Bilateral bidirectional cavopulmonary connection: a review of surgical techniques and clinical implications.

The presence of bilateral superior caval veins (bSCVs) could negatively influence the outcome of Fontan patients. In the setting of a bilateral bidirectional Glenn, the selective blood flow to the ipsilateral long with consequent flow stagnation in the connecting portion could lead to poor growth of the central portion of the pulmonary artery, potentially affecting the eligibility for Fontan completion and being associated with a higher incidence of thrombotic complications. Alternative surgical techniques have been described to perform a bidirectional cavopulmonary anastomosis in the presence of bSCVs aiming to achieve a balanced growth of the pulmonary bifurcation. The short-term results of these techniques such as the V- or Y-shape seem to be excellent; however, some anatomical settings could affect the feasibility of these techniques. The so-called "unifocalization" creates a configuration comparable to a "normal" bidirectional Glenn and could be a feasible alternative. However, the long-term results of this technique are not published yet. The positive effect of additional pulsatile pulmonary flow on pulmonary artery growth should be considered in case of bilateral bidirectional Glenn, despite the higher incidence of postoperative complications reported and the difficult calibration of the amount of additional flow. The role of computational fluid dynamic to simulate the surgical strategy in single ventricle patients is promising and could be worthwhile in the setting of bSCVs. In fact, the surgical techniques of bilateral bidirectional Glenn could be simulated testing their feasibility and allowing to identify the more favorable hemodynamic pattern, patient specific. This review article highlights the critical issues related to the presence of bSCVs in univentricular physiology, analyzing pros and cons of the different surgical techniques. Besides reviewing the literature, this manuscript focuses on the role of computational fluid simulation in identifying the most favorable surgical technique with an individualized approach, which could potentially improve the clinical outcome.

Intraoperative transit-time flow measurement of caval veins before and after bidirectional cavopulmonary anastomosis.

BACKGROUND: Haemodynamic changes in caval venous flow distribution occurring during bidirectional cavopulmonary anastomosis operation are still largely unknown. METHODS: Transit time flow measurements were performed in 15 cavopulmonary anastomosis operations. Superior and inferior caval vein flows were measured before and after the cavopulmonary anastomosis. Ratio of superior caval vein to overall caval veins flow was calculated. RESULTS: Mean superior caval vein flow ratio before cavopulmonary anastomosis was higher than previously reported for healthy children. Superior caval vein flow ratio decreased in 14/15 patients after cavopulmonary anastomosis: mean 0.63 ± 0.12 before versus 0.43 ± 0.14 after. No linear correlation between intraoperative superior caval vein pressure and superior caval vein flow after cavopulmonary anastomosis was found. Neither Nakata index nor pulmonary vascular resistance measured at preoperative cardiac catheterisation correlated with intraoperative flows. None of patients died or required a take down. CONCLUSIONS: The higher mean superior caval vein flow ratio before cavopulmonary anastomosis compared to healthy children suggests flow redistribution in univentricular physiology to protect brain and neurodevelopment. The decrease of superior caval vein flow ratio after cavopulmonary anastomosis may reflect the flow redistribution related to trans-pulmonary gradient. The lack of correlation between superior caval vein pressure and superior caval vein flow could be explained by limited sample size and multifactorial determinants of caval veins flow, although pressure remain essential. Larger sample of measurements are needed to find flow range potentially predictive for clinical failure. To authors' knowledge, this is the first intraoperative flow measurement of both caval veins during cavopulmonary operations.

Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study.

OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Thoracotomy Versus Sternotomy for Patent Ductus Arteriosus Closure in Preterm Neonates.

