Sarcoid Cardiomyopathy Research Articles

Perinatal outcomes in patients with biopsy confirmed sarcoidosis

Sarcoidosis is a multisystem inflammatory disease that is often diagnosed in women of reproductive age. The implications of this disease on pregnancy remains controversial, with most data derived from case reports and population-based cohort studies. This study aims to better understand the effects of sarcoidosis on perinatal outcomes. A retrospective chart review of patients delivering between 2010 to 2021 was completed. Patients with biopsy confirmed diagnosis or meeting clinical criteria for neurosarcoidosis and cardiac sarcoidosis with singleton pregnancies were included. Maternal demographic characteristics were collected, along with maternal and fetal complications during pregnancy. Obstetrical and adverse perinatal outcomes were also reviewed to determine the relationship between maternal sarcoidosis and pregnancy. Of the 20 patients included in the study, 90% had biopsy confirmed sarcoidosis. Majority of the patients were multiparous, African-American women, and diagnosed with sarcoidosis between the ages of 21-30 years. Sixty percent of patients had single organ systems affected by sarcoidosis with 55% having pulmonary disease. Approximately half of the patients required medical management prior to pregnancy, but none required therapy during pregnancy. Table 1 demonstrates patients’ disease states. Thirty-five percent of patients were diagnosed with hypertensive disorders during pregnancy, while 10% of patients were diagnosed with early-onset fetal growth restriction. Delivery outcomes demonstrated 70% of patients were delivered by cesarean section at term. Table 2 demonstrates maternal and neonatal morbidity. Postpartum hemorrhage and cardiovascular compromise, including peripartum sarcoid cardiomyopathy were notable maternal complications, while congenital heart disease and low birth weight were found to be pertinent neonatal morbidity in this population. Patients with biopsy confirmed sarcoidosis may be at risk for early-onset fetal growth restriction and maternal and neonatal morbidity.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Simultaneous Hyperammonemia Syndrome in Multiple Organ Recipients from a Single Donor

<h3>Introduction</h3> Hyperammonemia is a rare complication post solid organ transplant, with a high mortality. We report a case of hyperammonemic encephalopathy in separate cardiac and lung transplant recipients, likely related to donor derived infection acquired from the same donor. <h3>Case Report</h3> A 63-year-old male with sarcoid cardiomyopathy on LVAD support and no known hepatic disease received cardiac transplantation. He was extubated on day (D) 4 with normal allograft function and mentation. He received basiliximab induction, steroids, mycophenolate (MMF) and tacrolimus (TAC) on D6. He developed hallucinations on D8 followed by paranoia and reduced GCS on D9, with no focal neurological deficit. Cranial CT/MRI and CSF were unremarkable. Initial blood results showed raised CRP, WCC with mildly elevated urea, creatinine, bilirubin & transaminase. Serum ammonia (NH₃) was 549µmol/L. He was intubated and diagnosed with hyperammonemic encephalopathy. Hyperammonemic encephalopathy had also been diagnosed in the lung transplant recipient, a 65-year-old male with COPD, from the same donor. He received basiliximab induction, steroids, MMF and Cyclosporine A (CSA) on D1. He developed right sided hypertonia and reduced consciousness on D6, with elevated NH₃ (177µmol/L). Both received <i>Mycoplasma</i> / <i>Ureaplasma</i> combination antibiotics - moxifloxacin and azithromycin (lung), moxifloxacin and minocycline (cardiac); metronidazole, rifaximin, lactulose, ammonia scavengers and dialysis. NH₃ normalised by D12 (lung) and D15 (cardiac) with improvement in mental state. At D30, both remain dependent on dialysis with ongoing antibiotics, steroids, and moderate immunosuppression. Both patients required tracheostomy for critical illness myopathy. The aetiology of hyperammonemia is putative, however both recipients had <i>Mycoplasma hominis</i> identified on blood cultures. <i>M. hominis</i> and <i>Ureaplasma spp.</i> were present in bronchial washings in the lung recipient, and sputum, pleural and pericardial fluid from cardiac recipient. The donor had culture negative urethritis at donation with <i>Mycoplasma</i> and <i>Ureaplasma spp</i>. confirmed on retrospective bronchial washing PCR. <h3>Summary</h3> Hyperammonemia should be suspected in transplant recipients with altered mental state. Transplant units need increased awareness of the potential impact of infection by urease-producing organisms.

