Purpose: Residual ventricular septal defects (rVSDs) of small size are commonly seen on transesophageal echocardiography after surgical repair. This study aimed to determine the destiny of rVSD found on intraoperative echocardiogram. Methods: Patients undergoing surgical repair of VSD as the primary procedure with available intraoperative and discharge echocardiograms between 2007 and 2017 were reviewed. Presence of an rVSD on intraoperative echo triggered review of discharge echo and of subsequent follow-up echocardiograms. Results: One hundred four patients were analyzed. The mean age and weight for the entire cohort were 1.4 ± 2.9 years (median, 5.4 months; range, 29 days to 14 years) and 8.8 ± 9.9 kg (median, 5.1 kg; range, 2.7-58 kg), respectively. Sixty (57%) patients had rVSD at discharge, with mean size of residual VSD of 1.38 ± 0.92 mm (mode, 0.6; median, 2.2 mm; range, 0.5-3.9 mm). The mean follow-up time was 3.7 ± 3.1 years (range, 1 month to 9.3 years). Among those with rVSD at discharge, a residual shunt persisted in 73% at one-month follow-up. On follow-up at three years postdischarge, of the 60 patients with early rVSD, 6 had a persistent rVSD (10%) with a mean diameter of 3.0 ± 0.8 mm (range, 2.4-3.9 mm). Conclusions: Residual VSD after surgical repair is detected frequently on postoperative echocardiogram. The presence of rVSD was not associated with any preoperative, intraoperative, or postoperative factors. By three years of follow-up, only six patients continued to demonstrate rVSD with a mean diameter of 3 mm, suggesting that defects 3 mm or greater may be less likely to close spontaneously after three years.

This report describes 2 contrasting yet rare associations of hypoplastic left heart syndrome, 1 in a patient with pulmonary valve stenosis that was successfully surgically palliated and the other in a patient with an intact atrial septum and stenotic bilateral levoatriocardinal veins who was offered comfort care. These cases underscore the point that although both infants were born with hypoplastic left heart syndrome, the outcomes can dramatically differ as a result of anatomic and physiological variables. (Level of Difficulty: Advanced.)

We present the case of an infant with left atrial isomerism with complex pulmonary and systemic venous connections that resulted in physiological parallel circulation in the setting of ventriculoarterial concordance who was surgically treated using the Senning procedure. This case highlights a rare cause for cyanosis due to poor mixing from a parallel circulation and an issue with nomenclature. (Level of Difficulty: Advanced.)

OBJECTIVE:To develop quality metrics (QMs) for the ambulatory care of patients with transposition of the great arteries following arterial switch operation (TGA/ASO).DESIGN:Under the auspices of the American College of Cardiology Adult Congenital and Pediatric Cardiology (ACPC) Steering committee, the TGA/ASO team generated candidate QMs related to TGA/ASO ambulatory care. Candidate QMs were submitted to the ACPC Steering Committee and were reviewed for validity and feasibility using individual expert panel member scoring according to the RAND-UCLA methodology. QMs were then made available for review by the entire ACC ACPC during an "open comment period." Final approval of each QM was provided by a vote of the ACC ACPC Council.PATIENTS:Patients with TGA who had undergone an ASO were included. Patients with complex transposition were excluded.RESULTS:Twelve candidate QMs were generated. Seven metrics passed the RAND-UCLA process. Four passed the "open comment period" and were ultimately approved by the Council. These included: (1) at least 1 echocardiogram performed during the first year of life reporting on the function, aortic dimension, degree of neoaortic valve insufficiency, the patency of the systemic and pulmonary outflows, the patency of the branch pulmonary arteries and coronary arteries, (2) neurodevelopmental (ND) assessment after ASO; (3) lipid profile by age 11 years; and (4) documentation of a transition of care plan to an adult congenital heart disease (CHD) provider by 18 years of age.CONCLUSIONS:Application of the RAND-UCLA methodology and linkage of this methodology to the ACPC approval process led to successful generation of 4 QMs relevant to the care of TGA/ASO pediatric patients in the ambulatory setting. These metrics have now been incorporated into the ACPC Quality Network providing guidance for the care of TGA/ASO patients across 30 CHD centers.

