Myocardiopathies are diseases where the myocardium is weak, dilated or has some other structural problem. Often, the heart is unable to pump or work properly. In the case of inherited myocardiopathies, due to the fact they are often treated as part of uncommon diseases, specialist management is not within the scope of all clinical cardiologists. It requires specific treatment and involves specific technology.

We have an outpatient clinic connected to a day hospital and conventional hospitalisation, with support from the Imaging, Haemodynamics, Electrophysiology and Arrhythmias Units.

This Unit is also responsible for teaching and research. It is connected to the Genetics Unit with a unit for clinical research and a unit for basic research.

The main healthcare objectives of our Unit are:

• Excellence in care for index patients and family members with hereditary and/or genetic myocardial diseases.
• The creation of a protocol for medical action in these diseases.
• Homogenisation of care.
• The optimisation of care resources, reducing the overall cost of treatment.
• The provision of technology required for patients.
• The performance of genetic studies on patients (control index) and relatives.
• Genetic counselling for families and individuals, which predict the likelihood of passing the illness on to children.
• We provide consultations for other doctors, both at our hospital and other hospitals and primary care centres.
• Creation of fast-stream pathways for referral or interconsultations for cases where the patient must be referred to another professional.
• Reduction of barriers that often limit direct contact between patients and healthcare staff, as well as communication between staff and other institutions.

The Cardiovascular Critical Care Unit (initially known as the Coronary Care Unit) was created in 1971 to admit patients with acute myocardial infarction, or other acute cardiovascular diseases. It also dealt with cardiac surgery post-operative care. In 1989 the unit was divided into two. The current Cardiovascular Critical Care Unit became responsible for the patients and became a part of the Cardiology Department.

The unit has 10 hospital beds for critical patients and 4 for semi-critical patients.Around 1,000 patients are admitted each year. Out of these admissions, two-thirds are due to an acute myocardial infarction. The remaining admissions are the result of other acute cardiovascular diseases, such as:

• Heart failure
• Non-ischaemic cardiogenic shock
• Cardiopulmonary arrest and other serious tachyarrhythmias and bradyarrhythmias
• Acute aortic syndrome 
• Cardiac tamponade 
• Admissions after complicated structural or electrophysiological interventional procedures 

The unit also provides 24 hour support for cardiac emergencies via Vall d’Hebron Accident and Emergency Services. It also cares for outpatients.

The Cardiovascular Critical Care Unit offers the following advanced equipment and procedures: 

• Continuous electrocardiogram and haemodynamic monitoring
• Electrocatheter implants 
• Transthoracic and transoesophageal echocardiograms 
• Pericardiocentesis 
• Non-invasive and invasive ventilator support 
• Circulatory support with a balloon pump 
• Impella and ECMO veno-arterial device 
• Therapeutic hypothermia 
• Dialysis systems

Main diseases related to this speciality

Acute myocardial infarction and other acute heart diseases
This is the main cause for admission to the Cardiovascular Critical Care Unit. An acute myocardial infarction occurs when cholesterol plaques build up in the coronary arteries. These take the blood to the myocardium, the heart’s muscle tissue that is responsible for pumping the blood around the blood stream. When the blood flow in the coronary arteries decreases, a myocardial ischaemia occurs. It usually causes a pain similar to pressure on the chest, known as chest angina. When the coronary artery is completely obstructed, an acute myocardial infarction occurs which causes heart cells to die. 

It is a serious illness and requires the artery to be opened immediately. This is usually done with a catheterisation. The patient is then admitted to the Cardiovascular Critical Care Unit. Their vital signs are monitored and any drugs needed are administered.Imaging tests and invasive procedures are done, if necessary. Possible complications, such as arrhythmias, cardiac arrest or repeat ischaemic episodes, are also treated.

Acute, or chronic flare-up, heart failure and cardiogenic shock
Heart failure is the heart’s inability to supply the blood properly to the rest of the body.The most frequent causes are ischaemic and myocardial heart disease. The main symptoms are difficulty in breathing and congestion of the entire body.
The most extreme level of heart failure is cardiogenic shock.This occurs when the heart has almost no capacity to pump blood and impacts on other organs such as the kidneys, liver, lungs, intestine or the brain. The treatment consists of coronary revascularisation, which is surgery to recover blood flow in the artery. Drugs and mechanical circulatory support systems are also used. A heart transplant is the last resort.

