Heterotaxy Syndrome Diagnosed in an Adult
Ochsner Journal, cilt.24, sa.4, ss.288-297, 2024 (ESCI, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 24 Sayı: 4
- Basım Tarihi: 2024
- Doi Numarası: 10.31486/toj.24.0021
- Dergi Adı: Ochsner Journal
- Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus
- Sayfa Sayıları: ss.288-297
- Anahtar Kelimeler: Annular symbolscript symbolscript cardiovascular symbolscript symbolscript heterotaxy symbolscript symbolscript levocardia
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
Background: Heterotaxy syndrome, a condition in which the internal organs are abnormally arranged in the thorax or abdomen, is generally diagnosed early in life, often during the neonatal period. Case Report: We present the case of a 42-year-old male who was incidentally diagnosed with polysplenia syndrome and subse-quently diagnosed with heterotaxy syndrome. Upon further investigation, he was determined to have a sinus venosus type atrial septal defect. Furthermore, the patient’s inferior vena cava was interrupted in the infrarenal region and continued as the azygos vein with a coincidental retroaortic left renal vein, an anatomic variation unrelated to heterotaxy syndrome. Because of his minimal cardiac anomalies, the patient remained undiagnosed until adulthood. Conclusion: According to our research, this case is the first report of a patient with heterotaxy syndrome and a sinus venosus type atrial septal defect. This case augments the available information about the variations of this rare syndrome.