Scott E. Fletcher

Outcomes of congenital diaphragmatic hernia in the modern era of management.

OBJECTIVE To identify clinical factors associated with pulmonary hypertension (PH) and mortality in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN A prospective cohort of neonates with a diaphragm defect identified at 1 of 7 collaborating medical centers was studied. Echocardiograms were performed at 1 month and 3 months of age and analyzed at a central core by 2 cardiologists independently. Degree of PH and survival were tested for association with clinical variables using Fischer exact test, χ(2), and regression analysis. RESULTS Two hundred twenty patients met inclusion criteria. Worse PH measured at 1 month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation, patients inborn at the treating center, and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. CONCLUSIONS Severity of PH is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status, and need for extracorporeal membrane oxygenation.

Endovascular Stent Grafts for Large Thoracic Aneurysms After Coarctation Repair

BACKGROUND Aneurysm formation is a complication not infrequently seen after repair of aortic coarctation and some may enlarge over time. Conventional management of large thoracic aneurysms after aortic coarctation repair has been akin to the surgical treatment of nonspecific aneurysms; however, hypothermic circulatory arrest has been more frequently required because of reoperations. We describe the treatment of a series of patients with large aneurysms using novel endovascular techniques. METHODS The database of patients undergoing thoracic endograft placement was reviewed to identify those with thoracic aneurysms after aortic coarctation repair. Clinical, operative, and radiographic data were assessed. Follow-up imaging included spiral computed tomography (CT) scans immediately after deployment, at 6 months, and yearly thereafter. RESULTS Of 9 patients that were identified, 7 presented for elective repair and 2 were emergencies. The aneurysms measured 4.7 to 7.3 cm in diameter on spiral CT scans. Seven patients underwent carotid to subclavian bypass and subclavian ligation. Endografts were placed abutting the origin of the left common carotid artery. Seven patients were treated with Zenith endografts (Cook, Inc, Bloomington, IN), and 2 with TAG devices (W.L. Gore & Associates, Flagstaff, AZ). Left common carotid angioplasty and stenting was performed in 4 patients. No major complications occurred. A mean follow-up of 24 months (range, 6.4 to 48 months) demonstrated no late endoleaks, ruptures, conversions, or migration. CONCLUSIONS Placement of endovascular stent grafts is a less invasive approach for patients with thoracic aneurysm after aortic coarctation repair, provided there is no residual coarctation or arch hypoplasia. The potential to diminish the magnitude of the surgical procedure and consequences of aortic exposure in a reoperative field is promising and mandates further investigation.

Decade of experience with vascular rings at a single institution

OBJECTIVE. Over the past decade, the diagnostic workup of vascular rings has changed at our institution. Despite surgical intervention, we have observed long-term aerodigestive issues in some patients. In an effort to better characterize these pre- and postoperative issues, we reviewed a decade of experience at our institution. METHODS. We performed a complete chart review of all patients identified as having surgical intervention for vascular ring between January 1993 and December 2003. A questionnaire was mailed to the family of each patient to ascertain a subjective assessment of long-term postoperative issues. RESULTS. Thirty-eight patients were reviewed and categorized as to specific arch anomaly. Associated cardiac anomalies were present in 29% of patients. Chronic feeding difficulties persisted in 18% of patients, and airway issues remained in nearly half of the patients. Patients with underlying genetic syndromes had less resolution of symptoms compared with nonsyndromic children. Chronic respiratory symptoms were present in 80% of patients operated <6 months, 15% if operated between 6 months and 3 years, and 42% of patients operated after 3 years of age. CONCLUSIONS. Aerodigestive issues may persist after vascular ring division despite initial improvement. Accurate preoperative anatomic imaging is imperative to surgical planning.

