the cohort was inverse probability weighted on
variable proportional hazard model, overall mortality in the basis of the empirical age distribution of the cohort
the SAS group was lower (hazard ratio [HR], 0.26; 95% (Figures 2B and 3B). In this age-weighted analysis, the
CI, 0.09-0.74; P ¼ .012). Age at operation was not asso- effect of the SAS group on risk (vs HCM) of reoperation
ciated with survival in this young cohort. for recurrent LVOTO was attenuated from 3.06 (95% CI,
During follow-up, the 20-year cumulative risk of 1.40-6.67; P ¼ .005) to 1.80 (95% CI, 0.78-4.18; P ¼ .179).
reoperation for any cause was 26% (95% CI, 16%-42%)
for patients with HCM and 52% (37%-72%) for patients COMMENT
with SAS (P ¼ .001; Figure 2A; Supplemental Table 3).
This risk of reoperation for any cause was 3 times This study examines potential differences in clinical
higher in the SAS group compared with the HCM group presentation and long-term outcomes of young patients
(HR, 3.13; 95% CI, 1.56-6.26; P ¼ .001; Supplemental with SAS and HCM undergoing LVOTO resection. We
Table 5). found that patients with HCM were more symptomatic
In the HCM group, 11 patients required reoperation for and presented with LVOTO at an older age compared
recurrent or residual LVOTO a median of 11 years after with the SAS group. The risk of reoperation due to
initial surgery, and 1 patient underwent an additional recurrent LVOTO was 3 times higher in patients with SAS
procedure 3 years after the second operation. In the SAS than in those with HCM. However, despite the increased
group, 15 patients required reoperation at a median of 7 need for reoperation due to recurrent LVOTO in the SAS
years after the initial procedure; of those, 3 patients group, the late survival of young patients with HCM was
required a second reoperation after a median duration of poorer than that of those with membranous SAS. The
11 years from the second operation. Ten years after cause of this decrease in survival among young patients
initial repair, incidence of reoperation for recurrent with HCM is likely to be risk of arrhythmias and predi-
LVOTO was 5% (2%-13%) in patients with HCM and 31% lection for development of restrictive physiology.
(19%-50%) in those with SAS (Figure 3A; Supplemental To date, there are no studies in the pediatric popu-
Table 4). lation that compare the symptomatic presentation and
In the SAS group, the addition of a septal myectomy outcomes of patients with HCM and SAS. Etnel and co-
at the time of initial membranectomy did not influence workers9 in 2015 published a meta-analysis on outcomes
the risk of reoperation. As seen in Table 3, older age of of pediatric patients with SAS. The authors included 24
the patient was significantly associated with lower risk studies, and of these, 16 reports documented that the
of reoperation due to recurrent obstruction (HR, 0.87 pooled total incidence of reoperation was 2.04% per

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140 FATIMA ET AL Ann Thorac Surg
OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION 2023;115:136-43

FIGURE 3 (A) Reoperation (Reop) for recurrent or residual left ventricular outflow tract obstruction in patients with hy-
pertrophic cardiomyopathy (HCM) and congenital subaortic stenosis (SAS) after undergoing surgical correction (un-
weighted). (B) Reoperation for recurrent or residual left ventricular outflow tract obstruction in patients with HCM and
congenital SAS after undergoing surgical correction (weighted).

year, which correlates with our findings of 52% during groups, patients with HCM more frequently presented
20 years, resulting in an average 2.6% every year.9 In the with dyspnea, chest pain, and history of presyncope and
same meta-analysis, pooled risk of mortality was 0.12% syncope. Patients with HCM had significantly greater LV
per year, but these data include the natural history of wall thickness, LV septal thickness, and LV mass index
patients who were not operated on.9 Another study by compared with the SAS group. Although these differ-
Zhu and associates10 in 2020 reported the outcomes of ences might be expected because of differences in body
pediatric patients with obstructive HCM who size, greater degrees of hypertrophy in patients with
underwent septal myectomy. Inferences are limited by obstructive HCM are likely to be due to the underlying
the relatively short follow-up (median, 3.2 years), but disease. LV hypertrophy in patients with SAS is sec-
overall freedom from reoperation was 98% at 3 years, ondary to obstruction caused by the membrane, whereas
and overall survival was 96.5% in 3 years.10 Our results hypertrophy in patients with obstructive HCM is largely
are generally similar with a 3.5% risk of reoperation related to the underlying cardiomyopathy.
and survival of 95.6% at 5 years after operation for the Some clinical and hemodynamic features of obstruc-
HCM group. tive HCM and SAS may overlap, and in rare instances,
We found important differences in the clinical char- both conditions may be present in the same patient.11,12
acteristics of young patients with SAS and those with In our series, 1 patient with a documented family history
obstructive HCM. Patients with HCM were older at the of HCM was found intraoperatively to have a typical
time of surgery to relieve LVOTO and, as expected, had subaortic membrane distinct from the contact lesion on
greater height and weight. Although hemodynamics the septum that results from SAM of the mitral leaflets.
including LV outflow tract gradient and LV ejection Another patient who was clinically described as having
fraction were not significantly different between the HCM was discovered to have an unusual extension of

