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Introduction: Benign intracardiac tumours are the most common of the 5% of primary tumours and account for 90% of intracardiac tumours. Myxoma, which is the main benign tumour, rarely localizes to the mitral valve, in the order of 1-5%, associated with severe symptoms and enormous complications. The objective is to report a rare and severe case of mitral valve myxoma resulting in severe obstruction of the valve orifice. Presentation of Case: A 65-year-old Moroccan woman, without profession, the diabetic patient presented with progressive dyspnoea, in whom transthoracic echocardiography (TTE) completed by transesophageal echocardiography (TEE), a cardiac magnetic resonance imaging (MRI), a cerebro-throracoabdominopelvic Positron emission tomography-scan (PET-scan) showed a cauliflower-shaped mass embedded in the small mitral valve, evoking the diagnosis of myxoma, confirmed by the anatomopathological examination. A lumpectomy with mitral valve plasty was performed in association with coronary artery bypass surgery for a tri truncal lesion. The evolution was marked by an improvement in the clinical and echographic state. Discussion: Myxoma is the first benign tumour encountered in women between the 3rd and 6th decade, whose diagnosis is evoked by a TTE, better by a TEE or more, by a computed tomography (CT scan) or even a cardiac MRI which specifies the visualization of the soft parts with all the internal details of the myxoma, whose confirmation is carried out on the histopathological analysis of the operating room. Conclusion: The management was based on complete resection of the tumor associated with mitral valve plasty.

Objective: The present study aimed to investigate the relationship between brain natriuretic peptide (BNP) levels and blood pressure variability among hypertensive patients aged over 40 years. Methods: The study recruited 120 patients from a cardiology outpatient clinic who had been diagnosed with hypertension and taking antihypertensive medication for at least 6 months. Demographic and clinical information, blood pressure measurements, and blood samples were collected to measure BNP levels. The standard deviation of the mean arterial pressure over 24 hours was calculated as a measure of blood pressure variability. Linear regression was used to examine the association between BNP levels and blood pressure variability while controlling for age, sex, BMI, and medication history. Results: The study found a significant positive association between BNP levels and blood pressure variability (β=0.31, p=0.002), even after controlling for other variables. The linear regression model explained 20% of the variance in blood pressure variability (R2=0.20, F=9.52, p

Systemic lupus erythematosus is an autoimmune inflammatory disease of unknown etiology, the coronary lesions are extremely rare, and in particular the coronary aneurysms. We report a case of a coronary aneurysm of a patient diagnosed with Systemic lupus erythematosus who showed up at the emergency department suffering from a retro-sternal pain radiating to the jaws which he reported for 4 hours. An electrocardiogram was performed urgently and showed an offset of 2 mm in the inferior, right, basal and low lateral territories with a mirror V1, V2. The patient underwent coronary angiography which revealed an aneurysmal dilatation of the right coronary artery with multiple atheromatous aneurysms of different sizes without thrombi visualized in the lumen of the arteries. Coronary aneurysms are a fatal and has potentially fatal complications that should be considered in the diagnostic of systemic lupus erythematosus patients with cardiac symptoms as early detection may allow complete healing.

Background Systemic juvenile lupus erythematosus can affect any organ including the heart. While pericarditis and endocarditis are the most common cardiac complications, dilated cardiomyopathy remains rare. We report the full recovery of dilated cardiomyopathy in a girl with juvenile lupus. Case presentation A 7-year-old girl presented with persistent fever, weight loss, alopecia, positive anti-lupus antibodies, and decreased complement. Examination found dyspnea, tachycardia, and hypotension. Chest X-ray revealed an enlarged cardiac silhouette, and echocardiography showed a left ventricular ejection fraction of 35%. After excluding other causes, systemic lupus erythematosus related dilated cardiomyopathy was selected. Heart failure treatment, Prednisone, and hydroxychloroquine were prescribed. On day three, she developed macrophage activation syndrome which responded to methylprednisolone boluses. Clinical and biological improvement was observed after 4 weeks, normalization of echocardiography after 4 months. Conclusion A review of the literature confirmed the rare and severe nature of dilated cardiomyopathy in juvenile lupus emphasizing the importance of performing echocardiography at the slightest cardiac sign.

Hemolytic uremic syndrome (HUS) is a non-exceptional, progressive complication of acute gastroenteritis in children, especially secondary to Escherichia coli infection. It is responsible for significant morbidity and significant mortality (10% of deaths) because of acute renal failure which often complicates it. Dilated cardiomyopathy is a rare but critical extra renal manifestation of the HUS. This article highlights the importance of considering the diagnosis of associated cardiomyopathy in the acute phase of HUS and the following months. A five-year-old boy presented with HUS with acute renal failure requiring peritoneal dialysis for 24 days. No cardiac signs appeared during the acute phase of the disease. After dialysis and normalization of blood pressure, fluid, and electrolyte disturbance, severe dilated cardiomyopathy with cardiac failure appeared three months later without definite etiology. A review of the literature confirmed the rare and severe nature of cardiac lesions occurring in HUS. Dilated cardiomyopathy is a rare but important extra renal manifestation of the HUS and is best demonstrated by echocardiography. A cardiac manifestation should also be screened for in the acute phase of HUS and several months later.

