Circulation, Volume 148, Issue Suppl_1, Page A11597-A11597, November 6, 2023. Background:Pulmonary hypertension has varying effects on cardiac function. Though uncommon, a dilated pulmonary artery can cause decreased coronary perfusion through extrinsic compression of the left main coronary artery (LMCA). While the most common presentation of LMCA compression is angina, acute coronary syndrome is rare.Case Summary:An 81-year-old female with a history of group 3 pulmonary hypertension presenting with neck and shoulder pain was found to have elevated cardiac biomarkers and EKG findings remarkable for isolated ST elevation in aVR with diffuse ST depressions in all other leads, consistent with a non-ST elevation myocardial infarction. Coronary angiography revealed left main coronary artery compression that was successfully intervened on with placement of a single drug eluding stent. Right heart catheterization was remarkable for evidence of severe pulmonary hypertension with a mean pulmonary artery pressure of 60 mmHg.Discussion:Given the patient’s past medical history, presenting symptoms, coronary and right heart catheterization findings the etiology of her acute coronary syndrome presentation was thought to be driven by left main coronary artery compression from pulmonary artery dilation. Pulmonary artery dilation in individuals with a history of pulmonary hypertension is common, with several modalities available to evaluate for this anatomic compensatory measure. Management for left main coronary artery compression in this patient population is percutaneous coronary intervention. Future studies to determine if routine screening for coronary compression in patients with pulmonary hypertension is needed.
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Abstract 14560: Diabetes Mellitus and Hypertension Subtypes in Relation to Cardiovascular and All-Cause Mortality
Circulation, Volume 148, Issue Suppl_1, Page A14560-A14560, November 6, 2023. Background:Diabetes mellitus (DM) is associated with increased arterial stiffness indicated by isolated systolic hypertension (ISH). Whether this relates to greater cardiovascular disease (CVD) mortality remains unclear. We compare by DM status the association of hypertension subtypes with CVD and all-cause mortality.Methods:We included data from the National Health and Nutrition Examination Survey 1999-2008 with mortality follow-up until 2018. We used systolic and diastolic blood pressure cut points of 130 and 80 mmHg to define ISH, isolated diastolic hypertension (IDH), and systolic diastolic hypertension (SDH) with DM based on self-report, glucose ≥126 mg/dL if fasting or ≥200 mg/dL if non-fasting, use of DM medications or insulin, or HbA1c≥6.5%. The incidence of CVD and all-cause mortality was examined among hypertensive subtypes (vs. normotension) using Cox regression, adjusting for age, ethnicity, gender, tobacco use, dyslipidemia, and use of antihypertensive agents.Results:23,198 individuals (projected to 194 million) were included of which 14% had DM (projected to 19 million). In those with vs. without DM, 40% vs. 60% were normotensive, 5% vs. 9% had IDH, 39% vs. 18% had ISH, and 16% vs. 13% had SDH (p
Abstract 18940: Can the Religious Observation of Fasting Cause Prinzmetal’s Angina?
Circulation, Volume 148, Issue Suppl_1, Page A18940-A18940, November 6, 2023. CASE DESCRIPTION:A 73-year-old male with HTN, HLD, CAD/MI status post PCI to the RCA and LAD (2021), and former tobacco use presented with acute, severe, left-sided chest pain with nausea, diaphoresis and dizziness. He started fasting and adjusted his sleep schedule 3 days prior for Ramadan, but did not miss any of his medications including isosorbide and ranolazine. He was chest pain free on arrival, but subsequently developed severe chest pain becoming bradycardic, hypotensive and hypoxic. At that time the EKG (Figure 1) showed ST elevation (STEMI) in leads II, III and aVF with high degree atrioventricular block. He was started on intravenous fluids and a vasopressor but had spontaneous resolution of symptoms within 10 minutes. Emergent coronary angiography revealed stable 70% stenosis of an ostial left circumflex (LCx) artery and non-obstructive CAD of the RCA. Labs revealed normal blood glucose, negative urine toxicology and mild elevation of troponin (trended serially). Echocardiogram was normal with no regional wall motion abnormalities. He was treated as vasospastic angina with addition of a calcium channel blocker without recurrence of his symptoms.DISCUSSION:Vasospastic angina is an important cause of acute coronary syndrome. Our patient had severe vasospastic angina while being treated for CAD for over 10 years. No prior studies have linked fasting to vasospasm. We hypothesize that fasting and a diurnal sleep schedule may contribute to worsened episodes in susceptible individuals due to hormonal changes including glucose shifts and an increase in catecholamines and cortisol. Increased catecholamine levels may induce spasm by stimulation of alpha receptors in the coronary arteries, and cortisol can induce vascular inflammation, potentiating negative effects. We highlight the importance of keeping high clinical suspicion for a diagnosis of vasospasm, as well as the need to further understand the complex pathophysiology to counsel patients in the future.
