ABSTRACT
BACKGROUND AND AIMS: In patients with de novo heart failure with reduced ejection fraction (HFrEF), improvement of left ventricular ejection fraction (LVEF) is expected to occur when started on guideline-recommended medical therapy. However, improvement may not be completed within 90 days. METHODS: Patients with HFrEF and LVEF ≤ 35% prescribed a wearable cardioverter-defibrillator between 2017 and 2022 from 68 sites were enrolled, starting with a registry phase for 3 months and followed by a study phase up to 1 year. The primary endpoints were LVEF improvement > 35% between Days 90 and 180 following guideline-recommended medical therapy initiation and the percentage of target dose reached at Days 90 and 180. RESULTS: A total of 598 patients with de novo HFrEF [59 years (interquartile range 51-68), 27% female] entered the study phase. During the first 180 days, a significant increase in dosage of beta-blockers, renin-angiotensin system inhibitors, and mineralocorticoid receptor antagonists was observed (P < .001). At Day 90, 46% [95% confidence interval (CI) 41%-50%] of study phase patients had LVEF improvement > 35%; 46% (95% CI 40%-52%) of those with persistently low LVEF at Day 90 had LVEF improvement > 35% by Day 180, increasing the total rate of improvement > 35% to 68% (95% CI 63%-72%). In 392 patients followed for 360 days, improvement > 35% was observed in 77% (95% CI 72%-81%) of the patients. Until Day 90, sustained ventricular tachyarrhythmias were observed in 24 wearable cardioverter-defibrillator carriers (1.8%). After 90 days, no sustained ventricular tachyarrhythmia occurred in wearable cardioverter-defibrillator carriers. CONCLUSIONS: Continuous optimization of guideline-recommended medical therapy for at least 180 days in HFrEF is associated with additional LVEF improvement > 35%, allowing for better decision-making regarding preventive implantable cardioverter-defibrillator therapy.
Subject(s)
Heart Failure , Stroke Volume , Humans , Female , Male , Heart Failure/therapy , Heart Failure/physiopathology , Middle Aged , Aged , Stroke Volume/physiology , Mineralocorticoid Receptor Antagonists/therapeutic use , Ventricular Function, Left/physiology , Defibrillators, Implantable , Adrenergic beta-Antagonists/therapeutic use , Duration of Therapy , Wearable Electronic Devices , RegistriesABSTRACT
BACKGROUND: Ambulatory hemodynamic monitoring (AHM) using an implantable pulmonary artery pressure sensor (CardioMEMS) is effective in improving outcomes for patients with heart failure. The operations of AHM programs are crucial to clinical efficacy of AHM yet have not been described. METHODS AND RESULTS: An anonymous, voluntary, web-based survey was developed and emailed to clinicians at AHM centers in the United States. Survey questions were related to program volume, staffing, monitoring practices, and patient selection criteria. Fifty-four respondents (40%) completed the survey. Respondents were 44% (nâ¯=â¯24) advanced HF cardiologists and 30% (nâ¯=â¯16) advanced nurse practitioners. Most respondents practice at a center that implants left ventricular assist devices (70%) or performs heart transplantation (54%). Advanced practice providers provide day-to-day monitoring and management in most programs (78%), and use of protocol-driven care is limited (28%). Perceived patient nonadherence and inadequate insurance coverage are cited as the primary barriers to AHM. CONCLUSIONS: Despite broad US Food and Drug Administration approval for patients with symptoms and at increased risk for worsening heart failure, the adoption of pulmonary artery pressure monitoring is concentrated at advanced heart failure centers, and modest numbers of patients are implanted at most centers. Understanding and addressing the barriers to referral of eligible patients and to broader adoption in community heart failure programs is needed to maximize the clinical benefits of AHM.