Patients with single ventricle anatomy undergo staged surgical palliation which results in pulmonary and systemic circulations in series with a single systemic pump. Single right ventricular morphology has been found to be an independent risk factor for worse survival. We sought to compare patients with single left (SLV) and single right (SRV) ventricular morphology to identify hemodynamic differences that may contribute to worse survival in patients with a single right ventricular. Single center, retrospective review of invasive hemodynamic data. All patients with single ventricle anatomy who underwent superior cavo-pulmonary anastomosis (Stage 2 palliation) and/or total cavo-pulmonary anastomosis (Stage 3 palliation) from August 1995 through May 2011 were identified. Patients were compared over time, and SLV and SRV patients were compared. Seventy-nine single ventricle patients (56 % SRV) underwent staged palliation and were analyzed. There was no difference in overall mortality (12 % SLV, 11 % SRV). There was no difference in hemodynamics at pre-Stage 2 catheterization between ventricular morphology, but SRV patients had higher ventricular end-diastolic pressure at pre-Stage 3 catheterization (7.6 vs. 6.4 mmHg, p = 0.026). End-diastolic pressure decreased after Stage 2 surgery for SLV patients, but not SRV patients. Intrinsic differences in morphology, function, and response to performing as the systemic ventricle between single right and left ventricles may lead to an elevated ventricular end-diastolic pressure. This could limit passive flow through the pulmonary circuit and coronary perfusion after Stage 3 palliation and potentially lead to poorer long-term performance for SRV patients.

AbstractObjective: Acute kidney injury is common in neonates following surgery for congenital heart disease. We conducted a retrospective analysis to determine whether neonates with smaller pre-operative renal volume were more likely to develop post-operative acute kidney injury. Design/Setting: We conducted a retrospective review of 72 neonates who underwent congenital heart surgery for any lesion other than patent ductus arteriosus at our institution from January 2007 to December 2011. Renal volume was calculated by ultrasound using the prolate ellipsoid formula. The presence and severity of post-operative acute kidney injury was determined both by measuring the peak serum creatinine in the first 7 days post-operatively and by using the Acute Kidney Injury Network scoring system. Results: Using a linear change point model, a threshold renal volume of 17 cm3 was identified. Below this threshold, there was an inverse linear relationship between renal volume and peak post-operative creatinine for all patients (p = 0.036) and the subgroup with a single morphologic right ventricle (p = 0.046). There was a non-significant trend towards more acute kidney injury using Acute Kidney Injury Network criteria in all neonates with renal volume ≤17 cm3 (p = 0.11) and in the subgroup with a single morphologic right ventricle (p = 0.17). Conclusions: Pre-operative renal volume ≤17 cm3 is associated with a higher peak post-operative creatinine and potentially greater risk for post-operative acute kidney injury for neonates undergoing congenital heart surgery. Neonates with a single right ventricle may be at higher risk.

A 3-year-old boy with single-ventricle anatomy and a prior Glenn procedure (anastomosis of the superior vena cava [SVC] to the pulmonary arteries) underwent a fenestrated extracardiac Fontan procedure (connection of the inferior vena cava [IVC] to the pulmonary arteries) with use of an 18-mm Dacron conduit. The patient's immediate postoperative course was uneventful. He developed pleural effusions 4 months after surgery but appeared to be well. His medications were aspirin and furosemide. We performed cardiac catheterization because echocardiography yielded an insufficient view of the conduit, and because we needed to evaluate hemodynamic or structural causes of the pleural effusions. Angiograms showed occlusion of the extracardiac conduit (Fig. 1), a patent superior cavopulmonary anastomosis without thrombus (Fig. 2), and extensive collateral veins from the IVC to the SVC (Fig. 3). Right upper and lower pulmonary vein obstruction was also noted (Fig. 4). Fig. 4 Delayed angiographic image from the superior vena cava injection shown in Figure 2 shows no pulmonary venous return from the right upper pulmonary vein (black arrow) and apparent compression of the right lower pulmonary vein (white arrow). Fig. 3 Delayed angiographic image from the inferior vena cava injection shown in Figure 1 shows extensive decompressing venous collateral vessels connecting the venae cavae. Fig. 2 Angiogram after contrast injection into the superior vena cava shows a patent superior cavopulmonary anastomosis with no flow into the extracardiac Fontan conduit. Fig. 1 Angiogram after contrast injection into the inferior vena cava shows no flow into the extracardiac Fontan conduit. Bilateral pleural pigtail drains are in place. Hypercoagulability studies were negative. At the time of reoperation, the Dacron conduit was noted to be filled with thrombus. The patient's daily antithrombotic regimen was changed to aspirin (81 mg) and warfarin, and there was no recurrence of thrombus.

