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Echocardiography (Mount Kisco, N.Y.) Jun 2024The noninvasive right ventricular pressure-strain loop (PSL) represents a novel method for the quantitative assessment of right ventricular myocardial function. Given...
Quantitative evaluation of right ventricular myocardial function changes in patients with atrial septal defect before and after occlusion by noninvasive right ventricular pressure-strain loop.
OBJECTIVE
The noninvasive right ventricular pressure-strain loop (PSL) represents a novel method for the quantitative assessment of right ventricular myocardial function. Given that atrial septal defect (ASD) is a prevalent congenital heart anomaly associated with right ventricular volume overload, this study aimed to quantitatively assess the myocardial function of the right ventricle in ASD patients pre- and post-occlusion by noninvasive right ventricular PSL.
METHODS
This study included 36 patients diagnosed with secundum ASD group and 30 healthy adults (control group). We compared conventional right ventricular echocardiographic parameters, right ventricular strain, and myocardial work in the ASD group before occlusion, two days post-occlusion, and three months post-occlusion, with those in the control group.
RESULTS
Prior to and two days following occlusion, the ASD group exhibited higher right ventricular global work index (RVGWI), right ventricular global wasted work (RVGWW), and right ventricular global constructive work (RVGCW) compared to the control group (P < .05). Within the ASD group, post-occlusion, RVGWI, RVGCW, and RVGWW values were significantly reduced compared to pre-occlusion values (P < .001). Furthermore, RVGWI and RVGCW showed a significant decrease three months after occlusion compared to two days post-occlusion (P < .05). Multivariate regression analysis identified ASD diameter and pulmonary artery systolic pressure (PASP) as independent predictors of RVGWI (β = .405, P < .001; β = 2.307, P = .037) and RVGCW(β = .350, P<.001; β = 1.967, P = .023).
CONCLUSIONS
The noninvasive right ventricular PSL effectively demonstrates the alterations in right ventricular myocardial function in ASD patients, pre- and post-occlusion. The metrics of right ventricular myocardial work (RVMW) offer a novel indicator for evaluating right ventricular myocardial function in these patients. Moreover, ASD diameter and PASP emerge as independent determinants of RVGWI and RVGCW.
Topics: Humans; Female; Male; Heart Septal Defects, Atrial; Adult; Heart Ventricles; Echocardiography; Ventricular Function, Right; Ventricular Dysfunction, Right; Ventricular Pressure; Reproducibility of Results
PubMed: 38924593
DOI: 10.1111/echo.15868 -
Molecular Genetics & Genomic Medicine Jun 2024Dilated cardiomyopathy (DCM) is characterized by dilatation of the left ventricle, systolic dysfunction, and normal or reduced thickness of the left ventricular wall. It...
BACKGROUND
Dilated cardiomyopathy (DCM) is characterized by dilatation of the left ventricle, systolic dysfunction, and normal or reduced thickness of the left ventricular wall. It is a leading cause of heart failure and cardiac death at a young age. Cases with neonatal onset DCM were correlated with severe clinical presentation and poor prognosis. A monogenic molecular etiology accounts for nearly half of cases.
FAMILY DESCRIPTION
Here, we report a family with three deceased offspring at the age of 1 year old. The autopsy of the first deceased infant revealed a DCM. The second infant presented a DCM phenotype with a severely reduced Left Ventricular Ejection Fraction (LVEF) of 10%. Similarly, the third infant showed a severe DCM phenotype with LVEF of 30% as well, in addition to eccentric mitral insufficiency.
RESULTS
Exome sequencing was performed for the trio (the second deceased infant and her parents). Data analysis following the autosomal dominant and recessive patterns of inheritance was carried out along with a mitochondrial pathways-based analysis. We identified a homozygous frameshift variant in the TNNI3 gene (c.204delG; p.(Arg69AlafsTer8)). This variant has been recently reported in the ClinVar database in association with cardiac phenotypes as pathogenic or likely pathogenic and classified as pathogenic according to ACMG.
CONCLUSION
Genetic counseling was provided for the family and a prenatal diagnosis of choronic villus was proposed in the absence of pre-implantation genetic diagnosis possibilities. Our study expands the case series of early-onset DCM patients with a protein-truncating variant in the TNNI3 gene by reporting three affected infant siblings.
Topics: Humans; Cardiomyopathy, Dilated; Frameshift Mutation; Female; Homozygote; Pedigree; Consanguinity; Male; Infant; Phenotype; Troponin I
PubMed: 38924380
DOI: 10.1002/mgg3.2486 -
Journal of Cardiovascular... Jun 2024Pacemaker-mediated tachycardia is a known arrhythmia in patients with dual chamber pacemakers and defibrillators and intact ventriculoatrial (VA) conduction. We report a...
