Nguyen et al Adult

Apical myectomy for patients with hypertrophic

cardiomyopathy and advanced heart failure
Anita Nguyen, MBBS,a Hartzell V. Schaff, MD,a Rick A. Nishimura, MD,b Jeffrey B. Geske, MD,b
Joseph A. Dearani, MD,a Katherine S. King, MS,c and Steve R. Ommen, MDb

ABSTRACT 100

80
Objective: In patients with apical hypertrophic cardiomyopathy, extensive apical

Survival (%)
60
hypertrophy may reduce left ventricular end-diastolic volume and contribute to 40
diastolic dysfunction, angina, and ventricular arrhythmias. Transapical myectomy 20
to augment left ventricular cavity size can increase stroke volume and decrease 0
left ventricular end-diastolic pressure. In this study, we describe early outcomes 0 5 10
Follow-Up (Years)
15

of patients with apical hypertrophic cardiomyopathy after transapical myectomy Myectomy 108 61 29 7
Waitlist 1178 562 292 129
and compare survival with that of patients with hypertrophic cardiomyopathy Myectomy Waitlist
listed for heart transplantation.
Survival of patients after apical myectomy (blue) and
Methods: Between September 1993 and March 2017, 113 symptomatic patients patients with HCM on transplant waitlist (red).
with apical hypertrophic cardiomyopathy underwent transapical myectomy. Clin-
ical information, echocardiographic data, and follow-up were reviewed. With the Central Message
use of a national database, survival was compared with that of patients with hy- Apical myectomy is a unique treatment option
pertrophic cardiomyopathy listed for heart transplantation. for patients with apical HCM. This operation
has acceptably low surgical risks and leads to

ADULT
Results: In the surgical cohort, median (interquartile range) age was 50.8 (39.3- long-term improvement of symptoms.
60.7) years, and 49 (43%) were male. Preoperatively, 108 patients (96%) were in
New York Heart Association class III/IV. All patients underwent transapical Perspective
myectomy. There were 4 (4%) deaths within 30 days of operation. At last Apical myectomy is a unique treatment option
follow-up, 76% of patients reported improvement in symptoms, and 3 patients for patients with nonobstructive apical HCM.
Heart transplant, which is currently the only
(3%) subsequently underwent cardiac transplantation for recurrent heart failure. alternative surgical option, may be delayed or
The estimated 1-, 5-, and 10-year survivals were 96%, 87%, and 74%, respec- avoided entirely.
tively. Survival appeared superior to patients with hypertrophic cardiomyopathy
listed for heart transplant. See Commentary on page XXX.
Conclusions: Apical myectomy is beneficial in severely symptomatic patients
with apical hypertrophic cardiomyopathy. Early risk of the procedure is low,
and approximately 76% maintain clinical improvement with resolution of symp-
toms. Long-term survival appears better than for patients listed for heart
transplantation. (J Thorac Cardiovasc Surg 2019;-:1-8)

Apical hypertrophic cardiomyopathy (HCM) is a pheno-

From the Departments of aCardiovascular Surgery, bCardiovascular Medicine, and typic variant of HCM, in which hypertrophy is especially
c
Health Sciences Research, Mayo Clinic, Rochester, Minn.
This work was supported by the Paul and Ruby Tsai Family. This publication was sup-
prominent at the apex of the left ventricle (LV) (Figure 1).
ported by Grant Number UL1 TR002377 from the National Center for Advancing It is considered relatively uncommon in Western countries
Translational Sciences. Its contents are solely the responsibility of the authors and (1%-10% of all patients with HCM),1,2 but may occur
do not necessarily represent the official views of the National Institutes of Health.
Disclaimer: The data reported have been supplied by the Minneapolis Medical
more frequently in Asian populations, in whom it is
Research Foundation as the contractor for the Scientific Registry of Transplant Re- reported in as many as 40% of patients with HCM.3-5
cipients. The interpretation and reporting of these data are the responsibility of the
author(s) and in no way should be seen as an official policy of or interpretation by
the Scientific Registry of Transplant Recipients or the US Government.
Institutional Review Board 16-007286.
Received for publication Nov 16, 2018; revisions received March 14, 2019; accepted Scanning this QR code will
for publication March 16, 2019. take you to the article title
Address for reprints: Hartzell V. Schaff, MD, 200 First St SW, Rochester, MN 55905 page to access supplementary
(E-mail: schaff@mayo.edu).
0022-5223/$36.00 information.
Copyright Ó 2019 by The American Association for Thoracic Surgery
https://doi.org/10.1016/j.jtcvs.2019.03.088

