Fontan Completion Research Articles

Significance of additional pulmonary blood flow between second and third stage in Fontan pathway.

The benefit of additional pulmonary blood flow (APBF) in Fontan pathway remains controversial. The aim of the study was to analyze the systemic arterial saturation, ventricular and atrioventricular (AV) valve function, development of pulmonary arteries and postoperative course after Fontan operation in children with preserved or eliminated APBF between stages 2 and 3 of the Fontan pathway. A group of 180 consecutive children (median age: 2.92(1.83-16.83) years) with single ventricle underwent extracardiac conduit Fontan operation. The patients were retrospectively analyzed with respect to the presence of APBF between stages 2 and 3. Preoperative echocardiography showed no differences in ventricular function (good - 105[95%] vs. 65[98.5%]; reduced - 9[5%] vs 1[1.5%]; p = 0.07) or AV valve function (p = 0.78). There was no difference in oxygen saturation at admission (p = 0.48), in preoperative blood gas analysis (p = 0.62), or at the discharge (p = 0.73). There was no difference in size of the left pulmonary artery (9.5(0-16.2) vs 10.6 (4.9-21.7) mm; p = 0.13), however the right pulmonary artery was significantly larger (11.2(6.5-19.6) vs. 12.8(7.8-2.5) mm; p = 0.048) in APBF group. In this group the mean pressure in the pulmonary artery was significantly higher as well (8[4-17.5] vs 9.5[4-17.3] mmHg; p = 0.03). APBF does not result in higher oxygen saturation, both before and after Fontan completion. APBF can stimulate the growth of the pulmonary arteries and increase the pulmonary artery pressure. The presence of APBF does not affect the post-operative course after Fontan operation, however it can increase the need of AV valve reconstruction.

Late outcomes of Fontan operation from the EUROFONTAN experience

Abstract Background Fifty years after the first Fontan operation, the persisting challenges and evolving landscape of post-Fontan complications are explored in the EUROFONTAN experience. Aim of the study is the evaluation of long term outcomes of a large cohort of Fontan patients, focusing on age at Fontan and onset of Fontan-associated liver disease (FALD). Methods A retrospective clinical study across 21 european congenital cardiac centers across Europe. Clinical and operative data of patients who underwent Fontan operation between January 1990 and December 2022 were gathered through a RedCap online database. Results 2606 patients were included in this analysis (M/F: 59%/41%, median age and median weight at Fontan of 4.3 y.o (IQR 3.3-6.4), and 16kg (IQR 14-20), respectively. Most frequent cardiac anomaly were tricuspid atresia and pulmonary atresia with intact ventricular septum (781, 30%). Ventricular morphology was left in 1379 patients (53%), and right in 1029 patients (40%). Extra cardiac conduit (ECC) was performed in the majority of patients (2335, 90%), with a median conduit size of 20mm (IQR 18-20). Early post-operative complications were present in 52% (1340), with an early reintervention in 349 patients (249 surgical , 99 catheter driven) At a median follow-up median of 10 years (IQR 4-15), there were 79 late deaths (3%). Adverse events occurred in 49% (1288 , non-surgical in 962 , surgical in 249). The clinical status was stage A in 60% (1288 patients), while only a minority (1.3%) was in status D. Systemic Ventricular function was normal in 1898 patients (73%), while absent or mild Atrioventricular Valve regurgitation was presnet in 86% (1816). Of note, FALD was detected in 388 (15 %) , and sinus rhythm was present in 99%. Statistical analysis shows that late death and adverse events are significantly associated right ventricular morphology of the systemic ventricle (p-value 0.019). Of note, when compared to lateral tunnel, TCPC extracardiac conduit demonstrated lower late mortality (2.3% vs 8.2%, p-value <0.001) and complication rates (60% vs 48%, p-value 0.001). Analyzing age at Fontan, a linear relationship exists between age and late mortality, whereby mortality increases with advancing age (graph 1). However, concerning the development of late adverse events, the risk decreases with age at Fontan up to 7 years; beyond 7 years, the risk increases with advancing age (graph 2). The analysis demonstrated growing FALD-prevalence with increasing time from Fontan completion (significant especially after 10 years). Conclusion current long term outcomes of Fontan operation are highly satisfactory. However, the longer survival is associated to an increasing incidence of late term extracardiac complications (namely FALD) which are more evident if Fontan is performed at older age.Data of Fontan PatientsCorrelation between age at Fontan and AE

HeartMate 3 Ventricular Assist Device in a Single-Ventricle and Single-Lung Patient Palliated With a Bidirectional Glenn.

