Structural Heart Abnormalities Research Articles

P1412CARDIO PULMONARY AND CKD-MBD INTERACTIONS IN HEMODIALYSIS PATIENTS

Abstract Background and Aims Emerging evidence suggests that pulmonary hypertension (PHT) is closely associated with cardiac, pulmonary, and mineral bone diseases in hemodialysis patients. Previous results indicated that extra osseous calcifications might be a risk factor for PHT although results in human studies about this association have been conflicting. Multiple factors including abnormal calcium phosphate levels, uremia and inflammation are known to be important in vascular calcification development. In hemodialysis patients’ vascular calcifications may be evaluated by different techniques. The utilization of plain X-ray for screening vascular calcifications has been suggested by KDOQI and KDIGO .In this study, we examined the association between PHT, peripheral vascular calcification by plain X-Ray score, and echocardiographic parameters in hemodialysis patients. Method Chronic (> 6 months) hemodialysis patients (150) on a three times per week dialysis schedule were studied. Two-dimensional trans thoracic echocardiography was performed before dialysis. Global systolic function was evaluated by left ventricular ejection fraction (LVEF). Systolic pulmonary arterial pressure (SPAP) was recorded. Pulmonary hypertension was defined as, pulmonary artery pressure (PAP) greater than 35 mm Hg based on European Society of Cardiology Guideline. Vascular calcification score is evaluated in plain X-ray of pelvis and hands. Each film is divided into four sections. Any vascular calcification either in an irregular or linear pattern is considered. The presence of vascular calcifications in each section is rated as 1 and its absence as 0. Final score is the sum of all sections and ranges from 0 to 8.Demographic data and lab results including Calcium (Ca), Phosphorous (P), Ca X P product, 25OH-vitD and iPTH were collected for all enrolled patients. The effects of different peripheral vascular calcification scores in plain X-Ray, structural heart abnormalities in echocardiography and Ca-P-iPTH- vit D axis disorders on the development of pulmonary hypertension were evaluated to elucidate cardio, pulmonary and CKD-MBD interactions. Results Hemodialysis patients had a high prevalence of PHT (30%), which warrants special attention to this important complication in hemodialysis population. Severe peripheral VCs (VCs >3) were independent risk factor for PHT. In addition to the old age, duration of hemodialysis, and mitral valve disease, the presence of higher Ca- P product was another independent predictor of PHT in chronic hemodialysis patients. Conclusion Prevalence of PHT in dialysis patients seems to be high, and is closely associated with peripheral VC, mineral bone disorders and structural heart abnormalities suggesting further a strong cardio pulmonary renal interaction.

RIGHT VENTRICULAR AND PULMONARY HYPERTENSION IN CHRONIC OBSTRUCTIVE PULMONARY DISEASE AND RESTRICTIVE LUNG DISEASE

Background: Chronic lung disease is frequently associated with lung vascular lesion. We evaluated the structural-functional related changes of right ventricle under CLD with or without PH at RSUP dr. Kariadi, Semarang. Methods: We studied patients at RSUP dr. Kariadi who underwent routine evaluation that included resting spirometry and echocardiography. Patients with either COPD or RLD were studied, exclusion were made for patients with valvular heart disease and congestive heart disease. This study was performed during May through June of 2019 (n = 20). PASP, RVD, RVWT, TAPSE and spirometry values were analyzed for the association between PASP and RVD, RVWT, TAPSE, TAPSE/PASP ratio and FVC, FEV1, FEV1/FVC. Results: Thirteen (65%) of 20 patients who underwent echocardiography and spirometry evaluation were male and their average age were 55 years old. Mean PASP was 49.30 mmHg (range 2–111 mmHg). Ninety five percents patients had restrictive spirometry and 5% patients had moderate-severe mixed spirometry. The majority of the population of the study sample is dominated by a very severe degree of restriction spirometry. Out of the 20 subjects, 15 subjects (75%) had a diagnosis of COPD and 10 subjects (50%) had a history of pulmonary TB. In this study, 75% subjects had right ventricular dilatation, 85% subjects had right ventricular hypertrophy, and 15% subjects had decreased right ventricular systolic function (low TAPSE). The majority of structural and functional abnormalities of the right heart are found on patients with very severe degree of restriction spirometry. There were 13 subjects (65%) pulmonary hypertension, with the most findings being severe pulmonary hypertension as many as 8 subjects (40%). As many as 14 subjects (70%) had high TAPSE / PASP ratio.Conclusions: PH prevalence in patients with CLD is significantly associated with spirometry values. PH severity degree in patients with CLD is not significantly associated with spirometry values.Key words: spirometry; pulmonary hypertension; right heart echocardiography.