Background: Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that can affect any organ in the body, nonetheless cardiac involvement is frequent. Pericarditis is the most common type of heart attack followed by endocarditis, whereas myocarditis is rare. Despite improvements in the survival and quality of life of children with SLE, morbidity from the disease continues to be a major cause of death. Over the past three decades, lupus-related mortality has declined in all categories except cardiovascular disease. Heart disease is currently recognized as a major cause of death and morbidity in children with SLE. In fact, in the majority of cohort studies, the mean cardiovascular mortality is between 7 and 30%. We report full recovery of a little girl with SLE who developed dilated cardiomyopathy and review the literature. Case presentation: A 7-year-old girl presented with persistent fever, severe weight loss, alopecia, purpuric spots, positive anti-lupus antibodies, and decreased complement. She was diagnosed with SLE and treated with oral corticosteroids and hydroxychloroquine. On the third day of hospitalization, she developed a macrophage activation syndrome which improved with the methylprednisolone bolus. Additional examinations have been performed, including chest x-ray and echocardiography. They showed respectively marked enlargement of the cardiac silhouette and dilated cardiomyopathy with a left ventricular ejection fraction (LVEF) of approximately 35% associated with low pericardial effusion. She was diagnosed with SLE-related dilated cardiomyopathy and treated with furosemide, captopril, digoxin, spironolactone, and acetyl-salicylic acid. Clinical improvement was observed and the echocardiographic examination after 4 months of evolution was normalized, allowing cardiac treatments to be stopped while maintaining captopril.Conclusions: It is mandatory to check cardiac involvement in all patients with pediatric lupus to make an early diagnosis and ensure optimal management of cardiomyopathy, which is a rare, severe and silent complication.

Background Myeloproliferative neoplasms (MPNs) such as polycythemia Vera (PV) and Essential Thrombocythemia (ET) can be associated with a high risk of both venous and arterial thrombosis. However, the co-existence between these two complications is very rare and has never been described before, especially in young adults with no known history of MPNs. Case presentation We report the case of a 39 year-old Caucasian Moroccan male patient without cardiovascular risk factors (CVRF), who presented with acute chest pain. He also suffered from a severe headache since 2 weeks. Electrocardiogram (ECG) showed ST segment elevation myocardial infarction in the posterolateral leads. Cerebral Computed Tomography (CT) scan revealed subarachnoid hemorrhage (SAH), and cerebral Magnetic Resonance Angiography (MRA) found a Superior Sagittal Sinus Thrombosis (SSST). Routine blood tests showed raised hemoglobin and hematocrit in addition to leukocytosis and thrombocythemia. His coronary angiography revealed a thrombus in the ostial left circumflex artery (LCX). Further testing revealed positive Janus kinase 2 (JAK2) V617F mutation and low erythropoietin level, confirming the diagnosis of PV according to the 2008 World Health Organization (WHO) criteria. Antithrombotic and anti-ischemic treatments, in addition to myelosuppressive therapy with hydroxyurea, were initiated with a good clinical and biological evolution. Conclusion This case shows that MPNs are an important cause of thrombosis, especially in young patients with no other risk factors. Early diagnosis and appropriate management are fundamental before the occurrence of life-threatening complications that can sometimes present in unusual forms associating arterial and venous thrombotic events.

Background Carcinoid heart disease is a frequent complication of carcinoid syndrome. It is related to the release by the carcinoid tumor and/or its metastases of bioactive substances such as serotonin. It is characterized by right-sided valvular involvement and can lead to right-sided heart failure. It is a prognostic factor of carcinoid syndrome. Case presentation We report the case of a 53-year-old African woman with an endocrine tumor of her small intestine complicated by carcinoid heart disease, revealed by right-sided heart failure. The diagnosis was confirmed by transthoracic echocardiography, which showed a severe tricuspid regurgitation with a patent foramen ovale, and by increased serum chromogranin A and urinary 5-hydroxyindoleacetic acid. Initially she was treated with medical therapy (furosemide and injection of somatostatin). Afterwards she was proposed for surgery. The evolution of her treatment was good. Conclusions Carcinoid syndrome is complicated in 60% of the cases of a heart disease, and is responsible for an important morbidity and mortality. The prognosis of patients with carcinoid heart disease has improved in recent years through somatostatin analogues and the cardiac surgery.

