Here's the rub: A case of constrictive pericarditis in an adult with cystic fibrosis.

We present a rare coexistence of constrictive pericarditis in a patient with cystic fibrosis. Careful attention to cardiac friction rub auscultated on initial examination prompted echocardiography revealing constrictive pericarditis further confirmed by cardiac magnetic resonance imaging that allowed for dedicated treatment in addition to management of his concurrent respiratory infection.

Percutaneous Closure of Paravalvular Leak from a Rocking Mitral Valve in a 74-Year-Old Man at High Surgical Risk.

Dehiscence of a prosthetic heart valve or excessive rocking during the cardiac cycle is thought to preclude percutaneous paravalvular leak closure. However, surgical repair of paravalvular leak is associated with recurrent dehiscence and poor outcomes. We present the case of a symptomatic 74-year-old man in whom we performed percutaneous anchoring, involving multiple plugs and multimodal imaging, to stabilize a rocking mitral valve and close a substantial paravalvular leak caused by dehiscence. To our knowledge, using this technique to correct both conditions is novel.

Imaging for Predicting, Detecting, and Managing Complications After Transcatheter Aortic Valve Replacement.

The management of patients with valvular heart disease is increasingly reliant on multimodal cardiac imaging. In patients with severe aortic stenosis considered for transcatheter aortic valve replacement, careful pre-procedural planning with multimodal imaging is necessary to avoid and prevent complications during the procedure. During or immediately after the procedure, rapid echocardiographic assessment is important to assess the new valve's function and manage major complications. Echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging all share important roles in the post-procedural evaluation of abnormal transcatheter valve function. This review discusses the use of multimodal imaging for predicting, detecting, and managing complications after TAVR.

Role of Multimodal Cardiac Imaging in Diagnosing a Primary Intimal Sarcoma of the Left Atrial Appendage.

Primary cardiac tumors are rare, and most are benign. Intimal sarcomas are among the rarest of the malignant cardiac tumors; they are aggressive and associated with a poor prognosis. Whereas transesophageal echocardiography has been instrumental in evaluating cardiac masses, other imaging methods, such as cardiac magnetic resonance, have proved invaluable in accurately characterizing these masses. We present the case of a 49-year-old woman in whom we diagnosed a primary intimal sarcoma of the left atrial appendage, and we discuss the importance of multimodal imaging in the evaluation and diagnosis of cardiac masses.

Pulmonary Venous Waveforms Predict Rehospitalization and Mortality After Percutaneous Mitral Valve Repair.

OBJECTIVES: In this study, the authors hypothesized that intraprocedural improvement of pulmonary venous (PV) waveforms are predictive of improved outcomes. In this report, they analyzed intraprocedural invasive and echocardiographic changes with respect to rehospitalization and mortality. BACKGROUND: The effects of hemodynamic changes during percutaneous mitral valve repair (PMVR) with MitraClip (Abbott Vascular, Santa Clara, California) are incompletely characterized. METHODS: The authors retrospectively reviewed records and intraprocedural transesophageal echocardiograms of 115 consecutive patients (age 76 ± 12 years) who underwent PMVR for mitral regurgitation (MR) from May 2013 to January 2017 at Emory University Hospital. They assessed intraprocedural PV waveforms for improvement in morphology, measured change in MR grade by semiquantitative methods, evaluated invasive changes in left atrial pressure (LAP) and V-wave, and compared with 30-day and 1-year rehospitalization and all-cause mortality. RESULTS: Ninety-three cases (80%) had PV waveforms before and after clip placement sufficient for analysis, of which 67 (73%) demonstrated intraprocedural improvement in PV morphology and 25 (27%) did not. At 24 months, 57 (85%) of those with PV improvement were living, compared with only 10 (40%) of those without improvement. Proportional hazards models demonstrated a significant survival advantage in those with PV improvement (hazard ratio [HR]: 0.28, 95% confidence interval [CI]: 0.08 to 0.93, p = 0.038). By multivariable analysis, PV improvement predicted reduced 1-year cardiac rehospitalization (odds ratio [OR]: 0.18, p = 0.044). Intraprocedural assessment of MR grade and invasive hemodynamics did not consistently predict mortality and rehospitalization. CONCLUSIONS: PV waveforms are important markers of procedural success after PMVR. Our data show intraprocedural PV waveforms may predict rehospitalization and mortality after PMVR. A larger, multicenter cohort will be important to clarify this relationship.

