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What is it about? Tricuspid regurgitation is characterised by regurgitation from the ventricle into the right atrium. It may lead to dilatation and subsequently failure of the right-sided chambers of the heart (the right atrium and ventricle). The causes may be functional (common) or organic (rare). In the former case, regurgitation is secondary to incomplete leaflet coaptation because the tricuspid annulus is dilated as a result of increased pressure caused by a downstream obstruction (in the pulmonary circulation, at the mitral valve, etc.). This may occur in the context of pulmonary hypertension, mitral-aortic valve disease, ischaemic heart disease, cor pulmonale (chronic respiratory conditions increasing resistance to right ventricular ejection into the pulmonary vessels), or acute or chronic pulmonary embolism. Organic causes of tricuspid valve damage are rarer; these include certain congenital malformations, bacterial endocarditis, acute rheumatic fever, chest trauma, right ventricular infarction and carcinoid syndrome. Symptoms The patient generally presents with signs of right-sided heart failure: oedema of the lower limbs, hepatomegaly, distended jugular veins or, in cases of global heart failure, dyspnoea or fatigue. Treatment Treatment is initially medical. Surgery is indicated in cases of functional tricuspid regurgitation associated with mitral valve disease, tricuspid valve infection that does not respond to antibiotics, or symptomatic severe tricuspid regurgitation. Usually, tricuspid valve repair by annuloplasty is performed; more rarely, the valve needs to be replaced.

What is it about? Ascending aorta The first description was published in 1960 by Ellis et al., who associated disease of the aortic valve and ascending aorta (annuloaortic ectasia). The natural history of this disease usually leads to aortic regurgitation, but also to an aneurysm of the ascending thoracic aorta, the main complication of which is dissection. Marfan syndrome is one of the leading causes of annuloaortic ectasia. Surgery is indicated when aortic valve regurgitation is moderate to severe, with left ventricular involvement and/or dilatation of the ascending aorta to at least 45 mm in diameter. Surgical treatment is required because the natural course is rupture, with 100% mortality. Surgery is performed with cardiopulmonary bypass and consists of replacing the aortic valve and ascending aorta with a mechanical valve combined with a straight vascular graft, with reimplantation of the ostia of the left and right coronary arteries into the tubular prosthesis (the Bentall and Bono technique, described in 1968). There are other surgical techniques that are variants of the Bentall and Bono procedure, such as the Cabrol technique, which is now rarely used. There are also the David and Yacoub techniques, which have the advantage of preserving the native aortic valve if it is intact. The mortality associated with this surgery is close to that of aortic valve replacement surgery, i.e. less than 2%, except in an emergency situation without comorbidities, in which it is less than 10%. Descending Aneurysms Dissection

What is it about? Mitral stenosis The main cause of narrowing (less than 2 cm²) of the mitral valve is rheumatic heart disease, which occurs in patients who have had acute rheumatic fever. This disease has now become rare in Northern Europe. Other exceptional causes include lupus erythematosus, rheumatoid arthritis, radiotherapy, etc. Clinical manifestations Atrial fibrillation. Exertional or positional dyspnoea reflects an increase in left atrial pressure during exertion or when moving into a lying position. Pulmonary oedema. Cerebrovascular thromboembolic accident (CVA), resulting from the expulsion of a clot formed in the left atrium, most often associated with atrial fibrillation. Treatment If the patient initially needs to be treated with medication, valve repair (less frequently possible than in mitral regurgitation, see below) or valve replacement—either with a biological or a mechanical valve—is generally required as soon as symptoms occur.

What is it about? Patent ductus arteriosus is a congenital condition present at birth. It is characterised by the presence and persistence of a duct between the pulmonary artery and the aorta. It is very common in premature infants (80% of children born before the 28th week of gestation). Symptoms Primarily shortness of breath, breathing difficulties and possible episodes of apnea, as well as a decrease in heart rate. The diagnosis The electrocardiogram shows signs of left ventricular overload. The chest X-ray shows signs of left ventricular hypertrophy, which is the principal finding, and cardiomegaly. Echocardiography readily demonstrates the patent ductus arteriosus, measures pulmonary and aortic blood flow, and assesses the degree of arterial hypertension. Treatment Surgical intervention is mandatory because the risk of infective endocarditis and heart failure is significant. This duct must be closed.

