CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
26
AFRICA
Methods:
Forty-five children with a typical history of VVS were
enrolled as a study group, among them, 38 patients had a positive
response to HUTT while seven had a negative test. Twenty healthy
children with similar characteristics without syncope were matched
as a control group. HRV was calculated over a 24-hour period for the
time-domain indices and frequency-domain indices.
Results:
The study group had lower time-domain indices of SDNN
(
p
<
0.01), SDANN (
p
<
0.05), and higher frequency-domain indi-
ces of LF (
p
<
0.05) and LF/HF (
p
<
0.01) than the control group.
There were no significant differences in time-domain and frequency-
domain indices between HUTT+ VVS patients and HUTT– VVS
patients. VVS patients with vasodepressor responses had lower
SDNN (
p
<
0.01), SDANN (
p
<
0.05), rMSSD (
p
<
0.05) and PNN50
(
p
<
0.05) and higher frequency-domain indices of LF (
p
<
0.05) and
LF/HF (
p
<
0.01) when compared with cardio-inhibitory response
patients. The same occurred in the VD group, rMSSD (
p
<
0.05),
PNN
50
values (
p
<
0.05) were lower and LF (
p
<
0.05), LF/HF (
p
<
0.01) were higher when compared with mixed-response patients. In
the CI and MX groups there were no significant differences in HRV
values. Compared with the controls, rMSSD and PNN50 declined
obviously in the VD group while LF increased significantly.
Conclusion:
There were different changes in baseline autonomic
activity in VVS children with different haemodynamic types during
asymptomatic periods. Changes in RMSSD, PNN50 and LF param-
eters may be used as a predictor to identify vasodepressor type of
VVS in clinical diagnosis.
607: SUCCESSFUL TREATMENT OF THERAPY-RESIST-
ANT LEFT VENTRICULAR OUTFLOW OBSTRUCTION
IN CHILDHOOD HYPERTROPHIC CARDIOMYOPATHY
USING SHORT ATRIOVENTRICULAR DELAY DUAL-
CHAMBER PACING WITH A LEFT VENTRICULAR
EPICARDIAL ELECTRODE
Ulla Lundstrom, Anders Nygren, Ingegerd Ostman-Smith
Sahlgrenska University Hospital, Sweden
Background:
In hypertrophic obstructive cardiomyopathy (HOCM),
left ventricular outflow (LVOT) gradient is caused by the septum
bulging into the LVOT, and systolic anterior movement of the ante-
rior cusp of the mitral valve. Unrelieved LVOT obstruction is a risk
factor for death. Primary treatment for gradient reduction is pharma-
cological, with beta-blockers, calcium antagonists and disopyramide.
Optimal management of myectomy and drug-refractory LVOT gradi-
ent is unknown. In adults, short atrio-ventricular (AV) delay dual-
chamber (DDD) right ventricular pacing has been used.
Results are modest, with some non-responders.
Methods:
We report two consequtive paediatric patients with HOCM
and therapy-resistant LVOT gradient after short AV-delay DDD
pacing with an epicardial ventricular electrode placed at the left
ventricle apex. Age at pacing was seven months and 16 years. Both
had maximal pharmacotherapy with propranolol and disopyramide,
and had been through surgical myectomy of LVOT without any
significant relief of outflow obstruction. Optimal AV-delay veloc-
ity through the LVOT was measured by continuous-wave Doppler
from the apical view. The AV delay producing the least gradient was
chosen. The mitral inflow signal was then recorded and care was
taken that the atria would not contract against a closed mitral valve.
Both patients remained on their medical treatment during pacing.
Results:
Before pacing, the peak gradient was 81 and 61 mmHg. AV
delay producing the smallest gradient was 80 ms in both patients.
After pacing, at 12 and 3.5 months, the gradients were 7 and 9
mmHg, respectively
Conclusions:
Short-term results in our two paediatric patients who
failed medical treatment and myectomy showed complete relief of
the gradient. Pacing LV apex with short AV delay activates papillary
muscles before the outflow septum. The mitral valve apparatus moves
posteriorly and the LV apex has time to empty before the septum
bulges into the outflow tract. Pacing from the LV apex seems to be
more effective than RV pacing. It however requires thoracotomy.
