Normal Ventricular Function Research Articles

Use of ACE inhibitors in Fontan: Rational or irrational?

BackgroundDespite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry. MethodsAll patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase. ResultsIn 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p<0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation. ConclusionIt is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population.

Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy?

BackgroundIn patients with Fontan circulation, the liver is profoundly affected by chronic venous stasis. Little is known about early hepatic changes in this population. MethodsWe performed echocardiography, abdominal ultrasound, liver elastography, cardiac catheterization, esophago-gastro-duodenoscopy and calculated MELD-XI score in 64 Fontan patients (69% minors), at an interval of 1–15years since Fontan. ResultsCardiac output remained stable in the first 5years after Fontan, then significantly decreased (r=−0.45, p(r=0)=0.003). NYHA class significantly increased after Fontan. Patients in NYHA class II/III (n=21, 14 minors) had significantly higher hepatic pressures, but normal ventricular function and pulmonary vascular resistances (PVR). Patients with pulmonary arterial pressure (PAP) ≥15mmHg (n=12, 6 minors) and those with PVR≥2WU*m2 (n=27, 25 minors), had higher hepatic pressures (p<0.0001), a higher incidence of liver collaterals and/or esophageal varices (p<0.0001) and splenomegaly (p<0.02).Liver stiffness (LS) was elevated in most patients (median, 25th–75th percentile:17.3KPa, 14.1–21.4). It rapidly increased during the first 5-years after Fontan, compared to the following 5-years (from 12.2KPa, 9.8–14.1 to 17.5KPa, 14.3–24.5, p=0.007), then remained stable (19.1KPa, 16.9–22.6, p=0.60). MELD-XI score increased linearly with the time interval since Fontan (r=0.31, p(r=0)=0.01). For patients above 12years we found a linear correlation between LS and MELD-XI score in the 6–15years period after Fontan (r=0.40. p(r=0)=0.04). The overall incidence of established liver cirrhosis was 22%. ConclusionsThis is the largest study showing that Fontan circulation prompts early, progressive and eventually irreversible liver damage. Precautions should be taken immediately after Fontan, to protect this fragile population.

0201: Cardiac involvement in patients with sarcoidosis

Sarcoidosis is a systemic granulomatous disease of unknown etiology. Cardiac involvements in patients with sarcoidosis are rare with an estimated prevalence of 7%. These serious involvements are hard to diagnose at early stage of disease. We performed a retrospective study of patients’ files diagnosed with sarcoidosis in the Internal Medicine Department of Rabta University Hospital in Tunis. The diagnosis of sarcoidosis was based on clinical, paraclinical and histological criteria. Cardiac involvements were confirmed by the electrocardiogram and echocardiography. Cardiac involvement (CI) was found in seven patients among 138 with sarcoidosis (5%). They were 6 females and one male with a mean age at diagnosis of 49 years. Electrical disorders such as bundel branch block were seen in two cases. First degree atrioventricular block was noted in two cases. Ventricular hypertrophy was check out in two cases by electrocardiography and ultrasound. Mitral regurgitation with a flutter was noted in one patient and pericardial effusion with a normal ventricular function in another case. Only one patient was clinically symptomatic, he complained of palpitations. Thallium 201 scintigraphy was performed in one case and objectived reversible ischemia of the inferior and latero-inferior walls of myocardium. Other manifestations of sarcoidosis were dominantly associated to CI such as lung disease, which was found in all patients. Skin and neurological involvement were observed, each in three cases. Hepatosplenic involvements were noted in two patients and otorhinolaryngology involvements in one case. All patients received initially high dose of corticosteroids. Four of the patients had a good response to treatment and three were lost to follow up. Cardiac involvements in sarcoidosis are rare but serious, an early diagnosis and treatment with corticosteroid may rapidly improve patients.

High hemodialysis vascular access flow and impaired right ventricular function in chronic hemodialysis patients.

