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Circulation Journal : Official Journal... Apr 2024Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR),...
BACKGROUND
Complications arising from transcatheter closure of perimembranous ventricular septal defects (pmVSD) in children, such as residual shunts and aortic regurgitation (AR), have been observed. However, the associated risk factors remain unclear. This study identified risk factors linked with residual shunts and AR following transcatheter closure of pmVSD in children aged 2-12 years.Methods and Results: The medical records of 63 children with pmVSD and a pulmonary-to-systemic blood flow ratio <2.0 who underwent transcatheter closure between 2011 and 2018 were analyzed with a minimum 3-year follow-up. The success rate of transcatheter closure was 98.4%, with no emergency surgery, permanent high-degree atrioventricular block, or mortality. Defects ≥4.5 mm had significantly higher odds of persistent residual shunt (odds ratio [OR] 6.85; P=0.03). The use of an oversize device (≥1.5 mm) showed a trend towards reducing residual shunts (OR 0.23; P=0.06). Age <4 years (OR 27.38; 95% confidence interval [CI] 2.33-321.68) and perimembranous outlet-type VSD (OR 11.94, 95% CI 1.10-129.81) were independent risk factors for AR progression after closure.
CONCLUSIONS
Careful attention is crucial for pmVSDs ≥4.5 mm to prevent persistent residual shunts in transcatheter closure. Assessing AR risk, particularly in children aged <4 years, is essential while considering the benefits of pmVSD closure.
Topics: Humans; Heart Septal Defects, Ventricular; Child, Preschool; Child; Risk Factors; Male; Female; Cardiac Catheterization; Retrospective Studies; Septal Occluder Device; Treatment Outcome; Aortic Valve Insufficiency; Age Factors; Time Factors; Follow-Up Studies; Postoperative Complications
PubMed: 38325819
DOI: 10.1253/circj.CJ-23-0891 -
Brazilian Journal of Cardiovascular... Feb 2024Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor...
INTRODUCTION
Primary cardiac myxomas are rare tumors. Concurrent valvular lesion is a common finding on evaluation which is thought to be due to annular dilatation secondary to tumor movement across the valve, functional obstruction across the valve, and severe pulmonary hypertension secondary to chronic obstruction. A common belief among surgeons is that excision of myxoma leads to abatement of symptoms, and further valve intervention may not be warranted.
METHODS
A 10-year retrospective descriptive study was designed to analyze patients who underwent excision of cardiac myxoma at our center. Data was analyzed regarding presenting features, echocardiographic findings of myxoma and valve morphology, intraoperative assessment, and postoperative outcome with/without valve repair/replacement in all patients.
RESULTS
A total of 22 patients underwent surgery for myxoma. Six patients underwent successful mitral valve repair with ring annuloplasty, two had moderate mitral regurgitation, three had severe mitral regurgitation, and one patient had no mitral regurgitation on preoperative assessment, but moderate mitral regurgitation was found intraoperatively. Four of these patients had no residual mitral regurgitation in follow-up period while two had mild residual mitral regurgitation. One patient had severe mitral stenosis of concurrent rheumatic etiology and successfully underwent mitral valve replacement.
CONCLUSION
Cardiac myxomas are rare benign tumors commonly associated with mitral valve insufficiency. Mitral valve should be assessed intraoperatively after excision of mass as preoperative assessment might often be insufficient. Concomitant mitral valve intervention might be needed with a case-specific tailored approach, and mitral valve repair with ring annuloplasty offers best surgical outcome in such cases.
Topics: Humans; Mitral Valve Insufficiency; Retrospective Studies; Mitral Valve; Echocardiography; Heart Neoplasms; Myxoma; Treatment Outcome
PubMed: 38315074
DOI: 10.21470/1678-9741-2023-0040 -
The International Journal of... Mar 2024Evaluating right ventricular (RV) function remains a challenge. Recently, novel echocardiographic assessment of RV myocardial work (RVMW) by non-invasive pressure-strain...