We describe a 15 year old patient with failing second-stage single-ventricle palliation and left pulmonary artery thrombosis successfully supported with HeartMate 3 ventricular assist device and a Fontan completion as destination therapy.

Comparison of pulmonary artery growth between ductus stent and systemic-to-pulmonary shunt as single-ventricle palliation

ObjectiveWe aimed to compare the pulmonary artery (PA) growth between infants with univentricular hearts who underwent a ductus stenting (DS) and those who received a systemic to pulmonary shunt (SPS) as an initial palliation. MethodsAll infants with ductal-dependent pulmonary blood flow who underwent initial palliation with either a DS or SPS between 2009 and 2022 in our institution were reviewed. PA development was compared between the groups using the PA index and the symmetry index. ResultsA total of 130 patients were evaluated including 49 patients after DS and 81 after SPS. The most frequent primary diagnosis was tricuspid atresia in 27, followed by pulmonary atresia with intact ventricular septum in 19. At stage II palliation, PA index (p=0.926), right PA index (p=0.692), left PA index (p=0.297) and the symmetry index (p=0.650) were similar between the groups. At stage III Fontan completion, PA index (p=0.115), right PA index (p=0.868), and the symmetry index (p=0.144) were similar between the groups. However, left PA index (60 vs. 74mm2/m2, p=0.015) was lower, and the incidence of veno-venous collaterals (24.2 vs. 7.8%, p=0.036) was higher in DS group compared to SPS group. Freedom from PA interventions between stage II and III palliation was lower in DS group compared to SPS group (p=0.009). ConclusionsIn patients after DS in infants with univentricular heart, freedom from PA interventions after stage II was lower and the left PA index was smaller, compared to those after SPS. Long-term outcomes after the Fontan procedure should be addressed in patients after DS.

Utilisation of a post-Fontan management pathway reduces chest tube drainage and hospital readmission rates.

Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions. Fontan patients were identified by retrospective chart review (2017-2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion. A post-Fontan management pathway was implemented (2020-2021) utilising post-operative vasopressin, nasal cannula oxygen until chest tube removal, and discharge regimen of three times daily diuretics, sildenafil, and afterload reducing medications. Patients were followed to evaluate primary outcomes. The pre- and post-pathway groups were similar in single ventricle morphology, demographics, and pre-operative haemodynamics. Forty-three and 36 patients were included in the pre- and post-pathway cohorts, respectively. There were statistically significant reductions in chest tube duration (8 vs. 5 days, p ≤ 0.001), chest tube output on post-operative day 4 (20.4 vs. 9.9 mL/kg/day, p = 0.003), and hospital readmission rates for effusion (13[30%] vs. 3[8%], p = 0.02) compared to baseline. There was an absolute reduction in hospital length of stay (11 vs. 9.5 days, p = 0.052). When combining average cost savings for the Fontan hospitalisations, readmissions for effusion, and cardiac catheterisations within 6 months of Fontan completion, there was a $325,144 total cost savings for 36 patients following pathway implementation. Implementation of a post-Fontan management pathway resulted in significant reductions in chest tube duration and output, and readmission rates for effusion in the perioperative period.

Validity of Wrist-Worn Activity Tracker Heart Rate Detection in Fontan Patients During Exercise.

