Project description:Left ventricular outflow tract obstruction (LVOTO) can complicate percutaneous mitral valve replacement and may preclude patients considered high surgical risk from transcatheter therapies. We report a case of mitral valve-in-valve procedure in a patient at high risk for LVOTO. (Level of Difficulty: Advanced.).
Project description:ImportancePatients with hypertrophic obstructive cardiomyopathy (HOCM) and drug-refractory symptoms and outflow gradients have limited nonsurgical treatment options. The feasibility of percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) has been reported previously; however, procedural and medium-term outcomes are unknown.ObjectiveTo describe the safety and medium-term outcomes of PIMSRA in a large patient cohort with drug-refractory HOCM.Design, setting, and participantsThis was a single-arm, open-label study of PIMSRA in patients with drug-refractory HOCM. Patients presenting to the Xijing Hospital in Xi'an, China, between October 2016 to June 2020 with hypertrophic cardiomyopathy. Of 1314 patients presenting with HOCM, 244 fulfilled inclusion criteria of severe resting/provoked outflow gradients of 50 mm Hg or higher, and symptoms of New York Heart Association functional class of II or higher refractory to maximum tolerated medications. After discussion among the heart team, 40 patients underwent surgical or alcohol septal reduction therapy and 4 required treatment of significant coronary artery disease.InterventionsPIMSRA performed in patients.Main outcomes and measuresThe primary outcome was 30-day major adverse clinical events: death, emergency surgery, severe effusion requiring intervention, procedure-related stroke, bleeding, and stroke. Secondary outcomes included 30-day technical success and 90-day improvement in outflow obstruction.ResultsThe mean (SD) age of 200 patients was 46.9 (14.0) years, and 125 (62.5%) were men. Resting or provoked left ventricular outflow tract gradients were 50 mm Hg or higher. The median (IQR) follow-up for all patients was 19 (6-50) months. Thirty-day major adverse clinical events rate was 10.5% (n = 21): there were 2 in-hospital/30-day deaths (1.0%), 7 patients (3.5%) with pericardial effusion requiring mini-thoracotomy, 12 patients (6%) with pericardial effusion requiring pericardiocentesis, and no bleeding or strokes. Other periprocedural complications included permanent right bundle branch block in 5 patients (2.5%), resuscitated ventricular fibrillation in 2 (1.0%), and septal branch aneurysm in 2 (1.0%). There were no permanent pacemaker implantations. At follow-up, maximum septal thickness was reduced from a mean (SD) of 24.0 (5.1) mm to 17.3 (4.4) mm (P < .001), and left ventricular outflow tract gradient was decreased from a mean (SD) of 79.0 (53.0) mm Hg to 14.0 (24.0) mm Hg (P < .001). Overall, 190 patients (96%) with HOCM were in New York Heart Association functional class I or II at last follow-up.Conclusions and relevanceThis study found that PIMSRA in patients with drug-refractory HOCM may be an effective procedure for relief of left ventricular outflow tract obstruction and symptoms with acceptable complication rates. These results are encouraging and support the design of a randomized clinical trial against well-established septal reduction therapies.
Project description:BackgroundThis is a case report of a patient with Fabry disease (FD). We successfully treated a patient with ventricular septal hypertrophy and left ventricular outflow tract (LVOT) obstruction caused by FD. We report our exclusive new surgery for patients with LVOT obstruction, percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) procedure™ (percutaneous intramyocardial septal radiofrequency ablation). The patient recovered well 7 months after operation, and no LVOT obstruction and arrhythmia occurred.Case summaryA 51-year-old woman with chest tightness after satiety for ∼10 years, aggravated for 2 months. The patient had a history of multiple syncope, syncope after activity or emotional excitement. Echocardiogram demonstrated ventricular septal thickness of 22 mm by the report. Genetic testing revealed gene mutation, which was consistent with FD (GLA: c.643A > C). Biochemical evaluation reported reduced serum α-galactosidase A activity and abnormal serum Lyso-GL-3 enzyme activity. The patient and her families decided to perform PIMSRA operation. After the operation, the septal thickness steadily decreased to 16 mm over the course of 7 months. However, the patient's symptoms improved significantly.DiscussionThis is the first case of cardiac involvement caused by FD mimicking hypertrophic cardiomyopathy treated with the innovative PIMSRA procedure. Percutaneous intramyocardial septal radiofrequency ablation procedure can be one of the effective treatments to alleviate cardiac hypertrophy and left ventricular obstruction in FD.