Outcomes after heart transplantation in patients with cardiac sarcoidosis

BackgroundThe number of patients with sarcoidosis requiring heart transplantation (HT) is increasing. The aim of this study was to evaluate outcomes of isolated HT in patients with sarcoid cardiomyopathy and compare them to recipients with non‐ischaemic restrictive or dilated cardiomyopathy.Methods and resultsAdult HT recipients were identified in the UNOS Registry between 1990 and 2020. Patients were grouped according to diagnosis. The cumulative incidences for the all‐cause mortality and rejection were compared using Fine and Gray model analysis, accounting for re‐transplantation as a competing risk. Rejection was evaluated using logistic regression analysis. We also reviewed characteristics and outcomes of all HT recipients with previous diagnosis of sarcoid cardiomyopathy from a single centre. A total of 30 160 HT recipients were included in the present study (n = 239 sarcoidosis, n = 1411 non‐ischaemic restrictive cardiomyopathy, and n = 28 510 non‐ischaemic dilated cardiomyopathy). During a total of 194 733 patient‐years, all‐cause mortality at the latest follow‐up was not significantly different when comparing sarcoidosis to non‐ischaemic dilated cardiomyopathy [adjusted subhazard ratio (aSHR) 1.46, 95% confidence intervals (CIs): 0.9–2.4, P = 0.12] or restrictive cardiomyopathy (aSHR 1.12, 95% CI: 0.65–1.95, P = 0.67). Accordingly, multivariable analysis suggested that 1 year mortality was not significantly different between sarcoidosis and non‐ischaemic dilated cardiomyopathy (aSHR 1.56, 95% CI: 0.9–2.7, P = 0.12) or restrictive cardiomyopathy (aSHR 1.15, 95% CI: 0.61–2.18, P = 0.66). No differences were observed regarding 30 day mortality, treated and hospitalized acute rejection, and 30 day death from graft failure after HT. Thirty‐day mortality did not improve significantly in more recent HT eras whereas there was a trend towards improved 1 year mortality in the latest HT era (P = 0.06). Data from the single‐centre case review showed excellent long‐term outcomes with sirolimus‐based immunosuppression.ConclusionsShort‐term and long‐term post HT outcomes among patients with sarcoid cardiomyopathy are similar to those with common types of non‐ischaemic cardiomyopathy.

Risk and predictors of mortality after implantable cardioverter-defibrillator implantation in patients with sarcoid cardiomyopathy

Implantable cardioverter-defibrillators (ICDs) are recommended for patients with cardiac sarcoidosis (CS) with an indication for pacing, prior ventricular arrhythmias, cardiac arrest, or left ventricular ejection fraction <35%, but data on outcomes are limited. Using data from the National Cardiovascular Data Registry ICD Registry between April 1, 2010 and December 31, 2015, we evaluated a propensity matched cohort of CS patients implanted with ICDs versus non-ischemic cardiomyopathies (NICM). We compared mortality using Kaplan-Meier survival curves and Cox proportional hazards models. We identified 1,638 patients with CS and 8,190 propensity matched patients with NICM. The rate of death at 1 and 2 years was similar in patients with CS and patients with NICM (5.2% vs 5.4%, P = 0.75 and 9.0% vs 9.3%, P = 0.72, respectively). After adjusting for other covariates, patients with CS had similar mortality at 2 years after ICD implantations compared with NICM patients (RR 1.03, 95% CI 0.87-1.23). Among patients with CS, multivariable logistic regression identified 6 factors significantly associated with increased 2-year mortality: presence of heart failure (HR 1.92, 95% CI 1.44-3.22), New York Heart Association (NYHA) Class III heart failure (HR 1.68, 95% CI 1.16-2.45), NYHA Class IV heart failure (HR 3.08, 95% CI 1.49-6.39), atrial fibrillation/flutter (HR 1.66, 95% CI 1.17-2.35), chronic lung disease (HR 1.64, 95% CI 1.17-2.29), creatinine >2.0 mg/dL (HR 4.07, 95% CI 2.63-6.30), and paced rhythm (HR 2.66, 95% CI 1.07-6.59). Mortality following ICD implantation was similar in CS patients compared with propensity matched NICM patients. Presence of heart failure, NYHA class, atrial fibrillation/flutter, chronic lung disease, renal dysfunction, and paced rhythm at time of implantation were all predictors of increased 2-year mortality among CS patients with ICDs.

