Functional Single Ventricle Research Articles

Risk Factors for Semilunar Valve Insufficiency After the Damus-Kaye-Stansel Procedure

Cardiac function and survival after the Damus-Kaye-Stansel (DKS) procedure are encouraging, but only limited data exist related to postprocedural semilunar valve function. We investigated postprocedural midterm to long-term outcomes and changes in semilunar valve function and identified risk factors of semilunar valve function deterioration. Between 1996 and 2012, 63 patients with a single functional ventricle underwent the DKS procedure. Of them, 50 had previously undergone pulmonary artery banding. Cardiac function was measured by catheter examination 5.3 months (interquartile range, 2.6 to 9.7) preoperatively and 1.2 years (interquartile range, 1.0 to 1.4) postoperatively. Echocardiographic examination of the semilunar valve was performed concurrently with the catheter examination and at the last follow-up (5.3 years [interquartile range, 3.1 to 9.2] postoperatively). The overall survival rate at 1, 5, and 10 years postoperatively was 0.97, 0.92, and 0.89, respectively. Aortic and pulmonary valve regurgitation grade 1 year postoperatively and at the last follow-up increased compared with the preoperative grade. There was no significant difference between regurgitation at 1 year and the last follow-up. The duration from pulmonary artery banding to the DKS procedure was longer in the group with at least mild regurgitation (n = 6) than in the group with less than mild regurgitation (n = 54). Although the duration from pulmonary artery banding to the DKS procedure was associated with postoperative regurgitation, neither postprocedural aortic nor pulmonary valve regurgitation increased over time, and there were no deleterious effects on the clinical conditions.

Amino-Terminal proB-Type Natriuretic Peptide Levels in the Umbilical Cord Blood of Neonates Differ According to the Type of Prenatally Diagnosed Congenital Heart Disease.

The aim of this study was to investigate differences in amino-terminal proB-type natriuretic peptide (NT-proBNP) levels in the cord blood of neonates according to the type of congenital heart disease (CHD) and to evaluate the usefulness of NT-proBNP as a prognostic marker. We included 76 neonates with prenatally diagnosed CHD and 45 controls without CHD. Neonates were classified into five groups based on echocardiographic findings. The levels of NT-proBNP in the cord blood were examined and analyzed according to the neonatal outcomes. The levels of NT-proBNP were significantly elevated in the cord blood of neonates with CHD compared with that in the cord blood of controls. The levels of NT-proBNP in the group with right ventricular outflow tract obstruction without a ventricular septal defect were significantly increased compared to that in the other groups. The neonates that required acute surgical correction had higher levels of NT-proBNP in the cord blood, though they were not statistically significant. Meanwhile, NT-proBNP levels in the cord blood of neonates with functional single ventricle were significantly higher than that in the cord blood of those with functional biventricles. Significant differences in the levels of NT-proBNP between survivors and nonsurvivors were observed within 1year of birth. In this study, we found that the levels of NT-proBNP in the cord blood of neonates with CHD were higher than the levels in controls. This finding was striking in the group with right ventricular outflow tract obstruction, and it was associated with surgery for functional single ventricle and 1-year survival.

Fontan Completion for Younger than 3 Years of Age: Outcome in Patients with Functional Single Ventricle

Most single ventricle patients undergo Fontan procedure earlier in current era. However, optimal timing of Fontan completion after an intermediate staging surgery is controversial. We present children who had Fontan surgeries younger than 3years of age comparing the data with older patients. Between 2000 and 2013, 45 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) using GORE-TEX(®) conduit at a single institution. Children were divided into group A (age<36months; n=15) and group B (age≥36months; n=30), and retrospectively reviewed. Median follow-up was 2.9 [interquartile range (IQR) 2-4.4]years. There were two hospital deaths and one late death (all in group B, p=0.286). One TCPC was taken down in group A. The median intensive care unit and hospital stay were 90(IQR 46-140)h and 21(IQR 16-33)days for group A versus 65(IQR 45-115)h and 29 (IQR 20.8-38.5)days for group B, respectively (p=0.322 and p=0.057). Ventricular ejection fraction and GORE-TEX(®) conduit size were similar in both groups. The time of chest drain stay was significantly lower in group A (median 12days, IQR 7-22) than in group B (22days, IQR 16-32.5) (p=0.014). Extracardiac TCPC can be performed earlier with good intermediate results. Earlier Fontan procedure might be advantageous for reducing chest drains stay.

