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OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired. ispartof: JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY vol:163 issue:3 pages:1166-1175 ispartof: location:United States status: published

Background The frequency of arrhythmias increases after the Fontan operation over time; atrial tachycardia (AT) and sinus node dysfunction (SND) are frequently observed. Case summary Our patient was 63-year-old woman who underwent a lateral tunnel Fontan operation for double outlet right ventricle at age 36. She experienced paroxysmal AT for 1 year, and antiarrhythmic medication was not feasible due to symptomatic SND. Computed tomography revealed a 45 mm-sized thrombus in the high right atrium (RA). The patient had three coexisting conditions: paroxysmal AT, symptomatic SND, and the right atrial thrombus, for which total cavopulmonary connection conversion and epicardial pacemaker implantation (PMI) would have been effective; however, given her age and comorbidities, surgical treatment was considered high risk. Catheter ablation was avoided because of the right atrial thrombus. Finally, a transvenous pacemaker was implanted via the right femoral vein to avoid the right atrial thrombus and severe venous tortuosity from the left subclavian vein to the RA. After PMI, the patient was prescribed amiodarone and bisoprolol for AT suppression. Atrial tachycardia occurred once in the third month after discharge. We increased the dose of amiodarone, and she has been tachycardia-free. Discussion Transvenous PMI must be considered in cases where open thoracic surgery or catheter ablation cannot be performed. This is the first report of transvenous PMI via the right femoral vein and successful AT and SND management in an elderly Fontan patient.

Background: Pulmonary arteriovenous malformations (PAVMs) are common sequelae of palliated univentricular congenital heart disease, yet their pathogenesis remain poorly defined. In this preliminary study, we used paired patient blood samples to identify potential hepatic factor candidates enriched in hepatic vein blood. Methods: Paired venous blood samples were collected from the hepatic vein (HV) and superior vena cava (SVC) from children 0 to 10 years with univentricular and biventricular congenital heart disease (n = 40). We used three independent protein analyses to identify proteomic differences between HV and SVC blood. Subsequently, we investigated the relevance of our quantified protein differences with human lung microvascular endothelial assays. Results: Two independent protein arrays (semi-quantitative immunoblot and quantitative array) identified that soluble vascular endothelial growth factor receptor 1 (sVEGFR1) is significantly elevated in HV serum compared to SVC serum. Using ELISA, we confirmed the previous findings that sVEGFR1 is enriched in HV serum (n = 24, p < 0.0001). Finally, we studied the quantified HV and SVC serum levels of sVEGFR1 in vitro. HV levels of sVEGFR1 decreased tip cell selection (p = 0.0482) and tube formation (fewer tubes [p = 0.0246], shorter tube length [p = 0.0300]) in vitro compared to SVC levels of sVEGFR1. Conclusions: Based on a small heterogenous cohort, sVEGFR1 is elevated in HV serum compared to paired SVC samples, and the mean sVEGFR1 concentrations in these two systemic veins cause pulmonary endothelial phenotypic differences in vitro. Further research is needed to determine whether sVEGFR1 has a direct role in pulmonary microvascular remodeling and PAVMs in patients with palliated univentricular congenital heart disease.

Reduction of heart rate variability (HRV) parameters may be a risk factor and precede the occurrence of arrhythmias or the development of heart failure and complications in people with postinfarct left ventricular dysfunction and after coronary artery bypass grafting. Data on this issue in adults after a Fontan operation (FO) are scarce. This study assessed the association between HRV, exercise capacity, and multiorgan complications in adults after FO. Data were obtained from 30 FO patients (mean age 24 ± 5.4 years) and 30 healthy controls matched for age and sex. HRV was investigated in all patients by clinical examination, laboratory tests, echocardiography, a cardiopulmonary exercise test, and 24-h electrocardiogram. The HRV parameters were reduced in the FO group. Reduced HRV parameters were associated with patients’ age at the time of FO, time since surgery, impaired exercise capacity, chronotropic incompetence parameters, and multiorgan complications. Univariate analysis showed that saturated O2 at rest, percentage difference between adjacent NN intervals of &gt, 50 ms duration, and peak heart rate were associated with chronotropic index. Multivariable analysis revealed that all three variables were independent predictors of the chronotropic index. The results of this study suggest novel pathophysiological mechanisms that link HRV, physical performance, and organ damage in patients after FO.

