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JACC. Case Reports Dec 2023An adult with unrepaired tetralogy of Fallot presented with frequent tet spells. Her course was complicated by severe cyanotic spells and tachycardia-bradycardia...
An adult with unrepaired tetralogy of Fallot presented with frequent tet spells. Her course was complicated by severe cyanotic spells and tachycardia-bradycardia syndrome that limited beta blocker use to stabilize her spells. She markedly improved after disopyramide initiation and underwent successful tetralogy of Fallot repair with excellent functional outcome.
PubMed: 38204534
DOI: 10.1016/j.jaccas.2023.102093 -
Interdisciplinary Cardiovascular and... May 2024Extended septal myectomy and alcohol septal ablation are 2 invasive treatments for hypertrophic obstructive cardiomyopathy. Our goal was to compare which of these...
OBJECTIVES
Extended septal myectomy and alcohol septal ablation are 2 invasive treatments for hypertrophic obstructive cardiomyopathy. Our goal was to compare which of these techniques achieved a higher reduction in gradients, improvement in New York Heart Association (NYHA) functional class and reduction in medical treatment.
METHODS
It is a single-centre observational and retrospective analysis. We used multivariable regression analyses to assess the association of ablation/myectomy with different outcomes. The odds ratio or coefficient along with the 95% confidence interval was estimated according to the group and adjusted for the corresponding preprocedural variables and EuroSCORE II.
RESULTS
A total of 78 patients underwent septal myectomy, and 25 patients underwent alcohol septal ablation. Basal and Valsalva gradients after myectomy were reduced to a higher degree in comparison to ablation: 21.0 mmHg [P < 0.001, 95% confidence interval -30.7; -11.3], and 34.3 mmHg (P < 0.001, -49.1; -19.5) respectively. Those patients who received a myectomy had a lower probability of having moderate mitral regurgitation (odds ratio = 0.18, P = 0.054). Patients after septal myectomy were more likely to be NYHA functional class I (80.4%), whereas patients after ablation were more likely to be NYHA functional class III (48%). Both groups continued with beta-blocker therapy, but disopyramide could be discontinued after the myectomy in more cases (20%-36% vs 59%-1.3%; P < 0.001), and there was a tendency to discontinue calcium channel blockers (48%-16% vs 15.4-3.8%; P = 0.054).
CONCLUSIONS
After adjustment using preprocedural gradients and EuroSCORE II, myectomy achieves greater reduction in left ventricular outflow tract gradients compared to septal ablation.
PubMed: 38569884
DOI: 10.1093/icvts/ivae058 -
European Heart Journal. Case Reports Oct 2023Left ventricular outflow tract obstruction (LVOTO) sometimes presents with aortic stenosis (AS). Echocardiography is used to assess the diagnosis and severity of LVOTO...
Usefulness of catheter pressure measurement using the Navvus RXi system to determine left ventricular outflow tract obstruction and aortic stenosis severity: a case report.
BACKGROUND
Left ventricular outflow tract obstruction (LVOTO) sometimes presents with aortic stenosis (AS). Echocardiography is used to assess the diagnosis and severity of LVOTO or AS. However, LVOTO is one of the conditions that makes AS assessment difficult, and catheter pressure measurement is frequently useful in such cases.
CASE SUMMARY
An 84-year-old female patient presented with New York Heart Association functional Class III dyspnoea. Transthoracic echocardiography revealed LVOTO caused by upper septal hypertrophy, mitral valve systolic anterior motion with moderate mitral regurgitation, and a highly calcified aortic valve, which suggested the possibility of severe AS. The continuous Doppler echocardiography revealed a late-systolic peaking dagger-shaped profile with a peak jet velocity of 5.6 m/s. Cardiac catheterization was performed to determine the contribution of AS or LVOTO to her symptoms. Catheter pressures were measured at the ascending aorta (using a coronary catheter) and the LV (using the Navvus RXi system). The initial mean pressure gradient between the apex of the LV, just below the aortic valve and aorta, was measured at 65 and 25 mmHg, respectively. The mean pressure gradient between the apex and the aorta decreased from 65 to 40 mmHg after a 50 mg disopyramide administration. Oral medication therapy effectively stabilized her symptom after catheterization.
DISCUSSION
To the best of our knowledge, this is the first reported case of assessing the severity of LVOTO and AS using the Navvus RXi system. Catheter pressure measurement using the Navvus RXi system is a useful method of determining the severity of LVOTO and AS.
PubMed: 37811156
DOI: 10.1093/ehjcr/ytad471 -
Archives of Cardiovascular Diseases 2024The efficacy of current pharmacological therapies in hypertrophic cardiomyopathy is limited. A cardiac myosin inhibitor, mavacamten, has recently been approved as a...
Target population for a selective cardiac myosin inhibitor in hypertrophic obstructive cardiomyopathy: Real-life estimation from the French register of hypertrophic cardiomyopathy (REMY).
BACKGROUND
The efficacy of current pharmacological therapies in hypertrophic cardiomyopathy is limited. A cardiac myosin inhibitor, mavacamten, has recently been approved as a first-in-class treatment for symptomatic hypertrophic obstructive cardiomyopathy.
AIMS
To assess the profile and burden of cardiac myosin inhibitor candidates in the hypertrophic cardiomyopathy prospective Register of hypertrophic cardiomyopathy (REMY) held by the French Society of Cardiology.
METHODS
Data were collected at baseline and during follow-up from patients with hypertrophic cardiomyopathy enrolled in REMY by the three largest participating centres.
RESULTS
Among 1059 adults with hypertrophic cardiomyopathy, 461 (43.5%) had obstruction; 325 (30.7%) of these were also symptomatic, forming the "cardiac myosin inhibitor candidates" group. Baseline features of this group were: age 58±15years; male sex (n=196; 60.3%); diagnosis-to-inclusion delay 5 (1-12)years; maximum wall thickness 20±6mm; left ventricular ejection fraction 69±6%; family history of hypertrophic cardiomyopathy or sudden cardiac death (n=133; 40.9%); presence of a pathogenic sarcomere gene mutation (n=101; 31.1%); beta-blocker or verapamil treatment (n=304; 93.8%), combined with disopyramide (n=28; 8.7%); and eligibility for septal reduction therapy (n=96; 29%). At the end of a median follow-up of 66 (34-106) months, 319 (98.2%) were treated for obstruction (n=43 [13.2%] received disopyramide), 46 (14.2%) underwent septal reduction therapy and the all-cause mortality rate was 1.9/100 person-years (95% confidence interval 1.4-2.6) (46 deaths). Moreover, 41 (8.9%) patients from the initial hypertrophic obstructive cardiomyopathy group became eligible for a cardiac myosin inhibitor.
CONCLUSIONS
In this cohort of patients with hypertrophic cardiomyopathy selected from the REMY registry, one third were eligible for a cardiac myosin inhibitor.
Topics: Humans; Male; Cardiomyopathy, Hypertrophic; Female; Middle Aged; Registries; France; Treatment Outcome; Aged; Time Factors; Ventricular Function, Left; Cardiovascular Agents; Patient Selection; Prospective Studies; Cardiac Myosins; Benzylamines; Adult; Risk Factors; Ventricular Outflow Obstruction; Uracil
PubMed: 38762345
DOI: 10.1016/j.acvd.2024.04.001 -
European Journal of Preventive... Jun 2024
PubMed: 38850169
DOI: 10.1093/eurjpc/zwae198