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Patients with Fontan circulation are now surviving longer into adulthood and experience a multitude of extracardiac, non-hepatic sequelae that impact their quality of life and prognosis. These sequelae include pulmonary complications (pulmonary vascular disease, sleep-disordered breathing, restrictive lung disease, plastic bronchitis), gastrointestinal manifestations (protein losing enteropathy), chronic venous insufficiency, kidney abnormalities, reproductive issues (pregnancy, contraception and sexual dysfunction), neurocognitive changes, endocrinopathies (glycemic abnormalities, thyroid disorders) and hematological abnormalities (thromboembolic complications and secondary erythrocytosis). This review article aims to present a contemporary overview of the extracardiac, non-hepatic sequelae observed in adults with Fontan circulation.

Structured Abstract: Background: While percutaneous closure is preferred in adults with a patent ductus arteriosus (PDA), the extent of cardiac remodeling following percutaneous PDA closure in adults is not well understood. We performed a meta-analysis to characterize cardiac remodeling following percutaneous PDA closure in adults. Methods: MEDLINE and EMBASE were systematically searched for original studies that reported echocardiographic variables at baseline, immediately post-procedure (24 h), and at follow-up (>1 month) in adults undergoing percutaneous PDA closure. Additionally, we included data from patients >18 years of age that underwent percutaneous PDA closure between January 2015–December 2019 at our centre. Weighted averages were calculated for parameters with sufficient data. Pooled differences were presented as weighted mean differences (WMD). Results: After screening 278 abstracts, 5 studies were identified. When combined with our cohort, our meta-analysis encompassed 244 patients. Immediately post-procedure, LVEF was the only parameter to change significantly (64 vs 52%, WMD -8.64 95%, CI -14.04 to −3.25). All parameters significantly decreased from baseline to follow-up: LVEF: 64 vs 60% (WMD -3.95, CI -5.80 to −2.09), LVEDD: 62 vs 53 mm (WMD -8.79, CI -12.78 to −4.81) LVEDVI 172 vs 123 mL/m2 (WMD -26.60, CI -45.4 to −7.85), LVESD 42 vs 36 mm (WMD -4.64, CI -9.10 to −0.17) and LA dimension: 42 vs 37 mm (WMD -5.87, CI -9.85 to −1.90). Conclusions: As compared to the baseline values, left ventricular and left atrial sizes decreased. The LVEF decreased immediately following PDA closure and, while it recovers, it remained lower on follow-up testing.