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Background Patients with Fontan circulation may develop heart failure resulting in atrial fibrillation during the late phase. Inotropic effects to ameliorate hemodynamics on the Fontan circulation are not well understood, especially when in atrial fibrillation. This study was performed to determine whether dobutamine therapy in patients with Fontan circulation has limited effects on improving hemodynamics. Methods Lumped computational models (sinus and atrial fibrillation) were employed including biventricular, atriopulmonary connection (APC), and extracardiac total cavopulmonary connection (TCPC) Fontan models. The condition of atrial fibrillation including lack of atrial beat, irregular ventricular contraction, and time-varying elastance for the ventricle was introduced. A different dose of dobutamine was given by varying the elastance of the ventricle, heart rate, and peripheral resistance. Results In all models, the cardiac output decreased by 22.5% to 25.8% in atrial fibrillation. At 10 μg/kg/min of dobutamine in sinus rhythm, the cardiac output increased by 32.3% in the biventricular model but by only 9.2% (p Conclusions In the Fontan circulation, atrial fibrillation itself reduced the cardiac output by approximately 25%, and dobutamine had a limited effect on increasing the cardiac output, especially when in atrial fibrillation. Maintaining sinus rhythm in patients with Fontan circulation is very important.

This study investigated a 2-stage arterial switch operation (ASO) to treat transposition of the great arteries (TGA) with intact ventricular septum (TGA-IVS) in late referral patients.We retrospectively analyzed patients with TGA-IVS or TGA with restricted ventricular septal defects who had undergone 2-stage ASO at our institution from February 2007 to August 2018. Included were 41 patients: 21 (51.2%) who had undergone long-term 2-stage ASO and 20 (48.8%) who had undergone rapid 2-stage ASO.The long-term 2-stage group was older at ASO (3.5 vs 25 months; P.001). Results were more satisfactory in the long-term group than in the rapid group for intensive care unit time (P = .004), mechanical ventilation time (P = .004), and length of stay (P = .007). No in-hospital death occurred in the long-term group, and the postoperative course was more manageable in the long-term group than in the rapid group. However, the risk of significant neoaortic regurgitation was lower in the rapid group, which also had a better left ventricular ejection fraction.The long-term group achieved better early-term outcomes than the rapid group. However, a high risk of neoaortic regurgitation and myocardial dysfunction was also noted.

Unifocalization of the major aorto-pulmonary collaterals (MAPCAs) followed by intra-cardiac repair with ventricular septal defect (VSD) closure and restoration of right ventricle to pulmonary artery (RV-PA) continuity is the ultimate treatment goal in a case of VSD with pulmonary atresia (PA) and MAPCAs. It may be achieved in a single stage or may require multiple surgeries. We present a case of a 2 year old boy with VSD/PA who underwent single stage unifocalization of MAPCAs through midline followed by intra-cardiac repair using two polytetrafluoroethylene (PTFE) tube grafts; one for unifocalization and other as a bicuspid valved RV-PA conduit.

We previously reported the first successful implantation of the Heartmate 3TM in a Fontan patient. We now report his successful transplantation after 1,104 days of support, the longest reported bridge to transplantation of a Fontan patient. We describe our operative technique complicated by not only the Fontan anatomy and ventricular assist device (VAD) explanation but also a >10cm ascending and aortic arch aneurysm. Additionally, the post-transplant hemodynamics of this patient appear to demonstrate that effective VAD support may induce reversal of chronic effects of the failing Fontan circulation, which in this case was the elimination of his aorto-pulmonary collateral burden.

Few reports have described the implantation and management of an implantable ventricular assist device in patients with functional uni-ventricular anatomy. We herein report a case who underwent HVAD implantation due to severe cardiac dysfunction and Fontan failure in the remote period following total cavo-pulmonary connection. Double valve replacement was subsequently required one year later due to repeated Fontan failure caused by the progression of aortic and atrioventricular regurgitation. The low-profile and saddle-shaped sewing cuff design of the MITRIS were beneficial for avoiding the HVAD inflow and atrioventricular valve interference in the less-dilated ventricle.

Pentalogy of Cantrell (POC) is a rare congenital disorder characterized by defects of the anterior diaphragm, midline supraumbilical abdominal wall, diaphragmatic pericardium, lower sternum, and heart. The low incidence and significant heterogeneity of POC make it difficult for most surgeons to gain consistent experience in treating these congenital heart defects. Here we describe the treatment of a patient with POC, Tetralogy of Fallot, left ventricular (LV) diverticulum, and a muscular ventricular septal defect (VSD). An innovative approach through a left ventricular diverticulotomy was utilized which provided excellent exposure for repair of the muscular VSD.

Given its complex pathological anatomy, recurrent left atrioventricular valve regurgitation after partial atrioventricular septal defect repair remains a challenge for surgical correction. Here, we introduce a modified bridging technique by shortening the anteroposterior leaflet distance in selected patients with inadequate coaptation to compensate for the short leaflet height, specifically that of the anterior leaflet.

Background There is active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the nonfenestrated extracardiac Fontan including Fontan conversion. Methods We performed a retrospective review of all nonfenestrated extracardiac Fontan and Fontan conversion operations at our institution from December 1, 1994 to December 31, 2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis of risk factors for adverse outcomes. Results There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 were extracardiac nonfenestrated Fontan completion operations (57%) and 148 were Fontan conversions (43%). Length of stay was 11 days (SD, 6 days) with ventilator duration of 28 hours (SD, 26 hours). Six of the completion Fontan patients (3%) required Fontan takedown at a median time of 2.5 days. Upon multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length of stay. Kaplan-Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients. Conclusions Over the past 24 years, our strategy of nonfenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation; the key risk factor is preoperative ventricular dysfunction.

