ICD 10 patent ductus arteriosus. Patent ductus arteriosus. Q24.3 Infundibular stenosis of the pulmonary valve

RCHR (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical protocols of the Ministry of Health of the Republic of Kazakhstan - 2018

Patent ductus arteriosus (Q25.0)

Children's cardiac surgery, Pediatrics

general information

Short description

Approved
Joint Commission on Healthcare Quality
Ministry of Health of the Republic of Kazakhstan
from April 18, 2019
Protocol No. 62

Arterial duct - duct that provides fetal blood circulation in antenatal life. In full-term newborns, functional closure of the ductus arteriosus occurs in the first 10-15 hours after birth, anatomical closure - within 2-3 weeks. Typical localization is on the left side of the aorta. It starts from the junction of the aortic isthmus with the descending aorta and flows into the bifurcation area at the mouth of the left pulmonary artery. Other options for the location of the PDA are possible. as a rule, combined with various malformations of the cardiovascular system.

INTRODUCTORY PART

Protocol name: Patent ductus arteriosus

ICD code(s):

Date of protocol development/revision: 2013 (revised in 2018)

Abbreviations used in the protocol:

Classification

Angiographic classification:

• type A - the narrowest point of the duct is its pulmonary part, there is a well-differentiated aortic ampulla;
• type B - short duct, narrowest in the aortic part;
• type C - tubular structure of the duct without narrowing;
• type D - the duct has multiple narrowings;
• type E - a difficult-to-define configuration of an elongated conical shape with a stenotic part.

Diagnostics

DIAGNOSTIC METHODS, APPROACHES AND PROCEDURES

Diagnostic criteria

Complaints and anamnesis:
For babies with PDA is typical: shortness of breath, tachycardia, tachypnea, malnutrition, low weight gain.

In older children the presence of shortness of breath during physical activity, retardation in physical development, and frequent respiratory diseases prevail.
The clinical presentation of a PDA depends on the size of the PDA, the age of the child, and pulmonary vascular resistance.

Table 1 - Clinical symptoms of PDA

Physical examination:
Visual inspection: increased cardiac impulse, systolic trembling along the upper edge of the sternum on the left, high and rapid pulse.
Auscultation: the second tone is normal or increased over the area of ​​the pulmonary artery with the development of pulmonary hypertension. Systole-diastolic “machine” noise with maximum amplification on the left in the subclavian region and along the upper edge of the sternum. Increasing systolic murmur at point 3 (typical for newborns and with the development of pulmonary hypertension).

Laboratory research: NT-proBNP: Elevated levels of natriuretic propeptide in the presence of symptoms of heart failure.

• ECG: in older patients, hypertrophy of the left parts may occur; with a large PDA, combined hypertrophy of both parts of the heart is possible; with the development of PH, there are signs of hypertrophy of the right parts of the heart.
• cardiomegaly and enrichment of the pulmonary vascular pattern; with the development of PH, normal heart sizes with bulging of the pulmonary arch.
• Echocardiography (transthoracic and transesophageal): visualization of the PDA and associated anomalies, color Doppler examination allows one to determine the diameter and direction of the shunt, enlargement of the left chambers of the heart and relative mitral insufficiency (“mitralization” of the defect) - indirect signs of PDA.
• CT angiography/MRI of the thoracic aorta - according to indications.
• - according to indications: in infants it is performed extremely rarely; in older patients it is used as a one-step diagnostic and therapeutic procedure for installing an occluder.

1. Throat swab for patflora
2. HBsAg, antibodies to hepatitis B, C (ELISA)
3. Blood test for HIV
4. General urine analysis
5. General blood test (6 parameters)
6. Determination of total protein, glucose, creatinine, urea, ALT, AST, bilirubin - according to indications
7. TSH, T3, T4 - for Down syndrome (trisomy 21)

1. X-ray of the chest organs in one projection
2. Echocardiography
3. ECG

1. Consultation with a pediatric ENT doctor to exclude foci of chronic infection
2. Consultation with a pediatric dentist to exclude foci of chronic infection
3. Consultation with a pediatric cardiologist to clarify the diagnosis and prescribe conservative therapy

1. Testing for the causative agent of salmonellosis, dysentery, and typhoid fever
2. Examination of stool for helminth eggs
3. Microreaction or Wasserman reaction (RW)

1. Fluorography

1. General urine analysis
2. General blood analysis
3. Biochemical blood test (determination of total protein, glucose, electrolytes, ALT, AST, C-reactive protein, urea, creatinine, bilirubin)
4. Coagulogram (prothrombin time, fibrinogen, fibrinogen, INR, APTT, platelet aggregation)
5. Determination of blood group and Rh factor
6. Microbiological examination (throat swab), sensitivity to antibiotics

1. ECG
2. Echocardiography (transthoracic)
3. Plain radiography of the chest organs

Differential diagnosis

The main diagnostic method for PDA verification is transthoracic echocardiography. The same research method is the main one for making a differential diagnosis between the clinical diagnoses given below. To clarify the diagnosis, according to indications, transesophageal echocardiography, CT angiography, cardiac MRI, and catheterization of the cardiac cavities should be performed.

Treatment abroad

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Treatment

Treatment (outpatient clinic)

OUTPATIENT TREATMENT TACTICS

Preoperative preparation at the prehospital stage should include the following aspects:

• Prescription of conservative therapy.
• Preoperative examination aimed at identifying concomitant pathologies that may be a contraindication for elective surgery.
• Detection and treatment of intrauterine infection in patients with chronic leukocytosis and low-grade fever.
• Sanitation of foci of chronic infection (sanitation of the oral cavity).
• Assessment of patient transportability.
• Preparation of documents for registration on the portal of the hospitalization bureau.

• general mode;
• diet table No. 10, breastfeeding, artificial feeding.

Treatment (inpatient)

TREATMENT TACTICS AT THE INPATIENT LEVEL
Determination of operability criteria, exclusion of concomitant pathology that prevents surgical intervention, prevention of complications in the postoperative period. If possible, immediate radical correction of the defect. Preoperative preparation: additional examination, establishing an accurate diagnosis, choosing a method of surgical treatment, performing an operation, postoperative management, selection of conservative therapy.

