Hospital inpatient costs for single ventricle patients surviving the Fontan procedure

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95$264,703 to $516,499), corresponding to 164 (95$219,482 (95% CI $202,410 to$236,553) and the cost thereafter over 15 years was $146,820 (95$44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (

Hepatic and renal end-organ damage in the Fontan circulation: a report from the Australian and New Zealand Fontan Registry

Background: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. Methods: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). Results: Mean age and time since Fontan were 19.8 ± 9.3 and 14.1 ± 7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60–89 ml/min/1.73 m) in 46/131 (35%) and moderate (mGFR 30–59 ml/min/1.73 m) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (β = 0.89, 95% CI 0.54–1.25, p = 0.002) and decreased mGFR (β = −0.77, 95% CI −1.29–0.24, p = 0.005). Conclusions: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion

Fertility and pregnancy in the Fontan population

Background Women with a Fontan circulation are deemed at significantly increased risk of maternal morbidity and mortality during pregnancy. Publications describe a small number of pregnancies worldwide and a high rate of miscarriage. We compiled the experience of women enrolled in the Australia and New Zealand Fontan (ANZ) Registry with regard to menarche, contraceptive use, pregnancy advice and pregnancy outcomes. Methods Women within the ANZ Fontan Registry were contacted and asked to consent to receiving sequential questionnaires. Results 156 women ≥ 18 years of age (including 4 deceased individuals) were identified, 101 women consented and 97 completed the initial questionnaire. Women were aged (median) 25 years (23–32); menarche occurred at a median 14 years (13–16). A wide variety of contraceptive methods was reported. 81% of women reported having received advice that pregnancy carried an increased risk or was inadvisable. Pregnancy was reported in a minority (n = 27). Miscarriage (42.5%) and termination (7.5%) accounted for half the pregnancy outcomes and the babies were born early (median 31.5 weeks) and small (median 1350 g). Maternal complications of bleeding, arrhythmia and heart failure were reported with no early maternal mortality. Conclusions In women with a Fontan circulation the fertility onset is delayed and pregnancy has a higher rate of miscarriage. Successful pregnancy resulted in small and premature babies. Significant maternal morbidity occurred. Whether pregnancy with its volume loading has an adverse effect on the long-term outcome of women with a single ventricle remains to be elucidated

Long term follow-up after aortic valve replacement (Ross Procedure): Echocardiographic determinants of ventricular recovery

Aims and Methods: This prospective cohort study aimed to assess LV recovery post aortic valve replacement, stratified according to pre-operative valve lesion (aortic stenosis (AS), mixed disease (AS/AR) or aortic regurgitation (AR)), as well as define predictors of persistent LV dilatation post operation. We prospectively followed all patients post Ross procedure performed between 1992 and 2009 by a single surgeon. Echocardiography was performed pre-operatively, at approximately one year post operation then second yearly thereafter. Results: 265 patients were followed for a mean of 6.4 years (range 1–14 years, total 1702 patient-years). Seventy percent were male and mean age was 38.8 ± 12.6 years. The indication for surgery was AS in 44.5% (118), AS/AR in 23.4% (62), and AR in 32.1% (85). Overall mortality was 1.8% and 80% of deaths were non-cardiac. Morbidity was low and the need for pacing was less than 1%. Ninety-nine percent of patients were NYHA class 1 at one year follow up. The indexed LV end diastolic diameter (LVEDDi) decreased significantly post-operation in the AR (3.34 ± 0.39–2.66 ± 0.32 cm/m2, p 3.0 cm/m2 and the presence of mild aortic regurgitation at one year post-operation. Pre-operative valve lesion was not a predictor. The only independent predictor of a lesser reduction of LVEDDi at five year follow-up was mild post-operative AR, whilst predictors of a lesser reduction in indexed left ventricular end systolic diameter (LVESDi) at five year follow-up included mild post-operative AR and a larger pre-operative LVEDDi. LV wall thickness decreased significantly the AS and AS/AR groups within one year post operation, whilst the neo-aortic root size remained stable throughout follow-up. Conclusions: Recovery of LV size post Ross procedure is influenced predominantly by the pre-operative LV size, in particular the indexed LV end diastolic diameter. The pre-operative valve lesion was not predictive of larger ventricular dimensions post AVR, but independent predictors of a larger ventricular dimensions post operation included female gender, enlarged pre-operative LVEDDi and the presence of mild AR in the first post operative year. Those with mild post-operative AR did not have progressive LV enlargement, thus the clinical significance of this finding remains unclear