Efficacy of Drug-Coated Balloon Angioplasty in Pulmonary Vein Stenosis or Total Occlusion.

BACKGROUND: Current therapies for pulmonary vein stenosis (PVS) or pulmonary vein total occlusion (PVTO) involving angioplasty and stenting are hindered by high rates of restenosis. OBJECTIVES: This study compares a novel approach of drug-coated balloon (DCB) angioplasty and stenting with the current standard of care in PVS or PVTO due to pulmonary vein isolation (PVI). METHODS: A retrospective single-center study analyzed patients with PVS or PVTO due to PVI who underwent either angioplasty and stenting (NoDCB group; December 2012-December 2016) or DCB angioplasty and stenting (DCB group; January 2018-January 2021). Multivariable Andersen-Gill regression analysis assessed the risk of restenosis and target lesion revascularization (TLR). RESULTS: The NoDCB group comprised 58 patients and 89 veins, with a longer median follow-up of 35 months, whereas the DCB group included 26 patients and 33 veins, with a median follow-up of 11 months. The DCB group exhibited more PVTO (NoDCB: 12.3%; DCB: 42.4%; P = 0.0001), with a smaller reference vessel size (NoDCB: 10.2 mm; DCB: 8.4 mm; P = 0.0004). Follow-up computed tomography was performed in 82% of NoDCB and 85% of DCB, revealing lower unadjusted rates of restenosis (NoDCB: 26%; DCB: 14.3%) and TLR (NoDCB: 34.2%; DCB: 10.7%) in the DCB group. DCB use was associated with a significantly lower risk of restenosis and TLR (HR: 0.003: CI: 0.00009-0.118; P = 0.002). CONCLUSIONS: The novel approach of DCB angioplasty followed by stenting is effective and safe and significantly reduces the risk of restenosis and reintervention compared with the standard of care in PVS or PVTO due to PVI.

Contemporary risk factors for a longer hospital stay following bidirectional cavopulmonary anastomosis.

BACKGROUND: Despite high survival after bidirectional cavopulmonary anastomosis, a considerable number of patients suffer significant post-operative morbidities related to prolonged length of stay. METHODS: A single-center retrospective cohort study of all consecutive patients undergoing a first-time bidirectional cavopulmonary anastomosis from 2006 to 2019. RESULTS: Prolonged length of stay was defined as hospital stay greater than the 75th percentile for our cohort. Of 195 patients who met inclusion criteria, the median post-operative length of stay was 8 days (interquartile range, 4-15 days). Prolonged length of stay was defined as greater than 15 days. In multivariate analysis, greater than mild systemic atrioventricular valve regurgitation (odds ratio 3.7, 95% CI 1.05-13.068, p = 0.04), longer length of stay after the initial palliative procedure (odds ratio 1.028, 95% CI 1.004-1.05, p = 0.02), and pre-operative higher superior vena cava oxygen saturation (odds ratio 0.922, 95% CI 0.85-0.99, p = 0.04) maintained statistical significance as independent risk and protective factors for prolonged length of stay. A one-level increase in the severity of pre-operative systemic atrioventricular valve regurgitation was associated with a multiplicative change in the odds ratio of prolonged length of stay of 5.45 (p = 0.005) independent of the severity of systemic ventricular dysfunction. CONCLUSION: Pre-operative characteristics with greater than mild systemic atrioventricular valve regurgitation, longer length of stay after the initial palliative procedure, and lower superior vena cava oxygen saturation were associated with prolonged length of stay after a first-time bidirectional cavopulmonary anastomosis.

Melody valve-in-valve implantation in the tricuspid position through a Fontan conduit fenestration.

