Faculty Bibliography

Aortic wall complications can occur in unrepaired aortic coarctation (CoA) and after surgical repair or endovascular treatment. This review summarizes the available literature and current understanding of aortic wall injury (AWI) surrounding the management of CoA, focusing specifically on acute and follow-up AWI after endovascular treatment. There have been 23 reported cases of aortic rupture after endovascular treatment for CoA, including angioplasty alone, bare metal stenting, and primary covered stent therapy. Even if these published cases represent only a minority of ruptures that have actually occurred, the incidence is substantially <1%. The incidence of acute aneurysm formation was 0% to 13% after angioplasty, 0% to 5% after bare metal stent placement, and <1% after covered stent placement. The reported incidence and natural history of both acute and new AWI during follow-up after endovascular therapy for CoA varies considerably, likely secondary to ascertainment and reporting biases and inconsistent definitions. Although important AWI after endovascular treatment of CoA seems to be declining in frequency with increasing experience and improving technology, it remains one of the most important potential adverse outcomes. Long-term surveillance for new AWI and monitoring of existing AWI is mandatory, with institution of appropriate treatment when necessary. A central research focus in this population should be determination of the appropriate treatment for both native and recurrent CoA across various ages with regard to limiting recurrent CoA and preventing associated aortic wall complications, in addition to determining the appropriate treatment of various AWI. Consistent definitions and reporting are necessary to truly understand the incidence of, risk factors for, and measures protective against AWI after angioplasty or stent implantation for CoA.

BACKGROUND:Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. METHODS AND RESULTS/RESULTS:Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5-21.0; P<0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1-1.5; P<0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4-6.2; P<0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1-6.0; P=0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P<0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P<0.001). CONCLUSION/CONCLUSIONS:In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

The aim is to compare tricuspid valve (TV) atrioventricular junction (AVJ) annular motion parameters in unrepaired atrial septal defect (ASD) and repaired Tetralogy of Fallot (TOF) by cardiac magnetic resonance (CMR) imaging. We retrospectively reviewed CMR studies performed between November 2007 and November 2013 in patients 16-45 years of age with unrepaired ASD (with or without partial anomalous pulmonary venous return) and with repaired TOF, who had previous infundibulotomy, but have not undergone pulmonary valve replacement. Longitudinal motion of lateral TV in four-chamber view cine image was tracked through the cardiac cycle with custom software. Twenty TOF patients and 12 ASD patients were included, and values were compared with 80 controls. Right ventricular end-diastolic volume index and right ventricular end-systolic volume index were similar in the ASD and TOF groups and were significantly higher in both groups than in controls. Maximum displacement of the TV in systole, velocity at half-maximal displacement during systole, and velocity at half-maximal displacement during early diastole were all significantly lower in the TOF group than the ASD group [1.39 +/- 0.47 vs. 2.21 +/- 0.46 (cm, p < 0.01), 5.9 +/- 2.1 vs. 10.1 +/- 2.3 (cm/s, p < 0.01), and 7.7 +/- 2.6 vs. 10.9 +/- 3.1 (cm/s, p < 0.05)]. TOF patients have diminished early diastolic TV AVJ velocity compared to patients with an unrepaired ASD, despite similar RV volumes. This observation could suggest diastolic dysfunction or cardiac mechanics unique to the postoperative, volume-overloaded right ventricle in patients with repaired TOF.

Background: Complex muscular ventricular septal defects (CMVSD) are often difficult to surgically close and managed by device closure. The pre-intervention imaging is crucial in defining the anatomy and aids in patient selection. We hypothesized that 3D physical and virtual models in patients with CMVSD is feasible, would assist in patient selection and aid in the successful device closure. Methods: Virtual and physical 3D models on 3 patients with CMVSD were generated from CT or MRI data, using Mimics, and 3-Matic software. The first patient had history of complicated and unsuccessful prior device closures, with residual shunt. Two physical models, with and without devices in situ were printed (Figure 1A) for this patient. Two virtual models were generated in the other two patients. Results: The location, size, trabeculations, papillary and muscle bundles were clearly visualized in all patients. The two physical models were extensively studied, resulting in successful device closure of the residual VSD. The virtual model on patient 2 identified RV papillary muscles adjacent to the CMVSD (Figure 1B) precluding device closure. The patient 3 model identified muscle bundles crowding the VSD suggesting potential for spontaneous closure. Conclusion: Construction of 3D models in patients with CMVSD is feasible, assists in appropriate patient selection and allows for extensive examination and planning. This may facilitate a focused and informed procedure and improve the potential for successful closure. (Figure Presented)

Sinus of Valsalva aneurysm is a rare cardiac malformation that stems from incomplete fusion of the aortic media and the aortic valve annulus, a weakness that may result in rupture of the sinus, large left-to-right shunt, and severe congestive heart failure. Historically, this lesion has been repaired surgically, but percutaneous closure (PC) has emerged as a therapeutic intervention over the last 20 years. We review and contrast 34 studies detailing the PC approach with 16 studies on surgical closure (SC), together comprising a total of 877 patients who were treated for ruptured sinus of Valsalva aneurysm from 1956 to 2014. Both groups had similar sites of rupture, age distribution, and clinical symptoms at presentation. Selection bias ultimately prohibits a direct comparison between the 2 groups as patients who underwent SC often had worse aortic regurgitation and more complex associated lesions, including endocarditis, bicuspid aortic valve, tunnel-type fistulous connections, larger defect size, and multiple site of rupture. In conclusion, although SC is indicated and reserved for these more complicated patients, our review of previously published reports reveals that PC in patients who are too ill to undergo bypass, with mild or no aortic regurgitation and simple associated defects (muscular ventricular septal defects, secundum atrial septal defect, small patent ductus arteriosus), can be safe, effective, and practical.

