CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
117
258: CHANGING RISK FACTORS FOR ARRHYTHMIAS
AND SUDDEN DEATH AMONG ADULTS WITH REPAIRED
TETRALOGY OF FALLOT IN THE CURRENT ERA
Peter Karpawich, Swati Sehgal, Harinder Singh, Julie Kovach
Children’s Hospital of Michigan, Wayne State University School of
Medicine, Michigan, USA
Background
: Among adults patients (pts) with repaired congenital
heart disease, those with tetralogy of Fallot (TOF) historically have
exhibited increased risk for tachyarrhythmias (Ar), AV block (AVB)
and sudden cardiac death (SCD) associated with right ventricular
(RV) systolic pressures (RVSP)
>
60 mmHg, outflow tract (OT)
gradients
>
20 mmHg and QRS duration
>
180 ms. However, surgi-
cal practices have changed over the years. The purpose of this study
was to evaluate Ar/SCD risk with symptoms, echocardiographic
(ECHO), electrophysiologic (EP), haemodynamic and RV volume on
cardiac MRI (CMRI) findings among the current generation of older
repaired TOF pts.
Methods
: TOF pts seen in our institution’s Adult and Adolescent
Congenital Heart Programs were divided into 2 groups: group I:
+Ar/SCD; group II -Ar/SCD, and correlated with current pt age,
gender, age at surgical repair, repair types (transannular patch; no
patch; conduit; shunt), ECHO, CMRI, ECG/Holter, haemodynamic
and EP results.
Results
: Of 136 pts (66 male/70 female) ages 11–58 y (mean 26),
61 (45%) had Ar (22% atrial, 60% ventricular, 10% both) and 8%
AVB. Of these, SCD occurred in 3 (5%) (group I). These pts were
chronologically older (mean 32 vs 22 y) with repair performed at an
older age (mean 50 vs 22 mo) than those without Ar/SCD (group
II) (
p
<
0.05). QRS duration (mean 158 ms) and RVSP (mean 44
mmHg) were persistent risk factors. However, there was no correla-
tion with type of surgical repair, gender, RV pressure
>
60 mmHg,
RVOT gradient
>
20 mmHg, or RV volume. Ar were induced in 91%
of group I pts studied, requiring ablation or device implant.
Conclusions
: In the current era, repaired TOF pts still remain at risk
for Ar/SCD depending on their chronological age and age at surgical
repair. However, QRS duration is shorter (158 ms) and RVSP less (44
mmHg) than previously reported. Type of repair, RV outflow gradi-
ents or CMRI volumes did not correlate with Ar/SCD.
362: SURGERY FOR ADULTS WITH CONGENITAL HEART
DISEASE: AN 11-YEAR SINGLE-CENTRE EXPERIENCE
David Yang, Laura Linnemeier, Genevieve Yedlicka, Mark Rodefeld,
Mark Turrentine, John Brown
Department of Cardiothoracic Surgery, Indiana University School of
Medicine, Indiana, USA
Background/hypothesis:
Adults with congenital heart disease
(ACHD) are increasing in number as a result of asymptomatic lesions
during childhood or improved paediatric cardiac care. Some of these
patients are not discovered in childhood, while others require subse-
quent cardiac interventions. We retrospectively reviewed the early and
late outcomes of ACHD operations/re-operations over the last decade.
Materials and methods:
Between January 2001 and December
2011, 1 044 congenital lesions were repaired during 659 surgical
operations in 538 patients (287 males). Mean age at operation was
32.5
±
13.1 (range: 18–84) years. Excluding rhythm-related prob-
lems from postoperative analysis, 200/449 (45%) were first-time
operations, and 98/449 (21.9%) had NYHA Class symptoms 3 or 4
pre-operatively.
Results:
Early and late mortality (EM) (LM) were 1.3% (
n
=
6)
and 2.9% (
n
=
13) respectively. Actuarial survival at 1, 3, 5, and 10
years was 98%, 96%, 94%, and 86% respectively. Major postopera-
tive complications occurred in 54/449 (12.0%). Re-operations were
required in 7.1% (
n
=
32). Actuarial freedom from re-operation at
1, 3, 5, and 10 years was 98%, 92%, 87%, and 70% respectively. At
mean follow-up of 33.1
±
35.7 (range: 0–137) months, 3.1% (
n
=
14) had residual Class 3 or 4 symptoms, although 4.5% (
n
=
20) had
minimal follow-up.
