[Hypoplastic left heart syndrome].
BACKGROUND
Two cases of hypoplastic left heart syndrome diagnosed in the early neo-natal period are presented in this research. This syndrome is the most frequent cause of cardiac death in the infantile period. Its etiology is unknown. There are a few theories trying to explain the cause of the syndrome's occurrence, however, neither one of them has been confirmed with certainty. The hypoplastic left heart syndrome (LHHS) which is a complex congenital heart defect will take an advanced course followed by a bad prognosis unless treated surgically in time, i.e. within the first days of life after birth.


METHODS
During the research records of the Cardiology Department patients of the Health Care Institute for Children and Youth in Novi Sad have been used. A heart defect has been diagnosed using an noninvasive cardiology diagnostic procedure including heart examination, electrocardiogram (EKG), heart and lung X-rays and an ultrasound examination.


RESULTS
Two male infants of gestational age 39 weeks were our patients. The hypoplastic left heart syndrome was diagnosed by ultrasound with a distinctly small and undeveloped left ventricle reduced to a fissured area with hypoplasia and stenosis of the mitral valve as well as a very distinct aortal atresia. Both infants passed away in the early neo-natal period.


CONCLUSIONS
A classic LHHS was represented by both our cases. It is characterized by undeveloped aorta, aortal valve, mitral valve, the left ventricle and the left atrium with hypertrophy of right heart ventricle. Cases of the hypoplastic left heart syndrome (LHHS) with a dysplastic pulmonary valve have been described in professional literature. Infants born with the LHHS often have a usual body weight, however, within a few hours of life the respiration becomes excessively rapid with symptoms of cardiac congestion. They die unless treated surgically.


CONCLUSIONS
Our research of the LHHS points to seriousness and complexity of the described heart defect which demands an urgent surgery within the first days of life. A negative outcome follows if surgery is not performed.
Milić-Georgijević
L
L
Institut za zdravstvenu zastitu dece i omladine, Medicinski fakultet, Novi Sad.
D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis; D007231:Infant, Newborn; D008297:Male