6q25.1 (TAB2) microdeletion is a risk factor for hypoplastic left heart: a case report that expands the phenotype

IntroductionHypoplastic left heart syndrome (HLHS) is a rare but devastating congenital heart defect (CHD) accounting for 25% of all infant deaths due to a CHD. The etiology of HLHS remains elusive, but there is increasing evidence to support a genetic cause for HLHS; in particular, this syndrome is associated with abnormalities in genes involved in cardiac development. Consistent with the involvement of heritable genes in structural heart abnormalities, family members of HLHS patients have a higher incidence of both left- and right-sided valve abnormalities, including bicuspid aortic valve (BAV).Case presentationWe previously described (Am J Med Genet A 173:1848–1857, 2017) a 4-generation family with a 6q25.1 microdeletion encompassing TAB2, a gene known to play an important role in outflow tract and cardiac valve formation during embryonic development. Affected adult family members have short stature, dysmorphic facial features, and multiple valve dysplasia, including BAV. This follow-up report includes previously unpublished details of the cardiac phenotype of affected family members. It also describes a baby recently born into this family who was diagnosed prenatally with short long bones, intrauterine growth restriction (IUGR), and HLHS. He was the second family member to have HLHS; the first died several decades ago. Postnatal genetic testing confirmed the baby had inherited the familial TAB2 deletion.ConclusionsOur findings suggest TAB2 haploinsufficiency is a risk factor for HLHS and expands the phenotypic spectrum of this microdeletion syndrome. Chromosomal single nucleotide polymorphism (SNP) microarray analysis and molecular testing for a TAB2 loss of function variant should be considered for individuals with HLHS, particularly in those with additional non-cardiac findings such as IUGR, short stature, and/or dysmorphic facial features.

SAT-234 CARDIO-PULMUNARY AND CKD-MBD INTERACTIONS IN HEMODIALYSIS PATIENTS

Emerging evidence suggests that pulmonary hypertension (PHT) is closely associated with cardiac, pulmonary, and mineral bone diseases in hemodialysis patients. Previous results indicated that extra osseous calcifications might be a risk factor for PHT although results in human studies about this association have been conflicting. Multiple factors including abnormal calcium phosphate levels, uremia and inflammation are known to be important in vascular calcification development. In hemodialysis patients’ vascular calcifications may be evaluated by different techniques. The utilization of plain X-ray for screening vascular calcifications has been suggested by KDOQI and KDIGO. In this study, we examined the association between PHT, peripheral vascular calcification by plain X-Ray score, and echocardiographic parameters in hemodialysis patients. Chronic (> 6 months) hemodialysis patients (150) on a three times per week dialysis schedule were studied. Two-dimensional trans thoracic echocardiography was performed before dialysis. Global systolic function was evaluated by left ventricular ejection fraction (LVEF). Systolic pulmonary arterial pressure (SPAP) was recorded. Pulmonary hypertension was defined as, pulmonary artery pressure (PAP) greater than 35 mm Hg based on European Society of Cardiology Guideline. Vascularcalcification score is evaluated in plain X-ray of pelvis and hands. Each film is divided into four sections. Any vascular calcification either in an irregular or linear pattern is considered. The presence of vascular calcifications in each section is rated as 1 and its absence as 0. Final score is the sum of all sections and ranges from 0 to 8. Demographic data and lab results including Calcium (Ca), Phosphorous (P), Ca X P product, 25OH-vitD and iPTH were collected for all enrolled patients. The effects of different peripheral vascular calcification scores in plain X-Ray, structural heart abnormalities in echocardiography and Ca-P-iPTH- vit D axis disorders on the development of pulmonary hypertension were evaluated to elucidate cardio, pulmonary and CKD-MBD interactions. Hemodialysis patients had a high prevalence of PHT (30%), which warrants special attention to this important complication in hemodialysis population. Severe peripheral VCs (VCs >3) were independent risk factor for PHT. In addition to the old age, duration of hemodialysis, and mitral valve disease, the presence of higher Ca- P product was another independent predictor of PHT in chronic hemodialysis patients. View Large Image Figure ViewerDownload Hi-res image Download (PPT) Prevalence of PHT in dialysis patients seems to be high, and is closely associated with peripheral VC, mineral bone disorders and structural heart abnormalities suggesting further a strong cardio pulmonary renal interaction.

