Paradoxical septic embolism in an Ebstein's anomaly patient leading to brain abscess: A case report.

Ebstein's anomaly (EA), a congenital cardiac anomaly, is characterized by apical displacement of the tricuspid valve leaflet(s) into the right ventricle. We present the case of a 61-year-old female with a history of EA, Wolff-Parkinson-White syndrome, and patent foramen ovale (PFO), who presented with worsening hypoxia and confusion, in the setting of left lower extremity cellulitis and abscess. The computed tomography (CT) scan of the head showed a cerebellar infarct with hemorrhagic conversion. Magnetic resonance imaging of the head showed a satellite lesion raising concern for the embolic nature of infarcts. After ruling out cardioembolic causes of cerebellar infarction, her presenting symptoms were attributed to paradoxical septic emboli from the left leg abscess (demonstrated on CT scan of the leg). She was deemed a poor candidate for surgical closure of PFO due to contraindication to use heparin (due to the presence of hemorrhagic stroke) and underlying comorbidities. Septic embolization is a rare but dreaded complication in EA patients with PFO. Learning objective: •Paradoxical emboli can occur in patients with Ebstein's anomaly (EA) and patent foramen ovale (PFO).•The mainstay of management in case of paradoxical embolism lies with the identification and treatment of the underlying cause, such as infective endocarditis, deep vein thrombosis, or infectious source, as in the present case.•The surgical correction of PFO in EA patients should be considered when the patient becomes symptomatic with cyanosis, hypoxia, or manifestations of paradoxical emboli.

Comprehensive review of statin-intolerance and the practical application of Bempedoic Acid.

Statin-intolerance (SI) has prevalence between 8.0 % and 10 %, and muscular complaints are the most common reason for discontinuation. Bempedoic acid (BA), an ATP citrate lyase inhibitor, decreases hepatic generation of cholesterol, upregulates low-density lipoprotein (LDL) receptor expression in the liver, and eventually clears circulating LDL-cholesterol from the blood. Multiple randomized clinical trials studying BA demonstrate a reduction in LDL levels by 17-28 % in SI. The CLEAR OUTCOME trial established significant cardiovascular benefits with BA. A dose of 180 mg/day of BA showed promising results. BA alone or in combination with ezetimibe is US Food and Drug Administration-approved for use in adults with heterozygous familial hypercholesterolemia and/or established atherosclerotic cardiovascular disease. BA reduced HbA1c by 0.12 % (p < 0.0001) in patients with diabetes. Adverse events of BA include myalgia (4.7 %), anemia (3.4 %), and increased aminotransferases (0.3 %). BA can cause up to four times higher risk of gout in those with a previous gout diagnosis or high serum uric acid levels. Reports of increased blood urea nitrogen and serum creatinine were noted. Current evidence does not demonstrate a reduction in deaths from cardiovascular causes. More studies that include a diverse population and patients with both high and low LDL levels should be conducted. We recommend that providers consider BA as an adjunct to statin therapy in patients with a maximally tolerated dosage to specifically target LDL levels.

Heart transplantation from COVID-positive donors with 6-month follow-up: A case series.

BACKGROUND: Data on long term outcomes in heart transplant recipients from Coronavirus disease 2019 (COVID-19) positive donors are limited. METHODS AND RESULTS: We present a series of nine patients who underwent heart transplants from COVID-19 PCR-positive donors between November 2021 to August 2022 with mean follow-up of 12.12 ± 3 months. All the recipients received two doses of COVID-19 vaccine and had at least 6 months follow-up. Eight recipients had acceptable long-term outcomes; one patient died during index admission from primary graft dysfunction. Details regarding donor and recipient characteristics, management and outcomes are provided. Two patients developed deep vein thrombosis, and one patient underwent pacemaker implantation for sinus node dysfunction. Among the surviving eight patients, none developed COVID-19 infection during follow-up period. There was no significant difference in outcome parameters when compared to patients who received hearts from donors who tested negative for COVID-19 during the same time period at our center. CONCLUSION: Keeping in mind the significant waitlist mortality in patients awaiting heart transplantation, COVID-19-positive donors should be considered for heart transplantation to help expand the donor pool and potentially reduce waitlist mortality.