Radioprotective strategies for interventional echocardiographers during structural heart interventions.

OBJECTIVE: We investigated radioprotective strategies for the interventional echocardiographer (IE) during structural heart interventions in comparison with the interventional cardiologist (IC). BACKGROUND: Structural heart interventions are expanding in complexity with increased reliance on IE. Recent reports have demonstrated concerning exposure and higher radiation to the IE. METHODS: We monitored 32 structural interventions - 19 transcatheter aortic valve replacements (TAVR), 6 transcatheter mitral valve repairs, 5 paravalvular leak closures, and 2 atrial septal defect closures. Seventeen utilized transesophageal echocardiography (TEE) while 15 used transthoracic echocardiography (TTE). Members of the IC and IE teams wore multiple dosimeters on different sites of the body to measure radiation dose to the total body, lens of the eye, and hand. During each case, IE utilized dedicated radiation shielding. RESULTS: Mean doses were higher for the primary IC than the primary IE: IC#1-99, 222, 378; IE#1-48, 52, 416 (body, lens, and hand doses in µSv). IE radioprotective strategies were able to reduce body and lens doses compared to IC during both TTE and TEE-guided procedures. Hand equivalent dose remained higher for the IE driven by exposure during TEE-guided procedures (IC#1 294 vs. IE#1 676 µSv). In a subgroup using radioprotective drapes during TTE-guided TAVR, IC dose was reduced without effect on the IE. CONCLUSIONS: Radiation exposure during structural heart interventions is concerning. With dedicated shielding, IE received lower doses to the body and lens than IC. Further optimization of structural suite design and shielding is needed.

Atrial Fibrillation Predictors on Mobile Cardiac Telemetry in Cryptogenic Ischemic Stroke.

BACKGROUND AND PURPOSE: The objective of our study was to evaluate magnetic resonance imaging (MRI) and echocardiographic characteristics that would identify patients with cryptogenic ischemic stroke (IS) and transient ischemic attack (TIA) who subsequently developed paroxysmal atrial fibrillation (PAF) on mobile cardiac outpatient telemetry (MCOT). METHODS: All patients with cryptogenic IS or TIA seen at the Emory University Hospital and Emory University Hospital Midtown from January 1, 2009, to June 30, 2013, who underwent MCOT were included in this analysis. Location (cortical, high subcortical, or neither) of current and prior strokes on MRI and left atrial (LA) functional and anatomical echocardiographic parameters were evaluated to determine their association with subsequent detection of PAF. RESULTS: Of 132 patients, 17 (13%) had evidence of newly diagnosed PAF on MCOT (mean duration of monitoring = 25 days). The presence (vs absence) of ≥1 cortical infarct on baseline MRI was a significant predictor of identifying PAF (odds ratio: 5.2, 95% confidence interval: 1.3-19; P = .01). On baseline echocardiography, patients who had PAF (vs non-PAF) had significantly higher mean LA diameters (4.2 vs 3.7 cm, P = .03) and lower tissue Doppler velocity (a'; 5.5 vs 13.5 cm/s, P = .03). In receiver operating characteristic analysis, the ratio of LA volume index to the septal Doppler velocity (LAVI/a') of >4.6 was associated with a higher likelihood of PAF. Combining MRI with echocardiographic variables did not improve the predictive ability beyond echocardiography alone. CONCLUSION: Although the presence of cortical-based infarcts on MRI in patients with cryptogenic IS or TIA increases the likelihood of detecting PAF on MCOT, LA functional and anatomic parameters alone best predicted which patients subsequently had PAF.

Internal Jugular Venous Pseudoaneurysm in a Patient with Heart Failure and Severe Tricuspid Regurgitation.

The differential diagnosis of a lateral neck mass includes a number of possible etiologies. While jugular venous aneurysms and pseudoaneurysms are rare entities, they should be considered in the differential diagnosis of a pulsatile lateral neck mass. We present a case of an idiopathic jugular venous pseudoaneurysm and its association with worsening tricuspid regurgitation in a patient with heart failure with preserved ejection fraction.

Transapical closure of a pseudoaneurysm of the mitral-aortic intervalvular fibrosa.