Physical activity and a healthy lifestyle play an essential role in optimizing long-term health in patients with Fontan physiology. Wrist-worn activity trackers may be useful in medically directed exercise programs for patients with Fontan physiology. The objective of this study was to measure the validity of Garmin and Fitbit activity tracker heart rate detection in patients with Fontan circulation when compared to electrocardiogram (ECG) during cardiopulmonary exercise testing (CPET). 47 Fontan patients undergoing CPET for clinical indications were included and wore activity trackers during CPET. Heart rate via the activity tracker was collected at baseline, maximal exercise, and recovery. Patient heart rates, peak VO2, and peak respiratory exchange ratio (RER) were collected using standard CPET protocols and equipment. Heart rate at each time point was compared between the activity trackers and CPET ECG. Median age of participants was 17.1 years, 15.1 years since Fontan completion. Mean percent of predicted peak VO2 was 56.8%, z-score -3.2 with 61.7% of participants completing a maximal CPET (RER ≥ 1.09). Baseline oxygen saturation mean was 92.9%, 90.0% at maximal exercise. Activity trackers demonstrated mean absolute percentage error < 10% at most time points, comparable with other studies. Demographics, Fontan-associated comorbidities, and echocardiogram findings did not impact the accuracy. Consumer-oriented wrist-worn activity trackers show promising accuracy for heart rate monitoring in medically directed exercise programs for adolescents and young adults with Fontan physiology. Further validation across different exercise modalities is needed.

Single ventricle palliation in congenitally corrected transposition of the great arteries: An international multicenter study

BackgroundSingle ventricle palliation may be performed in patients with congenitally corrected transposition of the great arteries due to hypoplasia of 1 ventricle or anatomic complexity rendering biventricular repair unfeasible. There have been only a few small studies of the outcomes of single ventricle palliation in the setting of congenitally corrected transposition of the great arteries. MethodsA multicenter, international, retrospective cohort study of patients with congenitally corrected transposition of the great arteries undergoing single ventricle palliation was conducted in 29 tertiary hospitals in 6 countries from 1990 to 2018. ResultsA total of 194 patients with congenitally corrected transposition of the great arteries underwent single ventricle palliation. A functionally univentricular heart was present in 62.4% (121/194). Patients with 2 good-sized ventricles had more laterality defects (50.7% vs 28.1%, P = .002). Fontan completion was achieved in 80.3% (155/194). A tricuspid valve surgery was performed in 9.3% (18/194). Survival was 97.4% (95% CI, 92.1-99.2) at 15 years in the univentricular cohort and 89.8% (95% CI, 78.0–95.5) at 15 years in those with 2 adequate ventricles (P = .05). At last follow-up, 11.5% of patients had heart failure, whereas 6.0% had moderate or greater systemic right ventricle dysfunction and 8.4% exhibited moderate or greater tricuspid regurgitation. In multivariable analysis, aortic coarctation or hypoplasia (hazard ratio, 7.7; P = .005) was associated with mortality. ConclusionsSingle ventricle palliation in patients with congenitally corrected transposition of the great arteries is associated with excellent long-term survival and low rates of heart failure and atrioventricular valve failure. In patients who would require complex surgery to achieve a biventricular repair, single ventricle palliation appears to be a good alternative.

Long-term enlargement of the neo-aortic root and aortic arch following arch reconstruction in hypoplastic left heart syndrome

BackgroundLong-term enlargement of the aortic arch after aortic arch reconstruction in hypoplastic left heart syndrome is not well described. MethodsAortic arch measurements for 50 patients with hypoplastic left heart syndrome who achieved Fontan completion were converted to Pediatric Heart Network z-scores. Dimensions were assessed using linear mixed models, and differences among time points were evaluated with F-tests. Sub-analysis was conducted comparing Norwood (n = 36) with hybrid (n = 14) strategies. ResultsMedian time to last imaging was 6.4 (interquartile range, 3.5-11.3) years. Before intervention, the main pulmonary artery was dilated, whereas the ascending aorta, transverse arch, and isthmus were hypoplastic. With aortic arch reconstruction, there were expected increases in all arch z-scores. The aortic arch continued to dilate after aortic arch reconstruction, reaching peak values at 7 months (neo-aortic complex: z = 6.9 [5.6-8.0]) or 12 months after stage I (ascending aorta: z = 6.1 [2.9-8.3]; transverse arch: z = 4.7 [3.0-5.9]). After peak values, there was a gradual decline in z-scores with most components still at least mildly dilated at 16 years (neo-aortic complex: z = 3.2 [3.1-3.9], ascending aorta: z = 3.9 [3.3-4.2]; transverse arch: z = 3.1 [2.5-3.7]) with abrupt caliber change at the isthmus: z = −0.8 (−1.1 to −0.3). Norwood and hybrid strategies showed similar enlargement profiles after 7 months of age. ConclusionsNeo-aortic root and aortic arch in hypoplastic left heart syndrome are enlarged early after aortic arch reconstruction and continue to enlarge out of proportion to normal controls until 12 months of age, with gradual decline in enlargement up to adolescence. Further work should focus on modifiable surgical factors that may prove important to optimize arch growth and geometry.

