Abstract 13876: Squat-to-stand Provocation of Dynamic Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Lillian Peng ◽  
Darrell B Newman ◽  
Jeffrey B Geske
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Important Determinant ◽  
Valsalva Maneuver ◽  
Cost Savings ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Amyl Nitrite ◽  
Left Ventricular Outflow

Background: Left ventricular outflow tract (LVOT) obstruction is an important determinant in management of hypertrophic cardiomyopathy (HCM). Valsalva maneuver and amyl nitrite are used in clinical practice during resting echocardiography to induce latent obstruction (LVOT gradient <30 mmHg at rest, ≥30 mmHg with maneuver). With a nationwide shortage of amyl nitrite in 2019, we implemented a “repetitive squat-to-stand” maneuver to provoke LVOT obstruction during echocardiography. Methods: Patients with known or suspected HCM without resting obstruction who underwent repetitive squat-to-stand maneuver during echocardiography between February and September 2019 were included. Maximal instantaneous gradients were recorded. Provocable gradients were compared utilizing a two-sided t-test. Amyl nitrite was not available during the study period. Results: Squat-to-stand assessments were available in 125 patients without resting obstruction (age 58 ± 15 years, 67% male); 114 also performed Valsalva maneuver. 47 of 125 patients (37.6%) demonstrated provocable obstruction. 46 (36.8%) demonstrated latent obstruction provoked by squat-to-stand, which was severe (gradient ≥50 mmHg) in 29 (23.2%). Of the 46 patients, 19 demonstrated latent obstruction only with squat-to-stand but not with Valsalva, 13 of which demonstrated severe obstruction. Among patients with latent obstruction who performed both Valsalva and squat-to-stand maneuver (n=41), squat-to-stand resulted in a higher gradient than Valsalva maneuver (p<0.0001, Figure). Conclusion: Repetitive squat-to-stand maneuver is a clinically useful adjunct to the traditional echocardiographic exam in HCM. This maneuver appears to be more sensitive than the Valsalva maneuver for identifying severe dynamic LVOT obstruction. Use of repetitive squat-to-stand warrants further study in comparison to amyl nitrite, particularly regarding cost savings and diagnostic performance.

scholarly journals Squat-to-stand provocation of dynamic left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: a case report

2020 ◽  
Author(s):  
Lillian T Peng ◽  
D Brian Newman ◽  
Jeffrey B Geske
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Valsalva Manoeuvre ◽  
Pharmacologic Therapy ◽  
Alcohol Septal Ablation ◽  
Amyl Nitrite ◽  
Left Ventricular Outflow

Abstract Background Left ventricular outflow tract (LVOT) obstruction is an important determinant of the management of hypertrophic cardiomyopathy (HCM). With a nationwide shortage of amyl nitrite in 2019, we implemented a ‘repetitive squat-to-stand’ manoeuvre to provoke LVOT obstruction during echocardiography. Case summary A 64-year-old female was referred with symptomatic HCM refractory to pharmacologic therapy. Transthoracic echocardiography showed minor LVOT obstruction with conventional imaging at rest and during Valsalva manoeuvre, but severe obstruction was confirmed with the repetitive squat-to-stand manoeuvre. Alcohol septal ablation via the first septal perforator was performed with subsequent resolution of symptoms. Discussion Due to the dynamic nature of LVOT obstruction, a series of provocative manoeuvres including Valsalva manoeuvre, inhalation of amyl nitrite, and exercise are often necessary to maximally augment ventricular obstruction. The recent unavailability of amyl nitrite during a nationwide shortage prompted the implementation of a protocol of repetitive squat-to-stand manoeuvre in our echocardiography laboratory. Rising from the squatting position decreases preload and afterload, both of which augment dynamic LVOT obstruction. Repetition of squatting and standing appears to enhance the sensitivity of the manoeuvre, particularly when exertional symptoms are reproduced. In this case, repetitive squat-to-stand manoeuvre led to the identification of severe LVOT obstruction which may not have been diagnosed otherwise, alteration of treatment to septal reduction therapy, and subsequent resolution of symptoms.

scholarly journals Characteristics and Outcomes of Elderly Patients With Hypertrophic Cardiomyopathy

2021 ◽  
Vol 10 (3) ◽  
Author(s):  
Alaa Alashi ◽  
Nicholas G. Smedira ◽  
Zoran B. Popovic ◽  
Agostina Fava ◽  
Maran Thamilarasan ◽  
...  
Keyword(s):  
Risk Factors ◽  
Hypertrophic Cardiomyopathy ◽  
Elderly Patients ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Alcohol Ablation ◽  
Left Ventricular Outflow ◽  
Sudden Cardiac Death Risk

