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Patients with a single ventricle circulation continue to be a challenge for a heart transplant. The patients in this cohort, often in poor clinical condition with frequent hepatic and renal impairment having undergone previous multiple surgical procedures, are often allosensitive and consequently have an increased risk of post-transplant complications. Although the most recently published series results are improved, this group of patients, with preserved ventricular function, continues to have a higher mortality rate. Moreover, the operation can be complicated by anatomical differences among the pulmonary arteries. This case report presents a child with tricuspid atresia and pulmonary vascular resistance contraindicating Fontan surgery, unfavourable anatomy of the central pulmonary arteries and in poor clinical condition. An EXCOR ventricular assist device (Berlin Heart) was used for sub- pulmonary mechanical circulatory support and pulmonary bifurcation reconstruction, as a bridge to a transplant. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Up until the mid-1900s, tricuspid atresia - a birth defect of the tricuspid valve, was once categorized as a "death sentence." The challenge of achieving positive health outcomes for affected patients was compounded by a hesitancy to operate on children. The main concern was safely administering anesthesia to young patients who were going through a strenuous operation that was often poorly tolerated. Despite these assumed limitations, Francis Fontan, a pediatric cardiothoracic surgeon at the Hospital of Tondu in Bordeaux, was able to redirect blood flow from the superior and inferior vena cava to the pulmonary arteries in 1971, which elucidated the process of advancing clinical practice in medicine. With the support of mentors and a firm belief in this new technique, Fontan pioneered his eponymous procedure and ultimately paved the way for modern cardiovascular surgical techniques that helped to prolong the life of those with single functioning ventricles. The aim of this study is to examine the genesis and the evolution of the Fontan procedure to elucidate the process of advancing clinical practice in medicine by utilizing personal interviews, Fontan's works, associated primary and secondary sources in the context of 20th century cardiothoracic surgery and innovations. [ABSTRACT FROM AUTHOR]

Background: Endocardial cushion tissue is primordia of the valves and septa of the adult heart, and its malformation causes various congenital heart diseases (CHDs). Tricuspid atresia (TA) is defined as congenital absence or agenesis of the tricuspid valve caused by endocardial cushion defects. However, little is known about what type of endocardial cushion defect causes TA. Results: Using three‐dimensional volume rendering image analysis, we demonstrated morphological changes of endocardial cushion tissue in developing Hey2/Hrt2 KO mouse embryos that showed malformation of the tricuspid valve, which resembled human TA at neonatal period. In control embryos, atrioventricular (AV) endocardial cushion tissues showed rightward shift to form a tricuspid valve. However, the rightward shift of endocardial cushion tissue was disrupted in Hey2/Hrt2 KO embryos, leading to the misalignment of AV cushions. We also found that muscular tissue filled up the space between the right atrium and ventricle, resulting in the absence of the tricuspid valve. Moreover, analysis using tissue‐specific conditional KO mice showed that HEY2/HRT2‐expressing myocardium may physically regulate the AV shift. Conclusion: Disruption of rightward cushion movement is an initial cue of TA phenotype, and myocardial HEY2/HRT2 is necessary for the regulation of proper alignment of AV endocardial cushion tissue. Key Findings: HEY2/HRT2 expressed in the compact myocardial layer.Global Hey2/Hrt2 knockout mice showed abnormal AV cushion alignment.Loss of Hey2/Hrt2 function disrupted rightward shift of AV cushion.Developing compact myocardial layer may physically regulate the rightward shift of AV cushion. [ABSTRACT FROM AUTHOR]

BackgroundCOVID-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 presentationA 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.ConclusionsThis 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.