Non-drug treatment:
Mode: bed; crib
Diet: table No. 10; breastfeeding, artificial feeding.

Drug treatment: see clause 3.2

Other types of treatment:

• Closure of an arterial defect with an occluder.
• Newborns: indomethacin 0.2 mg/kg 3 times a day intravenously over 20 minutes. Occlusion of the PDA is achieved in 80% of cases. Contraindications are the presence of hemorrhagic syndrome, sepsis, and renal failure.

• Ligation of a large/medium-sized PDA with the presence of congestive heart failure and pulmonary hypertension: correction in the early stages (at the age of 3-6 months) ( ClassI).
• Average PDA without congestive heart failure: correction at the age of 6-12 months ( ClassI). If there is a delay in physical development, correction can be carried out at an earlier date (by lassIIA).
• Small PDA: correction at the age of 12-18 months ( ClassI).
• "Silent PDA": closure is not recommended ( ClassIII).

• Endovascular occlusion or ligation in children aged >6 months. Ligation and ligation with intersection and suturing in children aged<6 месяцев. Эндоваскулярная окклюзия у детей в возрасте <6 месяцев (кlassIIb). Treatment with indomethacin/ibuprofen should not be used in full-term infants ( ClassIII).

• Endoscopic clipping of PDA.

• normalization of intracardiac hemodynamics;
• disappearance of symptoms of heart failure;
• no signs of inflammation;
• primary healing of a postoperative wound;
• absence of shunt at the level of the PDA according to echocardiography;
• decrease in the level of natriuretic propeptide.

Hospitalization

INDICATIONS FOR HOSPITALIZATION, INDICATING THE TYPE OF HOSPITALIZATION

Indications for planned hospitalization:

• The presence of congenital heart disease with hemodynamic disturbances.

• Presence of congenital heart disease with heart failure, uncontrolled drug therapy

Information

Sources and literature

1. Minutes of meetings of the Joint Commission on the Quality of Medical Services of the Ministry of Health of the Republic of Kazakhstan, 2018
   1. 1) OhlssonA., Walia R., Shah S.S. Ibuprofen for the treatment of patent ductus arteriosus in preterm and/or low birth weight infants // Cochrane Datebase Syst. Rev. – 2013. 2) Karpova A.L. and others. Experience of drug closure of the hemodynamically significant patent ductus arteriosus in premature newborns at the regional level / Neonatology, - 2013, No. 2., - P.43-48. 3) Tefft R.G. The impact of an early Ibuprofen treatment protocol on the incidence of surgical ligation of the ductus arteriosus // Amer. J. Perinatol. – 2010/ - Vol. 27(1). – P.83-90. 4) FanosV., Pusceddu M., Dessi A. et al. Should we definitively abandon prophylaxis for patent ductus arteriosus in preterm newborns? CLINICS. – 2011. Vol. 66 (12). – P. 2141-2149. 5) Yuh D.D., Vricella L.A., Yang S.C., Doty J.R. Johns Hopkins Textbook of Cardiothoracic Surgery. – 2nd ed. 2014. 6) Kouchoukos N.T., Blackstone E.H., Hanley F.L., Kirklin J.K. Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. – 4th ed. Philadelphia: Elsevier; 2013.

Information

ORGANIZATIONAL ASPECTS OF THE PROTOCOL

List of protocol developers with qualification information:

1. Gorbunov Dmitry Valerievich - head of the children's cardiac surgery department of the National Scientific Cardiac Surgery Center, cardiac surgeon.
2. Ibraev Talgat Ergalievich - head of the department of anesthesiology and resuscitation (children) of the National Scientific Cardiac Surgery Center, anesthesiologist-resuscitator.
3. Utegenov Galymzhan Malikovich is a cardiac surgeon (pediatric) at the Department of Pediatric Cardiac Surgery of the National Scientific Cardiac Surgery Center.
4. Litvinova Liya Ravilyevna - clinical pharmacologist of the National Scientific Cardiac Surgery Center.

Disclosure of no conflict of interest: No.

List previewers:

1. Abzaliev Kuat Bayandiyevich - Doctor of Medical Sciences, Associate Professor, Head of the Department of Cardiac Surgery of JSC “Kazakh Medical University of Continuing Education”, independent accredited expert of the Republic of Kazakhstan.

Conditions for reviewing the protocol:review of the protocol 5 years after its publication and from the date of its entry into force or if new methods with a level of evidence are available.

Attached files

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PA - pulmonary artery

MRI - magnetic resonance imaging

NSAIDs - non-steroidal anti-inflammatory drugs

PDA - patent ductus arteriosus

TPR - total pulmonary resistance

ECG-electrocardiography

EchoCG - echocardiography

Terms and Definitions

Cardiac catheterization– an invasive procedure performed for therapeutic or diagnostic purposes for pathology of the cardiovascular system.

Pulse pressure- the difference between systolic and diastolic pressure.

Endocarditis- inflammation of the inner lining of the heart, is a common manifestation of other diseases.

Echocardiography- an ultrasound research method aimed at studying the morphological and functional changes of the heart and its valve apparatus.

1. Brief information

1.1. Definition

Patent ductus arteriosus (PDA)- a vessel through which the pathological communication between the aorta and the pulmonary artery (PA) remains after birth.

1.2 Etiology and pathogenesis

Normally, the PDA is necessarily present in the fetus, but closes soon after birth, turning into an arterial ligament. Risk factors for patent ductus arteriosus are premature birth and prematurity, family history, the presence of other congenital heart diseases, infectious and somatic diseases of the pregnant woman.

1.3 Epidemiology

The exact frequency of occurrence of the defect is unknown, since it is unclear at what point a patent ductus arteriosus should be considered a pathology. It is conventionally believed that normally it should close within the first or two weeks of life. PDA usually occurs in premature infants and is extremely rare in infants born at term. Under these criteria, the frequency of isolated pathology is about 0.14-0.3/1000 live births, 7% among all congenital heart defects (CHD) and 3% among critical congenital heart defects. Persistence of the duct largely depends on the degree of term of the child: the lower the weight, the more common this pathology is.

