Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Cardiology HomeNew OnlineCurrent IssueFor Authors Podcast Journals JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2024 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Cardiology journal

A 38-year-old female who was found to have severe pulmonary arterial hypertension (PAH) secondary to HIV in the postpartum period. This case illustrates the treatment and challenges of postpartum PAH. It also demonstrates the need for expert guidance and additional research on the optimal management of PAH in pregnancy and postpartum.

Klippel-Trénaunay syndrome (KTS) is a rare and complex congenital syndrome defined as the triad of cutaneous capillary malformation, bone and soft tissue hypertrophy, and venous and lymphatic malformations.KTS is thought to be due to a somatic mutation in phosphatidyl-inositol 3 kinase.It belongs to a group of syndromes termed the PI3CA-Related Overgrowth Spectrum (PROS) disorders.Because of the rarity and clinical heterogeneity of these disorders, management is patient specific, and best evidence guidelines are lacking.The most common clinical complications are thromboembolism, thrombophlebitis, pain, bleeding, and high-output heart failure.Surgery is recommended for hemangiomas and chronic venous insufficiency.The early identification of children with PROS disorders has allowed treatment with mTOR inhibitors which have been shown to be effective.The recent development of a direct PI3K inhibitor (alpelisib) has shown promise in preventing abnormal growth and long-term complications of KTS.This report documents a case of high-output heart failure due to the vascular malformations associated with KTS in a 57-year-old male patient and discusses current literature regarding the management of KTS with inhibitors of mTOR and PI3KCA.

Purpose of the Review This review focuses on broader perspectives of mitral regurgitation (MR) in patients with heart failure. Recent Findings The ratio of regurgitant volume to end-diastolic volume appears to help identify patients who may benefit from valve interventions. Secondary MR is not only attributed to geometric changes of the LV but also related to the structural changes in the mitral valve that include fibrosis of the mitral leaflets and changes in the extracellular matrix. The transition from mild to severe secondary MR can occur at different rates, from a slow LV remodeling process to a more abrupt process precipitated by an inciting event such as atrial fibrillation. Septal flash and apical rocking, two new visual markers of LV mechanical dyssynchrony, appear to be predictive of MR reduction following cardiac resynchronization therapy. Optimal guideline-directed medical therapy has been shown to decrease the severity of secondary MR effectively. Summary A theoretical framework to characterize secondary MR as it relates to the onset of MR is proposed. Type A: Early onset of MR contemporaneous with myocardial injury. The maladaptive LV remodeling occurs in parallel with MR. Type B: LV remodeling proceeds without significant MR until the LV is moderately dilated, which coincides with or without inciting factors such as atrial fibrillation. Type C: LV remodeling proceeds after myocardial injury without significant MR until the LV is severely dilated. MR is a late manifestation of LV remodeling.

Importance:Two randomized clinical trials of transcatheter edge-to-edge mitral valve repair in patients with secondary mitral regurgitation (the Multicentre Randomized Study of Percutaneous Mitral Valve Repair MitraClip Device in Patients With Severe Secondary Mitral Regurgitation [MITRA-FR] and the Cardiovascular Outcomes Assessment of the MitraClip Percutaneous Therapy for Heart Failure Patients with Functional Mitral Regurgitation [COAPT]) report clinical outcome disparities that are largely unexplained. This appraisal sought to provide insight and an explanation for the differences in clinical outcomes (survival and hospitalization rates) in the 2 clinical trials. The mean echocardiogram Doppler results (and derived volume parameters) from each of the 2 clinical trials were compared and examined relative to the clinical outcomes. Special emphasis was placed on the assessment of mitral regurgitation proportionality coefficients that were determined as the ratio of effective regurgitant orifice area (EROA) to end-diastolic volume and the ratio of mitral regurgitant volume to end-diastolic volume.Observations:In this analysis of the differences in the clinical outcomes of the MITRA-FR and COAPT clinical trials, the ratio of the EROA to the end-diastolic volume in the COAPT study was found to be twice that of the MITRA-FR study (0.002 cm-1 vs 0.001 cm-1, respectively). The finding of a larger proportional EROA in the COAPT study suggests more severe mitral regurgitation compared with the MITRA-FR study, thereby providing a potential explanation for the different outcomes in the 2 clinical trials. In contrast, the ratio of the mitral regurgitant volume to the end-diastolic volume in the COAPT study was similar to (but slightly lower than) that of the MITRA-FR study (0.15 vs 0.18, respectively), indicating that the proportional mitral regurgitant volume was comparable in the 2 clinical trials. This finding contradicts the conclusions of the EROA analysis.Conclusions and Relevance:The results of proportionality analyses based on EROA differ from those based on a volume analysis. This disparity casts doubt on the notion that an EROA analysis alone can explain the different results of the 2 randomized clinical trials.

