Challenge in mitral valve intervention: Super-elongated anterior and diminutive posterior mitral valve.

Diminutive posterior mitral valve leaflet with an extremely elongated anterior leaflet is a rare phenomenon. We share such a case discovered in an 82-year-old patient with severe, symptomatic mitral regurgitation. LA, left atrium; LV, left ventricle; MR, mitral regurgitation.

Hypertrophic Cardiomyopathy-Septal Perforator Arteries Doppler Flow Dynamics.

BACKGROUND: Patients with hypertrophic cardiomyopathy (HCM) have been shown to exhibit abnormal diastolic vessel flow; however, flow pattern profiles and their possible association with different grades of diastolic dysfunction have not been studied. Color Doppler two-dimensional echocardiography permits visualization of the septal perforator arteries, and pulsed-wave Doppler allows recording of diastolic septal artery flow (SAF). Through routine visualization of the septal perforator arteries and acquisition of SAF, we noticed 3 patterns of SAF in patients with HCM. In this study, we aimed to assess the feasibility of the acquisition of SAF and to describe types of SAF in an HCM cohort and their associations with diastolic function. METHODS: We reviewed two-dimensional echocardiograms and the electronic records of 108 patients with HCM in whom septal artery color and spectral Doppler had been performed. The peak diastolic and end-diastolic velocities, diastolic slope, diastolic flow time-velocity integral, and systolic flow reversal of the septal perforator arteries were recorded with pulsed-wave Doppler. Echocardiographic and clinical characteristics were analyzed. RESULTS: A reproducible pulsed-wave Doppler tracing was recorded in 54% of patients with HCM. Three distinct patterns of SAF were identified: type 1-smooth, linear holodiastolic velocity decrease; type 2-with presence of an atrial dip; and type 3-biphasic velocity decrease with an early, rapid diastolic slope and a mid-to-late gentle slope. These 3 SAFs correlated with different grades of diastolic dysfunction. CONCLUSION: Septal artery flow could be detected in more than 50% of patients with HCM. Three distinct types of SAF were identified, correlating with different grades of diastolic dysfunction. These 3 types of SAF can provide additional information about left ventricular end-diastolic pressure and diastolic function in patients with HCM in whom diastolic function may be difficult to determine.

Massive Left Ventricular Hypertrophy With Acromegaly: Hypertrophic Obstructive Cardiomyopathy Phenocopy or Phenotype?

Acromegaly-a rare endocrine disorder-results when a growth hormone-secreting somatotroph pituitary adenoma leads to increased insulin-like growth factor 1 production. Acromegaly is known to cause left ventricular hypertrophy. We present a case of acromegaly with massive left ventricular hypertrophy that was determined to be coexistent with gene-positive hypertrophic obstructive cardiomyopathy. (Level of Difficulty: Intermediate.).

Myocardial work indices in bileaflet mitral valve prolapse patients.

AIMS: Mechanisms underlying left ventricular dysfunction and arrhythmogenesis in bileaflet mitral valve prolapse (BMVP) patients are not well defined. Myocardial work index (MWI) is a noninvasive assessment that correlates with myocardial oxygen consumption. We aimed to compare global and regional MWI in BMVP patients with normal controls. METHODS AND RESULTS: In this retrospective study, we calculated MWI in BMVP patients and controls using GE EchoPAC (GE Healthcare, Chicago, IL) software. Of 147 BMVP patients (59% women, mean age 54 ± 15 years), 16 had a flail mitral leaflet. There was regional heterogeneity in MWIs, with increased posterolateral trident myocardial work (2099 ± 271 vs. 1895 ± 321 mm Hg%, P = .039), constructive work (2831 ± 366 vs. 2257 ± 338 mm Hg%, P < .001), wasted work (87 [52-194] vs. 71 [42-103] mm Hg%, P = .015), peak systolic strain (-23.0 ± 2.4 vs. -19 ± 3%, P < .001), and reduction in myocardial work efficiency (95.00 [93.50-97.75] vs. 96.75 (95.00-97.75) %, P = 0.020) in 100 BMVP patients compared with age- and sex-matched controls. BMVP patients' basal septal wall MWIs were lower than those of controls. The higher work indices in patients with BMVP were reduced in those who developed flail leaflets. No significant differences in work indices were seen between ventricular arrhythmia and non-arrhythmia BMVP patients. CONCLUSION: Regional differences in MWIs were noted in the BMVP patients compared with controls, with overall reduced myocardial efficiency in the posterolateral trident and basal septal regions. In cross-sectional analysis, MWIs were not different in patients with ventricular tachyarrhythmias. Impact of MWI in long-term prognosis needs to be determined.