Novel Nebulized Milrinone Formulation for the Treatment of Acute Heart Failure Requiring Inotropic Therapy: A Phase 1 Study.

BACKGROUND: Nonintravenous inotropic-delivery options are needed for patients with inotropic-dependent heart failure (HF) to reduce the costs, infections and thrombotic risks associated with chronic central venous catheters and home infusion services. METHODS: We developed a novel, concentrated formulation of nebulized milrinone for inhalation and evaluated the feasibility, safety and pharmacokinetic profile in a prospective, single-arm, phase I clinical trial. We enrolled 10 patients with stage D HF requiring inotropic therapy during a hospital admission for acute HF. Milrinone 60 mg/4 mL was inhaled via nebulization 3 times daily for 48 hours. The coprimary outcomes were adverse events and pharmacokinetic profiles of inhaled milrinone. Acute changes in hemodynamic parameters were secondary outcomes. RESULTS: A concentrated nebulized milrinone formulation was well tolerated, without hypotensive events, arrhythmias or inhalation-related adverse events requiring discontinuation. Nebulized milrinone produced serum concentrations in the goal therapeutic range with a median plasma milrinone trough concentration of 39 (17-66) ng/mL and a median peak concentration of 207 (134-293) ng/mL. There were no serious adverse events. From baseline to 24 hours, mean pulmonary artery saturation increased (60% ± 7%-65 ± 5%; P = 0.001), and mean cardiac index increased (2.0 ± 0.5 mL/min/1.73m2-2.5 ± 0.1 mL/min/1.73m2; P = 0.001) with nebulized milrinone. CONCLUSIONS: In a proof-of-concept study, a concentrated, nebulized milrinone formulation for inhalation was safe and produced therapeutic serum milrinone concentrations. Nebulized milrinone was associated with improved hemodynamic parameters of cardiac output in a population with advanced HF. These promising results require further investigation in a longer-term trial in patients with inotrope-dependent advanced HF.

Transcatheter aortic valve replacement for aortic insufficiency in a patient with aortic root Thrombus and left ventricular assist device: A risk worth taking?

A 61-year-old man with end-stage ischemic cardiomyopathy post HeartMate 3 (Abbott laboratories, Chicago, Illinois, USA) left ventricular assist device (LVAD) implant was hospitalized after he had recurrent ventricular tachycardia requiring implantable cardioverter-defibrillator shocks. His transthoracic echocardiogram and computed tomography angiography of the chest showed presence of trace aortic insufficiency (AI) and aortic root thrombus (ART) of non-coronary cusp without obstruction of right or left coronary artery ostium despite therapeutic international normalized ratio. He presented again 3 months later with worsening heart failure signs and symptoms. Transesophageal echocardiogram showed progression to severe AI and persistent ART. Despite hemodynamically guided LVAD speed optimization, inotropic support, and diuresis, the patient continued to deteriorate with worsening renal function. The patient was not a transplant candidate due to frailty. After multi-disciplinary discussion he underwent successful 29-Sapien S3 (Edwards Lifesciences, Irvine, CA, USA) transcatheter aortic valve replacement utilizing distal protection filters in bilateral internal carotid arteries for stroke prevention. This case provides novel insight to physicians treating LVAD patients regarding management of severe AI in the setting of ART. Learning objective: We report a rare approach employed for management of aortic insufficiency (AI) in a patient who also had an aortic root thrombus and left ventricular assist device (LVAD) that traditionally requires cardiac transplantation. Our patient had a favorable outcome with a minimally invasive transcatheter aortic valve replacement. With this case, we hope to generate awareness amongst physicians treating patients about management alternatives and approach of a commonly encountered, life-threatening complication of AI in patients with LVAD.

