CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013
164
AFRICA
415: A LONGITUDINAL STUDY OF VENTRICULAR
CONTRACTILE FUNCTION IN HYPOPLASTIC LEFT
HEART SYNDROME PRIOR TO FONTAN
Edythe Tham
1
, Shelby Kutty
2
, Timothy Colen
1
, Chodchanok
Vijarnsorn
1
, Akiko Hirose
1
, Jeffrey Smallhorn
1
, Nee Khoo
1
1
Stollery Children’s Hospital, University of Alberta, Canada
2
University of Nebraska College of Medicine, USA
Background:
The long-term prognosis in hypoplastic left heart
syndrome (HLHS) is limited by progressive ventricular dysfunction.
The objective of this longitudinal study was to determine changes in
HLHS ventricular function across staged palliative surgeries using
speckle tracking echocardiography.
Methods:
Twenty HLHS patients who survived to pre-Fontan surgery
assessment were prospectively studied. Two-dimensional echocardio-
grams were performed through palliation at: (1) pre-Norwood (6
±
7 days), (2) pre-BCPA (bidirectional cavo-pulmonary anastomosis,
5
±
2 months), and (3) pre-Fontan (2.6
±
0.6 years) stages. Speckle
tracking echocardiography measured global and segmental four-
chamber longitudinal and basal circumferential strain, strain rate
(SR), post-systolic strain index [PSSi
=
(peak strain–peak systolic
strain)/peak strain], rotation, myocardial dyssynchrony index (MDI
=
standard deviation of time to peak strain in 12 segments), and
longitudinal:circumferential strain ratio. Differences across the three
stages were analysed using one-way ANOVA for repeated measures
with post hoc testing (
p
<
0.05).
Results:
Both longitudinal and circumferential SR were decreased
at pre-BCPA and pre-Fontan when compared to pre-Norwood (
p
<
0.0001). Rotation also declined after the pre-Norwood stage
(
p
=
0.02). PSSi was greatest at pre-BCPA stage (longitudinal,
p
=
0.0002; circumferential,
p
=
0.03). Although global strain had
no detectable change between stages, longitudinal:circumferential
strain ratio decreased between pre-Norwood and pre-BCPA (
p
=
0.01). Interestingly, MDI was significantly greater at pre-Norwood
compared to pre-Fontan (
p
=
0.02). Fractional area change was
unchanged across the stages.
Conclusions:
Ventricular ejection appears to be preserved in HLHS
patients who survived to pre-Fontan assessment. However, at the
pre-BCPA assessment, there was a significant detrimental change in
ventricular contractility, coupled with an increase in PSSi, a potential
marker of myocardial ischaemia. We also observed reductions in
the longitudinal:circumferential strain ratio and ventricular rotation.
Whether this is a single RV adaptive process to chronic afterload
or evidence of subtle RV decline remains unclear. Except for PPSi,
recovery in the ventricular functional parameters was not observed at
pre-Fontan, despite volume unloading with BCPA.
416: PROTEIN-LOSING ENTEROPATHY AFTER FONTAN
OPERATION: GASTROINTESTINAL EVALUATION OFFERS
INSIGHT INTO THE PATHOPHYSIOLOGY
Jack Rychik
1
, Pierre Russo
1
, Edisio Semeao
1
, Katie Dodds
1
, David
Goldberg
1
, Simon Murch
2
1
Cardiac Centre, Children’s Hospital of Philadelphia, Philadelphia,
PA, USA
2
Warwick Medical School, Coventry, UK
Background:
Protein-losing enteropathy (PLE) remains an enig-
matic ailment seen after Fontan operation (FO). While the haemody-
namic disturbances of elevated central venous pressure and dimin-
ished cardiac output inherent in the Fontan circulation are likely
inciting factors, characterisation of the gastrointestinal (GI) tract may
offer clues to a better understanding of the pathophysiology. Sulfated
glycosaminoglycans (s-GAG) play an important role in maintaining
integrity of the intestinal mucosa. Congenital deficiency of intestinal
s-GAG, and acquired deficiency in disorders of glycosylation lead to
PLE. Altered s-GAG expression is also noted in patients with kwashi-
orkor, a condition with features similar to PLE after FO.
