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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.

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.

Background Subpulmonic membrane as a cause of right ventricular outflow tract obstruction in patients with concordant ventriculoarterial connection and intact ventricular septum is considered to be rare. Association with cor triatriatum dexter and success of subpulmonic balloon valvuloplasty have never been reported, at least to the best of our knowledge. Case presentation A 3-year-old Moroccan boy was referred to our tertiary care hospital with complaints of dyspnea on moderate exertion. A physical examination revealed parasternal lift, systolic thrill, and a 4/6 ejection systolic murmur, best heard over the left second intercostal space. His oxygen saturation was 99% on room air. Two-dimensional echocardiography showed a discrete circumferential membrane just below the pulmonic valve and a right atrial membrane. Continuous wave Doppler interrogation showed peak systolic pressure gradient of 85 mmHg across the subpulmonic membrane and no significant gradient across the right atrial membrane. Balloon dilation of the subpulmonic membrane was performed and the pressure gradient came down to 50 mmHg. During follow-up, he reported marked improvement in terms of exercise tolerance. Transthoracic echocardiography showed residual pressure gradient of approximately 40 mmHg across the membrane. Surgery resection of the two membranes was programmed, but he died after an extracardiac disease (appendicular peritonitis). Conclusions Subpulmonic membrane as an isolated cause of right ventricular outflow tract obstruction is rare. Its association with cor triatriatum dexter is even less common. The result of percutaneous balloon valvuloplasty of subpulmonic membrane is an interesting alternative while waiting for surgery. Surgery is currently the preferred modality of treatment with the resection of both right atrial and subpulmonic membranes.

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.

Background: Isolated cleft mitral valve (ICMV) may occur alone or in association with other congenital heart lesions. The aim of this study was to describe the profile of cardiac lesions associated with ICMV and their potential impact on therapeutic management. Methods: We conducted a descriptive study with data retrieved from the Congenital Heart Disease (CHD) single-center registry of our institution, including patients with ICMV registered between December 2008 and November 2014. Results: Among 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had Down syndrome. Seventeen patients (77.3%) had associated lesions, including 11 (64.7%) with accessory chordae in the left ventricular outflow tract (LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD), three had secundum atrial septal defect, and four had patent ductus arteriosus. Thirteen patients (59.1%) required surgical repair. The decision to proceed with surgery was mainly based on the severity of the associated lesion in eight patients (61.5%) and on the severity of the mitral regurgitation in four patients (30.8%). In one patient, surgery was decided based on the severity of both the associated lesion and mitral regurgitation. Conclusion: Our study shows that ICMV is rare and strongly associated with Down syndrome. The most common associated cardiac abnormalities were VSD and accessory chordae in the LVOT. We conclude that cardiac lesions associated with ICMV are of major interest, since in this study patients with cardiac lesions were diagnosed earlier. The decision to operate on these patients must take into account the severity of both mitral regurgitation and associated cardiac lesions. Resumo Fundamento: A fissura isolada da valva mitral (FIVM) pode ocorrer isoladamente ou em associação com outras lesões cardíacas congênitas. O objetivo deste estudo foi descrever o perfil das lesões cardíacas associadas à FIVM e o potencial impacto dessas lesões na terapêutica. Métodos: Realizamos um estudo descritivo com dados obtidos do registro unicêntrico Congenital Heart Disease (CHD) de nossa instituição incluindo pacientes com FIVM registrados entre dezembro de 2008 e novembro de 2014. Resultados: Entre 2177 pacientes identificados no registro CHD, 22 (1%) apresentavam FIVM. A mediana de idade ao diagnóstico foi de 5 anos (6 dias a 36 anos). Nove pacientes (40,9%) apresentavam síndrome de Down. Dezessete pacientes (77,3%) apresentavam lesões associadas, incluindo 11 (64,7%) com cordoalha acessória na via de saída do ventrículo esquerdo (VSVE) sem obstrução, 15 (88,2%) com comunicação interventricular (CIV), três com comunicação interatrial do tipo ostium secundum e quatro com persistência do canal arterial. Treze pacientes (59,1%) necessitaram reparo cirúrgico. A decisão de prosseguir com a cirurgia foi baseada principalmente na gravidade da lesão associada em oito pacientes (61,5%) e na gravidade da regurgitação mitral em quatro pacientes (30,8%). Em um paciente, a decisão por cirurgia foi baseada na gravidade tanto da lesão associada quanto da regurgitação mitral. Conclusão: Nosso estudo mostra que a FIVM é rara e está fortemente associada à síndrome de Down. As anormalidades cardíacas associadas mais comuns foram a CIV e cordoalha acessória na VSVE. Concluímos que as lesões cardíacas associadas à FIVM são de grande interesse, já que neste estudo, pacientes com lesões associadas foram diagnosticados mais precocemente. A decisão cirúrgica deve levar em conta a gravidade tanto da regurgitação mitral quanto das lesões cardíacas associadas.

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.

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.

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.

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 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.

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.