What is pentalogy of Cantrell?

We present the unusual case of an 8-month-old female with tetralogy of Fallot, coarctation of aorta, and complete presentation of pentalogy of Cantrell. A meta-analysis of 236 cases of Cantrell's syndrome reported in the literature was performed to compare intracardiac findings.

The Effect of Stenting on Post-Stenotic Dilatation in Coarctation of The Aorta.

The effect of stenting of native aortic coarctation (CoA) on post-stenotic dilatation (PSD) has not been previously described. We hypothesized that CoA stenting may lead to positive remodeling of PSD. Retrospective analysis of patients who underwent stent implantation for native CoA from 1999 to 2021 was performed. Primary outcome was incremental change PSD diameter and the PSD/DescAo (Descending Aorta) following stent implantation and comparison between covered and bare-metal stents. 90 consecutive patients, (26 female, average age at first intervention 12.0 years) were included. 35 patients (38.9%) underwent dilatation with bare-metal stents and 55 patients (61.1%) with covered stents. The covered stent subgroup was older (14.0 vs. 9.2 years old, p < 0.001) and PSD was larger (17.0 vs 14.0 mm, p < 0.001). Over a mean of 3.2 years, mean inter-catheterization growth of the PSD was blunted [- 0.05 mm, 95% CI (- 1.5 to 1.4)]. The covered stent subgroup demonstrated a negative inter-catheterization growth compared to the bare-metal stent subgroup (- 0.7 vs 1.6 mm, p < 0.001). When controlled for somatic growth, the PDS/DescAo decreased more significantly among those with covered stent vs bare metal (- 0.12 vs - 0.058, p = 0.004). Stenting of native CoA blunts the growth of PSD; covered stents were significantly associated with regression of the diameter of the PSD over time compared to bare-metal stents.

Antenatal Risk of Coarctation for Newborns at Hartford Hospital (ARCH) Pathway: A Predictor of Postnatal Management Strategy.

The diagnosis of coarctation of the aorta (CoA) prior to birth can be challenging due to the physiologic changes during postnatal transition. Prenatal risk stratification can standardize postnatal management and improve outcome. CT Children's Fetal Cardiology created the Antenatal Risk of CoA in Hartford (ARCH) clinical pathway defining four distinct postnatal order sets based on degree of suspicion for ductal dependency on fetal evaluation: low, low-moderate, moderate-high, and high risk. This study aims to evaluate safety and efficacy of the ARCH pathway in neonates with suspected CoA. This study was a single-center, retrospective chart review evaluating maternal-infant dyads with findings concerning for CoA between July 2004 and July 2021, before and after ARCH pathway implementation. Neonates were evaluated for the presence or absence of critical CoA and postnatal clinical data were collected. Statistical analysis was performed using chi square and Fisher's exact test. There were 108 maternal-infant dyads studied, comprising 53 non-pathway patients and 55 ARCH pathway participants. Thirty-three neonates had critical CoA, comprising 23 non-pathway and 10 ARCH pathway subjects. Patients categorized in the high-risk group were highly associated with critical CoA (P = 0.003). Non-pathway neonates with CoA demonstrated higher likelihood of hospital transfer compared to ARCH pathway neonates (56.5% vs. 10.0%, P = 0.021). NICU admission, prostaglandin administration, and intubation were not significantly different between before and after ARCH implementation (P < 0.05). More echocardiograms were performed in ARCH pathway neonates without CoA than their non-pathway counterparts (1.586 vs. 2.133, P = 0.049). The ARCH pathway is a safe, reliable prenatal risk stratification system to help guide management of patients with critical CoA. These results identify effective targets of modification to the pathway to reduce resource utilization without compromising safety.

Clinical features, hemodynamics, and outcomes of pulmonary hypertension in adults with coarctation of aorta.

