Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

BACKGROUND: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling. METHODS: SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively. RESULTS: SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased. CONCLUSIONS: Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes.

Growth of the right ventricular outflow tract in repaired tetralogy of Fallot: A longitudinal CMR study.

BACKGROUND: Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an equally effective and less invasive alternative to surgical PVR but many native right ventricular outflow tracts (RVOTs) are too large for TPVR at time of referral. Understanding the rate of growth of the RVOT may help optimize timing of referral. This study aims to examine the longitudinal growth of the native RVOT over time in repaired tetralogy of Fallot (TOF). METHODS: A retrospective review of serial cardiac MRI cardiovascular magnetic resonance (CMR) data from 121 patients with repaired TOF and a native RVOT (median age at first CMR 14.7 years, average interval between the first and last CMR of 8.1 years) was performed to measure serial changes in RVOT diameter, cross-sectional area, perimeter-derived diameter, and length. RESULTS: All parameters of RVOT size continued to grow with increasing age but growth was more rapid in the decade after TOF repair (for minimum systolic diameter, mean increase of 5.7 mm per 10 years up to year 12, subsequently 2.3 mm per 10 years). The RVOT was larger with a transannular patch and in patients without pulmonary stenosis (p < 0.001 for both), but this was not associated with rate of growth. More rapid RVOT enlargement was noted in patients with larger right ventricular end-diastolic volume (RVEDV), higher PR fraction, and greater rates of increases in RVEDV and PR (p < 0.001 for all) CONCLUSIONS: in patients with repaired TOF, using serial CMR data, we found that RVOT size increased progressively at all ages, but the rate was more rapid in the first decade after repair. More rapid RVOT enlargement was noted in patients with a larger RV, more PR, and greater rates of increases in RV size and PR severity. These results may be important in considering timing of referral for transcatheter pulmonary valves, in planning transcatheter and surgical valve replacement, and in designing future valves for the native RVOT.

Aortic growth after arch reconstruction with patch augmentation: a 2-decade experience.

OBJECTIVES: Optimal aortic sizing during aortic arch reconstruction remains unknown. Negative effects of arch under- or oversizing are well-published. We aimed to characterize longitudinal aortic growth after patch-augmented arch reconstruction to identify the initial reconstructed arch size that results in normal mid-term arch dimensions. METHODS: Single-centre, retrospective review of infants undergoing Damus-Kaye-Stansel (DKS) or non-DKS patch-augmented aortic arch reconstruction between 2000 and 2021. Ascending aorta, proximal and distal transverse arch, aortic isthmus (AIsth) and descending aorta dimensions were measured in postoperative echocardiograms (<3 months from index operation) and cross-sectional imaging (>12 months). Longitudinal changes to aortic dimensions and z-scores were analysed. Secondary outcomes included reintervention, valve and ventricular function, mortality and transplantation. RESULTS: Fifty-four patients (16 DKS, 38 non-DKS) were included. At 6.3 [2.2, 12.0]-year follow-up, all aortic segments grew significantly in both groups, while z-scores remained unchanged except for non-DKS proximal and distal transverse arch z-scores, which significantly increased (P < 0.05 each). When stratified by initial postoperative z-score (z < -1, -1 ≤ z ≤ 1, z > 1), non-DKS patients with initial AIsth z-score <-1 had a final z-score significantly smaller than both the targeted z-score zero (P = 0.014) and final z-score in a group with initial postoperative z-score ±1 (P = 0.009). Valve and ventricular function remained stable. Eighteen patients required reintervention, 1 died and 1 underwent transplant. CONCLUSIONS: Over mid-term follow-up, aortic growth after arch reconstruction with patch augmentation was proportional when repaired to normal z-score dimensions, aside from proximal transverse arch, which disproportionately dilated. AIsth undersizing prevailed mid-term and trended towards a higher reintervention rate. Initial reconstruction between z-score 0 and +1 resulted in maintenance of that z-score size at mid-term follow-up. Overall, it is crucial to achieve targeted aortic sizing at index operation to maintain appropriate aortic dimensions over time and reduce reintervention risk with specific focus on the AIsth.

