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Contemporary outcomes for adult congenital heart surgery in an adult tertiary care hospital

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Contemporary outcomes for adult congenital heart surgery in an adult tertiary care hospital

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Content CONTEMPORARY OUTCOMES FOR ADULT CONGENITAL HEART SURGERY IN AN
ADULT TERTIARY CARE HOSPITAL

by

Ramsey S. Elsayed, MD

______________________________________________________________________________

A Thesis Presented to the

FACULTY OF THE USC KECK SCHOOL OF MEDICINE
UNIVERSTIY OF SOUTHERN CALIFORNIA

In Partial Fulfillment of the
Requirements for the Degree

MASTER OF SCIENCE

(APPLIED BIOSTATISTICS AND EPIDEMIOLOGY)

December 2020

Copyright 2020 Ramsey S. Elsayed, MD
ii
DEDICATION
This work is dedicated to my wonderful family, for their endless love and support and
dedicated to the patients and their families who were born with congenital heart defects and had
to cope with lifelong medical and surgical care.

iii
ACKNOWLEDGEMENTS
I would like to thank my advisor, mentor, and friend, Dr. Michael Bowdish for being a
great mentor and encouraging me to pursue a master’s degree in applied Biostatistics and
Epidemiology. In addition, I would like to thank my committee members, Dr. Wendy Mack and
Dr. Meredith Franklin. Last, I would like to thank all the professors for tolerating a full-time
faculty member in their classes – I truly enjoyed learning about a new discipline and look
forward to continuing to work together as colleagues.

iv
TABLE OF CONTENTS
DEDICATION ii
ACKNOWLEDGEMENTS iii
LIST OF TABLES v
LIST OF FIGURES vi
ABBREVEATIONS vii
ABSTRACT viii
INTRODUCTION 1
METHODS 2
RESULTS 4
DISCUSSION 7
REFERENCES 11
TABLES 14
FIGURE LEGENDS 23
FIGURES 24

v
LIST OF TABLES
Table 1. Comparison of baseline and operative factors based on operative complexity. 14
Table 2. Distribution of primary congenital cardiac pathology among 15
simple and complex procedures.
Table 3. Comparison of postoperative and long-term outcomes 16
based on operative complexity.
Table 4. Mortality Summary. 17
Table 5. Multivariable logistic regression model to identify 19
predictors of mortality within 30 days of cardiac procedure.
Table 6. Multivariable Proportional hazards analysis for risk factors 20
for overall mortality.
Supplemental Table 1. Distribution of simple and complex congenital 21
cardiac procedures by year of surgery.
Supplemental Table 2. Confidence intervals for Kaplan Meier survival figure. 22

vi
LIST OF FIGURES
Figure 1. Classification of ACHD patient using a complexity-based 24
classification system.
Figure 2. Kaplan Meier survival curve of entire cohort of ACHD patients. 25
Figure 3. Kaplan Meier survival curve of ACHD patients based on 26
operative complexity.

vii
ABBREVIATIONS
ACHD Adult congenital heart disease
ASD Atrial septal defect
CHF Congestive heart failure
CHD Congenital heart disease
CI Confidence interval
HR Hazard ratio
IQR Interquartile range
NYHA New York Heart Association
OR Odds ratio
RACHS-1 Risk Adjustment for Congenital Heart Surgery 1
RVOT Right ventricular outflow tract
STS Society of Thoracic Surgeons
TAVR Transcatheter aortic valve replacement
TGV Transposition of great vessels
VSD Ventricular septal defect
viii
Abstract
Objective: The prevalence and heterogeneity of adults with congenital heart disease are rapidly
increasing. The optimal perioperative environment in which to provide surgical care for these
patients remains controversial. We used a complexity-based classification system to review
outcomes for adult congenital heart surgery patients receiving care in a free-standing adult
hospital.
Methods: From 2005 – 2019, 333 adults (age 18 years) underwent surgical correction of a
congenital cardiac defect at a single adult center. Two surgeons with formal congenital heart
surgery training performed 90% of procedures (n=300). Operations were classified into “simple”
and “complex” procedures. Thirty-day outcomes were compared using logistic regression while
overall survival was compared using Kaplan Meier methods and Cox proportional hazard
regression.
Results: Thirty-day mortality was 0.67% (n=1) in the simple and 3.8% (n=7) in the complex
cohort (p=0.06). After multivariable adjustment, reoperation was the only predictor of 30-day
mortality (odds ratio (OR) 17.1, confidence interval (CI) 1.7-170, p=0.02), while operative
complexity was not (OR 2.84, 95% CI 0.30-26.7, p=0.36). Kaplan-Meier survival at 1, 3, and 5
years was 98.6, 97.5, and 97.5% in the simple cohort and 96.1, 93.5, and 93.5% in the complex
cohort; survival did not significantly differ (log-rank p=0.12). After multivariable adjustment,
while age and presence of preoperative CHF were predictors of overall survival, while operative
complexity was not (hazard ratio 1.52, 95% CI 0.35-6.5, p=0.58).
Conclusion: Excellent outcomes for simple and complex lesions in adult congenital heart surgery
are achievable at centers caring exclusively for adult patients when performed by surgeons with
formal training in congenital cardiac surgery.
1
Introduction
Numerous medical and surgical advances in the past four decades have drastically altered
the life expectancy of children with congenital heart defects. With 4 out of 5 congenital heart
patients surviving into adulthood, there is an ever-evolving need for establishing benchmarks for
comprehensive care for these patients.
1-5
Despite efforts made worldwide to provide continuity of
care for this cohort of patients as they enter adulthood, many still suffer haphazard care, which can
be explained by the lack of a standard model of transition of care from childhood into adulthood.
6-
10
Controversy exists on where these patients should receive their continued care as well as
by whom. Should a 35-year old tetralogy of Fallot patient receive care at a pediatric or an adult
hospital? Should a 55-year old patient have their sinus venosus atrial septal defect repaired by an
adult or a pediatric heart surgeon? There are multiple supporting and opposing arguments for each
model of care. The pediatric care model is obviously well acquainted with the pathophysiology of
the cardiac disease but care for adults in a pediatric hospital creates unavoidable social
awkwardness, which undeniably burdens patients psychologically.
11
The adult care model relieves
patient discomfort, and more importantly, as this populations ages, so does their medical and
surgical complexity, that would require the proximity of multidisciplinary adult consult services.
Both models have certain limitations; however, the adult care model offers more comprehensive
care.
In the past decade, we have gradually transitioned care for this subset of patients from a
pediatric to an adult center of care. In this report, we sought to examine surgical outcomes and
predictors of early mortality for adult congenital cardiac surgery patients receiving care at a free-
standing adult medical center.

