Incidence and management of the left ventricular outflow obstruction in patients with atrioventricular septal defects.

OBJECTIVES: Left ventricular outflow tract obstruction (LVOTO) is a recognized complication after complete repair of atrioventricular septal defect (AVSD). This study reviewed the incidence and management of LVOTO following AVSD repair at a single institution. METHODS: From 1975 to 2019, 24 patients (3.3%, 24/730) underwent reoperation due to LVOTO following partial AVSD (pAVSD) and complete AVSD (cAVSD) repair. The data were retrospectively reviewed. RESULTS: The incidence of LVOTO following pAVSD and cAVSD repair was 4.4% (12/275) and 2.6% (12/455). Freedom from LVOTO reoperation following pAVSD and cAVSD repair at 25 years was 94.3% [95% confidence interval (CI); 89.7-96.7] and 95% (95% CI; 91.1-97.3). The median time from complete repair of pAVSD and cAVSD to LVOTO reoperation was 4.4 years [interquartile range (IQR): 3.4-6.7] and 2.6 years (IQR: 2.2-4.7). Freedom from second LVOTO reoperation at 5, 10 and 15 years was 83.7% (95% CI; 57.2-98.2), 59.2% (95% CI; 28.7, 80.3) and 39.5% (95% CI; 13.2-65.3). The median time between the first and the second LVOTO reoperation in the groups of pAVSD and cAVSD was 6.1 years (IQR: 3.4-8.9) and 8.6 years (IQR: 5.7-9.8). There was no significant difference regarding the first (P = 0.7406) and subsequent LVOTO (P = 0.7153) following complete repair of pAVSD and cAVSD. Combined access to the left ventricular outflow tract was not protective regarding LVOTO reoccurrence. Survival for both groups after LVOTO reoperation at 15 years was 95.6% (95% CI 99.4-72.9). CONCLUSIONS: Incidence of LVOTO after AVSD repair is low but the reoccurrence rate is high. Standard subaortic resection does not always provide definitive LVOTO relief. The survival after LVOTO reoperation is excellent.

Concurring hypertrophic cardiomyopathy and takotsubo cardiomyopathy: Assessment and management.

The prevalence of takotsubo cardiomyopathy (TCM) has been on the rise, but co-occurrence with hypertrophic cardiomyopathy (HOCM) remains rare. Although presenting patient demographics were similar to those in TCM, the potential for hemodynamic compromise was significantly compounded by the presence of underlying HOCM. Management was similar to standalone TCM, although use of inotropic agents and mechanical support appears to be more prevalent. Despite the increased potential for complications and the paucity of data regarding management, outcomes appear to be mostly favorable in both the hospitalization period and at follow-up. Interestingly, despite a new diagnosis of HOCM in about half the cases described, which signifies no significant left ventricular outflow tract (LVOT) gradient prior to TCM, half of those patients had a persistently elevated LVOT gradient after resolution of TCM. This poses a question of whether or not TCM can predispose to LVOT obstruction in HOCM patients even after its resolution.

Novel Multiphase Assessment for Predicting Left Ventricular Outflow Tract Obstruction Before Transcatheter Mitral Valve Replacement.

