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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.)

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.

Objectives: In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy., Methods: Two hundred and eleven patients were identified, 59 TA-TGA,152 DILV. Median follow-up was 17 years (range 4 days to 49.8 years). The Kaplan-Meier method was used for all of the time to event analyses and the log-rank test was used to compare the time-to-events. Cox proportional hazard models were used to test the association between potential predictors and time-to-event end-points., Results: TA-TGA had reduced survival compared to DILV (cumulative risk of death 28.8% vs 11%, hazard ratio (HR) 3.1 (95% confidence interval (CI) 1.6-6.1), P  = 0.001). In both groups, SOTO at birth carried a worse prognosis HR 3.54 (1.36-9.2, P  = 0.01). SOTO was not more common in either morphology at birth ( P  = 0.20). Periprocedural mortality accounted for 40% of deaths. Fontan was achieved in 82%, DILV were more likely to achieve Fontan than TA-TGA (91% vs 60%, P <0.001). After Fontan there were 9 deaths (4%) with no difference according to morphology., Conclusions: Patients with TA-TGA have poorer outcomes than those with DILV, affecting survival and likelihood of achieving Fontan. SOTO at birth carries a high risk of mortality suggesting that, when present, initial surgical management should address this., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken to optimize the patient's haemodynamic status prior to a Fontan completion., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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.

A 31-year old woman with tricuspid atresia, after Bjork Fontan modification and reoperation due to homograft narrowing, was evaluated because of progressive exercise intolerance; she was planning a pregnancy. The right atrium (RA) to right ventricle (RV) homograft narrowing and calcification with significant reversal flow were found. Successful reoperation was performed-calcified conduit was excised and extracardiac pulmonary homograft tipped with vascular prosthesis was implanted between the RA and the RV.

We report a case of an iatrogenic atrial septal defect following left atrial ablation for persistent atrial fibrillation in a patient with a Fontan circulation. Transseptal puncture was performed with two sheaths across a single puncture and left atrial ablation undertaken. Post procedure the patient became cyanosed with right to left shunting. Transcatheter closure immediately improved symptoms and oxygen saturation.

Objective: To assess the surgical results of the Norwood procedure and subsequent clinical outcome in the setting of transposition of the great arteries (TGA) with a dominant morphologic left ventricle., Methods: Among 486 patients who underwent the Norwood procedure from 1988 to 2007 at our institution, there were 37 patients with TGA and left ventricular dependant circulation with the following associated lesions: double inlet left ventricle (DILV) (n=24), tricuspid atresia (n=9), ventricular septal defect (VSD) with hypoplastic right ventricle (RV) (n=4). Outcomes for all three-staged procedure were compared with the overall Norwood group., Results: Early mortality was 21.6% (8/37) compared to 26.7% (120/449) in the overall Norwood group (p=ns). There was only one subsequent death giving a 5- and 10-year actuarial survival of 72.8+/-7.4% compared to 55.3+/-2.6% and 52+/-2.9% at 5 and 10 years for the overall series (p=0.06). Median follow-up was 4.7 (0.7-10.2) years. Eighteen patients underwent stage III completion at 3.9+/-1.5 years from the second stage with no mortality. Preoperative mean pulmonary artery (PA) pressure and transpulmonary gradient were respectively 11.6+/-3.4 and 5.2+/-3.3 mmHg. All patients had good left ventricle (LV) function at time of stage III. All patients except one are currently in NYHA I. One patient (with DILV) had congenital heart block and required a pacemaker. There was no postoperative heart block. The systemic outflow was unobstructed in all patients and no patient required any additional intracardiac procedure., Conclusions: The Norwood procedure provides good palliation in this subgroup of patients and avoids the need for subsequent intracardiac operations, maintaining unobstructed systemic outflow tract and avoiding the risk of postoperative heart block.

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.

A bilateral bidirectional cavopulmonary shunt was performed in a cyanotic 14-month-old girl who had tricuspid and pulmonary valve atresia, with right pulmonary artery (RPA) hypoplasia (3 mm), bilateral superior vena cavae and a ductus arteriosus-dependent pulmonary blood flow. Because of 62% postoperative arterial oxygen saturation and a right superior vena cava (RSVC) pressure of 30 mmHg, a 5 mm Gore-Tex tube was interposed to connect the two superior venae cavae. The creation of a 'new-innominate' vein allowed decompression of the right superior vena cava and an increase in arterial oxygen saturation to 86%.

Objective: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status., Methods and Results: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4-13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72 h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05)., Conclusions: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation.

