Mitral Annulus Calcium Score in Patients With Calcific Mitral Stenosis Undergoing Invasive Hemodynamic Assessment.

BACKGROUND: Aortic valve calcium score is associated with hemodynamic severity of aortic stenosis. Whether this association is present in calcific mitral stenosis remains unknown. METHODS AND RESULTS: This study was a retrospective analysis of consecutive patients with mitral stenosis secondary to mitral annular calcification (MAC) undergoing transseptal catheterization. All patients underwent invasive mitral valve assessment via direct left atrial and left ventricular pressure measurement. Computed tomography within 1 year of cardiac catheterization and with adequate visualization of the mitral annulus was included. MAC calcium score quantification by Agatston method was obtained offline using dedicated software (Aquarius, TeraRecon, V.4). Median patient age was 66.9±11.2 years, 47% of patients were women, 50% had coronary artery disease, 40% had atrial fibrillation, 47% had prior cardiac surgery, and 33% had prior chest radiation. Median diastolic mitral valve gradient was 9.4±3.4 mm Hg on echocardiography and 8.5±4 mm Hg invasively. Invasive median mitral valve area using the Gorlin formula was 1.87±0.9 cm2. Median MAC calcium score for the cohort was 7280±7937 Hounsfield units. MAC calcium score correlated with the presence of atrial fibrillation (P=0.02) but was not associated with other comorbidities. There was no correlation between MAC calcium score and mitral valve area (r=0.07; P=0.6) or mitral valve gradient (r=-0.03; P=0.8). CONCLUSIONS: MAC calcium score did not correlate with invasively measured mitral valve gradient and mitral valve area in patients with MAC-related mitral stenosis, suggesting that calcium score should not be used as a surrogate for invasive hemodynamic parameters.

Echo-Doppler and strain assessment of filling pressures in adults with congenitally corrected transposition and systemic right ventricles.

AIMS: Systolic dysfunction of the systemic right ventricle (sRV) is common in adults with transposition of the great arteries and sRV. In acquired disease, diastology analysis for assessment of filling pressures (FP) is paramount in patient care. METHODS AND RESULTS: Retrospective analysis of 47 adults with sRV without prior systemic tricuspid valve surgery undergoing catheterization and echocardiography within 7 days (median -2 [-1, -3]) from January 2000 to February 2021 at our institution. Median age was 48 (31, 55) years, and 16 (34.0%) patients were female. FPs were normal in 21 patients (44.7%). Left atrial size was enlarged in most patients (83.0%) with mean indexed value 58.3 ± 23.4 mL/m2. Tissue Doppler e' was not significantly different between those with high FPs vs. normal (medial 0.07 ± 0.03 vs. 0.08 ± 0.03 m/s, P = 0.63; lateral 0.08 ± 0.04 vs. 0.08 ± 0.04 m/s, P = 0.88). E velocity and subpulmonic mitral regurgitant velocity were higher in those with high FPs (0.9 ± 0.3 vs. 0.6 ± 0.2 m/s, P = 0.005; 3.8 ± 1.1 vs. 2.8 ± 0.9 m/s, P = 0.004). Left atrial reservoir strain, sRV global longitudinal strain, and subpulmonic left ventricular strain were worse in those with high FP (18.0 ± 7.6 vs. 27.9 ± 10.2%, P = 0.0009; -13.0 ± 4.4 vs. -17.9 ± 5.0%, P = 0.002; -16.8 ± 5.7 vs. -23.0 ± 3.8%, P = 0.001). CONCLUSION: Despite the complex anatomy, FPs can be assessed non-invasively in adults with sRV without prior systemic tricuspid valve surgery. The current guideline algorithm for diastolic dysfunction in acquired heart disease has limited applicability in this population. Given the limitations of Doppler in this heterogeneous population, strain analysis can be a helpful adjunct for estimation of FPs.

Surgical Repair of Truncus Arteriosus: A Long-Term Hemodynamic Assessment.