Patients with single right ventricle and tricuspid regurgitation have increased morbidity and mortality rates. The outcomes of surgical tricuspid valve repair and replacement are dismal. Although some centers prefer valve replacement with a bioprosthetic valve in the tricuspid position for the pediatric population, these valves have limited longevity and will eventually need to be replaced. Unfortunately, single ventricle patients are fragile and unlikely to tolerate a second replacement. Percutaneous valve-in-valve (ViV) implantation is an acceptable alternative with low morbidity and mortality. Studies have also shown acceptable short- and mid-term outcomes. We report a case of Melody ViV placement in the tricuspid position in a patient with hypoplastic left heart syndrome through the fenestration of a Fontan conduit.

Treatment of Congenital and Acquired Pulmonary Vein Stenosis.

PURPOSE OF REVIEW: Pulmonary vein stenosis (PVS) is a rare entity that until the last 2 decades was seen primarily in infants and children. Percutaneous and surgical interventions have limited success due to relentless restenosis, and mortality remains high. In adults, acquired PVS following ablation for atrial fibrillation has emerged as a new syndrome. This work will review these two entities with emphasis on current treatment. RECENT FINDINGS: Greater emphasis on understanding and addressing the mechanism of restenosis for congenital PVS has led to the use of drug-eluting stents (DES) and systemic drug therapy to target neo-intimal growth. Frequent reinterventions are positively affecting outcomes. Longer-term outcomes of percutaneous treatment for acquired PVS are emerging. Treatment of congenital PVS continues to be plagued by restenosis. DES show promise, but frequent reinterventions are required. Larger upstream vein diameter predicts success for congenital and acquired PVS interventions. Efforts to induce/maintain vessel growth are important for future treatment strategies.

Long-term outcome of percutaneous intervention for pulmonary vein stenosis after pulmonary vein isolation procedure.

OBJECTIVES: Report long-term outcomes of percutaneous intervention in patients with pulmonary vein stenosis (PVS) after pulmonary vein isolation (PVI) from a single center over 16 years. BACKGROUND: Outcome reports of percutaneous intervention for PVS resulting from PVI are limited. METHODS: Retrospective review of all patients with PVS after PVI who underwent percutaneous intervention at the Cleveland Clinic Foundation between January 2000 and December 2016. RESULTS: A total of 205 patients underwent cardiac catheterization for PVS during the study period. Completely occluded veins which could not be recanalized occurred in six patients. Of the remaining 199 patients, 27 (14%) were lost to follow-up, leaving 172 patients with 276 veins for analysis. Balloon angioplasty was performed in 62 veins and stent implantation in 250 (primary in 214, to treat postdilation restenosis in 36). Re-intervention occurred in 45/62 (73%) balloon-dilated veins and 45/250 (18%) stented veins. Freedom from re-intervention at 1 and 5 years was 90 and 73% following stenting versus 40 and 23% following balloon dilation (p < .001, Hazard ratio (HR) = 5.7). Veins with stent diameter ≥7 mm (n = 231) had greater freedom from re-intervention (95% at 1 year, 79% at 5 years) than veins with stents <7 mm (43% at 1 year, 9% at 5 years), p < .001. There was clear symptomatic improvement after intervention and no procedural mortality. CONCLUSIONS: Stent implantation at ≥7 mm for PVS after PVI is associated with low rates of re-intervention, in contrast to balloon dilation and stenting with small conventional stents.

Hammock effect and complete cusp prolapse: Rare mechanisms of Melody valve failure demonstrated by intracardiac echocardiography.

Transcatheter pulmonary valve replacement using Melody valve (Medtronic, Minneapolis MN) has significantly increased in the recent decades. Melody valve failures, although rare, can be problematic and require re-intervention. Through intracardiac echocardiography, we present two patients who each had a rare etiology for dysfunction of their Melody valve. Hammock effect, wherein the valve does not oppose the stent and complete cusp failure causing severe regurgitation have not been previously described as causes of Melody valve failure in the absence of endocarditis. Awareness and knowledge of these mechanisms is pivotal in the management of this patient population.

