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Diagnostic performance characteristics of planar quantitative and semi-quantitative parameters of Tc99m pyrophosphate (PYP) imaging for diagnosis of transthyretin (ATTR) cardiac amyloidosis: the SCAN-MP study

Pandey, Shivda; Teruya, Sergio; Rodriguez, Carlos; Deluca, Albert; Kinkhabwala, Mona; Johnson, Lynne L; Fine, Denise; Sabogal, Natalia; Winburn, Morgan; Castillo, Michelle; Bhatia, Ketan; Malkovskaya, Rita; Raiszadeh, Farbod; Kurian, Damian; Miller, Edward J; Einstein, Andrew J; Maurer, Mathew S; Ruberg, Frederick L
BACKGROUND:pyrophosphate (PYP) imaging in a population with low pretest probability is not known. METHODS:Using myocardial PYP retention by SPECT as the reference standard, we evaluated the diagnostic performance of different semi-quantitative and quantitative (H/CL chest ratio) planar parameters obtained from 3-hour PYP imaging in a prospectively recruited cohort of minority older adults with heart failure and increased LV wall thickness. RESULTS:Of 229 patients, 14 were found to have ATTR-CA (6.1%). No PYP uptake (grade 0) was observed in 77% of scans, all grade 3 scans were ATTR-CA, and only 4 of 11 (36%) grade 2 scans were ATTR-CA. An H/CL threshold of ≥ 1.4 maximized specificity (99%) and positive predictive value (93%) but resulted in decreased sensitivity (93%), compared to the ≥ 1.3 threshold which had 100% sensitivity. CONCLUSION/CONCLUSIONS:Among patients with a low pretest likelihood of ATTR-CA, planar interpretation, while useful to exclude disease, must be interpreted with caution. H/CL ratio threshold of ≥ 1.3 resulted in clinically important misclassifications. These data suggest that quantitative planar imaging thresholds may not be appropriate to apply in low pretest likelihood populations being evaluated for ATTR-CA.
PMID: 36823486
ISSN: 1532-6551
CID: 5522782

Design and Rationale the SCAN-MP (Screening for Cardiac Amyloidosis With Nuclear Imaging in Minority Populations) Study

Ruberg, Frederick L; Blaner, William S; Chiuzan, Codruta; Connors, Lawreen H; Einstein, Andrew J; Fine, Denise; Helmke, Stephen; Kurian, Damian; Pandey, Shivda; Raiszadeh, Farbod; Rodriguez, Carlos; Sabogal, Natalia; Teruya, Sergio; Winburn, Morgan; Chung, Wendy K; Cohn, Elizabeth; Miller, Edward J; Kelly, Jeffery W; Maurer, Mathew S
Background Transthyretin amyloid cardiomyopathy (ATTR-CM) is an important cause of heart failure in older individuals. Misfolding and deposition of transthyretin or prealbumin protein causes ATTR-CM in the context of a normal (wild-type) or variant TTR sequence. Variant ATTR-CM is most commonly caused by the substitution of valine for isoleucine at position 122 in transthyretin (Val122Ile or pV142I, almost exclusively observed in individuals of West African ancestry), demonstrated in 3.4% of self-identified Black individuals in the United States with an estimated 1.5 million carriers. Despite the large number of known pV142I carriers, the proportion of older Black patients with heart failure attributable to ATTR-CM remains unknown. Methods To address this knowledge gap, the SCAN-MP (Screening for Cardiac Amyloidosis with Nuclear Imaging in Minority Populations) study was funded by the National Institutes of Health/National Heart, Lung, and Blood Institute (R01HL139671) to enroll a targeted population of self-identified, community-dwelling Black or Caribbean Hispanic patients (many of whom are of West African ancestry) >60 years of age with heart failure and identify ATTR-CM by noninvasive nuclear imaging. The principal objective of SCAN-MP is to determine the prevalence of ATTR-CM in this population. Secondary objectives will explore TTR genotype, demographics, progression of variant versus wild-type ATTR-CM, and biochemical mechanisms of transthyretin amyloid fibril formation. Conclusions The SCAN-MP study is the largest, prospective study of cardiac amyloidosis in Black and Hispanic individuals. Both wild-type and variant ATTR-CM are now treatable with the US Food and Drug-approved drug tafamidis. The insights gained from SCAN-MP are likely to improve those at risk for or afflicted with ATTR-CM. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03812172.
PMCID:10227254
PMID: 37066788
ISSN: 2047-9980
CID: 5522792