Abstract 16284: Atypical Cause of Embolic Stroke; Intracardiac Metastasis of Mixed Germ Cell Tumor
Circulation, Volume 148, Issue Suppl_1, Page A16284-A16284, November 6, 2023. Testicular neoplasm constitutes the most common solid organ malignancy in males between the ages of 15 and 35. Metastatic disease to the heart is a rare entity. In most cardiac cases, metastasis is by direct hematogenous spread via the inferior vena cava to the right heart. Metastatic disease to the left heart is even more infrequent. We present the case of intracardiac metastasis of mixed germ cell tumor presenting with an embolic stroke. A 47-year-old Latinx male with a history of stage IV testicular cancer s/p left radical orchiectomy with multiple bilateral pulmonary masses (largest 6×9 cm), atrial fibrillation s/p ablation, left lower extremity iliac thrombus s/p thrombectomy, HTN, and diabetes who presented with sudden onset expressive aphasia and dysarthria. CT head showed a 3×5 cm left occipital intraparenchymal hemorrhage suspected to be an embolic stroke with hemorrhagic conversion. Further evaluation with TTE and TEE revealed a highly mobile and friable multi-lobar mass attached to the anterior mitral leaflet occupying most of the left atrial cavity (4.2×4.9 cm), a small mass attached to the right ventricular aspect of the tricuspid valve, an echo-dense mass in the left lower pulmonary vein at the left atrial junction, and a large mass abutting the rim of the superior vena cava. Definitive surgical management was considered; however, patient was deemed a high-risk candidate given high burden of cardiac metastases. After multidisciplinary discussion, it was decided to pursue systemic chemotherapy. This case illustrates an exceptionally rare presentation of advanced stage testicular cancer metastasizing to the heart with large intracardiac tumor burden resulting in embolic stroke. Early cardiac screening may be beneficial in individuals with history of cancer who present with stroke symptoms. Embolization of cardiac metastasis to other organs poses a great clinical challenge and multidisciplinary decision-making is crucial to clinical management.
Abstract 14503: Chemokine Ligand 18 (CCL18) is a Long-Term Predictor of All-Cause Mortality, but Did Not Predict CVD Events
Circulation, Volume 148, Issue Suppl_1, Page A14503-A14503, November 6, 2023. Introduction:Chemokines mediate leukocyte recruitment and activation. Chemokine ligand 18 (CCL18) is mainly expressed by a broad range of cells such as monocytes/macrophages and dendritic cells. It is highly expressed in chronic inflammatory diseases, and locally in atherosclerotic plaques, particularly at sites of reduced stability. Circulating CCL18 levels are transiently elevated in patients admitted with unstable angina pectoris, but data on myocardial infarction (MI) are scarce.Aim:To assess the utility of CCL18 as a prognostic marker of CVD in patients hospitalized for chest pain of suspected coronary origin.Methods:The population consisted of 871 consecutive patients (ClinicalTrials.gov Identifier: NCT00521976). Stepwise Cox regression models, applying continuous loge-transformed values, were fitted for each of the biomarkers with all-cause mortality and cardiac death within 24-months and all-cause mortality within median 7 years (y) as the dependent variables, adding an analysis of 1) all-cause mortality or MI, and 2) all-cause mortality or MI or stroke at 7 y follow up (FU). The hazard ratio (HR) (95% CI) was assessed in a univariate and multivariable model, adjusting for traditional clinical cardiovascular risk factors, adding BNP, hsCRP, and TnT.Results:Plasma samples from 849 patients were available. By 24 months FU, 138 (15.8%) patients had died; 86 were cardiac deaths. The univariate analysis showed a positive, significant association between CCL18 and total death [HR 1.55 (95% 1.30-1.83), p=0.000], and for cardiac death [HR 1.32 (95% 1.06-1.64), p=0.013]. Applying multivariable analysis, the associations were no longer significant.At 7 y FU, a total of 332 (38.1%) patients had died. After multivariable adjustment, CLL18 was independently associated with all-cause mortality [HR 1.14 (95% CI, 1.01-1.29), p=0.030], but not for MI (n=203) or stroke (n=55). The positive association between CCL18 and all-cause mortality was lost when combined with CVD events; all-cause mortality or MI (n=404, p=0.75), and all-cause mortality or MI or stroke (n=422, p=0.79).Conclusions:CCL18 predicts long-term all-cause mortality, but had no independent prognostic bearing on short-term cardiac death and CVD events during long-term FU.
