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Background Long‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. Methods and Results This nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow‐up was 98.7% complete (10 patients emigrated). Mean follow‐up was 11.3 years (maximum, 26.7 years). Long‐term survival was significantly higher in patients with left ventricular compared with RV dominance (10‐year survival: 91.0% [95% CI, 87.3%–93.6%] versus 71.1% [95% CI, 66.4%–75.2%]). RV dominance had a significant impact on outcomes after first‐stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology. Conclusions This study provides clinically relevant knowledge about the long‐term prognosis in patients with different underlying cardiac anomalies undergoing single‐ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.

Objective: This study applied preoperative computed tomography angiography (CTA) and computational fluid dynamics (CFD) simulation to predicte and verify the outcome of Y-shaped extracardiac conduits Fontan for functional single ventricle. Methods: Based on the preoperative CTA data of functional single ventricle (FSV), 4 types of spatial structures of extracardiac conduits were designed for 4 experimental groups: Group A, a traditional TCPC group (20 mm); Group B, a diameter-preserving Y-shaped TCPC (YCPC) group (branch 10 mm); Group C, YCPC group (branch 12 mm); and Group D, an area-preserving YCPC group (branch14 mm). Four indicators including flow velocity, pressure gradient (PG), energy efficiency and inferior vena cava (IVC) blood flow distribution were compared. The optimal procedure was applied. The radionuclide lung perfusion, CTA, echocardiography, cardiovascular angiography and catheterization were performed postoperatively. Results: There were the lowest PG, the lowest flow velocity of branches, the highest energy efficiency, and a relatively balanced and stable distribution of IVC flow for group D. Subsequently, the group D, a handcrafted Y-shaped conduit (14 mm) was used for the YCPC procedure. There was no postoperative PG between the conduit and pulmonary artery with normal pressure and resistance. IVC flow was distributed uniformly. Conclusion: CTA-based CFD provided more guidance for the clinical application of TCPC. A comprehensive surgical design could bring good postoperative outcome. Area-preserving YCPC has more advantages than TCPC and the diameter-preserving YCPC. The study effectively improved the feasibility of clinical applications of YCPC. [ABSTRACT FROM AUTHOR]

Background: To assess the impact of our surgical strategy for the treatment of patients with functional single ventricle and pulmonary hypertension, especially in patients>24 months old. Methods We retrospectively analyzed the clinical data of 97 patients with functional single ventricle and pulmonary hypertension undergoing pulmonary artery banding (PAB) in our hospital between April 2010 and December 2018. The surgical results, transition to Glenn operation and subsequent transition to Fontan operation were analyzed. Results: The 97 patients underwent PAB included 34 patients>24 months old, hospital mortality was 2.1% (2/97). 62 patients underwent the second-stage Glenn operation, and 21 patients underwent third-stage Fontan operation. On competing risk analysis, at 80 months after PAB, 81% had undergone the Glenn operation, and 13% were awaiting the Glenn operation. At 35 months after the Glenn operation, 2% of patients had died, 63% had undergone the Fontan operation, and 36% were awaiting the Fontan operation. Conclusion: PAB is an acceptable strategy for patients with functional single ventricle associated with pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good included patients>24 months. Accompanied with unbalanced atrioventricular septal defect for lower ratio of transition to Glenn and Fontan operation.

Ongoing concerns with single-ventricle palliation morbidity and poor outcomes from primary biventricular strategies for neonates with borderline left heart structures have led some centers to attempt alternative strategies to obviate the need for ultimate Fontan palliation and limit the risk to the child during the vulnerable neonatal period. In certain patients who are traditionally palliated toward single-ventricle circulation, biventricular circulation is possible. This review aims to delineate the current knowledge regarding converting certain patients with borderline left heart structures from single-ventricle palliation toward biventricular circulation. [ABSTRACT FROM AUTHOR]