Barlow's mitral valve with a dancing chorda.

We present a case of a ruptured mitral valve chorda visualized using the high temporal and axial resolution of transthoracic M-mode echocardiography.

Prevalence of dilated mid-ascending aorta in individuals 15 years and older: In search of optimal diagnostic criteria and their effect on the burden of disease.

BACKGROUND: Controversy regarding the definition of the upper limit of normal (ULN) for dilated mid-ascending aorta (mAA) stems from variation in criteria, based on several small-sized studies with small datasets of normal subjects (DONS). The present study was carried out to demonstrate this variation in the prevalence of mAA dilation and to identify the optimal definition by creating the largest DONS. METHODS: Echocardiographic studies of patients ≥ 15 years of age performed at a large tertiary care center over 4 years (n = 49,330) were retrospectively evaluated. The leading-edge-to-leading-edge technique was used to measure the mAA in diastole. The largest-to-date DONS (n = 2334) was created, including those who were normal on medical record review, did not have any of the 28 causes of dilated aorta, and had normal echocardiograms. Because age had the strongest correlation with mAA (multivariate adjusted R2 = 0.26), as compared with sex, height, and weight, we created a new ULN based on the DONS with narrow age stratification (10-year intervals). RESULTS: The prevalence of dilated mAA varied between 17% and 23% when absolute criteria were used with sex stratification, and it varied between 6% and 11% when relative criteria (relative to age, body surface area, and sex) were used. Based on new criteria from the DONS, it was 7.6%, with a ULN of 3.07-3.64 cm in women and 3.3-3.91 cm in men. CONCLUSIONS: These data demonstrate the undesirable variation in the prevalence of dilated mAA based on prior criteria and propose a new ULN for dilated mAA.

Age-Based Outcomes in Patients Who Underwent Septal Reduction Therapy.

There are no national data on age-based outcomes of septal reduction therapy. Using the National Inpatient Sample, we included all adult patients who underwent septal myectomy (SM) or alcohol septal ablation (ASA) from 2005 to 2019. The primary objective was to evaluate the in-hospital mortality and new permanent pacemaker (PPM) placement after SM and ASA in 3 age groups. In total, 9,564 patients underwent SM and 5,084 underwent ASA. Compared with the age group 18 to 39 years, the odds of in-hospital mortality after SM were similar in age group 40 to 64 years and 4.46 times higher than in age group ≥65 years; the higher mortality in the older group was explained by higher co-morbidity burden on the risk-adjusted analysis. Furthermore, compared with age group 18 to 39 years, the odds of new PPM placement after SM were higher in the age groups 40 to 64 years and ≥65 years, despite the risk adjustment (adjusted odds ratio [AOR] 3.17, 95% confidence interval [CI] 1.33 to 7.58 and AOR 4.39, 95% CI 1.78 to 10.8, respectively). The odds of in-hospital mortality after ASA were similar in age groups 65 to 79 years and 18 to 64 years. However, the odds of in-hospital mortality were higher in the age group ≥80 years than in the age group 18 to 64 years, although this difference were not present after risk adjustment. The odds of new PPM after ASA were higher for the age groups 65 to 79 years and ≥80 years than age group 18 to 64 years, despite the risk adjustment (AOR 1.78, 95% CI 1.22 to 2.60 and AOR 3.10, 95% CI 2.09 to 6.57, respectively). Finally, we also estimated these absolute risks in different age groups. In conclusion, this national data will inform health care providers to better understand the aged-based risks of outcomes after septal reduction therapy.

Survival Into the Seventh Decade of Life Following Mustard Repair.

Atrial switch procedures (Senning and Mustard) for transposition of the great arteries have largely been abandoned for arterial switch procedures. The number of surviving patients who have undergone atrial switch procedures is declining. We present a case of the oldest known survivor (aged 67 years) of the Mustard procedure. (Level of Difficulty: Beginner.).

Myocardial Work in Apical Hypertrophic Cardiomyopathy.

