Use of Interactive Visualization and 3D Printing in the Repair of Complex Congenital Heart Disease Presenting in Adult Life.

We report a case of a 25-year-old male with a heterotaxy-like constellation of congenital heart defects consisting of complete atrioventricular septal defect, transposition of the great arteries, subpulmonary stenosis, L-looped ventricles, hypoplastic right ventricle, and a distant aorta arising from the right ventricle. This case demonstrates how 3D printing and interactive 3D visualization may facilitate a unique surgical repair.

Hybrid palliation versus nonhybrid management for a multi-institutional cohort of infants with critical left heart obstruction.

OBJECTIVE: To compare patient characteristics and overall survival for infants with critical left heart obstruction after hybrid palliation (bilateral pulmonary artery banding with or without ductal stenting) versus nonhybrid management (eg, Norwood, primary transplantation, biventricular repair, or transcatheter/surgical aortic valvotomy). METHODS: From 2005 to 2019, 1045 infants in the Congenital Heart Surgeons' Society critical left heart obstruction cohort underwent interventions across 28 institutions. Using a balancing score propensity analysis, 214 infants who underwent hybrid palliation and 831 infants who underwent nonhybrid management were proportionately matched regarding variables significantly associated with mortality and variables noted to significantly differ between groups. Overall survival between the 2 groups was adjusted by applying balancing scores to nonparametric estimates. RESULTS: Compared with the nonhybrid management group, infants who underwent hybrid palliation had lower birth weight, smaller gestational age, and higher prevalence of in-utero interventions, noncardiac comorbidities, preoperative mechanical ventilation, absent interatrial communication, and moderate or severe mitral valve stenosis (all P values < .03). Unadjusted 12-year survival after hybrid palliation and nonhybrid management, was 55% versus 69%, respectively. After matching, 12-year survival after hybrid palliation versus nonhybrid management was 58% versus 63%, respectively (P = .37). Among matched infants born weighing <2.5 kg, 2-year survival after hybrid palliation versus nonhybrid management was 37% versus 51%, respectively (P = .22). CONCLUSIONS: Infants born with critical left heart obstruction who undergo hybrid palliation have more high-risk characteristics and anatomy versus infants who undergo nonhybrid management. Nonetheless, after adjustment, there was no significant difference in 12-year survival after hybrid palliation versus nonhybrid management. Mortality remains high, and hybrid palliation confers no survival advantage, even for lower-birth-weight infants.

Valve-Sparing Aortic Root Replacement in a Patient With an FLNA Variant.

We report a case of a 38-year-old female with an FLNA variant who underwent valve-sparing aortic root replacement. FLNA encodes Filamin A, an actin-binding protein. Our patient had aortic root dilation due to this variant. Aortic root repair was conducted using the David procedure, with modifications to account for tissue fragility associated with this genetic condition. This case demonstrates the value of patient-specific genetic information for the timing of surgery and operative course planning.

Management of 760-g Extremely Premature Infant With Dextro-Transposition of the Great Arteries.

Extremely low birth weight neonates with complex congenital heart disease have increased mortality risk. Multi-organ dysfunction, pulmonary disease, fluctuating pulmonary vascular resistance, and complex cardiovascular anatomy create a challenge for effective management. We present the case of a 760-g neonate with dextro-transposition of the great arteries, ventricular septal defect, patent ductus arteriosus, and single coronary artery with proximal intramural segment of the right coronary artery branch. We describe features of preoperative care, surgical intervention, and postoperative course that enabled this infant to survive.

Three-dimensional printing for surgical planning in complex congenital heart disease.

Surgical management of complex congenital heart disease (CHD) is challenging. Three-dimensional (3D) printing can improve multidisciplinary team decision-making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

Left Ventricular Pseudoaneurysm Following Surgical Repair of Ventricular Septal Defect in an Infant.

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication following cardiac surgery, let alone in the pediatric population. Other known causes of LV-PSA are trauma, percutaneous cardiac intervention, and infections. This report describes the development of LV-PSA following surgical repair of ventricular septal defect (VSD) and coarctation of aorta (CoA) in an infant.

Stenting and Reimplanting Disconnected Pulmonary Artery in Tetralogy of Fallot.

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.