Fontan Completion in Adult Patients with Functionally Univentricular Hearts.

Although there are considerable amounts of data on the outcomes of pediatric patients who have undergone Fontan repair, little is known about having Fontan completed in adulthood. The study presented the midterm results of our unit's experience with the Fontan completion procedure in adult patients with functionally univentricular hearts. Between 2014 and 2023, 16 adult patients underwent total cavopulmonary connection (TCPC) completion. Relevant information was retrospectively collected. Sixteen patients with a median age of 19 years (18-21 years) were included. Median arterial oxygen saturation was 76% (70-80.75%), and 62.5% of the patients were New York Heart Association (NYHA) Class III. The median mean pulmonary artery pressure was 14 mm Hg (9.5-14.5 mm Hg). Nine patients (56%) had heterotaxy syndrome, and the median time between the last operation and TCPC was 15.5 years (6.75-17.5 years). The median durations for bypass and cross-clamp were 160 minutes (130-201 minutes) and 120 minutes (84.5-137.5 minutes), consecutively. The postoperative course was straightforward in all. The median arterial oxygen saturation before discharge was 89.5% (85-90%), and 68.75% of the patients were NYHA Class II. Follow-up was complete for all patients with a median of 24 months. There was no early or late mortality or significant morbidity during the study period. We concluded that the intra-extracardiac Fontan technique was feasible for meticulously selected adults undergoing TCPC completion, as evidenced by an acceptable mortality rate and a satisfactory midterm outcome, including improvements in their NYHA functional class. However, the long-term consequences must be monitored.

Noncontrast MR Lymphangiography to Identify Progression of Lymphatic Abnormalities over the Course of Fontan Completion.

Noncontrast MR Lymphangiography to Identify Progression of Lymphatic Abnormalities over the Course of Fontan Completion.

Common atrial reservoir strain during the interstage period is a predictor of poor outcomes prior to Fontan completion in hypoplastic left heart syndrome.

The atrium augments ventricular function, but the significance of atrial function in hypoplastic left heart syndrome (HLHS) has not been well evaluated. We investigated the association of atrial reservoir strain (common atrial strain [CAS]) to death or need for transplantation in patients with HLHS. In this retrospective single-center study, echocardiograms from three timepoints (pre-stage 1 palliation [S1P], 4-8weeks post-S1P, and pre-Glenn) were analyzed in infants with classic HLHS. Patients were separated based on transplant-free survival to Fontan (survivors) versus death or heart transplant prior to Fontan (composite outcome). Echocardiographic parameters evaluated included CAS, right ventricle (RV) global longitudinal strain (RVGLS), RV fractional area change (FAC), and tricuspid annular plane systolic excursion (TAPSE). An equal variance t-test, regression, and receiver operating characteristic (ROC) analyses were performed. A total of 45 HLHS patients (25 survivors, 20 patients meeting endpoint) were included in this study. There were no significant differences in any of the functional parameters during the pre-stage 1 or post-stage 1 timepoints. Pre-Glenn CAS and RVGLS were significantly worse in those meeting composite endpoint compared to survivors. CAS was significantly correlated to RVGLS during the pre-S1P and pre-Glenn timepoints. A pre-Glenn CAS<19.5 had an area under the curve of .78 and a 75% sensitivity and 83% specificity for death or need for transplantation. Pre-Glenn CAS is significantly lower in patients with mortality or need for the transplantation prior to Fontan completion and may carry prognostic significance in patients with HLHS.

Retrospective Cohort Study of Additional Procedures and Transplant-Free Survival for Patients With Functionally Single Ventricle Disease Undergoing Staged Palliation in England and Wales.

