New Onset Cardiac Murmur and Exertional Dyspnea in an Apparently Healthy Child: A Rare Localization of Obstructive Myxoma in the Right Ventricle Outflow Tract without Pulmonary Embolization-A Case Report and Literature Review.

Myxomas are slowly growing benign neoplasms which are rare in children. Up to 80% can be located in the left atrium and generate symptoms such as embolism, cardiac failure, fever and weight loss. Rarely, myxomas can be detected in the right ventricle outflow tract, causing arrhythmias, pulmonary emboli and sudden death. We report the case of a 13-year-old healthy child brought to the Emergency Department (ED) of the Children's Hospital Bambino Gesù, Rome, for recent dyspnea, chest pain on exertion and new onset cardiac murmur. Patient underwent medical examination and echocardiogram with the finding of a rounded and lobulated voluminous mass in the right ventricle outflow tract (RVOT) which caused severe obstruction. The contrast computed tomography (CT) scan confirmed the presence of a heterogeneously enhancing soft-tissue mass occupying the RVOT with no evidence of pulmonary embolization. The mass was surgically excised, and the pathologic examination confirmed our suspicion of myxoma. Our experience suggests that myxoma can have mild clinical symptoms, the presentation may be non-specific, and diagnosis can be a challenge Careful examination and a diagnostic imaging workup, primarily with the transthoracic echocardiogram, are needful to make a rapid differential diagnosis and to better manage surgical treatment and follow-up.

The Diagnostic Utility of Computer-Assisted Auscultation for the Early Detection of Cardiac Murmurs of Structural Origin in the Periodic Health Evaluation.

BACKGROUND: Identification of the nature of cardiac murmurs during the periodic health evaluation (PHE) of athletes is challenging due to the difficulty in distinguishing between murmurs of physiological or structural origin. Previously, computer-assisted auscultation (CAA) has shown promise to support appropriate referrals in the nonathlete pediatric population. HYPOTHESIS: CAA has the ability to accurately detect cardiac murmurs of structural origin during a PHE in collegiate athletes. STUDY DESIGN: Cross-sectional, descriptive study. LEVEL OF EVIDENCE: Level 3. METHODS: A total of 131 collegiate athletes (104 men, 28 women; mean age, 20 ± 2 years) completed a sports physician (SP)-driven PHE consisting of a cardiac history questionnaire and a physical examination. An independent CAA assessment was performed by a technician who was blinded to the SP findings. Athletes with suspected structural murmurs or other clinical reasons for concern were referred to a cardiologist for confirmatory echocardiography (EC). RESULTS: Twenty-five athletes were referred for further investigation (17 murmurs, 6 abnormal electrocardiographs, 1 displaced apex, and 1 possible case of Marfan syndrome). EC confirmed 3 structural and 22 physiological murmurs. The SP flagged 5 individuals with possible underlying structural pathology; 2 of these murmurs were confirmed as structural in nature. Fourteen murmurs were referred by CAA; 3 of these were confirmed as structural in origin by EC. One such murmur was not detected by the SP, however, and detected by CAA. The sensitivity of CAA was 100% compared with 66.7% shown by the SP, while specificity was 50% and 66.7%, respectively. CONCLUSION: CAA shows potential to be a feasible adjunct for improving the identification of structural murmurs in the athlete population. Over-referral by CAA for EC requires further investigation and possible refinements to the current algorithm. Further studies are needed to determine the true sensitivity, specificity, and cost efficacy of the device among the athletic population. CLINICAL RELEVANCE: CAA may be a useful cardiac screening adjunct during the PHE of athletes, particularly as it may guide appropriate referral of suspected structural murmurs for further investigation.

Gigantic unruptured sinus of Valsalva aneurysm presenting as an incidental murmur.

We report a case of a 56-year-old man referred by his family physician with an asymptomatic cardiac murmur. Trans-thoracic echocardiography (TTE) suggested an unruptured right sinus of Valsalva aneurysm (SVA) causing extrinsic compression of the right ventricular outflow tract. This was confirmed with an ECG-gated cardiac CT showing a large right SVA measuring 35×37×42 mm in size. Coronary angiography demonstrated non-obstructive coronary artery disease. Ascending thoracic anterior in the right anterior oblique view delineated the right SVA. The patient underwent aortic valve sparing surgical repair of the aneurysm with an excellent result. Echocardiography confirmed obliteration of the aneurysm and normal aortic valve function postoperatively.

