Effect of graft sizing in valve-sparing aortic root replacement for bicuspid aortic valve: The Goldilocks ratio.

Objective: To investigate the effect of graft sizing on valve performance in valve-sparing aortic root replacement for bicuspid aortic valve. Methods: In addition to a diseased control model, 3 representative groups-free-edge length to aortic/graft diameter (FELAD) ratio <1.3, 1.5 to 1.64, and >1.7-were replicated in explanted porcine aortic roots (n = 3) using straight grafts sized respective to the native free-edge length. They were run on a validated ex vivo univentricular system under physiological parameters for 20 cycles. All groups were tested within the same aortic root to minimize inter-root differences. Outcomes included transvalvular gradient, regurgitation fraction, and orifice area. Linear mixed effects model and pairwise comparisons were employed to compare outcomes across groups. Results: The diseased control had mean transvalvular gradient 10.9 ± 6.30 mm Hg, regurgitation fraction 32.5 ± 4.91%, and orifice area 1.52 ± 0.12 cm2. In ex vivo analysis, all repair groups had improved regurgitation compared with control (P < .001). FELAD <1.3 had the greatest amount of regurgitation among the repair groups (P < .001) and 1.5-1.64 the least (P < .001). FELAD <1.3 and >1.7 exhibited greater mean gradient compared with both control and 1.5 to 1.64 (P < .001). Among the repair groups, 1.5 to 1.64 had the largest orifice area, and >1.7 the smallest (P < .001). Conclusions: For a symmetric bicuspid aortic valve, performance after valve-sparing aortic root replacement shows a bimodal distribution across graft size. As the FELAD ratio departs from 1.5 to 1.64 in either direction, significant increases in transvalvular gradient are observed. FELAD <1.3 may also result in suboptimal improvement of baseline regurgitation.

Uncommon presentations of type A quadricuspid aortic valve in the Septuagenarian.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital anomaly characterized by the presence of four cusps instead of the usual three. It is estimated to occur in less than 0.05% of the population, with Type A (four equal-sized leaflets) accounting for roughly 30% of QAV subtypes. Based on limited clinical series, the usual presentation is progressive aortic valve regurgitation (AR) with symptoms occurring in the fourth to sixth decade of life. Severe aortic valve stenosis (AS) and acute AR are very uncommon. CASE PRESENTATION: We describe two cases of Type A QAV in patients who remained asymptomatic until their seventies with very uncommon presentations: one with severe AS and one with acute, severe AR and flail leaflet. In Case A, a 72-year-old patient with history of moderate AS presents to clinic with progressive exertional dyspnea. During work-up for transcatheter vs. surgical replacement pre-operative computed tomography angiogram (CTA) reveals a quadricuspid aortic valve with severe AS, and the patient undergoes surgical aortic valve replacement. Pre-discharge transthoracic echocardiography (TTE) shows good prosthetic valve function with no gradient or regurgitation. In Case B, a 76-year-old patient is intubated upon arrival to the hospital for acute desaturation, found to have wide open AR on catheterization, and transferred for emergent intervention. Intraoperative TEE reveals QAV with flail leaflet and severe AR. Repair is considered but deferred ultimately due to emergent nature. Post-operative TTE demonstrates good prosthetic valve function with no regurgitation and normal biventricular function. CONCLUSIONS: QAV can present as progressive severe AS and acute AR, with symptoms first occurring in the seventh decade of life. The optimal treatment for QAV remains uncertain. Although aortic valve repair or transcatheter option may be feasible in some patients, aortic valve replacement remains a tenable option.

Simulation-based design of bicuspidization of the aortic valve.

