Utility of live/real time three-dimensional transesophageal echocardiography in the assessment and percutaneous intervention of bioprosthetic pulmonary valve stenosis.

Accurate echocardiographic evaluation of the pulmonary valve is technically difficult because of its close proximity to the left lung, which often limits decision making. Pulmonary valvotomy is the intervention of choice for symptomatic pulmonary valve stenosis, but fluoroscopy lacks appropriate real time anatomic detail. In this report, we present a case where direct imaging of the pulmonary valve with live/real time three-dimensional transesophageal echocardiography (3DTEE) aided in accurate evaluation and was then used to help guide and monitor successful valvuloplasty of a stenotic pulmonary valve bioprosthesis. We demonstrate that even in cases where two-dimensional (2D) evaluation of the pulmonary valve is difficult, the use of live/real time 3DTEE allows for accurate evaluation of bioprosthetic pulmonary valve structure and function, and enhances the precision and monitoring of percutaneous valvuloplasty.

Live/real time three-dimensional transesophageal echocardiographic assessment of the spinal cord.

We present an adult patient in whom live/real time three-dimensional transesophageal echocardiography (3DTEE) provided incremental value in the assessment of the spinal cord as compared to two-dimensional transesophageal echocardiographic (2DTEE) findings published in the literature. It improved accurate identification and assessment of the anterior radiculomedullary spinal arteries which may have an important clinical application in monitoring for spinal cord ischemia during thoracic aortic surgery. Because the spinal cord and spinal canal could be examined using not only transverse but also coronal (frontal), sagittal, and oblique planes, 3DTEE further allowed for three-dimensional measurements of the dimensions and volumetric analysis of the visualized spinal cord and spinal canal. These may have implications in the assessment of spinal cord edema due to trauma and other conditions which result in increase in the size and volume of the spinal cord.

Ultrasound- versus landmark-guided femoral catheterization in the pediatric catheterization laboratory: a randomized-controlled trial.

Ultrasound (US) is the standard of care for vascular access in many clinical scenarios. Limited data exist regarding the benefits of US- versus landmark (LM)-guided femoral vascular access in the pediatric catheterization laboratory. This study aimed to compare US- and LM-guided vascular access in the pediatric catheterization laboratory. A single operator randomized 95 patients (201 vessels) to undergo either LM- or US-guided vascular access. The primary end point was the access success rate. Number of attempts, inadvertent access, time to sheath placement, and complications also were compared between the two groups. No difference was seen in the overall access success rate: 98 % with US versus 93 % with LM (p = 0.17). The success rate for the targeted vessel was higher with US (89 %) than with LM (67 %) (p = 0.012). US facilitated fewer attempts (1.1 ± 0.4 vs 1.4 ± 0.9; p = 0.048) and improved the first-attempt success rate (87 vs 77 %; p = 0.049). The time to access did not differ significantly between the two groups (US 2:55 ± 4:03 vs LM 3:37 ± 2:54; p = 0.28). No differences in complication rates were noted. The benefits of US were accentuated in the subgroup weighing less than 10 kg. In this study, US access in the pediatric catheterization laboratory did not improve overall success. However, US improved accuracy and reduced the number attempts necessary for access without prolonging the access time of the procedure. Small children realized the greatest benefit of US-guided access.

Transcatheter Pulmonary Valve Implantation: A Comprehensive Systematic Review and Meta-Analyses of Observational Studies.

BACKGROUND: Transcatheter pulmonary valve implantation is approved for the treatment of dysfunctional right ventricle to pulmonary artery conduits. However, the literature is limited because of a small patient population, and it does not reflect changing procedural practice patterns over the last decade. METHODS AND RESULTS: A comprehensive search of Medline and Scopus databases from inception through August 31, 2016 was conducted using predefined criteria. We included studies reporting transcatheter pulmonary valve implantation in at least 5 patients with a follow-up duration of 6 months or more. In 19 eligible studies, 1044 patients underwent transcatheter pulmonary valve implantation with a pooled follow-up of 2271 person-years. Procedural success rate was 96.2% (95% confidence intervals [CI], 94.6-97.4) with a conduit rupture rate of 4.1% (95% CI, 2.5-6.8) and coronary complication rate of 1.3% (95% CI, 0.7-2.3). Incidence of reintervention was 4.4 per 100 person-years overall (95% CI, 3.0-5.9) with a marked reduction in studies reporting ≥75% prestenting (2.9 per 100 person-years [95% CI, 1.5-4.3] versus 6.5/100 person-years [95% CI, 4.6-8.5]; P<0.01). Pooled endocarditis rate was 1.4 per 100 person-years (95% CI, 0.9-2.0). CONCLUSIONS: Our study provides favorable updated estimates of procedural and follow-up outcomes after transcatheter pulmonary valve implantation. Widespread adoption of prestenting has improved longer-term outcomes in these patients.