What is this about? Fallot tetralogy is a complex heart defect characterised by cyanosis, meaning that patients have a bluish complexion due to inadequate oxygenation of the blood. In Fallot tetralogy, the heart is “not fully formed”. The pulmonary artery is underdeveloped and stenosed, and the ventricular septum is incomplete and absent beneath the roots of the aorta and pulmonary artery. In practice, blood enters the right ventricle. As it contracts, the right ventricle sends venous blood both into the pulmonary artery (but only in a small amount, given the resistance caused by the stenosis) and into the aorta. Arterial blood is therefore a mixture of non-oxygenated venous blood and red arterial blood, which accounts for the patient’s “bluish” appearance. Treatment It is surgical. Completely reparative surgery is performed as early as possible. This surgery consists of closing the ventricular septal defect and enlarging the pulmonary infundibulum and pulmonary arteries.

Presentation The Erasme-HUDERF Neuromuscular Reference Centre provides the services of an expert multidisciplinary team to offer integrated care to anyone with a neuromuscular disease. Neuromuscular diseases are conditions caused by dysfunction of the motor unit (consisting of the muscle, the neuromuscular junction and the nerve), which prevents the muscle from contracting normally. These diseases affect both children and adults. The age at which the disease appears, the initial symptoms and the severity of the condition vary depending on the disease. Motor, respiratory, cardiac and digestive functions may be affected to varying degrees and may require multidisciplinary care and follow-up.To request an appointment: %20Cons [dot] Neuromusculaire [dot] erasme [at] hubruxelles [dot] be (Cons[dot]Neuromusculaire[dot]erasme[at]hubruxelles[dot]be) or by telephone: +32 2 555 33 60. Image Image There are six groups of neuromuscular diseases:Myopathies (Steinert disease, dystrophies, myositis, myotonia, periodic paralysis, glycogen storage diseases, mitochondrial diseases, etc.).Diseases of the neuromuscular junction (myasthenia gravis and Lambert-Eaton syndrome).Motor neuron diseases (amyotrophic lateral sclerosis or “Charcot disease”, post-polio syndrome, spinal muscular atrophies, etc.).Neuropathies = polyneuritis = polyneuropathies (inflammatory, Guillain-Barré syndrome, CIDP/PIDC, Charcot-Marie-Tooth disease, diabetes-related, etc.).Degenerative syndromic conditions (often hereditary) (hereditary spastic paraplegias/Strümpell-Lorrain disease, Friedreich’s ataxia and spinocerebellar ataxias/SCA, etc.).Plexus disorders (cervicobrachial, lumbosacral).The care services cover all stages of the disease, from the most precise diagnosis through to treatment. The team’s work is primarily focused on optimising care, advising and guiding patients, their relatives and their care team, in close consultation and collaboration with local practitioners who are also involved in the patient’s care. The team’s main aim is to improve quality of life, slow disease progression, prevent and limit complications and, ultimately, improve the long-term prognosis. Structure and functioning The Erasme-HUDERF Neuromuscular Reference Centre (CRNM) brings together a multidisciplinary medical and paramedical team with expertise in neuromuscular diseases. Its activities are coordinated by the coordinating physicians, with the support of coordinating nurses and the secretariat. The core medical team, made up of specialists in neurology, paediatric neurology, physical medicine and genetics, works in conjunction with named referral physicians (for paediatric and adult patients) from various specialties (anaesthesiology, cardiology, dermatology, endocrinology, gastroenterology, gynaecology, internal medicine, emergency medicine, obstetrics, ophthalmology, orthopaedics, paediatrics, pneumology, psychiatry, stomatology and urology). This medical team’s expertise enables diagnosis and ensures medical follow-up and treatment during the multidisciplinary neuromuscular consultations held several times a month.All medical and technical examinations (for example, electromyography, neuromuscular biopsy, DNA analysis and cardiopulmonary assessment) are carried out within the Erasme-HUDERF Neuromuscular Reference Centre. Certain highly specialised laboratory analyses are performed in collaboration mainly with leading laboratories in Belgium and Europe. Neuromuscular biopsies are performed in close