The average life expectancy of patients with PDA is approximately 40 years. 20% of patients die before 30 years of age, 42% die before 45 years of age, and 60% die before 60 years of age. The main causes of death are heart failure, bacterial endocarditis (endarteritis), development and rupture of ductal aneurysm.

1.4 Coding according to ICD 10

Congenital anomalies of large arteries (Q25):

Q25.0 – Patent ductus arteriosus.

1.5. Classification

Taking into account the level of pressure in the pulmonary artery, 4 degrees of defect are distinguished:

• pressure in the pulmonary artery (PA) in systole does not exceed 40% of arterial pressure;
• PA pressure is 40-75% of arterial pressure (moderate pulmonary hypertension);
• pressure in the PA is more than 75% of the arterial pressure (severe pulmonary hypertension with preservation of the left-right shunt);
• the pressure in the PA is equal to or exceeds the systemic one (severe pulmonary hypertension, which leads to the occurrence of right-to-left shunting).

In the natural course of the patent ductus arteriosus there are 3 stages:

• Stage I primary adaptation (the first 2-3 years of a child’s life). Characterized by clinical manifestation of patent ductus arteriosus; often accompanied by the development of critical conditions, which in 20% of cases result in death without timely cardiac surgery.
• Stage II relative compensation (from 2-3 years to 20 years). It is characterized by the development and long-term existence of pulmonary hypervolemia, relative stenosis of the left atrioventricular orifice, and systolic overload of the right ventricle.
• Stage III sclerotic changes in the pulmonary vessels. The further natural course of the patent ductus arteriosus is accompanied by a restructuring of the pulmonary capillaries and arterioles with the development of irreversible sclerotic changes in them. At this stage, the clinical manifestations of patent ductus arteriosus are gradually replaced by symptoms of pulmonary hypertension.

2. Diagnostics

• It is recommended to carry out differential diagnosis with aortopulmonary septal defect, truncus arteriosus, large aortopulmonary collateral arteries, coronary pulmonary fistulas, sinus of Valsalva rupture and VSD with aortic insufficiency.

Comments:In the presence of severe pulmonary hypertension, the number of defects with which PDA has to be differentiated increases significantly; These include almost all congenital defects that occur with hypervolemia in the pulmonary circulation and can be complicated by the sclerotic form of pulmonary hypertension.

2.1. Complaints and anamnesis

• When collecting anamnesis, it is recommended to ask about family history, infectious and somatic diseases.

• When collecting complaints from a child patient, it is recommended to ask their parents about shortness of breath, fatigue that occurs during physical activity, and frequent infectious diseases of the lungs.

Comments:

• When collecting complaints from an adult patient, it is recommended to ask them about palpitations, feelings of interruptions in heart function, and a tendency to infectious diseases of the lungs.

Comments:Complaints from patients with PDA are nonspecific. Clinical manifestations depend on the size of the duct and the stage of hemodynamic disorders. The course of the defect varies from asymptomatic to extremely severe. With large duct sizes, the latter manifests itself already from the first weeks of life with signs of heart failure and retardation in physical development. In young children, when screaming (or straining), cyanosis may appear, which is more pronounced on the lower half of the body, especially on the lower extremities. It is typical that cyanosis disappears after the load is stopped. Persistent cyanosis occurs only in adults and is a sign of reverse blood discharge due to the sclerotic form of pulmonary hypertension.

2.2 Physical examination

• It is recommended to perform cardiac auscultation.

Comments:Auscultation reveals a systole-diastolic (“machine”) noise characteristic of the defect in the second or third intercostal space to the left of the sternum, radiating into the interscapular space and vessels of the neck. The strengthening of the second tone above the pulmonary artery has diagnostic significance. In most cases, the tone is not only amplified, but also split. Moreover, its second, pulmonary component is especially emphasized. By the intensity of its increase, one can get an idea of ​​the degree of pulmonary hypertension.

• It is recommended to measure your blood pressure.

Comments:Due to blood leakage from the aorta into the pulmonary artery, diastolic pressure decreases (sometimes to zero) and pulse pressure increases.

2.3 Laboratory diagnostics

There is no specific laboratory diagnosis for PDA.

• It is recommended that when a patient with a PDA is admitted to a specialized hospital for surgical treatment of the defect, his blood type and Rh factor are determined, then a blood sample is selected.

2.4 Instrumental diagnostics

• It is recommended to make a more precise diagnosis of PDA using imaging studies that demonstrate the presence of blood discharge through the aortopulmonary communication (with or without evidence of significant volume overload of the left heart).

Comments:In a patient with suspected PDA, diagnosis should be aimed at determining the presence and size of the aortopulmonary communication, functional changes in the left atrium and left ventricle, pulmonary circulation, as well as the presence of any concomitant defect.

• It is recommended to perform transthoracic echocardiography (EchoCG) using color Doppler mapping mode.

Comments:When conducting a study in the parasternal plane along the short axis, the PDA is clearly visualized.

• Cardiac catheterization with angiography is recommended to identify associated cardiac abnormalities and in patients with suspected pulmonary hypertension.

Comments:Cardiac catheterization makes it possible to assess the magnitude of the discharge, its direction, total pulmonary resistance (TPR) and the reactivity of the vascular bed. Angiography allows you to determine the size and shape of the duct.

• MRI is recommended when additional information about the anatomy and morphology of blood vessels is needed.

• A chest x-ray is recommended.

Comments:A plain X-ray of the chest shows an increase in the shadow of the heart due to the expansion of first the left and then both ventricles and the left atrium, bulging of the pulmonary artery and increased vascular pattern. In the absence of hypertension, there may be no changes on the x-ray. With a high OLR, due to the development of sclerotic changes in the pulmonary vessels and a decrease in the volume of discharge, the size of the heart decreases.

• It is recommended to perform electrocardiography.

Comments:In the absence of high hypertension, the ECG may show signs of left ventricular hypertrophy. In the hypervolemic form of hypertension there may be signs of hypertrophy of the left and right ventricles; in the sclerotic stage, signs of right ventricular hypertrophy come to the fore.

