Systolic Murmur Research Articles

LEFT VENTRICULAR NON COMPACTION CARDIOMYOPATHY ASSOCIATED WITH MITRAL VALVE PROLAPSE

Left ventricular non compaction (LVNC) is a rare condition characterized by prominent ventricular trabeculations and intertrabecular recesses,Mitral regurgitationwhen present is usually a result of annular dilation, mitral valve prolapse due to chordae tendinae rupture is quite rare.We present the case of a 48 year old male with no medical history who presented with worsening exertional dyspnea classified as NYHA class III. The physical examination found a systolic murmur heard at the apex. Transthoracic echocardiography and cardiac magnetic resonance confirmed the diagnosis of Left ventricular non compaction associated with posterior mitral valve prolapse with severe mitral regurgitation and preserved ventricular function who was successfully operated and found asymptomatic during the follow up.

A new wearable cardiac acoustic monitoring technology for the evaluation of subclinical leaflet thrombosis after transcatheter aortic valve replacement: a pilot study

Abstract Background Subclinical leaflet thrombosis characterized by hypoattenuated leaflet thickening (HALT) and reduced leaflet motion (RLM) is a common complication after transcatheter aortic valve replacement (TAVR), whether it affects hemodynamic change is still debated. Purpose We attempted to use a novel wearable heart acoustic monitoring device, and combined multi-dimensional computed tomography (MDCT) and echocardiography to diagnose SLT and analyze the changes of transvalvular hemodynamics after TAVR. Methods Patients with severe symptomatic aortic stenosis and underwent successful TAVR were consecutively enrolled. Clinical data, acoustic cardiographic parameters including the frequency, amplitude, and energy of heart sounds , and imaging results were collected and analyzed by Core laboratory at pre- and post TAVR, and follow-up at one month and six month. According to severity of SLT at one month follow-up, we divided the patients into three groups: Group1: no SLT, Group2: mild SLT, defined as existed HALT did not qualify for Group 3, and Group 3: moderate to severe SLT, defined as at least 1 leaflet with HALT of grade 3 or 4 or RLM of ≥50%, or 2 leaflets with HALT of grade 2 or RLM of ≥50%. A consistency analysis of SLT presence between MDCT findings and acoustic cardiographic variables was performed using Kappa statistics. Results The study included 116 TAVR patients. The prevalence of SLT as detected by MDCT at one-month follow-up was 25%, with moderate to severe SLT representing 11.2% of cases. A significant decrease in aortic valve orifice area (AVA) (-0.2±0.48cm2 vs. -0.1±0.33cm2, p=0.009) and an increase in mean gradient (MG) (3.5±5.85 mmHg vs. 0.1±11.34mmHg, p=0.028) were observed in moderate to severe SLT group at one-month follow-up versus post-TAVR via echocardiography. However, no significant differences were found in the mild SLT or no SLT group. Acoustic cardiographic characteristics exhibited a distinct pattern with early systolic, baseless, and high-energy murmurs in patients with moderate to severe SLT, but not in the mild SLT or no SLT group. The sensitivity, specificity, and Kappa values for acoustic cardiography in diagnosing moderate to severe SLT were 81.82%, 83.33%, and 0.41, respectively. At the 6-month follow-up, MDCT showed that 11 (85%) patients with moderate to severe SLT who received anticoagulant therapy reached complete dissolution of SLT. Conclusion Moderate to severe SLT presented notably transvalvular hemodynamic influences following TAVR. Acoustic cardiography demonstrated specific characteristics of moderate to severe SLT, providing a valuable tool for SLT diagnosis and evaluation in clinical follow-up.Figure 1.Baseline characteristicsFigure 2.SLT and acoustic cardiography

Challenges in the evaluation of aortic stenosis: A guide to multimodal imaging

Severe Aortic stenosis (AS) is characterised by a late peaking crescendo-decrescendo systolic murmur and a diminished/absent second heart sound. Echocardiographic assessment of AS severity, based on transvalvular velocities / pressure gradients and calculated aortic valve area (AVA), confirms the diagnosis and allows timely intervention. Nevertheless, diagnostic uncertainty exists in cases of discordant measurements, confounders, and symptoms-measurement discrepancies. This guide outlines an approach to the use of multimodal imaging in the diagnosis and staging of AS.