2

Patients and Methods
Patient selection, data collection, and study endpoints
We performed an observational retrospective cohort study of prospectively collected data
from 333 consecutive adult patients that required surgical management of congenital heart defects
between October 1, 2005 and August 31, 2019 at a single large adult quaternary center. The
institutional review board of the University of Southern California Health Sciences Campus
approved the study protocol (HS-18-00811) and waived patient consent requirement.
Baseline demographics and comorbidities, operative characteristics, and perioperative
outcomes were collected via our prospectively maintained cardiac surgery database. Mortality and
reintervention data were obtained through direct patient or provider contact. Follow-up closed on
December 31
st
, 2019. Patients with partial follow-up were included in the analysis but censored at
time of last contact. The primary study endpoint was 30-day mortality while overall mortality was
the secondary endpoint.

Complexity-based Classification System
In our analysis, procedures performed on patients (adults 18 years of age or older) with
congenital heart disease were divided in two cohorts; simple and complex (Figure 1). Simple
procedures were considered to be straightforward, uncomplicated, single structural defects which
included isolated atrial septal defects, non-complex left-sided valve repair or replacement, and
simple coronary abnormalities. Complex procedures were considered to require specialized
training in congenital heart surgery, and included complex left ventricular outflow reconstruction,
any right ventricular outflow reconstruction, congenital ventricular septal defects and complex
3
shunting lesions, single ventricle lesions, and more complex coronary artery anomalies. Multiple
patients had more than one lesion; we classified each patient based on the most technically
challenging lesion. Complex procedures were performed solely by adult cardiac surgeons with
formal training in congenital cardiac surgery while simple procedures were carried out by any
adult cardiac surgeon with adequate levels of experience and comfort in performing the procedure.
Statistical Analyses
Data analyses were performed using STATA version 16.1 (Statistical Software, College
Station, TX) and R version 4.0.2 (R Core Team, Vienna, Austria). Patient demographic, operative,
and postoperative outcome data were analyzed using standard descriptive statistics. Continuous
variables are presented as mean and standard deviation or median and interquartile range for
normal and non-normal variables, respectively, whereas categorical variables are presented as
number and percentage. Comparison between cohorts was conducted using chi square or Fisher’s
exact test for categorical variables and student’s t-test or Wilcoxon rank sum test, based on
normality, for continuous variables. Logistic regression analysis was used to assess association
between variables and 30-day mortality. Factors which were significant in univariate analyses
(p<0.1) were subsequently included in multivariable logistic regression analyses. Significant
variables identified on logistic regression, as well as age, sex, operative urgency, and repeat cardiac
operation were then analyzed for overall mortality in a multivariable Cox proportional hazard
model (“stcox” Stata package). Kaplan-Meier methods and log-rank test were used to analyze
overall survival for the entire cohort and for each of the operative complexity cohorts (Simple vs.
Complex).
4
Results
Patient population characteristics
A total of 350 operations were performed on 333 patients at our adult hospital during the
study period. Congenital heart surgeons performed 90% of these procedures. The complex cohort
was younger than the simple cohort [median 34 (IQR 26,49) vs. 45 (IQR 31,56) years,
p=0.0001]. There were more hypertensive patients in the simple cohort [58 (39%) vs 36 (20%),
chi-square p<0.001], while the complex cohort had more congestive heart failure (CHF) [23
(15%) vs 49 (27%), chi-square p=0.012], reoperations [28 (19%) vs 94 (51%), chi-square
p<0.001], and history of congenital disease [11 (7%) vs 99 (54%), chi-square p<0.001]. Other
preoperative and intraoperative characteristics did not differ between groups and are summarized
in Tables 1 and 2.
There was a higher prevalence of patients with complex pathology in our cohort [183
(54%) vs. 155 (46%)]. Systemic-to-pulmonary shunting lesions were the most frequent,
including 87 atrial septal defects (ASD), 36 ventricular septal defects (VSD), and 4 patent ductus
arteriosus lesions. Left-sided lesions were the second most common pathology with 88 bicuspid
aortic valves primarily managed by autograft, tissue, or mechanical valve replacement, three
mitral valve disease presenting after previous CHD repairs, and one truncus arteriosus patient.
Right-sided lesions were the third most common pathology with 43 tetralogies of Fallot, 9
Ebstein’s anomalies, 7 transposition of great vessels (TGV), 6 double outlet right ventricles, and
three single ventricle repairs requiring a Fontan revision. Less frequently observed were
coronary abnormalities mostly in the form of anomalous origin of the right coronary artery
(n=39), descending aortic aneurysms (n=10), and four other unclassified lesions (aberrant left
subclavian artery with a right-sided aortic arch, 2 vascular rings, and a D-transposition patient).
5
The distribution of primary congenital cardiac pathology among simple and complex cohorts as
well as operative classification are summarized in Table 3.
Perioperative outcomes and 30-day mortality
There was no difference in the length of intensive care unit (p=0.14) or hospital stay
(p=0.08) between simple and complex patients. The overall incidence of any postoperative
complication was 29% (96/333). These included reoperation within 30 days, bleeding, stroke,
arrythmia, renal failure requiring dialysis, anemia requiring blood transfusion, and pacemaker
insertion. The most common complication was atrial arrhythmia in 73 patients (22%) followed
by anemia requiring blood transfusion in 26 patients (7.8%). Complete postoperative
complications are displayed in Table 3.
One intraoperative death occurred in a 23-year old patient with DiGeorge syndrome after
an unsuccessful attempt of a transcatheter valve repair of a pulmonary autograft, which required
open conversion, but nonsurvivable profuse intraoperative hemorrhage occurred. Eight patients
expired within 30 days; 7 in the complex and one in the simple group ((3.8% vs 0.7%, Fischer
exact p=0.078). A complete summary of deaths in the cohort during the follow-up period are
presented in Table 4.
Survival and reinterventions
Median follow-up was 39 [IQR 12, 69] months. The simple cohort had a median follow-up of 36
(IQR 13, 67) months and the complex cohort had a median follow-up of 42 (IQR 12, 72) months.
During the follow-up period, 15 patients died; three in the simple group and 12 in the complex
group (Fischer exact, p=0.099). Overall Kapan Meier survival was 97%, 95%, and 95% at 1, 3,

