Darrell B. Newman

Echocardiographic Features of Cardiac Angiosarcomas: The Mayo Clinic Experience (1976–2013)

Cardiac angiosarcoma is the most common primary malignant cardiac tumor. The dismal prognosis and nonspecific symptomatology underscore the need for an accurate and cost‐effective approach to the identification and characterization of this rare tumor.

Low incidence of left atrial delayed enhancement with MRI in patients with AF: A single-centre experience

Background Atrial fibrillation (AF) is the most common sustained atrial arrhythmia. One potential target for ablation is left atrial (LA) scar (LAS) regions that may be the substrate for re-entry within the atria, thereby sustaining AF. Identification of LAS through LA delayed gadolinium enhancement (LADE) with MRI has been proposed. Objectives We sought to evaluate LADE in patients referred for catheter ablation of AF. Methods Prospective analysis was conducted of consecutive patients who underwent pulmonary vein antrum isolation (PVAI) ablation for AF at a single institution. Patients underwent LADE with MRI to determine LAS regions before ablation. MRI data were analysed independently in accordance with prespecified institutional protocol by two staff cardiac radiologists to whom patient outcomes were masked, and reports of LADE were documented. Where no initial consensus occurred regarding delayed enhancement (DE), a third staff cardiac radiologist independently reviewed the case and had the deciding vote. Results Of the 149 consecutive patients (mean (SD) age, 59 (9) years), AF was persistent in 64 (43%) and paroxysmal in 85 (57%); 45 (30%) had prior ablation. Only five patients (3%) had identifiable DE in LA walls (persistent AF, n=1; paroxysmal AF, n=4). LADE was present in two (4%) of the 45 patients with previous left PVAI. The presence of LADE was not associated with a higher recurrence rate of AF. Conclusions In contrast to previous studies, the finding of DE within LA walls was uncommon and, when present, did not correlate with AF type or risk of AF recurrence. It therefore is of unclear clinical significance.

Carcinoid Heart Disease without Severe Tricuspid Valve Involvement

Carcinoid syndrome causes a rare form of acquired valvular heart disease which typically occurs in the setting of liver metastases. In carcinoid-induced valvular heart disease, the tricuspid valve is almost universally affected; left-sided valve disease occurs infrequently in affected patients. Herein, we report 2 cases of carcinoid-induced valvular heart disease; one case had no evidence of tricuspid valve involvement despite severe involvement of all other valves, while the other case was without severe tricuspid valve involvement.

Intervention to Reduce Inappropriate Ionized Calcium Ordering Practices: A Quality-Improvement Project

CONTEXT The importance of an abnormal ionized calcium (iCa) measurement in noncritically ill patients is unclear. Furthermore, iCa monitoring is more expensive than measurement of total calcium and consumes more laboratory resources. We hypothesize that most iCa tests are ordered for routine monitoring in asymptomatic patients, and results do not influence clinical management. OBJECTIVE To characterize and to intervene on iCa test-ordering practices among our institutions hospital-based internal medicine clinicians. DESIGN A quality-improvement project, with retrospective review of clinical records. We retrospectively identified the first 100 consecutive patients admitted to our hospital internal medicine (HIM) services during January 2012 with an iCa test ordered during their hospitalization. We reviewed clinical records to determine the appropriateness of iCa test ordering and of the ordering department. An educational intervention regarding the appropriateness of iCa testing was undertaken targeting HIM clinicians. MAIN OUTCOME MEASURES The effect of the intervention was assessed by identifying a sample of the first 100 consecutive patients admitted to HIM services during November 2012 and by comparing the proportion of iCa tests ordered by HIM clinicians before and after the intervention. RESULTS HIM services were responsible for 38% of iCa measurements before the educational intervention, with the remainder originating primarily from the Emergency Department (29%) and intensive care units (28%). After the intervention, the internal medicine services were responsible for 13% of iCa measurements, which represented a 66% reduction (p = 0.0007). CONCLUSION A simple intervention based on clinician education can reduce the frequency of routine iCa monitoring in stable hospitalized patients.