Background Hepatic steatosis, caused by nonalcoholic fatty liver disease, is a leading cause of chronic liver disease. The interplay between hepatic steatosis and the development of liver disease following the Fontan procedure is not well understood. This study examined the prevalence and associations of hepatic steatosis in patients with a Fontan circulation. Methods and Results This was a single‐center retrospective study of 95 patients with a Fontan circulation with liver magnetic resonance imaging performed between 2012 and 2019. The average age at magnetic resonance imaging was 21.5±8.5 years. The percent liver fat signal was determined using magnetic resonance chemical shift‐encoded proton density fat fraction imaging. Hepatic steatosis was defined as liver fat ≥5% and was present in 10.5% of the cohort. The presence of hepatic steatosis was associated with higher body mass index (29±4 versus 24±6 kg/m 2 , P =0.006), a higher frequency of obesity (50% versus 12%, P =0.015), lower high‐density lipoprotein cholesterol (35±9 versus 43±14 mg/dL, P =0.050), and greater subcutaneous fat thickness (2.6±0.7 versus 1.8±1.0 cm, P =0.043). There was no association between hepatic steatosis and cardiovascular imaging or hemodynamic variables from cardiac catheterization. Conclusions Risk factors for hepatic steatosis in patients with Fontan circulation include obesity and dyslipidemia, similar to what is seen in the general population. Fontan hemodynamics were not associated with hepatic steatosis.

Background Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications. Methods This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1–4) and with application of an area score (0–12 points). Results Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13–60] vs. 13 [IQR: 2–22] days, p = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%, p = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min–max: 2–10] vs. 2 [min–max: 0–8]), p = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3–4: n = 5/9 vs. n = 1/33, p = 0.001). Conclusion Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.

Structural, hemodynamic, and morphological cardiac changes following Fontan operation (FO) can contribute to the development of arrhythmias and conduction disorders. Sinus node dysfunction, junction rhythms, tachyarrhythmias, and ventricular arrhythmias (VAs) are some of the commonly reported arrhythmias. Only a few studies have analyzed this condition in adults after FO. This study aimed to determine the type and prevalence of arrhythmias and conduction disorders among patients who underwent FO and were under the medical surveillance of the John Paul II Hospital in Krakow. Data for the study were obtained from 50 FO patients (mean age 24 ± 5.7 years; 28 men (56%)). The median follow-up time was 4 (2–9) years. Each patient received a physical examination, an echocardiographic assessment, and a 24 h electrocardiogram assessment. Bradyarrhythmia was diagnosed in 22 patients (44%), supraventricular tachyarrhythmias in 14 patients (28%), and VAs in 6 patients (12%). Six patients required pacemaker implantation, and three required radiofrequency catheter ablation (6%). Arrythmias is a widespread clinical problem in adults after FO. It can lead to serious haemodynamic impairment, and therefore requires early diagnosis and effective treatment with the use of modern approaches, including electrotherapy methods.

Background/Purpose Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center. Methods Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed. Results Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era. Conclusion Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.

The principal aim of this study was to evaluate changes in systolic function in the single right ventricle (SRV), during progression of the same patient through the three stages of surgical repair for hypoplastic left heart syndrome and during a 5-year follow-up. We hypothesize that, SRV global longitudinal strain (GLS) will be low during 3 stages of repair even in stable patients. We retrospectively evaluated 140 echocardiograms in 20 patients with HLHS (ages 0–11.3 years), before and after 3 stages of surgical palliation. Five-year follow-up data were available in all 20 patients. Controls with structurally normal hearts and in the same age group were used for comparison. We utilized speckle-tracking imaging for assessment of SRV segmental and global longitudinal and circumferential strains, from previously acquired 4-chamber and mid-cavity short-axis views prior to and within 1–3 months of each surgical stage. Longitudinal strain (LS) remained low through all 3 stages of repair and during follow-up. The pre-Fontan stage demonstrated significant interstage improvement compared to the post-Glenn stage despite similar volume status. Global LS was (− 15.6 ± 4.5% after Fontan surgery and remained similar (− 15.32 ± 3.2%) 5 years later. The SRV also showed increased dominance of circumferential strain compared to the normal RV, where the longitudinal deformation was dominant. In SRV, longitudinal strain may be a useful clinical index for evaluating both segmental and global function in an objective manner. Due to lack of significant clinical deterioration over a 10-year period, we speculate that a “lower-than-normal” longitudinal strain may be used as an objective measure of SRV function in clinically stable patients, particularly after the Fontan operation. Compensatory mechanisms where the longitudinal pattern of contraction switches to a more circumferential pattern, may play a role in asymptomatic patients with HLHS.

