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Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Competing Interests: Declaration of Conflicting InterestsThe author declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Although the terms "single ventricle" and "univentricular heart" are frequently used to describe a variety of complex congenital heart defects, in fact, nearly all hearts have two ventricles, although one of the two may be too small to be functional. A better term for these hearts would therefore be "functional single ventricle.", (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.

Introduction: Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia., Materials and Methods: A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro-Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described., Results: Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3 years (interquartile range 4.3-6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12 mmHg (interquartile range 10-15), the Nakata index 272 mm 2 /m 2 (interquartile range 204-327), and the McGoon index (interquartile range 1.86-2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19 days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%., Conclusion: The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.

Tricuspid atresia with common arterial trunk is a very rare association in complex CHD. This association has even more infrequently been documented concomitantly with interrupted aortic arch. We present the diagnosis and initial surgical management of an infant with a fetal diagnosis of tricuspid atresia and common arterial trunk, with additional postnatal finding of interrupted aortic arch with interruption between the left common carotid and left subclavian artery. Due to the infant's small size, she was initially palliated with bilateral pulmonary artery bands and a ductal stent. This was followed by septation of the common arterial trunk and interrupted aortic arch repair and 4 mm right subclavian artery to main pulmonary artery shunt placement at two months of age. She was discharged home on day of life 81.

Introduction: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature., Methods: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed., Results: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later., Conclusions: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

Tricuspid atresia with an absent pulmonary valve is a rare congenital cardiac defect. Although extensive pathological reviews have been published in the past, there are only a handful of cases that have been successfully palliated to the stage of Fontan. We hereby describe the successful management of one such case and review the surgical strategies described in the literature., (© 2022 Wiley Periodicals LLC.)

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative courses.

Isoproterenol stress test during cardiac catheterization unmasked dynamic bulbo-ventricular foramen restriction in a 5-year-old boy with bidirectional Glenn anastomosis for tricuspid atresia/transposed great arteries and unexplained syncope., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: Tricuspid atresia with normally related great vessels (TA) is considered the optimal substrate for the Fontan pathway. The factors associated with death or transplantation after cavopulmonary shunt (CPS) are underappreciated. We aimed to determine factors associated with CPS-Fontan interstage death/transplantation versus transition to Fontan in TA., Methods: A total of 417 infants younger than 3 months of age with TA were enrolled (January 1999 to February 2020) from 40 institutions into the Congenital Heart Surgeons' Society TA cohort. Parametric competing risk methodology was used to determine factors associated with the competing end points of death/transplantation without Fontan completion, and transition to Fontan., Results: CPS was performed in 382 patients with TA; of those, 5% died or underwent transplantation without transition to Fontan and 91% transitioned to Fontan by 5 years after CPS. Prenatal diagnosis (hazard ratio [HR], 0.74; P < .001) and pulmonary artery band (PAB) at CPS (HR, 0.50; P < .001) were negatively associated with Fontan completion. Preoperative moderate or greater mitral valve regurgitation (HR, 3.0; P < .001), concomitant mitral valve repair (HR, 11.0; P < .001), PAB at CPS (HR, 3.0; P < .001), postoperative superior vena cava interventions (HR, 9.0; P < .001), and CPS takedown (HR, 40.0; P < .001) were associated with death/transplantation., Conclusions: The mortality rate after CPS in patients with TA is notable. Those with preoperative mitral valve regurgitation remain a high-risk group. PAB at the time of CPS being associated with both increased risk of death and decreased Fontan completion may represent a deleterious effect of antegrade pulmonary blood flow in the CPS circulation., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Tricuspid atresia is a congenital malformation of the tricuspid valve resulting in a lack of blood flow between the right atrium and the right ventricle. Management generally involves staged surgical intervention enabling affected individuals to survive into adulthood. Although surgical intervention greatly improves morbidity and mortality in this patient population, there are many long-term complications associated with the creation of a surgical shunt. We report a case of a 33-year-old male with tricuspid atresia who underwent Fontan surgery as a child and presented to our hospital with acute liver failure.

