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Concept: Cardiology

169

BACKGROUND: The presence of myocardial fibrosis is associated with worse clinical outcomes in hypertrophic cardiomyopathy (HCM). Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) sequences can detect regional, but not diffuse myocardial fibrosis. Post-contrast T1 mapping is an emerging CMR technique that may enable the non-invasive evaluation of diffuse myocardial fibrosis in HCM. The purpose of this study was to non-invasively detect and quantify diffuse myocardial fibrosis in HCM with CMR and examine its relationship to diastolic performance. METHODS: We performed CMR on 76 patients - 51 with asymmetric septal hypertrophy due to HCM and 25 healthy controls. Left ventricular (LV) morphology, function and distribution of regional myocardial fibrosis were evaluated with cine imaging and LGE. A CMR T1 mapping sequence determined the post-contrast myocardial T1 time as an index of diffuse myocardial fibrosis. Diastolic function was assessed by transthoracic echocardiography. RESULTS: Regional myocardial fibrosis was observed in 84% of the HCM group. Post-contrast myocardial T1 time was significantly shorter in patients with HCM compared to controls, consistent with diffuse myocardial fibrosis (498 +/- 80 ms vs. 561 +/- 47 ms, p < 0.001). In HCM patients, post-contrast myocardial T1 time correlated with mean E/e' (r = -0.48, p < 0.001). CONCLUSIONS: Patients with HCM have shorter post-contrast myocardial T1 times, consistent with diffuse myocardial fibrosis, which correlate with estimated LV filling pressure, suggesting a mechanistic link between diffuse myocardial fibrosis and abnormal diastolic function in HCM.

Concepts: Cardiology, Cardiomyopathy, Heart, Echocardiography, Nuclear magnetic resonance, Hypertrophic cardiomyopathy, Diastolic dysfunction, Hypertrophy

169

Takayasu’s arteritis is an inflammatory arteriopathy which involves the aorta and its major branches, causing mainly stenosis of their lumen, though aneurysmal lesions can also occur. A young female with Takayasu’s disease presented to our hospital with acute lung oedema due to severe aortic insufficiency and ascending aorta dilatation. She had already undergone distal ascending aorta and hemiarch replacement due to Standford type A aortic dissection five years ago. The patient had also undergone reconstruction of abdominal arteries' stenoses with extraanatomical bypass. We performed a Bentall procedure with a valved conduit and implantation of the coronary ostia as buttons on the conduit. A mechanical valved graft was used instead of a bioprosthesis, due to possible early degradation of a bioprosthesis. The postoperative course was uneventful and the one year follow-up was normal. Valve-sparing aortic root replacement should be avoided in Takayasu’s arteritis due to high rate of recurrent regurgitation.

Concepts: Cardiology, Heart, Aortic aneurysm, Aortic dissection, Aorta, Ascending aorta, Takayasu's arteritis, Arteritis

168

BACKGROUND: Heart failure (HF) patients have a high risk of death, and implantable cardioverter defibrillators (ICDs) are effective in preventing sudden cardiac death (SCD). However, a certain percentage of patients may not be immediate candidates for ICDs, particularly those having a short duration of risk or an uncertain amount of risk. This includes the newly diagnosed patients, as well as those on the cardiac transplant list or NYHA class IV heart failure patients who do not already have an ICD. In these patients, a wearable cardioverter defibrillator (WCD) may be used until long term risk of SCD is defined. The purpose of this study was to determine the incidence of SCD in this population, and the efficacy of early defibrillation by a WCD. METHODS: Ten enrolling centers identified 89 eligible HF patients who were either listed for cardiac transplantation, diagnosed with dilated cardiomyopathy, or receiving inotropic medications. Data collected included medical history, device records, and outcomes (including 90 day mortality). RESULTS: Out of 89 patients, final data on 82 patients has been collected. Patients wore the device for 75+/-58 days. Mean age was 56.8+/-13.2, and 72% were male. Most patients (98.8%) were diagnosed with dilated cardiomyopathy with a low ejection fraction (<40%) and twelve were listed for cardiac transplantation. Four patients were on inotropes. There were no sudden cardiac arrests or deaths during the study. Interestingly, 41.5% of patients were much improved after WCD use, while 34.1% went on to receive an ICD. CONCLUSIONS: In conclusion, the WCD monitored HF patients until further assessment of risk. The leading reasons for end of WCD use were improvement in left ventricular ejection fraction (LVEF) or ICD implantation if there was no significant improvement in LVEF.