Background: The Fontan circulation is associated with low skeletal muscle mass, which affects the accuracy of creatinine-based glomerular filtration rate estimates (eGFR/GFR). Cystatin C, another filtration marker, is less impacted by muscle mass. We examined associations between characteristics of individuals with a Fontan circulation and both creatinine-based and cystatin C-based eGFR. Methods and results: This single-center retrospective study included 107 individuals with a Fontan circulation who had clinical measurements of serum cystatin C and creatinine. GFR was estimated using different equations for adults (≥19 years of age, Chronic Kidney Disease-Epidemiology Collaboration equations for both creatinine and cystatin C) and children (

Atrial arrhythmia is an important cause of late death in patients after the Fontan-Style operation. However, the detailed electrophysiological characteristics of the post-Fontan atrium and its underlying mechanisms are largely unknown. In this study, we investigated electrophysiological characteristics and the ionic remodeling in the right atrium (RA) of a canine model after the Fontan operation. We performed the operation of RA to pulmonary artery connection to mimic the Fontan operation. We undertook hemodynamic measurements, cardiac electrophysiological studies, and ion current measurements. The expression of ionic channels was analyzed by PCR and western-blotting. Our Fontan model induced RA hypertension, enlarged the size of RA, and increased atrial fibrosis, representing the classic characteristic of Fontan patients. In the Fontan group, the atrial effective refractory period and the active potential duration were reduced, and the atrial tachycardia has been more often to be induced. The electrical conduction mapping showed that the Fontan group reduced the conduction velocity. The Fontan operation significantly down-regulated the expression of KCND3/Kv4.3, CACNA1C/Cav1.2 and SCN5A, but up-regulated the expression of KCNJ2/Kir2.1. Correspondingly, The Fontan operation reduced transient-outward (Ito) and L-type Ca2 (ICa,L) and INa currents, while increasing the inward-rectifier current (IK1). Thus, the net shortening of the action potential in the post-Fontan atrium is associated with the altered expression of ionic channels which disturbed the balance between inward and outward currents. Taken together, the Fontan operation induces the ionic remodeling, and thus altered electrophysiological characteristics of the right atrium, improving our understanding on the pathophysiology of atrial arrhythmias in Fontan patients.

Background The accuracy of 2D and 3D strain analyses was evaluated by comparing strain and cardiac function parameters in Fontan repair patients and normal child volunteers. Methods We retrospectively enrolled 32 patients with Fontan circulation and 32 child volunteers who had undergone clinical cardiac magnetic resonance (CMR) assessment of the dominant ventricle with a 1.5-Tesla MRI scanner. Global and regional strain (2D and 3D) of the dominant ventricle in both groups was assessed using CMR feature-tracking. Correlations between cardiac function and strain data were assessed using Pearson’s correlation coefficient values. The intraclass correlation coefficient (ICC) and coefficient of variation (CoV) were determined to evaluate repeatability and agreement. Results The 2D GLS showed significant differences between the Fontan repair patients and volunteers (− 16.49 ± 5.00 vs. -19.49 ± 2.03; p = 0.002). The 2D GRS and 2D GCS showed no significant differences between two groups. 2D GRS: 38.96 ± 14.48 vs. 37.46 ± 7.77; 2D GCS: − 17.64 ± 5.00 vs. -16.89 ± 2.96, respectively; p > 0.05). The 3D global radial strain (GRS), global circumferential strain (GCS), and global longitudinal strain (GLS) showed significant differences between the Fontan repair patients and volunteers (3D GRS: 36.35 ± 16.72 vs. 44.96 ± 9.98; 3D GLS: − 8.86 ± 6.84 vs. -13.67 ± 2.44; 3D GCS: − 13.70 ± 7.84 vs. -18.01 ± 1.78; p r = − 0.491, p = 0.004). The 3D GCS showed correlations with the indexed end-diastolic volume (EDV) (r = 0.523, p = 0.002) and indexed end-systolic volume (ESV) (r = 0.602, p Conclusions 3D GCS is feasible, highly reproducible, and strongly correlated with conventional cardiac function measures. 3D GCS assessments may be useful for monitoring abnormal myocardial motion in patients with Fontan circulation.