Cytomegalovirus infection in heart transplant patient presenting as appendicitis.

Cytomegalovirus (CMV) may manifest in various ways. While immunocompetent hosts may be asymptomatic or present with a mononucleosis-like illness, immunocompromised patients can have organ-specific disease capable of significant morbidity and mortality. CMV appendicitis is a particularly rare presentation. A 22-year-old female with a history of orthotopic heart transplantation presented to our hospital with a three-day history of worsening abdominal pain. A computed tomography scan of her abdomen was consistent with acute uncomplicated appendicitis, and she underwent laparoscopic appendectomy. Pathology revealed acute appendicitis with numerous large cells with intranuclear "owl's eye" inclusions characteristic of CMV. Her CMV viral load was elevated at 327,018 IU/ml. She was started on ganciclovir which resulted in improvement of her CMV level to 30,118 IU/ml within three weeks. CMV is a frequent cause of opportunistic infection in solid organ transplant patients and commonly involves the gastrointestinal tract. Acute appendicitis is a rarely reported complication to consider in the differential diagnosis of abdominal pain in immunocompromised patients. Learning objective: Heart transplant recipients are at increased risk for opportunistic infections. Cytomegalovirus (CMV) is a frequent culprit and can present with a broad range of disease. A particularly rare presentation is that of acute appendicitis. We describe a case of a young woman with CMV appendicitis following orthotopic heart transplant.

Trends and Outcomes of Cardiogenic Shock in Patients With End-Stage Renal Disease: Insights From USRDS Database.

BACKGROUND: There is a paucity of data regarding epidemiology, temporal trends, and outcomes of patients with cardiogenic shock (CS) and end-stage renal disease (chronic kidney disease stage V on hemodialysis). METHODS: This is a retrospective cohort study using the United States Renal Data System database from January 1, 2006 to December 31, 2019. We analyzed trends of CS, percutaneous mechanical support (intraaortic balloon pump, percutaneous ventricular assist device [Impella and Tandemheart], and extracorporeal membrane oxygenation) utilization, index mortality, 30-day mortality, and 1-year all-cause mortality in end-stage renal disease patients. RESULTS: A total of 43 825 end-stage renal disease patients were hospitalized with CS (median age, 67.8 years [IQR, 59.4-75.8] and 59.1% men). From 2006 to 2019, the incidence of CS increased from 275 to 578 per 100 000 patients (Ptrend<0.001). The index mortality rate declined from 54.1% in 2006 to 40.8% in 2019 (Ptrend=0.44), and the 1-year all-cause mortality decreased from 63% in 2006 to 61.8% in 2018 (Ptrend=0.73), but neither trend was statistically significant. There was a significantly decreased utilization of intra-aortic balloon pumps from 17 832 to 7992 (Ptrend<0.001), increased utilization of percutaneous ventricular assist device from 137 to 5201 (Ptrend<0.001) and increase in extracorporeal membrane oxygenation use from 69 to 904 per 100 000 patients (Ptrend<0.001). After adjusting for covariates, there was no significant difference in index mortality between CS patients requiring percutaneous mechanical support versus those not requiring percutaneous mechanical support (odds ratio, 0.97 [CI, 0.91-1.02]; P=0.22). On multivariable regression analysis, older age, peripheral vascular disease, diabetes, and time on dialysis were independent predictors of higher index mortality. CONCLUSIONS: The incidence of CS in end-stage renal disease patients has doubled without significant change in the trend of index mortality despite the use of percutaneous mechanical support.

Endomyocardial biopsy-proven hydroxychloroquine-induced cardiomyopathy in a patient with rheumatoid arthritis.

