Coarctation Angioplasty in Young Children: Does IMPACT Registry Data Accurately Reflect Clinical Outcomes?

Balloon angioplasty for recoarctation of the aorta is routinely performed in young children and is considered successful when the systolic gradient is reduced to < 10 mmHg. IMPACT defines acute procedural success solely based on a final gradient of < 10 mmHg and stratifies participating institutions based on these acute outcomes. Between February 2012 and December 2020, IMPACT data was analyzed for 110 coarctation interventions. Electronic medical records were reviewed, and primary endpoints were one of the following: (1) final analysis end date (June 2021), (2) patient death, or (3) most recent transcatheter or surgical reintervention. 64 (58.2%) interventions had a post-procedure CA gradient < 10 mmHg. Comparison of clinical patient outcome for acute success demonstrated no significant relationship using IMPACT (p = 0.70) criteria. There was no statistically significant difference between clinical success and failure for: pre- and post-treatment systolic gradients; absolute or percent change in systolic gradient; and pre-treatment aorta diameter. Clinical outcome and patient age did show a significant difference (p = 0.0093) with better clinical outcomes in older patients. Our analysis did not reveal a statistically significant difference between IMPACT criteria for successful treatment of CA and clinical outcome. These findings underscore a need to identify other clinical metrics that better predict outcome following CA balloon angioplasty treatment.

Surgical Valvuloplasty Versus Balloon Dilation for Congenital Aortic Stenosis in Pediatric Patients.

BACKGROUND: For children with congenital aortic stenosis (AS) who are candidates for biventricular repair, valvuloplasty can be achieved by surgical aortic valvuloplasty (SAV) or by transcatheter balloon aortic dilation (BAD). We aimed to evaluate the longer term outcomes of SAV versus BAD at our institution. METHODS: We retrospectively reviewed the outcomes of 2 months to 18 years old patients who underwent SAV or BAD at our institution between January 1990 and July 2018. Baseline and follow-up characteristics were assessed by echocardiography. Long-term survival, freedom from reintervention, freedom from aortic valve replacement (AVR), and aortic regurgitation were evaluated. RESULTS: A total of 212 patients met inclusion criteria (SAV = 123; BAD = 89). Age, sex, aortic insufficiency (AI), and aortic valve gradient were similar between the groups. At 10 years, 27.9% (19/68) of SAV patients and 58.3% (28/48) of BAD patients had moderate or worse AI (P = .001), and reintervention occurred in 39.2% (29/74) of SAV patients and 78.6% (44/56) of BAD patients (P < .001). Kaplan-Meier analysis revealed overall survival was 96.8% (119/123) for SAV and 95.5% (85/89) for SAV (P = .87). At 10 years, 35% (23/66) of SAV patients and 54% (23/43) of BAD patients underwent AVR (P = .213). CONCLUSIONS: Surgical aortic valvuloplasty demonstrated greater gradient reduction, less postoperative and long-term AI, and a lower reintervention rate at 10 years than BAD. There was no difference in survival or AVR reintervention rate. Surgical aortic valvuloplasty is a durable and efficacious intervention and should continue to be considered a favorable choice for palliation of valvular AS.

Short- and intermediate-term results of balloon aortic valvuloplasty and surgical aortic valvotomy in neonates.

BACKGROUND: Balloon aortic valvuloplasty and open surgical valvotomy are procedures to treat neonatal aortic stenosis, and there is controversy as to which method has superior outcomes. METHODS: We reviewed the records of patients at our institution since 2000 who had a balloon aortic valvuloplasty or surgical valvotomy via an open commissurotomy prior to 2 months of age. RESULTS: Forty patients had balloon aortic valvuloplasty and 15 patients had surgical valvotomy via an open commissurotomy. There was no difference in post-procedure mean gradient by transthoracic echocardiogram, which were 25.8 mmHg for balloon aortic valvuloplasty and 26.2 mmHg for surgical valvotomy, p = 0.87. Post-procedure, 15% of balloon aortic valvuloplasty patients had moderate aortic insufficiency and 2.5% of patients had severe aortic insufficiency, while no surgical valvotomy patients had moderate or severe aortic insufficiency. The average number of post-procedure hospital days was 14.2 for balloon aortic valvuloplasty and 19.8 for surgical valvotomy (p = 0.52). Freedom from re-intervention was 69% for balloon aortic valvuloplasty and 67% for surgical valvotomy at 1 year, and 43% for balloon aortic valvuloplasty and 67% for surgical valvotomy at 5 years (p = 0.60). CONCLUSIONS: Balloon aortic valvuloplasty and surgical valvotomy provide similar short-term reduction in valve gradient. Balloon aortic valvuloplasty has a slightly shorter but not statistically significant hospital stay. Freedom from re-intervention is similar at 1 year. At 5 years, it is slightly higher in surgical valvotomy, though not statistically different. Balloon aortic valvuloplasty had a higher incidence of significant aortic insufficiency. Long-term comparisons cannot be made given the lack of long-term follow-up with surgical valvotomy.

