Factors associated with mortality or transplantation versus Fontan completion after cavopulmonary shunt for patients with tricuspid atresia.

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.

Major aortopulmonary collateral arteries in a case of unrepaired tricuspid and pulmonary atresia with single ventricle physiology.

Tricuspid and pulmonary atresia with single ventricle physiology and major aortopulmonary collateral arteries (MAPCAs) is a complex cyanotic congenital heart disease with heterogeneous pulmonary artery morphology and arborization. The complex anatomy and physiology, coupled with a dearth of existing literature, pose imitable challenges to treatment. Although the exact surgical algorithm is still unclear, the goal is a well-developed, low-resistance pulmonary vascular bed. A precise understanding of the blood supply to each lung is a requisite for successful surgery, and a multimodality and multidisciplinary approach is compulsory. Herein, we describe a case of tricuspid and pulmonary atresia with single ventricle, MAPCAs and aortopulmonary collateral arteries.

Bosentan Therapy in a Patient with Failed Fontan Procedure: A Case Report.

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.

Evaluation of the anatomic and hemodynamic abnormalities in tricuspid atresia before and after surgery using computational fluid dynamics.

Analysis of hemodynamics inside tricuspid atresia (TA) chamber is essential to the understanding of TA for optimal treatment. In this study, we introduced a combined computational fluid dynamics (CFD) to simulate blood flow in the left ventricle (LV) to study the diastolic flow changes in TA.Real-time 3-dimentional echocardiography loops (ECHO) were acquired in normal control group, in TA patients before surgery (pre-op group) and after surgery (post-op group). ECHO loops were reconstructed and simulated by CFD, the geometric, volumetric changes, and vortices in the LV were studies and compare among 3 groups.Compared with the control group, pre-op TA patients demonstrated significant LV remodeling, manifesting with smaller LV length, larger diameter, width and spherical index, as well as lager volumes; post-op TA group showed revisions in values of both geometric and volumetric measurements. CDF also demonstrated the abnormality of vortices in the pre-op TA patients and the alteration of existence and measurements of vortex in postoperation group.Echo-based CFD modeling can show the abnormality of TA in both LV geometric, volumetric measurements and intracardiac vortices; and CFD is capable to demonstrate the alterations of LV after Fontan and Glenn surgical procedure.

Successful staged Fontan completion for a tricuspid atresia patient with left ventricular non-compaction.

We report a case of Fontan completion for a tricuspid atresia (TA) patient with left ventricular non-compaction (LVNC). The patient was diagnosed with TA (Ia) with LVNC by fetal echocardiography. Because the unfavourable prognosis of LVNC was anticipated, Imidapril as well as Carvedilol were administered to improve cardiac function, from the early stages of infancy. Staged Fontan completion with fenestration was successfully achieved with improvement of LV function.

Improved Exercise Performance in Patients With Tricuspid Atresia After the Fontan-Björk Modification With Pulsatile Systolic Pulmonary Flow.

BACKGROUND: After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection. METHODS: According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30). RESULTS: Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N (p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation (p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg(-1) · min(-1), p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L(-1) · m(-2), p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01). CONCLUSIONS: Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow.

Surgical management of competing pulmonary blood flow affects survival before Fontan/Kreutzer completion in patients with tricuspid atresia type I.