Background: Unrepaired truncus arteriosus (TA) carries poor prognosis due to complications of unrestricted pulmonary flow, truncal valve insufficiency, and pulmonary vascular disease. Currently, the hemodynamic profile of adults late after TA repair is unknown. We reviewed the hemodynamics, prevalence, and pathophysiology of pulmonary hypertension (PH) in this population. Methods: Eighteen adult patients with repaired TA who underwent cardiac catheterization at Mayo Clinic, MN, between 1997 and 2021 were identified. PH was defined as either precapillary (mean pulmonary artery pressure [mPAP] ≥25 mm Hg, pulmonary artery wedge pressure [PAWP] ≤15 mm Hg, and pulmonary vascular resistance [PVR] >3 Wood units), isolated postcapillary (mPAP ≥25, PAWP >15, PVR ≤3), or combined (mPAP ≥25, PAWP >15, and PVR >3). Diastolic pressure and transpulmonary gradients were used as ancillary data for classification. Results: Mean age at catheterization was 34 ± 10 years. Mean right ventricular (RV) systolic pressure was 82 ± 22.6 mm Hg, mean right and left mPAPs 28.1 ± 16.2 and 27.9 ± 11.9 mm Hg, respectively. Seven patients (41.2%) had PAWP >15 mm Hg and, among those undergoing arterial catheterization, 7 (53.8%) had a left ventricular (LV) end-diastolic pressure >15 mm Hg. PH was diagnosed in 13 patients (72.2%): 6 (33.3%) precapillary, 4 (22.2%) isolated postcapillary, and 3 (16.7%) combined. PAWP >15 mm Hg was associated with male sex (P = .049),

Role of Echocardiography for Assessment of Cardiac Remodeling in Congenitally Corrected Transposition of Great Arteries.

BACKGROUND: The purpose of this study was to assess the role of echocardiography for a comprehensive assessment of cardiac remodeling, and the relationship between indices of cardiac remodeling and cardiovascular events (defined as the composite end point of heart failure hospitalization, heart transplant, or cardiovascular death) in adults with congenitally corrected transposition of great arteries (cc-TGA). METHODS: This is a retrospective study of adults with cc-TGA who underwent echocardiogram (2003-2020). Offline image analysis was performed in all patients. Chamber (atrial and ventricular) function and size were assessed by strain imaging and 2-dimensional echocardiography. RESULTS: Of 233 patients with cc-TGA (40±15 years), 123 (55%) had at least one cardiac procedure before baseline echocardiogram. Of 233 patients, 76% and 61% had left atrial dysfunction and systemic right ventricular dysfunction, respectively; while 43% and 11% had right atrial dysfunction and left ventricular dysfunction, respectively. During a median follow-up of 8.9 years, 114 (49%) underwent additional cardiac procedures, and 66 (28%) had cardiovascular events. Left atrial reservoir strain, right ventricular global longitudinal strain, right atrial reservoir strain, left ventricular systolic pressure, and left ventricular global longitudinal strain were independently associated with cardiovascular events. CONCLUSIONS: In addition to the clinical importance of right ventricular systolic dysfunction in cc-TGA that is already well described, the current study demonstrated, for the first time, that biatrial dysfunction was common and was associated with clinical outcomes. Since there are currently no effective therapies for atrial and ventricular dysfunction in patients with cc-TGA, there is a need for research to identify novel strategies to prevent atrial and ventricular dysfunction in this population.

Diamond-Forrester classification using echocardiography haemodynamic assessment in cardiac intensive care unit patients.

