Quadricuspid Aortic Valve: Characteristics, Associated Structural Cardiovascular Abnormalities, and Clinical Outcomes.

BACKGROUND: Quadricuspid aortic valve (QAV) is a rare congenital cardiac defect. This study sought to determine QAV frequency in a large echocardiography database, to characterize associated cardiovascular abnormalities, and to describe long-term outcomes. METHODS AND RESULTS: Fifty patients (mean ± SD age, 43.5 ± 21.8 years at the time of the index diagnosis; female sex, 52%) received a diagnosis of QAV between January 1, 1975, and March 14, 2014 (frequency, 0.006%). The QAV was type A in 32% and type B in 32% (Hurwitz and Roberts classification). Aortic dilatation was present in 29% of the patients, and 26% had moderate or severe aortic valve regurgitation at the index diagnosis. Stenosis affected only 8% of the valves and was mild. Other findings, including abnormalities of other cardiac valves, septal defects, persistent left superior vena cava, and patent ductus arteriosus, were present in 32% of patients. During a mean ± SD follow-up of 4.8 ± 5.6 years, 8 patients underwent aortic valve surgery, with severe aortic valve regurgitation being the surgical indication in 7 patients. One patient with mild to moderate aortic valve regurgitation underwent aortic valve repair for obstruction of the left coronary ostium by the accessory cusp of QAV. No infective endocarditis or aortic dissection was found. Overall survival was 91.5% and 87.7% at 5 and 10 years. CONCLUSIONS: Aortic dilatation and other structural cardiac abnormalities were relatively common among patients with QAV. Aortic valve regurgitation was the predominant hemodynamic abnormality and the indication for aortic valve surgery in most patients who received surgery. Long-term survival was excellent.

Quantitation of valve regurgitation severity by three-dimensional vena contracta area is superior to flow convergence method of quantitation on transesophageal echocardiography.

BACKGROUND: Quantitation of regurgitation severity using the proximal isovelocity acceleration (PISA) method to calculate effective regurgitant orifice (ERO) area has limitations. Measurement of three-dimensional (3D) vena contracta area (VCA) accurately grades mitral regurgitation (MR) severity on transthoracic echocardiography (TTE). METHODS: We evaluated 3D VCA quantitation of regurgitant jet severity using 3D transesophageal echocardiography (TEE) in 110 native mitral, aortic, and tricuspid valves and six prosthetic valves in patients with at least mild valvular regurgitation. The ASE-recommended integrative method comprising semiquantitative and quantitative assessment of valvular regurgitation was used as a reference method, including ERO area by 2D PISA for assigning severity of regurgitation grade. RESULTS: Mean age was 62.2±14.4 years; 3D VCA quantitation was feasible in 91% regurgitant valves compared to 78% by the PISA method. When both methods were feasible and in the presence of a single regurgitant jet, 3D VCA and 2D PISA were similar in differentiating assigned severity (ANOVAP<.001). In valves with multiple jets, however, 3D VCA had a better correlation to assigned severity (ANOVAP<.0001). The agreement of 2D PISA and 3D VCA with the integrative method was 47% and 58% for moderate and 65% and 88% for severe regurgitation, respectively. CONCLUSION: Measurement of 3D VCA by TEE is superior to the 2D PISA method in determination of regurgitation severity in multiple native and prosthetic valves.

Transient Apical Thickening Following Apical Ballooning Syndrome: A Case Series.

The authors describe transient apical thickening mimicking apical hypertrophic cardiomyopathy following apical ballooning syndrome. These findings are observed on multimodality cardiac imaging and appear to constitute a novel entity. Possible pathophysiologic mechanisms are explored.

Mean Right Atrial Pressure for Estimation of Left Ventricular Filling Pressure in Patients with Normal Left Ventricular Ejection Fraction: Invasive and Noninvasive Validation.

BACKGROUND: There is a paucity of data on the utility of right atrial pressure (RAP) for estimating pulmonary capillary wedge pressure (PCWP) in patients with normal ejection fraction (EF), including patients with heart failure with preserved EF. METHODS: Mean RAP was compared with PCWP in 129 patients (mean age, 61 ± 11 years; 45% men) with exertional dyspnea enrolled in a multicenter study. Measurements included left ventricular volumes, EF, and mitral inflow velocities. RESULTS: Mean PCWP was 14 ± 7 mm Hg, and mean RAP was 8 ± 5 mm Hg. A significant relation was present between mean RAP and mean PCWP (r2 = 0.5, P < .001). RAP > 8 mm Hg had 76% sensitivity and 86% specificity in detecting mean PCWP > 12 mm Hg. In 101 patients with inconclusive mitral filling pattern (defined according to American Society of Echocardiography/European Association of Cardiovascular Imaging 2016 diastolic function recommendations), RAP by catheterization had sensitivity of 73% and specificity of 91%. In a subset of 59 patients with echocardiographic assessment of mean RAP, RAP by echocardiography had sensitivity of 76% and specificity of 89%. CONCLUSIONS: Mean RAP provides useful information about mean PCWP in many patients with normal left ventricular EF. There is good sensitivity and excellent specificity when combining invasive or noninvasive RAP and mitral velocities to determine if PCWP is elevated.

