· web viewcava, asplenia and total anomalous pulmonary venous return (tapvr). characteristics...
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SUPPLEMENTAL MATERIALS
I. Supplemental Methods
Data Collection
Data collection included demographics, sex, race/ethnicity, cardiac and non-cardiac diagnoses,
cardiac and non-cardiac surgeries, morbidities (including infections, thrombosis, intestinal
perforation or volvulus and neurovascular complications), and mortality or need for transplant.
Descriptions of heart anatomy and morphology, cardiac function, bronchial pattern, and
positioning of the liver and spleen were collected from the following: records of chest X-ray
(CXR), echocardiography, cardiac catheterization with angiography, chest CT, cardiac MRI,
surgical reports and necropsy. Systemic ventricular function was assessed primarily by
echocardiography. For echocardiograms, the systemic ventricular function was designated from
the clinical report; any dysfunction (including mild, moderate, or severe) was characterized as
abnormal for this study. Undocumented arrhythmias reported in the clinical record were included
if they were treated.
Medical records including narrative history, ECGs, Holter monitors, exercise testing and
inpatient telemetry, electrophysiological studies, operative notes, and interrogations from
pacemakers (PMs) and implantable cardioverter defibrillators (ICDs) were reviewed for
documented arrhythmias.
Heterotaxy Classification
Heterotaxy cases were classified as right atrial isomerism (RAI) subtype, (also known as
asplenia syndrome) or left atrial isomerism (LAI) subtype (also known as polysplenia
syndrome).1 Classification schemes were adapted from prior publications.2-5 RAI and LAI
assignments were primarily based on atrial morphology, bronchial and/or lung anatomy. When
these indicators were unavailable or unclear, RAI was considered probable if ≥2 features typical
of RAI were present. These included abdominal juxtaposition of the aorta and inferior vena
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cava, asplenia and total anomalous pulmonary venous return (TAPVR). Characteristics used for
LAI classification included polysplenia, interruption of the inferior vena cava with azygos vein
continuation, and biliary atresia. LAI was considered probable if at least one of these features, in
addition to structural heart disease, was present. Although malformations associated with HS
may cluster and fall into either the RAI or LAI subtypes they can also overlap and defy
classification schemes.6,7 As such, patients with indeterminate atrial morphology or overlapping
RAI and LAI features were considered to have indeterminate/mixed type atrial isomerism.
Arrhythmia Classification
Clinically significant tachyarrhythmias were defined as: 1) hemodynamically significant or
requiring treatment with pharmacologic agents, temporary pacing wires (TPWs) or electrical
cardioversion/defibrillation; 2) nonsustained (≥ 3 beats, <30-s duration) or sustained (≥30-s
duration) ventricular tachycardia (VT) and 3) sustained (≥30-s duration) supraventricular
tachycardia (SVT). SVT was further categorized into ectopic atrial tachycardia (EAT), atrial
flutter and/or fibrillation (AFF), junctional tachycardia (JT), re-entrant SVT and SVT not
otherwise specified (NOS) when the distinction between EAT and re-entrant SVT could not be
made.
Tachyarrhythmia substrate definitions were adapted from prior publications.8,9
Recognizing that HS patients often have ≥1 baseline primary atrial rhythm with P wave
morphologies different from classic sinus, a diagnosis of EAT was made if there was an acute
change in P wave morphology during tachycardia at a rate ≥20% faster than the preceding
rate.8 Other supportive diagnostic features included varying tachycardia cycle length and
persistent atrial tachycardia in the setting of atrioventricular (AV) block following vagal
maneuvers or adenosine, as well as a P wave morphology in tachycardia different from those
documented on pre-existing 12-lead ECGs. JT was defined as tachycardia originating from the
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atrioventricular junction at a rate >95th percentile of heart rate for age; junctional rates ≤95th
percentile were reported as accelerated junctional rhythm (AJR).9
Additional rhythm disturbances noted, regardless of whether or not there was a history of
SVT included 1) ventricular pre-excitation on ECG (Wolff-Parkinson-White syndrome [WPW])
and 2) the presence of twin AV nodes (AVNs) characterized by two discrete QRS morphologies
without ventricular pre-excitation during sinus or baseline atrial rhythm on ECG or Holter
monitoring.
