Maximum survival age for Eisenmenger's syndrome. Eisenmenger's disease - symptoms and treatment. How do patients with Eisenmenger's syndrome live?

Eisenmenger's syndrome or complex is one of the congenital heart defects. The defect is quite rare - Eisenmenger's syndrome in children is about 4-5% of all congenital pathologies of the heart and blood vessels. It most often develops before the child reaches puberty. However, there are cases when the complex develops in adolescence and progresses throughout adolescence.

In many ways, Eisenmenger's syndrome is similar to the group of heart defects identified by Etienne-Louis Arthur Fallot - the triad, tetrad and pentad of Fallot. It differs from them in the absence of narrowing of the pulmonary artery.

Syndrome or Eisenmenger's complex is one of the congenital heart defects

Etiology and physiology

The most common cause of Eisenmenger's syndrome is a hole (shunt) between the two chambers of the heart. Such a hole can be congenital or artificially created during a surgical operation. The shunt provokes abnormal blood circulation not only in the heart, but also in the lungs. Arterial and venous blood mix. Instead of entering the body, the blood returns to the lungs. As a result, blood pressure in the lungs rises. Vessels become rigid and gradually become thinner, which can lead to damage to small vessels. This leads to oxygen starvation in all organs and tissues, the heart has to work at a faster pace.

Other pathologies of the heart that can lead to the development of the complex:

• open atrioventricular canal;
• cyanotic heart defects (conditions with increased pulmonary blood flow);
• common arterial trunk.

Heredity also increases the risk of having a child with this pathology.

Cardiovascular diseases

The physiological progress of Eisenmenger's syndrome is represented by a spectrum of changes in the structure of the bloodstream (the totality of the cavities of the heart and blood vessels), which can be reversible or irreversible.

Cases of Eisenmenger's syndrome are possible even in cats and dogs. Unfortunately, the animal, in most cases, quickly dies.

Symptoms

Often the disease is asymptomatic for a long time and is detected already at a later stage, when it is difficult to treat. Therefore, do not neglect preventive medical examinations. Regular examinations by a cardiologist can help to identify a serious and rare disease in time.

Signs of the Eisenmenger complex:

• dizziness;
• pathological fatigue, weakness;
• dyspnea;
• cardiopalmus;
• arrhythmia;
• feeling of constriction, pain in the left side of the chest;

One of the symptoms of this disease is arrhythmia.

• frequent bouts of coughing;
• fainting;
• increased sweating;
• swelling of the abdominal region;
• numbness and tingling in the fingers and toes;
• expectoration of blood;
• swelling in the joints.

Unlike the group of diseases that Fallot identified, cyanosis (cyanosis), fingers in the form of drumsticks and nail plates in the form of watch glasses occur much later. This is due to the fact that the pulmonary artery in this case does not narrow.

There are two periods of development of the disease:

• I - without cyanosis;
• II - with cyanosis.

In the first stage, oxygen saturation of the peripheral arterial blood is not disturbed, or the disturbances affect the body only slightly. This period can proceed without symptoms or with a blurry picture. In the second stage, blood oxygen saturation is significantly reduced, the symptoms of the disease are already clearly manifested.

Improper treatment can cause a heart attack

If the patient does not receive proper therapeutic assistance, complications arise:

• embolism;
• iron deficiency in the body;
• chronic heart failure;
• gout;
• heart attack;
• stroke;
• inflammation of the endocardium of a bacterial nature;
• cerebral hemorrhage;
• secondary polycythemia;
• increased blood viscosity (due to the ingress of cells of collapsing vessels);
• kidney failure;
• infectious processes in the brain;
• circulatory disorders of all organs, including the spinal cord and brain;
• sudden death.

Improper medical care can lead to kidney failure

Diagnostics

Already with a superficial examination, the specialist will reveal a violation of the rhythm of heart contractions. Additional tests are needed to confirm the diagnosis:

• echocardiography;
• chest x-ray;
• cardiac catheterization.

Since patients have strong changes in the blood picture, indicators of a general blood test with a hematocrit number (the ratio of blood cells) will be very important for diagnosis. As a result of the study, polycythemia can be detected, and the hematocrit will exceed 60%.

The most common differential diagnoses are: arterial stenosis, pentade of Fallot, non-closure of the arterial duct.

Treatment

Sometimes patients have some blood removed from the body (phlebotomy, earlier bloodletting). To compensate for the volume of lost fluid, saline solutions are administered to a person.

Patients may have some blood removed from the body - a phlebotomy

The patient may be given oxygen, although it is not currently established whether this procedure helps prevent worsening of the disease. In addition, sedatives and vasodilators are prescribed.

Surgical treatment is very difficult: during the operation, it is necessary to eliminate the defect of the interventricular septum and a possible anomaly of the aorta. Almost always, this requires the implantation of an artificial heart valve. Due to the complexity of the surgical intervention, in some cases, the procedure is extremely dangerous and can lead to the death of the patient.

In severe cases, a heart and lung transplant may be needed to save the patient's life.

Forecasts

Forms with early cyanosis are extremely unfavorable. Without surgery, sick children die before reaching adulthood.

If the disease is detected before the development of cyanosis, the prognosis is doubtfully favorable. After the onset of blueness, even with proper medical care, patients usually do not live more than 10 years.

Patients with the syndrome can live from 20 to 50 years. The course of the disease depends on the presence of other pathologies in the body, as well as age. As you know, blood pressure indicators increase with age, which has a negative impact on the health of patients with the Eisenmenger complex.

In general, the prognosis for this disease is better than for other malformations with cyanosis.

Eisenmenger's syndrome (ICD code 10) is an irreversible form of hypertension in the pulmonary artery system associated with congenital heart disease. It is believed that this condition can develop with any neglected defect with a significant discharge of blood from right to left. The Eisenmenger complex occurs in 10% of children and adults who have not undergone surgery, with a ventricular septal defect of any size, with an atrial septal defect - in 4-6% of patients. This serious condition has a significant impact on the life of patients: its duration is about 40 years.

Causes and pathophysiology of the development of Eisenmenger's syndrome

Eisenmenger's syndrome can be caused by both simple and complex congenital heart disease. This condition is a complication of "pale type" defects (without manifestations of cyanosis), which, with progression, can transform into "blue" ones (with pronounced cyanosis). Patients diagnosed with Eisenmenger's syndrome have at least one defect in the heart:

• interventricular septum (VSD);
• interatrial septum (ASD);
• open ductus arteriosus;
• tetralogy of Fallot (stenosis of the outflow department of the right ventricle, VSD, aortic dextrapposition, right ventricular hypertrophy);
• Fallot's pentad (Fallot's tetrad in combination with ASD);
• Ebstein's anomaly (tricuspid valve insufficiency, open foramen ovale, decreased right ventricular cavity).