Abstract 10663: Outcomes After Heart Transplantation in Patients with Cardiac Sarcoidosis

Introduction: The number of patients with sarcoidosis requiring heart transplantation (HT) is on the rise. Hypothesis: The aim of this study was to evaluate outcomes of isolated HT in patients with sarcoid cardiomyopathy. Methods: Adult HT recipients were identified in the UNOS Registry between 1990 and 2020. Patients were grouped according to diagnosis. The cumulative incidences for the all-cause mortality and rejection were compared using Fine and Gray model analysis, accounting for re-transplantation as a competing risk. Moreover, we reviewed characteristics and outcomes of all HT recipients with previous diagnosis of sarcoid cardiomyopathy from a single-center. Results: A total of 30,874 HT recipients were included in the present study (n=240 sarcoidosis, n=1,431 non-ischemic restrictive cardiomyopathy, and n=29,203 non-ischemic dilated cardiomyopathy). During a total of 196,985 patient-years, all-cause mortality at the latest follow-up was not significantly different when comparing sarcoidosis to non-ischemic dilated cardiomyopathy (adjusted subhazard ratio [aSHR] 1.4, 95% confidence intervals [CI]: 0.88-2.3, p=0.15) or restrictive cardiomyopathy (aSHR 1.12, 95% CI: 0.65-1.95, p=0.67). Accordingly, multivariable analysis suggested that 1-year mortality was not significantly different between sarcoidosis and non-ischemic dilated cardiomyopathy (aSHR 1.56, 95% CI: 0.9-2.7, p=0.12) or restrictive (aSHR 1.15, 95% CI: 0.61-2.18, p=0.66) cardiomyopathy. Additionally, no differences were observed regarding 30-day mortality after HT. Data from the single-center case review showed excellent long-term outcomes with sirolimus-based immunosuppression. Conclusions: Short- and long-term outcomes after isolated HT do not seem to be inferior in sarcoid cardiomyopathy when compared to common types of non-ischemic cardiomyopathy.

Abstract 15038: Gender Differences Amongst Caucasians and African Americans in Rates of Arrhythmias, Implantable Cardiac Devices, and In-hospital Mortality in Sarcoidosis Patients With Cardiomyopathy

Introduction: The prevalence of sarcoid cardiomyopathy (SCM) with female predominance is well known. However, gender differences amongst individual races remains poorly studied. Hypothesis: We sought to determine and compare within each gender and race, the rates of arrhythmias, implantable cardiac devices, and in-hospital mortality in patients with SCM after excluding those with a history of coronary artery disease. Methods: The Nationwide Inpatient Sample was queried from 2010 to 2014 using ICD-9 diagnosis code (135) for sarcoidosis among patients &gt;18 years old. We combined it with code (425.8) for cardiomyopathy in other diseases including sarcoid and sarcoid heart muscle disease. We excluded patients with a history of prior myocardial infarction, percutaneous coronary intervention and coronary artery bypass graft. Results: From 2010 to 2014, we identified 9,063 patients with SCM (mean age = 53.11 ± 11.28 years; men 51.8% and black 52.5%). Upon comparison of gender within races, event rates per 100 patients were (white male, white female, black male, black female; p-value): atrial fibrillation (20.7, 18.8, 18, 16.1; p=0.172), ventricular fibrillation (VF) (4.1, 1.7, 2.3, 2.1; p&lt;0.001), ventricular tachycardia (VT) (33.1, 19.3, 25.1, 21; p&lt;0.001), complete heart block (CHB) (9.1, 8.6, 3.4, 3.6; p=0.58), second degree Mobitz type II (0.3, 0.35, 0.8, 0.4; p=0.717), implantable cardioverter-defibrillator (ICD) (13.3, 9.3, 8.4, 9.2; p&lt;0.001), cardiac resynchronization therapy defibrillator (CRT-D) (5.5, 1.4, 3.4, 2.5; p&lt;0.001), permanent pacemaker (PPM) (2.2, 4.1, 1.1, 1.6; p&lt;0.001), sudden cardiac arrest (SCA) (1.3, 1.7, 1.3, 2.4; p=0.34), endomyocardial biopsy (EMB) (3.6, 2.1, 1.9, 2.3; p=0.011), cardiac MRI (CMR) (1.4, 0.3, 0.7, 1.3; p=0.002), cardiogenic shock (2.5, 1.4, 3, 3; p=0.027), orthotopic heart transplantation (OHT) (2.3, 1.4, 1.1, 0.2; p=0.05), catheter ablation (8.3, 4.5, 3.8, 1.9; p&lt;0.001), and in-hospital mortality (1.7, 3, 2.9, 2.6; p=0.008). Conclusion: White males had higher rates of VF, VT, CHB, but they also had a lower in-hospital mortality and SCA rates likely due to higher procedural rates such as EMB, ICD, CRT-D, catheter ablation and OHT as compared to others.