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right-sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10-month-old male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve stenosis with a large secundum atrial septal defect and a mildly hypoplastic right ventricle.

Radiation Reduction in the Pediatric Catheterization Laboratory Using a Novel Imaging System.

OBJECTIVES: Radiation dose was compared between two modern imaging systems with different x-ray tube technology (Megalix vs Gigalix) and detector type (amorphous vs crystalline silicon) at the same institution. BACKGROUND: Further reduction in radiation dose than currently reported may be achievable with advances in x-ray tube and detector technology. METHODS: Radiation dose (air kerma, dose-area product [DAP]) was retrospectively compared for post-transplant pediatric patients undergoing right heart catheterization/biopsy (fluoroscopy only) or "annual" catheterization with coronary angiography in one of two imaging systems between January 2014 and December 2016. Comparisons were also made with published radiation doses. RESULTS: A total of 122 right heart catheterizations with biopsy were performed in the Megalix/amorphous silicon (Si) lab and 168 in the Gigalix/crystalline Si lab. Age and weight were not statistically different for the two groups. There was a 50% decrease in median air kerma (2.2 mGy vs 1.1 mGy; P<.001) and 66% decrease in median DAP (52.2 µGy•m² vs 18.0 µGy•m²; P<.001) for the Gigalix/crystalline Si lab. A total of 24 "annual" catheterizations were performed in the Megalix/amorphous Si lab and 22 were performed in the Gigalix/crystalline Si lab. There was a 57% reduction in median air kerma (458.6 mGy vs 198.6 mGy; P<.001) and a 46% reduction in median DAP (2548.0 µGy•m² vs 1367.1 µGy•m²; P<.01) for the Gigalix/crystalline Si lab. Similar reductions were found on comparison with published doses. CONCLUSION: The Gigalix tube and crystalline Si detector decrease radiation dose by 50%-60% for fluoroscopy and cine acquisition in pediatric patients.

Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis.

To validate the feasibility and spatial accuracy of pre-procedural 3D images to 3D rotational fluoroscopy registration to guide interventional procedures in patients with congenital heart disease and acquired pulmonary vein stenosis. Cardiac interventions in patients with congenital and structural heart disease require complex catheter manipulation. Current technology allows registration of the anatomy obtained from 3D CT and/or MRI to be overlaid onto fluoroscopy. Thirty patients scheduled for interventional procedures from 12/2012 to 8/2015 were prospectively recruited. A C-arm CT using a biplane C-arm system (Artis zee, VC14H, Siemens Healthcare) was acquired to enable 3D3D registration with pre-procedural images. Following successful image fusion, the anatomic landmarks marked in pre-procedural images were overlaid on live fluoroscopy. The accuracy of image registration was determined by measuring the distance between overlay markers and a reference point in the image. The clinical utility of the registration was evaluated as either "High", "Medium" or "None". Seventeen patients with congenital heart disease and 13 with acquired pulmonary vein stenosis were enrolled. Accuracy and benefit of registration were not evaluated in two patients due to suboptimal images. The distance between the marker and the actual anatomical location was 0-2 mm in 18 (64%), 2-4 mm in 3 (11%) and >4 mm in 7 (25%) patients. 3D3D registration was highly beneficial in 18 (64%), intermediate in 3 (11%), and not beneficial in 7 (25%) patients. 3D3D registration can facilitate complex congenital and structural interventions. It may reduce procedure time, radiation and contrast dose.

Protein Losing Enteropathy After Cardiac Transplantation Successfully Treated by Stent Implantation.

Protein losing enteropathy (PLE) is a severe consequence of Fontan physiology that sometimes requires heart transplantation for definitive treatment. We describe transplant refractory PLE in a patient with heterotaxia and complex systemic and hepatic venous connections, successfully treated by transcatheter relief of multiple venous obstructions.

Percutaneous Pulmonary Valve Implantation in the Native Right Ventricular Outflow Tract Using a 29-mm Edwards SAPIEN 3 Valve.