Introduction: Congenital heart diseases (CHDs) are one of the most common birth defects worldwide with a prevalence of 1%. CHDs can be classified into cyanotic and acyanotic diseases based on the presence or absence of the characteristic bluish discoloration of skin and mucus membranes. A subset of cyanotic diseases is single ventricle malformations. This group of disorders comprises 1% of all CHDs. A remarkable yet rare and underreported entity of single ventricle malformations is combined tricuspid atresia (TA) and atrioventricular (AV) septal defect which is characterized by the anatomical features of both entities. Combined TA‐AV septal malformation was first anatomically described in 1953 and further explored through echocardiography and cardiac catheterization in 1987 and then 1991. Since then, no studies have been documented in the literature prompting us to share our findings. Methods: Herein we are describing a rare and underreported cardiac lesion based of a retrospective revision of medical charts at the American University of Beirut Medical Center (AUBMC) Children's Heart Center, a tertiary medical center in the Middle East Results: Out of 200 cases with confirmed single ventricle physiology, we identified a few patients with characteristics of combined TA‐AV septal defect. Our patients exhibited characteristic echocardiographic findings of primum ASD, VSD, atretic RAVV, and clefted LAVV. Conclusion: In short, TA‐AV septal defect is a rare, underreported congenital malformation. Tracking our patients' clinical profiles will help improve our understanding of the prognosis of this entity. Our findings may also improve treatment modalities since replacing the left‐sided valve is often overlooked if the defect is inaccurately diagnosed. In addition, such findings can help shed light on the embryological development of the rarely encountered variation of AV septal malformation. [ABSTRACT FROM AUTHOR]

Abstract: Dextro transposition of great arteries (dTGA) and tricuspid atresia (TA) are rare causes of cyanotic congenital heart disease (cCHD). Brain abscess is a dreaded complication in these patients due to high rates of morbidity and mortality. We present the anesthetic management of two pediatric patients with dTGA and TA, who developed brain abscess, for which burr-hole and drainage were done under general anesthesia. Anesthetic management included a thorough preoperative assessment and optimization, careful selection of anesthetic agents, and close intraoperative and postoperative monitoring. Both of our patients recovered and were discharged from the hospital. Although several cases of brain abscess in cCHD have been reported, both dTGA and TA are rare conditions and this is the first case report of brain abscess in untreated TA, to the best of our knowledge. [ABSTRACT FROM AUTHOR]

Key Clinical Message: The use of endocardial pacing in patients with univentricular hearts and intracardiac shunts is limited, primarily due to the increased risk of thromboembolism. However, when accompanied by proper long‐term anticoagulation therapy, it may be safer than epicardial interventions in selected patients at high risk for surgery. We report transvenous endocardial pacing through the atrial septal defect in a patient with tricuspid atresia, transposition of the great arteries, severe pulmonary hypertension, and complete heart block. This study is among the first reported cases using this pacing method in a patient with a univentricular heart and intracardiac shunt. [ABSTRACT FROM AUTHOR]

A neonate with type 1a tricuspid valve atresia with quadrivalvular involvement is described. There were other associated abnormalities as well. Such a combination is extremely unusual and is being reported here for its uniqueness.

Key Clinical Message The use of endocardial pacing in patients with univentricular hearts and intracardiac shunts is limited, primarily due to the increased risk of thromboembolism. However, when accompanied by proper long‐term anticoagulation therapy, it may be safer than epicardial interventions in selected patients at high risk for surgery. Abstract We report transvenous endocardial pacing through the atrial septal defect in a patient with tricuspid atresia, transposition of the great arteries, severe pulmonary hypertension, and complete heart block. This study is among the first reported cases using this pacing method in a patient with a univentricular heart and intracardiac shunt.

Background d-Transposition of the great arteries (d-TGA) is a congenital cardiac defect that is typically fatal. Those patients who survive without surgical repair and who are rare in number, need adequate intracardiac shunting and will suffer from chronic cyanosis. Here, we present a rare case of an adult with unrepaired cyanotic congenital heart disease (CHD) who developed infective endocarditis (IE) and also our approach to the medical decision-making process in this uncommonly encountered dilemma. Case summary A 52-year-old female with unrepaired d-TGA with tricuspid atresia, hypoplastic right ventricle, unrestricted atrial septal defect, ventricular septal defect, and sub-valvular as well as valvular pulmonic stenosis with a hypoplastic, bicuspid pulmonary valve presented with abdominal pain and hypoxia and was found to have an acute renal infarct. Transthoracic echocardiogram (TTE) revealed a large mobile mass on the mitral valve. Blood cultures grew Streptococcus mitis-oralis and she was diagnosed with streptococcal native mitral valve IE complicated by a renal embolus. Her large left-sided vegetation and embolic phenomenon favoured surgery. However, a right heart catheterization showed normal intracardiac pressures, likely a result of multi-level obstruction relating to sub-valvular and valvular pulmonary stenosis protecting the pulmonary vasculature from over-circulation and pulmonary hypertension. Cardiac surgery posed a significant risk of destabilizing her delicately balanced haemodynamics. Hence, she was treated with ceftriaxone for 4 weeks. A repeat TTE 8 weeks later showed a resolution of the vegetation. Discussion A decision for surgery vs. medical treatment for IE in adult patients with compensated CHD should be made following a multi-disciplinary assessment of each patient's unique cardiac haemodynamics and after shared decision-making with the patient. [ABSTRACT FROM AUTHOR]