AIMS: We sought to determine whether the Diamond-Forrester classification using non-invasive haemodynamic measurements by 2-D and Doppler echocardiography would predict hospital mortality in cardiac intensive care unit (CICU) patients. METHODS AND RESULTS: We retrospectively analysed unique patients admitted to the CICU at Mayo Clinic Rochester from 2007 to 2018. Doppler-derived cardiac index (CI) and ratio of mitral valve E velocity to medial mitral annulus e' velocity (E/e' ratio) were used to classify patients into four profiles: Profile I (warm/dry), Profile II (warm/wet), Profile III (cold/dry), and Profile IV (cold/wet). Logistic regression was used to determine predictors of hospital mortality, and Cox proportional-hazards analysis was used to determine predictors of mortality during one year of follow-up. We included 4563 patients with a mean age of 68.3 ± 14.3 years, including 36.2% female patients. The distribution of each profile was as follows: I, 47.4%; II, 36.2%; III, 7.9%; IV, 8.5%. A total of 5.8% patients died during hospitalization, and 18.1% died by 1 year. Patients with either low CI or elevated E/e' ratio had higher in-hospital and 1 year mortality. Patients with elevated E/e' ratio (i.e. Profiles II and IV) had an increased risk of death during hospitalization and at 1 year after multivariate adjustment (adjusted hazard ratio 1.72 and 2.17 for 1 year mortality, respectively, compared with Profile I, P < 0.01). CONCLUSIONS: Simple Doppler echocardiographic assessment can be used to identify haemodynamic profiles defined by the Diamond-Forester classification in patients admitted in CICU. These profiles predict outcomes and may be used to guide therapy in critically ill patients.

Assessment of left ventricular filling pressure with Doppler velocities across the patent foramen ovale.

BACKGROUND: The utility of Doppler velocities across the patent foramen ovale (PFO) to estimate left ventricular (LV) filling pressure is not well known. METHODS: The best cut-off value of peak interatrial septal velocity across a transeptal puncture site measured by transesophageal echocardiography for estimating high mean left atrial (LA) pressure (≥ 15 mmHg) was determined in 17 patients. This cut-off value was subsequently applied to 67 patients with a PFO undergoing transthoracic echocardiography (TTE) for assessing the value of PFO velocity in determining LV filling pressure. RESULTS: The peak systolic interatrial septal velocities significantly correlated with directly measured mean LA pressures during transcatheter mitral valve procedure (r = 0.77, P < 0.001). The best cut-off value was 1.7 m/s for predicting high LA pressure (AUC 0.91; sensitivity 90%, specificity 86%). When this cut-off was applied to patients undergoing TTE, peak PFO velocity ≥ 1.7 m/s correlated with reduced e', higher E/e', and higher tricuspid regurgitation velocity (P < 0.01). LV filling pressure according to the 2016 diastolic guideline was compared with peak PFO velocity in 51 patients. Among patients with high filling pressure according to the guidelines (n = 20), peak PFO velocity ≥ 1.7 m/s was present in 60% of patients. In patients with normal filling pressure per the guidelines (n = 31), PFO velocity < 1.7 m/s was present 84%. Sensitivity and specificity were 75% and 92%, respectively, in patients with sinus rhythm, but were only 50% and 57%, respectively, among patients with atrial fibrillation. CONCLUSIONS: Doppler-derived peak PFO velocities could be valuable in the assessment of increased LV filling pressure using 1.7 m/s as the cut-off value.

Assessment of Right Ventricular-Pulmonary Arterial Coupling in Chronic Pulmonary Regurgitation.

