Myocardial contrast echocardiography assessment of perfusion abnormalities in hypertrophic cardiomyopathy.

BACKGROUND: Perfusion defects during stress can occur in hypertrophic cardiomyopathy (HCM) from either structural or functional abnormalities of the coronary microcirculation. In this study, vasodilator stress myocardial contrast echocardiography (MCE) was used to quantify and spatially characterize hyperemic myocardial blood flow (MBF) deficits in HCM. METHODS: Regadenoson stress MCE was performed in patients with septal-variant HCM (n = 17) and healthy control subjects (n = 15). The presence and spatial distribution (transmural diffuse, patchy, subendocardial) of perfusion defects was determined by semiquantitative analysis. Kinetic analysis of time-intensity data was used to quantify MBF, microvascular flux rate (ß), and microvascular blood volume. In patients undergoing septal myectomy (n = 3), MCE was repeated > 1 years after surgery.  RESULTS: In HCM subjects, perfusion defects during stress occurred in the septum in 80%, and in non-hypertrophied regions in 40%. The majority of septal defects (83%) were patchy or subendocardial, while 67% of non-hypertrophied defects were transmural and diffuse. On quantitative analysis, hyperemic MBF was approximately 50% lower (p < 0.001) in the hypertrophied and non-hypertrophied regions of those with HCM compared to controls, largely based on an inability to augment ß, although hypertrophic regions also had blood volume deficits. There was no correlation between hyperemic MBF and either percent fibrosis on magnetic resonance imaging or outflow gradient, yet those with higher degrees of fibrosis (≥ 5%) or severe gradients all had low septal MBF during regadenoson. Substantial improvement in hyperemic MBF was observed in two of the three subjects undergoing myectomy, both of whom had severe pre-surgical outflow gradients at rest. CONCLUSION: Perfusion defects on vasodilator MCE are common in HCM, particularly in those with extensive fibrosis, but have a different spatial pattern for the hypertrophied and non-hypertrophied segments, likely reflecting different contributions of functional and structural abnormalities. Improvement in hyperemic perfusion is possible in those undergoing septal myectomy to relieve obstruction.  TRIAL REGISTRATION: ClinicalTrials.gov NCT02560467.

Standardized Goal-Directed Valsalva Maneuver for Assessment of Inducible Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy.

BACKGROUND: The Valsalva maneuver is widely used to provoke left ventricular outflow tract obstruction in hypertrophic cardiomyopathy (HCM). Whereas early experiments used a standardized, goal-directed approach by maintaining an intraoral pressure >40 mm Hg for >10 sec, current practice depends on patients' understanding and effort. The aim of this study was to evaluate the clinical effectiveness of the goal-directed Valsalva maneuver (GDV) in HCM as a method to provoke left ventricular outflow tract obstruction. METHODS: In this prospective study, patients blew into a syringe barrel connected to a manometer with rubber tubing and maintained an intraoral pressure of >40 mm Hg for >10 sec (GDV). Using Doppler echocardiography, peak left ventricular outflow tract gradient (pLVOTG) was measured at rest and using the provocative maneuvers of the self-directed Valsalva maneuver (SDV), GDV, and exercise. RESULTS: A total of 52 patients were included. Mean pLVOTG with GDV was higher compared with SDV (48 vs 38 mm Hg, P = .001, n = 52) and was similar to exercise (GDV, 52 mm Hg; exercise, 58 mm Hg; P = .42; n = 43). Reclassification to obstructive HCM (pLVOTG ≥ 30 mm Hg) with GDV was significantly higher than with SDV (38% vs 16.6%, P = .016) and comparable with exercise (50%, P = .51). Reclassification to severe obstruction (pLVOTG ≥ 50 mm Hg) was higher with GDV compared with SDV (28.3% vs 13.5%, P = .045) and was similar to exercise (29.7%). Furthermore, GDV identified two patients with occult severe obstruction in isolation. CONCLUSIONS: GDV is an objective, practical, and effective physiologic method of provoking left ventricular outflow tract obstruction. It can significantly alter patient management by reclassifying disease severity and should be incorporated in the routine clinical evaluation of patients with HCM.

The Role of Imaging with Cardiac Computed Tomography in Cardio-Oncology Patients.

Cardiovascular diseases and cancer represent the two most common causes of morbidity and mortality in industrialized countries. With the increase in long-term survival of cancer patients, cardiovascular diseases are the leading cause of mortality for many cancer survivors. In this article, we will review the most common cardiovascular toxicities of cancer therapies and will describe the role of cardiac CT in the detection and monitoring of cardiovascular disease. While there is limited evidence for the use of CT imaging in cancer patients, we will discuss the utility of cardiac CT in the detection and management of coronary artery disease, pericardial and valvular heart disease.