Tricuspid atresia is typically classified according to the relationship with the great arteries, the location of the ventricular septal defect, and the nature of the pulmonary flow. A condition where both great arteries arise from the left ventricle is an exceedingly uncommon occurrence. In this case study, we present a unique case of a young infant diagnosed with tricuspid atresia accompanied by a double-outlet left ventricle and pulmonary stenosis. We showcase this rare scenario through detailed multimodality imaging.

The influence of therapeutic hypothermia, known to improve neurodevelopmental outcomes in neonatal encephalopathy, remains unknown in newborns with severe congenital heart diseases. We report a neonate with tricuspid atresia type 1c suffering from moderate neonatal encephalopathy. A burst suppression pattern on amplitude-integrated electroencephalography recovered after inducing moderate therapeutic hypothermia, but exacerbated pulmonary overcirculation still persisted even after the rewarming. Since the medical treatment for pulmonary overcirculation had reached the limit, semi-urgent pulmonary trunk banding was performed on the 4th day of life. Postoperative brain magnetic resonance imaging showed no apparent brain injuries; the patient was discharged uneventfully. We share our perioperative management experience of a patient with tricuspid atresia type Ic who required therapeutic hypothermia for neonatal encephalopathy.

Patients with single left ventricle (SLV) and tricuspid atresia (TA) have impaired systolic and diastolic function. However, there are few comparative studies among patients with SLV, TA and children without heart disease. The current study includes 15 children in each group. The parameters measured by two-dimensional echocardiography, three-dimensional speckle tracking echocardiography (3DSTE), and vortexes calculated by computational fluid dynamics were compared among these three groups. Twist is best correlated with ejection fraction measured by 3DSTE. Twist, torsion, apical rotation, average radial strain, peak velocity of systolic wave in left lateral wall by tissue Doppler imaging (sL), and myocardial performance index are better in the TA group than those in the SLV group. sL by tissue Doppler imaging in the TA group are even higher than those in the Control group. In patients with SLV, blood flow spreads out in a fan-shaped manner and forms two small vortices. In the TA group, the main vortex is similar to the one in a normal LV chamber, but smaller. The vortex rings during diastolic phase are incomplete in the SLV and TA groups. In summary, patients with SLV or TA have impaired systolic and diastolic function. Patients with SLV had poorer cardiac function than those with TA due to less compensation and more disordered streamline. Twist may be good indicator for LV function.

Tricuspid valve atresia is a congenital heart defect and if left untreated, the lesion has a high mortality rate. This study aimed to report a case of tricuspid valve atresia in a low-birth-weight three-day-old infant, who was treated by intra-ductal stenting and established pulmonary blood flow instead of routine shunting.The patient was a three-day-old male infant, weighing 2800 grams with a gestational age of 38 weeks. He was admitted with symptoms of tachypnea, cyanosis, 45% saturation, and severe metabolic and respiratory acidosis. On echocardiography, tricuspid valve atresia was observed. The infant was treated with prostaglandin E1 immediately after admission to the NICU and necessary interventions were taken to keep the patent ductus arteriosus (PDA) open. Angiography was performed through the aortic artery. First, a balloon was inserted, and then a stent was successfully placed inside the PDA. Preoperative saturation increased from 45% to 93% after stenting, and the infant was extubated after 48 hours in a good general condition.As the results of the current case report showed, stent placement can effectively keep the PDA open and establish pulmonary blood flow in high-risk infants. It is suggested that ductal stenting should be considered as the first treatment selection in newborns with tricuspid valve atresia or as a good alternative method for Blalock-Taussig shunt.