Subject(s)
Heart Failure , Heart Transplantation , Hemodynamic Monitoring , Humans , United States/epidemiology , Heart Failure/diagnosis , Heart Failure/therapy , Monitoring, Ambulatory , Hemodynamics , Pulmonary Artery , Blood Pressure Monitoring, Ambulatory/methodsABSTRACT
AIMS: During the coronavirus disease 2019 (COVID-19) pandemic, important changes in heart failure (HF) event rates have been widely reported, but few data address potential causes for these changes; several possibilities were examined in the GUIDE-HF study. METHODS AND RESULTS: From 15 March 2018 to 20 December 2019, patients were randomized to haemodynamic-guided management (treatment) vs. control for 12 months, with a primary endpoint of all-cause mortality plus HF events. Pre-COVID-19, the primary endpoint rate was 0.553 vs. 0.682 events/patient-year in the treatment vs. control group [hazard ratio (HR) 0.81, P = 0.049]. Treatment difference was no longer evident during COVID-19 (HR 1.11, P = 0.526), with a 21% decrease in the control group (0.536 events/patient-year) and no change in the treatment group (0.597 events/patient-year). Data reflecting provider-, disease-, and patient-dependent factors that might change the primary endpoint rate during COVID-19 were examined. Subject contact frequency was similar in the treatment vs. control group before and during COVID-19. During COVID-19, the monthly rate of medication changes fell 19.2% in the treatment vs. 10.7% in the control group to levels not different between groups (P = 0.362). COVID-19 was infrequent and not different between groups. Pulmonary artery pressure area under the curve decreased -98â mmHg-days in the treatment group vs. -100â mmHg-days in the controls (P = 0.867). Patient compliance with the study protocol was maintained during COVID-19 in both groups. CONCLUSION: During COVID-19, the primary event rate decreased in the controls and remained low in the treatment group, resulting in an effacement of group differences that were present pre-COVID-19. These outcomes did not result from changes in provider- or disease-dependent factors; pulmonary artery pressure decreased despite fewer medication changes, suggesting that patient-dependent factors played an important role in these outcomes. Clinical Trials.gov: NCT03387813.
Subject(s)
COVID-19 , Heart Failure , Hemodynamics , Humans , Pandemics , Pulmonary ArteryABSTRACT
BACKGROUND: Sodium glucose cotransporter 2 inhibitors (SGLT2 inhibitors) prevent heart failure (HF) hospitalizations in patients with type 2 diabetes and improve outcomes in those with HF and reduced ejection fraction, regardless of type 2 diabetes. Mechanisms of HF benefits remain unclear, and the effects of SGLT2 inhibitor on hemodynamics (filling pressures) are not known. The EMBRACE-HF trial (Empagliflozin Evaluation by Measuring Impact on Hemodynamics in Patients With Heart Failure) was designed to address this knowledge gap. METHODS: EMBRACE-HF is an investigator-initiated, randomized, multicenter, double-blind, placebo-controlled trial. From July 2017 to November 2019, patients with HF (regardless of ejection fraction, with or without type 2 diabetes) and previously implanted pulmonary artery (PA) pressure sensor (CardioMEMS) were randomized across 10 US centers to empagliflozin 10 mg daily or placebo and treated for 12 weeks. The primary end point was change in PA diastolic pressure (PADP) from baseline to end of treatment (average PADP weeks 8-12). Secondary end points included health status (Kansas City Cardiomyopathy Questionnaire score), natriuretic peptides, and 6-min walking distance. RESULTS: Overall, 93 patients were screened, and 65 were randomized (33 to empagliflozin, 32 to placebo). The mean age was 66 years; 63% were male; 52% had type 2 diabetes; 54% were in New York Heart Association class III/IV; mean ejection fraction was 44%; median NT-proBNP (N-terminal pro B-type natriuretic peptide) was 637 pg/mL; and mean PADP was 22 mm Hg. Empagliflozin significantly reduced PADP, with effects that began at week 1 and amplified over time; average PADP (weeks 8-12) was 1.5 mm Hg lower (95% CI, 0.2-2.8; P=0.02); and at week 12, PADP was 1.7 mm Hg lower (95% CI, 0.3-3.2; P=0.02) with empagliflozin versus placebo. Results were consistent for PA systolic and PA mean pressures. There was no difference in mean loop diuretic management (daily furosemide equivalents) between treatment groups. No significant differences between treatment groups were observed in Kansas City Cardiomyopathy Questionnaire scores, natriuretic peptide levels, and 6-min walking distance. CONCLUSIONS: In patients with HF and CardioMEMS PA pressure sensor, empagliflozin produced rapid reductions in PA pressures that were amplified over time and appeared to be independent of loop diuretic management. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT03030222.