Echocardiographic Changes in Newly Diagnosed Type 2 Diabetes Mellitus Patients with and without Hypertension.

BackgroundWhether type 2 diabetes mellitus (T2DM) is independently associated with structural heart abnormalities is controversial because of confounders associated with T2DM. This study aimed to investigate echocardiographic features in patients with newly diagnosed T2DM, exploring changes in cardiac structure and function.Material/MethodsThis was a retrospective study of new T2DM cases treated at the Second People’s Hospital Affiliated to Nanjing Medical University (Changzhou) in 2014–2016. In all, 128 T2DM cases were included (62 hypertensive and 66 non-hypertensive individuals). Controls were selected among individuals who underwent examination at the same department/period. Interventricular septal thickness (IVST), left ventricular end-diastolic diameter (LVEDD), posterior left ventricular wall thickness (PWTD), left ventricle mass (LVM), end-diastolic thickness of left ventricular posterior wall (Dd), aortic root diameter, left atrial diameter (LAd), left atrial diameter fraction-shortening values, and left ventricular ejection fraction (LVEF) were determined routinely.ResultsIVST, LVEDD, PWTD, Dd, LAd, and left atrial diameter fraction-shortening values were larger in patients with T2DM (all P<0.05 vs. controls). LVM was higher in T2DM patients (median, 57.12 vs. 54.77 g, P=0.001). There were no differences in aortic root diameter and EF (both P>0.05). Multivariable analysis showed that IVST (OR=1.33, 95% CI: 1.01–1.76, P=0.04), LAd (OR=1.16, 95% CI: 1.07–1.25, P<0.001), TGs (OR=1.34, 95% CI: 1.09–1.63, P=0.005), and HDL (OR=1.46, 95% CI: 1.02–2.08, P=0.04) were independently associated with hypertension in patients with T2DM.ConclusionsPatients with newly diagnosed T2DM already display structural heart abnormalities. LAd and IVST are independently associated with hypertension in these patients, probably contributing to increased cardiovascular risk.

Алгоритмы ведения пациентов со структурными аномалиями сердца в терапевтической практике

Among structural heart abnormalities (SНA) in clinical practice, mitral valve prolapse, an open oval window, and atrial septal aneurysm, which can present a real threat to the health and life of patients, are most common. Frequent subclinical course makes it difficult to detect them in a timely manner and requires focused attention of practicing doctors in assessing the phenotypic features of the structure of the musculoskeletal system and connective tissue, which can serve as markers of latent defects in the development of the heart. The article provides data on the most typical symptoms and clinical manifestations of SНA, possible complications and therapeutic tactics. Early detection of structural abnormalities of the heart and the choice of the right tactics for passive or active monitoring of patients with varying degrees of severity of defects in the development of the heart will help preserve the health or even life of patients.

Brugada Syndrome: Clinical Features, Risk Stratification, and Management.

In 1992, the Brugada brothers published a patient series of aborted sudden death, who were successfully resuscitated from ventricular fibrillation (VF). These patients had a characteristic coved ST-segment elevation in the right precordial leads on their 12-lead electrocardiogram with no apparent structural heart abnormality. This disease was referred to as “right bundle branch block, persistent ST-segment elevation, and sudden death syndrome.” The term Brugada syndrome (BrS) was first coined for this new arrhythmogenic entity in 1996. BrS is more prevalent in Southeast Asian ethnic groups and was considered a familial disease due to the presence of syncope and/or sudden deaths in several members of the same family, however, the genetic alteration was only noted in 1998. The genetic characterization of BrS has proven to be challenging. The most common and well-established BrS genotype involves loss-of-function mutations in the SCN5A gene, but only represents between 15% and 30% of the diagnosed patients. Patients with BrS can present with a range of symptoms which can include syncope, seizures, and nocturnal agonal breathing due to polymorphic ventricular tachycardia or VF. If these arrhythmias are sustained, sudden cardiac death may result. Despite the significant progress on the understanding of BrS over the last two decades, there remain a number of uncertainties and challenges; we present an update review on the subject.

