CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
244
AFRICA
patients with a diagnosis of pulmonary vein stenosis. We excluded
patients with total anomalous pulmonary venous drainage, atrial
isomerism and gestational age
≤
35 weeks.
Results
: We identified 11 patients with pulmonary vein stenosis,
eight were male, median gestational age was 27 weeks (25–34),
median birth weight was 860 g (432–2 100 g), and three patients
were of twin pregnancies whose twin siblings were unaffected.
Most patients were diagnosed with chronic lung disease and needed
significant respiratory support after birth. In nine/11 initial neonatal
echocardiograms did not report abnormal pulmonary vein flow.
The median age at diagnosis was 5 months (3 months –2 years) the
diagnosis was most often made by or suspected by echocardiography
because of apparent worsening of chronic lung disease. Eight/11
patients underwent CT scan or MRI. The left pulmonary vein was the
most commonly stenosed (91%) and all patients underwent a suture-
less surgical repair. Median survival after pulmonary vein surgery
was six months (4–10). In seven/11 PVS recurred and four/11
patients died.
Conclusion
: Pulmonary vein stenosis should be considered if an
ex-premature baby has late deterioration of chronic lung disease or
evidence of pulmonary hypertension by echocardiogram. Further
imaging by CT scan or MRI may be required to complete the diag-
nosis. Pulmonary vein stenosis appears to develop postnatally but
the aetiology remains unknown. The response to surgery and late
recurrence appear to be similar to pulmonary vein stenosis in infants
born at term.
1483: SERIALMEASURES OF SYSTEMIC-TO-PULMONARY
ARTERIAL COLLATERAL FLOW IN PATIENTS WITH
SUPERIOR AND TOTAL CAVOPULMONARY CONNEC-
TIONS
Kevin Whitehead
1
, Matthew Harris
1
, Andrew Glatz
1
, Yoav Dori
1
,
Marc Keller
2
, Jonathan Rome
1
, Mark Fogel
1
1
Division of Cardiology, Children’s Hospital of Philadelphia, USA
2
Department of Radiology, Children’s Hospital of Philadelphia, USA
Background:
We have described a method of quantifying systemic-
to-pulmonary collateral (CollF) flow in patients with superior
(SCPC) and total (TCPC) cavopulmonary connections using magnet-
ic resonance phase-contrast velocity mapping (PC-MRI). Cross-
sectional data suggests that CollF decreases with time after TCPC
completion, but may take years. We wished to examine more acute
changes in physiology and CollF from SCPC to TCPC by comparing
serial (paired) PC-MRI data.
Methods:
We retrospectively reviewed PC-MRI data to identify
patients who had CollF quantified at both SCPC and TCPC. CollF,
systemic blood flow (Qs
=
total caval flow), and pulmonary blood
flow (Qp
=
total pulmonary vein flow) were quantified as previously
described. Fenestration flow was quantified in TCPC by the differ-
ence in caval and pulmonary artery flow.
Results:
Of 104 SCPC and 113 TCPC CollF studies, 26 patients had
both SCPC and TCPC studies performed on average five months
before and seven months after TCPC completion. Indexed CollF
was 1.4
±
0.7 l/min/m
2
at SCPC and 1.7
±
1.1 l/min/m
2
at TCPC (
p
=
0.12), which was a significantly higher fraction of aortic flow in
TCPC compared to SCPC (37
±
18 vs 31
±
14%,
p
=
0.03). There
was a significant decrease in Qs from 3.2
±
0.7 l/min/m
2
at SCPC to
2.8
±
0.8 l/min/m
2
at TCPC (
p
=
0.004). Q
P
increases significantly,
primarily as a result of increased cavopulmonary flow. Fenestration
flow averaged 48
±
42% of the inferior vena caval (IVC) flow at
TCPC, with a significant decrease in right-to-left shunt from SCPC
to TCPC. However, in four patients, fenestration flow equaled or
exceeded IVC flow with flow reversal in the Fontan baffle above the
fenestration in two patients.