Physical Performance in Black and Hispanic Outpatients With Heart Failure: The SCAN-MP Study

Valente, Christopher J; Chiuzan, Cody; Alreshq, Rabah; Blot, Tori; Fine, Denise; Helmke, Stephen; Rodriguez, Carlos; Sabogal, Natalia; Teruya, Sergio; Winburn, Morgan; Kurian, Damian; Raiszadeh, Farbod; Maurer, Mathew S; Ruberg, Frederick L
BACKGROUND/UNASSIGNED:Deficits of physical function are associated with poor quality of life and adverse health outcomes, but data informing the association of these assessments among Black and Hispanic outpatients with heart failure (HF) are limited. METHODS/UNASSIGNED:opulations (SCAN-MP) study identified Black and Hispanic subjects with stable HF, collected baseline characteristics, and took measures using the short physical performance battery. Subjects completed a Kansas City Cardiomyopathy Questionnaire (KCCQ), and the clinical outcomes of HF hospitalization and death were ascertained by telephone and review of the electronic health record. RESULTS/UNASSIGNED: = 0.090). CONCLUSIONS/UNASSIGNED:Reduced physical performance is highly prevalent among Black and Hispanic outpatients with HF, and it is associated with overall KCCQ score, as well as an increased risk for HF hospitalization.
PMCID:10140745
PMID: 37124967
ISSN: 2589-790x
CID: 5522802

Relation of Body Mass Index to Transthyretin Cardiac Amyloidosis Particularly in Black and Hispanic Patients (from the SCAN-MP Study)

Poterucha, Timothy J; Kurian, Damian; Raiszadeh, Farbod; Teruya, Sergio; Elias, Pierre; Kogan, Rebecca; Chiuzan, Codruta; Einstein, Andrew J; Ruberg, Frederick L; Maurer, Mathew S
Heart failure with preserved ejection fraction is a heterogeneous clinical syndrome that includes distinct subtypes with different pathophysiologies, genetics, and treatment. Distinguishing heart failure with preserved ejection fraction caused by transthyretin cardiac amyloidosis (ATTR-CA) is critical given its specific treatment. We analyzed a single-center retrospective cohort to determine the association of body mass index (BMI) with a composite of either ATTR-CA or the valine-to-isoleucine substitution (Val122Ile) variant genotype (ATTR-CA+Val122Ile). These BMI differences were prospectively evaluated in the multicenter Screening for Cardiac Amyloidosis using nuclear imaging for Minority Populations (SCAN-MP) study of Black and Hispanic patients with heart failure. The association of BMI with ATTR-CA+Val122Ile was compared by Wilcoxon rank sum analysis and combined with age, gender, and maximum left ventricle wall thickness in multivariable logistic regression. In the retrospective analysis (n = 469), ATTR-CA+Val122Ile was identified in n = 198 (40%), who had a lower median BMI (25.8 kg/m2, interquartile range [IQR] 23.4 to 28.9) than other patients (27.1 kg/m2, IQR 23.9 to 32.0) (p <0.001). In multivariable logistic regression, BMI <30 kg/m2 (odds ratio 2.6, 95% confidence interval 1.5 to 4.5) remained independently associated with ATTR-CA+Val122Ile with a greater association in Black and Hispanic patients (odds ratio 5.8, 95% confidence interval 1.7 to 19.6). In SCAN-MP (n = 201), 17 (8%) had either ATTR-CA (n = 10) or were Val122Ile carriers (n = 7) with negative pyrophosphate scans. BMI was lower (25.4 kg/m2 [IQR 24.3 to 28.2]) in ATTR-CA+Val122Ile patients than in non-amyloid patients (32.7 kg/m2 [28.3 to 38.6]) (p <0.001), a finding that persisted in multivariable analysis (p = 0.002). In conclusion, lower BMI is associated with ATTR-CA+Val122Ile in heart failure with increased left ventricle wall thickness, particularly in Black and Hispanic patients, and may aid in the identification of those benefiting from ATTR-CA evaluation.
PMID: 35705430
ISSN: 1879-1913
CID: 5522712

