Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II‑1A.4d)
Journal Title: Journal of Rare Cardiovascular Diseases - Year 2016, Vol 3, Issue 1
Abstract
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
Authors and Affiliations
Jacek Kuźma, Andrzej Rudziński, Grzegorz Kopeć, Piotr Weryński, Dorota Palczewska, Daniel Porada, Marek Rączka, Maciej Pitak
Keywords
Related Articles
- EP ID EP244134
- DOI 10.20418/jrcd.vol3no1.236
- Views 127
- Downloads 0
A 35-year old man with dyspnea on exertion, history of acute pulmonary embolism and ischaemic stroke
We present a case of a 35-year old man with a history of an acute pulmonary embolism and ischaemic stroke, who after several weeks of adequate antithrombotic treatment experienced functional deterioration with severe dys...
Facilitating recognition and grouping the expertise in the main fields of rare cardiovascular diseases
Dear Friends and Colleagues, Dear Readers,We are pleased to deliver at your hands the second issue of your quarterly Journal of Rare Cardiovascular Diseases. The feedback we have received, after launching the Journal in...
Right ventricular free wall motion abnormalities as a simple method of assessment in patients with pulmonary hypertension (RCD code: II‐1A.O)
Background: Pulmonary hypertension (PH) is a cardiovascular pathology leading to right-sided heart failure. A qualitative assessment of right ventricular (RV) function in echocardiography provides valuable information on...
A 50-year-old unrepaired patient with pulmonary atresia and ventricular septal defect
Pulmonary atresia with ventricular septal defect (PA + VSD) is a cyanotic congenital heart disease, also classified as Tetralogy of Fallot with pulmonary atresia. PA + VSD accounts for about 1–2% of congenital heart defe...
Isolated right ventricular endomyocardial fibrosis in a young male with chronic myeloid leukemia (RCD code: III‑3F.2)
Endomyocardial fibrosis (EMF) is a rare disease of unknown etiology which is more prevalent in tropical countries. EMF is associated with fibrosis of endomyocardium, involving one or both ventricles. It usually presents...