Surgical Intervention in Preterm Neonates with Patent Ductus Arteriosus.

The ductus arteriosus is a fetal vascular connection between the pulmonary and systemic circulations. It fails to close after birth in a small number of term infants, and in a larger number of infants with cyanotic congenital heart disease. In contemporary practice the majority of patients present with a patent ductus arteriosus (PDA) are premature infants before the gestational age of 28 weeks. The surgical management of PDA in preterm infants is critical for optimal outcomes and is discussed in this article.

Biventricular Repair of Pulmonary Atresia After Fontan Palliation.

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

Surgical Treatment of Neonate With Congenital Left Main Coronary Artery Atresia.

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively.

Methylenetetrahydrofolate Reductase C677T: Hypoplastic Left Heart and Thrombosis.

The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase (MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T. We present these cases and highlight the implications of MTHFR mutation in the management of complex congenital heart disease.

Postpartum pulmonary embolism in a patient with Fontan circulation.

Pregnancy is typically not recommended in patients with Fontan circulation. Although patients are well aware of the risks for the mother and fetus, an increasing number opt to become pregnant. The higher rate of survival into adulthood post-Fontan procedure is a likely factor as a result of improved management of single ventricle anatomy. Postpartum thromboembolism is a known complication, but its prevalence and management are not clearly defined. We present a case of massive pulmonary embolism two weeks postpartum in a patient with double inlet left ventricle palliated with lateral tunnel Fontan.

Midterm evaluation of biological prosthetic valves in the pulmonary position of grown-up patients.

OBJECTIVES: To examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves. METHODS: We reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months). RESULTS: There were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively. CONCLUSIONS: PVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

Aortic valve replacement in patients with previous coronary artery bypass grafting: 10-year experience.

UNLABELLED: OBJECTIVES; This study aimed to investigate the early and late outcomes of patients undergoing aortic valve replacement (AVR) with previous coronary artery bypass grafting (CABG) and patent grafts. METHODS: Between January 2000 and March 2010, 104 patients (87 males) with previous CABG ± concomitant surgery and patent grafts underwent AVR. The median age of the patients was 75 years (range: 37-90 years; inter-quartile range: 69-79 years) and the mean logistic EuroScore was 25.37 ± 16.8. The median time since the previous operation was 9 years (range 1-25; inter-quartile range: 7-14 years). The left internal mammary artery (LIMA) had been used in 75 patients (72.1%) and remained patent in 72 cases (96.0%). RESULTS: Thirty-day mortality was 7.7% (n = 8), which is less than the predicted mean logistic EuroScore. Isolated AVR was performed in 66 patients (63.5%). The LIMA was dissected and isolated (clamped or blocked with balloon) in 60 patients. The median hospital stay was 10 days (range: 4-183 days; inter-quartile range: 7-15.25 days). Nineteen patients (18.3%) had pulmonary complications, while 12 (11.5%) had acute kidney injury. Seven patients (6.7%) required permanent pacemaker. Six LIMAs (8.3%) were injured and repaired. Prolonged aortic cross-clamp (AXC) time (P = 0.038) and the presence of a previous LIMA graft (P = 0.045) were identified as independent predictors of 30-day mortality. The actuarial survival at 1 and 5 years was 89.4 ± 0.3 and 81.5 ± 0.5%, respectively. Perioperative intra-aortic balloon pump use (P = 0.036), prolonged AXC time (P = 0.004) and prolonged cardiopulmonary bypass time (P = 0.022) were associated with worse long-term overall survival on multivariate analysis. CONCLUSIONS: AVR post-CABG with patent grafts can be performed in high-risk patients with excellent short- and long-term outcomes and appears to be superior to published catheter-based interventions. In the absence of randomized trial data, we believe that open AVR remains the treatment of choice for aortic valve disease following prior CABG.

Long-term outcomes in octogenarians following aortic valve replacement.

