CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
256
AFRICA
lation, persistent PHT, or pulmonary haemorrhage warrants careful
evaluation for MAPCAs.
1633: PULMONARY VALVE GROWTH FOLLOWING VALVE
SPARING TETRALOGY OF FALLOT REPAIR
Adrian Dyer, Alan Nugent, Kristine Guleserian, Matthew Lemler,
Shannon Blalock, Claudio Ramaciotti, Joseph Forbess
University of Texas Southwestern Medical Center, Dallas, TX, USA
Background:
There has been increased focus on pulmonary valve
(PV) preservation in tetralogy of Fallot (TOF) repair. Valve sparing
(VS) techniques utilise a combined transatrial and transpulmonary
approach, a limited right ventriculotomy, sharp commissurotomies
and valvar/annular balloon dilation. The objective of this study is to
describe the VS TOF repair experience in terms of valve growth and
performance.
Materials and methods
: We did a retrospective review of pre-
operative echocardiograms in all patients who underwent repair of
isolated TOF from 2004 to 2012. PV annulus (PVA) dimensions were
assessed on immediate post-repair and most recent echocardiogram
studies in those undergoing a VS approach.
Results:
Ninety-seven patients were identified. Fifty-nine (61%)
underwent a VS repair at a median age of 4.1 months and 38 had a
transannular patch (TAP) at a median age of 3.5 months (
p
=
0.5).
Mean pre-operative PVA diameter was 7.4 mm (z-score, -1.7) in
the VS group versus 5.5 mm (z-score, -3.2) in the TAP group (
p
<
0.0001). In the VS group, the mean post-operative PVA was 9.7
mm (z-score of -0.2;
p
<
0.0001). Mean post-operative peak RVOT
velocity (Vmax) was 2.1 m/s and 93% had
<
mild pulmonary insuf-
ficiency (PI). Intra-operative pulmonary balloon valvuloplasty was
performed in 10 patients. In this group, mean PVA increased from
6.9 mm (z-score, -2.1) to 9.1 mm (z-score, -0.8) and post-operative
Vmax was 2.3 m/s with 80% having
<
mild PI. Nineteen patients are
followed at this institution with a mean follow-up of 3.7 years. In this
subset, the mean post-operative PVA grew from 9.6 mm to 14.9 mm
(
p
<
0.0001), while maintaining normal z-scores (-0.3 to -0.4), and no
increase in the minimal pulmonary stenosis (PS) or PI.
Conclusions:
VS TOF repair maintains the integrity of the PV and
allows ongoing annular growth with minimal PS or PI. This tech-
nique may prevent the long-term sequelae of right ventricular volume
overload secondary to chronic pulmonary insufficiency.
1643: CONCERNING OCULAR SIDE-EFFECT POSSIBLY
ASSOCIATED WITH PDE5 INHIBITOR IN AN INFANT
AFFECTED BY COMPLEX CONGENITAL HEART DISEASE
Alessandra Cristofaletti
1
, Marcella Gaffuri
2
, Maria Antonia Prioli
1
,
Caterina Mansoldo
3
, Luca Barozzi
4
, Giorgio Marchini
3
, Giovanni
Battista Luciani
4
, Paolo Biban
2
1
Department of Medicine, Section of Cardiology, University of
Verona, Verona, Italy
2
Department of Pediatrics, Pediatric and Neonatal Intensive Care
Unit, Azienda Ospedaliero, Parma, Italy
3
Eye Clinic, Department of Neurological and Visual Sciences,
University of Verona, Italy
4
Division of Cardiac Surgery, University of Verona, Verona, Italy.
We report a case of bilateral blindness possibly associated with
PDE5-inhibitor intake (sildenafil) in a 7-month-old baby affected by
a complex congenital heart defect.
Cavopulmonary anastomosis was performed at 4 months of
age for double inlet left ventricle, hypoplastic right ventricle,
bulbo-ventricular foramen and moderate subpulmonary obstruction.