6

and 5 years, respectively (Figure 2). Survival at 1, 3, and 5 years was 98%, 97%, and 97% in the
simple cohort and 96%, 93%, and 93% in the complex cohort and did not differ between cohorts
(log-rank p=0.12, Figure 3 and Supplemental Table 2). There was a total of 16 reinterventions,
eight in each cohort. Valve replacements were the most common reintervention followed by aortic
reinterventions (Table 3).

Predictors of 30-day and overall mortality
Risk factor analysis for 30-day mortality was performed. Multivariable analysis
demonstrated that preoperative CHF (Odds ratio (OR) 5.0, p=0.047) and redo operation (OR 17.1,
p=0.016) were associated with early mortality while operative complexity was not (OR 2.84 (0.30-
26.70, p=0.36) (Table 5). Cox proportional hazard modeling identified every 10 year increase in
age (HR 1.48, p=0.023), preoperative CHF (HR 5.67, p=0.003), and redo operation (HR 4.27,
p=0.031) as risk factors associated with overall mortality while operative complexity was not a
predictor of death (HR 1.50, p=0.58) (Table 6).

7

Discussion
Children with congenital heart disease are currently outnumbered by their adult
counterparts, with more than 85% surviving into adulthood.
12
According to the Society of Thoracic
Surgeons national database 2019 annual report, there has been an 45% increase in the number of
congenital cardiac operations from 354,846 in 2015 to 515,680 in 2019.
13,14
Similarly, at our adult
hospital, we witnessed a surge in number of adult congenital cardiac patients; a 174% rise in case
volume from the 2005-2009 period (n=43) to the 2010-2014 period (n=118) and a 46% rise from
the 2010-2014 period to the 2015-2019 period (n=172). This incremental increase in number of
congenital cardiac operations and adult survival rates necessitates complex multidisciplinary
treatment requirements that can be found in an adult hospital.
With a median post-surgical follow-up of xxx, multiple significant multivariate predictors
of overall death were identified in our series of ACHD patients, including reoperation, older age,
and preoperative CHF. However operative complexity was not a significant predictor of early
(give OR and CI) or late death (give HR and CI).
Redo sternotomy operations are known to increase risk of early and late mortality in adults
with and without congenital cardiac defects. Holst and colleagues found that increasing number of
sternotomies increased the risk of death, with a third sternotomy carrying a 1.5-fold and a fourth
sternotomy a 2-fold increase in risk of early mortality.
15
Another report by Nozohoor et. al. showed
a higher rate of major postoperative complications in those with previous history of congenital
heart surgery.
16
Increased frequency of sternotomies leads to complex anatomy, obscured
anatomical landmarks, extracardiac conduits or thoracic aortic invasion of chest wall, and
increased adhesions which make reoperations difficult.
17
Furthermore, those in the simple cohort
had less reoperations, which can be explained by the lack of previous diagnosis or symptoms
8
warranting surgical intervention as is the case in some ASD and BAV patients. Conversely, more
than half of patients in the complex cohort had previous operations for correction or palliation of
their congenital defect earlier in life and presented for further intervention.
Increasing age was a significant predictor of mortality in our cohort. Numerous reports
showed an inverse relation between patient age and survival rate. Putman and their group showed
that older age at surgery in a multivariate analysis led to increased 30-day, one year, and long-term
mortality rates.
18
Another report by Dore et. al. of 307 consecutive adult congenital operations
showed that reoperations and increasing age were independent predictors of early mortality in a
multivariable logistic regression analysis.
19
Additionally, in a study of over 3000 patients using
the Pediatric Health Information System database, Kim and colleagues showed that increasing age,
male sex, government issued insurance, and greater surgical complexity had the highest likelihood
of in-hospital death. Increased age-associated comorbidities may explain this finding.
20