First diagnosis of severe coarctation of the aorta necessitation percutaneous intervention during pregnancy, a case report

Abstract Background Coarctation of the aorta (CoA) is a common congenital heart defect that affects about 3-4 in every 10,000 live births. Despite clear signs on clinical examination, the diagnosis is sometimes not made until adulthood. An increasing number of patients with CoA are reaching child-bearing age. Unrepaired CoA or severe recurrent stenosis during pregnancy is a significant concern, as it poses a high risk of maternal and foetal complications and even death. Case summary A 21-year-old women was referred to the cardiology department at 17 weeks’ gestation for management of arterial hypertension and unexplained systolic murmur. She had been diagnosed with hypertension elsewhere the year before presentation, but unfortunately this remained unexplored. She had been started on labetalol early in the pregnancy. Clinical examination showed a loud systolic heart murmur extending from parasternal to subclavicular and scapular areas. Pulses in the lower extremities were very weak and blood pressure was slightly elevated with a significant gradient between the upper and lower extremities. Echocardiography showed remarkable absence of pulsatile flow in the abdominal aorta and narrowing just distal to the subclavian artery with typical diastolic tail pattern on suprasternal imaging. Cardiac magnetic resonance confirmed the presence of a severe coarctation distal to the subclavian artery and presence of multiple collaterals allocating this patient in the extremely high-risk category with a risk of up to up 40-100% of maternal cardiac event during pregnancy. An extensive multidisciplinary team meeting was convened. After initial medical optimisation, increased claudication and signs of placental hypoperfusion necessitated an endovascular procedure under general anaesthesia at 23 weeks’ gestation. A Bentley BeGraft Plus stent (16x38 mm) was successfully placed. Postoperative ultrasound showed biphasic placental perfusion and normalisation of blood pressure and ankle-brachial indices. At 36 weeks’ gestation, the patient gave birth to a healthy child. Conclusion CoA should be considered in any young patient with arterial hypertension. Altered maternal haemodynamics during pregnancy resulted in severe symptomatic CoA and reduced placental flow necessitating percutaneous intervention during pregnancy. A multidisciplinary pregnancy heart team is essential for optimal treatment management in these high-risk patients.

Clinical case on the use of the Bimod Vet® on a Doberman Pinscher puppy with atrial fibrillation

Similarly as in people, the diagnosis of cardiac diseases in dogs typically relies on echocardiography and electrocardiography, examinations that are usually reserved to cardiologists. A new medical device (footnote: Bimod Vet®) acquiring the electrocardiogram and phonocardiogram of dogs simultaneously and processing them with built-in algorithms has been developed to assist general practitioners in the management of cases suggestive of suffering from a cardiac disorder. This article displays the use of this device in a 5-month-old Doberman Pinscher puppy referred to a cardiologist and eventually diagnosed with cardiomyopathy of dilated phenotype with atrial fibrillation of unknown origin.In this case, Bimod Vet® enabled to acquire some cardiorespiratory parameters highly suggestive of a cardiac disorder, in a fast and stress-free manner. The rhythm analysis performed by the software lead to a suspicion of atrial fibrillation, based on a rapid and irregular rhythm, narrow QRS complexes and absence of P wave on the electrocardiogram tracing. A systolic murmur was also detected and the respiratory rate was measured.The device Bimod Vet® can therefore assist veterinarians in the detection of several abnormalities suggestive of an underlying cardiac disorder.

Storytelling of Hypertrophic Cardiomyopathy Discovery.