Subject(s)
Benzhydryl Compounds/therapeutic use , Glucosides/therapeutic use , Heart Failure/drug therapy , Pulmonary Artery/drug effects , Sodium-Glucose Transporter 2 Inhibitors/therapeutic use , Aged , Benzhydryl Compounds/pharmacology , Double-Blind Method , Female , Glucosides/pharmacology , Humans , Male , Middle Aged , Sodium-Glucose Transporter 2 Inhibitors/pharmacologyABSTRACT
Atrial myxoma is the most common primary left atrial tumor. Here we present a case report of a COVID-19 positive patient who was found to have an incidental left atrial appendage mass, concerning for thrombus. Further workup of the mass was suspicious for myxoma, warranting surgical resection.
Subject(s)
Atrial Appendage , COVID-19 , Heart Neoplasms , Myxoma , Atrial Appendage/diagnostic imaging , Atrial Appendage/surgery , Echocardiography, Transesophageal , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/surgery , Humans , Myxoma/diagnostic imaging , Myxoma/surgery , SARS-CoV-2ABSTRACT
The number of people older than 65 will double by 2060, and with it, the number of people suffering from heart failure will also surge. In the United States, it is estimated that there are close to 250,000-500,000 endstage/ advanced heart failure cases. Mechanical circulatory support (MCS) is an evolving advanced therapy for end-stage heart failure. MCS can be an interim measure along with acute mechanical circulatory support measures including but not limited to the intra-aortic balloon pump, extracorporeal membrane oxygenation, or temporary ventricular assist devices such as Impella, or MCS can be a more prolonged and ambulatory measure in conjunction with an implantable, durable left ventricular assist device (LVAD). As the technology of LVADs advances, the complication rate is decreasing, and the living LVAD patient population is expanding. This indicates that the probability of a non-heart failure specialist encountering these patients is also on the rise. In this article, we aim to expand the familiarity and basic knowledge of non-heart failure specialists by detailing the concepts and complications of LVADs, enabling them to more comfortably manage these patients.
Subject(s)
Extracorporeal Membrane Oxygenation , Heart Failure/diagnosis , Heart Failure/therapy , Heart-Assist Devices , Aged , Health Transition , Heart Failure/epidemiology , Heart-Assist Devices/trends , Humans , United States/epidemiologyABSTRACT
Trans-catheter aortic valve replacement (TAVR) was approved by the U.S. Food and Drug Administration in 2012 for treatment of severe symptomatic aortic stenosis in non-surgical and high risk patients. Implementation of this complex procedure requires a comprehensive heart team approach. Rural demographics in the Midwest pose many challenges related to low volumes of operations both at institutional and individual levels, leading to serious concerns about the quality of care delivered in such a setting. We compared the TAVR data at the University of South Dakota Sanford Medical Center to the national registry with the aim of looking at differences in outcomes of this procedure in a rural setting.
