Inpatient Resource Utilization for Hypoplastic Left Heart Syndrome from Birth Through Fontan.

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.

A Preliminary Study of One Year Safety and Tolerability of Attention-Deficit/Hyperactivity Disorder Medications in Youth with Fontan Palliation or Heart Transplant.

There are no published studies that examine the safety and tolerability of medication to treat attention-deficit/hyperactivity disorder (ADHD) in children with histories of Fontan palliation (Fontan) or heart transplant (HT), despite the high prevalence of ADHD in these populations. To address this gap, we examined the cardiac course, somatic growth, and incidence of side effects for one year after medication initiation amongst children with Fontan or HT and comorbid ADHD. The final sample comprised 24 children with Fontan (12 medication-treated, 12 control) and 20 children with HT (10 medication-treated, 10 control). Demographic, somatic growth (height and weight percentile-for age), and cardiac data (blood pressure, heart rate, results of 24 h Holter monitoring, electrocardiograms) were extracted from electronic medical records. Medication-treated and control subjects were matched by cardiac diagnosis (Fontan or HT), age, and sex. Nonparametric statistical tests were utilized to compare between- and within-group differences prior to, and one year post, medication initiation. There were no differences in somatic growth or cardiac data when comparing medication-treated participants to matched controls, regardless of cardiac diagnosis. Within the medication group, a statistically significant increase in blood pressure was observed, though the group average remained within clinically acceptable limits. While results are preliminary in nature due to our very limited sample size, our findings suggest that ADHD medications can be tolerated with minimal cardiac or somatic growth effects amongst complex cardiac patients. Our preliminary results favor treating ADHD with medication, which has considerable implications for long-term academic/employment outcomes and quality of life for this population. Close collaboration between pediatricians, psychologists, and cardiologists is essential to individualizing and optimizing interventions and outcomes for children with Fontan or HT.

Impact of 4D-Flow CMR Parameters on Functional Evaluation of Fontan Circulation.

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m2, p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO2/kg/min: R: - 0.45, p = 0.01 peak, minute ventilation (VE) R: - 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population.

"Quality of life" analysis in the long-term follow-up after "Fontan" palliation for CHDs-a single-centre experience.

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. METHOD AND RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. CONCLUSION: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.

Combined Atrial Volume is Associated with Significant Atrial Arrhythmias in Total Cavopulmonary Connection Fontan Patients.

Atrial arrhythmias are a common late manifestation after Fontan palliation and are known to contribute to significant morbidity and mortality. Atrial volume by cardiac magnetic resonance imaging has been increasingly used in patients with congenital heart disease with no reports in those with Fontan palliation. In acquired heart disease, left atrial volume has been shown to be a strong predictor of outcomes of sustained atrial arrhythmias, including recurrence of atrial fibrillation. We hypothesized that combined atrial volume (CAV) in patients with total cavopulmonary connection (TCPC) Fontan palliation may be associated with increased risk of significant atrial arrhythmias (SAA). This is a single center retrospective case-control study. Cases were defined as patients with TCPC Fontan palliation ≥ 18 years of age, with SAA requiring intervention. Only those with advanced imaging for 3D rendering between 2013 and 2022 were included. CAV was analyzed from a 3-dimensional (3D) data set, including both the left and right atria, excluding the Fontan baffle. Seventeen TCPC Fontan case patients and 17 control patients were included. There was no difference in age between the two groups. There was no difference between gender, type of Fontan palliation, atrio-ventricular valve regurgitation, or combined ventricular function between the two groups. CAV was higher in SAA group compared to controls, and all control patients had indexed CAV ≤ 80 mL/kg. This is the first data suggesting CAV is associated with SAA in TCPC Fontan patients. Indexed CAV ≥ 80 mL/kg may be a valuable marker for SAA risk.

Moderators of peak respiratory exchange ratio during exercise testing in children and adolescents with Fontan physiology.

OBJECTIVES: Many patients with Fontan physiology are unable to achieve the minimum criteria for peak effort during cardiopulmonary exercise testing. The purpose of this study is to determine the influence of physical activity and other clinical predictors related to achieving peak exercise criteria, signified by respiratory exchange ratio ≥ 1.1 in youth with Fontan physiology. METHODS: Secondary analysis of a cross-sectional study of 8-18-year-olds with single ventricle post-Fontan palliation who underwent cardiopulmonary exercise testing (James cycle protocol) and completed a past-year physical activity survey. Bivariate associations were assessed by Wilcoxon rank-sum test and simple regression. Conditional inference forest algorithm was used to classify participants achieving respiratory exchange ratio > 1.1 and to predict peak respiratory exchange ratio. RESULTS: Of the n = 43 participants, 65% were male, mean age was 14.0 ± 2.4 years, and 67.4% (n = 29) achieved respiratory exchange ratio ≥ 1.1. Despite some cardiopulmonary exercise stress test variables achieving statistical significance in bivariate associations with participants achieving respiratory exchange ratio > 1.1, the classification accuracy had area under the precision recall curve of 0.55. All variables together explained 21.4% of the variance in respiratory exchange ratio, with peak oxygen pulse being the most informative. CONCLUSION: Demographic, physical activity, and cardiopulmonary exercise test measures could not classify meeting peak exercise criteria (respiratory exchange ratio ≥ 1.1) at a satisfactory accuracy. Correlations between respiratory exchange ratio and oxygen pulse suggest the augmentation of stroke volume with exercise may affect the Fontan patient's ability to sustain high-intensity exercise.