The Journal of Thoracic and Cardiovascular Surgery c Volume -, Number - 1

 Adult
Abbreviations and Acronyms
HCM ¼ hypertrophic cardiomyopathy
IQR ¼ interquartile range
LV ¼ left ventricular
MRI ¼ magnetic resonance imaging
NDI ¼ National Death Index
NYHA ¼ New York Heart Association
SRTR ¼ Scientific Registry of Transplant
Recipients
TTE ¼ transthoracic echocardiography
Sakamoto and colleagues6 first described apical HCM in
a Japanese patient in 1976, and initial studies of the clinical
outcome described a relatively benign disease course and
good overall prognosis.1,3 However, recent investigations
suggest that apical HCM can be associated with
debilitating symptoms, including fatigue, angina, and
severe dyspnea.2,7-10 Furthermore, serious adverse
outcomes have been described, such as ventricular
ADULT
arrhythmias, stroke, heart failure, and sudden cardiac
death.10 Indeed, survival of patients with apical HCM is
significantly worse than in an age- and sex-matched popu-
lation without HCM.2,10
Medical therapy of apical HCM includes nonvasodila-
tory beta-blockers and non–dihydropyridine calcium
channel blockade,10 but in many patients, pharmacologic
therapy does not dramatically relieve symptoms. Until
recently, heart transplantation provided the only surgical
option for patients with severely symptomatic apical
HCM. However, transapical myectomy to enlarge the LV
cavity can improve diastolic dysfunction and associated
symptoms.7 Previously, we reported the first 44 patients
with apical HCM who underwent transapical myectomy
for enlargement of the ventricular cavity at our institution.7
The present study updates and extends this experience of
surgically managing patients with apical HCM and con-
trasts survival in patients receiving apical myectomy
with that in patients with HCM who are listed for heart
transplantation.
MATERIALS AND METHODS
Study Patients
After approval from the Institutional Review Board, we reviewed 113
consecutive adult patients who underwent transapical myectomy between
September 1993 and March 2017 at the Mayo Clinic, Rochester, Minne-
sota. All patients with apical HCM in whom operation was performed to
augment LV cavity size were included, but we excluded patients who
had transapical septal myectomy for relief of midventricular obstruc-
tion.11,12 Preoperatively, all patients remained limited with diastolic heart
failure despite best medical treatment, and the option of transplantation
was discussed with those who had New York Heart Association (NYHA)
class III or IV symptoms.
2 The Journal of Thoracic and Cardiovascular Surgery c - 2019
Nguyen et al
Diagnosis of Apical Hypertrophic Cardiomyopathy
and Patient Selection
Diagnosis of apical HCM and patient selection for myectomy have
evolved over this 24-year experience. The apical distribution of ventricular
hypertrophy can be confirmed by multiple imaging techniques including
transthoracic echocardiography (TTE) and cardiac magnetic resonance im-
aging (MRI) (Figure 1). Typical phenotypic characteristics include an api-
cal wall thickness 15 mm or greater, apical displacement of the
anterolateral and posteromedial papillary muscles, and asymmetric septal
hypertrophy mainly confined to the apex of the LV.3,4 If concomitant
subaortic or midventricular obstruction is present, resting and provoked
gradients are also measured on TTE.13 If an apical aneurysm is suspected,
echocardiographic contrast administration or cardiac MRI can clarify api-
cal anatomy.14,15
Patients who benefit from apical myectomy to augment LV chamber
size are those with diastolic heart failure and small LV end-diastolic vol-
ume. In our initial experience, we routinely performed left heart catheter-
ization to measure LV pressure and to visualize the LV chamber with
contrast ventriculography. Currently, we prefer cardiac MRI for visualizing
LV morphology and quantifying LV volume. Candidates for apical myec-
tomy will usually have LVend-diastolic volume less than 50 mL/m2 and LV
stroke volume less than 30 mL/m2, although these indices are age- and sex-
specific, and should be evaluated on an individual basis.16
Operative Details
To enlarge the LV cavity, septal myectomy is performed via a transap-
ical incision (Video 1), and our technique has been described in detail.7,17
In brief, operation is performed through a standard median sternotomy and
the LV is assessed using intraoperative transesophageal echocardiography.
It is important to examine carefully for subaortic obstruction with systolic
anterior motion of the mitral valve. A minority of patients will have some
degree of concomitant LV outflow tract obstruction that will require
additional transaortic septal myectomy.11 The heart is arrested using
antegrade cardioplegia, and the apex of the heart is delivered anteriorly.
The left ventriculotomy is made lateral to and far enough from the left ante-
rior descending coronary artery so that closure will not compromise the
vessel.
We then identify and protect the anterolateral and posteromedial papil-
lary muscles, and begin the myectomy on the ventricular septum to enlarge
the LV cavity. If prominent papillary muscles are present, these may be
shaved to further increase LV volume. The myectomy is extended proxi-
mally, beyond the midventricular level. Adequacy of muscle excision is
judged visually and by palpation. If an apical aneurysm is identified on pre-
operative TTE or intraoperative inspection, the outpouching is resected
completely. The ventriculotomy is closed using a 2-layer approximation
over strips of Teflon felt.15
Data Collection and Follow-up
Data were collected from our prospectively maintained cardiovascular
surgery database, from follow-up questionnaires, and from chart review
of electronic medical records. Follow-up questionnaires were sent to all pa-
tients at 1, 3, 5, 10, 15, and 20 years postoperatively. Postoperative health
status was determined with the following questions: ‘‘Please rate your gen-
eral state of health before your surgery (1 ¼ excellent, 2 ¼ very good,
3 ¼ good, 4 ¼ fair, 5 ¼ poor)’’ and ‘‘Please rate your general state of health
since your surgery (1 ¼ excellent, 2 ¼ very good, 3 ¼ good, 4 ¼ fair,
5 ¼ poor).’’ Vital status was determined using the Mayo Clinic electronic
medical records, the national death and location database (Accurint, Lex-
isNexis for Mayo Clinic patients), and correspondence with family mem-
bers and physicians.
Follow-up information regarding survival is complete for the entire pa-
tient cohort. However, functional outcomes were obtained in 62 patients;
questionnaires were completed by 41 patients.
Nguyen et al
FIGURE 1. Preoperative and postoperative cardiac MRI in a patient with apical HCM. A, Preoperative cardiac MRI illustrating obliteration of the LVapical
cavity. Dashed line on the illustration depicts site of myectomy. B, Postoperative cardiac MRI in the same patient demonstrating enlarged LV cavity after
apical myectomy.
National Death Index
Information on vital status was verified using records from the National
Death Index (NDI). The NDI is a centralized database that collects infor-
mation on causes of death compiled from US vital statistics offices.
VIDEO 1. Video showing transapical myectomy performed on a 20-year-
old woman with a history of dyspnea, syncope, and recurrent implantable
cardioverter defibrillator discharges. Preoperative TTE showed severe cav-
ity obliteration, which improved significantly after operation. Video avail-
able at: https://www.jtcvs.org/article/S0022-5223(19)30772-X/fulltext.
The Journal of Thoracic and Cardiovascular Surgery c Volume -, Number -
Adult
ADULT
Mortality data are provided by each US state to the National Center for
Health Statistics. Cause of death is coded according to the International
Classification of Diseases 9th or 10th Revision.
Scientific Registry of Transplant Recipients
This study used data from the Scientific Registry of Transplant Recipi-
ents (SRTR). The SRTR data system includes data on all donor, wait-listed
candidates, and transplant recipients in the United States, submitted by the
members of the Organ Procurement and Transplantation Network. The
Health Resources and Services Administration, US Department of Health
and Human Services provides oversight to the activities of the Organ Pro-
curement and Transplantation Network and SRTR contractors.
Informal comparison was made of survival in the apical HCM cohort
versus SRTR cohort with HCM listed for heart transplant. From January
2004 to August 2017, 1235 adult patients with a diagnosis of HCM were
waitlisted for heart or heart-lung transplantation within SRTR. Duplicate
listings (n ¼ 54) were removed, and patients who died on the same day
as their listing date (n ¼ 3) were excluded from analysis. Therefore, the
waitlist cohort included 1178 unique adult patients identified through
this national database. Of these, 878 (75%) underwent transplantation.
Statistical Analysis
Categoric data are presented as frequencies and percentages, and
continuous variables are expressed as medians and interquartile range
(IQR). To adjust for the age and sex differences of the transplant waitlist/
transplant cohort and the myectomy cohort (for those with NYHA class
III or IV), the data were reweighted to the empirical distribution of the
cohort.18 Survival analyses were carried out on the unweighted and the
3
 Adult Nguyen et al