Lastly, the presence of congestive heart failure symptoms preoperatively was significantly
associated with 30-day and overall mortality on univariate and multivariable analyses, regardless
of operative complexity. This finding has been abundantly reported in the literature.
2,21,22
Kogon
et. al. reported NYHA classes 3 or 4 as significant risk factors for operative mortality, major
adverse events, and a length of stay greater than 7 days while Tutarel et. al. reported similar
findings.
23
This frequent finding among studies and ours can be explained by reduced myocardial
function and vascular compliance, arrhythmogenicity, collateral circulation, ventricular
hypertrophy, and cardiovascular decompensation.
The lack of risk factor models as well as a clinical outcomes adult congenital registry
renders risk stratification challenging in this patient cohort. An STS risk factor calculator exists
9
for those with acquired heart disease, and the RACHS and Aristotle scores exist for those with
congenital heart disease, but does not accurately translate into the adult congenital population.
24

For example, RACHS-1 categories 4,5, and 6 are not well represented as they are generally
performed in childhood. This results in suboptimal classification of ACHD patients and possibly
contributing to the limited performance of this score.
In general, patients with CHD lack a comprehensive adult congenital risk-adjusted
classification system. This is in part due to the heterogeneity of the complex pathology unique to
each individual patient. The Bethesda congenital heart defect pathological classification system
created by Warnes and Liberthson in 2001 is formidable and established the cornerstone for
classification of CHD.
25
However, concomitant congenital defects, acquired cardiac conditions,
reoperations, and other comorbidities were not taken into consideration. In this study, we aimed to
identify preoperative risk factors for mortality while using a complexity-based classification
system. After reviewing each patient profile including age, number of previous cardiac
interventions, number of co-existing congenital and acquired cardiac diseases as well as operative
complexity, patients were classified into simple and complex cohorts. After multivariable
adjustment, operative complexity did not predict immediate mortality (OR 2.8, 95% CI 0.3-26.7,
p=0.36 ) or long-term mortality (HR 1.5, 95% CI 0.4-6.3, p=0.58) in our study. In contrast, Kim
and colleagues report of 3061 adult congenital heart surgery admissions from 42 hospitals showed
that operative complexity was a predictor of in-hospital mortality, inferred to by utilizing the
RACHS-1 risk categories. The authors found that patients in category 4+ carry highest risk of
mortality (OR 21.5, CI 3.4-136) Several other reports documented lower survival with higher
RACHS-1 scores.
20

The majority of procedures presented in this study were performed by two adult cardiac

