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

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- CASE REPORTRare congenital cardiovascular diseases Journal of Rare Cardiovascular Diseases 2016; 3 (1): 14-16 www.jrcd.eu Case presentationWe present a case of an 18 year-old female with a complex congenital heart defect and Eisenmenger syndrome treated with palliative cardiosurgical operations and pharmacotherapy for pulmonary arterial hypertension (PAH).The patient was delivered at term with body weight of 3700 g. Central cyanosis and soft systolic murmur with loudness of 2/6 in Levine scale were present at birth. Peripheral oxygen saturation (SpO 2 ) was very low (60-70%) with capillary blood partial oxygen pressure (pO 2 ) of 27 mm Hg. Transthoracic echocardiography (TTE) showed complex congenital heart defect: D-transposition of the great arteries (D-TGA), large inlet ventricular septal defect (VSD) with tricuspid valve (TV) straddling, patent foramen ovale (PFO) and sub-pulmonary stenosis (PS) due to the accessory connective tissue strands. Prostaglandin E1 (PGE1) infusion was started. Subsequent TTE study revealed moderate left ventricular outflow tract obstruction (LVOTO) with pressure gradient of 50 mmHg and no left-to-right shunt through the PFO.The child was qualified for Blalock-Taussig shunt (B-TS) at the age of 4 weeks. After the operation SpO 2 increased to 75-80% with pO 2 of 36 mm Hg. The patient was discharged home in a satisfactory clinical condition.At the age of 15 months cyanosis became intense with a drop of saturation to 60-70%. TTE showed D-TGA, large inlet VSD, mild LVOTO (pressure gradient of 17 mm Hg), extremely dilated pulmonary trunk and occluded B-TS. Cardiac catheterization revealed severe pulmonary hypertension (PH) with high pulmonary vascular resistance (PVR) of 10 Wood units (WU) and abnormal pulmonary-to-systemic resistance ratio (Rp/Rs) of 0.6 (Table 1, 2) unresponsive to 100% oxygen inhalation test. The patient was qualified for atrial septectomy to facilitate mixing of the blood at the atrial level. However, no improvement was observed after the operation. Therefore, the patient was qualified for palliative Senning procedure which was performed at the age of 29 months. During the operation a baffle within the atria was made in order to reroute