Balloon Angioplasty for the Treatment of Left Innominate Vein Obstruction Related Chylothorax after Congenital Heart Surgery.

OBJECTIVE: Chylothorax complicates the postoperative course of patients after congenital heart surgery. Innominate vein thrombosis and stenosis have been associated with postoperative chylothorax. Revascularization and angioplasty can be accomplished using transcatheter techniques. We report our experience with this procedure for the management of postoperative chylothorax. DESIGN: This is a retrospective case series of patients who underwent catheter revascularization and/or angioplasty of the innominate vein following cardiac surgery at our institution from January 1, 2008 through April 9, 2014. SETTING: The cardiovascular intensive care unit and cardiac catheterization laboratory at the University of Alabama at Birmingham and Benjamin Russell Hospital for Children in Birmingham, Alabama were used as settings for the study. PATIENTS: Out of 112 patients with postoperative chylothorax, 7 (6.3%) underwent transcatheter dilation of the innominate vein for occlusion/stenosis. The median age of the cohort was 1 month (15 days-6 years); median weight was 3 kg (2.7-22.2). Diagnosis was made a median 8 days (2-20) and persisted for a median of 24 days (9-44). Most patients failed medical management (low fat diet, nothing by mouth, and/or octreotide). RESULTS: Cardiac catheterization occurred at a median 9 days (2-29) after chylothorax diagnosis. Median chest tube output on the day prior to procedure was 63 (12-149) cc/kg/day and decreased to 23 (0-64) cc/kg/day 2 days postprocedure (P = .01). Effusions resolved in a median of 5 days (1-16). There were no clinical complications postcatheterization. All patients who have undergone repeat angiography have maintained patency of the innominate vein. There was no mortality. Complications from chylothorax included prolong hospitalization, hyponatremia, hypoproteinemia, coagulopathy, lymphopenia, and infection. CONCLUSIONS: Innominate vein occlusion and stenosis associated with chylous effusion are amenable to transcatheter revascularization and/or angioplasty, consistently leading to improvement, if not full resolution of chylothorax.

Transhepatic Cannulation for Venovenous Extracorporeal Membrane Oxygenation.

Limited vascular access because of vessel injury or thrombosis may complicate care of children with congenital heart disease. Although transhepatic venous access for cardiac catheterization and central venous catheter placement has been used in children, its use for extracorporeal membrane oxygenation (ECMO) has not been described. We report successful use of transhepatic cannulation for venovenous ECMO to support a 15 month-old child with bidirectional Glenn anatomy and intractable hypoxemia.

Novel technique of valve-sparing aortic root replacement in two children younger than 3 years of age.

A novel technique of valve-sparing aortic root replacement was applied to 2 children younger than 3 years of age with Marfan syndrome and large aortic root aneurysms. Using elements of both the remodeling and reimplantation techniques, circumferential rings from a 20-mm to 22-mm polyester graft provide stabilization at the subannular and sinotubular levels, and bovine pericardial patches create pseudosinuses. Follow-up at 2 years in 1 patient and 7 months in a second patient revealed satisfactory valve function with stable aortic root size.

Coronary artery fistula ligation in a child with use of the Starfish Heart Positioner.

The Starfish Heart Positioner aids off-pump coronary artery surgery in adult patients by providing posterior cardiac exposure without incurring hemodynamic instability. Herein, we describe its use in a 17-month-old girl who had a right coronary artery fistula that drained to the right ventricle. Use of the device enabled exposure that afforded closure of the fistula without cardiopulmonary bypass. The patient was discharged from the hospital 2 days postoperatively. Six weeks later, she was well, and echocardiography showed no residual fistulous flow.

Patency of internal jugular vein following repair after ECMO stabilization prior to Berlin Heart implantation: utility of repaired vein for subsequent cardiac catheterization and biopsy.

