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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 -
Journal of Perinatal Medicine Jun 2024To assess congenital fetal bradyarrhythmias with regard to etiological causes, features, risk factors, and prognosis.
OBJECTIVES
To assess congenital fetal bradyarrhythmias with regard to etiological causes, features, risk factors, and prognosis.
METHODS
This retrospective study involved fetuses with fetal bradyarrhythmias. All fetuses were evaluated by ultrasonography. Parental ECGs and family histories were obtained, and maternal autoantibodies were measured. Gestational age at diagnosis, fetal atrial and ventricular rates at presentation, type of bradyarrhythmias, the presence or absence of a congenital heart defect (CHD), fetal hydrops, fetal myocardial dysfunction, extra-cardiac abnormalities, maternal autoimmune diseases, maternal autoantibodies as well as prenatal treatment, and neonatal outcome were collected.
RESULTS
Of the 40 fetuses included in the study, 11 had maternal rheumatologic disease, 16 had complex cardiac anomalies such as left and right isomerism. Fetuses with CHD significantly differed from those without CHD with increased rates of extra-cardiac anomalies, hydrops, fetal deaths and shorter survival after 28 days (p<0.05). Survival was significantly better in fetuses with maternal rheumatic disease as compared with those with no maternal rheumatic disease (p<0.05). Maternal anti-arrhythmic therapy was administered in 11 fetuses. maternal treatment resulted in no significant difference in the course of arrhythmia or hydrops in fetuses with or without maternal rheumatic disease (p<0.05). In regression analysis, the absence of fetal hydrops was the only independent factor associated with survival (p=0.04).
CONCLUSIONS
The course of bradyarrhythmias, along with survival, seems to be more favorable in fetuses with maternal rheumatic disease than in those with CHD, especially left and right isomerism. Hydrops was the sole independent factor associated with poor survival.
PubMed: 38917319
DOI: 10.1515/jpm-2024-0131 -
Clinical Pharmacokinetics Jun 2024Vericiguat is an oral soluble guanylate cyclase stimulator and enhances the cyclic guanosine monophosphate pathway independently of nitric oxide as well as... (Review)
Review
Vericiguat is an oral soluble guanylate cyclase stimulator and enhances the cyclic guanosine monophosphate pathway independently of nitric oxide as well as synergistically in normal- and low-nitric oxide conditions. This review describes the pharmacokinetic and pharmacodynamic profile of vericiguat and summarizes the effect of vericiguat on cardiac electrophysiology and population pharmacokinetic/pharmacodynamic relationships. Vericiguat demonstrates virtually complete absorption and increased exposure with food. Vericiguat has high oral bioavailability when taken with food (93.0%) with dose-proportional pharmacokinetics in healthy volunteers. Vericiguat has slightly less than dose-proportional pharmacokinetics with a slight decrease in bioavailability at higher doses in patients with heart failure (HF) with reduced ejection fraction (HFrEF). Vericiguat is a low-clearance drug, with a half-life of approximately 20 h in healthy volunteers and 30 h in patients with HFrEF. Most drug metabolism is achieved by glucuronidation. Vericiguat has pharmacodynamic effects as expected from its pharmacological mechanism of action (i.e., relaxation of the smooth muscles in the vasculature leading to changes in hemodynamics). In the VICTORIA trial (NCT02861534), which enrolled patients with HFrEF, no meaningful exposure-response relationships for the incidence of symptomatic hypotension or syncope were evident. There were no significant imbalances in the incidence of undesirable hemodynamic-related effects (symptomatic hypotension and syncope) in subgroups with HFrEF defined by sex, age, race, and renal impairment. In addition, most patients achieved the 10-mg target dose per the blood pressure-guided titration regimen. No dose adjustments due to body weight, age, sex, race, or hepatic/renal impairment are necessary in adult patients with HFrEF. Observed and predicted changes in vericiguat exposure when co-administered with perpetrator drugs were small and not clinically meaningful. In addition, vericiguat has low potential as a perpetrator to affect exposure and/or pharmacodynamic effects of drugs commonly prescribed in patients with heart failure; therefore, no dose adjustment of these drugs is required in patients taking vericiguat. There is limited experience on the combined use of vericiguat with long-acting nitrates in patients with HFrEF. The ongoing VICTOR trial (NCT05093933), which is investigating vericiguat in patients with HFrEF, permits the co-administration of long-acting nitrates. Combined use of vericiguat and phosphodiesterase type-5 inhibitors has not been studied in patients with HFrEF and is therefore not recommended because of the potential increased risk for symptomatic hypotension. Vericiguat was not associated with electrophysiological abnormalities in preclinical and clinical studies up to the approved dose of 10 mg at steady state. Vericiguat is approved for the treatment of recently decompensated patients with worsening HFrEF. Vericiguat's safety and efficacy profile in patients with HFrEF will be further characterized by the VICTOR trial (NCT05093933) in adults without recent decompensation and in a pediatric population with HF due to left ventricular systolic dysfunction (VALOR trial, NCT05714085).