Patients with similar leaks in the heart chambers undergo intracardiac shunting of blood from the left (systemic, with higher pressure) to the right (pulmonary) compartments. This leads to supersaturation of the right atrium and ventricle with blood volume, which reflexively leads to spasm of the pulmonary arteries and increases pressure in the pulmonary artery system. Small vessels inside the lungs change structurally, which further increases intrapulmonary pressure and makes subsequent treatment more difficult. Due to a defect inside the heart, arterial blood saturated with oxygen mixes with venous blood, which leads to the development of hypoxia and cyanosis (blueness of the skin).

Clinical manifestations

Many children with Eisenmenger's syndrome have had at least one episode of pulmonary edema due to increased pulmonary blood flow resulting from an intracardiac left-to-right blood shunt. Later, with an increase in pulmonary vascular resistance, the movement of blood in the lungs slows down, and the symptoms of pulmonary congestion are reduced. When the left-right to right-left shunt transforms, cyanosis and erythrocytosis develop as a consequence of chronic hypoxia. Much less often, the clinic manifests itself in adulthood without specific manifestations: increasing weakness, shortness of breath, cyanosis. Most patients show the following symptoms:

• symptoms of hemodynamic disturbances in the systemic circulation (shortness of breath during exercise, weakness, fainting);
• neurological abnormalities (headaches, dizziness, visual disturbances) due to erythrocytosis and hyperventilation;
• symptoms of congestive heart failure;
• arrhythmias and hemoptysis (as causes of sudden death).

Diagnostics

Physical examination reveals central cyanosis, a symptom of "drumsticks" (the nail phalanges on the hands expand, resembling the shape of drumsticks), swelling of the cervical veins, the presence of a heart hump (deformities of the chest in the region of the heart), pulsation to the left of the sternum. During auscultation, an accent of the second tone is heard over the pulmonary valve, a rough systolic murmur over the entire surface of the heart with a characteristic symptom of "cat's purr" (trembling of the chest, similar to purring).

Phonocardiography is used to record sound effects. On a chest x-ray in a direct projection, an expansion of the trunk of the pulmonary artery is revealed. Standard ECG diagnostics registers signs of overload of the right heart. With catheter measurement of pressure in the pulmonary artery, its increase to 60-100 mm Hg is detected. (at a rate of up to 30 mm Hg). Echocardiography confirms the presence of one or more defects in the heart.

Treatment

A positive prognosis is possible with rapid surgical intervention, which consists in suturing defects in the interatrial and interventricular septa, eliminating the incorrect location of the aorta, and closing the open ductus arteriosus. Many studies have proven the ineffectiveness of surgical treatment of Eisenmenger's syndrome in adulthood: the earlier the operation is performed, the greater the likelihood of a positive outcome. But palliative (removing symptoms) operations are possible, for example, aimed at narrowing the pulmonary artery to reduce pressure in it.

Drug therapy for Eisenmenger's syndrome is symptomatic, aimed at improving the activity of the heart muscle, lowering intrapulmonary pressure, and reducing the symptoms of heart failure.

It is carried out in the preoperative period and with contraindications and the impossibility of performing cardiac surgery.

Drugs used in the treatment of Eisenmenger's syndrome:

Although primary PH is not directly related to CHD, children with this pathology are sometimes admitted to cardiac surgery clinics for differential diagnosis. Specialists repeatedly met patients with severe PH, which could not be associated with insignificant concomitant CHD. Cardiac surgeons are forced to take on advisory assistance in matters of not only diagnostics, but also therapeutic treatment of this difficult group of patients.

The first report of familial primary PH was made in 1927. Clarke et al described the clinical presentation and morphological findings on autopsy of primary PH in 5- and 8-year-old sisters. However, Dresdale et al. were the first to show familial transmission of the disease from one generation to the next. They described the case history of a family in which a woman and her son died of primary PH, respectively, at 43 and 21 years of age. In addition, her brother and sister died in early childhood at the age of 31 from right ventricular failure, probably due to primary PH. These early clinical descriptions contained many of the now well-established facts of familial primary PH, including vertical transmission, genetic prejudice, and the curious observation that in the family, the clinical course of the disease is more severe in males and they die at a younger age than females.

The frequency of familial primary PH is 1-2 cases per 1 million population and 6% in the US registry of PH of various etiologies, although there is reason to believe that quite a lot of cases are not counted. Familial primary PH differs from the sporadic form in that it is diagnosed earlier after the onset of symptoms. However, it does not differ from sporadic either clinically or in terms of the ratio of women to men - 2:1 in adults and 1.3:1 in childhood.

Familial primary PH is transmitted vertically. So, a family is known in which 5 generations suffered from this disease. It can be passed from male to male, but a case has been reported at the Toronto Pediatric PH Clinic in which a healthy father had two daughters with primary PH from different mothers. This example of transmission excludes the X-linkage of genes and strongly suggests the presence of an autosomal dominant gene.

Histology

The histological features of familial pulmonary arteriopathy are heterogeneous and often combine thrombotic and plexiform lesions. Histologically, familial, sporadic primary PH, and Eisenmenger's complex are indistinguishable. Lee et al showed that plexiform pulmonary vascular lesions in familial primary PH contain monoclonal proliferating endothelial cells as opposed to polyclonal endothelial cell proliferation in secondary PH. The presence of monoclonal proliferation of endothelial cells in primary PH indicates that somatic gene damage, similar to that in neoplastic processes, may contribute to clonal expansion of pulmonary endothelial cells. In primary PH, histological examination sometimes reveals occlusion of pulmonary venous microvessels and capillary hemangiomatosis.

Clinic

The etiology of primary pulmonary arterial hypertension is unknown. It affects predominantly young people, and the course of the disease is inexorably fatal, although isolated cases of spontaneous regression have been recorded. The diagnosis is established already at an early age, usually in advanced stages of the disease. The average life expectancy is 4 years. An important determinant of survival is right ventricular function. The life prognosis is better in patients with right atrial pressure less than 7 mm Hg. Art. A bad harbinger is low oxygen saturation of mixed venous blood. Children respond better to vasodilators than adults. The positive hemodynamic effect of treatment improves the prognosis, but not in everyone. According to lung biopsy in children, medial hypertrophy is more pronounced, which explains the tendency to vasoconstriction, and angiomatous changes and intimal fibrosis are less pronounced.

Pulmonary vascular hypertension in the absence of intracardiac shunts is poorly diagnosed in childhood, since objective symptoms are not pronounced. The most typical signs are:

• fainting or semi-consciousness;

  generalized convulsions;

• palpitations or cyanosis during exercise;

  swelling in the legs.

