Ruptured Sinus of Valsalva Aneurysm: Three Case Reports and Literature Review.

Sinus of Valsalva aneurysm rupture (SOVAR) into the right cardiac chambers is an uncommon complication with unusual presentation, high morbidity and mortality, and unique hemodynamics as well as cardiac imaging findings. Here, we present three SOVAR cases (two with rupture into the right atrium and one with rupture into the right ventricle) that were initially confused for ventricular septal defects and describe their initial presentation, cardiac imaging studies, invasive hemodynamics, as well as treatment options. Some of the unique findings of SOVAR patients include an acute presentation, often with hemodynamic decompensation, the presence of a continuous murmur on examination, and also hemodynamics that include wide pulse pressure and right heart volume overload.

A 65-Year-Old Male With Classic Cardiac Sarcoidosis: Case Report and Review of the Literature.

Sarcoidosis is a systemic disease characterized by the formation of non-necrotizing granulomas, primarily involving the lungs and other organs such as the heart. The diagnosis of cardiac sarcoidosis can be difficult. The last set of diagnostic guidelines for diagnosis and treatment of cardiac sarcoidosis was published in 2019 by the Japanese Circulation Society (JCS). We describe a case of classic cardiac sarcoidosis and review the literature on clinical presentation, imaging, and management.

Evaluation of Clinical Predictors for Major Outcomes in Patients Hospitalized With COVID-19: The Potential Role of the Rothman Index.

Introduction The Rothman Index (RI, PeraHealth, Inc. Charlotte, NC, USA) is a predictive model intended to provide continuous monitoring of a patient's clinical status. There is limited data to support its use in the risk stratification of patients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). We hypothesized that low admission RI scores would correlate with higher rates of adverse outcomes in patients hospitalized for coronavirus disease 2019 (COVID-19). Methods Medical records of adult patients admitted to a single 1,200-bed tertiary academic center were retrospectively reviewed for demographic data, baseline characteristics, RI scores, admission to intensive care unit (ICU), need for mechanical ventilation, and inpatient mortality. Statistical analyses were performed using STATA statistical software, version 17 (Stata Corp LLC, College Station, TX, USA). Continuous variables were analyzed using the Mann-Whitney test, and categorical variables were analyzed using Fisher's exact test. Both univariate and multivariate analyses were performed. A p-value <0.05 was considered statistically significant. Results Median admission RI score for the entire cohort was 63.0 (IQR 45.0 - 77.1). The cohort was divided by admission RI into a low-risk group (RI ≥70; n=70) and a high-risk group (RI <70; n=107). Compared to patients with low-risk RI, patients with high-risk RI had higher mortality (95.2%, 95% CI: 85.8 - 105 vs 4.8%, 95% CI: -5 - 14.2, p < 0.01), were more likely to require ICU admission (90.2%, 95% CI: 81.9 - 98.5 vs 9.8%, 95% CI: 1.5 - 18.1, p < 0.01) and mechanical ventilation (89.7%, 95% CI: 78.3 - 101 vs 10.3%, 95% CI: -1 - 21.7, p < 0.01), and had a longer median hospital length of stay (12 days, 95% CI: 9 - 14 vs 5 days, 95% CI: 4 - 7, p < 0.01). Conclusions High-risk RI was associated with increased admission to the ICU, mechanical ventilation, and mortality. These results suggest that it may be used as a tool to aid provider judgment in the setting of COVID-19.

Repeated extracorporeal membrane oxygenation for support of an Adult with Congenital Heart Disease and reperfusion pulmonary oedema.

We present the case of 28-year-old woman with a history of complex congenital cardiac surgery who developed cardiovascular collapse with reperfusion pulmonary oedema and right ventricular failure after surgical replacement of a severely stenosed right ventricle to pulmonary artery conduit. She required two separate episodes of support with extracorporeal membrane oxygenation and is alive and well 6 months after her initial cardiorespiratory crisis. We believe that consideration of a second period of support with extracorporeal membrane oxygenation is appropriate for select adults with CHD, provided they have a potentially reversible cause of postoperative cardiorespiratory collapse.Our case provides several important lessons: (1) adults with CHD with severe postcardiotomy cardiorespiratory failure may potentially be salvaged even if they require multiple runs of extracorporeal membrane oxygenation; (2) adults with CHD with severe postcardiotomy respiratory failure with adequate cardiac function may potentially be salvaged with veno-venous extracorporeal membrane oxygenation; and (3) patients supported with extracorporeal membrane oxygenation will benefit from care from a skilled multidisciplinary team who are able to focus on the support of the function of the organs of the patient whilst providing nutrition and mobilisation.