52-Year-Old Man With Liver Enzyme Abnormalities and Elevated Ferritin Level

94 Mayo Clin P A 52-year-old man presented for a general medical examination. His medical history included hypertriglyceridemia, obesity (body mass index of 32 kg/m), and impaired fasting blood glucose levels. Laboratory studies 4 years previously had shown persistent elevation of aminotransferase levels (reference ranges shown parenthetically)—aspartate aminotransferase (AST), 51 U/L (8-48 U/L) and alanine aminotransferase (ALT), 64 U/L (7-55 U/L)— and an elevated serum ferritin level of 675 g/L (24-336 g/L). At that time, he consumed alcohol regularly (2 drinks nightly). Additional laboratory abnormalities included hypertriglyceridemia (triglyceride level of 186 mg/dL). Testing for chronic hepatitis B and C virus infections yielded negative results. The patient failed to return for appointments over the next 4 years. At the current appointment, he stated that he had no symptoms and had come to the clinic only at his wife’s suggestion. Vital signs included an elevated blood pressure of 170/90 mm Hg. Physical examination revealed hepatomegaly characterized by a 17-cm liver span in the midclavicular line with a firm, nontender liver edge palpable 2 fingerbreadths below the right costal margin. There was notable absence of cutaneous stigmata of chronic liver disease and splenomegaly. Further review of the medical record showed an elevated blood pressure over the past several years consistent with hypertension. His liver enzyme and ferritin values remained elevated despite cessation of alcohol intake, with AST of 55 U/L, ALT of 88 U/L, and ferritin of 885 g/L. Hydrochlorothiazide treatment was initiated and follow-up scheduled with repeated laboratory testing.

Hypertrophic Obstructive Cardiomyopathy and Uncontrolled Hypertension : A Therapeutic Challenge

Hypertrophic obstructive cardiomyopathy and concomitant systemic hypertension can present a challenging diagnostic and therapeutic dilemma. Symptoms can occur from increased afterload from both dynamic outflow obstruction as well as the elevated systemic vascular resistance. Treatment of systemic

A not so typical pericardial effusion case

A 71-year-old woman with paroxysmal atrial fibrillation and hypothyroidism on hormone replacement presented with a 3-month history of progressive exertional dyspnoea and decline in functional capacity. She was evaluated and found to have a moderate circumferential pericardial effusion on transthoracic echocardiography without 2D or Doppler signs of tamponade physiology. Laboratory test showed normal thyroid-stimulating hormone and C-reactive protein levels, and sedimentation rate. Tuberculosis testing was negative and rheumatological panel was unrevealing. She was started on colchicine for idiopathic pericarditis. Due to progressive symptoms, she was referred …

Important Update for the Readers of Our Article

to the rapid development of overt carcinoid tricuspid valve disease from progressive metastatic disease, but other options should be considered. Could it be that the ‘sparing’ of the tricuspid valve that we reported was a variant of lead-time bias? Is the tricuspid valve always affected in patients with carcinoid heart disease if there is evidence of other (predominantly pulmonary) valve involvement, but the timing of manifest valve disease varies? Was there subclinical valve disease that, although not visible on gross evaluation by echocardiography or during surgical inspection, would possibly have been present on histopathologic assessment? Serial follow-up studies and pathologic evaluation of the apparently unaffected valves from patients with carcinoid heart disease may provide further insight. Another possibility is an alternative cause for the tricuspid valve disease; however, given the clinical history and appearance on transthoracic echocardiography, this seems unlikely. If faced with a similar clinical presentation to that which we initially reported, how would we manage it? Options include proceeding with the replacement of the affected valves only and monitoring the patient for tricuspid valve disease progression. Alternatively, one may wish to replace the normal-appearing tricuspid valve at the time of initial surgery, recognizing that recurrence of carcinoid disease has been reported on bioprosthetic valves [2, 3] . Our article entitled ‘Carcinoid heart disease without severe tricuspid valve involvement’ was recently published in Cardiology [1] . One patient included in our series had severe mitral and aortic valve regurgitation and moderate pulmonary valve regurgitation due to carcinoid heart disease. The tricuspid valve appeared normal on echocardiography and upon surgical inspection. Thus, the tricuspid valve was not replaced. Postoperatively, the patient initially did well. Seven months later, and following publication of our article, the patient developed effort intolerance, abdominal fullness and fluid retention. Echocardiography revealed thickened tricuspid valve leaflets with severe regurgitation and normal prostheses. The patient was simultaneously found to have a marked progression of her metastatic carcinoid disease, and advanced therapy was initiated. Following prolonged hospitalization, she decided to opt for hospice care. We feel it is important to update readers on the course of events, as it may impact the care of patients in the future. To our knowledge, carcinoid valvular heart disease that spares the tricuspid valve while affecting the mitral, aortic and pulmonary valves has not been previously described. However, in the light of this development of tricuspid valve disease within 7 months, 2 questions arise: What caused the tricuspid valve deterioration? This may merely have been due Received: January 13, 2016 Accepted: January 13, 2016 Published online: March 5, 2016