BACKGROUND: Pressure-strain loop analysis is a novel echocardiographic technique to calculate myocardial work indices that has not been applied to patients with apical hypertrophic cardiomyopathy (ApHCM). We hypothesized that myocardial work indices differ between patients with ApHCM and those with non-ApHCM. This study aimed to (1) evaluate myocardial work indices in patients with ApHCM compared with those with non-ApHCM, (2) describe associations with relevant clinical variables, and (3) examine associations with significant late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging. METHODS: We retrospectively identified 48 patients with ApHCM and 69 with non-ApHCM who had measurements of global longitudinal strain (GLS), global work index (GWI), global constructive work (GCW), global wasted work, and global work efficiency. We evaluated available cardiac magnetic resonance imaging data on 34 patients with ApHCM and 51 with non-ApHCM. Multivariable regression models correcting for traditional cardiac risk factors were used to evaluate the associations of myocardial work indices with relevant clinical variables. RESULTS: Median GLS (-11% vs -18%, P < .001), GWI (966 mm Hg% vs 1803 mm Hg%, P < .001), and GCW (1,050 mm Hg% vs 1,988 mm Hg%, P < .001) were significantly impaired in patients with ApHCM compared with those with non-ApHCM. Increasing N-terminal pro b-type natriuretic peptide, abnormal ultrasensitive troponin, and increasing maximal left ventricular wall thickness were significantly associated with reduced GWI and GCW in patients with ApHCM (P < .05). Global constructive work had only modest accuracy (area under the curve [AUC] = 0.70) to predict LGE in patients with ApHCM. However, in patients with non-ApHCM, GLS was the strongest predictor of LGE (AUC = 0.91), with a -17% cutoff yielding 81% sensitivity and 80% specificity. CONCLUSION: Myocardial work indices are significantly impaired in patients with ApHCM compared to those with non-ApHCM and correlate with important clinical variables. Global longitudinal strain, GWI, and GCW are more strongly predictive of fibrosis in patients with non-ApHCM than ApHCM.

Myocardial Work in Echocardiography.

Myocardial work is an emerging tool in echocardiography that incorporates left ventricular afterload into global longitudinal strain analysis. Myocardial work correlates with myocardial oxygen consumption, and work efficiency can also be assessed. Myocardial work has been evaluated in a variety of clinical conditions to assess the added value of myocardial work compared to left ventricular ejection fraction and global longitudinal strain. This review showcases the current use of myocardial work in adult echocardiography and its possible role in cardiac pathologies.

Hypertrophic cardiomyopathy is associated with dilated sinus of Valsalva: A case-control study.

Background: We aimed to test the hypothesis that there is an association between hypertrophic cardiomyopathy and dilated aorta in a case-control, matched-design fashion. Methods: Of 65,843 studies done from November 2011 to December 2015, we found, after detailed evaluation by a single author, 153 cases of hypertrophic cardiomyopathy and 3,213 controls who were classified as normal clinically and echocardiographically. Controls were defined as normal patients referred to the echocardiography laboratory with no diagnoses and no known risk factors for dilated aorta (e.g., aortic stenosis, hypertension, aortic regurgitation). Clinical chart review showed none of the risk factors for dilated aorta, and echocardiography did not reveal any abnormalities. Of these 3,213 patients, 153 controls were matched to cases by age and sex by propensity score. Dilated aorta was defined according to clinical, Goldstein, and Lang's criteria. Results: The prevalence of a dilated sinus of Valsalva was 9 times higher in hypertrophic cardiomyopathy patients than controls (OR = 9.4, P = 0.003). The 9-fold higher prevalence in hypertrophic cardiomyopathy patients persisted after adjusting for height, weight, and aortic pathology. Association of dilated mid-ascending aorta with hypertrophic cardiomyopathy was significant after adjustment for height and body surface area but became borderline insignificant after adjusting for weight and aortic valve pathology. Conclusion: Hypertrophic cardiomyopathy appears to be associated with a dilated sinus of Valsalva, even after adjusting for height, weight, and aortic valve pathology.

Regional Disparities in the Use of Septal Reduction Therapy and Associated Outcomes in the United States (from a Real-World Database).