Background: The high incidence of postoperative pulmonary venous obstruction (PVO) is a major mortality‐associated concern in patients with right atrial isomerism and extracardiac total anomalous pulmonary venous connection (TAPVC). We evaluated new anatomical risk factors for reducing the space behind the heart after TAPVC repair. Methods: Eighteen patients who underwent TAPVC repair between 2014 and 2020 were enrolled. Sutureless technique was used in 12 patients and conventional repair in six patients. The angle between the line perpendicular to the vertebral body and that from the vertebral body to the apex was defined as the "vertebral‐apex angle (V‐A angle)." The ratio of postoperative and preoperative angles, indicating the apex's lateral rotation, was compared between patients with and without PVO. Results: The median (interquartile range) age and body weight at repair were 102 (79–176) days and 3.8 (2.6–4.8) kg, respectively. The 1‐year survival rate was 83% (median follow‐up, 29 [11–36] months). PVO occurred in seven patients (39%), who showed an obstruction of one or two branches in the apex side. The postoperative V‐A angle (46° [45°–50°] vs. 36° [29°–38°], p = 0.001) and the ratio of postoperative and preoperative V‐A angles (1.27 [1.24–1.42] vs. 1.03 [0.98–1.07], p = 0.001) were significantly higher in the PVO group than in the non‐PVO group. The cut‐off values of the postoperative V‐A angle and ratio were 41° and 1.17, respectively. Conclusion: A postoperative rotation of the heart apex into the ipsilateral thorax was a risk factor for branch PVO after TAPVC repair. [ABSTRACT FROM AUTHOR]

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

Inducing anesthesia and securing the airway without disrupting the patient's hemodynamic state are challenging in pediatric patients with a functional single ventricle (FSV). Here, the authors report effective use of a high-flow nasal cannula (HFNC) as a tool in providing oxygen supplementation for airway management in pediatric FSV patients with a history of a difficult airway. A female patient, aged 5 years 7 months, was admitted for extracardiac conduit Fontan procedure. The patient had a history of multiple failed attempts at endotracheal intubation and was diagnosed with retrognathia and severe oral trismus of less than 1 finger width. The patient had another event of mask ventilation failure after propofol sedation during the preoperative computed tomography scan a day before the surgery. The patient's preoperative cardiac catheterization report revealed that the Qp/Qs ratio of 0.82 at room air, and the patient's peripheral oxygen saturation (SpO 2) ranged from 70% to 80% at room air. On entering the operating room, the preoxygenation process began with HFNC at an oxygen flow of 16 L/min, with a fraction of inspired oxygen (F I O 2) set at 95% after light sedation with an intravenous bolus of midazolam, 0.1 mg/kg. After 4 minutes of applying HFNC, with SpO 2 rising from 76% to 98%, anesthetic medications were administered intravenously without a neuromuscular blocking agent to preserve spontaneous breathing. The patient was not ventilated with a facemask but instead left with HFNC in place for continuous supplemental oxygenation. The patient's airway was secured in a single attempt in 80 seconds. HFNC is an ideal option for oxygen supplementation during airway management of pediatric FSV patients, as their balance of pulmonary and systemic flow is perturbed easily by subtle physiologic alteration and therapeutic maneuvers during the induction of general anesthesia and highly susceptible to rapid desaturation and cardiovascular collapse, and should be considered as having a physiologically difficult airway. [ABSTRACT FROM AUTHOR]

Background: To assess the impact of our surgical strategy for the treatment of patients with functional single ventricle and pulmonary hypertension, especially in patients>24 months old. Methods We retrospectively analyzed the clinical data of 97 patients with functional single ventricle and pulmonary hypertension undergoing pulmonary artery banding (PAB) in our hospital between April 2010 and December 2018. The surgical results, transition to Glenn operation and subsequent transition to Fontan operation were analyzed. Results: The 97 patients underwent PAB included 34 patients>24 months old, hospital mortality was 2.1% (2/97). 62 patients underwent the second-stage Glenn operation, and 21 patients underwent third-stage Fontan operation. On competing risk analysis, at 80 months after PAB, 81% had undergone the Glenn operation, and 13% were awaiting the Glenn operation. At 35 months after the Glenn operation, 2% of patients had died, 63% had undergone the Fontan operation, and 36% were awaiting the Fontan operation. Conclusion: PAB is an acceptable strategy for patients with functional single ventricle associated with pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good included patients>24 months. Accompanied with unbalanced atrioventricular septal defect for lower ratio of transition to Glenn and Fontan operation. [ABSTRACT FROM AUTHOR]