Background We report characteristics and outcomes of elderly patients with hypertrophic cardiomyopathy (HCM) with basal septal hypertrophy and dynamic left ventricular outflow tract obstruction. Methods and Results We studied 1110 consecutive elderly patients with HCM (excluding moderate or greater aortic stenosis or subaortic membrane, age 80±5 years [range, 75–92 years], 66% women), evaluated at our center between June 2002 and December 2018. Clinical and echocardiographic data, including maximal left ventricular outflow tract gradient, were recorded. The primary outcome was death and appropriate internal defibrillator discharge. Hypertension was observed in 72%, with a Society of Thoracic Surgeons (STS) score (8.6±6); while 80% had no HCM‐related sudden cardiac death risk factors. Left ventricular mass index, basal septal thickness, and maximal left ventricular outflow tract gradient were 127±43 g/m 2 , 1.7±0.4 cm, and 49±31 mm Hg, respectively. A total of 597 (54%) had a left ventricular outflow tract gradient >30 mm Hg, of which 195 (33%) underwent septal reduction therapy (SRT; 79% myectomy and 21% alcohol ablation). At 5.1±4 years, 556 (50%) had composite events (273 [53%] in nonobstructive, 220 [55%] in obstructive without SRT, and 63 [32%] in obstructive subgroup with SRT). One‐ and 5‐year survival, respectively were 93% and 63% in nonobstructive, 90% and 63% in obstructive subgroup without SRT, and 94% and 84% in the obstructive subgroup with SRT. Following SRT, there were 5 (2.5%) in‐hospital deaths (versus an expected Society of Thoracic Surgeons mortality of 9.2%). Conclusions Elderly patients with HCM have a high prevalence of traditional cardiovascular rather than HCM risk factors. Longer‐term outcomes of the obstructive SRT subgroup were similar to a normal age‐sex matched US population.

Abstract 13729: Surgical Myectomy Results in Significantly Improved Long-term Event Rate in Hypertrophic Cardiomyopathy Patients With Severe Left Ventricular Outflow Tract Obstruction

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Milind Y Desai ◽  
Nicholas G Smedira ◽  
Aditya Bhonsale ◽  
Nitesh Ainani ◽  
Maran THamilarasan ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Long Term Outcomes ◽  
Free Survival ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Background: In hypertrophic cardiomyopathy (HCM) with severe left ventricular outflow tract obstruction (LVOTO), surgical myectomy (SM) performed for relief of intractable symptoms is safe and associated with excellent long-term symptom-free survival. In such patients, we sought to determine if SM also results in improvement of long-term outcomes. Methods: We studied 1530 HCM patients with severe LVOTO (50±13 years, 63% men) that were evaluated at our center [excluding <18 years of age, LV ejection fraction (LVEF) <50%, LVOT gradient <30 mm Hg). Clinical, echocardiographic and surgical data were recorded. A composite endpoint of death and/or implantable defibrillator (ICD) discharge was recorded. Results: Hypertension, coronary artery disease (CAD), family history of HCM and syncope were present in 41%, 15%, 17% and 18%, while 73% patients were in Functional Class (FC) ≥ II. Mean LVEF, basal septal thickness, LVOT gradient (resting or provocable) and indexed left atrial dimensions were 62±5%, 2.2±1 cm, 101±39 mm Hg, 2.2±0.4 cm/m2. During 8±6 years of follow-up, 990 (65%) patients underwent SM (of which 65% were isolated SM), while 540 (35%) did not. 94 (6%) patients had alcohol septal ablation (66 in the non myectomy group), while 18% developed atrial fibrillation (AF), and 18% had ICD. There were 169 (11%) events (151 deaths), with 0% 30-day mortality in the SM group. On stepwise multivariable Cox Proportional Hazard analysis, increasing age (Hazard Ratio or HR 1.22 [1.06-1.40]), CAD (HR 1.57 [1.06-2.33]), worsening FC (HR 1.34 [1.05-1.71]) and AF (HR 1.73 [1.23-2.43]) predicted higher events, while SM as a time-dependent covariate (HR 0.58 [0.41-0.81]) was associated with improved event-free survival (all p<0.01). Kaplan-Meier curve showing impact of SM on outcomes is shown in Figure. Conclusion: In HCM patients with severe LVOTO, SM is associated with significant improvement in long-term outcomes when compared to watchful waiting.

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