Continuous murmur--the auscultatory expression of a variety of pathological conditions.

Continuous murmur is a peculiarity of cardiovascular auscultation, relatively rare, which often hides complex cardiovascular diseases. This article is a review of literature data related to the continuous murmurs accompanied by commenting and illustrating them through our own cases.Recognizing of a continuous murmur and understanding the cardiovascular pathologies that it can hide, is a challenge in current practice.

Electrocardiography Series. ECGs of structural heart disease: Part 1.

Electrocardiogram (ECG) is a useful but imperfect investigation in the diagnosis and possible follow-up of structural heart disease such as ventricular hypertrophy. Different ECG criteria with different sensitivity and specificity are available to aid the detection of left or right ventricular hypertrophy. Subsequent echocardiography can help in the quantification of ventricular mass and identification of the aetiology.

Digital Subtraction Phonocardiography (DSP) applied to the detection and characterization of heart murmurs.

BACKGROUND: During the cardiac cycle, the heart normally produces repeatable physiological sounds. However, under pathologic conditions, such as with heart valve stenosis or a ventricular septal defect, blood flow turbulence leads to the production of additional sounds, called murmurs. Murmurs are random in nature, while the underlying heart sounds are not (being deterministic). INNOVATION: We show that a new analytical technique, which we call Digital Subtraction Phonocardiography (DSP), can be used to separate the random murmur component of the phonocardiogram from the underlying deterministic heart sounds. METHODS: We digitally recorded the phonocardiogram from the anterior chest wall in 60 infants and adults using a high-speed USB interface and the program Gold Wave http://www.goldwave.com. The recordings included individuals with cardiac structural disease as well as recordings from normal individuals and from individuals with innocent heart murmurs. Digital Subtraction Analysis of the signal was performed using a custom computer program called Murmurgram. In essence, this program subtracts the recorded sound from two adjacent cardiac cycles to produce a difference signal, herein called a "murmurgram". Other software used included Spectrogram (Version 16), GoldWave (Version 5.55) as well as custom MATLAB code. RESULTS: Our preliminary data is presented as a series of eight cases. These cases show how advanced signal processing techniques can be used to separate heart sounds from murmurs. Note that these results are preliminary in that normal ranges for obtained test results have not yet been established. CONCLUSIONS: Cardiac murmurs can be separated from underlying deterministic heart sounds using DSP. DSP has the potential to become a reliable and economical new diagnostic approach to screening for structural heart disease. However, DSP must be further evaluated in a large series of patients with well-characterized pathology to determine its clinical potential.

Cause of heart murmurs in 57 apparently healthy cats.

Heart murmurs are caused by turbulent blood flow or by vibration of cardiac structures. Turbulent blood flow may originate from structural heart disease or from physiological phenomena. The aims of this study were to establish the cause of heart murmurs in apparently healthy adult cats and to determine whether a heart murmur is a reliable indicator of heart disease. In this retrospective study, we reviewed the medical records of cats in which a heart murmur was detected during physical examination by one of the authors in the period January 2008 to December 2009. Cats younger than 6 months and those with systemic disease were excluded. Timing, grade, and point of maximum intensity of the murmur were determined by one observer (MD) before 2D-, M-mode and Doppler echocardiography. Fifty-seven cats (median age 76 months, range 6-194) were included, 30 neutered females and 27 neutered males. All murmurs were systolic and varied in intensity from 2/6 to 5/6. The point of maximum intensity was the left or right parasternal region in 34/57 (61%) of murmurs. Murmurs were caused by dynamic left ventricular outflow tract obstruction in 25/57 (44%) cats, dynamic right ventricular outflow tract obstruction in 9/57 (16%) cats, and combined dynamic left and right outflow tract obstruction in 11/57 (19%) cats. In 5 (9%) cats the cause of the murmur could not be identified. Heart disease was present in 50 (88%) cats, namely, left ventricular hypertrophy in 44 (77%) and congenital defects in 6 (11%) cats. In conclusion, most heart murmurs in apparently healthy cats are detected in the left or right parasternal region and are caused by dynamic left and right ventricular outflow tract obstruction. Because most cats (88%) with a heart murmur had heart disease in this study, if a heart murmur is detected in an apparently healthy cat, echocardiography is recommended to determine the cause of the heart murmur and the presence of heart disease.