TABLE 1. Baseline characteristics and preoperative echocardiographic symptoms, including recurrent syncope, angina, and ar-
features rhythmias. Among the entire cohort, angina was present
n (%) or in 35 (31%), systemic hypertension was present in 50
Variable n missing median (IQR) (44%), and diabetes mellitus was present in 13 (12%). Ven-
Age, y 0 50.8 (39.3-60.7) tricular arrhythmias were common preoperatively; 20 pa-
Male 0 49 (43%) tients (18%) had at least 1 documented episode of
Dyspnea 0 112 (99%)
ventricular tachycardia, and intermittent or persistent atrial
fibrillation was present preoperatively in 30 patients (27%).
NYHA III or IV 0 108 (96%)
Angina 0 35 (31%)
Preoperative Echocardiography
Diabetes 0 13 (12%) All patients underwent transthoracic Doppler echocardi-
Hypertension 0 50 (44%) ography before surgery, and preoperative TTE data are pre-
Hyperlipidemia 0 63 (56%) sented in Table 1. The median (IQR) ejection fraction was
Cerebrovascular disease 0 9 (8%) 70.5 (66.0-75.0). Median LV end-diastolic diameter
Ventricular tachycardia 0 20 (18%) was 44.0 (39.0-50.0) mm, and LV end-systolic diameter
Atrial fibrillation 0 30 (27%)
was 25.0 (22.0-29.5) mm. Apical HCM distribution was
observed on TTE in all patients; concomitant subaortic
Permanent pacemaker 0 11 (10%)
obstruction was present in 23 patients (20%), midventricu-
Implantable cardioverter 0 37 (33%)
lar obstruction was present in 26 patients (23%), and apical
defibrillator
aneurysms were present in 25 patients (22%).
Beta-blockers 0 81 (72%)
ACE inhibitors 0 22 (19%) Operative Details and Postoperative Complications
ADULT