Evaluation of Left Ventricular Volumes and Function by Real Time Three-Dimensional Echocardiography in Children with Functional Single Left Ventricle: A Comparison between QLAB and TomTec.

Real time three-dimensional echocardiography (RT3DE) provides a reliable analysis of left ventricular (LV) volume. Despite a wide spectrum of tracking algorithms presently available, which software is most suitable for evaluating functional single left ventricle (FLSV) is unknown. Herein, we compared two of the most commonly used 3DE algorithms for quantification of LV volumes in the pediatric population with FLSV. Thirty-six children with FLSV were prospectively enrolled. The LV volume analysis was performed on QLAB 8.1 (semiautomated border tracking) and TomTec 4D LV 3.0 (manual dominant border tracking) and compared with MRI as the reference standard. 3DE volume quantification was achieved for 32 children with QLAB and 34 children with TomTec. Analysis time was much shorter for QLAB than TomTec (4.8 ± 1.2 vs. 6.3 ± 1.8 minutes, P < 0.05). Ejection fraction (EF) by either 3DE modality was significantly lower than the published normal values (P < 0.01 for each). End-diastolic volume (EDV), end-systolic volume (ESV), stroke volume, and EF calculated by both 3DE modalities underestimated MRI values. Compared to QLAB, TomTec showed better correlation and smaller intertechnique differences with MRI (the 95% limits of agreement, EDV: -20.84 to 5.18 mL in QLAB, -10.66 to 1.84 mL in TomTec; ESV: -8.94 to 3.07 mL in QLAB, -2.45 to 0.98 mL in TomTec; SV: -13.31 to 3.45 mL in QLAB, -9.34 to 2.0 mL in TomTec; EF: -12.07 to 7.76% in QLAB, -9.64 to 1.52% in TomTec), TomTec was more reproducible with better intraclass correlation coefficients and variation coefficients. Both 3DE modalities tend to underestimate LV volumes, but the correlation of LV volumes and EF between 3DE and MRI still holds well. Despite a longer operating time, TomTec analysis is more accurate and reproducible.

Elevated Aortic Augmentation Index in Children Following Fontan Palliation: Evidence of Stiffer Arteries?

Children born with a functional single ventricle who undergo Fontan palliation are prone to early pump failure. Whether they develop early arterial stiffness with resultant increase in afterload is not well known. We hypothesized that the aortic stiffness is higher in pediatric Fontan patients when compared to healthy controls. A prospective study was conducted at the Children's Hospital of Michigan. Twenty-two Fontan patients (aged 6-21 years) were compared with 22 healthy controls (aged 9-17 years) selected from children referred to our clinic who had normal cardiac anatomy and function on the echocardiogram. Aortic stiffness was assessed noninvasively by measuring the aortic augmentation index (AAI) using applanation tonometry (Sphygmocor, Atcor, IL). AAI was calculated as AP/PP where augmentation pressure (AP) is the increase in aortic systolic blood pressure (BP) and pulse pressure (PP) is the difference between aortic systolic and diastolic BP. Ten patients (45 %) had hypoplastic left ventricle, and 11 (50 %) had undergone aortic arch surgery. The median AAI was significantly higher in Fontan patients when compared to controls (12.5, IQR 4.8, 17.3 vs 0, IQR -6.3, 5.8; p = 0.0003). History of aortic arch surgery and single ventricle morphology did not have a significant impact on AAI. Pediatric patients who undergo Fontan palliation have significantly higher AAI, a marker of aortic stiffness and increased afterload, compared to healthy controls. Larger longitudinal studies are warranted to elucidate the possible contribution of elevated AAI on pump failure in these patients.