Portable digital esophageal stethoscope system.

Heart sound occurs when the heart contracts and expands. It provides information on myocardial contractility and blood vessels, which is not obtainable from ECG. For this reason, stethoscopy of heart sound in anesthesiology is a very crucial means for acquiring cardiac information and preventing intraoperative medical accidents, and it requires a system for precise objective measurement and analysis of heart sound and murmur. Thus, this study purposed to develop portable digital esophageal stethoscope (PDES) that can objectify and quantify heart sound and murmur. In this study, we designed PDES for precise measurement and analysis of heart sound and murmur data. Heart sound information obtained by inserting the sensor of the PDES into the patient's esophagus can be transmitted to a terminal or a PC and displayed on the screen The amplitude and waveform of heart sound are displayed using self-developed software Heart Sound 1.0. The results of experiment with the developed PDES showed that data on the amplitude and waveform of heart sound and murmur were produced stably in real-time. In addition, when heart sound was heard using a headphone, the sound was clear without external murmur. The PDES developed in this study, which complements the disadvantages of traditional esophageal stethoscope while preserving its advantages, could not only examine heart sound and murmur using an esophageal catheter but also display the amplitude and waveform of heart sound and murmur and measure the patient's body temperature. Accordingly, the developed PDES is expected to be useful in the continuous stethoscopy of heart sound during operation and to contribute to research on heart sound by providing heart sound data.

Intracardiac echocardiography imaging of periprosthetic valvular regurgitation.

A 62-year-old woman was referred for evaluation of recent-onset dyspnoea at rest and a newly found systolic heart murmur, heard best at the apex of the heart. The patient's history was notable for surgical mitral valve replacement 3 years ago, due to severe mitral regurgitation (myxomatous degeneration of the mitral valve). The transthoracic echocardiogram gave the impression of a mitral regurgitant jet, but the acoustic shadow of the prosthesis did not allow adequate evaluation of the regurgitation. A transoesophageal echocardiogram was performed showing a normally functioning mitral prosthesis, with a small periprosthetic leak (Figure 1, upper panel), disproportionate to the patient's symptoms and marked signs of haemolysis. An intracardiac ultrasound study revealed a large paravalvular regurgitant jet, indicating significant periprosthetic regurgitation (Figure 1, lower panel). Intracardiac echocardiography is increasingly being used to guide percutaneous interventions and electrophysiological procedures. The present case suggests a potentially useful widening of the range of intracardiac ultrasound clinical applications, out of the realm of device-closure interventions and electrophysiological procedures. It appears that intracardiac echocardiography could become a second-line alternative to transoesophageal echocardiography, especially in patients with contraindication to the latter.

Saw-tooth cardiomyopathy.

We present an unusual case of cardiomyopathy in a two month old male infant with a grade-I systolic murmur. Echocardiographic examination disclosed left ventricular (LV), dysplasia with saw-tooth like inwards myocardial projections extending from the lateral walls towards the LV cavity. There was mild LV systolic dysfunction with apical hypokinesia. Cardiovascular magnetic resonance demonstrated in detail these cross bridging muscular projections originating from the inferior interventricular septum and lateral LV wall, along with areas of hypokinesis at the LV septum and apex in a noncoronary distribution, without any late gadolinium enhancement. We have termed this condition saw-tooth cardiomyopathy because of the very characteristic appearance.

Small ventricular septal defect: long-term expectant clinical management.