OBJECTIVES: This study proposes a physiologic assessment of left ventricular outflow tract obstruction (LVOTO) that accommodates changes in systolic flow and accounts for the dynamic neo-left ventricular outflow tract (LVOT). BACKGROUND: Patients considered for transcatheter mitral valve replacement trials often screen-fail because of the perceived risk of LVOTO. In the Intrepid Global Pilot Study, assumed risk of LVOTO was based on computed tomography estimates of the neo-LVOT area computed at end-systole. However, this may overestimate actual risk. METHODS: Retrospective analyses were performed for screen-failed patients for potential LVOTO (n = 33) and treated patients (n = 29) with available dynamic computed tomography. A multiphase assessment of the neo-LVOT area was performed and represented as: 1) multiphase average; and 2) early systolic value. Prospective evaluation was performed in 9 patients approved for enrollment with multiphase and early systole methods that would have previously screen-failed with the end-systolic approach. RESULTS: Of 166 patients screened for possible inclusion; 32 were screen-failed for nonanatomical reasons. Screen failure for assumed LVOTO risk occurred in 37 of 134 (27.6%) patients. Retrospective analysis indicated a potential enrollment increase of 11 of 33 (33.3%) and 18 of 33 (54.5%) patients using multiphase and early systolic assessment methods. In the prospective cohort, there were no clinical observations of LVOTO 30 days post-procedure, despite assumed risk based on end-systolic estimates. CONCLUSIONS: Multiphase, and specifically early systolic, assessment of the neo-LVOT may better determine risk of LVOTO with transcatheter mitral valve replacement compared with end-systolic estimates. This novel approach has the potential to significantly increase patient eligibility, with over one-half of patients previously screen-failed now eligible for treatment.

The advantages of live/real-time three-dimensional echocardiograhy in the assessment of left ventricular myxoma, which causes partial left ventricular outflow tract obstruction.

Myxomas are the most common cardiac primary tumors; however, left ventricular myxomas are extremely rare. We describe a young female patient with a giant left ventricular myxoma causing partial obstruction of the left ventricular outflow tract, who underwent successful surgical resection. Real-time three-dimensional echocardiography yielded incremental value to two-dimensional echocardiography by allowing better assess of the true size, extent, attachment, and morphology of the tumor. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 46:273-277, 2018.

Recommendations on the Echocardiographic Assessment of Aortic Valve Stenosis: A Focused Update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Echocardiography is the key tool for the diagnosis and evaluation of aortic stenosis. Because clinical decision-making is based on the echocardiographic assessment of its severity, it is essential that standards are adopted to maintain accuracy and consistency across echocardiographic laboratories. Detailed recommendations for the echocardiographic assessment of valve stenosis were published by the European Association of Echocardiography and the American Society of Echocardiography in 2009. In the meantime, numerous new studies on aortic stenosis have been published with particular new insights into the difficult subgroup of low gradient aortic stenosis making an update of recommendations necessary. The document focuses in particular on the optimization of left ventricular outflow tract assessment, low flow, low gradient aortic stenosis with preserved ejection fraction, a new classification of aortic stenosis by gradient, flow and ejection fraction, and a grading algorithm for an integrated and stepwise approach of aortic stenosis assessment in clinical practice.

Septal Ablation and Hypertrophic Obstructive Cardiomyopathy: 7 Years US Experience.

BACKGROUND: Septal ablation (SA) is a key modality for left ventricular outflow tract gradient reduction in hypertrophic obstructive cardiomyopathy (HOCM) patients with refractory symptoms. The primary objective of our study was to evaluate post-procedural mortality, complications, length of stay (LOS), and cost of hospitalization following SA. METHODS: We queried the Nationwide Inpatient Sample (NIS) between 2005 and 2011 using the ICD9 procedure code of 37.34 for ablation of heart tissue. Only adult patients with HOCM (ICD-9-CM: 425.1) were included. Patients with any arrhythmia diagnosis or open surgical ablation procedure code were excluded. Hierarchical mixed effects models were generated in order to identify the independent multivariate predictors of outcomes. RESULTS: A total of 358 SAs were available for analysis. There was no reported mortality during the study period; permanent pacemaker implantation rate was 8.7%. Highest hospital volume tertile (OR, 95%CI, P- value) predicted significantly lower post-procedural complications (0.51, 0.26-0.98, P = 0.04). Univariate analysis of highest versus lowest tertile of hospital volume showed significant decrease in LOS (2.6 days vs. 3.8 days, P<0.01) and non-significant decrease hospitalization costs (16,800$ vs. 19,500$, P = 0.29). CONCLUSIONS: SA is a safe procedure and associated with low peri- procedural mortality rate. A higher burden of baseline comorbidities is associated with worse outcomes while higher annual hospital volume is associated with lower rate of post-procedural complications, length of stay, and cost of care following SA.