II. Supplemental Results
Automatic Tachycardias: Junctional Tachycardia
Of the 21 occurrences of junctional arrhythmias, nearly all (90%) occurred in the immediate
postoperative setting: 3 Blalock Taussig Shunts (BTS), 10 Fontans, 4 bidirectional Glenns
(BDGs), and 2 Rastellis. Management included cooling, sedation, atrial overdrive pacing
(n=10), and antiarrhythmic medications (n=11), including amiodarone, procainamide, or beta
anti-adrenergic blocker. At last follow-up encounter, only one of the perioperative cases
continued to have breakthrough JT as an outpatient and underwent an epicardial PM in
conjunction with amiodarone therapy for rhythm control. Notably, all but two patients with JT
had single ventricle substrates. The only anatomical factor associated with JT by univariate
analysis was ≥ moderate atrioventricular valve regurgitation (AVVR) (HR 4.28, 95% CI 1.72-
10.62, Online Supplemental Table S3).
Re-entrant supraventricular tachycardias
Twenty-four patients had a typical re-entrant SVT, 14 of whom underwent an electrophysiology
study (EPS): 4 had an accessory pathway (AP) mediated tachycardia, 2 had AV node re-entry
through a single AV node, 7 had AV node re-entry utilizing two AV nodes and 1 had His re-
entry. In only half of these patients was ablation successful without recurrence. Among the
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remaining 10 patients who did not have an EPS, 2 had ECG findings at baseline and during
SVT consistent with twin AVNs. The only anatomical factor associated with re-entry SVT by
univariate analysis was functional single ventricle (SV) physiology (HR 7.55, 95% CI 1.02-56.03,
Online Supplemental Table 3).
Twenty-five patients had AFF at a median age of 15 (IQR 6, 16) years. After excluding 4
AFF cases that occurred in infants < 1 year, the median age of onset shifted to 19 (IQR 8, 29)
years. Among all AFF patients, 80% had SV anatomy. Echo data available on 18 of 25 patients
at the time of AFF occurrence demonstrated normal ventricular function in 10. While 6 patients
had AFF in the perioperative setting, 75% presented with symptoms as outpatients at a median
age of 20 (IQR 10, 27) years. Half of those with AFF underwent PM implantation (including 2
ICDs for concomitant VT): 2 for sinus node dysfunction and symptomatic bradycardia preceding
onset of AFF, 9 as part of their antiarrhythmia management (either for symptomatic bradycardia
or to allow uptitration of antiarrhythmics), and one for neonatal complete AV block.. Three
patients had MAZE procedures performed. A majority of patients (n=20) were on an
antiarrhythmic at last follow-up. On univariate analysis, AFF was associated with ≥ moderate
AVVR (Table 4, HR 2.94, 95% CI 1.27-6.80).
SVT NOS
Seventeen patients had SVTs of undetermined etiology; a majority (n = 10) occurred in the
postoperative setting, 4 as outpatients following BDG (n=3) or Fontan (n=1), and 3 prior to any
surgery. Two patients, including one who had ECG findings consistent with twin AVNs,
underwent EPS prior to their Fontan palliation. Neither had inducible SVT during their EPS, nor
evidence of an AP; ablations were not attempted. At last follow-up, 11 of 17 were on an
antiarrhythmic.
Ventricular arrhythmias
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Ventricular arrhythmias occurred in 23 patients at a median age of 3 years (IQR 2 mo-16 yrs): 1
had a post-operative ventricular fibrillation (VF) arrest, 5 had sustained VT and 17 had NSVT.
Except for one, all sustained VT and VF episodes occurred in the perioperative setting among
SV patients. Among the 4 SV patients with sustained VT, the 2 with severe ventricular
dysfunction prior to surgery had poorer outcomes – 1 death, 1 transplant – than the 2 with
normal preoperative function. The latter were successfully weaned off antiarrhythmics without
subsequent VT recurrence. The remaining patient with sustained VT had a Rastelli repair prior
to VT onset. With an ICD and medical therapy, his VT is partially controlled; his ICD discharges
appropriate shocks infrequently.
The majority (14/17) of patients with NSVT had single ventricles. Among these, two
thirds (9/14) of cases were detected via routine Holter surveillance in the outpatient setting and
70% (n=10) of cases had normal ventricular function at the time of detection. Function at time
of detection, however, did not appear to correlate with overall outcomes; 40% (n=4) of those
with normal function later died or underwent transplant compared with 50% (n=2) of those with
ventricular dysfunction. In contrast, no patients with biventricular repairs and NSVT died. By
univariate analysis, there were no significant anatomical factors associated with VT (Table 4).