BACKGROUND: The small ventricular septal defect (VSD) usually presents good clinical evolution, even at long-term follow-up. OBJECTIVE: To verify the clinical evolution of patients with small VSD in order to determine the continuation or not of the expectant conduct, considering the low operative risk, which results in a more liberal indication for surgery. METHODS: From October 1976 to December 2007, 187 cases of small VSD (diameter < 3 mm at the echocardiogram) were evaluated and 155 of them were assessed at long-term follow-up. Time of the clinical manifestation of the murmur and evolution aspects such as the spontaneous closure of the defect (group I)--64 cases, persistence of the initial size (group II--74 cases and decrease in the size of the defect (group III)--17 cases, in addition to clinical complications, were studied. RESULTS: The clinical manifestation of the murmur occurred, in the majority of cases, during the first month of life, corresponding to 48 (75%), 54 (72.9%) and 12 (70.5%) patients, in the three groups, respectively and after the first year of life in 11 (5.8%) patients. Spontaneous closure occurred in the first year of life in 48 cases (75%), mean of 7.6 months and from 1 to 5.5 years in 15 patients (23.4%), with a maximum follow-up of 18 years. The persistence of the defect until 40 years of age was observed. The decrease in the size of the defect occurred on a mean of 15 months, followed for up to 9 years. The probability of VSD closure by the actuarial curve was 34.38% in 1 year and 49.89% in 5 years. There were no clinical complications. CONCLUSION: A favorable evolution of the small VSD at long-term follow-up does not require surgical intervention, with concerns regarding the strict antibiotic prophylaxis.

Comunicação interventricular pequena: conduta clínica expectante em longo prazo / Comunicación interventricular pequeña: conducta clínica expectante a largo plazo / Small ventricular septal defect: long-term expectant clinical management

FUNDAMENTO: A comunicação interventricular (CIV) pequena apresenta geralmente boa evolução clínica, mesmo em longo prazo. OBJETIVO: Verificar evolução clínica de pacientes com CIV pequena, a fim de determinar continuidade ou não da conduta expectante, em vista do baixo risco operatório, o que ocasiona maior liberalidade da indicação cirúrgica. MÉTODOS: No período de outubro de 1976 a dezembro de 2007, foram examinados 187 casos com CIV pequena (diâmetro menor que 3 mm pelo ecocardiograma) e seguidos 155 deles em longo prazo. Estudaram-se a época de exteriorização do sopro e aspectos evolutivos como fechamento espontâneo do defeito (grupo I) - 64 casos, continuidade do tamanho inicial (grupo II) - 74 casos e diminuição do mesmo (grupo III) -17 casos, além de intercorrências clínicas. RESULTADOS: A exteriorização do sopro ocorreu na maioria no primeiro mês de vida, correspondendo a 48 (75 por cento), 54 (72,9 por cento) e 12 (70,5 por cento) pacientes, nos três grupos respectivos, e acima do primeiro ano em 11 (5,8 por cento) pacientes. Fechamento espontâneo ocorreu no primeiro ano em 48 casos (75 por cento), média de 7,6 m e de 1 a 5,5 anos em 15 pacientes (23,4 por cento), em acompanhamento máximo de 18 anos. Verificou-se continuidade do defeito até 40 anos. A diminuição do defeito ocorreu em média de 15 meses, seguidos até 9 anos. Probabilidade de fechamento da CIV, pela curva actuarial, foi de 34,38 por cento em 1 ano e de 49,89 por cento em 5 anos. Não houve intercorrências clínicas. CONCLUSÃO: Evolução favorável da CIV pequena em longo prazo dispensa intervenção operatória, com preocupação da profilaxia antibiótica rigorosa.

BACKGROUND: The small ventricular septal defect (VSD) usually presents good clinical evolution, even at long-term follow-up. OBJECTIVE: To verify the clinical evolution of patients with small VSD in order to determine the continuation or not of the expectant conduct, considering the low operative risk, which results in a more liberal indication for surgery. METHODS: From October 1976 to December 2007, 187 cases of small VSD (diameter < 3 mm at the echocardiogram) were evaluated and 155 of them were assessed at long-term follow-up. Time of the clinical manifestation of the murmur and evolution aspects such as the spontaneous closure of the defect (group I) - 64 cases, persistence of the initial size (group II - 74 cases and decrease in the size of the defect (group III) - 17 cases, in addition to clinical complications, were studied. RESULTS: The clinical manifestation of the murmur occurred, in the majority of cases, during the first month of life, corresponding to 48 (75 percent), 54 (72.9 percent) and 12 (70.5 percent) patients, in the three groups, respectively and after the first year of life in 11 (5.8 percent) patients. Spontaneous closure occurred in the first year of life in 48 cases (75 percent), mean of 7.6 months and from 1 to 5.5 years in 15 patients (23.4 percent), with a maximum follow-up of 18 years. The persistence of the defect until 40 years of age was observed. The decrease in the size of the defect occurred on a mean of 15 months, followed for up to 9 years. The probability of VSD closure by the actuarial curve was 34.38 percent in 1 year and 49.89 percent in 5 years. There were no clinical complications. CONLCUSION: A favorable evolution of the small VSD at long-term follow-up does not require surgical intervention, with concerns regarding the strict antibiotic prophylaxis.