10

surgeons with training in congenital cardiac surgery, in collaboration with other adult cardiac
surgeons, adult congenital cardiologists, and radiologists. We believe that the proximity of
multidisciplinary adult consult services in addition to this collaborative approach led to our
excellent outcomes. We continue to advocate that repairs of complex congenital defects in adults
be repaired by formally trained surgeons and simple repairs be performed by an adult cardiac
surgeon with a sufficient amount of comfort and experience with the procedure.
11
References
1. Abarbanell G, Goldberg C, Devaney E, Ohye R, Bove E, Charpie J. Early surgical
morbidity and mortality in adults with congenital heart disease: the University of
Michigan experience. Congenit Heart Dis 2008;3:82-9.
2. Beurtheret S, Tutarel O, Diller GP, et al. Contemporary cardiac surgery for adults with
congenital heart disease. Heart 2017;103:1194-202.
3. Valente A, Emani S, Landzberg M, Bacha E. Adult Congenital Cardiac Surgery. Sabiston
and Spencer Surgery of the Chest Ninth Edition 2016:2347-61.
4. Burke R, Cohn L. Surgery for Adult Congenital Heart Disease: McGraw-Hill Education
LLC;; 2018.
5. Heery E, Sheehan A, While A, Coyne I. Experiences and Outcomes of Transition from
Pediatric to Adult Health Care Services for Young People with Congenital Heart Disease:
A Systematic Review. Congenit Heart Dis 2015;10:413-27.
6. Helm PC, Kaemmerer H, Breithardt G, et al. Transition in Patients with Congenital Heart
Disease in Germany: Results of a Nationwide Patient Survey. Front Pediatr 2017;5:115.
7. Said SM, Driscoll DJ, Dearani JA. Transition of care in congenital heart disease from
pediatrics to adulthood. Semin Pediatr Surg 2015;24:69-72.
8. Vaikunth SS, Williams RG, Uzunyan MY, Tun H, Barton C, Chang PM. Short-term
outcomes following implementation of a dedicated young adult congenital heart disease
transition program. Congenit Heart Dis 2018;13:85-91.
9. Everitt IK, Gerardin JF, Rodriguez FH, 3rd, Book WM. Improving the quality of
transition and transfer of care in young adults with congenital heart disease. Congenit
Heart Dis 2017;12:242-50.
12
10. Williams RG. Transitioning youth with congenital heart disease from pediatric to adult
health care. J Pediatr 2015;166:15-9.
11. Kogon BE, Plattner C, Leong T, et al. Adult congenital heart surgery: adult or pediatric
facility? Adult or pediatric surgeon? Ann Thorac Surg 2009;87:833-40.
12. Warnes CA. The adult with congenital heart disease: born to be bad? J Am Coll Cardiol
2005;46:1-8.
13. Jacobs JP, Shahian DM, Prager RL, et al. The Society of Thoracic Surgeons National
Database 2016 Annual Report. Ann Thorac Surg 2016;102:1790-7.
14. Fernandez FG, Shahian DM, Kormos R, et al. The Society of Thoracic Surgeons National
Database 2019 Annual Report. Ann Thorac Surg 2019;108:1625-32.
15. Holst KA, Dearani JA, Burkhart HM, et al. Risk factors and early outcomes of multiple
reoperations in adults with congenital heart disease. Ann Thorac Surg 2011;92:122-8;
discussion 9-30.
16. Nozohoor S, Gustafsson R, Kallonen J, Sjögren J. Midterm Results of Surgery for Adults
with Congenital Heart Disease Centralized to a Swedish Cardiothoracic Center. Congenit
Heart Dis 2013;8:273–80.
17. Bhat A, Sahn D. Congenital heart disease never goes away, even when it has been
‘treated’: the adult with congenital heart disease. Current opinion in pediatrics
2004;16:500-7.
18. Putman LM, van Gameren M, Meijboom FJ, et al. Seventeen years of adult congenital
heart surgery: a single centre experience. Eur J Cardiothorac Surg 2009;36:96-104;
discussion
13
19. Dore A, Glancy D, Stone S, Menashe V, Somerville J. Cardiac surgery for grown-up
congenital heart patients: survey of 307 consecutive operations from 1991 to 1994. Am J
Cardiol 1997;80:906-13.
20. Kim YY, Gauvreau K, Bacha EA, Landzberg MJ, Benavidez OJ. Risk factors for death
after adult congenital heart surgery in pediatric hospitals. Circ Cardiovasc Qual
Outcomes 2011;4:433-9.
21. Bhatt A, Rajabali A, He W, Benavidez O. High resource use among adult congenital
heart surgery admissions in adult hospitals: risk factors and association with death and
comorbidities. Congenit Heart Dis 2015;10:13-20.
22. Giamberti A, Chessa M, Abella R, et al. Morbidity and mortality risk factors in adults
with congenital heart disease undergoing cardiac reoperations. Ann Thorac Surg
2009;88:1284-9.
23. Kogon B, Grudziak J, Sahu A, et al. Surgery in adults with congenital heart disease: risk
factors for morbidity and mortality. Ann Thorac Surg 2013;95:1377-82; discussion 82.
24. Fuller SM, He X, Jacobs JP, et al. Estimating Mortality Risk for Adult Congenital Heart
Surgery: An Analysis of The Society of Thoracic Surgeons Congenital Heart Surgery
Database. Ann Thorac Surg 2015;100:1728-35; discussion 35-6.
25. Warnes CA, Liberthson R, Danielson GK, et al. Task Force 1: the changing profile of
congenital heart disease in adult life. Journal of the American College of Cardiology
2001;37:1170-5.

14

Table 1. Comparison of baseline and operative factors based on operative complexity.
1