INTRODUCTION
Pacemaker-mediated tachycardia is a known arrhythmia in patients with dual chamber pacemakers and defibrillators and intact ventriculoatrial (VA) conduction. We report a case of pacemaker-mediated reentrant arrhythmia (PMRA) in a patient with an atrioventricular (AV) synchronous leadless pacemaker.
METHODS AND RESULTS
A 91-year-old female presented with 2:1 AV conduction and received an AV synchronous leadless pacemaker. She had atrial mechanical sense-ventricular paced beats between heart rates of 80-100 bpm more than 80% of the time. She was found to have a new cardiomyopathy and was referred for placement of biventricular pacemaker. At the time of device implantation, her electrocardiogram showed ventricular pacing with a short RP interval and superiorly directed P waves. Changes in the ventricular pacing rate resulted in changes in the atrial rate. Following device placement, her heart rate decreased to the lower rate limit of her pacemaker. The atrial mechanical sense impulse most likely was generated by a retrograde conducted P wave resulting in near incessant PMRA.
CONCLUSION
PMRA may occur in patients who receive an AV synchronous leadless pacemaker with intact VA conduction and sinoatrial node dysfunction. Due to the lower rates of PMRA, this arrhythmia may be underrecognized. Interventions for and implications of PMRA need further investigation.
PubMed: 38923693
DOI: 10.1111/jce.16350 -
Arquivos Brasileiros de Cardiologia 2024The introduction of anthracyclines in the treatment of children and adolescents with cancer has promoted a significant increase in survival, but also in morbidity and...
BACKGROUND
The introduction of anthracyclines in the treatment of children and adolescents with cancer has promoted a significant increase in survival, but also in morbidity and mortality rates due to cardiovascular (CV) complications.
OBJECTIVES
To determine the cardiovascular profile of pediatric patients treated with anthracyclines at a cancer center in Brazil and the incidence of CV complications.
METHODS
The following data were collected from the medical records of patients of both sexes, aged younger than 19 years - frequency and form of clinical presentation of general CV complications (G1) and CV complications related to ventricular dysfunction (G2) - and correlated with risk factors, age range and vital status, cardiovascular and cardioprotective medications. A p<0.05 was considered statistically significant.
RESULTS
A total of 326 patients were included, 214 (65.6%) were younger than 10 years and 192 (58.9%) of male sex. G1 complications occurred in 141 (43.3%) patients, and the most frequent was systemic arterial hypertension; G2 complications occurred in 84 patients (25.8%). Cumulative dose (CD) of anthracyclines > 250mg/m2 was used in 26.7% of patients and the association of G2 complications with this CD was not statistically significant (p=0.305; OR=1.330 and [95% CI = 0.770- 2.296]). The most used cardiac medications were diuretics (34.7% of patients).
CONCLUSIONS
In accordance with literature, the study showed a high incidence of CV complications in the treatment of children and adolescents with cancer, with general CV complications as the most prevalent.
Topics: Humans; Male; Female; Child; Anthracyclines; Brazil; Adolescent; Child, Preschool; Incidence; Cardiovascular Diseases; Risk Factors; Infant; Neoplasms; Retrospective Studies; Antibiotics, Antineoplastic; Cardiotoxicity; Sex Distribution; Young Adult
PubMed: 38922271
DOI: 10.36660/abc.20210352 -
Arquivos Brasileiros de Cardiologia 2024Cardiomyopathy associated with partial lipodystrophy (PL) has not been well described yet.
BACKGROUND
Cardiomyopathy associated with partial lipodystrophy (PL) has not been well described yet.
OBJECTIVE
To characterize cardiac morphology and function in PL.
METHODS
Patients with familial PL and controls were prospectively assessed by transthoracic echocardiography and with speckle-tracking echocardiography (global longitudinal strain, GLS). The relationship between echocardiographic variables and PL diagnosis was tested with regression models, considering the effect of systolic blood pressure (SBP). Significance level of 5% was adopted.
RESULTS
Twenty-nine patients with PL were compared to 17 controls. They did not differ in age (p=0.94), gender or body mass index (p= 0.05). Patients with PL had statistically higher SBP (p=0.02) than controls. Also, PL patients had higher left atrial dimension (37.3 ± 4.4 vs. 32.1 ± 4.3 mm, p= 0.001) and left atrial (30.2 ± 7.2 vs. 24.9 ± 9.0 mL/m2,p=0.02), left ventricular (LV) mass (79.3 ± 17.4 vs. 67.1 ± 19.4, p=0.02), and reduced diastolic LV parameters (E' lateral, p= 0.001) (E' septal, p= 0.001), (E/E' ratio, p= 0.02). LV ejection fraction (64.7 ± 4.6 vs. 62.2 ± 4.4 %, p= 0.08) and GLS were not statistically different between groups (-17.1 ± 2.7 vs. -18.0 ± 2.0 %, p= 0.25). There was a positive relationship of left atrium (β 5.6, p<0.001), posterior wall thickness, (β 1.3, p=0.011), E' lateral (β -3.5, p=0.002) and E' septal (β -3.2, p<0.001) with PL diagnosis, even after adjusted for SBP.