Ejection fraction, % 1 70.5 (66.0-75.0) All patients underwent transapical myectomy to enlarge
LVEDD, mm 12 44.0 (39.0-50.0) the ventricular cavity. In 23 patients (20%) with complex
LVESD, mm 22 25.0 (22.0-29.5) long-segment septal hypertrophy involving both subaortic
Mitral valve deceleration time, ms 26 198.0 (164.0-230.0) area and LV apex, combined transaortic and transapical in-
cisions were made to excise septal muscle. Apical aneu-
E/E0 15 15.0 (10.0-20.0)
2
rysms were repaired in 25 patients (22%).
LV mass index, g/m 16 152.0 (108.0-202.0)
During the early postoperative period, there were 4 (4%)
Septal wall thickness, mm 7 18.0 (12.0-25.0) 30-day mortalities, and 6 patients (5%) required reopera-
Posterior wall thickness, mm 7 12.0 (10.0-14.0) tion for bleeding. Postoperative atrial fibrillation was
Subaortic obstruction 0 23 (20%) observed in 28 patients (25%), but there were no surgical
Midventricular obstruction 0 26 (23%) site infections or pacemaker insertions after surgery. There
Apical aneurysm 0 25 (22%) were no operative deaths in patients with concomitant sub-
IQR, Interquartile range; NYHA, New York Heart Association; ACE, angiotensin- aortic obstruction; 1 patient who died postprocedure had
converting enzyme; LVEDD, left ventricular end-diastolic dimension; LVESD, left midventricular obstruction, and 2 patients had apical aneu-
ventricular end-systolic dimension; LV, left ventricular. rysms. Early mortality rates improved as our experience
increased; in the first decade, 30-day mortality was 8%
weighted data using the Kaplan–Meier method. Statistical analyses were and decreased to 5% in the second decade, and there have
carried out in R statistical software (version 3.4.2, Vienna, Austria). been no 30-day mortalities in 35 patients undergoing oper-
ation since 2013 (Figure 2).7
RESULTS
Patient Characteristics Late Outcomes
The baseline characteristics of the study cohort are pre- There were 19 late deaths for overall estimated 1-, 5-, and
sented in Table 1. The median (IQR) age of patients was 10-year survivals of 96%, 87%, and 74%, respectively. By
50.8 (39.3-60.7) years, and 49 (43%) were male. A total using data from the NDI, causes of mortality could be deter-
of 112 (99%) had shortness of breath at presentation, and mined in 18 of these patients. Nine patients (50%) died of
108 (96%) were in preoperative NYHA class III or IV. HCM, and the remaining 9 patients (50%) died of unrelated
One patient with apical HCM and a small LV cavity had causes. During follow-up, 3 patients (3%) underwent car-
no dyspnea at presentation, and the main indication for diac transplantation and 1 patient (1%) required LV assist
operation was suspected fibroelastoma at the apex; apical device implantation for heart failure. Details on functional
myectomy was performed at the time of ventricular explo- status were ascertained through follow-up visits, as well
ration. The remaining 4 patients classified as NYHA class II as survey responses; data were available in 62 patients
had less severe dyspnea preoperatively but had other (55%) at a median follow-up of 3.6 (1.1-8.1) years.