Background: COVID-19 and Fontan physiology have each been associated with an elevated risk of venous thromboembolism (VTE), however little is known about the risks and potential consequences of having both., Case Presentation: A 51 year old male with tricuspid atresia status post Fontan and extracardiac Glenn shunt, atrial flutter, and sinus sick syndrome presented with phlegmasia cerulea dolens (PCD) of the left lower extremity in spite of supratherapeutic INR in the context of symptomatic COVID-10 pneumonia. He was treated with single session, catheter directed mechanical thrombectomy that was well-tolerated., Conclusions: This report of acute PCD despite therapeutic anticoagulation with a Vitamin K antagonist, managed with emergent mechanical thrombectomy, calls to attention the importance of altered flow dynamics in COVID positive patients with Fontan circulation that may compound these independent risk factors for developing deep venous thrombosis with the potential for even higher morbidity., (© 2021. The Author(s).)

Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure., (© 2021. The Japanese Association for Thoracic Surgery.)

We present the electrocardiographic findings in a 36-year-old female with tricuspid atresia with double-outlet right ventricle and malposition of great arteries who underwent classical or "atriopulmonary" Fontan procedure in childhood. Her electrocardiograms have consistently shown marked intra-atrial delay with an initial positive P wave deflection and terminal negative P deflection in all leads with progressive increase in P wave duration with time. She has had frequent episodes of intra-atrial tachycardia, atrial fibrillation and sinus and post ectopic pauses over the past few years. The findings in our patient, which have not been reported before, illustrate the atrial pathology, which is unique to Fontan physiology, particularly those with classical or atriopulmonary Fontan. We hope that the specific electrocardiographic findings presented will allow for their recognition., Competing Interests: Declaration of Competing Interest None of the authors have any conflict relevant to the work presented., (Copyright © 2021 Elsevier Inc. All rights reserved.)

As several complications can develop after the atriopulmonary Fontan procedure, total cavopulmonary connection conversion is often considered. We report two cases in which total cavopulmonary connection conversion was performed with a modified Glenn procedure. We used an expanded polytetrafluoroethylene graft between the superior vena cava and extracardiac conduit. This procedure ensures that a balanced hepatic blood flow is perfused into both lungs to avoid pulmonary arteriovenous malformations.

Background: Fenestration of the baffle/conduit is believed to reduce pleural effusion following the Fontan operation. However, equivocal results have been observed with or without fenestration. This study aims to evaluate the efficacy of fenestration on the amount and duration of pleural effusion following the Fontan operation., Methods: About 40 patients undergoing extracardiac Fontan (ECF) were randomized into two groups: one with fenestration (ECF-F; n = 20) or without fenestration (ECF-NF; n = 20). Primary outcome was the amount and duration of pleural effusions. Secondary outcomes were time to removal of the chest tubes, hospital stay, and readmission to the hospital because of recurrent pleural within 30 days of the operation., Results: Mean age was 11.5 ± 5.07 (range, 8.7-13.5) years in the ECF-F group and 13.6 ± 0.4 years (range, 10.5-15.5) in the (ECF-NF) group. The total drain output was 7.89 mL/kg/d in ECF-NF compared with 6.9 mL/kg/d in the ECF-group (P = .14). Time for removal of pleural tubes was 14.6 ± 0.95 days in the ECF-NF group compared with 11.6 ± days in the ECF-F group. Total duration of hospital stay was higher but not significant in the ECF-NF group compared with the ECF-F group. Two patients in ECF-NF required readmission to the hospital within 30 days following discharge, while there were no readmissions in the ECF-F group., Conclusion: Contrary to the literature, the creation of a fenestration in the ECF circuit was not clearly associated with a reduction in the amount and duration of pleural effusion compared with a non-fenestrated Fontan. These findings may be debatable in high risks versus low risk candidates. However in the present study, in a low risk canditates undergoing the Fontan operation, the daily amount of pleural drainage was no different. Larger studies are needed to confirm these findings., (© 2020 Wiley Periodicals LLC.)

