Anderson, Jason

Background: Inferior vena cava (IVC) size and collapsibility (IVC dynamics) are used for estimating right atrial pressure (RAP). However, the diagnostic performance of the American Society of Echocardiography IVC criteria for estimating RAP in patients with congenital heart disease are unknown. The purpose of this study was to assess the role of IVC dynamics for estimating RAP in adults with congenital heart disease. Methods: We conducted a retrospective study of adults with congenital heart disease that underwent cardiac catheterization and echocardiogram at Mayo Clinic (2003–2019). IVC diameter was measured at inspiration (IVC min ) and end-expiration (IVC max ), and IVC collapsibility index (IVC CI ) was calculated. Results: Based on 918 patients, we observed a good correlation between IVC max and invasive RAP ( r =0.56, P <0.001); IVC min and RAP ( r =0.58, P <0.001); and IVC CI ( r =−0.72, P <0.001). There was excellent correlation between invasive RAP and estimated RAP using IVC CI ( r =0.80, P <0.001). We observed that IVC CI <60% had superior diagnostic performance as compared with American Society of Echocardiography criteria (IVC max >2.1 cm, area under the curve difference 0.15, P <0.001; IVC CI <50%, area under the curve difference 0.09, P =0.008; combination of IVC max >2.1 cm; and IVC CI <50%, area under the curve difference 0.06, P =0.02). Estimated RAP >10 mm Hg based on IVC CI had comparable prognostic performance as invasive RAP but superior prognostic performance as the American Society of Echocardiography criteria. Conclusions: IVC CI <60% was the best criterion to identify patients with elevated RAP. IVC CI was comparable to invasively measured RAP in its relation to prognosis. Further studies are required to determine whether the use of IVC CI in clinical decision-making will improve clinical outcomes in this population.

The purpose of this study was to describe procedural outcomes, hemodynamic improvement, regression of left ventricular (LV) mass hypertrophy, and cardiovascular. The primary outcomes were procedural complications, reinterventions, and hemodynamic improvement after coarctation of aorta (COA) repair. The secondary outcomes were improvement in the severity of hypertension, regression of LV mass index, and incidence of cardiovascular events (atrial fibrillation, ventricular tachycardia, heart failure hospitalization, and cardiovascular death) after COA repair. Secondary outcomes were assessed only in patients with isolated COA who had clinical and imaging follow-up at 1 year and 3 years postintervention. Of 172 patients that underwent COA repair (surgical 161; transcatheter 11), there were no procedural deaths, and all patients had residual COA gradient <20 mm Hg. Of 128 patients that met criteria for secondary outcomes assessment, 39 (36%) had a reduction in the intensity of antihypertension therapy, and cardiovascular events occurred in 16 (13%) patients. There was no significant reduction in the overall prevalence of hypertension (stage 1 and stage 2) over time (78% versus 70% versus 73%, P =0.4 at baseline, 1 year and 3 years). Postintervention hypertension (both stage 1 and 2) were independent risk factors for suboptimal left ventricular mass index regression and cardiovascular events. Persistent hypertension was common after repair of native COA in adults and was associated with suboptimal left ventricular mass index regression and cardiovascular events. These results suggest that optimal blood pressure control with medical therapy after COA repair may result in improved clinical outcomes.

AbstractObjectivesThe purpose of this study was to investigate the influence of simulated reduced‐dose three‐dimensional angiography (3DA) on the accuracy and precision of linear measurements derived from 3DA datasets.BackgroundThree‐dimensional angiography is performed during X‐ray guided interventional procedures to aid diagnosis and inform treatment strategies for children and adults with congenital heart disease. However, 3DA contributes substantially to patient radiation dose and may lead to an increased radiation‐induced cancer risk.MethodsReduced‐dose patient 3DA images were simulated by adding quantum noise to the 2D projection angiograms, then reconstructing the projection angiograms into the 3DA dataset. Dose reduction in the range 33–72% was simulated. Five observers performed 46 vessel diameter measurements along prespecified axes within 23 vessel segments from 11 patient 3DA datasets. Statistical tests were performed to assess the influence of radiation dose reduction on the accuracy and precision of vessel diameter measurements.ResultsVessel diameter measurements were in the range 5.9– 22.7 mm. Considering all vessel segments and observers, the influence of dose level on the accuracy of diameter measurements was in the range 0.02 – 0.15 mm (p .05–.8). Interobserver variability increased modestly with vessel diameter, but was not influence by dose level (p = .52). The statistical test for observer recall bias was negative (p = .51).ConclusionsSimulated dose reduction up to 72% did not affect the accuracy or precision of the diameter measurements acquired from 3DA images. These findings may embolden 3DA radiation dose reduction for pediatric and congenital heart disease patients.

AbstractAntegrade transseptal approach was utilized in the first human case of transcatheter aortic valve replacement (TAVR) and in the early phase of TAVR. Various challenges with the antegrade transseptal approach including procedural complexity, need for atrial septal crossing, and potential for injury to the mitral valve apparatus led it to being supplanted by other approaches. These challenges have now largely been mitigated as structural interventionalists routinely perform left atrial procedures. We report a case of antegrade transseptal TAVR using a large bore sheath placed in the mid left ventricle across the mitral orifice to protect the mitral valve apparatus and facilitate valve deployment. An 84‐year‐old man with heart failure symptoms was seen for severe aortic stenosis. The severity of peripheral arterial disease precluded femoral, axillary, carotid, or transcaval routes. After transseptal puncture and creation of an arteriovenous rail, a long 26‐Fr sheath was advanced from the right femoral vein transseptally over the arteriovenous rail, past the mitral valve inflow to the mid left ventricular cavity. The sheath provided a stable platform with stable intraprocedure hemodynamics. Balloon valvuloplasty was performed in an antegrade manner, after which a 29‐mm SAPIEN S3 prosthesis was advanced into the aortic valve position and deployed under rapid pacing. We observed no injury to the mitral leaflets or subvalvular apparatus after the procedure. The antegrade transseptal approach should be revisited as an option for patients at high surgical risk with no other suitable access site. The use of a large bore sheath facilitates this approach.

