Annals of Diagnostic Paediatric Pathology

Annals of Diagnostic Paediatric Pathology 2004, 8(3-4):
© Copyright by Polish Paediatric Pathology Society
Congenital Heart Diseases Database: analysis of occurrence,
diagnostics and coexistence with anomalies of other systems.
Single institution experience
Annals of
Diagnostic
Paediatric
Pathology
Anna Rybak, Aneta Wójcik, Bogdan WoŸniewicz
Department of Pathology
The Children’s Memorial Health Institute
Warsaw, Poland
Abstract
The Congenital Heart Diseases Database was created in the Children’s Memorial Health Institute during
the period of 2001 – 2003. It contains over 1000 preparations of congenital heart defects of children aged
from 0 till 14 years. According to our data, the most common defect was ventricular septal defect. The
compatibility of the clinic and pathological diagnoses was high (about 80% of the diagnoses were confirmed),
however significantly higher after 1990. Although in our Database plenty of coexisting anomalies
and diseases of the other systems or organs occurred, we found no significant relations between them and
congenital heart defects.
Key words: congenital heart disease, database, heart defects
Introduction
Since 1980 until now, the Children’s Memorial Health Institute
of Warsaw has assembled over 1200 congenital heart defects
preparations from patients aged 0-14 years. Between
September 2001 and March 2003 we have made the repeated
sections of those preparations and created “The Congenital
Heart Diseases Database” (CHDDb) that includes 1037 records
with full descriptions of the hearts’ and defects’ anatomy, clinical
and sectional diagnoses, anomalies of other systems and
performed operations. This is to present the direct conclusions
that have arisen from this Database, including the frequency
of occurrence of the particular heart defects, compatibility of
the clinical and autopsy diagnoses and finally, regularity in
coexistence of the congenital heart defects with the anomalies
of the other systems.
Material and methods
The Congenital Heart Diseases Database is composed of 1037
preparations of the hearts with the congenital defects. They
are fully described in four files which contain personal patients’
data, clinical diagnoses with the descriptions of defects
Address for correspondence
Bogdan Wozniewicz
Department of Pathology
The Children's Memorial Health Institute
Aleja Dzieci Polskich 20, 04-736 Warsaw, Poland
of the other systems and types of performed operations, pathologic
diagnoses of the heart defect and coexisting diseases.
The last file shows complete measurement of all the hearts
including external dimensions as well as internal measurements
of the valves, arteries, veins or myocardium.
The patients’ age was between 1 day and 14 years.
There were 437 female and 600 male patients described and
they were all admitted to Children’s Memorial Health Institute
during the period 1980 – 2003 and almost half of them
were operated because of the heart defect.
A nomenclature for the congenital heart disease was
based on the International Nomenclature for Congenital Heart
Surgery that was officially adopted at the Annual Meeting of
the EACTS in Glasgow on September 6, 1999 [3].
Results and discussion
Occurrence of the congenital heart defects
The most common anomaly that appeared in our material was
ventricular septal defect (VSD) with the frequency just under
30% (Fig.1). In details, there were 31 cases of VSD in muscular
part of the septum (VSD single–29; VSD multiple-2 cases)
and 259 cases of this defect in other parts of the interventricular
septum (VSD; NOS).
Phone: +4822-815-1960
E-mail: b.wozniewicz@czd.waw.pl