2.5 Other diagnostics

3. Treatment

3.1 Conservative treatment

• The use of non-steroidal anti-inflammatory drugs (NSAIDs) is recommended.

Comments:NSAID therapy (indomethacin, ibuprofen**), started in the first days after birth, leads to a decrease and even closure of the duct. With enteral use of the drug, closure of the PDA occurs in 18-20%, and with intravenous administration in 88-90% of cases. Indomethacin is administered intravenously at a rate of 0.2 mg/kg/day for 2-3 days. Contraindications to treatment are renal failure, enterocolitis, blood coagulation disorders and bilirubinemia above 0.1 g/l.

• Patients with PDA complicated by bacterial endocarditis are recommended to undergo a course of antibiotic therapy.

Comments:PDA complicated by bacterial endocarditis and endarteritis or heart failure is currently successfully operated on after appropriate treatment.

• Drug therapy for pulmonary hypertension is recommended only for patients who have irreversible pulmonary hypertension.

3.2 Surgical treatment

• Surgical correction of PDA is recommended to be performed by surgeons with experience in treating congenital heart disease.

Comments:In cases where a PDA is combined with other congenital heart defects requiring surgical correction, the duct can be closed during the main operation.

• Surgical closure of the PDA is recommended when there is overload of the left side of the heart and/or signs of pulmonary hypertension in the presence of left-to-right shunting, as well as after previous endocarditis.

Comments:The optimal patient age for surgery is 2-5 years. However, with a complicated course of the disease, age is not a contraindication to surgery. Currently, most surgeons use the method of ligating the duct with a double ligature or clipping the vessel. There is no early mortality. Recanalization of the duct is rare. Complications may be associated with damage to the laryngeal or phrenic nerves and/or intrathoracic lymphatic duct. Long-term results of surgical treatment of PDA show that timely surgery allows for complete recovery. In patients with R Affected by pulmonary hypertension, the result of the operation depends on the reversibility of structural and functional changes in the pulmonary vessels and myocardium.

• Surgical removal of a PDA is not recommended for patients with pulmonary hypertension and right-to-left shunting.

3.3. Other treatment

• Endovascular closure of the PDA is recommended for overload of the left heart and/or signs of pulmonary hypertension in the presence of left-to-right shunting, as well as after previous endocarditis.

Comments:Contraindications to endovascular closure of a PDA are early childhood (up to 3 years) and the child’s low body weight.

• Endovascular closure of asymptomatic small PDA is recommended.

• Endovascular closure of the PDA is not recommended in patients with pulmonary hypertension and right-to-left shunting.

Comments:Complications that occur with percutaneous closure of a PDA include displacement of the implant with embolization of a vessel (mainly a branch of the pulmonary artery) or absence of a pulse, more often in young children.

4. Rehabilitation

• For 1-3 months after surgery, the patient is recommended to undergo rehabilitation treatment with limited physical activity.

5. Prevention and clinical observation

• It is recommended that a patient with a corrected PDA be monitored by a cardiovascular surgeon with follow-up examinations at least once every 6 months in the absence of hemodynamic disturbances.

• To make a decision on removing a patient from the dispensary register, it is recommended to perform an EchoCG and ECG.

Comments:EchoCG control is performed after 1, 3, 6, 12 months. after surgical treatment.

• It is recommended that a cardiovascular surgeon conduct follow-up examinations of a patient with a small PDA without signs of left heart overload at least once every 1-2 years.

6. Additional information affecting the course and outcome of the disease

• It is recommended to identify the defect in a timely manner, provide appropriate care for the child with a PDA, and perform optimal surgical intervention on time.

• Prevention of endocarditis is recommended for unoperated patients with PDA, as well as operated patients in the first 6 months after surgical treatment.

Criteria for assessing the quality of medical care

Bibliography

1. Sharykin A.S. Congenital heart defects. M.: Teremok; 2005.
2. Hoffman D. Pediatric cardiology. M.: Practice; 2006.
3. Burakovsky V.I., Bockeria L.A. Cardiovascular surgery. M.: Medicine; 1996.
4. Zinkovsky M.F. Congenital heart defects. K.: Book plus; 2008.
5. Yuh D.D., Vricella L.A., Yang S.C., Doty J.R. Johns Hopkins Textbook of Cardiothoracic Surgery. – 2nd ed. 2014.
6. Kouchoukos N.T., Blackstone E.H., Hanley F.L., Kirklin J.K. Kirklin/Barratt-Boyes cardiac surgery: morphology, diagnostic criteria, natural history, techniques, results, and indications. – 4th ed. Philadelphia: Elsevier; 2013.
7. Bilkis A.A., Alwi M., Hasri S. et al. The Amplatzer duct occluder: experience in 209 patients. J. Am. Coll. Cardiol. 2001; 37: 258–61.
8. Faella H.J., Hijazi Z.M. Closure of the patent ductus arteriosus with the Amplatzer PDA device: immediate results of the international clinical trial. Catheter Cardiovasc.Interv. 2000; 51:50–4.
9. Podnar T., Gavora P., Masura J. Percutaneous closure of patent ductus arteriosus: complementary use of detachable Cook patent ductus arteriosus coils and Amplatzerductoccluders. Eur. J. Pediatr. 2000; 159:293–6.
10. Celermajer D.S., Sholler G.F., Hughes C.F., Baird D.K. Persistent ductus arteriosus in adults. A review of surgical experience with 25 patients. Med J Aust. 1991;155:233–6.

There is no conflict of interest.

Appendix A1. Composition of the working group

1. Doctor of Medical Sciences I.V. Arnautova,
2. Ph.D. S.S. Volkov,
3. prof. S.V. Gorbachevsky,
4. V.P. Didyk,
5. Doctor of Medical Sciences Ermolenko M.L.,
6. prof. MM. Zelenikin,
7. prof. A.I. Kim,
8. prof. I.V. Kokshenev,
9. Doctor of Medical Sciences A.A. Kupryashov,
10. junior researcher A.B. Nikiforov,
11. Academician of the Russian Academy of Sciences V.P. Podzolkov,
12. Doctor of Medical Sciences B.N. Sabirov,
13. prof. M.R. Tumanyan,
14. prof. K.V. Shatalov,
15. Doctor of Medical Sciences A.A. Schmaltz,
16. Ph.D. I.A. Yurlov.

