Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has ...been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.
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.
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.
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.
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).
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.
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).
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.
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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.
The aim of this study was to investigate the early and late outcomes of patients undergoing pulmonary embolectomy for acute massive pulmonary embolus.
Twenty-one patients (15 male, 6 female) ...underwent pulmonary embolectomy at our institution between March 2001 and July 2010. The median age was 55 years (range, 24 to 70 years). Of these, 9 patients presented with out-of-hospital cardiac arrest and 8 presented with New York Heart Association class III or IV. Sixteen patients underwent preoperative transthoracic echocardiography, which showed evidence of right ventricular dilatation in all, whereas in 14 patients (66.6%) pulmonary artery pressures were significantly elevated with moderate to severe tricuspid regurgitation. The median preoperative Euroscore was 9 (range, 3 to 16), and 11 patients (52.1%) received systemic thrombolysis preoperatively. There were 6 salvage (28.5%), 10 emergency (47.6%), and 5 urgent (23.8%) procedures. Concomitant procedures were performed in 3 patients (14.2%), and surgery was performed without the use of cardiopulmonary bypass in 3 patients (14.2%). The median follow-up was 38 months (range, 0 to 114 months).
The in-hospital mortality was 19% (n = 4). Postoperative complications included stroke (n = 3, 14.2%), lower respiratory tract infection (n = 6, 28.5%), wound infection (n = 3, 14.2%), acute renal failure requiring hemofiltration (n = 4, 19%), and supraventricular tachyarrhythmias (n = 4, 19%). At discharge, transthoracic echocardiography showed mild to moderate right ventricular dysfunction and dilatation in 11 survivors (64.7%). Two patients died during follow-up, and actuarial survival at 5 years was 76.9% ± 10.1% and at 8 years was 51.2% ± 22.0%. At final follow-up, 11 of the 15 survivors (73.3%) were New York Heart Association class I, and no patients required further intervention.
Patients who undergo surgery for massive pulmonary embolism have an acceptable outcome despite being high-risk.
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.
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.
Sudden cardiac death (SCD) risk can be managed by implantable cardioverter defibrillators (ICD). Defibrillation shocks can be delivered via ICD generator and/or intracardiac or subcutaneous coil ...configurations. We present our single-centre use of childhood ICDs.
Twenty-three patients had ICD implantation, with median age and weight of 12.96 years and 41.35 kg. Indications included eight long QT; four hypertrophic cardiomyopathy; three Brugada syndrome; two idiopathic ventricular fibrillation; two post-congenital heart repair; two family history of SCD with abnormal repolarization; one catecholaminergic polymorphic ventricular tachycardia; and one left ventricle non-compaction. Twelve had out of hospital cardiac arrests prior to implantation. Techniques included 13 conventional ICD implants (pre-pectoral device with endocardial leads), 7 with subcutaneous defibrillation coils (sensing via epicardial or endocardial leads tunnelled to the ICD), and 3 with exclusive subcutaneous ICD (sensing and defibrillation via the same subcutaneous lead). Satisfactory defibrillation efficacy and ventricular arrhythmia sensing was confirmed at implantation. Follow-up ranged from 0.17 to 11.08 years. One child died with the ICD in situ. Ten children received appropriate shocks; five on more than one occasion. Five received inappropriate shocks (for inappropriate recognition of sinus tachycardia or supraventricular tachycardia). Five children underwent six further interventions; all had intracardiac leads.
Innovative shock delivery systems can be used in children requiring an ICD. The insertion technique and device used need to accommodate the age and weight of the child, and concomitant need for pacing therapy. We have demonstrated effective defibrillation with shocks delivered via configurations employing subcutaneous coils in children.