Reinterventions may influence the outcomes of children with functionally single-ventricle (f-SV) congenital heart disease. We undertook a retrospective cohort study of children starting treatment for f-SV between 2000 and 2018 in England, using the national procedure registry. Patients were categorized based on whether they survived free of transplant beyond 1 year of age. Among patients who had transplant-free survival beyond 1 year of age, we explored the relationship between reinterventions in infancy and the outcomes of survival and Fontan completion, adjusting for complexity. Of 3307 patients with f-SV, 909 (27.5%), had no follow-up beyond 1 year of age, among whom 323 (35.3%) had ≥1 reinterventions in infancy. A total of 2398 (72.5%) patients with f-SV had transplant-free survival beyond 1 year of age, among whom 756 (31.5%) had ≥1 reinterventions in infancy. The 5-year transplant-free survival and cumulative incidence of Fontan, among those who survived infancy, were 93.4% (95% CI, 92.4%-94.4%) and 79.3% (95% CI, 77.4%-81.2%), respectively. Both survival and Fontan completion were similar for those with a single reintervention and those who had no reinterventions. Patients who had >1 additional surgery (adjusted hazard ratio, 3.93 [95% CI, 1.87-8.27] P<0.001) had higher adjusted risk of mortality. Patients who had >1 additional interventional catheter (adjusted subdistribution hazard ratio, 0.71 [95% CI, 0.52-0.96] P=0.03) had a lower likelihood of achieving Fontan. Among children with f-SV, the occurrence of >1 reintervention in the first year of life, especially surgical reinterventions, was associated with poorer prognosis later in childhood.

Reprint of: How He Did It – The James S. Tweddell Approach to Single Ventricle Palliation in Patients With Hypoplastic Left Heart Syndrome

This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification), Bidirectional Glenn 28 (Superior Cavopulmonary Connection), and Extracardiac, Fenestrated Fontan Completion (Total 29 Cavopulmonary Connection). These techniques are the culmination of over 20 years of experience 30 and represent the final techniques he employed at both Children's Hospital of Wisconsin and 31 Cincinnati Children's Hospital Medical Center.

Sequential MRI Evaluation of Lymphatic Abnormalities over the Course of Fontan Completion.

Purpose To evaluate lymphatic abnormalities before and after Fontan completion using noncontrast lymphatic imaging and relate findings with postoperative outcomes. Materials and Methods This study is a retrospective review of noncontrast T2-weighted lymphatic imaging performed at The Children's Hospital of Philadelphia from June 2012 to February 2023 in patients with single ventricle physiology. All individuals with imaging at both pre-Fontan and Fontan stages were eligible. Lymphatic abnormalities were classified into four types based on severity and location of lymphatic vessels. Classifications were compared between images and related to clinical outcomes such as postoperative drainage and hospitalization, lymphatic complications, heart transplant, and death. Results Forty-three patients (median age, 10 years [IQR, 8-11]; 20 [47%] boys, 23 [53%] girls) were included in the study. Lymphatic abnormalities progressed in 19 individuals after Fontan completion (distribution of lymphatic classifications: type 1, 23; type 2, 11; type 3, 6; type 4, 3 vs type 1, 10; type 2, 18; type 3, 10; type 4, 5; P = .04). Compared with individuals showing no progression of lymphatic abnormalities, those progressing to a high-grade lymphatic classification had longer postoperative drainage (median time, 9 days [IQR, 6-14] vs 17 days [IQR, 10-23]; P = .04) and hospitalization (median time, 13 days [IQR, 9-25] vs 26 days [IQR, 18-30]; P = .03) after Fontan completion and were more likely to develop chylothorax (12% [three of 24] vs 75% [six of eight]; P < .01) and/or protein-losing enteropathy (0% [0 of 24] vs 38% [three of eight]; P < .01) during a median follow-up of 8 years (IQR, 5-9). Progression to any type was not associated with an increased risk of adverse events. Conclusion The study demonstrated that lymphatic structural abnormalities may progress in select individuals with single ventricle physiology after Fontan completion, and progression of abnormalities to a high-grade classification was associated with worse postoperative outcomes. Keywords: Congenital Heart Disease, Glenn, Fontan, Lymphatic Imaging, Cardiovascular MRI Supplemental material is available for this article. Published under a CC BY 4.0 license.