OBJECTIVE To investigate the regional flow distribution in patients with Fontan circulation by using magnetic resonance imaging (MRI). MATERIALS AND METHODS We identified 39 children (18 females and 21 males; mean age, 9.3 years; age range, 3.3-17.0 years) with Fontan circulation in whom flow volumes across the thoracic and abdominal arteries and veins were measured by using MRI. The patients were divided into three groups: fenestrated Fontan circulation group with MRI performed under general anesthesia (GA) (Group 1, 15 patients; average age, 5.9 years), completed Fontan circulation group with MRI performed under GA (Group 2, 6 patients; average age, 8.7 years), and completed Fontan circulation group with MRI performed without GA (Group 3, 18 patients; average age, 12.5 years). The patient data were compared with the reference ranges in healthy controls. RESULTS In comparison with the controls, Group 1 showed normal cardiac output (3.92 ± 0.40 vs. 3.72 ± 0.69 L/min/m², p = 0.30), while Group 3 showed decreased cardiac output (3.24 ± 0.71 vs. 3.96 ± 0.64 L/min/m², p = 0.003). Groups 1 and 3 showed reduced abdominal flow (1.21 ± 0.28 vs. 2.37 ± 0.45 L/min/m², p < 0.001 and 1.89 ± 0.39 vs. 2.64 ± 0.38 L/min/m², p < 0.001, respectively), which was mainly due to the diversion of the cardiac output to the aortopulmonary collaterals in Group 1 and the reduced cardiac output in Group 3. Superior mesenteric and portal venous flows were more severely reduced in Group 3 than in Group 1 (ratios between the flow volumes of the patients and healthy controls was 0.26 and 0.37 in Group 3 and 0.63 and 0.53 in Group 1, respectively). Hepatic arterial flow was decreased in Group 1 (0.11 ± 0.22 vs. 0.34 ± 0.38 L/min/m², p = 0.04) and markedly increased in Group 3 (0.38 ± 0.22 vs. -0.08 ± 0.29 L/min/m², p < 0.0001). Group 2 showed a mixture of the patterns seen in Groups 1 and 3. CONCLUSION Fontan circulation is associated with reduced abdominal flow, which can be attributed to reduced cardiac output and portal venous return in completed Fontan circulation, and diversion of the cardiac output to the aortopulmonary collaterals in fenestrated Fontan circulation.

Background Total cavopulmonary connection (TCPC) is associated with a lower risk of incident atrial arrhythmias as compared to atriopulmonary Fontan, but the risk of recurrent atrial arrhythmias is unknown in this population. The purpose of this study was to determine the incidence and risk factors for recurrent atrial arrhythmias and thromboembolic complications in patients with TCPC. Methods This is a retrospective multicenter study conducted by the Alliance for Adult Research in Congenital Cardiology (AARCC), 2000–2018. The inclusion criteria were TCPC patients (age > 15 years) with prior history of atrial arrhythmia. Results A total of 103 patients (age 26 ± 7 years; male 58 [56%]) met inclusion criteria. The mean age at initial arrhythmia diagnosis was 13 ± 5 years, and atrial arrhythmias were classified as atrial flutter/tachycardia in 85 (83%) and atrial fibrillation in 18 (17%). The median duration of follow-up from the first episode of atrial arrhythmia was 14.9 (12.1–17.3) years, and during this period 64 (62%) patients had recurrent atrial arrhythmias (atrial flutter/tachycardia 51 [80%] and atrial fibrillation 13 [20%]) with annual incidence of 4.4%. Older age was a risk factor for arrhythmia recurrence while the use of a class III anti-arrhythmic drug was associated with a lower risk of recurrent arrhythmias. The incidence of thromboembolic complication was 0.6% per year, and the cumulative incidence was 4% and 7% at 5 and 10 years respectively from the time of first atrial arrhythmia diagnosis. There were no identifiable risk factors for thromboembolic complications in this cohort. Conclusions Although TCPC provides superior flow dynamics and lower risk of incident atrial arrhythmias, there is a significant risk of recurrent arrhythmias among TCPC patients with a prior history of atrial arrhythmias. These patients may require more intensive arrhythmia surveillance as compared to other TCPC patients.

