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Echocardiography (Mount Kisco, N.Y.) Jun 2024Assessment of aortic stenosis (AS) is based on aortic valve (AV) gradients and calculation of aortic valve area (AVA). These parameters are influenced by flow and...
PURPOSE
Assessment of aortic stenosis (AS) is based on aortic valve (AV) gradients and calculation of aortic valve area (AVA). These parameters are influenced by flow and dependent on geometric assumptions. The dimensionless index (DI), the ratio of the LVOT time-velocity integral to that of the AV jet, is simple to perform, and is less susceptible to error but has only been examined in small selected groups of AS patients. The objective of this study was to assess the DI and prognosis in a large cohort.
METHODS
All subjects who underwent echocardiography with an assessment of the AV that included DI were included. Association between AV parameters including mean gradient, AVA, DI and AV resistance and mortality and cardiovascular hospitalizations was examined.
RESULTS
A total of 9393 patients (mean age 71 ± 16 years; 53% male) were included. 731 (7.7%) patients had DI less than .25. Increasing age and a diagnosis of heart failure were significantly associated with lower DI. Subjects with low DI had significantly lower ventricular function, a higher incidence of mitral and tricuspid regurgitation, worse diastolic function and more elevated pulmonary pressures. Decreasing DI was associated with significantly decreased survival and event-free survival which remained highly significant on multivariate analysis.
CONCLUSIONS
In a large population of patients with AV disease, decreased DI, was associated with increased mortality and decreased event-free survival. The easily obtained DI identifies a broad range of AS subjects with worse prognosis and should be integrated into the assessment of these complex patients.
Topics: Humans; Male; Female; Aged; Prognosis; Aortic Valve Stenosis; Aortic Valve; Echocardiography; Survival Rate; Aortic Valve Disease; Reproducibility of Results; Severity of Illness Index; Retrospective Studies
PubMed: 38814015
DOI: 10.1111/echo.15848 -
Cureus Apr 2024This case report describes a 75-year-old female with a medical history including recurrent bowel obstruction due to sigmoid stricture, atrial fibrillation managed with...
This case report describes a 75-year-old female with a medical history including recurrent bowel obstruction due to sigmoid stricture, atrial fibrillation managed with rivaroxaban, a 50-year one pack-per-day smoking history, hypertension, hyperlipidemia, peripheral vascular disease with bilateral iliac stents (2015), stage III chronic kidney disease, and renal artery stenosis with bilateral stenting. She was transferred from outside hospital for an elective sigmoidectomy with ileorectal anastomosis following several recent admissions due to bowel obstruction that had been managed non-operatively. She was deemed optimized for surgery by the primary care team; however, during induction, she developed pulseless ventricular tachycardia requiring extensive resuscitative efforts. Intraoperative findings revealed biventricular failure and a clot in the right pulmonary artery. Despite aggressive treatment, including veno-arterial extracorporeal membrane oxygenation (VA ECMO), the patient's condition deteriorated, and life support was ultimately withdrawn. This case highlights the challenges of managing complex surgical patients and underscores the importance of multidisciplinary care in such cases.
PubMed: 38813292
DOI: 10.7759/cureus.59282 -
European Heart Journal Open May 2024With the 2022 pulmonary hypertension (PH) definition, the mean pulmonary artery pressure (mPAP) threshold for any PH was lowered from ≥25 to >20 mmHg, and the...
AIMS
With the 2022 pulmonary hypertension (PH) definition, the mean pulmonary artery pressure (mPAP) threshold for any PH was lowered from ≥25 to >20 mmHg, and the pulmonary vascular resistance (PVR) value to differentiate between isolated post-capillary PH (IpcPH) and combined pre- and post-capillary PH (CpcPH) was reduced from >3 Wood units (WU) to >2 WU. We assessed the impact of this change in the PH definition in aortic stenosis (AS) patients undergoing aortic valve replacement (AVR).
METHODS AND RESULTS
Severe AS patients ( = 503) undergoing pre-AVR cardiac heart catheterization were classified according to both the 2015 and 2022 definitions. The post-AVR mortality [median follow-up 1348 (interquartile range 948-1885) days] was assessed. According to the 2015 definition, 219 (44% of the entire population) patients had PH: 63 (29%) CpcPH, 125 (57%) IpcPH, and 31 (14%) pre-capillary PH. According to the 2022 definition, 321 (+47%) patients were diagnosed with PH, and 156 patients (31%) were re-classified: 26 patients from no PH to IpcPH, 38 from no PH to pre-capillary PH, 38 from no PH to unclassified PH, 4 from pre-capillary PH to unclassified PH, and 50 from IpcPH to CpcPH (CpcPH: +79%). With both definitions, only the CpcPH patients displayed increased mortality (hazard ratios ≈ 4). Among the PH-defining haemodynamic components, PVR was the strongest predictor of death.