0450: Outcomes of pregnancy in women with Fontan palliation

Fontan palliation has improved long-term survival of (functional) single ventricles, allowing women to reach childbearing age. Outcomes of pregnancy are scarce and the management during pregnancy, as preventive anticoagulation, should be specified. We aimed to determine the outcomes of pregnancy in women with Fontan palliation. This retrospective multicentric study included women who had undergone Fontan palliation, followed in 12 French centers. All pregnancies were counted, including miscarriages and abortions. We observed maternal, obstetrical and neonatal outcomes, and we compared cardiovascular status before pregnancy and at the last follow-up. Twenty seven patients had 42 pregnancies (mean age=27±5 years old at first pregnancy). 15/42 (36%) were miscarriages and 24 were live births (62% of wanted pregnancies, 1 twin pregnancy). Cardiac complications occurred in 21% of pregnancies, there was no maternal death. They were essentially thrombo-embolic events (n=3) and supra-ventricular arrhythmias (n=2). Obstetrical complications occurred in 52% of pregnancies. They were premature rupture of membranes (n=5), preterm labour (n=3), placental abruption (n= 2). Hemorrhagic complications occurred in 3 women, 2 had curative anticoagulation. There were 78% of fetal/neonatal complications including one intrauterine death and one neonatal death. The main neonatal complication was the prematurity (n=17/24, 71%), and we observed 2 cases of congenital heart disease recurrence in fetuses (5%). Three severe complications occurred during postpartum period: 2 failing Fontan and 1 increase of NYHA functional class.Follow-up at mid-terms (median=5.6 months) showed no significant worsening of clinical status and cardiac function in patients. Women can successfully complete pregnancy after a Fontan palliation, only if pre-partum cardiac condition has been completely evaluated and is satisfactory. Anticoagulation should be recommended to prevent severe thrombo-embolic complications during pregnancy.

Intraatrial Conduit Fontan Procedure: Indications, Operative Techniques, and Clinical Outcomes

The intraatrial conduit (IAC) Fontan procedure is one of the Fontan modifications and is usually not considered the first choice. In this large series, we report our experience of the IAC Fontan procedure for the treatment of a functional single ventricle and review its indications, techniques, and clinical outcomes. Between 2009 and 2013, 101 patients with a functional single ventricle underwent an IAC Fontan procedure. The median surgical age was 44 months and weight was 15 kg. The cardiac malformations included tricuspid atresia in 10, double-inlet left ventricle in 8, double-inlet right ventricle in 15, double-outlet right ventricle in 18, congenitally corrected transposition of the great arteries in 11, complete atrioventricular septal defect in 8; criss-cross in 1, pulmonary atresia with intact ventricular septum in 3, Ebstein anomaly in 3, and others in 3. Heterotaxy syndrome was found in 21 patients. An intraatrial polytetrafluoroethylene conduit was implanted to construct the Fontan pathway. In 75 patients, a 2.7-mm to 4.5-mm fenestration was made to reduce the intraconduit pressure. Eighty-three patients had previously undergone a Glenn operation. Median follow-up was 29 months (range, 2 to 60 months). Doppler echocardiography, electrocardiography, and Holter monitoring were used to evaluate hemodynamic performance and arrhythmias. There were 2 hospital deaths, 1 Fontan takedown, and 3 midterm deaths. Conduit thrombosis developed in 1 patient. Atrial flutter developed in 2 patients, who underwent electrical cardioversion. Junctional bradycardia developed in 2 patients, but they did not require permanent pacemakers. Overall survival was 97.0% at 1 year and 94.1% at 5 years. The IAC modification provides excellent operative and midterm outcomes in most patients with a functional single ventricle. However, a longer follow-up time is required to demonstrate its real advantages.

