P Christian Schulze

Publication Details
Article Title: Transthyretin Val30Met mutation in an African American with cardiac amyloidosis.

First Author: P Christian Schulze

All Authors: Schulze PC, Maurer MS

Journal Title: Congestive heart failure (Greenwich, Conn.)


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Anesthesia and analgesia
Prognostic value of troponin I levels for predicting adverse cardiovascular outcomes in postmenopausal women undergoing cardiac surgery.

Authors: Stearns JD, Dávila-Román VG, Barzilai B, Thompson RE, Grogan KL, Thomas B, Hogue CW

Abstract: Adverse cardiac events that follow cardiac surgery are an important source of perioperative morbidity and mortality for women. Troponin I provides a sensitive measure of cardiac injury, but the levels after cardiac surgery may vary between sexes. Our purpose in this study was to evaluate the prognostic value of troponin I levels for predicting cardiovascular complications in postmenopausal women undergoing cardiac surgery.The cohort of this study were women enrolled in a previously reported clinical trial evaluating the neuroprotective potential of 17beta-estradiol in elderly women. In that study, 175 postmenopausal women not receiving estrogen replacement therapy and scheduled to undergo coronary artery bypass graft (with or without valve surgery) were prospectively randomized to receive 17beta-estradiol or placebo in a double-blind manner beginning the day before surgery and continuing for 5 days postoperatively. Serial 12-lead electrocardiograms were performed and serum troponin I concentrations were measured before surgery, after surgery on arrival in the intensive care unit, and for the first four postoperative days. The primary end-point of the present study was major adverse cardiovascular events (MACE) defined as a Q-wave myocardial infarction, low cardiac output state or death within 30 days of surgery. The diagnosis of Q-wave myocardial infarction was made independently by two physicians blinded to treatment and patient outcomes with the final diagnosis requiring consensus. Low cardiac output state was defined as cardiac index <2.0 L x min(-1) x m(-2) for >8 h regardless of treatment.Troponin I levels on postoperative day 1 were predictive of MACE (area under the receiver operator curve = 0.862). A cutoff point for troponin I of >7.6 ng/mL (95% confidence interval, 6.4-10.8) provided the optimal sensitivity and specificity for identifying patients at risk for MACE. The negative predictive value of a troponin I level for identifying a patient with a composite cardiovascular outcome was high (96%) and the positive predictive value moderate (40%). Postoperative troponin I levels were not different between women receiving perioperative 17beta-estradiol treatment compared with placebo and the frequency of MACE was not influenced by 17beta-estradiol treatment.In postmenopausal women, elevated troponin I levels on postoperative day 1 are predictive of MACE. Monitoring of perioperative troponin I levels might provide a means for stratifying patients at risk for adverse cardiovascular events.

Postgraduate medical journal
NICE guidelines for the investigation of stable chest pain: what are the implications for cardiac imaging?

Authors: Hoey ET


Revue neurologique
Cardiac arrhythmia and late-onset muscle weakness caused by a myofibrillar myopathy with unusual histopathological features due to a novel missense mutation in FLNC.

Authors: Avila-Smirnow D, Gueneau L, Batonnet-Pichon S, Delort F, Bécane HM, Claeys K, Beuvin M, Goudeau B, Jais JP, Nelson I, Richard P, Ben Yaou R, Romero NB, Wahbi K, Mathis S, Voit T, Furst D, van der Ven P, Gil R, Vicart P, Fardeau M, Bonne G, Behin A