Variable All patients
(n=333)
Simple
(n=150)
Complex
(n=183)
p-
value
*

Age (median, IQR) 38 (27, 52) 45 (31, 56) 34 (26, 49) 0.0001
Sex (male) 178 (53.5%) 81 (54%) 97 (53%) 0.86
Hypertension 94 (28.2%) 58 (38.7%) 36 (19.7%) <0.0001
Diabetes Mellitus 26 (7.8%) 10 (6.7%) 16 (8.7%) 0.51
Hyperlipidemia 51 (15.3%) 28 (18.7%) 23 (12.6%) 0.12
Chronic Obstructive Pulmonary Disease 10 (3%) 4 (2.7%) 6 (3.3%) 0.52
Peripheral Arterial Disease 2 (0.6%) 2 (1.3%) 0 (0) 0.12
Cerebrovascular Accident 18 (5.4%) 10 (6.7%) 8 (4.4%) 0.36
Family History of Congenital Heart Disease 27 (8.1%) 12 (8%) 15 (8.2%) 0.95
Body Mass Index 27 (23, 31) 27 (24, 31) 26 (23, 31) 0.39
History of Congenital Disease 110 (33%) 11 (7.3%) 99 (54.1%) <0.0001
Concomitant Acquired Cardiac Pathology 28 (8.4%) 15 (10%) 13 (7.1%) 0.25
Smoking History 54 (16.2%) 28 (18.7%) 26 (14.2%) 0.27
Endocarditis History 20 (6%) 5 (3.3%) 15 (8.2%) 0.10
Hemodialysis 1 (0.3%) 0 (0) 1 (0.5) 0.36
Non-elective Procedure 15 (4.5%) 5 (3.3%) 10 (5.5%) 0.36
Coronary Artery Disease 21 (6.3%) 9 (6%) 12 (6.6%) 0.97
Congestive Heart Failure 72 (21.6%) 23 (15.3%) 49 (26.8%) 0.012
Preoperative Hematocrit 42 (38, 45) 42 (37, 45) 42 (38, 46) 0.48
Preoperative Creatinine 0.8 (0.7, 0.9) 0.8 (0.7, 1) 0.8(0.7,0.9) 0.40
Preoperative Arrhythmia 64 (19.2%) 27 (18%) 37 (20%) 0.61
NYHA Classification 0.045
Class I 111 (33.3%) 58 (38.7%) 53 (29%)
Class II 147 (44.1%) 69 (46%) 78 (46.2%)
Class III 59 (17.7%) 18 (12%) 41 (22.4%)
Class VI 13 (3.9%) 3 (2%) 10 (5.5%)
Ejection fraction (%, median, IQR) 60 (55, 65) 60 (57, 65) 60 (55, 65) 0.14
Reoperation 122 (36.6%) 28 (18.7%) 94 (51.4%) <0.0001
Cardiopulmonary bypass time (minutes,
mean ± SD)
83 (55, 118) 82 (54, 106) 85(56,129) 0.26
Aortic cross clamp time (minutes, mean ±
SD)
54 (31, 84) 53 (32, 82) 55 (31, 85) 0.73
Intraoperative blood transfusion 160 (48.1%) 66 (44%) 94 (51.4%) 0.25

1
– Categorial variables are shown as count (%). Abbreviations: BMI, body mass index; IQR – interquartile range;
NYHA – New York Heart Association; SD – standard deviation
*p values generated using chi-square and Fischer exact tests for categorical variables and student’s t-test or
Wilcoxon rank sum tests for continuous variables

15
Table 2. Distribution of primary congenital cardiac pathology among simple and complex
procedures.
1

All patients
(n=333)
Simple
(n=150)
Complex
(n=183)
p-
value
*

Primary Pathology
Atrial Septal Defect 87 (26%) 62 (41.3%) 25 (13.7%) <0.0001
Ventricular Septal Defect 36 (10.8%) 2 (1.3%) 34 (18.6%) <0.0001
Patent Foramen Ovale 13 (3.9%) 4 (2.7%) 9 (4.9%) 0.29
Patent Ductus Arteriosus 4 (1.2%) 0 (0) 4 (2.2%) 0.07
Coarctation of Aorta 15 (4.5%) 15 (10%) 0 (0) <0.0001
Tetralogy of Fallot 43 (12.9%) 2 (4.7%) 41 (22.4%) <0.0001
Ebstein’s Anomaly 9 (2.7%) 0 (0) 9 (4.9%) 0.006
Hypoplastic Left Heart Syndrome 1 (0.3%) 0 (0) 1 (0.6%) 0.36
Transposition of Great Vessels 7 (2.1%) 0 (0) 7 (3.8%) 0.015
Truncus Arteriosus 1 (0.3%) 0 (0) 1 (0.6%) 0.36
Double Outlet Right Ventricle 6 (1.8%) 0 (0) 6 (3.3%) 0.025
Bicuspid Aortic Valve 88 (26.4%) 66 (44%) 22 (12%) <0.0001
Mitral Valve Prolapse 3 (0.9%) 3 (2%) 0 (0) 0.055
Anomalous Origin of Coronary Artery 40 (12%) 4 (2.7%) 36 (19.7%) <0.0001
Operative Classification <0.0001
Left Ventricular Outflow Tract 92 (27.6%) 68 (45.3%) 24 (13.1%)
Right Ventricular Outflow Tract 70 (21%) 0 (0) 70 (38.3%)
Left to Right Shunt 111 (33.3%) 66 (44%) 45 (24.6%)
Single Ventricle 7 (2.1%) 0 (0) 7 (3.8%)
Coronary Abnormalities 39 (11.7%) 6 (4%) 33 (18%)
Descending Aorta 10 (3%) 10 (6.7%) 0 (0)
Other 4 (1.2%) 0 (0) 4 (2.2%)
1
– Categorial variables are shown as count (%).
*p values generated using chi-square and Fischer exact tests for categorical variables and student’s t-test or
Wilcoxon rank sum tests for continuous variables
16
Table 3. Comparison of postoperative and long-term outcomes based on operative complexity.
1