Resilience of hospital and allied infrastructure during pandemic and post pandemic periods for maternal health care of pregnant women and infants in Tamil Nadu, India - A counterfactual analysis.

COVID-19 has impacted the healthcare system across the globe. The study will span three pandemic waves in 2020, 2021, and 2022. The goal is to learn how the pandemic affects antenatal care (ANC) and emergency delivery care for pregnant women in Tamil Nadu, India, and how medical services respond. The study employs counterfactual analysis to evaluate the causal impact of the pandemic. A feedforward in combination with a simple auto-regressive neural network (AR-Net) is used to predict the daily number of calls for ambulance services (CAS). Three categories of the daily CAS count between January 2016 and December 2022 are utilised. The total CAS includes all types of medical emergencies; the second group pertains to planned ANC for high-risk pregnant women and the third group comprises CAS from pregnant women for medical emergencies. The second wave's infection and mortality rates were up to six times higher than the first. The phases in wave-II, post-wave-II, wave-III, and post-wave-III experienced a significant increase in both total IFT (inter-facility transfer) and total non-IFT calls covering all emergencies relative to the counterfactual, as evidenced by reported effect sizes of 1 and a range of 0.65 to 0.85, respectively. This highlights overwhelmed health services. In Tamil Nadu, neither emergency prenatal care nor planned prenatal care was affected by the pandemic. In contrast, the increase in actual emergency-related IFT calls during wave-II, post-wave-II, wave-III, and post-wave-III was 62%, 160%, 141%, and 165%, respectively, relative to the counterfactual. During the same time periods, the mean daily CAS related to prenatal care increased by 47%, 51%, 38%, and 38%, respectively, compared to pre-pandemic levels. The expansion of ambulance services and increased awareness of these services during wave II and the ensuing phases of Covid-19 pandemic have enhanced emergency care delivery for all, including obstetric and neonatal cohorts.

Proposed competencies for the performance of cardiovascular computed tomography in pediatric and adult congenital heart disease.

Cardiovascular computed tomography (CCT) is rated appropriate by published guidelines for the initial evaluation and follow up of congenital heart disease (CHD) and is an essential modality in cardiac imaging programs for patients of all ages. However, no recommended core competencies exist to guide CCT in CHD imaging training pathways, curricula development, or establishment of a more formal educational platform. To fill this gap, a group of experienced congenital cardiac imagers, intentionally inclusive of adult and pediatric cardiologists and radiologists, was formed to propose core competencies fundamental to the expert-level performance of CCT in pediatric acquired and congenital heart disease and adult CHD. The 2020 SCCT Guideline for Training Cardiology and Radiology Trainees as Independent Practitioners (Level II) and Advanced Practitioners (Level III) in Cardiovascular Computed Tomography (1) for adult imaging were used as a framework to define pediatric and CHD-specific competencies. Established competencies will be immediately relevant for advanced cardiac imaging fellowships in both cardiology and radiology training pathways. Proposed future steps include radiology and cardiology society collaboration to establish provider certification levels, training case-volume recommendations, and continuing medical education (CME) requirements for expert-level performance of CCT in pediatric and adult CHD.

Comparison of aortic stiffness and hypertension in repaired coarctation patients with a bicuspid versus a tricuspid aortic valve.