The head of the working group is Academician of the Russian Academy of Sciences L.A. Boqueria

Target audience of the developed clinical recommendations:

1. Pediatrician;
2. Cardiologist;
3. Cardiovascular surgeon.

Table P1– Recommendation strength levels

Table A2 - Levels of evidence

Appendix A3. Related documents

1. On the basics of protecting the health of citizens in the Russian Federation (Federal Law of November 21, 2011 N 323-FZ)
2. The procedure for providing medical care to patients with cardiovascular diseases (Order of the Ministry of Health of Russia dated November 15, 2012 N 918n)
3. Order of the Ministry of Health and Social Development of the Russian Federation dated December 17, 2015 No. 1024n “On the classification and criteria used in the implementation of medical and social examination of citizens by federal state institutions of medical and social examination.”

Appendix B. Patient management algorithms

Appendix B: Patient Information

Regular monitoring by a cardiologist/pediatric cardiologist is necessary. In the first year of life (in the absence of indications for surgery) - once every 3 months, then - once every 6 months. For any invasive procedures, it is necessary to carry out antibacterial cover to prevent the occurrence of infective endocarditis.

Small anomalies of heart development: Brief description

Small anomalies heart development(MARS) - anatomical congenital changes in the heart and great vessels that do not lead to gross dysfunction of the cardiovascular system. A number of MARS are unstable and disappear with age.

Etiology

Hereditary determined connective tissue dysplasia. A number of MARS are dysembryogenetic in nature. The influence of various environmental factors (chemical, physical effects) cannot be excluded.

Code according to the international classification of diseases ICD-10:

Q24.9 Congenital heart defect, unspecified

Other diagnoses in the ICD 10 section

Q24.0 Dextrocardia Q24.1 Levocardia Q24.2 Triatrial heart Q24.3 Infundibular stenosis of the pulmonary valve Q24.4 Congenital subaortic stenosis

The information posted on the site is for informational purposes only and is not official.

Heart defects.congenital (classification)

Classification of congenital heart disease by severity classes (J. Kirklin et al. 1981) class I. It is possible to perform a planned operation later than 6 months: VSD, ASD, radical correction for tetralogy of Fallot class II. A planned operation can be performed within 3–6 months: radical correction for VSD, open atrioventricular canal (PAVC), palliative correction for class III TF. A planned operation can be performed within a period of up to several weeks: radical correction for transposition of the great vessels (TMS) class IV. Emergency surgery with a maximum preparation period of several days: radical correction for total anomalous pulmonary vein drainage (TAPDV), palliative correction for TMS, VSD, OAVC class V. The operation is performed urgently due to cardiogenic shock: various types of defects in the decompensation stage.

Classification of congenital heart disease by prognostic groups(Fyler D. 1980) 1 group. Relatively favorable prognosis (mortality during the first year of life does not exceed 8–11%): patent ductus arteriosus, VSD, ASD, pulmonary stenosis, etc. Group 2. Relatively unfavorable prognosis (mortality during the first year of life is 24–36%): tetralogy of Fallot, myocardial diseases, etc. Group 3. Poor prognosis (mortality during the first year of life is 36–52%): TMS, coarctation and stenosis of the aorta, tricuspid valve atresia, TADLV, single ventricle of the heart, OAVC, origin of the aorta and pulmonary artery from the right ventricle, etc. Group 4. Extremely unfavorable prognosis (mortality during the first year of life is 73–97%): hypoplasia of the left ventricle, pulmonary atresia with an intact interventricular septum, common truncus arteriosus, etc.

Classification of congenital heart disease according to the possibility of radical correction(Turley K. et al. 1980) 1 group. Defects for which only radical correction is possible: aortic stenosis, pulmonary artery stenosis, TADLV, triatrial heart, coarctation of the aorta, patent ductus arteriosus, aortic pulmonary septal defect, ASD, mitral valve stenosis or insufficiency group 2. Defects in which the advisability of radical or palliative surgery depends on the anatomy of the defect, the age of the child and the experience of the cardiology center: various variants of TMS, pulmonary atresia, common truncus arteriosus, tetralogy of Fallot, OAVC, VSD group 3. Defects for which only palliative operations are possible in infancy: a single ventricle of the heart, some variants of the origin of the great vessels from the right or left ventricle with pulmonary stenosis, atresia of the tricuspid valve, atresia of the mitral valve, hypoplasia of the ventricles of the heart.

Abbreviations OAVC - open atrioventricular canal TMS - transposition of the great vessels TADLV - total anomalous drainage of the pulmonary veins.

ICD-10 Q20 Congenital anomalies of the heart chambers and connections Q21 Congenital anomalies of the cardiac septum Q22 Congenital anomalies of the pulmonary and tricuspid valves Q23 Congenital anomalies of the aortic and mitral valves Q24 Other congenital anomalies of the heart.

More than 400 children with developmental anomalies were born in Kyrgyzstan during the year.

Since there are a lot of defects of the circulatory system, there cannot be only one VSP code according to ICD 10. In addition, the clinical picture of some of them is so similar that modern informative diagnostic techniques have to be used for differentiation.

There is a huge difference between acquired cardiac disorders and congenital developmental anomalies, as they are in different ICD classes. Although the disturbances in arterial and venous blood flow will be the same, the treatment and etiological factors will be completely different.

Congenital heart disease may not require therapeutic measures, however, more often planned operations are performed or even urgent ones for serious, incompatible with life, inconsistencies with the norm.

Heart defects are in the class of congenital anomalies of the structure of the body in the block of anomalies of the circulatory system. VSP in ICD 10 branches into 9 sections, each of which also has subparagraphs.