OBJECTIVES: To determine the association between surgical management of pulmonary blood flow (PBF) at initial and staged procedures with survival to Fontan/Kreutzer operation (Fontan) in patients with tricuspid atresia. METHODS: Infants aged <3 months with tricuspid atresia type I (n = 303) were enrolled from 34 institutions (1999-2013). Among those who underwent surgical intervention (n = 302), initial procedures were: systemic to pulmonary artery shunt (SPS; n = 189; 62%); pulmonary artery banding (PAB; n = 50; 17%); and superior cavopulmonary connection (SCPC; n = 63; 21%). Multiphase parametric-hazard models were used to analyze competing outcomes. RESULTS: Risk-adjusted 6-year survival was lower after SPS (85%; P = .04) versus PAB (93%) or SCPC (93%). Survival after SPS when the main pulmonary artery (MPA) was closed (n = 21) or banded (n = 4) was 60%, versus 93% without MPA intervention (P = .02). After SPS, survival before SCPC was lower with an open ductus arteriosus (n = 7; 76% vs 97%; P = .02). Similarly, after SPS, risk-adjusted survival was similar to that for patients who had an initial PAB or SCPC when MPA intervention was avoided and the ductus arteriosus either closed spontaneously before SPS, or was closed during SPS. For all patients reaching SCPC (n = 277), survival to Fontan was not significantly influenced by whether PBF persisted through the MPA. CONCLUSIONS: Tricuspid atresia patients with SPS represent a high-risk subgroup. Avoiding an open ductus arteriosus and concomitant MPA intervention during SPS may help mitigate the risk associated with SPS. The presence of antegrade PBF through the MPA, at initial and staged operations, did not significantly influence survival to Fontan operation.

Tricuspid valve implantation after Bjork procedure to establish biventricular physiology.

A 29-year-old male, who had undergone a Bjork procedure for tricuspid atresia with a ventricular septal defect, had redo surgery for relief of stenosis and systolic regurgitation of the right atrioventricular pathway. After revision using a valved bioprosthesis, effective biventricular physiology was established and the patient's functional status improved.

Tricuspid atresia with aortopulmonary window: challenges in achieving a balanced circulation.

Tricuspid atresia and aortopulmonary window are rare congenital cardiac anomalies. The occurrence of both these anomalies in the same patient is extremely rare, with only 1 case reported in the literature. We report the surgical management of one such patient and discuss the management issues with respect to Stage 1 single ventricle palliation.

Percutaneous closure of tricuspid paravalvular leak.

While transcatheter closure of mitral and aortic paravalvular leaks has been well-described, there are no prior reports of such procedures for prosthetic tricuspid valves. We describe our experience with percutaneous closure of a tricuspid paravalvular leak in a 34-year-old patient with a history of tricuspid atresia. He had previously undergone repair with placement of a right atrium-to-right ventricle conduit and most recently, placement of a bio-prosthetic tricuspid valve. He subsequently developed significant tricuspid paravalvular leak with symptoms of severe right heart failure. His clinical status was improved following successful transcatheter closure of the defect.

Long-term results of modified Fontan operation for single-ventricle patients associated with atrioventricular valve regurgitation.

BACKGROUND: The long-term outcome of modified Fontan operation concomitant with a valve operation for atrioventricular valve (AVV) regurgitation is not well described. METHODS: Between 1977 and 2003, 500 children who underwent modified Fontan operation were subdivided into 192 with AVV plasty (group P) and 308 without AVV plasty (group N). Factors associated with patient outcome were investigated retrospectively. RESULTS: Surgical techniques to correct valve incompetence included circular annuloplasty, partial annuloplasty with Kaye-Reed methods, edge-to-edge repair methods, and valvoplasty, which were combined according to the etiology of the valve lesion. The estimated actuarial survival rates at 10 and 20 years were, respectively, 82.0% and 76.6% in group P (p < 0.05) and 90.8% and 86.8% in group N (p = 0.001). The estimated actuarial survival rates at 10 years among patients with AVV plasty did not show a statistically significant difference (circular annuloplasty, 79.0%; partial annuloplasty, 81.6%; edge-to-edge, 83.3%; valvoplasty, 82.6%; p = 0.90). A Cox regression model revealed that a higher preoperative ventricular ejection fraction was associated with a significant reduction in long-term mortality in group P (hazard ratio, 0.921; 95% confidence interval, 0.873 to 0.972; p < 0.05). Each repair method showed acceptable durability at medium-term follow-up, without progression of lesions. CONCLUSIONS: Given worse results with poor ventricular function, early intervention against valve pathology before development of ventricular failure may improve long-term outcomes. Multiple methods are appropriate for a variety of valve lesions; however, circular annuloplasty remains a reliable repair option due to its technical simplicity.

Chronologic changes in P-wave characteristics after the Fontan procedure: the effect of surgical modification.

