Computed tomography to predict pacemaker need after transcatheter aortic valve replacement.

Transcatheter aortic valve replacement (TAVR) is preferred therapy for elderly patients with severe aortic stenosis (AS) and increasingly used in younger patient populations with good safety and efficacy outcomes. However, cardiac conduction abnormalities remain a frequent complication after TAVR ranging from relative benign interventriculair conduction delays to prognostically relevant left bundle branch block and complete atrio-ventricular (AV) block requiring permanent pacemaker implantation (PPI). Although clinical, procedural and electrocardiographic factors have been identified as predictors of this complication, there is a need for advanced strategies to control the burden of conduction defects particularly as TAVR shifts towards younger populations. This state of the art review highlights the value of ECG-synchronized computed tomographic angiography (CTA) evaluation of the aortic root to better understand and manage conduction problems post-TAVR. An update on CTA derived anatomic features related to conduction issues is provided and complemented with computational framework modelling. This CTA-derived 3-dimensional anatomical reconstruction tool generates patient-specific TAVR simulations enabling operators to adapt procedural strategy and implantation technique to mitigate conduction abnormality risks.

Moderate Aortic Stenosis-Advanced Imaging, Risk Assessment, and Treatment Strategies.

Moderate aortic stenosis is increasingly recognized as a disease entity with poor prognosis. Diagnosis of moderate aortic stenosis may be complemented by laboratory tests and advanced imaging techniques focused at detecting signs of cardiac damage such as increase of cardiac enzymes (N-terminal pro-B-type Natriuretic Peptide, troponin), left ventricular remodeling (hypertrophy, reduced left ventricular ejection fraction), or myocardial fibrosis. Therapy should include guideline-directed optimal medical therapy for heart failure. Patients with signs of cardiac damage may benefit from early intervention, which is the focus of several ongoing randomized controlled trials. As yet, no evidence-based therapy exists to halt the progression of aortic valve calcification.

Impact and limitations of 3D computational modelling in transcatheter mitral valve replacement-a two-centre Dutch experience.

BACKGROUND: Transcatheter mitral valve replacement (TMVR) has emerged as a minimally invasive alternative to mitral valve surgery for patients at high or prohibitive operative risk. Prospective studies reported favourable outcomes in patients with annulus calcification (valve-in-mitral annulus calcification; ViMAC), failed annuloplasty ring (mitral valve-in-ring; MViR), and bioprosthetic mitral valve dysfunction (mitral valve-in-valve; MViV). Multi-slice computed tomography (MSCT)-derived 3D-modelling and simulations may provide complementary anatomical perspectives for TMVR planning. AIMS: We aimed to illustrate the implementation of MSCT-derived modelling and simulations in the workup of TMVR for ViMAC, MViR, and MViV. METHODS: For this retrospective study, we included all consecutive patients screened for TMVR and compared MSCT data, echocardiographic outcomes and clinical outcomes. RESULTS: Sixteen out of 41 patients were treated with TMVR (ViMAC n = 9, MViR n = 3, MViV n = 4). Eleven patients were excluded for inappropriate sizing, 4 for anchoring issues and 10 for an unacceptable risk of left ventricular outflow tract obstruction (LVOTO) based on 3D modelling. There were 3 procedure-related deaths and 1 non-procedure-related cardiovascular death during 30 days of follow-up. LVOTO occurred in 3 ViMAC patients and 1 MViR patient, due to deeper valve implantation than planned in 3 patients, and anterior mitral leaflet displacement with recurrent basal septum thickening in 1 patient. TMVR significantly reduced mitral mean gradients as compared with baseline measurements (median mean gradient 9.5 (9.0-11.5) mm Hg before TMVR versus 5.0 (4.5-6.0) mm Hg after TMVR, p = 0.03). There was no residual mitral regurgitation at 30 days. CONCLUSION: MSCT-derived 3D modelling and simulation provide valuable anatomical insights for TMVR with transcatheter balloon expandable valves in ViMAC, MViR and MViV. Further planning iterations should target the persistent risk for neo-LVOTO.

