Management of circulatory failure after Fontan surgery

Abstract

With improvement in survival after Fontan surgery resulting in an increasing number of older survivors, there are more patients with a Fontan circulation experiencing circulatory failure each year. Fontan circulatory failure may have a number of underlying etiologies. Once Fontan failure manifests, prognosis is poor, with patient freedom from death or transplant at 10 years of only about 40%. Medical treatments used include traditional heart failure medications such as renin-angiotensin-aldosterone system blockers and beta-blockers, diuretics for symptomatic management, antiarrhythmics for rhythm control, and phosphodiesterase-5 inhibitors to decrease PVR and improve preload. These oral medical therapies are typically not very effective and have little data demonstrating benefit; if there are no surgical or catheter-based interventions to improve the Fontan circulation, patients with severe symptoms often require inotropic medications or mechanical circulatory support. Mechanical circulatory support benefits patients with ventricular dysfunction but may not be as useful in patients with other forms of Fontan failure. Transplant remains the definitive treatment for circulatory failure after Fontan, but patients with a Fontan circulation face many challenges both before and after transplant. There remains significant room and urgent need for improvement in the management and outcomes of patients with circulatory failure after Fontan surgery.

Intra-aortic balloon pump treatment in an adult patient with a Fontan circulation and acute heart failure: a case report

“… 10 Ventricular assist devices (VAD) are increasingly common amongst Fontan patients, particularly in those awaiting heart transplantation, whereas Impella support has only been described in a few case reports. 11 Extracorporeal membrane oxygenation and VAD, both far more invasive than IABP, were not considered appropriate in our patient.…”

Contemporary Management of the Failing Fontan

“…Cardiac output is affected in t circulation due to passive cavopulmonary flow without the contribution of a sub ventricle. The system is unable to deliver normal blood volume to the pulmona leading to a low preload of the systemic ventricle and a low stroke volume wi volume depletion of the systemic ventricle despite a paradoxically elevated passive filling of the pulmonary arteries is unable to be augmented sufficie exercise, leading to a chronically decreased functional capacity in most patients Fontan circulatory failure (FCF) constitutes a broad, term which dysfunction of the Fontan circulation, affecting a patient's ability to carry out activities, as per the consensus statement by Alsaied et al [18,19]. Table 1 show the common phenotypes of Fontan circulatory failure.…”

“…Fontan circulatory failure (FCF) constitutes a broad, term which describes dysfunction of the Fontan circulation, affecting a patient’s ability to carry out daily life activities, as per the consensus statement by Alsaied et al [ 18 , 19 ]. Table 1 shows some of the common phenotypes of Fontan circulatory failure.…”

Sequential MRI Evaluation of Lymphatic Abnormalities over the Course of Fontan Completion

“…Although perioperative mortality and 30-year survival have both improved dramatically, reaching 1% and 85%, respectively, morbidity throughout life remains substantial ( 1 – 3 ). An estimated 40% of the single ventricle population will experience Fontan failure over the time span of 2 decades ( 4 , 5 ). Early identification of individuals at risk for deterioration and Fontan failure is an important challenge in this growing population ( 3 ).…”