Concepts: Myocardial infarction, Cardiology, Cardiomyopathy, Heart failure, Ejection fraction, Implantable cardioverter-defibrillator, Cardiac arrest, Ventricular fibrillation

168

OBJECTIVESThe Trifecta valve (St. Jude Medical) was introduced into clinical practice as a tri-leaflet stented pericardial valve designed for supra-annular placement in the aortic position. The present study aims to evaluate the preliminary results with this new bioprosthesis.METHODSSeventy patients underwent aortic valve replacement (AVR) with the Trifecta valve between August 2010 and December 2011. Thirty-three patients were male and 37 were female (52.9%). Mean age was 74.65 ± 7.63 (range 47-90 years). Prevalent cause of AVR was aortic stenosis in 64 (91.43%) patients. The mean preoperative pressure gradient was 50 ± 17 (range 20-84 mmHg), and the mean aortic valve area was 0.77 ± 0.33. Five (7.14%) patients were operated on due to aortic valve endocarditis. One patient was operated on due to isolated, severe aortic insufficiency. All patients were in New York Heart Association functional class III or IV. Twenty-eight (40%) patients underwent concomitant procedures.RESULTSConcomitant procedures were coronary artery bypass grafting (n = 25), mitral valve replacement (n = 1), ablation of atrial fibrillation (n = 1) and septal myomectomy (n = 1). There were no intraoperative deaths. The 30-day in-hospital mortality was 2.85% (2 of 70). One late death occurred during the in-hospital stay due to a multiorgan failure on postoperative day 60. There were 2 (2.85%) perioperative strokes. Mean pressure gradient decreased significantly from a preoperative value of 50 ± 17 mmHg to an intraoperative gradient of 9 ± 4 mmHg (Table 3). The mean gradients were 14, 11, 11, 8 and 6 mmHg for the 19, 21, 23, 25 and 27 mm valve size, respectively. No prosthesis dislocation, endocarditis, valve thrombosis or relevant aortic regurgitation was observed at discharge.CONCLUSIONSThe initial experience with the Trifecta valve bioprosthesis shows excellent outcomes with favourable early haemodynamics. Further studies with longer follow-up are needed to confirm those preliminary results.

Concepts: Cardiology, Heart, Mitral valve prolapse, Aortic valve, Valvular heart disease, Aortic valve stenosis, Aortic valve replacement, Aortic insufficiency

168

BACKGROUND: Type VI dual left anterior descending artery (LAD) is a rare coronary anomaly, the first case of which has recently been described. This is the first report of type VI dual LAD anomaly in which the patient presented with non-ST-segment elevation myocardial infarction and percutaneous coronary intervention was performed in the anomalously originating LAD. CASE PRESENTATION: A 52-year-old man with diabetes, hypertension and hyperlipidemia presented with chest pain without ST elevation on EKG, although the patient’s troponin I level was elevated. Coronary angiography revealed a short LAD originating from the left main coronary artery and a long LAD originating from the proximal portion of the right coronary artery (RCA). Three-dimensional reconstruction of computed tomography of images revealed that the long LAD originated from the proximal RCA and coursed between the right ventricular outflow tract (RVOT) and the aortic root before entering the mid anterior interventricular groove. The high take-off RCA originated underneath the RVOT, pointing downwards and forming an acute angle with the proximal portion of the long LAD. The anomalous long LAD displayed significant stenosis. We performed successful percutaneous coronary intervention (PCI) in the anomalous artery. CONCLUSION: With accurate understanding of the coronary anatomy and appropriate hardware selection, successful PCI can be performed in the in the long LAD in patients with type VI dual LAD anomaly.

Concepts: Myocardial infarction, Atherosclerosis, Angina pectoris, Cardiology, Heart, Atheroma, Cardiovascular system, Left coronary artery

167

Chronic day-to-day symptoms of orthostatic intolerance are the most notable features of postural orthostatic tachycardia syndrome (POTS). However, we have encountered patients with such symptoms and excessive tachycardia but with no symptoms during the tilt-table test (TTT). We aimed to investigate whether POTS patients with chronic orthostatic intolerance always present orthostatic symptoms during the TTT and analyze the factors underlying symptom manifestation during this test.

Concepts: Medical terms, Cardiology, Symptoms, Symptom, Orthostatic hypotension, Post-concussion syndrome, Postural orthostatic tachycardia syndrome, Orthostatic intolerance

167

Aim: To determine the effect of a 12-month intent-to-treat tesosterone replacement therapy (TRT) trial on QTa interval variability (QTaVI) in hypogonadal (HG) men with spinal cord injury (SCI). Method: A prospective, controlled, 12-month TRT trial was completed in twenty-two healthy, chronic, non-ambulatory men with SCI. Based on serum T concentration, subjects were designated as HG (≤11.3 nmol/l) or eugonadal (EG, ≥11.4 nmol/l). Digital 3-lead electrocardiograms were performed. Heart rate (RR), heart rate variability [(HRV), including total power (TP(RR)), low frequency (LF(RR)) and high freguency (HF(RR))], QTa, QTe, and RT intervals, QTC (Bazett), QTVN, and QTaVI were calculated and evaluated at baseline and 12 months. Lipoprotein profiles (triglycerides, total cholesterol, low density and high-density lipoproteins) were obtained at the respective time points. Results: Based on serum T concentration, 13 subjects were designated as HG and 11 EG. During the trial, there were no group differences for RR, QTa, QTe or RT intervals, QTC, TP(RR), HF(RR), or lipoproteins. The HG was older (p < 0.05) and LF(RR) was lower (p < 0.05) at baseline. At baseline, QTaVI was significantly greater in HG compared to EG [-0.17 (0.92) vs. -1.07 (0.90); p < 0.05]. After TRT, this group difference was no longer present [-0.44 (0.87) vs. -0.65 (0.85)] and the change in HG was significant (p < 0.05). Conclusion: Hypogonadism in men with SCI was associated with elevated QTaVI at baseline. After 12 months of physiological TRT, the QTaVI improved in association with raising T into the normal range. These findings occurred independently from the prolongation of the QT interval.

