Vascular Workup

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Atrioventricular in India = overview

Atrioventricular in India = overview

Introduction

Background

Endocardial cushion defects, known as atrioventricular (AV) canal or septal defects, including a number of defects characterized by involvement of the atrial septum, ventricular septum, and one or both of the atrioventricular valves.

These defects can be classified by various methods. A distinction is usually between complete and partial defects. A complete atrioventricular septal defect indicates the presence of both handset and defects ventricular septum with a common atrioventricular valve. A partial defect indicates the involvement of the atrial septum with different holes in the mitral and tricuspid valve.

atrioventricular arise from abnormal development of endocardial cushions. In these patients, the upper and lower cushions do not close completely. Communication ASD is left at the bottom of the atrial septum. This is called an ostium primum defect. The failure of the endocardial cushions to fuse the results of a position Abnormally low atrioventricular valves and abnormal position of the aortic valve. A portion of the atrioventricular valves originates in the endocardium cushions, and results in improper fusion anterior and posterior components of the mitral valve leaflet.

Pathophysiology

Prevalence left to right shunting of blood through the heart occurs in these patients. In patients with partial defects, this occurs through the ostium primum defect the atrial septum. When complete endocardial cushion defect is present, a large ventricular septal defect and valvular insufficiency may develop, as resulting in volume overload of both the left and right ventricles associated with heart failure in early life. In patients with lung overload longstanding pulmonary vascular disease may develop and congestive heart failure (CHF) can improve symptoms. This improvement is an indicator of poor prognosis because it announces the development of a short circuit from right to left and irreversible pulmonary hypertension (ie, Eisenmenger syndrome).

Frequency

United States

The frequency rate is about 3% of children with congenital heart disease. Sixty to seventy percent of these defects are of the completed form. More than half of those affected with the complete form hAtrioventriculare Down syndrome.

International

The index frequency is about 3% of children hAtrioventriculare congenital heart disease.

Mortality / Morbidity

Patients with ostium primum atrial septal defect only and minimum impairment of the left atrioventricular valve (ie, mitral valve) do well without treatment during infancy, childhood and adolescence. During adulthood, these patients develop symptoms of CHF and atrial arrhythmias.

Patients with defects septal and mitral valve regurgitation develop CHF early in life, with high rates of morbidity and mortality if valvular insufficiency is pronounced. Patients with a complete absence of the ICC development in childhood, with frequent respiratory infections and poor weight gain.

Race

No racial predilection is evident.

Sex

Girls are affected slightly more often than males.

Age

ECD is a congenital defect present at birth. The severity of symptoms and presentation complex is directly dependent on the severity of the defect and the presence of renal mitral.

Clinical

History

A baby may be relatively asymptomatic. In severe cases, patients hAtrioventriculare a history of malnutrition, chronic infections of upper respiratory tract, pneumonia, and lack of growth. The mother may notice difficulties with crying, frequent breaks during feeding, and nasal flaring. As the child grows older, the most common manifestations of CHF may develop, including the business Atrioventricularersion and play, easy fatigability, dyspnea and edema.

Physical

• partial defects present with physical symptoms common to handset septal defects.
  • The second heart sound is deeply divided, without respiratory variation.
  • An ejection systolic murmur of the heart can be in the top left of the sternum.
  • Earlier low-pitched diastolic murmur may be the heart in the lower left sternal edge and is associated with increased tricuspid valve flow.
  • A murmur of mitral regurgitation may or may not be present.
• Additional findings in complete endocardial cushion defects are associated with ventricular septal defect and valvular insufficiency.
  • Poor physical, chest hyperinflated, bulging precordium, Harrison grooves, mild or intermittent cyanosis, and stigmata of Down syndrome (eg, oblique palpebral fissures, large protruding language, and short broad hands, simian crease, the inner skin epicanthal fold)
  • Arterial and jugular pulse - Water hammer pulse, wAtrioventriculare v dominant jugular pulse
  • precordial palpation and motion - emotion systolic palpable momentum in the second intercostal space and the third represents a dilated pulmonary artery, heAtrioventriculare prominent in the left sternal border
  • Auscultation
    • A single sound is first heard the heart, splitting can be fixed relatively soft second heart sound.
    • A systolic murmur of a ventricular septal defect can be heard and the systolic murmur of mitral regurgitation.
    • Pulmonary hypertension is associated with a strong component of the second pulmonary heart sound.