L'hémorragie intra-alvéolaire est une urgence médicale rare et grave ayant de nombreuses étiologies. Nous rapportons ainsi le cas clinique d'un patient qui pourrait contribuer à élargir la littérature sur ce sujet. Le patient est un homme âgé de 62 ans ayant un antécédent de fibrillation auriculaire sous anti-vitamines K et admis dans un tableau de dyspnée d'apparition brutale avec hémoptysie et notion d'automédication par anti-inflammatoires non stéroïdiens. La radiographie et le scanner thoracique ont montré des opacités alvéolaires bilatérales diffuses. Au bilan d'hémostase de l'admission, l'INR était incoagulable. Le diagnostic d'hémorragie intra-alvéolaire était suspecté cliniquement et radiologiquement puis confirmé par l'endoscopie bronchique avec lavage broncho-alvéolaire (LBA) retrouvant un liquide uniformément hémorragique. Les études précédentes présentant des cas similaires secondaires à la prise d'anti-vitamines K sont rares. En conclusion, Il parait très important de souligner l'intérêt d'une surveillance stricte et optimale clinico-biologique des patients traités en anti-vitamines K afin d'éviter un surdosage pouvant contribuer à un accident hémorragique grave engageant le pronostic vital.

Introduction Atrioventricular septal defect (AVSD) is a complex heart malformation affecting the lower part of atrial septum, the highest part of ventricular septum, mitral and tricuspid valves.It represents 3 to 7.3 of congenital heart diseasesand it is the congenital heart disease which is mostly associated with chromosomic abnormalities especially trisomy 21. Purpose The mane objective of this study is to assess features of AVSD in trisomic patientsand the prognostic impact of trisomy 21 in AVSD. Methods Descriptive transversal retrospective and monocentric study conducted on a period of 10 years betweenDecember 2008 and December 2018. Were included all patientspresenting with AVSD confirmed by echocardiographyand followed-up in the congenital heart diseases unit of our department. Results 313 patients were included. ACSD was associated with trisomy 21 in 30.2%. The comparison of frequency of trisomy 21 regarding the type of AVSD and according to Rastelli classificationfor complete AVSDwas not significant with p values respectively of 0,110 and 0,086. We noted less pulmonary arterial hypertensions in trisomic patients (33%), par rapport compared to non trisomic patients (33% vs. 67%;P = 0,026).The study of the association between trisomy 21 and Eisenmenger syndromehasn’t shown a statistical difference (P = 1,000), the presence of trisomy 21 didn’t cause more mortality compared to eusomic patients (P = 0,110), trisomic patients who did benefit from surgical curehad less mortality compared to non treated patients (28,6% vs. 87,5%; P = 0,005). Conclusion AVSD, particularly in its complete for is a pathology which is strongly associated with trisomy 21. This shows the importance of the diagnosis and genetic counselling. The treatment is essentially surgical and should be done early.

Introduction Atrioventricular septal defect (AVSD) is a complex heart malformation affecting the lower part of atrial septum, the highest part of ventricular septum, mitral and tricuspid valves.It represents 3 to 7.3 of congenital heart diseases and it is the congenital heart disease which is mostly associated with chromosomic abnormalities especially trisomy 21. Purpose The main objective of this study is to assess the prognostic factors and the overall mortality rate of patients with AVSD. Methods Descriptive transversal retrospective and monocentric study conducted on a period of 10 years between December 2008 and December 2018. Were included all patients presenting with AVSD confirmed by echocardiography and followed-up in the congenital heart diseases unit of our department. Results A total of 313 patients were included. The median age was 9 months [3 days;56 years]. Female gender represented 51.4%. Complete ACSD was more common (77%), followed by partial ACSD (19.2%). Overall mortality was 44.7%. Among these deaths 50.8% occurred during the first 6 months of life. Complete ACSD was associated with higher mortality (52.5% of deaths) compared to other types of ACSD P = 0.002. Operated patients had a mortality of 28.9%, significantly lower than non-operated (64.9%) P Conclusion AVSD, particularly in its complete form is a serious condition with a poor prognosis. This shows the importance of the diagnosis and genetic counselling. The treatment is essentially surgical and when it is early, it considerably improves the prognosis.

Background Congenital heart disease (CHD) is frequently described in patients with Down syndrome (DS) and is the main cause of death in this population during the first two years of life. The spectrum of CHD patterns in DS varies widely worldwide; this variation could be due to sociodemographic, genetic and geographic factors. Methods A six-year retrospective, descriptive study was carried out from December 2008 to October 2014, based on the Paediatric Unit CHD registry of Ibn Rochd University Hospital. Clinical, echocardiographic and outcomes data were collected and sorted according to confirmation of the syndrome. Results Among 2 156 patients with CHD, 128 were identified with Down syndrome. The genders were equally represented (gender ratio 1) and the median age at diagnosis was 9.5 months (2 days to 16 years). The median age of mothers at delivery was 39 years (16-47). Of the 186 CHD lesions reported, the most common was atrioventricular septal defect (AVSD, 29%), followed by ventricular septal defect (VSD, 21.5%) and atrial septal defect (ASD, 19.9%). The most common associations of CHD were AVSD + ASD (10%) and VSD + ASD (7.8%). Surgery was the most common modality of treatment (54.3%). The overall mortality rate was 14.1%. Conclusion Our study confirmed that the profile and type of CHD in DS in the Moroccan setting exhibited slight differences in the distribution of these CHDs compared with European neighbours and other Western countries. Further studies are needed to determine which variables have an impact on these differences.