AbstractObjectivePerforate the expanded polytetrafluoroethylene membrane of the GORE® CARDIOFORM Septal Occluder (GSO) and GORE® CARDIOFORM ASD Occluder (GCA) after implantation.BackgroundPercutaneous transseptal access to the left atrium is necessary for many structural and electrophysiological procedures. The potential need to access the left atrium may influence decision‐making for patent foramen ovale or atrial septal defect closure.MethodsSixteen canines underwent implantation of equal number GSO or GCA devices. A transseptal crossing procedure was performed through the device 85 (±1) days postoccluder implantation. The crossing procedure was performed utilizing commercially available equipment: radiofrequency/SureFlex sheath and standard needle/Mullin's sheath. Progressive dilation of the perforation was performed to allow passage of a 12 French Mullin's sheath into the left atrium.ResultsLeft atrial access was achieved in all cases. Postmortem analysis demonstrated passage through both occluder discs in all radiofrequency/SureFlex sheath cases (4 GSO, 4 GCA) and half of the standard needle/Mullin's sheath cases (3 GSO, 1 GCA). The remaining standard needle/Mullin's sheath cases demonstrated perforation through the right atrial disc but passage around the septal aspect of the left atrial disc, thus not perforating the left atrial disc. No acute embolic complications from the procedure were observed.ConclusionsLeft atrial access may be achieved through the GSO or GCA devices after implantation and endothelialization. The combination of a radiofrequency needle and steerable sheath provides benefit over a standard needle and Mullin's sheath in accomplishing passage through both occluder discs.

ObjectivesTo develop a novel delivery technique to overcome vascular size limitations for device closure of a secundum ASD in the young patient.BackgroundThe efficacy, clinical utility, and safety of transcatheter closure in comparison to surgical closure are well established; however, access vessel size remains a potential limitation to device selection in the young patient.MethodsA retrospective medical record review of all cases of secundum ASD closure between April 2015 and April 2016 utilizing a novel delivery method described herein at two separate academic institutions. The device is disassembled allowing the delivery sheath to serve as the introducer. It is advanced to the RA with re‐introduction of the device allowing for deployment in the standard fashion.ResultsOverall, 10 patients underwent secundum ASD closure via this novel delivery technique. There were six females (60%) and the average age at time of the procedure was 4.2 years ± 1.6 years (range 2–6 years). The majority of patients had an isolated secundum ASD (70%) with the primary indication for closure being right ventricular volume overload (90%). All patients had successful placement of a GORE® CARDIOFORM® Septal Occluder (GCSO) with no or trivial residual shunt. No patients had vascular complications related to the procedure.ConclusionsWe describe a novel technique for ASD closure using the GCSO delivery sheath as the access sheath, which reduces the vascular access size requirement by 25%, thus addressing one common limitation for percutaneous device closure of a secundum ASD in young patients. © 2016 Wiley Periodicals, Inc.

Background— Targeted postmortem genetic testing of the 4 major channelopathy-susceptibility genes ( KCNQ1 , KCNH2 , SCN5A , and RYR2 ) have yielded putative pathogenic mutations in ≤30% of autopsy-negative sudden unexplained death in the young (SUDY) cases with highest yields derived from the subset of exertion-related SUDY. Here, we evaluate the role of whole-exome sequencing in exertion-related SUDY cases. Methods and Results— From 1998 to 2010, 32 cases of exertion-related SUDY were referred by Medical Examiners for a cardiac channel molecular autopsy. A mutational analysis of the major long-QT syndrome–susceptibility genes ( KCNQ1 , KCNH2 , and SCN5A ) and catecholaminergic polymorphic ventricular tachycardia–susceptibility gene ( RYR2 ) identified a putative pathogenic mutation in 11 cases. Whole-exome sequencing was performed on the remaining 21 targeted gene–negative SUDY cases. After whole-exome sequencing, a gene-specific surveillance of all genes (N=100) implicated in sudden death was performed to identify putative pathogenic mutation(s). Three of these 21 decedents had a clinically actionable, pathogenic mutation (CALM2-F90L, CALM2-N98S, and PKP2-N634fs). Of the 18 remaining cases, 7 hosted at least 1 variant of unknown significance with a minor allele frequency <1:20 000. The overall yield of pathogenic mutations was higher among decedents aged 1 to 10 years (10/11, 91%) than those aged 11 to 19 years (4/21, 19%, P =0.0001). Conclusions— Molecular screening in this clinical scenario is appropriate with a pathogenic mutation detection rate of 44% using direct DNA sequencing followed by whole-exome sequencing. Only 5 of the 100 interrogated sudden death genes hosted actionable pathogenic mutations for more than one third of these exertion-related, autopsy-negative SUDY cases.

AbstractSVC obstruction is a rare acute complication after bicaval cardiac transplantation and can be catastrophic for the patient. Herein, we report the case of a child who developed SVC thrombosis causing acute SVC syndrome nine days after heart transplantation. Thrombolysis was successfully performed using the AngioJet™ system and subsequent balloon angioplasty with re‐establishment of flow. This procedure allowed for revascularization without repeat sternotomy or stent implantation. The patient is well without recurrence at last follow‐up.