Causes

• Genetics
  • The characteristic pattern of malformation has been attributed to trisomy 21 and Down syndrome in some cases. There is some evidence that a critical region of chromosome band 21q22 may contribute particularly to cardiac malformation in this syndrome.
  • Other chromosome abnormalities can also lead to atrioventricular septal defects, in particular, the removal of 8p, 10q partial monosomy, partial monosomy 13q, Ring 22 14 q +, and 3-p 1p.
  • In most cases of major chromosomal aberrations, atrioventricular septal defects are associated with other non-cardiac birth defects. However, isolated atrioventricular septal defects may be transmitted in families as an autosomal dominant trait.
  • HAtrioventriculare Linkage analysis suggests a locus for autosomal dominant atrioventricular septal defects on chromosome 1p but no specific gene defect has been determined yet.
• Aberrations growth factor: In the developing fetus, the formation of cardiac tissue depends on appropriate stimulation transforming growth factor beta as a growth factor and platelet-derived growth. Alterations in the concentration or effectiveness of these factors during embryogenesis may contribute to heart malformations.

Differential Diagnosis

Atrial septal defect
Regurgitation mitral
Ventricular septal defect

Workup

Laboratory studies

• CBC count: Blood tests determine the presence of polycythemia in a potentially cyanotic.
• The prothrombin time / activated partial thromboplastin time (PT / aPTT): In children with cyanotic heart disease, coagulation profile may be abnormal due to the associated polycythemia.
• Electrolytes: This test detects incurred any abnormalities that treatment of CHF.

Imaging Studies

• Chest X-ray
  • This test is a test of general interest shows cardiac enlargement, particularly of the right atrium and ventricle.
  • The main pulmonary artery usually stands with increased pulmonary vascular markings. After pulmonary hypertension develops, a reduction in pulmonary vascular markings observed.
• Echocardiography
  • M-mode displays the diastolic motion of the mitral valve with an enlarged right ventricle and paradoxical movement of interventricular septum.
  • Two-dimensional echocardiography is very reliable in identifying septal defects. Echocardiography identifies the absence of the interventricular septum. The findings may include right ventricular dilatation and paradoxical septal motion. The extension of septal defects, as well as left to right shunt and the degree of valvular insufficiency can be determined, and the estimated pressure pulmonary artery. The lack of movement on the left and right atrioventricular valves is a characteristic finding in this condition. Prolonged diastolic contact the anterior mitral leaflet and the interventricular septum can also be observed. Associated defects that may require attention also can be detected.
  • Abnormalities of the atrioventricular valves can be identified reliably. Transesophageal echocardiography clearly identifies the atrioventricular valve morphology.
• MRI: This test easily visually deficiency in the ventricular septum and the atrioventricular valve morphology.
• Cardiac catheterization: This test is indicated when clinically significant questions remain unanswered after a comprehensive noninvasive assessment. If other injuries are suspected or if the operational planning can not be performed properly after a noninvasive test, then catheterization should be performed. Left ventricular angiography in the frontal plane shows an elongated outflow tract of the left ventricle, called goose neck deformity "," which is characteristic of this condition. Catheterization should include the quantification of shunts and valvular insufficiency and calculation of pulmonary vascular resistance. Aortography may be performed to determine whether a ductus arteriosus is present.

Other tests

• Electrocardiograph
  • The typical ECG in patients with partial atrioventricular septal defects sample of first-degree Atrioventricular block and axis deviation to the left (by the depolarization later left anterior fascicular). Patients with right ventricular dilation usually right hAtrioventriculare block partial or complete branch. Complete atrioventricular block and atrial fibrillation commonly occur in older patients. See Medscape Resource Center atrial fibrillation.
  • A prolonged PR interval accompanied by left ventricular or biventricular hypertrophy can also be seen.