Hydroxychloroquine is a disease-modifying antirheumatic drug used for various rheumatological conditions. Its long-term use is well-known to have toxic effects on cardiac muscle cells. We present a biopsy-proven case of hydroxychloroquine-induced cardiotoxicity with detailed histopathological and imaging findings. The patient was referred to our heart failure clinic for concerns of reduction in left ventricular ejection fraction despite being on guideline-directed medical therapy. She had been diagnosed with rheumatoid arthritis, pulmonary hypertension and then subsequently heart failure with reduced ejection fraction 5 years ago. The evaluation included right heart catheterisation, cardiac MRI and endomyocardial biopsy. Light and electron microscopy showed myocyte hypertrophy and vacuolar change, abnormal mitochondria, myeloid bodies and curvilinear bodies. These findings were specific for hydroxychloroquine-induced cardiomyopathy. This case highlights the importance of clinical monitoring, early suspicion and consideration of drug-induced toxicities as a culprit for heart failure.

Impact of Biopsy Proven Liver Fibrosis on Patients Undergoing Evaluation and Treatment for Advanced Heart Failure Surgical Therapies.

There is a paucity of data regarding the impact of liver fibrosis on patients with stage D heart failure (HF). We conducted a retrospective study (January 1, 2017 to December 12, 2020) in patients with stage D HF who underwent liver biopsy as part of their advanced HF therapy evaluation. Baseline characteristics and 1-year outcomes were compared between no- or mild-to-moderate-fibrosis (grade 0 to 2) and advanced-fibrosis (grade 3 to 4) groups. Of 519 patients with stage D HF, 136 who underwent liver biopsy (113 [83%] no or mild-to-moderate fibrosis and 23 [17%] advanced fibrosis) were included. A total of 71 patients (52%) received advanced HF therapies (23 heart transplantation, 48 left ventricular assist devices). One-year mortality was higher among patients with advanced fibrosis (52% vs 18%, p <0.001). Further subgroup analysis suggested a trend toward increased 1-year mortality among patients with advanced fibrosis who underwent advanced therapies (37% vs 13%, p = 0.09). There was a trend of lower likelihood of receiving advanced HF therapies in the advanced-fibrosis group, only 1 heart transplantation and 7 left ventricular assist devices, but it did not reach statistical significance (35% vs 56%, p = 0.06). After adjustment for confounders, degree of liver fibrosis was an independent predictor of mortality (odds ratio 6.2; 95% 1.27 to 30.29, p = 0.02). We conclude that advanced liver fibrosis is common among patients with stage D HF who undergo evaluation for advanced HF surgical therapies and significantly increases 1-year mortality. Further larger studies are needed to support our findings.

Loperamide-induced cardiogenic syncope: a case report of a life-threatening presentation of an over-the-counter drug.

Background: Loperamide at supratherapeutic doses can cause cardiac toxicity, presenting as cardiogenic shock, prolonged QT, malignant arrhythmias, or in severe cases sudden cardiac death. Surreptitious loperamide use is difficult to diagnose. We present an interesting case of loperamide use presenting with polymorphic ventricular tachycardia, cardiogenic shock. Case summary: A 25-year-old female presented with multiple syncopal episodes for 12 months with an electrocardiogram showing a Brugada-like pattern for which she underwent implantable cardioverter-defibrillator placement. One day following the procedure, she developed cardiogenic shock and was transferred to our tertiary care centre. Extensive workup was unrevealing. She responded well to supportive management, recovering from shock and was transferred to the floor. Unfortunately, she again developed cardiogenic shock, ultimately leading to cardiac arrest. Given the unclear cause for her cardiovascular symptoms, futher medication history was obtained. It was revealed that she was taking 100-150 tablets of loperamide per day. The decision was made to treat with intralipid emulsion therapy empirically given the strong suspicion for loperamide toxicity. The patient recovered well with supportive care. Loperamide levels returned elevated at 190 ng/mL. Repeated studies showed improvement of the conduction block, resolution of arrhythmias, and recovery of right and left ventricular function. Discussion: Acute loperamide toxicity can present as biventricular failure, with difficult-to-control arrhythmias. It requires a high index of suspicion. Treatment for loperamide toxicity is mainly supportive, lipid emulsion therapy can be considered in severe or refractory cases.

