RESUMEN
BACKGROUND: Latent left ventricular outflow tract obstruction (LVOTO) is an important cause of symptoms in patients with hypertrophic cardiomyopathy (HCM) but can be challenging to provoke. OBJECTIVES AND METHODS: To examine the value of postprandial resting and stress echocardiography and utilization of invasive or enhanced drug therapies (surgical myectomy, alcohol septal ablation, disopyramide, and mavacamten) in patients with postprandial LVOTO. Consecutive HCM patients without LVOTO underwent routine and postprandial echocardiography at rest, with provocation (Valsalva and standing) and after symptom-limited treadmill stress. RESULTS: Among 252 patients (mean age, 58 years, 39% women), postprandial LVOT gradients were higher compared with routine echocardiography at rest (median, 9.0 [0-38.0] vs 0 [0-14.0] mm Hg; P < .0001) and with provocation (18.5 [0-70.3] vs 1.5 [0-41.0] mm Hg; P < .0001). Postprandial exercise stress echocardiogram (PPXSE) gradients were higher in a subset of 44 patients who underwent both postprandial and fasting stress echocardiography (47.0 [5.3-81.0] vs 17.5 [0-46.0] mm Hg; P < .0001). In total, 49 (19.5%) patients achieved the ≥50 mm Hg threshold under routine conditions (rest/provocation); 90 (35.7%) additional patients achieved postprandial gradients ≥50 mm Hg (rest/provocation/exercise), 38 (15.1%) with PPXSE alone. A total of 71 patients were treated with 91 invasive or enhanced drug therapies, 32 (45.1%) of whom had gradients ≥50 mm Hg only after eating (rest/provocation) and 8 (11.3%) only with PPXSE, with symptom relief in the majority. CONCLUSIONS: Postprandial echocardiography was useful at unmasking LVOTO in more than one-third of patients who did not have high gradients otherwise. Eating before echocardiography is a powerful provocative tool in the evaluation of patients with HCM.
RESUMEN
BACKGROUND: Mitral leaflet elongation is common in hypertrophic cardiomyopathy (HCM), contributes to obstructive physiology, and presents a challenge to the dual surgical goals of abolition of outflow gradients and abolition of mitral regurgitation. Anterior leaflet shortening, performed as an ancillary surgical procedure during myectomy, is controversial. METHODS: This was a retrospective study of all patients undergoing myectomy from January 2010 to March 2020, with analysis of survival and echocardiographic results. The study compared outcomes of patients treated with myectomy and concomitant mitral leaflet shortening with patients treated with myectomy alone. Over this time, the technique for mitral shortening evolved from anterior leaflet plication to residual leaflet excision (ReLex). RESULTS: Myectomy was performed in 416 patients aged 57.5 ± 13.6 years, and 204 (49%) patients were female. Average follow-up was 5.4 ± 2.8 years. Survival follow-up was complete in 415 patients. Myectomy without valve replacement was performed in 332 patients, of whom 192 had mitral valve shortening (58%). Mitral leaflet plication was performed in 73 patients, ReLex in 151, and both procedures in 32. Hospital mortality for patients undergoing myectomy was 0.7%. At 8 years, cumulative survival was 95% for both the myectomy combined with leaflet shortening group and the myectomy alone group, with no difference in survival between the 2 groups. There was no difference in survival between the anterior leaflet plication and ReLex groups. Echocardiography 2.5 years after surgery showed a decrease in resting and provoked gradients, mitral regurgitation, and left atrial volume and no difference in key variables between patients who underwent ancillary leaflet shortening and patients who underwent myectomy alone. CONCLUSIONS: These results affirm that mitral shortening may be an appropriate surgical judgment for selected patients.