Treatment

Medical Care

Medical treatment is designed to relieve the symptoms of CHF until surgical correction is feasible. The objective of therapy is the development of atrioventricular pulmonary vascular obstructive disease. When heart failure and associated pulmonary congestion are present, diuretics and digoxin are shown.

Surgical Care

• Babies with partial atrioventricular septal defects that are symptomatic are referred for corrective surgery, including mitral valve replacement and closure of atrial septal defect. Asymptomatic patients with ostium primum defect referred to elective repair after infancy.
• Patients with complete atrioventricular septal defects not associated with right ventricular hAtrioventriculare general outlet obstruction pulmonary arterial pressures hAtrioventriculare near systemic levels. These patients develop pulmonary vascular disease after first year of life and usually are referred for corrective surgery in infancy.
• Historically, children were treated with pulmonary artery childhood band to protect the pulmonary vasculature of excessive blood flow and development of pulmonary vascular disease. Patients were referred for corrective surgery when they are older than 3-4 years.
• Corrective surgery can be performed even in early childhood in several ways. A single Dacron patch can be used to close the septal defect and ventricular fibrillation. The left and right sides of the common atrioventricular valve are then resuspended in the review. A two-patch technique can also be used.
• Serious and irreversible pulmonary vascular disease is a contraindication to corrective surgery, and these children may be referred for cardiopulmonary transplantation.

Diet

For infants in the ICC, the discretion with fluid intake and salt use is encouraged.

Activity

Rest during feeding is encouraged since a manifestation of dyspnea in these children is the inability to feed. Generally, the child activity limits of breath.

Medication

Digital and diuretics are used to control volume overload found in these patients until palliative or corrective surgery may be performed.

Diuretics

These agents are used to decrease volume overload.

Monitoring

Hospitalization services

Postoperative Recovery requires 5-10 days of hospitalization, depending on the child's condition before surgery and whether palliative or complete correction takes out. With the mitigation (ie, pulmonary artery Gaza), the preoperative condition of volume overload must still be regulated. With full correction, Recovery is usually without complications.

Furthermore Care

It is necessary to continue the observation with echocardiography regularly scheduled in order to assess the integrity of the atrioventricular valve reconstruction. The area is prone to the development of valvular insufficiency that may require further intervention as the child grows older.

Inpatient and outpatient drugs

Digitalis: This agent provides support for postoperative myocardial and can be stopped after 2-3 years.

Diuretics: In general, furosemide is prescribed for several months after repair to correct volume overload, but stops once he euvolemia.

Deterrence and prevention

The current limited knowledge of genetic abnormalities that predispose to the formation of endocardial cushion defect can greatly expand the current developments in the human genome project. As the knowledge base expands, the prenatal detection and treatment can possibly be possible in the future.

Complications

Since synthetic material used to repair the ventricular and atrial septal defect, the child is at risk of infection. Other potential complications include complete heart block, ventricular arrhythmia, and atrioventricular valve stenosis and / or failure.

Prognosis

The long-term results of surgical correction of this anomaly depends on the degree of preoperative pulmonary vascular disease and the amount of atrioventricular valve regurgitation waste. If the pulmonary vasculature is protected and the amount of valvular regurgitation is substantially reduced, the prognosis is good. When severe pulmonary vascular disease is present before surgery, morbidity and mortality rates are high. Complete heart block and arrhythmias may occur after the correction, and the incidence increases with age. As the patient grows, mitral valve replacement may be necessary.

The mortality rate in surgical patients with partial endocardial cushion defects is 0-6%, while for the full default range of 3-10%.

Education Patient

Parents should be instructed to ensure that antibiotic prophylaxis for dental procedures is instituted for the child. Good children's dental hygiene is essential.

Miscellaneous

Medical-Legal Mistakes

A frank discussion should be conducted with the parents of these children from all possible operative complications related implications for the child in the future. The question future of the mitral valve replacement should be covered with them before the initial surgery. The risk of endocarditis, cardiac arrhythmia, and complications long-term lung must be covered.