BACKGROUND: The aging of the population has resulted in an increasing number of elderly patients undergoing cardiac operations. We reviewed our experience in patients over the age of 80 undergoing primary aortic valve replacement (AVR) with or without CABG. METHODS: Between 2000 and 2008, 345 patients (226 male) ≥80 years underwent primary AVR in our unit. The notes of these patients were retrospectively reviewed and follow-up information was obtained from their general practitioners. They had a mean age of 82.9 ± 2.3 years and a median logistic EuroSCORE of 13.4 (IQR 9.4, 19.1). Isolated AVR was performed in 161 patients (45.5%), and 184 (51.6%) patients underwent combined AVR and CABG. A quality of life questionnaire was sent to all survivors. RESULTS: Hospital mortality occurred in 17 patients (4.9%), which was significantly lower than the mortality predicted by logistic EuroSCORE (16.2%, p < 0.01). Hospital mortality was comparable between patients undergoing isolated AVR and those undergoing additional CABG (4.3% vs. 5.4%, respectively). Actuarial survival at one and five years was 90.1 ± 1.6% and 77.2 ± 2.9%, respectively. There was a 62% response on the questionnaire showing 70% of the patients were NYHA I and 83.7% were satisfied with the operation outcome. CONCLUSIONS: AVR can be undertaken with excellent results in octogenarians and the current risk is significantly lower than what is predicted with conventional risk-scoring systems. Patients with advanced age should not necessarily be excluded from being candidates for AVR.

Restoring anterior leaflet continuity: the Spinnaker repair of Ebstein's anomaly.

Several repair techniques for Ebstein's anomaly include aggressive mobilization of the anterior leaflet and the construction of a monobicuspid valve coapting against the interventricular septum. This can prove difficult when leaflet tissue is of poor quality or several fenestrations reduce leaflet surface. We present a simple and highly reproducible technique for restoring tissue continuity of the anterior leaflet that could represent a possible addendum to the existing array of surgical solutions. Two surgical cases are described. Both patients showed good valve function and trivial tricuspid regurgitation at short-term follow-up.

Long-term outcome following repair of acute type A aortic dissection after previous cardiac surgery.

We evaluated the outcome after repair for acute spontaneous type A aortic dissection in patients with previous cardiac surgery. From January 2000 to December 2009, 114 patients underwent emergency repair for acute spontaneous type A dissection at Southampton University Hospital. Eleven (median age 64 years; range 36-83 years; two females) patients (9.8%) had undergone previous cardiac surgery and were included in this study. Aortic root replacement was performed in three patients (27%), aortic arch replacement in four patients (36%) and two patients (18%) required aortic valve re-suspension. The elephant trunk operation was performed in two patients (18%). There were two hospital deaths (18%). Two patients (18%) suffered a stroke, two needed re-opening for bleeding (18%) and two patients (18%) required haemofiltration postoperatively. Median length of hospital stay was 16 days (range 6-34 days). Actuarial survival at five and eight years for redo compared to first-time surgery was 68±3.63% vs. 81±5.34% and 51±3.8% vs. 61±5.4%, respectively (P=0.365). In conclusion, acute type A aortic dissection repair in patients with previous cardiac surgery has an acceptable mortality and comparable long-term outcome to first-time surgery.

Sustainable knowledge transfer in pediatric cardiac surgery: a team approach to shared learning.

The study assesses the impact of a program using a model of knowledge transfer on the long-term development of a pediatric cardiac service in Lithuania. A team from the United Kingdom evaluated Lithuanian pediatric cardiac services and provided support in areas targeted for improvement. The total number of infant operations performed in Lithuania from 1990 to 2008, together with in-hospital mortality rates, was broken down into 3 time periods and analyzed to estimate the efficacy of the program: (1) 1990-1998, before the program; (2) 1999-2002, during the program; (3) 2003-2008, following the end of the program. Lithuanian results in children older than 1 year were comparable with other European centers. However, only 315 infant and neonatal cardiac procedures were performed between 1990 and 1998, and there was an in-hospital mortality of 34.2%. Between 1999 and 2002, the UK team performed 23 highly complex demonstration procedures (in-hospital mortality, 13%). During the same period the Lithuanian team performed 305 additional operations in neonates and infants, and in-hospital mortality decreased to 18.7%. From 2003 to 2008 results continued to improve-559 infant operations were performed, with in-hospital mortality of 11.3% (P < .0001). Knowledge transfer has led to substantial and sustainable long-term improvement in the results of infant cardiac surgery in Lithuania. Demonstrating techniques and care on a limited number of more complex cases is an efficient way of transferring knowledge and skills to the developing pediatric cardiac centers.