Abstract 78: Late Gadolinium Enhancement on Cardiac MRI Identifies Ventricular Dysfunction and Regional Myocardial Dyssynchrony in Patients with Univentricular Heart Diseases

Backgrounds: The impact of myocardial fibrosis on cardiac performance and clinical outcomes in patients with a functional single ventricle before stage-3 operation is unknown. Objective: The purpose of this study is to investigate the prognostic value of myocardial fibrosis identified by cardiac magnetic resonance imaging (cMRI) in patients with univentricular heart diseases. Methods: Consecutive 23 patients undergoing staged-3 surgical palliation with single ventricle physiology were prospectively scheduled to have cMRI study with late gadolinium enhancement (LGE) imaging and ventricle circumferential strain were examined. Results: Of 23 patients (mean age 3.3±0.9 years), 6 (26%) had positive late gadolinium enhancement (LGE+) in the ventricular myocardium, median percent LGE was 3.0% (interquartile range 3.0% to 14.0%). Pre-Fontan examinations revealed that patients with LGE+ had increased end-diastolic volume index (142.8 ml/BSA vs. 113.8 ml/BSA; P=0.02), increased end-systolic volume index (101.0 ml/BSA vs. 72.2 ml/BSA); P=0.01) compared with those without LGE (LGE-). Patients with LGE have shown to have lower ventricular circumferential strain compared with the area without LGE (basal: −1.9±1.9% vs. −4.0±3.0%, P=0.046; mid: −3.9±2.1% vs −8.0±3.9%, P=0.007; apical: −3.9±2.4% vs. −8.2±2.8%, P=0.004). In contrast to LGE- group, patients in LGE+ group had decreased right ventricular ejection fraction (27.7±8.8% vs. 38.2±8.4%; P=0.02) as well as higher levels of BNP (99.2±75.7 pg/ml vs. 32.6±44.3 pg/ml, P=0.02). In addition, patients with LGE+ had higher score of Ross classification (2.5±0.55 vs. 2.0±0, P=0.02) and New York University Pediatric Heart Failure Index (11.0±3.5 vs. 7.8±1.1, P=0.01) than in LGE- group. Age at stage-2 palliation was significantly older in patients with LGE+ group than LGE- subjects (16.8±16 months vs. 8.8±3.4 months, P=0.03). Conclusion: In this pre-stage-3 cMRI study, the age to stage-2 palliation may attribute to substantial myocardial fibrosis. The area of LGE was associated with impaired regional circumferential strain as well as disturbed ventricular performance. This novel strategy may provide a possible prognostic value of latent myocardial dysfunction after staged palliation.

Primary Central Pulmonary Artery Plasty for Single Ventricle With Ductal-Associated Pulmonary Artery Coarctation

Infants with a single ventricle who require a modified Blalock-Taussig shunt can have stenosis at the ductal insertion site on the pulmonary artery (PA). We hypothesized that primary PA plasty in the first palliation would limit PA stenosis and unbalanced pulmonary perfusion, and thereby facilitate safe accomplishment of the next operation. From 1998 to 2012, functional single-ventricle patients with pulmonary atresia were managed as follows: initial operative strategy based on midline approach, using cardiopulmonary bypass, and central PA plasty (resection of all ductal tissue). All patients underwent catheter study and lung perfusion test before bidirectional cavopulmonary shunt (BCPS). Forty consecutive patients (heterotaxy, n = 27; others, n = 13) were enrolled in this study. Seven patients (17.5%) had repair of obstructed total anomalous pulmonary venous drainage, and 4 (10%) had repair of common atrioventricular valve at the first palliative operation. Fifteen patients (37.5%) underwent first palliation in the neonatal period. The mean PA index (mm(2)/m(2)) before BCPS was 230.7 ± 101.7. No pulmonary stenosis was detected before BCPS. Pulmonary artery diameter ratio (nonshunt side/shunt side) was 0.93 ± 0.25; pulmonary lung perfusion ratio (nonshunt side/shunt side) was 0.9 ± 0.2. A multivariate analysis identified persistent total anomalous pulmonary venous drainage (extracardiac type; p = 0.002) as a factor associated with unbalanced branch pulmonary arteries at BCPS. Thirty-five patients underwent BCPS, and 31 underwent Fontan procedures. There were 4 early deaths and 4 late deaths. The strategies we have employed facilitate safe accomplishment of the next-stage operation in diverse anatomic groups, with avoidance of unbalanced pulmonary vascular bed.

