| QUESTION Respected gentlemen: ABDOMINAL AORTA REQUESTED HIS COLLABORATION ON THE TOPIC OF THE ANEURISMA; WHICH WANTED TO KNOW THE FOLLOWING THING:
1. IF DOES A PERSON WHO HAS THE SYNDROME OF MARFAN HAVE A HIGH PERCENTAGE OF RISK OF SUFFERING FROM ABDOMINAL ANEURISMA?.
2. WHEN DOES A PERSON SUFFER FROM ABDOMINAL ANEURISMA, WHICH EXAMINATIONS MUST PRACTISE TO DIAGNOSE THIS ILLNESS?.
3. CAN THIS PERSON HAVE POSSIBILITIES OF LIFE, WHEN ONE HAS GIVEN HIM AN EXACT DIAGNOSIS AND TREATMENT TO HIS HIS ILLNESS?.
4. WITH WHAT NAME IS HE MET TO THE SURGICAL INTERVENTION OF THIS ILLNESS?.
5. WHAT SPECIALTIES OF THE MEDICINE CAN THEY DIAGNOSE AND TREAT THIS ILLNESS?.
6. IF IS A 33-YEAR-OLD WOMAN WHO ENDURES THIS ILLNESS IN A PERIOD OF GESTATION (TWINS) OF 7 MONTHS AND A HALF, TO WHICH IS REALIZED A RADIOGRAPHY (ACCORDING TO INFORMATION OF THE CLINIC, WHICH THREW THE RESULT OF WHAT THE BABIES WERE IN PERFECT CONDITIONS), THERE EXISTS POSSIBILITY OF LIVING THE BABIES EVEN IF THE MOTHER SUFFERS THIS ILLNESS?.
7. CAN EXIST THE POSSIBILITY OF CONFUSING THE SYMPTOMATOLOGY OF THE ABDOMINAL ANEURISMA WITH THE SHARP GASTRITIS?.
8. IS IT OF VERY DIFFICULT DIAGNOSTIC THIS ILLNESS?.
9. IS THIS ILLNESS HEREDITARY, AND IN A FAMILY THERE CAN EXIST HER SEVERAL MEMBERS WHO SUFFER IT?. ANSWER Heredity.
The gene of the Syndrome of Marfan (placed in the chromosome 15) is inherited from the father who is affected. It is a domineering gene for it 50 % of the children can be affected. Sometimes, in 25 % of the cases, the gene is altered by spontaneous mutation and is the first one in enduring it in his family.
Mechanisms.
In the Marfan a Fibrilina deficit exists (Glicoproteina 350kd), which is a component of the microfibrillas associated with the elastina. The affected ones in the Marfan syndrome differ for having a typical morphology, with very long members, height as enzyme of the normality, long face and the packed teeth. Diagnosis and control panel. Before a Marfán syndrome the regular examinations are recommended of:
For the heart - Ecocardiograma
For the aorta - Ultrasounds
For the eyes - Control panel for the detachment of retina. Advisable preventive treatments in the Marfán syndrome: - of the Hypertension
- replacement of aortic valve
- replacement of other valves of the heart
- anticoagulant medication if the valves change
- preventive endocarditis antibiotics, in the surgical interventions of any type
- physical therapy of back column
- teeth treatment for the ortodoncista Complications.
The women with Marfan syndrome think of high risk if they are embarrassed, the increase of the dissection of aorta is very big.
The loss of the flexible textiles of the arterial wall is the most frequent cause of the aneurisma of aorta. It can appear like an abdominal pulsatile mass without other symptoms accompanists, then light pains appear, sometimes the break of an aneurisma is preceded only of unspecific light symptoms. The diagnosis is realized by means of Ultrasound scan (ultrasounds), detects it and values his size, by means of the axial Scanner Computarizada and the nuclear magnetic Resonance, the anatomical knowledge of the aneurisma is improved and to see the commitment of the renal arteries, mesentéricas, coeliac and iliacas. The aortografia with contrast is the most specific test and it is necessary to realize previously to a surgical intervention.
The replacement of the area with aneurisma as synthetic textile has a mortality of 5 %, but if it is necessary to realize of urgency this intervention the risk increases 25-50 %. For it if the aneurisma is a 5 cm minor, periodic control panel is realized and it is not taken control.
Medicines beta - bloqueantes are used to diminish the speed of progression of the aneurisma. The aneurisma disecante meets with more frequency in the Marfan syndrome and is diagnosed well by means of Ultrasonography after esofágica and ecodoppler to see the flows dynamics. Treatment in sharp Aneurisma disecante:
I medicate, to lower the arterial tension with nitroprusiato sódico, propanolol.
If there is hemo-pericardium it is necessary to realize the rapid aortografía to confirm the ascending dissection and to realize urgency surgery. If the cardiac mistake is imminent it is taken control before the aortografía. If the dissection is for both sides the intervention it must be realized not beyond 24 hours. The appearance of new aneurismas disecantes is 15 % if they are thoracic and of 40 % if they are lower than the diaphragm.
There is recommended an annual ecocardiograma and the prophylactic treatment with Betabloquantes if the diameter of the aorta is major than the normal thing. If it exceeds 6 cm of the normal thing it is necessary to intervene like precaution. It usually associate to a valve prolapse mitral. The pregnancy has a big risk of dissection of the abdominal aorta. The skill of intervention and other informations can see them in: |