EPS with programmed ventricular stimulation was performed in 3 patients with single
ventricles and NSVT: 1 patient had inducible VT and underwent ICD implantation; the other 2
did not have inducible VT and remain on antiarrhythmic therapy.
Atrioventricular block
Characteristics of 22 patients with AV block, including 3 patients with acquired surgical AV block
are described in Online Supplemental Table S4. The majority (67%) with congenital complete
atrioventricular block (CCAVB) were prenatally diagnosed; the remaining 5 were diagnosed at
birth. Four patients had progressive hydrops prompting delivery, two of which were pre-term.
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Both premature infants (30 and 34 weeks) died shortly after delivery. All but one of the
remaining 13 patients had PMs successfully implanted within the first month of life (the
exception was a relatively asymptomatic patient with CCAVB, asplenia syndrome, s/p VSD
repair in infancy who was referred at age 3 for progressive bradycardia and PM implantation).
Of the 4 patients with high grade AVB, 2 were diagnosed at birth with intermittent
complete AVB and had pacemakers implanted by 2 weeks of age. The other two patients
developed conduction disease after surgical palliations: one between the Glenn and Fontan
palliations, and the other after palliation (i.e. BTS) for duct dependent pulmonary circulation.
III. Supplemental Discussion
In this review of 337 patients with heterotaxy syndrome (HS), more than half (51%) were
Hispanic. Compared to the demographic composition of inpatient admissions with congenital
heart disease at our center (Hispanic 36%, Non-Hispanic White 36%, Non-Hispanic Black 14%,
Other 14%),10 there was an increased representation by Hispanics in our heterotaxy cohort. This
finding echoes those from a recent population-based epidemiologic study on HS in Texas by
Lopez et al.11 Utilizing Texas Birth Defects Registry data from 1999—2006, Lopez et al found an
increased prevalence of HS in Hispanics (57.5% of their cohort). The increased incidence of
heterotaxy among Hispanic infants has also been reported in other national12 and regional
studies (in New York13 and Nevada14). This Hispanic predominance in heterotaxy patients
suggests that the interaction between environmental and genetic variants plays an important
role in HS.
IV. Supplemental References
1. Van Praagh, S. Cardiac Malpositions and the Heterotaxy Syndromes in Keane JF, Lock JE, Fyler DC. ed., Nadas' Pediatric Cardiology 2006. W B Saunders Company; 2006; p.675–697
2. Lim HG, Bacha EA, Marx GR, Marshall A, Fynn-Thompson F, Mayer JE, Del Nido P, Pigula FA: Biventricular repair in patients with heterotaxy syndrome. The Journal of Thoracic and Cardiovascular Surgery 2009; 137:371–379.e373.
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3. Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM: Management and outcomes of right atrial isomerism: a 26-year experience. Journal of the American College of Cardiology 1998; 31:1120–1126.
4. Gilljam T, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM: Outcomes of left atrial isomerism over a 28-year period at a single institution. Journal of the American College of Cardiology 2000; 36:908–916.
5. Lim JSL, McCrindle BW, Smallhorn JF, Golding F, Caldarone CA, Taketazu M, Jaeggi ET: Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes. Circulation Lippincott Williams & Wilkins, 2005; 112:2454–2461.
6. Van Praagh R, Van Praagh S: Atrial isomerism in the heterotaxy syndromes with asplenia, or polysplenia, or normally formed spleen: an erroneous concept. AJC 1990; 66:1504–1506.
7. Anderson C, Devine WA, Anderson RH, Debich DE, Zuberbuhler JR: Abnormalities of the spleen in relation to congenital malformations of the heart: a survey of necropsy findings in children. Br Heart J 1990; 63:122–128.
8. Shamszad P, Cabrera AG, Kim JJ, Moffett BS, Graves DE, Heinle JS, Rossano JW: Perioperative atrial tachycardia is associated with increased mortality in infants undergoing cardiac surgery. The Journal of Thoracic and Cardiovascular Surgery The American Association for Thoracic Surgery, 2012; 144:396–401.
9. Collins KK, Van Hare GF, Kertesz NJ, et al.: Pediatric Nonpost-Operative Junctional Ectopic Tachycardia. Journal of the American College of Cardiology American College of Cardiology Foundation, 2009; 53:690–697.
