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INFORMATION

Children can suffer congenital cardiopathies and gained, but with a disease spectrum different to the adults. Currently congenital cardiopathies can be diagnosed in foetus time allowing an adjustment that minimized its risks and prepare the delivery type and its treatment. Fortunately, the vast majority can be treated, and they can have a normal life, though they will need to be in contact to a medical team who will manage their problems and guide their medical treatments, kind of activity, supports, capacities, etc. In this Unit the assistance is custom made for each child with these pathologies.
The Cardiology Unit and the Paediatric Cardiac surgery and the Adult’s congenital cardiopathies are formed by the Paediatric Cardiology service and the Children Cardiac Surgery.
The Ruber International Hospital’s paediatric cardiology service is headed by Dr. F. Gutiérrez- Larraya. It is formed by the Paediatric Service (Dr. F. Mar) and teamed up with the Adults’ Cardiology Service (Dr. J. Ros). Emergency services are locally available during 24 h a day, all days a year and the Scheduled Consultations are on Wednesday and Friday afternoon. Caring for all cardiac problems, congenital or not, of children from their prenatal lifetime to 14 years old. The Hospital has from the most basic diagnostic and therapeutic technology to most update: electrocardiography, echocardiografía (two-dimensional, three-dimensional, color …), endurance test, ECG and TA Holter, catheterism diagnosis and therapeutic (digital laboratory), electrophysiology, multi-slice CT, magnetic resonance, operating theater of cardiac surgery, in addition to a magnificent laboratory of clinical analyses.
The Ruber International Hospital Paediatric cardiac surgery service is headed by Dr. Ángel Aroca Peinado. It attends all the children’s cardiac surgery problems from neonatal to 14 periods and it is coordinated with the Adults’ Cardiac Surgery Service (Dr. J.E. Rodríguez) to provide treatment to teenagers and adults carriers of congenital cardiopathies who need surgical operation or reoperation.
The Hospital arrangement and the qualification of its staff warranties the medical care excellence and efficacy to patients with a commitment in teaching, research and continued training, performing clinical sessions, attending congresses, meetings and national symposiums and promoting the quality medical newsletter.

WHAT DO WE TREAT?

Clinical Cardiology

It is not a disease, but a kind of audible noise in auscultation. The heart, in his cycles, produces noises, the most spectaculars, and therefore, easier to listen / auscultate, which are generated by the valves in its movements. The blood in its movement generates sound but this sound is getting depressed in intensity from the heart on having to cross many layers to be heard from the exterior: cardiac muscle itself and its coverings, visceral fat, lungs interposition, bony structures (breastbone, ribs), muscles, breast tissue, fat, skin. Although all these slices, when we ear by auscultating the heart the blood movement, we named it “murmur” because it really resembles a blast. Luckily, in children, it is the regular specific sound in most cases since, logically, they have less thickness in layers, described above, filtrating the standard sound. Certain circumstances, also the normal ones (fever, exercise, stress…), are going to produce a more vigorous beat easing its perception; in fact, most of the children sent to a cardiologist due to a murmur, is because this one has been perceived in these circumstances. Heart murmurs are graded depending how loud the murmur sounds, and not on seriousness. There are perceived six grades: the less intense are grade I, and the more intense are grade VI. Murmurs in normal people are known by several names, indifferent: Functional murmurs, innocents…, they are of minimum intensity and are not accompanied by other signs nor symptoms.
The great majority of cardiac diseases are congenital injuries, and most of the acquired ones generate murmurs, which features are different to the functional murmurs. Injuries are not only diagnosed by the murmur, but also for further details of auscultation, medical examination and patients’ symptoms.
Although the auscultation and the medical examination by experimented doctors often accurately register murmurs, it is normal to perform some complementary examination (electrocardiogram, radiography, echocardiograms).