BACKGROUND: Pulmonary hypertension (pH) and secondary right ventricle dysfunction is present in 20 % of adults with coarctation of aorta (COA) based on echocardiographic studies. There are limited data about invasive hemodynamic characterization of PH in COA. The purpose of this study was to delineate the clinical features, hemodynamics, and outcomes of PH in COA. METHOD: Retrospective cohort study of adults with repaired COA that underwent right heart catheterization (RHC). PH was defined as pulmonary artery (PA) mean pressure > 20 mmHg, and PH was classified as isolated precapillary PH and combined pre/postcapillary PH. RESULTS: Of 99 COA patients that underwent RHC, 57 (58 %) had PH. Of the patients with PH, 14 (25 %) had isolated precapillary PH while 43 (75 %) had postcapillary PH with or without precapillary disease. The correlates of PH were PA compliance (adjusted OR 0.79, 95 % CI 0.71-0.86 per 1 ml/mmHg), left atrial reservoir strain (adjusted OR 0.95, 95 % CI 0921-0.98 per 1 %), and atrial fibrillation (adjusted OR 2.18, 95 % CI 1.20-13.5). Higher PA mean pressure was associated with risk of cardiovascular events (adjusted HR 1.04, 95 % CI 1.02-1.06 per 1 mmHg) and all-cause mortality (adjusted HR 1.05, 95 % CI 1.02-1.08 per 1 mmHg). CONCLUSIONS: PH was present in over half of adults with COA referred for RHC, and one-quarter of the patients with PH presented with isolated precapillary PH suggesting an underlying PA vascular dysfunction as a contributing mechanism. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population. CLINICAL SUMMARY: Of 99 adults with repaired coarctation of aorta (COA) that underwent right heart catheterization, 57 % had pulmonary hypertension (pH). Of the patients with PH, 25 % had isolated precapillary PH while 75 % had combined pre/postcapillary PH. The correlates of PH were pulmonary artery (PA) compliance, left atrial reservoir strain and atrial fibrillation. PH was associated with cardiovascular events and all-cause mortality. These data suggest PA vascular dysfunction in addition to left-sided heart disease as potential etiologies for PH in this population. Further studies are required to determine optimal therapies and strategies for prevention and treatment of PH in this population.

The Enduring Impact of Shape Following Perfect Coarctation of the Aorta Repair.

Objectives: Aortic arch appearances can be associated with worse cardiac function and chronic hypertension late after coarctation of the aorta (CoA) repair, even without residual obstruction. Statistical shape modeling (SSM) has identified specific 3D arch shapes linked to poorer cardiovascular outcomes. We sought a mechanistic explanation. Methods: From 53 asymptomatic patients late after CoA repair with no residual obstruction (age: 22.3 ± 5.6 years; 12-38 years after operation), eight aortic arch shapes associated with the four best and four worst cardiovascular parameters were obtained from 3D SSM. Four favorable shapes were affiliated with left ventricular (LV) ejection fraction +2 standard deviation (SD) values from the mean, and indexed LV end diastolic volume/indexed LV mass/resting systolic blood pressure that were -2SD. Four unfavorable shapes were defined by the reverse. Computational Fluid Dynamics modeling was carried out to assess differences in pressure gradient across the aortic arch and viscous energy loss (VEL) between favorable and unfavorable aortic arches. Results: In all aortic arches, the pressure gradients were clinically insignificant (<8 mm Hg). However, in the four unfavorable aortic arches, VEL were uniformly higher than those in the favorable shapes (VEL difference: 15%-32%). There was increased turbulence and more complex propagation of VEL along with the unfavorable aortic arches. Conclusions: This study reveals the variable flow dynamics that underpin the association of aortic arch shapes with worse cardiovascular outcomes late after successful CoA repair. Higher VEL persists in the unfavorable aortic arch shapes. Further understanding of the mechanism of viscous energy loss in cardiovascular maladaptation may afford mitigating strategies to monitor and modify this unrelenting liability.