OBJECTIVE: Severe congenital aortic valve pathology in the growing patient remains a challenging clinical scenario. Bicuspidization of the diseased aortic valve has proven to be a promising repair technique with acceptable durability. However, most understanding of the procedure is empirical and retrospective. This work seeks to design the optimal gross morphology associated with surgical bicuspidization with simulations based on the hypothesis that modifications to the free edge length cause or relieve stenosis. METHODS: Model bicuspid valves were constructed with varying free edge lengths and gross morphology. Fluid-structure interaction simulations were conducted in a single patient-specific model geometry. The models were evaluated for primary targets of stenosis and regurgitation. Secondary targets were assessed and included qualitative hemodynamics, geometric height, effective height, orifice area, and billow. RESULTS: Stenosis decreased with increasing free edge length and was pronounced with free edge length less than or equal to 1.3 times the annular diameter d. With free edge length 1.5d or greater, no stenosis occurred. All models were free of regurgitation. Substantial billow occurred with free edge length 1.7d or greater. CONCLUSIONS: Free edge length 1.5d or greater was required to avoid aortic stenosis in simulations. Cases with free edge length 1.7d or greater showed excessive billow and other changes in gross morphology. Cases with free edge length 1.5d to 1.6d have a total free edge length approximately equal to the annular circumference and appeared optimal. These effects should be studied in vitro and in animal studies.

SDF4CHD: Generative Modeling of Cardiac Anatomies with Congenital Heart Defects.

Congenital heart disease (CHD) encompasses a spectrum of cardiovascular structural abnormalities, often requiring customized treatment plans for individual patients. Computational modeling and analysis of these unique cardiac anatomies can improve diagnosis and treatment planning and may ultimately lead to improved outcomes. Deep learning (DL) methods have demonstrated the potential to enable efficient treatment planning by automating cardiac segmentation and mesh construction for patients with normal cardiac anatomies. However, CHDs are often rare, making it challenging to acquire sufficiently large patient cohorts for training such DL models. Generative modeling of cardiac anatomies has the potential to fill this gap via the generation of virtual cohorts; however, prior approaches were largely designed for normal anatomies and cannot readily capture the significant topological variations seen in CHD patients. Therefore, we propose a type- and shape-disentangled generative approach suitable to capture the wide spectrum of cardiac anatomies observed in different CHD types and synthesize differently shaped cardiac anatomies that preserve the unique topology for specific CHD types. Our DL approach represents generic whole heart anatomies with CHD type-specific abnormalities implicitly using signed distance fields (SDF) based on CHD type diagnosis, which conveniently captures divergent anatomical variations across different types and represents meaningful intermediate CHD states. To capture the shape-specific variations, we then learn invertible deformations to morph the learned CHD type-specific anatomies and reconstruct patient-specific shapes. Our approach has the potential to augment the image-segmentation pairs for rarer CHD types for cardiac segmentation and generate cohorts of CHD cardiac meshes for computational simulation.

Simulation-Based Design of Bicuspidization of the Aortic Valve.

OBJECTIVE: Severe congenital aortic valve pathology in the growing patient remains a challenging clinical scenario. Bicuspidization of the diseased aortic valve has proven to be a promising repair technique with acceptable durability. However, most understanding of the procedure is empirical and retrospective. This work seeks to design the optimal gross morphology associated with surgical bicuspidization with simulations, based on the hypothesis that modifications to the free edge length cause or relieve stenosis. METHODS: Model bicuspid valves were constructed with varying free edge lengths and gross morphology. Fluid-structure interaction simulations were conducted in a single patient-specific model geometry. The models were evaluated for primary targets of stenosis and regurgitation. Secondary targets were assessed and included qualitative hemodynamics, geometric height, effective height, orifice area and prolapse. RESULTS: Stenosis decreased with increasing free edge length and was pronounced with free edge length less than or equal to 1.3 times the annular diameter d. With free edge length 1.5d or greater, no stenosis occurred. All models were free of regurgitation. Substantial prolapse occurred with free edge length greater than or equal to 1.7d. CONCLUSIONS: Free edge length greater than or equal to 1.5d was required to avoid aortic stenosis in simulations. Cases with free edge length greater than or equal to 1.7d showed excessive prolapse and other changes in gross morphology. Cases with free edge length 1.5-1.6d have a total free edge length approximately equal to the annular circumference and appeared optimal. These effects should be studied in vitro and in animal studies.