Management of Severe Coarctation of the Aorta During Pregnancy.

An 18-year-old primigravida woman underwent emergent percutaneous balloon stent repair of a severe coarctation of the aorta, allowing her to undergo an uneventful remaining pregnancy and the delivery of a healthy baby. Her case also demonstrates the association between maternal coarctation and fetal Shone complex. (Level of Difficulty: Beginner.).

Successful TPV Implantation in a Pregnant Patient With Right Ventricle to Pulmonary Artery Conduit Obstruction.

A patient with repaired double outlet right ventricle presented during early gestation with heart failure symptoms due to severe right ventricle-pulmonary artery conduit stenosis and insufficiency. In the first trimester, she underwent transcatheter therapy with Melody pulmonary valve implantation with excellent hemodynamic results and completed pregnancy without significant maternal complications. (Level of Difficulty: Advanced.).

Unusual interatrial fistula discovered during device closure of an atrial septal defect.

Atrial septal defect (ASD) closure is a common reason for referral to the cardiac catheterization laboratory. We report a case in which a relatively large Qp:Qs of 1.85:1 was demonstrated in the face of a small secundum ASD. This led to further investigation, ultimately leading to the discovery of an unusual atrial fistula. Rare cases of interatrial tunnels have been described in the literature, however, this is a unique case of a left atrial appendage to right atrial appendage fistula in the setting of a right juxtaposed left atrial appendage.

Early and late presentation of coronary artery fistula: a possible natural progression?

We present two patients, one 10 years old and another 43 years old, who both had successful transcatheter closure of left main coronary artery to right atrium fistulas. The older patient had a larger fistula as well as more symptoms and a complicated post-procedure course. Closure of medium or large coronary artery fistulas should be considered at younger ages to minimise future complications.

Resolution of inferior venous baffle leak and total venous occlusion using a bare stent and the gore(®) excluder( ®) aortic extension in transposition of the great arteries after mustard procedure.

We present a patient with a history of Mustard repair for transposition of the great arteries. The patient presented with complete inferior venous baffle obstruction and a large baffle leak after several years of cyanosis. Complete relief of the obstruction and exclusion of the baffle leak were accomplished with the use of a combination of bare metal stenting and the Gore(®) Excluder(®) aortic extender. To our knowledge, this represents the first reported use of the Gore(®) Excluder(®) aortic extender in the setting of inferior venous baffle leak and associated total inferior vena cava obstruction. © 2015 Wiley Periodicals, Inc.

Thromboexclusion of an atypical left ventricular pseudoaneurysm.

Left ventricular pseudoaneurysm is rare in children. The gold standard for treatment has been surgical repair. Here, we describe a boy in whom an atypical left ventricular pseudoaneurysm was treated through staged transcatheter procedures. In addition, we highlight the importance of the preprocedure cardiac magnetic resonance imaging in providing invaluable information that allowed detailed planning of a management strategy for this unusual pseudoaneurysm.

Results of the combined U.S. Multicenter Pivotal Study and the Continuing Access Study of the Nit-Occlud PDA device for percutaneous closure of patent ductus arteriosus.