Ongoing concerns with single-ventricle palliation morbidity and poor outcomes from primary biventricular strategies for neonates with borderline left heart structures have led some centers to attempt alternative strategies to obviate the need for ultimate Fontan palliation and limit the risk to the child during the vulnerable neonatal period. In certain patients who are traditionally palliated toward single-ventricle circulation, biventricular circulation is possible. This review aims to delineate the current knowledge regarding converting certain patients with borderline left heart structures from single-ventricle palliation toward biventricular circulation.

To date, despite improvement in survival rate following a Fontan operation, postoperative prolonged pleural effusion (PPE) has still remained a confounding complication of this procedure, which significantly contributes to morbidity and prolonged hospitalization. Our study aims to investigate risk factors associated with PPE after the extracardiac Fontan operation. From August 2012 to December 2019, we obtained clinical data from the medical records of 145 consecutive patients who were diagnosed with single-ventricle lesions and received an extracardiac Fontan operation at the E Hospital (Hanoi, Vietnam). PPE was defined as the need for a chest tube for > 14 days. Patients were divided into two groups, those with PPE (n = 29, 20.00%) and those without PPE (n = 116, 80.00%). During the pre-Fontan evaluation, significant differences between two groups were observed in PPE (p = 0.00), chylothorax (p = 0.045), pleurodesis (p = 0.045), position of thoracic and abdominal organs (p = 0.018), atrioventricular (AV) valve regurgitation (p = 0.030), and large aortapulmonary circulation (p = 0.041). During the Fontan evaluation, significant differences among two groups were seen in aortic cross-clamp time (p = 0.04), cardiopulmonary bypass time (p = 0.014), and mean pulmonary artery pressure (PAP) at Fontan (p = 0.0072). In multivariable analysis with logistic regression, a reduced model including independent predictors for PPE was found to be the NYHA class III (OR 4.93, 95% CI 1.19–20.50, p = 0.028), double-outlet right ventricle (DORV) with transposition of great arteries (TGA) (OR 31.00, 95% CI 1.35–711.63, p = 0.032), AV valve regurgitation (OR 70.73, 95% CI 3.28–1523.28, p = 0.007), ventricle-to-pulmonary artery shunt (OR 8.29, 95% CI 1.60–42.78, p = 0.012), PAI (OR 0.98, 95% CI 0.97–0.99, p = 0.002) at pre-Fontan, while, at Fontan, high PAP (OR 1.24, 95% CI 1.01–1.53, p = 0.046) was an independent predictor for PPE. In conclusion, the incidence of PPE was relatively low. The NYHA class III, primary anatomical diagnosis of DORV with TGA, pre-Fontan AV valve regurgitation, the existence of pre-Fontan ventricle-to-pulmonary artery shunt, low pulmonary artery index (PAI), and high PAP in the operation were identified as independent risk factors to predict PPE following a Fontan operation. As prior studies also investigated various risk factors influencing PPE, a preventive strategy that targets these factors combined with previous identified other risk factors might reduce the PPE incidence. [ABSTRACT FROM AUTHOR]