OBJECTIVES: The Fontan-type procedure has undergone 2 major modifications, including intra-atrial baffling and extracardiac conduit. To clarify the effect of these modifications on arrhythmia propensity, we analyzed chronologic changes in P-wave characteristics after atriopulmonary connection, intra-atrial baffling, or extracardiac conduit. METHODS: A retrospective analysis was conducted on electrocardiographic data from 40 patients with tricuspid atresia who underwent the Fontan-type procedure and follow-up for greater than 5 years: 18 had atriopulmonary connection, 13 had intra-atrial baffling, and 9 had extracardiac conduit. The mean follow-up period in years was 19.8 for atriopulmonary connection, 13.3 for intra-atrial baffling, and 8.0 for extracardiac conduit. We analyzed chronologic changes in P-wave duration, dispersion, and amplitude and prevalence of sinus node dysfunction. RESULTS: Atrial tachyarrhythmia was documented in 9 patients with atriopulmonary connection but not in any patients with extracardiac conduit or intra-atrial baffling. Both P-wave maximum duration and dispersion decreased slightly over time with extracardiac conduit but increased progressively in the intra-atrial baffling and atriopulmonary connection groups. Intra-atrial baffling resulted in significantly shorter P-wave duration than atriopulmonary connection, whereas extracardiac conduit had significantly shorter P-wave duration and smaller dispersion than atriopulmonary connection and intra-atrial baffling. P-wave amplitude decreased markedly immediately after surgical intervention with intra-atrial baffling and extracardiac conduit but remained unchanged in patients undergoing atriopulmonary connection. Sinus node dysfunction was found commonly in all 3 groups. CONCLUSION: After intra-atrial baffling, patients increasingly had prolonged P-wave duration and larger dispersion associated with sinus node dysfunction, suggesting a propensity to arrhythmia, although less progressive than seen in those undergoing atriopulmonary connection. In contrast, despite an equal prevalence of sinus node dysfunction after extracardiac conduit, the lack of important changes in P-wave characteristics over time suggests that the extracardiac conduit procedure is the preferred option for optimal rhythm prognosis.

The Fontan/Kreutzer procedure at 40: an operation for the correction of tricuspid atresia.

The first atriopulmonary anastomosis (APA) with neither a valve in the inferior vena cava (IVC) nor an Glenn shunt was performed in 1971. A fenestration was intentionally left in the atrial septum. In a second patient, the APA incorporated the patient's own pulmonary valve, which had been removed from the outflow tract of the right ventricle. Since the early days, our rationale was that the right atrium would only function as a pathway, and the end diastolic pressure and the systole of the main ventricle would be the principal "pump" of this system. The late hemodynamic problems of the APA have decreased with newer and better surgical techniques, such as the lateral tunnel (LT) or the extracardiac conduit (EC). Although these procedures have improved the prognosis and quality of life of patients with a univentricular heart (UH), in the long run, deterioration frequently occurs because of chronic low cardiac output and high central venous pressure. Progressive increase in pulmonary vascular resistances and ventricular dysfunction are frequently the underlying reasons for this deterioration. However, such deterioration is not inevitable in every case, as shown in the longest survivor of the world after 34 years of follow-up. The Fontan Kreutzer (FK) palliation represents the best surgical option despite its uncertain late outcome. Certainly, it is one of the triumphs of cardiac surgery in congenital heart disease.

Diagnosis and management of cyanotic congenital heart disease: part I.

Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.

Tricuspid atresia with normal axis on ECG palliated with a central shunt; a case report.

BACKGROUND: Tricuspid Atresia is the 3rd commonest cyanotic congenital Heart disease. It is characterized by lack of communication between the right atrium and right ventricle. The treatment often requires a palliative systemic to pulmonary shunt before definite surgery. The use of a central shunt via a median sternotomy is suggested here as an alternative to other traditional shunts via a thoracotomy. METHOD: The management of a 3-month-old boy who presented with dyspnoea, fever, cough and cyanosis is presented here as obtained from Clinical records. RESULT: Following resuscitation, a central shunt (Ascending Aorta to main Pulmonary Artery) was constructed and the patient did well despite a turbulent post-operative period. CONCLUSION: The management of tricuspid atresia likes other cyanotic heart disease is daunting but palliative treatment is possible in our environment and definitive treatment where possible affords a fairly satisfactory prognosis.