Tricuspid valve atresia with severe pulmonary stenosis is one of the common cyanotic diseases in neonate. Child can succumb due to profound cyanosis and arterial hypoxaemia after closure of patent ductus arteriosus. Evolving procedure of right ventricular outflow tract stenting may be considered as a palliative procedure in such vulnerable group, destined for a later definitive management. The right ventricular outflow tract stenting is described essentially for tetralogy of Fallot physiology with a catheter course across tricuspid valve. We describe a case of successful right ventricular outflow tract stenting in a 5-day-old symptomatic neonate. We discuss the possible routes and the tips to facilitate right ventricular outflow tract stenting in such a case. This happens to be the first reported case description with successful stenting of neonate with tricuspid atresia with critical pulmonic stenosis.

Neoaortic root dilation is a common phenomenon after the Norwood procedure, but the real incidence and its natural history are unclear. Regular surveillance in these patients after the operation is necessary. Herein, we present an 11-year-old boy born with tricuspid atresia, a discordant ventriculo-arterial connection and a hypoplastic aortic arch, who was palliated initially with a hybrid stage I procedure involving a reversed Blalock-Taussig shunt, followed by comprehensive stage II and then, an extra-cardiac fenestrated Fontan operation. The patient developed an aortic root aneurysm and severe aortic regurgitation. He was electively taken into the operating room, where cardiopulmonary bypass was established through a peripheral cannulation of the femoral vessels due to the high risk nature of the reoperation. A mechanical Bentall procedure was performed without residual lesions and the native ascending aorta was anastomosed as a single coronary button to the anterior wall of the graft., (© The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive., (© 2020 The Authors. Journal of Cardiac Surgery published by Wiley Periodicals LLC.)

It is unsettled whether increased exercise ventilation in Fontan subjects is due to increased pulmonary dead space or augmented ventilatory drive. Twenty-six Fontan patients underwent symptom-limited treadmill cardiopulmonary exercise testing. Two groups of age- and sex- matched subjects served as controls: the biventricularly repaired (Bi, n = 18), and the "true" control (C, n = 29) groups. Peak oxygen uptake (V̇O 2peak ) was not different among groups (41.0 +/- 8.4 ml/min/kg, 43.5 +/- 6.6 ml/min/kg, and 45.9 +/- 11.6 ml/min/kg for Fontan, Bi, and C groups, respectively, p = 0.16). Fontan subjects, however, showed steeper alveolar ventilation/carbon-dioxide (V̇A/V̇CO 2 ) regression slope (35.5 +/- 5.3, 28.7 +/- 3.8, and 29.5 +/- 3.0 l/ml, for Fontan, Bi, and C groups, respectively, p<0.0001), and lower end-expiratory carbon-dioxide fraction (FetCO 2VAT ) at ventilatory threshold (VAT) (4.4 +/- 0.5%, 5.5 +/- 0.5%, and 5.5 +/- 0.4%, for Fontan, Bi, and C groups, respectively, p<0.001). The dead-space ventilation fraction at VAT was similar among groups (0.33 +/- 0.06, 0.33 +/- 0.04, 0.35 +/- 0.05 for Fontan, Bi, and C groups, respectively, p = 0.54). In Fontan subjects, arterial oxygen saturation at rest (SaO 2rest ) was correlated with V̇A/V̇CO 2 regression slope (r = -0.41, p = 0.04) and with FetCO 2VAT (p = -0.53, p<0.01). We conclude that Fontan patients show exercise hyperventilation due to augmented central and/or peripheral ventilatory drive, which is further augmented by residual hypoxemia., Competing Interests: None.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.