Abstract: Myofibrillar myopathies (MFM) are mostly adult-onset diseases characterized by progressive morphological alterations of the muscle fibers beginning in the Z-disk and the presence of protein aggregates in the sarcoplasm. They are mostly caused by mutations in different genes that encode Z-disk proteins, including DES, CRYAB, LDB3, MYOT, FLNC and BAG3. A large family of French origin, presenting an autosomal dominant pattern, characterized by cardiac arrhythmia associated to late-onset muscle weakness, was evaluated to clarify clinical, morphological and genetic diagnosis. Muscle weakness began during adult life (over 30 years of age), and had a proximal distribution. Histology showed clear signs of a myofibrillar myopathy, but with unusual, large inclusions. Subsequently, genetic testing was performed in MFM genes available for screening at the time of clinical/histological diagnosis, and desmin (DES), ?B-crystallin (CRYAB), myotilin (MYOT) and ZASP (LDB3), were excluded. LMNA gene screening found the p.R296C variant which did not co-segregate with the disease. Genome wide scan revealed linkage to 7q.32, containing the FLNC gene. FLNC direct sequencing revealed a heterozygous c.3646T>A p.Tyr1216Asn change, co-segregating with the disease, in a highly conserved amino acid of the protein. Normal filamin C levels were detected by Western-blot analysis in patient muscle biopsies and expression of the mutant protein in NIH3T3 showed filamin C aggregates. This is an original FLNC mutation in a MFM family with an atypical clinical and histopathological presentation, given the presence of significantly focal lesions and prominent sarcoplasmic masses in muscle biopsies and the constant heart involvement preceding significantly the onset of the myopathy. Though a rare etiology, FLNC gene should not be excluded in early-onset arrhythmia, even in the absence of myopathy, which occurs later in the disease course.

Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation
Evaluation of quality in adult cardiac surgery: let us speak the same language.

Authors: Noyez L, Kievit PC, Verkroost MW, van Wetten HB, Verhagen AF, van Swieten HA

Abstract: Over the last years, measurements of quality of care have become more and more a public product, used by providers, purchasers and consumers, and patients. This information serves as an important guide for improvement, as well as a decision support tool for everybody taking part in medical treatment. This evolution can be compared with advertising and as in commercials it is important to use the right information. In this report we focus on the quality of adult cardiac surgery. Honest information is of course essential, but in this article attention is asked for the variables used to evaluate the quality of cardiac surgery. (Neth Heart J 2010;18:365-9.).

Prenatal diagnosis
Normal and abnormal fetal cardiac anatomy.

Authors: Cook AC, Yates RW, Anderson RH

Abstract: The heart is often perceived as a difficult organ to understand by ultrasound during fetal life. This is undoubtedly reflected in the low detection rate of cardiac abnormalities as compared to those of most other organ systems in the fetus. In this article we start by updating classical concepts of cardiac embryology, many of which were previously difficult to understand since they were overly simplistic or purely observational. We then lead on to the structure and growth of the fully formed fetal heart where we review the anatomy and ultrasound appearances in detail and provide comparisons with major abnormalities. We emphasise the fact that a solid understanding of cardiac anatomy can enable those involved in fetal medicine to make full use of the views of the heart that are obtained by ultrasound and which are often only transient.

Conference proceedings : ... Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual Conference
A sensitive algorithm for automatic detection of space-time alternating signals in cardiac tissue.

Authors: Jia Z, Bien H, Entcheva E

Abstract: Alternans, a beat-to-beat alternation in cardiac signals, may serve as a precursor to lethal cardiac arrhythmias, including ventricular tachycardia and ventricular fibrillation. Therefore, alternans is a desirable target of early arrhythmia prediction/detection. For long-term records and in the presence of noise, the definition of alternans is qualitative and ambiguous. This makes their automatic detection in large spatiotemporal data sets almost impossible. We present here a quantitative combinatorics-derived definition of alternans in the presence of random noise and a novel algorithm for automatic alternans detection using criteria like temporal persistence (TP), representative phase (RP) and alternans ratio (AR). This technique is validated by comparison to theoretically-derived probabilities and by test data sets with white noise. Finally, the algorithm is applied to ultra-high resolution optical mapping data from cultured cell monolayers, exhibiting calcium alternans. Early fine-scale alternans, close to the noise level, were revealed and linked to the later formation of larger regions and evolution of spatially discordant alternans (SDA). This robust new technique can be useful in quantification and better understanding of the onset of arrhythmias and in general analysis of space-time alternating signals.

The American surgeon
Validation of an updated approach to preoperative cardiac risk assessment in vascular surgery.