CT Angiography for Aortic Arch Anomalies: Prevalence, Diagnostic Efficacy, and Illustrative Findings.

Aortic arch anomalies encompass a diverse spectrum of conditions. Elucidating the prevalence of these anomalies, their impact on patient wellbeing, and the most effective diagnostic tools are crucial steps in ensuring optimal patient care. This paper aims to explore the various presentations of aortic arch anomalies, emphasizing the remarkable utility of computed tomography (CT) angiography in their definitive diagnosis and characterization. We conducted a retrospective study on patients who were submitted to the CT angiography of the thoracic aorta or supra-aortic trunks, or the contrast-enhanced CT scans of the thorax and/or cervical region between January 2021 and February 2024 in our Hospital. Out of the total of 2350 patients, 18 were diagnosed with aortic arch anomalies, with an average age of approximately 55 years. The aortic arch anomalies identified in the study were as follows: left aortic arch with the aberrant origin of the right subclavian artery, right aortic arch (types I and II), double aortic arch, aortic coarctation, aortic pseudocoarctation, and ductus diverticulum. Although often asymptomatic, aortic arch anomalies require recognition and CT using advanced post-processing techniques is the optimal diagnostic method with the ability to also identify other associated cardiac or vascular malformations.

Hemodynamics in the treatment of pseudoaneurysm caused by extreme constriction of aortic arch with coated stent.

Objectives: To evaluate the changes in distal vascular morphology and hemodynamics in patients with extremely severe aortic coarctation (CoA) after covered palliative (CP) stent dilation with different surgical strategies. Materials and methods: Perioperative computed tomography angiography and digital subtraction angiography were utilized to construct three aortic models with varying stenosis rates and one follow-up model in a patient with extremely severe CoA. The models included: an idealized non-stenosed model (A: 0%), a model post initial stent deployment (B: 28%), a model post balloon expansion (C: 39%), and a model 18 months after post-balloon expansion (D: 39%). Consistent boundary conditions were applied to all models, and hemodynamic simulation was conducted using the pure fluid method. Results: The narrowest and distal diameter of the stent increased by 34.71% and 59.29%, respectively, from model B to C. Additionally, the distal diameter of the stent increased by -13.80% and +43.68% compared to the descending aorta diameter, respectively. Furthermore, the ellipticity of the maximum cross-section of the aneurysm region in model A to D continued to increase. The oscillatory shear index at the stenosis to the region of the aneurysm were found to be higher in Models A and B, and lower in Models C and D. At the moment of maximum flow velocity, the blood flow distribution in models A and B was more uniform in the widest section of the blood vessels at the distal end of the stenosis, whereas models C and D exhibited disturbed blood flow with more than 2 eddy currents. The time-averaged wall shear stress (TAWSS) decreased in the distal and basal aneurysms, while it significantly increased at the step position. The aneurysmal region exhibited an endothelial cell activation potential value lower than 0.4 Pa-1. Conclusion: In patients with extremely severe CoA, it is crucial to ensure that the expanded diameter at both ends of the CP stent does not exceed the native vascular diameter during deployment. Our simulation results demonstrate that overdilation leads to a decrease in the TAWSS above the injured vessel, creating an abnormal hemodynamic environment that may contribute to the development and enlargement of false aneurysms in the early postoperative period. Clinical Trial Registration: ClinicalTrials.gov, (NCT02917980).

Predictors of left ventricular outflow tract obstruction after biventricular repair in interrupted aortic arch or aortic coarctation.