Conclusions:
CollF did not decrease after TCPC completion and as a
fraction of aortic flow initially increased, despite a significant reduc-
tion in right-to-left shunt. Q
S
decreased and Q
P
increased significantly
thereafter.
1486: CORONARY ARTERY DYSPLASIA WITH PERSIS-
TENT SINUSOIDS: RARE CAUSE OF FATAL MYOCARDIAL
INFARCTION IN AN INFANT
Pankaj Gupta
1
, Ella Sugo
2
, Owen Jones
1
1
Sydney Children’s Hospital Network, New South Wales, Australia
2
Prince of Wales Hospital, Randwick, New South Wales, Australia
Introduction:
Myocardial infarction in infancy is a very rare and
serious event. Coronary artery dysplasia with persistent sinusoids
is an extremely rare defect. We report on a previously healthy infant
with a normal heart, who developed a massive myocardial infarction
and died secondary to severe coronary artery dysplasia.
Case report:
A two-and-a-half-month old boy was transferred to
intensive care unit of our hospital with a brief history of feeding
intolerance and respiratory distress leading to cardiorespiratory
arrest and requiring cardiopulmonary resuscitation with inotropic
infusion. Other than congenital abnormalities such as mildly dysplas-
tic left hip, multiple cortical renal cyst, undescended left testis and
Talipes left foot, he had an asymptomatic postnatal course. Initial
arterial blood gas showed severe lactic acidosis. Chest roentogram
showed cardiomegaly with pulmonary congestion. ECG revealed
persistent pulseless electrical activity. Echocardiogram demonstrated
structurally normal heart with severely depressed biventricular func-
tion. Prolonged cardiopulmonary resuscitation was ceased after
failure to gain any significant cardiac output and he was pronounced
deceased. Micro-organism investigations including bacterial culture
and nucleic acid analysis for viruses were all negative. Post-mortem
examination showed a significantly enlarged heart, acute and suba-
cute ischaemic changes, widespread myocardial fibrosis, normal
coronary artery origin and course, and most importantly, persistent
myocardial sinusoids. Histology confirmed marked coagulative
necrosis with acellular fibrosis in the subendocardial region of the
ventricles, dystrophic calcification, and persistent sinusoids. There
were no inflammatory infiltrates suggestive of myocarditis. A coro-
nary specimen revealed intimal thickening and myxoid changes,
duplication and fragmentation of the internal elastic lamina and
hypertrophy of media smooth muscle, along with the presence of
myocardial bridging pattern.
Conclusion:
Physicians and pathologists should be aware of unusual
coronary artery dysplasia when managing infants with severe
ventricular dysfunction.
1487: AORTIC DISEASEAND RECURRENCE OF CONGENI-
TAL HEART DISEASE IN FIRST- AND SECOND-DEGREE
RELATIVES OF PATIENTS WITH PERSISTENT DUCTUS
ARTERIOSUS
Regina Bokenkamp, Liesbeth de Bruijn, Janine J Houwing-
Duistermaat, Marco C DeRuiter, Robert E Poelmann, Adriana C
Gittenberger-de Groot, Nico A Blom
Leiden University Medical Centre, Leiden, The Netherlands
Aims:
Persistent ductus arteriosus (PDA) and familial thoracic aneu-
rysm and dissection (TAAD) are genetically heterogeneous. Data on
the prevalence of aortic disease including TAAD, the familial recur-
rence of PDA, and other congenital heart disease (CHD) provide
essential information for the investigation of genetic factors of these
diseases.
Methods and Results:
This was a retrospective patient-based study
using a questionnaire assessing the prevalence of cardiovascular
disease in family members of children with PDA. A thorough family
history provided information on three generations. The data were
analysed by a binominal test. The prevalence in the general popula-
tion was derived from a Dutch population-based study. Over the last
30 years, 320 patients older than three months with isolated PDA
have been treated in our institution. Data on 175 patients and their 2
336 family members were available. The prevalence of aortic disease
was higher in the study group compared to the general Dutch popula-
tion. Aortic disease was reported in four/590
=
0.7% of first-degree
relatives and 29/1339
=
2% of second-degreee relatives. In second-