The regional differences in the use of septal reduction therapies and the associated outcomes in patients with Hypertrophic obstructive cardiomyopathy (HOCM) are unknown. The primary objective of our study was to evaluate the regional disparities in the use of septal reduction therapies, including septal myectomy and alcohol septal ablation, in patients with HOCM. The secondary objective was to analyze the regional differences in the outcomes in these patients. Patients with HOCM had 87% higher risk-adjusted odds of getting septal myectomy (adjusted odds ratio 1.87, p = 0.03) and 37% lower risk-adjusted odds of getting alcohol septal ablation (adjusted odds ratio 0.63, p = 0.03) in the Midwest than in the Northeast. The in-hospital mortality rate was higher for patients who underwent septal myectomy in the South versus the Northeast on the unadjusted analysis. These differences persisted despite the adjustment for demographic and clinical characteristics. Additional adjustment for hospital volume partially explained these disparities, but the adjustment for both hospital volume and hospital teaching status completely explained these disparities. The risk-adjusted in-hospital mortality in patients who underwent alcohol septal ablation was similar in the South versus other regions. In conclusion, regional disparities may exist in the use of septal myectomy and alcohol septal ablation, and patients with HOCM should be referred to high-volume teaching hospitals for septal myectomy for better outcomes, which may also eliminate the extra burden of hospital mortality in the South.

Case report: Metastatic melanoma masquerading as apical hypertrophic cardiomyopathy.

Background: Cardiac tumors are usually metastatic. Melanoma is the tumor with the highest rate of cardiac metastasis. Clinicians need to be aware of the metastatic involvement of the left ventricular apex as a differential diagnosis of apical hypertrophic cardiomyopathy. Case summary: A 74-year-old woman presented for evaluation of fatigue. The initial electrocardiogram and echocardiogram showed features of apical hypertrophic cardiomyopathy. The patient reported a lesion on her right forearm that had been present for many years, leading to its biopsy, which showed melanoma. Further evaluation with a chest-computed tomography (CT) scan showed left lung nodules and nodular thickening of the left ventricular apex. Positron emission tomography showed an increased uptake of fluorodeoxyglucose in the left lung nodule and left ventricular apex, suggestive of metastatic spread of the melanoma. A CT-guided biopsy of the left lung nodule revealed melanoma. The patient was treated with ipilimumab initially, followed by paclitaxel with poor response to treatment, and later passed under hospice care. Conclusion: Metastatic tumors involving the left ventricular apex should be considered in the differential diagnosis of apical hypertrophic cardiomyopathy, especially in patients with a history of melanoma, and advanced cardiac imaging, including cardiac magnetic resonance imaging, CT, and/or positron emission tomography (PET) may help with narrowing down the differential diagnosis.

Refining the upper limit of normal for the ascending aorta: In search of optimal criteria -- a large database study of normal individuals.

OBJECTIVES: The cutoff for dilated mid-ascending aorta (mAA) is controversial and has several definitions. The present study was carried out to determine the prevalence of mAA dilation based on published definitions and to identify the optimal cutoff. METHODS: Echocardiographic studies of patients >15 years of age performed at a large tertiary care center over 4 years, n = 49,330, were retrospectively evaluated. Leading-edge-to-leading-edge technique was used to measure the mAA in diastole. Several cutoff criteria were included. In addition, we defined normals in our database as those who, after 28 causes of dilated aorta were excluded, were normal both clinically and echocardiographically (n = 2334). RESULTS: The mean age was 64.2 ± 17.1 years, and 31.5% were men. The prevalence of dilated mAA based on absolute criteria with sex stratification varied between 17% and 23% and based on relative criteria (to age, body surface area, and sex) varied between 6% and 11%. It further decreased to 7.6% on the addition of narrow age stratification (10 year intervals) performed on normals in our database. The multivariate adjusted R2 (for variation in mAA diameter) was 0.25 for age, decreasing to 0.12 for weight and 0.07 for sex and height. CONCLUSIONS: The lowest prevalence of 7.6% probably represents the optimal cutoff for dilated mAA because it includes age, which explains most of the variation in mAA, in narrow (10 year) intervals only performed in our normals, which represents the largest sample size to date.

Monckeberg calcification of coronary arteries: a string of pearls.

Coronary artery calcium scores are derived from cardiac-gated noncontrast computed tomography scans that are used in cardiac risk stratification. However, an elevated calcium score does not always translate to coronary artery luminal obstruction. Our case demonstrates an extremely high coronary artery calcium score despite nonobstructive coronaries on angiogram.