Left Ventricular Strain Is Associated With Myocardial Recovery Following ST-Elevation Myocardial Infarction, a Prospective Longitudinal CMR Study.

Background: Infarct size following ST-elevation myocardial infarction (STEMI) is an important determinate of left ventricular (LV) dysfunction and cardiovascular morbidity and mortality. Cardiac magnetic resonance feature tracking (CMR-FT) is a technique that allows for the assessment of myocardial function via quantification of longitudinal, radial, and circumferential strain. We investigated the association between CMR-FT-derived myocardial global strain and myocardial recovery. Methods: A prospective study on patients presenting with STEMI treated with primary percutaneous coronary intervention (PCI) was conducted. CMR imaging was obtained at two interval time points, the baseline within 2 weeks of hospital discharge and follow-up at 6 months. Strain analysis was performed via FT-CMR, and recovery was quantified by the area of late gadolinium enhancement (LGE). Results: A total of n = 14 patients met inclusion and exclusion criteria and were analyzed. There was a significant reduction in the infarct size, as measured by LGE mass percentage of the left ventricular muscle mass, between the initial and follow-up CMR (19.7%, IQR 12.2-23.9 vs. 17.1%, IQR 8.3-22.5, p = 0.04). Initial strain parameters were inversely correlated with the initial edema mass and the decrease in LGE mass between the initial and follow-up CMR. All LV global strains had high accuracy for the prediction of a reduction in LGE mass by 50% or more. Conclusions: LV global strains measured after primary PCI can predict the extent of myocardial recovery.

Takayasu's arteritis presenting as acute myocardial infarction: case series and review of literature.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu's arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu's arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu's arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu's arteritis within the paediatric community.

Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors.

OBJECTIVE: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation. DESIGN: Retrospective analysis. SETTING: Single center, quaternary care children's hospital. PATIENTS: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016. INTERVENTIONS: None. OUTCOME MEASURES: The primary outcome was early postoperative atrial fibrillation. Secondary outcomes included preoperative comorbidities, preoperative echocardiographic findings, operative details, and postoperative morbidities, such length of stay, reintubation, stroke, and death. RESULTS: The incidence of early postoperative atrial fibrillation was 21%. Those who developed early postoperative atrial fibrillation were older (50 years vs 38 years, P =< .001), had a history of atrial fibrillation prior to surgery, had preoperative pulmonary hypertension, and had longer cardiopulmonary bypass times (103 minutes vs 84 minutes, P = .025) when compared to those who did not develop postoperative atrial fibrillation. Multivariate analysis identified age greater than 60, preoperative pulmonary hypertension, mitral valve intervention, and the need for postoperative inotropic support as being independent predictors of postoperative atrial fibrillation. Those who developed postoperative atrial fibrillation remained in the hospital longer (9 days vs 7 days, P =< .001). CONCLUSIONS: Atrial fibrillation is a common complication following cardiac surgery in adults with congenital heart disease. Age, preoperative comorbidities, type of surgical intervention, and the need for perioperative inotropic infusions may predict the risk of atrial fibrillation in this unique patient population.

A 61-Year-Old Man With Shortness of Breath, Ascites, and Lower Extremity Edema.

A 61-year-old man presented with an 18-month history of progressive shortness of breath on exertion, fatigue, worsening bilateral lower extremity edema, abdominal swelling, and increased assistance with activities of daily living. Pertinent past medical history included right-sided pneumonia secondary to Streptococcus pneumoniae that was complicated by empyema, requiring right-sided video-assisted thoracoscopic surgery with decortication 2 years earlier. He had a negative cardiac history, no recent travel in the last 3 years, and no known exposure to tuberculosis. His medications included aspirin and daily furosemide. His symptoms appeared to be refractory to diuretic therapy. Previous workup 6 months earlier included an echocardiography (ECHO) showing enlarged left and right atria with a normal ejection fraction, and a catheterization of the left side of the heart with reported normal left ventricular function and unobstructed coronary arteries.

Effectiveness of late gadolinium enhancement to improve outcomes prediction in patients referred for cardiovascular magnetic resonance after echocardiography.