610: AORTICO-LEFT VENTRICULAR TUNNEL: CASE
REPORT AND LITERATURE REVIEW
Luis Enrique Vargas Portugal, Mauricio Laerte Silva, Cacero
Brommelstroet Ramos, Gabriel Gustavo Longo, Tito Lavio Baiao
Filho, Vera Regina Fernandes, Sargio Luiz Lopes, Mariana De Souza
Parreiras, Silvia Meyer Cardoso, Leandro Latorraca Ponce
Hospital Infantil Joana De Gusmao, Floriana, POLIS/SC, Brazil
Background:
ALVT is a rare congenital anomaly. Echocardiography
can identify the ALVT and associated lesions. Catheterisation should
be reserved for patients with unclear non-invasive findings or percu-
taneous closure. Neonatal surgery has been advocated in all patients
due to long-term concern of aortic regurgitation (AR).
Objectives:
The purpose of this case report was to review the litera-
ture on aortico-left ventricular tunnel (ALVT) and describe a case
that was misdiagnosed as ventricular septal defect (VSD).
Methods:
We describe an ALVT case that was misdiagnosed initially
as a VSD. A two-year-old boy, presenting with heart failure and
murmur was investigated by echocardiography that misdiagnosed a
VSD. Clinical, echocardiographic and surgical case details as well as
the literature were reviewed.
Case:
Congestive heart failure and a murmur were diagnosed in
the physical examination and furosemide, enalapril and digoxin
were prescribed. Pre-operative echocardiogram revealed a peri-
membranous VSD of 4.6 mm with moderate repercussion, aortic
dilatation and mild AR. The patient underwent surgery to occlude
the VSD, which was not detected. A right sinus of Valsalva aneurysm
was detected and aortotomy revealed an ALVT that was corrected
by patch closure of the aortic end of the ALVT and a plasty of the
sinus aneurysm. At six months’ follow up, the patient was asympto-
matic, without AR and distal tunnel flow in the interventricular peri-
membranous septum was observed.
Conclusion:
Aortico-left ventricular tunnel is a rare cardiac malfor-
mation. The literature shows a good postoperative long-term outcome
and surgical correction is recommended soon after diagnosis in
symptomatic patients. In our case two-dimensional and pulsed
colour-flow Doppler imaging did not establish the diagnosis. Three-
dimensional or transoesophageal echocardiogram and more clinical
suspicion was helpful in its diagnosis. This article is to remind us that
sometimes the diagnosis of rare congenital heart diseases can be done
in the operating room if not diagnosed pre-operatively.
614: SCIMITARSYNDROME: SURGICALDIRECTCONNEC-
TION BETWEEN RIGHT PULMONARYANOMALOUSVEIN
AND LEFT ATRIUM
Luis Enrique Vargas Portugal, Mauricio Laerte Silva, Cicero
Brommelstroet Ramos, Gabriel Gustavo Longo, Vera Regina
Fernandes, Marcia Mallmann Cappellari, Sargio Luiz Lopez, Silvia
Meyer Cardoso, Mariana De Souza Parreiras, Leandro Latorraca
Ponce
Hospital Infantil Joana De Gusmao, Floriana, POLIS/SC, Brazil
Background
: Scimitar syndrome is a rare congenital heart disease.
Different surgical approaches have been described.
Objectives:
The aim of this study was to review the case of a patient
of scimitar syndrome who underwent surgery to directly connect the
right pulmonary anomalous vein (RPAV) to the left atrium (LA).
Methods:
A four-year-old girl presenting with pneumonia was inves-
tigated by means of computed tomography, which diagnosed RAPV
connected to the inferior vena cava. Clinical, echocardiographic,
computed tomography and surgical case details as well as the
literature were reviewed. Her pre-operative echocardiogram revealed
enlargement of the right atrium and right ventricle with an anomalous
connection from the right pulmonary vein to the inferior vena cava.
The patient was submitted to surgery and a direct connection between
the right pulmonary anomalous vein and left atrium was done. At
10 months’ follow up she was asymptomatic. Right phrenic nerve
paralysis was observed.
Conclusion:
In this case a direct connection between RAPV and LA
was feasible because of the anatomical features.