BACKGROUND: Left ventricular outflow tract obstruction (LVOTO) re-intervention is a significant cause of morbidity and mortality in patients with coarctation of the aorta (CoA) or interrupted aortic arch (IAA) after aortoplasty. METHODS: This retrospective study analyzed data from neonates with IAA/CoA who underwent biventricular repair between 2012 and 2022. LVOTO events were defined by the detection of color Doppler flow acceleration ≥3.0 m/s at the valvular, subvalvular, or supravalvular regions via transthoracic echocardiography, and the necessity for surgical or catheter intervention to relieve the obstruction. RESULTS: Among 121 neonates with CoA/IAA, 16 (13.7%) primary aortoplasty patients developed LVOTO. Additionally, one patient (25%) who underwent a staged Yasui operation developed LVOTO due to a narrowed ventricular septal defect-pulmonary atresia tunnel. During follow-up, 58% of patients with a bicuspid valve and 25% of patients with a subaortic ridge developed LVOTO. The combination of either a bicuspid valve, subaortic ridge, or an aortic valve annulus Z-score < -3.0 predicted a high re-intervention rate (7/8 [87.5%]). CONCLUSIONS: In patients with IAA/CoA, the presence of multiple risk factors, including a bicuspid valve, subaortic ridge, and an aortic valve annulus Z-score < -3.0, is associated with a significantly increased rate of re-intervention for LVOTO.

Aortic isthmus flow reversal in fetal coarctation of the aorta and the associated factors.

INTRODUCTION: The aim of this study was to investigate the presence of aortic isthmus flow reversal and its associated factors in fetuses with positive and false-positive coarctation of the aorta (CoA) compared with normal controls. MATERIAL AND METHODS: Pregnant women with fetuses suspected of CoA and normal control were enrolled, and these women experienced prenatal ultrasound scan and followed up for 6 months after birth to confirm the presence of CoA. All the ultrasound parameters were analyzed. RESULTS: A total of 134 pregnant women were enrolled, with 43 CoA-positive fetuses and 91 CoA false-positive fetuses, and 334 matched pregnant women were enrolled in the control group. Aortic isthmus flow reversal occurred in 28 (65.1%) fetuses in the CoA-positive group, significantly (p < 0.05) more than in the false-positive (37 or 40.7%) or control group (64 or 19.2%). Aortic isthmus flow reversal was mostly in the full systole (n = 17 or 60.7%) or late systole and early-middle diastole (n = 10 or 35.7%) in the CoA-positive fetuses (n = 27 or 96.4%), significantly (p < 0.001) different from that in the false-positive or control group. The aortic isthmus flow reversal peak systolic velocity (PSV), flow volume, and ratio of reversed flow/forward flow were significantly (p < 0.05) increased in the CoA-positive and false-positive groups than in the control group. The aortic isthmus flow reversal incidence was significantly (p < 0.05) correlated with the middle cerebral artery (MCA) PSV in the total three groups or in the false-positive group but was significantly (p < 0001) negatively correlated with the MCA resistance index (RI) in the CoA-positive group. The incidence of the aortic isthmus flow reversal was significantly (p < 0.05) positively correlated with the umbilical artery (UA) RI in the false-positive group and with the UA RI in the total three groups. Independently associated factors for aortic isthmus flow reversal were isthmic flow volume/CCO (combined cardiac output) in the CoA-positive group. CONCLUSIONS: Reversal of flow in the aortic isthmus is much more common in true-positive cases of CoA as compared to controls, and isthmic flow reversal in the full systolic phase only suggests presence of CoA. The aortic isthmic reversed flow volume accounts for over half of the isthmic forward flow volume in the CoA-positive fetuses than in the normal controls.

Pulmonary Overcirculation Requiring Surgical and Pulmonary Flow Restrictor Device Intervention in Critical Coarctation of the Aorta-A Case Series.

The use of prostaglandin infusion to maintain patency of the ductus arteriosus in patients with critical coarctation of the aorta (CoA) to support systemic circulation is the standard of care. However, pulmonary overcirculation resulting from a patent ductus arteriosus in patients with critical CoA is not well described in the literature. We report two cases of critical CoA that required invasive measures to control pulmonary blood flow before surgical repair of the CoA. Both patients had signs of decreased oxygen delivery, hyperlactatemia, and systemic to pulmonary flow via the ductus arteriosus. One patient required surgical pulmonary artery banding and the second patient underwent pulmonary flow restrictor device placement for the control of pulmonary blood flow. A rapid improvement in oxygen delivery and normalization of lactate levels were observed after control of pulmonary overcirculation. Both patients underwent successful surgical repair of the coarctation A and were discharged home.