Regional aortic wall shear stress increases over time in patients with a bicuspid aortic valve.

BACKGROUND: Aortic wall shear stress (WSS) is a known predictor of ascending aortic growth in patients with a bicuspid aortic valve (BAV). The aim of this study was to study regional WSS and changes over time in BAV patients. METHODS: BAV patients and age-matched healthy controls underwent four-dimensional (4D) flow cardiovascular magnetic resonance (CMR). Regional, peak systolic ascending aortic WSS, aortic valve function, aortic stiffness measures, and aortic dimensions were assessed. In BAV patients, 4D flow CMR was repeated after 3 years of follow-up and both at baseline and follow-up computed tomography angiography (CTA) were acquired. Aortic growth (volume increase of ≥5%) was measured on CTA. Regional WSS differences within patients' aorta and WSS changes over time were analyzed using linear mixed-effect models and were associated with clinical parameters. RESULTS: Thirty BAV patients (aged 34 years [interquartile range (IQR) 25-41]) were included in the follow-up analysis. Additionally, another 16 BAV patients and 32 healthy controls (aged 33 years [IQR 28-48]) were included for other regional analyses. Magnitude, axial, and circumferential WSS increased over time (all p < 0.001) irrespective of aortic growth. The percentage of regions exposed to a magnitude WSS >95th percentile of healthy controls increased from 21% (baseline 506/2400 regions) to 31% (follow-up 734/2400 regions) (p < 0.001). WSS angle, a measure of helicity near the aortic wall, decreased during follow-up. Magnitude WSS changes over time were associated with systolic blood pressure, peak aortic valve velocity, aortic valve regurgitation fraction, aortic stiffness indexes, and normalized flow displacement (all p < 0.05). CONCLUSION: An increase in regional WSS over time was observed in BAV patients, irrespective of aortic growth. The increasing WSSs, comprising a larger area of the aorta, warrant further research to investigate the possible predictive value for aortic dissection.

The cardiovascular exercise response in children with overweight or obesity measured by cardiovascular magnetic resonance imaging.

BACKGROUND: Overweight and obesity are among the main causes of cardiovascular diseases. Exercise testing can aid in the early detection of subtle cardiac dysfunction not present in rest. We hypothesized that the cardiovascular response to exercise is impaired among children with overweight or obesity, characterized by the inability of the cardiovascular system to adapt to exercise by increasing cardiac volumes and blood pressure. We performed a cardiovascular stress test to investigate whether the cardiovascular exercise response is altered in children with overweight and obesity, as compared to children with a normal weight. SUBJECTS: A subgroup of the Generation R population-based prospective cohort study, consisting of 41 children with overweight or obesity and 166 children with a normal weight with a mean age of 16 years, performed an isometric exercise. METHODS: Continuous heart rate and blood pressure were measured during rest, exercise and recovery. Cardiovascular magnetic resonance (CMR) measurements were performed during rest and exercise. RESULTS: Higher BMI was associated with a higher resting systolic and diastolic blood pressure (difference: 0.24 SDS (95% CI 0.10, 0.37) and 0.20 SDS (95% CI 0.06, 0.33)) and lower systolic and diastolic blood pressure increases from rest to peak exercise (-0.11 SDS (95% CI -0.20, -0.03) and -0.07 SDS (95% CI -0.07, -0.01)). BMI was also associated with a slower decrease in systolic and diastolic blood pressure during recovery (p values < 0.05). Higher childhood BMI was associated with lower BSA corrected left ventricular mass, end-diastolic volume and stroke volume (p values < 0.05). There were no associations of childhood BMI with the cardiac response to exercise measured by heart rate and CMR measurements. CONCLUSION: Childhood BMI is, across the full range, associated with a blunted blood pressure response to static exercise but there were no differences in cardiac response to exercise. Our findings suggest that adiposity may especially affect the vascular exercise reaction without affecting cardiac response.

Computed Tomography in Infectious Endocarditis.