Load intolerance is always noted. Pain in the heart for children is atypical, unlike adults. However, myocardial ischemia can also occur in children when the pressure in the pulmonary artery exceeds the systemic pressure, as well as during exercise.

The characteristic features of PH are seen on a chest x-ray:

expansion of the heart shadow;

bulging of the second arc along the left edge of the heart;

expansion of the proximal pulmonary arteries with "chopped off" peripheral branches.

The electrocardiogram shows hypertrophy of the right atrium and ventricle with signs of overload in 70-80% of patients.

Echocardiography allows diagnosing an increase in pressure in the right ventricle, the absence of pathology of the mitral valve and pulmonary veins, as well as the absence of other probable causes of right ventricular hypertension - subvalvular, valvular and supravalvular stenosis of the pulmonary artery. The presence of narrow proximal pulmonary arteries with constant distal flow on Doppler cardiography indicates multiple peripheral pulmonary artery obstructions. Extra- and intracardiac shunts should also be excluded.

Cardiac catheterization and angiocardiography are the most important studies for establishing the correct diagnosis.

Treatment of primary PH

Until the last 10 years, conventional therapy was mainly symptomatic and limited to digoxin, diuretics, calcium channel blockers, and anticoagulants. However, the latest advances in vascular biology and molecular genetics are rapidly being introduced into practice in the form of etiopathogenetically substantiated treatment.

Calcium channel blockers

In 1992, Rich et al showed that large doses of calcium channel blockers reduced pulmonary artery pressure and resistance by more than 20% in 26% of patients with primary PH. Oral administration of nifedipine or diltiazem in patients showed a 94% survival rate for 5 years and signs of regression of right ventricular hypertrophy, improved exercise tolerance and quality of life. However, in the part of the subjects in whom the decrease in PVR was not accompanied by a decrease in pressure in the pulmonary artery, there was no decrease in symptoms during long-term therapy. It has been noted that calcium channel blockers may exacerbate right ventricular failure and should be used with caution. Calcium channel blockers are only effective in a small proportion of patients and have been superseded by newer drugs.

Vasoctive mediators and pharmacological treatment

As mentioned above, prostacyclin is an endogenous vasoactive mediator that promotes vasodilation, inhibition of platelet aggregation, and proliferation of vascular smooth muscle. Thromboxane has the opposite effect and worsens the course of pulmonary vascular disease. The ratio of prostacyclin to thromboxane is reduced in primary PH, the Eisenmenger complex, and in children with a left-to-right intracardiac shunt, and returns to normal after successful correction of the defect.

Prolonged infusion of prostocycline

Higenbottam et al. were the first to report the beneficial effects of continuous prostocycline infusion in patients with primary PH. There was an improvement in well-being, exercise tolerance and survival. Subsequently, these results were confirmed by other studies. The one-year survival rate of patients awaiting heart and lung transplantation increased by 66%. Interestingly, after 2 years of such treatment, no advantages over conventional therapy were noted, with the exception of more severe stages of the disease. In these patients, the long-term effect was not associated with the vasodilation that occurs at the beginning of a course of prolonged infusion of prostocycline. The effect is explained by mechanisms other than vasodilation, namely, inhibition of platelet aggregation and remodeling of the vascular wall.

Side effects in the form of headache, redness of the skin and abdominal pain are usually transient, lasting for 24 hours, but may reappear with an increase in dose.

Complications are mainly associated with a long standing venous catheter, pump malfunction. On average, a patient has two episodes of sepsis per year. If the infusion is interrupted, shortness of breath and loss of consciousness may occur. Over time, the need for prostacyclin and the need for dose adjustments to maintain normal cardiac output increases. However, prolonged intravenous administration of prostocycline significantly improves survival at 1, 2, and 3 years and is 88%, 76%, and 63%, respectively, which is significantly better than controls.

The leading factors determining the survival of patients are:

tolerance for physical activity;

functional class MUNA;

pressure in the right atrium;

direct vasodilating response to adenosine or inhaled NO.

After a year of treatment, cardiac output and mean pulmonary artery pressure become additional prognostic factors.

Continuous intravenous administration of prostocycline has revolutionized the chronic management of PH. However, the above disadvantages and complications are particularly burdensome in the treatment of children. Not surprisingly, many patients are reluctant to make decisions about such treatment. This stimulates the search for alternative methods of administration of prostocycline - aerosols, oral or subcutaneous analogues. Beraprost is an orally active prostocycline analogue that has been shown to be effective in both short-term and long-term treatment of PH. The efficacy of the oral analogue is comparable to that of intravenous prostocycline and is also maintained for 1 year. Adverse reactions - facial flushing, arthralgia, muscle pain, nausea or diarrhea - were noted quite often, however, serious complications associated with the catheter were excluded.

The inhaled aerosol form of prostocycline is comparable in efficacy to inhaled NO, however, their combination does not provide an additional answer. More favorable is the combination of inhaled iloprost with oral drugs such as bosentan or sildenafil.

Recent studies have opened up a promising alternative to the continuous intravenous administration of prostocycline in PH. The transfer of the human prostocyclin synthase gene to the liver of rats with monocrotaline-induced PH made it possible to achieve a high level of expression of the prostocyclin synthase gene in animal liver hepatocytes. As a result, the pressure in the pulmonary artery decreased from 88% to 60% relative to the systemic one, and the content of ET-1 in the lung tissue decreased by 2 times compared to the control. The survival rate of animals has increased significantly.

Inhalation NO

Inhaled NO is an instantaneous selective pulmonary vasodilator that improves intrapulmonary shunt fraction and has a short half-life. It is ideal for performing functional tests during catheterization and in neonates with persistent PH, who are mechanically ventilated in intensive care units, and in children after CHD surgery. It should be noted that, despite oxidative stress, manifested by increased lipid peroxidation in patients with PH, inhaled NO does not contribute to a further increase in the formation of peroxynitrites. Nitric oxide has become the method of choice in the treatment of pulmonary hypertensive crises in pediatric cardiac surgery. However, serious technical difficulties limit its practical application for the long-term treatment of patients with chronic PH.