Percutaneous pulmonary valve implantation is now an acceptable treatment option. The Edwards SAPIEN valve, intended for transcatheter aortic valve implantation, has been used in patients whose landing zone diameter is >22 to 24 mm which is not suitable for Melody valve. We report a patient in whom we successfully placed the latest generation, Edwards SAPIEN 3 (ES3) in the pulmonary position. However, because of the short covered portion of this valve, there was a significant paravalvular leak through the stent struts requiring implantation of a second ES3 valve.

Percutaneous removal of unraveled HELEX® septal occluder 4 months post deployment.

The GORE® HELEX® Septal Occluder (HSO: W.L. Gore & Associates; Flagstaff, AZ) is preferentially used at our institution for percutaneous closure of the patent foramen ovale (PFO). Adequate deployment of the device requires capture of three sequential eyelets by the locking loop. At times, the right atrial eyelet is not caught, particularly when a long tunnel PFO causes too much separation between the discs. Although rarely, unlocked devices have been left in the atrial septum with no untoward events provided they appear stable in the catheterization laboratory and the shunt has been eliminated. We report a patient in whom an unlocked, but otherwise well positioned, HSO subsequently unraveled with the right atrial disc migrating through the tricuspid valve while the left atrial disc remained well apposed to the left side of the atrial septum. The PFO was closed prior to liver transplantation to prevent an embolic event during the transplant. The patient required placement of several internal jugular central lines prior to transplant, and this instrumentation in the right atrium may have caused unraveling of the device. The HSO was removed percutaneously 15 weeks after implantation despite a well-seated and likely partially endothelialized left atrial disc.

Cryoablation of AVNRT when sustained tachycardia cannot be induced.

BACKGROUND: Inducibility of sustained tachycardia is preferred prior to cryoablation for atrioventricular nodal reentrant tachycardia (AVNRT). METHOD: The Pediatric Electrophysiology Database of a single institution was retrospectively reviewed for patients with clinical sustained (≥ 30 seconds of symptoms) AVNRT documented by noninvasive means who underwent cryoablation for AVNRT to determine if intermediate-term success with cryoablation for AVNRT can be achieved without inducibility of sustained AVNRT during electrophysiology study (EPS). RESULTS: There were no differences between patients with sustained (≥ 30 seconds of tachycardia) AVNRT (N = 67) and patients with nonsustained (ns, ≥ 3 beats and <30 seconds of tachycardia) AVNRT at EPS (N = 16). Acute success was achieved without PR prolongation in all patients. Although duration of follow-up was shorter for the sustained group than the nonsustained group (2.7 ± 1.6 years vs 3.8 ± 1.4 years, P = 0.008), recurrence rate was similar (6% vs 6.3%, P = 0.6). In patients with only nonsustained AVNRT at EPS, supportive findings for procedural effectiveness seen: (1) Dual atrioventricular node physiology (DAVNP) was eliminated in 14/14, (2) the fast pathway effective refractory period (FPERP) decreased after ablation in 10/11, (3) sustained slow pathway conduction was eliminated in 8/8 including both patients without discrete DAVNP prior to ablation, and (4) FPERP increased during lesion formation in 10/10. CONCLUSION: Intermediate-term success can be achieved with cryoablation of ns AVNRT. Attention should be paid to supportive indicators of damage to slow pathway.

Right thoracic ectopic kidney in a child with breathing difficulties--sonographic diagnosis.

Congenital intrathoracic ectopic kidney is a rare congenital abnormality that is usually found as an incidental lesion on chest radiographs. We report the case of a 6-month-old male with a 1-day history of breathing difficulties whose chest radiograph revealed a soft tissue right basilar mass. Further investigation utilizing ultrasound revealed a thoracic kidney. This case demonstrates the importance of sonography as a diagnostic tool in identifying thoracic kidneys in the pediatric age group.