Imaging is one of the cornerstones in diagnosis and management of infective endocarditis, underlined by recent guidelines. Echocardiography is the first-line imaging technique, however, computed tomography (CT) has a class I recommendation in native and prosthetic valve endocarditis to detect valvular lesions in case of possible endocarditis and to detect paravalvular and periprosthetic complications in case of inconclusive echocardiography. Echocardiography has a higher diagnostic accuracy than CT in detecting valvular lesions, but not for diagnosing paravalvular lesions where CT is superior. Additionally, CT is useful and recommended by guidelines to detect extracardiac manifestations of endocarditis and in planning surgical treatment including assessment of the coronary arteries. The advent of photon-counting CT and its improved spatial resolution and spectral imaging is expected to expand the role of CT in the diagnosis of infective endocarditis. In this review, we provide an overview of the current role of CT in infective endocarditis focusing on image acquisition, image reconstruction, interpretation, and diagnostic accuracy.

Coronary computed tomography angiography improves assessment of patients with acute chest pain and inconclusively elevated high-sensitivity troponins.

OBJECTIVES: To determine whether coronary computed tomography angiography (CCTA) can improve the diagnostic work-up of patients with acute chest pain and inconclusively high-sensitivity troponins (hs-troponin). METHODS: We conducted a prospective, blinded, observational, multicentre study. Patients aged 30-80 years presenting to the emergency department with acute chest pain and inconclusively elevated hs-troponins were included and underwent CCTA. The primary outcome was the diagnostic accuracy of ≥ 50% stenosis on CCTA to identify patients with type-1 non-ST-segment elevation acute coronary syndrome (NSTE-ACS). RESULTS: A total of 106 patients (mean age 65 ± 10, 29% women) were enrolled of whom 20 patients (19%) had an adjudicated diagnosis of type-1 NSTE-ACS. In 45 patients, CCTA revealed non-obstructive coronary artery disease (CAD) or no CAD. Sensitivity, specificity, negative predictive value (NPV), positive predictive value and area-under-the-curve (AUC) of ≥ 50% stenosis on CCTA to identify patients with type 1 NSTE-ACS, was 95% (95% confidence interval: 74-100), 56% (45-68), 98% (87-100), 35% (29-41) and 0.83 (0.73-0.94), respectively. When only coronary segments with a diameter ≥ 2 mm were considered for the adjudication of type 1 NSTE-ACS, the sensitivity and NPV increased to 100%. In 8 patients, CCTA enabled the detection of clinically relevant non-coronary findings. CONCLUSION: The absence of ≥ 50% coronary artery stenosis on CCTA can be used to rule out type 1 NSTE-ACS in acute chest pain patients with inconclusively elevated hs-troponins. Additionally, CCTA can help improve the diagnostic work-up by detecting other relevant conditions that cause acute chest pain and inconclusively elevated hs-troponins. CLINICAL RELEVANCE STATEMENT: Coronary CTA (CCTA) can safely rule out type 1 non-ST-segment elevation acute coronary syndrome (NSTE-ACS) in patients presenting to the ED with acute chest pain and inconclusively elevated hs-troponins, while also detecting other relevant non-coronary conditions. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03129659). Registered on 26 April 2017 KEY POINTS: Acute chest discomfort is a common presenting complaint in the emergency department. CCTA achieved very high negative predictive values for type 1 NSTE-ACS in this population. CCTA can serve as an adjunct for evaluating equivocal ACS and evaluates for other pathology.

Stomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management.

OBJECTIVE: To identify risk factors for stenosis and compare management strategies for stenosis etiology and to examine the efficacy of each approach. Patients with classic bladder exstrophy (CBE), a rare genitourinary malformation, may require construction of a continent urinary stoma (CUS) if incontinence persists. Stomal stenosis is a challenging complication as it is common, progressive, and recurrent. METHODS: CBE patients who underwent CUS were retrospectively reviewed for risk factors for stenosis including stoma type, prior midline laparotomy number, and umbilicoplasty suture material. Stenosis etiology and management strategies were further reviewed. RESULTS: A total of 260 CBE patients underwent CUS creation. Stenosis developed in 65 patients (25.0%) at a median interval of 1.9 years. Etiology included scar contracture (n = 41), keloid (n = 17), and hypertrophic scar (n = 7). Multifilament suture was the only variable associated with an increased risk of stenosis compared to monofilament suture (P = .009). Almost all patients required surgical intervention. Most scar contractures underwent stomal incision with success in 100%. Hypertrophic scars and keloids responded best to excision with local tissue rearrangement (66.7%). At last follow-up, all patients achieved success. CONCLUSION: Stomal stenosis is common and challenging for the reconstructive surgeon. Strategies to prevent and effectively manage this are greatly desired. Use of multifilament suture for the umbilicoplasty increased stenosis perhaps from a greater inflammatory response and scarring, while monofilament suture may reduce its incidence. Stomal incision for treating scar contractures, and excision with local tissue rearrangement for hypertrophic scars and keloids may improve successful primary surgical intervention.