However, heart problems include:

Q20 – anatomical disorders in the structure of the cardiac chambers and their connections (for example, various clefts of the oval window); Q21 – pathologies of the cardiac septum (defects of the atrial and interventricular septa and others); Q22 – problems with the pulmonary and tricuspid valves (insufficiency and stenosis); Q23 – pathologies of the aortic and mitral valves (insufficiency and stenosis); Q24 – other congenital heart defects (change in the number of chambers, dextracardia, etc.).

Each of the listed points requires further differentiation, which will allow us to determine the treatment plan and prognosis for the child. For example, with valve damage There may be symptoms of insufficiency or stenosis. In this case, the hemodynamic features of the disease will differ.

In ICD, congenital heart disease implies some kind of blood flow disturbance.

That is why in all encodings complete inversion of organs or their structures with full functioning is excluded.

Excluded: endocardial fibroelastosis (

Excludes: dextrocardia with localization inversion (Q89.3) atrial appendage isomerism (with asplenia or polysplenia) (Q20.6) mirror image of the atria with localization inversion (Q89.3)

Q24.1 Levocardia

Q24.2 Triatrial heart

Q24.3 Infundibular stenosis of the pulmonary valve

Q24.4 Congenital subaortic stenosis

Q24.5 Malformation of coronary vessels

Congenital coronary (arterial) aneurysm

Q24.6 Congenital heart block

Q24.8 Other specified congenital cardiac anomalies

Congenital: . left ventricular diverticulum. vice: . myocardium. pericardium Abnormal position of the heart Uhl's disease

Q24.9 Congenital heart defect, unspecified

Congenital: . anomaly). heart disease NOS

Congenital heart disease refers to the isolation of diseases that are combined by anatomical defects of the heart or valve apparatus. Their formation begins during the process of intrauterine development. The consequences of defects lead to disturbances in intracardiac or systemic hemodynamics.

Symptoms differ depending on the type of pathology. The most common signs are pale or blue skin, a heart murmur, and physical and mental developmental delays.

It is important to diagnose the pathology in time, since such disorders provoke the development of respiratory and heart failure.

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Congenital heart defects – ICD-10 code Q24 – include various pathologies of the cardiovascular system, accompanied by changes in blood flow. Subsequently, heart failure is often diagnosed, which leads to death.

According to statistics, every year in the world 0.8-1.2% of the total number of newborns are born with this pathology. Moreover, these defects account for about 30% of the total number of diagnosed congenital defects in fetal development.

Often the pathology in question is not the only disease. Children are also born with other developmental disorders, of which a third are musculoskeletal defects. Taken together, all the violations lead to a rather sad picture.

Congenital heart defects include the following list of defects:

ventricular or atrial septal defect; stenosis or coarctation of the aorta; pulmonary stenosis; open form of the ductus arteriosus; transposition of large great vessels.

Causes

Among the causes of this pathology in newborns, I highlight the following factors:

The cause of pathology in newborns in the form of maternal diseases during pregnancy accounts for 90% of cases. Risk factors also include toxicosis during pregnancy in the first trimester, threats of miscarriage, genetic predisposition, endocrine system disorders and “inappropriate” age for pregnancy.

Classification

Depending on the principle of changes in hemodynamics, there is a certain classification of the presented pathology. The classification includes several types of heart disease, where the influence on pulmonary blood flow plays a key role.

In practice, experts divide the heart pathologies under consideration into three groups.

Here they highlight:

Hemodynamic disturbance

When these factors-causes are exposed and manifested in the fetus during development, characteristic disturbances occur in the form of incomplete or untimely closure of the membranes, underdevelopment of the ventricles and other anomalies.

Intrauterine development of the fetus is distinguished by the functioning of the ductus arteriosus and the oval window, which is in an open state. The defect is diagnosed when they still remain open.

The presented pathology is characterized by the absence of manifestations in intrauterine development. But after birth, characteristic disorders begin to appear.

Such phenomena are explained by the time of closure of the communication between the systemic and pulmonary circulation, individual characteristics and other defects. As a result, the pathology can make itself felt some time after birth.

Often, hemodynamic disorders are accompanied by respiratory infections and other concomitant diseases. For example, the presence of a pale type pathology, where arteriovenous discharge is noted, provokes the development of pulmonary hypertension, while a blue type pathology with a venoarterial shunt promotes hypoxemia.

The danger of the disease in question lies in the high mortality rate. Thus, a large discharge of blood from the pulmonary circulation, provoking heart failure, in half of the cases ends in the death of the baby before the age of one, which is preceded by the lack of timely surgical care.

The condition of a child over 1 year of age improves noticeably due to a decrease in the amount of blood entering the pulmonary circulation. But at this stage, sclerotic changes often develop in the vessels of the lungs, which gradually provokes pulmonary hypertension.

Symptoms

Symptoms appear depending on the type of anomaly, the nature and time of development of circulatory disorders. When the cyanotic form of the pathology develops in a sick child, a characteristic blueness of the skin and mucous membranes is noted, which increases its manifestation with each strain. The white defect is characterized by pallor, constantly cold hands and feet of the baby.

The baby himself with the presented disease differs from others in hyperexcitability. The baby refuses to breastfeed, and if he starts sucking, he quickly gets tired. Often, children with this pathology are diagnosed with tachycardia or arrhythmia; external manifestations include sweating, shortness of breath and pulsation of neck vessels.

In the case of a chronic disorder, the child lags behind his peers in weight, height, and there is a physical delay in development. As a rule, at the initial stage of diagnosis, a congenital heart defect is listened to, where heart rhythms are determined. In the further development of the pathology, edema, hepatomegaly and other characteristic symptoms are noted.

Complications include bacterial endocarditis, venous thrombosis, including cerebral thromboembolism, congestive pneumonia, angina syndrome and myocardial infarction.

Diagnostic measures

The disease in question is determined by using several methods of examining the child:

How to treat congenital heart disease

The presented disease is complicated by performing surgery on a sick child under one year of age. Here, specialists are guided by the diagnosis of cyanotic pathologies. In other cases, operations are postponed because there is no risk of developing heart failure. Cardiology specialists work with the child.

Treatment methods and methods depend on the types and severity of the pathology in question. If an anomaly of the interatrial or interventricular septum is detected, the child undergoes plastic surgery or suturing.

