Truncus arteriosus

January 2017
The following recommendations concern persistent truncus arteriosus a long-term disease, and aim at improving the way in which patients suffering from this complex congenital heart disease are dealt with.


Definition



Truncus arteriosus is a heart malformation. Only one vessel leaves the heart and form the aorta (the aorta and the pulmonary artery meet).

Diagnostic

Clinical diagnosis


The diagnosis of this disease is done before the birth or at birth.

The symptoms of the malformation are:
  • A huge pulmonary output in the month which follows the birth, the symptoms are:
    • Polypnea (rapid breathing),
    • Difficulties with feeding-bottles,
    • A break in the weight curve,
    • Hepatomegaly (increase in the size of the liver),
    • Paleness,
    • Full pulse,
    • Light cyanosis,
  • Diastolic breathing (escape truncal valve)
  • An absence of femoral pulse (interruption of the aortic arch).

Para-clinical diagnosis

  • Examinations will confirm the diagnosis:
    • Chest x-ray (pulmonary x-ray),
    • Echocardiography.
  • Further examinations can be necessary:
    • Angioscanner,
    • Cytogenetic search (diagnosis of a genetic syndrome).

Treatment


In some cases, when there is an interruption of the aortic arch for example, there is a need for neonatal management, as from the birth.

Medical treatment


If diagnosis was made before birth:
  • Delivery must be carried out in an appropriate maternity
  • A medical management of the heart failure
    • Diuretics
    • IEC
    • Hypercaloric diet
    • Enteral feeding probe

Surgical Treatment

  • Possible interventions:
    • Ensuring the proper flow in the right ventricle with the lung arteries,
    • Plastic surgery of the lung arteries,
    • Closing of the VSD (ventricular septal malformation),
    • Plastic surgery of the truncal valve,
    • Rebuilding of the aortic arch (if interruption or coarctation of the aorta).

Postoperative management

Therapeutic management


Therapeutic education is recommended. It encourages:
  • A better knowledge of the disease by the family, then the patient, when he gets older;
  • To prevent infective endocarditis (hygiene...);
  • Educating about the time of taking an anticoagulant;
  • An education about cardiac pacemakers or implants when it is necessary;
  • Information about the risks of pregnancy;
  • Advice on contraception;
  • Change the patient's lifestyle (do sports);
  • To prevent RSV bronchiolitis;
  • An education on treatment by antivitamine K.

Treatment

  • Medicinal
    • If heart failure: diuretics, IEC;
    • If disorder of the arterial or jonctional rhythm: antiarythmic treatment;
    • If right ventircle-Pulmonary artery or pulmonary valvular bioprosthesis is carried out: aspirin.
  • Implants in the case of a residual pulmonary hypertension:
    • Cardiac pacemaker,
    • Implantable defibrillator.
  • Surgery
    • If residual obstruction of the airway of right ventricle ejection and/or residual stenoses of the pulmonary branches;
    • If there is a residual interventricular communication;
    • If there is an important pulmonary failure;
    • If there is an aortic failure;
    • Rythm disorder.

Follow-up

  • Search for symptom at rest or during efforts.
  • ECG (electrocardiogram).
  • Doppler echocardiography.
  • INR (patient under anticoagulant).
  • Creatininemia and kaliemy (taken IEC).

Sources


Photographic copyright: Oguzaral | Dreamstime.com

Related


Le truncus arteriosus (HAS)
Le truncus arteriosus (HAS)
Latest update on April 11, 2010 at 02:06 PM by owliance.en_ctrl_002.
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