10. Texas Inpatient Public Use Data File, 1999-2012. https://www.dshs.texas.gov/thcic/hospitals/Inpatientpudf.shtm
11. Lopez KN, Marengo LK, Canfield MA, Belmont JW, Dickerson HA: Racial disparities in heterotaxy syndrome. Kirby R, Browne M, eds: Birth Defects Research Part A: Clinical and Molecular Teratology 2015; 103:941–950.
12. Lin AE, Ticho BS, Houde K, Westgate M-N, Holmes LB: Heterotaxy: Associated conditions and hospital-based prevalence in newborns. Genetics in Medicine Nature Publishing Group, 2000; 2:157–172.
13. Rigler SL, Kay DM, Sicko RJ, Fan R, Liu A, Caggana M, Browne ML, Druschel CM, Romitti PA, Brody LC, Mills JL: Novel copy-number variants in a population-based investigation of classic heterotaxy. Genetics in Medicine 2014; 17:348–357.
14. Evans WN, Acherman RJ, Restrepo H: Heterotaxy in Southern Nevada: Prenatal Detection and Epidemiology. Pediatr Cardiol Springer US, 2015; 1–5.
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V. Supplemental Figures
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50
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250 237
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Number of Tachyarrhythmias
Num
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f Pat
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s
Figure S1. Number of Tachyarrhythmias in Patients with Heterotaxy Syndrome
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VI. Supplemental Tables
Table S1. Patient characteristics and arrhythmia occurrence
All No Arrhythmia Arrhythmia P valuen=337 n=208 n=129
CharacteristicsMale 178 (53%) 115 (55%) 63 (49%) 0.08Race/Ethnicity Hispanic NH White NH Black Other
171 (51%)106 (31%)37 (11%)23 (7%)
114 (55%)62 (30%)19 (9%)13 (4%)
57 (44%)44 (34%)18 (14%)10 (8%)
0.47
Age at HS Presentation Fetus Neonate Childhood Unknown
117 (35%)163 (48%)
24 (7%)33 (10%)
62 (30%)107 (51%)
19 (9%)20 (10%)
55 (43%)56 (43%)
5 (4%)13 (10%)
< 0.001
Biventricular anatomy 109 (32%) 68 (32%) 41 (32%) 0.73Single ventricle anatomy Single RV Single LV Undetermined SV
228 (68%)160 (47%)50 (15%)18 (5%)
140 (67%)98 (47%)34 (16%)
8 (4%)
88 (68%)62 (48%)16 (12%)10 (8%)
0.68
Isomerism TypeRAILAIUndetermined
130 (39%)121 (36%)86 (25%)
84 (40%)68 (33%)56 (27%)
46 (36%)53 (41%)30 (23%)
0.41
Pulmonary venous obstruction 77 (23%) 46 (22%) 31 (24%)
0.009
Pulmonary Venous ConnectionsPartial AnomalousTotal AnomalousNormal
52 (50%)169 (16%)116 (34%)
31 (15%)99 (48%)78 (37%)
21 (16%)70 (54%)38 (30%)
0.19
Ventricular Outflow TractsPulmonary ObstructionAortic ObstructionAortic and Pulmonary ObstructionNo Obstruction
228 (68%)39 (12%)
8 (2%)
62 (18%)
139 (67%)28 (13%)
2 (1%)
39 (19%)
89 (69%)11 (8%)6 (5%)
23 (18%)
0.02
Age at last follow-up (IQR)
7 y(2 y, 13 y)
6 y(1 y, 12 y)
9 y(4 y, 16 y)
0.07
Age at last follow-up reported as median. Relationship between age at last follow-up and arrhythmia occurrence analyzed by Independent Samples Median Test. All other analyses performed using time to event (arrhythmia) analysis; p values calculated by Cox regression.NH indicates Non-Hispanic; HS, Heterotaxy syndrome, LV, left ventricle; RV, right ventricle; RAI, right atrial isomerism, LAI, left atrial isomerism; IQR, interquartile range (25th percentile, 75th percentile); d, days; m, months; and y, years.