The discovery of hypertrophic cardiomyopathy (HCM) dates back to 1958, when the pathologist Donald Teare of the St. George's Hospital in London performed autopsies in eight cases with asymmetric hypertrophy of the ventricular septum and bizarre disorganization (disarray) at histology, first interpreted as hamartoma. Seven had died suddenly. The cardiac specimens were cut along the long axis, similar to the 2D echo. In the same year, at the National Institute of Health U.S.A., Eugene Braunwald, a hemodynamist, and Andrew Glenn Morrow, a cardiac surgeon, clinically faced a patient with an apparently similar morbid entity, with a systolic murmur and subaortic valve gradient. "Discrete" subaortic stenosis was postulated. However, at surgery, Dr. Morrow observed only hypertrophy and performed myectomy to relieve the obstruction. This first Braunwald-Morrow patient underwent a successful cardiac transplant later at the disease end stage. The same Dr. Morrow was found to be affected by the familial HCM and died suddenly in 1992. The term "functional subaortic stenosis" was used in 1959 and "idiopathic hypertrophic subaortic stenosis" in 1960. Years before, in 1957, Lord Brock, a cardiac surgeon at the Guy's Hospital in London, during alleged aortic valve surgery in extracorporeal circulation, did not find any valvular or discrete subaortic stenoses. In 1980, John F. Goodwin of the Westminster Hospital in London, the head of an international WHO committee, put forward the first classification of heart muscle diseases, introducing the term cardiomyopathy (dilated, hypertrophic, and endomyocardial restrictive). In 1995, the WHO classification was revisited, with the addition of two new entities, namely arrhythmogenic and purely myocardial restrictive, the latter a paradox of a small heart accounting for severe congestive heart failure by ventricular diastolic impairment. A familial occurrence was noticed earlier in HCM and published by Teare and Goodwin in 1960. In 1989-1990, the same family underwent molecular genetics investigation by the Seidman team in Boston, and a missense mutation of the β-cardiac myosin heavy chain in chromosome 14 was found. Thus, 21 years elapsed from HCM gross discovery to molecular discoveries. The same original family was the source of both the gross and genetic explanations of HCM, which is now named sarcomere disease. Restrictive cardiomyopathy, characterized grossly without hypertrophy and histologically by myocardial disarray, was found to also have a sarcomeric genetic mutation, labeled "HCM without hypertrophy". Sarcomere missense mutations have also been reported in dilated cardiomyopathy (DCM) and non-compaction cardiomyopathy. Moreover, sarcomeric gene defects have been detected in some DNA non-coding regions of HCM patients. The same mutation in the family may express different phenotypes (HCM, DCM, and RCM). Large ischemic scars have been reported by pathologists and are nowadays easily detectable in vivo by cardiac magnetic resonance with gadolinium. The ischemic arrhythmic substrate enhances the risk of sudden death.

Large ventricular myxoma causing inflow and outflow obstruction of the right ventricle; A Case Report

BackgroundMyxomas are the most common primary benign heart tumors, typically found in the left atrium, with only 2–4% occurring in the right ventricle. Clinical presentations vary widely, including congestive heart failure and systemic embolic phenomena. This case report describes a rare right ventricular myxoma causing both inflow and outflow obstruction, presenting as progressive exertional dyspnea.Case PresentationA 23-year-old male presented with two weeks of worsening exertional dyspnea. He was stable but tachypneic with a systolic murmur over the tricuspid area. Elevated erythrocyte sedimentation rate (ESR) and C-Reactive protein (CRP) were noted, while other lab tests were normal. Imaging, including echocardiography and chest tomography scan (CT) revealed a 4 × 3.8 × 4.6 cm mass in the right ventricle extending to the pulmonary trunk. Surgical resection via right ventriculotomy was performed, and histopathology confirmed myxoma. The patient recovered uneventfully.ConclusionRight ventricular myxomas, though rare, can cause significant obstruction and present with diverse symptoms. Timely diagnosis using imaging techniques like echocardiography is crucial. Surgical resection remains the definitive treatment, offering excellent outcomes and low recurrence rates. Early intervention is vital to prevent serious complications and ensure favorable patient prognosis.