Implantation of a permanent pacemaker is a negative prognostic marker in patients with Fontan palliation; however, data delineating outcomes in adult patients with pacemaker requirements are lacking. We hypothesize that high ventricular pacing burden is associated with adverse outcomes in adult Fontan patients. We performed a retrospective review comprising adult patients with history of Fontan repair. A high burden of ventricular pacing was defined as ≥40% pacing. Major adverse clinical events (MACE) were defined as all-cause mortality or need for advanced cardiac therapies (ventricular assist device or heart transplant). A total of 145 adult patients with Fontan were studied for a median of 3.1 years. Twenty (14%) patients had implanted pacemakers with ≥40% ventricular pacing. Twelve events occurred in those with ≥40% ventricular pacing (incidence 60.0%) versus 11 in those without (incidence 8.8%). In multivariable analysis, ≥40% ventricular-pacing (odds ratio 12.51, confidence interval [CI] 3.56 to 43.83, p <0.001) was associated with MACE independent of initial Fontan type, New York Heart Association functional class at baseline, or history of atrial tachyarrythmia. In survival analysis, patients with ≥40% ventricular pacing had nearly 8 times the risk of MACE compared with those with a lower ventricular pacing burden (hazard ratio 7.79, 95% CI 2.56 to 23.66, p <0.001), whereas patients with atrial-only or <40% ventricular pacing burden had a trend toward higher hazard of MACE compared with those without permanent pacemaker (hazard ratio 3.38, 95% CI 0.92 to 12.47, p = 0.07) that did not meet statistical significance. These findings suggest that high ventricular pacing burden contributes to poor outcomes in the adult Fontan patients and bear consideration when determining optimal treatment of tachyarrhythmias in this population., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: This study aimed to develop non-invasive machine learning classifiers for predicting post-Glenn shunt patients with low and high risks of a mean pulmonary arterial pressure (mPAP) > 15 mmHg based on preoperative cardiac computed tomography (CT)., Methods: This retrospective study included 96 patients with functional single ventricle who underwent a bidirectional Glenn procedure between November 1, 2009, and July, 31, 2017. All patients underwent post-procedure CT, followed by cardiac catheterization. Overall, 23 morphologic parameters were manually extracted from cardiac CT images for each patient. The Mann-Whitney U or chi-square test was applied to select the most significant predictors. Six machine learning algorithms including logistic regression, Naive Bayes, random forest (RF), linear discriminant analysis, support vector machine, and K-nearest neighbor were used for modeling. These algorithms were independently trained on 100 train-validation random splits with a 3:1 ratio. Their average performance was evaluated by area under the curve (AUC), accuracy, sensitivity, and specificity., Results: Seven CT morphologic parameters were selected for modeling. RF obtained the best performance, with mean AUC of 0.840 (confidence interval [CI] 0.832-0.850) and 0.787 (95% CI 0.780-0.794); sensitivity of 0.815 (95% CI 0.797-0.833) and 0.778 (95% CI 0.767-0.788), specificity of 0.766 (95% CI 0.748-0.785) and 0.746 (95% CI 0.735-0.757); and accuracy of 0.782 (95% CI 0.771-0.793) and 0.756 (95% CI 0.748-0.764) in the training and validation cohorts, respectively., Conclusions: The CT-based RF model demonstrates a good performance in the prediction of mPAP, which may reduce the need for right heart catheterization in post-Glenn shunt patients with suspected mPAP > 15 mmHg., Key Points: • Twenty-three candidate descriptors were manually extracted from cardiac computed tomography images, and seven of them were selected for subsequent modeling. • The random forest model presents the best predictive performance for pulmonary pressure among all methods. • The computed tomography-based machine learning model could predict post-Glenn shunt pulmonary pressure non-invasively.

Cavopulmonary anastomosis was first described by Carlon, Mondini, De Marchi in a canine model in 1951 and later, in the clinical practice, by Glenn in 1958. Total right heart bypass was first introduced by Fontan and Kreutzer in 1971, in each instance as treatment for tricuspid atresia. Several modifications of such a procedure followed the initial concept of the right atrium as a pumping chamber, including modifications aimed to minimize energy loss at the anastomotic level and arrhythmias. Tribute is given to our pioneers who developed such an operation aimed to treat any child with functionally univentricular hearts.

Enlargement of the bulboventricular foramen (BVF) in double-inlet left ventricle or the ventricular septal defect (VSD) in tricuspid atresia with transposition of the great arteries is one approach for prevention or treatment of systemic ventricular outflow obstruction. Most often, BVF/VSD restriction is bypassed preemptively or addressed directly at the time of Glenn/Fontan procedures as part of staged univentricular palliation. We describe a patient who underwent enlargement of a restrictive VSD during Fontan completion and subsequently presented with an asymptomatic pseudoaneurysm of the right ventricle at the ventriculotomy site.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.