Sildenafil

Sildenafil is a selective inhibitor of V-phosphodiesterase, which is an enzyme that degrades cGMP and thus limits NO-mediated vasodilation. The effect of inhibition of phosphodiesterase on the vessels of the penis and its use in the treatment of erectile dysfunction is well known. It is also known that there are high concentrations of the type V enzyme in the pulmonary vessels. Preliminary reports have shown that sildenafil may have a vasodilating effect in PH, in particular, attenuate the acute rise in pulmonary artery pressure after the cessation of NO inhalation, and may also be used as a drug for the treatment of chronic PH. Oral sildenafil abolishes hypoxic pulmonary vasoconstriction in humans. Sildenafil is well tolerated, available as an oral agent, and may be an alternative to prostacyclin, especially for patients whose symptoms do not justify continuous intravenous infusion. Sildenafil can act as an adjuvant in treatment with inhaled prostocycline or in combination with continuous inhaled NO. Sildenafil causes rapid and relatively selective pulmonary vasodilation, which is maintained for a sufficiently long time. The synergistic and additional action to prostocycline is due to an increase in the content of cAMP and cGMP. Interestingly, inhaled sildenafil reduces intrapulmonary shunting in animal studies and oral sildenafil reduces intrapulmonary shunting in patients with pulmonary fibrosis and secondary PH. Sildenafil is a selective pulmonary vasodilator, in contrast to other intravenous and oral drugs of the same purpose.

Blockade of ET receptors

Endothelin is a powerful vasoconstrictor that promotes the proliferation of vascular smooth muscle. There is evidence that an abnormally high level of circulating ET deepens vascular disorders in the lungs. Elevated ET, accompanied by a decrease in NO synthesis, is implicated in the pathophysiology of PH that occurs after cardiopulmonary bypass, persistent neonatal PH, and Eisenmenger's syndrome. Chronic prostocycline therapy in patients with primary PH improves pulmonary clearance of ET along with hemodynamic and clinical parameters. The action of ET is mediated through two types of receptors - ETA and ETP. ETA is present on smooth muscle cells and mediates vasoconstriction and proliferation, while the ETP receptor is found predominantly on endothelial cells. When ET binds to the ETV receptor, it causes vasorelaxation through the release of NO and prostocycline. This explains the paradox found in early work, in which ET infusion in healthy mammals caused pulmonary vasodilation even at doses that would normally result in systemic vasoconstriction. These data led to the idea of ​​an important role of endothelial cells in maintaining pulmonary vascular homeostasis. It is possible that ETA receptors predominate in the damaged pulmonary vascular bed. While it remains unclear, pharmacological agents should act on the ETA or ETV receptors. Non-selective blockade of ET receptors may reduce the beneficial effects of ET. However, the most promising ET receptor blocker acts on both types of receptors. Intravenous administration of bosentan reduces pulmonary artery pressure and resistance in patients with primary PH, but this effect is not selective. Despite the non-selective effect of intravenous bosentan, the tablet form of the drug in two placebo-controlled studies in patients with primary and secondary PH due to scleroderma improved physical performance, hemodynamics and alleviated symptoms.

In addition to the vasodilating effect, bosentan inhibits the development of fibrosis and proliferation. Its use contributes to an increase in life expectancy. According to data published in 2005, 86% of patients treated with bosentan survived the 3-year period, compared to 48% in the comparison group. The advantage of the drug is also its oral administration, eliminating the difficulties and complications of parenteral administration.

The drug is well tolerated, has no side effects, except for a dose-dependent increase in the level of pulmonary enzymes, which decreased to normal after 2-6 weeks. after its cancellation.

Anticoagulants

Follow-up of a group of patients for 15 years showed better survival of patients treated with warfarin compared to those who did not receive it. There is histological evidence for a role for vascular thrombosis in primary PH.

Knife atrioseptostomy

It is known that patients with Eisenmenger's syndrome and an open foramen ovale live longer than patients with an intact atrial septum. Taking this observation into account, a number of cardiologists have reported that they perform knife septostomy in patients with severe forms of PH.

Animal studies and experience with Fontan fenestration procedures show that atrial communication provides decompression of congested right hearts and maintenance of cardiac output at the cost of a drop in arterial oxygen saturation, while improving systemic oxygen delivery and reducing symptoms of right ventricular failure. Knife atrial septostomy increases cardiac output and systemic oxygen transport despite a drop in arterial oxygen saturation. Survival at 1, 2, and 3 years was 80%, 73%, and 65%, respectively, which is significantly better than the predicted survival curve derived from the New York Heart Association Primary PH Registry Equation.

Knife atrioseptostomy improves the condition of patients with syncope. The procedure involves some risk. Volume loading, elevated hematocrit, and inotropic support are recommended to prevent early mortality in the perioperative period. From a technical point of view, a gradual, in several steps, balloon dilatation of the septostomy is safer.

Lung transplant

Despite advances in the understanding of PH, lung transplantation is the last resort for patients who have exhausted treatment options. The number of children who have undergone transplantation is still small. The survival rate of children during the year is 73%. Mortality after transplantation is due to four factors:

cytomegalovirus infection;

obliterating bronchiolitis;

post-transplant lymphoproliferative disease;

bronchial stenosis.

Ten-year survival in children is 30-40%.

The timing of transplantation remains ambiguous. With effective drug therapy, expectant tactics are followed. Indications for transplantation are right ventricular failure or MUNA class IV when life expectancy is less than 6 months. Lack of effect from vasodilatory therapy, suprasystemic pulmonary artery pressure, syncope, or low cardiac output are signals to the transplant team. Simple quantitative hemodynamic criteria for survival in the natural course of the disease have also been developed. If the mean right atrial pressure times the PVR index is less than 160, survival is better than after lung transplantation.

Persistent PH in newborns

Persistent pulmonary hypertension of the newborn occurs in 1 in 1500 live births and is characterized by persistent PH and cyanosis due to a right-to-left shunt through the PDA or patent foramen ovale. There is no heart defect.

The causes of PLGN are divided into 3 groups depending on the structure of the pulmonary vascular bed:

Pronounced pulmonary vasoconstriction with a normally developed pulmonary vascular bed. May occur with perinatal asphyxia, meconium aspiration, circulatory shock, streptococcal pneumonia, increased blood viscosity, hypoglycemia, and hypocalcemia. Alveolar hypoxia and acidosis, vasoactive agents - thromboxane, vasoconstrictive prostaglandins, leukotrienes, endothelin play an important role in the mechanisms of vasocontraction.

Hypertrophy of the media of the pulmonary arterioles may develop with chronic fetal hypoxia, may be a consequence of the mother taking non-steroidal anti-inflammatory drugs during pregnancy.

Underdevelopment of the pulmonary arteries, accompanied by a decrease in the cross section of the pulmonary vascular bed in congenital diaphragmatic hernia and primary pulmonary hypoplasia.

Pulmonary hypertension of a functional nature is easily reversible when the causes that caused it are eliminated: PH of the 2nd group requires intensive treatment; Group 3 PH is usually irreversible.

PLGN is accompanied by a decrease in myocardial contractility and tricuspid insufficiency due to general or subendocardial myocardial ischemia. Hypoglycemia and hypocalcemia increase mocardial hypoxia.