Fetal Cardiology

During gestation, the cardiovascular apparatus is gradually being formed, in the two fist months, by the dividing walls growing, creases… and from two months, the cardiac flow itself determines the heart’s development and growth and big arteries and veins. In fetal life, congenital and not congenital problems may arise, which are in a more general context (certain maternal diseases as diabetes mellitus poorly controlled). Congenital and hereditary are not synonyms and, in fact, most of the congenital malformations have not been inherited from ancestors.
The cardiovascular system function in the embryotic life (the first two months of gestation) and fetal (the rest) has nothing to do with postnatal, in fact, the transition between the fetal model and the “adult” is not completed until the last two months after birth. During gestation, oxygenated blood and nutrients come to the embryo/fetus, from the mother, once filtered by placenta, through a vein and not an artery. Thanks to a complicated system of embryonic and foetal circulation, the nutrition and oxygenation (firstly cerebral and then cardiac) is a priority.
Most of malformations are produced by unknown reasons, although the severest can be in context of some well-known syndrome. Most of the analytical and echographical examinations done by obstreticians are pointed to identify them.
Fetal echocardiography is a method that allows to identify injuries and tries to qualify their consequences or from general fetal situations with fetal cardiovascular aftermath using varieties of ultrasounds. It is not a perfect method and, this means, that it cannot diagnoses absolutely firmly and accuracy all the injuries and their aftermaths. This stems from multiple natural reasons: technological limitations (in spite of its vertiginous progress) and maternal (hair presence, fat volume, personal tissues characteristics can make difficult examinations, and fetal (its size, position within the uterus, arms’ interposition, legs, placenta…) and physician’s degree of experience. Findings should be interpreted regarding fetal circulation.
The examination can be done in any moment of pregnancy (the first quarter ones must be done via transvaginal given the small size of embryo/fetus) and the last quarter of pregnancy are more difficult due to the fetus position and the arms interposition, legs and ribs calcification degree. The best moment to do a cardiac examination is around the second quarter.
These explorations are done, as mentioned above, by echocardiography and the physician that usually performs it is an expert obstetric in fetal physiopathology, although sometimes can be a radiologist. After this first filter, if any anomaly is observed, or simply a difficulty in the exploration does not satisfy the physician, the gestating mother is referred to a pediatric cardiologist, expert in fetal cardiology. The discovery of a cardiac malformation or a fetal cardiovascular disease of a more complicated general situation allows reporting and advising the family and preparing the delivery (moment, place, type of delivery) and the immediate assistance to the newborn in order to minimize the possibility of complications and implies a more synchronized teamwork (obstetrics, neonatologist, anesthetists and cardiologists). A diagnosis in fetal life requires a precise postnatal verification and research, having now many other techniques (echocardiographies, blood analysis, radiographies, electrocardiograms, MRs, CTs… ) easing an accurate diagnosis that allows to establish a detailed diagnosis.
Fortunately in most cases gestating women referred to fetal cardiologic examination or without having any problem or this one is common. At present, though there are very concrete situations in which is possible to perform a foetal surgery, in most of the cases, these are not advisable due to their risk for the foetal live and the maternal well-being.

Interventional Cardiology

The striking technological development lets tackling cardiopathies that used to be untreated. Most of these cardiopathies have as a unique feature, or it is substantial for its intricacy the existence of blockages or harmful septal. Since one decade ago, many diseases could only be diagnosed by invasive techniques, as catheterism; now most of these have a therapeutic purpose.
Interventional cardiology allows the treatment of these injuries through a not surgery approach. By a puncture of one or more arteries or shorter veins from the body, preferably in groin area (since they are out of the thoracic and abdominal cavity), basically with the same technique as blood extraction is performed, there are thin plastic tubes (catheters) to be moved forward to analyse, act in certain points (drilling, dilating, administering medications and releasing devices). The cardiologist gently inserts these catheters by soft movements “sailing” through the bloodstream to the key points. The catheter itself provides information regarding the way and controls it, its screen display is by a fluoroscopy screen; therefore, in a great part of cases low dosage of ray-x are used. Sometimes the information is complemented applying ultrasounds (echography) using probes to emit from various possible locations (through the esophagus, endovascular, transthoracic.) The anatomical complexity of the cardiovascular apparatus and the great variety of injuries (in location expansion and severity) oblige to use diverse tools for helping (guides) and a considerable variety of catheters. There are countless devices specifically intended for the injuries treatment: “stents” (meshes made from inert metals alloys, covered or not by medicines), devices for occluding septal, stoppers of different types (coils, balls, particles), several sizes balloons, lengths, forms and composition.
While in adults can be used sedation and local anaesthesia, in children is used general anaesthesia and a hospital stay of, at least, one night and the same day. In each case, the cardiologist establishes and informs the family of the previous fasting hours, what to do with medication if there is and the watching over after the discharge to home.
Nowadays, the eventual complications are low and are related to the patient’s age, his circumstances and the type of catheterism to be performed.
Listing:
The proposed index should be considered as a guide, since the successive technical innovations and the results analysis of the already established, can modify this list.
Closures:
• Certain type of atrial septal.
• Certain type of ventricular septal defect.
• Inter arterial septal closure (arteriosus ductus).
• Fistula occlusion at different levels, intra and extra cardiac.
Opening:
• Occluded cardiac valves congenital or acquired.
• Occluded veins or arteries.
• Occluded surgical ducts.

Surgical Treatment of congenital cardiopathies from born to adult age. Various examples.