4 The Journal of Thoracic and Cardiovascular Surgery c - 2019

Nguyen et al Adult

70 9 80
60 8
7
Patient numbers

50 60

Mortality (%)
6

Percentage
40 5
4 40
30
3
20
2 20
10 1
0 0
1993 - 2002 2003 - 2012 After 2013 0
Health status at follow-up
No. of operations Early mortality (%)
Improved Unchanged Worsened
FIGURE 2. Case numbers and early mortality (defined as 30-day mortal- A
ity) by surgical era. The number of patients who underwent transapical
myectomy for cavity obliteration is shown in blue bars. Early operative
80
mortality, defined as death within 30 days of operation, is shown on the
red line.
60

Percentage
Preoperatively, 59 patients (95%) were in NYHA III or IV,
and postoperatively this decreased to 28 patients (45%). 40
More specific data from follow-up questionnaires were

available in 41 patients (36%) at a median follow-up of
5.2 (3.2-10.1) years; in these patients, 31 (76%) reported
improvement in general health compared with their preop-
erative condition, 9 (22%) stated that their general health
had stayed the same, and only 1 (2%) reported worsening
health status (Figure 3).
Comparison With Cardiac Transplantation
To provide perspective on survival of patients undergoing
apical myectomy, we contrasted survival of patients with
NYHA class III or IV symptoms (n ¼ 108) with that of pa-
tients with HCM listed for heart transplantation. Patients in
our surgical cohort were older than those listed for trans-
plant (P ¼ .001) and less likely to be male (P ¼ .006). Sur-
vival of patients receiving apical myectomy appeared
superior to those listed for transplant in both the unweighted
and age- and sex-weighted data (Figure 4, A and B). In
further analysis, survival of patients who received heart
transplant seemed similar to survival of patients receiving
apical myectomy (unweighted data in Figure 4, C, age-
and sex-weighted data in Figure 4, D).
DISCUSSION
Historically, apical HCM has been considered a benign
condition, but recent data suggest that apical HCM can be
associated with a variety of debilitating symptoms, as
well as life-threatening complications.9 Medical manage-
ment is often inadequate, and heart transplantation is the
only option for many patients with advanced symp-
toms.7,17,19 In the present surgical series of 113 patients
with apical phenotype of HCM, small LV diastolic
volume appeared to contribute to preoperative diastolic
ADULT
20
0
Preoperative Postoperative
NYHA I NYHA II NYHA III NYHA IV
B
FIGURE 3. Health status and functional outcomes of patients who under-
went transapical myectomy. A, Changes in health status from preoperative
baseline at a median (IQR) follow-up of 5.2 (3.2-10.1) years measured
through patient surveys in 41 patients. This shows improvement in health
status in 76%. B, Preoperative and postoperative functional status
described as NYHA class I to IV in 62 patients over median (IQR)
follow-up of 3.6 (1.1-8.1) years. NYHA, New York Heart Association.
heart failure. More than 95% of patients had NYHA class
III or IV dyspnea, and the extent of heart failure is
considerably higher than previously reported in
nonsurgical cohorts.8,20,21 The present study confirms that
surgical management with transapical myectomy to
enlarge the LV cavity has acceptable early mortality and
decreases symptoms related to diastolic dysfunction.
Overall survival compared favorably to that of patients
with HCM listed for heart transplantation in a national
database.
Early Operative Mortality
In this series, early mortality declined as surgical experi-
ence increased over the past 2 decades (Figure 2). In-
hospital (30-day) death occurred in a total of 4%, with no
early mortality in our 35 most recent cases since 2013.
The improving early outcomes may be due to a number of

The Journal of Thoracic and Cardiovascular Surgery c Volume -, Number - 5

 Adult Nguyen et al

100 100

80 80
Survival (%)

Survival (%)
60 60

40 40

20 20

0 0
0 5 10 15 0 5 10 15
Follow-Up (Years) Follow-Up (Years)
Myectomy 108 61 29 7 Myectomy 108 61 29 7
Waitlist 1178 562 292 129 Transplant 878 516 277 120
Myectomy Waitlist Myectomy Transplant
A C

100 100

80 80
Survival (%)

