Anthony Ralph-Edwards

Myocardial fibrosis in hypertrophic cardiomyopathy: accurate reflection of histopathological findings by CMR.

OBJECTIVES In this study we sought to explore the relationship between cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and histopathological parameters including interstitial fibrosis and replacement fibrosis (scar) in patients with hypertrophic cardiomyopathy (HCM). BACKGROUND CMR-LGE is a well-established tool for the assessment of scar in ischemic heart disease. Its role in HCM has evolved in recent years, and an association with nonsustained ventricular tachycardia has been demonstrated. METHODS HCM patients who underwent septal myectomy during the period 2004 through 2010 and had undergone CMR-LGE no more than 6 months before surgery were selected. Histopathological assessment of the myectomy specimens included quantitative digital analysis (interstitial and replacement fibrosis) and semiquantitative assessment (small intramural coronary arteriole dysplasia and disarray). Correlations between CMR-LGE measured with various techniques, SD above the signal intensity for the normal remote myocardium (2, 4, 5, 6, and 10 SD) and the full width at half maximum (FWHM) technique, at the myectomy site, and interstitial fibrosis, replacement fibrosis (scar), and their sum (fibrosis + scar) were evaluated. RESULTS Twenty-nine patients were included. Statistically significant correlations between CMR-LGE (at 2, 4, 5, 6, 10 SD and by the FWHM technique), and both interstitial fibrosis and the combined interstitial and replacement fibrosis were found. The strongest correlation was between combined interstitial and replacement fibrosis and CMR-LGE measured at 5 SD (r = 0.78, p < 0.0001). LGE measured at 10 SD demonstrated the best correlation with replacement fibrosis (r = 0.42, p = 0.02). Bland-Altman analysis revealed optimum agreement between the combined interstitial and replacement fibrosis found at pathology and LGE measured at 4 SD. In addition, moderate and severe small intramural coronary artery dysplasia showed a statistically significant correlation with replacement fibrosis (p = 0.01) and CMR-LGE at 10 SD (p = 0.04). CONCLUSIONS CMR-LGE measured at 4 SD and 5 SD yields the closest approximation to the extent of total fibrosis measured by the histopathological standard of reference. These findings have implications for future investigations of CMR-LGE and its association with important clinical endpoints in HCM, including sudden cardiac death.

Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment.

OBJECTIVES The aim of this study was to compare the survival of patients with hypertrophic cardiomyopathy (HCM) and resting left ventricular outflow tract (LVOT) obstruction managed with an invasive versus a conservative strategy. BACKGROUND In patients with resting obstructive HCM, clinical benefit can be achieved after invasive septal reduction therapy. However, it remains controversial whether invasive treatment improves long-term survival. METHODS We studied a consecutive cohort of 649 patients with resting obstructive HCM. Total and HCM-related mortality were compared in 246 patients who were conservatively managed with 403 patients who were invasively managed by surgical myectomy, septal ethanol ablation, or dual-chamber pacing. RESULTS Multivariable analyses (with invasive therapy treated as a time-dependent covariate) showed that an invasive intervention was a significant determinant of overall mortality (hazard ratio: 0.6, 95% confidence interval: 0.4 to 0.97, p = 0.04). Overall survival rates were greater in the invasive (99.2% 1-year, 95.7% 5-year, and 87.8% 10-year survival) than in the conservative (97.3% 1-year, 91.1% 5-year, and 75.8% 10-year survival, p = 0.008) cohort. However, invasive therapy was not found to be a significant independent predictor of HCM-related mortality (hazard ratio: 0.7, 95% confidence interval: 0.4 to 1.3, p = 0.3). The HCM-related survival was 99.5% (1 year), 96.3% (5 years), and 90.2% (10 years) in the invasive cohort, and 97.8% (1 year), 94.6% (5 years), and 86.9% (10 years) in the conservative cohort (p = 0.3). CONCLUSIONS Patients treated invasively have an overall survival advantage compared with conservatively treated patients, with the latter group more likely to die from noncardiac causes. The HCM-related mortality is similar, regardless of a conservative versus invasive strategy.

Reoperation for recurrent aortic coarctation

BACKGROUND Recurrence of stenosis is a complication of coarctation repair associated with major long-term morbidity. Persistent or exercise-provoked hypertension may indicate recurrent coarctation. Patients failing or not amenable to balloon dilation should be managed surgically. METHODS A retrospective chart review was performed. RESULTS Forty-three patients were identified as having undergone repeat surgical intervention for recurrent aortic coarctation between the years 1976 and 1993 at The Hospital for Sick Children in Toronto. Seventy percent of the children had other congenital cardiac anomalies. Eighty-six percent of patients initially treated by subclavian flap aortoplasty or end-to-end anastomosis were managed at reoperation by patch aortoplasty, and 26% of patients also required augmentation of the transverse arch (under hypothermic circulatory arrest) for accompanying hypoplasia. Three patients underwent a second reoperation; all were treated at this reoperation with tube graft interposition. CONCLUSIONS No ischemic spinal injury occurred in patients managed with either simple proximal aortic cross-clamping or cardiopulmonary bypass. No patient treated with transverse arch augmentation required further surgical intervention. Mortality at reoperation was 7% (3 patients), similar to that of first-time coarctation repair. At follow-up (mean duration, 4.5 years), 57% of patients are normotensive, with no measurable arm-leg gradient.