Mucopolysaccharidosis Type I (Hurler – Scheie Syndrome): Case Report

Background. Mucopolysaccharidosis type I (MPS I) is an inherited disease caused by pathogenic variants in the IDUA gene, which encodes the lysosomal enzyme alpha-L-iduronidase. This clinical case demonstrates the importance of diagnosing rare diseases when patients have phenotypic features indicating hereditary pathology presence. Phenotype changes can be so-called “red flag” that helps specialists suspect and diagnose lysosomal storage diseases. Clinical case description. The patient was observed with delayed psychomotor development; she also had hepatomegaly and on auscultation of the heart there was a functional systolic murmur above the apex and in the Botkin–Erb point on heart auscultation. Coarse “garholoid” facial features attracted attention at external examination: large nose, wide sunken nose bridge, macroglossia, thick lips, synophrysis, scaphocephaly, wide chest, and flat-valgus feet. Echocardiography has revealed mitral valve insufficiency and hardened valve leaflets. Ophthalmologist examination: hypermetropic astigmatism. Psychiatrist examination: mental and speech development delay. Enzymatic diagnostics: significant decrease in alpha-Liduronidase activity. No further examinations were carried out due to the onset of the COVID-19 epidemic and parents' doubts about the diagnosis. The patient was readmitted to the hospital at the age of 6 years due to aggravation of her condition. A molecular genetic study has revealed two pathogenic variants in the IDUA gene. Diagnosis of MPS I was established according to the clinical data, results of biochemical and molecular genetic studies. Conclusion. This clinical case demonstrates the classic course of MPS I with typical phenotypic signs and lesions in various organs and systems. The presented case demonstrates the importance of professional vigilance among doctors of various specialties regarding orphan diseases.

Challenges in the approach to a patient with aortic stenosis and cardiac amyloidosis with ATTR mutation associated with negative scintigraphy - A case report

IntroductionCardiac amyloidosis (CA) poses significant diagnostic and therapeutic challenges. In this case report, we detail a patient with CA due to a rare transthyretin (CA-TTR) mutation, manifesting with negative myocardial scintigraphy and requiring genetic testing for diagnosis. The patient also had severe aortic stenosis (AS), necessitating discussion with a heart team to determine the optimal treatment strategy. Case reportA 70-year-old male with a family history of sudden death was previously diagnosed with third-degree atrioventricular block and treated with a pacemaker. He presented with worsening exertional dyspnoea, and examination revealed a third heart sound, a systolic murmur indicative of AS and bilateral muscular atrophy in the thenar region. Transthoracic echocardiography indicated severe AS and moderate left ventricular dysfunction, with images suggesting infiltrative disease. Pyrophosphate scintigraphy revealed no abnormal cardiac tracer uptake. Cardiac magnetic resonance imaging (MRI) revealed extensive, heterogeneous, subendocardial late gadolinium enhancement in both the atria and ventricles, which was consistent with CA. Genetic testing identified the Phe84Leu mutation in the TTR gene. Following heart team discussions, the patient underwent successful transcatheter aortic valve implantation (TAVI) and remained asymptomatic in follow-up, being monitored at an outpatient clinic specializing in CA and using tafamidis. DiscussionCA-TTR can be an autosomal dominant disease with variable penetrance involving abnormal amyloid protein deposition in tissues and can often be diagnosed noninvasively via myocardial scintigraphy. However, some TTR mutations do not affect scintigraphy results, necessitating genetic testing when clinical suspicion is high, potentially avoiding endomyocardial biopsy. Moreover, AS occurs in up to 16 % of TTR amyloidosis patients, with the conditions mutually exacerbating each other. Recent consensus suggests that TAVI reduces mortality in patients with severe AS and amyloidosis. ConclusionsVarious diagnostic algorithms emphasize the use of myocardial scintigraphy for suspected CA-TTR. Genetic testing is crucial when scintigraphy results are negative, but clinical suspicion remains high, potentially circumventing invasive procedures. Compared with medical management alone, TAVI has been shown to improve quality of life and survival in patients with concurrent severe AS and CA.