New mechanistic and therapeutic targets for pediatric heart failure: report from a National Heart, Lung, and Blood Institute working group.

Pediatric heart failure (HF) is the inability of the heart of an infant, child, or adolescent to meet the body’s metabolic demands. It involves circulatory, neurohumoral, and molecular abnormalities that manifest as edema, respiratory distress, growth failure, and exercise intolerance. The myriad causes include inherited and acquired myocardial anomalies (cardiomyopathy [CM]), volume overload (intracardiac shunts, valvular regurgitation), and the unique hemodynamics predicated by a functional single ventricle (palliated complex congenital heart disease [CHD]). Although the societal and financial costs of adult HF are well known, the burden of pediatric HF is less familiar, but no less onerous. New-onset HF requiring hospital admission occurs in 0.87 per 100 000 children,1 yet that does not include the growing population with CHD-related HF. In 2006, there were nearly 14 000 pediatric hospitalizations for HF from all causes in the United States.2 The rate of HF-related admissions was nearly 18 per 100 000 children,2 which is comparable to severe sepsis.3 The mortality for pediatric HF hospitalizations is significant. The 7% overall hospital mortality rate exceeds the 4% mortality of adult HF admissions4 and represents a 20-fold increase over children without HF.2 With comorbidities like renal failure, sepsis, or stroke, hospital mortality in pediatric HF can exceed 20%,2 yet the risk does not end with discharge. After an initial HF hospitalization, only 21% of children in 1 study avoided readmission, death, or transplantation.5 Pediatric HF treatment is resource intensive. Although the total healthcare costs for pediatric HF are lower than for adults, per-patient costs are higher. The estimated hospital charge per pediatric HF admission in 2006 was >$135 000, with aggregate charges exceeding $1.8 billion.6 Certain subpopulations of pediatric HF incurred disproportionally higher costs. For example, single-ventricle CHD averaged >$200 000 per hospitalization,7 whereas adult …

Completion of the Three-Stage Fontan Pathway Without Cardiopulmonary Bypass.

The three-stage surgical approach is now accepted as the standard for management of children born with functional single ventricle. However, there is little consensus on the cardiopulmonary bypass strategies employed for these procedures. We have attempted to avoid cardiopulmonary bypass in patients with single ventricle whenever possible to eliminate the adverse effects that are induced by this process. The purpose of this study was to review our experience in patients who underwent all three stages of the Fontan pathway without ever being exposed to bypass. A total of 52 patients with single ventricle underwent "off-pump" treatment at all three stages of their surgical management. The time period of the study was from 2002 to 2013. There were 31 males and 21 females. Anatomic diagnoses included double inlet left ventricle (n = 11), pulmonary atresia with intact ventricular septum (n = 11), tricuspid atresia (n = 10), double outlet right ventricle (n = 9), and other (n = 11). There was no operative mortality in the 52 patients undergoing Fontan completion. The patients have been followed for an average of 5.1 ± 2.5 years, with one late mortality. The median length of hospital stay for the three stages was 17, 5, and 9 days, respectively. Of the 52 patients, 42 were able to undergo all three stages without the need for a blood transfusion. This series demonstrates the feasibility of achieving a Fontan circulation without patients exposed to cardiopulmonary bypass. There was no operative mortality and low mid-term mortality. It is notable that 80% of patients never required a blood transfusion with this approach. The elimination of cardiopulmonary bypass provides several potential clinical benefits in this highly select subset of patients with single ventricle.