Role of a schematic figure of echocardiography in congenital heart diseasessurgery: which method of reporting do you prefer?

Congenital structural heart diseases are the most commonly reported fatal anomalies in children. These anatomical disorders often can be corrected surgically .Today, echocardiography is the best and most important diagnostic technique for anatomical abnormalities in the heart. In this method, by using the ultrasound waves (sonography), anatomical details, and hemodynamic examination of the heart can be determined . Most pediatric cardiologists provide echocardiographic reports in the form of handwriting or typed text. This method of reporting is sometimes unable to understand the structural anomalies precisely and accurately for the final decision. Nowadays, 2-dimensional echocardiography is acceptable for the diagnosis of some structural heart abnormalities accurately. Even there is no necessary to angiography for surgical planning .2 But sometimes lack matching echocardiographic findings with surgical reports, it seems that adding a schematic figure base on echocardiography findings from the viewpoint of pediatric cardiologist will be the helper to the congenital cardiac surgeon. This proposed method of reporting will lead to the promotion of surgical treatment in congenital heart diseases. The history of the schematic picture in the anatomical textbook for a better understanding of subjects and also diseases has been shown previously by famous painters like Leonardo Davin chi. This suggestion shows that mapping can be viewed as a common language for better understanding of echocardiographic reports and more adaptation between comments of a pediatric cardiologist with designing congenital cardiac surgeon for more precise and complete treatment.

Prognosis Analysis of Heart Failure Based on Recurrent Attention Model

ObjectivesHeart failure is a group of complex clinical syndromes that lead to ventricular filling or impaired ejection ability due to abnormal heart structure or function. Difficult treatment, poor prognosis and high mortality are the main characteristics of heart failure. According to admission data and past medical use, the 30-day mortality rate of patients with heart failure was obtained and the main characteristics affecting the 30-day mortality of patients with heart failure were determined. Material and methodsBased on the data of April 2016 to July 2018 of Shanxi Acadeny of Medical Sciences, and we chose 4,682 information on heart failure patients, of which 539 died in the hospital by screening. We built a 30-day mortality prediction model for patients with heart failure. The model can fuse clinical data and text data through multiple kernel learning, and input the fused data into the recurrent attention model. It can not only predict the 30-day mortality of patients with heart failure, but also the influencing factors of prognosis of patients with heart failure were also obtained. ResultsThe prediction accuracy of the recurrent attention network is obviously higher than that of other machine learning models, and the accuracy rate reaches 93.4%. The AUC value of the area under the ROC curve of the model reaches 87%, which is obviously higher than that of the traditional machine learning models such as decision tree, naive Bayesian and support vector machine. In addition, the model can also reach a conclusion that New York heart function classification, age, NT—ProBNP, LVEF, β-blockers, ventricular arrhythmia, high blood pressure, coronary heart disease (CHD) and bronchitis were independent risk factors for death. And patients with revascularization, ACEI/ARB drugs, β-blockers, spironolactone have a better prognosis than non-users. This provides an important reference for doctors to better treat and manage patients with heart failure. ConclusionExperiments show that the prognostic effect of the recurrent attention model is significantly higher than that of other traditional machine learning models. Because the model increases the attention mechanism, the important features affecting the prognostic results are obtained, which enables doctors to prescribe drugs according to the symptoms, take timely precautions and help patients to treat in time.

The first 200 years of cardiac auscultation and future perspectives.

