Impact of Telehealth on Failure to Attend Rates and Patient Re-Engagement in Adult Congenital Heart Disease Clinic.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic paved the way for telehealth consultations. We aimed to determine the impact of telehealth on rates of failure to attend (FTA) in adult congenital heart disease (ACHD) clinics and whether telehealth could re-engage patients with previous FTA face-to-face appointments. METHODS: This was a retrospective audit of a tertiary ACHD clinic over a 12-month pre-telehealth (26 March 2019-17 March 2020) and 12-month post-telehealth implementation period (24 March 2020-16 March 2021). Patients with one or more FTAs during the 24-month study period were included. Our ACHD clinic is run three times per month. Patients with ACHD are offered lifelong follow-up and reviewed annually on average. Re-engagement was defined as two or more consecutive face-to-face FTAs immediately before the telehealth period with subsequent attendance of their telehealth appointment. RESULTS: A total of 359 patients with a total of 623 FTAs were included. Complexity of congenital heart disease was moderate in 56% (202/359) and severe in 19% (69/359) of patients. Overall FTA rate was 18% (623/3,452). FTA rate was significantly lower in the post-telehealth period (15%, 257/1,664) compared with the pre-telehealth period (20%, 366/1,788) (p<0.00001). At study conclusion, 1% of patients had died (5/359). Of the 354 remaining patients, 42% (150/354) were considered lost to follow-up (two or more FTAs including telehealth), 37% (132/354) missed only one clinic appointment, and 20% (72/354) previously considered lost to follow-up had re-engaged in the telehealth period. CONCLUSIONS: Rates of FTA in a tertiary ACHD clinic significantly reduced after the introduction of telehealth consultation. A fifth of patients considered lost to follow-up were re-engaged with telehealth. Additional strategies to further reduce FTA should be explored.

Pregnancy Outcomes in Women After the Arterial Switch Operation.

BACKGROUND: Pregnancy outcomes after the arterial switch operation (ASO) are rare. We sought to determine outcomes of ASO survivors who underwent pregnancy. METHODS: Female patients who had an ASO and underwent pregnancy were identified from the congenital heart disease pregnancy clinic at The Royal Melbourne Hospital. All follow-up data were collected retrospectively by medical record review. RESULTS: Eleven (11) women were identified as having undergone medical care during pregnancy, from the adult congenital database, at The Royal Melbourne Hospital. There were 17 successful pregnancies, and nine women have been followed post pregnancy. Of the 17 successful deliveries, eight were delivered by Caesarean section, seven were vaginal deliveries and two were instrumented vaginal deliveries. Of the eight Caesarean sections, five were emergency and three were elective. The indications for emergency Caesarean section were obstructed labour (n = 2), abnormal cardiotocography (n = 1), obstructed labour and abnormal cardiotocography (n = 1) and congestive cardiac failure (n = 1). There was one neonatal complication (respiratory distress requiring intubation) in a child born at 31 weeks. There were maternal obstetric complications in 10 patients. There were two maternal cardiac complications during pregnancy (heart failure and rapid atrial fibrillation/flutter). There was no change in left ventricular function post-pregnancy. There was progression of severity of neo-aortic valve regurgitation in two patients post pregnancy (trivial to mild and moderate-severe to severe respectively). CONCLUSION: Pregnancy post ASO appears to be safe in the majority of women. Maternal cardiac complications are uncommon in patients without residual significant haemodynamic lesions, although maternal obstetric complications may be common.

Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand.

BACKGROUND: The life expectancy of patients undergoing a Fontan procedure is unknown. METHODS AND RESULTS: Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975-1995), 271 lateral tunnels (1988-2006), and 532 extracardiac conduits (1997-2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%-93%) for AP and 97% (95% confidence interval [CI], 94%-99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%-82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4-16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%-76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0-7.1). Ten-year freedom from failure was 79% (95% CI, 61%-89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%-95%) for other morphologies. CONCLUSIONS: The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

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/m(2), p<0.001) and mixed (2.85 ± 0.38-2.65 ± 0.30 cm/m(2), p = 0.01) groups, whilst the indexed systolic LV dimension decreased significantly post-operation in the AR group (2.26 ± 0.34-1.87 ± 0.27, p<0.001). At five years post operation, independent predictors for a larger LVEDDi were female gender, a pre-operative LVEDDi >3.0 cm/m(2) 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.

Favourable anatomy after end-to-side repair of interrupted aortic arch.

OBJECTIVE: To evaluate cardiovascular outcomes in patients with aortic arch repair and their possible correlation with arch geometry. METHODS: Ten patients who underwent end-to-side repair for aortic arch interruption (IAA), older than 10 years were compared to a cohort of 10 post coarctation (CoA) repair patients matched for age, sex and age at repair. Mean age at operation was 9.7±6.5 days. Patients underwent a resting and 24 h blood pressure measurements, exercise study, MRI, transthoracic echocardiography and vascular studies. RESULTS: Seven patients developed hypertension, two from IAA group and five from CoA group. Nine patients (45%) had gothic arch geometry, three from IAA group and six from CoA group. Despite differences in arch geometry, both groups had normal LV mass, LV function and vascular function. CONCLUSION: No differences in functional or morphologic outcomes could be demonstrated between the end-to-side repair of the arch by sternotomy and the conventional coarctation repair by thoracotomy. A favourable arch geometry can be achieved after the end-to-side repair of the aortic arch. In the present study, we could not correlate adverse arch geometry with any adverse cardio-vascular outcomes. After neonatal arch repair, the contributive role of aortic arch geometry to late hypertension remains uncertain.