Effect of the ellipsoid shape of the left ventricular outflow tract on the echocardiographic assessment of aortic valve area in aortic stenosis.

BACKGROUND: Previous studies showed discrepancies between echocardiographic and multidector row CT (MDCT) measurements of aortic valve area (AVA). OBJECTIVE: Our aim was to evaluate the effect of the ellipsoid shape of the left ventricular outflow tract (LVOT), as shown and measured by MDCT, on the assessment of AVA by transthoracic echocardiography (TTE) in patients with severe aortic stenosis. METHODS: This retrospective single-center study involved 49 patients with severe aortic stenosis referred before transcatheter aortic valve implantation. The AVA was deduced from the continuity equation on TTE and from planimetry on cardiac MDCT. Area of the LVOT was calculated as follows: on TTE, from the measurement of LVOT diameter on parasternal long-axis view; on MDCT, from manual planimetry by using multiplanar reconstruction perpendicular to LVOT. RESULTS: At baseline, correlation of TTE vs MDCT AVA measurements was moderate (R = 0.622; P < .001). TTE underestimated AVA compared with MDCT (0.66 ± 0.15 cm2 vs. 0.87 ± 0.15 cm2; P < .001). After correcting the continuity equation with the LVOT area as measured by MDCT, mean AVA drawn from TTE did not differ from MDCT (0.86 ± 0.2 cm2) and correlation between TTE and MDCT measurements increased (R = 0.704; P < .001). CONCLUSION: Assuming that LVOT area is circular with TTE results in constant underestimation of the AVA with the continuity equation compared with MDCT planimetry. The elliptical not circular shape of LVOT largely explains these discrepancies.

Assessment of the ductus arteriosus in fetuses with tetralogy of Fallot and the implication for postnatal management.

OBJECTIVE: To describe the antenatal and neonatal echocardiographic morphology and flow pattern of the ductus arteriosus in patients with tetralogy of Fallot. PATIENTS AND METHODS: We included patients with a prenatal diagnosis of tetralogy of Fallot between January 2006 and December 2012. RESULTS: Among the 52 fetuses with tetralogy of Fallot the severity of right ventricular outflow obstruction was considered mild in 32, moderate in 14, and severe in 6. In the mild right ventricular outflow obstruction group (n = 32) all had normal ductal morphology and flow pattern, eight (25%) elected for termination of pregnancy and two died in the neonatal period from extracardiac causes. In the moderate right ventricular outflow obstruction group (n = 14) the fetuses had a small ductus arteriosus with antegrade but abnormal flow velocity, one (7%) elected for termination of pregnancy. Immediately after birth the ductus arteriosus was very small or already closed at echocardiographic examination. Two out of 13 patients (15%) developed severe hypoxic spells and underwent modified Blalock-Taussig shunt during the neonatal period. Six fetuses were considered to have severe right ventricular outflow obstruction with flow reversal in the ductus arteriosus, three (50%) of whom elected for termination of pregnancy. The other three newborns underwent modified Blalock-Taussig shunt. CONCLUSION: In fetuses with tetralogy of Fallot, ductal diameter can be reduced even up to prenatal closure. Prenatal ductal morphology assessment may be useful for improving management of patients with moderate right ventricular outflow obstruction and small ductus arteriosus who may become cyanotic at birth.

Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment.

Adult patients with congenital heart disease (ACHD) represent a growing population due to progress in management. Surgical procedures generally fall short of restoring entirely normal anatomical and functional relations. Further procedures can be needed and lifelong follow-up is required. The right ventricle (RV) plays an important role in congenital heart disease and cardiac magnetic resonance (CMR) imaging has become the imaging method of choice for its assessment. CMR can provide relatively accurate measurements of RV volume and function, and arterial flow, with additional anatomical information provided by three-dimensional contrast angiography and late gadolinium imaging of fibrosis. Here we focus our review on three categories of ACHD in which evaluation of the RV is important: repaired tetralogy of Fallot, the systemic RV and Ebstein anomaly. We demonstrate how CMR contributes to decision-making regarding the types and timings of interventions. A dedicated CMR service should be regarded as a necessary facility of a centre specializing in the care of ACHD patients.