Evaluating right ventricular (RV) function remains a challenge. Recently, novel echocardiographic assessment of RV myocardial work (RVMW) by non-invasive pressure-strain loops was proposed. This enables evaluation of right ventriculoarterial coupling and quantifies RV dyssynchrony and post-systolic shortening. We aimed to assess RVMW in patients with different etiologies of RV dysfunction and healthy controls. We investigated healthy controls (n=17), patients with severe functional tricuspid regurgitation (FTR; n=22), and patients with precapillary pulmonary hypertension (PCPH; n=20). Echocardiography and right heart catheterization were performed to assess 1) RV global constructive work (RVGCW; work needed for systolic myocardial shortening and isovolumic relaxation), 2) RV global wasted work (RVGWW; myocardial shortening following pulmonic valve closure), and 3) RV global work efficiency (RVGWE; describes the relation between RV constructive and wasted work). RVGCW correlated with invasive RV stroke work index (r=0.66, P<0.001) and increased in tandem with higher afterload, i.e., was low in healthy controls (454±73 mmHg%), moderate in patients with FTR (687±203 mmHg%), and highest among patients with PCPH (881±255 mmHg%). RVGWE was lower and RVGWW was higher in patients with FTR (86±8% and 91 mmHg% [53-140]) or PCPH (86±10% and 110 mmHg% [66-159]) as compared with healthy controls (96±3% and 10 mmHg%). RVMW by echocardiography provides a promising index of RV function to discriminate between patients with RV volume or pressure overload. The prognostic value of this measure needs to be settled in future studies.
Topics: Humans; Ventricular Dysfunction, Right; Predictive Value of Tests; Echocardiography; Systole; Tricuspid Valve Insufficiency; Ventricular Function, Right; Stroke Volume
PubMed: 38305942
DOI: 10.1007/s10554-023-03038-y -
Arquivos Brasileiros de Cardiologia Dec 2023Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular...
Central Illustration : Position Statement on the Use of Myocardial Strain in Cardiology Routines by the Brazilian Society of Cardiology's Department Of Cardiovascular Imaging - 2023 Proposal for including strain in the integrated diastolic function assessment algorithm, adapted from Nagueh et al.67 Am: mitral A-wave duration; Ap: reverse pulmonary A-wave duration; DD: diastolic dysfunction; LA: left atrium; LASr: LA strain reserve; LVGLS: left ventricular global longitudinal strain; TI: tricuspid insufficiency. Confirm concentric remodeling with LVGLS. In LVEF, mitral E wave deceleration time < 160 ms and pulmonary S-wave < D-wave are also parameters of increased filling pressure. This algorithm does not apply to patients with atrial fibrillation (AF), mitral annulus calcification, > mild mitral valve disease, left bundle branch block, paced rhythm, prosthetic valves, or severe primary pulmonary hypertension.
Topics: Humans; Echocardiography, Doppler; Brazil; Atrial Fibrillation; Heart Atria; Cardiology; Ventricular Function, Left; Ventricular Dysfunction, Left
PubMed: 38232246
DOI: 10.36660/abc.20230646 -
Annals of Medicine and Surgery (2012) Jan 2024Edward syndrome is a severe chromosomal defect that occurs as a result of non-disjunction through meiosis. It presents with cardiac septal defects, horseshoe kidneys,...
INTRODUCTION AND IMPORTANCE
Edward syndrome is a severe chromosomal defect that occurs as a result of non-disjunction through meiosis. It presents with cardiac septal defects, horseshoe kidneys, patent ductus arteriosus, central nervous system dysgenesis, distinctive craniofacial deformities, and overriding or overlapping fingers. Klinefelter syndrome (47, XXY) is found in 1 in 660 newborn males. It is considered to be one of the most common genetic causes of infertility. It manifests with small firm testes, androgen insufficiency, and azoospermia.
CASE PRESENTATION
A 2-month-old male infant with a history of weakness in feeding, frequent convulsions, and an increase in cyanosis two days ago. There were multiple skeletal deformities and a tendency to spasm in the extremities, left ventricular atrophy, mitral atresia, atrial septal defect, ventricular septal defect with dilated right cavities, tricuspid valve regurgitation, pulmonary valve stenosis; and the aorta exits in the right ventricle. There is a widening of the subdural space, which was observed in the left frontal-parietal side with cortical atrophy in that area and a widening of the Sylvian fissure. A karyotype test confirmed the presence of Edward and Klinefelter syndromes.
CLINICAL DISCUSSION
Aneuploidy is a chromosomal issue characterized by an abnormal number of a chromosome copies. The coexistence of two aneuploidies is called "double aneuploidy" which is a rare occurrence. Herein, we report a case of a 2-month-old male with Edward syndrome and Klinefelter syndrome.
CONCLUSION
This publication aims to highlight the challenges in diagnosing and treating a complicated genetic disease.