Influence of Bidirectional Cavopulmonary Anastomosis and Concomitant Valve Repair on Atrioventricular Valve Annulus and Function

The relationship between atrioventricular valve regurgitation (AVVR) and valve annulus after bidirectional cavopulmonary anastomosis (BCPA) and adequate indications for valve repair are unclear. We evaluated the size of the valve annulus and the grade of AVVR before and immediately after BCPA, and at the most recent follow-up before the Fontan operation in 37 patients with a functional single ventricle. Nine patients underwent concomitant valve surgery. The mean z value of the valve annulus was significantly lower postoperatively than preoperatively in the 28 patients who were not treated by valve surgery (0.45 vs 1.51, p=0.01). However, mean regurgitation scores did not significantly change after BCPA (1.60 vs 1.78, p=0.08). The most recent assessment showed that the mean z value increased compared with that immediately after BCPA (1.36 vs 0.45, p=0.005). This increase was significant in the patients with moderate regurgitation. The mean z value of the valve annulus of the patients treated by concomitant valvuloplasty was significantly lower postoperatively than preoperatively (-0.25 vs 3.9, p=0.0001) and remained low at the latest evaluation. Mean regurgitation scores also significantly decreased after BCPA (2.25 vs 3.37, p=0.007). Unloading the systemic ventricle by BCPA leads to a decrease in the relative size of the atrioventricular valve. However, this decrease does not improve the degree of AVVR in the absence of concomitant valve repair. Concomitant valve repair is justified in patients with moderate or worse AVVR and an abnormal valve structure.

MRI of lymphatic abnormalities after functional single-ventricle palliation surgery.

Protein-losing enteropathy (PLE) and plastic bronchitis are serious complications that occur after single-ventricle surgery. A lymphatic cause for these conditions has been proposed, but imaging correlation has not been reported. The objective of this study was to evaluate lymphatic abnormalities in patients after functional single-ventricle palliation compared with patients with non-single-ventricle congenital heart conditions using T2-weighted MR lymphangiography. We retrospectively reviewed imaging data from 48 patients who underwent T2-weighted MR lymphangiography in our institution between May 1, 2012, and October 24, 2012. The patients were divided into four groups: patients who underwent superior cavopulmonary connection, patients who underwent total cavopulmonary connection, patients with total cavopulmonary connection and lymphatic complications, and patients with non-single-ventricle cardiac anomalies. There were 38 patients with single ventricles in this study. The lymphatic abnormalities observed in these patients included thoracic duct dilation greater than 3 mm (31%), lymphangiectasia and lymphatic collateralization (78%), and tissue edema (86%). There were five patients with PLE, one patient with plastic bronchitis, and one patient with chronic chylous effusions and superior cavopulmonary connection. The patients with PLE and plastic bronchitis had statistically significant larger thoracic duct maximal diameters (median, 3.9 mm; range, 3-7.2 mm) than did the other patients with total cavopulmonary connection (p < 0.01). In the two-ventricle patient group, there were no abnormal lymphatic findings. Lymphatic abnormalities are found in many patients after functional single-ventricle palliation. T2-weighted unenhanced MRI is capable of anatomic assessment of the lymphatic system in this patient population and has promise for guiding treatment in the future.