Left ventricular strain-volume loops in bicuspid aortic valve disease: new insights in cardiomechanics.

Aims: By combining temporal changes in left ventricular (LV) global longitudinal strain (GLS) with LV volume, LV strain-volume loops can assess cardiac function across the cardiac cycle. This study compared LV strain-volume loops between bicuspid aortic valve (BAV) patients and controls, and investigated the loop's prognostic value for clinical events. Methods and results: From a prospective cohort of congenital heart disease patients, BAV patients were selected and compared with healthy volunteers, who were matched for age and sex at group level. GLS analysis from apical views was used to construct strain-volume loops. Associations with clinical events, i.e. a composite of all-cause mortality, heart failure, arrhythmias, and aortic valve replacement, were assessed by Cox regression. A total of 113 BAV patients were included (median age 32 years, 40% female). BAV patients demonstrated lower Sslope (0.21%/mL, [Q1-Q3: 0.17-0.28] vs. 0.27%/mL [0.24-0.34], P < 0.001) and ESslope (0.19%/mL [0.12-0.25] vs. 0.29%/mL [0.21-0.43], P < 0.001) compared with controls, but also greater uncoupling during early (0.48 ± 1.29 vs. 0.05 ± 1.21, P = 0.04) and late diastole (0.66 ± 1.02 vs. -0.07 ± 1.07, P < 0.001). Median follow-up duration was 9.9 [9.3-10.4] years. Peak aortic jet velocity (HR 1.22, P = 0.03), enlarged left atrium (HR 3.16, P = 0.003), E/e' ratio (HR 1.17, P = 0.002), GLS (HR 1.16, P = 0.008), and ESslope (HR 0.66, P = 0.04) were associated with the occurrence of clinical events. Conclusion: Greater uncoupling and lower systolic and diastolic slopes were observed in BAV patients compared with healthy controls, suggesting presence of altered LV cardiomechanics. Moreover, lower ESslope was associated with clinical events, highlighting the strain-volume loop's potential as prognostic marker.

A Rocky Road: Bladder Stones in the Augmented Exstrophy-Epispadias Complex Patient.

OBJECTIVE: To determine the rate of stone formation amongst patients of the exstrophy-epispadias complex with augmentation cystoplasty. We hypothesize that bowel segment choice influences the rate of stone formation after bladder augmentation and the rate of complications from bladder stone surgery. METHODS: An IRB-approved institutional database of 1512 exstrophy-epispadias patients was reviewed retrospectively. Patients that had a history of bladder augmentation and were seen at our institution between 2003 and 2023 were included. RESULTS: Out of 259 patients, bladder stones developed in 21.6% (56), of which the bowel segment used was colon in 147 patients and ileum in 100. Stones formed in 19% of colon augments compared to 29% ileal augments, however, this was not statistically significant (P = .07). The most common primary stone component was dahllite, followed by struvite for all augments (Table 1). The median time to stone treatment after augmentation was 4.14 years (0.75-31). Seventy-four percentage of patients had a recurrence that required a second surgery. The median time from first to second surgery and second to third surgery was 1.4 years and 2.22 years, respectively. Bladder stone surgery complications occurred in 14% of patients, vesicocutaneous fistula being the most common, and complications did not differ by augment type. Median follow-up after first stone intervention was 6.07 years (0-19.5). CONCLUSION: The treatment of bladder stones in the exstrophy-epispadias complex remains challenging. Interventions to prevent recurrence are crucial as the majority of patients will require 2 or more stone surgeries in their lifetime.