Contents Vol. 133, 2016

D.H. Adams, New York, N.Y. C.W. Akins, Boston, Mass. J.S. Alpert, Tucson, Ariz. E.A. Amsterdam, Davis, Calif. W.S. Aronow, Valhalla, N.Y. J.J. Badimon, New York, N.Y. J. Bax, Leiden R.C. Becker, Durham, N.C. G.A. Beller, Charlottesville, Va. P.C. Block, Atlanta, Ga. A.S. Budzikowski, Brooklyn, N.Y. A.J. Camm, London B.A. Carabello, Houston, Tex. P.F. Cohn, Stony Brook, N.Y. J. Coromilas, New Brunswick, N.J. M.H. Crawford, San Francisco, Calif. J.E. Dalen, Tucson, Ariz. S. Dalla Volta, Padova A. Davidson, Philadelphia, Pa. P.C. Deedwania, Fresno, Calif. A.N. De Maria, San Diego, Calif. J.A. Eleft eriades, New Haven, Conn. U. Elkayam, Los Angeles, Calif. C. Erol, Ankara M.D. Ezekowitz, Wynnewood, Pa. R. Ferrari, Ferrara G. Filippatos, Athens G.I. Fishman, New York, N.Y. K. Fox, London G.S. Francis, Minneapolis, Minn. V. Fuster, New York, N.Y. B.J. Gersh, Rochester, Minn. W. Gersony, New York, N.Y. J.P. Gold, Toledo, Ohio R.J. Goldberg, Worcester, Mass. M.E. Goldman, New York, N.Y. P.J. Goldschmidt, Miami, Fla. J. Gore, Worcester, Mass. J.L. Halperin, New York, N.Y. Z.-X. He, Beijing D. Heistad, Iowa City, Iowa C.H. Hennekens, Boca Raton, Fla. I.M. Herling, Wynnewood, Pa. E.M. Herrold, Brooklyn, N.Y. G. Heusch, Essen C.A. Hochreiter, New York, N.Y. M. Hori, Osaka S.K.S. Huang, Temple, Tex. B.K. Kantharia, Houston, Tex. J.S. Karliner, San Francisco, Calif. International Journal of Cardiovascular Medicine, Surgery, Pathology and Pharmacology

The maverick heart sound

Clinical introduction An asymptomatic 29-year-old woman presented for prenatal counselling. She had a history of a heart murmur since childhood and a previous echocardiogram suggesting ‘enlargement of the heart’. Physical exam revealed normal jugular venous pressure and contour. Precordial palpation was unremarkable. Auscultation, however, was abnormal; findings on inspiration and expiration are presented in Figure 1, sound clip. Question Based on the phonocardiogram and online supplementary audio clip, which of the following is correct? An early diastolic filling sound (S3) is heard, indicating increased right ventricular filling pressures. An ejection click without respiratory variation and a systolic ejection murmur are heard, consistent with bicuspid aortic valve stenosis. An ejection click with respiratory variation and a systolic ejection murmur are heard, consistent with pulmonic valve stenosis. A holosystolic murmur with inspiratory augmentation is heard, indicating tricuspid regurgitation.