Abstract 17431: A Rare Cause of Constrictive Pericarditis in a Case of Adult T-cell Lymphoma
Circulation, Volume 148, Issue Suppl_1, Page A17431-A17431, November 6, 2023. Introduction:Adult T-cell lymphoma (ATCL) is a rare T cell lymphoproliferative malignancy that typically presents with lymphadenopathy, hepatosplenomegaly, lytic lesions, hypercalcemia or skin lesions. Cardiac involvement is rare.Case presentation:A 66-year-old male with a history of chronic kidney disease and hypertension presented with dyspnea. Transthoracic echocardiography revealed a moderate pericardial effusion and a left ventricular ejection fraction of 45%. Further workup with MRI and transesophageal echocardiography found extensive circumferential pericardial thickening with enhancement and extensive mediastinal and hilar lymphadenopathy along with a mass along the course of the superior vena cava with extension of disease into the right atrium along the right atrial wall (Figure 1). Endobronchial biopsy of the mediastinal lymph nodes was positive for T-cell lymphoma. Chemotherapy was initiated; however, the patient decided to focus on comfort measures only.Discussion:Cardiac involvement in ATCL is rare and usually detected in postmortem autopsies. Incidence is unknown, and experience is limited to case reports. The largest case series to date found 16 patients who had antemortem diagnosis of symptomatic cardiac involvement of ATCL. The characteristic features included infiltration of the myocardium, pericardial effusion, valvular lesions, and masses in the atrium, similar to what was seen in our patient. To our knowledge, there have been no reported cases of ATCL causing constriction. Prognosis is poor, with median survival of 5 months after development of symptoms (n = 15, range 0-120 months).Conclusion:The incidence of cardiac ATCL is unknown, but it is an extremely rare occurrence. It can present with a wide variety of cardiac involvement, including constriction. The prognosis of cardiac ATCL remains poor.
Abstract 13163: Clonal Hematopoiesis Risk Score is Associated With All-Cause and Cardiovascular Mortality in Older Adults: The Atherosclerosis Risk in Communities (ARIC) Study
Circulation, Volume 148, Issue Suppl_1, Page A13163-A13163, November 6, 2023. Introduction:Clonal hematopoiesis of indeterminate potential (CHIP) is common in older adults, but the prognostic significance is unknown. We studied the prevalence of CHIP and its mortality in older adults and investigated whether the CHIP risk score (CHRS) -tool to predict hematologic malignancies (HM)- is associated with mortality in this population.Method:In ARIC cohort, CHIP was identified using exome sequencing, and CHRS scores were calculated using demographic, blood parameters, and molecular features. Individuals with CHIP were categorized into low- (CHRS≤9.5), intermediate- (9.5
Abstract 15724: All-Cause Death and Major Adverse Events in Atrial Fibrillation With Frailty: Observations From the Korea National Health Insurance Service Data