CONCLUSION
In severe AS, the application of the 2022 PH definition results in a substantially higher number of patients with any PH as well as CpcPH. With either definition, CpcPH patients have a significantly increased post-AVR mortality.
PubMed: 38812478
DOI: 10.1093/ehjopen/oeae037 -
Journal of the American College of... Jun 2024Transcatheter correction of sinus venosus defects (SVDs) using balloon-mounted covered stents provides an attractive surgical alternative. Surgery may be complicated by...
BACKGROUND
Transcatheter correction of sinus venosus defects (SVDs) using balloon-mounted covered stents provides an attractive surgical alternative. Surgery may be complicated by superior vena caval or right upper pulmonary vein (RUPV) stenosis, sinus nodal dysfunction, and residual additional pulmonary veins.
OBJECTIVES
Being a new intervention, technical modifications would simplify the procedure, improve universal applicability, and reduce or tackle complications.
METHODS
Patients were included if balloon interrogation of cavoatrial junction confirmed closure of SVD and redirected RUPV to the left atrium. A single-center experience was analyzed to summarize the procedural modifications over 8 years. Transesophageal echocardiogram (TEE) on follow-up was done to identify residual shunt, RUPV flows, and stent thrombosis.
RESULTS
A total of 100 patients including 9 children with a median age of 35 years (range, 4-69 years) underwent SVD closure after balloon interrogation. Among 57 patients balloon interrogated in the first 5 years, 70% underwent transcatheter closure, with 2 failures. RUPV occlusion caused the exclusions. Inclusions improved to 94% among the subsequent 65 balloon interrogations when RUPV protection was implemented, with 1 failure. Stent embolization caused the 3 failures warranting surgery. Recent modifications included limited transesophageal echocardiogram without anesthesia, avoiding venovenous circuit, interrogation with semicompliant balloons, trans-septal RUPV protection, overlapping stents to permit additional vein drainage to superior vena cava and tackle embolizations. There were no deaths. Minor complications included stent embolizations stabilized in catheterization laboratory in 2 patients, left innominate vein jailing in 2 patients, insignificant residual flows, and nonocclusive asymptomatic stent thrombosis in 4 patients.
CONCLUSIONS
Procedural success was 97%. Recent modifications increased patient inclusions, decreased complications, and simplified the intervention.
Topics: Humans; Child; Adolescent; Stents; Child, Preschool; Male; Female; Middle Aged; Adult; Aged; Young Adult; Cardiac Catheterization; Echocardiography, Transesophageal; Heart Septal Defects, Atrial; Retrospective Studies; Treatment Outcome; Follow-Up Studies
PubMed: 38811095
DOI: 10.1016/j.jacc.2024.03.417 -
Journal of Biomechanics Jun 2024To better understand the impact of valvular heart disease (VHD) on the hemodynamics of the circulatory system, investigations can be carried out using a model of the...
To better understand the impact of valvular heart disease (VHD) on the hemodynamics of the circulatory system, investigations can be carried out using a model of the cardiovascular system. In this study, a previously developed hybrid (hydro-numerical) simulator of the cardiovascular system (HCS) was adapted and used. In our HCS Björk-Shiley mechanical heart valves were used, playing the role of mitral and aortic ones. In order to simulate aortic stenosis (AS) and mitral regurgitation (MR), special mechanical devices have been developed and integrated with the HCS. The simulation results proved that the system works correctly. Namely, in the case of AS - the mean pulmonary arterial pressure was increased due to increased preload of the left ventricle and the decrease in right ventricular preload was caused by a decrease in systemic arterial pressure. The severity of AS was performed based on the transaortic pressure gradient as well as using the Gorlin and Aaslid equations. In the case of severe AS, when the mean gradient was above 40 mmHg, the aortic valve orifice area was 0.5 cm, which is in line with ACC/AHA guidelines. For the case of MR - with increasing severity of MR, there was a decrease in the left ventricular pressure and an increase in left atrial pressure. Using mechanical heart valves to simulate VHD by the HCS can be a valuable tool for biomedical research, providing a safe and controlled environment to study and understand the pathophysiology of VHD.