Clinical manifestations in the form of cyanosis, tachypnea, groaning breathing with retraction begin 6-12 hours after birth. Complications in childbirth, anamnestic data on the mother taking non-steroidal anti-inflammatory drugs in the third trimester, help to suggest PLGN.

Characterized by an increased heart beat, a loud unsplit II tone, a gallop rhythm, a soft systolic murmur of tricuspid insufficiency, and in severe cases, hypotension.

Saturation of arterial blood in samples obtained from the umbilical artery is reduced with normal saturation in the preductal arteries. Sometimes there is a difference in the color of the upper and lower half of the body. With a large discharge of blood from right to left through the open oval window, there is no difference in saturation in the upper and lower arterial pools.

The ECG is usually normal for age, sometimes there is right ventricular overload or an abnormal T wave, indicating myocardial dysfunction.

On the radiograph, cardiomegaly, enhanced pulmonary pattern, atelectasis can be detected. However, these signs may be absent.

Echocardiography showed no signs of cyanotic malformation. The only find is a large PDA with right-left or bi-directional discharge. The right ventricle is dilated, the atrial septum bulges to the left, there is an open foramen ovale. The aortic arch is normal, with no evidence of aortic coarctation or rupture of the aortic arch. The left ventricle may be enlarged, with a reduced ejection fraction.

Catheterization is not usually indicated, but if the diagnosis is unclear or the patient is refractory, catheterization and arteriography are performed to avoid misdiagnosis.

Treatment has 3 goals:

decrease in PVR and pressure in the pulmonary artery by inhalation of oxygen, the creation of respiratory alkalosis and the use of pulmonary vasodilators;

correction of myocardial dysfunction;

stabilization of the patient and treatment of concomitant pathology.

Carry out general maintenance therapy: correction of hypoglycemia, hypocalcemia, hypomagnesemia, polycythemia. Body temperature is maintained within 36.5-37.2 0 С.

To achieve arterial pO 2 100 mm Hg. Art. carry out inhalation of 100% oxygen without intubation. If there is no effect, intubation is carried out with the creation of a positive airway pressure of 2-10 cm of water. Art. with spontaneous breathing.

If these measures are ineffective, mechanical ventilation is carried out to improve oxygenation and achieve respiratory alkalosis. The following ventilation mode is used: 100% oxygen concentration, respiratory rate 40-80 per minute, inspiratory pressure 40 cm of water. Art., positive expiratory pressure 4-10 cm of water. Art., the ratio of inhalation-exhalation time 1:1. The patient is relaxed. Upon reaching normal saturation of arterial blood with oxygen within 12-24 hours, a gradual weaning from the apparatus is carried out.

Vasodilators, as a rule, are nonspecific and dilate not only pulmonary, but also systemic resistive arterioles, so they have not been used recently.

The most effective method aimed at reducing PVR is the addition of small doses of gaseous NO, a selective pulmonary vasodilator, to the respiratory mixture. This effective method, widely used in Western medical institutions, has not yet been introduced in Ukraine and is at the stage of clinical testing.

Treatment of heart failure is carried out using conventional means: dopamine at a dose of 10 mg / kg / min by intravenous administration, dobutamine p-adrenergic agent at an initial dose of 5-8 mg / kg / min by continuous intravenous administration, digoxin for chronic congestive heart failure on later stage, diuretics.

Correction of acidosis, hypocalcemia, hypoglycemia helps to improve myocardial function.

In the arsenal of leading Western clinics in some severe cases of PLGN there is such an aggressive method as extracorporeal membrane oxygenation. However, the introduction of NO inhalation has limited its scope.

Forecast

With moderate PLGN, therapeutic actions are usually effective and the prognosis is favorable. Most newborns recover without pulmonary or neurological disease. Among patients requiring prolonged ventilation, survival is worse, bronchopulmonary dysplasia and other complications develop. With underdevelopment of the pulmonary vascular bed, patients are resistant to treatment and their prognosis is poor. Many show signs of underdevelopment of the central nervous system, the frequency of hearing loss is high. These complications are directly related to the degree of alkalosis, the duration of ventilation, the use of furosemide and aminoglycosides. In 80% of patients, there are deviations in the encephalogram and in 45% - cerebral strokes.

Eisenmenger syndrome in children

In 1897, Eisenmenger described the pathological findings in a 32-year-old man with a large VSD and PH. It was not until 60 years later, in 1958, that Wood gave a definitive definition of the disease that is consistent with our understanding of this clinical syndrome today. Wood used the term "Eisenmenger's syndrome" to describe patients with systemic pulmonary artery pressure due to high PVR and a right-to-left or bidirectional shunt at the level of the great vessels, the interventricular or interatrial septum. The term "Eisenmenger complex" is used when the underlying malformation is VSD.

With the development of cardiac surgery at an early age, the incidence of Eisenmenger's syndrome decreases. It is determined by the level of culture of the population and primary health care. Eisenmenger's syndrome occurs even at the age of 2 months.

Clinic

Typical signs of the disease are cyanosis, polycythemia, right ventricular failure. In patients with complex congenital malformations - OSA, AVSD, univentricular atrioventricular junction and transposition - the symptoms of Eisenmenger's syndrome develop early and worse prognosis. In patients with trisomy 21, the disease is also severe. Usually, symptoms progress slowly and are pronounced in adolescence and in adults. All patients have reduced physical performance.

Cyanosis first appears on exertion and then becomes permanent, reflecting the magnitude of the right-left shunt. Saturation of arterial blood with oxygen is 80-85%. A constant symptom in cyanotic patients is a thickening of the terminal phalanges of the fingers in the form of drumsticks. Hypertrophic osteoarthropathy may progress with arthralgia and articular synovitis.

Arterial hypoxemia is the cause of erythrocytosis. An increase in hemoglobin increases the oxygen capacity of the blood. The increase in blood viscosity associated with polycythemia does not manifest itself until the hemoglobin level does not exceed 18-20 g / l. Symptoms of increased blood viscosity:

headache;

dizziness;

visual impairment due to occlusion of the central retinal vein.

An increase in blood viscosity is a risk factor for thrombosis and cerebral hemorrhage. Due to thrombocytopenia, prolongation of clotting time, deficiency of coagulation factors and fibrinolysis, patients are prone to bleeding during surgery and tooth extraction. In 20% of patients, pulmonary hemorrhages occur as a result of ruptures of the bronchial arteries or aneurysms of the pulmonary arteries, which develop as a result of progressive dilatation of the central pulmonary arteries.

The cause of hemoptysis can be embolism and thrombosis of dilated pulmonary arteries.