OBJECTIVES: This study aimed to compare the efficacy and safety of the Nit-Occlud PDA device (PFM Medical, Cologne, Germany) to benchmarks designed as objective performance criteria (OPC). BACKGROUND: The Nit-Occlud PDA is a nitinol coil-type patent ductus arteriosus (PDA) occluder with a reverse cone configuration, which is implanted using a controlled delivery system. METHODS: Patients with <4-mm minimum diameter PDA were prospectively enrolled in the Pivotal and the Continuing Access Studies from 15 sites in the United States and were followed up for 12 months post-procedure. Investigator-reported outcomes were compared to OPC including a composite success criterion, efficacy criteria of successful closure (clinical and echocardiographic), and safety criteria incidence of adverse events (serious and of total). RESULTS: The Pivotal Study enrolled patients between November 1, 2002 and October 31, 2005, and the Continuing Access Study enrolled additional patients between September 1, 2006 and October 31, 2007. A total of 357 patients were enrolled, and 347 had successful device implantations. After 12 months, 96.8% had complete echocardiographic closure (OPC = 85%) and 98.1% had clinical closure (OPC = 95%). There were no deaths or serious adverse events (OPC = 1%). The total adverse event rate was 4.7% (OPC = 6%). Composite success was 95.1% in the study patients (OPC = 80%). CONCLUSIONS: Closure of small- and medium-sized PDA with the Nit-Occlud PDA is effective and safe when compared with OPC.

Delayed success of balloon dilation for coexisting pulmonary valve stenosis and sinotubular narrowing.

OBJECTIVES: We evaluated the outcomes of children at a single institution who underwent balloon pulmonary valvuloplasty (BPV) for coexisting pulmonary valve stenosis (PVS) and sinotubular narrowing (STN). BACKGROUND: BPV is the treatment of choice for PVS in children. Current practice favors surgical repair of moderate, severe, and symptomatic pulmonary stenosis when STN exists. This practice arose from lack of reduction in total pulmonary gradient (TPG) and frequent adverse events from BPV. METHODS: A retrospective analysis of outcomes in children with coexisting PVS and STN following BPV at a single institution was performed. RESULTS: Twenty-three patients were identified. Median age at BPV was 0.5 years (interquartile range (IQR) 0.3-2). Surgery was avoided in 15/23 (65%) (Group 1) and required in 8/23 (35%) (Group 2) following BPV. Group 1 had a mean baseline peak echo TPG of 60 mm Hg (±12) that decreased to 44 mm Hg (±10) following BPV (P < 0.01) and further to 21 mm Hg (±13) at 3.6 years (±2.2) following BPV (P < 0.01). Group 2 had a mean baseline peak echo TPG of 68 mm Hg (±17). TPG was unchanged by first echo after BPV at 56 mm Hg (±13) and just prior to surgery at 63 mm Hg (±15) (P > 0.10). CONCLUSIONS: BPV has minimal acute effect on PVS when STN exists; however; long-term benefits are achieved in most. BPV should be considered first-line therapy given its safety and effectiveness. Only those with worsening clinical signs and symptoms should be referred for surgical repair following BPV.

Mid-term follow up of perventricular device closure of muscular ventricular septal defects.

BACKGROUND: Surgical and transcatheter management of muscular ventricular septal defects (MVSD) have independent drawbacks. Hybrid procedures are becoming increasingly utilized to manage congenital cardiac lesions including MVSDs. This report describes the mid-term results of perventricular device closure of MVSDs at a single institution. METHODS: The cardiology database of patients who underwent attempted perventricular MVSD closure from a single institution was reviewed. RESULTS: Between January 2004 and December 2009, six patients underwent attempted perventricular MVSD closure in the operating room. Mean age was 9.8 ± 9.1 months; mean weight was 7.2 ± 3.7 kg. In five patients, closure was successful without use of bypass. In one patient, the device embolized to the left ventricle after release and patch closure of the MVSD was performed on cardiopulmonary bypass. The mean interval from the procedure to the most recent echocardiogram for the patients with successful perventricular closure was 39.8 ± 25.2 months. Three patients demonstrated no residual shunt at the last echocardiogram. Two patients had mild, hemodynamically insignificant shunting; one had a left ventricular pseudoaneurysm that was embolized during repeat catheterization. CONCLUSIONS: Perventricular closure of MVSDs is attractive because it overcomes the limitations of surgery and catheterization. Additionally, it spares the need for cardiopulmonary bypass and its comorbidities. In some instances, however, successful deployment of the device is not possible. Our mid-term results demonstrate overall success but identify possible complications that are not immediately identified in the short term.