In case of hypoxemia, at the initial stage of treatment, specialists perform palliative intervention, which involves the application of intersystem anastomoses. Such actions can significantly improve blood oxygenation and reduce the risk of complications, as a result of which further planned surgery will take place with favorable results.

Aortic disease is treated by resection or balloon dilatation of coarctation of the aorta, or plastic stenosis. In the case of a patent ductus arteriosus, simple ligation is performed. Pulmonary stenosis undergoes open or endovascular valvuloplasty.

If a newborn is diagnosed with a heart defect in a complicated form, where it is impossible to talk about radical surgery, specialists resort to actions to separate the arterial and venous ducts.

The anomaly itself does not disappear. It talks about the possibility of performing Fontannes, Senning and other types of operations. If surgery does not help in treatment, they resort to a heart transplant.

As for the conservative method of treatment, they resort to the use of medications, the action of which is aimed at preventing attacks of shortness of breath, acute left ventricular failure and other heart damage.

Prevention

Preventive actions for the development of this pathology in children should include careful planning of pregnancy, complete exclusion of unfavorable factors, as well as a preliminary examination to identify a risk factor.

Women who are on such an unfavorable list must undergo a comprehensive examination, which includes ultrasound and timely chorionic villus biopsy. If necessary, issues of indications for termination of pregnancy should be addressed.

If a pregnant woman is already informed about the development of pathology during fetal development, she should undergo a more thorough examination and consult with an obstetrician-gynecologist and cardiologist much more often.

Forecasts

According to statistics, mortality due to the development of congenital heart disease occupies a leading position.

In the absence of timely assistance in the form of surgical intervention, 50-75% of children die before reaching their first birthday.

Then comes a period of compensation, during which mortality rates drop to 5% of cases. It is important to identify the pathology in a timely manner - this will improve the prognosis and condition of the child.

Patent ductus arteriosus (PDA)- a vessel through which the pathological communication between the aorta and the pulmonary artery (PA) remains after birth. In healthy children, the functioning of the duct stops immediately after birth or continues in a sharply reduced volume for no more than 20 hours. Subsequently, the ductus arteriosus gradually obliterates and turns into an arterial ligament. Normally, obliteration of the duct ends in 2-8 weeks. The ductus arteriosus is considered an anomaly if it is functioning 2 weeks after birth.

Code according to the international classification of diseases ICD-10:

• P29.3
• Q21.4
• Q25.0

Statistical data: PDA is one of the most common defects (6.1% of all congenital heart defects in infants, 11-20% of all congenital heart defects diagnosed in the clinic, 9.8% according to autopsies); The ratio of male to female is 1:2.
Etiology: family cases of the defect are described; often the mother has a history of rubella, herpes, influenza at 4-8 weeks of pregnancy; Prematurity and neonatal respiratory distress syndrome, hypoxia of the newborn with a high Pg content are predisposing factors.
Pathophysiology. The direction of blood discharge is determined by the pressure difference between the aorta and the pulmonary artery and depends on the resistance value of the pulmonary and systemic vascular beds (while the pulmonary vascular resistance is lower than the systemic resistance, blood is discharged from left to right; when pulmonary resistance predominates, the direction of shunting changes). With large PDA sizes, changes in the pulmonary vessels occur early (Eisenmenger syndrome).

Symptoms (signs)

Clinical picture and diagnosis
Complaints: fatigue, shortness of breath, a feeling of interruptions in the heart, frequent infections, paradoxical embolism.
Objective examination. Retarded physical development. Pale skin, unstable cyanosis when screaming or straining. Symptoms of “drumsticks” and “watch glasses”. Persistent cyanosis when shunting blood from right to left. “Cardiac hump”, increased apical impulse, systolic tremors with a maximum in the second intercostal space to the left of the sternum. The borders of the heart are expanded to the left and right. A decrease in diastolic and an increase in pulse blood pressure, an increase in the apical impulse, an increase in both heart sounds (the volume of the second heart sound above the PA correlates with the severity of pulmonary hypertension). Rough machine systolic diastolic murmur in the second intercostal space to the left of the sternum, radiating into the interscapular space and to the great vessels. As pulmonary hypertension progresses and the left-to-right shunt decreases, the murmur weakens and shortens until it disappears completely (at this stage, a Graham Still diastolic murmur may appear, arising from relative insufficiency of the PA valve), followed by a repeated increase when a right-to-left shunt occurs. Sometimes above the apex of the heart there is a murmur of relative stenosis or mitral valve insufficiency.
Instrumental diagnostics
. ECG: signs of hypertrophy and overload of the right and then left parts of the heart; rarely - blockade of the His bundle branches.
. X-ray examination of the chest organs. Bulging of the PA arches, right and left ventricles. Enrichment of the pulmonary pattern, expansion and lack of structure of the roots of the lungs. Dilation of the ascending aorta. In adults, a calcified PDA can be visualized relatively rarely.
. EchoCG. Hypertrophy and dilatation of the right and left ventricles. Visualization of the PDA, determination of its shape, length and internal diameters (to assess the prognosis and select the size of the endovascular occlusion device). In the Doppler mode, a specific form of shift of the Doppler frequency spectrum in the PA is identified, the degree of shunt and the ratio of pulmonary blood flow to systemic blood flow (Qp/Qs) are determined.
. Catheterization of the left and right parts of the heart. The letter  symptom is the passage of a catheter from the PA through the PDA into the descending aorta. An increase in blood oxygenation in the LA compared to the right ventricle by more than 2 volume percent. Tests with aminophylline and oxygen inhalation are performed to determine the prognosis regarding the reversibility of pulmonary hypertension.
. Ascending aortography. Receipt of contrast agent from the ascending aorta into the PA. Diagnosis of concomitant coarctation of the aorta.
Drug therapy. Before closing the PDA, prophylaxis against bacterial endocarditis is necessary. The use of indomethacin is indicated for narrow PDAs identified during the neonatal period, and is contraindicated in renal failure. Intravenous administration of indomethacin is recommended:
. less than 2 days: initial dose 200 mcg/kg; then 2 doses of 100 mcg/kg with an interval of 12-24 hours;
. 2-7 days: initial dose 200 mcg/kg; then 2 doses of 200 mcg/kg with an interval of 12-24 hours;
. more than 7 days: initial dose 200 mcg/kg; then 2 doses of 250 mcg/kg with an interval of 12-24 hours.

