The combined (difficult) heart diseases – the congenital or acquired anomalies of heart at which there is a simultaneous damage of several heart valves or a combination of several anatomic defects. Displays of the combined heart diseases depend on prevalence in structure of defect of this or that anatomic defect, a combination of defeats and their mutual influence. Diagnosis of the combined heart diseases is based on data of an echocardiography (including fetalny), an electrocardiography, a X-ray analysis of a thorax, an aortografiya, a ventrikulografiya, a kateterization of cavities of heart. At the combined heart diseases conservative treatment of the haemo dynamic violations (pulmonary hypertensia, heart failure) accompanying them or surgical correction is carried out (multivalvate prosthetics, etc.).
Combined heart diseases
Combined (difficult, multivalvate) heart diseases are characterized by simultaneous damage of two or three heart valves and openings – mitralny, trikuspidalny, aortal and pulmonary. Existence at one patient of eight heart diseases – a mitralny stenosis and mitralny insufficiency, a trikuspidalny stenosis and trikuspidalny insufficiency, a stenosis of the mouth of an aorta and aortal insufficiency, a stenosis of the mouth of a pulmonary artery and insufficiency of the valve of a pulmonary artery is theoretically allowed at once. However such multiple defeat in cardiology meets extremely seldom.
Among the difficult acquired heart diseases the combined defeat of mitralny and aortal valves more often (mitralno-aortal defect – 44%), mitralny and three-leaved valves (mitralno-trikuspidalny defect – 10%) and all three listed valves meets ( - aortal defect – 11%). More rare combinations are mitralno-aortal insufficiency, aortal insufficiency in combination with the combined mitralny defect, the combined aortal defect in combination with the combined mitralny defect, etc. Involvement of valves in pathological process can happen at the same time or consistently.
Fallo's triad, Fallo's tetrad, Sean's syndrome, Ebstein's anomaly, Eyzenmenger's complex, etc. at which valvate defects are combined with defects of partitions and the main vessels of heart also belong to the combined congenital heart diseases.
Reasons of the combined heart diseases
Difficult congenital heart diseases are formed vnutriutrobno owing to cardiogenesis violation. Anomalies of cardiovascular system of a fruit can develop in the presence of the following risk factors: toxicoses of the I trimester, diseases of the pregnant woman (a rubella, diabetes), threats of spontaneous termination of pregnancy, still birth cases in the anamnesis, a hereditary otyagoshchennost on VPS in this family etc. possess Teratogenny action some medicines accepted during pregnancy (thalidomide, progestagena, lithium medicines, anticonvulsive means, etc.), alcohol, the ionizing radiation.
Formation of the combined acquired heart diseases is more often connected with the postponed rheumatism, a system red volchanka, a bacterial endocarditis, atherosclerosis, a heart injury. The combined heart diseases sharply negatively influence haemo dynamics as the blood circulation violations peculiar to each isolated defect, at their combination are summarized, leading to bystry development of a decompensation.
Symptoms of the combined heart diseases
The clinical picture of the combined heart diseases of a polimorfn is also defined by a prevalence of symptoms of one defect over other or their mutual strengthening. In certain cases manifestations of one defect can smooth out the compensating influence of another.
So, at mitralno-aortal defect with prevalence of a stenosis the clinical picture is defined by signs of a mitralny stenosis – short wind, heartbeat, feelings of interruptions in work of heart, the blood spitting caused by pulmonary hypertensia. At fizikalny inspection of patients with the combined mitralno-aortal heart disease the mitralny flush, a phenomenon of "cat's purring" come to light . Development of left ventricular insufficiency is followed by attacks of cardiac asthma and hypostasis of lungs.
Domination during defect of a stenosis of the mouth of an aorta more blagoyopriyatno – such combined heart disease is longer remains compensated. Existence in clinic of the combined heart disease of attacks of headaches, dizzinesses, short-term unconscious states forces to think of a prevalence of aortal insufficiency.