BACKGROUND: We hypothesized that noninvasively measured right ventricular (RV) to pulmonary arterial (RV-PA) coupling would be abnormal in chronic pulmonary regurgitation (PR) even in the setting of normal RV ejection fraction, and that RV-PA coupling indices would have a better correlation with peak oxygen consumption (VO2) compared with RV systolic indices alone. METHODS: This was a retrospective study of 129 adults (repaired tetralogy of Fallot [TOF] n = 84 and valvular pulmonic stenosis [VPS] with previous intervention n = 45) with ≥ moderate native PR and RV ejection fraction > 50%. The 84 TOF patients were propensity matched with 84 patients with normal echocardiogram (control); age 28 ± 7 years and male sex n = 39 (46%). RV-PA coupling was measured according to fractional area change (FAC)/RV systolic pressure (RVSP) and tricuspid annular plane systolic excursion (TAPSE)/RVSP. RESULTS: RV systolic function indices were similar between TOF and control groups (FAC 43 ± 6% vs 41 ± 5% [P = 0.164] and TAPSE 22 ± 5 mm vs 24 ± 6 mm [P = 0.263]). However, RV-PA coupling was lower in the TOF group (FAC/RVSP 1.10 ± 0.29 vs 1.48 ± 0.22 [P < 0.001]; TAPSE/RVSP 0.51 ± 0.15 vs 0.78 ± 0.11 [P < 0.001]) because of higher RV afterload (RVSP 42 ± 3 mm Hg vs 31 ± 3 mm Hg [P = 0.012]). FAC/RVSP (r = 0.61; P < 0.001) and TAPSE/RVSP (r = 0.69; P < 0.001) correlated with peak VO2 especially in the patients with impaired exercise capacity whereas FAC and TAPSE were independent of peak VO2. Similar comparisons between VPS and control groups showed no difference in TAPSE and FAC between groups, but lower FAC/RVSP and TAPSE/RVSP in the VPS group. CONCLUSIONS: There is abnormal RV-PA coupling in chronic PR, and noninvasively measured RV-PA coupling might potentially be prognostic because of its correlation with exercise capacity.

Cost Efficacy of α-Galactosidase A Enzyme Screening for Fabry Disease.

The prevalence of Fabry disease (FD) in adult patients with suspected hypertrophic cardiomyopathy (HCM) has been reported between 0.3% and 4%. Fabry disease-specific therapy necessitates early diagnosis; however, the optimal screening strategy and cost efficacy of routine α-galactosidase A (α-gal A) vs comprehensive galactosidase alpha gene (GLA) testing remain poorly understood. We identified 1192 patients who underwent routine α-gal A screening between January 1, 2011, and December 31, 2017, for suspected HCM. Cost efficacy was explored using prevalence and cost estimates. Ten patients had reduced α-gal A enzyme activity, and 5 (3 women) were ultimately diagnosed with FD (prevalence estimate, 0.42%). An alternative cardiac diagnosis was made in 3 patients with mildly reduced enzyme activity. Two women with reduced borderline enzyme levels did not undergo confirmatory testing, but FD was not suspected. The number needed to screen to diagnose 1 patient with FD in a similar cohort is estimated at 238 (5 new cases per 1192 at-risk individuals) at a cost of approximately US $24,000 per diagnosis. We identified a 0.42% prevalence of FD using routine α-gal A screening in adult patients referred to a dedicated HCM center in the United States. Compared with more comprehensive genetic testing strategies, we identified a similar prevalence of FD at a lower cost per diagnosis.

Echo-Doppler assessment of ventricular filling pressures in adult Fontan patients.

BACKGROUND: Diastolic dysfunction is felt to be part of the natural history of patients with prior Fontan operation. Despite that, data on noninvasive assessment of diastolic function and ventricular filling pressures in Fontan patients are limited. METHODS: We included 45 adult Fontan patients who underwent right heart catheterization with pulmonary artery wedge pressure (PAWP) measurement and transthoracic echo-Doppler assessment within 7 days. Offline measurement of systemic atrioventricular valve pulsed-wave (PW) and medial/lateral atrioventricular annular tissue Doppler velocities was performed and correlated to PAWP. RESULTS: Median age was 31.4 years (IQR 24.8-37); 51% of patients were females. Median ventricular ejection fraction was 55% (IQR 50.3-57.5) and median PAWP was 10 mm Hg (IQR 8-11). PW Doppler E velocity (r = 0.64, p ≤ 0.0001), deceleration time (r = -0.40, p = 0.008), and E/A ratio (r = 0.33, p = 0.03) correlated with PAWP. Median medial and lateral E/e' ratios also correlated to PAWP (r = 0.71, p = 0.002 and r = 0.75, p < 0.0001; respectively). An E velocity >75 cm/s had 88% sensitivity and 86% specificity [97% negative predictive value (NPV); 58% positive predictive value (PPV)], E/A ratio >1.7 had 100% sensitivity and 61% specificity (100% NPV; 26% PPV), and deceleration time <135 ms had 88% sensitivity and 83% specificity (97% NPV; 58% PPV) for predicting PAWP >12 mm Hg. CONCLUSION: Our data suggest that PW Doppler atrioventricular E velocity, E/A ratio, and deceleration time could be used to identify adult Fontan patients with normal filling pressures. However, PPVs for PAWP >12 mm Hg were poor. Further studies, particularly prospective, simultaneous echo-catheterization correlation, are critically needed.

