Diagnosis and management of the adult patient with a failing Fontan circulation

Broda, Christopher R.; Downing, Tacy E.; John, Anitha S.

doi:10.1007/s10741-020-09932-0

Cited by 17 publications

(9 citation statements)

References 132 publications

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“…It results from various causes, such as structural issues (obstruction in the Fontan pathway), rhythm disturbances, elevated pulmonary vascular resistance, and single-ventricle dysfunction. 24) CT is an effective modality to diagnose most of these complications. The imaging features of common post-operative complications are discussed below.…”

Section: Complications and Their Imaging Featuresmentioning

confidence: 99%

Computed Tomography in the Evaluation of Fontan Circulation

Kumar

Bhatia

2021

J Cardiovasc Imaging

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The Fontan procedure is a well-established surgical technique to improve survival in patients with univentricular heart disease. The procedure reroutes the systemic venous flow to the lungs, bypassing the right ventricle. The originally proposed method involved direct anastomosis of the right atrium to the pulmonary artery. Since then, several modifications have been made in the original technique leading to the modern Fontan, or total cavopulmonary connection. The modern Fontan technique has shown improved surgical outcomes and increased life expectancy in patients with univentricular disease. Due to the increased survival of these patients, long-term complications are becoming more prevalent. Common complications of Fontan procedure include right atrial dilatation and thrombosis; conduit stenosis and thrombosis; right-to-left and left-to-right shunts; hepatic congestion and cirrhosis; and lymphovascular. Computed tomography (CT) can reliably depict the normal Fontan anatomy and various postoperative complications. A fundamental understanding of the techniques of CT, including imaging protocols and common interpretive pitfalls, allows targeted imaging and precise reporting of clinically significant findings. Radiologists should be familiar with the multiple stages of single-ventricle palliation, normal Fontan anatomy, pathophysiology, and imaging features of common Fontan-related complications.

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Section: Complications and Their Imaging Featuresmentioning

confidence: 99%

Computed Tomography in the Evaluation of Fontan Circulation

Kumar

Bhatia

2021

J Cardiovasc Imaging

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“…1,2 This pathologic physiology disrupts the serial flow of blood in circulation, making it often fatal in the absence of treatment. 2 In 1971, the revolutionary Fontan procedure was introduced to palliate FSV which greatly improved the prognosis for these patients. 3,4 The goal of surgery is to create the Fontan circulation that allows for passive caval blood flow into pulmonary circulation and the use of the FSV for systemic circulation.…”

Section: Introductionmentioning

confidence: 99%

“…Congenital heart defects (CHDs) resulting in a functional single ventricle (FSV) heart occur at a prevalence of ∼35 per 100 000 live births. 1,2 This pathologic physiology disrupts the serial flow of blood in circulation, making it often fatal in the absence of treatment. 2 In 1971, the revolutionary Fontan procedure was introduced to palliate FSV which greatly improved the prognosis for these patients.…”

Section: Introductionmentioning

confidence: 99%

“…7,9 Exercise intolerance revealed during CPET can be an important indicator of Fontan failure, and decreased VO 2 max has been shown to predict mortality in Fontan patients. 2 Exercise capacity is markedly reduced in individuals with a Fontan compared to healthy controls, along with several other differences in exercise response. Less is known about how Fontan subgroups compare.…”

Section: Introductionmentioning

confidence: 99%

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Cardiopulmonary Exercise Test Outcomes in Fontan Patients With Right Versus Left Single Ventricle Morphology

Dhauna¹,

Aboulhosn²,

Lluri³

2022

World J Pediatr Congenit Heart Surg

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Background: Congenital heart defects (CHDs) palliated with Fontan surgery often result in a functional single ventricle that is either a morphologically right or left ventricle, and much less commonly undefined. Given this departure from normal physiology, especially for systemic right ventricle Fontan patients, our study sought to compare cardiopulmonary exercise test (CPET) results of adult patients with single right ventricle (SRV) and single left ventricle (SLV) morphology. Methods: Of 237 Fontan patients from the Ahmanson/UCLA Adult Congenital Heart Disease Center database, 135 patients met the inclusion criteria and were split into 2 groups: SRV (n = 44) and SLV (n = 91). Data were collected on baseline demographics, cardiac history, and CPET results. The 2 groups were compared using unpaired t-test, Mann-Whitney, or Chi-square test. Results: Regarding baseline demographics, SRV patients underwent CPET at a slightly younger age than the SLV group (26.5 ± 6.2 vs 29.6 ± 8.5 years, P = .03). There were no significant differences in CPET parameters (including peak heart rate, oxygen saturation, and maximum VO2/kg) between the SRV and SLV groups. When evaluated subsequent CPET at 3 to 4 years, there was no difference in CPET peak heart rate, peak oxygen saturation, and maximum VO2/kg between the 2 groups. Conclusions: This single-center retrospective analysis suggests that dominant single ventricle morphology may not be associated with an appreciable difference in exercise performance in adult survivors with a Fontan palliation.

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“…Following on, Mueller et al cover heart failure in the largest such group of patients with ACHD, that being tetralogy of Fallot [16]. The intricacies of heart failure in the setting of the adult with Ebstein anomaly [17] is then addressed before the edition turns attention to the systemic right ventricle [18,19] and ultimately to perhaps the most challenging group, those with a failing Fontan circulation [20]. The value of exercise testing in the assessment of heart failure in ACHD is then reviewed by Burstein et al [21].…”

mentioning

confidence: 99%

The success and failure of the adult congenital heart

Collins

2020

Heart Fail Rev

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Diagnosis and management of the adult patient with a failing Fontan circulation

Cited by 17 publications

References 132 publications

Computed Tomography in the Evaluation of Fontan Circulation

Computed Tomography in the Evaluation of Fontan Circulation

Cardiopulmonary Exercise Test Outcomes in Fontan Patients With Right Versus Left Single Ventricle Morphology

The success and failure of the adult congenital heart

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