Pre- and Post-operative determinants of transplantation-free survival after Fontan. The Australia and New Zealand experience.

BACKGROUND: This review identifies the predictors of late mortality and heart transplantation that remain relevant in the contemporary population of patients with a Fontan circulation, focusing on the potential impact of post-Fontan morbidities on the late outlook of these patients. METHODS AND RESULTS: A total of 1561 patients who had survived the Fontan operation in Australia or New Zealand from 1975 to 2018 were included in this review. Over a median duration of 11.4 years, there was a total of 117 deaths (7%) and 32 heart transplantations (2%). Freedom from death and heart transplantation at 10, 20 and 35 years post Fontan surgery were 94% (95% CI 93-95%), 87% (95 %CI 85-90%) and 66% (95 %CI 57-78%) respectively. Being male, having an atriopulmonary Fontan, pre-Fontan atrioventricular valve intervention, or prolonged pleural effusions post Fontan were predictive of late death or heart transplantation. However, time-dependent variables such as the development of atrial arrhythmia, protein/losing enteropathy or late ventricular dysfunction were stronger predictors of the same outcome. Patients who developed a time-dependent risk factor had a freedom from death and heart transplantation rate of 54% (95 %CI 43-66) at 15 years and 44% (95 %CI 33-57) at 25 years post Fontan. However, 95% (95 %CI 91-99) of patients without any of the identified risk factors were free from death or heart transplantation rate at 25 years post Fontan. CONCLUSION: In conclusion, the occurrence of post-operative complications such as PLE, arrhythmia and ventricular dysfunction will likely precede the late demise of these patients.

Pulmonary Valve Function Late After Ross Procedure in 443 Adult Patients.

BACKGROUND: Limited data exist on long-term pulmonary valve function after the Ross procedure. This study sought to determine the long-term function of the pulmonary valve in 443 consecutive adult patients who underwent a Ross procedure. METHODS: All 443 patients who underwent a Ross procedure between November 1992 and March 2018 were reviewed retrospectively. All underwent pulmonary valve replacement using a cryopreserved pulmonary allograft. Freedom from the study's outcomes were calculated using Kaplan Meier survival. Risk factors for valve failure were analyzed using Cox regression. RESULTS: Mean age at time of operation was 39 years (range: 15-66 years). There was 1 (0.2%, 1 of 443) operative mortality. Nine patients required reintervention on the pulmonary allograft at a mean 6.1 years (range: 1-12 years) after Ross procedure. Patients required pulmonary allograft reintervention for infective endocarditis (n = 4), severe pulmonary stenosis (n = 4), or severe pulmonary regurgitation (n = 1). Freedom from pulmonary allograft reintervention was 98.9% (95% confidence interval [CI] 97.1%-99.6%), 97.7% (95% CI 95.1%-98.9%), 96.6% (95% CI 93.3%-98.3%), and 96.6% (95% CI 93.3%-98.3%) at 5, 10, 15, and 20 years, respectively. Freedom from pulmonary allograft dysfunction (at least moderate pulmonary regurgitation and/or mean systolic gradient ≥ 25 mm Hg and/or reintervention) was 94.5% (95% CI 91.6%-96.4%), 88.1% (95% CI 83.6%-91.4%), 84.9% (95% CI 79.6%-88.9%), and 78.3% (95% CI 69.5%-84.9%) at 5, 10, 15, and 20 years, respectively. No risk factors were identified to influence pulmonary valve durability. CONCLUSIONS: The pulmonary valve allograft gives excellent long-term function when used in adults undergoing the Ross procedure. Reintervention on the pulmonary valve is rare and significant pulmonary allograft dysfunction is uncommon.

Fontan-associated liver disease: pathophysiology, investigations, predictors of severity and management.

Cardiac hepatopathy is the liver injury resulting from congestion and ischaemia associated with acute or chronic heart failure. The improved longevity of adults with operated congenital heart disease who develop heart failure as an increasingly late event makes this form of liver injury increasingly clinically relevant. Patients with congenital heart disease with a single ventricle anomaly, who require creation of a Fontan circulation, are particularly vulnerable as they have elevated venous filling pressures with chronic liver congestion. Progression to liver fibrosis and eventually cirrhosis may occur, with its associated risks of liver failure and hepatocellular carcinoma. This risk likely increases over the patient's lifetime, related to the duration post-surgical repair and reflects the chronicity of congestion. Liver biopsy is rarely performed due to a higher risk of complications in the setting of elevated venous pressures, and the frequent use of anticoagulation. Non-invasive methods of liver assessment are poorly validated and different factors require consideration compared to other chronic liver diseases. This review discusses the current understanding of cardiac hepatopathy in congenital heart disease patients with a Fontan circulation. This entity has recently been called Fontan Associated Liver Disease in the literature, with the term useful in recognising that the pathophysiology is incompletely understood, and that long-standing venous pressure elevation and hypoxaemia are presumed to play an additional significant role in the pathogenesis of the liver injury.

