Echocardiographic evaluation of left atrial volume and comparative analysis to left atrial to aortic root ratio in premature neonates and infants with patent ductus arteriosus.

PURPOSE: Left atrium to aortic root ratio (LA/Ao) is an echocardiographic marker of hemodynamically significant patent ductus arteriosus (PDA). Since 2-dimensional measurement of the ratio is geometrically limited, left atrial volume (LAV) which has 3-dimensional characteristics was investigated. The aim of this study was to determine a correlation between LA/Ao ratio and LAV as well as holodiastolic flow reversal in preterm neonates with and without a PDA. METHODS: A retrospective evaluation of neonates with and without PDA was performed. Targeted neonatal echocardiography evaluation of LA/Ao and LAV was measured from parasternal long-axis view and the apical 4 and 2-chamber views, respectively. Univariate and linear regression analysis were performed. RESULTS: 200 patients were included of whom 158 (79.0%) had a PDA shunt. The median gestational age at the time of echo was 27.4 weeks (IQR: 25.7-29.4 weeks). The median LA/Ao ratio was 1.51 (IQR: 1.26-1.83) and median LAV indexed to weight was  .91 mL/kg (IQR: .65-1.18 mL/kg). There was a significant correlation between LA/Ao and LAV indexed to weight in the PDA group (r2 = .080, p = .0003). LA/Ao ratio and LAV indexed to weight differed significantly between those with diastolic flow reversal versus no-flow reversal (LA/Ao, p = .003; LAV, p = .001). CONCLUSIONS: This study demonstrated a significant correlation between LA/Ao and LAV in preterm infants with PDA, with greater magnitude of discordance for LAV. The power of LAV versus LA/Ao in monitoring hemodynamically significant PDA requires prospective evaluation.

Pouch Transfer for Single Coronary Artery With Nodal Artery Variant in Arterial Switch Operation.

The arterial switch operation with single coronary artery variance is an independent risk factor for increased operative mortality. There are reports of technical modifications, such as the double-barreled sinus pouch configuration, to improve geometric reimplantation of the single coronary into the neoaortic sinus. We describe the novel application of this technique for transferring a single coronary artery with a separate nodal artery emanating from the opposite sinus during an arterial switch operation.

Palliative Procedures for Congenital Heart Disease: Imaging Findings and Complications.

Palliative procedures are performed for congenital heart diseases that are not amenable for definitive surgical procedures or as a component of hybrid procedures along with transcatheter interventions. Multimodality imaging plays an important role in the follow-up of these palliative procedures, mainly for the timely detection of complications and for planning any subsequent palliative or definitive procedure. Echocardiography is the first-line imaging modality, with CT and MRI used as complementary techniques in indeterminate cases. MRI provides anatomic, functional, flow, and tissue characterization information. CT is performed for the evaluation of vascular anatomy and when MRI cannot be performed due to contraindications, challenges, or artifacts. The modified Blalock-Taussig shunt procedure is the most common systemic-pulmonary artery (PA) shunt procedure, with thrombus being the most serious complication. Other complications of systemic-PA shunts include shunt stenosis, infection, pulmonary overcirculation, and cardiac failure. The Glenn shunt procedure is the second stage of palliation in single ventricle physiology, with thrombus, stenosis, superior vena cava syndrome, and infection being the common complications. The Fontan shunt procedure is the third stage of palliation in single ventricle physiology. Complications can be cardiovascular (heart failure, valve regurgitation, thromboembolism, shunt stenosis, arteriovenous malformation), venolymphatic (collaterals, protein-losing enteropathy, plastic bronchitis), or hepatic (congestion, cirrhosis, portal hypertension). PA banding is used to decrease pulmonary flow or to train the systemic ventricle. Complications include stenosis, thrombus, erosion, pseudoaneurysm, and subaortic obstruction. Atrial septostomy and atrial switch procedures are performed for increasing intracardiac mixing. Complications of atrial septostomy can be mechanical, traumatic, embolic, or electrical. Complications of the atrial switch procedure include baffle stenosis, baffle leak, and systemic ventricle failure. The authors review the role of multimodality imaging in the evaluation of these palliative procedures. © RSNA, 2023 See the invited commentary by Bardo and Popescu in this issue. Quiz questions for this article are available through the Online Learning Center.