Circulation, Volume 148, Issue Suppl_1, Page A15724-A15724, November 6, 2023. Introduction:Atrial fibrillation (AF) may be a marker of frailty. Previous evidence indicated that frailty in patients with AF has been associated with increased adverse events.Hypothesis:This study aimed to investigate the effect of frailty on the use of oral anticoagulants (OAC) and clinical outcomes in a nationwide cohort of patients with AF.Methods:We included 451,368 participants without AF from the Korea National Health Insurance Service-Health Screening cohort from 2005 to 2010. Patients’ Hospital Frailty Risk Score was calculated and patients were divided into two groups: without frailty (< 5 points) and with frailty (≥ 5 points).Results:Over 7.2 years, 11,953 participants (median age, 67 years; 60.2% men) developed new-onset AF. Among these patients, 26.9% had frailty. Frailty was significantly associated with older age, female sex, polypharmacy, and comorbidities. Frailty was negatively associated with OAC prescription after new-onset AF (P< 0.001). Patients with frailty had significantly higher risk of all-cause death (hazard ratio [HR] 2.87, 95% confidence interval [CI] 2.66-3.11), cardiovascular death (HR 2.60, 95% CI 2.77-2.98), stroke (HR 2.49, 95% CI 2.25-2.75), major bleeding (HR 2.54, 95% CI 2.28-2.83), and heart failure admission (HR 1.30, 95% CI 1.11-1.53). The increased risks of all-cause death, cardiovascular death, stroke, and heart failure admission due to frailty were lower with OAC prescription.Conclusions:Frailty was negatively associated with OAC use and was a predictor of worse prognosis. However, the increased risks associated with frailty were reduced by OAC in patients with AF.
Abstract 18878: Right Heart Valves Affected by Cancer-Related Non-Bacterial Thrombotic Endocarditis as Potential Cause of Pulmonary Embolism
Circulation, Volume 148, Issue Suppl_1, Page A18878-A18878, November 6, 2023. Background:Ca-NBTE primarily affects the mitral valve (MV) and aortic valve (AV), with rare involvement of the right heart valves. Premortem descriptions of this condition are limited to a few case reports. This study presents an initial clinical series focusing on patients with Ca-NBTE specifically affecting their right heart valves. We describe the underlying cancers, types of valve lesions, and associated embolic complications.Patients and Methods:A electronic search of medical records (March 31, 2002, to June 30, 2022) was followed by a manual review to identify adult patients with echocardiographically detected NBTE, active cancer, and no infectious endocarditis or lupus anticoagulant/antiphospholipid antibodies.Results:Out of 115 identified patients with Ca-NBTE (mean age 63.2±9.7 years, 66.1% female), there were 8 cases (7.0%) affecting right heart valves (mean age 64.5±12.1 years, 3 females) with underlying cancer: pancreatic (n=3), lung (n= 2), genitourinary (n=1), gastric (n=1) and neck sarcomatoid carcinoma (n=1). TV was affected in 8 patients and PV in one case; 4 patients had Ca-NBTE limited only to PV, 2 patients had MV and TV, 1 patient had mitral, aortic and TV, and 1 patient with pancreatic cancer had all four valves affected by NBTE. 5 patients had embolic complications to the brain with one patient in that group also experiencing emboli to the kidney and spleen. Out of 4 patients with NBTE limited to TV, two still had brain emboli, one had only pulmonary embolism, and one patient had no embolic complications.Conclusion:Around 7% of Ca-NBTE cases involve the right heart valves, with half of these cases limited to the TV. The cancer profile in these cases resembles that seen in left heart valve involvement.