A Rare Case of Late Onset-Pompe’s Disease: Presented as Heart Failure

Pompe disease is a hereditary lysosomal storage disorder characterized by a deficiency in the acid alpha-glucosidase (GAA) enzyme, leading to glycogen accumulation in muscle and neurons. Clinical manifestations vary from severe infantile-onset with hypertrophic cardiomyopathy and early mortality due to respiratory insufficiency to late-onset with proximal muscle weakness, gross motor delay, and progressive respiratory insufficiency. A case of an 11-year-old boy who reported to the pediatric emergency department with a nine-year history of progressive muscle weakness and a one-month history of anemia symptoms (easy fatigue, shortness of breath, pale appearance) and heart failure (orthopnea, dyspnea). His family history included consanguineous marriages and similar conditions in his brother and maternal uncle. On examination, he appeared pale, malnourished, and exhibited signs of respiratory distress and tachypnea. His cardiovascular examination revealed apex beat displacement, elevated JVP, bilateral pedal edema, mild ascites, positive hepatojugular reflux, and systolic murmurs. Respiratory examination indicated bilateral crepitation and wheezes. Musculoskeletal examination showed decreased muscle mass and power, especially in proximal muscles. Abdominal examination revealed hepatosplenomegaly and mild ascites. Radiological findings included an enlarged cardiac shadow with pleural effusion and bilateral radio-opaque shadows on chest x-ray, while echocardiography showed impaired left ventricular systolic function with mild to moderate mitral and tricuspid regurgitation. Laboratory tests indicated elevated aspartate aminotransferase, LDH, and creatine kinase levels, along with normocytic, normochromic anemia. Muscle biopsy from the hamstring revealed PAS stain positive granules. These clinical, radiological, and laboratory findings strongly suggest late-onset Pompe disease, marking this as potentially the second reported case in Pakistan.

Atypical Presentation of a Cardiac Lipoma

Cardiac masses can have atypical presentations and diagnosis can be difficult because of limitations in imaging modalities. We report the case of a 69-year-old man with a history of hypertension and hyperlipidemia who presented with several months of dyspnea with exertion. There was a soft systolic murmur on exam. Transthoracic echocardiogram showed a large right atrial mass that appeared to be adherent to the interatrial septum. Considering the size and apparent attachment to the interatrial septum the suspicion was a myxoma. After further imaging however, including transesophageal echocardiogram, cardiac computed tomography (CT) and cardiac magnetic resonance imaging (MRI) the diagnosis changed to a lipoma. The patient had surgical resection of the mass which confirmed the diagnosis. A multi-imaging approach may be helpful and required to accurately diagnose cardiac masses.

Hematologic mask of infective endocarditis as a cause of fever of unknown origin: a case report

Fever of unknown origin (FUO), despite the wide diagnostic potential of modern medicine, remains a difficult problem for clinicians. Often, making a correct diagnosis requires a comprehensive examination of the patient, as well as the joint work of doctors from different specialties.A 57-year-old male patient was hospitalized due to an episode of loss of consciousness, unmotivated weight loss and daily evening temperature rises to 38-39о C, accompanied by chills and increased sweating. Previously, he was examined by a general practitioner, a cardiologist, and repeatedly by a hematologist. There was no evidence of an infectious disease, arrhythmias, multiple myeloma, lymphoproliferative disorders. Monoclonal gammopathy of undetermined significance (MGUS) was diagnosed. Blood tests revealed neutrophilia (9800 cells/ μl) and moderate anemia. The patient was hospitalized to determine the FUO cause. Heart auscultation revealed a systolic murmur in the mitral valve, which was previously absent. Echocardiography revealed a mobile masses on the mitral valve, severe mitral regurgitation, and therefore infective endocarditis was diagnosed. Empirical antibiotic therapy with ceftriaxone and levofloxacin was administered. Further blood culture revealed growth of a HACEK representative Aggregatibacter actinomycetemcomitans, sensitive to both drugs. Examination established the odontogenic nature of endocarditis. During treatment, stable normothermia and significant blood count improvement were achieved. However, fluoroquinolone therapy led to tendinopathy. Due to persistent grade 3 mitral regurgitation, the patient underwent routine mitral valve replacement. Further follow-up revealed satisfactory condition of the patient.A feature of this case is the atypical course of infective endocarditis, occurring under a hematological mask, which made its diagnosis difficult. When examining a FUO patient, infective endocarditis should be included in the range of possible causes, taking into account not only the typical clinical picture, but also the numerous masks of this disease.