Rare case of chronic Q fever myocarditis in end stage heart failure patient: A case report.

BACKGROUND: Q fever myocarditis is a rare disease manifestation of Q fever infection caused by Coxiella burnetii. It is associated with significant morbidity and mortality if left untreated. Prior studies have reported myocarditis in patients with acute Q fever. We present the first case of chronic myocarditis in an end-stage heart failure patient with chronic Q fever infection. CASE SUMMARY: A 69-year-old male was admitted with dyspnea on exertion, hypotension and bilateral lower extremity edema for a few months. He has a past medical history of ischemic cardiomyopathy with left ventricular ejection fraction of 25%, implantable cardioverter defibrillator in place, bioprosthetic aortic valve and mitral valve replacement. He continued to have shortness of breath despite diuresis along with low grade fevers. Initial infectious work up came back negative. On further questioning, the patient was found to have close contact with farm animals and the recurrent fevers prompted the work-up for Q fever. Q fever serologies and cardiac positron emission tomography confirmed the diagnosis of chronic Q fever myocarditis. He was then successfully treated with doxycycline and hydroxychloroquine for 18 mo. CONCLUSION: Chronic Q fever myocarditis, if left untreated, carries a poor prognosis. It should be kept in differentials, especially in patients with recurrent fevers and contact with farm animals.

Palliative Inotropes in Advanced Heart Failure: Comparing Outcomes Between Milrinone and Dobutamine.

BACKGROUND: We sought to describe and compare outcomes among advanced patients with heart failure (not candidates for orthotopic heart transplant/left ventricular assist device) on long-term milrinone or dobutamine, which are not well-studied in the contemporary era. METHODS AND RESULTS: We included adults with refractory stage D heart failure who were not candidates for orthotopic heart transplant or left ventricular assist device and discharged on palliative dobutamine or milrinone. The primary outcome was 1-year survival. A 6-month predictor of survival analysis was conducted. A total of 248 patients (133 on milrinone, 115 on dobutamine) were included. There were no differences in baseline comorbidities between milrinone and dobutamine cohorts, except for the prevalence of chronic kidney disease, which was higher in the dobutamine group. On discharge, the proportion of patients on beta-blockers and mineralocorticoid antagonists was higher in milrinone group. Overall, the 1-year mortality rate was 70%. The dobutamine cohort had a significantly higher 1-year mortality rate (84% vs 58%, P <0.001). The type of inotrope did not predict survival at 6 months when adjusted for discharge medications and comorbidities. Beta-blockers and angiotensin-converting enzyme/angiotensin receptor blocker/angiotensin receptor neprilysin inhibitor continued at discharge predicted survival at 6 months. CONCLUSIONS: The 1-year mortality from palliative inotropes remains high. Compared with dobutamine, use of milrinone was associated with improved survival owing to better optimization of guideline-directed medical therapy, primarily beta-blocker therapy.

Emergency department use and hospital admissions among adult orthotopic heart transplant patients.

Objective: To study the demographics, clinical presentations, and outcomes of emergency department (ED) visits of patients with heart transplantation (HT) in the United States. Methods: We performed a secondary analysis of the National Emergency Department Sample database from 2016 to 2018. All ED visits of patients with HT aged ≥ 18 years were identified using International Classification of Diseases, Tenth Revision codes. Results: Out of a total 308,182,495 national ED visits, 55,583 were HT-related visits. The median age was 61.07 years (interquartile range [IQR]: 46.91-69.38) and 69.44% were males. The hospital admission rate was 54.3% and median inpatient length of stay was 3.19 days (IQR: 1.63-5.92). The mortality rate during inpatient stay was 1.16%. Median inpatient and ED charges among admitted patients were $37,911 (IQR: $21,487-$71,262). The most common primary diagnosis of HT-related ED visits was sepsis (4.3%) followed by acute kidney injury (3.57%) and chest pain (3%). Conclusion: More than half of total ED visits among HT patients resulted in hospital admission. The most common cause for ED visit in these patients was sepsis followed by acute kidney injury and chest pain.