Cardiac auscultation – even with its limitations – is still a valid and economical technique for the diagnosis of cardiovascular diseases, and despite the growing demand for sophisticated imaging techniques, clinical use of the stethoscope in medical practice has not yet been abandoned. In 1816, René-Théophile-Hyacinthe Laënnec invented the stethoscope, while examining a young woman with suspected heart disease, giving rise to mediated auscultation. He described in detail several heart and lung sounds, correlating them with postmortem pathology. Even today, a correct interpretation of heart sounds, integrated with the clinical history and physical examination, allows to detect properly most of the structural heart abnormalities or to evaluate them in a differential diagnosis. However, the lack of organic teaching of auscultation and its inadequate practice have a negative impact on the clinical competence of physicians in training, also reflecting a diminished academic interest in physical semiotic. Medical simulation could be an effective instructional tool in teaching and deepening auscultation. Handheld ultrasound devices could be used for screening or for integrating and improving auscultatory abilities of physicians; the electronic stethoscope, with its new digital capabilities, will help to achieve a correct diagnosis. The availability of innovative representations of the sounds with phono- and spectrograms provides an important aid in diagnosis, in teaching practice and pedagogy. Technological innovations, despite their undoubted value, must complement and not supplant a complete physical examination; clinical auscultation remains an important and cost-effective screening method for the physicians in cardiorespiratory diagnosis. Cardiac auscultation has a future, and the stethoscope has not yet become a medical heirloom.

Early repolarization and risk of lone atrial fibrillation.

Early repolarization syndrome is a recently proposed condition characterized by an early repolarization pattern in the electrocardiogram (ECG) and ventricular fibrillation in the absence of structural heart abnormalities. Although some studies have suggested that early repolarization is associated with frequency of atrial fibrillation, the association of early repolarization with atrial fibrillation is not well known. Early repolarization indicates the substrate for atrial fibrillation in addition to that for ventricular fibrillation. This study included 79 patients (57 men [72%]; age, 45 ± 12 years) aged less than 60 years who had paroxysmal lone atrial fibrillation and 395 age- and sex-matched healthy controls (patient:control ratio, 1:5). Patients who had structural heart disease, hypertension, diabetes, hyperthyroidism, history of successful resuscitation, or the Brugada type ECG were excluded. ECGs recorded during sinus rhythm were compared between patients with atrial fibrillation and healthy controls. Early repolarization in the inferior and/or lateral leads was more common in patients with atrial fibrillation (25%) than controls (10%; P = 0.001). The location and magnitude of early repolarization were similar between the two groups. Other electrocardiographic measurements were not different between the two groups. Among patients with atrial fibrillation, there was no difference in clinical characteristics including age at atrial fibrillation development, sex, and body mass index between patients with early repolarization and those without early repolarization. Electrocardiographic measurements were not different between patients with early repolarization and those without early repolarization. Early repolarization was associated with lone atrial fibrillation. Early repolarization may indicate increased susceptibility to atrial fibrillation.

Integration of 12-Lead Electrocardiograms Into Preparticipation Screenings to Prevent Sudden Cardiac Death in High School Athletes

Sudden cardiac arrest continues to be the leading cause of death in young athletes despite preparticipation sports screening (PPS). The devastating loss of children to sudden cardiac arrest has led communities to demand more comprehensive heart screenings. The purpose of this study was to evaluate a community youth heart screening program in Minnesota. A retrospective chart review of 840 participants aged 14 to 18 years from August 2015 through March 2017 was conducted. Referrals were made for two abnormal electrocardiography results, 12 abnormal echocardiography results, and 59 elevated blood pressure readings. Cross-tabulations showed one participant with an abnormal electrocardiogram and six participants with abnormal echocardiograms who had no other abnormal findings at the screening. Findings were not statistically significant. It was clinically significant that six participants were found to have potentially lethal electrical or structural heart abnormalities who would have otherwise have remained unidentified with the standard preparticipation sports screening guidelines.

A study of time-frequency features for CNN-based automatic heart sound classification for pathology detection

This study concerns the task of automatic structural heart abnormality risk detection from digital phonocardiogram (PCG) signals aiming at pediatric heart disease screening applications. Recently, various systems based on convolutional neural networks trained on time-frequency representations of segmental PCG frames have been presented that outperform systems using hand-crafted features. This study focuses on the segmentation and time-frequency representation components of the CNN-based designs. We consider the most commonly used features (MFCC and Mel-Spectrogram) used in state-of-the-art systems and a time-frequency representation influenced by domain-knowledge, namely sub-band envelopes as an alternative feature. Via tests carried on two high quality databases with a large set of possible settings, we show that sub-band envelopes are preferable to the most commonly used features and period synchronous windowing is preferable over asynchronous windowing.