The Fontan procedure is a well-established surgical technique to improve survival in patients with univentricular heart disease. The procedure reroutes the systemic venous flow to the lungs, bypassing the right ventricle. The originally proposed method involved direct anastomosis of the right atrium to the pulmonary artery. Since then, several modifications have been made in the original technique leading to the modern Fontan, or total cavopulmonary connection. The modern Fontan technique has shown improved surgical outcomes and increased life expectancy in patients with univentricular disease. Due to the increased survival of these patients, long-term complications are becoming more prevalent. Common complications of Fontan procedure include right atrial dilatation and thrombosis; conduit stenosis and thrombosis; right-to-left and left-to-right shunts; hepatic congestion and cirrhosis; and lymphovascular. Computed tomography (CT) can reliably depict the normal Fontan anatomy and various postoperative complications. A fundamental understanding of the techniques of CT, including imaging protocols and common interpretive pitfalls, allows targeted imaging and precise reporting of clinically significant findings. Radiologists should be familiar with the multiple stages of single-ventricle palliation, normal Fontan anatomy, pathophysiology, and imaging features of common Fontan-related complications.

Background: We assess whether the lower exercise tolerance in children with univentricular heart (UVH) after Fontan operation is associated with altered peripheral muscular and cerebral tissue oxygenation. Methods: 18 children with UVH and 20 healthy subjects performed an incremental ramp exercise test. Changes in the cerebral and muscular pattern of oxygenated (O 2Hb) and deoxygenated hemoglobin (HHb) and local tissue oxygenation (TOI) were analyzed by means of Near Infrared Spectroscopy (NIRS). Correlations between arterial saturation during exercise and tissue oxygenation were evaluated. Results: In UVH, maximal oxygen consumption (VO 2peak/kg, 28.9 ± 7.9 vs. 46.3 ± 11.9 ml/min/kg, P < 0.001), heart rate (HRpeak, 168 ± 13 vs. 193 ± 12 bpm, P < 0.001) and load (Ppeak, 73 ± 19 vs. 133 ± 68 W, P < 0.001) were lower, VE/VCO 2 slope was higher (34.5 ± 5.9 vs. 27.1 ± 3.9, P < 0.001). A faster and steeper course up to the same level of HHb and absent increase in O 2Hb was seen at cerebral level in UVH; tissue oxygenation index (TOI) demonstrated a steady decrease from the start of exercise. At the muscular level, HHb curve has a similar pattern compared to controls, with an early cessation. O 2Hb has a similar pattern, but with early discontinuation at a higher O 2Hb-level. Muscular TOI has the same course throughout exercise, starting from a lower level. Lower arterial saturation and higher age correlated with lower VO 2peak; higher amplitude of muscular TOI and lower amplitude cerebral TOI correlated with higher VO 2peak. Conclusion: Children after Fontan procedure have different oxygenation mechanisms at muscular and cerebral level. This reflects a different balance between O 2 supply to O 2 demand which might contribute to the reduced exercise tolerance in this patient population.

Background: The Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used. Methods: We retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed. Results: There was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up. Conclusion: The DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

Patients with adult congenital heart disease (ACHD) are at increased risk of developing late cardiovascular complication. However, little is known about the predictive factors for long-term outcome. The Model for End-Stage Liver Disease eXcluding INR (MELD-XI) score was originally developed to assess cirrhotic patients and has the prognostic value for heart failure (HF) patients. In the present study, we examined whether the score also has the prognostic value in this population. We retrospectively examined 637 ACHD patients (mean age 31.0 years) who visited our Tohoku University hospital from 1995 to 2015. MELD-XI score was calculated as follows; 11.76 x ln(serum creatinine) + 5.11 x ln(serum total bilirubin) + 9.44. We compared the long-term outcomes between the high (≥10.4) and the low (