Two-staged surgical repair of Berry syndrome type 2B.

Berry syndrome is a rare congenital heart disease that requires complete corrective surgery. In certain extreme cases, such as ours, a two-stage as opposed to single-stage repair is a possibility. In doing so, we also used annotated and segmented three-dimensional models for the first time in Berry syndrome, adding to growing evidence that such models enhance the understanding of complex anatomy for surgical planning.

Accuracy of Cardiac Magnetic Resonance Imaging in Diagnosing Pediatric Cardiac Masses: A Multicenter Study.

BACKGROUND: After diagnosis of a cardiac mass, clinicians must weigh the benefits and risks of ascertaining a tissue diagnosis. Limited data are available on the accuracy of previously developed noninvasive pediatric cardiac magnetic resonance (CMR)-based diagnostic criteria. OBJECTIVES: The goals of this study were to: 1) evaluate the CMR characteristics of pediatric cardiac masses from a large international cohort; 2) test the accuracy of previously developed CMR-based diagnostic criteria; and 3) expand diagnostic criteria using new information. METHODS: CMR studies (children 0-18 years of age) with confirmatory histological and/or genetic diagnosis were analyzed by 2 reviewers, without knowledge of prior diagnosis. Diagnostic accuracy was graded as: 1) single correct diagnosis; 2) correct diagnosis among a differential; or 3) incorrect diagnosis. RESULTS: Of 213 cases, 174 (82%) had diagnoses that were represented in the previously published diagnostic criteria. In 70% of 174 cases, both reviewers achieved a single correct diagnosis (94% of fibromas, 71% of rhabdomyomas, and 50% of myxomas). When ≤2 differential diagnoses were included, both reviewers reached a correct diagnosis in 86% of cases. Of 29 malignant tumors, both reviewers indicated malignancy as a single diagnosis in 52% of cases. Including ≤2 differential diagnoses, both reviewers indicated malignancy in 83% of cases. Of 6 CMR sequences examined, acquisition of first-pass perfusion and late gadolinium enhancement were independently associated with a higher likelihood of a single correct diagnosis. CONCLUSIONS: CMR of cardiac masses in children leads to an accurate diagnosis in most cases. A comprehensive imaging protocol is associated with higher diagnostic accuracy.

Anomalous Left Anterior Descending Coronary Artery With Retroaortic Left Circumflex Artery: A Novel Coronary Pattern.

A novel coronary anatomy in the form of anomalous left anterior descending coronary artery from pulmonary artery with a retroaortic left circumflex arising from the right coronary artery is presented. This unreported anatomy was discovered in a 7-month-old girl with failure to thrive. (Level of Difficulty: Intermediate.).

Patient-Specific Patch for an Intra-Atrial Rerouting Procedure Developed Through Surgical Simulation.

BACKGROUND: In pediatric cardiac surgery, an application of three-dimensional (3D) modeling to develop custom-made prostheses is limited, and currently surgeons use their intraoperative visual estimation to develop 3D complex structures from 2D patch materials. Contemporary 3D designers are developing complex surfaces using surface modeling in other industries, which can be applied to pediatric cardiac surgery. However, its free-form nature may lead to intradesigner variability. METHODS: A patient with a body weight of 4 kg with partial anomalous pulmonary venous connection and preoperative computed tomography data was selected, and a patient-specific 3D heart model was obtained. Through collaboration with a pediatric cardiologist and a pediatric cardiac surgeon, a 3D designer developed two patient-specific 3D patches for an intra-atrial rerouting procedure (IAR) for the patient using different methods of surface modeling. The shape and size of two flattened patches were analyzed using a geometric morphometrics (GM) approach. Computational fluid dynamics (CFD) analysis was also performed to calculate pressure drop across streamlines and flow energy loss in the right atrium for both patches. RESULTS: The GM analysis showed that the size and shape of the two patches around the systemic vein orifice, crucial to prevent systemic venous obstruction, were almost equivalent. However, the CFD analysis showed that the pressure drop and flow energy loss were almost twice for one patch compared with the other. CONCLUSIONS: Our platform of developing a patient-specific 3D patch for an IAR procedure using surface modeling seemed promising, although intradesigner patch variability was not neglectable in our small-sized patient.