OBJECTIVESTo identify and predict neo-aortic arch obstruction (NAAO) in children after Norwood/Sano operation (NO) for hypoplastic left heart syndrome (HLHS).BACKGROUNDNAAO is associated with morbidity and mortality after NO for HLHS and no objective measure has predicted the initial occurrence of NAAO. Computational flow models of aortic coarctation demonstrate increased wall shear stress (WSS) in vessels proximal to the coarctation segment, which we believe also occurs with NAAO. These vessels respond by increasing their luminal diameter to maintain normal WSS. We hypothesized that the relative increase in diameters of head and neck vessels to the isthmus, as measured by angiography, would identify hemodynamically significant NAAO and predict future NAAO.METHODSRetrospective review of patients with HLHS and at least one catheterization with aortic angiography after NO. Diameters of head and neck vessels were totaled and divided by the isthmus diameter to give a head and neck index (HNI), which was compared to coarctation index (CI) for identifying and predicting future NAAO.RESULTSForty-four patients were identified, 17 with and 27 without NAAO. Receiver operator characteristic analysis using a value for CI ≤0.5 showed a sensitivity of 47% and specificity of 89%. For HNI, a value >2.65 gave a sensitivity of 77% and specificity of 93%. Three patients who developed NAAO after their initial catheterization had CI >0.5, but abnormally high HNI >2.65.CONCLUSIONSHNI is a more robust indicator of hemodynamically significant NAAO than CI and may predict its future occurrence after NO for HLHS.

Neo-aortic arch obstruction (NAAO) is a common complication following the Norwood/Sano procedure (NP) for hypoplastic left heart syndrome (HLHS) and is associated with increased morbidity and mortality. However, there is currently no objective method for predicting which patients will develop NAAO. This study was designed to test the hypothesis that hemodynamic changes from development of NAAO after NP in patients with HLHS will lead to changes in myocardial dynamics that could be detected before clinical symptoms develop with strain analysis using velocity vector imaging. Patients with HLHS who had at least one cardiac catheterization after NP were identified retrospectively. Strain analysis was performed on all echocardiograms preceding the first catheterization and any subsequent catheterization performed for intervention on NAAO. Twelve patients developed NAAO and 30 patients never developed NAAO. Right ventricular strain was worse in the group that developed NAAO (−6.2 vs. −8.6 %, p = 0.040) at a median of 59 days prior to diagnosis of NAAO. Those patients that developed NAAO following NP were significantly younger at the time of first catheterization than those that did not develop NAAO (92 ± 50 vs. 140 ± 36 days, p = 0.001). This study demonstrates that right ventricular GLS is abnormal in HLHS patients following NP and worsening right ventricular strain may be predictive of the future development of NAAO.

Purpose Dilated cardiomyopathy (DCM) is a common indication for heart transplantation (Tx) in children but not all progress to Tx. Standard 2D echocardiography (2DE) and Doppler techniques do not predict which patients will recover function. Myocardial function can be reliably determined using Velocity Vector Imaging (VVI), an angle independent method of evaluating myocardial mechanics using conventional 2DE images. We believe VVI provides a valid and reproducible measure of myocardial function in DCM patients with correlation to clinical outcomes. Methods and Materials Medical records and 2DEs were retrospectively reviewed on patients with DCM. VVI analysis was performed offline using Siemens Syngo software. Using VVI on a 4 chamber image, longitudinal velocity (LVel), strain (S), strain rate (SR) were obtained. Ventricular functional parameters were compared to normal. Results 22 patients were treated for DCM − 8 patients recovered function, 4 patients underwent Tx, 3 patients were listed for Tx, 7 patients died without recovering or while awaiting Tx. Mortality was associated with mechanical, ventilatory support, and renal failure. The overall mortality rate was 36% (8/28). LVel, S, and SR were decreased compared to normal in all groups. LVel, S, and SR were statistically different in those who progressed to Tx and listed for Tx compared to those who did recover. S and SR did not return to normal even in the group who clinically recovered. Ejection fraction and 2DE dimensions correlated with VVI parameters. Conclusions VVI derived myocardial functional parameters do not return to normal despite clinical recovery and return to normal of conventional 2DE parameters. VVI is an effective tool to predict degree of myocardial recovery and serves as an adjunct to conventional 2DE measures of systolic and diastolic function.

