ABSTRACT
Pulmonary arteriovenous fistulae are known to develop in patients who have functional single-ventricle heart disease and interruption of the inferior vena cava with direct hepatic drainage to the heart, in which a bidirectional Glenn shunt is the only source of pulmonary blood flow. The progressive systemic arterial hypoxemia that is associated with pulmonary arteriovenous fistulae can have important clinical consequences. Baffling the hepatic venous return to the pulmonary circulation can alleviate pulmonary arteriovenous fistulae.Herein, we present the case of a 13-year-old patient with modified Fontan anatomy and pulmonary arteriovenous fistulae, in whom redirection of a previously placed hepatic venous-to-right pulmonary artery conduit was required in order to increase systemic arterial oxygen saturation. Revision of the conduit improved mixing of hepatic venous effluent with blood flow from the bidirectional Glenn shunt. Three years after this revision, the patient's oxygen saturation remained stable at 90%, and his physical activity was markedly improved. We present our rationale for selected redirection of the conduit and discuss other surgical options that can improve hypoxemia that is associated with pulmonary arteriovenous fistulae.
Subject(s)
Arteriovenous Fistula/surgery , Blood Vessel Prosthesis Implantation , Fontan Procedure/adverse effects , Hepatic Veins/surgery , Hypoxia/surgery , Pulmonary Artery/surgery , Pulmonary Veins/surgery , Adolescent , Arteriovenous Fistula/blood , Arteriovenous Fistula/etiology , Arteriovenous Fistula/physiopathology , Cardiopulmonary Bypass , Hemodynamics , Hepatic Veins/physiopathology , Humans , Hypoxia/blood , Hypoxia/etiology , Hypoxia/physiopathology , Male , Oxygen/blood , Phlebography , Pulmonary Artery/physiopathology , Pulmonary Veins/physiopathology , ReoperationABSTRACT
Integrated cardioplegia techniques have gained wide acceptance by surgeons performing adult cardiac surgery, because patients being referred are likely to have poor ventricular function and energy-depleted hearts. In addition, the increasing complexity of available procedures has led to an increased threat of reperfusion injury and calcium contracture ("stone heart") after prolonged ischemia. In this report, we describe the case of a newborn with transposition of the great arteries that survived almost 6 hours of ischemic time and has normal ventricular function postoperatively. We attribute this outcome to the myocardial protection employed throughout the procedure which allowed successful correction of a technical problem.