Subject(s)
Aortic Valve Stenosis/surgery , Rural Population , Transcatheter Aortic Valve Replacement , Aged, 80 and over , Female , Humans , Male , Patient Care Team , Program Evaluation , South Dakota , Treatment OutcomeABSTRACT
Heart failure is a major public health concern. It is the most common reason for hospitalization. It commonly affects older population and given the increasing life expectancy coupled with improved management of chronic medical conditions, the number of patients with heart failure is expected to increase. Heart failure has been recently categorized into heart failure with reduced and preserved ejection fraction. Despite differences in the two types, mortality remains high and similar in both conditions. Over the past few decades, numerous medical and device based therapies have been developed for the management of heart failure. These therapies have improved outcomes for the patients both in terms of morbidity and mortality. The objective of this review is to provide an overview of management of heart failure, specifically heart failure with reduced ejection fraction. It is of paramount importance for all health care providers to be aware of therapies for management of heart failure. We will also briefly discuss the role of mechanical circulatory support as an emerging new therapy for patients with advanced heart failure.
Subject(s)
Heart Failure/therapy , Hospitalization , Public Health , Aged , Heart Failure/epidemiology , Heart Failure/physiopathology , Humans , Risk FactorsABSTRACT
BACKGROUND: Monitoring supine pulmonary artery pressures to guide heart failure (HF) management has reduced HF hospitalizations in select patients. OBJECTIVES: The purpose of this study was to evaluate the effect of managing seated mean pulmonary artery pressure (mPAP) with the Cordella Pulmonary Artery sensor on outcomes in patients with HF. METHODS: Following GUIDE-HF (Hemodynamic-GUIDEd Management of Heart Failure Trial), with U.S. Food and Drug Administration input, PROACTIVE-HF (A Prospective, Multi-Center, Open Label, Single Arm Clinical Trial Evaluating the Safety and Efficacy of the Cordella Pulmonary Artery Sensor System in NYHA Class III Heart Failure Patients trial) was changed from a randomized to a single-arm, open label trial, conducted at 75 centers in the USA and Europe. Eligible patients had chronic HF with NYHA functional class III symptoms, irrespective of the ejection fraction, and recent HF hospitalization and/or elevated natriuretic peptides. The primary effectiveness endpoint at 6 months required the HF hospitalization or all-cause mortality rate to be lower than a performance goal of 0.43 events/patient, established from previous hemodynamic monitoring trials. Primary safety endpoints at 6 months were freedom from device- or system-related complications or pressure sensor failure. RESULTS: Between February 7, 2020, and March 31, 2023, 456 patients were successfully implanted in modified intent-to-treat cohort. The 6-month event rate was 0.15 (95% CI: 0.12-0.20) which was significantly lower than performance goal (0.15 vs 0.43; P < 0.0001). Freedom from device- or system-related complications was 99.2% and freedom from sensor failure was 99.8% through 6 months. CONCLUSIONS: Remote management of seated mPAP is safe and results in a low rate of HF hospitalizations and mortality. These results support the use of seated mPAP monitoring and extend the growing body of evidence that pulmonary artery pressure-guided management improves outcomes in heart failure. (Multi-Center, Open Label, Single Arm Clinical Trial Evaluating the Safety and Efficacy of the Cordella Pulmonary Artery Sensor System in NYHA Class III Heart Failure Patients trial [PROACTIVE-HF]; NCT04089059).
ABSTRACT
Background: In this multicenter prospective study, we explored the relationship between pulmonary artery pressure (PAP) at rest and in response to a 6-min walk test (6MWT) in ambulatory patients with heart failure (HF) with an implantable PAP sensor (CardioMEMS, Abbott). Methods: Between 5/2019 and 2/2021, HF patients with a CardioMEMS sensor were recruited from seven sites. PAP was recorded in the supine and seated position at rest and in the seated position immediately post-exercise. Results: In our cohort of 66 patients, mean age was 70 ± 12 years, 67% male, left ventricular ejection fraction (LVEF) < 50% in 53%, mean 6MWT distance was 277 ± 95 meters. Resting seated PAPs were 31 ± 15 mmHg (systolic), 13 ± 8 mmHg (diastolic), and 20 ± 11 mmHg (mean). The pressures were lower in the seated rather than the supine position. After 6MWT, the pressures increased to PAP systolic 37 ± 19 mmHg (p < 0.0001), diastolic 15 ± 10 mmHg (p = 0.006), and mean 24 ± 13 mmHg (p < 0.0001). Patients with elevated PAP diastolic at rest (>15 mmHg) demonstrated a greater increase in post-exercise PAP. Conclusion: The measurement of PAP with CardioMEMS is feasible immediately post-exercise. Despite being well-managed, patients had severely limited functional capacity. We observed a significant increase in PAP with ambulation which was greater in patients with higher baseline pressures.