This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.

Changes in right ventricle (RV) loading conditions and pressure alter left ventricle (LV) myocardial mechanics. Pediatric patients with chronic RV pressure load [valvar pulmonary stenosis – PS] or volume load [secundum atrial septal defect – ASD], who underwent catheter intervention

The objective of this study was to determine angiographic predictors of future pulmonary artery stenosis (PS) in patients with hypoplastic left heart syndrome (HLHS) at the time of pre-stage 2 cardiac catheterization (PS2C). The Sano modification of the Norwood operation (NSO) for HLHS includes placement of a right ventricle-to-pulmonary artery (RV-PA) conduit. Branch PS is a recognized complication. Data from patients with HLHS who underwent NSO from 2005 to 2009 and who underwent PS2C were reviewed retrospectively. Nakata and McGoon indices were calculated in the traditional fashion, and modified Nakata and McGoon indices were calculated using the narrowest branch PA diameters. Thirty-three patients underwent NSO and 28 patients underwent PS2C. Mean follow-up was 35.8 ± 7.5 months. Ten (36 %) patients had significant left branch PS, with two requiring balloon angioplasty and eight requiring stent placement, a median of 15.2 months after PS2C (interquartile range 1.2, 32.8). The modified Nakata index was predictive of future intervention for left PS (receiver operating characteristic curve area under the curve 0.811), with a cut-off of 135 mm(2)/m(2) and a sensitivity of 100 % and specificity of 72.2 %. A modified Nakata index <135 mm(2)/m(2) at PS2C predicts future need for intervention on left-branch PS in patients with HLHS after the NSO. Surgical pulmonary arterioplasty at the time of stage 2 surgical palliation may obviate the need for future interventions.

Maternal lupus can be complicated by complete heart block (CHB) and fetal myocarditis. Conventional myocardial quantification methods can be technically challenging in fetuses. Velocity Vector Imaging (VVI) is an angle independent method of determining myocardial mechanics. The objective was to determine myocardial function using VVI prenatally (PrN), postnatally (PN) and following pacemaker placement (PMP) in infants born to mothers with lupus. Analysis was done offline using Syngo VVI software (Siemens, California) in the 4 chamber view. Longitudinal velocity, strain, strain rate, longitudinal displacement and ejection fraction, were determined in both ventricles of 5 fetuses with a history of maternal lupus and 1 with L-transposition (L-TGA) and CHB. 4 fetuses had CHB and 2 did not but had siblings with CHB. Similar parameters were determined PN and following PMP. These were compared to normal fetuses and infants. Similar to normal fetuses, all parameters were maximal at the ventricular bases and least at the apices. Quantitatively, all were significantly decreased compared to normals. All parameters improved PN. Following PMP, parameters decreased, but were higher than PrN. In opposition to the patient with L-TGA/CHB, in all other patients free wall indices of both ventricles were dominant compared to the interventricular septum. The 2 patients without CHB had globally decreased parameters that improved PN although lower than normals. Post-processing took approximately 4 + 2 minutes. Myocardial dysfunction is evident in all fetuses and infants with a history of maternal lupus even in the absence of CHB. This implies that normal function using conventional methods does not exclude myocarditis. There is evidence that PMP exacerbates dysfunction. The differences in denervation patterns between L-TGA and normal hearts are demonstrated using this technique. VVI is a practical modality of evaluating myocardial mechanics.