Catheter closure of atrial septal defect in anatomically corrected malposition with left juxtaposition of the atrial appendages using intracardiac echocardiography.

Anatomically corrected malposition is characterized by abnormally related great vessels and atrioventricular and ventriculoarterial concordance. It is often associated with juxtaposition of the atrial appendages where the plane of the atrial septum is abnormally oriented. We describe the device closure of an atrial septal defect in a patient with such a lesion under intracardiac echocardiography guidance.

Percutaneous closure of patent ductus arteriosus: a multiinstitutional registry comparing multiple devices.

BACKGROUND: There are no guidelines and multiple devices available now for closure of PDA. OBJECTIVE: We sought to determine which devices are used and their outcomes. METHODS: Prospectively collected data on closure of PDA at 12 US catheterization labs was entered into the MAGIC data registry between November 2005 and September 2008. RESULTS: PDA closure was accomplished in 357 of 359 attempted closures during the study period. Mean age, weight, and minimum ductal diameter were 4.3 ± 6.8 years, 18.7 ± 18.4 kg, and 2.1 ± 1.4 mm, respectively. Devices used were Gianturco coils (161/359), Amplatzer Duct Occluder (ADO, 174/359), Flipper coils (18/359), and other devices (6/359). Gianturco coils had the least fluoroscopy and contrast exposure (8.7 ± 7.5 min, P = 0.00001, 2.6 ± 1.4 cm(3) kg(-1), P = 0.00001). Immediate angiographic success rates were 96.9% (156/161) for Gianturco coils, 89.7% (156/174) for the ADO, 100% (18/18) for Flipper coils, and 66.7% (4/6) for other devices. Major complication rates were 0.6% for Gianturco coils (1/161), 1.7% for ADO (3/174), and 83.3% for other devices (4/6). Minor complication rates by device were 1.9% for Gianturco coils (3/161), 2.3% for ADO (4/174), and 16.7% (1/6) for other devices. CONCLUSIONS: Gianturco coils, the ADO and Flipper coils were immediately successful with low complication profiles. The ADO and Gianturco coils were the predominate devices. The ADO was the device of choice for PDA > 3 mm with good success. In PDA < 3 mm, the Gianturco coil is as effective and safe as the ADO with less cost, less radiation, and less contrast exposure. © 2010 Wiley-Liss, Inc.

Recurrent coarctation: is surgical repair of recurrent coarctation of the aorta safe and effective?

BACKGROUND: Persistence or recurrence of stenosis is a complication of coarctation repair and is associated with major long-term morbidity. The rate of recurrence varies significantly, depending on the age of the patient, technique at initial repair, and the arch anatomy. We reviewed our experience with surgical repair of recurrent coarctation of the aorta and compared it with our institutional experience with balloon aortoplasty. METHODS: We retrospectively reviewed our experience with 1,012 patients undergoing initial repair of coarctation between 1960 and 2008. During that time, 103 patients (10%) required reintervention. Median age at reintervention was 6.5 years (range, 2 weeks to 44 years) and median weight was 12 kg (range, 1.9 to 94 kg). Fifty-nine patients with recoarctation had surgical repair, and 44 patients were treated with balloon aortoplasty with or without stent placement. RESULTS: Ninety-five percent of patients have been followed up (median time, 14.2 years; range, 2 months to 42 years). There were 5 late deaths. Actuarial survival was 98% at 15 and 40 years in patients with surgical reintervention, and it was 91% (p = 0.001) at 15 years in patients with balloon aortoplasty reintervention. A second redo coarctation of the aorta reintervention was performed in 12 patients: 8 patients after percutaneous intervention (nonsurgical) and 4 patients after surgical recoarctation repair. The median interval between first and second reintervention was 3.5 years (range, 1 month to 14 years). One patient who had two dilations underwent a third and fourth reintervention: patch enlargement and pseudoaneurysm resection. Freedom from reintervention in the surgical group was 96% at 15 years and 94% at 40 years, which was compared with actuarial freedom from reintervention for patients with percutaneous intervention (balloon/stent) at 15 years (82%; p < 0.001). CONCLUSIONS: Our study demonstrates that surgical repair of recurrent coarctation of the aorta can be performed safely and with excellent results. The recurrence after surgical reintervention is low, and most patients to date have not required further intervention. Balloon aortoplasty as an alternative method of managing recoarctation is efficient and less invasive than surgery; however, well-described complications may occur. Recurrence rates with angioplasty are significantly higher than with surgery.