Introduction Eisenmenger's syndrome is defined by the appearance of cyanosis during the evolution of left-right shunts, due to a progressive alteration of the pulmonary arteriolar bed, leading to an increase in pulmonary vascular resistance. This evolution of the shunt towards a reaction of Eisenmenger, makes that its simple surgical closure becomes useless. The only surgical treatment is pulmonary transplantation, or cardiopulmonary transplantation. The interest of specific medical treatment of pulmonary arterial hypertension in Eisenmenger Syndrome remains controversial. Objective Evaluate the impact of the specific treatment of PAH on Eisenmenger patients. Methods We report the evolution over 1 year of 4 patients with congenital heart diseases in Eisenmenger. For each patient we evaluated NYHA functional class, 6 MWT, echocardiographic and hemodynamic parameters. Results Two of our patients had atrioventricular canal defect: – one complete; – the other partial; 2 patients with patent arterial duct. These patients were put under specific treatment of PAH: sildenafil + macitentan. Clinically, there was a slight improvement in the dyspnoea stage from stage III to II, with an average of 6MWT at 287 m. On the echocardiographic plan Peak tricuspid regurgitation velocity which averaged 4.59 m/s decreased to 3.5 m/s after one year of treatment with PASP by tricuspid regurgitation on average at 93 mmHg at the beginning of the treatment then at 70 mmHg at 1 year. Humodynamically, the PAPm (mmHg) were at 38 before specific treatment and decreased to 32 at the end of treatment, with a cardiac output that went from 2.3 to 2.5 l/min/m2, and an RVP of 7 to 5 wood units. We did not notice any complications and no deaths were reported. Conclusion We noted that despite improved dyspnoea and quality of life in these patients, the prognosis of patients undergoing Eisenmenger treatment under PAH specific therapy remains reserved.

Background The last decades have witnessed expanding evidence corroborating the major role of numerous cytokines involved in the pro-inflammatory and immunomodulatory responses. Macrophage migration inhibitory factor (MIF) may have major impact on the outcome of heart diseases; this pleiotropic cytokine is known to be implicated in myocardial inflammation. Single nucleotide polymorphism (SNP) in the MIF promoter (rs755622) regulates MIF expression and can lead to worse prognosis. The aim of our study was to discuss the possible association between MIF-173 genetic polymorphism and pediatric dilated cardiomyopathy (DCM). Methods Our study enrolled 105 unrelated individuals (53patients with DCM, and 52age- and sex-matched healthy controls). Genetic polymorphisms of several cytokine genes are known to result in altered gene expression. TaqMan genotyping assay and Polymerase chain restriction fragment length polymorphism (PCR-RFLP) methods were used. Results The overall data showed a significant correlation between MIF gene polymorphism and DCM patients. In codominant model, the frequencies of the GG, GC, and CC genotypes of MIF 173G/C were 54.7%, 34%, and 11.3% in cases and were 22%, 78%, and 0% in controls, respectively. A protective effect was observed under dominant genetic model CC + CG vs. GG ( p = 0.0009, OR = 0.2334, 95% CI = 0.0987–0.5518). Conclusion The results obtained in our analysis provide evidence on the potential protective role that MIF-173G/C play in the development of DCM disease in the pediatric population. MIF 173G/C rs755622 was significantly associated with a reduction in risk of pediatric DCM.

Background Functional analysis of the right ventricle cannot be reliably evaluated by conventional echocardiography, because of its complex geometry and load dependence of ejection phase indices. The Tei index, dP/dt, and myocardial acceleration during isovolumic contraction are parameters of right ventricular (RV) contractility unaffected by RV geometry. However, the effect of loading conditions on these parameters is controversial. The aim of this study was to examine how afterload reduction observed after percutaneous transverse mitral commissurotomy (PTMC) in patients with mitral stenosis affects these measures of RV contractility. Methods Fifty-eight patients (mean age, 30.0 ± 8.3 years seven men, 52 women) with isolated rheumatic mitral stenosis, eight of whom had atrial fibrillation, were studied prospectively before and 24 to 48 hours after PTMC. Results Immediately after PTMC, mitral valve area increased from 1.0 ± 0.2 to 1.8 ± 0.3 cm 2 ( P = .0001). There was a significant decrease in systolic pulmonary artery pressure from 50.2 ± 26.9 to 33.2 ± 12.3 mm Hg ( P = .0001), a decrease in the RV Tei index from 0.5 ± 0.2 to 0.3 ± 0.2 ( P = .0001), and an increase in RV dP/dt from 321.0 ± 59.9 to 494.6 ± 139.5 mm Hg/sec ( P = .0001). RV myocardial acceleration during isovolumic contraction and systolic velocity at the lateral tricuspid annulus assessed by Doppler tissue imaging did not change. There were weak positive correlations among the Tei index, dP/dt, and systolic pulmonary artery pressure before PTMC (respectively, r = 0.39, r = 0.28, and P = .02, P = .05) but not afterward (respectively, r = 0.17, r = 0.02, and P = .20, P = .90). Conclusions This study suggests that RV dP/dt and Tei index are weakly load dependent, whereas myocardial acceleration during isovolumic contraction is unaffected by acute change in RV afterload.