Low operative mortality achieved with surgical septal myectomy role of dedicated hypertrophic cardiomyopathy centers in the management of dynamic subaortic obstruction

Treatment of progressive heart failure, due to left ventricular (LV) outflow tract obstruction and elevated intraventricular systolic pressures, has been a major component of hypertrophic cardiomyopathy (HCM) disease management for 50 years [(1–3)][1]. Throughout this time, septal myectomy has

Hypertrophic cardiomyopathy : current understanding and treatment objectives

The understanding of hypertrophic cardiomyopathy (HCM) has changed dramatically over the last few decades, and it is now understood to be caused by a mutation in one of several cardiac sarcomeric genes. Due to complications such as outflow tract obstruction, diastolic dysfunction, arrhythmias, stroke, infective endocarditis and sudden cardiac death, appropriate and early identification of these patients is imperative. This review attempts to summarise the current state of knowledge on HCM, and provide insight of the appropriate investigations needed in patients with HCM. It also outlines treatment strategies for these patients. Much remains unknown about this complex and intriguing disease, and continued research in identifying the genetic basis of HCM, along with the assessment of therapeutic strategies, will help to optimise patient care.

Atypical Presentation of Spontaneous Pneumomediastinum

Spontaneous pneumomediastinum is a rare clinical entity; when diagnosis is certain, no treatment is required and symptoms rarely recur. The clinical presentation is usually diagnostic; however, atypical symptoms may mandate further investigation before diagnosis can be established. We describe 2 patients with spontaneous pneumomediastinum who presented with dominant esophageal symptoms (odynophagia and dysphagia) suggestive of esophageal perforation. Investigation and management are discussed.

Infective endocarditis in patients who had replacement of the aortic root

In 12 patients who had had composite replacement of the aortic valve and ascending aorta, infective endocarditis developed 2 months to 17 years after operation. Six patients had mechanical valves and 6 had biological ones (four homograft and two porcine valves). All patients needed operation because of shock, heart failure, persistent sepsis in spite of adequate antibiotic therapy, or the development of a paravalvular false aneurysm. The predominant microorganism was Staphylococcus. All 6 patients who had mechanical valves were found to have an abscess in the junction between the aortic annulus and the prosthesis; in patients who had biological valves the infection was limited to the leaflets in 3 (one homograft and two porcine valves) and leaflets and annulus abscess in 3 (three homograft valves). Operation consisted of radical resection of tissues suspected of being infected and reconstruction of the left ventricular outflow tract and of the surrounding structures with glutaraldehyde-fixed bovine pericardium. The aortic valve and ascending aorta were replaced with a new valved conduit. An aortic homograft was used in only 1 patient. There was only one operative death due to right ventricular infarction but most patients experienced serious postoperative complications. Operative survivors were followed up from 3 to 156 months (mean, 42 months). One patient died 35 months postoperatively due to bleeding complications of anticoagulation; 1 patient suffered a cardiac arrest at home 2 months after operation, sustained permanent cerebral damage, and died 4 months later. The remaining patients are asymptomatic from the cardiovascular viewpoint.(ABSTRACT TRUNCATED AT 250 WORDS)

Significance of left ventricular apical–basal muscle bundle identified by cardiovascular magnetic resonance imaging in patients with hypertrophic cardiomyopathy

AIMS Cardiovascular magnetic resonance (CMR) has improved diagnostic and management strategies in hypertrophic cardiomyopathy (HCM) by expanding our appreciation for the diverse phenotypic expression. We sought to characterize the prevalence and clinical significance of a recently identified accessory left ventricular (LV) muscle bundle extending from the apex to the basal septum or anterior wall (i.e. apical-basal). METHODS AND RESULTS CMR was performed in 230 genotyped HCM patients (48 ± 15 years, 69% male), 30 genotype-positive/phenotype-negative (G+/P-) family members (32 ± 15 years, 30% male), and 126 controls. Left ventricular apical-basal muscle bundle was identified in 145 of 230 (63%) HCM patients, 18 of 30 (60%) G+/P- family members, and 12 of 126 (10%) controls (G+/P- vs. controls; P < 0.01). In HCM patients, the prevalence of an apical-basal muscle bundle was similar among those with disease-causing sarcomere mutations compared with patients without mutation (64 vs. 62%; P = 0.88). The presence of an LV apical-basal muscle bundle was not associated with LV outflow tract obstruction (P = 0.61). In follow-up, 33 patients underwent surgical myectomy of whom 22 (67%) were identified to have an accessory LV apical-basal muscle bundle, which was resected in all patients. CONCLUSION Apical-basal muscle bundles are a unique myocardial structure commonly present in HCM patients as well as in G+/P- family members and may represent an additional morphologic marker for HCM diagnosis in genotype-positive status.

Successful treatment of massive pulmonary embolism after coronary artery bypass grafting due to heparin-induced thrombocytopenia

Massive pulmonary embolism is a rare complication in patients undergoing coronary artery bypass grafting. Frequently patients have had exposure to heparin before the operation. In this article we report a patient who 6 days after a cardiac operation suffered a massive pulmonary embolism. The patient was later discovered to have heparin-associated thrombocytopenia with serum heparin antibody. We recommend patients receiving heparin have frequent platelet counts and those with induced thrombocytopenia undergo sensitivity testing.

Why we need more septal myectomy surgeons: An emerging recognition

From the HCM Institute, Division of Cardiology, Tufts Medical Center, Boston, Mass; Mayo Clinic, Rochester, Minn; New York University Langone Medical Center, New York, NY; Toronto General Hospital, University Health Network, Toronto, Ontario, Canada; Cleveland Clinic Foundation, Cleveland, Ohio; and Policlinico di Monza, Monza, Italy. Received for publication Oct 15, 2016; revisions received Nov 21, 2016; accepted for publication Dec 15, 2016. Address for reprints: Barry J. Maron, MD, HCM Institute, Division of Cardiology, Tufts Medical Center, 800 Washington St, #70, Boston, MA 02111 (E-mail: barrymaron1@gmail.com). J Thorac Cardiovasc Surg 2017;-:1-5 0022-5223/