Senning operation as a palliative therapy for a girl with complex heart defect and Eisenmenger syndrome (RCD code: II‑1A.4d)

Q4 Medicine
Jacek Kuźma, A. Rudziński, G. Kopeć, P. Weryński, Dorota Palczewska, D. Porada, M. Rączka, M. Pitak
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引用次数: 0

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
Senning手术作为姑息性治疗女孩复杂心脏缺陷和艾森曼格综合征(RCD代码:II‑1A.4d)
大动脉d -转位合并室间隔缺损(VSD)并发不可逆肺动脉高压是一个众所周知的现象。共存的左心室流出道梗阻(LVOTO)理论上应该保护患者免受肺动脉高压(PAH)。Blalock-Taussig分流术(B-TS)的应用暂时改善了患者的病情,但可能导致不可逆的PAH,需要姑息性综合治疗。我们报告一例18岁的女性先天性复杂心脏缺陷:D-TGA合并流入性室性室性缺损和肺下狭窄,艾森曼格综合征,她接受了几次后续姑息性心脏外科手术,包括B-TS和Senning手术,最终,联合pah特异性治疗,导致临床状态稳定。JRCD 2016;3 (1): 14-16
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来源期刊
Journal of Rare Cardiovascular Diseases
Journal of Rare Cardiovascular Diseases Medicine-Cardiology and Cardiovascular Medicine
自引率
0.00%
发文量
0
审稿时长
23 weeks
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
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