PubMed: 38222680
DOI: 10.1097/MS9.0000000000001468 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Jan 2024Moderate to severe mitral regurgitation (MR) and tricuspid regurgitation (TR) are present in approximately 20-60% of patients undergoing transcatheter aortic valve...
OBJECTIVE
Moderate to severe mitral regurgitation (MR) and tricuspid regurgitation (TR) are present in approximately 20-60% of patients undergoing transcatheter aortic valve implantation (TAVI). This study aims to evaluate the impact of TAVI on MR and TR, pulmonary hypertension, and reverse cardiac remodeling in these patients. Methods: Out of 240 patients who underwent TAVI, 79 who met the inclusion and exclusion criteria were analyzed.
RESULTS
In our study, 46.8% (n = 37) of the patients were male. Nineteen (24.1%) patients died within two years. Before TAVI, 34 (43%) patients had moderate-to-severe MR, which decreased to 18 (22.7%) after the procedure (P < 0.05). Similarly, the number of patients with moderate-to-severe TR decreased from 26 (32.9%) before TAVI to 12 (15%) after the procedure (P < 0.05). Of the patients, 50.6% (n = 40) did not require hospitalization after the procedure, while 25 were hospitalized once, 12 twice, and 2 three times. The mean systolic pulmonary artery pressure (sPAP) values of the patients decreased from 44.30 ± 14.42 mmHg before the procedure to 39.09 ± 11.77 mmHg after the procedure (Z=-3.506, P < 0.001). No correlation was found between changes in MR and TR grades after TAVI and mortality or hospitalization during follow-up. Furthermore, there was no statistically significant difference in tricuspid annular plane systolic excursion (TAPSE), free wall annular S' velocity, left atrial volume (LAV), or LAV index (LAVI) before and after TAVI. Conclusion: There was a significant decrease in moderate-to-severe MR and TR after TAVI; however, this did not impact hospitalization or mortality rates. Additionally, no significant differences were observed in right ventricular systolic function or in LAV and LAVI before and after TAVI.
Topics: Humans; Male; Female; Transcatheter Aortic Valve Replacement; Tricuspid Valve Insufficiency; Mitral Valve Insufficiency; Treatment Outcome; Heart Valve Prosthesis Implantation; Aortic Valve Stenosis; Aortic Valve
PubMed: 38221830
DOI: 10.5543/tkda.2023.08130 -
Journal of Cardiovascular Magnetic...Many patients with repaired tetralogy of Fallot require pulmonary valve replacement (PVR) due to significant pulmonary regurgitation (PR). Transcatheter PVR (TPVR) is an...
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.
Topics: Humans; Tetralogy of Fallot; Retrospective Studies; Adolescent; Time Factors; Child; Young Adult; Male; Female; Cardiac Surgical Procedures; Treatment Outcome; Adult; Pulmonary Valve Insufficiency; Longitudinal Studies; Child, Preschool; Ventricular Function, Right; Predictive Value of Tests; Magnetic Resonance Imaging, Cine; Age Factors; Pulmonary Valve; Infant; Heart Ventricles; Magnetic Resonance Imaging; Middle Aged
PubMed: 38211659
DOI: 10.1016/j.jocmr.2023.100002 -
European Heart Journal. Case Reports Jan 2024Cardiac complications occur in 1-6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less...
Association of endomyocardial fibrosis and minor myocarditis sequelae with intracardiac thrombus and Ebstein like valvulopathy in a patient with Behçet disease: a case report.
BACKGROUND
Cardiac complications occur in 1-6% of cases of Behçet disease (BD) with intracardiac thrombus being the most frequent complication. Endomyocardial fibrosis, less common and occasionally associated with intracardiac thrombus, is reported in <20 case reports of BD, among which, three cases are described to mimic Ebstein disease based on echocardiography. We present the first case in the literature of a 34-year-old man with BD diagnosed with multiple cardiovascular complications, highlighting the challenging diagnosis and treatment of this pathology, especially regarding anticoagulation therapy.
CASE SUMMARY
A 34-year-old man, diagnosed with BD, presented to the Emergency Room with haemoptysis. Computed tomography study of the thorax diagnosed pulmonary arterial aneurysm with multiple arterial thrombi, associated with multiple intracardiac thrombi in the right ventricle and atrium. The echocardiography confirmed the presence of voluminous thrombi in the right ventricle and atrium and showed hypertrabeculation of the right ventricle and a high insertion of the posterior leaflet of the tricuspid valve inducing a moderate tricuspid insufficiency compatible with an Ebstein disease. The cardiac MRI later revealed right ventricular fibrosis consistent with endomyocardial fibrosis and sequelae of myocarditis, also described as BD rare cardiac manifestations. The patient had a favourable outcome under anticoagulant treatment and immunosuppressive drugs.
