CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
AFRICA
265
Aim:
To analyse the clinical course and echocardiographic features
of isolated left ventricular non-compaction (LVNC) in children.
Methods:
The study group consisted of 16 patients. Clinical evalu-
ation, chest X-ray, ECG, Holter ECG and echocardiography were
performed. For statistical analysis Student’s t test was used.
Results:
The age at diagnosis was 2 weeks to 18 years (average
6.44 years), follow-up 2 months to 9 years (mean 3.15 years). In
two brothers Barth syndrome was recognised. Signs of acute heart
failure occurred in 4 children. Chest X-ray revealed cardiomegaly in
7 with features of pulmonary congestion in 2. The ECG was within
normal limits in 8, and detected signs of LV hypertrophy in 3, 1st
degree atrio-ventricular block in 1, QTc prolongation in 1, ST-T
abnormalities in the form of fluttering and/or biphasic T in 3. In 5
patients arrhythmia was diagnosed. In all the evidence of 2-layered
myocardium with prominent trabeculations was diagnosed by ECHO,
the non-compacted to compacted ratio ranged from 1.6 to 3:1 (mean
2:1). Dilated left ventricle was found in 8 patients, reduced systolic
function in 7 (EF 22% to 57%). The cardiac function progressively
improved during follow-up in all patients. The mean age of patients
with impaired left ventricular function was 5.9 months and the
non-compacted to compacted ratio was 2.09:1; in those without left
ventricular dysfunction the mean age was 60.8 months (
p
<
0,05) and
the ratio 1.93 (
p
>
0,05). Pharmacological treatment with ACEI and
spironolacton was used in 8 patients and digoxin in 4 of them.
Conclusions:
1. The clinical course of severe heart failure and
significant systolic dysfunction is observed in infants with LVNC. 2.
Progressive improvement of the left ventricular function with age is
characteristic for LVNC. 3. The non-compacted to compacted ratio
has no impact on the left ventricular dysfunction.
1773: PAEDIATRIC CARDIOVERTER-DEFIBRILLATOR
IMPLANTATION: STILL A CHALLENGE BUT SAFE AND
FEASIBLE
Fabrizio De Rita
1
, Luca Tomasi
2
, Luca Barozzi
1
, Alessandra
Cristofaletti
2
, Lucia Rossetti
2
, Corrado Vassanelli
2
, Giovanni Battista
Luciani
1
1
Division of Pediatric Cardiac Surgery, University of Verona, Italy
2
Division of Cardiology, University of Verona, Italy
Background:
Implantable cardioverter defibrillator
(ICD) implanta-
tion in children remains a challenge because of their limited body
surface area (BSA), making implantation of percutaneous leads
difficult, and because of adhesions generated by epicardial patches,
which may interfere with normal development of the heart muscle. A
new technique of subcutaneous AICD implantation in children with
defibrillation coil is reported.
Methods:
With the patient supine decubitus, a left anterolateral thora-
cotomy is performed. The pericardium is opened and an epicardial
bipolar electrode for sensing is placed on the left ventricle. The sens-
ing wire is tunnelled subcutaneously and connected to the generator,
placed in an abdominal box pocket. A stimulating lead linked to the
generator is then tunnelled subcutaneously from the pocket to the
back, with the distal end of the coil placed medially to the left scapula.
Results:
The technique was successfully applied in 2 children (age
14 and 12 years at implantation) affected by LQTS1 and hyper-
trophic cardiomyopathy respectively, with hisotry of resuscitation for
cardiac arrest. Early after procedure, good values of sensing, pacing
impedance, and excellent pacing threshold were found in both cases.
Furthermore, the defibrillation threshold was optimal in both cases
(14 J). The AICD-PM setting parameters were: brady therapy VVI 40
bpm and tachy therapy with a single VF-window from 194 bpm. At
follow-up of 9 and 23 months respectively, electrical parameters and
position of subcutaneous lead remained stable. No events of inap-
propriate shocks were recorded during follow-up.