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare but serious sequela of endocarditis or valve replacement surgery. Because open-heart surgery is a high-risk treatment option, alternative methods are sought. We present the case of a 77-year-old man with a noninfected mechanical mitral valve whose pseudoaneurysm was repaired by introducing an occluder device into the defect by a transapical approach. Upon follow-up imaging, the defect was successfully closed. We conclude that percutaneous closure of pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a viable alternative to surgery and that a transapical approach is an appropriate method of access.

The prognostic value of standardized reference values for speckle-tracking global longitudinal strain in hypertrophic cardiomyopathy.

Speckle-tracking left ventricular global longitudinal strain (GLS) assessment may provide substantial prognostic information for hypertrophic cardiomyopathy (HCM) patients. Reference values for GLS have been recently published. We aimed to evaluate the prognostic value of standardized reference values for GLS in HCM patients. An analysis of HCM clinic patients who underwent GLS was performed. GLS was defined as normal (more negative or equal to -16%) and abnormal (less negative than -16%) based on recently published reference values. Patients were followed for a composite of events including heart failure hospitalization, sustained ventricular arrhythmia, and all-cause death. The power of GLS to predict outcomes was assessed relative to traditional clinical and echocardiographic variables present in HCM. 79 HCM patients were followed for a median of 22 months (interquartile range 9-30 months) after imaging. During follow-up, 15 patients (19%) met the primary outcome. Abnormal GLS was the only echocardiographic variable independently predictive of the primary outcome [multivariate Hazard ratio 5.05 (95% confidence interval 1.09-23.4, p = 0.038)]. When combined with traditional clinical variables, abnormal GLS remained independently predictive of the primary outcome [multivariate Hazard ratio 5.31 (95 % confidence interval 1.18-24, p = 0.030)]. In a model including the strongest clinical and echocardiographic predictors of the primary outcome, abnormal GLS demonstrated significant incremental benefit for risk stratification [net reclassification improvement 0.75 (95 % confidence interval 0.21-1.23, p < 0.0001)]. Abnormal GLS is an independent predictor of adverse outcomes in HCM patients. Standardized use of GLS may provide significant incremental value over traditional variables for risk stratification.

Bad company: supracristal VSD presenting with ruptured sinus of valsalva aneurysm. a case presentation with echocardiographic depiction and an analysis of contemporary literature.

Supracristal ventricular septal defect (SCVSD), a defect of the infundibular portion of the interventricular septum just below the right aortic cusp, occurs more frequently in Eastern Asian populations. SCVSD may be complicated by right sinus of Valsalva aneurysm (SoVA). We present the case of a 26-year-old male of Korean descent with a history of a childhood murmur who was referred to our institution for progressive heart failure symptoms. He was diagnosed with SCVSD and ruptured right SoVA based on history, physical exam, and echocardiography including three-dimensional transesophageal echocardiography with reconstructed surgical views. The patient underwent SCVSD closure, SoVA excision, and valve-sparing aortic root replacement. We reviewed the echocardiography literature regarding SCVSD and SoVA, and analyzed contemporary literature of SoVA and its relationship with SCVSD. We conclude that a higher prevalence of ruptured SoVA in Eastern Asians is likely related to a higher prevalence of underlying SCVSD in this population.

The role of cardiovascular magnetic resonance in stratifying paravalvular leak severity after transcatheter aortic valve replacement: an observational outcome study.

BACKGROUND: Significant paravalvular leak (PVL) after transcatheter aortic valve replacement (TAVR) confers a worse prognosis. Symptoms related to significant PVL may be difficult to differentiate from those related to other causes of heart failure. Cardiovascular magnetic resonance (CMR) directly quantifies valvular regurgitation, but has not been extensively studied in symptomatic post-TAVR patients. METHODS: CMR was compared to qualitative (QE) and semi-quantitative echocardiography (SQE) for classifying PVL and prognostic value at one year post-imaging in 23 symptomatic post-TAVR patients. The primary outcome was a composite of all-cause death, heart failure hospitalization, and intractable symptoms necessitating repeat invasive therapy; the secondary outcome was a composite of all-cause death and heart failure hospitalization. The difference in event-free survival according to greater than mild PVL versus mild or less PVL by QE, SQE, and CMR were evaluated by Kaplan-Meier survival analysis. RESULTS: Compared to QE, CMR reclassified PVL severity in 48% of patients, with most patients (31%) reclassified to at least one grade higher. Compared to SQE, CMR reclassified PVL severity in 57% of patients, all being reclassified to at least one grade lower; SQE overestimated PVL severity (mean grade 2.5 versus 1.7, p=0.001). The primary and secondary outcomes occurred in 48% and 35% of patients, respectively. Greater than mild PVL by CMR was associated with reduced event-free survival for the primary outcome (p<0.0001), however greater than mild PVL by QE and SQE were not (p=0.83 and p=0.068). Greater than mild PVL by CMR was associated with reduced event-free survival for the secondary outcome, as well (p=0.012). CONCLUSION: In symptomatic post-TAVR patients, CMR commonly reclassifies PVL grade compared with QE and SQE. CMR provides superior prognostic value compared to QE and SQE, as patients with greater than mild PVL by CMR (RF>20%) had a higher incidence of adverse events.