Objectives: Patients with unoperated functional single ventricle (FSV) rarely survive into adulthood with good functional status and there are few reports about surgical results of adult patients with FSV. This study retrospectively reviews our experience with surgery in adult patients with FSV.Methods: From January 2008 to September 2017, 65 adult patients with FSV underwent surgery in our hospital. Twenty underwent Blalock-Taussig shunt or bidirectional Glenn procedures in other hospitals prior, and four were lost to follow-up. Finally, 41 patients were included in this study.Results: The early postoperative mortality was 7.3% (3/41). Postoperative systemic arterial oxygen saturation (SpO2 , 83.7% ± 4.8%) was significantly higher than preoperative SpO2 (77.9% ± 10.1%, P < .01). The mean follow-up time was 3.9 ± 3.1 years (range 1-11 years). There was only one case of late mortality and massive hemoptysis was the cause of death. Right ventricular morphology and severe atrioventricular valve regurgitation (AVVR) were the risk factors of postoperative death for patients with pulmonary stenosis(PS). There was no death case in patients who received Fontan procedure. In a follow-up, we found the grade of AVVR was reduced and the grade of heart function (New York Heart Association) was improved.Conclusions: Adult patients with FSV can also undergo surgery. The mortality was acceptable and late results were satisfactory. After surgery, oxygen saturations increased, grades of AVVR decreased and the heart functions improved. Right ventricular morphology and severe AVVR were risk factors for patients with PS. [ABSTRACT FROM AUTHOR]

Prolonged pleural effusion after Fontan operation is a significant morbidity that leads to long hospital stays. We investigated the association of multiple risk factors, including clinical characteristics, hemodynamic parameters, and preoperative, operative, and postoperative factors, with prolonged pleural effusion after Fontan operation. Eighty-five patients who underwent a Fontan operation between January 2005 and June 2018 in our center were included in this retrospective study. Patients were divided into two groups: group 1 (n = 36, 42.4%) included those with prolonged pleural effusion, defined as lasting > 14 days after the Fontan operation, and group 2 included patients without prolonged pleural effusion. Patients with hypoplastic left heart syndrome (HLHS) were more prevalent in group 1 (n = 15, P = 0.006). No differences in age at Fontan operation, central venous pressure at Fontan operation, or hemodynamic parameters during the pre-Fontan evaluation were found between the two groups. In multivariable analysis, HLHS (P = 0.002), non-fenestration (P = 0.018), and high central venous pressure at bidirectional cavopulmonary shunt (BCPS) operation (P = 0.043) were independent risk factors for prolonged pleural effusion after Fontan operation. Adverse outcomes such as death, need for heart transplantation, and Fontan failure were not associated with prolonged pleural effusion. In conclusion, patients with HLHS and higher central venous pressure at BCPS were more likely to have a prolonged pleural effusion after Fontan operation, but fenestration was more likely to decrease prolonged effusion. We should consider closer management of fluid status before, during, and after surgery in patients with these risk factors after Fontan operation. [ABSTRACT FROM AUTHOR]

Background: Pacemaker implantation in patients with single ventricle is associated with poor outcomes.Objective: The purpose of this study was to determine the reasons for the poor outcomes of pacemaker implantation.Methods: We performed a retrospective chart review of patients with single ventricle who had undergone permanent pacemaker implantation. Patients were categorized into 3 groups based on the site of pacing and the proportion of ventricular pacing (VP) as follows: (1) atrial pacing group with atrial pacing only (n = 11); (2) low VP group with low daily VP proportion (<50%; n = 12); and (3) high VP group with high daily VP proportion (≥50%; n = 15). Pacing leads were placed at the epicardium in all patients.Results: No patients in the atrial pacing or low VP groups died, whereas the survival rate in the high VP group was 58.9% and 39.3% at 10 and 20 years, respectively, after pacemaker implantation. Among the post-Fontan patients, plasma brain natriuretic peptide (BNP) levels significantly increased with the proportion of VP: 11.7, 20.3, and 28.4 pg/mL in the atrial pacing, low VP, and high VP groups, respectively (P = 0.04). In the high VP group, the plasma BNP level was significantly lower in patients with an apical pacing lead than in those with a nonapical pacing lead (27.0 pg/mL vs 82.8 pg/mL, respectively; P = .03).Conclusion: A higher proportion of VP was associated with poor outcome and higher plasma BNP levels, probably due to ventricular dyssynchrony. In epicardial ventricular pacing, apical pacing is better to avoid the increase in ventricular stress and plasma BNP level. [ABSTRACT FROM AUTHOR]