Concepts: Cholesterol, Cardiology, Lipoprotein, Lipoproteins, Cardiac electrophysiology, Testosterone, QT interval, Hypogonadism

167

Abstract Objective. We studied the relationship between systolic blood pressure and hemodynamics using impedance cardiography in elderly Han residents in order to evaluate how different hemodynamic variables are altered with normal aging and with hypertension superimposed on aging. Methods. A total of 670 subjects, aged 60-93 years, were evaluated with impedance cardiography for non-invasive hemodynamic variables. The subjects were categorized as hypertensives or normotensives, and then they were also divided into six subgroups according to actual systolic blood pressure values. Results. Hypertensive patients had significantly lower values of cardiac output (4.4 ± 1.5 L/min) and cardiac index (2.6 ± 1.0 L/min/m(2)) than those in the normotensive group (4.7 ± 1.5 L/min, and 2.8 ± 0.8 L/min/m(2), respectively; P < 0.05 for both). Compared to the normotensive group, stroke volume and stroke index values were also lower and systemic vascular resistance and systemic vascular resistance index were higher in the hypertensive group. There were no significant differences in left ventricular stroke work and left ventricular stroke work index between the two groups. When all 670 subjects were stratified to actual blood pressure, cardiac output of group 6 patients (systolic blood pressure ≥180 mmHg) was 19% lower than that of group 1 subjects (SBP <140 mmHg; P < 0.05). Similarly, systemic vascular resistance of group 6 patients was 56% higher than that of group 1 subjects (P < 0.05). Conclusion. With aging, arterial systolic blood pressure is elevated as a result of increased arterial stiffness and increased systemic vascular resistance. With hypertension, these values are further elevated. Non-invasive impedance cardiography helps to characterize the hemodynamic mechanisms, which can improve hypertension management.

Concepts: Blood, Myocardial infarction, Hypertension, Cardiology, Blood pressure, Artery, Vein, Total peripheral resistance

166

Anomalies of coronary number and course represent an opinion-dividing topic in cardiopathology, particularly for their relationship with sudden cardiac death. To the best of our knowledge, we herein report the first fatal case of a young female whose coronary anatomy was characterised by the absence of any septal perforator branch in the proximal segment of the LAD. This case could be useful for pathologists, coronary angiographers, and interventional cardiologists in detecting this infrequent anomaly, thus providing a more accurate estimation of its incidence. Virtual Slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3570015858473043.

Concepts: Atherosclerosis, Cardiology, Heart, Cardiac arrest, Artery, Coronary circulation, Interventional cardiology, Sudden cardiac death

166

BACKGROUND: Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) experience impaired health-related quality of life (HRQL). The objective of this study was to evaluate HRQL in a nation-wide sample. PATIENTS AND METHODS: This is a prospective, multicenter, non-interventional study of HRQL including 139 (89%) PAH and 17 (11%) CTEPH patients (women 70.5%; mean age, 52.2) recruited from 21 Spanish hospitals. 55% had idiopathic PAH, 34% other PAH and 11% CTEPH. HRQL was measured using the Short Form 36 Health Survey (SF-36) and EuroQoL-5D (baseline and after 6 months). RESULTS: HRQL in the patients with PAH or CTEPH was impaired. The physical component of SF-36 and the EuroQol-5D correlated with the functional class (FC). Mean EuroQol-5D visual analogical scale (EQ-5D VAS) scores were 73.5±18.4, 62.9±20.7 and 51.3±16.0 (P<.0001) in patients with FC I, II and III, respectively. Every increase of one FC represented a loss of 4.0 on the PCS SF-36 and a loss of 9.5 on the EQ-5D VAS. Eight patients who died or received a transplant during the study period presented poorer initial HRQL compared with the rest of the population. No significant changes in HRQL were observed in survivors after 6 months of follow-up. CONCLUSIONS: HRQL is impaired in this population, especially in PAH/CTEPH patients near death. HRQL measurements could help predict the prognosis in PAH and CTPH and provide additional information in these patients.

Concepts: Pulmonology, Cardiology, Blood pressure, Pulmonary artery, Pulmonary hypertension, Iloprost