ABSTRACT
Background: Hemodynamic Frontiers in Heart Failure (HF2) is a multicenter academic research consortium comprised of 14 US institutions with mature remote monitoring programs for ambulatory patients with heart failure (HF). The consortium developed a retrospective and prospective registry of patients implanted with a wireless pulmonary artery pressure (PAP) sensor. Goals/aims: HF2 registry collects demographic, clinical, laboratory, echocardiographic (ECHO), and hemodynamic data from patients with PAP sensors. The aims of HF2 are to advance understanding of HF and to accelerate development of novel diagnostic and therapeutic innovations. Methods: HF2 includes adult patients implanted with a PAP sensor as per FDA indications (New York Heart Association (NYHA) Class III HF functional class with a prior hospitalization, or patients with NYHA Class II or brain natriuretic peptide (BNP) elevation without hospitalization) at a HF2 member site between 1/1/19 to present. HF2 registry is maintained at University of Kansas Medical Center (KUMC). The registry was approved by the institutional review board (IRB) at all participating institutions with required data use agreements. Institutions report data into the electronic registry database using REDCap, housed at KUMC. Results: This initial data set includes 254 patients implanted from the start of 2019 until May 2023. At time of device implant, the cohort average age is 73 years old, 59.8% are male, 72% have NYHA Class III HF, 40% have left ventricular ejection fraction (LVEF) < 40%, 35% have LVEF > 50%, mean BNP is 560â pg/ml, mean N-Terminal pro-BNP (NTproBNP) is 5,490â pg/ml, mean creatinine is 1.65â mg/dl. Average baseline hemodynamics at device implant are right atrial pressure (RAP) of 11â mmHg, pulmonary artery systolic pressure (PASP) of 47â mmHg, pulmonary artery diastolic pressure (PADP) 21â mmHg, mean pulmonary artery pressure (mPAP) of 20â mmHg, pulmonary capillary wedge pressure (PCWP) of 19â mmHg, cardiac output (CO) of 5.3â L/min, and cardiac index (CI) of 2.5â L/min/m2. Conclusion: A real-world registry of patients implanted with a PAP sensor enables long-term evaluation of hemodynamic and clinic outcomes in highly-phenotyped ambulatory HF patients, and creates a unique opportunity to validate and test novel diagnostic and therapeutic approaches to HF.
ABSTRACT
BACKGROUND: Ambulatory hemodynamic monitoring with an implantable pulmonary artery (PA) sensor is approved for patients with New York Heart Association Class III heart failure (HF) and a prior HF hospitalization (HFH) within 12 months. The objective of this study was to assess the efficacy and safety of PA pressure-guided therapy in routine clinical practice with special focus on subgroups defined by sex, race, and ejection fraction. METHODS: This multi-center, prospective, open-label, observational, single-arm trial of 1200 patients across 104 centers within the United States with New York Heart Association class III HF and a prior HFH within 12 months evaluated patients undergoing PA pressure sensor implantation between September 1, 2014, and October 11, 2017. The primary efficacy outcome was the difference between rates of adjudicated HFH 1 year after compared with the 1 year before sensor implantation. Safety end points were freedom from device- or system-related complications at 2 years and freedom from pressure sensor failure at 2 years. RESULTS: Mean age for the population was 69 years, 37.7% were women, 17.2% were non-White, and 46.8% had preserved ejection fraction. During the year after sensor implantation, the mean rate of daily pressure transmission was 76±24% and PA pressures declined significantly. The rate of HFH was significantly lower at 1 year compared with the year before implantation (0.54 versus 1.25 events/patient-years, hazard ratio 0.43 [95% CI, 0.39-0.47], P<0.0001). The rate of all-cause hospitalization was also lower following sensor implantation (1.67 versus 2.28 events/patient-years, hazard ratio 0.73 [95% CI, 0.68-0.78], P<0.0001). Results were consistent across subgroups defined by ejection fraction, sex, race, cause of cardiomyopathy, presence/absence of implantable cardiac defibrillator or cardiac resynchronization therapy and ejection fraction. Freedom from device- or system-related complications was 99.6%, and freedom from pressure sensor failure was 99.9% at 1 year. CONCLUSIONS: In routine clinical practice as in clinical trials, PA pressure-guided therapy for HF was associated with lower PA pressures, lower rates of HFH and all-cause hospitalization, and low rates of adverse events across a broad range of patients with symptomatic HF and prior HFH. Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02279888.