To review the outcome after atrioventricular valve replacement in single ventricle patients. The medical records of 37 consecutive patients who underwent initial valve replacement between 2001 and 2016 were reviewed. Actuarial survival rates were 73%, 65%, and 59% at 1, 5, and 10 years, respectively. Multivariate analysis indicated body surface area <0.28 m2 (P = 0.007; hazard ratio, 31.1), preoperative inotropic support (P < 0.001; hazard ratio, 24.5), primary valve replacement (P = 0.044; hazard ratio, 6.1), oversized prosthesis (P = 0.001; hazard ratio, 14.5), and intra- or postoperative extracorporeal membrane oxygenation support (P < 0.001; hazard ratio, 53.2) were the risk factors for mortality. Cumulative incidences of redo replacement were 11%, 17%, and 17% at 1, 5, and 10 years, respectively. There was no risk factor for redo replacement. Among 11 patients undergoing valve replacement before or at the time of bidirectional cavopulmonary shunt, all 4 patients who reached Fontan completion survived. Among 13 patients undergoing valve replacement after bidirectional cavopulmonary shunt, only 3 patients reached Fontan completion. Among 11 patients undergoing valve replacement after Fontan completion, there were 3 operative mortalities and 2 late mortalities. For 7 of 8 hospital survivors, cardiac index improved from 2.2 L/min/m2 (interquartile range, 2.0-2.4) to 3.4 (2.8-3.7) (P = 0.002). Atrioventricular valve replacement was a reasonable choice of treatment for single ventricle patients. For small patients with impaired ventricular function who had no choice other than valve replacement, commercially available valves were oversized and outcomes remained poor. [ABSTRACT FROM AUTHOR]

Objective: To determine the clinical outcomes and factors influencing the results of late Fontan completion in functional single ventricle patients. Material and Methods: The authors retrospectively analyzed data in functional single ventricle patients that underwent late Fontan completion (age more than four-year-old) between January 2007 and December 2017. Results: The extracardiac conduit Fontan procedure was performed in thirty-six patients over four years old (median age 10 years). The early mortality rate was 11% and the overall mortality was 16%. According to the Cox regression analysis, pulmonary artery pressure greater than 15 mmHg was an unfavorable factor for hospital death. Other morbidities included persistent pleural effusion (44%), chylothorax (2.78%), early atrial tachycardia (5.56%), early graft thrombosis (5.56%), and late graft thrombosis (2.78%). Conclusion: Late extracardiac conduit Fontan completion yielded acceptable outcomes. The authors found pulmonary artery pressure greater than 15 mmHg to be a risk factor for early mortality. Prevalence of postoperative persistent pleural effusion was higher in older patients, while rates of other complications did not differ from other age groups. [ABSTRACT FROM AUTHOR]

Treatment of patients with functional single-ventricle physiology is achieved using surgical corrections known as the “Fontan Procedure”. Two separate blood circulations are created from the single-ventricle function. These are non-cyanogenic patients, but at high risk of developing HF. CRT implantation is an option to improve the functional class and EF.

In the followed article authors talking about the creation the need for developing a physical rehabilitation technology for this contingent of patients, bringing their physical development. The aim of the performed research was to determine the features of the physical development and its connection with the body of the children of school age who have a congenital heart disease with a functionally single heart' ventricle for developing the physical rehabilitation technology. There were performed an examination of the 163 children with functionally single heart' ventricle hospitalized for operative treatment in the State Center «Scientific and Practical Medical Center of the Pediatric Cardiology and Cardiosurgery of the Ministry of Health of Ukraine» (Kyiv, Ukraine) in the 2013 and 2014 years. The analysis of the physical development indicators of the children with functionally single heart' ventricle allowed to establish a more pronounced lagging in the physical development by dividing the length and body mass index into the centile corridors and Roerre's index, comparing with children with other congenital heart disease, determining the need for development for patients with functionally single heart' ventricle special physical rehabilitation technology. The physical rehabilitation technology of the children with functionally single heart' ventricle after a hemodynamic correction should include the principles of physical rehabilitation, pedagogical principles, age characteristics of children, the indicators of physical development, the quantitative indicators of the biogeometrical posture profile, features of hemodynamics, functional condition of cardiovascular and respiratory systems. [ABSTRACT FROM AUTHOR]