Cardiac surgery and acute hepatitis B - Use of the Seraph™ 100 hemofilter.

We present a case of a 55-year-old male, presenting with angina symptoms with electrocardiographic changes and a panfocal systolic murmur radiating to the carotids. He had a primary HBV infection 8 months ago, without antiviral treatment. Echocardiography showed critical aortic valve stenosis (area: 0.53 cm2/m2). No coronary lesions were found on coronary angiography. Blood analysis revealed AST/GOT of 96 U/L and ALT/GPT 150 U/L. The serological profile revealed positive IgM anti-HBc, anti-HBc, anti-HBs and anti-HBe antibodies, with an increasing viral load (VL). The abdominal ultrasound identified mild hepatic fibrosis (F3) with minimal steatosis. Mechanical aortic prosthetic valve replacement was performed under CPB. The Seraph™ 100 filter was incorporated into the CPB circuit to reduce the risk of HBV contamination, infection and liver failure. The postoperative VL was monitored (Table 1). Liver function tests showed peak levels of bilirubin 0.66 mg/dL, AST/GOT 58 U/L, ALT/GPT 74 U/L at 6 hours post-surgery, with recovery of normal ranges at 48 hours post-surgery.

Etude de la cardiomyopathie du péri-partum dans un contexte à ressources limitées : cas de la région de Zinder, Niger

To describe the epidemiological, clinical, paraclinical, therapeutic and evolutionary characteristics of of peripartum cardiomyopathy (PPCM) in the internal medicine department of the Zinder National Hospital (ZNH). This was a descriptive cross-sectional study carried out from 2018 to 2022 at the ZNH Department of Internal Medicine. Included were all patients admitted for PPCM who met National Heart Blood and Lung Institute criteria. The data collected was analyzed using Excel and EPI INFO v7. We had collected 100 cases of PPCM out of a total of 8706 hospitalized patients, i.e. a hospital prevalence of 1.14%. The mean age of the patients was 27.9 years ± 7.4 [17-45]. The majority of patients were from underprivileged social strata (n=64). The risk factors for PMPC found were essentially hot bath (n=66), home birth (n=40), natron porridge (n=35) and multiparity (n=57). Cardiac symptomatology appeared postpartum in 56% of patients. Dyspnea was the main symptom in 98% of cases. The physical signs were dominated by the functional systolic murmur (66%). Three quarters (75%) of the patients had congestive heart failure. Electrocardiographic signs were dominated by left ventricular hypertrophy (n=65). Cardiomegaly was present in 94% of patients. Left ventricular ejection fraction was altered in all patients. Impaired renal function was found in 31% of patients. Management was based on a low-sodium diet tripod, diuretics and converting enzyme inhibitors. Two cases of death were recorded. PPCM is common in the Zinder region. It affects young women with several risk factors and is revealed by signs of congestive heart failure. For a better understanding of this still poorly elucidated condition, it is necessary to pursue research efforts.

Shorten τ<sub>1</sub> in Chronic Early vs. Late Systolic LV Load for Systolic Dysfunction in Ascending vs. Descending Thoracic Aortic Stenosis