Temporal changes in CT-derived fractional flow reserve in patients after heart transplantation.

BACKGROUND: Adding functional information by CT-derived fractional flow reserve (FFRct) to coronary CT angiography (CCTA) and assessing its temporal change may provide insight into the natural history and physiopathology of cardiac allograft vasculopathy (CAV) in heart transplantation (HTx) patients. We assessed FFRct changes as well as CAV progression over a 2-year period in HTx patients undergoing serial CT imaging. METHODS: HTx patients from Erasmus MC and Mount Sinai Hospital, who had consecutive CCTAs 2 years apart were evaluated. FFRct analysis was performed for both scans. FFRct values at the most distal point in the left anterior descending (LAD), left circumflex (LCX), and right coronary artery (RCA) were measured after precisely matching the anatomical locations in both analyses. Also, the number of anatomical coronary stenoses of > 30% was scored. RESULTS: In total, 106 patients (median age 57 [interquartile range 47-67] years, 67% male) at 9 [6-13] years after HTx at the time of the baseline CCTA were included. Median distal FFRct values significantly decreased from baseline to follow-up for the LAD from 0.85 [0.79-0.90] to 0.84 [0.76-0.90] (p = 0.001), LCX from 0.92 [0.88-0.96] to 0.91 [0.85-0.95] (p = 0.009), and RCA from 0.92 [0.86-0.95] to 0.90 [0.86-0.94] (p = 0.004). The number of focal anatomical stenoses of > 30% increased from a median of 1 [0-2] at baseline to 2 [0-3] at follow-up (p = 0.009). CONCLUSIONS: The distal coronary FFRct values in post-HTX patients in each of the three major coronary arteries decreased, and the number of focal coronary stenoses increased over a 2-year period. Temporal FFRct change rate may become an additional parameter in the follow-up of HTx patients, but more research is needed to elucidate its role. CLINICAL RELEVANCE STATEMENT: CT-derived fractional flow reserve (FFRct) is important post-heart transplant because of additional information on coronary CT angiography for cardiac allograft vasculopathy (CAV) detection. The decrease and degree of reduction in distal FFRct value may indicate progression in anatomic CAV burden. KEY POINTS: CT-derived fractional flow reserve (FFRct) is important for monitoring cardiac allograft vasculopathy (CAV) in heart transplant patients. Over time, transplant patients showed a decrease in distal FFRct and an increase in coronary stenoses. Temporal changes in FFRct could be crucial for transplant follow-up, aiding in CAV detection.

Evaluation of the 2021 ESC recommendations for family screening in hereditary transthyretin cardiac amyloidosis.

AIMS: The 2021 European Society of Cardiology (ESC) screening recommendations for individuals carrying a pathogenic transthyretin amyloidosis variant (ATTRv) are based on expert opinion. We aimed to (i) determine the penetrance of ATTRv cardiomyopathy (ATTRv-CM) at baseline; (ii) examine the value of serial evaluation; and (iii) establish the yield of first-line diagnostic tests (i.e. electrocardiogram, echocardiogram, and laboratory tests) as per 2021 ESC position statement. METHODS AND RESULTS: We included 159 relatives (median age 55.6 [43.2-65.9] years, 52% male) at risk for ATTRv-CM from 10 centres. The primary endpoint, ATTRv-CM diagnosis, was defined as the presence of (i) cardiac tracer uptake in bone scintigraphy; or (ii) transthyretin-positive cardiac biopsy. The secondary endpoint was a composite of heart failure (New York Heart Association class ≥II) and pacemaker-requiring conduction disorders. At baseline, 40/159 (25%) relatives were diagnosed with ATTRv-CM. Of those, 20 (50%) met the secondary endpoint. Indication to screen (≤10 years prior to predicted disease onset and absence of extracardiac amyloidosis) had an excellent negative predictive value (97%). Other pre-screening predictors for ATTRv-CM were infrequently identified variants and male sex. Importantly, 13% of relatives with ATTRv-CM did not show any signs of cardiac involvement on first-line diagnostic tests. The yield of serial evaluation (n = 41 relatives; follow-up 3.1 [2.2-5.2] years) at 3-year interval was 9.4%. CONCLUSIONS: Screening according to the 2021 ESC position statement performs well in daily clinical practice. Clinicians should adhere to repeating bone scintigraphy after 3 years, as progressing to ATTRv-CM without signs of ATTRv-CM on first-line diagnostic tests or symptoms is common.

