Optimizing 3D Rotational Angiography for Congenital Cardiac Catheterization.

The aim of the study was to determine the variables associated with high-quality (HQ) versus low-quality (LQ) three-dimensional rotational angiography (3DRA) and create guides for optimization of approach to 3DRA in congenital cardiac catheterization (CCC). CCC has adopted 3DRA as a mainstay, but there has not been systematic analysis of approach to and factors associated with HQ 3DRA. This was a single-center, retrospective study of 3DRAs using Canon Infinix-I platform. Reconstructions were graded by 3 interventionalists. Quality was dichotomized into HQ and LQ. Univariable analyses and multivariable logistic regression models were performed. From 8/2016 to 12/2018, 208 3DRAs were performed in 195 CCCs; median age 7 years (2, 16), weight 23 kg (12, 57). The majority of 3DRAs were performed in patients with biventricular physiology (N = 137, 66%) and in pulsatile sites (N = 144, 69%). HQ 3DRA (N = 182, 88%) was associated with greater total injection volume [2.20 mL/kg (1.44, 3.29) vs. 1.62 mL/kg (1.10, 1.98), p = 0.005] and more dilute contrast solution [60% (50, 100) vs. 100% (60, 100), p = 0.007], but not with contrast volume administered (p = 0.2) on univariable analysis. On multivariable logistic regression, HQ 3DRA was significantly associated with patient weight [OR 0.97 (95% CI (0.94, 0.99), p = 0.018], total injection volume [OR 1.04 (95% CI 1.01, 1.07) p = 0.011], and percent contrast solution [OR 0.97 (95% CI 0.95, 1.00), p = 0.022]. These data resulted in creation of scatter plots and a novel 3DRA Nomogram for estimating the probability of HQ 3DRA. This is the first study to create evidence-based contrast dose guides and nomogram for 3DRA in CCC. HQ 3DRA was associated with lower weight, higher total injection volumes, and more dilute contrast solution.

Tissue engineered vascular grafts transform into autologous neovessels capable of native function and growth.

Background: Tissue-engineered vascular grafts (TEVGs) have the potential to advance the surgical management of infants and children requiring congenital heart surgery by creating functional vascular conduits with growth capacity. Methods: Herein, we used an integrative computational-experimental approach to elucidate the natural history of neovessel formation in a large animal preclinical model; combining an in vitro accelerated degradation study with mechanical testing, large animal implantation studies with in vivo imaging and histology, and data-informed computational growth and remodeling models. Results: Our findings demonstrate that the structural integrity of the polymeric scaffold is lost over the first 26 weeks in vivo, while polymeric fragments persist for up to 52 weeks. Our models predict that early neotissue accumulation is driven primarily by inflammatory processes in response to the implanted polymeric scaffold, but that turnover becomes progressively mechano-mediated as the scaffold degrades. Using a lamb model, we confirm that early neotissue formation results primarily from the foreign body reaction induced by the scaffold, resulting in an early period of dynamic remodeling characterized by transient TEVG narrowing. As the scaffold degrades, mechano-mediated neotissue remodeling becomes dominant around 26 weeks. After the scaffold degrades completely, the resulting neovessel undergoes growth and remodeling that mimicks native vessel behavior, including biological growth capacity, further supported by fluid-structure interaction simulations providing detailed hemodynamic and wall stress information. Conclusions: These findings provide insights into TEVG remodeling, and have important implications for clinical use and future development of TEVGs for children with congenital heart disease.

Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial.

BACKGROUND: The Melody valve was developed to extend the useful life of previously implanted right ventricular outflow tract (RVOT) conduits or bioprosthetic pulmonary valves, while preserving RV function and reducing the lifetime burden of surgery for patients with complex congenital heart disease. METHODS: Enrollment for the US Investigational Device Exemption study of the Melody valve began in 2007. Extended follow-up was completed in 2020. The primary outcome was freedom from transcatheter pulmonary valve (TPV) dysfunction (freedom from reoperation, reintervention, moderate or severe pulmonary regurgitation, and/or mean RVOT gradient >40 mm Hg). Secondary end points included stent fracture, catheter reintervention, surgical conduit replacement, and death. RESULTS: One hundred seventy-one subjects with RVOT conduit or bioprosthetic pulmonary valve dysfunction were enrolled. One hundred fifty underwent Melody TPV replacement. Median age was 19 years (Q1-Q3: 15-26). Median discharge mean RVOT Doppler gradient was 17 mm Hg (Q1-Q3: 12-22). The 149 patients implanted >24 hours were followed for a median of 8.4 years (Q1-Q3: 5.4-10.1). At 10 years, estimated freedom from mortality was 90%, from reoperation 79%, and from any reintervention 60%. Ten-year freedom from TPV dysfunction was 53% and was significantly shorter in children than in adults. Estimated freedom from TPV-related endocarditis was 81% at 10 years (95% CI, 69%-89%), with an annualized rate of 2.0% per patient-year. CONCLUSIONS: Ten-year outcomes from the Melody Investigational Device Exemption trial affirm the benefits of Melody TPV replacement in the lifetime management of patients with RVOT conduits and bioprosthetic pulmonary valves by providing sustained symptomatic and hemodynamic improvement in the majority of patients. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT00740870.

Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis.

BACKGROUND: There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. OBJECTIVES: This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. METHODS: Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. RESULTS: Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. CONCLUSIONS: Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Implantable pulmonary artery pressure monitoring device in patients with palliated congenital heart disease: Technical considerations and procedural outcomes.

OBJECTIVE: We describe the technical considerations of transcatheter implantation of the CardioMEMS™ HF System (Abbott, Abbott Park, IL) in adult patients with complex palliated congenital heart disease (CHD) and advanced heart failure (HF). BACKGROUND: Ambulatory pulmonary artery (PA) pressure monitoring with implantable hemodynamic monitors (IHMs) has been shown to reduce HF-related hospital admissions in non-CHD populations. HF is a common late cardiovascular complication in adult CHD necessitating better understanding of IHM application in this population. METHODS: We analyzed adults with complex CHD and advanced HF who were referred for CardioMEMS™ device implantation (2015-2018). Feasibility of device implantation, defined by successful device implantation and calibration, and procedural outcomes were evaluated. RESULTS: CardioMEMS™ was successfully implanted in all 14 adults (35.5 ± 9.2 years old, 72 ± 12 kg) with complex CHD (single ventricle/Fontan, n = 8 [57%]; d-transposition of the great arteries/atrial switch, n = 6 [43%]). The device was delivered via femoral venous access in 13 (93%) patients and implanted in the left PA in 12 (86%). A long sheath was used in 8 (57%) patients, including 5/6 with an atrial switch operation. There was one device migration that did not require retrieval. CONCLUSIONS: Transcatheter implantation of an IHM is feasible in select complex adult CHD patients with advanced HF. Further studies evaluating integration of ambulatory hemodynamics and the impact on clinical care are needed. This technology has the potential to improve medical management of advanced HF in patients with Fontan and atrial switch physiologies and provide new insights into their ambulatory hemodynamics.

Use of the Gore Viabahn VBX balloon-expandable endoprosthesis in the congenital heart disease population.

The Gore Viabahn VBX balloon-expandable endoprosthesis (W. L. Gore & Associates, Flagstaff, AZ) is a flexible covered stent that is FDA-approved for the treatment of iliac artery stenosis, including lesions at the aortic bifurcation. In this case series, we report the first use of the VBX covered stent in congenital heart disease, highlighting several of its unique advantages.

Intravascular Ultrasound Characterization of a Tissue-Engineered Vascular Graft in an Ovine Model.

Patients who undergo implantation of a tissue-engineered vascular graft (TEVG) for congenital cardiac anomalies are monitored with echocardiography, followed by magnetic resonance imaging or angiography when indicated. While these methods provide data regarding the lumen, minimal information regarding neotissue formation is obtained. Intravascular ultrasound (IVUS) has previously been used in a variety of conditions to evaluate the vessel wall. The purpose of this study was to evaluate the utility of IVUS for evaluation of TEVGs in our ovine model. Eight sheep underwent implantation of TEVGs either unseeded or seeded with bone marrow-derived mononuclear cells. Angiography, IVUS, and histology were directly compared. Endothelium, tunica media, and graft were identifiable on IVUS and histology at multiple time points. There was strong agreement between IVUS and angiography for evaluation of luminal diameter. IVUS offers a valuable tool to evaluate the changes within TEVGs, and clinical translation of this application is warranted.

Aortic root thrombosis with coronary embolization following neo-aortic reconstruction in a child with hypoplastic left heart syndrome.