Simultaneous apicoaortic conduit placement and mitral valve replacement in an adolescent with porcelain aorta, aortic stenosis, and mitral stenosis.

We describe the treatment of a 16-year-old girl with calcific aortic stenosis, porcelain aorta, and calcific mitral stenosis with insufficiency using a valved apicoaortic conduit and mitral valve prosthesis. Both valve replacements were porcine bioprostheses, and the apicoaortic conduit was implanted without the use of cardiopulmonary bypass. In cases in which the degree of aortic calcification pre-empts manipulation of the coronary ostia, an apicoaortic conduit may offer a viable solution to improve left ventricular outflow obstruction.

Successful embolization of a left ventricular pseudoaneurysm after perventricular ventricular septal defect device closure.

Perventricular device closure has become an important management adjunct for ventricular septal defects deemed inoperable and difficult to address in the catheterization laboratory. Complications directly related to the procedure are rarely reported, and all have been manifest in the periprocedural period. We present the case of a 6-week-old boy who underwent perventricular closure of a muscular ventricular septal defect and had a left ventricle pseudoaneurysm identified 10 months later during catheterization to close residual septal defects. We describe the subsequent percutaneous coil embolization of the pseudoaneurysm and clinical follow-up.

Single pediatric center experience with multiple device implantation for complex secundum atrial septal defects.

OBJECTIVES: We analyzed the efficacy and complications of placing multiple transcatheter devices to correct complex ASD in a primarily pediatric population. BACKGROUND: Limited pediatric information exists regarding the safety and efficacy of using multiple devices for secundum atrial septal defects (ASD) when multiple defects are present. METHODS: Cath lab, echo, and clinical data were reviewed for 238 patients who underwent device closure of secundum ASD from 2000 to 2007 at a single pediatric center. Of those requiring multiple devices (n = 15), residual shunting and complications were assessed. RESULTS: 238 patients (mean age 12 yrs; 80% < 16 yrs) underwent transcatheter ASD closure. 34 (14%) had multiple ASD, but only 15 received multiple devices (2 devices in 14 and 3 devices in 1). The mean age and weight (12 yrs; 35 kg) represent a younger population than previously reported. Only 3 were > 15 yrs, with a median age of 10.8 yrs (range 2-31). There was no compromise or obstruction to surrounding structures, and no device embolization or erosion was noted. A tiny thrombus was observed on the right sided screw of one device with no clinical sequelae. One patient (age 31 yrs) died at home 30 days post procedure from cardiac tamponade. Autopsy revealed the 3 intact devices in stable position without evidence of erosion. Latest available echo follow-up for the group revealed functional resolution of atrial level shunting in all patients. CONCLUSION: Multiple devices can safely and effectively be implanted in the pediatric population to close complex ASD.

Delivery sheath tear after modification for ASD closure.

During transcatheter closure of an atrial septal defect with insufficient aortic rim, a standard delivery sheath was modified by cutting a bevel at the distal tip to improve device orientation. The sheath split longitudinally when attempting to recapture the closure device. Troubleshooting allowed a device to be implanted successfully. Pitfalls regarding our sheath modification and methods to overcome prolapse of the left atrial disk into the right atrium are discussed.

Transcatheter closure of atypical right-to-left shunts after Fontan surgery.

Atypical right-to-left shunts following Fontan surgery were closed by transcatheter techniques using various devices in 4 patients. Three had variants of hypoplastic left heart syndrome, while the other had tricuspid atresia. Large Fontan baffle leaks were present in 2, a residual fistula from the inferior vena cava to the coronary sinus was present in 1, and another patient had surgical tube fenestration of an extracardiac conduit. When careful catheter and transesophageal echocardiographic exploration is performed, the selection of appropriate devices makes closure of these defects feasible.