BACKGROUND: To date, a posterolateral thoracotomy approach is considered the gold standard for surgical closure of patent ductus arteriosus (PDA), also in preterm neonates. However, a posterolateral thoracotomy approach can induce post-thoracotomy lung injury of the immature and vulnerable lungs of preterm neonates. Therefore, this study aims to compare a posterolateral thoracotomy and median sternotomy for surgical closure of PDA in preterm neonates. METHODS: Between September 2010 and November 2014, both surgical approaches were used to treat a symptomatic PDA in very and extremely preterm neonates. The hospital records of all these neonates were retrospectively reviewed to assess all-cause mortality and postoperative morbidity in both groups. RESULTS: Despite comparable preoperative patient profiles, the postoperative pulmonary complication rate was significantly lower in the median sternotomy group (52.9% vs 94.7%; P = .006). Moreover, significantly lower mean airway pressures (MAPs) were seen in the median sternotomy group directly after surgery (ΔMAP median [interquartile range], 0.00 [2.13] vs 0.80 [1.67] cmH2O; P = .025). Postoperative blood transfusion (median [interquartile range], 20 [14] vs 17 [16] mL; P = .661) rates did not differ between both approaches. In addition, Kaplan-Meier survival analysis demonstrated no statistically significant differences between both groups. CONCLUSIONS: In our experience, a median sternotomy approach for surgical PDA closure is at least noninferior to a posterolateral thoracotomy approach. Given the lower postoperative pulmonary complication rate and lower postoperative MAPs directly after surgery, the median sternotomy approach may be considered superior for preterm neonates with immature and vulnerable lungs.

A stented bovine pericardial prosthesis in the pulmonary position.

BACKGROUND: Pulmonary valve replacement is very common among patients with congenital heart disease. The Carpentier Edwards Perimount valve (Edwards Lifesciences, Irvine, Calif), which was originally designed for the aortic position is among the most implanted valves. We aim to describe the follow-up of this valve in the pulmonary position. METHODS: Patients with a Perimount valve implanted between 2003 and 2013 in the University Medical Center Groningen were followed for the primary end point reintervention, defined as surgical or transcatheter valve replacement. Secondary end point was the occurrence of valve failure, defined as significant valvular regurgitation or stenosis. Explanted valves were histologically examined. RESULTS: Forty-five patients (median age at operation 27.8 years, 55.6% women) had a mean follow-up duration of 5.8 ± 3.3 years. There were 7 reinterventions (5 surgical and 2 transcatheter). Freedom from reintervention was respectively 95% ± 4% and 83% ± 8% at 5- and 10- years of follow-up. Freedom from valve failure was 75% ± 4% at 2 years, 65% ± 8% at 5 years of follow-up and 57% ± 10% at 10 years of follow-up. Morphology evaluation (n = 4) showed stiffened valves in the open position, with extensive fibrous tissue overgrowth on the leaflets and a variable proliferation of myofibroblasts. CONCLUSIONS: The Perimount valve has adequate function in the pulmonary valve position at 5 years of follow-up, although after 10 years of follow-up valve failure and reinterventions are common. Explanted valves show retraction and stiffening of the leaflets due to a fibrotic layer on both sides of the leaflet.

Autologous Right Auricular Versus PTFE Cavopulmonary Lateral Tunnel: Influence of Surgical Technique on Arrhythmias.

BACKGROUND: To compare the incidence of arrhythmias and the overall survival at long-term follow-up of the right auricular baffle technique (RA) versus Gore-Tex® (GT) baffle as intra-atrial cavopulmonary lateral tunnel, as well as the Nakata index and tunnel dimensions on cardiac magnetic resonance. METHODS: Data were retrospectively collected. Serial 24-hour Holter recordings and cardiac magnetic resonance findings of the two groups were compared. RESULTS: There was no significant difference in the estimated freedom from arrhythmias (87% at 10 years and 78% at 15 years vs 80% at 10 years and 70% at 15 years in RA and GT, respectively; P = .44) nor cumulative survival (86% at 10 years and 84% at 15 years vs 97% at 10 years and 81% at 15 years in RA and GT, respectively; P = .8). Also, no difference between the groups was observed in the Nakata index. The tunnel dimensions on cardiac magnetic resonance were significantly wider in the RA group. In reference to other potential risk indicators, using Cox proportional hazard regression analysis, only age (5 years or older at the time of total cavopulmonary connection) was associated with an increased risk for both arrhythmia and mortality. CONCLUSIONS: This study demonstrated that there was no difference in freedom from arrhythmias, Nakata index, or survival between the two groups. This study confirmed the growth potential of the right auricular tunnel. However, the growth of the tunnel did not influence the incidence of arrhythmias.

Resection of Kommerell Diverticulum and Reimplantation of Aberrant Left Subclavian Artery in Right Aortic Arch Vascular Ring.

