Selected Publication:
Breuer, T.
Klinisches Spektrum und Outcome von Erwachsenen mit angeborenem Herzfehler (EMAH) und Syndromen
[ Diplomarbeit ] Medical University of Graz; 2010. pp. 58
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- Authors Med Uni Graz:
- Advisor:
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Gamillscheg Andreas
-
Nagel Bert Hermann Philipp
- Altmetrics:
- Abstract:
- Introduction: Despite the growing number of adult patients with congenital heart
defects (ACHD), there is no sufficient data for long- term follow- up of ACHD with
syndrome. The aim of this study was to evaluate ACHD with syndrome at the
Division of Pediatric Cardiology from the Medical University of Graz in comparison
to ACHD without syndrome and to detect risk factors for outcome.
Methods: Retrospectively data reviewed of 69 ACHD with syndrome (35 male, 34
female) with a mean age of 26.7 years (range:18.8 to 53.3 years) was analyzed.
The largest group of patients in this study were 32 ACHD with Down¿s syndrome
(20 male, 12 female), who also were compared to ACHD without syndrome.
Results: The most common diagnoses were VSD (n=17; 24%), ASD I or ASD II
(n=16; 23%), prolapse of the mitral valve (n=10; 14%), TOF (n=8; 11%) and
atrioventricular canal (n=8; 11%). 18 surgical procedures on 14 adult patients had
been performed, in detail there were 11 pacemaker- ops ( 61%), 5 correction ops
(27 %), and 1 revision op and one palliative op (each 5 %). 49 patients underwent
92 cardiac catheter procedures, 84 in childhood (91%) and 8 during adulthood
(8%). There had been 7 cardiac catheter procedures for diagnostic reasons and 1
for interventional reason.
6 postoperative complications occurred in 5 cases: 2 AV- block¿s III (33 %), 2
residual VSD¿s (33 %), 1 fistula between VCS and LA (16 %) and 1 sinus node
dysfunction (16 %). 2 patients suffering from Down¿s syndrome and dysmorphic
syndrome died, but cause of death remains unclear.
50 % of all evaluated ACHD- patients were classified NYHA I, another 49 % were
in NYHA II and above. Even 53 % of ACHD with Down¿s syndrome were placed in
NYHA ¿ II and showed cardiological disabilities.
Discussion: This study showed a syndrome- specific variety of congenital heart
defects. The most common surgical procedures were pacemaker ops.
Postoperative complications occurred frequently (27 %) and mortality was rare.
ACHD with syndrome were more frequently classified to have cardiological
disabilities than ACHD without syndrome.