Objective: The extracardiac Fontan procedure, as compared with classic atriopulmonary connections, may have the potential for optimizing ventricular and pulmonary vascular function by maximizing the laminar flow principle, by the avoidance of intra-atrial suture lines and cardiac manipulation, and by minimizing cardiopulmonary bypass time. In this study the clinical results of this procedure are assessed., Methods: From January 1990 until January 1997, 45 patients (33 males and 12 females) with a median age of 4.0 years (range 2.7-38 years) underwent an extracardiac Fontan procedure for univentricular physiology. The underlying diagnoses included tricuspid atresia (n=19), double-inlet left ventricle (n=11), and complex anomalies (n=15). Forty patients (89%) were in sinus rhythm. The median ventricular ejection fraction was 60%. In 37 patients (82%) the procedure was staged., Results: Median cardiopulmonary bypass time was 72 min, with a decrease to a median time of 24 min in the last ten patients. Aortic cross-clamping was avoided in 33 patients (73%). The intraoperative Fontan pressure and transpulmonary gradient were low: 13.6+/-3.2 and 8.5+/-3.9 mmHg, respectively. Transient supraventricular tachyarrhythmias were observed in six patients (13%). There was no early or late mortality. At a median follow-up of 64 months (range 26-105 months), 39 patients (87%) were in NYHA class I, four (9%) were in NYHA class II, and two (4%) were in class III. Forty patients (89%) remained in sinus rhythm. The median ventricular ejection fraction was 59%. The median arterial oxygen saturation raised from 82% preoperatively to 97%. Functional class (P=0.02), maintenance of sinus rhythm (P=0.04), and preservation of ventricular function (P=0.05) was superior in patients who were appropriately staged. None of the patients had atrial thrombus, chronic pleural effusions, or protein losing enteropathy., Conclusions: In the majority of patients, the extracardiac Fontan procedure, when performed as a staged procedure, provides excellent early and midterm results in terms of quality of life, maintenance of sinus rhythm, and preservation of ventricular function.

Objective: Obstruction of the atriopulmonary anastomosis or the lateral atrial tunnel cavopulmonary anastomosis in the Fontan circulation for univentricular physiology may result in dilation of the right atrium or the right atrial free wall that is incorporated in the lateral atrial tunnel, respectively. Secondary detrimental sequelae may consist of supraventricular dysrhythmias, thromboembolism, right pulmonary vein compression, pleural effusions, and protein-losing enteropathy. Conversion of these Fontan connections to an extracardiac conduit cavopulmonary anastomosis may improve central systemic venous flow patterns and provide clinical improvement in these patients., Methods: Eighteen patients (7-40 years old) with atriopulmonary anastomosis (n = 15) or obstructed lateral atrial tunnel cavopulmonary anastomosis (n = 3) presented at 5.7 +/- 3.9 years with moderate to severe right atrial dilation (n = 15), Fontan pathway obstruction (n = 12), atrial dysrhythmia (n = 13), pleural effusion (n = 8), right atrial thrombus (n = 3), right pulmonary vein compression (n = 3), and protein-losing enteropathy (n = 3). All patients underwent conversion to an extracardiac conduit cavopulmonary anastomosis., Results: Two of the three patients with protein-losing enteropathy died (2/18; 11%) on the 30th and 52nd postoperative days. At a mean follow-up of 19 months, the remaining 16 patients had marked (n = 11) or moderate (n = 5) clinical improvement. The SaO2 improved from 90.7 +/- 5.3% to 96.0 +/- 4.1%. None of the patients had obstruction in the systemic venous pathway. In the 13 surviving patients with previous atriopulmonary anastomosis there was a drastic reduction in right atrial size. Four of 13 patients with atrial dysrhythmias converted to sinus rhythm. The right pulmonary vein compression as present in three patients resolved after conversion. Pleural effusions disappeared in four patients., Conclusions: Conversion to an extracardiac cavopulmonary connection may lead to clinical improvement in patients with atriopulmonary or lateral atrial tunnel Fontan connection associated with specific target conditions such as obstruction, pulmonary vein compression, right atrial enlargement, atrial dysrhythmia, or atrial thrombus. The conversion operation should not be unduly delayed to prevent irreversible deterioration of clinical status with chronic rhythm disturbances or protein-losing enteropathy. The benefit of the conversion operation is questionable in patients with poor clinical condition and protein-losing enteropathy.

Objective: Continued follow-up of the Fontan population group is mandatory in order to evaluate the best approach for long term treatment. We studied exercise capacity and survival in patients with either right atrial to right ventricular (Fontan-Bjoerk, RA-RV) anastomosis or right atrial to pulmonary artery (RA-PA) connection., Methods: Between January 1980 and December 1995 Fontan-Bjoerk modifications were performed in 73 patients with tricuspid atresia. A RA-PA anastomosis (performed either with direct atrio-pulmonary connection or with a lateral tunnel of autologous atrial tissue) was used in 118 patients with single ventricle or complex cardiac malformations. Using bicycle ergospirometry and impedance cardiography standard variables of exercise testing were measured in 15 patients with RA-RV and in 18 patients with RA-PA connection. A group of 23 healthy pupils served as controls., Results: Follow-up was complete for 97.9% (n = 187) of all operated patients. Survival (% mean +/- SEM) at 5, 10 and 15 years was 89.3 +/- 3.6, 76.8 +/- 0.6 and 63.6 +/- 10.5 for RA-RV connection and 80.2 +/- 4.0, 75.3 +/- 4.5 and 64.6 +/- 10.7 for RA-PA connection (P = 0.12) respectively. Exercise capacity was tested after a median time of 6.0 (0.8-19.8) years after Fontan operation in RA-RV and of 7.8 (4.3-18.2) years in RA-PA patients. Total work load was equal in the two Fontan groups, but it was below normal. Heart rate, respiratory rate, oxygen uptake and ventilatory equivalent for oxygen were not different between the two Fontan groups. Cardiac index and stroke volume index were consistently lower at anaerobic threshold and at maximal exercise in RA-PA patients compared with controls., Conclusion: Survival analysis between RA-RV and RA-PA Fontan connection failed to demonstrate a better outcome for patients with either Fontan modification. Although there was a tendency for RA-RV connection to adapt cardiac output more efficient to exercise compared with RA-PA patients, total work load and ventilatory equivalent was not significantly different between the two Fontan modifications. We conclude, that by incorporation of a residual subpulmonary ventricular chamber within the Fontan circulation no additional benefit for exercise capacity could be observed.