Fontan associated liver disease is associated with morbidity and mortality in palliated single-ventricle congenital heart disease patients. Magnetic resonance elastography (MRE) provides a quantitative assessment of liver stiffness in Fontan patients. We hypothesized that MRE liver stiffness correlates with liver enzymes, hemodynamics, portal hypertension, and Fontan failure (FF). All adult Fontan patients who had MRE between 2011 and 2018 were included. Radiologic portal hypertension was defined as splenomegaly, ascites, and/or varices. FF was defined as death, transplantation, or heart failure symptoms requiring escalation of diuretics. Seventy patients with a median age of 24.7 years and a median follow-up from MRE of 3.9 years were included. The median liver stiffness was 4.3 kPa (interquartile range [IQR]: 3.8 to 5.0 kPa). There was a weak, positive correlation between liver stiffness and Fontan pathway pressure (r = 0.34, p = 0.03). There was a moderate negative correlation of liver stiffness with ventricular ejection fraction (r = -0.52, p = 0.03). Liver stiffness was weakly positively correlated with liver transaminases and gamma glutamyl transferase. Patients with portal hypertension had higher liver stiffness compared to patients without (5.2 ± 1.3 vs 4.2 ± 0.8 kPa, p = 0.03). At MRE or during follow-up, 13 patients (19%) met definition of FF and had significantly higher liver stiffness compared to patients without FF (5.1 [IQR: 4.3 to 6.3] vs 4.2 [IQR: 3.7 to 4.7] kPa, p = 0.01). Liver stiffness above 4.5 kPa differentiated FF with a sensitivity of 77% and specificity of 77%. In conclusion, elevated MRE-derived liver stiffness is associated with worse hemodynamics, liver enzymes and clinical outcomes in Fontan patients. This measure may serve as a global imaging biomarker of Fontan health., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Coexistence of tricuspid atresia and truncus arteriosus (common arterial trunk) is an extremely rare combination of anomalies and requires staged palliation. Initial palliation involves disconnection of the pulmonary arteries from the common trunk and creation of a systemic-to-pulmonary artery shunt. This has generally been accomplished with the use of cardiopulmonary bypass. We describe a technique of off-pump palliation using the common trunk and a modified Blalock-Taussig shunt as sources of pulmonary blood flow at various stages of reconstruction. The child has recovered well and has undergone second-stage palliation with bilateral bidirectional superior cavopulmonary anastomoses at one year of age.

Wolff-Parkinson-White (WPW) syndrome causes paroxysmal supraventricular tachycardia in which short PR intervals and delta waves are seen in electrocardiography, which may cause sudden cardiac death. A 19-year female presented with increasing episodes of wide and narrow QRS complex tachycardia for the past 5 years. She had tricuspid atresia and Fontan Bjork operation in her past history. She was then diagnosed with narrow QRS complex tachycardia; and WPW syndrome was discovered when she returned to sinus rhythm. Ablation was performed from the coronary sinus ostium region via the left subclavian vein. Fontan Bjork procedure leading to accessory connections stemming from the surgery in the atrio-infundibular anastomosis may be one reason for WPW syndrome. In this case, since the ablation area was close to the Fontan anastomotic line, it could not be determined clearly whether WPW syndrome was secondary to Fontan anastomosis or it was congenital occult WPW syndrome, which became overt following Fontan surgery.

A 42-year-old man was referred to our hospital with heart failure and unoperated tricuspid atresia with pulmonary valve stenosis. His condition was initially managed with medical therapy; however, he required repeat hospitalisations for congestive heart failure. We diagnosed the chief cause of his heart failure as aortic valve regurgitation secondary to aortic root dilatation. Aortic root replacement was performed and then his heart failure was controlled.