There are limited data showing right ventricular preload increase due to high-flow arteriovenous fistulas (AVFs). This cross-sectional study investigated whether high AVF flow had an impact on right ventricular function in patients undergoing hemodialysis. Sixty-four patients aged between 18 and 85 years who were on routine hemodialysis with >2 hemodialysis sessions per week for at least 3 months via an AVF were studied. Patients with inadequate flow fistulas, severe chronic obstructive pulmonary disease, history of pulmonary embolism, primary pulmonary hypertension, severe mitral, aortic or pulmonary regurgitation, and/or stenosis were excluded. After an initial evaluation, 44 patients (mean age: 58.50 ± 16.84, male:female = 23:21) were considered eligible. Right ventricular function was assessed by tricuspid annular plane systolic excursion (TAPSE). AVF blood flow was measured with duplex ultrasound. There were 15 patients (34.1%) with a TAPSE of <16 mm. AVF blood flow was significantly higher in patients with impaired versus normal right ventricular function (1631.53 ± 738.17 vs. 1060.55 ± 539.92 min/ml, respectively, P = 0.003). Low left ventricular ejection fraction (odds ratio [OR]: 1.15, 95% confidence intervals [CI]: 1.007-1.334, P = 0.04), high interventricular septum thickness (OR: 1.64, 95% CI: 1.104–2.464, P = 0.01), and high AVF blood flow (OR: 1.00, 95% CI: 1.000-1.003, P = 0.03) were independent predictors of impaired right ventricular function. In addition to known risk factors that predominantly increase right ventricular afterload, excessive AVF blood flow was found to be independently associated with impaired right ventricular function, possibly by increasing right ventricular preload.

ECG In Evaluation for Pulmonary Thromboembolism- Occam's Razor or Hickam's Dictum?

The electrocardiogram (ECG) is a simple, universally available tool in emergency departments used in evaluation of acute chest pain. Among the differential diagnosis, Acute Pulmonary Thromboembolism (PTE) is important to recognize as the diagnosis, carries serious therapeutic and prognostic implications. A 28-year-old male presented to the emergency department with acute onset shortness of breath and chest pain for two days and two episodes of haemoptysis. He had sustained a road traffic accident with fracture of both bones of right leg one month back for which he had undergone open reduction and internal fixation. On examination, he was tachycardic (pulse rate-152/min) and tachypnoeic (respiratory rate-26/min) with normal blood pressure (116/78 mm of Hg) and oxygen saturation (96%) on breathing ambient air. His right lower limb was warm and swollen with tenderness along the femoral vein. The electrocardiogram (ECG) showed sinus tachycardia, right ventricular strain, right bundle branch block pattern and S1Q3T3 pattern [Table/Fig-1] suggestive of PTE. Compression ultrasound scan of right lower limb revealed echogenic contents in right femoro-popliteal veins indicative of Deep Vein Thrombosis (DVT). Computed tomographic angiogram of pulmonary arteries showed hypodense filling defect suggestive of thrombus involving the right main pulmonary artery [Table/Fig-2], extending into its descending branch. A 2-dimensional echocardiogram showed mild tricuspid regurgitation and mild right ventricular dilatation. Since the patient was haemodynamically stable, thrombolysis was not attempted. He was started on subcutaneous enoxaparin at 1 mg/kg/dose twice daily along with oral anticoagulation with warfarin. Patient improved and was discharged. On follow-up at one month, patient is asymptomatic for DVT or PTE and INR is in therapeutic range. [Table/Fig-1]: A 12-lead electrocardiogram (ECG) showing sinus tachycardia (heart rate- 150/min), right bundle branch block pattern (black arrows), T-inversion in aVF and typical S1Q3T3 pattern (red arrows) suggestive of pulmonary thromboembolism. [Table/Fig-2]: Axial computed tomographic angiogram section of pulmonary arteries showing hypodense filling defect (arrowhead) suggestive of thrombus involving the right main pulmonary artery. Common ECG abnormalities in PTE include sinus tachycardia and T wave inversions in precordial leads and a ventricular fibrillation (VF), right bundle branch block pattern, S1Q3T3 pattern, QR pattern in V1, QS pattern in precordial leads, ST-elevation in precordial leads and low voltage in limb leads. Classic changes like S1Q3T3 pattern tend to occur with more severe forms of PTE and correlate well with mortality [1]. S1Q3T3 pattern is a marker of acute cor-pulmonale and can occur with conditions like severe bronchospasm and pneumothorax [2]. Composite scores based on ECG in PTE can help in patient management. Using a 21-point scale, a score ≤3 in predicted normal right ventricular function [3]. QR in V1, ST-depression in V4-V6 and ST-elevation in lead III and V1 are associated with in-hospital mortality [1]. This case illustrates the classical ECG findings of PTE and the importance of an easily available, simple diagnostic test in diagnosing a life threatening condition. The classical signs in ECG can be used as a decision tool for further imaging and can also be used as an indicator for early referral of a patient especially in resource poor settings. To conclude, ECG in PTE can show a spectrum of changes from being normal to having multiple suggestive abnormalities. The ECG changes in PTE are not specific and similar changes may result from wide array of causes conferring to the principle of Hickam’s dictum. In general, the number of abnormalities on ECG correlates well with pulmonary artery pressures and the severity of PTE. Even though ECG can give a clue to the diagnosis of PTE, its actual role lies in prognostic assessment and hence can be used as a decision tool for further imaging and investigations especially in resource poor settings.