Outcomes of Acute Heart Failure in Patients with Amyloid or Sarcoid Cardiomyopathy: A 10 Year Nationwide Analysis

Background Ischemic Cardiomyopathy is the leading cause of heart failure in the United States. Restrictive cardiomyopathy etiologies like sarcoid and amyloid are less common causes of heart failure and in hospital outcome descriptions are scant across the literature. Purpose describe the outcomes of acute heart failure exacerbations in patients with sarcoid or amyloid cardiomiopathy Methods A retrospective analysis using the 2005-2014 United States Nationwide Inpatient Sample was performed. Patients above 18 years with primary diagnosis of acute heart failure (AHF), systolic heart failure (SHF) and diastolic heart failure (DHF) were included. Secondary diagnosis of Sarcoid Cardiomyopathy (SCM) or Amyloid Cardiomyopathy (ACM) was generated. End stage renal disease patients were excluded. Primary outcome was in-hospital mortality. Secondary outcomes included length of stay, cost of stay, shock, utilization of mechanical ventilation, acute kidney injury requiring dialysis (AKID), use of non-invasive ventilation (NIV), cardiac arrest, and short term mechanical circulatory support. Diagnoses were identified using ICD-9- CM codes . Multivariate logistic regression analysis adjusting for age, race, gender, hospital location was done using STATA 15. Results 2,821,201 patients with primary diagnosis of AHF were identified. In AHF cohort 2,893 patients had a history of ACM, corresponding to 42% patients with SHF and 58% patients with DHF. The SCM cohort was composed of 1,905 with AHF of which 83% had SHF and 17% had DHF. Mean age in ACM with AHF was 73 years. 63% were males. In the SCM, 52 % males with mean age of 53 years. ACM patients had increased mortality in the AHF(OR 2.5 p Conclusions In patients with AHF, mortality is increased in those with ACM compared to those without. SCM is associated with increased mechanical support use and development of shock

443 Role of electro-anatomical mapping of the right ventricle in evaluation of patients with unexplained cardiac arrest and familial sudden death

Cardiac arrest without evident cardiac disease may be caused by uncommon genetic conditions, including primary electrical disease and latent arrhythmogenic right ventricular cardiomyopathy (ARVC). The Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER) was designed to assess the results of systematic assessment of patients with unexplained cardiac arrest (UCA) and their 1st degree relatives. The objective of this study was to assess the role of electro-anatomical mapping of the right ventricle (RV) to aid in the diagnosis of latent ARVC in these patients. After exclusion of structural heart disease and manifest primary electrical disease (normal echo and ECG), patients with UCA and select family members underwent an electro-anatomic map of the RV during SR using a 4 mm Navistar catheter (Biosense-Webster, Diamond Bar, California). The unipolar (filtered at 0.5 to 400 Hz) and bipolar (filtered at 30 to 400 Hz) intracardiac electrograms were sampled at each endocardial site. Areas of abnormal voltage were defined as < 1mV. A total of 18 patients (15 UCA and 3 first degree relatives, age 37 ± 13 years, 61% male) underwent electro-anatomic map of the RV during SR. Three patients had abnormal RV voltage maps; all 3 patients had a history of UCA. Patient 1 is a 57 yo male with additional phenotypic evidence suggestive but not diagnostic of ARVC (T Wave inversion in V2, minor RV abnormality on MRI and borderline SAECG), and is therefore considered likely to have ARVC. Patient 2 was a 55 yo male who had additional phenotypic evidence suggestive but not diagnostic of ARVC. The patient succumbed from electrical strorm and at autopsy was found to have sarcoid cardiomyopathy. Patient 3 is a 71 yo male who developed progressively severe mitral regurgitation secondary to mitral valve prolapse and monomorphic VT originating from below the mitral valve and is felt not to have ARVC. Another patient with a normal RV voltage map had phenotypic evidence suggestive but not diagnostic of ARVC. We found abnormal electro-anatomic maps of the right ventricle in 3/15 patients with UCA. Additional investigations suggested a diagnosis of ARVC in 1/3 patients. Another patient was initially felt to have ARVC but at autopsy was found to have sarcoid cardiomyopathy. The role of electro-anatomical mapping of the RV in evaluation of patients with unexplained cardiac arrest requires further investigation in a large cohort.