Peripartum cardiomyopathy is an idiopathic reduction in left ventricular systolic function (ejection fraction <45%) toward the end of pregnancy or in the months after delivery. A multidisciplinary approach to management with shock team support is key to identifying and adequately treating patients with refractory heart failure and peripartum cardiomyopathy. (Level of Difficulty: Intermediate.)

A 62-year-old woman was evaluated for worsening dyspnoea. She had previously undergone coronary artery bypass surgery and had a history of a chronic loculated left pleural effusion (PE), which had previously been treated with repeated thoracentesis and talc pleurodesis. An echocardiogram was obtained, which was of suboptimal image quality, but it did demonstrate a small left ventricular (LV) chamber and normal LV ejection fraction. Pulsed wave Doppler of mitral valve inflow revealed significant respirophasic changes of the E-wave velocity (>25%), a short deceleration time (<160 ms), and an ‘L’ wave. There was also apparent ventricular interaction. Together these findings raised suspicion for constrictive pericarditis (CP), with the thought being that this condition resulted in dyspnoea due to impaired LV compliance, elevated filling pressures, and an inability to augment stroke volume with exertion. Cardiac magnetic resonance (CMR) imaging was performed to evaluate the possibility of CP. Cine CMR was performed at a 1.5Tesla MR scanner using an electrocardiogram gated steady-state free precession pulse sequence. As shown in a short-axis orientation of the heart, there is dyssynchronous contraction of the LV posterior wall but normal wall thickening (Videos 1–3). During systole, there is pronounced posterior motion of the LV posterior wall followed by flattening and compression of the same wall during diastole (Figures 1 and 2 and Supplementary material online, Figure S1). This phenomenon has been described in two-dimensional echocardiography as pseudodyskinesis and is commonly associated with liver or parenchymal lung disease. The abnormal systolic motion of the posterior wall has previously been attributed to diaphragmatic elevation resulting from intrathoracic or intrabdominal pathology. Pleural effusions, likewise, can have a significant impact on cardiac haemodynamics. A study of 47 subjects with large PE by Wang et al. demonstrated that drainage of the PE resulted in significant increases in LV enddiastolic volume index, LV ejection fraction, and stroke volume. In rare instances, large left-sided PEs have even been shown to result in cardiac tamponade. Video 1 Basal segment. Cardiac magnetic resonance steady-state free precession imaging of a short-axis view of the left ventricle which is surrounded by a large pleural effusion. During systole, there is pronounced outward motion of the posterior wall of the left ventricle; in diastole, there is inward motion of the same wall.

Secondary mitral regurgitation (MR) develops as a consequence of postinfarction remodelling of the ventricle or other causes of left ventricular (LV) dilatation and dysfunction. The presence of MR amplifies the poor prognosis of the failing ventricle, but it has not been established whether the adverse outcomes stem from the MR or whether the MR is simply a marker of progressive LV dysfunction. In this article, an attempt will be made to clarify the clinical impact of mitral surgery and transcatheter repair in patients with secondary MR. Observational studies indicate symptomatic improvement, but the results of randomised trials are mixed. Furthermore, neither mitral surgery nor transcatheter repair consistently leads to reversal of the adverse LV remodelling. There is, however, general agreement that these procedures do not have a salutary effect on survival. Certainly mitral surgery and transcatheter repair can substantially reduce the mitral regurgitant flow, but inconsistencies and uncertainties regarding clinical outcomes persist in the published literature. Some such problems could be resolved by utilisation of more accurate and reproducible imaging modalities in randomised studies of patients who are most likely to benefit from a reduction in the regurgitant volume—namely those with the most severe MR.