Background: Patent ductus arteriosus (PDA) is a frequent congenital heart disease. Its transcatheter closure has become the treatment of choice in children and adults. However, the device closure of PDA in children with low weight is still challenging with high rate of complications. The aim of this study was to report further experience with trancatheter closure of PDA using the Amplatzer Duct Occluder(ADO) for children weighing less or equal to 8 kg. Methods: Twenty-two patients (5 male, 17 female) underwent transcatheter closure of a PDA using ADO at a median age of 10 months (range 4 to 26) and a median weight of 7 kg (range 4.3 to 8). Follow-up evaluations were performed with Doppler echocardiography at 24h, and at 6 and 12 months. Results: The device was implanted successfully in all patients. The median fluoroscopy time was 17.25 min (range 10 to 29). Within 24h, color Doppler revealed complete closure in 15 patients (68%), the other patients had a small residual shunt. No deaths were associated with the procedure. Two patients had a slight aortic protrusion of the device without coarctation and in one patient the device encroached partially on the left pulmonary artery without significant acceleration on Doppler echocardiography. All patients were discharged home the next day. All patients completed the 6-month follow-up with complete closure in 18 patients (81%). At the last evaluation in all patients at any time, there has been documentation of complete PDA closure in 20 (91%) of 22 patients. Conclusion: In patients weighing less or equal to 8 kg, percutaneous closure of PDA using an ADO is a safe and effective procedure. (J Interven Cardiol 2012;25:391–394)

Aims Conventional echocardiographic (ECHO) parameters of left ventricular (LV) and right ventricular (RV) systolic and diastolic function have been shown to be load-dependent; however, the impact of preload reduction on tissue Doppler (TD) parameters of LV and RV function is incompletely understood. The aim of this study was to examine the effect of acute preload reduction by haemodialysis (HD) on conventional (ECHO) and TD imaging (TDI) indices of systolic and diastolic function of the left and right ventricles. Methods and results Seventeen chronically uremic patients (age 31 ± 10 years), without overt heart disease underwent conventional 2D and Doppler ECHO together with measurement of longitudinal mitral and tricuspid annular motion velocities. Fluid volume removed by HD was 2706 ± 1047 cm3. Haemodialysis led to reduction in LV end-diastolic volume ( P < 0.0001), end-systolic volume ( P < 0.001), peak early (E wave) transmitral flow velocity ( P = 0.0001), and the ratio of early to late Doppler velocities of diastolic mitral inflow ( P = 0.021). For the LV, early diastolic (E0) TDI velocities and the ratio of early to late TDI diastolic velocities (E0/A0) only on the septal side of the mitral annulus decreased significantly after HD ( P = 0.0001 and P = 0.009, respectively). In a subgroup of seven patients who sustained significantly larger fluid volume loses following HD, E0 and the ratio of E0/A0 at the lateral side of mitral annulus also decreased suggesting a greater resistance of the lateral annulus to preload changes. Systolic velocities decreased after HD on both sides of mitral annulus (septal 6.90 ± 1.10 vs. 5.97 ± 1.48 cm/s, P = 0.006; lateral 8.68 ± 2.67 vs. 6.94 ± 1.52 cm/s, P = 0.011). For the RV, systolic tricuspid annular velocities decreased (13.45 ± 1.47 vs.11.73 ± 1.90 cm/s, P = 0.002) together with early diastolic velocities after HD (13.95 ± 2.90 vs.10.62 ± 2.45 cm/s, P = 0.0001). Both systolic and early diastolic tricuspid annular velocities correlated directly with fluid removal ( P < 0.01). Conclusion This study shows that both systolic and diastolic TDI velocities of the LV and RV are preload-dependent. However, the lateral mitral annulus is more resistant to preload changes than either the septal mitral annulus or the lateral tricuspid annulus.