DISCUSSION
The association of multiple cardiovascular complications can occur in a single patient with BD. The endomyocardial fibrosis in the right heart chambers acting as a substrate for thrombus formation and subsequent pulmonary embolism; fibrosis extending to the tricuspid valve inducing an Ebstein-like morphology.
PubMed: 38173783
DOI: 10.1093/ehjcr/ytad631 -
BMC Cardiovascular Disorders Jan 2024Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and...
BACKGROUND
Patients with repaired Tetralogy of Fallot (rTOF) often develop pulmonary regurgitation (PR) and right ventricle (RV) dysfunction, experiencing increased mortality and morbidity rates in adulthood. Pulmonary valve replacement (PVR) timing to address PR is controversial. Cardiac Magnetic Resonance (CMR) is the gold standard for morpho-functional evaluation of complex cardiopathies. This study aims to identify CMR parameters predictive of adverse outcomes to help defining the best therapeutic management of rTOF patients.
METHODS
130 rTOF patients who underwent CMR (2006-2019) were enrolled in this retrospective single-center study. CMR, clinical, ECG and exercise data were analyzed. Univariate and multivariate analyses identified clinical and CMR parameters predictive of adverse outcomes both individually (e.g., death, arrhythmias, heart failure (HF), pharmacological therapy, QRS ≥ 160ms) and as composite outcome.
RESULTS
Univariate analysis confirmed RV volumes and RV ejection fraction corrected for PR as adverse outcome predictors and identified interesting correlations: pulmonary artery bifurcation geometry and abnormal interventricular septum (IVS) motion with arrhythmias (p < .001; p = .037), HF (p = .049; p = .005), composite outcome (p = .039; p = .009); right atrium (RA) dimensions with the composite outcome and the outcomes individually (p < .001). The best predictive models by multivariate analysis included sex (male), RV and RA dilation for QRS ≥ 160ms, time form repair to CMR, age at TOF repair and IVS fibrosis for pharmacological therapy.
CONCLUSIONS
Besides RV volumes, new adverse prognostic factors could guide rTOF therapeutic management: pulmonary arteries morphology, abnormal IVS motion, RV dysfunction, RA dilation. Perspective multicentric evaluation is needed to specify their effective role.
Topics: Humans; Male; Tetralogy of Fallot; Retrospective Studies; Magnetic Resonance Imaging; Pulmonary Valve Insufficiency; Heart Failure; Magnetic Resonance Spectroscopy; Ventricular Function, Right; Ventricular Dysfunction, Right
PubMed: 38172687
DOI: 10.1186/s12872-023-03671-4 -
Frontiers in Cardiovascular Medicine 2023Despite numerous advantages of the Ross procedure, it presents a risk of late autograft and right ventricular outflow tract conduit failure. This study aimed to analyze...
BACKGROUND
Despite numerous advantages of the Ross procedure, it presents a risk of late autograft and right ventricular outflow tract conduit failure. This study aimed to analyze the outcomes of autograft dysfunction reoperations using autograft-sparing and root replacement techniques.
METHODS
Between 2015 and 2023, 49 patients underwent redo root surgery in our institution. Autograft valve-sparing procedures (VSP) were performed in 20 cases and the Bentall procedure (BP) in 29 patients. The short and long-term clinical outcomes along with echocardiographic results of VSP and BP were investigated.
RESULTS
Overall early mortality rate was 2.0% with no significant difference between the groups. Severe autograft valve insufficiency at the time of redo (OR 4.07, = 0.03) and patient age (OR 1.07, = 0.04) were associated with a valve replacement procedure instead of VSP. The median follow-up duration was 34 months. No late deaths occurred in either group. Freedom from VSP failure and aortic prosthesis dysfunction were 93.8% and 94.1% in the VSP and BP groups, respectively. No reoperations were necessary in either group.
CONCLUSION
Redo aortic root surgery can be safely performed in patients with autograft failure. Both root replacement and autograft valve-sparing procedures demonstrated acceptable results at mid-term follow-up. Early redo surgery pre-empting severe aortic insufficiency increases the likelihood of preservation of the dilated autograft valve.
PubMed: 38155981
DOI: 10.3389/fcvm.2023.1306445