Background : The optimal timing, need for primary/staged procedure in patients undergoing univentricular palliation, is debatable. Aims : We performed this study to assess the exercise performance of patients undergoing various forms of univentricular palliation. Setting and Design : This was a retrospective, prospective comparative study conducted at a multispecialty tertiary referral center. Patients and Methods : Between January 2012 and June 2015, 117 patients undergoing either bidirectional Glenn (BDG) (n = 43) or Fontan (total cavopulmonary connection [TCPC]) (n = 74) underwent exercise testing. Statistical Analysis : Comparisons between subgroups for continuous data were made with Student's ttest if normally distributed and Wilcoxon ranksum test otherwise. Tests between subgroups for qualitative data were made with Pearson's Chisquare test. Results : Patients who underwent BDG with open antegrade pulmonary blood flow (APBF) had higher saturations (oxygen saturation [SpO2]) compared to those without it (87.5 ± 5.0% vs. 81.1 ± 4.8%; P = 0.0001). However, we found no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC (n = 42) patients demonstrated better exercise capacity (15.0 ± 7.7 vs. 11.2 ± 6.2 min; P = 0.02) and increased SpO2 on exercise (87.0 ± 8.0% vs. 83.4 ± 7.6%; P ≤ 0.05) compared to lateral tunnel TCPC (n = 32). Fenestrated TCPC (n = 30) patients had higher exercise capacity reflected by higher metabolic equivalents (METs) consumption (6.4 ± 2.3 vs. 5.2 ± 2.0 METs, P = 0.02), fewer pleural effusions (7.0 ± 3.2 vs. 9.2 ± 6.2 days, P ≤ 0.05), and lower hospital stay (9.5 ± 4.0 vs. 12.7 ± 7.7 days, P = 0.04) compared to nonfenestrated TCPC (n = 44) patients. Conclusions : We observed no differences in exercise parameters of patients undergoing BDG with or without APBF. Extracardiac TCPC patients had better exercise capacity but longer postoperative hospital stay and pleural effusions than patients with lateral tunnel Fontan. Fenestrated TCPC patients seemed to fare better than nonfenestrated ones. Patients undergoing TCPC had better exercise capacity than patients undergoing BDG alone.

Objectives The aim of this study was to describe exercise performance during the first 2 decades of life in Fontan survivors by a cross-sectional study and to identify factors that influence exercise performance. Background Exercise performance after the Fontan procedure is reduced relative to performance in healthy subjects. Data on pre-adolescents are limited, and the patterns of exercise performance in different ages are unexplored. Methods Ramp cycle ergometry was performed with expired gas. Data were analyzed for the entire study population and for subpopulations that did and did not achieve a maximal aerobic capacity. Results Of 411 subjects tested (12.4 ± 3.2 years of age), 166 achieved a maximal aerobic capacity. Peak oxygen consumption (VO 2 ) was 26.3 ml/kg/min (65% of predicted for age and gender [% predicted]) for the entire population and was lower in the submaximal capacity subgroup compared with the maximal capacity subgroup (63% predicted and 67% predicted, respectively; p = 0.02). Oxygen consumption at ventilatory anaerobic threshold (VAT) was better preserved (78% predicted for the total population) than peak VO 2 . Higher % predicted O 2 pulse at peak exercise was associated with greater % predicted peak VO 2 , work rate, and VAT. Adolescence and male gender were associated with decreased % predicted peak VO 2 . The relationship between echocardiographic indexes of ventricular function and exercise function was surprisingly weak. Conclusions In Fontan patients, maximal aerobic capacity is reduced compared with healthy subjects, with better preservation of submaximal performance. Higher O 2 pulse is associated with better exercise performance, whereas adolescence and male gender are associated with decreased performance compared with healthy subjects.

Hypoplastic left heart syndrome (HLHS) accounts for 4-9% of congenital heart disease in children. The mortality rate among children with HLHS undergoing cardiac repair is well documented, but comparable data for noncardiac surgical procedures are not well known. Medically intractable epilepsy is one of those problems, which arises as a complication of cardiovascular surgery in HLHS and necessitates neurosurgical intervention. There is no published knowledge about neurosurgical procedures performed in children with HLHS in the English literature. Thus, we present a 10-year-old boy who developed medically intractable epilepsy after cardiac surgery for HLHS. The aim of this study is to outline the pre-, intra- and postoperative precautions needed for neurosurgical intervention in HLHS patients to decrease morbidity and mortality. Copyright © 2007 S. Karger AG.