PubMed: 38916717
DOI: 10.1007/s40262-024-01384-1 -
Giornale Italiano Di Cardiologia (2006) Jul 2024Every year, approximately 5 out of 1000 patients receive a diagnosis of advanced heart failure, with a prevalence of 1-2% in the adult population. This figure is likely... (Review)
Review
Every year, approximately 5 out of 1000 patients receive a diagnosis of advanced heart failure, with a prevalence of 1-2% in the adult population. This figure is likely underestimated, considering undiagnosed cases. Despite significant progress in medical therapy for heart failure, mortality rates persist around 20% within the first year, reaching 50-60% at 5 years from the initial diagnosis. For patients with severe end-stage heart failure, the 1-year mortality rate can reach up to 70%. Heart transplantation remains the preferred treatment for terminal stages of the disease; however, the significant challenge lies in the mismatch between available donors and recipients. Given this dilemma, both short-term solutions including extracorporeal membrane oxygenation and long-term options such as left ventricular assist devices have gained prominence. These mechanical circulatory support systems become crucial for patients in critical conditions, temporarily ineligible for heart transplantation, such as those with severe irreversible pulmonary hypertension or acute organ failure. Despite these advancements, a growing number of patients on the waiting list develops severe biventricular dysfunction, precluding the use of a left ventricular assist device as a bridge to transplant. In such cases, a total artificial heart emerges as a viable therapeutic option.
Topics: Humans; Heart, Artificial; Heart Failure; Heart Transplantation; Heart-Assist Devices; Forecasting; Extracorporeal Membrane Oxygenation
PubMed: 38916464
DOI: 10.1714/4282.42636 -
Giornale Italiano Di Cardiologia (2006) Jul 2024Ischemic left ventricular dysfunction results from the combination of scar and areas of dysfunctional but viable myocardium that may improve contractile function with... (Review)
Review
Ischemic left ventricular dysfunction results from the combination of scar and areas of dysfunctional but viable myocardium that may improve contractile function with revascularization. The traditional paradigm states that an improvement in function after revascularization leads to improved health outcomes and that assessment of myocardial viability has a key role in predicting the benefit of revascularization and, therefore, is a prerequisite for the selection of patients to undergo this treatment option. A range of retrospective observational studies supported this "viability hypothesis". However, randomized prospective trials have not confirmed the interaction between myocardial viability and the treatment effect of revascularization, challenging the recovery of left ventricular function as the principal mechanism by which surgical revascularization improves prognosis. A conceptual shift from the traditional paradigm centered on the assessment of viability as a dichotomous variable to a more comprehensive approach founded on an alternative concept that the main goal of revascularization is to prevent further damage by protecting the residual viable myocardium from subsequent acute coronary events and ventricular arrhythmias is required.
Topics: Humans; Myocardial Revascularization; Prognosis; Myocardial Ischemia; Ventricular Dysfunction, Left; Randomized Controlled Trials as Topic; Myocardium; Tissue Survival
PubMed: 38916462
DOI: 10.1714/4282.42634 -
BioRxiv : the Preprint Server For... Jun 2024ATP-citrate lyase (ACLY) converts citrate into acetyl-CoA and oxaloacetate in the cytosol. It plays a prominent role in lipogenesis and fat accumulation coupled to...
BACKGROUND
ATP-citrate lyase (ACLY) converts citrate into acetyl-CoA and oxaloacetate in the cytosol. It plays a prominent role in lipogenesis and fat accumulation coupled to excess glucose, and its inhibition is approved for treating hyperlipidemia. In RNAseq analysis of human failing myocardium, we found ACLY gene expression is reduced; however the impact this might have on cardiac function and/or metabolism has not been previously studied. As new ACLY inhibitors are in development for cancer and other disorders, such understanding has added importance.
METHODS
Cardiomyocytes, ex-vivo beating hearts, and in vivo hearts with ACLY inhibited by selective pharmacologic (BMS303141, ACLYi) or genetic suppression, were studied. Regulation of ACLY gene/protein expression, and effects of ACLYi on function, cytotoxicity, tricarboxylic acid (TCA)-cycle metabolism, and redox and NAD+/NADH balance were assessed. Mice with cardiac ACLY knockdown induced by AAV9-acly-shRNA or cardiomyocyte tamoxifen-inducible Acly knockdown were studied.