Our aim was to evaluate the results of stage 2 palliation by means of bidirectional cavopulmonary shunt (BCPS) and to identify risk factors for failed Fontan completion.Between 1998 and 2018, BCPS was performed on 525 patients with functional single ventricle. Patient demographics, surgical data, and echocardiographic and cardiac catheterization measurements were analyzed, and outcomes after BCPS were evaluated.The median age at BCPS was 4.7 months (interquartile range, 3 to 7.4). Hypoplastic left heart syndrome was the most frequent diagnosis. The median follow-up after BCPS was 3.4 years (interquartile range, 1.5 to 8.7); 407 patients underwent the Fontan procedure, 50 were waiting for Fontan completion, 47 died, 6 were considered not suitable for Fontan completion, and 15 were lost to follow-up. Of the 407 patients who underwent Fontan completion, there were 5 early deaths. Freedom from failed Fontan completion (mortality before, unsuitability for, and early mortality after the Fontan procedure) at 1, 2, and 3 years was 91.9%, 87.3%, and 86.1%, respectively. Risk factor analysis for failed Fontan completion revealed hypoplastic left heart syndrome (hazard ratio [HR] 4.1, P = .001), unbalanced atrioventricular septal defect (HR 10.1, P.001), higher pulmonary artery pressure (HR 1.1, P = .040), and reduced ventricular function (HR 4.2, P = .001) as risks.Stage 2 palliation can be performed with minimal risk and provides excellent subsequent Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure, and reduced ventricular function at the time of BCPS were identified as risk factors for failure to successfully complete the Fontan procedure.

Pulmonary hamartoma is a common benign tumor that rarely degenerates into malignancy. We document a unique case of pulmonary hamartoma with malignant transformation into well differentiated liposarcoma, coexisting in proximity to pulmonary nodules representing benign metastasizing leiomyoma in a sixty-year-old lady.

Cardiac or aortic surgeries requiring cardiopulmonary bypass in combination with patent ductus arteriosus in adults present a special problem that requires a tailored approach. We report a case of ruptured type A acute aortic dissection in a 73-year-old woman with patent ductus arteriosus. In this case, we highlight that transpulmonary direct ductus arteriosus closure and subsequent frozen elephant deployment with total arch replacement are reasonable and reliable treatments for type A acute aortic dissection in a patient with patent ductus arteriosus.

Truncus arteriosus is a congenital heart defect with high resource use, cost, and mortality. Value assessment (outcome relative to cost) can improve quality of care and decrease cost. This study hypothesized that truncus arteriosus repair at a high-volume center would result in better outcomes at lower cost (higher value) compared with a low-volume center.This study retrospectively analyzed a multicenter cohort of neonates undergoing truncus arteriosus repair (2004 to 2015) by using the Pediatric Health Information Systems database. Multivariate quantile, logistic, and negative binomial regression models were used to evaluate total hospital cost, in-hospital mortality, ventilation days, intensive care unit length of stay (LOS), hospital LOS, and days of inotropic agent use by center volume (high-volume3/year) and age at repair while adjusting for sex, ethnicity, race, genetic abnormality, prematurity, low birth weight, concurrent interrupted arch repair, and truncal valve repair.Of 1024 neonates with truncus arteriosus, 495 (48%) were treated at high-volume centers. Costs at the 75th percentile were lower at high-volume vs low-volume centers by $28,456 (P = .02) at all ages at repair. Patients at high-volume centers had lower median postoperative ventilation days (5 days vs 6 days; P.001), intensive care unit LOS (13 days vs 19 days; P.001), hospital LOS (23 days vs 28 days; P = .02), and inotropic agent use (3 days vs 4 days; P = .004). In-hospital mortality did not differ by center volume.In neonates undergoing truncus arteriosus repair, costs are lower and outcomes are better at high-volume centers, thus resulting in higher value at all ages of repair. Value-based interventions should be considered to improve outcomes and decrease cost in truncus arteriosus care.

Surgery for truncus arteriosus has an early mortality of 3% to 20%, with a long-term survival of approximately 75% at 20 years. Nowadays, truncus arteriosus repair is mostly done in the neonatal period together with a single-staged repair of concomitant cardiovascular anomalies. There are many challenging subgroups of patients with truncus arteriosus, including those with clinically significant truncal valve insufficiency, an interrupted aortic arch, or a coronary artery anomaly. In fact, truncal valve competency appears to be the most important factor influencing the outcomes after truncus arteriosus repair. The use of a conduit during truncus arteriosus repair invariably requires reoperation on the right ventricular outflow tract. Through improvements in perioperative techniques over time, many children are now living well into adulthood after repair of truncus arteriosus, albeit with a high rate of reoperation. Despite this, the long-term outcomes of truncus arteriosus repair are good, with many patients being asymptomatic and with a quality of life comparable to the general population.

The native tricuspid leaflets are sometimes preserved with tricuspid valve replacement to prevent atrioventricular block or maintain right ventricular function. However, after a tricuspid valve replacement using bioprosthetic valve, a rare complication involving adhesion of the pannus formation to the prosthetic valve may occur. We report the case of a 48-year-old woman who underwent a third tricuspid valve replacement with a bioprosthetic valve for severe tricuspid regurgitation 2.5 years after the redo tricuspid valve replacement.