OBJECTIVES: Left atrial decompression using cardiac catheterization techniques has been described at centers with extracorporeal membrane oxygenation programs. Left atrial decompression can decrease cardiogenic edema, minimize ventricular distension, and allow myocardial recovery. We describe Boston Children's Hospital's experience with percutaneous left atrial decompression techniques, acute outcomes, and clinical impact of left atrial decompression in extracorporeal membrane oxygenation patients. SUBJECTS: Patients supported with extracorporeal membrane oxygenation undergoing percutaneous left atrial decompression were identified and assigned to two groups 1) myocarditis/suspected myocarditis or 2) nonmyocarditis cardiac disease. INTERVENTIONS: Three techniques including vent placement, static balloon dilation, and stent implantation were used. MEASUREMENTS AND MAIN RESULTS: Change in left atrial pressure and severity of pulmonary edema on chest radiography pre and post procedure, impact of timing and technique of left atrial decompression on resolution of left atrial hypertension, and extracorporeal membrane oxygenation survival were evaluated. Furthermore, we evaluated the presence of residual atrial septal defect during follow-up. Percutaneous left atrial decompression was performed in 44 of 419 extracorporeal membrane oxygenation cases (10.5%) and was frequently used for myocarditis (22 of 44 patients; 50%). Techniques included 25 vents, 17 static balloon dilations, and two stents. All techniques were equally successful and significantly reduced left atrial pressure and pulmonary edema. Survival to hospital discharge was not associated with extracorporeal membrane oxygenation duration prior to left atrial decompression, change in left atrial pressure, or technique used. Persistent atrial septal defect was noted in five surviving patients (excluding transplant recipients and deceased), two required closure. CONCLUSIONS: Left atrial decompression can be performed effectively in children on extracorporeal membrane oxygenation using various percutaneous techniques. Reduction in pulmonary venous congestion is usually evident by chest radiography within 48 hours of intervention. Persistent atrial septal defect may require closure at the time of extracorporeal membrane oxygenation decannulation or during long-term follow-up.

Background: A recent multi-center study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD) found that 1/3rd of live-born patients (pts) died prior to hospital discharge. The purpose of this study was to explore differences in postnatal management and the relationship to outcome. Methods: This 23-center, retrospective study included 243 fetuses with EA/TVD from 2005 to 2011. Neonatal procedure (NP) was defined as surgery or interventional catheterization (cath) prior to discharge. Associations between postnatal management and outcome at discharge were explored. Results: Of 176 live-born pts, 7 received comfort care only, 11 died <24 hrs of life, and 4 had insufficient data. Among 154 remaining pts, 38 (25%) did not survive to discharge. Pts who required ECMO at any point (n=18) had 83% mortality. More than half of pts (54%) did not have an NP, 34% had surgery, 8% had interventional cath, and 4% had both. The median age at 1st NP was 6 days (quartiles: 1-11). Survival did not differ between pts who had an NP and those who did not (70% vs. 80%; p=0.19) or between pts who had surgery and those who did not (68% vs. 80%; p=0.09). However, mortality differed by NP performed and whether pulmonary regurgitation, an indicator of high risk, was present prenatally (Figure). No pts with a right ventricular exclusion (RVE) died. Of 49 surviving neonates with >1 procedure, 28 (57%) were palliated with a shunt or RVE and 21 (43%) had a biventricular circulation. Thus, in total, 86 of 154 live-born pts (56%) survived with a biventricular circulation: 65 with medical management only and 21 with >1 NP. Conclusion: Among live-born pts diagnosed with EA/TVD in utero, a variety of postnatal management strategies were employed with overall poor outcomes. If surgery beyond PDA ligation is necessary, then RVE or other palliative procedure may need to be considered. A prospective, multi-center study utilizing a management algorithm would help elucidate the optimal strategy

OBJECTIVE: Isolated pulmonary artery of ductal origin (IPADO) is a rare disease with diverse presentations. Diagnostic and therapeutic approaches vary widely given the low incidence. Reporting additional cases may help inform medical decision making. METHODS: We reviewed diagnostic data-including echocardiography, catheterization, and MRI-and outcomes for all patients with IPADO at our institution. RESULTS: Thirty-seven patients who met inclusion criteria were identified. The mean age at diagnosis was 3 months (range, 1 day-45 years). In 11 patients, the duct supplying the IPADO was patent (ie, patent ductus arteriosis [PDA]); the duct had closed (ligamentum) in the other 26 patients. When performed, catheterization delineated the anatomy in 90% (100% if PDA) versus 54% with magnetic resonance imaging. Patients with a PDA were more likely to undergo intervention (100% vs 58%, P = .02) and had earlier first intervention (1 vs 20 months; P < .001). Patients diagnosed at age