RESULTS
Acly gene expression was reduced more in obese patients with heart failure and preserved EF (HFpEF) than HF with reduced EF. In vivo pressure-overload and in vitro hormonal stress increased ACLY protein expression, whereas it declined upon fatty-acid exposure. Acute ACLYi (1-hr) dose-dependently induced cytotoxicity in adult and neonatal cardiomyocytes, and caused substantial reduction of systolic and diastolic function in myocytes and ex-vivo beating hearts. In the latter, ATP/ADP ratio also fell and lactate increased. U13C-glucose tracing revealed an ACLYdependent TCA-bypass circuit in myocytes, where citrate generated in mitochondria is transported to the cytosol, metabolized by ACLY and then converted to malate to re-enter mitochondria,bypassing several NADH-generating steps. ACLYi lowered NAD+/NADH ratio and restoring this balance ameliorated cardiomyocyte toxicity. Oxidative stress was undetected with ACLYi. Adult hearts following 8-weeks of reduced cardiac and/or cardiomyocyte ACLY downregulation exhibited ventricular dilation and reduced function that was prevented by NAD augmentation. Cardiac dysfunction from ACLY knockdown was worse in hearts subjected to sustained pressureoverload, supporting a role in stress responses.
CONCLUSIONS
ACLY supports normal cardiac function through maintenance of the NAD+/NADH balance and is upregulated by hemodynamic and hormonal stress, but depressed by lipid excess. ACLY levels are most reduced in human HFpEF with obesity potentially worsening cardio-metabolic reserve.
PubMed: 38915649
DOI: 10.1101/2024.06.09.598152 -
Cardiovascular Diabetology Jun 2024Diabetic peripheral neuropathy (DPN) is the most prevalent complication of diabetes, and has been demonstrated to be independently associated with cardiovascular events...
BACKGROUND
Diabetic peripheral neuropathy (DPN) is the most prevalent complication of diabetes, and has been demonstrated to be independently associated with cardiovascular events and mortality. This aim of this study was to investigate the subclinical left ventricular (LV) myocardial dysfunction in type 2 diabetes mellitus (T2DM) patients with and without DPN.
METHODS
One hundred and thirty T2DM patients without DPN, 61 patients with DPN and 65 age and sex-matched controls who underwent cardiovascular magnetic resonance (CMR) imaging were included, all subjects had no symptoms of heart failure and LV ejection fraction ≥ 50%. LV myocardial non-infarct late gadolinium enhancement (LGE) was determined. LV global strains, including radial, circumferential and longitudinal peak strain (PS) and peak systolic and diastolic strain rates (PSSR and PDSR, respectively), were evaluated using CMR feature tracking and compared among the three groups. Multivariable linear regression analyses were performed to determine the independent factors of reduced LV global myocardial strains in T2DM patients.
RESULTS
The prevalence of non-infarct LGE was higher in patients with DPN than those without DPN (37.7% vs. 19.2%, p = 0.008). The LV radial and longitudinal PS (radial: 36.60 ± 7.24% vs. 33.57 ± 7.30% vs. 30.72 ± 8.68%; longitudinal: - 15.03 ± 2.52% vs. - 13.39 ± 2.48% vs. - 11.89 ± 3.02%), as well as longitudinal PDSR [0.89 (0.76, 1.05) 1/s vs. 0.80 (0.71, 0.93) 1/s vs. 0.77 (0.63, 0.87) 1/s] were decreased significantly from controls through T2DM patients without DPN to patients with DPN (all p < 0.001). LV radial and circumferential PDSR, as well as circumferential PS were reduced in both patient groups (all p < 0.05), but were not different between the two groups (all p > 0.05). Radial and longitudinal PSSR were decreased in patients with DPN (p = 0.006 and 0.003, respectively) but preserved in those without DPN (all p > 0.05). Multivariable linear regression analyses adjusting for confounders demonstrated that DPN was independently associated with LV radial and longitudinal PS (β = - 3.025 and 1.187, p = 0.014 and 0.003, respectively) and PDSR (β = 0.283 and - 0.086, p = 0.016 and 0.001, respectively), as well as radial PSSR (β = - 0.266, p = 0.007).
CONCLUSIONS
There was more severe subclinical LV dysfunction in T2DM patients complicated with DPN than those without DPN, suggesting further prospective study with more active intervention in this cohort of patients.
Topics: Humans; Male; Female; Middle Aged; Ventricular Dysfunction, Left; Ventricular Function, Left; Diabetes Mellitus, Type 2; Diabetic Neuropathies; Aged; Predictive Value of Tests; Asymptomatic Diseases; Magnetic Resonance Imaging, Cine; Case-Control Studies; Diabetic Cardiomyopathies; Risk Factors; Prevalence; Cross-Sectional Studies; Stroke Volume; Myocardial Contraction
PubMed: 38915040
DOI: 10.1186/s12933-024-02307-x