Conclusions:
Placement of AICD with a surgical approach, epicar-
dial pacing and sensing electrode and subcutaneous defibrillation
coil, is a feasible, safe and effective procedure in paediatrics. This
approach ensures better functioning of the device over time and does
not interfere with child growth. Key to success is regular patient
follow-up to ensure constant adjustment of sensing parameters for
their growth in order to avoid inadequate shocks.
1777: PHARMACOLOGICAL TREATMENT AND LONG-
TERM OUTCOME IN PATIENTS WITH LONG QT
SYNDROME
Beata J Pietrucha
1
, Maciej Pitak
1
, Beata Zaluska-Pitak
1
, Jacek
Kuzma
1
, Jolanta Oko-Lagan
1
, Agata Sulik
1
, Artur Pietrucha
2
, Andrzej
Rudzinski
1
1
Children’s Cardiology Department, Children’s University Hospital,
Jagiellonian University, Krakow, Poland
2
Coronary Disease Department, Jagiellonian University Medical
College, Krakow, Poland
Methods:
We observed 17 children at the age from 6 months to
17.5yrs (mean age 10 yrs), 10 girls and 7 boys, with diagnosed long
QT syndrome. Patients (pts) were divided into 2 groups: group I – 11
pts (7 girls, 4 boys) with symptomatic long QT syndrome (syncope,
dyspnoea); group II – 6 pts (3 girls and 3 boys) with prolongation
of QTc segment on ECG but without any clinical symptoms. All pts
were in long-term follow-up from 19 months to 7 years (mean obser-
vation time 3.5 yrs). Standard 12-lead ECG with assessment of QT
interval, corrected QT interval according to Bazett formula and QTc
dispersion were done at the beginning and the end of observation
period; 24-hour Holter ECG monitoring with QT evaluation was also
performed in all pts. All pts from group I were administered beta-
blockers (propranolol or metoprolol) with a mean dose of 1.2 mg/kg.
Mean standard ECG QTC duration in pts in group I was significantly
higher than in group II both in the beginning and at the end of the
observation period (471.82 vs 435 ms and 460.91 vs 423.33 ms). A
similar result was observed in Qtc duration in Holter monitoring (535
vs 455 ms; 515.91 vs 452.5 ms).There were no significant differences
between QTc duration before and after beta-blocker treatment in
group I (471.82 vs 460.91 ms) although frequency and intensity of
symptoms in this group of pts was expressively diminished. Three pts
(27.3%) from group I were referred for ICD implantation.
Conclusions:
Beta-blockers diminished clinical symptoms in long
QT syndrome. Syncope can be an evident risk factor of sudden
cardiac death (SCD) in children. Pharmacological treatment could
decrease the SCD risk in children with long QT syndrome.
1778: PARTIAL ANOMALOUS CONNECTION OF PULMO-
NARY VEINS: ANATOMICAL VARIANTS, SURGICAL
TREATMENT AND DEVELOPMENT
Jorge Luis Cervantes Salazar, Samuel Ramírez Marroquín, Alfonso
Buendía Hernández, Juan Calderón-Colmenero, Lizbeth Gómez
Martínez
Instituto Nacional de Cardiología, Mexico
Introduction (objective):
To spread the institute’s experience in the
surgical treatment of partial anomalous connection of pulmonary
veins (PACPV) and its mid-term results in terms of morbidity and
mortality.
Method:
A retrospective, longitudinal, observational, descriptive
study was undertaken of all patients with PACPV undergoing surgical
correction, in the period between January 2000 and December 2010.
Results:
Of all 86 patients, 62.5% were male, the average age was
19 years, and the vast majority (91%) were in functional class I, II
of NYHA (New York Heart Association). The most common variant
of PACPV was two vessels (64.28%), the surgical technique used the
most was redirecting the flow through pericardium (66%), followed
by the Warden technique (19.6%), and finally direct reimplantation
of the anomalous vein (14.2%). There were 3 deaths in the early post-
operative period; there were no deaths in late follow-up.
Conclusions:
Surgical results of PACPV at our institution were satis-
factory in terms of morbidity and mortality, since rates are low and
are within the worldwide average. The mid-term prognosis is satis-
factory among the remaining patients in NYHA functional class I.