La bronquitis plástica es una rara manifestación consistente en la formación y expectoración de moldes bronquiales de origen linfático que provocan obstrucción de la vía respiratoria. Se asocia sobre todo al postoperatorio de cardiopatías congénitas corregidas con la operación de Fontan. Como tratamiento de base se han utilizado mucolíticos y fisioterapia respiratoria y en los casos más graves la extracción de los moldes por broncoscopia así como la nebulización de urocinasa o activador tisular del plasminógeno recombinante (rt-PA). Se presenta el caso de un varón con atresia pulmonar con tabique íntegro y ventrículo derecho hipoplásico operado a los 7 años con una intervención de Fontan, que a los 12 años presentó episodios frecuentes de tos, disnea y desaturación en los cuales el paciente expectoraba moldes bronquiales de aspecto blanquecino. Se realizó una conversión a Fontan extracardíaca para mejorar la clínica y hemodinámica, presentando en el postoperatorio frecuentes episodios de obstrucción respiratoria por moldes bronquiales que requirieron extracción mediante broncoscopia y mejoraron con la administración de urocinasa nebulizada. : Plastic bronchitis is a rare complication in which bronchial casts of lymphatic origin develop in the tracheobronchial tree and cause airway obstruction. The main feature is expectoration of bronchial casts. This condition usually occurs in the postoperative period after the Fontan operation for congenital heart disease. Treatment modalities include mucolytics and chest physiotherapy, and in the most severe cases bronchoscopy to remove the casts and aerosolized urokinase or r-TPA. We describe a 12-year-old boy with pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle who underwent a modified Fontan operation at the age of 7 years. At the age of 12 years the patient started to present frequent episodes of coughing, dyspnea, and desaturation followed by difficult emission of white bronchial casts. An extracardiac Fontan conversion was performed to improve hemodynamics and symptoms. During the postoperative period frequent episodes of airway obstruction required bronchoscopy and finally improved with administration of aerosolized urokinase.

Two cases are presented. In the first patient (8-year-old boy) after Glenn operation without closure of pulmonary outflow from the common ventricle (SV-PA) despite transcatheter SV-PA closure increased mean pulmonary artery pressure (mPAP) (22 mm Hg) remained. After 6 months of sildenafil therapy he was catheterized again. His mPAP dropped to 10 mm Hg and a Fontan operation was subsequently performed. The second patient was a 25-year-old man, 20 years after a Fontan operation, presenting protein los ing enteropathy and severe heart failure. All syndromes diminished significantly after medical therapy with sildenafil. Good clinical results of sildenafil therapy were maintained after 1 and 4 years of treatment. Chronic therapy with sildenafil can be beneficial in patients before and after the Fontan operation with elevated pulmonary artery pressure.

In patients with a right to left intracardiac shunt, air embolism results in an obligatory systemic embolization. Nonembolization of entrained air is described in a child with a single ventricle physiology who had earlier undergone bidirectional Glenn shunt construction and Damus-Kaye-Stansel anastomosis. The air entrainment was detected by intra-operative transesophageal echocardiography. The combined effect of a "diving bell" phenomenon and mild aortic valve regurgitation are suggested as the reasons for the confinement of air into the ventricle preventing catastrophic systemic embolization.