BACKGROUND: Echocardiography (echo) is a first line test to assess cardiac structure and function. It is not known if cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) ordered during routine clinical practice in selected patients can add additional prognostic information after routine echo. We assessed whether CMR improves outcomes prediction after contemporaneous echo, which may have implications for efforts to optimize processes of care, assess effectiveness, and allocate limited health care resources. METHODS AND RESULTS: We prospectively enrolled 1044 consecutive patients referred for CMR. There were 38 deaths and 3 cardiac transplants over a median follow-up of 1.0 years (IQR 0.4-1.5). We first reproduced previous survival curve strata (presence of LGE and ejection fraction (EF) < 50%) for transplant free survival, to support generalizability of any findings. Then, in a subset (n = 444) with contemporaneous echo (median 3 days apart, IQR 1-9), EF by echo (assessed visually) or CMR were modestly correlated (R(2) = 0.66, p < 0.001), and 30 deaths and 3 transplants occurred over a median follow-up of 0.83 years (IQR 0.29-1.40). CMR EF predicted mortality better than echo EF in univariable Cox models (Integrated Discrimination Improvement (IDI) 0.018, 95% CI 0.008-0.034; Net Reclassification Improvement (NRI) 0.51, 95% CI 0.11-0.85). Finally, LGE further improved prediction beyond EF as determined by hazard ratios, NRI, and IDI in all Cox models predicting mortality or transplant free survival, adjusting for age, gender, wall motion, and EF. CONCLUSIONS: Among those referred for CMR after echocardiography, CMR with LGE further improves risk stratification of individuals at risk for death or death/cardiac transplant.

Perioperative management of a patient with recently placed drug-eluting stents requiring urgent spinal surgery.

Patients receiving drug-eluting coronary stents (DES) require antiplatelet therapy for at least 12 months to prevent stent thrombosis (ST), a potentially calamitous event. Since interruption of antiplatelet therapy is the greatest risk factor for ST, it is imperative that the decision to discontinue these agents be based on an accurate assessment of the patient's risk for bleeding complications. Individuals who are regarded as being at a high risk are those undergoing intracranial, spinal or intraocular surgeries. These patients require alternative agents during the perioperative period to minimize both their risk of perioperative thrombosis and intraoperative hemorrhage. We report the case of a woman who required spinal surgery 3 months after she underwent placement of two drug-eluting stents. The patient's clopidogrel was stopped 5 days prior to surgery and an infusion of eptifibatide was used to "bridge" antiplatelet therapy during the perioperative period. Postoperatively, anticoagulation therapy was reinstituted using aspirin with clopidogrel. This case serves as a successful example of bridging therapy using a short acting and gycoprotein (GP) IIb/IIIa inhibitor as a means of maintaining antiplatelet therapy during the perioperative period to minimize the risk of stent thrombosis and the risk of intraoperative bleeding.

Preoperative management in patients with single-ventricle physiology.

OBJECTIVE: Advancements in the preoperative management of patients with single-ventricle physiology continue to evolve. Previous reports have questioned the benefit of using inhaled nitrogen in single-ventricle patients, suggesting that this therapeutic modality may not provide adequate systemic cardiac output. The objective of this study was to review our institutional experience managing preoperative patients with single-ventricle physiology using a combination of afterload reduction and inhaled hypoxemic therapy. DESIGN, SETTING, AND PATIENTS: This is a retrospective review of 49 consecutive single-ventricle patients admitted preoperatively between July 2004 and January 2009, to the cardiac intensive care unit at Children's Hospital of Pittsburgh who underwent single-ventricle palliation, and treated preoperatively with milrinone and inhaled nitrogen. Therapeutic interventions and indirect indicators of cardiac output were collected on day of admission (time 0) and compared with those collected on the morning of surgery (time 1); data included clinical assessment, hemodynamic measurements, and laboratory values. RESULTS: When comparing time 0 to time 1, there was a statistically significant decrease in lactate (from 2.2 to 1.8 mEq/L [P < 0.001]) and an increase in pH (from 7.36 to 7.41 [P < 0.001]), serum bicarbonate (from 24.16 to 27.55 mmol/L [P < 0.001]) and arterial PaO2 (from 38.10 to 41.82 mm Hg [P = 0.027]). Preoperatively, there were no deaths, and only two patients had an evidence of multiorgan dysfunction on day of surgery (time 1). CONCLUSION: Our results suggest that a combination of afterload reduction and hypoxemic therapy was able to maintain an appropriate distribution of the cardiac output in the majority of preoperative patients with single-ventricle physiology. An adequate balance of systemic and pulmonary blood flow was successfully achieved with an increase in arterial PaO2 values.

Quantification of plaque neovascularization using contrast ultrasound: a histologic validation.