OBJECTIVE: The objective of this study was to examine the patency and utility for subsequent vascular access of the internal jugular vein following use in short-term extracorporeal membrane oxygenation. DESIGN: Retrospective review. SETTING: Pediatric cardiac intensive care unit, pediatric cardiac catheterization laboratory, and pediatric cardiac clinic. PATIENTS: Four children, ages 7-178 months, requiring mechanical circulatory support. Interventions. Extracorporeal membrane oxygenation support, internal jugular vein repair, Berlin Heart mechanical circulatory support, heart transplantation, cardiac catheterization. OUTCOME MEASURES: Following surgical repair, the internal jugular vein was studied with bedside ultrasound for assessment of patency. When appropriate, subsequent vascular access of the vessel was attempted and the success was reported. RESULTS: Follow-up ultrasound examination demonstrated vessel patency in all cases. In patients requiring subsequent catheterization (3/4), successful vessel entry and catheterization were performed. CONCLUSIONS: Repair of the internal jugular vein following use for short-term extracorporeal membrane oxygenation support can be accomplished with success. The vessel can be used for subsequent vascular access when necessary.

Is the "perfect Fontan" operation routinely achievable in the modern era?

OBJECTIVE: In 1990, Fontan, Kirklin, and colleagues published equations for survival after the so-called "Perfect Fontan" operation. After 1988, we evolved a protocol using an internal or external polytetraflouroethylene tube of 16 to 19 millimetres diameter placed from the inferior caval vein to either the right or left pulmonary artery along with a bidirectional cava-pulmonary connection. The objective of this study was to test the hypothesis that a "perfect" outcome is routinely achievable in the current era when using a standardized surgical procedure. METHODS: Between 1 January, 1988, and 12 December, 2005, 112 patients underwent the Fontan procedure using an internal or external polytetraflouroethylene tube plus a bidirectional cava-pulmonary connection, the latter usually having been constructed as a previous procedure. This constituted 45% of our overall experience in constructing the Fontan circulation between 1988 and 1996, and 96% of the experience between 1996 and 2005. Among all surviving patients, the median follow-up was 7.3 years. We calculated the expected survival for an optimal candidate, given from the initial equations, and compared this to our entire experience in constructing the Fontan circulation. RESULTS: An internal tube was utilized in 61 patients, 97% of whom were operated prior to 1998, and an external tube in 51 patients, the latter accounting for 95% of all operations since 1999. At 1, 5, 10 and 15 years, survival of the entire cohort receiving polytetraflouroethylene tubes is superimposable on the curve calculated for a "perfect" outcome. Freedom from replacement or revision of the tube was 97% at 10 years. CONCLUSION: Using a standardized operative procedure, combining a bidirectional cavopulmonary connection with a polytetraflouroethylene tube placed from the inferior caval vein to the pulmonary arteries for nearly all patients with functionally univentricular hearts, early and late survival within the "perfect" outcome as predicted by the initial equations of Fontan and Kirklin is routinely achievable in the current era. The need for late revision or replacement of the tube is rare.

Usefulness of live three-dimensional transthoracic echocardiography in the characterization of atrial septal defects in adults.

In this report we present 12 adult patients in whom surgical or percutaneous intervention was considered for repair of atrial septal defect (ASD). Location, size, and surrounding atrial anatomy of the ASD were assessed prior to intervention in all patients with standard and live three-dimensional transthoracic echocardiography (3D TTE). In the four patients in whom intraoperative three-dimensional transesophageal echocardiographic reconstruction (3D TEE) was done, 3D TTE measurements of maximum dimension, maximum circumference, and maximum area of ASD agreed well with 3D TEE. In the seven patients who underwent transcatheter closure device insertion, live 3D TTE measurements of maximum dimension, maximum circumference, and maximum area of ASD agreed well with the sizing balloon. Additionally, since the sizing balloon measures a stretched diameter and area, a live 3D TTE stretched ASD diameter and area (derived from the actual live 3D TTE maximum circumference) were calculated and demonstrated improved agreement with the sizing balloon measurements. In all patients, > or =5 mm of atrial tissue was visualized surrounding the ASD. Further, with the addition of contrast enhancement, characterization of a small patent foramen ovale (<5 mm) was possible in one patient. Live 3D TTE accurately defined ASD location, size, and surrounding atrial anatomy in all patients studied by us. ASD characterization by live 3D TTE agreed well with 3D TEE and sizing balloon measurements.