BACKGROUND: Coarctation of the aorta (COA) is associated with reduced aortic distensibility and systemic hypertension (HTN). 60-85% of COA patients have a bicuspid aortic valve (BAV). It is not known if the presence of a BAV accentuates the aortopathy and HTN in CoA patients. We examined whether patients with COA and a BAV had lower aortic distensibility by CMR, and a higher prevalence of systemic HTN compared with COA patients with a tricuspid aortic valve (TAV). METHODS: In successfully repaired COA patients excluding those with residual COA, ascending aorta (AAO) and descending aorta (DAO) distensibility was calculated by CMR. HTN was assessed using standard pediatric and adult criteria. RESULTS: Among 215 COA patients (median age 25.3 years), 67% had a BAV, and 33% had a TAV. Median AAO distensibility z-score was lower in the BAV group (- 1.2 versus - 0.7; p = 0.014) but DAO distensibility was similar in BAV and TAV patients. HTN prevalence was similar in BAV (32%) and TAV groups (36%, p = 0.56). On multivariable analysis controlling for confounders, HTN was not associated with BAV but was associated with male gender (p = 0.003) and older age at follow-up (p = 0.004). CONCLUSIONS: In young adults with treated COA, those with a BAV had stiffer AAO compared to those with a TAV, but DAO stiffness was similar. HTN was not related to BAV. These results suggest that although the presence of a BAV in COA exacerbates the AAO aortopathy, it does not exacerbate the more generalized vascular dysfunction and associated HTN.

Pacemaker lead insertion sites contribute to abnormalities of myocardial function and histopathology.

BACKGROUND: Ventricular pacing can cause myocardial dysfunction, but how lead anchoring to the myocardium affects function has not been studied. OBJECTIVE: The purpose of this study was to evaluate patterns of regional and global ventricular function in patients with a ventricular lead using cine cardiac computed tomography (CCT) and histology. METHODS: This was a single-center retrospective study with 2 groups of patients with a ventricular lead: (1) those who underwent cine CCT from September 2020 to June 2021 and (2) those whose cardiac specimen was analyzed histologically. Regional wall motion abnormalities on CCT were assessed in relation to lead characteristics. RESULTS: For the CCT group, 122 ventricular lead insertion sites were analyzed in 43 patients (47% female; median age 19 years; range 3-57 years). Regional wall motion abnormalities were present at 51 of 122 lead insertion sites (42%) in 23 of 43 patients (53%). The prevalence of a lead insertion-associated regional wall motion abnormality was higher with active pacing (55% vs 18%; P < .001). Patients with lead insertion-associated regional wall motion abnormalities had a lower systemic ventricular ejection fraction (median 38% vs 53%; P < .001) than did those without regional wall motion abnormalities. For the histology group, 3 patients with 10 epicardial lead insertion sites were studied. Myocardial compression, fibrosis, and calcifications were commonly present directly under active leads. CONCLUSION: Lead insertion site-associated regional wall motion abnormalities are common and associated with systemic ventricular dysfunction. Histopathological alterations including myocardial compression, fibrosis, and calcifications beneath active leads may explain this finding.

Double Aortic Arch with Atresia of the Left Aortic Arch Proximal to the Left Common Carotid Artery: First Report of Documentation by Surgical Observation.

Double aortic arch associated with atresia of the left arch proximal to the left common carotid artery has been considered a theoretical possibility. To our knowledge, we report the first patient with this anatomy confirmed by surgical observation.

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation.

BACKGROUND: Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. METHODS: This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. RESULTS: Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2-9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (- 0.61%/year vs. - 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). CONCLUSIONS: Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

Follow-up cardiac magnetic resonance in children with vaccine-associated myocarditis.