Laparoscopic Surgery Requiring Abdominal Insufflation in Patients With Congenital Heart Disease.

OBJECTIVE: To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach. DESIGN, SETTING, PARTICIPANTS: This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation. MEASUREMENTS AND MAIN RESULTS: The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p < 0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p < 0.0001). CONCLUSIONS: Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

Transcatheter Fontan completion using novel balloon and stent system.

Despite advances in percutaneous interventions, transcatheter Fontan completion remains experimental and performed only in select cases. Non-surgical Fontan completion requires surgical preconditioning at an earlier stage of palliation. We describe transcatheter Fontan completion in a 15-year-old male with previously failed surgical Fontan palliation without surgical preconditioning.

A milestone in congenital cardiac surgery: Four decades of the Norwood procedure.

Hypoplastic left heart syndrome (HLHS) was first described by Lev in 1952, but it was not until 1958 that it received a name from Noonan and Nadas. For the next several decades, the defect was considered untreatable. In 1979, William Norwood and his colleagues from Boston initiated a program to evaluate staged surgical management for infants with HLHS. The Norwood operation has became a milestone in the effective palliation for neonates born with HLHS. Today, the Norwood procedure is the first step of a three-stage heart surgery aimed at creating a new circulatory pathway (i.e., the Fontan pathway).

The Identification and Impact of Abnormal Spirometry Patterns on Exercise Capacity in Pediatric Patients with Fontan Palliation.

Reduced exercise capacity and restrictive lung physiology are common in patients after Fontan palliation (FP). However, there is paucity of data regarding the association between specific spirometry patterns and key exercise parameters in this population. This is a single-center, cross-sectional, study correlating pulmonary function and exercise parameters in children with FP. Patients who were ≤ 18 years of age and underwent a comprehensive cardiopulmonary treadmill exercise stress test (CPT) and spirometry at the same time, were included. Patients were categorized as (i) normal or (ii) abnormal based on the results of spirometry. The abnormal group was subdivided into (a) restrictive, (b) obstructive, and (c) mixed patterns. Demographic and key exercise parameters were compared between groups. Our study included 82 patients who underwent CPT at 13.6 (IQR, 11.3-15.4) years of age. A reduced exercise capacity (%VO2 ≤ 85%) was noted in the majority (n = 50, 61%). Spirometry was abnormal in 47 (57%) patients [restrictive (n = 25, 30%), obstructive (n = 12, 15%), and mixed (n = 10, 12%)]. The abnormal spirometry group had significant lower %VO2 (77% vs. 92%, p = 0.01) and METS (8.4 vs. 9.6, p = 0.02). Subgroup analysis revealed that obstructive (p = 0.04) and mixed (p = 0.02) patterns were associated with a significant decrease in % VO2. Majority of the children demonstrated an abnormal spirometry pattern post-FP. Abnormal pulmonary function was associated with the reduced exercise capacity. Identification and treatment of the abnormal lung function may improve the exercise capacity in these patients and improve the morbidity.

Severe conduit stenosis in a patient with Fontan circulation with a Y-shaped Dacron conduit.

Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

Elevated ventricular filling pressures and long-term survival in adults post-Fontan.