Outcome of Heart Transplantation in Patients with Sarcoid Cardiomyopathy

Background Cardiac transplantation has been utilized increasingly in patients with sarcoid cardiomyopathy. We sought to review outcomes in patients with sarcoidosis who had heart transplants and compared their outcomes with those of transplanted patients without sarcoidosis. Methods We retrospectively reviewed the United Network for Organ Sharing (UNOS) database to assess the survival of patients with sarcoidosis who were treated by heart transplantation. Results Over an 18-year period, 65 patients (40 men and 25 women) with sarcoidosis underwent orthotopic heart transplantation. There were 4 operative deaths and 12 late deaths at a mean follow-up of 40 months. One-year post-transplant survival was significantly better for sarcoid patients receiving orthotopic transplantation compared with contemporaneous patients receiving transplantation for all other diagnoses (87.7% vs 84.5%, p = 0.030). Conclusions Patients with sarcoidosis undergoing orthotopic heart transplant had better short- and intermediate-term survival than the majority of heart transplant recipients. The diagnosis of sarcoidosis should not disqualify potential transplant candidates.

Outcome of Heart Transplantation in Patients With Sarcoid Cardiomyopathy

Cardiac transplantation has been utilized increasingly in patients with sarcoid cardiomyopathy. We sought to review outcomes in patients with sarcoidosis who had heart transplants and compared their outcomes with those of transplanted patients without sarcoidosis. We retrospectively reviewed the United Network for Organ Sharing (UNOS) database to assess the survival of patients with sarcoidosis who were treated by heart transplantation. Over an 18-year period, 65 patients (40 men and 25 women) with sarcoidosis underwent orthotopic heart transplantation. There were 4 operative deaths and 12 late deaths at a mean follow-up of 40 months. One-year post-transplant survival was significantly better for sarcoid patients receiving orthotopic transplantation compared with contemporaneous patients receiving transplantation for all other diagnoses (87.7% vs 84.5%, p = 0.030). Patients with sarcoidosis undergoing orthotopic heart transplant had better short- and intermediate-term survival than the majority of heart transplant recipients. The diagnosis of sarcoidosis should not disqualify potential transplant candidates.

Recurrence of Cardiac Sarcoidosis in a Heart Transplant Recipient

We present the case of a young man who received a left ventricular assist device (LVAD) as a bridge to transplantation for end-stage heart failure, in whom the diagnosis of sarcoid cardiomyopathy was made at the time of LVAD implantation. He subsequently underwent uncomplicated heart transplantation. Nineteen months later, a routine surveillance endomyocardial biopsy specimen demonstrated recurrence of sarcoidosis in the transplanted heart.