Topics: Humans; Models, Cardiovascular; Computer Simulation; Hemodynamics; Mitral Valve Insufficiency; Aortic Valve Stenosis; Heart Valve Diseases; Heart Valve Prosthesis; Mitral Valve
PubMed: 38805856
DOI: 10.1016/j.jbiomech.2024.112173 -
Circulation May 2024
Topics: Humans; Hypertension, Pulmonary
PubMed: 38805576
DOI: 10.1161/CIRCULATIONAHA.123.066807 -
Andes Pediatrica : Revista Chilena de... Apr 2024Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder that typically presents with cholestasis, cardiac, ocular, skeletal, vascular and renal...
UNLABELLED
Alagille syndrome (ALGS) is an autosomal dominant, multisystem disorder that typically presents with cholestasis, cardiac, ocular, skeletal, vascular and renal abnormalities, and distinct facial features. Most cases are due to variants in the JAG1 gene, with only a small percentage involving a complete gene deletion.
OBJECTIVE
to contribute to the phenotype delineation and interpretation of a microdeletion not previously described in the literature on chromosome 20.
CLINICAL CASE
A 4-month-old female patient was diagnosed with a heart murmur. An echocardiogram revealed pulmonary artery stenosis, which, combined with a prominent forehead observed on physical examination, determined her referral to clinical genetics. Because ALGS was suspected, complementary studies were performed, revealing butterfly vertebras and a genetic panel identified a pathogenic heterozygous deletion, encompassing the entire coding sequence of the JAG1 gene. To rule out a more extensive deletion, a chromosome microarray was performed, confirming a pathogenic microdeletion on chromosome 20 of 378 kb (arr[GRCh37] 20p12.2(10414643_10792802)x1).
CONCLUSIONS
A targeted sequencing panel followed by confirmation with a chromosome microarray allowed the identification and delineation of a pathogenic microdeletion not previously reported in the literature, including the complete JAG1 gene in a Chilean patient whose phenotype is consistent with ALGS.
Topics: Humans; Alagille Syndrome; Jagged-1 Protein; Female; Gene Deletion; Infant; Phenotype
PubMed: 38801368
DOI: 10.32641/andespediatr.v95i2.4820 -
Structural Heart : the Journal of the... May 2024The interplay between the right ventricle and the pulmonary artery, known as right ventricular to pulmonary artery (RV-PA) coupling, is crucial for assessing right...
Invasive Assessment of Right Ventricular to Pulmonary Artery Coupling Improves 1-year Mortality Prediction After Transcatheter Aortic Valve Replacement and Anticipates the Persistence of Extra-Aortic Valve Cardiac Damage.
BACKGROUND
The interplay between the right ventricle and the pulmonary artery, known as right ventricular to pulmonary artery (RV-PA) coupling, is crucial for assessing right ventricular systolic function against the afterload from the pulmonary circulation. Pulmonary artery pressure levels are ideally measured by right heart catheterization. Yet, echocardiography represents the most utilized method for evaluating pulmonary artery pressure levels, albeit with limitations in accuracy. This study therefore aims to evaluate the prognostic significance of right ventricular to pulmonary artery (RV-PA) coupling expressed as tricuspid annular plane systolic excursion (TAPSE) related to systolic pulmonary artery pressure (sPAP) levels measured by right heart catheterization (TAPSE/sPAP) or estimated by transthoracic echocardiography (TAPSE/sPAP) in patients with severe aortic stenosis undergoing transcatheter aortic valve replacement (TAVR).
METHODS
Using data from a bicentric registry, this study compares TAPSE/sPAP vs. TAPSE/sPAP in predicting 1-year all-cause mortality after TAVR.
RESULTS
Among 333 patients with complete echocardiography and right heart catheterization data obtained before TAVR, their mean age was 79.8 ± 6.74 years, 39.6% were female, and general 1-year survival was 89.8%. sPAP and sPAP showed only moderate correlation (Pearson correlation coefficient : 0.53, value: <0.0001). TAPSE/sPAP was superior to TAPSE/sPAP in predicting 1-year all-cause mortality after TAVR (area under the curve: 0.662 vs. 0.569, value: 0.025). Patients with reduced TAPSE/sPAP levels (< 0.365 mm/mmHg) evidenced significantly lower 1-year survival rates than patients with preserved TAPSE/sPAP levels (81.8 vs. 93.6%, value: 0.001; hazard ratio for 1-year mortality: 3.09 [95% confidence interval: 1.55-6.17]). Echocardiographic follow-up data revealed that patients with reduced RV-PA coupling suffer from persistent right ventricular dysfunction (TAPSE: 16.6 ± 4.05 mm vs. 21.6 ± 4.81 mm in patients with preserved RV-PA coupling) and severe tricuspid regurgitation (diagnosed in 19.7 vs. 6.58% in patients with preserved RV-PA coupling).