In the followed article authors talking about the creation the need for developing a physical rehabilitation technology for this contingent of patients, bringing their physical development. The aim of the performed research was to determine the features of the physical development and its connection with the body of the children of school age who have a congenital heart disease with a functionally single heart' ventricle for developing the physical rehabilitation technology. There were performed an examination of the 163 children with functionally single heart' ventricle hospitalized for operative treatment in the State Center «Scientific and Practical Medical Center of the Pediatric Cardiology and Cardiosurgery of the Ministry of Health of Ukraine» (Kyiv, Ukraine) in the 2013 and 2014 years. The analysis of the physical development indicators of the children with functionally single heart' ventricle allowed to establish a more pronounced lagging in the physical development by dividing the length and body mass index into the centile corridors and Roerre's index, comparing with children with other congenital heart disease, determining the need for development for patients with functionally single heart' ventricle special physical rehabilitation technology. The physical rehabilitation technology of the children with functionally single heart' ventricle after a hemodynamic correction should include the principles of physical rehabilitation, pedagogical principles, age characteristics of children, the indicators of physical development, the quantitative indicators of the biogeometrical posture profile, features of hemodynamics, functional condition of cardiovascular and respiratory systems. [ABSTRACT FROM AUTHOR]

Introduction A functionally single cardiac ventricle seen on foetal ultrasound scan carries a guarded prognosis. The antenatal diagnosis of anomalous pulmonary venous connection (APVC) remains challenging, if there is no associated structural cardiac abnormality. Antenatally, a combination of complex cardiac anomaly with suspected isomerism should raise the possibility of associated total anomalous pulmonary venous connection (TAPVC). There needs to be a high index of suspicion for TAPVC, in functional single ventricle and suspected isomerism, as this carries a very grim outcome postnatally. We illustrate foetal echocardiographic findings of suspected TAPVC and review outcomes of antenatal versus postnatal diagnosis of TAPVC with functional single ventricle. Methods We retrospectively reviewed our database over 13 years, focusing on foetal cardiac diagnosis, pregnancy outcomes, management and outcomes of livebirths with diagnosis of TAPVC with functional single ventricle. Results Thirteen patients were included in the review. For the nine antenatal patients, three pregnancies were terminated and six babies were born alive (four babies had compassionate care, two babies had cardiac surgery). One baby is alive at 8.5 years, after Fontan surgery. For the four postnatal patients, three babies had compassionate care (one alive at age 8.1 years) and one baby had cardiac surgery (died age nine weeks). Ten of the 13 patients have right atrial isomerism. Of these 10 patients, only two are alive. For the three non-isomeric babies, only one baby is still alive. There is heterogeneity of the type of TAPVC diagnosed with no particular group that offered better survival. Conclusion Antenatal diagnosis of TAPVC, even in the context of functional single ventricle remains challenging. If isomerism is suspected, targeted evaluation of pulmonary venous connection should be done. This combination of cardiac lesion carries a very grim outcome. The ability to make this diagnosis antenatally will add to the information and counselling given to these parents. [ABSTRACT FROM AUTHOR]