collaboration with the neuropathology laboratory of Brugmann University Hospital. The rehabilitation team (present at both the Erasme and HUDERF sites) consists of paramedical specialists representing the following professions: coordinating nurse, occupational therapist, physiotherapist, psychologist, social worker, speech therapist and dietitian.Patients’ files are systematically discussed during multidisciplinary team meetings, which makes it possible to shorten and refine the diagnostic process and accelerate care. A personalised and coordinated programme of medical and rehabilitation care is systematically proposed to the patient, their relatives and their home care team. There is close collaboration between the CRNM (Erasme site) and the rehabilitation centre located on the same site, which has both inpatient and outpatient facilities where neuromuscular patients are regularly treated during the acute phase and/or over the long term. Care management ExaminationsCardiac assessmentThe cardiac assessment as part of the Neuromuscular Reference Centre (CRNM) comprises five examinations: electrocardiogram, echocardiogram, Holter ECG, bone densitometry, and muscle and/or nerve biopsy.Electromyography (EMG)This examination involves inserting a fine needle into the muscle being studied in order to record the electrical activity produced by the muscle fibres when the muscles are activated or when they are at rest and fully relaxed.Cardiopulmonary exercise testingCardiopulmonary exercise testing is used to assess your exercise capacity (cycling). The examination is performed on a bicycle. Measurements are taken throughout the exercise to assess your respiratory system and your heart.Nerve conduction velocity testingNerve conduction velocity testing determines the speed at which electrical signals travel along a particular peripheral nerve. It is used to diagnose a nerve injury or dysfunction.Evoked potentialsThis technique involves stimulating the sensory organs to assess the function of specialised nerve structures involved in transmitting information related to touch, hearing and vision, using electrical stimuli, headphones and a screen, respectively.Repetitive stimulationRepetitive nerve stimulation (RNS) is a neurophysiological tool used to reveal certain neuromuscular transmission and muscle conduction deficits.Ischaemic forearm exercise testThe purpose of this test is to detect dysfunctions in muscle metabolism, particularly the muscle’s ability to use energy resources. After a cuff has been inflated around the upper arm and a catheter (a small plastic tube) has been placed in a vein in the forearm, the patient is asked to repeatedly clench their fist against resistance for several minutes.Treatments MedicationIn the context of neuromuscular diseases, drug treatments are numerous and vary considerably depending on the diagnosis made. It is therefore impossible to detail all the possible options here for the many diseases grouped under the term “neuromuscular diseases”. Each treatment will be adapted with your doctor to your condition and to your individual circumstances (medical history, age, etc.).Non-pharmacological treatmentsA significant proportion of patients with a neuromuscular disease experience limitations in their activities of daily living, and the disease often affects various areas of life: professional, family, social, psychological and economic consequences, etc. In this context, the centre’s team offers you integrated, continuous multidisciplinary follow-up over time, in order to identify the difficulties related to the disease as effectively as possible and to try to provide the most appropriate responses according to each patient’s specific needs. Regular physiotherapy is, in most situations, an essential treatment to recover and/or maintain the best possible motor function and functional independence in the medium and long term. A range of measures may be implemented to facilitate daily functioning when necessary: adaptations to the home, various forms of home assistance, different allowances, a reduced-mobility parking permit, etc.  Useful documents and brochures Brochure of the Neuromuscular Reference Centre External request – nerve biopsy External request – muscle biopsy Our specialists Our associated services In 2024, the H.U.B’s CRNM celebrates its 10th anniversary. On this occasion, the CRNM is organising a symposium on 23 November 2024 at the Museum of Medicine. Discover the programme