In patients with interrupted aortic arch and ventricular septal defect (VSD) with a small left ventricular outflow tract (LVOT), either aortopulmonary amalgamation or a Ross-Konno type procedure can be performed to create stable systemic outflow. We sought to analyze factors associated with these different surgical approaches.We retrospectively identified patients who underwent surgical repair for interrupted aortic arch/VSD at our institution between 1998 and 2017. Of these, 43 patients had a small, native LVOT that was unsuitable for systemic outflow. Patient data were retrospectively collected for this cohort and analyzed.Aortopulmonary amalgamation was performed at 7 days (interquartile range [IQR], 5-10) in 30 patients (group I). Within group I a primary Yasui repair with ventricular septation was performed in 3 patients and a Norwood-type repair in the other 27. Of these 27, 19 underwent subsequent biventricular conversion at 9 months (IQR, 7-11). In contrast 13 patients underwent a Ross procedure at 12 days (IQR, 6-27) (group II). Compared with group I, group II patients had a smaller VSD (3.5 vs 5.1 mm, P.001) that was more often remote from the semilunar valves (38% vs 13%, P = .02). Operative mortality occurred in 1 group I patient (4%) at the time of biventricular conversion and 2 group II patients (15%) during the Ross procedure. After a 5.2-year (IQR, 3.2-7.4) follow-up there were 2 additional mortalities in each group, all unrelated to cardiac disease.When native LVOT in interrupted aortic arch/VSD is unsuitable for systemic outflow, size and location of the VSD can be used to tailor the surgical approach to establish biventricular circulation with favorable intermediate-term outcomes.

BACKGROUND. Patients with acute type A aortic dissection with a previous cardiac surgery (PCS) and malperfusion syndrome (MPS) are extremely difficult to manage and have poor outcomes. METHODS. From 1996 to 2018, 668 patients underwent emergent open aortic repair or endovascular fenestration/stenting for MPS for an acute type A aortic dissection, including those with PCS (PCS, n = 64) and those without PCS (No-PCS, n = 604). The groups were further divided into PCS+MPS, PCS+No-MPS, No-PCS+MPS, and No-PCS+No-MPS. RESULTS. Compared with the No-PCS group, the PCS group had significantly more coronary artery disease, acute renal failure, and mesenteric and renal MPS. Forty-two percent of patients with PCS underwent upfront endovascular fenestration/stenting for endovascular-amendable MPS. The in-hospital mortality was significantly higher in patients with PCS+MPS (40%) compared with PCS+No-MPS (5.9%), No-PCS+MPS (30%) and No-PCS+No-MPS (6.7%). Multivariable logistic regression showed cardiogenic shock (odds ratio, 7.3) and MPS (odds ratio, 6.6) were risk factors for in-hospital mortality (P < .0001). After recovering from MPS the PCS group (n = 54) had similar rates of postoperative complications, including 30-day mortality (7.4% vs 6.3%, P = .77), compared with the No-PCS group (n = 557). The 5-year survival was significantly lower in the PCS group compared with the No-PCS group (60% vs 72%, P = .004) and was lowest in those with PCS+MPS (46%). PCS was not a significant risk factor for in-hospital (odds ratio, 1.2; P = .63) or late (hazard ratio, 1.3; P = .27) mortality. CONCLUSIONS. Because of severe preoperative comorbidities and the complexity of open aortic repair, in acute type A aortic dissection patients with PCS and MPS, endovascular fenestration and stenting first with delayed redo sternotomy and central aortic repair was a valid approach.

Ring annuloplasty (Ring) and suture bicuspidization annuloplasty (Suture-bicuspidization) are 2 techniques for tricuspid valve repair. With the emergence of transcatheter tricuspid valve repair technologies that mimic these conventional surgical techniques, we compared their midterm outcomes and longitudinal echocardiographic profiles.From 2002 to 2016, 650 patients underwent tricuspid valve repair (324 Ring and 326 Suture-bicuspidization) for primary or functional tricuspid regurgitation (TR). Concomitant aortic valve, mitral valve, or coronary artery bypass graft procedures were included but tricuspid valve replacement, concomitant aortic procedures, heart transplantation or ventricular assist device placement procedures were excluded. Tricuspid valve event-free survival was estimated using Kaplan-Meier competing risk analysis. Freedom from TR recurrence (defined as at least moderate TR) and freedom from tricuspid valve reoperation were also assessed.There were no statistical differences in terms of age, gender, and most baseline comorbidities (P .05). In-hospital outcomes also did not significantly differ between groups (P.05). However, Suture-bicuspidization was associated with significantly lower tricuspid valve event-free survival, and freedom from TR recurrence and tricuspid valve reoperation compared with Ring at 1, 5, and 8 years (all P.05). After adjusting for significant confounders, Suture-bicuspidization continued to be associated with significantly decreased tricuspid valve event-free survival (hazard ratio = 2.13; 95% confidence interval, 1.3-3.4).The superiority of Ring over Suture-bicuspidization annuloplasty for tricuspid valve repair appeared to be evident in terms of event-free survival and freedom from tricuspid valve insufficiency and reoperation. These findings raise concern in the context of emerging transcatheter technologies that mimic the bicuspidization technique.

Neurological disorders are the most unpredictable and feared complications after open surgery or endovascular aortic repair. Paraplegia because of spinal cord injury is well known after thoracoabdominal aortic surgery but not after valvular surgery. We herein present a case of paraplegia after mitral and tricuspid valve surgery in a patient with a history of surgery involving the thoracoabdominal and abdominal aorta. The paraplegia was likely caused by temporary postoperative hypotension as low as 40 mm Hg for more than 10 minutes with decreased spinal perfusion in the intensive care unit.

Hypoplastic left heart syndrome is almost always fatal in neonates unless it is palliated surgically. The conventional Norwood procedure with a Blalock-Taussig shunt or a Sano modification and a hybrid approach with branch pulmonary artery banding and ductal stenting are common surgical options. An alternate technique with a different strategy to restore pulmonary blood flow is described in this report.