Exercise stress echocardiographic assessment of outflow tract and ventricular function in patients with an obstructed right ventricular-to-pulmonary artery conduit after repair of conotruncal heart defects.

This study assessed right ventricular (RV) and RV outflow tract (RVOT) function and pressure in response to exercise in patients with an obstructed RV-pulmonary artery (PA) conduit using exercise stress echocardiography (ESE) to evaluate these parameters. RV-PA conduits inevitably develop stenosis and/or regurgitation over time. Assessment of conduit obstruction only at rest may not reveal the extent of physiologic perturbation related to RV pressure loading. Patients with a stenotic RV-PA conduit who were being considered for transcatheter pulmonary valve placement were approached prospectively. ESE was performed and ventricular images were obtained at rest and at peak exercise. Forty patients (median age 17 years) were enrolled. Most patients had tetralogy of Fallot (63%) and were in New York Heart Association class II (59%). Exercise stress echocardiographic images were adequate in 38 patients (95%). With exercise there was a significant increase in maximum instantaneous RVOT gradient from rest (59 vs 96 mm Hg, p <0.001); exercise-induced change in RVOT gradient correlated with global RV strain at rest (r = -0.3, p = 0.05). Compared to measurements at rest there were significant increases in median peak longitudinal strain of the left ventricular free wall, interventricular septum, and global left ventricular strain at peak exercise. There were no significant changes in median RV strain at peak exercise (RV free wall -14.3 [-26, -8] at rest vs -15.2 [-27, -3] at peak exercise, p = 0.87; global RV strain -13.9 [-32, -9] vs -15.1 [-23, -6], p = 0.11). In conclusion, using ESE it was possible to evaluate abnormal ventricular function and conduit dysfunction at peak exercise in patients with an obstructed RV-PA conduit.

Assessment of left ventricular outflow gradient: hypertrophic cardiomyopathy versus aortic valvular stenosis.

OBJECTIVES: This study examined the relationship between peak-to-peak (common invasive measurement), peak instantaneous (common Doppler measurement), and mean pressure gradients in patients with hypertrophic cardiomyopathy (HCM) and aortic stenosis (AS). BACKGROUND: In patients with AS, the peak-to-peak gradient and peak instantaneous gradient are discrepant, and the mean gradient best represents obstruction severity. The pathophysiology of outflow obstruction differs in HCM, with the maximum gradient occurring in late systole, thus the optimal method for quantifying gradient severity in HCM remains undefined. METHODS: Fifty patients with HCM and 50 patients with AS underwent gradient characterization at cardiac catheterization (age 55 ± 15 years vs. 72 ± 9 years; 48% vs. 42% male, respectively). All HCM patients were studied with high-fidelity, micromanometer-tip catheters and transseptal measurement of left ventricular inflow and central aortic pressures. In AS, simultaneous left ventricular and central aortic pressures were recorded. RESULTS: The peak instantaneous gradient was linearly correlated with peak-to-peak gradient in HCM (R(2) = 0.98, p < 0.0001), with the relationship close to the line of identity. In AS, more scatter and further deviation from the line of identity occurred when comparing the peak instantaneous gradient to the peak-to-peak gradient (R(2) = 0.70, p < 0.0001). Both peak-to-peak and peak instantaneous gradients were consistently higher than the mean gradient in HCM, with wide 95% confidence limits of agreement (26.7 ± 46.5 mm Hg and 16.4 ± 47.2 mm Hg, respectively). CONCLUSIONS: In HCM, peak instantaneous and peak-to-peak gradient demonstrate excellent correlation. Consequently, both peak instantaneous and peak-to-peak gradients can be used to classify obstruction severity in HCM. By contrast, the mean gradient should direct clinical management in AS.

Subaortic stenosis missed by invasive hemodynamic assessment.