Impact of surgical strategy and postrepair transverse aortic arch size on late hypertension after coarctation repair during infancy.

BACKGROUND: Late hypertension (HTN) after coarctation of the aorta (CoA) repair contributes to higher morbidity and mortality. An association between transverse aortic arch (TAA) hypoplasia and HTN has been found, but its relationship with surgical strategy is unclear. We studied the association between late HTN and initial surgical strategy pertaining to the TAA. METHODS: We retrospectively reviewed patients who underwent surgical repair of CoA during infancy with at least 10 years of follow-up, excluding those with atypical coarctation, major associated heart defects, and residual isthmic narrowing. TAA diameter z-score immediately postrepair was measured as a marker of surgical strategy. Systemic HTN at latest follow-up was assessed using standard criteria. RESULTS: A total of 130 patients underwent surgical repair of CoA (76% via thoracotomy, 24% via sternotomy) with resection and end-to-end anastomosis (62%), extended end-to-end anastomosis (30%), subclavian flap (5%), or arch repair with patch (4%), at a median age of 14 days (interquartile range [IQR], 7-62 days). The median postrepair TAA diameter z-score was -2.04 (IQR, -2.69 to 1.24). At a mean follow-up of 17.3 years, 43 of the 130 patients (33%) developed HTN. After controlling for age at repair, sex, and presence of a genetic syndrome, HTN was not associated with immediate postrepair TAA diameter z-score (P = .41), type of surgical incision (P = .99), or type of surgical repair (P = .66). CONCLUSIONS: In patients undergoing surgical repair of CoA during infancy, late HTN was not associated with immediate postrepair TAA size or surgical strategy pertaining to the TAA. These results suggest that factors other than surgical strategy, such as differential growth of the TAA during childhood, may be important.

The Fate of the Aorta after Coarctation Repair: Open Surgical Replacement of Descending Aorta in a High-Volume Unit.

Objectives: Complications after aortic coarctation repair are associated with high mortality and require surgical or endovascular reintervention. For patients unsuitable for endovascular therapies, reoperation remains the only therapeutic option. However, surgical experience and up-to-date follow-up data concerning this overall rare entity in the spectrum of aortic reoperations are still highly limited. Thus, the aim of this study was to analyze the short-term outcomes and long-term survival of patients undergoing surgical descending aorta repair after previous coarctation repair in a high-volume unit. Methods: We present a retrospective single-center analysis of 25 patients who underwent open descending aorta replacement after initial coarctation repair. The surgical history, concomitant cardiovascular malformations, and preoperative characteristics as well as postoperative complications and long-term survival were analyzed. Results: The mean age at operation was 45.4 ± 12.8 years. A proportion of 68% (n = 17) of the patients were male. The most common complication necessitating reoperation after coarctation repair was aneurysm formation (68%) and re-stenosis (16%). The average time between initial repair and reoperation was 26.3 ± 9.9 years. Technical success was achieved in all the operations, while recurrent nerve damage (24%) and bleeding requiring rethoracotomy (20%) were identified as the most common perioperative complications. The one-year mortality was 0% and the overall long-term survival was 88% at 15 years. Conclusions: Open surgical descending aorta replacement can be performed safely and with excellent survival outcomes even in the challenging subgroup of patients after previous coarctation repair. Thus, reoperation should be considered a feasible approach for patients who are unsuitable for endovascular therapies. Nonetheless, concomitant cardiovascular anomalies and frequent preoperations may complicate the redo operation in this patient population.

Stress Echocardiography in the Follow-Up of Young Patients with Repaired Aortic Coarctation.