Background: Physical development of children is an important indicator of health. The study aimed to evaluate the dynamics of physical development indicators of children with functional single ventricle heart disease at the individual stages of physical rehabilitation. Methods: A group of 35 patients aged 6 to 14 years was examined during hospitalization; 31 of them underwent follow-up examinations after completion of the full course of physical rehabilitation. The following indicators were measured: the body weight, height, chest circumference, body surface area, and Rohrer index. Results: We did not find significant differences between the groups of boys and girls of the same age. A substantial share of children with functional single ventricle was characterized by reduced body weight, which was reflected by the distribution of Rohrer index, where the percentage of children with low values was 31.5%. After completion of the course of physical rehabilitation, the indicators of physical development were statistically improved and the share of children with low values of the Rohrer index decreased to 16.1%. Conclusions: Physical therapy positively affects the physical development of children with functional single ventricle heart disease. [ABSTRACT FROM AUTHOR]

OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern. [ABSTRACT FROM AUTHOR]

Background Real time three-dimensional echocardiography ( RT3 DE) 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 3 DE algorithms for quantification of LV volumes in the pediatric population with FLSV. Methods 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. Results 3 DE 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 3 DE 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 3 DE 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. Conclusions Both 3 DE modalities tend to underestimate LV volumes, but the correlation of LV volumes and EF between 3 DE and MRI still holds well. Despite a longer operating time, TomTec analysis is more accurate and reproducible. [ABSTRACT FROM AUTHOR]

Abstract: Objective: Fontan completion in patients with atrial isomerism, in which the inferior vena cava (IVC) and the hepatic vein (HV) drain separately, is technically challenging. Herein, we review our surgical approach to these patients. Methods: The medical records of 50 consecutive patients with atrial isomerism who underwent Fontan completion between 1998 and 2008 were reviewed retrospectively. Results: Separate HV drainage was present in 17 patients. Patients with interrupted IVC were excluded. Patient characteristics were as follows: median age, 26 months (range 15–149); median weight, 9.6kg (range 8.1–47.2); right atrial isomerism, 16 patients; and left atrial isomerism, one. The IVC and the separate HV at the level of diaphragm were contralateral in 16 patients, and ipsilateral in one. The surgical procedures for directing blood flow from the IVC and the separate HV to the pulmonary arteries were as follows: en bloc resection of the IVC and the HV and anastomosing these veins to an extracardiac conduit in 10 patients; connecting the IVC to the HV in a side-to-side fashion before anastomosing them to an extracardiac conduit in one; and lateral tunnel in another. When the IVC and the HV were widely separated by the vertebrae, we chose an intra-extracardiac conduit (intra-atrial septation) in four patients and an extracardiac conduit for the IVC and the right HV and lateral tunnel for the separate left HV in one. There was no mortality. Five re-operations were performed (pacemaker in two patients; one each of fenestration, release of outflow obstruction and ligation of collateral arteries). Sixteen patients underwent follow-up catheterisation, which revealed central venous pressure of 12.0±2.0mmHg and arterial oxygen saturation of 92%±6%. Conclusions: The mid-term results of the Fontan completion in patients with atrial isomerism and separate HV drainage were excellent. The distance between the IVC and the separate HV and the position of the vertebrae should be considered when choosing a surgical technique. [Copyright &y& Elsevier]

Abstract: Patients with functional single ventricle heart disease undergo a series of palliative surgeries, typically culminating in a Fontan-type circulation. For the past three decades, the primary cardiac imaging modalities used to follow such patients have been echocardiography and catheter angiography. With the advent and rapid development of cardiac magnetic resonance and cardiac computed tomography over the past decade, these modalities offer novel techniques and capabilities to evaluate the single ventricle circulation. This article reviews the surgical management of the patient with functional single ventricle, and then explores the role of various imaging modalities in this setting with an emphasis on these newer techniques. [Copyright &y& Elsevier]

Abstract: Objective: The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). Methods: Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0–744) days; median weight 3.2 (range 2.0–9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. Results: The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39–77%) and 54% (95% CI, 34–73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34–78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p =0.001, hazard ratio, 13.4; 95% CI, 2.8–64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p =0.024, hazard ratio, 23.4; 95% CI, 1.5–363.4) was a risk factor for recurrent PVS. Conclusions: The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved. [Copyright &y& Elsevier]