BACKGROUND Increased QT dispersion (QTd) has been associated with an increased risk for ventricular arrhythmias and sudden death in the general population and in various clinical states. METHODS We investigated the impact of hemodialysis (HD) on QT, QTd, and T-wave amplitude in subjects with end-stage renal failure. Data on 49 patients on chronic HD were studied. The QT, QTd, and the sum of amplitude of T waves (SigmaT) in millimetre in the 12 ECG leads, along with a host of other ECG parameters, body weight, blood pressure, heart rate, electrolytes, and hemoglobin/hematocrit were measured before and immediately after HD. RESULTS QT decreased (380.9 +/- 38.4-363.5 +/- 36.8 ms, P = 0.001), the QTc did not change (406.2 +/- 30.8-405.4 +/- 32.2 ms, P = 0.8), the QTd increased (31.3 +/- 14.6-43.9 +/- 18.6 ms, P = 0.003), and the SigmaT decreased (32.3 +/- 15.7-25.9 +/- 12.6 mm, P = 0.0001) after HD. There was no correlation between the change in QTd and the changes in serum cations, heart rate, the subjects' weight, T-wave duration, and SigmaT. However, the change in QTc correlated inversely with the change in serum Ca(++) (r =-0.339, P = 0.021). CONCLUSION QTd increased, the SigmaT decreased, and the QTc and T-wave duration remained stable, after HD. The QTd increase, although may be real, could also reflect measurement errors stemming from the decrease in the amplitude of T waves (as shown recently), imparted by HD; this requires clarification, to use QTd in patient on HD.

Background Down's syndrome is the most common chromosomal abnormality caused by trisomy of chromosome 21; this anomaly is frequently associated with congenital heart defects (CHDs). Objectives The aim of the study was to identify types, frequency and outcomes of CHD associated with Down syndrome. Methods This retrospective descriptive study was conducted in pediatric cardiology department of the University Hospital IBN ROCHD of Casablanca in Morocco, based on the CHD registry identified between 2008 and 2014. Results 2156 patients with CHD were identified including 128 patients with Trisomy 21(6%). The median age of diagnosis was 9.5 months (Min: 2d -Max: 16 years), the sex ratio was 1. The most common CHD was the atrioventricular septal defect (AVSD) (42.2%), followed by ventricular septal defect VSD (31.3%) then atrial septal defect ASD (28,9%) and patent ductus arteriosus PDA(24,2%). The most common associations of CHD were: AVSD+ASD (10%), VSD+ASD (7,8%), VSD+PDA (7,8%), ASVD+PDA (7%) and ASD+PDA (6%). The indication for surgery was the most common treatment's modality (54.3%) and the total mortality was 20%. Conclusion In our study, the spectrum of congenital heart disease in Down syndrome was similar to the one described in the literature, and also in our Moroccan patients the AVSD was the most described abnormality, the mortality rate in this population remain high therefore we should insist on the early screening of congenital heart disease in this specific population.

Infective endocarditis is a rare complication of hypertrophic cardiomyopathy. It's estimated incidence is 1.4 per 1000 person/year in all patients and it increases to 3.8 per 1000 person/year in patients with left ventricular outflow obstruction. The most common site of vegetation is the ventricular aspect of anterior mitral valve leaflet. We report a case of a 43-year-old man who was admitted for mitral infective endocarditis resulting in severe mitral regurgitation complicating a hypertrophic obstructive cardiomyopathy. The patient underwent mitral valve replacement. Post-operative outcome was good with relieve of symptom and resolution of left ventricular outflow obstruction. Literature data are reviewed.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a clinical entity characterized by myocardial ischemia which becomes symptomatic shortly after birth leading to left heart failure and death. It is rare for an ALCAPA patient to survive to adulthood. In this case, the diagnosis is sometimes missed because of atypical clinical presentation or of the unawareness of doctors. Here, we report a case of an 11-year-old boy with ALCAPA syndrome whose diagnosis took many years to be set-up.

Patent ductus arteriosus (PDA) is a frequent congenital heart disease. Its transcatheter closure has become the treatment of choice in children and adults. However, the device closure of PDA in children with low weight is still challenging with high rate of complications. The aim of this study was to report further experience with trancatheter closure of PDA using the Amplatzer Duct Occluder(ADO) for children weighing less or equal to 8 kg.Twenty-two patients (5 male, 17 female) underwent transcatheter closure of a PDA using ADO at a median age of 10 months (range 4 to 26) and a median weight of 7 kg (range 4.3 to 8). Follow-up evaluations were performed with Doppler echocardiography at 24h, and at 6 and 12 months.The device was implanted successfully in all patients. The median fluoroscopy time was 17.25 min (range 10 to 29). Within 24h, color Doppler revealed complete closure in 15 patients (68%), the other patients had a small residual shunt. No deaths were associated with the procedure. Two patients had a slight aortic protrusion of the device without coarctation and in one patient the device encroached partially on the left pulmonary artery without significant acceleration on Doppler echocardiography. All patients were discharged home the next day. All patients completed the 6-month follow-up with complete closure in 18 patients (81%). At the last evaluation in all patients at any time, there has been documentation of complete PDA closure in 20 (91%) of 22 patients.In patients weighing less or equal to 8 kg, percutaneous closure of PDA using an ADO is a safe and effective procedure.