OBJECTIVE: To assess the association between elevated ventricular-end diastolic pressures (VEDP) and pulmonary artery wedge pressure (PAWP) on long-term survival in adult Fontan patients. BACKGROUND: The impact of ventricular filling pressures on long-term survival in adults post-Fontan palliation is unknown. METHODS: We included 148 adult Fontan patients (age ≥ 18 years) without atrioventricular valve prosthesis or pulmonary vein stenosis undergoing arterial and venous catheterization between December 1999 and November 2017. VEDP was defined as ≥12 mmHg and PAWP as >12 mmHg based on optimal cut-offs for prediction of mortality on receiver-operator curves (AUC 0.63 and 0.66, respectively). RESULTS: Mean age was 31.3 ± 9.2 years and 48.6% of patients were females. Most common congenital defects were tricuspid atresia (36.4%) and double-inlet left ventricle (28.3%); 59.5% patients had atriopulmonary Fontan connections. Mean VEDP was 11.5 ± 4.7 mmHg and PAWP 10.6 ± 4.5 mmHg (correlation coefficient .76). During a follow-up of 6.0 ± 4.8 years (median 5.4, IQR 1.4-9.4), there were 45 deaths (30.4%). Overall survival was lower in patients with VEDP ≥ 12 compared to those with VEDP < 12 mmHg (p = .02). Similarly, survival was lower in patients with PAWP>12 compared to patients with PAWP ≤ 12 mmHg (p < .0001). In the multivariate model, PAWP was an independent predictor of death (HR 1.1 per mmHg, 95% CI 1.02-1.15, p = .009) whereas VEDP was not (HR 1.1 per mmHg, 95% CI 1.0-1.13; p = .08). CONCLUSION: PAWP but not VEDP was independently associated with long-term overall mortality in adult Fontan patients. Perhaps PAWP rather than VEDP should be used in the risk stratification of these patients.

Silent in situ thrombus within a hypoplastic left ventricle in a teenager with Fontan palliation.

Thrombosis, especially in the Fontan pathway, is one of the major concerns in patients who underwent Fontan surgery, with reported prevalence of 5-33%. We report a case of thrombus in a rudimentary left ventricle in teenager with no arrhythmia or neurological complications. We also report the special concerns of silent thrombus and role of cardiac MRI in diagnosing an intracardiac thrombus.

Physical exercise may improve sleep quality in children and adolescents with Fontan circulation.

OBJECTIVE: To study physical activity and sleep in Fontan patients and healthy controls before and after an endurance training program, and after 1 year. METHOD: Fontan patients (n = 30) and healthy controls (n = 25) wore accelerometers for seven consecutive days and nights during a school week before and after a 12-week endurance training program and after 1 year. RESULTS: Patients had similar sleep duration and sleep efficiency as healthy controls. Latency to sleep onset in minutes was longer for patients than controls (22.4 (4.3-55.3) minutes versus 14.8 (8.6-29.4) minutes, p < 0.01). More time in moderate-to-vigorous activity daytime was correlated with increased sleep time (p < 0.05; r2 = 0.20), improved sleep efficiency (p < 0.01; r2 = 0.24) and less time as wake after sleep onset (p < 0.05; r2 = 0.21) for patients but not controls. Sleep variables did not change after the exercise intervention for patients or controls. After 1 year, patients had decreased total sleep time, decreased sleep efficiency, increased accelerometer counts during sleep and more time as wake after sleep onset during sleep time, but not controls. CONCLUSIONS: Fontan patients have prolonged latency to sleep onset compared with controls. More time in physical activities was correlated with better sleep quality for the patients. Also, subjects with low sleep efficiency and long latency to sleep onset may benefit most from physical exercise. These patients should be encouraged to engage in individually designed physical exercise as this could improve sleep quality.

Two rare cases of paraganglioma in patients with Fontan physiology.

Pheochromocytoma/paraganglioma is an exceedingly rare tumour, thought to share an association with cyanotic CHD. This association is thought to be a result of chronic hypoxaemia (Antonio et al, Revista Española de Cardiología (English Edition) 2017; 70: 673-675; Folger et al, Circulation 1964; 29: 750-757; Opotowsky et al, J Clin Endocrinol Metab 2015; 100: 1325-1334) We report two cases of paraganglioma over a 4-year period in patients with hypoplastic left heart syndrome who had undergone Fontan completion by ages 2 and 4. Based on a very small number of reported cases of CHD, the mechanism of tumourigenesis is unclear. It is imperative that cases associated with CHD continue to be reported so that we may learn more about the pathogenesis and epidemiology of this entity.

Longitudinal Outcomes of Epicardial and Endocardial Pacemaker Leads in the Adult Fontan Patient.

Placement of an epicardial pacemaker system is often preferred over an endocardial system in patients who have undergone a Fontan operation, but data are limited on how these two systems perform over time in patients with Fontan palliation. We performed a retrospective review of adults with Fontan palliation who had pacemaker implantation and interrogation data at Mayo Clinic from 1994 to 2014. Lead parameters, pacing mode, and polarity were collected at the earliest device interrogation report. Clinic notes and device interrogation reports were reviewed at implantation, 6 months, and yearly after implantation to determine impedance, capture threshold (CT), and energy threshold (ET). There were 87 patients with 168 leads in the study cohort. The mean follow-up time was 7.7 years (6 months-19 years). There were 143 epicardial leads (57 atrial and 86 ventricular) and 25 endocardial leads (20 atrial and 5 ventricular). There was no difference in the baseline lead parameters between epicardial and endocardial leads for impedance (610 ± 259 versus 583 ± 156 Ω, p = 0.93), CT (2.0 ± 1.3 versus 1.8 ± 1.3 V, p = 0.28), or ET (7.1 ± 12.5 versus 6.8 ± 18.1 µJ, p = 0.29). Compared to endocardial leads, ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Regarding clinical outcomes, epicardial leads had higher rates of failure but similar generator longevity in comparison to endocardial leads. Ventricular epicardial leads were associated with temporal decrease in impedance and increase in ET. Epicardial leads had a higher rate of failure but similar generator longevity compared to endocardial leads.