Role of Doppler echocardiography for cardiac output assessment in Fontan patients.

BACKGROUND: To determine (1) correlation between Doppler stroke volume index (SVI) and cardiac magnetic resonance imaging (CMRI) SVI and (2) association between Doppler SVI and Fontan-associated diseases (FAD) and Fontan failure. METHODS: Review of Fontan patients who underwent same-day CMRI and transthoracic echocardiography (TTE), 2005 to 2015. We defined FAD as cardiac thrombus, protein-losing enteropathy, arrhythmia, and hospitalization for heart failure. Fontan failure was defined as Fontan conversion or revision, heart transplantation or listing, or death. RESULTS: Fifty-three patients with systemic left ventricle (LV) underwent 86 sets of TTE/CMRI. Mean (SD) age 31 (6) years. SVI (45 [16] vs 42 [13] mL/m2), CI (3.0 [1.1] vs 2.8 [0.8] L min-1 m-2), and ejection fraction (53 [4]% vs 51 [5]%) were similar for both modalities (P>.05 for all). Doppler SVI correlated with CMRI (r=0.68; P<.001). Sixteen patients had cirrhosis, and these patients had a higher CI (3.9 [0.9] vs 2.8 [1.0] L min-1 m-2; P<.01). Among the 37 patients without cirrhosis, Doppler SVI <39 mL/m2 was associated with FAD (odds ratio [OR], 2.11; 95% confidence limit, 1.26-3.14; P=.02); Fontan failure was more common in patients with CI was <2.5 L min-1 m-2 (3/9 [33%] vs 0/28 [0%], P=.01). Another 11 patients with systemic right ventricle (RV) underwent 17 sets of TTE/CMRI, mean (SD) age 17 (3) years, and CMRI SVI also correlated with Doppler SVI (r=0.75; P<.001). CONCLUSION: Doppler SVI correlated with CMRI SVI in patients with systemic LV and systemic RV. The association between output measures (SVI and CI) and FAD were seen only in single LV patients (single RV patients not assessed for this outcome due to small numbers). An association between low Doppler CI and Fontan failure was suggested in a small number of single LV patients.

Constrictive Pericarditis: A Practical Clinical Approach.

Constrictive pericarditis (CP) represents a form of severe diastolic heart failure (HF), secondary to a noncompliant pericardium. The true prevalence of CP is unknown but it is observed in 0.2-0.4% of patients who have undergone cardiac surgery or have had pericardial trauma or inflammation due to a variety of etiologies. Despite its poor prognosis if untreated, CP is a potentially curable disease and surgical pericardiectomy can now be performed at low perioperative mortality in tertiary centers with surgical expertise in pericardial diseases. Cardiologists should have a high index of suspicion for CP in patients presenting with predominant right-sided (HF), particularly when a history of cardiac surgery, pericarditis or pericardial effusion is present. Transthoracic two-dimensional and Doppler echocardiography is usually the first diagnostic tool in the evaluation of HF and can reliably identify CP in most patients by characteristic real-time motion of the heart and hemodynamic features. Computerized tomography and magnetic resonance imaging provide incremental data for the diagnosis and management of CP and are especially helpful when clinical or echocardiographic findings are inconclusive. Cardiac catheterization has been the gold-standard for the diagnosis of CP, but may not be necessary if non-invasive test(s) demonstrate diagnostic features of CP; it should then be reserved for selected cases or for assessment of concomitant coronary disease. Although most patients with CP require pericardiectomy, anti-inflammatory therapy may be curative in patients presenting with subacute symptoms, especially when evidence of marked ongoing inflammation is seen.