Background: Aortic coarctation (CoA) is a congenital heart disease affecting 5-8% of patients, with long-term complications persisting despite successful correction. Stress echocardiography (SE) is increasingly used for evaluating cardiac function under stress, yet its role in repaired CoA remains under-explored. Objective: This study aimed to assess the predictive value of SE and myocardial strain in repaired CoA patients with a history of hypertension without significant gradients or with borderline gradients at rest. Methods: Between June 2020 and March 2024, we enrolled 35 consecutive CoA patients with successful repairs and either a history of hypertension or borderline Doppler gradients. Baseline and peak exercise echocardiographic measurements, including left ventricular mass index (LVMi) and global longitudinal strain (LVGLS), were recorded. Patients were followed for up to 4 years. Results: At baseline, the positive SE group had higher systolic blood pressure (SBP) and diastolic blood pressure (DBP) compared to the negative SE group. The positive SE group also exhibited significantly higher basal and peak trans-isthmic gradients. Positive SE was found in 45.7% of patients, with 68.7% of these requiring re-intervention during follow-up. A peak trans-isthmic gradient > 61 mmHg during exercise predicted recoarctation with 100% sensitivity and 71% specificity (AUC = 0.836, p < 0.004). Conclusions: SE identifies at-risk patients post-CoA repair, aiding in early intervention. A peak trans-isthmic gradient > 61 mmHg during exercise is a strong predictor of recoarctation. These findings support incorporating SE into routine follow-up protocols for CoA patients, particularly those with a history of hypertension and borderline gradients, to improve long-term outcomes and quality of life.

Correlation of Near-Infrared Spectroscopy (NIRS) with Invasive Arterial Pressure Monitoring during Aortic Coarctation Surgery in Pediatric Patients.

Aortic coarctation surgery in pediatric patients requires the placement of two arterial cannulas to monitor pressure gradients and surgical correction adequacy. Near-infrared spectroscopy (NIRS) monitoring provides insight into regional blood flow. This study aimed to investigate the correlation between NIRS values and invasive arterial pressures, exploring NIRS monitoring as a potential substitute for arterial cannulation. In a cohort of 21 consecutive pediatric patients undergoing aortic coarctation surgery, recordings of NIRS and invasive arterial pressure values were evaluated at various time intervals. Pearson correlation evaluated the relationship between NIRS values and invasively measured arterial pressures. Moderate to strong correlations were observed between the mean arterial pressure (MAP) of the upper and lower arteries and cerebral (rSO2-C) and somatic (rSO2-S) NIRS values 5 min after cross-clamp placement (r = 0.621, p = 0.003; r = 0.757, p < 0.001). Strong correlations were found 15 min after cross-clamp placement (r = 0.828, p = 0.002; r = 0.783, p = 0.004). Before transfer to the ICU, a strong correlation existed between the upper artery MAP and rSO2-C (r = 0.730, p < 0.001), but there was no correlation between the lower artery MAP and rSO2-S. These findings are promising, but further studies are required to validate it as a reliable substitute for invasive pressure monitoring in this patient population.

Surgical Management of Juxtaductal Coarctation of the Aorta in Adults: A Novel Technique.

Coarctation of the aorta (CoA) is a rare congenital malformation, the symptoms of which may remain subtle in childhood and appear at a later age. It can manifest only with symptoms of upper body hypertension. Various methods have been described for managing coarctation of the aorta in adults, including surgical or percutaneous balloon angioplasty with or without stent placement and medical therapy. Surgical approaches include an extra-anatomical bypass through a left lateral thoracotomy, a median sternotomy, or a combined median sternotomy and a laparotomy incision; all have their merit in overcoming the symptoms. We went ahead with an extra-anatomical tube graft between the ascending aorta and the descending thoracic aorta in a 24-year-old patient who presented to us with a diagnosis of coarctation of the aorta.

