P160 RIGHT SISTEMIC VENTRICLE AND PREGNANCY

Abstract

Abstract Patient with complex congenital heart disease: situs inversus with dextrocardia and transposition of the great vessels (D–TGA), right aortic arch and inferior cava agenesis with azygos continuation; at 4 months Mustard atrial switch (intratrial baffle for redirection of the systemic venous circulation in the left subpulmonary left ventricle and of the pulmonary venous circulation in the right subaortic systemic ventricle). At 22 years, 1st pregnancy without complications, caesarean section, birth weight 2.2 kg. Two pregnancies spontaneously terminated in the firts trimester. At 29 years echocardiography: right systemic ventricle hypertrophic, dilated and slightly hypokinetic with tricuspid (systemic) valve with mild to moderate insufficiency; Mustard circuit normally functioning on the systemic side, mild stenosis of the pulmonary buffle (Gmax 6mmHg). At 33 years monochorionic biamniotic twins pregnancy; the risk linked to pregnancy for the patient on the basis of her heart disease was included in a WHO class III worsened by twinning. Because of the high probability of maternal and fetal complications related to the continuation of pregnancy, it was necessary to recommend a selective interruption of the pregnancy of the fetus affected by severe selective underdevelopment with signs of twin to twin transfusion at the 20th week of gestation. Maternal clinical and haemodynamic conditions have been stable during pregnancy. At the 24th week of gestation echocardiography: worsening of the systemic right function ventricle, moderate tricuspid insufficiency and increased gradients in the Mustard circuit (systemic Gmax 13mmHg and pulmonary venous Gmed 8mmHg). After the pregnancy team‘s discussion, a delivery plan was proposed to transfer the patient to a third level cardiac surgery center with experience in congenital heart disease adult between the 35th and 36th week. This was not possible because the patient at 32th week presented preterm labor with caesarean section without maternal and newborn complications (birth weight 1,650 kg). In the puerperium ehocardiography: hypertrophic, dilated and moderately hypokinetic systemic right ventricle with moderate tricuspid insufficiency; Mustard circuit: systemic baffle max gradient 16mmHg and pulmonary venous medium gradient 9mmHg. 7 years after this pregnancy at the last visit: stable clinical and echocardiographic situation with persistence of moderate dysfunction of the systemic right ventricle.