Personalizing the Management of Heart Failure in Congenital Heart Disease: Challenges and Opportunities

Heart defects are among the most prevalent congenital anomalies, afflicting an estimated nine per 1000 live births [1]. Over the past few decades, major advances in care have resulted in a dramatic reduction in mortality, particularly in children with severe forms of congenital heart disease (CHD), giving rise to a rapidly growing and aging population of survivors [2]. This medical triumph is, however, tempered by the realization that following corrective surgery, patients cannot be considered cured by virtue of common sequelae and long-term complications related to their CHD, surgical repair, and hypoxic, thromboembolic, and/or hemodynamic ramifications. Heart failure (HF) is a major source of morbidity in CHD that, in some series of patients, has surpassed sudden cardiac death as the leading cause of mortality [3]. Mechanisms for HF are varied, reflecting heterogeneous physiologies that may include circulatory overload from shunts, pressure or volume overload from dysfunctional or underdeveloped valves, a morphologic subaortic right ventricle exposed to the systemic circulation, a single functional ventricle, and disorders of the electrical conduction system, pulmonary vasculature, myocardial structure and coronary blood supply [4]. Moreover, characteristics that define HF in the setting of CHD remain poorly defined. For example, the New York Heart Association functional class is of questionable relevance to patients with life-long adaptation to their inborn heart defect and in whom limited functional capacity is the norm. While indicators of neurohormonal activation are increased in the setting of complex CHD [5], concentrations are generally lower than standard adult

Neurodevelopmental outcomes in preschool survivors of the Fontan procedure

The study objectives were to compare the neurodevelopmental outcomes of preschool survivors of the Fontan procedure with those of children with congenital heart disease undergoing biventricular repair and to investigate predictors of neurodevelopmental outcome for those with single ventricle congenital heart disease, including hypoplastic left heart syndrome. Neurodevelopmental outcomes were assessed at 4 years of age, including cognition, visual-motor integration, behavior, social skills, and academic achievement. Unadjusted outcomes were compared between patients with biventricular circulation and patients with single ventricles. Predictors of neurodevelopmental outcome were assessed in the patients with single ventricles. Multiple covariate models were evaluated using patient-related, operative, and postoperative covariates. Neurodevelopmental evaluation was performed in 365 children, 112 after the Fontan procedure (hypoplastic left heart syndrome, n=91; other single ventricle, n=21) and 253 after biventricular repair. Compared with patients with biventricular circulation, patients with single ventricles performed worse in terms of processing speed, inattention, and impulsivity. Otherwise, there were no significant differences between the groups for any domain. There was a trend toward lower performance for patients with single ventricles on visual motor integration. Outcomes for patients with hypoplastic left heart syndrome were not worse than for other forms of functional single ventricle. Patient factors were more important predictors of neurodevelopmental outcomes than were operative management variables. In this cohort, unadjusted neurodevelopmental outcomes for preschool survivors of the Fontan procedure are similar to those for children with congenital heart disease undergoing biventricular repair for most domains. Among the patients undergoing the Fontan procedure, hypoplastic left heart syndrome was not associated with worse outcomes compared with other forms of single ventricle.

Functional Assessment for Congenital Heart Disease

Significant improvement in survival of children with congenital cardiac malformations has resulted in an increasing population of adolescent and adult patients with congenital heart disease. Of the long-term cardiac problems, ventricular dysfunction remains an important issue of concern. Despite corrective or palliative repair of congenital heart lesions, the right ventricle, which may be the subpulmonary or systemic ventricular chamber, and the functional single ventricle are particularly vulnerable to functional impairment. Regular assessment of cardiac function constitutes an important aspect in the long-term follow up of patients with congenital heart disease. Echocardiography remains the most useful imaging modality for longitudinal monitoring of cardiac function. Conventional echocardiographic assessment has focused primarily on quantification of changes in ventricular size and blood flow velocities during the cardiac cycles. Advances in echocardiographic technologies including tissue Doppler imaging and speckle tracking echocardiography have enabled direct interrogation of myocardial deformation. In this review, the issues of ventricular dysfunction in congenital heart disease, conventional echocardiographic and novel myocardial deformation imaging techniques, and clinical applications of these techniques in the functional assessment of congenital heart disease are discussed.