Aims Conventional echocardiographic parameters of LV and RV systolic and diastolic function have been shown to be load-dependent. The impact of preload reduction on tissue Doppler parameters of LV and RV function is incompletely understood. The aim of this study was to examine the effect of acute preload reduction by haemodialysis on conventional and TDI indices of systolic and diastolic function of the LV and RV. Methods and results 17 uremic patients (age 31+10 years), without overt heart disease underwent conventional 2D and Doppler ECHO together with measurement of longitudinal mitral and tricuspid annular motion velocities. Haemodialysis led to reduction in LV end-diastolic volume (P,0.0001), end-systolic volume (P, 0.001), peak early (E wave) transmitral flow velocity (P 1/4 0.0001), and the ratio of early to late Doppler velocities of diastolic mitral inflow (P 1/4 0.021).For the LV, early diastolic (E0) TDI velocities and the ratio of early to late TDI diastolic velocities (E0/A0) only on the septal side of the mitral annulus decreased significantly after HD (P 1/4 0.0001and P 1/4 0.009, respectively). In a subgroup of 7 patients who sustained significantly larger fluid volume loses following HD, E0 and the ratio of E0/A0 at the lateral side of mitral annulus also decreased suggesting a greater resistance of the lateral annulus to preload changes. Systolic velocities decreased after HD on both sides of mitral annulus (septal P 1/4 0.006; lateral, P 1/4 0.011). For the RV, systolic tricuspid annular velocities decreased (P 1/4 0.002) together with early diastolic velocities after HD (P 1/4 0.0001). Both systolic and early diastolic tricuspid annular velocities correlated directly with fluid removal (P,0.01). Conclusion This study shows that both systolic and diastolic TDI velocities of the LV and RV are preload dependent.The lateral mitral annulus is more resistant to preload changes than either the septal mitral annulus or the lateral tricuspid annulus.

Aims Pulmonary atresia with intact ventricular septum (PA–IVS) is a complex congenital heart malformation with multitude therapeutic approaches. Recently, balloon valvotomy has been used as an alternative to primary surgery. This study aimed to identify echocardiographic markers of balloon dilation success in PA–IVS. Methods and results The echocardiograms of 26 patients diagnosed with PA–IVS who underwent primary pulmonary balloon valvotomy were reviewed. Tricuspid annulus Z -score, pulmonary annulus Z -score, right ventricular (RV) to left ventricular (LV) length ratio, RV to LV transverse diameter ratio, and tricuspid valve (TV) to mitral valve (MV) annulus diameter ratio were measured. The tricuspid Z -score, pulmonary Z -score, RV/LV length ratio, RV/LV diameter ratio, and the TV/MV ratio were significantly different in the group which had successful balloon dilation compared with that failed. Based on decision trees using the Weka classifier package, only RV/LV diameter ratio >0.76 predicts a 92.3% success rate. In contrast, an RV/LV diameter ratio ≤0.76 associated with RV/LV length ratio ≤0.70 predicts 100% failure. Conclusion Successful balloon dilation in membranous type PA–IVS can be predicted by a scoring system using RV/LV diameter ratio and RV/LV length ratio.

Aims Mitral stenosis (MS) affects right ventricular (RV) function as a result of myocardial and haemodynamic factors. Although the long-term effects of mitral commissurotomy are well known, the aim of this study was to evaluate the immediate impact of percutaneous mitral commissurotomy (PTMC) on RV function in patients with MS. Methods and results Twelve female patients (mean age 29 ± 7 years) with isolated rheumatic MS, all in sinus rhythm, were studied before and 24–48 h after PTMC. Multiple parameters of global and longitudinal RV function were assessed by conventional and tissue Doppler imaging echocardiography. Immediately following PTMC, mitral valve area increased from 0.91 ± 0.29 cm2 to 1.86 ± 0.43 cm2 ( P < 0.0001) and RV outflow tract fractional shortening (RVOTfs) increased from 57 ± 15% to 72 ± 12% ( P = 0.002). There was a significant decrease in systolic pulmonary artery pressure from 46.4 ± 32.1 mmHg to 29.1 ± 13.4 mmHg ( P = 0.02), in the RV Tei index from 0.44 ± 0.025 to 0.29 ± 0.17 ( P = 0.021), in myocardial acceleration during isovolumic contraction (IVA) at the lateral tricuspid annulus from 0.36 ± 0.11 m/s[2][1] to 0.25 ± 0.07 m/s[2][1] ( P = 0.023), and in isovolumic contraction velocities at the lateral tricuspid annulus from 11.03 ± 3.37 cm/s to 8.50 ± 2.04 cm/s ( P = 0.034). In contrast, tissue Doppler velocities at the septal tricuspid annulus remained unchanged. The RV Tei index correlated with systolic pulmonary artery pressure before but not after PTMC ( r = 0.70, P = 0.01, and r = 0.270, P = 0.053). Conclusion Immediately after successful PTMC, significant decrease in RV contractility as assessed by IVA was observed whereas other parameters of infundibular and global RV function as assessed by RVOTfs and Tei index showed significant improvement. These discordant results may be related to the relative insensitivity of currently available echocardiography parameters of RV function that are not completely immune to loading conditions. Further work using larger numbers of patients is needed to confirm our findings and to assess their utility in patient follow-up and management. [1]: #ref-2