Background: In this study we sought to evaluate risk factors (RFs) for death or heart transplantation (D-HT) in single-ventricle (SV) physiology due to tricuspid atresia (TA), pulmonary atresia‒intact ventricular septum (PA-IVS), and heterotaxy with SV (HX), clinical conditions for which outcome data are limited., Methods: To conduct a systematic review, we included citations that evaluated occurrence of D-HT in SV physiology of TA, PA-IVS, and HX in English articles published between January 1998 and December 2017 based on inclusion and exclusion criteria, following the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. The Cochrane Risk of Bias in Non-Randomized Studies-Interventions (ROBINS-I) tool for non-randomized studies was used to assess the risk of bias. Meta-analysis was performed if RF data were available in more than 3 studies., Results: Of 11,629 citations reviewed, 30 met inclusion criteria. All 30 were observational, retrospective studies. In all, 1,770 patients were included, 481 died and 21 underwent HT (63 lost to follow-up); 723 patients reached Fontan completion. We found that systemic ventricular dysfunction (odds ratio [OR] 20.7, confidence interval [CI] 10.0-42.5, I 2  = 0%) and atrioventricular valve regurgitation (AVR) were associated with risk of D-HT (OR 3.7, CI 1.9-6.9, I 2  = 14%). RF associations with D-HT could not be derived for right ventricle‒dependent coronary circulation, pulmonary arteriovenous malformations, total anomalous pulmonary venous return, arrhythmias, and pulmonary atresia., Conclusions: This systematic review and meta-analysis has identified a high mortality rate in children born with non-HLHS SV heart disease and points to potential under-utilization of HT. Systemic ventricular dysfunction and AVR were identified as RFs for D-HT in this subset of patients SV with TA, PA-IVS, and HX., (Copyright © 2019 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Congenital heart disease (CHD) is the most common birth defect, affecting 1 in 100 babies. Among CHDs, single ventricle (SV) physiologies, such as hypoplastic left heart syndrome and tricuspid atresia, are particularly severe conditions that require multiple palliative surgeries, including the Fontan procedure. Although the management strategies for SV patients have markedly improved, the prevalence of ventricular dysfunction continues to increase over time, especially after the Fontan procedure. At present, the final treatment for SV patients who develop heart failure is heart transplantation; however, transplantation is difficult to achieve because of severe donor shortages. Recently, various regenerative therapies for heart failure have been developed that increase cardiomyocytes and restore cardiac function, with promising results in adults. The clinical application of various forms of regenerative medicine for CHD patients with heart failure is highly anticipated, and the latest research in this field is reviewed here. In addition, regenerative therapy is important for children with CHD because of their natural growth. The ideal pediatric cardiovascular device would have the potential to adapt to a child's growth. Therefore, if a device that increases in size in accordance with the patient's growth could be developed using regenerative medicine, it would be highly beneficial. This review provides an overview of the available regenerative technologies for CHD patients.

Background: Increased pulmonary vascular resistance index (PVR) leads to several complications in patients after a Fontan operation. This increase is mainly attributed to the overexpression of endothelin-1 for a long duration after the Fontan procedure. Here, we describe the case of a 3-year-old boy with a failed Fontan operation who was treated with bosentan, an endothelin-1 receptor blocker., Case Report: Cardiac catheterization was performed, which showed a main pulmonary artery pressure (MPAP) of 19 mmHg and PVRI of 5.6 woods/m2. Oral bosentan regimen at a dose of 31.25 mg was initiated twice a day. The treatment was continued as pleural effusion and ascites persisted. No adverse events were observed, and the treatment was well tolerated. Pleural effusion disappeared, and ascites decreased markedly after 4 weeks, whereas the MPAP was 15 mmHg and the PVRI was 4.3 woods/m2. After 3 months of bosentan therapy, the MPAP was 12 mmHg and the PVRI was 4.1 woods/m2., Conclusion: We observed that bosentan reduces the PVRI and complications such as pleural effusion and ascites after a failed Fontan procedure.