Secondary mitral regurgitation (MR) develops as a consequence of left ventricular (LV) dilatation and dysfunction, which complicates its evaluation and management. The goal of this article is to review the assessment of secondary MR with special emphasis on quantification and analysis of LV volume data. At the present time, the optimal method for making these measurements appears to be cardiac MRI. In severe MR (both primary and secondary), the regurgitant fraction (RF) exceeds 50%, and as a result, the LV end diastolic volume (EDV) is increased. In secondary MR, the ejection fraction is depressed (generally <40%) and despite an RF >50%, the regurgitant volume (RegV) rarely meets the current published criteria for severe MR (>60 mL). The ratio of the RegV to EDV, which is very low in secondary MR, reflects the effect of the RegV on the ventricle and it may be predictive of the fractional change in LV size that can be expected after correction of MR. Accurate measurement of the volumetric parameters is essential to proper management of patients with secondary MR.

A 57 year-old male with Klippel-Trenaunay Syndrome (KTS) presented with four weeks of dyspnea on exertion with unilateral right lower extremity swelling and pain.Vital signs were unremarkable though exam demonstrated elevated jugular venous distension and unilateral pitting edema in the right

BACKGROUND:Recurrent heart failure (HF) events are common in patients discharged after acute decompensated heart failure (ADHF). New patient-centered technologies are needed to aid in detecting HF decompensation. Transthoracic bioimpedance noninvasively measures pulmonary fluid retention.OBJECTIVE:The objectives of our study were to (1) determine whether transthoracic bioimpedance can be measured daily with a novel, noninvasive, wearable fluid accumulation vest (FAV) and transmitted using a mobile phone and (2) establish whether an automated algorithm analyzing daily thoracic bioimpedance values would predict recurrent HF events.METHODS:We prospectively enrolled patients admitted for ADHF. Participants were trained to use a FAV-mobile phone dyad and asked to transmit bioimpedance measurements for 45 consecutive days. We examined the performance of an algorithm analyzing changes in transthoracic bioimpedance as a predictor of HF events (HF readmission, diuretic uptitration) over a 75-day follow-up.RESULTS:We observed 64 HF events (18 HF readmissions and 46 diuretic uptitrations) in the 106 participants (67 years; 63.2%, 67/106, male; 48.1%, 51/106, with prior HF) who completed follow-up. History of HF was the only clinical or laboratory factor related to recurrent HF events (P=.04). Among study participants with sufficient FAV data (n=57), an algorithm analyzing thoracic bioimpedance showed 87% sensitivity (95% CI 82-92), 70% specificity (95% CI 68-72), and 72% accuracy (95% CI 70-74) for identifying recurrent HF events.CONCLUSIONS:Patients discharged after ADHF can measure and transmit daily transthoracic bioimpedance using a FAV-mobile phone dyad. Algorithms analyzing thoracic bioimpedance may help identify patients at risk for recurrent HF events after hospital discharge.

Background: Cardiac amyloidosis is seen in a third of patients undergoing transcatheter aortic valve replacement (TAVR) and might lead to poor outcomes in these patients. Case: 87 year old man, with history of severe aortic stenosis (AS, mean trans-aortic gradient 37mmHg, calculated valve area 0.6cm2) and paroxysmal atrial fibrillation, presented to the cardiology clinic with symptoms of biventricular failure. He underwent TAVR with CoreValve two months ago for recurrent heart failure with preserved ejection fraction (HFpEF) exacerbations. His hospital course was complicated by post-procedure cardiac arrest, dual-chamber atrioventricular permanent pacemaker placement for complete heart block and left anterior descending artery dissection. A repeat echocardiogram revealed normal left ventricular ejection fraction, severe concentric increase in left ventricular thickness, normal trans-aortic gradients for CoreValve and moderate aortic insufficiency (Figure 1). Longitudinal ventricular strain analysis by Spe...