Survival (%)
60 60

40 40

20 20
ADULT

0 0
0 5 10 15 0 5 10 15
Follow-Up (Years) Follow-Up (Years)
Myectomy 108.0 63.6 30.7 6.6 Myectomy 108.0 63.6 31.1 6.9
Waitlist 1178.0 559.4 290.9 128.4 Transplant 878.0 512.2 275.4 119.0
Myectomy Waitlist Myectomy Transplant
B D
FIGURE 4. Survival of patients with preoperative NYHA class III or IV dyspnea undergoing apical myectomy compared with transplant waitlist patients
and recipients. A, Unweighted survival of patients receiving apical myectomy (blue) and patients with HCM on the SRTR list (red). B, Age- and sex-
weighted survival of patients receiving apical myectomy (blue) and SRTR transplant list (red). C, Unweighted survival of patients receiving apical myec-
tomy (blue) and transplant recipients (red). D, Age- and sex-weighted survival of patients receiving apical myectomy (blue) and transplant recipients (red).

factors, including better patient selection and refinement of
surgical technique. Patients who are most likely to benefit
from apical myectomy are those with diastolic heart failure,
a small LV cavity, and reduced stroke volume. Those with
normal LV end-diastolic dimensions are unlikely to experi-
ence symptomatic improvement and thus should not be
considered for surgery. LV cavity size can be assessed by
TTE or cardiac MRI. Cardiac MRI is especially useful in
determining ventricular volumes and stroke volume. At
operation, adequate resection of muscle is necessary to
ensure enlargement of the LV cavity. It is important to pre-
serve the papillary muscles, because injury to these struc-
tures can lead to adverse events, including mortality.
Midventricular Obstruction and Apical Aneurysms
Apical HCM with concomitant midventricular obstruc-
tion may be a more severe phenotype than isolated apical
HCM.8,20,22 Apical aneurysms are reported to occur in
10% to 20% of patients with nonobstructive HCM.22,23
In the present series, apically distributed HCM with
midventricular obstruction was observed in 23%, and
concomitant aneurysms were present in 22%. Of the 4 in-
hospital (30-day) deaths, 1 occurred in a patient with asso-
ciated midventricular obstruction, and 2 occurred in
patients with concomitant aneurysms. However, it should
be noted that adverse event rates in unoperated patients
with apical aneurysms are high. For patients with ventricu-
lar arrhythmias associated with apical aneurysms, surgery
appears to decrease adverse event rates.15,24
Functional Improvement
In a previous study, we documented that apical myec-
tomy enlarges LV end-diastolic volume, thereby increasing
stroke volume and reducing left atrial pressure.7 These he-
modynamic changes were associated with early and late
functional improvement. In the present study, self-
reported health status at last follow-up was improved in
the majority of patients, and general health had improved