A curious case of Lyme carditis in an urban hospital [Case Report]

Brissett, Shantel; Myint, Khaing T; Lopez, Yanira; Raiszadeh, Farbod; Sivapalan, Vel; Kurian, Damian
Lyme carditis (LC), a manifestation of early disseminated Lyme disease, most commonly presents with cardiac conduction abnormalities. It is a transient condition with good prognosis but in extremely rare cases may be life-threatening. We describe a 42-year-old man who presented with progressively worsening generalized weakness, presyncope and dyspnea on exertion for 2 weeks after sustaining a tick bite. He subsequently developed a 'bull's eye rash' on his flank 2 days before his presentation. He was found to have symptomatic third-degree AV conduction blockade with a ventricular escape rhythm resulting in a brief cardiac arrest. Intravenous (IV) ceftriaxone was commenced empirically and a temporary transvenous pacemaker was placed. In a few days he showed dramatic, rapid improvement; the pacemaker was removed, and the patient was discharged on oral doxycycline to complete a 24-day course. This case is unique due to its occurrence in an urban hospital where such cases are uncommon. Cardiac arrest, although brief in this case, is a rare occurrence. Lyme carditis was a surprise diagnosis in our hospital due to the patient's geographical dislocation during the COVID-19 pandemic.
PMCID:8225969
PMID: 34194998
ISSN: 2214-2509
CID: 5522772

SOCIOECONOMIC AND GEOGRAPHIC DISPARITIES IN CARDIOVASCULAR MORTALITY IN NYC [Meeting Abstract]

Adekunle, Adewumi; Olubowale, Olusola; Brissett, Shantel; Kurian, Damian; Farbod, Raiszadeh
ISI:000522979101936
ISSN: 0735-1097
CID: 5353862

A Case of Right Ventricular Dysfunction with Right Ventricular Failure Secondary to Obesity Hypoventilation Syndrome [Case Report]

Terla, Vikhyath; Rajbhandari, Griwan Lal; Kurian, Damian; Pesola, Gene R
BACKGROUND Obesity hypoventilation syndrome (OHS) is characterized by a body mass index (BMI) ≥30 kg/m², daytime hypercapnia, an arterial carbon dioxide tension ≥45 mmHg, and obstructive sleep apnea (OSA). OHS can lead to pulmonary hypertension. It has not been clearly demonstrated that OHS with pulmonary hypertension can lead to right ventricular dysfunction and right heart failure. A case is presented of right ventricular dysfunction and right ventricular failure secondary to OHS. CASE REPORT A 53-year-old man, who was morbidly obese with a BMI of 75 kg/m², presented with shortness of breath (SOB) and hypercapnia. He had never smoked but had a history of severe OSA and hypertension. On examination, the patient was obese with normal lung auscultation and mild pitting edema of the lower extremities. A spiral computed tomography (CT) angiogram showed no evidence of pulmonary embolism or interstitial lung disease. Pulmonary function testing showed no obstructive airway disease and a normal diffusion capacity. Two-dimensional transthoracic echocardiogram (TTE) showed normal left ventricular function and a dilated right ventricle (RV) with a flattened septal wall, moderate tricuspid regurgitation, and an estimated right ventricular systolic pressure of 55-60 mmHg. The patient was discharged on continuous positive airway pressure (CPAP) and oxygen at night, and as needed during the day. CONCLUSIONS This report has shown that OHS without underlying causes of alveolar hypoventilation can result in isolated right ventricular dysfunction and right ventricular failure.
PMCID:6796192
PMID: 31594915
ISSN: 1941-5923
CID: 5522762