Conclusions:
A wide variety of CHD pathology is seen in adults.
Surgical repair can be accomplished with low mortality and morbid-
ity. Pulmonary valve, aortic valve, aortic pathologies and rhythm
abnormalities dominate the spectrum of ACHD pathology.
379: MODE OF DELIVERY AND PREGNANCY OUTCOME
IN A TERTIARY CENTRE FOR ADULT CONGENITAL
HEART DISEASE
Rafael Hirsch
1,3
, Alexander Dadashev
1,3
, Yael Schertz
2,3
, Galia Oron
2,3
,
Leonard Blieden
1,3
1
Adult Congenital Heart Unit, Department of Cardiology, Rabin
Medical Center, Israel
2
Department of Obstetrics and Gynecology, Rabin Medical Center,
Israel
3
Sackler School of Medicine, Tel Aviv University, Israel
Background
: As a result of the continuing improvement of congeni-
tal heart disease (CHD) therapy, a growing number of women are
reaching child-bearing age. Contemporary maternal risks of preg-
nancy and preferred modes of delivery are not well known.
Aim
: To review all deliveries of CHD patients in our centre since
2000, regarding mode of delivery and maternal outcome.
Results
: There were 391 deliveries. Mean age at delivery was 30.3 (
±
5.5) years, age range 17.4–48.5 years; 208 women had 1 delivery (4
twins), 58 had 2, 17 had 3 and 4 had 4.
Spontaneous deliveries occurred in 231 (59%), 94 (24%) were
by caesarean section (CS) and 66 (17%) after medical induction of
labour; 120/231 (52%) spontaneous deliveries were entirely unevent-
ful. The rest had premature rupture of membranes (26), fetal distress
(23), bleeding requiring transfusion (10), maternal arrhythmia (3),
vacuum delivery (30), forceps (6) and perineal laceration grade II or
more and/or uterine exploration.
Of 66 medical inductions of labour, 56 were for cardiac indica-
tions; 18 had shunt lesions, 17 valve disease, 10 complex transpo-
sition of the great arteries (TGA), 7 tetralogy of Fallot (TOF), 2
Fontans and 2 PFO post stroke.
Of 94 CS, 58 (62%) were elective (both term and preterm); 7
were for very high risk CHD – 4 Eisenmenger syndrome, 2 PHT,
1 TGA-Rastelli, 1 Fontan. Thirty-three CS were urgent. Two of
four Eisenmenger patients died. Two more patients died, both with
repaired DSS: one after induction and one died at home 3 days after
an uneventful delivery. There were 7 more serious complications but
with good outcome.
Conclusions
: This large single-centre series of deliveries in CHD
shows that except for Eisenmenger patients, maternal outcome is
very satisfactory with a very low cardiac complication rate in all
modes of delivery. A higher than expected rate of CS suggests that
the threshold for this intervention was lower in CHD patients.
448: SUDDEN CARDIAC DEATH IN CHILDREN AND
ADOLESCENTS: IT IS PREVENTABLE WITH A MULTI-
DISCIPLINARY, MULTI-INSTITUTIONAL APPROACH
Stuart Berger
1
, Debra Klich
1
, Robert Campbell
2
, Vickie Vetter
3
,
Joseph Marek
4
, Chris Anderson
5
, Yung Lau
6
, Deborah Schutte
7
,
Augustin Ramos
8
, Geoff Jennings
9
1
Children’s Hospital of Wisconsin, Medical College of Wisconsin,
USA
2
Children’s Health Care of Atlanta, Emory University Medical
School, Atlanta, USA
3
Children’s Hospital of Philadelphia, University of Pennsylvania,
USA
4
Midwest Heart Specialists, USA
5
Providence Center for CHD, USA
6
Children’s Hospital of UAB, University of Alabama, Birmingham,
USA
7
Cook Children’s Hospital, Texas, USA
8
Children’s Hospital of Orlando, Philadelphia, USA
9
East Tennessee Children’s Hospital, University of Tennessee, USA
