Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ KEY FEATURES +++ ESSENTIALS OF DIAGNOSIS ++ Young adult with history of multiple staged interventions and surgeries in infancy Physical findings of: – Low-amplitude waveform on jugular venous pulse – Single S2 (may be quite loud if presence of transposed great vessels) – Prominent liver edge (as patients may develop Fontan-associated liver disease) – Evidence of multiple sternal scars +/– thoracotomies Echocardiographic findings reveal a single dominant ventricle of either right or left ventricular morphology +++ GENERAL CONSIDERATIONS ++ True prevalence of univentricular hearts is unknown. It is estimated to be ~0.05–0.1 per 1000 live births Final common pathway for a variety of lesions that exhibit a single ventricular physiology or in which a biventricular repair could not be performed. Most common lesions are tricuspid atresia, hypoplastic left heart syndrome, double inlet left ventricle, unbalanced atrioventricular (AV) septal defects, and double outlet right ventricle Deoxygenated blood from the superior and inferior vena cava is redirected passively to the pulmonary artery Atriopulmonary Fontan, lateral tunnel Fontan, and extracardiac total cavopulmonary connections are the various forms of palliative repairs seen in patients with the Fontan circulation Features of the "failing Fontan" should be monitored. Patients can present with: – Arrhythmias, heart failure, venous thromboembolism, lymphatic obstruction, protein-losing enteropathy, plastic bronchitis, and liver disease +++ CLINICAL PRESENTATION +++ SYMPTOMS AND SIGNS ++ Dyspnea Diminished exercise tolerance Chest pain Palpitations Symptoms more common after the third decade of life Signs and symptoms of volume overload due to either heart failure, liver failure, or hypoproteinemia +++ PHYSICAL EXAM FINDINGS ++ Blunting of the jugular venous pressure waveform Single S2 (maybe loud in the presence of transposed great vessels) Holosystolic murmurs in the presence of significant AV valve regurgitation Mild cyanosis (due to either a Fontan fenestration or venovenous collaterals) Palpable liver edge Sternotomy scars Cyanosis in patients with +++ DIFFERENTIAL DIAGNOSIS ++ Differential is based on the initial anatomy of the congenital lesion However, different phenotypes of Fontan failure include: – Fontan failure with reduced ejection fraction – Fontan failure with normal ejection fraction – Fontan failure with normal pressures and hemodynamics but portovenous outflow obstruction, ascites, and cirrhosis +++ DIAGNOSTIC EVALUATION +++ LABORATORY TEST ++ Liver functions tests, CBC, N-terminal pro-B-type natriuretic peptide, alpha1-antitrypsin Incomplete right bundle branch block in 90% of cases Absence of right-sided forces in tricuspid atresia Sinus node dysfunction requiring a pacing device and atrial pacing Atrial arrhythmias (atrial fibrillation, flutter, and intra-atrial reentrant tachycardia) Sequential AV pacing +++ IMAGING STUDIES ++ Chest radiograph: may be used if pulmonary edema is suspected in single ventricular dysfunction Transthoracic echocardiography (preferred initial imaging modality): – Dominant single ventricle with an adjacent smaller rudimentary ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free a profile for additional features.