TABLE 3 Univariable Proportional Hazard Model Predicting the Cause-Specific Hazard Ratio for Late Reoperation in
Patients With HCM and Congenital SAS Related to Recurrent or Residual LVOTO

Unweighted Age Weighted

Variable HR Lower 95% CI Upper 95% CI P Value HR Lower 95% CI Upper 95% CI P Value

Diagnosis, SAS vs HCM 3.055 1.399 6.669 .005 1.802 0.777 4.178 .170
Older age at surgery (per year) 0.874 0.811 0.943 <.001 0.881 0.811 0.956 .002
Greater height (per cm) 0.976 0.964 0.988 <.001 0.978 0.966 0.990 <.001
Greater weight (per kg) 0.959 0.937 0.982 <.001 0.960 0.938 0.983 <.001
Higher body mass index (per kg/m2) 0.897 0.818 0.982 .019 0.870 0.782 0.967 .010
Septal myectomy in SAS patients 0.991 0.279 3.518 .989 0.667 0.184 2.415 .537
Membranectomy in SAS patients 1.322 0.451 3.878 .611 1.007 0.305 3.325 .990

HCM, hypertrophic cardiomyopathy; HR, hazard ratio; LVOTO, left ventricular outflow tract obstruction; SAS, subaortic stenosis.

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Ann Thorac Surg FATIMA ET AL 141
2023;115:136-43 OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION

the membrane involving the anterior leaflet of the mitral myectomy would eliminate the substrate for recurrent
valve and the ventricular aspects of the left and subvalvular stenosis. Similarly, Brauner and
noncoronary cusp, findings typical of SAS. As a general colleagues19 hypothesized that turbulence caused by
clinical rule, patients with SAS have hemodynamic incomplete removal of LVOTO promotes fibrosis and
features of fixed obstruction and no SAM of the mitral subsequent restenosis, and septal myectomy can
valve. In this study, however, we recognized 6 patients reduce obstruction and therefore will decrease
(9.5%) who had SAM associated with a discrete recurrence. The rationale behind performing a septal
membrane characteristic of SAS. Iwata and colleagues13 myectomy is based on the pathologic process of SAS, a
in 2008 described a similar unusual patient with SAS progressive lesion characterized by thickening, fibrosis,
associated with SAM of the mitral valve who had, in and scarring of the LVOT.16
addition, an anterior cleft in the mitral leaflet and an In our study, the need for reoperation due to recurrence
accessory papillary muscle. of SAS was 1.8 times higher after 5 years and 6.2 times
Although perioperative mortality was similar for the higher after 10 years of index surgery compared with the
patients in the SAS and HCM groups, late survival of HCM group. Several studies have identified risk factors for
patients with HCM was reduced. Unfortunately, data on reoperation, including age <2 years at first intervention,
causes of death were incomplete in our study, but Nor- postoperative gradient >20 mm Hg, presence of a hypo-
rish and Kaski14 reported that beyond infancy, sudden plastic aortic annulus, <5-mm distance of membrane to
cardiac death is the most common cause of death in aortic valve, and associated left-sided heart lesions.20,21
pediatric patients with HCM; arrhythmias account for Ramog  lu and coworkers22 recognized a higher risk of
>50% of adverse events occurring within 10 years of reoperation in isolated SAS but similar risk in
diagnosis. Of note, this study did not include patients membranous and fibromuscular types. Early surgical
who had surgical correction for obstructive HCM.14 Zhu procedure may be beneficial in preventing aortic
and associates10 documented an overall 2.6% incidence insufficiency but does not affect the rate of reoperation.22
of sudden cardiac death after surgical correction of This study has several limitations including the
HCM in a median follow-up period of 3.2 years, possible bias associated with a single-center retrospec-
emphasizing the importance of risk stratification for ICD tive investigation. As reoperation was an important
in children with HCM regardless of prior septal myec- variable of the study, we had limited access to data for
tomy. More recently, Hughes and colleagues15 reported the patients who underwent index surgery in other in-
that myocardial perfusion is impaired in patients who stitutions, and therefore these patients were excluded
are genotype positive for HCM, even in the absence of from the study. The study patients were identified
hypertrophy and scarring, and resulting ischemia may during a long period during which surgical and diag-
predispose the patient to major adverse cardiac events. nostic techniques have improved; it is unclear, however,
In our study, relief of LVOTO was late in HCM patients whether current imaging techniques for surveillance
compared with SAS patients, and it is possible that this would improve late results. Last, our institution receives
too increases the risk of underlying fibrosis and patients from a global referral base, and detailed follow-
concern of ischemia secondary to LV hypertrophy and up in many was not possible.
subsequent diastolic dysfunction. In summary, among patients 21 years of age with
The incidence of reoperation for any cause was 3 obstructive HCM and SAS undergoing corrective surgery,
times higher for patients with SAS than for those with those with HCM were older and more symptomatic
HCM. It is notable, too, that the incidence of reoperation preoperatively compared with those with SAS, but the
for recurrent LVOTO after corrective surgery was also 3 severity of LVOTO was similar between the groups. The
times higher in the SAS group. It appears, however, that greater prevalence and severity of symptoms in those
some of this difference was related to younger age at the with HCM may be related to underlying myocardial
time of initial corrective surgery. In our cohort, the disease and diastolic dysfunction or to SAM of the mitral
addition of septal myectomy at the time of mem- valve causing variable degrees of regurgitation. Despite
branectomy did not appear to decrease the risk of a higher incidence of later reoperation, survival of pa-
reoperation, and this is similar to the reports of Mazurek tients with LVOTO due to SAS was superior to that of
and coworkers,16 Karamlou and coworkers,17 and van patients with HCM.
der Linde and coworkers.5 Others have hypothesized
that the addition of septal myectomy reduces the risk
FUNDING SOURCES
of reoperation. Lupinetti and colleagues18 suggested This work was supported by the Paul & Ruby Tsai Family.
that performing myectomy reduces turbulence, which
DISCLOSURES
may discourage progressive scar formation in the
The authors have no conflicts of interest to disclose.
subvalvular area during healing; thus, performing a