Myocarditis is a rare complication of the COVID-19 mRNA vaccine. We previously reported a case series of 15 adolescents with vaccine-associated myocarditis, 87% of whom had abnormalities on initial cardiac magnetic resonance (CMR), including late gadolinium enhancement (LGE) in 80%. We performed follow-up CMRs to determine the trajectory of myocardial recovery and better understand the natural history of vaccine-associated myocarditis. Case series of patients age < 19 years admitted to Boston Children's Hospital with acute vaccine-associated myocarditis following the BNT162b2 vaccine who had abnormal CMR at the time of initial presentation, and underwent follow-up testing. CMR assessment included left ventricular (LV) ejection fraction, T2-weighted myocardial imaging, LV global native T1, LV global T2, extracellular volume (ECV), and late gadolinium enhancement (LGE). Ten patients (9 male, median age 15 years) with vaccine-associated myocarditis underwent follow-up CMR at a median of 92 days (range 76-119) after hospital discharge. LGE was persistent in 80% of patients, though improved from prior in all cases. Two patients (20%) had abnormal LV global T1 at presentation, which normalized on follow-up. ECV decreased between acute presentation and follow-up in 6/10 patients; it remained elevated at follow-up in 1 patient and borderline in 3 patients. CONCLUSION: CMR performed ~3 months after admission for COVID-19 vaccine-associated myocarditis showed improvement of LGE in all patients, but persistent in the majority. Follow-up CMR 6-12 months after acute episode should be considered to better understand the long-term cardiac risks. WHAT IS KNOWN: • Myocarditis is a rare side effect of COVID-19 mRNA vaccine. •Late gadolinium enhancement is present on most cardiac magnetic resonance at the time of acute presentation. WHAT IS NEW: •Late gadolinium enhancement improved on all repeat cardiac magnetic resonance at 3-month follow-up. •Most patients still had a small amount of late gadolinium enhancement, the clinical significance of which is yet to be determined.

Utility of cardiac CT in infants with congenital heart disease: Diagnostic performance and impact on management.

BACKGROUND: Advances in cardiac CT (CCT) scanner technology allow imaging without anesthesia, and with low radiation dose, making it an attractive technique in infants with congenital heart disease. However, the utility of CCT using a dual-source scanner with respect to diagnostic performance and impact on management has not been systematically studied in this population. METHODS: Retrospective review of infants who underwent CCT to determine the utility of CCT with respect to the following: answering the primary diagnostic question, providing new diagnostic information, prompting a change in management, and concordance with catheterization or surgical inspection. RESULTS: A total of 156 infants underwent 172 scans at a median age of 64 days, (IQR 4-188) from Jan 2016-Dec 2019. The most frequent diagnostic question was related to the pulmonary arteries (43%), followed by the aortic arch (30%), pulmonary veins (26%), coronary arteries (17%), patent ductus arteriosus (10%) and others (9%). A high-pitch spiral scan was frequently used (90%). The median effective radiation dose was low (0.66 â€‹mSv) and general anesthesia was used infrequently (23%). CCT answered the primary diagnostic question in 168/172 (98%) and added to the diagnostic information already available by echocardiography in 161/172 (96%) scans. CCT led to a change in management following 78/172 (53%) scans and had an impact on management following 167/172 (97%) scans. On follow-up, after 107/172 (62%) scans, subjects underwent cardiac surgery, and after 55/172 (32%) scans, they had cardiac catheterization. CCT findings were concordant with catheterization and/or surgical inspection in 156/159 (98%) scans. CONCLUSIONS: In infants with complex congenital heart disease, CCT was accurate, answered the diagnostic questions in nearly all cases, and frequently added diagnostic information that impacted management. Radiation exposure was low, and anesthesia was needed infrequently.

Association of Myocarditis With BNT162b2 Messenger RNA COVID-19 Vaccine in a Case Series of Children.