In Belgium, fewer than 1,000 cases per year of the active form, which is contagious because it is pulmonary in 75% of cases, are diagnosed and monitored. These forms are most often confirmed bacteriologically following an appropriate clinical assessment carried out in close collaboration with the laboratory. Isolating the organism makes it possible to assess the sensitivity of the Koch bacillus to the various antituberculosis drugs. Patients can often return home quickly, avoiding hospital isolation, which is difficult to organise at a time when there is a shortage of beds related to Covid; however, treatment is lengthy and must be adapted to any resistance (fortunately rare in Belgium). It often needs to be supervised, as perfect adherence is essential to ensure its efficacy and, consequently, patients’ recovery. Toxicities must also be monitored. Fares (French-speaking) and VRGT (Dutch-speaking) carry out this task through the BeltaTBnet programme while keeping the Belgian register up to date. Erasme Hospital is particularly involved in the operation of these non-profit organisations. Work towards eradication In our country, where the eradication of the disease is being targeted in the medium term in accordance with the WHO, it is also important to detect latent forms and treat them so that they do not become reactivated. The aim is to halt the disease cycle. The interpretation of in vivo tests (tuberculin skin test) is complex because of their lack of specificity, while the sensitivity of in vitro assays following stimulation (QuantiFERON), which are now advocated by many, is insufficient.The people most at risk of developing active tuberculosis from a “dormant” form are immunocompromised individuals and those with recent exposure (conversion of screening tests).The pneumology department provides care for these individuals, particularly in preparation for immunotherapy (especially biological therapy, for which screening for a latent form is mandatory), in the presence of a disease affecting the body’s defences, or following contact in the workplace (employees referred by the occupational health service).There are frequent and excellent clinical interactions with the internal medicine department (AIDS reference centre and infectious diseases clinic). Other infectious lung diseases Pulmonary infections caused by atypical mycobacteria (induced by these less virulent environmental organisms) are also part of the specialist expertise provided by the pulmonology department.They are rarer but very often occur in the context of pre-existing and predisposing pulmonary conditions (COPD, mucoviscidosis, etc.).Detecting the organism does not make it a pathogen; it is the progression of the lesions attributed to it that determines whether or not to initiate a long and burdensome treatment. Each case must therefore be considered through targeted multidisciplinary discussion.The disappearance of the mycobacterium does not establish a cure, which is rather a stabilisation. Follow-up is therefore ongoing.

What is it about? Heart failure occurs when the heart’s pumping function is reduced below normal. This results in an insufficient blood supply to other organs, such as the liver and kidneys.The heart failure you are experiencing is sometimes of unknown origin. It is most often the consequence of coronary artery disease; your medical history then includes one or more myocardial infarctions, and possibly coronary artery bypass surgery.It may follow heart valve disease, in which case you have probably already undergone replacement of one or more valves. It may also be caused by a viral infection, drug toxicity (chemotherapy), dietary toxicity (alcoholism), or even pregnancy (very rare!).The course of your heart disease invariably and irreversibly leads to cardiac dilatation. Your heart is no longer able to ensure adequate perfusion of the organs, causing symptoms and dysfunction of other organs despite appropriate drug treatment. What are the causes? Coronary artery disease (= ischaemic cardiomyopathies, sequelae of myocardial infarction, etc.), diabetes, valvular heart disease, high blood pressure, pulmonary embolism, and myocarditis caused by a viral infection or toxins. Diagnosis A number of symptoms are associated with heart failure. Shortness of breath, which may occur at rest or during exertion, is undoubtedly one of the symptoms characteristic of heart failure. Other symptoms include rapid fatigue and fluid retention (oedema). Other symptoms, such as tachycardia, weight gain over a few days and reduced urine output, are observed in heart failure.The diagnosis is readily suspected based on the symptoms and confirmed by cardiac ultrasound. The cause of the decompensation must then be sought among the diseases mentioned above. Treatment Several types of medication are useful in the treatment of heart failure. These medications aim to improve the heart’s pumping function, reduce the signs and symptoms of heart failure, or reduce stress on the heart. Currently, medications known as “angiotensin-converting enzyme inhibitors” (e.g. Capoten, Zestril, Coversyl, Tritace, etc.) can be considered the cornerstone of treatment. In second place are beta-blockers (e.g. Kredex, Isoten, etc.), which, by reducing the heart’s energy requirements and oxygen consumption, are also a basic treatment for decompensated heart failure.It should not be forgotten that certain simple dietary measures (a salt-free diet and fluid restriction) are easy-to-implement treatments for reducing the signs of heart failure.