Diversifying cardiac intensive care unit models: Successful example of an operating surgeon-led unit.

Objective: The intensivist-led cardiovascular intensive care unit model is the standard of care in cardiac surgery. This study examines whether a cardiovascular intensive care unit model that uses operating cardiac surgeons, cardiothoracic surgery residents, and advanced practice providers is associated with comparable outcomes. Methods: This is a single-institution review of the first 400 cardiac surgery patients admitted to an operating surgeon-led cardiovascular intensive care unit from 2020 to 2022. Inclusion criteria are elective status and operations managed by both cardiovascular intensive care unit models (aortic operations, valve operations, coronary operations, septal myectomy). Patients from the surgeon-led cardiovascular intensive care unit were exact matched by operation type and 1:1 propensity score matched with controls from the traditional cardiovascular intensive care unit using a logistic regression model that included age, sex, preoperative mortality risk, incision type, and use of cardiopulmonary bypass and circulatory arrest. Primary outcome was total postoperative length of stay. Secondary outcomes included postoperative intensive care unit length of stay, 30-day mortality, 30-day Society of Thoracic Surgeons-defined morbidity (permanent stroke, renal failure, cardiac reoperation, prolonged intubation, deep sternal infection), packed red cell transfusions, and vasopressor use. Outcomes between the 2 groups were compared using chi-square, Fisher exact test, or 2-sample t test as appropriate. Results: A total of 400 patients from the surgeon-led cardiovascular intensive care unit (mean age 61.2 ± 12.8 years, 131 female patients [33%], 346 patients [86.5%] with European System for Cardiac Operative Risk Evaluation II <2%) and their matched controls were included. The most common operations across both units were coronary artery bypass grafting (n = 318, 39.8%) and mitral valve repair or replacement (n = 238, 29.8%). Approximately half of the operations were performed via sternotomy (n = 462, 57.8%). There were 3 (0.2%) in-hospital deaths, and 47 patients (5.9%) had a 30-day complication. The total length of stay was significantly shorter for the surgeon-led cardiovascular intensive care unit patients (6.3 vs 7.0 days, P = .028), and intensive care unit length of stay trended in the same direction (2.5 vs 2.9 days, P = .16). Intensive care unit readmission rates, 30-day mortality, and 30-day morbidity were not significantly different between cardiovascular intensive care unit models. The surgeon-led cardiovascular intensive care unit was associated with fewer postoperative red blood cell transfusions in the cardiovascular intensive care unit (P = .002) and decreased vasopressor use (P = .001). Conclusions: In its first 2 years, the surgeon-led cardiovascular intensive care unit demonstrated comparable outcomes to the traditional cardiovascular intensive care unit with significant improvements in total length of stay, postoperative transfusions in the cardiovascular intensive care unit, and vasopressor use. This early success exemplifies how an operating surgeon-led cardiovascular intensive care unit can provide similar outcomes to the standard-of-care model for patients undergoing elective cardiac surgery.

Innominate artery patency after direct cannulation in neonates.

Objective: The study objective was to determine the short-term incidence of innominate artery stenosis for neonates who underwent direct innominate artery cannulation during the Norwood procedure. Methods: This is a retrospective, single-institution review of 92 patients who underwent the Norwood procedure with direct innominate artery cannulation from 2006 to 2017. The primary outcome was angiographic evidence of patency at pre-Glenn cardiac catheterization. Patient characteristics, intraoperative surgical and hemodynamic measurements, and postoperative neurologic findings were recorded. Results: At a median age of 5.0 days, 92 neonates underwent the Norwood procedure with direct innominate artery cannulation. These patients underwent cardiac catheterization at a median of 3.0 months after the index operation. In 5 of 92 patients with catheterization images available for review, there was angiographic evidence of mild innominate artery stenosis, and none had moderate or severe stenosis. Review of follow-up records did not reveal evidence of clinically significant stenosis or innominate artery reintervention. Conclusions: In neonates undergoing the Norwood procedure with direct innominate cannulation, innominate artery stenosis was uncommon and clinically significant stenosis did not occur.