Importance: The BNT162b2 (Pfizer-BioNTech) messenger RNA COVID-19 vaccine was authorized on May 10, 2021, for emergency use in children aged 12 years and older. Initial reports showed that the vaccine was well tolerated without serious adverse events; however, cases of myocarditis have been reported since approval. Objective: To review results of comprehensive cardiac imaging in children with myocarditis after COVID-19 vaccine. Design, Setting, and Participants: This study was a case series of children younger than 19 years hospitalized with myocarditis within 30 days of BNT162b2 messenger RNA COVID-19 vaccine. The setting was a single-center pediatric referral facility, and admissions occurred between May 1 and July 15, 2021. Main Outcomes and Measures: All patients underwent cardiac evaluation including an electrocardiogram, echocardiogram, and cardiac magnetic resonance imaging. Results: Fifteen patients (14 male patients [93%]; median age, 15 years [range, 12-18 years]) were hospitalized for management of myocarditis after receiving the BNT162b2 (Pfizer) vaccine. Symptoms started 1 to 6 days after receipt of the vaccine and included chest pain in 15 patients (100%), fever in 10 patients (67%), myalgia in 8 patients (53%), and headache in 6 patients (40%). Troponin levels were elevated in all patients at admission (median, 0.25 ng/mL [range, 0.08-3.15 ng/mL]) and peaked 0.1 to 2.3 days after admission. By echocardiographic examination, decreased left ventricular (LV) ejection fraction (EF) was present in 3 patients (20%), and abnormal global longitudinal or circumferential strain was present in 5 patients (33%). No patient had a pericardial effusion. Cardiac magnetic resonance imaging findings were consistent with myocarditis in 13 patients (87%) including late gadolinium enhancement in 12 patients (80%), regional hyperintensity on T2-weighted imaging in 2 patients (13%), elevated extracellular volume fraction in 3 patients (20%), and elevated LV global native T1 in 2 patients (20%). No patient required intensive care unit admission, and median hospital length of stay was 2 days (range 1-5). At follow-up 1 to 13 days after hospital discharge, 11 patients (73%) had resolution of symptoms. One patient (7%) had persistent borderline low LV systolic function on echocardiogram (EF 54%). Troponin levels remained mildly elevated in 3 patients (20%). One patient (7%) had nonsustained ventricular tachycardia on ambulatory monitor. Conclusions and Relevance: In this small case series study, myocarditis was diagnosed in children after COVID-19 vaccination, most commonly in boys after the second dose. In this case series, in short-term follow-up, patients were mildly affected. The long-term risks associated with postvaccination myocarditis remain unknown. Larger studies with longer follow-up are needed to inform recommendations for COVID-19 vaccination in this population.

Longitudinal changes in extent of late gadolinium enhancement in repaired Tetralogy of Fallot: a retrospective analysis of serial CMRs.

BACKGROUND: Right ventricular (RV) late gadolinium enhancement (LGE) occurs due to surgical scarring and RV remodeling, and has been shown to be associated with clinical outcomes in Tetralogy of Fallot (TOF). However, it is not known if cardiovascular magnetic resonance (CMR) LGE extent progresses over time, and therefore, it is not known if serial reassessment of LGE is necessary. We determined the rate of progression in the extent of RV LGE on serial CMR examinations in repaired TOF. METHODS: Retrospective review of 127 patients after TOF repair (49% male, median age at first CMR 18.9 years (Interquartile range (IQR) 13.3,27.0) who had at least two CMRs (median follow-up duration of 4.0 years (IQR 2.1,5.9)) was performed. 84/127 patients had no interventions between serial CMRs (Group 1) while 43/127 patients had transcatheter or surgical intervention between CMRs (Group 2). The extent of RV LGE was assessed using 2 methods: a semiquantitative RV LGE score and a quantitative RV LGE extent expressed as % of RV mass. Mixed effects linear regression modeling to estimate changes in LGE over time. RESULTS: RV LGE was present in all patients on the first CMR. % RV LGE extent and LGE score did not increase over time in either patient group. The mean 5 year rates of change were small and negative for both % RV LGE extent [- 2.3 (95% CI - 2.9, - 1.8, p < 0.001) in Group 1, and - 1.9 (95% CI - 3.2, - 0.7, p = 0.004) in Group 2], and RV LGE score [- 0.9 (95% CI - 1.1, - 0.6, p < 0.001) in Group 1, and - 0.5 (95% CI - 1.1, - 0.0, p = 0.047) in Group 2]. CONCLUSIONS: In this serial CMR evaluation of children and adults with repaired TOF, no significant progression in the extent of RV LGE was seen on intermediate term follow-up. Given recent concerns regarding the safety of gadolinium-based contrast agents, frequent assessment of LGE may not be necessary in follow-up.