Impact of COVID-19 on the Diagnosis of Coarctation of the Aorta in Infants.

Newborns with coarctation of the aorta are a potentially vulnerable population whose diagnosis could have been impacted by the COVID-19 pandemic. The objectives of this study were to assess if there was delayed diagnosis of infants with coarctation and if they had higher acuity prior to repair after the start of the pandemic. The Pediatric Health Information Systems database was queried for patients less than three months of age who underwent surgical repair or palliation of coarctation of the aorta. Patients were divided into three time periods: (1) pre-COVID (October 2017-December 2019), (2) early COVID (January 2020-December 2020), and (3) late COVID (January 2021-December 2021). The outcomes were age at repair and pre-procedure acuity. Among the 4885 patients, the median time to repair was 10 days during all time periods. Use of pre-procedure mechanical ventilation, vasopressors, and extracorporeal membranous oxygenation did not increase after the start of the pandemic. Median length of hospital stay increased after the start of the pandemic and did not return to baseline (22 days, 24 days, and 25 days, sequentially, p < 0.01). When analyzing patients who presented to the surgical hospital after 3 days of life, there were no differences in age at repair, pre-procedural acuity, or other outcomes. Age at repair of coarctation of the aorta and acuity did not change after the start of the COVID-19 pandemic. This suggests that the safeguards in place to ensure timely diagnosis of critical heart disease were adequate during this time of disruption.

Role and Mechanism of Lamellar Derived Growth Factor /AKT Pathway in Ventricular Remodeling Induced by Pressure Overload.

This study aimed to investigate the role and underlying mechanisms of the platelet-derived growth factor (PDGF)/protein kinase B (AKT) signaling pathway in pressure overload-induced ventricular remodeling. Ventricular remodeling, a critical pathological process in heart failure, is commonly triggered by pressure overload. While PDGF is known to promote cell proliferation and growth, the AKT pathway is crucial for cell growth, survival, and metabolism. However, the specific role of the PDGF/AKT pathway in pressure overload-induced ventricular remodeling remains unclear. Thus, this study aimed to elucidate the precise mechanisms of PDGF/AKT involvement in this process using animal models and cell experiments. 45 female C57BL/6 mice were utilized, randomly divided into three groups: model group (M group, n = 15), control group (C group, n = 15), and experimental group (E group, n = 15). M group mice underwent thoracotomy without aortic constriction (AC). C group mice received phosphate-buffered saline (PBS) and dimethyl sulfoxide (DMSO) treatment following AC surgery. E group mice were treated with the PDGF receptor inhibitor AG1296 and PBS solution after AC surgery. Additionally, 293 T cells were categorized into three groups: PDGF shRNA transfected group (downregulating PDGF expression, D group), PDGF overexpression group (B group), and control group (NV group). Left ventricular end-systolic volume (LVESV) and ejection fraction (FS) of the mice were measured via echocardiography. Western blot analysis was conducted to assess the expression levels of p-AKT and t-AKT in myocardial tissues. Furthermore, myocardial cell area was measured using hematoxylin and eosin (HE) staining and image analysis software. The LVESV in the C group was significantly higher than in the M and E groups (48.32 ± 3.08 mL vs. 18.24 ± 3.19 mL and 25.44 ± 3.12 mL, P < 0.05). The FS in the C group was significantly lower compared to the M and E groups (21.18 ± 2.99% vs. 42.45 ± 3.02% and 26.89 ± 2.54%, P < 0.05). Western blot analysis revealed that p-AKT and t-AKT levels were significantly elevated in the C group and PDGF overexpression group (B group) compared to the M and PDGF shRNA groups (D group) (P < 0.05). HE staining showed a significant increase in myocardial cell cross-sectional area in the C and D groups, with the most pronounced enlargement in the D group (P < 0.05). PDGF facilitates pressure overload-induced ventricular remodeling and myocardial fibrosis. Inhibition of the PDGF/AKT signaling pathway effectively mitigates myocardial cell hypertrophy and ventricular remodeling. These findings offer novel potential targets and therapeutic strategies for the treatment of pressure overload-related heart failure.