Cardiopulmonary arrest
Ventricular arrhythmias are a heartbeat disorder coming from the ventricles, which are the heart’s lower cavities. They can cause cardiopulmonary arrest if they occur in the context of an acute myocardial infarction or any other heart disease. Breathing and the heartbeat suddenly stop. They may cause the person's death if they are not immediately reanimated. Reanimation is done with heart massage, defibrillating with an electric shock and assisted breathing. 

The most feared outcome after reanimation is brain damage. This may occur due to the lack of blood supply during the arrest. These patients are admitted to the Cardiovascular Critical Care Unit where they get brain support and protection while they recover.

Ventricular tachycardia and arrhythmic storm
Another reason for admissions is sustained or repeated ventricular arrhythmias. They are known as arrhythmic storms and can have serious consequences.They occur in patients affected by an infarction or other advanced structural heart disease. Patients with arrhythmias caused by hereditary heart disease also suffer from them.

Anti-arrhythmia treatment and ablation of the tachycardias, or fitting a defibrillator, are needed to stabilise the patient. In the most serious cases assisted breathing and circulation support devices may be necessary. 

Atrioventricular block
This consists of the loss of electrical connection between the heart’s atria and ventricles. It frequently occurs in the elderly due to degeneration. It causes a very slow heartbeat. It can turn into fainting (syncope), a sudden, temporary loss of consciousness or heart failure. It is treated by fitting a pacemaker. 

Acute aortic syndrome
The aorta is the body’s main artery. It may split, suffer internal bleeding or an ulcer on the walls. This can lead to very serious consequences, such as a rupture to the artery or a lack of blood flow to any organ. It frequently requires emergency surgical treatment.

Complications in congenital heart disease in adults and complications during pregnancy and postpartum in patients with congenital heart disease or other heart disease
The Cardiology Department is a benchmark for congenital heart disease in adults. The Cardiovascular Critical Care Unit cares for complications arising from the disease. For example, those occurring during pregnancy or postpartum. The unit provides complex surgical procedures for these cases. 

Research

The Cardiovascular Critical Care Unit has a long tradition of research. It researches the fields of thrombosis, prognostic factors and complications in acute myocardial infarction, heart failure and cardiopulmonary arrest. It also studies nursing care for cardiovascular disease. 

Its members have their own lines of research and participate in several research networks. The results of the research are regularly presented at cardiology conferences. They are also published in Spanish and international scientific journals. Several members of the unit belong to Catalan, Spanish and European scientific associations focusing on acute cardiovascular diseases.

Education 

The Cardiovascular Critical Care Unit's team of professionals take part in academic education and practical training on the medicine and nursing degree courses. Cardiology residents usually spend time at the unit, along with residents in other specialities at the hospital. Residents from other hospitals in the Vall d’Hebron area and from other Spanish, European and Latin American hospitals also do so, where they are interested in training in caring for acute cardiovascular disease. 

The unit's professionals regularly take part in training activities outside the hospital. They run master’s courses in cardiovascular disease, and collaborate on them. One of the unit’s members currently coordinates the Advanced Clinical Simulation Centre at Vall d’Hebron.

Experts

The Cardiovascular Critical Care Unit consists of medical and nursing professionals, assistants, porters and administrative staff. They work as a team and in close collaboration with the rest of the Cardiology Department and other departments at the hospital. The medical staff consists of a section head and four assistants, plus on-call doctors and residents. 

The nursing team consists of a supervisor and thirty-two professionals. They are spread over four 12 hour shifts. Mercè Andorrà López is the current nursing supervisor.

Why does a transversal line make sense for treating aortic disease?