<i>Background:</i> Arterial hypertension (HTA) results with diastolic LV dysfunction (DD), important to develop systolic LV dysfunction and exercise intolerance with HF. Separating between chronic late (LL) to early LV load (EL) during systole, impaired LV relaxation is present earlier in chronic LL vs. EL, having early HF, as result of myocardial ischemia and systolic LV dysfunction in HTA. <i>Objective and Methods:</i> to assess early systolic in diastolic LV dysfunction from biexponential τ regression assessment, using single beat and mono-exponential regression analysis, with nonzero asymptote with special software in LL and EL, between 4<sup>th</sup> and 8<sup>th</sup> week in a porcine model. This assesses early HF and systolic LV pump dysfunction, from fast_τ (τ<sub>1</sub>), for early systolic LV dysfunction, in LVH remodeling in moderate LV afterload increase. Fourteen domestic male pigs, underwent LV pressure measurements with conductance Millar 5F catheter having moderate ascending aortic banding (EL=6), and in descending thoracic aortic stenosis, as in hypertension (LL=8). τ<sub>1</sub> (τ_fast) and τ<sub>2</sub> (for τ_slow) component of bi-exponential τ analyzed LV dysfunction at 4<sup>th</sup> vs. 8<sup>th</sup> week. Under reduced LV load (m3), during ventilation preserved (m1) or suspended transitionally (m2), fast τ assess early systolic dysfunction in LL vs. EL. Associated murmurs were assessed to detect LV valves dysfunction. Data was compared statistically, using two-way repeated measurement ANOVA, after Leven normality test. Results are means±SEM or medians (quartiles), for significant p<0.05. <i>Results:</i> mono-exponential τ was not different, neither changed in LL vs. EL at 4<sup>th</sup> and 8<sup>th</sup> week in m1, m2 or m3, that reduced in both groups with mechanical LV load reduction at 4<sup>th</sup> and 8<sup>th</sup> week (p<0.05). Prolonged bi-exponential asynchronous τ<sub>2</sub>/τ<sub>1</sub> ratio in EL was different from LL at 8<sup>th</sup> week, resulted from LV afterload (τ<sub>2</sub>τ<sub>1</sub> interaction p<0.05). τ_fast was different, being shorten in EL vs. LL at 4<sup>th</sup> and 8<sup>th</sup> week. Reduced bi-exponential τ<sub>2</sub>τ<sub>1</sub> ratio in EL and increased in LL, with mechanical load reduction, improved LV ischemia with DD in EL at 4<sup>th</sup> and 8<sup>th</sup> week of moderate LV afterload increase, but did not respond in LL. There was predominant systolic murmur in EL and diastolic murmur in LL, pronounced with load reduction. <i>Conclusion:</i> Prolonged bi-exponential τ<sub>1</sub> in LL shows early systolic LV dysfunction within DD. LV ischemia and systolic with diastolic LV pump dysfunction in EL presents shorten fast_τ, being unresponsive to mechanical LV load reduction in LL.

A Comprehensive Overview of a Rare Case of Type 2 Autoimmune Polyglandular Syndrome

Introduction: Polyglandular deficiency syndromes reflects a wide spectrum of disorders. Autoimmune polyglandular syndrome (APS) is a rare condition generally divided into two categories APS -1 and APS -2.  Case Report: We report a case of APS-2 in a 28 years old male with marfanoid habitus, presented with significant weight loss, fever and hemoptysis. upon examination, he had exophthalmos, pallor, marfanoid habitus with systolic murmur in all cardiac areas and coarse crepitation in left hemithorax. Laboratory evaluation revealed left upper lobe pneumonia secondary to Acinetobacter. Further evaluation revealed severe hyperthyroidism, anti TPO, TRAB antibody was elevated, megaloblastic anemia with atrophic gastritis and positive 21 alpha hydroxylase.  Diagnosis: The above findings were consistent with the diagnosis of APS type 2 (Graves’ Disease, Adrenal Insufficiency, Pernicious Anaemia, Vitiligo). The presence of two or more endocrine deficiencies defines APS-2 which may include graves’ disease, type 1 Dm, primary adrenal insufficiency, hypogonadism and features like pernicious anaemia, vitiligo and alopecia.  Conclusion: Circulating antibodies may precede development of clinical disease by many years but would allow clinician to follow the patient and identify the disease onset at the earliest.