The post-op period was uneventful, except for the occurrence of
chylothorax, which was treated with multiple drainages, parenteral
nutrition and diuretics. Echocardiographic evaluation showed satis-
factory results at the anastomosis level, with good left ventricle and
atrioventricular valve function. Nonetheless, intermittent episodes of
chylothorax were still present after 2 months. Thus, despite the lack
of obvious echographic signs of pulmonary hypertension, we raised
the hypothesis that a relative increase of pulmonary vascular resist-
ances might have been involved in the aetiology of the persistent
chylothorax. Then,
ex adiuvantibus
, we decided to start sildenafil at
0.2 mg/kg TDS.
Four weeks later a lack of visual focus on moving objects was
noticed, with poor pupillary light reflex. An ophthalmologic examina-
tion revealed bilateral pendular nystagmus, absent papillary reflex,
transparent crystalline lens, light pallor papilla, arterial venous tortu-
ous vessels, peripapillary retinal haemorrhages and macular exudation.
The sudden onset of visual loss associated with light pallor optic disc,
in the absence of other neurological and orbital abnormalities, prompt-
ed the diagnosis of posterior ischaemic optic neuropathy (PION).
Sildenafil was immediately suspended, while a trial with methy-
prednisolone intravenously was attempted for the treatment of PION.
One month later, complete reduction of retinal haemorrhages,
macular exudation and arterial venous tortuosity was noticed; howev-
er visual evoked potentials were absent. At one year follow-up no
visual recovery was observed, with further worsening of bilateral optic
disc atrophy. This alarming case raises questions about the safety of
sildenafil in young infants and the necessity of ongoing ophthalmo-
logical evaluations before and during therapy with PDE5 inhibitors.
1647: OVERVIEW OF PAEDIATRIC DILATED CARDIOMY-
OPATHY IN A TERTIARY HOSPITAL
Joana Miranda
1
, Esmeralda Rodrigues
2
, Teresa Vaz
1
, Elisa Leão
Teles
2
, Maria João Baptista
1
1
Pediatric Cardiology Department, Hospital S. João, Porto, Portugal
2
Metabolic Diseases Unit, Pediatric Department, Hospital S. João,
Porto, Portugal
Introduction:
Dilated cardiomyopathy (DCM) is the most common
form of cardiomyopathy in the paediatric population and an impor-
tant cause of heart transplantation in children.
Objective:
To characterise the paediatric population diagnosed with
DCM in our paediatric cardiology referral centre in the north of
Portugal.
Methods:
We performed a retrospective review of patients diagnosed
with DCM from January 2005 to June 2012, including demographic
data, clinical presentation, aetiology, treatment and outcome.
Results:
We identified 61 patients with DCM (37 female; 24 male).
Median age at diagnosis was 16 months. Heart failure was present
in 83.6% (
n
=
51) of patients and 44.3% (
n
=
27) needed intensive
care. Median left ventricular ejection fraction at clinical presentation
was 32.0% (SD 13.0%). The most prevalent causes were idiopathic
(
n
=
29 [47.5%]), viral myocarditis (
n
=
11 [18.0%]) and inherited
metabolic diseases (
n
=
7 [11.5%]). In viral myocarditis parvovirus
B19 was the most common identified agent. Anticongestive agents,
angiotensin-converting enzyme inhibitor and anti-aggregant therapy
were used in the majority of patients. Overall mortality was 16.1%
(
n
=
10) and 5 patients underwent heart transplantation. Age under 1
year was associated with higher mortality (
p
<
0.001) and increased
need of intensive care (
p
<
0.05). A higher rate of transplantation was
observed in children over 1 year of age (
p
<
0.05) and with intensive
care admission (
p
<
0.05). We report no mortality in the 5 patients
who underwent heart transplantation, after 1 year of follow-up.
Discussion:
The most important prognostic factors identified were
age at DCM diagnosis and requirement for intensive care. Although
the majority of patients had an idiopathic form of DCM, a significant
number of patients presented with inherited metabolic diseases. This
reinforces the importance of considering them in the differential
diagnosis of paediatric DCM, as an appropriate approach to inherited
metabolic diseases could alter the outcome of affected patients.
1655: THE EFFECT OF SPIRONOLACTONE, FUROSEMIDE
AND CAPTOPRIL ON BLOOD ELECTROLYTE LEVELS IN
CONGENITAL HEART DEFECT INFANTS WITH UNCON-
TROLLED CONGESTIVE HEART FAILURE