Analyzing the prevalence of proximal aorta dilatation and its risk factors using Z-score

Objective Using Z-score to assess the prevalence of proximal aorta dilatation in middle-aged and aged individuals during routine transthoracic echocardiogram examinations and to identify its risk factors. Methods A total of 823 middle-aged or elderly patients on routine transthoracic echocardiogram examinations were consecutively enrolled.The internal diameters of the sinus of Valsalva(SoV)and the ascending aorta(AA)were measured.Z-scores were calculated according to the proposed equation for SoV and AA.A dilated aortic root was defined as a Z-score ≥1.96 or the diameter of SoV or AA ≥40 mm.The prevalence of proximal aorta dilatation and associated factors were analyzed. Results The prevalences of proximal aorta dilatation, SoV dilatation, and AA dilatation were 26.1%(25/823), 6.0%(49/823), and 23.7%(195/823), respectively.In the aortic root dilatation group, age and the proportion of obesity were higher (both P<0.05), and there were more female subjects (30.5% or 117/384 vs.22.3% or 98/439, P<0.01). The incidences of left atrial dilation, left ventricular dilation, left ventricular hypertrophy, and aortic regurgitation in the aortic root dilatation group were higher than those in the non-aortic root dilatation group(P<0.05). Logistic regression analysis demonstrated that sex (OR=1.827, 95%CI: 1.248-2.673, P=0.002), hypertension (OR=1.441, 95%CI: 1.000-2.075, P=0.050)and left ventricular hypertrophy (OR=1.827, 95%CI: 1.248-2.673, P=0.002)were independently correlated with aortic root dilatation. Conclusions The prevalence of proximal aorta dilatation is high in middle-aged and aged individuals.Proximal aorta dilatation is related to sex, age, and body size, and it is often accompanied by structural abnormalities of the heart. Key words: Aorta; Dilatation, pathologic; Echocardiography

Clinical and epidemiological features of Heart-Hand Syndrome: a hospital-based study in China

Heart–hand syndrome (HHS) is a clinically and genetically heterogeneous disorder characterized by the co-occurrence of a congenital cardiac disease and an upper limb malformation. This study revealed the clinical and epidemiological features of HHS in China. The study was based on patients with congenital upper limb malformation treated in Beijing Ji Shui Tan hospital from October 1st, 2013 to October 1st, 2016. We reviewed the patients’ medical records and identified patients with abnormal ultrasonic cardiogram and/or electrocardiogram (ECG). A total of 1462 patients (910 male and 552 female) were identified to be treated for congenital upper limb malformation. Among them, 172 (11.8%) had abnormal ultrasonic cardiogram and/or ECG. Abnormal heart structure were discovered in 121 patients and 51 patients had abnormal ECG. The most common type of abnormal heart structure was tricuspid regurgitation (53/121, 43.8%), while the most common abnormal ECG was wave patterns (22/51, 43.1%). This hospital-based study suggests that the rate of congenital heart disease is high in patients treated for congenital upper extremity malformation in China. Surgeons and anesthetists should be aware of the comorbidity and preoperational examination of congenital heart diseases is highly needed to avoid complications during operation.

Brugada Syndrome: A Primer for Nurse Practitioners

Abstract Brugada syndrome (BrS) is a less known cardiac condition which falls into the category of a channelopathies. BrS has been diagnosed for more than 2 decades. Currently BrS remains a major cause of sudden cardiac death in young adults who have no known abnormal heart structure. Nurse practitioners' awareness of the clinical presentation, diagnosis, treatment, and ongoing care is essential for maximizing patient outcomes.

CARDIOVASCULAR COMPLICATIONS OF ACROMEGALY.

Acromegaly is associated with increased mortality and decreased life expectancy. Cardiovascular disease is the principal cause of premature mortality in patients with acromegaly, accounting for about 60% of deaths. GH and/or IGF-I exert direct cardiac effects: enhance cardiac contractility, stimulate cardiomyocyte growth, influence calcium influx in cardiomyocytes. Cardiac remodelling is influenced by hypertension and insulin resistance. Among cardiovascular risk factors arterial hypertension, reported in 35% of patients with acromegaly, ranks among most important negative prognostic factors for mortality. Hypertension plays significant role in the development of cardiac hypertrophy, especially in older acromegalic patients and diastolic blood pressure is best predictive factor for cardiac hypertrophy. Therefore, early and aggressive hypertension treatment is essential for prognosis in acromegaly. Other important risk factors are: valvular defects, arrhythmias, endothelial dysfunction, heart failure, lipid abnormalities and coronary artery disease. Numerous studies suggest that patients with acromegaly are under threat of arrhythmias, especially those with structural heart abnormalities. Congestive heart failure as end-stage acromegalic cardiomyopathy occurs usually in older patients, with long-term uncontrolled disease and other cardiovascular and metabolic complications. Relation between acromegaly and coronary artery disease is controversial as it seems to be connected rather with classical cardiovascular risk factors than GH and IGF-1 overexpresion.