Hutchinson-Gilford progeria syndrome is an autosomal dominant, rare, fatal pediatric segmental premature aging disease. Cardiovascular and cerebrovascular diseases constitute the major cause of morbidity and mortality. Patients with the syndrome and severe aortic valve stenosis have been described in the literature, and for all of them a strategy of conservative management has been followed. We describe the first successful treatment of a 23-year-old Hutchinson-Gilford progeria syndrome patient with severe aortic stenosis who underwent transapical transcatheter aortic valve replacement.

Therapeutic interventions are limited for high-risk burn patients with caval and intracardiac septic thrombi. Percutaneous thrombectomy represents a less invasive alternative to median sternotomy. However, there is limited literature on application of this approach and outcomes in these patients. We report two cases of patients with large total body surface area burns with similar caval and right intracardiac septic thrombi. Both patients were successfully treated using a percutaneous aspiration device.

A redo surgical approach to residual ventricular septal defect (VSD) is technically demanding. Sternum reentry has to be performed very carefully to avoid any uncontrolled bleeding, and preparation of the adhesions must be made to expose the most important structures (ascending aorta, caval veins). However, percutaneous transcatheter device closure of the residual VSD with radiation exposure can cause arrhythmia, valve injury, and vascular complications. This report presents a less invasive technique to treat residual VSD, without arterial access, radiation exposure, or cardiopulmonary bypass.

Tetralogy of Fallot with unilateral absence of the pulmonary artery is a rare congenital cardiac disorder that makes the ipsilateral lung susceptible to opportunistic infections. This disorder adds to the complexity of the case and tests the surgical and decision-making skills of the surgeon. We present an adult male patient with tetralogy of Fallot and absent left pulmonary artery who underwent physiologic exclusion of the left lung for aspergilloma along with intracardiac repair as a single stage procedure.

Dr Dwight C. McGoon, a prolific surgeon at the Mayo Clinic in Rochester, Minnesota, achieved an amazing feat during the early days of cardiac surgery. In 1965, he reported a series of 100 consecutive aortic valve replacements with no in-hospital mortality. As a pioneer in adult and congenital cardiac surgery, his other contributions included novel uses of left ventricular and biventricular extracardiac conduits and describing repairs for mitral regurgitation resulting from ruptured chordae, transposition of the great arteries, truncus arteriosus, pulmonary stenosis, and pulmonary atresia. More than 50 years later, Dr McGoon is still remembered for his technical prowess, innovation, mentorship, and humanism.

Rheumatic involvement of the tricuspid valve frequently occurs in patients with mitral and aortic valve disease. Inadequate attention to tricuspid valve function can lead to increased morbidity and may necessitate reoperation. A surgical algorithm is developed and presented for treatment of rheumatic heart disease. Seventy-four patients underwent tricuspid valve repair. Competent tricuspid valve (not more than mild tricuspid regurgitation) could be achieved in all of these patients.

There is growing awareness of the long-term impact of a Fontan circulation on the associated morbidity and mortality. Comparative data on the incidence of supraventricular arrhythmia and sinus node dysfunction following extracardiac conduit (EC) and lateral tunnel (LT) Fontans are controversial. We performed a meta-analysis pooling all available long-term results comparing the EC and LT Fontan, with a special focus on arrhythmia.We performed a systematic search of PubMed, Embase, and the Cochrane Library for articles reporting long-term results of Fontan comparing the EC and the LT Fontan.Twelve studies were selected with 3,330 patients (1,729 EC, 1,601 LT). Freedom from tachyarrhythmia was significantly higher in the EC group (92% versus 83% at 15 years; p0.0001), while there was no difference in term of bradyarrhythmias (p = 0.7). The survival was 93% and 89% at 20 years in the EC and LT groups, respectively (p = 0.007). The risk of thromboembolic events was 2.87% patient-years in the EC group versus 0.9% in the LT group (odds ratio, 2.15; 95% confidence interval, 0.95 to 4.85; p = 0.07).The EC Fontan confers long-term survival advantage over the LT Fontan without a higher rate of reoperations. The EC Fontan preserves the sinus node function and reduces significantly the incidence of long-term postoperative arrhythmia.

Aortic root translocation (Nikaidoh), Rastelli, and réparation à l'etage ventriculaire (REV) are repair options for transposition of the great arteries (TGA) with ventricular septal defects and left ventricular outflow tract obstruction (VSD-LVOTO) or double outlet right ventricle TGA type (DORV-TGA).This retrospective study using The Society of Thoracic Surgeons Congenital Heart Surgery Database evaluates surgical procedure utilization and outcomes of patients undergoing repair of TGA-VSD-LVOTO and DORV-TGA with a Nikaidoh, Rastelli, or REV procedure.A total of 293 patients underwent repair at 82 centers (January 2010 to June 2019). Most patients underwent a Rastelli (n = 165, 56.3%) or a Nikaidoh (n = 119, 40.6%) operation; only 3.1% (n = 9) underwent a REV. High-volume centers performed the majority of the repairs. Fewer Nikaidoh than Rastelli patients had prior cardiac operations (n = 57 [48.7%] vs n = 102 [63.0%]; P = .004). Nikaidohs had longer median cardiopulmonary bypass time (227 [interquartile range (IQR), 167-299] minutes vs 175 [IQR, 133-225] minutes; P.001) and median aortic cross-clamp times (131 [IQR, 91-175] minutes vs 105 [IQR, 82-141] minutes; P = .0015). Operative mortality was 3.1% (95% confidence interval [CI], 1.0%-7.0%; n = 5) for Rastelli, 4.4% (95% CI, 1.4%-9.9%; n = 5) for Nikaidoh, and 11.1% (95% CI, 0.3%-48.3%, n = 1) for REV. The rates of cardiac arrest, unplanned reoperation, mechanical circulatory support, prolonged ventilation, and permanent pacemaker placement were higher in the Nikaidoh population but with 95% CIs overlapping those of the other procedures.Rastelli and Nikaidoh procedures are the prevalent repair strategies for patients with DORV-TGA and TGA-VSD-LVOTO. Most are performed at high-volume institutions, and early outcomes are similar.