Screening for Cardiac Abnormalities in Asymptomatic Patients Undergoing low to Intermediate Risk Non-Cardiac Surgery

Abstract Background Perioperative cardiovascular complications are the predominant cause of morbidity and mortality in patients undergoing non cardiac surgery. Major cardiovascular and cerebrovascular complications, including death, myocardial infarction or stroke, occur in approximately 1 of every 33 hospitalizations for non-cardiac surgery. Objective To uncover hidden cardiac problems that may cause complications during and after non- cardiac major surgery. Patients and Methods This observational cross-sectional study was conducted on 250 asymptomatic patients coming to Ain Shams University Hospitals and police hospitals clinics for preoperative assessment to undergo low to intermediate risk non cardiac surgery. Results most of the study group were males (68%) with a mean age of 47 years. Diabetes and hypertension were found in 45% and 41% of patients respectively. Most patients had GIT operations (34%) followed by cataract extraction (14%), orthopedic operation was done in 11% of patients. clinical examination was normal in 98% of patients with 2% had AF and systolic murmur. Significantly abnormal ECG was found in 10% of patients. Preoperative significantly abnormal TTE was detected in 9% of patients. post-operative cardiovascular complications occurred in 7% of patients who had completely normal preoperative clinical, ECG and echocardiographic data. Most of these patients had orthopedic operations and hysterectomy. Conclusion The clinical and predictive value of routine preoperative ECG and echocardiography for major cardiac adverse events is limited especially in low-risk patients groups undergoing low to intermediate risk operations. However, it helps uncovering of hidden conditions that alter patient's plan of treatment. The type of surgery was an important factor for the occurrence of post-operative complications.

Cardiac murmurs in children.

Approximately 50% of children experience a cardiac murmur at some point in their lives; <1% of these murmurs are attributed to congenital heart disease (CHD). Cardiac murmur might be the first clinical sign of a significant CHD in children. Despite careful routine medical examinations at birth, approximately 50% of CHD cases could remain unrecognised. Cardiovascular symptoms and signs could be specific or non-specific in neonates and children with heart murmurs. Knowledge about red flags in history and physical examinations, and syndromic associations of common CHDs are important. Auscultatory skills to identify systolic, diastolic and continuous murmurs and heart sounds are essential. Differential diagnosis should be formulated based on the location of maximum intensity of murmurs. Younger infants and children with pathological murmurs and red-flag signs should be promptly referred to local paediatric cardiology services for further investigations. Significant skill and knowledge are required for the identification of critical murmurs and associated cardiovascular problems. This review provides a simplified comprehensive update on cardiac murmurs and associated conditions in neonates and children.

Aortic stenosis: Update in monitoring and management.

Aortic stenosis (AS) remains one of the most commonly encountered valvular pathologies. Medical management does not alter the progression of the disease, making assessment of severity and timing of referral for valve replacement the most important aspects of caring for patients with AS. To review the contemporary management of AS, including signs and symptoms, echocardiography and decision making in management. Severe symptomatic AS is frequently accompanied by dyspnoea, chest pain or syncope and a physical examination might reveal the presence of an ejection systolic murmur. Echocardiography is the first and most useful investigation to stratify risk and determine requirement for valve replacement by assessing valve gradients and left ventricular function. Surgical and transcatheter options now exist for treatment of AS and decision making is usually multidisciplinary and based on individual patient parameters.

The study of the impact of murmurs on heart sounds by using multiple signal classification pseudo-spectrum

Abstract The aim of this study was to present a novel framework for the analysis of the impact of murmurs on heart sounds recorded in real clinical environment. Heart sound records were rigorously selected from the PASCAL dataset using an automated signal quality assessment algorithm. Recordings from 159 patients were analyzed for spectral differences in normal, systolic and diastolic murmurs using a Multiple signal classification algorithm pseudo-spectrum. The spectral features evaluated for the first heart sound (S1) and the second heart sound (S2) were: energy, frequency and frequency density. Results show increased energy of fundamental heart sounds in systolic and diastolic murmurs similarly, whilst frequency is decreased inversely. Furthermore, the frequency density of the first and second heart sounds decreases in murmurs and it is shown to be the lowest in systolic murmur cases.