Mechanistic studies on ketamine-induced mitochondrial toxicity in zebrafish embryos

Ketamine, a phencyclidine derivative, is an antagonist of the Ca2+-permeable N-methyl-d-aspartate (NMDA)-type glutamate receptors. It is a pediatric anesthetic and has been implicated in developmental neurotoxicity. Ketamine has also been shown to deplete ATP in mammalian cells. Our previous studies showed that acetyl l-carnitine (ALCAR) prevented ketamine-induced cardiotoxicity and neurotoxicity in zebrafish embryos. Based on our finding that ALCAR's protective effect was blunted by oligomycin A, an inhibitor of ATP synthase, we further investigated the effects of ketamine and ALCAR on ATP levels, mitochondria and ATP synthase in zebrafish embryos. The results demonstrated that ketamine reduced ATP levels in the embryos but not in the presence of ALCAR. Ketamine reduced total mitochondrial protein levels and mitochondrial potential, which were prevented with ALCAR co-treatment. To determine the cause of ketamine-induced ATP deficiency, we explored the status of ATP synthase. The results showed that a subunit of ATP synthase, atp5α1, was transcriptionally down-regulated by ketamine, but not in the presence of ALCAR, although ketamine caused a significant upregulation in another ATP synthase subunit, atp5β and total ATP synthase protein levels. Most of the ATP generated by heart mitochondria are utilized for its contraction and relaxation. Ketamine-treated embryos showed abnormal heart structure, which was abolished with ALCAR co-treatment. This study offers evidence for a potential mechanism by which ketamine could cause ATP deficiency mediated by mitochondrial dysfunction.

The History of Ultrasound and Its Use at Point of Care: Neonatal Ultrasound in Transport

Purpose of review The purposes of this review are to explore the history of the diagnostic ultrasound and its use in medical care and to report on the latest findings on the utility of ultrasound during the transport of patients. Recent findings The Neonatal Ultrasound in Transport (NUiT) study demonstrated the feasibility and utility of ultrasound assessment prior to the retrieval of critically ill newborns to a higher level of care. It is feasible to carry a laptop ultrasound with the transport team without hindering flight or retrieval processes. The NUiT study demonstrated that many term babies had evidence of clinically unsuspected haemodynamic compromise. Other babies had clinically unsuspected abnormal heart structure allowing for redirection of transport toward paediatric cardiology centres. The preterm cohort revealed many babies with clinically unsuspected low systemic blood flow and stable head ultrasound findings pre- and post transport. This has implications for therapeutic targeting of treatment and for counselling parents. Summary Clinician-performed ultrasound (CPU) during the retrieval of neonates confirms a high incidence of haemodynamic pathology. Ultrasound in retrieval is feasible, allows the accurate triage of babies to cardiac centres and may allow more accurate and earlier targeting of fluid and inotropic support.

Progress in Molecular Genetic Study of Mitochondrial Cardiomyopathy.

Mitochondria plays a key role in providing ATP for the energy-consuming cardiac tissues. Mitochondrial cardiomyopathy is a myocardial condition characterized by abnormal heart structure and/or function secondary to genetic defects involving the mitochondrial respiratory chain. The typical cardiac manifestations of mitochondrial cardiomyopathy include hypertrophic and dilated cardiomyopathy,while left ventricular myocardial noncompaction is less common. Recent research has suggested that most mitochondrial diseases result from mitochondrial DNA mutation,which can be found in genes that encode ancillary proteins needed for genetic transcription (tRNA),in genes that encode subunits of the electron transport chain complexes,or in genes that control the activities of the mitochondria called D-loop zone. However,the exact physiological mechanisms remain unclear. This review summarizes the recent advances in the molecular mechanism of mitochondrial cardiomyopathy.