Senning Procedure for Physiological Atrial Inversion With Left Atrial Isomerism.

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.).

Reports of 2 Rare Associations of Hypoplastic Left Heart Syndrome.

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.).

Development of quality metrics for ambulatory pediatric cardiology: Transposition of the great arteries after arterial switch operation.

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.

Pre-operative renal volume predicts peak creatinine after congenital heart surgery in neonates.

OBJECTIVE: 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.

Right-ventricular global longitudinal strain may predict neo-aortic arch obstruction after Norwood/Sano procedure in children with hypoplastic left heart syndrome.

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.

Head and neck vessel size by angiography predicts neo-aortic arch obstruction after Norwood/Sano operation for hypoplastic left heart syndrome.

OBJECTIVES: To identify and predict neo-aortic arch obstruction (NAAO) in children after Norwood/Sano operation (NO) for hypoplastic left heart syndrome (HLHS). BACKGROUND: NAAO 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. METHODS: Retrospective 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. RESULTS: Forty-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. CONCLUSIONS: HNI is a more robust indicator of hemodynamically significant NAAO than CI and may predict its future occurrence after NO for HLHS.

Hypoplastic left heart syndrome and pulmonary veno-occlusive disease in an infant.

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.

An angiographic predictor of pulmonary artery stenosis after the Norwood-Sano operation for hypoplastic left heart syndrome.

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.

Anatomically corrected malposition of the great arteries.

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.

Cardiac transplantation after the Fontan or Glenn procedure.

OBJECTIVES: The purpose of this study was to review the clinical course and outcome of cardiac transplantation after a failed Glenn or Fontan procedure. BACKGROUND: Late complications of the Glenn or Fontan procedure, including ventricular failure, cyanosis, protein-losing enteropathy, thromboembolism, and dysrhythmias often lead to significant morbidity and mortality. If other therapies are ineffective, cardiac transplantation is the only therapeutic recourse. Transplantation in this unique population presents significant challenges in the operative and perioperative periods. METHODS: The anatomic diagnoses, previous operations, clinical status, and indications for transplantation were characterized in patients transplanted after a Glenn or Fontan procedure. Outcomes after transplantation, including postoperative complications and mortality, were reviewed. Comparisons were made between survivors and nonsurvivors. RESULTS: Primary orthotopic cardiac transplantation was performed in 35 patients (mean age 15.7 +/- 8.5 years) with a mean follow-up of 54 +/- 46 months. A total of 11 patients had undergone a Glenn shunt and 24 patients a Fontan procedure. Indications for transplantation were a combination of causes including ventricular dysfunction, failed Fontan physiology, and/or cyanosis. Ten patients died

Endovascular stent implantation in the pulmonary arteries of infants and children without the use of a long vascular sheath.

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.