Background The purpose of this investigation was to: (1) determine incidence and predictors of mitoxantrone‐induced early cardiotoxicity and (2) study left ventricular mechanics before and after receiving mitoxantrone. Method and Results We retrospectively analyzed 80 subjects diagnosed with acute myeloid leukemia ( AML ) who underwent chemotherapy with bolus high‐dose mitoxantrone. Echocardiographic measurements were taken at baseline and at a median interval of 55 days after receiving mitoxantrone. Thirty‐five (44%) of the patients developed clinically defined early cardiotoxicity, 29 (36%) of which developed heart failure. There was a significant decrease in the ejection fraction ( EF ) not only in the cardiotoxicity group (17.6 ± 14.8%, P < 0.001) but also in the noncardiotoxicity group (5.3 ± 8.4%, P < 0.001). Decrease in global longitudinal strain ( GLS ) (−3.7 ± 4.5, P < 0.001 vs. −2.4 ± 4.3, P = 0.01) and global circumferential strain ( GCS ) (−5.6 ± 9, P = 0.003 vs. −5.3 ± 8.7, P < 0.001) was significant in both the cardiotoxicity and noncardiotoxicity group, respectively. A multivariate model including baseline left ventricular end‐systolic diameter, baseline pre‐E/A ratio, and baseline pre‐E/e′ ratio was found to be the best‐fitted model for prediction of mitoxantrone‐induced early clinical cardiotoxicity. Conclusion High‐dose mitoxantrone therapy is associated with an excellent remission rate but with a significantly increased risk of clinical and subclinical early cardiotoxicity and heart failure. Mitoxantrone‐induced systolic dysfunction is evident from reduction in EF , increase in Tei index, and significant reduction in GLS and GCS . Baseline impaired ventricular relaxation evident from higher E/e′ ratio and lower E/A ratio independently predicts increased risk of mitoxantrone‐induced early cardiotoxicity.

Heart failure is a common complication seen in patients with Type 2 Diabetes Mellitus (T2DM) and is associated with significant morbidity and mortality in these patients [ 1 Dei Cas A. Khan S.S. Butler J. et al. Impact of diabetes on epidemiology, treatment, and outcomes of patients with heart failure. JACC Heart Fail. 2015; 3: 136-145 Crossref PubMed Scopus (223) Google Scholar , 2 Nichols G.A. Gullion C.M. Koro C.E. et al. The incidence of congestive heart failure in type 2 diabetes: an update. Diabetes Care. 2004; 27: 1879-1884 Crossref PubMed Scopus (589) Google Scholar ]. Although improved glycemic control has been shown to reduce microvascular complications in patients with T2DM [ [3] McMurray J.J. Gerstein H.C. Holman R.R. Pfeffer M.A. Heart failure: a cardiovascular outcome in diabetes that can no longer be ignored. Lancet Diab Endocrinol. 2014; 2: 843-851 Abstract Full Text Full Text PDF PubMed Scopus (258) Google Scholar ], it is uncertain whether any particular hypoglycemic regimen can be considered safe from a cardiovascular standpoint. Many novel glucose-lowering strategies have recently been tested in randomized trials of patients with type 2 diabetes to establish whether use of these newer therapeutic agents translates into reductions in adverse outcomes such as risk of developing of heart failure. Dipeptidyl Peptidase-4 (DDP-4) inhibitors are a new class of oral hypoglycemic drugs used in patients with T2DM. They act by blocking the degradation of glucagon like peptide-1 (GLP-1), gastric inhibitory peptide (GIP) and a number of other peptides, thereby raising levels of these ‘incretins’ (GLP-1/GIP), which subsequently enhance the insulinotropic effects of glucose [ [4] Zhong J. Goud A. Rajagopalan S. Glycemia lowering and risk for heart failure: recent evidence from studies of dipeptidyl peptidase inhibition. Circ Heart Fail. 2015; 8: 819-825 Crossref PubMed Scopus (12) Google Scholar ]. Recent clinical trials have raised concerns for an increased risk of heart failure with DDP-4 inhibitor use in diabetic patients [ [5] Scirica B.M. Bhatt D.L. Braunwald E. et al. SAVOR-TIMI 53 steering committee and investigators. Saxagliptin and cardiovascular outcomes in patients with type 2 diabetes mellitus. N Engl J Med. 2013; 369: 1317-1326 Crossref PubMed Scopus (2731) Google Scholar ], but concrete data regarding their safety profile is lacking. The purpose of this meta-analysis was to evaluate the absolute risk of heart failure with use of DDP-4 inhibitors in patients with T2DM, using data from the most recent randomized controlled trials (RCTs).