Long-Term Outcomes of the Non-fenestrated Strategy for Extracardiac Total Cavopulmonary Connection

BackgroundThe discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy. MethodsBetween August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy. Primary outcomes were Fontan-related events, including death, reoperation, catheter intervention, and postoperative complications such as arrhythmias, protein-losing enteropathy, plastic bronchitis, thrombosis, bleeding, and drainage of pleural effusion after discharge. ResultsThe median follow-up duration was 15.7 years (interquartile range, 15.3–18.4). The 1-, 2-, 3-, 5-, 10-, and 15-year occurrence of Fontan-related events was 24%, 43%, 44%, 55%, 67%, and 76%, respectively. The 1-, 2-, 3-, 5-, and 10-year occurrence of venovenous collaterals was 16%, 60%, 65%, 72%, and 81%, respectively. Ventricular end-diastolic pressure was higher in patients with venovenous collaterals (median, 5.0 mm Hg; interquartile range, 4.0-7.0 mm Hg) than in those without (median, 3.5 mm Hg; interquartile range, 3.0-4.25 mm Hg) (P = .01). Multivariable Cox regression analysis showed that higher ventricular end-diastolic pressure and younger age at Fontan completion significantly increased the risk of developing venovenous collaterals, with hazard ratios of 1.22 for each (95% CI, 1.052–1.41; P = .0085 and .016, respectively). ConclusionsThe occurrence rate of Fontan-related events was acceptable with the non-fenestrated Fontan strategy, whereby venovenous collateral development was common. The results suggest that patients with high ventricle end-diastolic pressure and young patients might benefit from fenestration.

Bilateral bidirectional cavopulmonary connection: a review of surgical techniques and clinical implications.

The presence of bilateral superior caval veins (bSCVs) could negatively influence the outcome of Fontan patients. In the setting of a bilateral bidirectional Glenn, the selective blood flow to the ipsilateral long with consequent flow stagnation in the connecting portion could lead to poor growth of the central portion of the pulmonary artery, potentially affecting the eligibility for Fontan completion and being associated with a higher incidence of thrombotic complications. Alternative surgical techniques have been described to perform a bidirectional cavopulmonary anastomosis in the presence of bSCVs aiming to achieve a balanced growth of the pulmonary bifurcation. The short-term results of these techniques such as the V- or Y-shape seem to be excellent; however, some anatomical settings could affect the feasibility of these techniques. The so-called "unifocalization" creates a configuration comparable to a "normal" bidirectional Glenn and could be a feasible alternative. However, the long-term results of this technique are not published yet. The positive effect of additional pulsatile pulmonary flow on pulmonary artery growth should be considered in case of bilateral bidirectional Glenn, despite the higher incidence of postoperative complications reported and the difficult calibration of the amount of additional flow. The role of computational fluid dynamic to simulate the surgical strategy in single ventricle patients is promising and could be worthwhile in the setting of bSCVs. In fact, the surgical techniques of bilateral bidirectional Glenn could be simulated testing their feasibility and allowing to identify the more favorable hemodynamic pattern, patient specific. This review article highlights the critical issues related to the presence of bSCVs in univentricular physiology, analyzing pros and cons of the different surgical techniques. Besides reviewing the literature, this manuscript focuses on the role of computational fluid simulation in identifying the most favorable surgical technique with an individualized approach, which could potentially improve the clinical outcome.

Relationship of Aortopulmonary Collaterals and Pulmonary Artery Development During Staged Single Ventricle Reconstruction.

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%). The interventional closure of aortopulmonary collaterals was performed before stage II in 12 (4%) patients, before Fontan completion in 38 (13%), and afterwards in 39 (14%). Presence of aortopulmonary collaterals before stage II was not associated with the pulmonary artery index (129 vs. 150 mm2/m2, p = 0.176) at stage II. In contrast, aortopulmonary collaterals before the Fontan completion were associated with lower pulmonary artery index (154 vs. 172 mm2/m2, p = 0.005), and right pulmonary artery index (99 vs. 106 mm2/m2, p = 0.006). Patients who underwent interventional closure of aortopulmonary collaterals before total cavopulmonary connection had lower pulmonary artery index (141 vs. 169 mm2/m2, p < 0.001), lower right pulmonary artery index (93 vs. 106 mm2/m2, p = 0.007), and left pulmonary artery index (54 vs. 60 mm2/m2, p = 0.013) at Fontan completion. The presence of aortopulmonary collaterals did not influence pulmonary artery size by the time of stage II. However, presence of aortopulmonary collaterals was associated with under-developed pulmonary arteries at Fontan completion, especially in patients who needed interventional closure of aortopulmonary collaterals.