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142 FATIMA ET AL Ann Thorac Surg
OUTCOMES OF OPERATION FOR LVOT OBSTRUCTION 2023;115:136-43

REFERENCES

1. Serraf A, Zoghby J, Lacour-Gayet F, et al. Surgical treatment of subaortic 11. Anderson MJ, Arruda-Olson A, Gersh B, Geske J. Subaortic
stenosis: a seventeen-year experience. J Thorac Cardiovasc Surg. membrane mimicking hypertrophic cardiomyopathy. BMJ Case Rep.
1999;117:669-678. 2015;2015:bcr2015212321. https://doi.org/10.1136/bcr-2015-212321

2. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Long- 12. Mushtaque RS, Mushtaque R, Baloch S. Co-existing subaortic stenosis
term results of surgical repair in patients with congenital subaortic stenosis. in a patient with hypertrophic obstructive cardiomyopathy: a rare and
Interact Cardiovasc Thorac Surg. 2006;5:227-233. interesting finding. Cureus. 2020;12:e11891.

13. Iwata Y, Imai Y, Shin’oka T, Kurosawa H. Subaortic stenosis associated


3. Minakata K, Dearani JA, Schaff HV, O’Leary PW, Ommen SR,
with systolic anterior motion. Heart Vessels. 2008;23:436-439.
Danielson GK. Mechanisms for recurrent left ventricular outflow tract
obstruction after septal myectomy for obstructive hypertrophic cardiomy- 14. Norrish G, Kaski JP. The risk of sudden death in children with hyper-
opathy. Ann Thorac Surg. 2005;80:851-856. trophic cardiomyopathy. Heart Fail Clin. 2022;18:9-18.

4. Mukadam S, Gordon BM, Olson JT, et al. Subaortic stenosis resection in 15. Hughes RK, Camaioni C, Augusto JB, et al. Myocardial perfusion de-
children: emphasis on recurrence and the fate of the aortic valve. World J fects in hypertrophic cardiomyopathy mutation carriers. J Am Heart Assoc.
Pediatr Congenit Heart Surg. 2018;9:522-528. 2021;10:e020227.