CONCLUSION
LP patients have LV hypertrophy, left atrial enlargement, and LV diastolic dysfunction although preserved LVEF and GLS. Echocardiographic parameters are related to PL diagnosis independent of SBP.
Topics: Humans; Female; Male; Adult; Echocardiography; Case-Control Studies; Lipodystrophy, Familial Partial; Middle Aged; Prospective Studies; Blood Pressure; Heart Atria; Cardiomyopathies; Reference Values; Stroke Volume
PubMed: 38922260
DOI: 10.36660/abc.20230442 -
Annals of the Academy of Medicine,... Oct 2023Although severe acute respiratory failure is the primary cause of morbidity and mortality in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, this... (Review)
Review
INTRODUCTION
Although severe acute respiratory failure is the primary cause of morbidity and mortality in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, this viral infection leads to cardiovascular disease in some individuals. Cardiac effects of the virus include myocarditis, pericarditis, arrhythmias, coronary aneurysms and cardiomyopathy, and can result in cardiogenic shock and multisystem organ failure.
METHOD
This review summarises cardiac manifesta-tions of SARS-CoV-2 in the paediatric population. We performed a scoping review of cardiovascular disease associated with acute coronavirus disease 2019 (COVID-19) infection, multisystem inflammatory syndrome in children (MIS-C), and mRNA COVID-19 vaccines. Also examined are special considerations for paediatric athletes and return to play following COVID-19 infection.
RESULTS
Children presenting with acute COVID-19 should be screened for cardiac dysfunction and a thorough history should be obtained. Further cardiovascular evaluation should be considered following any signs/symptoms of arrhythmias, low cardiac output, and/or myopericarditis. Patients admitted with severe acute COVID-19 should be monitored with continuous cardiac monitoring. Laboratory testing, as clinically indicated, includes tests for troponin and B-type natriuretic peptide or N-terminal pro-brain natriuretic peptide. Echocardiography with strain evaluation and/or cardiac magnetic resonance imaging should be considered to evaluate diastolic and systolic dysfunction, coronary anatomy, the pericardium and the myocardium. For patients with MIS-C, combination therapy with intravenous immunoglobulin and glucocorticoid therapy is safe and potentially disease altering. Treatment of MIS-C targets the hyperimmune response. Supportive care, including mechanical support, is needed in some cases.
CONCLUSION
Cardiovascular disease is a striking feature of SARS-CoV-2 infection. Most infants, children and adolescents with COVID-19 cardiac disease fully recover with no lasting cardiac dysfunction. However, long-term studies and further research are needed to assess cardiovascular risk with variants of SARS-CoV-2 and to understand the pathophysiology of cardiac dysfunction with COVID-19.
Topics: Humans; COVID-19; Child; Systemic Inflammatory Response Syndrome; Cardiovascular Diseases; COVID-19 Vaccines; SARS-CoV-2
PubMed: 38920204
DOI: 10.47102/annals-acadmedsg.202386 -
Cureus Jun 2024Takotsubo cardiomyopathy (TC) mimics myocardial infarction with symptoms like chest pain, electrocardiogram (EKG) changes, and elevated troponin levels, although it...
Case Report and Literature Review of an Anomalous Course of the Left Main Coronary Artery (LMCA) Arising From the Right Sinus of Valsalva (RSV) Presenting as Takotsubo Cardiomyopathy.
Takotsubo cardiomyopathy (TC) mimics myocardial infarction with symptoms like chest pain, electrocardiogram (EKG) changes, and elevated troponin levels, although it typically features normal coronary arteries upon angiography. While often asymptomatic, coronary artery anomalies (CAAs) can cause intermittent vasospasm and endothelial dysfunction, potentially inducing TC. We report the case of a 74-year-old female with a history of hypertension, hyperlipidemia, and peripheral artery disease, who presented with sudden onset chest pain. Initial EKG and elevated troponin suggested myocardial infarction. However, coronary angiography revealed an anomalous left main coronary artery (LMCA) originating from the right coronary artery (RCA), with no significant stenosis. Subsequent transthoracic echocardiography indicated TC, with the left ventricular ejection fraction improving from 35-40% to 60-65% within days. Cardiac computed tomography angiography (CCTA) revealed that the anomalous LMCA originated from the common trunk at the right sinus of Valsalva (RSV), which further continued as a large, dominant RCA. The LMCA branched into a small to moderate left anterior descending artery (LAD) and a non-dominant left circumflex artery (LCx). The LMCA followed a prepulmonic/anterior course, while the LCx took an interarterial course between the aorta and pulmonary artery. The patient was referred for further surgical evaluation. We conclude that the CAA was an incidental finding and was not related to underlying TC. Although rare, this case suggests a possible correlation between CAAs and a predisposition to stress-induced cardiomyopathy, warranting further investigation.