We present a case with posterior tricuspid leaflet mass involving a tricuspid annulus and the right ventricle. Echocardiography demonstrated a lobulated mass arising from posterior tricuspid valve and partially obstructing the orifice of the tricuspid valve. At the operation, a yellowish, walnut-sized mass arising from the posterior tricuspid leaflet tightly adherent to the posterior tricuspid annular was excised en bloc with posterior tricuspid leaflet and adherent ventricular myocardium and annulus. Autologous pericardium was used to repair the valve. The pathologic analysis confirmed the mass as a lipoma. In the follow-up, no recurrent mass or symptom was found.

A 21-year old woman with a history of Turner syndrome presented with a diastolic heart murmur, dizziness, dyspnea, and intermittent chest pain. Preoperative imaging revealed a fistula from the right sinus of Valsalva into the right atrium. Turner syndrome is associated with both aortopathy and congenital heart malformations. Acquired sinus of Valsalva fistula is a rare disorder, and this report describes its presence in association with Turner syndrome.

This study evaluated the impact of a staged surgical strategy incorporating a modified Blalock-Taussig shunt (BTS) for tetralogy of Fallot on pulmonary valve annulus growth, the rate of valve-sparing repair at the time of intracardiac repair, and long-term functional outcomes.This retrospective study involved 330 patients with tetralogy of Fallot who underwent intracardiac repair between 1991 and 2019 and included 57 patients (17%) who underwent BTS. The mean follow-up period was 15.0 ± 7.3 years. We compared the data of patients who underwent BTS and patients who did not undergo BTS before intracardiac repair.The median age before BTS was 71 days (range, 28 to 199) and the median body weight was 4.3 kg (range, 3.3 to 6.8 kg). There were no inhospital or interstage deaths after BTS. The pulmonary valve annulus Z scores of patients with BTS revealed significant growth after BTS (from -4.2 ± 1.8 to -3.0 ± 1.7, P.001). Valve-sparing repair was eventually performed in 207 patients (63%), including 26 (46%) who underwent staged repair. The overall freedom from pulmonary regurgitation-related reintervention was 99.7%, 99.1%, and 95.8% at 1, 5, and 20 years, respectively.A staged surgical strategy incorporating BTS as the first palliation for symptomatic patients resulted in no mortality. Blalock-Taussig shunt may have contributed to the avoidance of primary transannular patch repair and facilitated pulmonary valve annulus growth; therefore, approximately half of the symptomatic neonates and infants were recruited for valve-sparing repair. Staged repair may have led to functionally reliable delayed transannular patch repair, thereby resulting in fewer surgical reinterventions.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve, and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the right ventricle-to-pulmonary artery conduit as the patient grows. We present a successful complete repair cone tricuspid valvuloplasty in a 9-day-old (2.8 kg) neonate with trisomy 21, pulmonary atresia with intact ventricular septum, and an Ebsteinoid tricuspid valve.

Valve-sparing aortic root replacement (VSRR) is an established treatment for aortic root pathology for trileaflet valves. The safety and durability of VSRR in bicuspid aortopathy is unclear. In this study, outcomes of performing VSRR in the setting of bicuspid and trileaflet valves were compared.An institutional database identified 294 patients who underwent VSRR from 2005 to 2017. Of these, 225 had trileaflet valves and 69 had bicuspid valves. Patients were followed prospectively and had annual postoperative echocardiograms. Propensity-matched comparisons were made between trileaflet and bicuspid valve patients.The average patient age for trileaflet valves was 46.0 ± 13.5 versus 42.7 ± 12.2 years for bicuspid patients (p = 0.07). There was a higher presence of preoperative2+ aortic insufficiency (AI) present in bicuspid patients (63.8%) compared with trileaflet patients (31.1%) (p0.01). Mean follow-up was 39 months and was 98% complete. At 5 years, the cumulative incidence of2+ AI and aortic valve replacement (AVR) was 2.0% and 4.3% in trileaflet patients and 7.7% (p = 0.75) and 7.7% (p = 0.81) in bicuspid patients. Preoperative2+ AI was not predictive of2+ postoperative AI (p = 0.62) nor AVR (p = 0.49). Five-year survival was no different between groups (trileaflet: 98%, bicuspid: 84%, p = 0.24).VSRR can be safely and effectively performed in patients with trileaflet and bicuspid valves. Operative outcomes and valve function were equivalent in bicuspid and trileaflet patients in midterm follow-up. Performance of VSRR is a viable term option in the setting bicuspid aortic valve aortopathy.

We report 3 cases of functional tricuspid regurgitation and demonstrate a novel tricuspid repair technique through the right atrioventricular groove without cardiopulmonary bypass or open heart surgery, which provides a new idea for the treatment of functional tricuspid regurgitation.

Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes.Fontan patients undergoing Fontan revision or transplantation from January 2010 to June 2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and composite mortality and major morbidity.A total of 1135 patients underwent Fontan revision (n = 598) or transplantation (n = 537) at 100 centers. Transplantations increased from 34 in 2010 to 76 in 2017, largely owing to an increase in patients with hypoplastic left heart syndrome (HLHS) (18 in 2010 to 49 in 2017), while Fontan revision decreased (75 in 2010 to 49 in 2017). Transplantation patients were younger (median 14 years of age vs 18 years of age; P.001), more often had preoperative risk factors (66% vs 40%; P.001), and more often had HLHS (51% vs 15%; P.001). Operative mortality and composite major morbidity and mortality were 7.6% and 35% for transplantation and 7.1% and 22% for Fontan revision, respectively. Multivariable risk factors for mortality included older age (odds ratio [OR], 1.08/y; P = .007), presence of preoperative risk factors (OR, 3.33; P = .002), and concomitant pulmonary artery reconstruction (OR, 2.7; P = .029) for Fontan revision but only older age (OR, 1.06/y; P = .020) for transplantation.Both transplantation and Fontan revision are associated with high morbidity and mortality. There has been evolution of practices in surgical therapy for Fontan failure, perhaps related to rising prevalence of HLHS staged palliation.

Background In this study we sought to verify the existence of primary cardiac impairment in patients with Marfan syndrome (MFS) undergoing a Bentall procedure and to explore its clinical significance. Methods Between October 2010 and April 2019, 164 patients with MFS and 748 patients without MFS with aortic root aneurysms underwent a Bentall procedure. Clinical characteristics and perioperative cardiac structure and function between both groups were compared after propensity score matching. Multivariate regression analysis was performed to identify factors that influenced the postoperative left ventricular ejection fraction and any adverse cardiogenic events. The midterm survival between these 2 groups were compared. Results After propensity score matching both groups had similar preoperative baseline characteristics. The MFS patients presented a higher preoperative New York Heart Association classification, larger left ventricular size, and poorer postoperative cardiac function compared with non-MFS patients. In our multivariate regression analysis MFS was associated with a lower postoperative left ventricular ejection fraction. Furthermore MFS was an independent risk factor for adverse postoperative cardiogenic events during patients’ hospital stay (odds ratio, 6.243; 95% confidence interval, 1.71-22.75; P = .006). There was no difference in midterm survival between the 2 groups (P = .772). Conclusions Clinical manifestations observed in MFS patients supported the existence of primary cardiac impairment. Because of a higher rate of postoperative cardiogenic adverse events in MFS patients, the potential impact of primary cardiac impairment in MFS should be carefully considered in cardiovascular surgery.

BACKGROUND Vertical-right-axillary-mini-thoracotomy (VRAMT) is the standard approach for correction of atrial septal defect (ASD) and partial atrioventricular septal defects (PAVSD) at our institution. This observational single-centre study compares our initial results with the VRAMT approach for the repair of ventricular septal defects (VSD) and complete atrioventricular septal defects (CAVSD) in infants and children to an approach using standard median sternotomy (MS). METHODS Perioperative course of patients undergoing VSD and CAVSD correction through either a VRAMT or a MS were analysed retrospectively. The surgical technique for the VRAMT involved a 4-5 cm vertical incision in the right axillary fold. RESULTS Of 84 patients, 25 patients (VSD, n=15; CAVSD, n=10) underwent correction through a VRAMT approach, whereas 59 patients (VSD, n=35; CAVSD, n=24) had repair through MS. VSD and CAVSD-groups were comparable with respect to age and weight. No significant differences were observed for aortic cross-clamp duration, intensive care unit stay, hospital stay and echocardiographic follow-up. There was no need for any conversion from VRAMT to MS in any case. No wound infection nor thoracic deformities were observed in both groups. CONCLUSIONS VRAMT can be considered as a safe and effective approach for the repair of VSD and CAVSD in selected patient groups, and the outcome data appears comparable to those of median sternotomy.

Background Different techniques have been used for exposure of ventricular septal defect (VSD) margins when there is crowding of the VSD anatomy by tricuspid valve subvalvar apparatus. This study compared surgical outcomes for the two techniques of tricuspid valve leaflet detachment and the rarely described tricuspid valve chordal detachment for hard-to-expose VSDs. Methods Patients undergoing transatrial VSD repair were identified from our institutional database. Follow-up echocardiography and patient data were obtained from medical records. Between January 2005 and August 2016, 130 isolated conoventricular VSDs were repaired. Among these, 26 patients underwent leaflet detachment, 15 underwent chordal detachment, and 89 underwent regular VSD repair (reference group). Results The groups did not differ significantly in age, weight, postoperative length of stay, genetic/syndromic abnormalities, time to extubation, and left and right ventricular systolic function. The cardiopulmonary bypass and cross-clamp time were significantly longer in the leaflet detachment group than in the reference group (118 ± 28 vs 102 ± 32 minutes [p = 0.02] and 73 ± 20 vs 61 ± 23 minutes [p = 0.01], respectively). Echocardiographic follow-up was available for 87 patients at a mean of 2.6 years (range, 1 month to 11 years). Tricuspid regurgitation was rated as none or trivial in 66 (76%), mild in 20 (23%), and moderate in 1 reference group patient. There was no difference in presence of residual VSD or degree of tricuspid regurgitation among the three groups. There was no reoperation for tricuspid regurgitation. Conclusions Tricuspid valve leaflet and chordal detachment techniques provide an equally viable and safe alternative to closure of hard-to-expose VSDs while maintaining appropriate tricuspid valve function. Their use in our series did not lead to increased tricuspid valve dysfunction at early-to-midterm echocardiographic assessment.