Often observed uremia is due to increased production and reduced renal clearance of uric acid. Gout develops in 13-23% of patients. Increased erythropoiesis and destruction of erythrocytes leads to bilirubinemia and an increase in bilirubin in the bile, so cholelithiasis and cholecystitis are observed in 15% of patients. In 65% of patients, kidney dysfunction with proteinuria and the development of nephrotic syndrome with an increase in serum creatinine are noted. This serves as an additional factor that reduces survival.

Cerebral complications are characteristic: a stroke at the age of about 30 years and brain abscesses at 20-25 years. Rhythm disturbances in the form of supraventricular and ventricular extrasystole, flutter and atrial fibrillation are not uncommon. Every fifth patient has fainting and presyncope conditions associated with ventricular tachycardia.

Endocarditis occurs with a frequency of about 4%. Some patients present with hoarseness and cough associated with laryngeal nerve compression, dilated pulmonary arteries. Dilated pulmonary arteries can compress the left coronary artery with anginal pain. 30% of patients die suddenly. Although the presence of a shunt prolongs the life of patients with Eisenmenger's syndrome compared with patients with primary PH, 40-50% of them die from heart failure. The latter is especially common with complex underlying defects and is due to stenosis or insufficiency of the atrioventricular or semilunar valves.

Patients with Eisenmenger's syndrome need qualified management. About 20% of deaths are due to avoidable mistakes. Non-cardiac surgery is responsible for 24% of deaths. Venesections must be performed with caution. Patients should be warned about the risk of pregnancy, staying at altitude, taking estrogen, anesthesia.

The use of vasodilators and anticoagulants should be carried out under qualified supervision, taking into account the balance between pulmonary and systemic vascular resistance and the risk of bleeding and thrombosis. Pregnancy outcomes are usually unfavorable:

spontaneous abortion or preterm birth occurs in 25%;

therapeutic abortion - in 27%;

prematurity or low weight of the child - in 26%;

maternal death - in 16%;

deterioration of the mother's condition - in 54% of patients.

Conservative treatment is ineffective. However, recently there have been reports of partial preservation of pulmonary vascular reactivity and some regression of advanced OBLS, which has renewed interest in treatment with the latest generations of vasodilators. Gorenflo et al conducted a comparative study of the effectiveness of various vasodilators in children with CHD, PH and an average Wood index of 10 U/m 2 . Pulmonary artery pressure and PVR decreased in response to oxygen inhalation in 2 of 14 patients, to NO inhalation in 4 of 14 patients, and to intravenous prostacyclin supplementation with NO in 2 of 7 patients. Oxygen inhalation did not affect the level of vasoactive mediators. Nitric oxide at a dose of up to 80 ppm increased the cGMP level by an average of 2 times, but there was no relationship between the cGMP level and the hemodynamic response.

Rosenzweig et al used long-term prostacyclin infusion to alleviate symptoms of Eisenmenger's syndrome and showed a 20% reduction in mean pulmonary artery pressure, an increase in cardiac index from 3.5 to 5.9 L/min/m 2 , an improvement in functional class from 3.2 to 2.0, increased exercise capacity and oxygen delivery, but arterial oxygen saturation did not increase.

Closing of the VSD after pre-narrowing of the pulmonary artery in Eisenmenger's syndrome was first described in 1971 by Azzolina and has generated a lot of debate. The regression of medial hypertrophy and intimal proliferation after pressor unloading of pulmonary vessels were well documented experimentally and in the clinic, but it remained unclear whether far-reaching changes such as intimal fibrosis, fibrinoid necrosis, or plexiform lesions after narrowing of the pulmonary artery undergo regression. Interestingly, PVR may decrease after ductus arteriosus closure despite plexiform arteriopathy.

Nowick and co-authors proposed to close the VSD in patients with high pulmonary vascular resistance with a double patch-valve with a hole to ensure right-left shunting of blood when the pressure in the right ventricle rises above the systemic one. In 18 operated patients, vascular resistance averaged 11.4 U/m 2 , and all had cyanosis despite a predominantly left-to-right shunt. The publication did not provide data on pulmonary vascular reactivity, so it is difficult to assess the severity of obstructive vascular disease in these patients.

Lung transplant

Lung transplantation is rarely used in children with Eisenmenger syndrome. The results of these interventions in children and adults with CHD and PH are identical. Hospital mortality is 23%, 5-year survival - 57%. Lung transplantation without a heart transplant is possible in patients with ASD and PDA. In Eisenmenger complex, survival is better if the heart and lungs are transplanted at the same time, as opposed to lung-only transplantation and closure of the VSD. In adults, 1-, 5-, and 10-year survival rates are 73%, 51%, and 28%, respectively.

Despite the fact that the progress in the world of medicine is clear today, yet cardiac pathologies are among the first to significantly reduce the quality and life expectancy of a person. In addition, physicians are discovering relatively new varieties of heart disease. One of them is the Eisenmenger syndrome, which was discovered by the Austrian cardiologist and pediatrician Viktor Eisenmenger. Pathology is quite rare and not fully understood. In the article below, we will analyze what Eisenmenger's syndrome is, what its clinical picture is, and how pathology is treated in modern cardiology.

What is Eisenmenger's Syndrome?

Regarding Eisenmenger's disease, two terms are used - complex and syndrome. The term "complex" means the disease itself, and the term "syndrome" means a symptom complex (combination of symptoms) in a patient.

The Eisenmenger complex itself is a congenital anomalous structure of the heart, in which the interventricular septum has extensive and high defects. In addition, the patient has a right heart chamber and some abnormal origin of the aorta in the area of ​​both heart chambers. ICD pathology code - Q21.8 (other congenital cardiac anomalies).

Important: among patients with the Eisenmenger complex is noted in 10% of cases.

Causes of the development of pathology in the baby

A cardiac pathology of this type develops even in utero in the first 8 weeks of embryonic growth. It is during this period that the baby's heart is laid. Reliable and scientifically proven reasons for the development of the anomaly have not been identified. However, doctors tend to believe that it is the course of pregnancy and third-party effects on the body of the mother and fetus that provoke pathology. It is assumed that the following factors can influence the development and structure of the fetal heart:

• heredity (if there are heart defects in the family history of the baby's parents);
• taking certain medications during pregnancy by the expectant mother;
• toxic and physical effects on the mother's body (alcohol, salts of heavy metals, radiation exposure, magnetic effects, vibrations, etc.);
• viral infections carried by the mother in the first trimester of pregnancy.

Symptoms of heart disease

Eisenmenger's syndrome in children and adults may be asymptomatic in the case of normal blood flow. If hemodynamics occurs from right to left, then the defect is manifested by such signs and symptoms:

1. Explicit cyanosis of the upper body (cyanosis of the named zone).
2. Constant shortness of breath even in a state of relative rest.
3. Increased fatigue and severe physical weakness.
4. Constant aching pain in the chest.
5. Tachycardia and palpitations.
6. Frequent headaches.
7. Frequent pathological bleeding from the nose (including hemoptysis).
8. Visible swelling of the neck veins.