Abstract 18280: Five-Year Major Amputation Risk in Patients Undergoing Lower Extremity Bypass Taking into Account All-Cause Mortality Risk and Age Groups
Circulation, Volume 148, Issue Suppl_1, Page A18280-A18280, November 6, 2023. Background:Selecting good lower extremity bypass (LEB) surgery candidates for chronic limb threatening ischemia (CLTI) is important to optimize risk-benefit ratios for amputation and death. As mortality risks may be different across age groups, we aimed to examine whether amputation risks across age groups differ by their differential competing risk of death.Methods:Individuals with CLTI undergoing infrapopliteal LEB from 2014 to 2019 in the Medicare linked Vascular Quality Initiative registry were included. The cumulative incidence Function (CIF) for 5-year mortality and major amputation in age groups
Abstract 12083: Symptoms of Depression and All-Cause and Cardiovascular Disease Mortality Among US Adults, NHANES 2005-2018
Circulation, Volume 148, Issue Suppl_1, Page A12083-A12083, November 6, 2023. Introduction:Depression is a common mental health disorder among the US population. Depressive symptoms are associated with increased cardiovascular disease (CVD) incidence and mortality rates. Few recent studies have examined the association between depressive symptoms and mortality among US adults.Hypothesis:Depressive symptoms increase all-cause and CVD mortality among US adults.Methods:The present study used the National Health and Nutrition Examination Survey (NHANES) 2005-2018 data with the NHANES 2019 Linked Mortality Files among adults aged ≥20 years. Depressive symptoms were defined by the Patient Health Questionnaire-9 (PHQ-9) scores and categorized into 3 groups: (1) none or minimal (0-4); (2) mild (5-9); and (3) moderate to severe (10-27). Cox proportional hazard models were used to evaluate the association between depressive symptoms and all-cause, CVD, and ischemic heart disease mortality, adjusting for sociodemographic factors, lifestyle factors, and clinical characteristics.Results:Prevalences of none or minimal, mild, and moderate to severe depression were 77.9%, 14.9%, and 7.2%, respectively. For all-cause mortality, hazard ratios were 1.35 (95% confidence interval, 1.06, 1.72) for mild depressive symptoms vs none, and 1.61 (1.24, 2.10) for moderate to severe depressive symptoms vs none. The corresponding hazard ratios were 1.49 (1.11, 2.00) and 1.79 (1.22, 2.62) for CVD mortality, and 0.96 (0.58, 1.60) and 2.21 (1.24, 3.91) for ischemic heart disease mortality. The associations were largely consistent across subgroups. Approximately 11.0%-16.1% of the associations between depression and mortality could be explained by lifestyle factors.Conclusions:Overall, there was a graded and positive association between depressive symptoms and mortality among US adults. Public health and healthcare efforts to improve awareness and treatment of depression and its related risk factors can support a comprehensive strategy to reduce the overall burden of depression nationwide.
Abstract 13245: Neural Network-Derived Electrocardiographic Features Predict Future Cardiovascular Disease and All-Cause Mortality
Circulation, Volume 148, Issue Suppl_1, Page A13245-A13245, November 6, 2023. BackgroundSubtle prognostically-important ECG features may not be apparent to physicians. In the course of supervised machine learning (ML), many thousands of ECG features are identified. These are not limited to conventional ECG parameters and morphology.Hypothesis:Novel neural network (NN)-derived ECG features can predict future cardiovascular disease and mortalityMethods and Results:We extracted 5120 NN-derived ECG features from an AI-ECG model trained for six simple diagnoses and applied unsupervised machine learning to identify three phenogroups. In the derivation cohort (CODE, 1,558,421 subjects), the three phenogroups had significantly different mortality profiles. After adjusting for known covariates, phenogroup B had a 20% increase in long-term mortality compared to phenogroup A (HR 1.20, 95% CI 1.17-1.23, p < 0.0001). The predictive ability of the phenogroups was retained in a group with physician confirmed normal ECGs. We externally validated our findings in five diverse cohorts (Figure) and found phenogroup B had a significantly greater risk of mortality in all cohorts. Phenome-wide association study (PheWAS) showed phenogroup B had a higher rate of future AF, ischaemic heart disease, AV block, heart failure, VT and cardiac arrest. Phenogroup B had increased cardiac chamber volumes and decreased cardiac output. A single-trait GWAS yielded four loci. SCN10A, SCN5A and CAV1 have roles in cardiac conduction and arrhythmia. ARHGAP24 does not have a clear cardiac role and may be a novel target. Gradient-weighted Class Activation Mapping (Grad-CAM) identified the terminal QRS and terminal T wave as important regions of the ECG for identification of phenogroup B.Conclusion:NN-derived ECG features can be used to predict all-cause mortality and future cardiovascular diseases. We have identified biologically plausible and novel phenotypic and genotypic associations that describe mechanisms for the increased risk identified.