Right ventricular outflow tract function in chronic heart failure

BackgroundHeart failure (HF) is a common, progressive, complex clinical syndrome and a subset of HF patients has symptoms out of proportion to the resting hemodynamics and left ventricular ejection fraction (LVEF). Right ventricular (RV) function is a powerful prognostic factor in HF, but assessing it is a challenge because of the right ventricle's complex geometry. ObjectiveThe aim of this study was to investigate the clinical application value of RV outflow tract (RVOT) function measured by transthoracic echocardiography in HF patients. MethodWe prospectively investigated 36 chronic HF patients with dilated heart and LV systolic dysfunction and 21 healthy control subjects (normal ventricular function and ECG, and no cardiac risk factors). In addition to clinical and conventional echocardiographic parameters, RVOT size and fractional shortening (RVOT-FS) parameters were analyzed. ResultsThe RVOT-FS was less in HF patients than healthy controls (18.8±15.7 vs 55.8±6.7, p<0.001) and correlated positively with TAPSE (r=0.814, p<0.001) and inversely with SPAP (r=−0.728, p<0.001) and functional capacity (r=−0.842, p<0.001). There was a statistically significant difference in RVOT-FS among the HF subgroups with regard to NYHA functional capacity (p<0.001), although there was no statistically significant difference with regard to LVEF. ConclusionAlthough the apparent discordance between LVEF and the degree of functional impairment in HF is not well understood, it may be explained in part by alterations in RV function. We found that the RVOT-FS was a noninvasive and easily applicable measure of RV function and might be used for a comprehensive evaluation and follow-up of HF patients with a combined assessment of RV by other RV parameters.

Abstract 9909: Selenium Supplementation Rescues Cardiac Function in Patients With Severe Heart Failure Who are Intractable to Renin-Angiotensin System Inhibitors and β-blockers