Shunt operations improved thrombocytopenia in a patient with congenital cyanotic heart disease.

Cardiac and vascular intervention in thrombocytopenic congenitally cyanotic patients is more dangerous. Thrombocytopenia in these patients is related to immune thrombocytopenia, polycythemia, hyperviscosity, pseudothrombocytopenia, and drugs. Herein we report on a thrombocytopenic 8-year-old girl with tricuspid valve atresia and pulmonary valve stenosis admitted for catheterization. Thrombocytopenia (21,000/mm3) and shunt occlusion was noticed. Thrombocytopenia did not recover after intravenous immunoglobulin (IVIG) and phlebotomy therapies. During preparation for surgery, she suffered cardiopulmonary arrest. A Gore-tex graft was placed in the right pulmonary artery and truncus brachiocephalicus. After surgery, her platelet count spontaneously increased to within the normal range (178,000/mm3 to 250,000/mm3). After resuscitation, she had right-sided hemiplegia sequelae, though there were no hemorrhagic findings on cranial magnetic resonance imaging (MRI) or computed tomography (CT) scans. Two months after surgery, the Blalock-Taussig (BT) shunt blood flow decreased, thrombocyte count dropped, and peripheral cyanosis reappeared. A Fontan operation was performed without hemorrhagic events, and after surgery the thrombocyte count reached 330,000/mm3. We suggest that if a patient with cyanotic heart disease has thrombocytopenia and there is no apparent cause, hypoxia-related thrombocytopenia must be considered. After reoxygenation by shunt or corrective surgeries, thrombocyte count and functions will recover.

Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis.

A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.

Nephrotic syndrome complicating cyanotic congenital heart disease: a report of two cases.

BACKGROUND: Renal complications are said to be common in cyanotic congenital heart disease (CCHD), but have not been documented frequently in Nigerian children. Nephrotic syndrome (NS) is an uncommon complication of CCHD. OBJECTIVES: To report two cases of NS complicating CCHD who presented within months of each other, in order to draw attention to the problem. METHODS: The details of the clinical presentation, course and outcome of two children with CCHD, presenting with features of NS within months of each other, were reviewed. The patients were fully assessed clinically and were further investigated with chest X-ray, ECG ad echocardiography. RESULTS: A 12-year old girl with Fallot's tetralogy underwent a Blalock-Taussig shunt at the age of 2 years, but subsequently defaulted from follow-up. She reappeared 10 years later with features of NS. Echocardiography revealed impaired myocardial function. Despite initial clinical improvement following three plasma exchanges and Enalapril therapy, she suddenly died on the 15th day of admission. The second patient was a 7-year old boy with tricuspid atresia, diagnosed at the age of 10 months, and similarly defaulted, reappearing six years later with features of NS. Oedema regressed with similar treatment, but his renal function deteriorated. He was stable enough to be discharged after six weeks on admission. CONCLUSION: Proteinuria is likely to be more common in Nigerian children with CCHD than has been previously appreciated. Early intervention in patients with CCHD is desirable in order to prevent development of complications which worsen the prognosis. Patients with CCHD should be screened regularly for proteinuria in order to detect and address renal complications early.

Transvenous right atrial and left ventricular pacing after the Fontan operation: long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization.

We report a case of long-term, successful, endocardial atrioventricular pacing in a 32-year-old man who had severe heart failure and ascites after having undergone a Fontan procedure for tricuspid atresia 9 years earlier. The patient was referred to our hospital for Fontan revision. However, electroanatomic mapping of the right atrium revealed viable tissue at the interatrial septum above the os of the coronary sinus, and it appeared that the left ventricle could be paced from a coronary sinus branch. Therefore, instead of Fontan revision, an endocardial atrioventricular pacemaker was implanted transvenously. On 5-year follow-up, the patient remained in New York Heart Association functional class I and had not been readmitted to the hospital for congestive heart failure or arrhythmias. His atrial and ventricular leads continued to show excellent pacing and sensing results.