Abstract 14479: Compound Heterozygosity With Two Novel Mutations in MYBPC3 as a Cause of Severe and Early-Onset Hypertrophic Cardiomyopathy
Circulation, Volume 148, Issue Suppl_1, Page A14479-A14479, November 6, 2023. Case presentation:A 8-year-old male was diagnosed with severe hypertrophic cardiomyopathy (HCM) after a cardiac arrest from which he was successfully defibrillated. Maximal wall thickness was 23 mm (z-score 6.36) on echocardiography. An implantable cardioverter defibrillator (ICD) was implanted and atenolol was started. After 4 ventricular fibrillation-terminating ICD shocks in the following 2 months, further treatment is under consideration. A genetic test identified the likely pathogenic intronic variant (c.1927+337G >T) in the MYBPC3 gene. An additional variant in the same gene (p.Arg817Gly), of uncertain significance but potentially relevant for the phenotype, was also found. His 10-year-old sister was also diagnosed with severe HCM, genetic test identified the two previously referred variants and ICD was also implanted. In the 4-year-old one sister, who is apparently healthy, only the likely pathogenic intronic variant (c.1927+337G >T) was found. Regarding their parents, both with no evidence of cardiomyopathy, c.1927+337G >T variant was identified in the father, while p.Arg817Gly was present in the mother.Discussion:This clinical case is noteworthy as an example of compound heterozygosity in which each parent donates one alternate allele, being these alleles located at different loci within the same gen, which emphasizes the importance of family genetic testing. The variant of uncertain significance p.Arg817Gly appears to be a disease modifier, causing the phenotype to be expressed early and severely in the presence of the probably pathogenic intronic variant c.1927+337G >T in MYBPC3. This deep intronic variant has not been described previously but was found in thirteen index cases referred for HCM and a history of familial sudden death at an early age, being overrepresented in the cohort of HCM in the north of Spain. Being a carrier of both these two novel variants appears to cause an extremely aggressive childhood-onset phenotype.
Abstract 12340: Association of Liver Enzymes With Incident Cardiovascular Disease Events and All-Cause Mortality in the Hispanic Community Health Study/Study of Latinos (HCHS/SOL)
Circulation, Volume 148, Issue Suppl_1, Page A12340-A12340, November 6, 2023. Introduction:Metabolic associated fatty liver disease (MAFLD) is a significant contributor to chronic liver disease on a global scale, encompassing a broad range of systemic impacts, including an increased risk of cardiovascular disease (CVD). Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels are commonly utilized indicators of liver injury in this context. Despite the high prevalence of MAFLD among Hispanic/Latino populations, there is a scarcity of research focusing on this specific demographic.Aim:To investigate the relationship between liver disease, incident CVD, and mortality in a representative Hispanic/Latino population, using non-invasive markers.Methods:A total of 14,149 participants recruited between 2008-2011 in HCHS/SOL aged 18-74 years with no pre-existing CVD at study baseline were included in this study. The outcome of interest was a composite of adjudicated incident CVD and all-cause mortality. Participants who had liver function tests (LFTs) AST > 37 IU/mL or ALT > 40 IU/mL for men, and AST or ALT > 31 IU/mL for women were classified as having elevated LFTs. MAFLD was defined as a fatty liver index score >60 and: type 2 diabetes, body mass index >25 kg/m2or at least two metabolic risk abnormalities. Survey-weighted cox proportional hazards models estimated hazard ratios (HR) and 95% confidence intervals (CI) for our composite outcome by elevated LFTs. Interaction terms assessed the relationship between elevated LFTs and MAFLD.Results:The study population was 40 years old on average, 53.4% female and had 403 weighted CVD and all-cause mortality events. Elevated LFTs and MAFLD alone were not associated with incident CVD and all-cause mortality (HR: 1.24; CI: 0.89-1.71). MAFLD modified the relationship between elevated LFTs and CVD (P=0.04). Among those with MAFLD, elevated LFTs were not associated with CVD (HR: 1.01; CI:0.70-1.46), but among those without MAFLD, elevated LFTs were associated with CVD (HR: 2.05; CI:1.17-3.58).Conclusions:Among Hispanic/Latinos without MAFLD, elevated LFTs were associated with CVD and all-cause mortality. Additional research is needed to elucidate the relationship between liver disease and CVD in the Hispanic/Latino population.