CONCLUSIONS
RV-PA coupling expressed as TAPSE/sPAP can refine stratification of severe aortic stenosis patients into low-risk and high-risk cohorts for mortality after TAVR. Moreover, it can help to anticipate persistent extra-aortic valve cardiac damage, which will demand further treatment.
PubMed: 38799808
DOI: 10.1016/j.shj.2024.100282 -
Prenatal Diagnosis Jun 2024In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot... (Observational Study)
Observational Study
OBJECTIVE
In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot (ToF) and its variations. This study aimed to determine whether there is an association of RAA and ADA in fetuses with ToF. Distribution of genetic anomalies and impact on postnatal outcome were further evaluated.
METHOD
Single-center retrospective observational study including pregnancies with prenatal diagnosis of ToF from 2010 to 2023. All cases were subdivided into ToF with pulmonary stenosis (PS) and pulmonary atresia (PA). Clinical and echocardiographic databases were reviewed for pregnancy outcome, genetic anomalies, and postnatal course.
RESULTS
The cohort included 169 cases, 124 (73.4%) with ToF/PS and 45(26.6%) with ToF/PA. Agenesis of the ductus arteriosus was significantly associated with RAA in both subtypes of ToF (p = 0.001) compared to left aortic arch and found in 82.5% (33/40) versus 10.7% (9/84) of fetuses with ToF/PS and in 57.1% (8/14) versus 12.9% (4/31) of fetuses with ToF/PA. In both ToF/PS and ToF/PA, RAA/ADA versus RAA/patent DA revealed a significantly higher risk for the presence of genetic abnormalities, especially microdeletion 22q11.2, major aorto-pulmonary collateral arteries and a shorter time to complete surgical repair.
CONCLUSION
We demonstrated a significantly increased frequency of RAA/ADA in patients with prenatally diagnosed ToF. Although this association revealed no significant impact on overall survival, the prenatal detection of RAA/ADA has implications for counseling, genetic evaluation and postnatal management.
Topics: Humans; Tetralogy of Fallot; Female; Retrospective Studies; Pregnancy; Aorta, Thoracic; Ultrasonography, Prenatal; Adult; Ductus Arteriosus; Pulmonary Atresia; Infant, Newborn; Abnormalities, Multiple
PubMed: 38797960
DOI: 10.1002/pd.6611 -
International Journal of Surgery Case... Jul 2024Partially anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, often concomitant with atrial septal defects (ASDs). PAPVC usually tends to...
INTRODUCTION AND IMPORTANCE
Partially anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, often concomitant with atrial septal defects (ASDs). PAPVC usually tends to be treated by surgery, but the case we report will open up new perspectives for the interventional treatment of PAPVC present with ASD.
CASE PRESENTATION
We present a case of a 2-year-old 11 kg boy transthoracic echocardiography showed secundum-type ASD. A supracardiac-PAPVC was accidentally detected during cardiac catheterization, and an abnormal pulmonary vein connection was detected with a vertical vein (VV) opening. Ultimately, ASD and VV were both occluded.
CLINICAL DISCUSSION
Surgical therapy of PAPVC is the first line treatment of most centers in the world. However, the main complications after surgical repair of PAPVC raise our concerns, such as pulmonary stenosis, caval vein stenosis and sinus node dysfunction. Therefore, percutaneous closure of PAPVC can be an alternative method. This case of percutaneous interventional closure of ASD and supracardiac PAPVC through a vertical vein in the same surgery was first reported. Patients with ASD tend to have missed diagnoses of PAPVC. We can evaluate it by transesophageal echocardiography (TEE), cardiac magnetic resonance imaging (CMR) and computed tomography (CT).
CONCLUSIONS
This case suggests that the effect of interventional therapy is quite reliable. For children with ASD, attention should be paid to the omission of the presence or absence of PAPVC before surgery. During interventional therapy, a guide wire rather than a catheter should be preferred to explore the atrial septum and pulmonary veins to avoid a missed diagnosis of PAPVC.
PubMed: 38795410
DOI: 10.1016/j.ijscr.2024.109783