The Prevalence of Scoliosis after Fontan Circulation Surgery Followed-Up to Adolescence.

The advancement of surgical techniques and perioperative management for congenital heart disease (CHD) has increased life expectancy. The surgical creation of the Fontan circulation maintains pulmonary blood flow without relying on an effective pump from the abnormal heart, relying on peripheral vascular resistance to maintain effective flow through the lungs. Unfortunately, this delicate mechanism is compromised when scoliosis restricts ventilation, leading to Fontan failure and a poor prognosis for life. This report describes the prevalence of scoliosis with Fontan completion surgery and the role of screening and surgical correction. Ninety-six consecutive Japanese patients undergoing Fontan completion surgery for CHD between 2000 and 2017 were identified in our institutional records. The inclusion criterion was at least 7 years of follow-up after Fontan completion surgery, while the exclusion criteria were congenital, syndromic, and neuromuscular scoliosis. Radiographic and clinical parameters, including cardio-thoracic ratio (CTR) for cardiomegaly and cyanosis saturation, were compared between with and without scoliosis. There were 23 and 40 patients in the scoliosis and no scoliosis groups, respectively. The mean age at the final follow-up was 18.5 and 16.7 years in the scoliosis and no scoliosis groups, respectively (p=0.02). Mean CTR was 43.7% and 39.4% in the scoliosis and no scoliosis groups (p=0.016), and the mean saturation in room air at the final follow-up was 88.8% and 93.2%, respectively (p=0.036). There were no significant differences to clarify the risk factors with multivariate logistic regression analysis. The prevalence of scoliosis with Fontan completion surgery was 36.5%. Screening for scoliosis is important for children with Fontan circulation surgery as part of their routine follow-up at least until they reach adolescence.

Prognostic Value of RV Function Analysis During the Interstage Period in Patients with Hypoplastic Left Heart Syndrome.

Cardiac dysfunction is associated with mortality in children with hypoplastic left heart syndrome (HLHS). We evaluated the ability of qualitative and quantitative RV functional parameters to predict outcomes in HLHS patients. In this retrospective, single-center study, echocardiograms from 3 timepoints (pre-stage 1 palliation, 4-8weeks post-stage 1 palliation, and pre-Glenn) were analyzed in infants with HLHS. Patients were stratified into two groups based on outcome of transplant-free survival post-Glenn (survivors) versus mortality or transplantation prior to Fontan (non-survivors). Images were retrospectively reviewed to obtain RV global longitudinal strain (RVGLS), RV-free wall strain (RVFWS), fractional area change (FAC), tricuspid annular systolic plane excursion (TAPSE), tissue motion annular displacement of the tricuspid valve (TMAD-TV) and qualitative systolic function assessment during the predetermined timepoints. An equal variance t-test and chi-square were used to determine significant differences and ROC curve analysis was performed to derive optimal cutoff values to predict mortality/transplant. A total of 47 patients met inclusion criteria, of which, 21 patients met composite endpoint. There were no significant differences in any RV functional parameter during the pre- or post-stage 1 palliation timepoints. The absolute values of RVFWS, RVGLS, and TMAD-TV were significantly greater in survivors than non-survivors during the pre-Glenn timepoint. A pre-Glenn RVGLS > -15.6 (AUC 0.79), RVFWS > -18.6 (AUC 0.75), and TMAD-TV < 12.6% (AUC 0.82) were sensitive and specific for predicting death or need for transplantation prior to Fontan completion. RVGLS, RVFWS, and TMAD-TV may help identify higher-risk HLHS patients during the interstage period.

A Rare Right-to-Left Shunt in a Child Following Fontan Completion Contributing to Cyanosis

A Rare Right-to-Left Shunt in a Child Following Fontan Completion Contributing to Cyanosis