Introduction: Increases in oxidative stress may be involved in the progression of heart diseases. Selenium (Se) is an integral part of the enzyme glutathione peroxidase (GSHPx), one of the central players of the heart’s antioxidant system. This study evaluated Se supplementation in patients with severe heart failure who were intractable to renin-angiotensin system (RAS) inhibitors and β-blockers. Methods: In order to confirm the activation of myocardial oxidative stress and the local deficiency of GSHPx, we examined immunohistochemical staining of endomyocardial biopsy specimens obtained from 24 dilated cardiomyopathy (DCM) patients and analyzed serum Se concentrations and GSHPx activities. We enrolled patients who had shown no functional improvements after RAS inhibitors and β -blockers (n = 11). The patients were supplied with 25 μg of Se chloride twice per day while their other medications remained unchanged. Results: Compared with patients with normal ventricular function (n = 30), the serum Se concentrations and GSHPx activities from the patients with DCM (n = 24) were decreased by 55% (0.167 μg/ml vs 0.076 μg/ml, p &lt; 0.01), and by 33% (328 μmol/min/L vs 221 μmol/min/L, p &lt; 0.01), respectively. Myocardial biopsy from DCM patients, but not control patients, uniformly showed marked reduction of GSHPx stains (24/24 vs 0/3, respectively) that was accompanied by positive staining for both 4-hydroxy-2nonenal (20/24 vs 1/3) and 8-hydroxy-2`-deoxyguanosine (16/24 vs 0/3). Left ventricular (LV) ejection fraction increased from 26% ± 8% to 34% ± 8%, 37% ± 12%, and 38% ± 11% at the 3-month follow-up, 6-month follow-up, and 12-month follow-up, respectively, and then maintained thereafter. Conclusions: Our study suggests that myocardial oxidative stress in chronic heart failure may be augmented at least in part by concomitant GSHPx deficiency, and that the administration of Se could rescue the exhaustion of this selenoprotein, resulting in improved LV function.

Abstract 15700: L-type Calcium Channels Localization and Function are Affected by Microdomain Remodelling in Human Failing Cardiomyocytes

Introduction: L-type calcium channels (LTCCs) are essential in determining the electrical and mechanical properties of the cardiac muscle. LTCCs are located in specialized membrane microdomains in cardiac cells. Disruption of their microdomain localization in heart failure may contribute to the pathophysiology of cardiac disease. Methods: We studied isolated ventricular cardiomyocytes from patients with dilated cardiomyopathy with or without Left Ventricular Assistance Device (DCM or DCM+LVAD) versus those with normal ventricular function (nonfailing). Super-resolution scanning patch clamp provided topography images of the cell surface which enabled us to record LTCC current in distinct cellular compartments (T-tubules openings and areas between them, crests). Confocal microscopy was used to score T-tubular density and regularity in Di-8-ANEPPS stained cells. Expression of β2 subunit of LTCC was detected by immunofluorescence, Western blot and qPCR. Results: In failing cells T-tubular density is lower than in normal. The loss of cellular micro-architecture in failing cardiomyocytes leads to a redistribution of functional LTCCs from their usual location (T-tubules) to non-native crests of the sarcolemma. These relocated LTCCs in the crest of DCM cardiomyocytes have increased open probability and lower amplitude as compared with the channels located on the T-tubules. Interestingly, in cardiomyocytes isolated from patients with LVAD the open probability of LTCC is reduced dramatically and the amplitude recovers to the normal level, as does the T-tubular density. Immunostaining of DCM cardiomyocytes shows an increased amount of LTCC β2 subunit in the crest, and Western blot/qPCR shows more abundant expression of LTCC β2 subunit. Conclusion: In DCM cardiomyocytes a considerable amount of LTCCs improperly located on the crest microdomain display a pathologically high activity; this study suggests that the increased expression of LTCC β2-subunit could be responsible. Although LVAD cannot reverse the remodelling in DCM cardiomyocytes it helps to restore part of the microarchitecture of the cells and to normalize the function of the LTCCs.

Effects of acute kidney injury and chronic hypoxemia on fibroblast growth factor 23 levels in pediatric cardiac surgery patients.

