Jacek Kuźma, A. Rudziński, G. Kopeć, P. Weryński, Dorota Palczewska, D. Porada, M. Rączka, M. Pitak
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Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II‑1A.4d)
Development of irreversible pulmonary hypertension in D-transposition of the great arteries (D-TGA) with ventricular septal defect (VSD) is a well-known phenomenon. Coexistence of left ventricular outflow tract obstruction (LVOTO) should theoretically protect the patient against pulmonary arterial hypertension (PAH). Application of the Blalock-Taussig shunt (B-TS) temporarily improves condition of the patient, but may contribute to irreversible PAH requiring palliative complex therapy. We present a case of a 18 year-old female with congenital complex heart defect: D-TGA with inflow VSD and subpulmonary stenosis, Eisenmenger syndrome, who was treated with several subsequent palliative cardiosurgical operations, including B-TS and Senning procedure and eventually, combined PAH-specific therapy, what resulted in stabilization of clinical status. JRCD 2016; 3 (1): 14–16
期刊介绍:
Journal of Rare Cardiovascular Diseases (JRCD) is an international, quarterly issued, peer-reviewed, open access, online journal that keeps cardiologists and non-cardiologists up-to-date with rare disorders of the heart and vessels. The Journal publishes fine quality review articles, original, basic and clinical sciences research papers, either positive or negative, case reports and articles on public health issues in the field of rare cardiovascular diseases and orphan cardiovascular drugs. Topics of interest include, but are not limited to the following areas: (1) rare diseases of systemic circulation (2) rare diseases of pulmonary circulation (3) rare diseases of the heart (cardiomyopathies) (4) rare congenital cardiovascular diseases (5) rare arrhythmogenic disorders (6) cardiac tumors and cardiovascular diseases in malignancy (7) cardiovascular diseases in pregnancy (8) basic science (9) quality of life