Background: Hemodialysis (HD) leads to an augmentation in the amplitude of QRS complexes (QRS-c), and R waves (R-w); some correlates of this phenomenon have been identified, but the exact mechanism for these ECG changes remains elusive. The objective of this study is to search for the underlying mechanism(s) of the post-HD augmentation of QRS-c and R-w. Methods: The sum of the amplitudes of ECG QRS-c and R-w, along with a host of other parameters (body weight, fluid volumes, echocardiographically-derived left ventricular dimensions and volumes, serum potassium, hemoglobin, hematocrit, and others) was measured, before and after HD, in 17 patients with end-stage renal failure. Results: While there were many correlations noted between the changes in the QRS-c and R-w and some of the above variables in numerous univariate analyses carried out, multivariate analyses did not identify any of the examined variables as exerting an independent influence on the observed ECG changes after HD. Conclusion: Augmentation of QRS-c and R-w following HD is engendered by an interplay of a decrease in the LVEDD/LVEDV, and K+, loss of fluid volume, and a rise in Hb and Ht, without any of the above being an independent variable; also other factor(s) (e.g., increase in the body electrical impedance) exerting an influence in this ECG phenomenon cannot be excluded.

Introduction The value of routine use of transesophageal echocardiography (TOE) in cardiac surgery has been rarely studied in a consecutive series of patients. The aim of this study was to evaluate the contribution of perioperatively TOE in cardiac surgery. Material and methods This is a prospective study of thirty two consecutive patients undergoing cardiac surgery, in a programmed manner. The data of TOE were compared with preoperative diagnosis retained. In some cases, changing diagnostic led to a modification of the planned intervention. The contributions of TOE were to elucidate of the mechanism of valvular disease, assess its severity, to diagnose morphological, guiding surgery and checking results at the end of the intervention Results The main types of interventions were: valve replacement or valvuloplasty (51%), CABG (37%), aortic surgery (12%). The diagnosis made preoperatively was modified in 10% of cases (n=21). In 54% of cases, TOE confirmed the diagnosis previously established by transthoracic echocardiography, in 10% of cases the diagnosis made preoperatively was changed, and in 28% of TOE has provided diagnostic and therapeutic solution. The results found for each type of surgical pathology are reported in Table Coronary artery disease Pathology of the mitral valve Pathology of the tricuspid valve Pathology of the aorta Change in diagnosis 3% 20% 11% 16% Change in the intervention planned 1% 13% 9% 16% Discussion TOE performed routinely during cardiac surgery may lead to changes in surgery, particularly in the aortic pathology and valvular diseases. In about half of the indications for which review is requested, the supply of TOE is decisive and modifies the treatment instituted. Conclusion Our study confirms the value of TOE in cardiac surgery, in addition to the indications found in our series of patients, others such as aortic dissection or pulmonary embolism major benefit from the addition of TOE.

To evaluate the impact of long term permanent hypoxemia noticed in patients with non operated congenital cyanogenic cyanotic cardiopathy on liver stiffness.We included ten adult patients with non operated inoperate cyanotic cardiopathy and ten matched patients for age and gender admitted to the gastroenterology department for proctologic diseases; Clinical and laboratory data were collected [age, gender, body mass index, oxygen saturation, glutamate oxaloacetate transaminase (GOT), glutamate pyruvate transaminase (GPT), glycemia and cholesterol]. Measurement of hepatic stiffness by transient elastography was carried out in all patients using the Fibroscan device. All patients underwent an echocardiography to eliminate congestive heart failure.Among the patients with cyanotic cardiopathy, median liver stiffness 5.9 ± 1.3 kPa was greater than control group (4.7 ± 0.4 kPa) (P = 0.008). Median levels of GOT, GPT, gamma-glutamyltransferase, glycemia and cholesterol were comparable in cardiopathy and control group. In regression analysis including age, gender, body mass index, oxygen saturation, GOT, GPT, glycemia, cholesterol showed that only oxygen saturation was related to liver stiffness (r = -0.63 P = 0.002).Chronic permanent hypoxemia can induce mild increase of liver stiffness, but further studies are needed to explore the histological aspects of liver injury induced by chronic permanent hypoxemia.