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Table S2. Characteristics and Onset Of Tachycardia By Type
EAT Atrial Fibrillation
Atrial Flutter
Re-entrant SVT
SVT NOS Junctional Ventricular
38 25 23 17 21 23CharacteristicMale 20 (53%) 13 (52%) 14 (61%) 9 (53%) 8 (38%) 10 (43%)Median age at onset(IQR)
3.5 m(13 d, 6 y)
15 y(6 y, 26 y)
22 m(2 m, 9 y)
8 m(9 d, 5 y)
3 y(11 m, 6 y)
3 y(2 m, 16 y)
Arrhythmia OnsetPerioperative 25 (65%) 6 (24%) 8 (35%) 10 (59%) 19 (81%) 8 (34%)Inpt hospitalization 1 (3) 0 1 (4) 0 0Outpatient 11 (29%) 18 (72%) 10 (44%) 4 (24%) 2 (10%) 12 (52%)Other 1 (3%) 1 (4%) 4 (17%) 3 (18%) 0 3 (13%)
Associated Surgical Palliation
Prior to any surgery 2 (5%) 2 (8%) 6 (26%) 4 (24%) 0 2 (9%)S/p Stage I palliation 3 (8%) 0 2 (9%) 0 0 0S/p Stage 2 palliation 6 (16%) 6 (24%) 7 (30%) 5 (29%) 5 (24%) 8 (35%)S/p Fontan 6 (16%) 7 (28%) 2 (8%) 3 (18%) 11 (52%) 2 (9%)S/p other surgical palliation
21 (55%) 10 (40%) 6 (26%) 5 (29%) 5 (24%) 11 (47%)
SVT indicates supraventricular tachycardia; NOS, not otherwise specified; IQR, interquartile range (25th percentile, 75th percentile); d, days; m, months; and y, years; inpt, inpatient.
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Table S3. Univariate Analysis of Patient Factors by Tachyarrhythmia Type
EAT
N = 38
Atrial Fibrillation Atrial Flutter
N = 25
Re-entrant SVT
N = 23
SVT NOS
N = 17
Junctional
N = 21
Ventricular
N = 23
HR(95% CI)
HR(95% CI)
HR(95% CI)
HR(95% CI)
HR(95% CI)
HR(95% CI)
AVVR ≥ moderate 2.08*(1.09-3.96)
2.94*(1.27-6.80)
1.30(0.56-2.99)
3.74*(1.38-10.10)
4.28*(1.72-10.62)
2.32(1.00-5.40)
Functional Single Ventricle
4.00*(1.23-13.07)
1.29(0.48-3.51)
7.55*(1.02-56.03)
5.16(0.68-38.92)
6.64(0.89-49.48)
1.68(0.57-4.97)
Pulmonary venous obstruction
5.56*(2.83-10.93)
0.43(0.06-3.30)
1.48(0.54-4.06)
4.96*(1.91-12.93)
2.46(0.92-6.53)
1.06(0.30-3.69)
Isomerism TypeLAI (ref)
RAI
Undetermined
1.00
2.76*(1.21-6.27)
2.26(0.86-5.95)
1.00
0.84(0.34- 2.10)
0.99(0.33-3.00)
1.00
2.56(0.90-7.26)
1.65(0.50-5.41)
1.00
5.21(1.14-23.81)
3.53(0.68-18.17)
1.00
1.30(0.43-3.86)
2.36(0.77-7.22)
1.00
0.58(0.21-1.57)
0.95(0.34-2.63)
Ventricular dysfunction
0.91(0.40-2.07)
1.51(0.64-3.60)
0.40(0.09-1.72)
1.94(0.68-5.51)
1.42(0.52-3.89)
1.52(0.61-3.76)
EAT indicates ectopic atrial tachycardia; SVT, supraventricular tachycardia; NOS, not otherwise specified; HR, hazard ratio; CI, confidence interval; AVVR, atrioventricular valve regurgitation; LAI, left atrial isomerism; RAI, right atrial isomerism; ref, reference..All univariate analyses performed with univariate Cox regression model. For multiple comparisons, p < 0.017 was considered significant after Bonferroni adjustment was applied. Otherwise, p < 0.05 was considered significant. * Variables achieving significant p values.