Revisiting prosthesis choice in mitral valve replacement in children: Durable alternatives to traditional bioprostheses.

OBJECTIVE: To determine risk factors for re-replacement and death or transplant following mitral valve replacement (MVR) in children METHODS: This is a retrospective 26-year review of patients younger than 20 years of age undergoing MVR between 1992 and 2018 at single institution. Outcomes included freedom from re-MVR and transplant-free survival. Cox proportional hazards regression models assessed association between outcomes and potential risk factors. RESULTS: At median age 4.2 years, 190 children underwent 290 MVR: 180 mechanical, 63 porcine, 13 pericardial, and 34 stented bovine jugular vein valves. Re-MVR occurred in 100 valves. Freedom from re-MVR at 5 and 10 years was 76% and 44%. Times to re-MVR were associated with prosthesis type (P < .001), with porcine and pericardial valves at greatest risk. Other risk factors for prosthetic failure included smaller prosthesis size and left ventricular hypoplasia. There were 9 transplants and 44 deaths. Transplant-free survival at 5 and 10 years was 81% and 76%. Prosthesis type was significantly associated with time to death/transplant in univariate analysis only (P = .021), with porcine at greater risk than mechanical. Independent risk factors for death/transplant included larger indexed geometric orifice area and longer bypass time. CONCLUSIONS: In pediatric patients undergoing MVR, mechanical and stented bovine jugular vein valves were associated with increased durability compared with fixed-diameter bioprosthetic alternatives.

Three-Dimensional Modeling of Surgically Implanted Stent-Based Valves in the Mitral Position in Children.

PURPOSE: In children with a mitral annulus too small to accommodate traditional prostheses, surgical implantation of stent-based valves is a promising option. However no reliable preoperative methods exist to guide patient selection, device sizing, and positioning. We describe a novel methodology to visualize and quantify device fit in 3-dimensional echocardiogram (3DE)-derived heart models. DESCRIPTION: Heart models were created from existing preoperative 3DEs using custom software. Valve models were virtually implanted into the models, and both device fit and left ventricular outflow tract (LVOT) area were quantified. EVALUATION: The 3DEs of 3 patients who underwent Melody valve placement in the mitral position were retrospectively modeled: 1 with LVOT obstruction, 1 with perivalvar leak, and 1 without complications. In all cases 2-dimensional measurements underestimated 3D annular dimensions, and the patient with clinical LVOT obstruction had the lowest predicted LVOT area-to-aortic area ratio (0.5). CONCLUSIONS: 3DE-based preoperative modeling of surgical implantation of stent-based valves in the mitral position may improve quantification of mitral valve dimensions and inform risk stratification for potential LVOT obstruction.

Magnetic Resonance-Based Diagnostics for Bleeding Assessment in Neonatal Cardiac Surgery.

PURPOSE: Infants undergoing a cardiac operation are at high risk for postsurgical bleeding. To date, there are no highly predictive models for postsurgical bleeding in this population. This study's objective was to assess the predictive ability of T2 magnetic resonance (T2MR). DESCRIPTION: T2MR uses magnetic resonance to detect clot formation characteristics on a small blood sample and provides hemostatic indicators that can assess bleeding risk. EVALUATION: This prospective, single-institution study enrolled 100 patients younger than 12 months old undergoing a cardiac operation from April 27, 2015, to September 21, 2016. The primary end point was postsurgical bleeding within 24 hours after the procedure. T2MR data were modeled with a binary recursive partitioning algorithm with randomized cross-validation. The tight clot metric produced the highest univariate discrimination of bleeding (receiver operator characteristic curve, 0.64; classification accuracy, 72%), and along with the platelet function metric, demonstrated highest relative importance based on Gini index splitting (Salford Systems, San Diego, CA). Multivariate modeling with cross-validation showed mean receiver operator characteristic curve area of 0.74 and classification accuracy of 82%. CONCLUSIONS: T2MR tight clot and platelet function metrics were associated with bleeding events.