Background This study summarizes the clinical experience and 8-year follow-up results of the intraoperative device closure of ventricular septal defects (VSD). Methods From January 2009 to June 2017, 1,090 patients with isolated VSD were enrolled at our institute to participate in the study. The procedure involved a small lower sternal incision that was performed after full evaluation of VSD by transesophageal/transthoracic echocardiographic (TEE/TTE) guidance and the deployment of a domestically made occluder to close the VSD periventricularly and off cardiopulmonary bypass. Follow-up was in the first 3 months and then every 1 to 2 years with TTE, electrocardiography, and upon symptoms. Results In all, 1,033 patients were occluded successfully under this approach, and the remaining patients were converted to surgical repair. The size of the occluder deployed ranged from 6 to 14 mm and included 825 symmetric devices and 208 asymmetric devices. A total of 25 patients developed severe arrhythmias, including 11 cases of complete atrioventricular block and 14 cases of Mobitz type II atrioventricular block, during and after the procedure. Conclusions Minimally invasive transthoracic device closure of VSD with an asymmetric or symmetric domestically made device on a beating heart is a safe and feasible alternative to conventional surgical repair. More experience in multicenter and long-term follow-up is necessary to assess the actual feasibility and safety of this procedure.

We sought to evaluate the incidence of Fontan failure or complication and its relation to death in patients having contemporary Fontan strategies over 2 decades.Five hundred patients who underwent Fontan completion (extracardiac, n = 326; lateral tunnel, n = 174) from 1985 to 2012 were reviewed. Patient characteristics, modes of Fontan failure/complication and death, and predictors for Fontan failure/complication and death were analyzed.There were 23 early deaths (4.6%) and 17 late deaths (3.4%), with no early death since 2000. Survival has improved over time (p0.001). Twenty-three of 40 patients who died were identified as Fontan failure before death, including ventricular dysfunction (n = 14), pulmonary vascular dysfunction (n = 4), thromboembolism (n = 2), and arrhythmia (n = 4). Mode of death was circulatory failure (n = 18), multiorgan failure (n = 6), pulmonary failure (n = 3), cerebral/renal (n = 5), and sudden death (n = 4). Modes of failure/complication were directly (65%) or conceivably (10%) related to death in 30 of 40 patients (75%). Forty-eight percent of survivors had late Fontan complication(s). Five-year freedom from late Fontan complication was lower among patients who died compared with patients who survived (29.4% versus 53.3%, p0.001). Ventricular dysfunction (p = 0.001) and higher pulmonary artery pressures (p0.001) after Fontan were predictors for death. Longer cardiopulmonary bypass time (p = 0.032) and reinterventions (p0.001) were predictors for late Fontan complication.Early death in the early era has been overcome. Yet the incidence and causes of late death remain unchanged. There was a strong causative relationship between the mode of Fontan failure/complication and death, indicating the importance of early recognition and treatment of Fontan failure/complication.

Background Patients with dextro (D)-transposition of the great arteries (TGA) who have undergone a previous atrial switch and for some patients with levo (L)-TGA (ie, no ventricular septal defect or outflow tract obstruction), the left ventricle (LV) may require retraining before late arterial switch. The purpose of this study was to analyze the results of LV retraining for these two entities. Methods This was a retrospective review of 51 patients enrolled in an LV retraining program. There were 25 patients with D-TGA and 26 with L-TGA. The median age of the D-TGA patients was 15 years, and 22 of 25 were in New York Heart Association class III or IV. The median age of the L-TGA patients was 12 months, and 3 of 26 patients were in New York Heart Association class III or IV. Results LV retraining was successful in 13 of the 25 patients (52%) with D-TGA, and 10 these 13 patients (77%) underwent successful late arterial switch. Of the 26 patients with L-TGA, LV retraining was successful in 24 (92%), and a double-switch operation was successful in 19 of 19 (100%) who subsequently underwent that procedure. A mathematical formula based on the incremental gain in left-to-right ventricular pressure ratio correctly predicted the success or failure in 94% of the patients. Conclusions The data demonstrate differences in the success of LV retraining and late arterial switch for D-TGA and L-TGA. We would propose that the LV retraining ratio may be useful in objectively selecting patients eligible for late arterial switch.

Neurodevelopmental impairments have frequently been described in children and adolescents with dextro-transposition of the great arteries (d-TGA). The arterial switch operation (ASO) to correct d-TGA has been used for more than 30 years, and more than 90% of these patients now reach adulthood. However, very little is known about their long-term functional outcomes. The present study investigated neurocognitive outcomes and the prevalence of psychiatric disorders in adults with d-TGA corrected by ASO.Neurocognitive functioning was comprehensively assessed (general intellectual functioning, language, attention, visual-spatial skills, executive functions, memory) in 67 adults (59.7% men) with d-TGA (aged 22.9 ± 3.4 years) and in 43 healthy individuals. The prevalence of psychiatric disorders, including depression and anxiety, was evaluated using a structured diagnostic interview. We also analyzed patient- and operative-related risk factors associated with outcomes.Compared with the general population and the control group, adults with d-TGA displayed reduced performance in tasks assessing attention, visual-spatial skills, executive functions, and memory (all p0.05). Compared with controls, patients had also a higher lifetime prevalence of depression (43% vs 19%, p = 0.008) and anxiety disorders (54% vs 33%, p = 0.025). Predictors of long-term outcomes included gender and parental socioeconomic and educational status (all p0.05).Adults who have undergone a neonatal ASO to correct d-TGA have an increased risk of cognitive deficits and psychiatric disorders. Evaluation of long-term neuropsychological and psychosocial outcomes in early adulthood is a crucial step to anticipate for adapted treatment strategies in adults with congenital heart disease.

Kartagener's syndrome is a rare genetic disorder of ciliated epithelial cells associated with recurrent respiratory tract infections, bronchiectasis, and situs inversus. In some patients, the accumulation of airway secretions and recurrent infections lead to end-stage lung disease, for which lung transplantation is the only effective treatment. Anatomical variations, such as dextrocardia and pulmonary situs inversus, make the procedure challenging, yet feasible with certain technical modifications and careful preparation of donor lungs. We report a case of bilateral lung transplantation without the use of cardiopulmonary bypass in a patient with Kartagener's syndrome while describing important technical details of the operation.