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Table S4. Characteristics of Patients with Atrioventricular Block
Total CCAVB HG AVB Surgical AVB22 15 4 3
CharacteristicMale 10 (46%) 6 (40%) 2 (50%) 2 (67%)Death or transplant 9 (41%) 6 (40%) 2 (50%) 1 (33%)Median age at diagnosis(range)
Prenatal(PN, 2 y)
Prenatal(PN, Birth)
8 d(PN, 10 m)
6 m(1 m, 2 y)
Hydrops 4 (18%) 4 (27%) 0 NAFunctional Single Ventricle
10 (46%) 7 (47%) 3 (75%) 0
Permanent PM 19 (86%) 13 (87%) 3 (75%) 3 (100%)Median Age at PM(IQR)
6 d(2 d, 1 m)
5 d (1 d, 9 d)
11 d(3 d, 3 y)*
6 m(1 m, 2 y)*
CCAVB indicates congenital complete atrioventricular block; HG, high grade; AVB, atrioventricular block; PN, prenatal; SV, single ventricle; IQR, interquartile range (25th percentile, 75th percentile); d, days; m, months; and y, years.*Expressed as range
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Table S5. Characteristics of Patients with Pacemakers
Reason for Pacemaker
Sinus Node Dysfunction and/or Sinus Bradycardia
Low Grade 2nd degree
AVB
HG AVB CCAVB Surgical AVB Tachycardia Related
Treatment
Unknown
13 3 3 13 3 15 1CharacteristicsMale 4 (31%) 2 (67%) 1 (33%) 5 (38%) 2 (67%) 8 (53%) 1 (100%)Functional Single Ventricle
6 (46%) 3 (100%) 2 (67%) 6 (46%) 0 12 (80%) 1 (100%)
ICD Upgrade - 1 (33%) - - - 2 (13%) -Median Age at Implant (IQR)
4 y(11 m, 7.5 y)
6 y(10 m, 9 y)**
11 d(3 d – 3 y)**
5 d(1 d, 9 d)
6 m(1 m, 2 y)**
9 y(5 y, 24 y)
6 m
AVB indicates atrioventricular block; HG, high grade; CCAVB, congenital complete atrioventricular block; PN, prenatal; SV, single ventricle; ICD, implantable cardioverter defibrillator; IQR, interquartile range (25th percentile, 75th percentile); d, days; m, months; and y, years.**Expressed as range
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Table S6. Arrhythmia Related Deaths
Pt Sex Race/ Ethnicity
Age at death
Isomerism Significant History
Tachyarrhythmia Deaths
1 M Hispanic 2 d RAI Post-operative narrow complex tachycardia with subsequent degeneration into terminal bradycardia. Occurred following TAPVR repair and placement of BTS.
Single ventricle.
2 F White 3 y LAI Post-operative narrow complex tachycardia with subsequent degeneration into ventricular fibrillation. Occurred following Fontan palliation with concomitant placement of a single chamber pacemaker for bradycardia.
Single ventricle.
3 F Hispanic 8 m RAI Expired in the hospital at 8 months of age with an intractable episode of atrial tachycardia.
Single ventricle. Could not be palliated beyond a BTS due to persistently elevated pulmonary vascular resistances.
4 F White 10 y LAI In hospital torsades de pointes arrest during initiation of Sotalol for persistent atrial tachycardia.
Single ventricle. Fontan palliation with progressive cyanosis from intrapulmonary arteriovenous malformations.
5 F Black 17 y LAI VF arrest occurring within 24 hours of a diagnostic catheterization. No antecedent history of arrhythmias
Unrepaired unobstructed TAPVR and primum atrial septal defect with pulmonary hypertension and mild ventricular dysfunction.
Bradyarrhythmia Deaths
6 M Black 1 d Mixed Fetal CCAVB with hydrops prompting premature delivery at 30 weeks. Single right ventricle with severe dysfunction.
7 F Hispanic 11 d LAI Fetal CCAVB with hydrops prompting premature delivery at 34 weeks.
Biventricular anatomy. Sinus venosus ASD with partial anomalous pulmonary venous return. Dysmorphic. Congenital hydrocephalus. Anomalous corpus callosum.
8 M White 17 d RAI Post-operative high grade AVB following BTS placement for ductal dependent pulmonary circulation. Died from hemodynamic instability despite placement of temporary pacing wires.
Single ventricle. Clinical sepsis.
Pt indicates patient; M, male; F, female; d, days; w, weeks; m, months; y, years; RAI, right atrial isomerism; LAI, left atrial isomerism; TAPVR, total anomalous pulmonary venous return; BTS, Blalock-Taussig shunt; CCAVB, congenital complete atrioventricular block; ASD, atrial septal defect; AVB, atrioventricular block.
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