Classically this condition has been treated by surgeons. In recent years it has been proven that the only way to improve diagnosis and treatment is to treat the subject in a multidisciplinary manner. Certainly, when the aorta is broken it is vital to offer prompt, careful surgical treatment, but the medical goal is to try not to break it, or to indicate the surgery before the risk is too high. There are two reasons behind aortic disease developing. Firstly, we find genetic disorders such as Marfan syndrome, bicuspid aorta, etc. And on the other hand, we find hypertension or atherosclerosis. It is therefore very important to have a genetics unit, to take into account family history and risk factors, excellent support through imaging techniques (echocardiography, CT and MRI) and a good cardiovascular surgery team. The Multidisciplinary Aortic Diseases Unit at the Vall d'Hebron University Hospital was set up because most of these requirements are already met.

When did we start and how have we evolved?

For 20 years now we have been monitoring all sick people receiving medical discharge after having overcome an acute aortic syndrome, which is a risky condition that can lead to very serious complications. We are one of the leading groups in the protocolised monitoring of this disease. This experience has provided us with a unique knowledge of this disease.

More recently, our interest has expanded to genetic or congenital diseases that may affect the aorta. An important turning point was the creation of the Marfan Syndrome Multidisciplinary Unit in 2009. This Unit, a leading centre in Catalonia, has the purpose of offering patients integral assistance for all their related problems.

Programme aim

To improve the care provided for aortic pathology in terms of prevention, diagnosis and treatment.

Prevention and diagnosis

To inform specialists and general practitioners when the disease should be suspected: family history, genetic diseases, valvular heart disease, atherosclerosis. Proper diagnostic strategy and control of risk factors (ADHD, smoking, etc.)

Surgical / endovascular treatment

Appropriate indications, selection of cases, and intraoperative support for optimisation of surgical or endovascular treatment, in both urgent and scheduled actions.

The Echocardiography and Cardiac Imaging Unit looks after and manages the needs of a great number of people. It has the most advanced technology and heads up knowledge about the speciality. It is a multi-disciplinary unit and brings together caring for people, research and training in day-to-day activities. It has extensive experience in achieving clinical and research breakthroughs. The unit has achieved and published many research milestones.

The unit's services portfolio includes the following: 

Echocardiography laboratory
Each year, this services carries out:

• 23,000 transthoracic echocardiograms. These include bi-dimensional and advanced 3D and 4D echocardiograms, using Strain to analyse myocardial deformation. 
• 1,000 transoesophagal echocardiograms with bi-dimensional and advanced 3D and 4D imaging and 3D-CAT fusion. 
• 150 stress echocardiograms, including exercise and pharmaceutical stress. The unit is certified by the Spanish Cardiology Society, under the SCS Excellence programme, and by the European Cardiology Society.

Transoesophagal echocardiogram guiding for heart valve surgery or structural procedures
Over the past few years, this technique has been included into new procedures to guide the cardiac surgery team. For example, it is used in the aortic valve percutaneous implant, percutaneous repair to the mitral valve, closing the left atrium and closing intracardiac shunts. 

Basal and stress cardiac MRI, cardiac and coronary CAT scans and cardiac and large vessel CT angiography
The unit carries out studies on the diagnostics using images of the myocardial, coronary, pericardial and aorta disease. These are done in collaboration with the Radiology Department. The unit is certified by the European Cardiology Society.

Evolution of the department

The Echocardiography and Cardiac Imaging Unit was formed in 1975, within the Cardiology Department. It was responsible for phonocardiograms - a graphic record of cardiac sounds obtained from a stethoscope - and echocardiograms - an ultrasound of the heart. With the passage of time, and technological advances, the unit has brought in other techniques in the field of cardiac imaging, such as: 

• transoesophagal echocardiogram, from inside the oesophagus
• 3D and 4D echocardiograms 
• strain studies on myocardial deformation
• computerised axial tomography (CAT)
• cardiac MRI 

These advances mean that the unit’s team are specialists in each one of the technologies. 

The team works together with the Arrhythmia Unit, the Congenital Heart Disease Unit and the Genetics, Cardiac Surgery, Vascular surgery and Paediatric Cardiology departments. Daily collaboration enables advances in the field of heart disease from the clinical and scientific research points of view.
The unit also has the following specialist sub-units: 

• The Familial Heart Disease Unit has been recognised as a Centre of Excellence in the National Health System, due to its growth in care and research. 
• The Valve Disease Unit works with the Haemodynamic Unit to offer quality care to patients with aortic and mitral heart valve disease. 
• The Complex Aortic Disease and Genetic Aortic Disease Unit has become a benchmark in the field, both in Spain and Europe. It is a part of the European Reference Network on Rare Multisystemic Vascular Diseases. 