Outcomes in Patients With Chronic Kidney Disease and End-stage Renal Disease and Durable Left Ventricular Assist Device: Insights From the United States Renal Data System Database.

BACKGROUND: There is paucity of data regarding durable left ventricular assist device (LVAD) outcomes in patients with chronic kidney disease (CKD) stages 3-5 and CKD stage 5 on dialysis (end-stage renal disease [ESRD]). METHODS AND RESULTS: We conducted a retrospective study of Medicare beneficiaries with ESRD and a 5% sample of patients with CKD with an LVAD (2006-2018) to determine 1-year outcomes using the United States Renal Data System database. The LVAD implantation, comorbidities, and outcomes were identified using appropriate International Classification of Diseases, 9th and 10th edition codes. We identified 496 patients with CKD and 95 patients with ESRD who underwent LVAD implantation. The patients with ESRD were younger (59 years vs 66 years; P < .001), had more Blacks (40% vs 24.6%, P = .009), compared with the CKD group. The 1-year mortality (49.5% vs 30.9%, P < .001) and index mortality (27.4% vs 16.7%, P = .014) rates were higher for patients with ESRD. A subgroup analysis showed significantly higher mortality in ESRD vs CKD 3 (49.5% vs 30.2%, adjusted P = .009), but no significant difference in mortality between stage 3 vs 4/5 (30.2% vs 30.8%, adjusted P = .941). There was no significant difference in secondary outcomes (bleeding, stroke, and sepsis/infection) during follow-up between the 2 groups. CONCLUSIONS: Patients with ESRD undergoing LVAD implantation had significantly higher index and 1-year mortality rates compared with patients with CKD.

Effects of Vitamin D Supplementation and 25-Hydroxyvitamin D Levels on the Risk of Atrial Fibrillation.

The effects of vitamin D (Vit-D) deficiency and Vit-D treatment (VDT) on atrial fibrillation (AF) remain inconclusive. This study sought to determine the effects of VDT and nontreatment on AF risk in Vit-D-deficient patients without a previous history of AF. In this nested case-control study, 39,845 individuals with low 25-hydroxy-Vit-D ([25-OH]D) levels (<20 ng/ml) were divided into group-A (untreated, levels ≤20 ng/ml), group-B (treated, levels 21 to 29 ng/ml), and group-C (treated, levels ≥30 ng/ml). The risk of AF was compared utilizing propensity score-weighted Cox proportional hazard models. Among the individuals receiving VDT for ≥6 months, the risk of AF was significantly lower in group-B (hazard ratio [HR] 0.89, 95% confidence interval [CI] 0.80 to 0.98, p = 0.03] and group-C (HR 0.84, 95% CI 0.73 to 0.0.95, p = 0.007] than in group-A. A subgroup analysis of men >65 years showed individuals with hypertension had a significantly lower risk of AF in group-C than in group-B (HR 0.79, CI 0.65 to 0.94, p = 0.02) and group-A (HR 0.78, CI 0.64 to 0.96, p = 0.012). A similar result was found in men >65 years with diabetes mellitus in group-C compared with group-B (HR 0.69, CI 0.51 to 0.93, p = 0.012) and group-A (HR 0.63, CI 0.47 to 0.84, p = 0.002). In what is, to best of our knowledge, the largest observational study to date of patients with Vit-D deficiency and no previous history of AF, (25-OH)D level of >20 ng/ml with VDT for ≥6 months was associated with a significantly lower risk of AF. Additionally, men >65 years with hypertension or diabetes mellitus had a further decrease in AF risk when the (25-OH)D levels were ≥30 ng/ml.

Inpatient characteristics, complications, and outcomes of patients with cardiac sarcoidosis: A study from the National Inpatient Sample.