16. Mazurek AA, Yu S, Lowery R, Ohye RG. Routine septal myectomy


5. van der Linde D, Roos-Hesselink JW, Rizopoulos D, et al. Surgical
during subaortic stenosis membrane resection: effect on recurrence rates.
outcome of discrete subaortic stenosis in adults. Circulation. 2013;127:
Pediatr Cardiol. 2018;39:1627-1634.
1184-1191.
17. Karamlou T, Gurofsky R, Bojcevski A, et al. Prevalence and associated
6. Lang RM, Bierig M, Devereux RB, et al. Recommendations for chamber
risk factors for intervention in 313 children with subaortic stenosis. Ann
quantification: a report from the American Society of Echocardiography’s
Thorac Surg. 2007;84:900-906 [discussion: 906].
Guidelines and Standards Committee and the Chamber Quantification
Writing Group, developed in conjunction with the European Association of 18. Lupinetti FM, Pridjian AK, Callow LB, Crowley DC, Beekman RH,
Echocardiography, a branch of the European Society of Cardiology. J Am Bove EL. Optimum treatment of discrete subaortic stenosis. Ann Thorac
Soc Echocardiogr. 2005;18:1440-1463. Surg. 1992;54:467-470 [discussion: 470-471].

19. Brauner R, Laks H, Drinkwater DC Jr, Shvarts O, Eghbali K, Galindo A.


7. Arghami A, Dearani JA, Said SM, O’Leary PW, Schaff HV. Hypertrophic
Benefits of early surgical repair in fixed subaortic stenosis. J Am Coll Car-
cardiomyopathy in children. Ann Cardiothorac Surg. 2017;6:376-385.
diol. 1997;30:1835-1842.
8. van Son JA, Schaff HV, Danielson GK, Hagler DJ, Puga FJ. Surgical
20. Carlson L, Pickard S, Gauvreau K, et al. Preoperative factors that pre-
treatment of discrete and tunnel subaortic stenosis. Late survival and risk of
dict recurrence after repair of discrete subaortic stenosis. Ann Thorac Surg.
reoperation. Circulation. 1993;88(pt 2):II159-II169.
2021;111:1613-1619.
9. Etnel JR, Takkenberg JJ, Spaans LG, Bogers AJ, Helbing WA. Paediatric  T, et al. Paediatric subaortic stenosis:
21. De Wolf R, François K, Bove
subvalvular aortic stenosis: a systematic review and meta-analysis of natural long-term outcome and risk factors for reoperation. Interact Cardiovasc
history and surgical outcome. Eur J Cardiothorac Surg. 2015;48:212-220. Thorac Surg. 2021;33:588-596.

10. Zhu C, Wang S, Ma Y, et al. Childhood hypertrophic obstructive car- 22. Ramog  lu MG, Karago € zlu
€ S, Uçar T, et al. Long-term follow-up of sub-
diomyopathy and its relevant surgical outcome. Ann Thorac Surg. 2020;110: valvular aortic stenosis in children: a single-centre experience. Cardiol
207-213. Young. 2022;32:980-987.

ª 2023 by The Society of Thoracic Surgeons 0003-4975/$36.00


Published by Elsevier Inc. https://doi.org/10.1016/j.athoracsur.2022.09.004

Obstruction on the Left Can Be Hard reoperation for recurrent left ventricular outflow tract
to Make Right obstruction. This is the first paper directly comparing the
differences between these two pathologies in young
INVITED COMMENTARY: patients. It is a good-size cohort from a leading institu-
In this issue of The Annals of Thoracic Surgery, the nicely tion in HCM surgery. The manuscript is well written, the
1
prepared article by Fatima and colleagues describes a data clearly presented, and the discussion appropriately
retrospective review of patients aged less than 21 years contextualizes the results without overstatement.
who had surgery for hypertrophic cardiomyopathy Although well addressed by the authors, this study
(HCM) or subaortic stenosis (SAS) at Mayo Clinic over has limitations. The 2011 North American guidelines
the last 6 decades. They found that patients with HCM consider septal myectomy as the gold standard tech-
were older and more symptomatic than patients with nique for septal reduction2; however, the operation is
SAS. Thirty-day mortality was slightly higher in the notoriously challenging and operative outcomes vary
HCM group, but survival was similar through the first 10 dramatically based on experience.3 The Mayo
years. However, during the second decade, patients with experience in HCM treatment is one of the largest in
HCM had worse survival. They also found that at 10 and the world, so their outcomes may not be
20 years after repair, more patients with SAS required representative of HCM treatment at lower volume

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