Treatment

Surgery
Indications. Failure of conservative therapy for 5 days or more, contraindications to the use of NSAIDs. Decompensation of circulatory failure. PDA of medium or large diameter in all children under 1 year of age.
Contraindications. Severe concomitant pathology that threatens the patient’s life. Terminal stage of circulatory failure. Irreversible pulmonary hypertension.
Methods of surgical treatment. In most cases, endovascular closure of the duct using occlusion devices (Gianturco coils, Cook coils, or umbrella devices) is feasible. If the duct is very wide or endovascular correction is unsuccessful, an open operation is performed: ligation or (less often) intersection of the PDA, followed by suturing of both ends. Thoracoscopic clipping of a PDA has no advantages over endovascular and open interventions, so it is rarely performed.
Specific postoperative complications: injury to the left recurrent laryngeal nerve, bleeding, deformation of the aorta with the formation of coarctation, residual discharge of blood through the duct due to inadequate correction.

Forecast. A narrow PDA generally does not affect life expectancy, but increases the risk of infective endocarditis. Medium and wide PDAs almost never close on their own; spontaneous closure after 3 months is rare. The effectiveness of conservative treatment of narrow PDA reaches 90%. With PDA, mortality during the first year of life is 20%. Eisenmenger syndrome in older children is observed in 14% of cases, infective endocarditis and endarteritis - in 9% of cases. Duct aneurysm and its ruptures are isolated cases. The average life expectancy for medium PDA is 40 years, for wide PDA it is 25 years. Postoperative mortality is 3%. Clinical rehabilitation, depending on hemodynamic disorders, takes place over 1-5 years.
Pregnancy. In women with a small or medium-sized PDA and left-to-right shunting, an uncomplicated course of physiological pregnancy can be expected. Women with high pulmonary resistance and a right-to-left shunt have an increased risk of complications.
Synonyms: Open botal duct; Patent ductus arteriosus; Non-closure of the ductus botallus.
Abbreviations. PDA - patent ductus arteriosus. PA - pulmonary artery.

ICD-10. P29.3 Persistent fetal circulation in the newborn. Q21.4 Defect of the septum between the aorta and pulmonary artery. Q25.0 Patent ductus arteriosus

Keywords

Dyspnea; heartbeat; cyanosis; persistent cyanosis; pulmonary; endocarditis; enterocolitis.

List of abbreviations

CHD - congenital heart defects

PA - pulmonary artery

MRI - magnetic resonance imaging

NSAIDs - non-steroidal anti-inflammatory drugs

PDA - patent ductus arteriosus

TPR - total pulmonary resistance

ECG - electrocardiography

EchoCG - echocardiography

Terms and Definitions

Cardiac catheterization is an invasive procedure performed for therapeutic or diagnostic purposes for pathology of the cardiovascular system.

Pulse pressure is the difference between systolic and diastolic pressure. Endocarditis - inflammation of the inner lining of the heart, is a common manifestation of other diseases.

Echocardiography is an ultrasound method aimed at studying the morphological and functional changes of the heart and its valve apparatus.

1. Brief information

1.1. Definition

Patent ductus arteriosus (PDA) is a vessel through which pathological communication between the aorta and the pulmonary artery (PA) remains after birth.

Comments: Normally, the PDA is necessarily present in the fetus, but closes soon after birth, turning into an arterial ligament.

1.2 Etiology and pathogenesis

Risk factors for patent ductus arteriosus are premature birth and prematurity, family history, the presence of other congenital heart diseases, infectious and somatic diseases of the pregnant woman.

1.3 Epidemiology

The exact frequency of occurrence of the defect is unknown, since it is unclear at what point a patent ductus arteriosus should be considered a pathology. It is conventionally believed that normally it should close within the first or two weeks of life. PDA usually occurs in premature infants and is extremely rare in infants born at term. Under these criteria, the incidence of isolated pathology is about 0.14-0.3/1000 live births, 7% among all congenital heart defects (CHD) and 3% among critical congenital heart defects. Persistence of the duct largely depends on the degree of term of the child: the lower the weight, the more common this pathology is.

The average life expectancy of patients with PDA is approximately 40 years. 20% of patients die before 30 years of age, 42% die before 45 years of age, and 60% die before 60 years of age. The main causes of death are bacterial endocarditis (endarteritis), development and rupture of ductal aneurysm.

1.4 Coding according to ICD 10

Congenital anomalies of large arteries (Q25):

Q25.0 - Patent ductus arteriosus.

1.5. Classification

Taking into account the level of pressure in the pulmonary artery, 4 degrees of defect are distinguished:

The pressure in the pulmonary artery (PA) in systole does not exceed 40% of the arterial pressure;

The pressure in the PA is 40-75% of the arterial pressure (moderate pulmonary);

Pressure in the PA is more than 75% of arterial pressure (pronounced pulmonary pressure with preservation of left-right blood discharge);

The pressure in the PA is equal to or exceeds the systemic one (severe pulmonary hypertension, which leads to the occurrence of right-to-left shunting).

In the natural course of the patent ductus arteriosus there are 3 stages:

Stage I of primary adaptation (the first 2-3 years of a child’s life). Characterized by clinical manifestation of patent ductus arteriosus; often accompanied by the development of critical conditions, which in 20% of cases result in death without timely cardiac surgery.

Stage II of relative compensation (from 2-3 years to 20 years). Characterized by the development and long-term existence of pulmonary hypervolemia, relative

stenosis of the left atrioventricular orifice, systolic overload of the right ventricle.

Stage III of sclerotic changes in the pulmonary vessels. The further natural course of the patent ductus arteriosus is accompanied by a restructuring of the pulmonary capillaries and arterioles with the development of irreversible sclerotic changes in them. At this stage, the clinical manifestations of patent ductus arteriosus are gradually replaced by symptoms of pulmonary hypertension.