Fibroblast growth factor-23 (FGF23) levels are elevated in cardiopulmonary bypass (CPB)-associated acute kidney injury (AKI); however, it is unknown how much of the circulating FGF23 is intact and bioactive. Hypoxia may induce FGF23 production, yet its impact in humans is unknown. Pediatric cardiac surgery patients have both a high incidence of CPB-associated AKI and a high prevalence of chronic hypoxemia. We assessed the effects of hypoxemia and CPB-associated AKI on C-terminal FGF23 (cFGF23) and intact FGF23 (iFGF23) levels in 32 pediatric cardiac surgery patients with normal estimated glomerular filtration rate (eGFR). Plasma cFGF23 and iFGF23 were measured preoperatively and serially postoperatively. Despite normal renal and ventricular function, preoperative cFGF23 levels were high and elevated out of proportion to iFGF23 levels. Preoperative oxygen saturation measurements correlated inversely with FGF23 levels. Preoperative cFGF23 and oxygen saturation both predicted postoperative AKI. Postoperatively, cFGF23 and iFGF23 increased by 2h postreperfusion; iFGF23 then returned to baseline, but cFGF23 remained elevated through 24 h postreperfusion. Group status (AKI vs. non-AKI) modified the effect of time on changes in iFGF23 levels but not cFGF23 levels. Preoperative cFGF23 may predict CPB-associated kidney dysfunction. Changes over time in cFGF23 and iFGF23 levels post-CPB differ. Chronic hypoxemia may affect FGF23 production in humans.

Trauma induced myocardial infarction

Chest Trauma in athletes is a common health problem. However, myocardial infarction secondary to coronary dissection in the setting of blunt chest trauma is extremely rare. We report a case of acute inferior wall myocardial infarction following blunt chest trauma. A 32-year-old male with no relevant medical problems was transferred to our medical center for retrosternal chest pain after being elbowed in the chest during a soccer game. Few seconds later, he started experiencing sharp retrosternal chest pain that was severe to that point where he called the emergency medical service. Upon arrival to the trauma department patient was still complaining of chest pain. ECG demonstrated ST segment elevation in the inferior leads with reciprocal changes in the lateral leads all consistent with active ischemia. After rolling out aortic dissection, patient was loaded with ASA, ticagerlor, heparin and was emergently taken to the cardiac catheterization lab. Coronary angiography demonstrated 100% thrombotic occlusion in the distal right coronary artery with TIMI 0 flow distally. After thrombus aspiration, a focal dissection was noted on the angiogram that was successfully stented. Two days after admission patient was discharged home. Echocardiography prior to discharge showed inferior wall akinesis, normal right ventricular systolic function and normal overall ejection fraction.

Changes in cardiac function after pedicle subtraction osteotomy in patients with a kyphosis due to ankylosing spondylitis.

Cardiac disease in patients with ankylosing spondylitis (AS) has previously been studied but not in patients with a kyphosis or in those who have undergone an operation to correct it. The aim of this study was to measure the post-operative changes in cardiac function of patients with an AS kyphosis after pedicle subtraction osteotomy (PSO). The original cohort consisted of 39 patients (33 men, six women). Of these, four patients (two men, two women) were lost to follow-up leaving 35 patients (31 men, four women) to study. The mean age of the remaining patients was 37.4 years (22.3 to 47.8) and their mean duration of AS was 17.0 years (4.6 to 26.4). Echocardiographic measurements, resting heart rate (RHR), physical function score (PFS), and full-length standing spinal radiographs were obtained before surgery and at the two-year follow-up. The mean pre-operative RHR was 80.2 bpm (60.6 to 112.3) which dropped to a mean of 73.7 bpm (60.7 to 90.6) at the two-year follow-up (p = 0.0000). Of 15 patients with normal ventricular function pre-operatively, two developed mild left ventricular diastolic dysfunction (LVDD) at the two-year follow-up. Of 20 patients with mild LVDD pre-operatively only five had this post-operatively. Overall, 15 patients had normal LV diastolic function before their operation and 28 patients had normal LV function at the two-year follow-up. The clinical improvement was 15 out of 20 (75.0%): cardiac function in patients with AS whose kyphosis was treated by PSO was significantly improved.