Role of cystic fibrosis transmembrane conductance regulator in patients with chronic sinopulmonary disease.

BACKGROUND: Previous studies report a high frequency of mutations in the cystic fibrosis (CF) transmembrane conductance regulator gene (CFTR) in patients with idiopathic bronchiectasis. However, most studies have based their findings on preselected patient groups or have performed limited testing for CF transmembrane conductance regulator (CFTR) dysfunction. The objective of our study was to evaluate the prevalence of CFTR gene mutations and/or CFTR-related ion channel abnormalities among subjects with idiopathic chronic sinopulmonary disease and the prevalence of CF or a CFTR-related disorder in this population. METHODS: We evaluated 72 prospectively enrolled patients from 1995 to 2005 at the Hospital for Sick Children and St. Michael's Hospital with idiopathic chronic sinopulmonary disease for evidence of CFTR-mediated abnormalities. We performed CFTR genotyping and assessed CFTR function using sweat testing and nasal potential difference testing. The results were compared with data from healthy control subjects, CF heterozygotes, and patients with CF. RESULTS: The CFTR functional tests in idiopathic sinopulmonary patients showed a continuous spectrum, ranging from normal to values typically seen in individuals with CF. Forty-eight patients (66%) demonstrated CFTR mutations and/or abnormalities of CFTR function. Twenty-two (31%) fulfilled criteria for a diagnosis of CF and 26 (36%) for a CFTR-related disorder with a strong female preponderance. Functional tests, more than genotyping, were instrumental in establishing a CF diagnosis. Clinical features failed to distinguish subjects with CF from those with CFTR-related or idiopathic disease. CONCLUSIONS: The high prevalence of CF and CFTR dysfunction among patients with idiopathic chronic sinopulmonary disease underscores the need for extensive diagnostic evaluation for CF.

The experience of conducting Mortality and Morbidity reviews in a pediatric interventional radiology service: a retrospective study.

PURPOSE: To review the experience and impact of conducting multidisciplinary Morbidity and Mortality (M&M) reviews in pediatric interventional radiology (IR) and describe issues, lessons, and recommendations. MATERIALS AND METHODS: A dedicated functionality of an existing database was developed to retrospectively analyze pediatric IR M&M issues. Patient demographics, sedation/anesthesia, and procedure type were recorded. M&M issues were assigned to at least one of 10 categories and graded as major or minor per Society of Interventional Radiology (SIR) guidelines. Issues could result in recommendations, which were divided into six categories; each was classified as implemented or not implemented. RESULTS: Of 31,983 patient encounters over a period of 10 years, 516 patient events (1.6%) were discussed at M&M reviews. A total of 772 categories were assigned; they related to the procedure (34%), patient comorbidity (20%), processes (15%), device (10%), management (8%), sedation/anesthesia (4%), medication (2%), ethical issues (1%), "near-misses" (1%), and other (5%). A total of 292 issues (57%) were graded as minor (SIR class A, n = 202; class B, n = 90) and 224 (43%) as major (SIR class C, n = 42; class D, n = 151; class E, n = 6; class F, n = 27). Twenty-seven deaths were reviewed. Of 397 recommendations made, 80% were fully implemented, 11% partially implemented, and 9% not implemented. Recommendations made related to process improvements (49%), technical changes (20%), interdisciplinary discussions (15%), educational endeavors (9%), contacting manufacturers (6%), and other issues (1%). CONCLUSIONS: As a result of regular multidisciplinary M&M reviews being conducted, a large number of practical recommendations were made for improvements in quality of care, and implemented over a 10-year period. M&M reviews provide a useful forum for team discussions and are a vehicle for change and potential improvement in the delivery of care in a pediatric IR service.