A right aortic arch vascular ring consists of an anomalous aortic arch course with a ligamentum arteriosum and an aberrant left subclavian artery arising from a Kommerell diverticulum. Division of the ligamentum arteriosum is required for the relief of symptoms in these patients. However, to prevent recurrent symptoms, resection of the Kommerell diverticulum and reimplantation of the subclavian artery to the left carotid artery is advocated. Here we describe our standardized surgical approach to this pathology.

Acute Type A Dissection 18 Years After a Ross Operation: The Old Prejudice.

An acute aortic dissection of the pulmonary autograft with a free rupture of the neoaortic wall is a rare complication after a Ross procedure. In this case, the intimal tear was localized in the noncoronary sinus of the pulmonary autograft and the neoaortic wall was ruptured in the involved sinus area. The dissection was contained in the pulmonary autograft. This report describes a successful aortic root replacement of a patient with the clinical symptoms of an acute aortic type A dissection with a free rupture of the neoaortic wall.

Surgical Correction of Supravalvar Aortic Stenosis: 52 Years' Experience.

OBJECTIVES: Supravalvar aortic stenosis (SVAS) is a rare congenital anomaly. The "single-patch technique," "'two sinus augmentation with an inverted Y-patch" (both nonsymmetrical corrections), "three-patch technique," and the "slide aortoplasty" (both symmetrical corrections) are the techniques implemented by the majority of surgeons for the correction of SVAS. In the few studies that compared these techniques, no technique was shown to be superior over another. The aim of the present study is to review the 52-year experience with the surgical correction of SVAS in two of four congenital cardiothoracic surgical centers in the Netherlands. METHODS: We retrospectively reviewed all patient files of those who underwent an operation to correct their SVAS, between 1962 and 2014 in our centers. Patients were divided according to their operating technique. These groups were compared using the end points freedom from reoperation and mortality. RESULTS: A total of 49 patients were included, 23 (46.9%) patients in the nonsymmetrical group and 26 (53.1%) patients in the symmetrical group. Survival after 20 years in the nonsymmetrical group was 80% (standard error [SE]: 0.091) and in the symmetrical group was 85% (SE: 0.085; P = .163). Freedom from reoperation after 20 years in the nonsymmetrical group was 88% (SE: 0.079) and in the symmetrical group was 71% (SE: 0.107; P = 0.313). CONCLUSION: In this patient group, there is no significant difference in survival and freedom from reoperation between the different surgical techniques for SVAS repair. Compared to the survival in the general population, the survival of SVAS patients is remarkably low. Apparently, SVAS is not a benign disease and probably patients should be followed more closely for the rest of their lives.

Effects of a multidisciplinary weight loss intervention in overweight and obese children and adolescents: 11 years of experience.

AIMS: To evaluate the effects of an outpatient multidisciplinary weight loss intervention in reducing body mass index (BMI) in children and adolescents suffering overweight and obesity, changes in A Body Shape Index (ABSI, waist circumference normalized to height and weight) and Hip Index (HI, normalized hip circumference) during treatment and correlation between the ABSI and HI with change in BMI z score. METHODS: We analyze anthropometric data from pediatric patients affected by overweight and obesity aged 2 to 18 years old who entered our multidisciplinary weight loss intervention, which included medical, psychological and nutritional sessions, from January 1st 2006 to December 31st 2016. Lifestyle modification counselling was delivered. Follow-up visits were planned every month for 3 months and subsequently every 2-4 months. BMI, ABSI, and HI were converted to z scores using age and sex specific population normals. RESULTS: 864 patients entered our intervention. 453 patients (208 males), mean age 11.2 ±3.1 years, 392 with obesity (86%, z-BMI 2.90 ±0.80 SD) and 61 patients with overweight (z-BMI 1.73±0.21 SD) attended at least 1 follow-up visit. The mean number of visits was 3.5 (± 1.8 SD) in overweight subjects and 3.9 (±2.2 SD) in ones with obesity. At the last attended follow-up visit (at 16 ± 12 months SD) we observed a reduction in mean z-BMI in patients with obesity (to 2.52 ±0.71 SD) and patients with overweight (to 1.46 ±0.5 SD). Most patients (80.8%) reduced their BMI z scores. Mean ABSI and HI z scores showed no significant change. 78/392 patients (19.8%) recovered from obese to overweight, 5/392 (1.2%) from obese to normal weight. The recovery rate from overweight to normal weight was 13.1%. In a multivariate model, initial BMI z score and number of follow-up visits were significant predictors of weight change, while age, sex, ABSI, and HI were not significant predictors. CONCLUSIONS: Patients affected by overweight and obesity involved in a multidisciplinary weight loss intervention reduced their mean BMI z score, while ABSI and HI were stable. Weight loss was not predicted by initial ABSI or HI. More visits predict more weight loss, but dropout rates are high. The great majority of patients leave the weight management program before having normalized their BMI.

Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.

OBJECTIVES: An almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas. METHODS: We analyzed 11 patients with arteriovenous fistulas that developed after the Kawashima operation. The hepatic-to-azygos shunts were performed with an off-pump technique through a lateral thoracotomy in all but one. Operative and postoperative data were retrospectively collected. RESULTS: No intraoperative complications occurred, and no patient died in the hospital. Up to 10-year follow-up showed a significant postoperative improvement of patients' oxygen saturation and New York Heart Association class. Apart from 2 re-thoracotomies for bleeding in 1 patient, no complications occurred and no patient died during follow-up. Two other patients underwent reoperation for an undiagnosed additional hepatic vein. The improvement of patients' oxygen saturation and New York Heart Association class persisted during the follow-up period. CONCLUSIONS: The surgical connection can be performed safely with an off-pump technique that avoids the risks related to extracorporeal circulation and circulatory arrest. The results at 10 years follow-up confirmed the efficacy and safety of the surgical technique described.

Off-pump no-touch technique: 3-year results compared with the SYNTAX trial.

OBJECTIVES: A 3-year follow-up of a retrospective, single-centre clinical study of OPCAB (off-pump coronary artery bypass) no-touch technique with arterial composite grafts and an indirect comparison with clinical outcomes of the 3-year follow-up of the SYNTAX trial. METHODS: A total of 400 consecutive patients ('all-comers') who underwent OPCAB no-touch coronary surgery. Primary endpoint was 3-year MACCE (major adverse cardiovascular and cerebrovascular events). These results were compared with the randomized arms of the SYNTAX trial. RESULTS: The observed 3-year survival was 82.2%. The cumulative 3-year freedom from MACCE was 89.7%. The percentage of MACCE was significantly lower (P < 0.001) in the OPCAB no-touch group (10.3%) compared with both arms of the SYNTAX trial (20.2 and 28.0%, respectively). Repeat revascularization in the OPCAB no-touch group (4.3%) was significantly lower (P < 0.001) compared with both arms of the SYNTAX trial (10.7 and 19.7%, respectively). The percentage of stroke was significantly lower (P = 0.032) in the OPCAB no-touch group compared with the CABG arm in the SYNTAX trial (1.3 vs 3.4%). There was no significant difference of stroke rate between the OPCAB no-touch group and the PCI arm of the SYNTAX trial (1.3 vs 2%, P = 0.347). CONCLUSIONS: The results at the 3-year follow-up showed the safety and efficacy of OPCAB no-touch technique. The OPCAB no-touch technique may improve patients' outcome by minimizing the morbidity and the neurological complications. Prospective controlled trials are needed to confirm these results.

Lusoria flap for the management of aortic coarctation in an eight-year-old child.

Anomalous origin of right subclavian artery arising from the descending aorta is known as "arteria lusoria." The diagnosis in asymptomatic children is usually the by-product of other symptomatic-associated anomalies, such as aortic coarctation. We describe a case of an eight-year-old boy with juxtaductal aortic coarctation and rare origin of the arteria lusoria proximal to the aortic coarctation. The anomalous arteria lusoria was used as a flap to repair the aortic coarctation. To the authors' knowledge, this is the first reported application of lusoria flap in a young child (not newborn) with ductal aortic coarctation.

Patients' satisfaction and wound-site complications after radial artery harvesting for coronary artery bypass.

The aim of the study was to establish patients' satisfaction and the incidence of wound-site complications after radial artery (RA) harvesting for bypass surgery. A telephonic quality of life questionnaire was performed in 306 consecutive patients who had undergone coronary artery bypass grafting with the use of RA with the open technique. A psychometric Likert scale was used to define the degree of patients' satisfaction. The questionnaire concerned arm pain, cosmesis and mobility, sensory and neurological complications, and patients' general health state. The median values and the modal scores achieved the maximal value of satisfaction for all site-related complications and the mean scores were >4 out of 5. The median patients' general health state was 4. Cosmetic result of the wound was considered at least acceptable by 98% of patients. The incidence of impairing peripheral neurological complications was 16.7%. The degree of patients' satisfaction after RA harvesting was more than satisfactory and the incidence of wound-site complications was acceptable. The incidence of neurological injuries was lower than previously described.