Role of Transesophageal Echocardiography in the Diagnosis of Paradoxical Low Flow, Low Gradient Severe Aortic Stenosis.

BACKGROUND AND OBJECTIVES: Prior studies indicate that up to 35% of cases of severe aortic stenosis (AS) have paradoxical low flow, low gradient despite preserved left ventricular ejection fraction (LVEF). However, error in left ventricular outflow tract (LVOT) diameter may lead to misclassification. Herein, we determined whether measurement of LVOT diameter by transesophageal echocardiography (TEE) results in reclassification of cases to non-severe AS. SUBJECTS AND METHODS: Patients with severe AS with aortic valve area (AVA) <1 cm2 by transthoracic echocardiography (TTE) within 6 months were studied. Paradoxical low flow, low gradient was defined as mean Doppler gradient (MG) <40 mm Hg and stroke volume index (SVI) ≤35 mL/m2. Preserved LVEF was defined as ≥0.50. RESULTS: Among 108 patients, 12 (15%) had paradoxical low flow, low gradient severe AS despite preserved LVEF based on TTE measurement. When LVOT diameter by TEE in 2D was used, only 5 (6.3%) patients had low flow, low gradient severe AS (p<0.001). Coefficients of variability for intraobserver and interobserver measurement of LVOT were <10%. However, the limits of agreement between TTE and TEE measurement of LVOT ranged from 0.43 cm (95% confidence interval [CI]: 0.36 to 0.5) to -0.31 cm (95% CI: -0.38 to -0.23). CONCLUSION: TEE measured LVOT diameter may result in reclassification to moderate AS in some patients due to low prevalence of true paradoxical low flow, low gradient (PLFLG) severe AS.

Doppler Echocardiography for the Estimation of LV Filling Pressure in Patients With Mitral Annular Calcification.

OBJECTIVES: This study sought to identify Doppler parameters useful for the assessment of left ventricular filling pressure (LVFP) in patients with mitral annular calcification (MAC) and to develop and validate a decision algorithm for assessing LVFP in such patients. BACKGROUND: Predicting LVFP in the presence of MAC is problematic. METHODS: Prospectively, 50 patients with MAC (mean 72 ± 11 years of age) underwent a complete Doppler echocardiographic study and right or left heart catheterization. Standard and nonstandard parameters of ventricular filling and relaxation were correlated with LVFP. Classification and regression tree analysis was used to develop a decision tree for prediction of LVFP. Validation was performed prospectively using an additional cohort with MAC and invasive hemodynamics (n = 21). RESULTS: In the initial study group, 26 patients had mild MAC, and 24 had moderate or severe MAC. Mean LVFP was 17.0 ± 8.1 mm Hg (range 4 to 50 mm Hg). Of the variables tested, the best predictor of LVFP was the ratio of early-to-late diastolic filling velocity (mitral E/A) (r = 0.66; p < 0.001). This finding was observed in subjects with mild as well as moderate-to-severe MAC. Importantly, the ratio of early diastolic filling velocity-to-mitral annulus velocity (E/e') demonstrated weak correlation (r = 0.42; p = 0.003). A clinical algorithm using mitral E/A and isovolumic relaxation time (IVRT) was associated with good specificity (100%) and positive predictive value (100%), and moderate sensitivity (81%) and negative predictive value (67%) for high LVFP. Validation of the clinical algorithm in a separate prospective cohort yielded a diagnostic accuracy of 94%. CONCLUSIONS: The E/e' ratio should not be used to estimate LVFP in subjects with significant MAC. However, mitral E/A ratio and IVRT are useful predictors of LVFP in this patient population. The proposed decision algorithm combining these Doppler parameters is accurate in estimating LVFP in patients with MAC.

Estimating Left Ventricular Filling Pressure by Echocardiography.

BACKGROUND: The diagnosis of heart failure may be challenging because symptoms are rather nonspecific. Elevated left ventricular (LV) filling pressure may be used to confirm the diagnosis, but cardiac catheterization is often not practical. Echocardiographic indexes are therefore used as markers of filling pressure. OBJECTIVES: This study investigated the feasibility and accuracy of comprehensive echocardiography in identifying patients with elevated LV filling pressure. METHODS: We conducted a multicenter study of 450 patients with a wide spectrum of cardiac diseases referred for cardiac catheterization. Left atrial volume index, in combination with flow velocities and tissue Doppler velocities, was used to estimate LV filling pressure. Invasively measured pressure was used as the gold standard. RESULTS: Mean left ventricular ejection fraction (LVEF) was 47%, with 209 patients having an LVEF <50%. Invasive measurements showed elevated LV filling pressure in 58% of patients. Clinical assessment had an accuracy of 72% in identifying patients with elevated filling pressure, whereas echocardiography had an accuracy of 87% (p < 0.001 vs. clinical assessment). The combination of clinical and echocardiographic assessment was incremental, with a net reclassification improvement of 1.5 versus clinical assessment (p < 0.001). CONCLUSIONS: Echocardiographic assessment of LV filling pressure is feasible and accurate. When combined with clinical data, it leads to a more accurate diagnosis, regardless of LVEF.