Background: Total Cavopulmonary connection (Fontan) is the final palliation for patients with a functionally univentricular heart. This is commonly accomplished after a prior bidirectional Glenn on cardiopulmonary bypass (CPB) with separate cannulation of the aorta, superior vena cava (SVC), and inferior vena cava. We describe an alternative technique of Fontan completion that eliminates the need for cannulation and dissection of the SVC, and pulmonary artery dissection., Methods: Between January and October 2018, 17 patients underwent completion Fontan using an alternate technique at our institute. All operations were conducted on CPB at normothermia without cannulating the SVC RESULTS: Mean CPB time was 60 ± 16.8 minutes (range, 39-102 minutes). There were no early deaths. Mean postoperative Fontan pressures were 15.6 ± 1.2 mm Hg with no gradient between the SVC and IVC pressures. Mean duration of hospital stay was 15.6 ± 3.6 days (range, 10-22 days). No patient developed phrenic nerve paresis or palsy., Conclusions: Completion without cannulating the SVC is simple, reproducible, and easy to teach. It avoids the disadvantages associated with routine techniques., (© 2019 Wiley Periodicals, Inc.)

Background: Patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries are predisposed to systemic outflow tract obstruction necessitating systemic outflow relief operations (SORO)., Methods: Between January 2000 and February 2018, 30 patients with double-inlet left ventricle (n = 20) or tricuspid atresia (n = 10) with transposition of the great arteries underwent single-ventricle palliation. Arch obstruction was observed in 14 patients. Initial palliative procedures comprised pulmonary artery banding with (n = 4) or without (n = 14) arch repair, bilateral pulmonary artery banding with ductal stenting (n = 5), primary Norwood operation (n = 4), and palliative arterial switch operation (n = 1). Cox proportional hazards model was fitted in 15 patients with initial postnatal echocardiography to identify risk factors for decreased time to SORO., Results: One early and one late death occurred during the median follow-up period of 66 months (10-year survival rate, 93.3%). Various types of SORO were required in 20 of 30 patients (66.7%): Damus-Kaye-Stansel procedure (n = 12), primary Norwood-type palliation (n = 4), palliative arterial switch operation (n = 1), and bulboventricular foramen extension (n = 3). Freedom from SORO at 5 years was 34.5% in all patients (N = 30). Cox regression for the subgroup (n = 15) revealed that arch obstruction (hazard ratio, 20.6; 95% confidence interval, 2.9 to 148.2; p = 0.003) and smaller systemic outflow tract area index at end-systolic phase (hazard ratio, 1.5 at 10 mm 2 /m 2 decrease; 95% confidence interval, 1.0 to 2.1; p = 0.033) were identified as risk factors for decreased time to SORO., Conclusions: Arch obstruction and a smaller systemic outflow tract area index at end-systolic phase at initial presentation are predictors of subsequent need for SORO in patients with double-inlet left ventricle or tricuspid atresia with transposition of the great arteries., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. Although rarely performed now, patients with this physiology often face severe complications requiring reintervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present 2 successful cases to illustrate this idea., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Rationale: Atrioventricular reentrant tachycardia (AVRT) is the most common supraventricular tachycardia occurring in children. However, in complex congenital heart disease patients with a different heart anatomy and conduction system morphology, accessory pathway modification may be particularly challenging because of distortion of typical anatomic landmarks., Patient Concerns: A 10-year-old boy with tricuspid atresia and history of bidirectional Glenn operation had recurrent chest distress and palpitation for 3 months. He had multiple hospitalizations for narrow-QRS tachycardia with poor hemodynamic tolerance, despite the use of adenosine and amiodarone., Diagnoses: AVRT. Tricuspid atresia with secundum atrial septal defect, large ventricular septal defect, and right ventricular outflow tract stenosis., Interventions: Cardiac catheterization, electrophysiological examination, and ablation., Outcomes: The child has not had a recurrent AVRT during 6 months of follow-up and is waiting for Fontan operation., Lessons: Since there is an increased risk of accessory pathways in patients with tricuspid atresia, all these patients should be checked before the Fontan operation to exclude congenital accessory pathways.

Persistent right valve of the systemic venous sinus is a rare anomaly with anatomical variations. This anomaly may present as an obstructive structure that can inhibit the antegrade flow through the tricuspid valve. We report on a 4-day-old neonate who presented with pronounced peripheral and central cyanosis. Echocardiographic examination showed a giant Eustachian valve moving towards the tricuspid valve during late systole and leading to complete obstruction of the inflow. Blood flow was redirected through the persistent foramen ovale, producing a right-to-left shunt.