Acute and Short-Term Outcomes of Percutaneous Transcatheter Mitral Valve Replacement in Children.

[Figure: see text].

Rapid ascending aorta stiffening in bicuspid aortic valve on serial cardiovascular magnetic resonance evaluation: comparison with connective tissue disorders.

BACKGROUND: Aortic stiffness has been shown to be abnormal in patients with bicuspid aortic valve (BAV), and is considered a component of the aortopathy associated with this condition. Progressive aortic stiffening associated with aging has been previously described in normal adults. However, it is not known if aging related aortic stiffening occurs at the same rate in BAV patients. We determined the longitudinal rate of decline in segmental distensibility in BAV patients using serial cardiovascular magnetic resonance (CMR) studies, and compared to previously published results from a group of patients with connective tissue disorders (CTD). METHODS: A retrospective review of CMR and clinical data on children and adults with BAV (n = 49, 73% male; 23 ± 11 years) with at least two CMRs (total 98 examinations) over a median follow-up of 4.1 years (range 1-9 years) was performed to measure aortic distensibility at the ascending (AAo) and descending aorta (DAo). Longitudinal changes in aortic stiffness were assessed using linear mixed-effects modeling. The comparison group of CTD patients had a similar age and gender profile (n = 50, 64% male; 20.6 ± 12 years). RESULTS: Compared to CTD patients, BAV patients had a more distensible AAo early in life but showed a steeper decline in distensibility on serial examinations [mean 10-year decline in AAo distensibility (× 10-3 mmHg-1) 2.4 in BAV vs 1.3 in CTD, p = 0.005]. In contrast, the DAo was more distensible in BAV patients throughout the age spectrum, and DAo distensibility declined with aging at a rate similar to CTD patients [mean 10 year decline in DAo distensibility (× 10-3 mmHg-1) 0.3 in BAV vs 0.4 in CTD, p = 0.58]. CONCLUSIONS: On serial CMR measurements, AAo distensibility declined at significantly steeper rate in BAV patients compared to a comparison group with CTDs, while DAo distensibility declined at similar rates in both groups. These findings offer new mechanistic insights into the differing pathogenesis of the aortopathy seen in BAV and CTD patients.

Pediatric Heart Network Echocardiographic Z Scores: Comparison with Other Published Models.

BACKGROUND: Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models. METHODS: Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit). Model comparisons were performed by evaluating (1) overlaid graphs of measurement versus body surface area with curves at Z = -2, 0, and +2; (2) scatterplots of PHN versus other Z scores with correlation coefficients; (3) Bland-Altman plots of PHN versus other Z scores; and (4) comparison of median Z scores for each model. RESULTS: For most measurements, PHN Z score curves were similar to Boston and Italian curves but diverged from Detroit curves at high body surface areas. Correlation coefficients were high when comparing the PHN model with the others, highest with Boston (mean, 0.99) and lowest with Detroit (mean, 0.90). Scatterplots suggested systematic differences despite high correlations. Bland-Altman plots also revealed poor agreement at both extremes of size and a systematic bias for most when comparing PHN against Italian and Detroit Z scores. There were statistically significant differences when comparing median Z scores between the PHN and other models. CONCLUSIONS: Z scores from the multicenter PHN model correlated well with previous single-center models, especially the Boston model, which also had a large sample size and similar methodology. The Detroit Z scores diverged from the PHN Z scores at high body surface area, possibly because there were more subjects in this category in the PHN database. Despite excellent correlation, significant differences in Z scores between the PHN model and others were seen for many measurements. This is important when comparing publications using different models and for clinical care, particularly when Z score thresholds are used to guide diagnosis and management.