PulseCath(R) as a right ventricular assist device.

The PulseCath(®) is a pulsatile pump that offers a circulatory support up to 3 l/min. The PulseCath(®) is indicated for patients who require a higher degree of support than that offered by the intra-aortic balloon pump. We describe the first two cases of the use of the PulseCath(®) as a temporary support for the right ventricle after insertion through the pulmonary artery trunk. Two patients developed an acute right ventricular failure with severe hemodynamic instability after cardiac surgery. The PulseCath(®) was chosen to assist the right ventricle. An immediate improvement of hemodynamic parameters was observed in both cases. In the first patient an irreversible metabolic unbalance, already present prior to PulseCath(®) insertion, led to multi-organ failure and eventually to death. In the second case the early utilization of PulseCath(®) led to a complete recovery of the right ventricle and the patient was discharged in good clinical condition. Besides the technical feasibility, this report would suggest that a correct timing is the key to success for the PulseCath(®) as a right ventricular assist device.

Off-pump Y-graft coronary artery bypass in a patient with situs inversus totalis.

Situs inversus is the mirror image of situs solitus. Situs inversus with dextrocardia is termed 'situs inversus totalis'. Since situs inversus totalis is a rare condition, there are a only a few reports about off-pump coronary artery bypass (OPCAB) in these patients. A 67-year-old man with a diagnosis of situs inversus totalis and three-vessel disease, underwent an elective surgical revascularization. The operation consisted of an OPCAB using a total arterial composite Y-graft. The right internal mammary artery (RIMA) and the left radial artery (RA) were harvested as pedicles and they were anastomized as Y-grafts. Three distal anastomoses were performed: the RIMA to the anterior descending artery and the RA sequentially to the obtuse marginal branch and the posterior descending artery. The peculiarity of OPCAB no-touch with a composite graft in a patient with situs inversus totalis consisted in the mirroring of the whole surgical set-up and of the surgical strategy. The surgery as well as the postoperative period were uneventful. This case report shows the feasibility of OPCAB 'no-touch' with composite arterial grafting in a patient with situs inversus totalis.

Four-year outcome of OPCAB no-touch with total arterial Y-graft: making the best treatment a daily practice.

BACKGROUND: A retrospective, single-center 4-year clinical study of the off-pump coronary artery bypass grafting no-touch technique with arterial conduits (Y-graft) was compared with the Syntax trial. METHODS: Four hundred consecutive patients ("all-comers") who underwent coronary surgery between 2004 and 2008 at the Thorax Center Twente (TCT) formed the study group. The primary end point was in-hospital and 12-month major cardiovascular or cerebrovascular event (MACCE). Event rates of MACCE were based on life tables, and overall MACCE was determined by Kaplan-Meier analysis. RESULTS: In-hospital mortality was 0.2%. Cumulative 1-year survival was 98.2%, and freedom from MACCE was 94.7% +/- 1.1%. Cumulative 4-year survival and freedom from MACCE were 91.2% +/- 2.4% and 82.1% +/- 3.0%, respectively. There were no significant differences in the baseline characteristics between the patients of the TCT group and the surgical arm of the Syntax trial. Repeat revascularization, MACCE, and symptomatic graft occlusion in the TCT group were significantly lower than in the Syntax trial. The event rate of myocardial infarction and all-cause death in the TCT group were significantly lower than those of the percutaneous coronary intervention arm of the Syntax trial. There was a clear trend toward a reduction of the event rate of stroke in the TCT group (0.8%) compared with the surgical arm of the Syntax trial (2.2%). There was no significant difference of stroke rate between the TCT group and the percutaneous coronary intervention arm of the Syntax trial. CONCLUSIONS: A state-of-the-art surgical technique such as off-pump coronary artery bypass grafting no-touch can further improve the advantage of surgical treatment with respect to percutaneous coronary intervention. Off-pump coronary artery bypass grafting no-touch surgery can be the treatment of choice for patients with three-vessel disease and left main stenosis.