Comparison of myocardial rotation and torsion in asymptomatic children with single RV, single LV and normal hearts

Background Abnormal myocardial rotation and torsion have been investigated earlier as markers of ventricular failure1-2. However, there are no previous studies on global or regional torsion in functional single ventricle (SV) or the contribution of torsion to biomechanical differences between single RV (SRV) and single LV (SLV) hearts. Additionally, regional variations in rotation between the ventricular free wall and the septal wall which borders the hypoplastic chamber in SV have not been studied. Purpose: In normal subjects and in asymptomatic patients with SLV and SRV after total cavopulmonary connection (TCPC), to compare: 1) Global rotation (ROTglo). 2) Regional rotation in systole at free wall (ROTfree) and septal wall (ROT sept) for each slice location. 3) Torsion across the ventricle from apex to base.

横隔神経麻痺を合併した機能的単心室の肺循環動態：横隔膜縫縮術およびFontan型手術前後で左右の肺循環評価を行った1例

横隔神経麻痺を合併した機能的単心室の肺循環動態：横隔膜縫縮術およびFontan型手術前後で左右の肺循環評価を行った1例

Long-term outcomes of common atrioventricular valve plasty in patients with functional single ventricle

Common atrioventricular valve (CAVV) regurgitation is widely known as a risk factor for mortality and Fontan completion in patients with functional single ventricle. Hence, we reviewed our surgical experience with CAVV plasty in Fontan candidates. Staged Fontan strategy and extracardiac total cavopulmonary connection as Fontan modification were our principal approaches in 1995. Since then, 38 consecutive Fontan candidates (21 males, median weight at operation was 7.0 kg and median age was 17 months old) underwent CAVV plasty. Right atrial isomerism was associated with 24 patients. The initial CAVV plasty was performed before inter-stage bidirectional Glenn (BDG) in 3 patients, at BDG in 23, before Fontan in 4 and during Fontan in 8. Since 1995, the modified Alfieri technique with a tailed, expanded, polytetrafluoroethylene tube as a bridging strip was the procedure for repair and 27 patients underwent the procedure. The mean follow-up period was 7.1 years (range 0-17 years). Actuarial survival and freedom from CAVV replacement rates at 1, 5 and 10 years were 81, 70 and 67% and 89, 85 and 75%, respectively. Seven patients ultimately underwent CAVV replacement with one death. Twenty-three of the 38 patients completed Fontan operation (61%). Association with total anomalous pulmonary venous connection (P= 0.01) and CAVV plasty before BDG (P= 0.05) were risk factors for mortality. CAVV plasty for patients with functional single ventricle is still challenging; however, the aggressive and repeated surgical intervention may contribute to provide better life-prognosis. The ventricular volume unloading effect of BDG without additional pulmonary blood flow or Fontan operation did not contribute to maintain CAVV function. Therefore, there would not be any hesitation for CAVV replacement to control CAVVR in the setting of systemic ventricular failure. Although the statistically significant therapeutic superiority of the modified Alfieri technique was not shown so far, further follow-up may reveal the advantage of this easy and simple technique.

Magnetic resonance imaging-guided surgical design: can we optimise the Fontan operation?

The Fontan procedure, although an imperfect solution for children born with a single functional ventricle, is the only reconstruction at present short of transplantation. The haemodynamics associated with the total cavopulmonary connection, the modern approach to Fontan, are severely altered from the normal biventricular circulation and may contribute to the long-term complications that are frequently noted. Through recent technological advances, spear-headed by advances in medical imaging, it is now possible to virtually model these surgical procedures and evaluate the patient-specific haemodynamics as part of the pre-operative planning process. This is a novel paradigm with the potential to revolutionise the approach to Fontan surgery, help to optimise the haemodynamic results, and improve patient outcomes. This review provides a brief overview of these methods, presents preliminary results of their clinical usage, and offers insights into its potential future directions.