Acute Hemodynamic Response to Cardiac Resynchronization in Dilated Cardiomyopathy: Effect on Late Mitral Regurgitation.

Functional mitral regurgitation (FMR) is associated with reduced survival in dilated cardiomyopathy (DCM). Cardiac resynchronization therapy (CRT) can improve FMR. We sought to identify the predictors of FMR improvement after CRT in DCM. From January 2003 to December 2013, 430 DCM patients consecutively enrolled were retrospectively analyzed. Inclusion criteria were successful CRT implantation in the presence of conventional indications (i.e., left bundle branch block, left ventricular ejection fraction ≤35%, New York Heart Association functional class ≥II) and moderate-to-severe FMR at the time of procedure. Early echocardiographic evaluation after CRT implantation (median 2.5 days) has been performed in each patient. Improvement in FMR (absent/mild) at midterm (7 months; interquartile range 4-10) was considered as the primary study end point. A total of 44 patients (10% of the overall cohort) were included. A significant reduction in FMR severity was observed in 21 patients (48%) at midterm after CRT (median time 7 months). No preimplantation variables predicted FMR evolution, but FMR improvement at midterm was strongly predicted by an acute favorable hemodynamic response (persistence/development of normal right ventricular function and 10-mm Hg decrease or normalization [≤35 mm Hg] of systolic pulmonary artery pressure) at postimplantation echocardiography (odds ratio: 13.7; 95% confidence interval: 1.27-42.8; P = 0.016). FMR improvement at midterm was stable during follow-up and was associated with superior long-term transplant-free survival (P = 0.022). Stable FMR improvement frequently occurs after CRT implantation in DCM and is associated with improved transplant-free survival. Echocardiographic evaluation of acute hemodynamic response to CRT is helpful to early identification of the favorable FMR evolution.

Role of Extracorporeal Membrane Oxygenation in Aluminum Phosphide Poisoning–Induced Reversible Myocardial Dysfunction: A Novel Therapeutic Modality

BackgroundAluminum phosphide (AlP) poisoning carries a high rate of mortality despite intensive care management, primarily because of refractory myocardial depression, resistant hypotension, and severe metabolic acidosis as well as acute respiratory distress syndrome. Extracorporeal membrane oxygenation (ECMO) is a modified “heart–lung” machine to provide temporary cardiorespiratory support. We studied the novel use of ECMO in the management of a subset of patients with AlP poisoning. Case ReportIn this case series, seven patients with AlP poisoning suffering from severe metabolic acidosis and refractory cardiogenic shock with a reduced left ventricular ejection fraction (<35%) received ECMO treatment. The acidosis and hemodynamic status improved within 6–12 h and 12–24 h, respectively, in five patients. Two patients did not survive because of a long delay in presentation after ingestion. The majority of the patients developed dysrhythmias, ECMO cannulation site bleeding, and thrombocytopenia. Two patients required surgical exploration of the femoral artery. At 9 months of follow-up, all five surviving patients were doing well, with the near normalization of ventricular function. Why Should an Emergency Physician be Aware of This?We have found that timely intervention with ECMO in patients with AlP poisoning–induced severe metabolic acidosis and refractory cardiogenic shock may lead to a significant improvement in overall survival. Therefore, ECMO might be considered as a bridge therapy for patients with intractable cardiorespiratory failure caused by AlP poisoning who are not responding to conventional treatment. ECMO, however, also is associated with significant complication rates, which must be incorporated into the risk-benefit analysis while considering treatment options.

Evaluation of tricuspid annular plane systolic excursion measured with cardiac MRI in children with tetralogy of Fallot.