Accurate prenatal diagnosis of coarctation of the aorta by 3-step echocardiographic diagnostic protocol.

BACKGROUND: Coarctation of the aorta (CoA) is the most common undiagnosed congenital heart defect during prenatal screening. High false positive and false negative rates seriously affect prenatal consultation and postnatal management. The objective of the study was to assess the utility of various measurements to predict prenatal CoA and to derive a diagnostic algorithm. METHODS: One hundred and fifty-four fetuses with suspected CoA who presented at Fuwai Hospital between December 2017 and August 2021 were enrolled and divided into confirmed CoA cases (n = 47) and false positive cases (n = 107), according to their postnatal outcomes. The transverse aortic arch, isthmus, and descending aorta were measured in the long-axis view of the aortic arch. The angle between the transverse aortic arch (TAO) and the descending aortic arch (DAO) was defined as the TAO-DAO angle and measured in the long axis or sagittal view. Based on the database in GE Voluson E10 and the formula (Z = [Formula: see text]), the standard score (Z-score) of the dimensions of the aorta were calculated in relation to the gestational age. The main echocardiographic indices were combined to design a 3-step diagnostic protocol. The TAO-DAO angle was used as the first step in the diagnostic model. The diameter of the transverse arch and the Z-score of the isthmus were the second step. The third-step indices included a Z-score of the transverse arch, diameter of the isthmus, distance from the left subclavian artery (LSA) to left common carotid artery (LCCA), the ratio of isthmus diameter and LSA diameter and ratio of the distances (the distance between the LSA and LCCA to the distance between the right innominate artery and LCCA). The receiver operating characteristic (ROC) curve determined the predictive capability of each diagnostic parameter, and the kappa test determined the diagnostic accuracy of the proposed model. RESULTS: The cases with confirmed CoA had thinner transverse arches (1.92 ± 0.32 mm vs. 3.06 ± 0.67 mm, P = 0.0001), lower Z-scores of the isthmus (-8.97 ± 1.45 vs. -5.65 ± 1.60, P = 0.0001), smaller TAO-DAO angles (105.54 ± 11.51° vs. 125.29 ± 8.97°, P = 0.0001) and larger distance between the LSA and LCCA (4.45 ± 1.75 mm vs. 2.74 ± 1.07 mm, P = 0.0001) than the false positive cases. The area under the curve (AUC) was 0.947 (95% CI 0.91-0.98) for the TAO-DAO angle ≤ 115.75°, 0.942 (95% CI 0.91-0.98) for the transverse arch diameter ≤ 2.31 mm, 0.937 (95% CI 0.90-0.98) for the Z-score of the isthmus ≤ -7.5, and 0.975 (95% CI 0.95-1.00) for the 3-step diagnostic protocol with 97.8% sensitivity and 97.2% specificity. The kappa test showed that the model's diagnostic accuracy was consistent with postnatal outcomes (kappa value 0.936, P = 0.0001). CONCLUSIONS: The 3-step diagnostic protocol included the three most useful measurements and the additional indices with appropriate cut-off values. The algorithm is useful for the detection of aortic coarctation in fetuses with a high degree of accuracy. TRIAL REGISTRATION: Retrospectively registered.

Transcarotid TAVR for Severe Bicuspid Aortic Stenosis With Virtually Atretic Coarctation of the Thoracic Aorta.

Transcatheter aortic valve replacement by alternate access sites allows for treatment of patients with unfavorable anatomy for a transfemoral approach. To our knowledge, we present the first reported case of successful transcatheter aortic valve replacement via the transcarotid approach in a 65-year-old man with a symptomatic severe bicuspid aortic valve stenosis and virtually atretic coarctation of the thoracic aorta.