Abstract 11836: A Rare Cause of Recurrent Myocardial Infarction: Genetic Testing Cracks the Case
Circulation, Volume 148, Issue Suppl_1, Page A11836-A11836, November 6, 2023. A 29-year-old man presented to our institution with acute, severe substernal chest pain. An ECG on arrival revealed 3mm ST-elevations in the lateral leads with reciprocal depressions in the inferior leads. Troponin-I was above assay ( >50 ng/mL). Emergent coronary angiography showed normal coronary arteries, and left ventriculogram showed no wall motion abnormalities. A transthoracic echocardiogram showed normal biventricular size, wall thickness, and ejection fraction (EF). This was his fifth episode of chest pain over the past 10 years, consistent with prior presentations of ST-elevation myocardial infarction and angiographically normal coronary arteries. A prior cardiac magnetic resonance imaging (MRI, 6 years before the current presentation) revealed increased T2 signal intensity and subepicardial late gadolinium enhancement (LGE) in the septum, inferior, and inferolateral walls, suggestive of nonischemic acute myocardial injury. A repeat cardiac MRI during the current presentation showed mildly reduced biventricular systolic function (LVEF 52%, RVEF 36%), and again showed increased T2 signal intensity and subepicardial LGE involving the septum, inferior and inferolateral walls. The anterior and anterolateral walls were also now involved. Endomyocardial biopsy (EMB) showed focal interstitial fibrosis with no evidence of active myocarditis. On telemetry he was noted to have runs of non-sustained ventricular tachycardia (NSVT). Serologic evaluation was negative for parvovirus, cytomegalovirus, and Chagas disease. Erythrocyte sedimentation rate and C-reactive protein levels were normal. Genetic testing was performed at our newly established cardiovascular genetics program, which showed a pathogenic truncating mutation in the desmoplakin gene (DSP). DSP cardiomyopathy (CM) is an LV-predominant arrhythmogenic CM, which can present with episodic myocardial injury, and has a high disposition for ventricular arrhythmias with subepicardial LGE pattern often proceeding LV systolic dysfunction. A primary prevention implantable cardioverter-defibrillator was placed given extent of LGE and NSVT burden in the context of DSP variant. This case illustrates the importance of genetic testing in identifying rare CM phenotypes.
Abstract 15580: Blood Transfusion in Ascending Aortic Surgery: No Inherent Cause for Concern
Circulation, Volume 148, Issue Suppl_1, Page A15580-A15580, November 6, 2023. Introduction:There is widespread concern that blood transfusion has a strong adverse impact on early/late surgical survival, so hematocrit is often allowed to drift very low. We investigate whether this adverse impact of transfusion is seen in aortic surgery.Methods:The records of 870 patients who underwent open ascending aortic or arch surgery from 2004 to 2016 by one surgeon at a large hospital were reviewed. Of these patients, 347 (39.9%) received a blood transfusion. The mean number of intra-op units transfused was 0.89, and the mean total units transfused (intra+post-op) was 2.45. High-volume transfusion (over 5 units) was needed for only 24 (6.9%) patients. We aimed to maintain a post-operative Hct of above 28%. Mean follow-up was 6.5 years.Results:We found no significant difference in 30-day or 1-year mortality between the transfused and non-transfused groups, nor between high- and low-volume transfused patients. However, the long-term mortality beyond 1 year was higher in the transfused group than in the non-transfused group (13% vs 5.7%; p=0.0003). Notably, the transfused group was older than the non-transfused group (64.9 vs 57.7). On Kaplan-Meier analysis (Fig.1), there remained a significant adverse effect of transfusion overall (p=0.004), but this effect was eliminated if patients were propensity matched for age (p=0.18). There was no difference in high- vs low-volume K-M survival (p=0.58). Amongst those transfused, the total units transfused was not a predictor of mortality in any time-frame on multivariate regression when adjusting for pre-op Hct, blood transfusions, DHCA, type A dissection, and operation urgency (p=0.95, 0.56, and 0.45 for 30d, 1yr, and beyond 1yr respectively).Conclusions:Transfusion and transfusion volume had no significant effect on early mortality, or even on late mortality after appropriate age adjustment. Should patient condition warrant, surgeons need not be concerned that transfusion will further reduce survival.