The patient, a 17 year old boy, had undergone a Rastelli operation for transposition of the great arteries plus ventricular septal defect and left ventricular outflow tract obstruction at the age of 7 years. Associated tricuspid valve stenosis was treated with insertion of a second homograft shortly after the Rastelli operation. The patient remained symptom-free for 10 years, when he suffered femoral artery embolus. Investigation showed severe obstruction of both conduits and biventricular dysfunction with thrombus in the left ventricle. After embolectomy and stabilisation of his general condition and prolonged treatment with heparin, he underwent uneventful replacement of the two conduits. He remains well, 8 months after the operation.

Objective: Atrio ventricular septal defects (AVSD) with normal caryotype represent in average 25% of AVSD. They constitute a more complex group of patients characterized by frequent left sided heart obstructive lesions, raising the problem of the appropriate indications between biventricular and univentricular procedures., Methods: Sixty-nine consecutive patients, who had AVSD with normal caryotype underwent surgery. According to the anatomical complexity there were 22 intermediate AVSD, 36 complete AVSD and 11 complex AVSD. Associated lesions were present in 68% of the patients including left sided heart anomalies in 57%. According to the size of the left ventricle (LV) evaluated on the LV/RV end diastolic diameter ratio calculated at 2D echocardiogram: right ventricular (RV) dominance was found in 29%; with border line LV in 13 patients and truly hypoplastic LV in 7 patients. Biventricular repair was always favored in case of border line LV and precluded when the LV/RV ratio was less than 0.33., Results: There were 57 biventricular repairs with 10 years actuarial survival of 70 +/- 8% and respectively, 100% in the complex AVSD, 86% in the intermediate AVSD and 51% in the complete AVSD (P = 0.006). The risk factors for biventricular repair were the association to a subaortic stenosis (P = 0.01) and the severity of the mitral valve lesions (P = 0.03) that led to 38% reoperation. There were 12 univentricular palliation with 10 years survival of 66 +/- 14%. The risk factor for univentricular palliation was the association to a severe pre-operative mitral regurgitation (P = 0.005)., Conclusions: Biventricular repair should be precluded in patients presenting with subaortic stenosis. Severe mitral valve anomalies lead to elevated mortality and morbidity with frequent reoperations. Univentricular repair might have larger indications and cardiac transplantation might be considered in patients with truly hypoplastic LV presenting with severe pre-operative AV valve regurgitation.

Objectives: The optimal technique of the Fontan operation remains disputable. This investigation was aimed at the comparison of atriopulmonary and total cavopulmonary anastomoses., Methods: The results of 81 operations of total cavopulmonary and 69 operations of atriopulmonary anastomosis, performed from 1983 to 1995 were analysed. A control study was carried out 1-10 (3.7 + 0.2) years after the operation in 80 patients (36 after total cavopulmonary and 44 after atriopulmonary anastomoses). 70 patients were studied several times. Cardiac catheterization was done in 70 patients. In 78 patients central hemodynamical indices were studied during exercise., Results: Preoperative hemodynamical indices were not reliably different in the two groups. Early mortality after total cavopulmonary anastomosis was 15%, after atriopulmonary anastomosis 23%. The highest mortality was seen when the criteria of Choussat et al. (Choussat et al. Pediatric Cardiology. Edinburgh: 1977:559-566) were surpassed. In the early postoperative period after total cavopulmonary anastomosis circulatory insufficiency was less marked, transsudation from pleural cavities was reliably lower (15.3 + 1.2 versus 25.5 + 1.8 ml/kg/day, P < 0.01). Arrhythmias were more common after total cavopulmonary anastomosis (18.5 versus 12%). Late mortality after total cavopulmonary anastomosis was 6%, after atriopulmonary anastomosis 12%. 82% of patients after atriopulmonary anastomosis and 81% after total cavopulmonary anastomosis were in NYHA classes 1 and 2.7% of patients after total cavopulmonary anastomosis and 11% after atriopulmonary anastomosis were reoperated. Physical tolerance rose stepwise in both groups and by the third post-operative year reached 75% of normal level. At this time we saw the most optimal hemodynamical indices during exercise. However, the best hemodynamics during exercise were seen after atriopulmonary anastomosis., Conclusions: Total cavopulmonary anastomosis is accompanied by lower mortality rate and a more favourable course of early postoperative period. However, the best long-term functional results are obtained after atriopulmonary anastomosis.