Fetal function has been difficult to quantify due to issues related to angle dependency, excessive fetal movement and less than perfect windows. Our goal was to evaluate the feasibility of using 2D Velocity Vector Imaging (VVI) to determine right ventricular myocardial performance in the setting of hypoplastic left heart syndrome and compare to normal fetal heart parameters. Gestational range was 22–33 weeks. All fetal echo images were obtained using the GE Voluson E8. Measurements were obtained from the 4 chamber view. Analysis was done offline using software from Siemens Syngo VVI (Siemens Medical Solutions, Mountain View, California, USA). 2D velocity vector imaging (VVI) is an angle independent method of determining regional myocardial mechanics. Longitudinal velocity (LVel), strain(S), and strain rate (SR), were determined at the base free wall of the right ventricle in seven fetuses with hypoplastic left heart syndrome. Strain values were then compared to published criteria of normal right ventricles (base free wall) in the fetal heart. Data were obtained from all seven fetuses. Offline post-processing took approximately 4 ± 2 minutes. Longitudinal Velocity was similar in all patients. Strain and strain rate were similar in all but one patient who was confirmed post-natally to have endocardial fibroelastosis. In this patient, strain was markedly reduced. When compared to published normal fetal right base heart strain values our right ventricular function measurements were decreased in the fetus with hypoplastic left heart syndrome. Inter and intra-observer variability was not significant. VVI is a technically feasible and practical modality of evaluating fetal myocardial mechanics and function in the presence of congenital heart disease. Future comparison studies are needed.

We present cardiac magnetic resonance images of a 6-year-old with a suspected great artery abnormality in the setting of mesocardia, atrial situs solitus, D-looping of the ventricles, and anatomically corrected malposition of the great arteries with subaortic and subpulmonary conus.

Endovascular stent implantation for pulmonary artery stenosis requires the use of a long, large-bore vascular sheath to insure precise implantation without embolization or malposition. A long vascular sheath may be difficult to position and usage may be associated with vascular compromise and/or hemodynamic embarrassment, especially in infants and small children. We report a new technique for pulmonary artery endovascular stent implantation without the use of a long sheath. From December 2000 to May 2001, 10 patients underwent implantation of 13 Palmaz Corinthian premounted biliary transhepatic stents for pulmonary artery stenosis. Median age was 0.8 years (range, 0.5-18.5) and median weight was 11.8 kg (range, 4.6-65). Patient diagnoses were tetralogy of Fallot (five), double outlet right ventricle (three), branch peripheral pulmonary artery stenosis (two), single ventricle s/p cavopulmonary shunt (one), and truncus arteriosus (one). All Palmaz Corinthian stents were delivered uncovered on Cordis Opta LP balloon catheters via short sheaths (6-7 Fr); super-stiff guidewires were not always necessary. These stents, with a maximal expanded diameter of 12 mm, were placed for peripheral pulmonary artery stenosis as a definitive procedure or at the pulmonary artery bifurcation in patients who were expected to undergo future open heart surgery. The stents were initially implanted on 4, 6, or 8 mm balloon catheters and further expanded if needed. Stents were placed in the right pulmonary artery alone in three patients, left pulmonary artery alone in four patients, and side-by-side stents were implanted simultaneously in three patients. All thirteen stents were implanted successfully in the desired location without stent malposition or embolization. Mean angiographic diameter increased from 2.5 +/- 1.5 to 5.7 +/- 1.4 mm (P < 0.01) and peak systolic ejection gradients decreased from 44 +/- 22 to 14 +/- 11.6 mm Hg (P < 0.01). The uncovered delivery of the premounted Palmaz Corinthian stent allowed for precise and safe endovascular stent implantation without the hemodynamic and technical problems associated with long vascular sheath usage. This technique is useful for the palliation of proximal pulmonary artery stenosis and is effective definitive treatment for peripheral pulmonary artery stenosis in small infants and children.

Survival following cardiac transplantation in children and adults, including the group with complex congenital heart disease, has improved over the last decade secondary to medical and surgical advances in management. There have been rare reports of superior vena cava obstruction at anastomotic sites following transplantation. In patients following heart transplantation, venous stenosis can limit the ability to perform endomyocardial biopsies. We reviewed our experience in three patients who underwent cardiac transplantation and developed significant venous stenosis requiring intervention. All three were successfully treated by transcatheter implantation of endovascular stents. Endovascular stent implantation for venous obstruction in patients following cardiac transplantation was safe and effective, allowing improved ease of catheterization for future posttransplantation monitoring and surveillance.