Cardiac dysfunction during exercise in young adults with bronchopulmonary dysplasia.

Background: Worldwide, 1-2% of children are born premature and at risk for developing bronchopulmonary dysplasia (BPD). Preterm-born adults are at risk for early cardiovascular disease. The role of BPD is unclear. This study aims to examine cardiorespiratory function during submaximal exercise in young adult survivors of extreme prematurity, with or without BPD. Methods: 40 preterm-born young adults, 20 with BPD (median gestational age 27 weeks, interquartile range (IQR) 26-28 weeks) and 20 without BPD (median gestational age 28 weeks, IQR 27-29 weeks) were prospectively compared to age-matched at term-born adults (median gestational age 39 weeks, IQR 38-40 weeks). Participants underwent exercise testing and cardiovascular magnetic resonance with submaximal exercise. Results: Resting heart rate in BPD subjects was higher than in at term-born subjects (69±10 mL versus 61±7 mL, p=0.01). Peak oxygen uptake during maximal cardiopulmonary exercise testing was decreased in BPD subjects (91±18% versus 106±17% of predicted, p=0.01). In BPD subjects, cardiac stroke volume change with exercise was impaired compared to at term-born subjects (11±13% versus 25±10%; p<0.001). With exercise, left ventricular end-diastolic volume decreased more in preterm-born subjects with versus without BPD (-10±8% versus -3±8%; p=0.01) and compared to at term-born subjects (0±5%; p<0.001). Exploratory data analysis revealed that exercise stroke volume and end-diastolic volume change were inversely correlated with oxygen dependency in those born prematurely. Conclusions: In preterm-born young adults, particularly those with BPD, resting cardiac function, exercise performance and cardiac response to exercise is impaired compared to controls. Exercise cardiovascular magnetic resonance may reveal an important predisposition for heart disease later in life.

Community-Acquired Pneumonia Diagnosis Following Emergency Department Visits for Respiratory Illness.

Community-acquired pneumonia (CAP) is often considered for children presenting to the emergency department (ED) with respiratory symptoms. It is unclear how often children are diagnosed with CAP following an ED visit for respiratory illness. We performed a retrospective case-control study to evaluate 7-day CAP diagnosis among children 3 months to 18 years discharged from the ED with respiratory illness from 2011 to 2021 and who receive care at 4 hospital-affiliated primary care clinics. Logistic regression was performed to assess for predictors of 7-day CAP diagnosis. Seventy-four (0.7%, 95% confidence interval [CI] = 0.6%, 0.9%) of 10 329 children were diagnosed with CAP within 7 days, and fever at the index visit was associated with increased odds of diagnosis (odds ratio [OR] = 3.32, 95% CI = 1.75-6.28). Community-acquired pneumonia diagnosis after discharge from the ED with respiratory illness is rare, even among children who are febrile at time of initial evaluation.

Aortic geometry and long-term outcome in patients with a repaired coarctation.