Objective: Overall mortality of the patients with a functional single ventricle is still high, though excellent mid- and long-term results of the modified Fontan procedure have been reported. This study assessed the factors that affect the surgical outcomes mainly in the pre-Fontan stage and performed long-term survival analysis. Methods: Between January 1988 and December 2000, 405 patients with a functional single ventricle underwent surgical interventions and were followed up until June 2001. The mean follow-up period was 74.5±69.4 months and 95% of the patients were followed up completely. Their median age was 2.5 months at the time of shunt or pulmonary artery banding (PAB), 8.6 months at BCPS, and 28.6 months at the Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, surgical pathways leading to the Fontan stage, age at operation, study periods, and type of the Fontan procedure were analyzed. The role of BCPS in the long-term results was evaluated. Results: Overall survival after birth was 60.1±2.8% at 10 years. In multivariate analysis, complete atrioventricular septal defect-typed lesion, pulmonary venous obstruction, total anomalous pulmonary venous connection, and interruption of aortic arch were risk factors for long-term survival, while pulmonary stenosis was demonstrated as a favorable prognostic factor. In this study, there was no significant survival difference between the early and late study period. Actuarial mortality in the pre-Fontan stage was 41.3% in the non-BCPS group and 16.3% in the BCPS group (P<0.001). The 10-year survival rates of the populations in staged and primary Fontan groups were not significantly different (P=0.24). The long-term survival rate of the atriopulmonary Fontan group was significantly lower than that of lateral tunnel Fontan (60.3±6.3% vs. 86.8±3.1% at 10 years, P=0.0001). Conclusion: This study revealed that the overall survival was disappointing and there were still problems that need to be solved in the pre-Fontan stage to improve the overall survival. The role of BCPS was not to contribute to the longer survival after Fontan operation, but to lower mortality in the pre-Fontan stage, which can offer a higher probability to proceed to the Fontan procedure successfully. [Copyright &y& Elsevier]

Background Functional single ventricle (FSV) is a spectrum of severe congenital heart disease, with multiple anatomic variations but similar surgical treatment strategies. FSV patients are living longer into adulthood compared to two or three decades ago, and they are more frequently undergoing imaging to assist in clinical and surgical management. Objective: To determine impact of ventricular morphology on myocardial deformation in patients with single ventricle. Material and Methods: From November 2017 to July 2018, 41 patients with single ventricle morphology or Physiology who were referred to Ain-Shams University hospitals for follow up Echocardiography or diagnostic cardiac catheterization were enrolled in the study. All patients subjected to history, clinical examination Echocardiography (conventional 2D and speckle tracking "global longitudinal strain and strain rate".Dominant ventricular strain was analyzed using conventional two-dimensional echocardiographic. The region of interest was obtained by tracing the dominant ventricle endocardial borders at endsystole. An automated software program was used to calculate the frame-to-frame displacements of speckle pattern within the region of interest throughout the cardiac cycle. Results: There is statiscially significance between the basic anatomy of the single ventricle and the ejection fraction (p value 0.001) and global longitudinal strain pattern by speckle tracking echocardiography with (p value 0.031) with no statistically effect of the side of dominance. Conclusion:: Regarding the side of dominant ventricle there is no significant relation between the side of dominant ventricle on myocardial deformation (by ejection fraction, global strain and strain rate by speckle tracking echocardiography) but there is significant relation between the ventricular morphology and the myocardial deformation that was assessed by by the ejection fraction and global longitudinal strain (by speckle tracking echocardiography) but with no significant effect on the strain rate (by speckle tracking echocardiography). [ABSTRACT FROM AUTHOR]

Objectives Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. Methods Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post–total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. Results The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality ( P = .20), intensive care unit stay ( P = .20), and incidence of prolonged effusion ( P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients ( P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). Conclusions The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort. [ABSTRACT FROM AUTHOR]

Objective The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. Methods Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. Results Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years ( P < .01) were worse than in those who did not require atrioventricular valve surgery. Conclusions Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory. [ABSTRACT FROM AUTHOR]