Project description:BackgroundEstimated plasma volume status (ePVS) estimated by the Duarte formula is associated with clinical outcomes in patients with heart failure. It remains unclear the predictive value of the ePVS to the postoperative hypotension (POH) in percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) treating hypertrophic obstructive cardiomyopathy (HOCM).MethodsData of HOCM patients who underwent PIMSRA were retrospectively collected. Preoperative ePVS was calculated using the Duarte formulas which derived from hemoglobin and hematocrit ratios. Clinical variables including physical assessment, biological and echocardiographic parameters were recorded. Patients were labeled with or without POH according to the medical record in the hospital. Univariable and multivariable logistic regression were performed to evaluate the association between ePVS and POH. Using different thresholds derived from quartiles and the best cutoff value of the receiver operating characteristic curve, the diagnostic performance of ePVS was quantified.ResultsAmong the 405 patients included in this study, 53 (13.1%) patients were observed with symptomatic POH. Median (IQR) of ePVS in overall patients was 3.77 (3.27~4.40) mL/g and in patients with POH were higher than those without POH. The ePVS was associated with POH, with the odds ratio of 1.669 (95% CI 1.299 ~ 2.144) per mL/g. After adjusted by potential confounders, ePVS remained independently associated with POH, with the approximate odds ratio in different models.ConclusionThe preoperative ePVS derived from the Duarte formulas was independently associated with postoperative hypotension in HOCM patients who underwent PIMSRA and showed prognostic value to the risk stratification of postoperative management.Trial registrationNCT06003478 (22/08/2023).
Project description:BackgroundAlcohol septal ablation (ASA) can be recommended for patients with drug-refractory hypertrophic obstructive cardiomyopathy (HOCM). Recently, percutaneous intramyocardial septal radiofrequency ablation (PIMSRA) was reported as a safe and effective treatment for HOCM.Case summaryWe present a case report of pulseless electrical activity (PEA), cardiac arrest, and pericardial tamponade occurring post-PIMSRA. We performed PIMSRA for the patient with HOCM after failed ASA. Two hours post-PIMSRA, transthoracic echocardiography (TTE) revealed that the hypokinetic basal intraventricular septal (IVS) thickness increased with aggravation of systolic anterior motion of the mitral valve. After the occurrence of subsequent PEA cardiac arrest, veno-arterial extracorporeal membrane oxygenation (VA-ECMO) support was provided. With sinus rhythm restoration and blood pressure stabilization after ECMO removal, the patient had pericardial tamponade on Day 3 post-PIMSRA. After excluding apparent myocardial perforation and draining haemorrhagic effusion under TTE guidance, her symptoms and haemodynamic status improved. She was asymptomatic at her one-year follow-up. The left ventricular outflow tract gradient (LVOTG) at rest and the thickness of the basal IVS reduced to 5 mmHg and 12 mm, respectively.DiscussionWe assumed that the main causes of PEA cardiac arrest and pericardial tamponade in our case were ablation-related tissue oedema at the basal IVS and blood leakage possibly related to puncture haemorrhage, respectively. While waiting for myocardial oedema to resolve, ECMO was applied as a bridge-to-recovery therapeutic approach. Pericardiocentesis is a strategy for the emergency drainage of pericardial effusion. It is essential to distinguish life-threatening complications with TTE for management planning post-PIMSRA.