6 The Journal of Thoracic and Cardiovascular Surgery c - 2019

Nguyen et al
in more than 75% (Figure 3). Postoperative imaging studies
other than TTE were not performed routinely in our pa-
tients. Indeed, visualization of the apical region of the LV
by echocardiography can be problematic early after
operation.
Survival and Comparison With Waitlisted Patients
Data on long-term survival in patients with apical HCM
are scarce, but a recent review by Jan and colleagues10 re-
ported overall mortality rates of 9% to 10.5% over a
mean/median follow-up of 2 to 6.5 years. Further, in
follow-up of 193 patients with nonsurgical apical HCM
from our Clinic, Klarich and colleagues2 described an over-
all mortality rate of 29% over a median follow-up of
6.5 years. In these patients, survival was reduced in older
and female patients. In the present surgical cohort, survival
estimates at 1, 5, and 10 years were 96%, 87%, and 74%,
respectively, which is consistent with our earlier
experience.7
When compared with patients listed for heart transplant,
survival appeared better in patients who underwent apical
myectomy (Figure 4, A and B). Patients who were waitlisted
for transplantation were younger than our surgical cohort
and more likely to be male, characteristics associated with
more favorable prognosis.13 Survival of patients with apical
HCM who had apical myectomy was similar to patients
with HCM who underwent cardiac transplantation
(Figure 4, C and D). In view of the shortage of organ donors
in the United States, apical myectomy should be considered
as initial treatment in appropriately selected patients with
apical HCM and advanced heart failure symptoms.25,26
Risk of Worsening Heart Failure After Myectomy
In patients with apical hypertrophy, percutaneous treat-
ment options such as alcohol septal ablation have been
discouraged, because targeted ablation would not signifi-
cantly alter chamber size and stroke volume.10 Thus,
advanced treatment options in patients with apical HCM
who are not amenable to medical therapy include heart
transplantation and our unique surgical technique of apical
myectomy.7 The present study shows that apical myectomy
can avert or delay the need for cardiac transplant in the ma-
jority of patients. At late follow-up, cardiac transplantation
had been performed in only 3 patients, and 1 needed an LV
assist device implanted because of worsening heart failure.
Study Limitations
This is a retrospective study of a single institution and is
limited by inherent selection bias. The relatively small size
of our study is due to apical HCM being an uncommon dis-
ease in Western countries. Echocardiographic and other im-
aging follow-up were not available in the entire cohort.
Patients with HCM who were waitlisted for heart transplant
were identified using a national database, but the SRTR
The Journal of Thoracic and Cardiovascular Surgery c Volume -, Number -
Adult
dataset does not include specific diagnosis of apical HCM
or preoperative echocardiographic variables; this should
be considered when interpreting our results. Causes of mor-
tality were obtained through the NDI, but data were avail-
able only for the small subset of patients who died during
the study period.
CONCLUSIONS
Apical myectomy is a unique treatment option for pa-
tients with nonobstructive apical HCM and small LV cavity
size who have severe symptoms due to diastolic heart fail-
ure. Early postoperative outcomes are encouraging, and
risk of the procedure is acceptably low. Survival in surgical
patients appears to be superior to patients with HCM listed
for cardiac transplantation. The majority of patients under-
going apical myectomy have sustained improvement in
heart failure symptoms, and heart transplant, which is
currently the only alternative surgical option, may be de-
layed or avoided entirely.
Conflict of Interest Statement
ADULT
Authors have nothing to disclose with regard to commercial
support.
References
1. Kitaoka H, Doi Y, Casey SA, Hitomi N, Furuno T, Maron BJ. Comparison of
prevalence of apical hypertrophic cardiomyopathy in Japan and the United
States. Am J Cardiol. 2003;92:1183-6.
2. Klarich KW, Attenhofer Jost CH, Binder J, Connolly HM, Scott CG,
Freeman WK, et al. Risk of death in long-term follow-up of patients with apical
hypertrophic cardiomyopathy. Am J Cardiol. 2013;111:1784-91.
3. Eriksson MJ, Sonnenberg B, Woo A, Rakowski P, Parker TG, Wigle ED, et al.
Long-term outcome in patients with apical hypertrophic cardiomyopathy. J Am
Coll Cardiol. 2002;39:638-45.
4. Binder J, Ommen SR, Gersh BJ, Van Driest SL, Tajik AJ, Nishimura RA, et al.
Echocardiography-guided genetic testing in hypertrophic cardiomyopathy:
septal morphological features predict the presence of myofilament mutations.
Mayo Clin Proc. 2006;81:459-67.
5. Moon J, Shim CY, Ha J-W, Cho IJ, Kang MK, Yang W-I, et al. Clinical and echo-
cardiographic predictors of outcomes in patients with apical hypertrophic cardio-
myopathy. Am J Cardiol. 2011;108:1614-9.