Variable All patients
(n=333)
Simple
(n=150)
Complex
(n=183)
p-
value
*

Perioperative
Stroke 5 (1.5%) 1 (0.7%) 4 (2.2%) 0.26
Pacemaker Insertion 19 (5.7%) 5 (3.3%) 14 (7.7%) 0.09
Bleeding 20 (6%) 9 (6%) 11 (6%) 0.44
Renal failure requiring dialysis 7 (2%) 3 (2%) 4 (2.2%) 0.98
Arrhythmia 73 (21.9%) 30 (20%) 43 (23.5%) 0.44
Blood transfusion 26 (7.8%) 11 (7.3%) 15 (8.2%) 0.95
Reoperation within 30 days 24 (7.2%) 10 (6.7%) 14 (7.7%) 0.62
Operative mortality 1 (0.3%) 0 (0) 1 (0.6%) 0.37
Mortality within 30 days 8 (2.4%) 1 (0.7%) 7 (3.8%) 0.06
ICU Length of stay (days, median, IQR) 2 (2, 3) 2 (1, 3) 2 (2, 4) 0.14
Length of stay (days, median, IQR) 6 (5, 9) 6 (5, 8) 6 (5, 10) 0.08
Long-term Outcomes
Follow-up time (months, median, IQR) 39 (13, 69) 36 (13, 67) 42 (12, 72) 0.75
Overall mortality 15 (4.5%) 3 (2%) 12 (6.6%) 0.11
Reinterventions 16 (4.8%) 8 (5.3%) 8 (4.4%) 0.68
Aortic valve replacement 4 (25%) 4 (50%) 0 (0)
Mitral valve replacement 2 (12.5%) 0 (0) 2 (25%)
Pulmonary valve replacement 2 (12.5%) 0 (0) 2 (12.5%)
Aortic surgery 4 (25%) 4 (50%) 0 (0)
Ventricular assist device 1 (6.3%) 0 (0) 1 (12.5%)
Arterial switch operation 2 (12.5%) 0 (0) 2 (25%)
Pulmonary artery stent placement 1 (6.3%) 0 (0) 1 (12.5%)
1
– Categorial variables are shown as count (%). Abbreviations: ICU: Intensive care unit; IQR – interquartile range
*p values generated using chi-square and Fischer exact tests for categorical variables and student’s t-test or
Wilcoxon rank sum tests for continuous variables
17
Table 4. Mortality Summary.
1

ID
Age
(Y)
Procedure
Classification
Initial Congenital
Diagnosis
Procedure performed
at Adult Hospital
Surgeon Surgical History Cause of Death
Days to
Death
1 20 C
Shone’s
Syndrome
Bentall, MVR, TVr CCS
Ross-Konno,
Prosthetic MVR,
multiple aortic
dilatation procedures
Unknown 214
2 78 S
Secundum ASD,
MV endocarditis
Pericardial patch
closure of ASD, MVr,
Maze
CCS BAV replacement Unknown 68
3 24 C TGA
Pulmonary artery
banding
CCS Mustard procedure
Thrombotic
complications
after ASO
1024
4 79 S ASD
AVR, MVr, Maze,
CABG, Pericardial
patch closure of ASD
CCS None
Bronchogenic
cancer metastases
845
5 39 C
Williams
syndrome with
SAS
Bentall CCS
Proximal aortic
replacement
Diffuse cerebral
edema
7
6 60 C
Right-left shunt
VSD, MR, TR
MVR, TVr, Dacron
patch closure of VSD
CCS None Septic shock 876
7 45 C
Hypoplastic right
ventricle with TA
Fontan revision CCS
Fontan and Glenn
procedures
MOSF 18
8 26 C TOF
PVR, Distal ascending
aortic repair
CCS
Blalock-Taussig
shunt, Failed PA
stenting requiring
RVOT
reconstruction
LV dysfunction,
CV collapse
13
9 66 S BAV AVR, PVR CCS
Ross procedure 25
years prior
Unknown 21
10 30 C TGA
RVOT revision, PVR,
ascending aortic
repair, VSD closure
CCS ASO MOSF 2
18
11 68 C
Turner syndrome
with vascular
ring
Ascending aortic
replacement and
vascular ring repair
CCS None
Diffuse anoxic
brain injury
25
12 25 C TGA
Orthotopic cardiac
transplantation
CCS
ASO via Senning
operation
Transplant
rejection
939
13 29 C
Pulmonary
atresia, VSD
RVOT reconstruction
(Redo Rastelli)
CCS
Blalock-Taussig,
Rastelli, RV-PA
conduit
Septic shock 130
14 39 C
Endocardial
cushion defect,
BAV
MVR, AVR, VSD CCS
Multiple AV and
MV repairs
Cardiogenic
shock, MOSF
4
15 23 S
DiGeorge
syndrome, IAA
type B
Aortic root
replacement, PA
conduit exchange,
PVR
CCS
Ross procedure,
aortic arch repair
Coagulopathy 0
1
–Abbreviations: C: Complex; S: Simple; ASD: atrial septal defect; MV: mitral valve; MVR: mitral valve replacement; MVr: mitral valve repair; TVr: tricuspid
valve repair; TR: tricuspid regurgitation; BAV: bicuspid aortic valve; TGA: transposition of great arteries; ASO: arterial switch operation; CABG: coronary
artery bypass surgery; SAS: Supravalvular aortic stenosis; TA: tricuspid atresia; TOF: tetralogy of Fallot; LV: left ventricle; CV: cardiovascular; PVR:
pulmonary valve replacement; RVOT: right ventricular outflow tract; MOSF: multi-organ system dysfunction; RV: right ventricle; PA: pulmonary artery; IAA:
interrupted aortic arch; AV: aortic valve; CCS: congenital cardiac surgeon
19
Table 5. Multivariable logistic regression model to identify predictors of mortality within 30
days of cardiac procedure
1