At the same time, the presence of the Eisenmenger complex in a person can also be determined by its appearance. Often such patients try to squat, thus alleviating the state of lack of oxygen. At the same time, in patients with the Eisenmenger complex, spinal deformity and a clear protrusion of the chest above the heart location zone are observed.

It is also worth noting here that although in general such people do not suffer from developmental delays, they nevertheless experience some visual impairment and speech disorders. Also, Eisenmenger's syndrome can be characterized by frequent pneumonia, SARS and bronchitis.

Important: with an asymptomatic course of the pathology, sudden death may occur against the background of ventricular fibrillation. Patients with an overt clinical picture may die from bleeding from the upper respiratory tract or from acute heart failure.

Diagnostics

Simply examining a newborn or adult patient and listening to heart sounds does not give the doctor a complete picture. To confirm the diagnosis, it is necessary to conduct a number of studies:

• Electrocardiogram (ECG). Allows the doctor to track the conduction of electrical impulses in the myocardium.
• X-ray of organs in the chest. It is necessary for assessing the parameters of the heart and its contours, as well as assessing the condition of the main vessels.
• Echocardiography (ultrasound of the heart). It makes it possible to see the work of the heart, blood vessels and valvular system. Detects hemodynamic disturbances.
• Cardiac catheterization. It makes it possible to assess the state of the heart in each of its departments.

Important: Eisenmenger's syndrome is often disguised as other diseases, which makes it difficult to diagnose and takes precious time from the patient's life. For example, frequent bleeding can be mistaken for von Willebrand disease.

Treatment of cardiac pathology

There is no cure for Eisenmenger's syndrome. All drug therapy is aimed at relieving symptoms and maintaining an optimal quality of life for the patient. Also, therapy includes preventive measures that are aimed at preventing possible complications such as high pulmonary hypertension, etc. Basically, drug therapy is carried out either at the stage of preparation for surgery, or if it is impossible to carry it out. The patient is prescribed a number of medications that allow him to maintain his condition at a more or less normal level:

• Phosphodiesterase type 5 inhibitors. They give the opportunity to act on all smooth muscle tissues of blood vessels.
• Prostacyclins. They reduce pressure in the area of ​​the pulmonary artery, which significantly improves oxygen consumption by the muscles. In addition, such drugs prevent damage to the pulmonary vessels.
• Anticoagulants. Prevent blood clotting and thrombosis.
• Antiarrhythmic drugs. Prevent arrhythmia and stabilize the heart rhythm.
• Endothelin receptor antagonists. Assign to maintain the functioning of the vessels of the lungs. But these drugs can have a negative effect on the heart. Therefore, they are used infrequently and for a short time.

An operation for this disease is done either with the aim of setting a pacemaker, which normalizes the patient's heart rhythm, or with the aim of eliminating it. Particularly good chances for life are given by the second type of surgery at an early age.

Important: with the low efficiency of all the above methods, the patient is shown a transplant of the lungs and heart. The prognosis in this case is quite favorable.

Prevention

As for preventive measures, everything here depends on genetics and on the mother herself. And if genetics is not defeated, then the expectant mother can do everything in her power to prevent the possible abnormal structure of the fetal heart. To do this, during pregnancy, it is necessary to refrain from self-medication, protect yourself from viral infections and eat right. In addition, it is desirable to avoid places with possible toxic effects on the body (chemical industry enterprises, radiation zones, etc.).

It is important to understand that Eisenmenger's syndrome is a pathology that not only reduces the patient's quality of life, but also takes away a significant part of it from him. Therefore, the main task of a pregnant woman is to do everything so that the baby is born healthy and strong.

Vice is compatible with uterine life. After birth, the foramen ovale and ductus arteriosus close normally. Venous blood flows from the right atrium into the right ventricle. From here, most of the blood is ejected into the pulmonary artery and lungs, from where it returns through the pulmonary veins to the left atrium. Since the aorta "sits on top" of the ventricular septal defect, some of the blood from the right ventricle is ejected into the aorta. With this defect, the pressure in the pulmonary artery increases significantly. It can reach the pressure in the aorta or even exceed it. An increase in pressure in the pulmonary circulation is explained by an increase in resistance in the pulmonary arterioles (the minute volume of blood in the pulmonary circle does not increase, it is normal or, more often, reduced). It can be equal to the value of resistance in the systemic circulation. This is an expedient compensatory reaction that protects the pulmonary circulation from hypervolemia, and the patient from pulmonary edema. The mechanism of pressure increase in the pulmonary circulation is not clear. Assumptions are made about spasm of arterioles, about anatomical changes in the structure of arterioles. The latter is more likely, as it is confirmed by histological examination.

Violation of blood circulation in the Eisenmenger complex leads to hypertrophy of the right ventricle. Sooner or later, cyanosis appears. This is due to venous-arterial shunting. The volume of venous blood discharged into the aorta depends on several factors: the size of the ventricular septal defect, the degree of dextroposition of the aorta, the ratio of systolic pressure in the right ventricle and aorta. The volume of the veno-arterial shunt increases with the increase in pulmonary hypertension.

From a pathological and physiological point of view, we can talk about two periods in the development of the Eisenmenger complex: the stage without cyanosis and the stage with cyanosis. In the first stage, the oxygen saturation of the peripheral arterial blood is normal or almost normal, since shunting goes from left to right, i.e., according to the arterial-venous type (due to lower vascular resistance in the pulmonary circulation compared to the large one). In the second, Dianetic, stage, the oxygen saturation of the peripheral arterial blood is reduced, since shunting goes from right to left, i.e., along a veno-arterial shunt (due to higher vascular resistance in the pulmonary circulation compared to the large one).

Pathological anatomy.

The position of the aorta "riding" on the ventricle is usually moderate. The defect, located in the membranous part of the interventricular septum, is large (usually 1.5-3 cm in diameter). The size of the pulmonary artery is always normal; in most cases, the trunk and branches are sometimes significantly enlarged. Pronounced morphological changes in the pulmonary arterioles are observed: thickening of the muscles of the media with a decrease in the lumen of the vessel. These changes are associated with the absence of reverse development during extrauterine life, when the lungs begin to function, and the remaining intrauterine type of blood circulation in the lungs. In the future, secondary changes develop in the arterioles (secondary fibrous thickening of the intima), due to increased pressure in the pulmonary artery.

The right ventricle and right atrium are hypertrophied and dilated. The left ventricle is not enlarged. In 25-20% of patients, a right-sided aortic arch is observed.