Multimodality Imaging of Transposition of the Great Arteries.

Transposition of the great arteries (TGA) is a congenital conotruncal abnormality characterized by discordant connections between the ventricles and great arteries, with the aorta originating from the right ventricle (RV), and the pulmonary artery (PA) originating from the left ventricle (LV). The two main types of TGA are complete transposition or dextro-transposition of the great arteries (D-TGA), commonly referred to as d-loop, and congenitally corrected transposition (CCTGA), commonly referred to as l-loop or L-TGA. In D-TGA, the connections between the ventricles and atria are concordant, whereas in CCTGA they are discordant, with the left atrium connected to the RV, and the right atrium connected to the LV. D-TGA manifests during the neonatal period and can be surgically managed by atrial switch operation (AtrSO), arterial switch operation (ASO), Rastelli procedure, or Nikaidoh procedure. Arrhythmia, systemic ventricular dysfunction, baffle stenosis, and baffle leak are the common complications of AtrSO, whereas supravalvular pulmonary or branch PA stenosis, neoaortic dilatation, and coronary artery narrowing are the common complications of ASO. CCTGA may manifest late in life, even in adulthood. Surgeries for associated lesions such as tricuspid regurgitation, subpulmonic stenosis, and ventricular septal defect may be performed. A double-switch operation that includes both the atrial and arterial switch operations constitutes anatomic correction for CCTGA. Imaging plays an important role in the evaluation of TGA, both before and after surgery, for helping define the anatomy, quantify hemodynamics, and evaluate complications. Transthoracic echocardiography is the first-line imaging modality for presurgical planning in children with TGA. MRI provides comprehensive morphologic and functional information, particularly in adults after surgery. CT is performed when MRI is contraindicated or expected to generate artifacts. The authors review the imaging appearances of TGA, with a focus on pre- and postsurgical imaging. Online supplemental material is available for this article. ©RSNA, 2021.

Evaluation of Transcatheter Pulmonary Valve Endocarditis by Dual-Energy Computed Tomography.

Transcatheter pulmonary valve implantation (TPVI) is now an established alternative to surgery in patients with congenital heart disease and dysfunctional right ventricular outflow tract (RVOT) conduit. However, there is recognition of a higher incidence of infective endocarditis in the patients after TPVI. Transthoracic and transesophageal echocardiography is limited in the evaluation of prosthetic pulmonary valve endocarditis secondary to a metallic artifact and degenerative calcified conduit. Additionally, the anterior position of RVOT also limits evaluation by echocardiography. Conventional single-energy CTA can also be sub-optimal in evaluating prosthetic pulmonary valve stent frame due to streak artifacts from the metallic cage and poor contrast to noise ratio if higher kV is used for single-energy CTA to avoid metallic artifacts. Dual-energy CTA can overcome these limitations using reconstructed virtual monoenergetic and iodine-only images for metal artifact reduction and improve intra-stent luminal visualization. Reconstructed iodine perfusion maps may also help differentiate vegetation from a thrombus. In this case report, we discuss the diagnostic utility of dual-energy cardiac CT in the evaluation of endocarditis after TPVI and discuss the imaging protocol.

Novel Application of Geometric Morphometrics to Cardiac Surgical Simulation.

There may exist intersurgeon and intrasurgeon variability in the shape and size of patches developed during intracardiac rerouting procedures, especially among junior surgeons. In this report geometric morphometrics, approaches of shape analysis developed in biology, is applied to analyze patch shapes created by surgeons through surgical simulation for an intraatrial rerouting procedure. Geometric morphometrics can be a useful toll in the armamentarium of pediatric cardiac surgery in which shape matters.