Anomalous coronary artery in a transplanted heart: a rare incidental diagnosis.

Coronary artery anomaly is a rare postoperative coronary angiographic finding in heart transplant recipients. We report a case of anomalous origin of the right coronary artery in an asymptomatic 70-year-old heart transplant patient. Most coronary artery anomalies are benign, but surgical treatment may be necessary in major coronary artery anomalies that are known to have adverse outcomes.

Decreased exercise capacity and sleep-disordered breathing in patients with hypertrophic cardiomyopathy.

BACKGROUND: Mechanisms of decreased exercise capacity in patients with hypertrophic cardiomyopathy (HCM) are not well understood. Sleep-disordered breathing (SDB) is a highly prevalent but treatable disorder in patients with HCM. The role of comorbid SDB in the attenuated exercise capacity in HCM has not been studied previously. METHODS: Overnight oximetry, cardiopulmonary exercise testing, and echocardiographic studies were performed in consecutive patients with HCM seen at the Mayo Clinic. SDB was considered present if the oxygen desaturation index (number of ≥ 4% desaturations/h) was ≥ 10. Peak oxygen consumption (VO2 peak) (the most reproducible and prognostic measure of cardiovascular fitness) was then correlated with the presence and severity of SDB. RESULTS: A total of 198 patients with HCM were studied (age, 53 ± 16 years; 122 men), of whom 32% met the criteria for the SDB diagnosis. Patients with SDB had decreased VO2 peak compared with those without SDB (16 mL O2/kg/min vs 21 mL O2/kg/min, P < .001). SDB remained significantly associated with VO2 peak after accounting for confounding clinical variables (P < .001) including age, sex, BMI, atrial fibrillation, and coronary artery disease. CONCLUSIONS: In patients with HCM, the presence of SDB is associated with decreased VO2 peak. SDB may represent an important and potentially modifiable contributor to impaired exercise tolerance in this unique population.

Late aminophylline reversal of regadenoson stress testing in patients with end stage renal disease.

Regadenoson is a widely used and well-tolerated vasodilator agent for pharmacologic stress myocardial perfusing imaging. In patients at higher risk of adverse reactions such as those with end stage renal disease, it can be reversed with early administration of aminophylline. However, little is known about late administration of aminophylline. This case report describes the efficacy of late aminophylline use in patients with end stage renal disease. Possible explanations for the prolonged pharmacodynamic effect of regadenoson in this group are discussed.

Paradoxical coronary artery embolism - a rare cause of myocardial infarction.

Paradoxical coronary artery embolism is a rare, but often an underdiagnosed cause of acute myocardial infarction. It should be considered in patient who presents with chest pain and otherwise having a low risk profile for atherosclerosis coronary artery disease. We describe a case of paradoxical coronary artery embolism causing ST segment elevation myocardial infarction in a patient with upper extremity venous thrombosis. Echocardiography demonstrated a patent foramen ovale (PFO) with bidirectional shunt. In addition to treatment of acute coronary event closure of the PFO should be considered to prevent a recurrence.

Cardiac output response to exercise in relation to metabolic demand in heart failure with preserved ejection fraction.

AIMS: Exercise intolerance is a hallmark of heart failure with preserved ejection fraction (HFpEF), yet its mechanisms remain unclear. The current study sought to determine whether increases in cardiac output (CO) during exercise are appropriately matched to metabolic demands in HFpEF. METHODS AND RESULTS: Patients with HFpEF (n = 109) and controls (n = 73) exercised to volitional fatigue with simultaneous invasive (n = 96) or non-invasive (n = 86) haemodynamic assessment and expired gas analysis to determine oxygen consumption (VO2) during upright or supine exercise. At rest, HFpEF patients had higher LV filling pressures but similar heart rate, stroke volume, EF, and CO. During supine and upright exercise, HFpEF patients displayed lower peak VO2 coupled with blunted increases in heart rate, stroke volume, EF, and CO compared with controls. LV filling pressures increased dramatically in HFpEF patients, with secondary elevation in pulmonary artery pressures. Reduced peak VO2 in HFpEF patients was predominantly attributable to CO limitation, as the slope of the increase in CO relative to VO2 was 20% lower in HFpEF patients (5.9 ± 2.5 vs. 7.4 ± 2.6 L blood/L O2, P = 0.0005). While absolute increases in arterial-venous O2 difference with exercise were similar in HFpEF patients and controls, augmentation in arterial-venous O2 difference relative to VO2 was greater in HFpEF patients (8.9 ± 3.4 vs. 5.5 ± 2.0 min/dL, P < 0.0001). These differences were observed in the total cohort and when upright and supine exercise modalities were examined individually. CONCLUSION: While diastolic dysfunction promotes congestion and pulmonary hypertension with stress in HFpEF, reduction in exercise capacity is predominantly related to inadequate CO relative to metabolic needs.