Percutaneous translumbar cardiac catheterization and central venous line insertion: An alternative approach in children with congenital heart disease

Children with congenital heart disease present major problems with venous access, eliminating conventional routes for cardiac catheterization. Although the transhepatic approach has recently gained popularity, we describe here an alternative approach using percutaneous translumbar approach for cardiac catheterization and/or in‐dwelling central line insertion in three children with congenital heart disease. Diagnostic hemodynamic studies, transcatheter delivery of an intravascular stent for left pulmonary artery (LPA) stenosis, and chronic central venous line insertion were performed using this technique. Disadvantages include interventionalists unfamiliarity with technique, awkward patient positioning, technically more difficult than transhepatic, and potential injury to kidney and bowel. Advantages include avoidance of vascular‐rich hepatic parenchyma, thus reducing risk of hemorrhage; providing an alternative where transhepatic entry may be contraindicated; avoidance of bile duct, portal vein, and hepatic artery injury; and providing another alternative for not only transvenous, but also transarterial access that may be required for intravascular aortic stent delivery. The interventional radiologist should be utilized as a valuable resource to the cardiologist to help teach and supervise this technique in selected infants and children with limited vascular access. Cathet. Cardiovasc. Intervent. 46:187–192, 1999.

Children With Heart Murmurs: Can Ventricular Septal Defect Be Diagnosed Reliably Without an Echocardiogram?

OBJECTIVES This study was undertaken to determine the accuracy of expert examination for ventricular septal defect (VSD) among children with a heart murmur. BACKGROUND Because the frequency and nature of errors that might be made by reliance solely on expert examination for diagnosis of VSD are speculative, the role of echocardiography in such diagnosis is controversial. METHODS Two hundred eighty-seven consecutive previously unevaluated pediatric subjects were enrolled in the study. For each child, the pediatric cardiologists prospectively recorded a working diagnosis and their level of confidence in the diagnosis, categorizing any VSD diagnosed as small or moderate to large. After echocardiography, VSDs were subcategorized by location and requirement for treatment as minor, intermediate or major. Receiver-operating characteristic (ROC) curves described the accuracy of the clinical examination. RESULTS Seventy-three subjects had a VSD (minor in 52, intermediate in 10 and major in 11). ROC areas (1.0 = perfect discrimination, 0.5 = indiscriminate) were minor VSD 0.92 +/- 0.02 and major/intermediate VSD 0.69 +/- 0.07 (p = 0.0016). Four of 52 minor VSDs were not identified at any level of suspicion; the clinical diagnoses were moderate to large VSD in two patients and atrial septal defect and unlimited differential diagnosis in one patient each. Fourteen of 235 patients without a minor VSD were believed with confidence to have a small VSD, but the final diagnosis was intermediate VSD in 4, innocent murmur in 3, major VSD in 2, pulmonary stenosis in 2 and subaortic membrane, atrial septal defect and mitral regurgitation in 1 patient each. CONCLUSIONS Almost all minor VSDs are recognized without echocardiography; however, errors can occur even when an expert examiner is confident. Clinical recognition of an intermediate or major VSD is less accurate than clinical recognition of a minor VSD. Failure to distinguish VSDs of major or intermediate importance from minor VSDs is a weakness of the expert clinical examination.

Management of pediatric patients with isolated valvar aortic stenosis by balloon aortic valvuloplasty.

Moderate to severe aortic stenosis in children requires an initial procedure to improve the stenosis and often additional procedures for recurrent stenosis or aortic insufficiency before adulthood. The purpose of this study was to evaluate children who underwent balloon valvuloplasty and were followed with a specific management plan. Twenty-two children with aortic stenosis underwent balloon valvuloplasty and were followed on a regular basis. Repeat valvuloplasty was performed if indicated. The initial gradient was reduced from 63 +/- 9 mmHg to 28 +/- 8 mmHg (P < 0.001). There were no deaths and only one major complication, which had no sequelae. Average follow-up was 61 +/- 23 months. Three patients required valve replacement 39-76 months after valvuloplasty for progressive insufficiency. Seven patients underwent successful repeat valvuloplasty. The overall probability of survival without surgical intervention was 75% at 100 months. Balloon valvuloplasty is an effective intermediate palliation for aortic stenosis and is an acceptable alternative to surgical valvotomy. Repeat valvuloplasty is successful without additional risk. In a subgroup of patients, aortic insufficiency is progressive and will require surgical intervention.