6. Sakamoto T, Tei C, Murayama M, Ichiyasu H, Hada Y. Giant T wave inversion as
a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle.
Echocardiographic and ultrasono-cardiotomographic study. Jpn Heart J. 1976;
17:611-29.
7. Schaff HV, Brown ML, Dearani JA, Abel MD, Ommen SR, Sorajja P, et al. Apical
myectomy: a new surgical technique for management of severely symptomatic
patients with apical hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg.
2010;139:634-40.
8. Yan L, Wang Z, Xu Z, Li Y, Tao Y, Fan C. Two hundred eight patients
with apical hypertrophic cardiomyopathy in China: clinical feature, prog-
nosis, and comparison of pure and mixed forms. Clin Cardiol. 2012;35:
101-6.
9. Kim EK, Lee S-C, Hwang JW, Chang S-A, Park S-J, On YK, et al. Differences
in apical and non-apical types of hypertrophic cardiomyopathy: a prospective
analysis of clinical, echocardiographic, and cardiac magnetic resonance find-
ings and outcome from 350 patients. Eur Heart J Cardiovasc Imaging. 2016;
17:678-86.
10. Jan MF, Todaro MC, Oreto L, Tajik AJ. Apical hypertrophic cardiomyopathy:
present status. Int J Cardiol. 2016;222:745-59.
11. Hang D, Schaff HV, Ommen SR, Dearani JA, Nishimura RA. Combined
transaortic and transapical approach to septal myectomy in patients with
7
 Adult
complex hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg. 2018;
155:2096-102.
12. Kunkala MR, Schaff HV, Nishimura RA, Abel MD, Sorajja P, Dearani JA, et al.
Transapical approach to myectomy for midventricular obstruction in hypertro-
phic cardiomyopathy. Ann Thorac Surg. 2013;96:564-70.
13. Geske JB, Ong KC, Siontis KC, Hebl VB, Ackerman MJ, Hodge DO, et al.
Women with hypertrophic cardiomyopathy have worse survival. Eur Heart J.
2017;38:3434-40.
14. Kherada N, Vinardell JM, Mihos CG, Santana O. Apical hypertrophic cardiomy-
opathy with left ventricular apical aneurysm: importance of multi-modality im-
aging. Echocardiography. 2017;34:1392-5.
15. Nguyen A, Schaff HV, Nishimura RA, Dearani JA, Ommen SR. Early outcomes
of repair of left ventricular apical aneurysms in patients with hypertrophic cardio-
myopathy. Circulation. 2017;136:1979-81.
16. Cain PA, Ahl R, Hedstrom E, Ugander M, Allansdotter-Johnsson A, Friberg P,
et al. Age and gender specific normal values of left ventricular mass, volume
and function for gradient echo magnetic resonance imaging: a cross sectional
study. BMC Med Imaging. 2009;9:2.
17. Said SM, Schaff HV, Abel MD, Dearani JA. Transapical approach for apical
myectomy and relief of midventricular obstruction in hypertrophic cardiomyop-
athy. J Card Surg. 2012;27:443-8.
18. Nieto FJ, Coresh J. Adjusting survival curves for confounders: a review and a new
method. Am J Epidemiol. 1996;143:1059-68.
19. Ong KC, Geske JB, Schaff HV, Nishimura RA. Transapical myectomy for severe
mid-ventricular obstructive hypertrophic cardiomyopathy. Eur Heart J. 2014;35:
2713.
ADULT
8 The Journal of Thoracic and Cardiovascular Surgery c - 2019
Nguyen et al
20. Cai C, Duan F-J, Yang Y-J, Guo X-Y, Liu Y-L, Liu Y-Q, et al. Comparison of the
prevalence, clinical features, and long-term outcomes of midventricular hyper-
trophy vs apical phenotype in patients with hypertrophic cardiomyopathy. Can
J Cardiol. 2014;30:441-7.
21. Lee S-E, Park J-K, Uhm J-S, Kim JY, Pak H-N, Lee M-H, et al. Impact of atrial
fibrillation on the clinical course of apical hypertrophic cardiomyopathy. Heart.
2017;103:1496-501.
22. Minami Y, Haruki S, Hagiwara N. Phenotypic overlap in hypertrophic cardiomy-
opathy: apical hypertrophy, midventricular obstruction, and apical aneurysm. J
Cardiol. 2014;64:463-9.
23. Matsubara K, Nakamura T, Kuribayashi T, Azuma A, Nakagawa M. Sustained
cavity obliteration and apical aneurysm formation in apical hypertrophic cardio-
myopathy. J Am Coll Cardiol. 2003;42:288-95.
24. Nguyen A, Schaff HV. Electrical storms in patients with apical aneurysms and
hypertrophic cardiomyopathy with midventricular obstruction: a case series. J
Thorac Cardiovasc Surg. 2017;154:e101-3.
25. Hsich EM, Rogers JG, McNamara DM, Taylor DO, Starling RC, Blackstone EH,
et al. Does survival on the heart transplant waiting list depend on the underlying
heart disease? JACC Heart Fail. 2016;4:689-97.
26. Zu~niga Cisneros J, Stehlik J, Selzman CH, Drakos SG, McKellar SH, Wever-
Pinzon O. Outcomes in patients with hypertrophic cardiomyopathy awaiting
heart transplantation. Circ Heart Fail. 2018;11:e004378.
Key Words: apical, hypertrophic cardiomyopathy,
myectomy
Nguyen et al Adult

000 Apical myectomy for patients with hypertrophic cardiomyopathy and

advanced heart failure
Anita Nguyen, MBBS, Hartzell V. Schaff, MD, Rick A. Nishimura, MD, Jeffrey B. Geske, MD,
Joseph A. Dearani, MD, Katherine S. King, MS, and Steve R. Ommen, MD, Rochester, Minn

Apical myectomy is a unique treatment option for patients with apical HCM. This operation has
acceptably low surgical risks and leads to long-term improvement of symptoms.

ADULT

The Journal of Thoracic and Cardiovascular Surgery c Volume -, Number -