AIMS: The density of vasa vasorum within atherosclerotic plaque correlates with histologic features of plaque vulnerability in post-mortem studies. Imaging methods to non-invasively detect vasa vasorum are limited. We hypothesized that contrast ultrasound (CUS) can quantify vasa vasorum during atherosclerosis progression. METHODS AND RESULTS: New Zealand white rabbits received a high-fat diet for 3 weeks, and bilateral femoral artery stenosis was induced by balloon injury. Contrast ultrasound femoral imaging was performed at baseline and 2, 4, and 6 weeks post injury to quantify adventitial videointensity. At each imaging time point 10 vessels were sectioned and stained with haematoxylin and eosin and von-Willebrand factor. Adventitial vasa vasorum density was quantified by counting the number of stained microvessels and their total cross-sectional area. Plaque size (per cent lumen area) progressed over time (P < 0.001), as did adventitial vasa vasorum density (P < 0.001). Plateau peak videointensity also progressed, demonstrating a strong linear correlation with histologic vasa vasorum density (P < 0.001). Receiver operating characteristic analysis indicated that a three-fold increase in median adventitial videointensity had a sensitivity of 100% and specificity of 88% for predicting abnormal neovascularization. CONCLUSION: We have histologically validated that CUS quantifies the development of adventitial vasa vasorum associated with atherosclerosis progression. This imaging technique has the potential for characterizing prognostically significant plaque features.

Role of high-pressure balloon valvotomy for resistant pulmonary valve stenosis.

BACKGROUND: Pulmonary valve (PV) balloon valvotomy (BV) is considered the treatment of choice for isolated pulmonary valve stenosis (IPVS). While immediate and long-term results of PVBV are usually excellent, the reported results in dysplastic valves are variable. High-pressure (HP) PVBV in dysplastic valves that fail low-pressure (LP) PVBV may increase success rate, reducing the need for surgical interventions. METHODS: We reviewed all cases of IPVS in patients <3 years old, who underwent PVBV between August 1999 and March 2004. Study outcomes were initial success rate (gradient post PVBV < 30 mm Hg) and freedom from reintervention. Possible predictors of failure to LP-PVBV were explored (age, hemodynamic data, PV leaflet maximal thickness, diameter/z-scores for PV annulus, sinotubular junction, and subvalvar area). RESULTS: All 35 patients (16 neonates, 5 with critical IPVS) underwent LP-PVBV with immediate success in 27 (80%). All eight patients who failed LP-PVBV successfully underwent HP-PVBV. Upon follow-up (27 +/- 24 months), two patients (6.9%) required reintervention after LP-PVBV (LP-PVBV at 3 months, HP-PVBV at 2 months with success, both reintervention free thereafter), and one patient (12.5%) after HP-PVBV (surgical right ventricular outflow tract patch at 33 months) (Fisher's exact test = 0.5). There were no major immediate or long-term complications. After nonparametric median regression, age (2 vs. 11 months, P < .001) and PV maximal thickness (0.13 vs. 0.24 cm, P= .026) were the only predictors of failure to LP-PVBV. CONCLUSION: HP-PVBV can be performed safely in patients with IPVS that fail LP-PVBV, with high success rate and acceptable long-term results. Failure to LP-PVBV is difficult to predict.

Detection of elevated right atrial pressure using a simple bedside ultrasound measure.

AIMS: Accurate assessment of right atrial pressure (RAP) often requires invasive measurement. With normal RAP, Valsalva increases right internal jugular vein (RIJV) cross sectional area (CSA) 20% to 30%. With high RAP, when venous compliance is low, we hypothesized that the increase in CSA would be blunted and could be detected non-invasively with bedside ultrasound. METHODS AND RESULTS: RIJV ultrasound images were obtained in 67 patients undergoing right heart catheterization. The median RAP at end-expiration was 7 mm Hg (interquartile range [IQR] 5-9 mm Hg) in patients with normal RAP (n = 47) versus 15 mm Hg (IQR 12-22 mm Hg) in patients with elevated RAP (n = 20). With Valsalva, the median percent change in RIJV CSA was 35% (IQR 19%-79%) versus 5% (IQR 3%-14%) for normal and high RAP, respectively. By receiver operating curve analysis, a <17% increase in RIJV CSA with Valsalva predicted elevated RAP (> or =12 mmHg) with 90% sensitivity, 74% specificity, 94% negative predictive value, and 60% positive predictive value (area under the curve 0.86, P < .001). CONCLUSIONS: An increase in RIJV CSA >17% during Valsalva effectively rules out elevated RAP. This simple bedside technique may be useful to assess central venous pressure and reduce the need for invasive pressure measurement.