We present a case of 61-year-old man that was evaluated for possible aortic stenosis but did not show a left ventricular outflow gradient on invasive assessment in the catheterization laboratory. Transthoracic echocardiography showed subaortic stenosis secondary to a discrete membranous structure in the left ventricular outflow tract. This is the first case in the literature of a patient with discrete subaortic stenosis missed by invasive hemodynamic assessment.

Prevalence of pannus formation after aortic valve replacement: clinical aspects and surgical management.

Pannus formation after aortic valve replacement is not common, but obstruction due to chronic pannus is one of the most serious complications of valve replacement. The causes of pannus formation are still unknown and effective preventive methods have not been fully elucidated. We reviewed our clinical experience of all patients who underwent reoperation for prosthetic aortic valve obstruction due to pannus formation between 1973 and 2004. We compared the initial 18-year period of surgery, when the Björk-Shiley tilting-disk valve was used, and the subsequent 13-year period of surgery, when the St. Jude Medical valve was used. Seven of a total of 390 patients (1.8%) required reoperation for prosthetic aortic valve obstruction due to pannus formation. All seven patients were women; four patients underwent resection of the pannus and three patients needed replacement of the valve. The frequency of pannus formation in the early group was 2.4% (6/253), whereas it was 0.73% (1/137) in the late group (P < 0.05). Pannus was localized at the minor orifice of the Björk-Shiley valve in the early group and turbulent transvalvular blood flow was considered to be one of the important factors triggering its growth. We also consider that small bileaflet valves have the possibility of promoting pannus formation and that the implantation of a larger prosthesis can contribute to reducing the occurrence of pannus.

[The role of transesophageal echocardiography (TEE) in assessment of left ventricular tract obstruction]. / Rola echokardiografii przezprzelykowej (TEE) w diagnostyce podzastawkowego zwezenia aorty.

Twelve children with subaortic stenosis were analysed. Nine of them developed left ventricular outflow obstruction after surgery for congenital heart disease (VSD + IAA, VSD + DORV, VSD + TGA, VSD + CoA) and then developed an isolated form of primary stenosis. Both echocardiographic exams, TTE and TEE were performed in all patients. TTE was sufficient to assess isolated subaortic stenosis. In children after cardiosurgery, TEE was more reliable and provided more detailed visualisation of the stenosis and its relationship to surrounding structures.

Echocardiographic assessment of subvalvular aortic stenosis before and after operation.

The development of two-dimensional and Doppler echocardiography has provided a noninvasive technique for the diagnosis and serial assessment of patients with subvalvular aortic stenosis. The clinical records and echocardiographic data were reviewed of all patients with subaortic stenosis diagnosed between 1983 and 1991. Of the 77 patients identified (45 male and 32 female), 28 had isolated subaortic stenosis and 49 had associated cardiac lesions. The most frequently encountered associated lesions were ventricular septal defect (n = 19) and coarctation of the aorta/interrupted aortic arch (n = 14). Serial echocardiographic studies, performed in 38 of the 77 patients, documented significant progression of the left ventricular outflow tract gradient in 25 patients (66%) and development of aortic regurgitation in 25 patients (66%). Surgical resection was performed in 36 patients. The preoperative outflow tract peak gradient was 62.9 +/- 31 mm Hg (range 0 to 153), whereas the immediate postoperative gradient was 14.4 +/- 14 mm Hg (range 0 to 67). The two patients with a significant residual gradient (37 and 67 mm Hg, respectively) in the immediate postoperative period had severe subaortic stenosis preoperatively with marked left ventricular hypertrophy and intracavitary gradient. The immediate postoperative echocardiograms demonstrated no worsening of aortic regurgitation in any patient and regression of regurgitation in one patient from mild to none. Intermediate-term follow-up studies were available for review in 13 postoperative patients at a mean of 4 years postoperatively. In 2(15%) of these 13 patients, subaortic stenosis recurred; however, the degree of aortic regurgitation did not increase in any patient.(ABSTRACT TRUNCATED AT 250 WORDS)