Main diseases related to this speciality

Familial heart diseases
A significant proportion of the people seen in the unit suffer from some kind of familial cardiomyopathy. This is a disease caused by a defect that is genetic in origin, that causes abnormal development of the heart. In some cases it causes exaggerated thickening of the walls and, in others, dilation of the cavities. In both cases abnormal functioning of the heart occurs. Advice from other specialists in genetics and arrhythmia is essential to treat this disease. Collaboration with paediatric cardiology experts is also needed. Treatments include medicines and inserting intracardiac devices that enable the heart to work better and even, in serious cases, prevent sudden death. 

Complex and genetic aortic disease
This is an alteration to the wall of the aorta, which is the body’s main artery. It may increase in size or become more fragile. These changes may cause the artery to rupture or fissure and put the person’s life at risk. The change must be controlled and, if necessary, a surgical or endovascular procedure should be carried out. 

Valve diseases
One of the most frequent appointments at the unit is for disease in one of the four heart valves. The disease is diagnosed with an echocardiogram. The heart’s valves may deteriorate due to ageing, infectious illnesses or some congenital cause. The deterioration may cause narrowing of the valve opening. This prevents the blood from circulating properly. It may also cause incorrect closure which makes the blood go backwards, instead of moving on in the blood system. Currently, early diagnosis, follow-up and suitable treatment enable a valve diseases to be solved or improved to a considerable extent. 

The main treatment for valve disease is the surgical implant of a mechanical or biological prosthesis. Over the last decade implantable prostheses and devices have been developed to repair valves using catheterisation. This is a simple technique that is minimally invasive.

Treatments and diagnostic tests related to this speciality

Transthoracic echocardiogram
This studies the morphology and function of the heart using ultrasound. It is a non-invasive test, with no side effects and highly effective in diagnostics using imaging in cardiology. It enables a detailed study to be made of the heart walls’ mobility, the valves and the blood flow within the heart. Echocardiography currently uses 3D technology. This allows the heart to be studied from a more real perspective and improves the diagnosis.

Transoesophagal echocardiogram
This test also studies the morphology and function of the heart using ultrasound. In this case, it is done using a probe in the inside of the oesophagus. The patient needs to be sedated. The advantage of this test is the high resolution image it gives, which is much higher than conventional echocardiography. For this reason, where there are doubts about a diagnosis, both are done.

Transthoracic stress echocardiogram
This test also studiesthe morphologyandthe function of theheartusingultrasound. In this case,it is done with the patient pedalling a staticbicycle.It enables assessmentof the increase in coronary blood flowin patientswithischaemiccardiopathy.During the test, theelectrocardiogram andbloodpressurearecontinuouslymonitored.Thetestprovidesinformationabout possibleobstructions ofthecoronaryarteriesand, in some cases,aboutthe function of thevalves.

Multi-detector computed tomography
This technique uses X-Rays to provide multiple images of cuts or sections of the body, including the heart. A computer program puts the images obtained together again and creates a 3D view of the heart which enables precise exploration of the coronary arteries, the aorta, valve prostheses or abnormal intracardiac communications. 

Cardiac MRI
This technique also provides images of sections of the heart and the main arteries in the body. It does not use X-Rays. However, it is not advisable for patients with chronic kidney failure or implanted heart devices. It provides information about the heart’s structure and allows precise imaging of its tissue. It determines whether the heart’s tissue is made up of, or occupied by, dead cells, fibrous tissue, fat or liquid.

Research

The research group at the Echocardiography and Cardiac Imaging Unit has an excellent reputation in its field in Spain, Europe and worldwide. The research programme mainly receives external, highly competitive finance. It works to get to know more about heart disease and, therefore, improve people’s health care. The research group works with some organisations, such as the association of patients affected by Marfan’s syndrome (SIMA) and the José Durán Spanish Sudden Death Society.