Successful transcatheter perforation of the atretic pulmonary valve membrane in a newborn using the new Coe radiofrequency end hole catheter

The new 2 French Coe radiofrequency (RF) end hole catheter was first used to successfully perforate the atretic pulmonary valve membrane using an antegrade approach in a newborn with intact ventricular septum (IVS). Nine watts of energy for 8 sec was required with simultaneous delivery of a 0.014 in. coronary guidewire coaxially through the end hole RF catheter for balloon valvuloplasty. This new ringed-tip end hole RF catheter offers considerable advantages to the pediatric interventionalist in the transcatheter therapy in neonates with pulmonary atresia (PA) and IVS.

Ultrasound contrast and real-time perfusion in conjunction with supine bicycle stress echocardiography for comprehensive evaluation of surgically corrected congenital heart disease

AIMS We sought to evaluate the efficacy of ultrasound contrast (UC) and low mechanical index real-time perfusion (RTP) in the haemodynamic and anatomic assessment of repaired congenital heart disease (CHD) at rest and during supine bicycle stress echocardiography (BSE). METHODS AND RESULTS Patients with CHD (n = 51, median age 21.5 years) were prospectively studied. All had compromised image quality, 20 (39%) had arrhythmias, and 10 (20%) had pacemakers. RTP was performed at rest and during BSE using Definity and Contrast Pulse Sequencing, with assessment of Doppler pressure gradients. Diagnoses included tetralogy of Fallot (n = 27), transposition of the great arteries (TGA) atrial switch (n = 10), TGA arterial switch (n = 2), aortic valve disease (n = 4), Fontan (n = 4), and Kawasaki disease (n = 4). UC with RTP improved endocardial border definition, with increased number of left ventricular (LV) and right ventricular (RV) segments visualized at rest (P < 0.0001) and during stress. LV ejection fraction (EF) and RV fractional area change (FAC) were measurable at rest and peak stress, RV FAC correlating closely with same-day magnetic resonance EFs (r = 0.72; P < 0.001). UC enhanced Doppler signals, enabling subpulmonary ventricular systolic pressure measurements at rest and stress. In six patients, marked elevations of subpulmonary ventricular systolic pressure were detected with UC during BSE, and quantifiable ventricular dysfunction. No adverse events occurred, other than transient low back pain in one patient. CONCLUSION UC at rest and with supine BSE enables safe and comprehensive assessment of anatomy, haemodynamics, and biventricular functional and perfusion reserve in adolescents and young adults with surgically modified CHD.

Patent ductus arteriosus closure using the amplatzer® vascular plug ii for all anatomic variants

To evaluate the safety and efficacy of the Amplatzer® Vascular Plug II (AVPII) for closure of the patent ductus arteriosus (PDA).

Sonothrombolysis of Intra-Catheter Aged Venous Thrombi Using Microbubble Enhancement and Guided Three-Dimensional Ultrasound Pulses

BACKGROUND Central venous and arterial catheters are a major source of thromboembolic disease in children. The investigators hypothesized that guided high-mechanical index (MI) impulses from diagnostic three-dimensional (3D) ultrasound during an intravenous microbubble infusion could dissolve these thrombi. METHODS An in vitro system simulating intracatheter thrombi was created and then treated with guided high-MI impulses from 3D ultrasound, using low-MI microbubble sensitive imaging pulse sequence schemes to detect the microbubbles. Ten aged thrombi >24 hours old were tested using 3D ultrasound coupled with a continuous diluted microbubble infusion (group A) and 10 with 3D ultrasound alone (group B). RESULTS The mean thrombus age was 28.6 hours (range, 26.6-30.3 hours). Group A exhibited a 55 +/- 19% reduction in venous thrombus size compared with 31 +/- 10% in group B (P = .008). Feasibility testing was performed in four pigs, establishing an in vivo model to investigate further the efficacy of this approach. CONCLUSIONS Sonothrombolysis of aged intracatheter venous thrombi can be achieved with commercially available microbubbles and guided high-MI ultrasound from a diagnostic 3D transducer.