-Neonatal period preparation, transposition of the great arteries (TGA):
It represents the 8% of all the CC (Congenitally Corrected). The D-TGA with entire ventricular septal is an arterial ventricular defect. The right ventricle with venous blood return of all the body is related to the aorta, in previous position, the left ventricle with venous return blood; send again to lung by the pulmonary artery. This seriously determines cyanosis from birth with rapid appearance of metabolic acidosis and shock. As there is not surgical treatment, more than 80% of patients have died before having a year old.
The pre-operative management looks to ensure points of venous blood and arterial mixture to maintain the oxygen saturation (SaO2) between 75-85% and balance between systemic and lung circulation (QP/QS = 1). This is achieved by keeping open the ductus with prostaglandin E1 IV and performing a CIA or percutaneous Rashkind with balloon catheter via umbilical with ECOcardio monitoring.
Anatomical repair surgery or Jatene procedure is carried out electively on the 4-14th day of life, before the lung vascular resistances decrease and the “preparation” to VI are lost. The surgery is done with extracorporeal circulation and is to cut the aorta and pulmonary which come from the wrong ventricle and taking coronary arteries (which supply to heart itself) to neo-aortic going out already from the left ventricle.
Most of the patients will remain asymptomatic after surgery and without medication, but they will need cardiologic revisions permanently, since a small percentage could suffer residual lesions or future aftermaths.
-Repair during lactation, the Tetralogy of Fallot (TOG):
It is the cyanotic CC most frequent, it is almost the 10% of all the congenital cardiopaties. It comprises the presence of an infra crystal ventricular septal defect (VSD), obstruction of the right ventricle exit (EP), overriding aorta on the septal defect and right ventricle hypertrophy (tetralogy). A 10% is associated to a atrial septal (CIA) and are called Pentalogy of Fallot. It causes a progressive cyanosis, less remarkable than in the TGA at birth. Hypoxic crisis by infundibular spasm may occur, more frequent from the 2ond month of life. Repair surgery is indicated at any age, even neonatal, if there are symptoms: SaO2 kept idle < of 80-85% or isolated < if 65%. Optionally between 3rd and 9th month of life. Without any surgical treatment, more than 50% of patients would have died before having a year old. Surgery is performed with extracorporeal circulation, and is the closing of VSD with patch and EP resolution (CP, infundibular resection, transannular patch, trunk enlargement and/or branches, VD-AP conduct in complex cases…) A great greater part of patients, after surgery, reach the adult life almost asymptomatic, although also they will need lifelong cardiological reviews on considering that among a 25 and a 30% of them will need surgical treatment or haemodynamic of the residual pulmonary insufficiency.
-Repair during childhood, atrial septal (CIA):
It is a frequent congenital heart disease (10%). It is the defect in atrial septum wall continuity at fossa ovalis level. Pulmonary hyperflow course (left shunt ->Right of blood at an intercardiac level). The prevalence is almost double in women. It is related to many CC, in some it is imperative for the initial survival: Hypoplastic left heart syndrome, pulmonary vein drain absolutely abnormal… It produces poor symptomatology in first decade (frequent respiratory infections, dyspnoea if a great shunt). Rarely stature-ponderal deficiency. There are spontaneous closures in first years of life, but less than in CIV. Without correction, the estimated mortality is a 25% due to cardiac insufficiency, Eisenmenger, embolism,… from the 3th decade of life.
The closure is indicated if the Qp/Qs is > than 1.5, or the deficiency is bigger than 8 mm diameter.
In ostium secundum CIA (most frequent), the current treatment chosen is the closing with percutaneous device insertion through interventional cardiology (Amplatzer…), if it has a sufficient rim of insertion, and it is < than 25mm diameter.
Electively it is done before schooling, but if the percutaneous closing is chosen, it will be delayed to decrease the vascular morbidity.
Operated patients at childhood, after a favourable revision few months after surgery, they are considered to be cured.

MEDICAL TEAM

Dr. Gutiérrez-Larraya Aguado, Federico Team leader
Más información

DrLarraya
  • Doctor en Medicina.
  • Especialista en Cardiología Pediátrica
  • Profesor Asociado de Pediatría, Universidad Autónoma de Madrid.
  • Jefe de Servicio de Cardiología Pediátrica del Hospital Ruber Internacional y del Hospital Universitario La Paz, Madrid.
  • Fellow American Society for Cardiac Angiography and Interventions.
  • Máster en Dirección de Organizaciones Sanitarias. ESADE.
  • Máster en Dirección y Gestión de Servicios de Salud. Instituto Europeo de Salud y Bienestar Social.
  • Miembro de las principales Sociedades Profesionales Internacionales y Nacionales.
  • Numerosas publicaciones, comunicaciones, conferencias y libros de la especialidad, nacionales e internacionales.
  • Editor de Revistas de la especialidad, nacionales e internacionales.
  • Director de Instalaciones de Rayos X con fines diagnósticos.
  • Investigador en Proyectos con financiación pública y privada relativos a la especialidad.
  • Responsable cardiológico de la ONG Fundación Adelias. España
  • Numerosos premios relativos a la Especialidad.
  • Variados cargos administrativos a nivel de gestión, en las diversas instituciones sanitarias locales, de la comunidad y nacionales.
  • Miembro de comisiones de mortalidad.
  • Presidente Comisión Permanente de Dirección del Hospital Infantil La Paz.
  • Asesor de la Consejería de Sanidad la CAM.
  • Miembro de Plataformas de Innovación.
  • Secretario Grupo de Trabajo de Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas.
SERVICES PORTFOLIO

Diagnostic tests

Electrocardiography, ECG Holter and arterial pressure, endurance test, foetal and paediatric echocardiography, MR, CT.

Specific percutaneous treatments

Valve openings and dilations, arterial occlusions and anomalous veins, stents placing in arteries and veins, percutaneous valve placing, intra and extra cardiac impairment closing.

Specific percutaneous treatments

2nd cardiological opinion 2nd surgical opinion.