Subject(s)
Blood Pressure Monitoring, Ambulatory , Heart Failure/therapy , Hospitalization/statistics & numerical data , Pulmonary Wedge Pressure/physiology , Aged , Female , Heart Failure/epidemiology , Heart Failure/physiopathology , Hemodynamics , Humans , Male , Prospective Studies , Pulmonary Artery , United States/epidemiologyABSTRACT
Importance: In a randomized clinical trial, heart failure (HF) hospitalizations were lower in patients managed with guidance from an implantable pulmonary artery pressure sensor compared with usual care. It remains unclear if ambulatory monitoring could also improve long-term clinical outcomes in real-world practice. Objective: To determine the association between ambulatory hemodynamic monitoring and rates of HF hospitalization at 12 months in clinical practice. Design, Setting, and Participants: This matched cohort study of Medicare beneficiaries used claims data collected between June 1, 2014, and March 31, 2016. Medicare patients who received implants of a pulmonary artery pressure sensor were identified from the 100% Medicare claims database. Each patient who received an implant was matched to a control patient by demographic features, history of HF hospitalization, and number of all-cause hospitalizations. Propensity scoring based on comorbidities (arrhythmia, hypertension, diabetes, pulmonary disease, and renal disease) was used for additional matching. Data analysis was completed from July 2017 through January 2019. Exposures: Implantable pulmonary artery pressure monitoring system. Main Outcomes and Measures: The rates of HF hospitalization were compared using the Andersen-Gill method. Days lost owing to events were compared using a nonparametric bootstrap method. Results: The study cohort consisted of 1087 patients who received an implantable pulmonary artery pressure sensors and 1087 matched control patients. The treatment and control cohorts were well matched by age (mean [SD], 72.7 [10.2] years vs 72.9 [10.1] years) and sex (381 of 1087 female patients [35.1%] in each group), medical history, comorbidities, and timing of preimplant HF hospitalization. At 12 months postimplant, 616 HF hospitalizations occurred in the treatment cohort compared with 784 HF hospitalizations in the control cohort. The rate of HF hospitalization was lower in the treatment cohort at 12 months postimplant (hazard ratio [HR], 0.76 [95% CI, 0.65-0.89]; P < .001). The percentage of days lost to HF hospitalizations or death were lower in the treatment group (HR, 0.73 [95% CI, 0.64-0.84]; P < .001) and the percentage of days lost owing to all-cause hospitalization or death were also lower (HR, 0.77 [95% CI, 0.68-0.88]; P < .001). Conclusions and Relevance: Patients with HF who were implanted with a pulmonary artery pressure sensor had lower rates of HF hospitalization than matched controls and spent more time alive out of hospital. Ambulatory hemodynamic monitoring may improve outcomes in patients with chronic HF.