Clinic and symptoms of the Eisenmenger complex

The clinic is quite diverse. Among congenital heart defects accompanied by cyanosis, the Eisenmenger complex is better tolerated than other anomalies. If the pulmonary artery is not dilated, there is no cyanosis. With its expansion, observed in most patients, cyanosis appears during puberty or in early youth. Sometimes, however, cyanosis is noted already from birth or appears during the first two years of life and for the most part no later than 10 years. In any case, late than early cyanosis is more characteristic of the Eisenmenger complex (unlike Fallot's tetrad, in which cyanosis appears earlier). The time of occurrence of cyanosis depends on the degree of dextroposition of the aorta. The more pronounced it is, the earlier and more significant cyanosis. Cyanosis for a long time can be moderate. It would be constant or appear only during physical exertion. With the course of the disease, cyanosis gradually increases and is most pronounced before death.

Polycythemia is usually mild; fingers in the form of drumsticks are not always observed and are a rather late symptom. They are not as pronounced as in Fallot's tetrad, since after the appearance of cyanosis, death occurs after a relatively short period of time.

Frequent complaints of shortness of breath, general weakness and fatigue. There is also a headache, a tendency to fainting. Shortness of breath with the advent of cyanosis becomes more pronounced, although it is not too painful. There is relief in the squatting position. Respiratory tract infections are frequent. Frequent hemoptysis and nosebleeds. Compression of the pulmonary artery on the left recurrent nerve can lead to hoarseness. Physical development and growth suffer only in the case of early and prolonged (since childhood) cyanosis.

In the region of the heart, a cardiac hump is visible, sometimes a pulsation to the left of the sternum. Cardiac dullness is generally slightly dilated or unchanged. A mostly loud and rough systolic murmur of a ventricular septal defect is heard, which is accompanied by a cat's purr. The epicenter of the noise in the third - fourth intercostal space on the left at the edge of the sternum. Here, a diastolic murmur of relative insufficiency of the pulmonary valve (Graham-Still murmur) can also be heard, usually limited to only the specified area, soft. However, it should be borne in mind that diastolic murmur of aortic insufficiency (rough, accompanied by peripheral symptoms) and diastolic murmur (as part of a continuous systolic-diastolic murmur) of the patent ductus arteriosus can be heard in the region of the heart, with which the Eisenmenger complex is often combined. Above the pulmonary artery, the accent of the 2nd tone is expressed. There is a positive ether test when the clinical veno-arterial shunt is not yet detected.

The radiographic picture is different.

The size of the cardiac shadow is normal or slightly enlarged with a slight protrusion of the arch of the pulmonary artery trunk. In rare cases, the shadow takes the form of a wooden shoe. The shadows of the branches of the pulmonary artery are also without features. Suspicion of the presence of a defect can only be caused by a lively pulsation of the shadows of the pulmonary roots. In most cases, nevertheless, the cardiac shadow is moderately enlarged due to the right ventricle, and sometimes the right atrium. The arch of the pulmonary artery is distinctly bulging and pulsating. The cardiac shadow is often spherical in shape. The branches of the pulmonary artery in the roots of the lungs are dilated and pulsate, the vascular pattern of the lungs is enhanced.

In some cases, all sizes of the heart are enlarged and it is impossible to determine at the expense of which departments. The pulmonary artery is aneurysmically dilated, the roots of the lungs, large branches of the pulmonary artery pulsate. However, the pulsation is not traced to the periphery of the lungs, as is observed with a significant atrial septal defect.

Electrocardiographic data are not specific, often do not differ from the norm. The electrical axis of the heart may be in the normal position, but usually it is deviated to the right, a vertical or semi-vertical position according to Wilson is noted. The prong may be enlarged R, but most of the time it's normal. Signs of overload of the right or both ventricles are often expressed, sometimes lengthening of the interval is recorded R-Q, blockade of the right leg of the bundle of His.

Significant help in the diagnosis are the results of cardiac sounding, cardiomanometry and angiocardiography. Systolic pressure in the right ventricle and pulmonary artery is increased, in the right atrium and pulmonary capillaries is normal. With an increase in pulmonary hypertension, diastolic pressure in the pulmonary arterioles increases. The saturation of blood with oxygen in the right ventricle is greater than in the right atrium. The contrast mass introduced into the right ventricle simultaneously fills the aorta and the pulmonary artery, the pulmonary artery is dilated, the contrast mass is delayed in it and its branches, the contrasting of the left half of the heart is delayed and less pronounced than normal.

Complications

Complications of the Eisenmenger complex: bacterial endocarditis, catarrh of the upper respiratory tract, embolism, including paradoxical ones, to the brain.

Differential diagnosis of defect

If there is no cyanosis, it is necessary to differentiate the Eisenmenger complex and congenital heart defects, in which there is an expansion of the trunk and branches of the pulmonary artery (pure pulmonary artery stenosis, patent ductus arteriosus, significant ventricular defect and atrial septal defect).

An uncomplicated isolated patent ductus arteriosus is easy to rule out. Difficulties arise in cases of a combination of an open arterial duct with pulmonary hypertension and a veno-arterial shunt. An atrial septal defect, especially when combined with cyanosis, gives a clinical and radiological picture similar to the Eisenmenger complex. However, cyanosis in atrial septal defect is usually intermittent. The Eisenmenger complex is most difficult to distinguish from an isolated large high ventricular septal defect with severe pulmonary hypertension. The main distinguishing feature is the dextroposition of the aorta in the Eisenmenger complex, however, with a moderate dextroposition of the aorta and a change in the predominant arterial-venous shunt to a veno-arterial shunt, it is almost impossible to distinguish these two diseases clinically in vivo.

The Eisenmenger complex with cyanosis must be distinguished primarily from Fallot's tetrad. However, with the Eisenmenger complex, there are no such pronounced cyanosis, "drum fingers" and polycythemia, there is an expansion and a sharp pulsation of the trunk and branches of the pulmonary artery. In all cases that are doubtful and difficult for differential diagnosis, catheterization of the cavities of the heart, pulmonary artery and aorta, measurement of pressure in them, examination of blood oxygen saturation, and angiocardiography are performed.

Treatment of the Eisenmenger complex

An operation to close the defect, undertaken before the development of persistent hypertension of the pulmonary circulation and irreversible changes in the wall of the pulmonary arteries, is effective.

The prognosis is doubtfully favorable until cyanosis develops. The form with late cyanosis is more favorable, although rarely patients live more than 10 years from the time of the onset of overt cyanosis. Death in most cases occurs at the age of 20-30 years as a result of right heart failure. The form with early cyanosis proceeds, as a rule, unfavorably from the very beginning. Patients die, usually before reaching adulthood. Despite the fact that this defect lives longer than other congenital malformations with cyanosis, the average life expectancy is about 25 years.