Sarcoid cardiomyopathy

The patient is a 40 year old African-American female with no significant past medical history who presented to the ED with a 2 week history of lower extremity edema and shortness of breath. She also noticed intermittent palpitations, dry cough, easy fatigue and a 30 pound weight loss. On examination, the patient’s temperature was 98.9F orally, pulse was 155-170 beats per minute, and blood pressure was 133/93 mmHg. EKG revealed long P-R tachycardia with heart rate of 170. Chest X- ray was done and showed large right pleural effusion with compressive atelectasis and small left pleural effusion. Transthoracic echocardiogram at the time of admission was consistent with severe global LV dysfunction with EF-20% and moderate pulmonary hypertension. CT Chest, which showed bilateral hilar adenopathy and subsequent biopsy was consistent with non-caseating granulomatous lesion. Her initial basic laboratory values, including TSH level, were normal except for BNP which was 569. She underwent left and right heart catheterization, which revealed normal coronary arteries and decreased cardiac index. During the hospital stay, telemetry monitors revealed rhythms consistent with various degrees of AV conduction abnormalities. She was started on steroids and intravenous milrinone for possible sarcoid cardiomyopathy and listed for heart transplant. Her symptoms were gradually improved and she was discharged home with steroids, beta blockers, diuretics, ace inhibitors and an implantable defibrillator. She is currently followed as an outpatient and doing extremely well without any cardiac symptoms.

Sarcoidosis and the Heart.

The treatment of sarcoid cardiomyopathy can be considered in part the treatment of the systemic disorder, and in part cardiac involvement, the manifestations of which may differ greatly. Therapy for the systemic disease is corticosteroid. Therapy for cardiac involvement includes prednisone, but because treatment must ameliorate or abolish many differing manifestations, therapy differs among patients. Asymptomatic patients (the majority) who are free from serious manifestations of the disease do not require pharmacologic or interventional treatment. Patients with dilated cardiomyopathy require treatment for congestive heart failure. High-grade atrioventricular conduction delay usually necessitates a permanent electronic pacemaker. Life-threatening arrhythmia usually requires implantation of an automatic implantable cardiac defibrillator (AICD). Antiarrhythmic drugs may also be needed. Cardiac tamponade should be treated by drainage of pericardial fluid. Pericardiectomy is usually the appropriate treatment for patients who develop significant constrictive pericarditis. Calcium channel blockers may be helpful for severe diastolic dysfunction that occurs in those with restrictive cardiomyopathy. Therapy should be given to those few patients who manifest hypertrophic cardiomyopathy to relieve left ventricular outflow obstruction. Cardiac transplantation for intractable heart failure or arrhythmia may be needed.

Doppler Echocardiographic Detection of Left Ventricular Diastolic Dysfunction in Patients With Pulmonary Sarcoidosis

To determine the prevalence of left ventricular diastolic dysfunction in patients with biopsy specimen-proved pulmonary sarcoidosis without clinical evidence of cardiac disease. A cross-sectional study. A large tertiary care university teaching hospital. Fifty consecutive subjects had biopsy specimen-proved pulmonary sarcoidosis without suspected cardiac involvement. Those with other conditions known to affect diastolic function were excluded. The control group comprised 30 healthy hospital workers. Clinical examination, 12-lead ECG, and combined echocardiographic/phonocardiographic examination. Indexes of left ventricular diastolic function, including isovolumic relaxation time, peak velocity of early (E) and late (A) ventricular filling, deceleration rate of early diastolic flow, and the sum of the time velocity integrals of E and A were obtained in each patient and control subject. Systolic function was determined using a modification of Simpson's rule. Diastolic dysfunction was present in 7 (14%) patients, 6 of whom had normal systolic function and normal two-dimensional echocardiographic examination. Those with diastolic dysfunction had a longer duration of illness (15 +/- 7 vs 6 +/- 5 years; p = 0.0004), were significantly older (52 +/- 11 vs 38 +/- 9 years; p = 0.0009), and had higher systolic BP (130 +/- 13 vs 117 +/- 12 mm Hg; p = 0.01) than the sarcoid patients with normal diastolic function. These results demonstrate a significant prevalence of left ventricular diastolic dysfunction in patients with pulmonary sarcoidosis. The cause of this abnormality may be a subclinical sarcoid cardiomyopathy.

Sarcoid Cardiomyopathy Precipitated by Pregnancy With Cocaine Complications

We report the case of a 27-year-old previously healthy multiparous black woman who developed congestive heart failure 5 days postpartum. It was initially diagnosed and treated as idiopathic peripartum cardiomyopathy. Five weeks later, pulmonary sarcoidosis was diagnosed by transbronchial biopsy specimen, and steroid therapy was begun. An endomyocardial biopsy specimen 1 month later indicated cardiac sarcoidosis. She responded well to steroid therapy; however, 16 months later, she had a cardiac arrest and died. Autopsy confirmed the diagnosis of cardiac sarcoidosis.