Research is an area of work that is well consolidated and multi-disciplinary. It creates original ideas and moves forward with technological improvements. Over the past few years, the group's engineering team has developed several innovative clinical imaging analysis tools to get to know about and solve important problems. It has also published several articles in scientific journals and has taken part in several clinical trials, and multi-centre logs and research. The group also actively collaborates in projects associated with Barcelona University and Pompeu Fabra University. The unit is currently coordinating: 

• four Spanish projects: FIS and RETOS
• five European projects: Discharge, Cardiateam, iModern, EIT Health and CT-Pro
• an international log: Pfizer in cardiac amyloidosis
• five clinical trials BICATOR, LOAT, IONIS, ARRAY and DAPA-Moda

The members of the unit are a part of the European Cardiology Society’s Aortic Disease Group, the European Association of Cardiovascular Imaging (EACVI) and the American Society for Cardiovascular Magnetic Resonance (SCMR).

Education 

The training programme includes:

• a specialist medical programme in Cardiology 
• for the bachelors, masters and doctorate students at the Universidad Autónoma de Barcelona (UAB) 
• sub-specialities within the Cardiology speciality 
• an international grants programme 
• internationally renowned weekly scientific seminars 
• continuous medical training 

The individual members of the unit regularly take part in training activities outside the hospital. They run masters courses in cardiovascular disease, and collaborate on them. Each year the unit organises two echocardiogram courses and a meeting on cardiac MRI and CAT. 200 experts from all over the world take part in these events.

Experts

The Echocardiography and Cardiac Imaging Unit is made up of medical, nursing, engineering and administrative personnel, as well as technical and support staff. It works closely with other sections of the Cardiology Department and with the other hospital departments. Furthermore, over the past few years, three people with a degree in engineering have joined who are specialists in fluid dynamics. 

The medical staff consists of a coordinator and seven assistants. The nursing team consists of four specialist experts. The technical team has four experts, qualified with a specific masters from the Spanish Cardiology Society. 

The Adolescent and Adult Congenital Heart Disease Unit (UCCAA) was set up at Vall d'Hebron University Hospital more  than 40 years ago, and we have been providing paediatric care for congenital heart diseases ever since. This Unit is the result of a collaboration agreement between our Hospital and the University Hospital of Santa Creu i Sant Pau, to bring together the effort, resources and experience of these two leading hospitals in the treatment of this disease.

Outpatient visits to the UCCAA Unit take place in the Outpatient Clinic of the Maternity and Children's Hospital, next to the Paediatric Cardiology Department, meaning we can group outpatient care for cardiology of congenital heart disease in a single area. This means that we can share work stations, databases and tools (the ECO 3D) and it makes the transfer and continuity of treatment simple, from paediatric age to adulthood, for what is a congenital and life-threatening disease.

Patients who require admission are directed to the General Hospital, and stay in the cardiology ward. Depending on their clinical circumstances, some of these adult patients with congenital heart disease must be admitted onto special wards:

• Patients in critical condition: Coronary and Intensive Cardiological Care Unit of the Cardiology Department.
• Patients just out of operations: Cardiology Department Postoperative Cardiac Surgery Unit.
• Pregnant patients with congenital heart disease: the Obstetrics, Foetal Medicine and Anaesthesia Departments have created a Working Unit for High-Risk Pregnancies for women with congenital heart disease. Following an established protocol, the checks carried out by the Obstetrics Unit are set to match any necessary cardiac checks to monitor the well-being of mother and foetus during pregnancy, birth and postpartum, with a care protocol that accounts for both the heart disease and the clinical circumstances. We also provide care for cases of postpartum with cardiological risk.

In parallel with these services, there is also a clinic for reproductive counselling for women with congenital heart disease. This clinic is a part of the Adolescent and Adult Congenital Heart Disease Unit (UCCAA), carried out jointly with the Gynaecology Department at the Outpatient Clinic of the Maternity and Children's Hospital. There, a cardiologist and a gynaecologist/angiologist, experts in congenital heart disease, work together to analyse the patient depending on their individual clinical and heart disease condition and explore their chances of conceiving children. Their mission is to inform, evaluate and offer sexual health planning, discussing the reproductive possibilities of the patients living with congenital heart disease.