Relationship of cardiac magnetic resonance imaging and myocardial biopsy in the evaluation of nonischemic cardiomyopathy.

This study was performed to determine the relative role of cardiac magnetic resonance (CMR) imaging and endomyocardial biopsy (EMB) in the evaluation of cardiomyopathy. Sixty-six patients with a clinical diagnosis of nonischemic dilated cardiomyopathy or restrictive cardiomyopathy underwent both EMB and CMR imaging as part of their diagnostic evaluation. The authors retrospectively reviewed the results of these two methods to determine their diagnostic impact and congruency. CMR imaging provided data on cardiac anatomy, left ventricular volumes, mass, and function in 85% of the patients, uncovered fibrosis in 31%, myocardial ischemia in 7%, and fibrofatty infiltration in two patients. EMB provided the histologic findings of cardiomyocyte hypertrophy in 77% of patients and substantial interstitial fibrosis in 59%. Six patients had EMB-proven amyloid heart disease, which was detected by CMR imaging in two. CMR imaging showed patterns of late gadolinium enhancement supportive of infiltrative disease or inflammation in 6 patients with EMB-proven definite (n=3) or borderline (n=3) myocarditis, but failed to do so in two other patients with borderline and two with resolving myocarditis. At the present time, CMR imaging and EMB remain complementary procedures in the evaluation of cardiomyopathic conditions.

Mid-myocardial fibrosis by cardiac magnetic resonance in patients with lamin A/C cardiomyopathy: possible substrate for diastolic dysfunction.

AIMS: We sought to identify patterns of myocardial fibrosis in vivo in patients with lamin cardiomyopathy, and to determine its functional significance. METHODS AND RESULTS: Eleven patients sharing the identical mutation in LMNA without contraindication to magnetic resonance were identified from a 1016-member pedigree. Eight autopsy hearts from deceased relatives were reviewed. Patients and age-matched controls underwent cardiac magnetic resonance that included measures of cardiac function and late gadolinium enhancement (LGE). LGE-CMR identified midmyocardial fibrosis of the basal interventricular septum in 5 of 11 LMNA patients that was identical to that seen in 6 autopsy specimens of related genotype-positive family members; this was not present in any of 11 controls. LGE-CMR was positive in the 5 oldest patients in the cohort, age 46 +/- 6 years compared to 24 +/- 10 years for LGE-negative subjects (p = 0.003). Systolic function was abnormal in 2 subjects, both with myocardial fibrosis. LGE-positivity distinguished patients with diastolic dysfunction by mitral inflow velocities from those with normal diastolic function; these patients also had significant left atrial enlargement compared to controls (p < 0.05). CONCLUSIONS: LGE-CMR can identify myocardial fibrosis under genetic control in vivo in patients with heritable cardiomyopathy similar in distribution to that observed at autopsy. Mid-myocardial fibrosis may form the substrate for diastolic dysfunction in these patients.

Splenectomy in haematology--a 5-year single centre experience.

OBJECTIVE: To define indications and outcome in haematologic cases undergoing splenectomy. STUDY DESIGN: A retrospective review of clinical records from consecutive patients having open or laparoscopic removal of the spleen in an academic centre in the private sector. Endpoints were survival, operating time, spleen size, histopathology, requirements for blood or related products complications and average costs. RESULTS: In the total group (n = 69) there were two deaths. Referrals were for immune thrombocytopaenia (41%), acquired haemolytic anaemia (10%), myeloproliferative syndrome (9%), acute or chronic leukaemia (19%), lymphoma (13%) and a miscellaneous group (8%), comprising cholelithiasis, aplasia or as a diagnostic procedure for otherwise unexplained splenomegaly. An open midline approach was predicated by spleens greater than twice normal size and a history of any bleeding disorder. Here the mean operating time was 83 min (range 40-295) whereas for laparoscopy this was 251 min (range 181-272). SUMMARY: Careful stratification between the two options facilitated optimum haemostasis and consequently reduced requirement for packed red cells and platelets. Neither underlying pathology nor the choice of treatment influenced morbidity or mortality. Overall local experience is consistent with published international standards of surgical practice. Outcome is directly proportional to the number of each procedure carried out by a single team, observance of consistent protocols for preoperative evaluation and standardized proactive management through the recovery period.