Cardiac plasmacytoma: a rare clinical entity.

Primary malignant cardiac tumors are rare. Among these tumors, cardiac plasmacytoma is extremely rare and is the subject of few case reports. We present the case of a 73-year-old man who had isolated cardiac plasmacytoma 26 years after successful treatment of an axillary plasmacytoma. Multiple imaging methods-including echocardiography, cardiac magnetic resonance, and positron-emission tomography/computed tomography-were valuable and complementary to each other in this patient's diagnosis and optimal management. His case illustrates the use of these techniques in the successful diagnosis and treatment of a rare clinical entity, cardiac plasmacytoma.

Multimodality imaging of aortitis.

Multimodality imaging of aortitis is useful for identification of acute and chronic mural changes due to inflammation, edema, and fibrosis, as well as characterization of structural luminal changes including aneurysm and stenosis or occlusion. Identification of related complications such as dissection, hematoma, ulceration, rupture, and thrombosis is also important. Imaging is often vital for obtaining specific diagnoses (i.e., Takayasu arteritis) or is used adjunctively in atypical cases (i.e., giant cell arteritis). The extent of disease is established at baseline, with associated therapeutic and prognostic implications. Imaging of aortitis may be useful for screening, routine follow up, and evaluation of treatment response in certain clinical settings. Localization of disease activity and structural abnormality is useful for guiding biopsy or surgical revascularization or repair. In this review, we discuss the available imaging modalities for diagnosis and management of the spectrum of aortitis disorders that cardiovascular physicians should be familiar with for facilitating optimal patient care.

Partial papillary muscle rupture: a cause of acute mitral regurgitation.

A 56-year-old woman presented to the cardiology clinic with a 7-day history of dyspnea and chest pressure. An echocardiogram showed a flail posterior mitral valve leaflet, and subsequent left heart catheterization showed complete occlusion of the first obtuse marginal coronary artery. Further investigation demonstrated the culprit lesion to be ischemic partial rupture of the posteromedial papillary muscle. Timely recognition of this condition allowed for an optimal clinical outcome. In this Cardiology Grand Rounds, the authors further describe the above presentation of ischemic partial papillary muscle rupture and provide a brief review of the pathophysiology, diagnosis and treatment of this condition.

Cardiomyopathy with inverted tako-tsubo pattern in the setting of subarachnoid hemorrhage: a series of four cases.

BACKGROUND: Severe subarachnoid hemorrhage may be associated with regional wall motion abnormalities (RWMA) in the absence of epicardial coronary occlusion. The RWMA extends beyond the distribution of a single coronary artery and may present in a typical tako-tsubo cardiomyopathy pattern. Other variants have also been recognized, including an inverted tako-tsubo pattern of severe basal hypokinesis that spares the apex. The mechanism of this cardiomyopathy is not well understood but likely involves catecholamine excess. While classic tako-tsubo cardiomyopathy from emotional stress carries a favorable prognosis, cardiac dysfunction from subarachnoid hemorrhage is a marker of overall poor prognosis. METHODS: We collected cases over a period of 4 years at a large teaching hospital. The cases represent cardiac dysfunction in the setting of subarachnoid hemorrhage in the unusual distribution of basal hypokinesis with relative sparing of the apex (inverted tako-tsubo pattern). RESULTS: A total of four cases were identified. All cases were female between the ages 43-67 years and had echocardiographic evidence of basal hypokinesis after suffering from an intracranial hemorrhage. CONCLUSIONS: The typical and inverted patterns may represent a spectrum within the same disease process or distinct clinical entities with dramatically different prognostic implications. Larger studies comparing the two presentations will help elucidate this further.