Associazione Nazionale Medici Cardiologi Ospedalieri



a very complex case of dorv type fallot:a case report

Testa SABRINA Chieti(CH) – SS ANNNUNZIATA | Ricci Mirella Chieti(CH) – SS ANNUNZIATA | Sabina Gallina Chieti(CH) – SS ANNUNZIATA

Double outlet right ventricle (DORV) is a rare CHD characterized by ventriculoarterial connection in which both great arteries arise entirely or predominantly from the right ventricle. Its prevalence is 0.9% of all CHDs. Several chromosomal abnormalities have been associated with DORV, including trisomy 13, trisomy 18, and chromosome 22q11 deletion. Hearts with DORV are an extremely heterogeneous group showing various morphological features, connections, and relationships at each level of the cardiac segments and intersegmental junctions. As a consequence, the clinical manifestations and required surgical procedures for patients with DORV are variable. The anatomical spectrum is higly variable: it can be associated with ventricular septal defect, transposition of the great arteries (TGA) and functional single ventricle. We report the case of an infant with prenatal diagnosis of CHD and finding partial duplication of the short arm of the X chromosome.

Pregnant woman at 22 weeks comes to the outpatient clinic to perform follow-up ultrasound. At that site finding of CHD which was later confirmed at birth and therefore prostaglandin therapy was started. Echocardiogram showed a wide DIV from malalignment with antero-cephalic deviation of the conal septum with significant aortic destroposition configuring picture of double outlet right ventricle. It also presents a complex right efflux obstruction already at subvalvular pulmonary departure. The pulmonary valve presented dysplastic, with en dome motion, hypoplastic. Pulmonary perfusion appears to be provided predominantly by the Botallo ‘s duct originating from the isthmic region of the aorta and leading with a long and tortuous course to the pulmonary confluence near the left pulmnary artery with high velocity flow. The remaining findings are normal. Therefore, in view of the clinical picture, he was subjected to palliative surgery of right systemic-pulmonary shunt between the brachiocephalic trunk and right pulmonary artery ( modified Blalock-Taussing surgery) in 3.5mm PTFE and ligation of Botallo ‘s duct. At subsequent follow-up, the systemic-pulmonary shunt appeared pervious with regular flow. Wide DIV from anterior malalignment with aorta cavalier to DIV. Severe stenosis at complex pulmonary outflow with max gradient up to 124mmHg with dynamic and valvular component. For the subsequent course of treatment, the patient will be discussed collegially.