Aneurysmal dilation of the right ventricular outflow tract complicates assessment of right ventricular function in patients with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is commonly used to estimate ejection fraction. We hypothesised that tricuspid annular plane systolic excursion measured by cardiac MRI approximates global and segmental right ventricular function, specifically right ventricular sinus ejection fraction, in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion was measured retrospectively on cardiac MRIs in 54 patients with repaired tetralogy of Fallot. Values were compared with right ventricular global, sinus, and infundibular ejection fractions. Tricuspid annular plane systolic excursion was indexed to body surface area, converted into a fractional value, and converted into published paediatric Z-scores. Tricuspid annular plane systolic excursion measurements had good agreement between observers. Right ventricular ejection fraction did not correlate with the absolute or indexed tricuspid annular plane systolic excursion and correlated weakly with fractional tricuspid annular plane systolic excursion (r=0.41 and p=0.002). Segmental right ventricular function did not appreciably improve correlation with any of the tricuspid annular plane systolic excursion measures. Paediatric Z-scores were unable to differentiate patients with normal and abnormal right ventricular function. Tricuspid annular plane systolic excursion measured by cardiac MRI correlates poorly with global and segmental right ventricular ejection fraction in children with repaired tetralogy of Fallot. Tricuspid annular plane systolic excursion is an unreliable approximation of right ventricular function in this patient population.

Frequent Premature Ventricular Contractions: An Electrical Link to Cardiomyopathy.

Heart failure is common and is associated with significant morbidity and mortality. Identifying potentially modifiable risk factors for the development of ventricular dysfunction is important in both the prevention and the treatment of this condition. Arrhythmia disorders are increasingly recognized as contributory to the development of ventricular failure. Poorly controlled supraventricular tachyarrhythmias, altered left ventricular activation due to left bundle branch block or right ventricular pacing, and frequent premature ventricular contractions (PVCs) constitute the main subtypes of arrhythmia disorders that are associated with the development of ventricular dysfunction. PVCs are common and are considered benign in the absence of structural heart disease. Frequent PVCs, defined as greater than 20% of all QRS complexes on standard 24-hour Holter monitoring, are associated with the presence or subsequent development of left ventricular dilatation and dysfunction. Catheter ablation of frequent PVCs has been demonstrated to be effective at PVC suppression and is associated with improvement or normalization of ventricular function; thus defining a specific, reversible form of ventricular dysfunction termed PVC cardiomyopathy. In patients presenting with high burden PVCs, an assessment for symptoms and associated cardiomyopathy is warranted and, in the appropriate clinical setting, PVC catheter ablation may be a reasonable treatment option.

Heart beats: not to be beaten.

Heart beats: not to be beaten.

Cardiac syndrome X: Clinical characteristics revisited

BackgroundCardiac syndrome X includes a heterogenous group of patients with angina but normal epicardial coronaries in angiography. ObjectiveOur objective was to study the clinical characteristics of patients with cardiac syndrome X. MethodsData of patients who underwent coronary angiography over a period of one year was retrospectively analyzed. Those with normal or non-obstructive coronaries in angiography with chest pain were included in this study. Results1203 patients underwent coronary angiography during the study period. 105 (8.7%) patients fulfilled the inclusion criteria. There were 52 (49.5%) males and 53 (50.5%) females including 31 (29.5%) postmenopausal women. Many patients had atherosclerotic risk factors. Typical angina and atypical chest pain were reported by 63 (60%) and 42 (40%) patients, respectively. ECG was normal in 46 (43.8%) and abnormal in 59 (56.2%) patients. The most common abnormal finding in ECG was ST-T changes seen in 49 (46.7%) patients. Regional wall motion abnormality with mild left ventricular systolic dysfunction was seen in 4 (3.8%) patients while 101 (96.2%) patients had normal ventricular function in echocardiography. TMT was positive for inducible ischemia in 35 (33.3%) patients and inconclusive in 10 (9.5%) patients. Angiography showed normal epicardial coronaries in 85 (80.9%) patients. ConclusionsCardiac syndrome X constitutes a significant subset of patients undergoing coronary angiography. It is essential to identify and treat them specifically for microvascular angina. Many of them have atherosclerotic risk factors but their presentation is different from those with obstructive coronaries.