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. Although rarely performed now, patients with this physiology often face severe complications requiring reintervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present 2 successful cases to illustrate this idea.

Background Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. Methods Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after. Mean follow-up time was 10.9 ± 3.1 years. Measurements included interventricular septum thickness and ventricular length. The LV/RV length ratio at birth was calculated, and its impact on later LV and septal growth was examined. The primary endpoint was a composite of death, transplantation, or Fontan takedown. Results A cutoff LV/RV length ratio of 0.55 was identified: length ratio was 0.55 or less in group A (18 patients) and more than 0.55 in group B (12 patients) The LV/RV length ratio and interventricular septum thickness decreased over time in group A while remaining static in group B. The LV length at birth did not affect late adverse outcomes (hazard ratio 2.7, 95% confidence interval: 0.31 to 23.4, p = 0.37), whereas aortic atresia and mitral atresia were the most potent predictors of death or transplantation or takedown (hazard ratio 8.5, 95% confidence interval: 1.2 to 57.7, p = 0.029). Conclusions Patients with aortic atresia and mitral atresia have worse outcomes even after Fontan independently of a small, thick, hypertrophied LV. The most severely hypoplastic LVs do not grow proportionally as much as the RVs.

Background Fontan conversion from a classic Fontan operation such as atriopulmonary connection to total cavopulmonary connection with antiarrhythmia surgery is currently not indicated for patients without any late Fontan complications. Methods Thirty-two consecutive patients who underwent Fontan conversion between 1991 and 2012 were divided into 2 groups by the presence (group 1: n = 25, atrial tachyarrhythmia [AT] in 24 and protein-losing enteropathy in 4) or absence (group 2: n = 7) of late Fontan complications, and the surgical outcomes were retrospectively compared. During the study period, heart transplantation was not indicated for patients with failed Fontan circulation in Japan. Results The mean follow-up period was 6.2 ± 3.7 years in group 1 and 4.6 ± 3.8 years in group 2 ( p = 0.29). Overall survival rate at 10 years after conversion was 71% in group 1 and 100% in group 2 ( p = 0.12). Whereas preoperative AT and protein-losing enteropathy remained after conversion in 8 patients (33%) and all 4 patients (100%), respectively, in group 1, neither were observed in group 2. Cardiac catheter examinations presurgery (n = 32) at 1 year (n = 28), and at 5 years (n = 19) after the conversion showed that the cardiac index significantly and similarly improved in both groups after the conversion, and maintained for at least 5 years. Conclusions Cardiac output similarly improved after Fontan conversion in patients with or without late Fontan complications by elimination of venous blood congestion on Fontan pathways. Although long-term follow-up is mandatory, newly onset AT was not observed after prophylactic Fontan conversion.

The use of the left ventricle as the subpulmonary ventricle to achieve a 1.5 or biventricular circulation is feasible in heterotaxy patients with complex intracardiac anatomy and acceptable right ventricular function. It is an alternative in patients who are not ideal candidates for single-ventricle palliation. We highlight 2 cases in which patients were rescued from a failed Fontan palliation and demonstrated improved functional status with normal saturations.

Truncus arteriosus with interrupted aortic arch represents a complex lesion with high rates of reintervention. We present a novel technique of a composite flap, made possible by aberrant right subclavian anatomy.

Approximately half of all tricuspid valves consist of four or more leaflets; however, no previous reports have discussed how to implant an annuloplasty ring in these multi-leaflet valves. We herein define the shoulder point of the tricuspid annulus and the annuloplasty ring and advocate a simple and universal fitting method to avoid deforming the tricuspid valve after ring implantation.

Transcatheter edge-to-edge tricuspid valve repair is emerging as an alternative therapeutic option in symptomatic patients with severe tricuspid regurgitation deemed unfit for open surgery. However, the presence of a dehiscent ring usually prevents transcatheter tricuspid valve intervention. Herein, we report a case of torrential tricuspid regurgitation secondary to ring dehiscence successfully treated with a MitraClip.

Heterotaxy is considered a risk factor for poor outcomes after the Fontan operation. However, long-term data to support this notion are lacking. The aims of this study were to ascertain the long-term outcomes of patients with heterotaxy after hospital discharge after Fontan completion and to compare these outcomes with those of a contemporary nonheterotaxy cohort.A binational Fontan registry (n = 1540) was analyzed to identify patients with heterotaxy and compare them with patients without heterotaxy. The primary composite end point was Fontan failure, encompassing death, heart transplantation, Fontan takedown or conversion, protein-losing enteropathy, plastic bronchitis, or New York Heart Association functional class III or IV.A total of 109 patients with heterotaxy were identified and they were compared with 1431 nonheterotaxy patients after Fontan completion. There was no difference in unadjusted 15-year freedom from Fontan failure (heterotaxy, 78% vs nonheterotaxy, 85%; P = .2). Patients in the heterotaxy group had a significantly higher cumulative incidence of post-Fontan arrhythmias (P.001). Propensity-score matching for confounders yielded 73 patients with heterotaxy matched with 439 patients without heterotaxy, in whom 15-year freedom from Fontan failure was also not different (heterotaxy, 76% vs nonheterotaxy, 81%; P = .2). There was no difference in 15-year freedom from Fontan failure in patients with right vs left isomerism (right isomerism, 80% vs left isomerism, 76%; P = .7).Although heterotaxy may complicate the pre-Fontan course, once the Fontan procedure is successfully completed, heterotaxy does not appear to be an important risk factor for Fontan failure. Patients with heterotaxy are at a higher risk of post-Fontan arrhythmias compared with patients without heterotaxy.