Subject(s)
Blood Pressure , Heart Failure/therapy , Hospitalization/statistics & numerical data , Monitoring, Ambulatory/methods , Prostheses and Implants , Pulmonary Artery , Aged , Aged, 80 and over , Cohort Studies , Disease Management , Female , Heart Failure/diagnosis , Heart Failure/physiopathology , Hemodynamics , Humans , Kaplan-Meier Estimate , Male , Medicare , Patient Care Planning , Propensity Score , Proportional Hazards Models , United StatesABSTRACT
Spontaneous coronary artery dissection is an uncommon cause of acute myocardial infarction in the general population but is relatively more common in the peripartum period. Regardless of clinical setting, the management strategy is individualised, ranging from conservative to invasive. We report a case of peripartum myocardial infarction due to spontaneous coronary dissection that propagated during diagnostic angiography and ultimately required emergent bypass surgery.
Subject(s)
Coronary Angiography/adverse effects , Coronary Vessel Anomalies/diagnosis , Puerperal Disorders/diagnosis , Vascular Diseases/congenital , Adult , Chest Pain/etiology , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessel Anomalies/etiology , Coronary Vessel Anomalies/surgery , Diagnosis, Differential , Electrocardiography , Female , Humans , Iatrogenic Disease , Pregnancy , Puerperal Disorders/diagnostic imaging , Puerperal Disorders/etiology , Puerperal Disorders/surgery , Twins , Vascular Diseases/diagnosis , Vascular Diseases/diagnostic imaging , Vascular Diseases/etiology , Vascular Diseases/surgeryABSTRACT
Alport syndrome (AS) is a genetic disorder due to inheritance of genetic mutations which lead to production of abnormal type IV collagen. AS has been associated with renal, auditory, and ocular diseases due to the presence of abnormal alpha chains of type IV collagen in the glomerulus, cochlea, cornea, lens, and retina. The resulting disorder includes hereditary nephritis, corneal opacities, anterior lenticonus, fleck retinopathy, temporal retinal thinning, and sensorineural deafness. Aortic and aortic valve pathologies have been described as extrarenal manifestations of AS in multiple case reports. One case report described intramural hematoma of the coronary artery. We report the first case of true spontaneous coronary artery dissection (SCAD) with an intimal flap as a very rare manifestation of AS. The patient is a 36-year-old female with history of AS with chronic kidney disease, hypertension, and obesity who presented to the emergency room with acute onset of substernal chest pain radiating to her neck and arms. Troponin was elevated, and ECG showed transient 1 mm ST-segment elevation in the inferior leads. Subsequent coronary angiography revealed localized dissection of the left circumflex artery. Percutaneous coronary angioplasty was performed and her symptoms improved. This case illustrates that SCAD may be a manifestation of AS patients with chest pain.
ABSTRACT
Sinus venosus atrial septal defect is a rare congenital, interatrial communication defect at the junction of the right atrium and the vena cava. It accounts for 5-10% of cases of all atrial septal defects. Due to the rare prevalence and anatomical complexity, diagnosing sinus venous atrial septal defects poses clinical challenges which may delay diagnosis and treatment. Advanced cardiac imaging studies are useful tools to diagnose this clinical entity and to delineate the anatomy and any associated communications. Surgical correction of the anomaly is the primary treatment. We discuss a 43-year-old Hispanic female patient who presented with dyspnea and hypoxia following a laparoscopic myomectomy. She had been diagnosed with peripartum cardiomyopathy nine years ago at another hospital. Transesophageal echocardiography and computed tomographic angiography of the chest confirmed a diagnosis of sinus venosus atrial septal defect. She was also found to have pulmonary arterial hypertension and Eisenmenger syndrome. During a hemodynamic study, she responded to vasodilator and she was treated with Ambrisentan and Tadalafil. After six months, her symptoms improved and her pulmonary arterial hypertension decreased. We also observed progressive reversal of the right-to-left shunt. This case illustrates the potential benefit of vasodilator therapy in reversing Eisenmenger physiology, which may lead to surgical repair of the atrial septal defect as the primary treatment.