Atypical aortic coarctation as a cause of a cardiomyopathy.

Atypical locations for aortic coarctation have been previously described. However, to our knowledge, no case has been described of a rapidly progressive dilated cardiomyopathy caused by an atypical coarctation, with a rapid normalisation of ventricular function after treatment.

Abstract 144: Lung Parenchymal Consolidation during typical Kawasaki Disease

Introduction: Kawasaki disease (KD) is a systemic vasculitis of childhood throughout the body. We report a patient who complicated by severe pneumonia of lung parenchymal consolidation (LPC) during typical KD clinical course. Case reports: A 7-yr-old male who had a past history of KD at 3-yr-old, visited a general practitioner with fever, sore throat and neck pain. Three days later, after being treated with CTRX/TBPMPI, he was transferred to our hospital because of bilateral lymph nodes swelling and a generalized erythema and rash. On admission he was still febrile and had bilateral conjunctival injections, cervical and axillary lymphadenopathy and markedly reddened pharynx, without edema. We diagnosed him as KD and treated him with aspirin and IVIG at a dose of 2.0g/kg, but the condition worsened; he developed dyspnea and dry cough. Crackles were noted on rt-lung auscultation, along with rt-basal dullness to percussion of the thorax. Chest radiography and a subsequent computed tomography scan revealed disseminated patchy infiltrates and ground-glass alterations with massive pleural effusions on rt-lung, and enlarged axillary and mediastinal lymph nodes. Some kinds of microbiology (including Mycoplasma) and virology (Epstein-Barr virus, cytomegalovirus, parvovirus B19), from all sampled sites (pleural effusion, blood, pharyngeal swab and cerebrospinal fluid), were negative for pathogenic specimens. So we treated him as intractable KD with second-IVIG and m-PSL, which led to the quick resolution of fever and progressive amelioration of pneumonia. Echocardiography showed normal ventricular size and function without coronary artery lesion (CAL). Thereafter, he was finally afebrile. At the first follow-up visit, 1 month after discharge, he had fully recovered. Clinically, echocardiographically and radiographically, no residues were noted. Conclusion: Several manifestations of broncho-pulmonary involvement in KD have been described. But there are very few case of LPC. We should consider the possibility that LPC were secondary to pulmonary arteritis.

La normalización precoz de la fracción de eyección tras resincronización en miocardiopatía dilatada idiopática es mantenida a largo plazo e identifica el mejor pronóstico clínico y arrítmico

Introduction and objectivePilot study was conducted in order to ascertain whether normalization of ventricular function after cardiac resynchronization (CRT) is maintained in the long term and its impact on ICD therapies and cardiovascular mortality. MethodsIn patients with idiopathic dilated cardiomyopathy with ICD-CRT analyzed the behavior of the ejection fraction (EF) to 6 months after implantation, defining a group (A) normalized FE (≥55%) and a (B) without normalizing it. We performed long-term monitoring to identify changes in left ventricular ejection fraction in both groups and their potential clinical-arrhythmic impact. ResultsOf the 47 patients included, 10 patients achieved normal EF at 6 months after implantation. The only predictors of normalization of EF were left ventricular end-systolic volume (OR: 0.94, 95% CI 0.92-0.97) and the regurgitant orifice mitral (OR: 0.89, 95% CI 0.86 to 0.93).After echocardiographic follow through 46 months patients in group A remained preserved EF (61±6 vs. 64±6%, P=.342), recorded by echocardiography, and showed no arrhythmic event compared to patients in group B, with no significant improvement in LVEF, had a higher incidence of arrhythmic events (0 vs. 29.7%, P=.039). Furthermore, in group A was not observed cardiovascular death compared to group B with 12 (0 vs. 32.4%, P=.031). ConclusionsNormalization of ejection fraction in the short term in patients with idiopathic dilated cardiomyopathy after CRT is maintained over time, and identifies patients with an excellent long-term prognosis.