In the recent era, the diagnosis, treatment options, postoperative management and outcomes of infants born with hypoplastic left heart syndrome (HLHS) have undergone dramatic changes. As is the case with many other novel treatment modalities used for congenital heart diseases, data concerning the long-term outcomes and complications of the various strategies become gradually more available as the numbers of survivors grow. In general, complications of the three-stage surgical palliation used for HLHS tend to occur most commonly following the first-stage surgery. Post-stage 2 complications are substantially less common, and centre on the procedure itself and the unique physiology of the cavopulmonary connection. In the following case report, we describe a relatively rare adverse outcome that occurred following a stage 2 surgery in the form of native aortic root thrombosis extending to the coronary arteries. The selected methods of treatment used in the catheterization laboratory and later in the operating theatre, as well as its outcomes are described.

The use of three-dimensional rotational angiography to assess the pulmonary circulation following cavo-pulmonary connection in patients with single ventricle.

BACKGROUND: Bi-plane angiography is the gold standard for imaging the pulmonary arteries (PAs) after cavo-pulmonary connection (CPC). Three-dimensional rotational angiography (3DRA) is emerging technology that we hypothesized may enhance diagnostic capabilities in this patient group. METHODS: Retrospective review of patients who underwent 3DRA to assess the PAs after CPC. 3DRA images were examined for diagnostic quality and compared, both qualitatively and quantitatively with corresponding 2D images. RESULTS: Between 1/2010 and 12/2010, 37 3DRAs were obtained in 32 pts with prior CPC. Median age and weight were 4.3 years (0.3-19) and 15.7 kg (4.3-114). Previous surgery included: bi-directional Glenn (16), bilateral bi-directional Glenn (2), and Fontan (14). Thirty-three (89%) acquisitions in 28 pts were of diagnostic quality. In 19/28 pts (68%) 3DRA provided significant additional information pertaining to 22 lesions when compared with bi-plane angiography. These included delineating the mechanism of PA stenosis in 13 (aortic compression: 7, PA torsion: 6), demonstrating a discrete PA stenosis not seen or under appreciated on corresponding 2D images in 9. This additional information led to the performance of 9 interventions (stent implantation 8, angioplasty 1). Corresponding 2D and 3DRA images of the right and left PA were compared using quantitative analysis with a correlation coefficient of 0.94 (P < 0.001) and 0.97 (P < 0.001), for the right and left PAs respectively. CONCLUSIONS: 3DRA can be performed safely and effectively following CPC and may be more sensitive in diagnosing PA stenoses than 2D angiography. Quantitative measurements of PA diameter using 3DRA correlates well with 2D angiography.

Case Report of a 2-year-old boy with Takayasu's arteritis: an atypical, severe presentation of a rare disease.

Takayasu's arteritis is a granulomatous giant-cell arteritis of the aorta and its major branches. It primarily affects East Asian women in their second or third decade of life but is well known to affect all ethnicities across the world. Given its systemic nature, Takayasu's arteritis has multiorgan involvement, with the majority of disease morbidity related to the cardiovascular, central nervous, and renal systems. This report describes an unusual presentation of Takayasu's arteritis in a 2½-year-old boy. This is the first report in the literature describing the presence of both severe aortic regurgitation and diffuse coronary artery involvement in a patient so young.

Prenatal screening for major congenital heart disease: superiority of outflow tracts over the 4-chamber view.

OBJECTIVE: The purpose of this study was to determine the relative importance of the 4-chamber view (4CV) compared with the outflow tract views (OFTVs) in prenatal screening for major congenital heart disease (CHD). METHODS: We prospectively evaluated 200 consecutive infants undergoing cardiac surgery at our institution for major CHD. By reviewing the infants' medical records and conducting bedside interviews with their parents or guardians, we evaluated detection rates both prenatally and postnatally (before and after discharge to home), and we noted any prenatally identifiable risk factors for CHD. For each infant, we determined whether the 4CV or OFTVs would be expected to have been normal or abnormal on routine midgestation screening fetal sonography. RESULTS: A prenatal diagnosis of CHD was made in 65 infants (33%): 30 of 124 low-risk pregnancies (24%) and 35 of 76 high-risk pregnancies (46%). An abnormal screening midgestation 4CV would have been expected in up to 63% of the infants, whereas abnormal midgestation OFTVs would have been expected in up to 91% of the infants. Thus, the potential sensitivity for detecting major CHD was higher with the OFTVs than with the 4CV (91% versus 63%; P < .001). Moreover, the OFTVs were more sensitive than the 4CV for detecting ductal-dependent forms of CHD. Diagnosis after discharge to home occurred in 39 of 135 postnatal diagnoses (29%), including many cases of isolated outflow tract abnormalities requiring early invasive intervention. CONCLUSIONS: Cases of major neonatal CHD with OFTV abnormalities predominate over cases with 4CV abnormalities, particularly among those forms of CHD requiring early invasive intervention.