Although seen in ∼5% of sarcoidosis patients, cardiac sarcoidosis (CS) accounts for nearly 25% of disease-related deaths. This study aimed to describe characteristics and outcomes among CS patients. Patients diagnosed with CS in 2016-2017 in the US National Inpatient Sample Database were evaluated to study patient characteristics, reasons ascribed to admission, in-hospital outcomes, and complications. A total of 2420 patients (median age 56 years) were included in the analysis. Most admissions occurred due to ventricular tachycardia (12.8%), followed by myocarditis (9.9%) with a mean length of stay of 7 ± 7 days. The overall incidence of in-hospital mortality was 2.5%.

Gender-specific outcomes after percutaneous left atrial appendage closure: A nationwide readmission database analysis.

INTRODUCTION: Thromboembolism-associated stroke is the most feared complication of atrial fibrillation (AF). Percutaneous left atrial appendage closure (pLAAC) is indicated for stroke prevention in patients with AF who can not tolerate long-term anticoagulation. We aim to study gender differences in peri-procedural and readmissions outcomes in pLAAC patients. METHODS: Using the national readmission database from January 2016 to December 2018, AF patients undergoing the pLAAC procedure were identified. We used multivariate logistic regression analyses and time-to-event Cox regression analyses to conduct the study. Propensity matching with the Greedy method was done for the accuracy of results. RESULT: A total of 28 819 patients were included in our study. Among them 11 946 (41.5%) were women and 16 873 (58.6%) were men. The mean age of overall population was 76.1 ± 8.5 years, with women ~1 year older than men. The overall rate of complications was higher in women (8.6% vs. 6.6%, p < .001), primarily driven by bleeding-related complications, that is, major bleed (odds ratio [OR]: 1.32 95% confidence interval [CI]: 1.03-1.69, p = .029), blood transfusion (OR: 1.45, 95% CI: 1.06-1.97, p = .019), and cardiac tamponade (OR: 1.80, 95% CI: 1.13-2.89, p = .014). Women had two times higher peri-procedural ischemic stroke. There was no difference in peri-procedural mortality. Women remained at 20% and 13% higher risk for readmission at 30 days and 6 months of discharge. CONCLUSION: Women had higher peri-procedural complications and were at higher risk of readmissions at 30 days and 6 months. However, there was no difference in mortality during the index hospitalization. Further studies are necessary to determine causality.

Early and aggressive presentation of wild-type transthyretin amyloid cardiomyopathy: A case report.

BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt) is the most common form of transthyretin amyloid cardiomyopathy, occurring mostly over age of 60 years (mean age of 80 years). Mean survival without treatment is 3.6 years, making early detection imperative. We report an unusual case of a 58-year-old patient with ATTRwt cardiomyopathy requiring heart transplantation. CASE SUMMARY: A 58-year-old male presented with progressive fatigue, shortness of breath, weight gain, leg swelling, orthopnoea, and paroxysmal nocturnal dyspnoea for several months. Approximately ten months before this clinical presentation, the patient had first received a diagnosis of heart failure with reduced ejection fraction (EF) of 15% to 20%. The patient was started on appropriate guideline-directed medical therapy with only mild improvement in his EF. Upon further investigation, echocardiogram, technetium pyrophosphate scan (Tc PYP), and cardiac magnetic resonance imaging (cMRI) suggested a diagnosis of amyloidosis, and ATTRwt was subsequently confirmed with native heart tissue biopsy, congo red staining, liquid chromatography-tandem mass spectrometry, and genetic testing. The patient was successfully treated with heart transplantation and is doing well post-transplant. CONCLUSION: Wild-type ATTR amyloidosis should be kept on differentials in all patients (even less than 60 years old) with non-ischemic cardiomyopathy, especially in the setting of increased ventricular wall thickness and other classic echocardiogram, cMRI, and Tc PYP findings. Early diagnosis and management can be consequential in improving patient outcomes.