Truncus arteriosus with retrograde aortic flow: a unique case.

Truncus arteriosus is a rare cyanotic congenital heart defect that involves septation failure of the heart's main arterial outflow tract. Varying morphologies of the truncal valve and aorta have been reported; however, the ascending aorta is typically supplied via anterograde blood flow through the truncal valve. We present the first reported case of neonatal truncus arteriosus with the ascending aorta being supplied entirely by retrograde flow.

Venous Myocardial Infarction in an Infant with Obstructed Totally Anomalous Pulmonary Venous Drainage and Coronary Sinus Ostial Atresia.

We report a rare causal association between obstructed supracardiac totally anomalous pulmonary venous drainage and coronary sinus ostial atresia. Our 12-week-old patient developed venous myocardial infarction secondary to coronary venous hypertension because her sole route of coronary venous drainage was obstructed. She recovered after the obstruction was relieved by balloon dilation. Surgical repair then included anastomosis of the pulmonary venous confluence to the left atrium, ligation of the vertical vein, and unroofing of the coronary sinus. Coronary sinus ostial atresia is rarely diagnosed before autopsy.

Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate.

Isolated left subclavian artery is one of the rarer aortic arch anomalies. It has been associated with other congenital heart diseases, typically tetralogy of Fallot, double-outlet right ventricle, and atrial and ventricular septal defects. Its significant clinical implications include a left-to-right shunt from the vertebrobasilar system, which causes pulmonary overcirculation and subclavian steal. We present an unusual case of a premature infant who was diagnosed prenatally with congenital complete atrioventricular block and tricuspid atresia and was found to have an isolated left subclavian artery postnatally. The patient underwent implantation of a permanent single-chamber epicardial pacing system. To our knowledge, this combination of lesions has not been reported-and in our case, it influenced our surgical planning.

Fetal diagnosis of superior vena cava aneurysm.

Superior vena cava aneurysm is a rare intrathoracic vascular lesion with only 27 cases reported in the literature. The majority are fusiform and can be associated with cystic hygroma due to the close embryonic relationship between lymphatic vessels and systemic veins. This is the first report of superior vena cava aneurysm diagnosed with fetal echocardiography in a fetus with a cystic hygroma. There is a need of a prospective registry to further delineate all aspects of this condition and establish the most appropriate therapeutic approach.

Percutaneous stent placement in children weighing less than 10 kilograms.

The objectives of this study were to evaluate the safety, efficacy, and outcomes of palliative percutaneous stent placement in infants with congenital heart disease (CHD). There is interest in improving outcomes of infants with CHD through interventional/surgical collaboration. Small, high-risk patients may benefit from delayed open operations, and endovascular stents may provide a means to defer surgery to more advantageous times in select infants. Patients weighing < or =10 kg in whom stent placement was attempted during 2003-2006 were identified. Diagnoses, indications, angiographic and hemodynamic data, complications, and outcomes were reviewed. Seventeen stents were successfully placed in 15 patients. The mean age was 10.21 months; the mean weight was 6.08 kg. Indications were branch pulmonary artery stenosis, coarctation, total anomalous pulmonary venous return with obstructed venous egress, right ventricle-pulmonary artery conduit, and shunt stenosis. Premounted Palmaz Genesis and Driver Mx stents were used. Average vessel diameter increased from 3.65 to 6.72 mm (p < 0.001). The pressure gradient fell from 24 to 15 mm Hg (p < 0.01). Two stents migrated. One was recaptured and implanted at the target site and one implanted away from the target site. None needed surgical intervention. Mean follow-up was 270 days. Five patients have had additional surgery; seven patients underwent 10 additional stent dilations. There was no procedural-related mortality. Five patients (33%) died during the follow-up period, none attributable to the stent placement. We conclude that stent placement can be successfully accomplished in select, small, high-risk patients. A collaborative interventional/surgical paradigm is important to ensure that the risk-benefit ratio is properly evaluated during the patient selection.