PubMed: 38919862
DOI: 10.7759/cureus.63028 -
PCN Reports : Psychiatry and Clinical... Jun 2024Takotsubo cardiomyopathy (TCM) is a left ventricular dysfunction resembling acute coronary syndrome. Its prognosis is generally favorable; however, a subset of patients...
A case of reinitiation of modified electroconvulsive therapy 2 weeks after modified electroconvulsive therapy-induced Takotsubo cardiomyopathy in a male patient with major depressive disorder.
BACKGROUND
Takotsubo cardiomyopathy (TCM) is a left ventricular dysfunction resembling acute coronary syndrome. Its prognosis is generally favorable; however, a subset of patients may present with severe complications. TCM is a rare side-effect of modified electroconvulsive therapy (ECT); it has been reported in 22 female and two male patients. Eight cases of ECT reinitiation after TCM have been reported (all females), with the shortest duration being 3 weeks.
CASE PRESENTATION
We report the case of a 61-year-old man with a history of major depressive disorder and no history of heart disease or previous ECT treatment. Antidepressants had been ineffective, and ECT was indicated. After the third ECT session, the patient complained of chest pain and shortness of breath. Electrocardiography revealed ST elevation, and catheter angiography was used to diagnose TCM. The patient had mild heart failure but remained stable. Recognizing that ECT was effective, the patient asked for it to be reinitiated. We confirmed that the cardiac function had been normalized, applied a bisoprolol fumarate patch as a preventive measure, and reinitiated ECT 14 days after the onset of TCM. ECT was performed five times, with no recurrence of TCM and a marked improvement in depression.
CONCLUSION
We describe a male patient with major depressive disorder who underwent reinitiation of ECT 2 weeks after ECT-induced TCM. Therefore, TCM should be recognized as a side-effect of ECT, even in men. Moreover, depending on whether the patient's condition is stable, ECT can be successfully performed in patients with TCM.
PubMed: 38919546
DOI: 10.1002/pcn5.221 -
BMC Cardiovascular Disorders Jun 2024Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal...
BACKGROUND
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources.
OBJECTIVE
This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated.
METHODOLOGY
This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation.
RESULTS
Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team.
CONCLUSION
The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.
Topics: Humans; Tetralogy of Fallot; Male; Retrospective Studies; Female; Uganda; Child, Preschool; Child; Adolescent; Infant; Cardiac Surgical Procedures; Treatment Outcome; Time Factors; Risk Factors; Postoperative Complications; Risk Assessment
PubMed: 38918721
DOI: 10.1186/s12872-024-03991-z -
BMC Nephrology Jun 2024Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited cystic disease characterized by bilateral renal cyst formation and congenital liver fibrosis....
BACKGROUND
Autosomal recessive polycystic kidney disease (ARPKD) is a rare inherited cystic disease characterized by bilateral renal cyst formation and congenital liver fibrosis. Cardiovascular disorders such as noncompaction of ventricular myocardium (NVM) have not been reported with ARPKD.
CASE PRESENTATION
A 5-month-old girl was examined after presenting with a fever and turbid urine for one day and was diagnosed as urinary tract infection. Urinary ultrasound showed multiple round, small cysts varying in size in both kidneys. Genetic testing revealed two heterozygous mutations and one exon deletion in the polycystic kidney and hepatic disease 1 gene, indicating a diagnosis of ARPKD. During hospitalization, she was found to have chronic heart failure after respiratory tract infection, with an ejection fraction of 29% and fraction shortening of 13%. When the patient was 15 months old, it was found that she had prominent trabeculations and deep intertrabecular recesses with the appearance of blood flow from the ventricular cavity into the intertrabecular recesses by echocardiography. The noncompaction myocardium was 0.716 cm and compaction myocardium was 0.221 cm (N/C = 3.27), indicating a diagnosis of NVM. Liver and kidney function remained normal during four-year follow-up.
CONCLUSIONS
This is the first report of NVM in a patient with ARPKD. It is unsure if the coexistence of NVM and ARPKD is a coincidence or they are different manifestations of ciliary dysfunction in the heart and kidneys.
Topics: Humans; Female; Polycystic Kidney, Autosomal Recessive; Infant; Isolated Noncompaction of the Ventricular Myocardium; Ciliopathies
PubMed: 38918687
DOI: 10.1186/s12882-024-03642-7