The combined mitralno-trikuspidalny heart disease with prevalence from insufficiency of the three-leaved valve is characterized by pallor of integuments with the icteric shade expressed akrotsianozy, short wind, swelling and a pulsation of veins of a neck, by early development of heart failure. As manifestations - as aortal heart disease serve widespread cyanosis, arterial hypotonia, permanent increase in a liver, hypostases of a waist and the lower extremities, , ascites.
In general the combined heart diseases proceed heavier, than isolated. Cardiovascular insufficiency, a thrombembolia, pneumonia becomes frequent causes of death of patients with the acquired combined heart diseases.
According to domestic cardiologists and heart surgeons (A. Gorbachevsky, L. Bokeria), 14% of the children born with the heavy combined heart diseases die in the first week of life, 25% - in the first month, 40% - within the first year of life. The similar statistics testifies to need of carrying out careful prenatal screening, identification of VPS at a fruit, adoption of the weighed decision on expediency of prolongation or abortion.
Diagnosis of the combined heart diseases
Diagnosis of the combined heart diseases is difficult in view of mutual stratification of various clinical symptoms. The cardiologist obtains primary information on the available heart disease at survey of the patient, percussion and an auskultation of heart.
The further diagnostic search necessary for differential diagnosis of the combined heart diseases includes carrying out tool inspection. An electrocardiography and daily ECGs monitoring allow to reveal signs of a hypertrophy of a cardiac muscle, the available violations of a warm rhythm. By means of a phonocardiography the tones and noise in heart which are indirectly demonstrating defeat of the valvate device are registered and estimated.
According to the roentgenogram at the combined heart diseases the myocardium hypertrophy, expansion of cameras of heart, developments of stagnation in lungs etc. is quite often visualized. As the most informative type of diagnosis of the combined heart diseases serves the echocardiography with a dopplerkardiografiya giving a clear idea of a condition of the valvate device, existence of defects of partitions, pathological intracardial haemo dynamics.
MSKT or MRT of heart allow to consider the layer-by-layer spatial image of structures of body that is especially valuable to diagnostics of the combined defects. More exact topichesky and differential diagnosis of the combined heart diseases is performed by means of special methods of a research – a ventrikulografiya, an aortografiya, sounding of cavities of heart, a flebografiya. Patients with the combined heart diseases and IBS need carrying out coronary angiography.
Treatment of the combined heart diseases
In treatment of the combined heart diseases conservative medicamentous and radical surgical methods are applied. Medicinal therapy is directed, mainly, to correction of heart failure (diuretics, warm glycosides), prevention of tromboembolichesky complications (anticoagulants), improvement of metabolism of a cardiac muscle (inosine, potassium medicines), elimination of arrhythmias (β-adrenoblokator, etc.). Medical actions at the combined heart diseases concern also main disease in this connection courses of antirheumatic therapy, prevention of an infectious endocarditis etc. are conducted.
Expeditious treatment of the acquired combined heart diseases can consist in performing multivalvate prosthetics, prosthetics of one valve and plasticity of another, sometimes – combinations of double prosthetics to a komissurotomiya. In literature there are single messages on successful prosthetics of three valves of heart. Possibilities of a modern heart surgery allow to carry out prosthetics of valves of heart at an active infectious endocarditis.
Cardiac intervention at the combined heart diseases is contraindicated at a diffusion cardiosclerosis, emphysema of lungs, hepatitis, a heavy glomerulonefrit, an aggravation of a revmokardit, malignant tumors. One-stage or stage-by-stage ways of radical correction are developed for difficult congenital heart diseases.
Forecast and prevention of the combined heart diseases
The forecast of the natural course of the combined heart diseases is extremely adverse. Five-year survival at multivalvate defects makes 35,5 – 44,5%, ten-year-old – 6-9,6%. Results of operations at the combined heart diseases it is worse, than at the isolated defeat of one of valves. Nevertheless, postoperative 5-year survival is quite high - 72%.
Prevention of the combined heart diseases consists in an exception of teratogenny influences, the prevention of primary and returnable revmokardit and a septic endocarditis. Patients with the combined heart diseases have to be in a constant field of vision of the cardiologist, rheumatologist, heart surgeon; to receive rational medicamentous therapy.