OBJECTIVE: This study aims to compare aortic morphology between repaired coarctation patients and controls, and to identify aortic morphological risk factors for hypertension and cardiovascular events (CVEs) in coarctation patients. METHODS: Repaired coarctation patients with computed tomography angiography (CTA) or magnetic resonance angiography (MRA) were included, followed-up and compared with sex-matched and age-matched controls. Three-dimensional aortic shape was reconstructed using patients' CTA or MRA, or four-dimensional flow cardiovascular magnetic resonance in controls, and advanced geometrical characteristics were calculated and visualised using statistical shape modelling. In patients, we examined the association of geometrical characteristics with (1) baseline hypertension, using multivariable logistic regression; and (2) cardiovascular events (CVE, composite of aortic complications, coronary artery disease, ventricular arrhythmias, heart failure hospitalisation, stroke, transient ischaemic attacks and cardiovascular death), using multivariable Cox regression. The least absolute shrinkage and selection operator (LASSO) method selected the most informative multivariable model. RESULTS: Sixty-five repaired coarctation patients (23 years (IQR 19-38)) were included, of which 44 (68%) patients were hypertensive at baseline. After a median follow-up of 8.7 years (IQR 4.8-15.4), 27 CVEs occurred in 20 patients. Aortic arch dimensions were smaller in patients compared with controls (diameter p<0.001, wall surface area p=0.026, volume p=0.007). Patients had more aortic arch torsion (p<0.001) and a higher curvature (p<0.001). No geometrical characteristics were associated with hypertension. LASSO selected left ventricular mass, male sex, tortuosity and age for the multivariable model. Left ventricular mass (p=0.014) was independently associated with CVE, and aortic tortuosity showed a trend towards significance (p=0.070). CONCLUSION: Repaired coarctation patients have a smaller aortic arch and a more tortuous course of the aorta compared with controls. Besides left ventricular mass index, geometrical features might be of importance in long-term risk assessment in coarctation patients.

Optimizing prenatal diagnosis and referral of classic bladder exstrophy: Lessons from a single-institution experience.

INTRODUCTION: Classic bladder exstrophy (CBE) is a malformation of the genitourinary system that occurs due to failure of abdominal wall closure. Unlike other malformations of similar incidence, prenatal diagnosis of CBE relies on suggested, rather than formal, diagnostic criteria. OBJECTIVE: This report describes prenatal diagnosis of CBE in the largest single-institutional cohort to date and delineates key sonographic findings and protocols for specialist referral. MATERIALS AND METHODS: A single-institutional database was reviewed for CBE patients born since 2000. Data on screening ultrasound use, gestational age at ultrasound, and abnormal findings were extracted. Where possible, time of prenatal diagnosis (pre- or postnatal and gestational age), ultrasound findings and other imaging data, specialist referral, institution of birth and closure, and outcome of primary closure attempt were compared. RESULTS: Of 557 patients born with CBE between 2000 and 2022, 284 met inclusion criteria and complete data were available for 280 (229 born domestically and 51 born internationally) who were included for analysis. Abnormal sonography suggestive of CBE was present for 48% (n = 134) of patients, for whom absent bladder was the most common abnormal finding (76% [102/134]). Of domestic patients, 46% (n = 106) were diagnosed prenatally at a median gestational age of 22 weeks (inter-quartile range [IQR]: 20-24), and 14% (n = 32) underwent confirmatory fetal magnetic resonance imaging. Of domestic patients with abnormal prenatal findings, 75% (n = 80/106) consulted with maternal-fetal medicine and 58% (n = 62/106) consulted with pediatric urology. On univariate analysis, prenatal diagnosis was positively associated with primary repair at Association for the Bladder Exstrophy Community-recognized centers of excellence (54% vs. 38%, p = 0.02) and negatively associated with osteotomy at primary closure (41% vs 59%, p = 0.003) but not success of primary closure (74% vs. 82%, p = 0.07). DISCUSSION: Rates of prenatal diagnosis in this cohort were similar to previous reports of smaller cohorts. Diagnosis allows for comprehensive pre- and postnatal follow-up with a pediatric urologist, with implications on birth planning and decisions on termination of pregnancy. Because of the previously-reported association between exstrophy and in vitro fertilization, these pregnancies should undergo detailed sonography. Any nonvisualization of the fetal bladder should prompt a detailed exam, and any finding characteristic of bladder exstrophy warrants referral to pediatric urology. CONCLUSIONS: Although CBE is a rare disorder, it is underdiagnosed during pregnancy. Sonographers and obstetricians should be aware of characteristic findings and best practices following diagnosis. Early referral to pediatric urology and maternal-fetal medicine is important for counseling and postnatal planning.