What is it about? An atrial septal defect is a “hole” in the interatrial septum, thereby creating a connection between the left atrium and the right atrium. It is a common congenital malformation (6–10% of congenital heart diseases; 1 in 1,500 births). As a result of this atrial septal defect, oxygenated blood mixes with “deoxygenated” blood. This leads to an increased workload for the right atrium, the right ventricle and the lungs. Symptoms Shortness of breath or fatigue during exertion, palpitations... People with an atrial septal defect are more prone to cerebrovascular accidents. Treatment It depends on their size, location, and effects on the heart and lungs. Very often, the atrial septal defect will be closed surgically. It is possible to close them percutaneously (that is, without surgically opening the chest, using cardiac catheterisation techniques).

What is it about? A ventricular septal defect is an opening between the left and right ventricles. It is a common malformation. This ventricular septal defect allows oxygenated blood to mix with “deoxygenated” blood. The result is an increased workload for the ventricles and therefore heart failure. Symptoms Shortness of breath or fatigue during exertion, palpitations... (symptoms of heart failure). Affected infants are more prone to respiratory infections. Treatments The ventricular septal defect will be closed surgically. Diuretics, digitalis, sodium restriction, and treatment of respiratory infections in the meantime if the situation is stable.

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What is pulsatile tinnitus? Pulsatile tinnitus is the perception of a rhythmic noise, often described as a beating or whooshing sound, that is synchronised with the patient’s pulse. Unlike conventional tinnitus, which is generally constant and non-rhythmic, pulsatile tinnitus is often a sign of a vascular abnormality or a blood circulation problem near the ear. SymptomsSymptoms of pulsatile tinnitus include:A beating or pulsating sound in one or both ears.A sensation of sound that may increase with exercise or when the head is tilted, or decrease with gentle compression of certain parts of the neck.Sometimes accompanied by hearing loss, dizziness or ear pain. Care Imaging examinationsTo identify the possible causes of pulsatile tinnitus, several imaging examinations are used:Brain MRI and magnetic resonance angiography (MRA): to visualise the blood vessels in the brain and neck and detect masses, vascular malformations or other structural abnormalities.Computed tomography angiography (CT angiography) and temporal bone CT scan: to assess vascular abnormalities.Doppler ultrasound: to examine blood flow in the cervical and intracranial vessels.Conventional angiography: sometimes necessary for a more detailed examination of the blood vessels.Medical care pathwayPatients suffering from pulsatile tinnitus should consult several specialists, including:ENT specialist (otorhinolaryngologist): initial consultation to assess the symptoms and perform the first examinations.Radiologist: to perform and interpret the imaging examinations.Neurologist: if intracranial vascular abnormalities are suspected.Cardiologist: if a cardiovascular cause is suspected.Interventional neuroradiologist: for cases requiring an endovascular procedure, particularly venous sinus stenting.TreatmentsTreatment options for pulsatile tinnitus depend on the underlying cause:Medical treatment: for causes related to hypertension or metabolic abnormalities.Surgical or endovascular intervention: for arteriovenous malformations, glomus tumours or dural fistulas.Endovascular embolisation: to treat certain types of vascular malformations, particularly using a stent.Auditory therapies: such as hearing aids or white-noise generators to reduce the perception of tinnitus.Behavioural therapy: to help patients manage the stress and anxiety associated with pulsatile tinnitus.The level of evidence for these treatments varies, with surgical and endovascular interventions often based on case reports and clinical series, whereas medical and behavioural treatments have more robust evidence for symptom management.Multidisciplinary discussionThe management of pulsatile tinnitus often requires a multidisciplinary approach, involving regular discussions between ENT specialists, radiologists, interventional neuroradiologists, neurologists, cardiologists and neurosurgeons to determine the exact cause and choose the appropriate treatment.Follow-upFollow-up of patients with pulsatile tinnitus is crucial in order to:Monitor treatment effectiveness: particularly after surgical or endovascular intervention.Manage residual symptoms: with ongoing auditory or behavioural therapies.Control risk factors: such as hypertension or other underlying medical conditions.Follow-up is provided by a multidisciplinary team to ensure comprehensive care and improve patients’ quality of life.  Our specialists Make an appointment Associated services