Three-dimensional motion of liver tumors using cine-magnetic resonance imaging.

PURPOSE: To measure the three-dimensional motion of liver tumors using cine-magnetic resonance imaging (MRI) and compare it to the liver motion assessed using fluoroscopy. METHODS AND MATERIALS: Liver and liver tumor motion were investigated in the first 36 patients with primary (n = 20) and metastatic (n = 16) liver cancer accrued to our Phase I stereotactic radiotherapy study. At simulation, all patients underwent anteroposterior fluoroscopy, and the maximal diaphragm excursion in the craniocaudal (CC) direction was observed. Cine-MRI using T(2)-weighted single shot fast spin echo sequences were acquired in three orthogonal planes during free breathing through the centroid of the most dominant liver tumor. ImageJ software was used to measure the maximal motion of the tumor edges in each plane. The intra- and interobserver reproducibility was also quantified. RESULTS: The average CC motion of the liver at fluoroscopy was 15 mm (range, 5-41). On cine-MRI, the average CC tumor motion was 15.5 mm (range, 6.9-35.4), the anteroposterior motion was 10 mm (range, 3.7-21.6), and the mediolateral motion was 7.5 mm (range, 3.8-14.8). The fluoroscopic CC diaphragm motion did not correlate well with the MRI CC tumor motion (r = 0.25). The mean intraobserver error was <2 mm in the CC, anteroposterior, and mediolateral directions, and 90% of measurements between observers were within 3 mm. CONCLUSIONS: The results of our study have shown that cine-MRI can be used to directly assess liver tumor motion in three dimensions. Tumor motion did not correlate well with the diaphragm motion measured using kilovoltage fluoroscopy. The tumor motion data from cine-MRI can be used to facilitate individualized planning target volume margins to account for breathing motion.

Gaps in the care of patients admitted to hospital with an exacerbation of chronic obstructive pulmonary disease.

BACKGROUND: Patients admitted to hospital because of an exacerbation of chronic obstructive pulmonary disease (COPD) are at high risk of adverse events. We evaluated the association between gaps in care and adverse events during the hospital stay and after discharge. METHODS: We retrospectively reviewed the charts of 105 consecutive patients discharged from hospital between Jan. 1 and Dec. 31, 2001, with a diagnosis of COPD exacerbation. On the basis of published guidelines, prior studies and discussions with colleagues, we defined a care gap as having occurred if any of 9 important inpatient and 7 discharge-related processes of care did not take place correctly. Inpatient adverse events included worsening of condition after admission, transfer to a higher level of care, cardiac arrest and death. Discharge-related adverse events were defined as including readmission to the hospital, revisit to the emergency department or death within 30 days after discharge. RESULTS: Of the 105 patients studied, 88 (84%) had at least 1 inpatient gap in care and 16 (15%) an inpatient adverse event; 2 of the 16 died. Patients who had an inpatient adverse event had more gaps in their care (2.0 v. 1.3 gaps, p = 0.004) and longer stays (16.4 v. 8.6 days, p = 0.007). There were 6 adverse events (frequency 38%) among the 16 patients with 3 or more gaps in their care, 6 adverse events (28%) among the 21 patients with 2 gaps, 1 adverse event (2%) among the 51 patients with 1 gap and 3 adverse events (18%) among the 17 patients with no gaps in their care (p = 0.001 for trend). Of the 103 patients discharged alive, 102 (99%) had at least 1 gap in discharge-related care, but we found no association between these gaps and adverse events within 30 days after discharge. INTERPRETATION: Gaps in the inpatient care of patients with COPD exacerbation were common and were associated with inpatient adverse events. Gaps in discharge-related care were also common but were not associated with postdischarge adverse events.