OBJECTIVE:Mitral regurgitation (MR) is generally characterised as exhibiting a 'low impedance leak into the left atrium'. This notion is widely accepted without measured impedance data. The aim of this study was to define the impedance to retrograde and forward blood flow and to examine hydraulic (pressure-volume) and mechanical (stress-shortening) function in chronic severe MR.METHODS:A mathematical model of a double outlet ventricle was developed and the ratio of retrograde to forward impedance was plotted over a wide range of regurgitant fraction (RF). The model predicts that an impedance ratio >1 indicates that the impedance to retrograde flow exceeds that of forward flow. Left ventricular (LV) systolic pressure/flow rate was used as an index of impedance (mm Hg/mL/s). Data from 10 patients with severe MR were used to assess the clinical applicability of the model. All patients had degenerative valve disease with partial flail leaflet, an RF >50% and an ejection fraction (EF) >0.60. There were seven males and three females, aged 59±10. LV volumes as well as retrograde and forward flow rates were determined with echocardiographic and Doppler techniques.RESULTS:The model indicates that the impedance ratio is >1 when the RF ranges from zero to 57%. Clinical data: end-diastolic volume=184±47 mL; EF=0.63±3%; RF=53±4%. Values for retrograde and forward impedance were 0.77±0.17 and 0.63±0.12 (p=0.003); the impedance ratio was 1.22±0.19. Total impedance to LV emptying was low (0.35±0.06). The ratio of systolic wall stress to EF (580±81 g/cm2) was normal. Data are mean±SD.CONCLUSIONS:The model, supported by clinical data, indicates that the impedance to retrograde flow exceeds the impedance to forward flow in chronic severe MR. These findings refute the notion of a low impedance leak into the left atrium. The double outlet of an enlarged ventricle provides a mechanism for low total impedance to ejection in the presence of a normal stress-shortening relation.

Introduction: Patients and health systems are focused on reducing readmissions for patients with acute decompensated heart failure (ADHF). Readmission after hospitalization is often secondary to HF decompensation, but it remains challenging to identify patients at-risk. Bioimpedance is a validated marker of thoracic fluid accumulation. We examined whether changes in bioimpedance, measured using a Fluid Accumulation Vest (FAV), predicted subsequent HF decompensation in patients discharged after ADHF. Methods: Participants included 83 patients hospitalized for ADHF. Subjects were trained on the use of a FAV-smartphone dyad to obtain and transmit a 5-minute bioimpedance measurement once daily for 45-days after discharge.(see Figure) The outcome of interest, HF-related readmission was assessed using participant report and medical records. Sensitivity, specificity, negative and positive predictive values were calculated to describe the efficacy of the bioimpedance alert algorithm as a predictor of HF readmission. Results: Subject characteristics: mean age 68 ± 11 years, 36% female, 92% white, mean ejection fraction of 44 ± 19%. 49 participants completed the 45-day follow-up and had sufficient, daily FAV data for analysis. Our main outcome of HF-related rehospitalization occurred in 8% of patients during follow-up. The decompensation detection algorithm demonstrated a sensitivity of 75%, specificity of 47%, positive/negative predictive values of 11% and 96%, respectively. Conclusions: The preliminary results of this ongoing study suggest that HF readmissions may be predicted with modest sensitivity by our current decompensation detection algorithm. Further refinement of our transthoracic bioimpedance system may offer possibilities for reducing HF readmissions by enabling identification and treatment of outpatients at risk for readmission.

BackgroundWhile heart failure with preserved ejection fraction (HFpEF) is primarily a disease of old age, risk factors that contribute to HFpEF are not limited to older patients. The objectives of this population-based observational study were to describe the clinical epidemiology of HFpEF in younger (<65years) as compared with older (≥65years) patients hospitalized with acute decompensated heart failure.Methods and resultsWe reviewed the medical records of residents of central Massachusetts hospitalized with HFpEF at all 11 greater Worcester (MA) medical centers during the 5 study years of 1995, 2000, 2002, 2004, and 2006. Among the 2398 patients hospitalized with confirmed HFpEF, 357 (14.9%) were <65years old. Younger patients were more likely to be male, non-Caucasian, obese, and to have a history of diabetes and chronic kidney disease than older patients with HFpEF. Younger patients hospitalized with HFpEF were less likely to have received commonly prescribed cardiac medications, had a longer hospital stay, and experienced significantly lower post-discharge death rates than older hospitalized patients.ConclusionWhile HFpEF is predominantly a disease of old age, data from longitudinal studies remain needed to identify risk factors in younger individuals that may predispose them to the development of HFpEF.