Background: Tricuspid atresia (TA) is the second most common form of functionally univentricular heart. For patients with TA and normally related great arteries (Type I), left ventricular outflow tract obstruction (LVOTO) is rare., Methods: From the Congenital Heart Surgeons' Society multi-institutional cohort of 445 patients with Type I TA enrolled from 1999 to 2024 from 42 sites, 14 infants (3%) had interventions for associated LVOTO, either at presentation or after their first TA-related intervention., Results: Of seven infants initially undergoing Norwood/Damus-Kaye-Stansel (DKS), six survived to Stage II, of whom five survived with one developing pulmonary hypertension and four achieving Fontan. An additional seven infants who were first managed with pulmonary artery band placement subsequently had bidirectional superior cavopulmonary anastomosis (BCPA) and a DKS procedure; there were six survivors, all achieving Fontan. All ten survivors who underwent the Fontan procedure had normal left ventricular and mitral valve function at the latest follow-up. The overall Kaplan-Meier survival estimate at 20 years for these 14 patients was 79% (70% CI, 66%-88%), and the median follow-up was 8.3 years (0.24-21.5)., Conclusions: While infants with TA and transposition of the great arteries are more likely to have LVOTO, this can also occur in the setting of normally related great arteries. Infants with Type I TA and LVOTO can be managed in the neonatal period with the Norwood procedure ensuring complete arch relief with acceptable outcomes. If LVOTO becomes evident after initial pulmonary artery band placement, a subsequent DKS procedure facilitates satisfactory success to Fontan., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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. [ABSTRACT FROM AUTHOR]

Background: The horseshoe lung is a congenital malformation in which the bases of the right and the left lung are fused. Case report: We describe a monochorionic twin gestation with malformation discordance. The abnormal twin had a horseshoe lung with hypoplasia of the right lung, tricuspid atresia, cleft lip, and a pelvic right kidney. Conclusion: The discordance of anomalies in this monochorionic twin suggests that a postzygotic mutation, epigenetic change, or environmental factors may be responsible for these malformations. [ABSTRACT FROM AUTHOR]

Juxtaposition of the atrial appendages is known to occur in specific congenital heart lesions. Recognition of these variants is critical in balloon atrial septostomy and atrial switch operations. There remains little clinical data on the prevalence and associations of these lesions. This is the largest echocardiographic study of juxtaposition of the atrial appendages. This is a retrospective study using the Mt. Sinai echocardiogram database (EchoLAN), which contains echocardiogram reports performed or reviewed at Mt. Sinai Hospital (New York, NY) between 1992 and 2019. Each report was reviewed for associated intracardiac and extracardiac anomalies and the prevalence of juxtaposed atrial appendage among specific diagnosis, including tricuspid atresia, transposition of the great arteries, and double outlet right ventricle, was calculated. Descriptive and analytical statistics were performed as applicable, including Fisher's exact test, with p value < 0.05 considered statistically significant. Forty-nine patients had juxtaposed atrial appendages (1.2/1000 patients): Thirty-eight had LJRAA (0.9 per 1000 patients) and eleven had RJLAA (0.3 per 1000 patients). LJRAA was seen in 22% of tricuspid atresia, 6% of d-TGA, 1% of l-TGA, and 5% of DORV. RJLAA was seen in 2% of DORV, and none of the other lesions. Of associated lesions, dextrocardia, mesocardia, transposition, tricuspid atresia, double inlet left ventricle, and pulmonary valve stenosis were more likely to be present in LJRAA, while bicuspid aortic valve and left ventricular hypoplasia were more likely to be present in RJLAA. This study reaffirms our understanding of juxtaposition of the atrial appendages, its prevalence, and clinical importance. [ABSTRACT FROM AUTHOR]

Congenital heart disorders (CHDs) are a major cause of juvenile morbidity and mortality in many poor nations, owing to late detection and a lack of talent and facilities for decisive treatments. A case of a newborn baby with atrial septal defect (ASD) with patent ductus arteriosus (PDA), tricuspid atresia (TA), and pulmonary valve stenosis is admitted to the pediatric ward. It is a complex cardiac anomaly that leads to mortality and morbidity. We rarely get to see a baby dealing with four major complex heart conditions except in the condition of tetralogy of Fallot. The child was a known case of congenital heart disease. Symptomatic treatment was given and treated with antibiotics.