Fontan-associated nephropathy: Predictors and outcomes.

BACKGROUND: Nephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown. METHODS: The Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV. RESULTS: Serum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR <90 mL/min/1.72m2. Renal dysfunction was present in 67/328 (20%) and 3/328 (1%) patients had an eGFR <60 mL/min/1.72m2. The 10-year survival and 10-year freedom from death and transplantation were the same, 96% (95% CI: 0.9-1) for those with renal dysfunction, and 89% (0.83-0.95; p = 0.1) and 87% (95% CI: 0.81-0.94; p = 0.05) for patients without dysfunction. The 10-year freedom from failure were also similar, 83% (95% CI: 0.70-0.97) for those without renal dysfunction vs 80% (95% CI: 0.74-0.89; p = 0.84). There was no change in mean eGFR for the renal dysfunction group over a mean of 8 ± 5.5 years. CONCLUSION: By the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated.

Pacemakers are associated with a higher risk of late death and transplantation in the Fontan population.

BACKGROUND: The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation. OBJECTIVES: We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker. METHODS: Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker. RESULTS: There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (n = 198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, p = 0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, p < 0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced >50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, p = 0.002). CONCLUSIONS: Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation.

Creatinine-based estimation of glomerular filtration rate in patients with a Fontan circulation.

BACKGROUND: Patients with a Fontan circulation are at risk of renal dysfunction. We analyzed cross-sectional data in pediatric and adult Fontan patients in order to assess the accuracy of commonly used serum creatinine-based methods in estimating glomerular filtration rate (GFR). METHODS: A total of 124 Fontan patients (58 children, 66 adults) were enrolled across three study centers. Measurement of GFR (mGFR) using in vivo 99m Tc-DTPA clearance was performed. Various serum creatinine-based equations were used to calculate estimated GFR (eGFR). RESULTS: Mean mGFR was 108 ± 28 mL/min/1.73 m2 in children and 92 ± 20 mL/min/1.73 m2 in adults. Fourteen children (25%) and 28 adults (45%) had an mGFR <90 mL/min/1.73 m2 . There was no significant correlation between mGFR and eGFR (Schwartz) in children (r = 0.22, P = .1), which substantially overestimated mGFR (bias 50.8, 95%CI: 41.1-60.5 mL/min/1.73 m2 , P < .0001). The Bedside Schwartz equation also performed poorly in the children (r = 0.08, P = .5; bias 5.9, 95%CI: -2.9-14.6 mL/min/1.73 m2 , P < .0001). There was a strong correlation between mGFR and both eGFR (CKD-EPI) and eGFR (MDRD) in adults (r = 0.67, P < .0001 in both cases), however, both methods overestimated mGFR (eGFR(CKD-EPI):bias 23.8, 95%CI: 20-27.6 mL/min/1.73 m2 , P < .0001; eGFR (MDRD):bias 16.1, 95%CI: 11.8-20.4 mL/min/1.73 m2 , P < .0001). None of the children with an mGFR <90 mL/min/1.73 m2 had an eGFR (Schwartz) <90 mL/min/1.73 m2 . Sensitivity and specificity of eGFR (CKD-EPI) and eGFR (MDRD) for mGFR <90 mL/min/1.73 m2 in adults were 25% and 92% and 39% and 100%, respectively. CONCLUSIONS: This study identifies the unreliability of using creatinine-based equations to estimate GFR in children with a Fontan circulation. The accuracy of formulas incorporating cystatin C should be further investigated and may aid noninvasive surveillance of renal function in this population.

The Fontan procedure: contemporary techniques have improved long-term outcomes.

BACKGROUND: To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection. METHODS AND RESULTS: Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12+/-6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed. CONCLUSIONS: The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.

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 ±â€¯9.3 and 14.1 ±â€¯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 m2) in 46/131 (35%) and moderate (mGFR 30-59 ml/min/1.73 m2) 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 (ß = 0.89, 95% CI 0.54-1.25, p = 0.002) and decreased mGFR (ß = -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.

Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome.

OBJECTIVE: Eisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT). METHODS: From January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively. RESULTS: The patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having 'complex' anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; p<0.001). WHO ≥3 at presentation was associated with a worse prognosis (mortality HR 1.82, 95% CI 1.19 to 2.78; p=0.006). CONCLUSION: Treatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.

Common atrioventricular valve failure during single ventricle palliation†.

OBJECTIVES: To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve. METHODS: Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation. RESULTS: From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% [95% confidence interval (CI), 88-100%] and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%). CONCLUSIONS: Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.