Authors: Bui H, Lee JT, Greenway S, Donayre C, de Virgilio C

Abstract: To validate a more selective approach to cardiac assessment which consisted of limiting stress testing and coronary revascularization to highly selected patients and limiting coronary revascularization to patients with severe cardiac symptoms, we compared two time periods (1994-1995 and 2000-2001) with respect to cardiac work-up and cardiac morbidity and mortality. Our method involved a retrospective review of patients undergoing vascular procedures from 2000 to 2001 at a single institution. In group 1 (2000-2001), 139 operations were performed on 120 patients. In group 2 (1994-1995), 145 procedures were performed on 109 patients. Preoperative stress testing was reduced from 42 patients (29%) in group 2 to 20 patients (14%) in group 1 (P < 0.01), and preoperative coronary artery bypass grafting was reduced from six (4.1%) to two (1.4%) (P < 0.28), respectively. Coronary angiography was unchanged: 8 (5.8%) patients in group 1 versus 11 (7.9%) patients in group 2 (P = NS). Two (1.4%) patients underwent percutaneous transluminal coronary angioplasty in group 1 and group 2. Cardiac event rates were similar: seven (5%) patients in both groups. Cardiac death was not significantly different: two (1.4%) in group 1 versus one (0.7%) in group 2. Cardiac morbidity and mortality after major vascular surgery remain the same despite using a more selective cardiac stress protocol.

Experimental and toxicologic pathology : official journal of the Gesellschaft fur Toxikologische Pathologie
Pyruvate attenuates cardiac dysfunction and oxidative stress in isoproterenol-induced cardiotoxicity.

Authors: Ojha S, Goyal S, Kumari S, Arya DS

Abstract: Pyruvate, a potent endogenous antioxidant and an important metabolic fuel is essential for the cardiac function and tissue defense mechanism. The present study was evaluated to investigate whether pyruvate attenuates the development of cardiotoxicity in isoproterenol (ISO)-induced myocardial infarction by assessing hemodynamic, biochemical and histopathological parameters. Subcutaneous injection of ISO (85 mg/kg) administered for 2 days at an interval of 24h was used for induction of cardiotoxicity. ISO administration significantly decreased arterial pressure indices, heart rate, contractility {(+)LVdP/dt} and relaxation {(-)LVdP/dt} and increased left ventricular end-diastolic pressure. In addition, a significant reduction in activities of myocardial creatine phosphokinase-MB, lactate dehydrogenase, superoxide dismutase, catalase, glutathione peroxidase and reduced glutathione levels along with increase in thiobarbituric acid reactive substances were also observed following ISO administration. However, pretreatment with pyruvate (0.25, 0.5 and 1.0 g/kg, p.o.) favorably modulated all most every studied parameters in ISO-induced myocardial injury. Furthermore, protective effect of pyruvate was confirmed by histopathological studies. Rats pretreated only with pyruvate did not produce significant change in hemodynamic, biochemical and histopathological parameters. Pyruvate at 0.50 and 1.0 g/kg doses was found to exert optimal cardioprotective effect against ISO-induced myocardial infarction. The results of our study suggest that pyruvate possessing antioxidant activity has a significant cardioprotective effect against ISO-induced myocardial injury.

JPMA. The Journal of the Pakistan Medical Association
Cardiac tamponade after removal of temporary pace maker in multidiscipinary intensive care unit.

Authors: Khan MF, Zubairi AB

Abstract: Cardiac tamponade is a medical and surgical emergency, which needs early recognition and treatment. Myocardial perforation leading to cardiac tamponade is a rare complication after pace maker insertion. We are reporting a case of cardiac tamponade after removal of temporary pace maker in a multidisciplinary intensive care unit.

Expert review of cardiovascular therapy
The prominent role of cardiac magnetic resonance imaging in coronary artery disease.

Authors: Palios J, Karangelis D, Roubelakis A, Lerakis S

Abstract: The role of cardiac magnetic resonance (CMR) in coronary artery disease is prominent. CMR provides functional and structural heart disease assessment with high accuracy. It allows accurate cardiac volume and flow quantification and wall motion analysis both at rest and at stress. CMR myocardial perfusion studies detect myocardial ischemia and provide insights into the morphology of the myocardial tissue. CMR imaging noninvasively differentiates causes of myocardial injury such as ischemia or inflammation; stages of myocardial injury, such as acute or chronic; grade of myocardial damage, such as reversible or irreversible; myocardial fibrosis or scar. There is an emerging role of CMR in patients with acute chest presentation since it can demonstrate causes of chest pain other than coronary artery disease such as myocarditis, pericarditis, aortic dissection and pulmonary embolism. CMR is noninvasive and radiation-free. It's combined approach of functional and structural cardiac assessment makes it unique compared with other imaging modalities.

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