Tricuspid valve atresia is a congenital heart defect and if left untreated, the lesion has a high mortality rate. This study aimed to report a case of tricuspid valve atresia in a low-birth-weight three-day-old infant, who was treated by intra-ductal stenting and established pulmonary blood flow instead of routine shunting. The patient was a three-day-old male infant, weighing 2800 grams with a gestational age of 38 weeks. He was admitted with symptoms of tachypnea, cyanosis, 45% saturation, and severe metabolic and respiratory acidosis. On echocardiography, tricuspid valve atresia was observed. The infant was treated with prostaglandin E1 immediately after admission to the NICU and necessary interventions were taken to keep the patent ductus arteriosus (PDA) open. Angiography was performed through the aortic artery. First, a balloon was inserted, and then a stent was successfully placed inside the PDA. Preoperative saturation increased from 45% to 93% after stenting, and the infant was extubated after 48 hours in a good general condition. As the results of the current case report showed, stent placement can effectively keep the PDA open and establish pulmonary blood flow in high-risk infants. It is suggested that ductal stenting should be considered as the first treatment selection in newborns with tricuspid valve atresia or as a good alternative method for Blalock-Taussig shunt. [ABSTRACT FROM AUTHOR]

Background: Cyanotic congenital heart disease (CCHD) children often have malnutrition and iron-deficiency anemia along with poor weight gain. Low arterial oxygen saturation due to CCHD affects hematological indices. Objective: The objective of the study is to find iron status and hematological indices in children having CCHD and to study the impact of iron therapy in the sample population under study. Materials and Methods: This hospital-based, prospective, cross-sectional, observational type of study evaluated 50 CCHD children aged more than 1 year with informed written consent from the parents. Sample size is calculated at 95% confidence level and alpha error 4.005 assuming 52.2% iron deficiency in patient with CCHD with hematocrit value

Congenital heart disorders (CHDs) are a major cause of juvenile morbidity and mortality in many poor nations, owing to late detection and a lack of talent and facilities for decisive treatments. A case of a newborn baby with atrial septal defect (ASD) with patent ductus arteriosus (PDA), tricuspid atresia (TA), and pulmonary valve stenosis is admitted to the pediatric ward. It is a complex cardiac anomaly that leads to mortality and morbidity. We rarely get to see a baby dealing with four major complex heart conditions except in the condition of tetralogy of Fallot. The child was a known case of congenital heart disease. Symptomatic treatment was given and treated with antibiotics. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Patients who have undergone a Fontan's procedure have an abnormal circulation that presents a unique challenge when performing computed tomography pulmonary angiograms. In a standard imaging protocol, contrast is injected into the upper limb veins that feed into the superior vena cava. In Fontan's patients the Computed tomography pulmonary angiograms bypasses the heart and preferentially fills the right lung, with only a small amount of mixture of contrast and noncontrast blood in the pulmonary arteries.In this article, we present the case of a 35-year-old female complaining of chest and abdominal pain with oxygen saturations of 85% on room air. Computed tomography pulmonary angiograms showed suboptimal imaging of the left lung and apparent filling defects in the right lung suggesting a radiological diagnosis of a pulmonary embolism. The abnormal flow and distribution of contrast in the pulmonary arteries can result in a false positive diagnosis of pulmonary embolism. To overcome this, experts advise using a dual-injection of contrast via upper and lower limb central veins to achieve optimal imaging.

Abstract Background The anesthetic management of cesarean sections in Fontan-palliated parturients requires strict hemodynamic control. However, patient management with central venous oxygen saturation (ScvO2) and oxygen consumption (VO2) has never been reported. Case presentation A 30-year-old woman, who had received a total cavopulmonary connection for tricuspid atresia, was planned to undergo cesarean section at 38 weeks’ gestation. During combined spinal-epidural anesthesia, ScvO2 in addition to arterial pressure-based cardiac output (APCO) and central venous pressure (CVP) was monitored, and the change of VO2 was evaluated. After delivery, her APCO was almost unchanged. However, her ScvO2 increased dramatically from 42.1 to 67.3% and her CVP increased from 9 to 11 mm Hg. The calculated mean maternal VO2 changed from 443 to 295 mL/min. Conclusions In a cesarean section for a Fontan-palliated parturient, ScvO2 dramatically increased and maternal VO2 decreased by more than 25% after delivery.

Various combinations of congenital heart defects are included in complex congenital heart disease. Various pathologies of cardiac structures have varied embryological dysmorphology. Lesions described are single ventricle pathologies, hypoplastic left heart syndrome, tricuspid atresia, pulmonary atresia and intact ventricular septum, and crisscross atrioventricular connections. Irrespective of underlying structural abnormalities, most of these lesions will need a unified single ventricle pathway of palliative surgeries for continued survival. A few complex lesions are amenable to corrective surgery. Approach to diagnosis, echocardiography of different lesions, a unified approach toward treatment is discussed.