Can endurance training improve physical capacity and quality of life in young Fontan patients?

OBJECTIVE: Children after Fontan palliation have reduced exercise capacity and quality of life. Our aim was to study whether endurance training could improve physical capacity and quality of life in Fontan patients. METHODS: Fontan patients (n=30) and healthy age- and gender-matched control subjects (n=25) performed a 6-minute walk test at submaximal capacity and a maximal cycle ergometer test. Quality of life was assessed with Pediatric Quality of Life Inventory Version 4.0 questionnaires for children and parents. All tests were repeated after a 12-week endurance training programme and after 1 year. RESULTS: Patients had decreased submaximal and maximal exercise capacity (maximal oxygen uptake 35.0±5.1 ml/minute per·kg versus 43.7±8.4 ml/minute·per·kg, p<0.001) and reported a lower quality of life score (70.9±9.9 versus 85.7±8.0, p<0.001) than controls. After training, patients improved their submaximal exercise capacity in a 6-minute walk test (from 590.7±65.5 m to 611.8±70.9 m, p<0.05) and reported a higher quality of life (p<0.01), but did not improve maximal exercise capacity. At follow-up, submaximal exercise capacity had increased further and improved quality of life was sustained. The controls improved their maximal exercise capacity (p<0.05), but not submaximal exercise capacity or quality of life after training. At follow-up, improvement of maximal exercise capacity was sustained. CONCLUSIONS: We believe that an individualised endurance training programme for Fontan patients improves submaximal exercise capacity and quality of life in Fontan patients and the effect on quality of life appears to be long-lasting.

Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training.

OBJECTIVES: The objective of this research was to study lung function, physical capacity, and effect of endurance training in children and adolescents after Fontan palliation compared with healthy matched controls. METHODS: Fontan patients (n=30) and healthy matched control patients (n=25) performed dynamic and static spirometry, and pulmonary diffusing capacity and maximal oxygen uptake tests, before and after a 12-week endurance training programme and at follow-up after 1 year. RESULTS: Fontan patients had a restrictive lung pattern, reduced pulmonary diffusing capacity (4.27±1.16 versus 6.61±1.88 mmol/kPa/minute, p<0.001), and a reduced maximal oxygen uptake (35.0±5.1 versus 43.7±8.4 ml/minute/kg, p<0.001) compared with controls. Patients had air trapping with a higher portion of residual volume of total lung capacity compared with controls (26±6 versus 22±5%, p<0.05). Vital capacity increased for patients, from 2.80±0.97 to 2.91±0.95 L, p<0.05, but not for controls after endurance training. The difference in diffusing capacity between patients and controls appeared to be greater with increasing age. CONCLUSIONS: Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.

Reduced physical exercise and health-related quality of life after Fontan palliation.

AIM: A growing number of patients with Fontan circulation are reaching adulthood, and there is increasing concern about their physical performance and quality of life. This study compared self-reported exercise and measured activity with quality of life in patients after Fontan palliation and healthy controls. METHODS: Physical exercise during an average school week was reported by 30 Fontan circulation patients aged eight to 20 years, and 25 healthy controls, followed by accelerometer recordings over seven days. All subjects and their parents answered a questionnaire on quality of life. RESULTS: Patients reported spending less time exercising each week than the controls (114 ± 66 minutes vs. 228 ± 147 minutes, p < 0.001). However, the overall measured activity and moderate-to-vigorous activity was similar for patients and controls. Patients reported a lower quality of life score than the controls (70.9 ± 9.9 vs. 85.7 ± 8.0, p < 0.001). CONCLUSION: In spite of similar measured total activity, Fontan patients reported less time engaged in regular physical exercise than healthy controls and their quality of life was lower than the controls. We speculate that promoting structured regular physical exercise could improve the quality of life of Fontan patients.

Twin pregnancy in a Fontan-palliated patient.

The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of CHDs that cannot support a biventricular circulation. An increasing number of females who have undergone these connections in childhood are now surviving into adulthood and some are becoming pregnant. We report a case of a 29-year-old woman who presented with a twin pregnancy at 33 weeks of gestation. She had significant deterioration of her cardiovascular status before the twin babies were delivered by emergency caesarean section owing to associated obstetric complications. This report also highlights the various maternal and fetal complications occurring in pregnancy of Fontan-palliated patients and suggests the need for meticulous pre-conception counselling and strict perinatal care.