Saddle Pulmonary Embolism with Thrombus in Transit across a Patent Foramen Ovale [Case Report]

Shepherd, Fitzgerald; White-Stern, Ashley; Rahaman, Oloruntobi; Kurian, Damian; Simon, Karen
This is the case of a 25-year-old obese man who presented with acute shortness of breath, chest pain, and palpitations. Of note, he lives a sedentary lifestyle and was recently hospitalized for incision and drainage of a left foot abscess. On presentation he was tachypnoeic, tachycardiac, and hypoxic but blood pressure was stable. Laboratory studies were significant for elevated D-dimer and mildly increased troponin. On further investigation he was found to have a saddle pulmonary embolism with massive clot burden. Echocardiogram revealed thrombus in transit and McConnell's sign. He underwent surgical embolectomy and closure of a patent foramen ovale. This is a particularly rare case, especially in such a young patient. Because this is a rare diagnosis, with insufficient data, there is no formally established treatment guideline. However, in patients who are good surgical candidates, studies have shown better outcome with surgical embolectomy as compared to anticoagulation alone or thrombolysis.
PMCID:5299177
PMID: 28246560
ISSN: 2090-6404
CID: 5522752

The optimal duration of dual antiplatelet therapy in patients receiving percutaneous coronary intervention with drug-eluting stents

Sheyin, Olusegun; Perez, Xavier; Pierre-Louis, Bredy; Kurian, Damian
BACKGROUND:The optimal duration of dual antiplatelet therapy (DAPT) following drugeluting stent (DES) implantation remains a subject of an ongoing debate. METHODS:MEDLINE, EMBASE, Scopus and CENTRAL databases were searched for eligible randomized controlled trials (RCTs) that compared short-term (£ 6 months) DAPT with long-term (≥ 12 months) DAPT following DES implantation. The primary endpoint was a composite of all-cause mortality, myocardial infarction (MI), target vessel revascularization (TVR), stroke, or major bleeding. The secondary outcome were the individual components of the primary outcome, cardiovascular death, stent thrombosis and any bleeding episode. RESULTS:A total of 15,378 patients from 7 RCTs were studied. There were no statistically significant differences between the short-term and long-term DAPT groups with respect to the occurrence of the primary outcome (risk ratio [RR] 1.017; 0.872-1.186; I2 = 0%), all cause death (RR 0.896; 0.708-1.134), cardiovascular death (RR 0.924; 0.668-1.279), MI (RR 1.139; 0.887-1.461), TVR (RR 1.174; 0.916-1.505), stent thrombosis (RR 1.264; 0.786-2.032), and stroke (RR 0.876; 0.685-1.611). However, there was a statistically significant lower risk of major bleeding in the short-term DAPT group (RR 0.57; 0.36-0.90; p = 0.02). There were no statistically significant differences in the sub-group analysis of patients with diabetes and patients presenting with acute coronary syndrome, RR 1.029; 0.745-1.421 and RR 1.062; 0.785-1.438, respectively. CONCLUSIONS:There was no difference in efficacy outcomes between short-term and long-term DAPT following DES, even among high-risk patients. However, longer duration of DAPT was found to be associated with increased risk of major bleeding.
PMID: 26711462
ISSN: 1898-018x
CID: 5522732

Right heart serpiginous mass

Davies, Oluwaseun; Greenbaum, Dennis; Clarke, Aubrey; Pierre-Louis, Bredy; Kurian, Damian
PMID: 27276900
ISSN: 1880-344x
CID: 5522742