FUNDAMENTO: La comunicación interventricular (CIV) pequeña presenta generalmente buena evolución clínica, aun a largo plazo. OBJETIVO: Verificar la evolución clínica de pacientes con CIV pequeña, a fin de determinar continuidad o no de la conducta expectante, en vista del bajo riesgo operatorio, resultando una mayor liberalidad de la indicación quirúrgica. MÉTODOS: En el período de octubre de 1976 a diciembre de 2007, se examinaron 187 casos con CIV pequeña (diámetro menor que 3 mm por el ecocardiograma) y se siguieron a 155 de ellos a largo plazo. Se estudiaron la época de exteriorización del soplo y los aspectos evolutivos, como cierre espontáneo del defecto (grupo I): 64 casos; continuidad del tamaño inicial (grupo II): 74 casos; y disminución del mismo (grupo III):17 casos; además de intercurrencias clínicas. RESULTADOS: En la mayoría de los casos, la exteriorización del soplo tuvo lugar en el primer mes de vida, correspondiendo a 48 (75 por ciento), 54 (72,9 por ciento) y 12 (70,5 por ciento) pacientes, en los tres grupos respectivos, y después del primer año en 11 (5,8 por ciento) pacientes. Cierre espontáneo ocurrió en el primer año en 48 casos (75 por ciento), promedio de 7,6 m y de 1 a 5,5 años en 15 pacientes (23,4 por ciento), en seguimiento máximo de 18 años. Se verificó continuidad del defecto hasta 40 años. La disminución del defecto ocurrió en promedio de 15 meses, seguidos hasta 9 años. Probabilidad de cierre de la CIV, por la curva actuarial, fue de un 34,38 por ciento en 1 año y de un 49,89 por ciento en 5 años. No hubo intercurrencias clínicas. CONCLUSIÓN: Evolución favorable de la CIV pequeña a largo plazo dispensa intervención operatoria, con preocupación de la profilaxis antibiótica rigurosa.

Late presentation of cor triatriatum with persistent levoatrial cardinal vein.

An asymptomatic 10-year-old boy presented with reduced exercise tolerance and an echocardiographic diagnosis of cor triatriatum. Transthoracic and transesophageal echocardiography failed to reveal the persistent levoatrial cardinal vein discovered at surgery. In patients with late presentation of cor triatriatum with severe mitral inflow obstruction and a small patent foramen ovale, an alternative communication between the posterior collecting chamber and the systemic venous circulation should be sought with alternative imaging techniques.

Sudden onset congestive heart failure with a continuous murmur: ruptured sinus of Valsalva aneurysm complicated by anomalous origin of the left coronary artery.

Ruptured sinus of Valsalva aneurysm is an unusual cause for congestive heart failure, and anomalous coronary arteries have rarely been found in association. A 47-year-old man developed sudden onset heart failure due to a ruptured noncoronary sinus of Valsalva fistula to the right atrium. Coronary angiography revealed an anomalous left coronary artery arising from the right coronary sinus, limiting percutaneous options for repair. We review the incidence, complications, and management of sinus of Valsalva aneurysms and anomalous left coronary arteries.

Should I do this case? - The Paediatric Murmur

Cardiac murmurs in children may have serious haemodynamic implications during anaesthesia and surgery. Innocuous and pathological murmurs have characteristics that differ; but there is unfortunately also overlap. Most murmurs (70) are innocuous and anaesthesia can be safely administered. It is essential to investigate any murmur detected preoperatively to exclude dangerous murmurs. An appropriate history and thorough clinical examination will enable diagnosis of most innocuous murmurs. For some cardiac lesions perioperative antimicrobial prophylaxis is necessary for some procedures. Patients should be referred for postoperative evaluation and follow-up. Patients and parents should be informed of the implications and need for prophylaxis and follow-up