Variable 30-day mortality
OR (95% CI) p-value
Age 1.03 (0.98-1.09) 0.16
Sex (male) 4.83 (0.84-27.81) 0.08
Body mass index 1.08 (0.98-1.19) 0.10
Preoperative CHF 4.99 (1.02-24.45) 0.047
Redo operation 17.08 (1.71-170.60) 0.016
Complex Operative Complexity 2.84 (0.30-26.70) 0.36
1
–Abbreviations: CHF: congestive heart failure; OR – odds ratio; CI – confidence interval

20

Table 6. Multivariable? Proportional hazards analysis for risk factors for overall mortality

Variable Overall mortality
HR (95% CI) p-value
Age (per 10-year increase) 1.48 (1.06-2.07) 0.023
Sex (male) 1.46 (0.48-4.47) 0.50
Non-elective Procedure 3.71 (0.81-17.1) 0.09
Preoperative CHF 5.67 (1.81-17.7) 0.003
Redo operation 4.27 (1.14-15.9) 0.031

Complex Operative complexity 1.50 (0.36-6.28) 0.58

1
–Abbreviations: CHF: congestive heart failure; HR – hazard ratio; CI – confidence interval

21

Supplemental Table 1. Distribution of simple and complex congenital cardiac procedures by
year of surgery.

Year of Surgery All procedures Simple procedures Complex procedures
2001 1 1 0
2004 1 1 0
2005 2 0 2
2006 6 3 3
2007 6 2 4
2008 13 7 6
2009 14 7 7
2010 29 11 18
2011 21 7 14
2012 24 9 15
2013 17 9 8
2014 27 13 14
2015 33 13 20
2016 37 16 21
2017 43 20 23
2018 39 20 19
2019 20 11 9
Total 333 150 183

22

Supplemental Table 2. Confidence intervals for Kaplan Meier survival figure.
1

1 year
(95% CI)
3 year
(95% CI)
5 year
(95% CI)
Entire cohort 0.97 (0.94-0.98) 0.95 (0.92-0.97) 0.95 (0.92-0.97)
Simple cohort 0.98 (0.94-0.99) 0.97 (0.92-0.99) 0.97 (0.92-0.99)
Complex cohort 0.96 (0.92-0.98) 0.93 (0.88-0.96) 0.93 (0.88-0.96)

1
– Abbreviations: CI – Confidence Interval

23

Figure Legends
Figure 1. Classification of ACHD patient using a complexity-based classification system.

Figure 2. Kaplan Meier survival curve of entire cohort of ACHD patients.

Figure 3. Kaplan Meier survival curve of ACHD patients based on operative complexity.

Video Legend
Video of repair of a large Secundum atrial septal defect (2.7cm) using a glutaraldehyde-treated
pericardial patch in a 57-year-old female after failed device closure due to insufficient rim.

24

Figure 1.

25

Figure 2. Kaplan Meier survival curve of entire cohort of ACHD patients

26

Figure 3. Kaplan Meier survival curve of ACHD patients based on operative complexity

Abstract (if available)

Abstract Objective: The prevalence and heterogeneity of adults with congenital heart disease are rapidly increasing. The optimal perioperative environment in which to provide surgical care for these patients remains controversial. We used a complexity-based classification system to review outcomes for adult congenital heart surgery patients receiving care in a free-standing adult hospital. ❧ Methods: From 2005 – 2019, 333 adults (age ≥18 years) underwent surgical correction of a congenital cardiac defect at a single adult center. Two surgeons with formal congenital heart surgery training performed 90% of procedures (n=300). Operations were classified into “simple” and “complex” procedures. Thirty-day outcomes were compared using logistic regression while overall survival was compared using Kaplan Meier methods and Cox proportional hazard regression. ❧ Results: Thirty-day mortality was 0.67% (n=1) in the simple and 3.8% (n=7) in the complex cohort (p=0.06). After multivariable adjustment, reoperation was the only predictor of 30-day mortality (odds ratio (OR) 17.1, confidence interval (CI) 1.7-170, p=0.02), while operative complexity was not (OR 2.84, 95% CI 0.30-26.7, p=0.36). Kaplan-Meier survival at 1, 3, and 5 years was 98.6, 97.5, and 97.5% in the simple cohort and 96.1, 93.5, and 93.5% in the complex cohort

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Asset Metadata

Creator Elsayed, Ramsey S. (author)

Core Title Contemporary outcomes for adult congenital heart surgery in an adult tertiary care hospital

School Keck School of Medicine

Degree Master of Science

Degree Program Applied Biostatistics and Epidemiology

Publication Date 12/12/2020

Defense Date 12/10/2020

Publisher University of Southern California (original), University of Southern California. Libraries (digital)

Tag cardiac surgery,cardiovascular disease,congenital heart defects,OAI-PMH Harvest

Language English

Contributor Electronically uploaded by the author (provenance)

Advisor Bowdish, Michael E. (committee chair), Franklin, Meredith (committee member), Mack, Wendy J. (committee member)

Creator Email relsayed@usc.edu

Permanent Link (DOI) https://doi.org/10.25549/usctheses-c89-405585

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Type texts

Source University of Southern California (contributing entity), University of Southern California Dissertations and Theses (collection)

Access Conditions The author retains rights to his/her dissertation, thesis or other graduate work according to U.S. copyright law. Electronic access is being provided by the USC Libraries in agreement with the a...

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