Objective:
To describe the GI histological findings in patients with
PLE after FO with focus on intestinal s-GAG expression.
Methods:
Twenty-eight combined upper GI endoscopy/colonoscopy
studies were performed in 26 patients with PLE after FO. Gross
histology was assessed. In a random select group of 10 patients,
immunohistochemistry s-GAG staining was performed on tissue
specimens from the duodenum and terminal ileum.
Results:
Age at study was 15.8
±
5.6 years; albumin was 2.8
±
0.9
gm/dl. Of the 28 GI studies, tissue histology revealed inflammation
(oesophagitis, gastritis, small bowel inflammation, cryptitis or coli-
tis) in 15 (53%), lymphangiectasia in 12 (43%), and eosinophilia in
four (14%). Seven of 10 patients demonstrated variable degrees of
s-GAG deficiency, with more prominent depletion seen in the termi-
nal ileum than in the duodenum.
Conclusions:
Intestinal lymphangiectasia and inflammation are
common in PLE after FO. Enteric mucosal s-GAG deficiency is
evident and may be a commonly shared molecular pathway to PLE
among various conditions. Our findings support the model that circu-
latory stressors in combination with inflammation trigger change at
the enteric mucosa in predisposed individuals, which leads to enteric
protein loss after FO. Effective treatment strategies should target
modification of circulatory stressors, reduce inflammation, or replen-
ish tissue s-GAG (i.e. heparin sulfate).
421: ARE WE IGNORING APICAL NON-COMPACTION OF
BOTH VENTRICLES
IB Vijayalakshmi
Sri Jayadeva Institute of Cardiovascular Sciences and Research,
Bengaluru, Bengalore, India
Background:
Isolated left ventricular non-compaction (LVN) is
reported extensively but apical non-compaction (ANC) of both
ventricles and the inter-ventricular septum (IVS) is not reported
much in literature.
A
im:
The aim of our study was to analyse and evolve echocardio-
graphic (TTE) diagnostic criteria for apical non-compaction.
Methods and Results:
From January 2011 to July 2012, 60 consecu-
tive cases that fulfilled standard echocardiographic criteria for
LVNC, and the additional criteria: (1) Swiss-cheese appearance of
IVS, or looking like the delta of a river, and (2) non-compaction of
the right ventricle (RV) on TTE, comprised the study material. The
diagnosis of ANC was totally missed by the echocardiographer and
either only specific lesions or Swiss cheese VSD were diagnosed.
The diagnosis was made after review. Age ranged from three days to
35 years, with 36 males and 24 females. The associated lesions were
present in all 60 cases; 52 patients had acyanotic heart disease (86.7
%) and eight had cyanotic heart disease (13.3 %). Sixteen of 60 cases
had pump failure (26.7 %), seven had LV dysfunction, seven had RV
dysfunction and two had biventricular dysfunction, 28 patients (46.7
%) had pulmonary hypertension, and two (3.3 %) had thrombus.
Three cases of VSDs were post-operative residual shunts. ANC is
known to happen in the chicken heart, but thus far there has been no
evidence to suggest a similar mechanism in humans.
Conclusion:
For the first time worldwide, we are presenting the larg-
est series of ANC. ANC is probably ignored and is invariably asso-
ciated with other serious congenital cardiac malformations, which
worsen the pump failure.
424: IMPACT OFTHE PERINATALTRANSITION ON CARDI-
OVASCULAR FUNCTION AND CEREBRAL DOPPLER
PROFILES IN HYPOPLASTIC LEFT HEART SYNDROME
Akiko Hirose, Nee S Khoo, Timothy Colen, Chodchanok Vijarnsorn,
Winnie Savard, Edythe Tham, Lisa K Hornberger
University of Alberta, Canada
Background:
Rapid changes in myocardial loading occur during the
transition from foetal to postnatal circulation. In healthy neonates
previous studies have shown that the LV output acutely doubles
within hours of delivery but decreases at 24 hours to levels only
slightly more than that of the foetus. Little data exists regarding the