Seit Einführung der palliativen chirurgischen Behandlung des singulären Ventrikels erreichen mehr und mehr Patienten das Erwachsenalter, sodass heutzutage bereits bei größeren Kohorten Langzeitergebnisse vorliegen. Die modifizierte Fontan-Operation erlaubt den meisten Patienten mit singulärem Ventrikel, das Erwachsenenalter mit niedriger Mortalität und Morbidität zu erreichen. Dadurch entstand in den letzten 40 Jahren eine neue und weiter wachsende Patientengruppe mit sehr individuellen anatomischen und physiologischen Prinzipien. In den bisherigen Beobachtungen zeigten Patienten einen stabilen Verlauf unter nicht zyanotischen Bedingungen. Die besten Voraussetzungen für eine stabile Langzeithämodynamik sind strenge präoperative Auswahlkriterien und eine kontinuierliche, postoperative Überwachung. Durch rechtzeitig eingeleitete Therapien werden die Langzeitergebnisse verbessert. Trotzdem bleibt die Fontan-Zirkulation durch ihre nicht physiologische Hämodynamik und mindestens leicht eingeschränkte Funktion des Univentrikels zeitlich limitiert. Die Senkung des pulmonalarteriellen Widerstandes und die Besserung der ventrikulären Füllung sind von entscheidender Bedeutung; konventionelle medikamentöse Therapien der Herzinsuffizienz sind jedoch kaum anwendbar. Schwere Komplikationen wie das Eiweißverlustsyndrom mit chronischem Aszites bzw. ein multifaktoriell bedingtes Failing-Fontan sind selten, und als Ultima-Ratio-Therapie steht in dem Fall nur noch eine Herztransplantation zur Verfügung., Since the introduction of palliative surgical therapy for single ventricle defect more and more patients have reached adulthood. Thus, nowadays the long- term results of large cohorts can be analyzed. The results of the modified Fontan operation performed with modern surgical technique are characterized by low morbidity and mortality rates. Therefore in the past 40 years a new patient population with very specific anatomical and physiological principles has arisen and continues to increase in size. The long-term follow-up reveals stable hemodynamics under non-cyanotic conditions. The preconditions for optimal long-term outcome are strict preoperative selection and subject- specific postoperative supervision. The long-term results can be significantly improved if therapy is started early enough. Nevertheless the Fontan circulation is limited through the non-physiological flow principle and decreased function of the single ventricle. Essential for the long-term stability of the hemodynamics are the reduction of pulmonary vascular resistance and improvement of ventricular filling. The conventional medication for heart failure is not applicable intreatment of the failing Fontan circulation. Severe Fontan complications, such as proteinlosing enteropathy with chronic ascites and chronic Fontan failure are rare. Nevertheless the possibility for conventional treatment in such cases is limited and heart transplantation is the ultima ratio therapy.

Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services.

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

Oitenta e quatro pacientes foram submetidos a operação tipo Fontan, no Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, entre 1979 e 1989; as idades variaram entre 5 meses e 31 anos. Estes pacientes foram colocados em 5 grupos anatômicos: 1) atresia tricúspide la, 3 pacientes (1 óbito, 33,3%); 2) atresia tricúspide lb, 48 pacientes (5 óbitos, 10,4%); 3) ventrículo único: 18 pacientes (2 óbitos, 11,1%); 4) atresia pulmonar com septo interventricular íntegro, 5 pacientes (1 óbito, 20,0%); 5) anomalias complexas, 10 pacientes (zero óbitos). O grupo das anomalias complexas foi dividido em 2 subgrupos: pacientes com situs solitus e pacientes com situs inversus. A maioria destes pacientes foi submetida a procedimentos adicionais: em 5 pacientes, uma das valvas A-V foi fechada; em 1 paciente, foram realizadas valvoplastia mitral e septação do átrio único; em 1 paciente, foi realizada a secção de feixe anômalo (feixe de Kent). A evolução tardia (6 meses a 8 anos) revelou que 9 pacientes encontram-se em classe funcional I e 1 na classe funcional II (NYHA). Baseados nos resultados obtidos, concluímos que a operação de Fontan modificada é uma alternativa válida em anomalias cianóticas complexas. Between 1979 and 1989, 84 patients underwent a Fontan operation or one of its modifications; the ages ranged from 5 months to 31 years. Patients were placed into five anatomic groups: 1) tricuspid atresia la (3 cases, 1 death, 33.3%); 2) tricuspid atresia lb (48 cases, 5 deaths, 10.4%); 3) single ventricle (18 cases, 2 deaths, 11.1%); 4) pulmonary atresia, intact ventricular septum (5 cases, 1 death, 20.0%); 5) complex anomalies (10 cases, no deaths). The subgroup of complex anomalies was divided in: with situs inversus and with situs solitus. In the majority of these patients, additional procedures were performed; in 5, one A-V valve was closed; in 2, complex atrial septation was done; in 1, a mitral valvuloplasty and the septation of the single atrium were performed; in 1, the section of an anomalous pathway (Kent bundle) was carried out.