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

Jacek Kuźma, Andrzej Rudziński, Grzegorz Kopeć, Piotr Weryński, Dorota Palczewska, Daniel Porada, Marek Rączka, Maciej Pitak

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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


Keywords


right heart catheterization; echocardiography; pulmonary hypertension; endothelin receptor antagonist; phosphodiesterase type 5 inhibitor; combination therapy

References


Paul MH, Wernovsky G. Transposition of the great arteries. Moss and Adams Heart Disease in Infants, Children, and Adolescents 1995; 2: 1154–1224.

Vázquez-Antona CA, Muñoz-Castellanos L, Kuri-Nivón M, et al. Left Ventricular Outflow Tract Obstruction in Transposition of the Great Arteries. Correlation Between Anatomic and Echocardiographic Findings. Rev Esp Cardiol 2003; 56: 695–702.

Suesaowalak M, Cleary JP, Chang AC. Advances in diagnosis and treatment of pulmonary arterial hypertension in neonates and children with congenital heart disease. World J Pediatr 2010; 6: 13–31.

Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev 2009; 18: 154–161.

Hislop AA, Moledina S, Foster H et al. Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children. Eur Respir J 2011; 38: 70–77.




DOI: http://dx.doi.org/10.20418%2Fjrcd.vol3no1.236

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